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Moore, MD" +breadcrumbs: + - + name: "Pediatrics" + slug: "pediatrics" + treeNodeId: "a915965c-d436-44cf-ae65-2f22e7246ea4" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "2b5cea64-a083-489e-ac0c-ec14ba059026" + - + name: "Pediatric Neuroradiology" + slug: "pediatric-neuroradiology" + treeNodeId: "d0eb8f4a-e769-43dd-896c-8c9c27ce8759" + - + name: "Brain" + slug: "brain" + treeNodeId: "feaaadba-649b-4f0a-9aad-9188a8f9926a" + - + name: "Pathology-Based Diagnoses" + slug: "pathology-based-diagnoses" + treeNodeId: "2d26053f-23a7-4062-bf35-a93775ae1209" + - + name: "Inflammatory and Demyelinating Disease" + slug: "inflammatory-and-demyelinating-dis-" + treeNodeId: "cb319228-da96-4d29-8276-c72388a57656" + - + name: "ADEM, Brain" + slug: "adem-brain" + treeNodeId: null +category: "Pediatrics" +documentVersionId: "d15d7c30-1933-4a0f-afc7-3e9b13ce1ff7" +imageCount: 26 +lastUpdated: "02/05/24" +pageDescription: "ADEM, Brain" +pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Inflammatory and Demyelinating Disease, ADEM, Brain" +pageTitle: "ADEM, Brain | STATdx" +enhancedTitle: "ADEM, Brain" +type: "DX" +references: true +breadcrumbs: + - "Pediatrics" + - "Diagnosis" + - "Pediatric Neuroradiology" + - "Brain" + - "Pathology-Based Diagnoses" + - "Inflammatory and Demyelinating Disease" + - "ADEM, Brain" +--- +# KEY FACTS + +- ## Terminology + + + - Autoimmune-mediated white matter (WM) demyelination of brain &/or spinal cord, usually with remyelination +- ## Imaging + + + - Best diagnostic clue: Multifocal WM and deep gray lesions days to weeks following infection/vaccination + - May involve both brain and spinal cord; WM > gray matter, but usually both affected + - Both supratentorial and infratentorial lesions + - Multifocal punctate to large, flocculent FLAIR hyperintensities + - Deep/juxtacortical WM > periventricular WM + - Do not usually involve callososeptal interface + - Most lesions show increased signal on DWI (T2 shine-through) + - Punctate, ring, incomplete ring, peripheral enhancement + - Absence of enhancement does not exclude diagnosis + - MRS: ↓ NAA within lesions; may see ↑ Cho, ↑ lactate +- ## Top Differential Diagnoses + + + - Multiple sclerosis (MS) + - Collagen-vascular disorders + - Neoplasms + - Hemophagocytic lymphohistiocytosis (HLH) + - Mitochondrial diseases + - Leukodystrophies + - Acute hypertensive encephalopathy, PRES + - Autoimmune-mediated vasculitis + - Fabry disease +- ## Pathology + + + - > 30 different infectious agents reported + - Anti-MOG IgG antibodies found more commonly in younger patients +- ## Clinical Issues + + + - Mean age: 5-8 years but can occur at any age + - Male predominance (M:F = 1:0.6-0.8), unlike MS + - Usually monophasic, self-limited + - Complete recovery within 1 month: 50-60% + - Mortality: 10-30% +- ## Diagnostic Checklist + + + - Imaging findings often lag behind symptom onset, resolution + +# TERMINOLOGY + +- ## Abbreviations + + + - Acute disseminated encephalomyelitis (ADEM) +- ## Definitions + + + - Autoimmune-mediated white matter (WM) demyelination of brain &/or spinal cord, usually with remyelination + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Multifocal WM/basal ganglia lesions days to weeks following preceding infection + - 93% within 3 weeks of infection + - ### Location + + + - Widespread WM and gray matter brain abnormalities + - Both supratentorial and infratentorial lesions + - Most conspicuous on T2-weighted and fluid-attenuated inversion recovery (FLAIR) sequences + - May involve both brain and spinal cord; WM > gray matter, but usually both affected + - Deep/juxtacortical WM > periventricular WM + - ### Size + + + - Tumefactive lesions may be large but with less mass effect than expected from similar size tumor + - ### Morphology + + + - Punctate to flocculent + - Tumefactive, mass-like lesions possible +- ## CT Findings + + + - ### NECT + + + - Initial CT normal in 40% of patients + - ### CECT + + + - Normal or multifocal punctate or ring-enhancing lesions +- ## MR Findings + + + - ### T1WI + + + - Hypointense "black holes" uncommon + - ### T2WI + + + - Hyperintensities may be better visualized in brainstem and posterior fossa than on T2 FLAIR + - ### FLAIR + + + - Widespread multifocal punctate to large, flocculent FLAIR hyperintensities + - Bilateral, asymmetric, frequently poorly marginated + - Involve peripheral WM-gray matter junction subcortical WM + - Thalami and basal ganglia symmetrically involved, particularly in children + - Can involve brainstem and posterior fossa + - Usually do **not** involve callososeptal interface + - ### DWI + + + - Variably hyperintense lesions on DWI (trace) images + - ADC may be increased or decreased + - Most lesions show increased signal (T2 shine-through) + - Diffusion restriction uncommon, suggests worse prognosis + - Diffusivity normal within normal-appearing WM (NAWM), unlike MS + - ### T1WI C+ + + + - Punctate, ring, incomplete ring, peripheral enhancement + - Cranial nerve(s) may enhance + - Absence of enhancement does not exclude ADEM + - ### MRS + + + - NAA low within lesions; lactate may be elevated + - Choline often elevated in acute lesions + - NAA normalizes with resolution of symptoms/MR abnormalities + - Magnetization transfer ratio (MTR) + - ADEM MTR normal within NAWM, unlike MS +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Contrast-enhanced brain MR, including FLAIR T2 imaging + - Initial imaging often normal but more sensitive than CT + - May appear identical to MS; repeat MR necessary to distinguish with certainty +- ## Nuclear Medicine Findings + + + - Tc-99m-HMPAO SPECT shows more extensive hypoperfusion than T2 lesions + +# DIFFERENTIAL DIAGNOSIS + +- [Multiple Sclerosis](/document/pediatric-multiple-sclerosis-brain/f2592b04-f800-4235-9eea-a43f2bf4adfe) + - Predilection for periventricular WM (callososeptal interface), subcortical U fibers, posterior fossa + - Lesions often more symmetric than ADEM + - Relapsing-remitting course common +- ## Collagen-Vascular Disorders + + + - e.g., systemic lupus erythematosus (SLE), Sjögren syndrome, and neuro-Behçet disease + - Can present with primarily CNS involvement in children + - Imaging findings mimic ADEM + - Elevated markers of systemic inflammation help distinguish + - Abnormal antinuclear antibody profile, positive double-stranded DNA antibodies, elevated angiotensin converting enzyme, antiphospholipid antibodies + - Behcet: Classic triad of oral and genital ulcerations with uveitis +- ## Neoplasms + + + - Mimic features of pediatric demyelinating processes + - Optic pathway neoplasms, primary brain tumors, intramedullary spinal cord tumors + - Specific imaging features and clinical history can help distinguish from pediatric demyelinating disorders +- ## Hemophagocytic Lymphohistiocytosis + + + - With primary CNS involvement can (rarely) precede systemic disease manifestations + - Mimics clinical presentation of ADEM + - Imaging manifestations are protean +- ## Mitochondrial Diseases + + + - MELAS and MERRF may present in setting of intercurrent acute infection + - Typical brain MR features include abnormal T2-hyperintense signal in basal ganglia and nonvascular distribution hemispheric lesions + - DWI MR will show acute restriction reflecting "metabolic stroke" + - Helps distinguish from nonrestricting demyelinating processes + - Elevation of serum, CSF lactate levels +- [Leukodystrophies](/document/leukodystrophies/f4ff3738-131c-46bf-be71-1811f2c1776c) + - Genetic WM diseases are typically symmetrical, sometimes with characteristic location or appearance + - e.g., metachromatic leukodystrophy, X-linked adrenoleukodystrophy, Alexander disease, globoid cell leukodystrophy (Krabbe disease) +- [Autoimmune-Mediated Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0) + - Multifocal gray matter-WM lesions + - Bilateral, usually cortical/subcortical, basal ganglia/thalami + - Ring-enhancing lesions may mimic infection +- [Acute Hypertensive Encephalopathy, PRES](/document/acute-hypertensive-encephalopathy--/efc6f9c2-dad9-4eb8-bad2-421bfaf1ec57) + - Typically posterior circulation in cortex/subcortical WM + - May affect deep gray nuclei +- [Aging Brain With Hyperintense White Matter Lesions](/document/normal-aging-brain/2a315550-b2ea-4afe-a2ef-f93a2209f276) + - Atherosclerotic brain changes in 50% patients > 50 years old + - Found in normotensive patients; more common in hypertensives + - Present in 10-30% of cognitively normal older adult patients + - MR: Scattered, asymmetric WM lesions, without enhancement + - Often periatrial; posterior fossa uncommon + - Spares callososeptal interface, subcortical U fibers +- [Fabry Disease](/document/fabry-disease/83fd222a-9b37-4087-afab-34ba74525887) + - Synonym: Angiokeratoma corporis diffusum universalis + - X-linked recessive; incidence 1/40,000 + - Deficiency α-galactosidase A; overaccumulation of glycosphingolipids within lysosomes + - MR: Scattered, asymmetric WM lesions without enhancement + - May involve brainstem and posterior fossa + - Spares callososeptal interface and subcortical U fibers + - Cranial MR sensitive to identify neurologic involvement in asymptomatic patients + - Present with renal failure/heart disease + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Autoimmune-mediated severe acute demyelination + - Pathogenesis incompletely understood, but thought to be triggered by environmental stimulus in genetically susceptible individuals + - Myelin autoantigens, such as myelin basic protein (MBP), proteolipid protein (PLP), and myelin oligodendrocyte glycoprotein (MOG), share antigenic determinants with those of infecting pathogen + - Often follows antecedent nonspecific upper respiratory tract infection + - > 30 different infectious agents and immunizations reported + - Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2 or COVID) infection has been reported with ADEM, but overall incidence appears to be low + - Some cases are spontaneous (no known cause) + - Neurologic symptoms typically appear 4-13 days after preceding infection + - Early reports suggested that minority of ADEM cases followed immunization + - Current studies show little or no association between ADEM and immunizations (postvaccinal ADEM) + - Implicated viral pathogens associated with ADEM are myriad and include coronavirus, coxsackievirus, cytomegalovirus, Epstein-Barr, herpes simplex, hepatitis A, HIV, influenza, measles, rubella, varicella zoster, and West Nile viruses + - Bacterial organisms associated with ADEM include *Borrelia burgdorferi*, *Chlamydia trachomatis*, *Leptospira*, *Mycoplasma pneumoniae*,*rickettsia*, and beta hemolytic *Streptococcus* + - ### Genetics + + + - ADEM associated with DRB1*01 and DRB1*017(03) in Russian population + - Acute hemorrhagic leukoencephalopathy variant associated with ulcerative colitis and asthma + - Anti-MOG IgG antibodies found more commonly in younger patients + - Most cases probably reflect MOGAD +- ## Gross Pathologic & Surgical Features + + + - None, unless hemorrhage (rare) or tumefactive edema +- ## Microscopic Features + + + - Acute myelin breakdown + - Perivenous inflammation; lymphocytic infiltrates + - Relative axonal preservation; atypical astrogliosis + - Virus particles generally not found, unlike viral encephalitides + - Similar to experimental allergic encephalomyelitis, supporting autoimmune-related etiology + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Usually preceded by prodromal phase: Fever, malaise, myalgia + - Multifocal neurologic symptoms, 2 days to 4 weeks after viral illness + - Initial symptoms: Headache, fever, drowsiness + - Cranial nerve palsies, hemiparesis + - Decreased consciousness (from lethargy to coma) + - Behavioral changes + - ### Other signs/symptoms + + + - Seizures in 10-35% + - ### Clinical profile + + + - CSF normal in 60% + - If abnormal (lymphocyte pleocytosis, elevated protein) usually lacks CSF oligoclonal bands +- ## Demographics + + + - ### Age + + + - Children > adults + - Mean age: 5-8 years but can occur at any age + - ### Sex + + + - Male predominance (M:F = 1.0:0.6-0.8), unlike MS + - ### Epidemiology + + + - Pediatric ADEM is uncommon illness + - Estimated annual incidence 0.2-0.5 per 100,000 children + - Typically delay between symptom onset, imaging findings + - Rare, yet most common para-/postinfectious disorder + - Most common in winter and spring + - Exact epidemiology unknown but increasingly reported +- ## Natural History & Prognosis + + + - Usually monophasic and self-limited + - Variable prognosis + - Complete recovery within 1 month (50-60%) + - Neurologic sequelae (most commonly seizures) (20-30%) + - Mortality (10-30%) + - Relapses are rare + - "Relapsing disseminated encephalomyelitis" + - Many of these cases may actually be MOG antibody disease (MOGAD) + - May be difficult to distinguish from relapsing-remitting MS + - Varicella and rubella ADEM have preferential patterns + - Varicella ADEM characterized by cerebellar ataxia and mild pyramidal dysfunction + - Rubella ADEM characterized by acute explosive onset, seizures, coma, and moderate pyramidal signs + - Rare manifestations of ADEM + - Acute hemorrhagic leukoencephalopathy (2%) + - Young patients with abrupt symptom onset and fulminant clinical course + - Inflammatory hemorrhagic WM demyelination + - These variants are more rapidly progressive and clinically severe than typical ADEM + - Otherwise, their symptomatology is similar to typical ADEM with meningismus, headache, seizures, multifocal and asymmetric neurologic deficits, &/or coma + - Prognosis for survival or recovery of neurologic function is worse than for classic ADEM + - Bilateral striatal necrosis (usually in infants, may be reversible) +- ## Treatment + + + - Immunosuppressive/immunomodulatory therapy + - MR may show prompt improvement after therapy + - Plasma exchange therapy + - 40% of patients failing steroid treatment may show marked improvement + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - Imaging findings often lag behind both symptom onset and resolution + + 31576fd8-140b-43b2-9f58-b6f33ef20ca3 + +## References + +# Selected References + +1. [Kalita IR et al: Acute abducens nerve palsy with acute disseminated encephalomyelitis-like presentation following COVID-19 vaccination. Indian J Ophthalmol. 71(5):2279-81, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37202971%5Bpmid%5D) +1. [Chen LW et al: Prognostic factors for functional recovery in children with moderate to severe acute disseminated encephalomyelitis. Mult Scler Relat Disord. 66:104056, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35878513%5Bpmid%5D) +1. [Wang Y et al: SARS-CoV-2-associated acute disseminated encephalomyelitis: a systematic review of the literature. J Neurol. 269(3):1071-92, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=34459986%5Bpmid%5D) +1. [Benallegue N et al: Neurological involvement in secondary hemophagocytic lymphohistiocytosis in children. Eur J Paediatr Neurol. 34:110-7, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34482125%5Bpmid%5D) +1. [Pujari SS et al: Acute haemorrhagic leukoencephalitis (AHLE) - our experience and a short review. J Neuroimmunol. 361:577751, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34739912%5Bpmid%5D) +1. [Baumann M et al: MRI of the first event in pediatric acquired demyelinating syndromes with antibodies to myelin oligodendrocyte glycoprotein. J Neurol. 265(4):845-55, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29423614%5Bpmid%5D) +1. [Aubert-Broche B et al: Monophasic demyelination reduces brain growth in children. Neurology. 88(18):1744-50, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28381515%5Bpmid%5D) +1. [Bester M et al: Neuroimaging of multiple sclerosis, acute disseminated encephalomyelitis, and other demyelinating diseases. Semin Roentgenol. 49(1):76-85, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24342677%5Bpmid%5D) +1. [Longoni G et al: White matter changes in paediatric multiple sclerosis and monophasic demyelinating disorders. Brain. 140(5):1300-15, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28334875%5Bpmid%5D) +1. [Koelman DLH et al: Acute disseminated encephalomyelitis: prognostic value of early follow-up brain MRI. J Neurol. 264(8):1754-62, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28695361%5Bpmid%5D) +1. [Marziali S et al: Acute disseminated encephalomyelitis following Campylobacter jejuni gastroenteritis: Case report and review of the literature. Neuroradiol J. 30(1):65-70, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27888275%5Bpmid%5D) +1. [Kanekar S et al: A pattern approach to focal white matter hyperintensities on magnetic resonance imaging. Radiol Clin North Am. 52(2):241-61, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24582339%5Bpmid%5D) +1. [Daida K et al: Cytomegalovirus-associated encephalomyelitis in an immunocompetent adult: a two-stage attack of direct viral and delayed immune-mediated invasions. case report. BMC Neurol. 16(1):223, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27855658%5Bpmid%5D) +1. [Pohl D et al: Acute disseminated encephalomyelitis: updates on an inflammatory CNS syndrome. Neurology. 87(9 Suppl 2):S38-45, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27572859%5Bpmid%5D) +1. [Yuan JL et al: Acute disseminated encephalomyelitis following vaccination against hepatitis B in a child: a case report and literature review. Case Rep Neurol Med. 2016:2401809, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27478662%5Bpmid%5D) +1. [Baumann M et al: Clinical and neuroradiological differences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein. J Neurol Neurosurg Psychiatry. 86(3):265-72, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25121570%5Bpmid%5D) +1. [Karussis D: The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review. J Autoimmun. 48-49:134-42, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24524923%5Bpmid%5D) +1. [Mariotto S et al: Clinical spectrum and IgG subclass analysis of anti-myelin oligodendrocyte glycoprotein antibody-associated syndromes: a multicenter study. J Neurol. 264(12):2420-30, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29063242%5Bpmid%5D) +1. [Nakamura Y et al: Anti-MOG antibody-positive ADEM following infectious mononucleosis due to a primary EBV infection: a case report. BMC Neurol. 17(1):76, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28420330%5Bpmid%5D) +1. [Tenembaum SN: Acute disseminated encephalomyelitis. Handb Clin Neurol. 112:1253-62, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23622336%5Bpmid%5D) +1. [Wingerchuk DM et al: Acute disseminated encephalomyelitis, transverse myelitis, and neuromyelitis optica. Continuum (Minneap Minn). 19(4 Multiple Sclerosis):944-67, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23917095%5Bpmid%5D) +1. [Callen DJ et al: Role of MRI in the differentiation of ADEM from MS in children. Neurology. 72(11):968-73, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19038851%5Bpmid%5D) +1. [Noorbakhsh F et al: Acute disseminated encephalomyelitis: clinical and pathogenesis features. Neurol Clin. 26(3):759-80, ix, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18657725%5Bpmid%5D) +1. [Rossi A: Imaging of acute disseminated encephalomyelitis. Neuroimaging Clin N Am. 18(1):149-61; ix, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18319160%5Bpmid%5D) +1. [Tenembaum S et al: Acute disseminated encephalomyelitis. Neurology. 68(16 Suppl 2):S23-36, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17438235%5Bpmid%5D) +1. [Menge T et al: Acute disseminated encephalomyelitis: an update. Arch Neurol. 62(11):1673-80, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16286539%5Bpmid%5D) +1. [Yeh EA et al: Detection of coronavirus in the central nervous system of a child with acute disseminated encephalomyelitis. Pediatrics. 113(1 Pt 1):e73-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14702500%5Bpmid%5D) +1. [Dale RC: Acute disseminated encephalomyelitis. Semin Pediatr Infect Dis. 14(2):90-5, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12881796%5Bpmid%5D) +1. [Garg RK: Acute disseminated encephalomyelitis. Postgrad Med J. 79(927):11-17, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12566545%5Bpmid%5D) +1. [Idrissova ZhR et al: Acute disseminated encephalomyelitis in children: clinical features and HLA-DR linkage. Eur J Neurol. 10(5):537-46, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12940836%5Bpmid%5D) +1. [Okamoto K et al: MR features of diseases involving bilateral middle cerebellar peduncles. AJNR Am J Neuroradiol. 24(10):1946-54, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14625215%5Bpmid%5D) +1. [Sener RN: Neuro-Behcet's disease: diffusion MR imaging and proton MR spectroscopy. AJNR Am J Neuroradiol. 24(8):1612-4, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=13679280%5Bpmid%5D) +1. [Stonehouse M et al: Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians. Arch Dis Child. 88(2):122-4, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12538312%5Bpmid%5D) +1. [Inglese M et al: Magnetization transfer and diffusion tensor MR imaging of acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 23(2):267-72, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11847052%5Bpmid%5D) +1. [Murthy JM: Acute disseminated encephalomyelitis. Neurol India. 50(3):238-43, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12391446%5Bpmid%5D) +1. [Tenembaum S et al: Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology. 59(8):1224-31, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12391351%5Bpmid%5D) +1. [Bizzi A et al: Quantitative proton MR spectroscopic imaging in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 22(6):1125-30, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11415908%5Bpmid%5D) +1. [Honkaniemi J et al: Delayed MR imaging changes in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 22(6):1117-24, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11415907%5Bpmid%5D) +1. [Straussberg R et al: Improvement of atypical acute disseminated encephalomyelitis with steroids and intravenous immunoglobulins. Pediatr Neurol. 24(2):139-43, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11275464%5Bpmid%5D) +1. [Dale RC et al: Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain. 123 Pt 12:2407-22, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11099444%5Bpmid%5D) +1. [Rust RS: Multiple sclerosis, acute disseminated encephalomyelitis, and related conditions. Semin Pediatr Neurol. 7(2):66-90, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10914409%5Bpmid%5D) +1. [Schaefer PW et al: Diffusion-weighted MR imaging of the brain. Radiology. 217(2):331-45, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11058626%5Bpmid%5D) +1. [Kocer N et al: CNS involvement in neuro-Behcet syndrome: an MR study. AJNR Am J Neuroradiol. 20(6):1015-24, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10445437%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial FLAIR MR in a child with ADEM shows peripheral, confluent areas of hyperintensity predominantly involving subcortical white matter. Bilateral but asymmetric pattern is typical of ADEM.](images/app.statdx.com_image_thumbnail_0089d6c8-c46e-47c9-a72d-9b6ee9284d04_annotated_true_size_900_quality_90_69c8127b_20251018T152316Z.jpg) +*Axial FLAIR MR in a child with ADEM shows peripheral, confluent areas of hyperintensity predominantly involving subcortical white matter. Bilateral but asymmetric pattern is typical of ADEM.* + +![Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of most lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis lesions is more variable.](images/app.statdx.com_image_thumbnail_64ffcf26-7ca7-4104-bfa4-b6548f582028_annotated_true_size_900_quality_90_04a7a3f4_20251018T152315Z.jpg) +*Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of most lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis lesions is more variable.* + +![Axial FLAIR MR in a 5-year-old with ADEM following an EBV infection demonstrates more subtle findings with abnormal hyperintensity in the left basal ganglia and right parietal cortex/subcortical white matter .](images/app.statdx.com_image_thumbnail_903a6a28-4877-4eb7-801d-dee46bf162bd_annotated_true_size_900_quality_90_85ce8fb7_20251018T152316Z.jpg) +*Axial FLAIR MR in a 5-year-old with ADEM following an EBV infection demonstrates more subtle findings with abnormal hyperintensity in the left basal ganglia and right parietal cortex/subcortical white matter .* + +![Axial T1 C+ MR in the same patient reveals no abnormal lesional contrast enhancement.](images/app.statdx.com_image_thumbnail_6ec7cb98-de90-4247-963e-31be1fa7ee52_annotated_true_size_900_quality_90_7360436d_20251018T152315Z.jpg) +*Axial T1 C+ MR in the same patient reveals no abnormal lesional contrast enhancement.* + +![Axial T2 MR in a 21-month-old who presented with seizures and encephalopathy demonstrates numerous lesions involving both subcortical white matter and gray matter (caudate nuclei, left basal ganglia, both thalami).](images/app.statdx.com_image_thumbnail_fb52c1ce-8553-441d-a1cb-c49fe6580667_annotated_true_size_900_quality_90_dc29ce16_20251018T152316Z.jpg) +*Axial T2 MR in a 21-month-old who presented with seizures and encephalopathy demonstrates numerous lesions involving both subcortical white matter and gray matter (caudate nuclei, left basal ganglia, both thalami).* + +![Coronal FLAIR MR in the same patient confirms characteristic lesion distribution. Contrast-enhanced imaging (not shown) demonstrated no lesional enhancement.](images/app.statdx.com_image_thumbnail_1e485e05-a9d9-4ec8-9ff1-359d53745e1c_annotated_true_size_900_quality_90_18d4794b_20251018T152315Z.jpg) +*Coronal FLAIR MR in the same patient confirms characteristic lesion distribution. Contrast-enhanced imaging (not shown) demonstrated no lesional enhancement.* + +![Axial T2WI MR shows hyperintense lesions in the brachium pontis bilaterally, typical for demyelination. The right-sided lesion shows a targetoid appearance. Enhancement of several lesions was present on postcontrast T1 images (not shown).](images/app.statdx.com_image_thumbnail_18b81bfe-32d0-46ae-9261-89c5ac6de92e_annotated_true_size_900_quality_90_67a641ed_20251018T152316Z.jpg) +*Axial T2WI MR shows hyperintense lesions in the brachium pontis bilaterally, typical for demyelination. The right-sided lesion shows a targetoid appearance. Enhancement of several lesions was present on postcontrast T1 images (not shown).* + +![Axial FLAIR MR shows large, confluent regions of hyperintense signal in the periventricular and subcortical white matter in a 14-year-old who presented with neck stiffness, fatigue, and seizures.](images/app.statdx.com_image_thumbnail_9d547a6a-7b9d-43b8-b2bf-3cb459f98fe8_annotated_true_size_900_quality_90_04b3f6ff_20251018T152315Z.jpg) +*Axial FLAIR MR shows large, confluent regions of hyperintense signal in the periventricular and subcortical white matter in a 14-year-old who presented with neck stiffness, fatigue, and seizures.* + +![Axial SWI MR in the same patient shows petechial hemorrhages in regions of FLAIR signal abnormality.](images/app.statdx.com_image_thumbnail_c88bfbb2-5923-4952-8c80-65ac436e467b_annotated_true_size_900_quality_90_68ef6089_20251018T152316Z.jpg) +*Axial SWI MR in the same patient shows petechial hemorrhages in regions of FLAIR signal abnormality.* + +![Sagittal T1 C+ MR in same patient shows extensive irregular ring enhancement involving multiple subcortical white matter lesions. Acute hemorrhagic leukoencephalopathy (AHL) is a rare manifestation of ADEM, occurring in 2% of cases. AHL is associated with a very poor prognosis. Aggressive therapeutic management is a prerequisite to avoid usual disease course with fatal outcome.](images/app.statdx.com_image_thumbnail_64cb6152-89e6-4027-b778-5d559cb3efeb_annotated_true_size_900_quality_90_a908aeb7_20251018T152321Z.jpg) +*Sagittal T1 C+ MR in same patient shows extensive irregular ring enhancement involving multiple subcortical white matter lesions. Acute hemorrhagic leukoencephalopathy (AHL) is a rare manifestation of ADEM, occurring in 2% of cases. AHL is associated with a very poor prognosis. Aggressive therapeutic management is a prerequisite to avoid usual disease course with fatal outcome.* + + +### Additional Images + +![Axial T1WI C+ MR shows an incomplete ring of peripheral enhancement, typical of a demyelinating process. Other contrast enhancement patterns include ovoid or punctate homogeneous enhancement.](images/app.statdx.com_image_thumbnail_1c49d689-a60d-45a3-8c9b-52aa69ea64be_annotated_true_size_900_quality_90_e7866374_20251018T152321Z.jpg) +*Axial T1WI C+ MR shows an incomplete ring of peripheral enhancement, typical of a demyelinating process. Other contrast enhancement patterns include ovoid or punctate homogeneous enhancement.* + +![Axial DWI MR shows increased signal in areas of FLAIR hyperintensity (not shown). The foci were hypointense on ADC images, indicating diffusion restriction. Both white matter and gray matter involvement is present. Diffusion restriction is an uncommon imaging finding and is associated with a worse prognosis.](images/app.statdx.com_image_thumbnail_68ac872b-ffc7-45df-944b-c92acff8b7cb_annotated_true_size_900_quality_90_bb79bfc6_20251018T152321Z.jpg) +*Axial DWI MR shows increased signal in areas of FLAIR hyperintensity (not shown). The foci were hypointense on ADC images, indicating diffusion restriction. Both white matter and gray matter involvement is present. Diffusion restriction is an uncommon imaging finding and is associated with a worse prognosis.* + +![Coronal T2WI MR shows large, confluent regions of hyperintense signal in the white matter and deep gray nuclei of a child with ADEM. Although ADEM predominantly involves white matter, gray matter is often affected.](images/app.statdx.com_image_thumbnail_4c698420-da7a-4ef8-a9a5-486dae2fb2ad_annotated_true_size_900_quality_90_b8fc7613_20251018T152322Z.jpg) +*Coronal T2WI MR shows large, confluent regions of hyperintense signal in the white matter and deep gray nuclei of a child with ADEM. Although ADEM predominantly involves white matter, gray matter is often affected.* + +![MRS at long echo time (TE) in a patient with acute lesions in ADEM demonstrates an ↑ choline , ↓ NAA , and the presence of a lactate doublet . Increase in choline with corresponding reductions in NAA normalize as the clinical and conventional neuroimaging abnormalities resolve.](images/app.statdx.com_image_thumbnail_d84ff7a6-ae85-4c5d-b939-1711e1a097ef_annotated_true_size_900_quality_90_fdcfb7a4_20251018T152322Z.jpg) +*MRS at long echo time (TE) in a patient with acute lesions in ADEM demonstrates an ↑ choline , ↓ NAA , and the presence of a lactate doublet . Increase in choline with corresponding reductions in NAA normalize as the clinical and conventional neuroimaging abnormalities resolve.* + +![Axial FLAIR MR shows bilateral, multiple asymmetric, flocculent, hyperintense lesions of ADEM.](images/app.statdx.com_image_thumbnail_ec8663a5-52f2-4149-8d68-5f8c603f1b79_annotated_true_size_900_quality_90_4d798e7a_20251018T152322Z.jpg) +*Axial FLAIR MR shows bilateral, multiple asymmetric, flocculent, hyperintense lesions of ADEM.* + +![Coronal T1WI C+ MR demonstrates partial peripheral enhancement around multiple asymmetric flocculent lesions of acute disseminated encephalomyelitis. Note the supra- and infratentorial lesions.](images/app.statdx.com_image_thumbnail_4068f5f5-1f2b-4355-837a-8b17a894bdca_annotated_true_size_900_quality_90_24f28941_20251018T152323Z.jpg) +*Coronal T1WI C+ MR demonstrates partial peripheral enhancement around multiple asymmetric flocculent lesions of acute disseminated encephalomyelitis. Note the supra- and infratentorial lesions.* + +![Axial FLAIR MR shows asymmetric, flocculent, nearly confluent, hyperintense lesions of ADEM within posterior fossa structures.](fed48635-3311-4ff4-ae2c-68e8330b75ed) +*Axial FLAIR MR shows asymmetric, flocculent, nearly confluent, hyperintense lesions of ADEM within posterior fossa structures.* + +![Axial FLAIR MR reveals multiple asymmetric, primarily punctate, hyperintense lesions of ADEM.](f35db971-737f-4cce-afba-e70659b67143) +*Axial FLAIR MR reveals multiple asymmetric, primarily punctate, hyperintense lesions of ADEM.* + +![Axial FLAIR MR demonstrates a large, tumefactive, hyperintense lesion. Less mass effect is present than expected for lesion size. Smaller lesions were also present at other locations.](afe5abf8-901e-47eb-90f1-68c8271cf4ab) +*Axial FLAIR MR demonstrates a large, tumefactive, hyperintense lesion. Less mass effect is present than expected for lesion size. Smaller lesions were also present at other locations.* + +![Axial T1WI C+ MR in the same patient demonstrates a large, tumefactive, hypointense lesion with minimal partial peripheral enhancement. Less mass effect is present than expected for lesion size. More lesions were seen elsewhere.](1855f77d-0e73-47ea-a0c1-5db86dc083e7) +*Axial T1WI C+ MR in the same patient demonstrates a large, tumefactive, hypointense lesion with minimal partial peripheral enhancement. Less mass effect is present than expected for lesion size. More lesions were seen elsewhere.* + +![Axial FLAIR MR demonstrates a rare manifestation of ADEM: Bilateral striatal necrosis, evidenced by asymmetric confluent hyperintensity involving the gray matter and white matter of bilateral corpus striatum.](5c6dc644-142a-4303-afb7-09c16c07363a) +*Axial FLAIR MR demonstrates a rare manifestation of ADEM: Bilateral striatal necrosis, evidenced by asymmetric confluent hyperintensity involving the gray matter and white matter of bilateral corpus striatum.* + +![Axial DWI MR confirms the rare manifestation of ADEM, displaying bilateral striatal necrosis, as evidenced by asymmetric confluent restricted diffusion involving gray matter and white matter of bilateral corpus striatum.](f0e2bac7-bbc9-45da-8e3f-ddd9151ed43a) +*Axial DWI MR confirms the rare manifestation of ADEM, displaying bilateral striatal necrosis, as evidenced by asymmetric confluent restricted diffusion involving gray matter and white matter of bilateral corpus striatum.* + +![Axial T2WI MR shows multiple bilateral but asymmetric, T2-hyperintense foci . None of the lesions demonstrates significant mass effect in this adult patient with ADEM. Imaging mimics multiple sclerosis, vasculitis, and microvascular ischemia.](36c82d94-ffcc-4bee-8ea6-91d4e8368bb8) +*Axial T2WI MR shows multiple bilateral but asymmetric, T2-hyperintense foci . None of the lesions demonstrates significant mass effect in this adult patient with ADEM. Imaging mimics multiple sclerosis, vasculitis, and microvascular ischemia.* + +![Axial FLAIR MR shows a large, tumefactive, hyperintense ADEM lesion with mass effect less than expected for the size of the lesion. Another clue to its nonneoplastic nature is the right-sided lesion .](84bb9f5f-d05b-4391-bd16-8beea63e1fbb) +*Axial FLAIR MR shows a large, tumefactive, hyperintense ADEM lesion with mass effect less than expected for the size of the lesion. Another clue to its nonneoplastic nature is the right-sided lesion .* + +![MRS at a long TE in the same patient shows the tumefactive lesion has a depressed choline and NAA metabolites in the presence of a large lactate doublet . This MRS helps distinguish this lesion from a neoplasm. MRS of ADEM may show elevated choline acutely.](8d4a240b-4ac5-4002-86f6-76f8dd742664) +*MRS at a long TE in the same patient shows the tumefactive lesion has a depressed choline and NAA metabolites in the presence of a large lactate doublet . This MRS helps distinguish this lesion from a neoplasm. MRS of ADEM may show elevated choline acutely.* + +![Axial FLAIR MR shows typical findings of ADEM with peripheral, subcortical hyperintense foci . Bilateral insular involvement is seen . Periventricular and callososeptal lesions, which are typical of multiple sclerosis, are not commonly seen in ADEM.](261b8d47-c109-457f-b28e-2151acebd773) +*Axial FLAIR MR shows typical findings of ADEM with peripheral, subcortical hyperintense foci . Bilateral insular involvement is seen . Periventricular and callososeptal lesions, which are typical of multiple sclerosis, are not commonly seen in ADEM.* + diff --git a/docs_md/articles/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md b/docs_md/articles/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md index f21352a..18ecf4f 100644 --- a/docs_md/articles/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md +++ b/docs_md/articles/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md @@ -403,57 +403,33 @@ breadcrumbs: ### Selected Images -![Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.](images/app.statdx.com_image_deba3310-8e09-466c-97d2-1e6bccb28edf_dafbadc1_20251018T064947Z.jpg) -*Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.* - ![Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.](images/app.statdx.com_image_thumbnail_deba3310-8e09-466c-97d2-1e6bccb28edf_size_168_quality_85_3dfef960_20251018T064936Z.jpg) *Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.* -![Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of the majority of lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis (MS) lesions is more variable.](images/app.statdx.com_image_e18458d3-5f6e-43fd-ab6d-72c7a86dca92_0f3893d6_20251018T064949Z.jpg) -*Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of the majority of lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis (MS) lesions is more variable.* +![Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.](images/app.statdx.com_image_thumbnail_deba3310-8e09-466c-97d2-1e6bccb28edf_size_174_quality_85_61e31a0b_20251018T152348Z.jpg) +*Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.* ![Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of the majority of lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis (MS) lesions is more variable.](images/app.statdx.com_image_thumbnail_e18458d3-5f6e-43fd-ab6d-72c7a86dca92_size_168_quality_85_70272c40_20251018T064936Z.jpg) *Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of the majority of lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis (MS) lesions is more variable.* -![Axial T1 C+ MR shows an incomplete ring of peripheral enhancement, typical of a demyelinating process. Other contrast enhancement patterns include ovoid or punctate homogeneous enhancement.](images/app.statdx.com_image_53135c0f-4c9e-4fb9-ab09-df46c5b40f70_c986514a_20251018T064950Z.jpg) -*Axial T1 C+ MR shows an incomplete ring of peripheral enhancement, typical of a demyelinating process. Other contrast enhancement patterns include ovoid or punctate homogeneous enhancement.* - ![Axial T1 C+ MR shows an incomplete ring of peripheral enhancement, typical of a demyelinating process. Other contrast enhancement patterns include ovoid or punctate homogeneous enhancement.](images/app.statdx.com_image_thumbnail_53135c0f-4c9e-4fb9-ab09-df46c5b40f70_size_168_quality_85_3f185a62_20251018T064936Z.jpg) *Axial T1 C+ MR shows an incomplete ring of peripheral enhancement, typical of a demyelinating process. Other contrast enhancement patterns include ovoid or punctate homogeneous enhancement.* -![Axial DWI MR shows increased signal in areas of FLAIR hyperintensity. The foci were hypointense on ADC images, indicating diffusion restriction. Both WM and gray matter involvement is present. Diffusion restriction is an uncommon imaging finding and is associated with a worse prognosis.](images/app.statdx.com_image_b79995c4-6bff-49f1-af71-cb56fe4b4aa1_119a9c33_20251018T064951Z.jpg) -*Axial DWI MR shows increased signal in areas of FLAIR hyperintensity. The foci were hypointense on ADC images, indicating diffusion restriction. Both WM and gray matter involvement is present. Diffusion restriction is an uncommon imaging finding and is associated with a worse prognosis.* - ![Axial DWI MR shows increased signal in areas of FLAIR hyperintensity. The foci were hypointense on ADC images, indicating diffusion restriction. Both WM and gray matter involvement is present. Diffusion restriction is an uncommon imaging finding and is associated with a worse prognosis.](images/app.statdx.com_image_thumbnail_b79995c4-6bff-49f1-af71-cb56fe4b4aa1_size_168_quality_85_03275787_20251018T064936Z.jpg) *Axial DWI MR shows increased signal in areas of FLAIR hyperintensity. The foci were hypointense on ADC images, indicating diffusion restriction. Both WM and gray matter involvement is present. Diffusion restriction is an uncommon imaging finding and is associated with a worse prognosis.* -![Axial T2 MR shows hyperintense lesions in the brachium pontis bilaterally, typical for demyelination. The right-sided lesion shows a targetoid appearance. Enhancement of several lesions was present on postcontrast T1 images (not shown).](images/app.statdx.com_image_f74d958e-f845-47e7-884e-331bf3dcb299_89b1781b_20251018T064952Z.jpg) -*Axial T2 MR shows hyperintense lesions in the brachium pontis bilaterally, typical for demyelination. The right-sided lesion shows a targetoid appearance. Enhancement of several lesions was present on postcontrast T1 images (not shown).* - ![Axial T2 MR shows hyperintense lesions in the brachium pontis bilaterally, typical for demyelination. The right-sided lesion shows a targetoid appearance. Enhancement of several lesions was present on postcontrast T1 images (not shown).](images/app.statdx.com_image_thumbnail_f74d958e-f845-47e7-884e-331bf3dcb299_size_168_quality_85_10b7c767_20251018T064936Z.jpg) *Axial T2 MR shows hyperintense lesions in the brachium pontis bilaterally, typical for demyelination. The right-sided lesion shows a targetoid appearance. Enhancement of several lesions was present on postcontrast T1 images (not shown).* -![Axial FLAIR MR shows large, confluent regions of hyperintense signal in the periventricular and subcortical WM in a 14 year old who presented with neck stiffness, fatigue, and seizures.](images/app.statdx.com_image_3e8a9c1e-560e-4c5c-93bc-dda9b3773622_eda9eb67_20251018T064954Z.jpg) -*Axial FLAIR MR shows large, confluent regions of hyperintense signal in the periventricular and subcortical WM in a 14 year old who presented with neck stiffness, fatigue, and seizures.* - ![Axial FLAIR MR shows large, confluent regions of hyperintense signal in the periventricular and subcortical WM in a 14 year old who presented with neck stiffness, fatigue, and seizures.](images/app.statdx.com_image_thumbnail_3e8a9c1e-560e-4c5c-93bc-dda9b3773622_size_168_quality_85_caf40da9_20251018T064936Z.jpg) *Axial FLAIR MR shows large, confluent regions of hyperintense signal in the periventricular and subcortical WM in a 14 year old who presented with neck stiffness, fatigue, and seizures.* -![Axial SWI MR in the same patient shows petechial hemorrhages in regions of FLAIR signal abnormality.](images/app.statdx.com_image_fa96c2d2-8153-44c2-bd76-be0296382093_e2505a5f_20251018T064955Z.jpg) -*Axial SWI MR in the same patient shows petechial hemorrhages in regions of FLAIR signal abnormality.* - ![Axial SWI MR in the same patient shows petechial hemorrhages in regions of FLAIR signal abnormality.](images/app.statdx.com_image_thumbnail_fa96c2d2-8153-44c2-bd76-be0296382093_size_168_quality_85_93dd4c72_20251018T064936Z.jpg) *Axial SWI MR in the same patient shows petechial hemorrhages in regions of FLAIR signal abnormality.* -![Sagittal T1 C+ MR in the same patient shows extensive irregular ring enhancement in multiple subcortical WM lesions. Acute hemorrhagic leukoencephalopathy (AHL) is a rare manifestation of ADEM occurring in 2% of cases. AHL is associated with a very poor prognosis. Aggressive therapeutic management is a prerequisite to avoid usual disease course with fatal outcome.](images/app.statdx.com_image_b9452b6b-6772-4d91-8ca4-47ceaafa25f4_ddfedaec_20251018T064957Z.jpg) -*Sagittal T1 C+ MR in the same patient shows extensive irregular ring enhancement in multiple subcortical WM lesions. Acute hemorrhagic leukoencephalopathy (AHL) is a rare manifestation of ADEM occurring in 2% of cases. AHL is associated with a very poor prognosis. Aggressive therapeutic management is a prerequisite to avoid usual disease course with fatal outcome.* - ![Sagittal T1 C+ MR in the same patient shows extensive irregular ring enhancement in multiple subcortical WM lesions. Acute hemorrhagic leukoencephalopathy (AHL) is a rare manifestation of ADEM occurring in 2% of cases. AHL is associated with a very poor prognosis. Aggressive therapeutic management is a prerequisite to avoid usual disease course with fatal outcome.](images/app.statdx.com_image_thumbnail_b9452b6b-6772-4d91-8ca4-47ceaafa25f4_size_168_quality_85_3dcd4458_20251018T064936Z.jpg) *Sagittal T1 C+ MR in the same patient shows extensive irregular ring enhancement in multiple subcortical WM lesions. Acute hemorrhagic leukoencephalopathy (AHL) is a rare manifestation of ADEM occurring in 2% of cases. AHL is associated with a very poor prognosis. Aggressive therapeutic management is a prerequisite to avoid usual disease course with fatal outcome.* -![Coronal T2 MR shows large, confluent regions of hyperintense signal in the WM and deep gray nuclei of a child with ADEM. Although ADEM predominantly involves WM, gray matter is often affected.](images/app.statdx.com_image_113e918b-8d8f-46b8-8664-e0f617904251_b9bf7b86_20251018T064958Z.jpg) -*Coronal T2 MR shows large, confluent regions of hyperintense signal in the WM and deep gray nuclei of a child with ADEM. Although ADEM predominantly involves WM, gray matter is often affected.* - ![Coronal T2 MR shows large, confluent regions of hyperintense signal in the WM and deep gray nuclei of a child with ADEM. Although ADEM predominantly involves WM, gray matter is often affected.](images/app.statdx.com_image_thumbnail_113e918b-8d8f-46b8-8664-e0f617904251_size_168_quality_85_1b06d2b7_20251018T064936Z.jpg) *Coronal T2 MR shows large, confluent regions of hyperintense signal in the WM and deep gray nuclei of a child with ADEM. Although ADEM predominantly involves WM, gray matter is often affected.* @@ -466,15 +442,9 @@ breadcrumbs: ### Additional Images -![Axial FLAIR MR shows multiple bilateral, asymmetric, flocculent, hyperintense lesions of acute disseminated encephalomyelitis.](images/app.statdx.com_image_bac2d338-fffe-48e4-be97-bb8258970076_425b9699_20251018T065002Z.jpg) -*Axial FLAIR MR shows multiple bilateral, asymmetric, flocculent, hyperintense lesions of acute disseminated encephalomyelitis.* - ![Axial FLAIR MR shows multiple bilateral, asymmetric, flocculent, hyperintense lesions of acute disseminated encephalomyelitis.](images/app.statdx.com_image_thumbnail_bac2d338-fffe-48e4-be97-bb8258970076_size_168_quality_85_4b79e54c_20251018T064936Z.jpg) *Axial FLAIR MR shows multiple bilateral, asymmetric, flocculent, hyperintense lesions of acute disseminated encephalomyelitis.* -![Coronal T1 C+ MR demonstrates partial peripheral enhancement around multiple asymmetric, flocculent lesions of acute disseminated encephalomyelitis. Note the supra- and infratentorial lesions.](images/app.statdx.com_image_674fcfe9-2db8-4d36-a55c-9ca0a0672252_4fcbd413_20251018T065003Z.jpg) -*Coronal T1 C+ MR demonstrates partial peripheral enhancement around multiple asymmetric, flocculent lesions of acute disseminated encephalomyelitis. Note the supra- and infratentorial lesions.* - ![Coronal T1 C+ MR demonstrates partial peripheral enhancement around multiple asymmetric, flocculent lesions of acute disseminated encephalomyelitis. Note the supra- and infratentorial lesions.](images/app.statdx.com_image_thumbnail_674fcfe9-2db8-4d36-a55c-9ca0a0672252_size_168_quality_85_05b2865d_20251018T064936Z.jpg) *Coronal T1 C+ MR demonstrates partial peripheral enhancement around multiple asymmetric, flocculent lesions of acute disseminated encephalomyelitis. Note the supra- and infratentorial lesions.* diff --git a/docs_md/articles/aqueductal-stenosis_6dfa6261-3945-4606-850b-51484d05e70c.md b/docs_md/articles/aqueductal-stenosis_6dfa6261-3945-4606-850b-51484d05e70c.md new file mode 100644 index 0000000..bbf73bf --- /dev/null +++ b/docs_md/articles/aqueductal-stenosis_6dfa6261-3945-4606-850b-51484d05e70c.md @@ -0,0 +1,430 @@ +--- +title: "Aqueductal Stenosis" +docid: "6dfa6261-3945-4606-850b-51484d05e70c" +authors: + - key: "2c9d2e67-05db-4d26-b8cb-02e0f7566179" + value: "Usha D. Nagaraj, MD" + - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607" + value: "Surjith Vattoth, MD, FRCR" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "Aqueductal Stenosis" + slug: "aqueductal-stenosis" + treeNodeId: null +category: "Brain" +cmeTopicId: "22225b65-7fc8-4415-b55f-20fb85f6ecf5" +documentVersionId: "f14f3356-bf6d-4b97-a2e5-77751c20492b" +imageCount: 15 +lastUpdated: "07/16/20" +pageDescription: "Aqueductal Stenosis" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Aqueductal Stenosis" +pageTitle: "Aqueductal Stenosis | STATdx" +enhancedTitle: "Aqueductal Stenosis" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "Aqueductal Stenosis" +--- +# KEY FACTS + +- ## Terminology + + + - Aqueductal stenosis (AS) +- ## Imaging + + + - Ventriculomegaly of lateral and 3rd ventricles with normal-sized 4th ventricle + - Obstruction of cerebral aqueduct ± tectal thickening + - Macrocephaly in fetus and infant + - Multiplanar MR with sagittal 3D True FISP/bFFE sequence to evaluate aqueduct +- ## Top Differential Diagnoses + + + - Supratentorial volume loss + - Benign enlargement of subarachnoid fluid spaces of infancy + - Communicating hydrocephalus + - Secondary obstructive hydrocephalus +- ## Pathology + + + - Congenital AS is common cause of fetal hydrocephalus + - Can be acquired (isolated) or associated with genetic disorder + - Subsets include stenosis from tectal thickening, obstructing web/gliotic tissue, or forking +- ## Clinical Issues + + + - Though may present at any time from birth to adulthood, bimodal distribution in 1st year of life and adolescence + - Headache, papilledema, 6th nerve palsy, macrocephaly, bulging fontanelle + - C-section may be required for prenatally diagnosed cases due to macrocephaly +- ## Diagnostic Checklist + + + - Look for coexisting brain anomalies, such as rhombencephalosynapsis or diencephalic-mesencephalic dysplasia + +# TERMINOLOGY + +- ## Abbreviations + + + - Aqueductal stenosis (AS) +- ## Definitions + + + - Ventriculomegaly involving lateral and 3rd ventricles as result of complete or partial obstruction to CSF flow within cerebral aqueduct + - AS diagnosis generally refers to congenital form characterized by varying degree of obstruction at level of cerebral aqueduct + - However, tumors, germinal matrix hemorrhage, or vascular lesions can obstruct aqueduct as well + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Ventriculomegaly of lateral and 3rd ventricles with normal-sized 4th ventricle + - Macrocephaly (typical of AS) can help differentiate from supratentorial volume loss (usually normal or decreased head circumference) + - ### Location + + + - Cerebral aqueduct + - Most commonly at superior colliculi or intercollicular sulcus level + - ### Size + + + - Normal mean aqueductal cross-sectional area at birth is 0.2-1.8 mm² + - ### Morphology + + + - Funnel-shaped enlargement of proximal cerebral aqueduct or diffuse ↓ caliber of entire aqueduct +- ## CT Findings + + + - ### NECT + + + - Ventriculomegaly of lateral and 3rd ventricles, normal-sized 4th ventricle + - ± periventricular interstitial edema from uncompensated hydrocephalus +- ## MR Findings + + + - ### T1WI + + + - Ventriculomegaly of lateral and 3rd ventricles, foramina of Monro + - Corpus callosum (CC) thinned, stretched upward + - Often limits evaluation for coexisting callosal dysgenesis + - ± lateral ventricular diverticulum (a.k.a. ventricular rupture or dehiscence) + - Extraaxial CSF effacement + - Limits ability to evaluate gyral-sulcal pattern + - Normal size of 4th ventricle, basilar foramina + - Aqueductal web: Thin tissue membrane separating dilated aqueduct from normal-sized 4th ventricle + - Look for coexisting brain anomalies, such as rhombencephalosynapsis or diencephalic-mesencephalic dysplasia (incomplete segmentation between diencephalon and mesencephalon) + - ### T2WI + + + - Presence of dephasing jet or flow void through aqueduct may suggest that AS is less likely, though does not completely exclude diagnosis + - ± periventricular interstitial edema + - Tectal plate thickening + - Loss of differentiation between superior and inferior colliculi + - May be difficult to differentiate from tectal plate glioma in certain cases + - AS should **not** have T2-/FLAIR hyperintense signal or enhancement in tectal plate (tectum should be isointense to rest of midbrain on all pulse sequences) + - Unlikely to be tectal plate glioma < 3 years of age + - Septum pellucidum often absent secondary to perforation + - ### T2* GRE + + + - May have trace amounts of blood products in aqueduct and ventricular system but no frank germinal matrix hemorrhage or other cause for bleeding + - ### T1WI C+ + + + - Presence of tumor enhancement excludes congenital AS + - Hydrocephalus may induce leptomeningeal venous stasis → mimics meningitis or CSF metastases + - ### MRA + + + - Upward displacement of anterior cerebral artery branches secondary to hydrocephalus + - ### MRV + + + - Downward displacement of internal cerebral veins secondary to hydrocephalus + - ### MR cine + + + - Phase-contrast imaging may demonstrate absent or diminished CSF flow in aqueduct +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Ventriculomegaly of lateral and 3rd ventricles with normal-sized 4th ventricle in newborn with macrocephaly is highly suggestive of AS + - Obstetrical ultrasound may permit prenatal diagnosis + - Usually severe lateral ventriculomegaly (> 15 mm) + - Decreased transverse cerebellar diameter with coexisting rhombencephalosynapsis; fetal MR can help confirm + - Adducted thumbs in male fetus raise possibility of X-linked hydrocephalus +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Multiplanar MR with sagittal 3D True FISP/bFFE sequence to evaluate aqueduct + +# DIFFERENTIAL DIAGNOSIS + +- ## Supratentorial Volume Loss + + + - Should have normal or decreased head circumference +- ## Benign Enlargement of Subarachnoid Spaces in Infancy + + + - a.k.a "benign macrocrania," thought to be mild form of communicating hydrocephalus from immaturity of CSF absorption mechanisms + - Patients have normal neurologic exam + - Ventricles are normal in size or mildly enlarged +- ## Communicating Hydrocephalus + + + - Secondary to impaired absorption of CSF in subarachnoid spaces rather than anatomic obstruction + - Causes include meningitis, leptomeningeal metastatic disease, venous hypertension +- ## Secondary Obstructive Hydrocephalus + + + - Cause of obstruction is extrinsic to aqueduct: Tumor, germinal matrix hemorrhage, vascular lesion, etc. + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - AS + - Aqueductal lumen normally decreases in size beginning in 2nd month of fetal life and continuing until birth + - Normal mean cross-sectional area of aqueduct is 0.5 mm² (range: 0.2-1.8 mm²) + - Narrowing caused by growth pressures upon aqueduct from adjacent mesencephalic structures + - AS pathologically obstructs CSF flow into 4th ventricle + - CSF production in choroid plexus continues → lateral/3rd ventricular fluid ↑ pressure, ventriculomegaly + - Ventricles expand, compress adjacent parenchyma, stretch CC + - May rupture/open ependymal cell junctions → periventricular edema, ventricular diverticulum + - May be acquired or genetic + - Isolated acquired forms from prior injury/insult, such as trace hemorrhage or infection, which results in webs or gliotic tissue obstructing aqueduct + - Genetic forms often have other associated anomalies + - ### Genetics + + + - X-linked hydrocephalus + - One of most common inherited causes of AS + - Caused by mutation of *L1CAM*gene + - Gene located on X chromosome (Xq28) + - *L1CAM* expression is essential during normal embryonic development of nervous system + - Codes for neural cell adhesion molecule transmembrane glycoprotein in immunoglobulin superfamily of cell adhesion molecules + - Site of mutation within L1 protein correlates with disease severity + - Patients have poor prognosis despite early shunting + - Associated syndromes + - MASA syndrome: **Mental disability**, **a**phasia, **s**huffling gait, and **a**dducted thumbs + - CRASH syndrome: **C**allosal hypoplasia, mental disability, **a**dducted thumbs, **s**pastic paraplegia, and X-linked **h**ydrocephalus + - ### Associated abnormalities + + + - Rhombencephalosynapsis + - Up to 65% of patients with rhombencephalosynapsis have coexisting AS + - CRASH syndrome + - Absence/diminution of corticospinal tracts, thalamic fusion, collicular fusion, absence of septum pellucidum, CC dysgenesis + - Thin cerebral mantle, malformations of cortical development, hypoplastic white matter + - Dystroglycanopathy (a.k.a. congenital muscular dystrophies, such as Walker-Warburg) + - Usually associated with cerebellar dysplasia and abnormally small brainstem + - Chiari 2 malformation + - Not well described, but coexisting AS suspected in cases with increased head circumference (majority of Chiari 2 patients have decreased head circumference) +- ## Microscopic Features + + + - Can have associated malformations of cortical development with poor differentiation and maturation of cortical neurons on histology + - Aqueductal fork shows branching of aqueduct into dorsal and ventral channels + - Dorsal channel usually divided into several ductules + - These channels cannot be resolved on imaging due to microscopic size + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Symptoms depend upon patient age at time of diagnosis + - Onset can be insidious, may occur from birth to adulthood though typically bimodal distribution + - ### Other signs/symptoms + + + - Headache, papilledema, 6th nerve palsy, bulging fontanelles + - Macrocrania, especially if sutures open + - Parinaud syndrome + - Sun-setting eyes + - Lid retraction + - Tonic downgaze + - Bobble-head doll syndrome (rare) +- ## Demographics + + + - ### Age + + + - Presentation, 2 peaks of distribution: 1 in 1st year of life (more common), other in adolescence + - ### Sex + + + - M:F = 2:1 + - ### Epidemiology + + + - 0.5-1 per 1,000 births, recurrence rate of 1-4.5% in siblings + - AS responsible for ~ 20% of congenital hydrocephalus + - Most common cause of prenatal obstructive hydrocephalus +- ## Natural History & Prognosis + + + - Hydrocephalus usually progressive unless treated + - May stabilize as "arrested" or compensated hydrocephalus + - While isolated congenital AS has much better prognosis than AS with genetic disorder or other brain anomalies, only ~ 1/3 of patients with isolated AS have normal neurodevelopmental outcomes +- ## Treatment + + + - CSF shunt diversion + - Endoscopic 3rd ventriculostomy + - Cerebral aqueductoplasty for membranous and short-segment aqueductal stenoses (selected cases) + - Prenatally diagnosed cases may require C-section due to macrocephaly + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Look for coexisting brain anomalies as they make difference in prognosis +- ## Image Interpretation Pearls + + + - Use thin-section 3D True FISP/bFFE to better delineate aqueduct + + 51b0f452-75a6-4416-baed-960891a1f404 + +## References + +# Selected References + +1. [Guo D et al: A novel nonsense mutation in the L1CAM gene responsible for X-linked congenital hydrocephalus. J Gene Med. e3180, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32128973%5Bpmid%5D) +1. [Alhousseini A et al: Familial hydrocephalus and dysgenesis of the corpus callosum associated with Xp22.33 duplication and stenosis of the aqueduct of sylvius with X-linked recessive inheritance pattern. Gynecol Obstet Invest. 84(4):412-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30965333%5Bpmid%5D) +1. [Heaphy-Henault KJ et al: Congenital aqueductal stenosis: findings at fetal mri that accurately predict a postnatal diagnosis. AJNR Am J Neuroradiol. 39(5):942-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29519789%5Bpmid%5D) +1. [Kline-Fath BM et al: Congenital aqueduct stenosis: progressive brain findings in utero to birth in the presence of severe hydrocephalus. Prenat Diagn. 38(9):706-12, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29927492%5Bpmid%5D) +1. [Tonetti DA et al: Clinical outcomes of isolated congenital aqueductal stenosis. World Neurosurg. 114:e976-81, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29588243%5Bpmid%5D) +1. [Yamada S et al: Current and emerging MR imaging techniques for the diagnosis and management of CSF flow disorders: a review of phase-contrast and time-spatial labeling inversion pulse. AJNR Am J Neuroradiol. 36(4):623-30, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25012672%5Bpmid%5D) +1. [Griessenauer CJ et al: Pediatric tectal plate gliomas: clinical and radiological progression, MR imaging characteristics, and management of hydrocephalus. J Neurosurg Pediatr. 13(1):13-20, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24180680%5Bpmid%5D) +1. [Kartal MG et al: Evaluation of hydrocephalus and other cerebrospinal fluid disorders with MRI: an update. Insights Imaging. 5(4):531-41, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24903254%5Bpmid%5D) +1. [Tully HM et al: Infantile hydrocephalus: a review of epidemiology, classification and causes. Eur J Med Genet. 57(8):359-68, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24932902%5Bpmid%5D) +1. [Ucar M et al: Evaluation of aqueductal patency in patients with hydrocephalus: three-dimensional high-sampling-efficiency technique (SPACE) versus two-dimensional turbo spin echo at 3 Tesla. Korean J Radiol. 15(6):827-35, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25469096%5Bpmid%5D) +1. [Whitehead MT et al: Rhombencephalosynapsis as a cause of aqueductal stenosis: an under-recognized association in hydrocephalic children. Pediatr Radiol. 44(7):849-56, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24633306%5Bpmid%5D) +1. [Muehlmann M et al: Magnetic resonance-based estimation of intracranial pressure correlates with ventriculoperitoneal shunt valve opening pressure setting in children with hydrocephalus. Invest Radiol. 48(7):543-7, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23695081%5Bpmid%5D) +1. [O'Neill BR et al: Rapid sequence magnetic resonance imaging in the assessment of children with hydrocephalus. World Neurosurg. 80(6):e307-12, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23111234%5Bpmid%5D) +1. [Rush ET et al: Four new patients with Gomez-Lopez-Hernandez syndrome and proposed diagnostic criteria. Am J Med Genet A. 161A(2):320-6, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23292994%5Bpmid%5D) +1. [Gallo P et al: The endoscopic trans-fourth ventricle aqueductoplasty and stent placement for the treatment of trapped fourth ventricle: long-term results in a series of 18 consecutive patients. Neurol India. 60(3):271-7, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22824682%5Bpmid%5D) +1. [Ishak GE et al: Rhombencephalosynapsis: a hindbrain malformation associated with incomplete separation of midbrain and forebrain, hydrocephalus and a broad spectrum of severity. Brain. 135(Pt 5):1370-86, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22451504%5Bpmid%5D) +1. [Schroeder C et al: Why does endoscopic aqueductoplasty fail so frequently? Analysis of cerebrospinal fluid flow after endoscopic third ventriculostomy and aqueductoplasty using cine phase-contrast magnetic resonance imaging. J Neurosurg. 117(1):141-9, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=-1%5Bpmid%5D) +1. [Cinalli G et al: Hydrocephalus in aqueductal stenosis. Childs Nerv Syst. 27(10):1621-42, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928028%5Bpmid%5D) +1. [Algin O et al: Phase-contrast MRI and 3D-CISS versus contrast-enhanced MR cisternography on the evaluation of the aqueductal stenosis. Neuroradiology. 52(2):99-108, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19756563%5Bpmid%5D) +1. [Stoquart-El Sankari S et al: Phase-contrast MR imaging support for the diagnosis of aqueductal stenosis. AJNR Am J Neuroradiol. 30(1):209-14, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18832663%5Bpmid%5D) +1. [Bateman GA: Magnetic resonance imaging quantification of compliance and collateral flow in late-onset idiopathic aqueductal stenosis: venous pathophysiology revisited. J Neurosurg. 107(5):951-8, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17977266%5Bpmid%5D) +1. [da Silva LR et al: Endoscopic aqueductoplasty in the treatment of aqueductal stenosis. Childs Nerv Syst. 23(11):1263-8, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17676325%5Bpmid%5D) +1. [Koch-Wiewrodt D et al: Success and failure of endoscopic third ventriculostomy in young infants: are there different age distributions?. Childs Nerv Syst. 22(12):1537-41, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16944172%5Bpmid%5D) +1. [Sansone JM et al: Endoscopic cerebral aqueductoplasty: a trans-fourth ventricle approach. J Neurosurg. 103(5 Suppl):388-92, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16302609%5Bpmid%5D) +1. [Bhattacharyya KB et al: Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature. Acta Neurol Scand. 108(3):216-20, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12911467%5Bpmid%5D) +1. [Tisell M et al: Neurological symptoms and signs in adult aqueductal stenosis. Acta Neurol Scand. 107(5):311-7, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12713521%5Bpmid%5D) +1. [Fukuhara T et al: Clinical features of late-onset idiopathic aqueductal stenosis. Surg Neurol. 55(3):132-6; discussion 136-7, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11311904%5Bpmid%5D) +1. [Partington MD: Congenital hydrocephalus. Neurosurg Clin N Am. 12(4):737-42, ix, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11524294%5Bpmid%5D) +1. [Schroeder HW et al: Endoscopic aqueductoplasty: technique and results. Neurosurgery. 45(3):508-15; discussion 515-8, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10493373%5Bpmid%5D) +1. [Graf WD et al: The pachygyria-polymicrogyria spectrum of cortical dysplasia in X-linked hydrocephalus. Eur J Pediatr Surg. 8 Suppl 1:10-4, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9926316%5Bpmid%5D) +1. [Castro-Gago M et al: Autosomal recessive hydrocephalus with aqueductal stenosis. Childs Nerv Syst. 12(4):188-91, 1996](http://www.ncbi.nlm.nih.gov/pubmed/?term=8739404%5Bpmid%5D) +1. [Kadowaki C et al: Cine magnetic resonance imaging of aqueductal stenosis. Childs Nerv Syst. 11(2):107-11, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7758008%5Bpmid%5D) +1. [Villani R et al: Long-term outcome in aqueductal stenosis. Childs Nerv Syst. 11(3):180-5, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7773981%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal graphic shows obstructive hydrocephalus with markedly enlarged lateral and 3rd ventricles, a stretched (thinned) corpus callosum, and a funnel-shaped cerebral aqueduct related to distal obstruction. Note the normal size of the 4th ventricle and depression of the floor of the 3rd ventricle from the hydrocephalus.](images/app.statdx.com_image_thumbnail_b8037593-f2c6-448f-890c-33e3c832cfb8_annotated_true_size_900_quality_90_d08fbaed_20251018T165041Z.jpg) +*Sagittal graphic shows obstructive hydrocephalus with markedly enlarged lateral and 3rd ventricles, a stretched (thinned) corpus callosum, and a funnel-shaped cerebral aqueduct related to distal obstruction. Note the normal size of the 4th ventricle and depression of the floor of the 3rd ventricle from the hydrocephalus.* + +![Sagittal T2WI from a fetal MR at 25 weeks gestational age with aqueductal stenosis shows macrocephaly, lateral and 3rd ventriculomegaly, and no CSF in the cerebral aqueduct .](images/app.statdx.com_image_thumbnail_cfcc833d-ed65-4463-8b21-0a16f998b61a_annotated_true_size_900_quality_90_0f9aeb64_20251018T165041Z.jpg) +*Sagittal T2WI from a fetal MR at 25 weeks gestational age with aqueductal stenosis shows macrocephaly, lateral and 3rd ventriculomegaly, and no CSF in the cerebral aqueduct .* + +![Sagittal T1WI MR depicts proximal aqueductal stenosis producing enlargement of the lateral and 3rd ventricles with depression of the fornices in conjunction with normal 4th ventricle size. The tectum is dysplastic and thickened with collicular fusion .](images/app.statdx.com_image_thumbnail_691b95bc-abde-45e4-9c41-f468bff7e576_annotated_true_size_900_quality_90_5ca20c30_20251018T165041Z.jpg) +*Sagittal T1WI MR depicts proximal aqueductal stenosis producing enlargement of the lateral and 3rd ventricles with depression of the fornices in conjunction with normal 4th ventricle size. The tectum is dysplastic and thickened with collicular fusion .* + +![Sagittal FIESTA of a 5 year old with aqueductal stenosis secondary to a small obstructing web is shown. This patient underwent a 3rd ventriculostomy and is doing well. There are no other brain anomalies.](images/app.statdx.com_image_thumbnail_7e7c9735-6651-40b7-92d2-9ba4c6ba4eb9_annotated_true_size_900_quality_90_58d84b56_20251018T165041Z.jpg) +*Sagittal FIESTA of a 5 year old with aqueductal stenosis secondary to a small obstructing web is shown. This patient underwent a 3rd ventriculostomy and is doing well. There are no other brain anomalies.* + +![Sagittal T2WI MR of a 5 day old with prenatal diagnosis of aqueductal stenosis demonstrates effacement of the cerebral aqueduct with thickening of the tectum .](images/app.statdx.com_image_thumbnail_6e7dc665-e001-4483-982c-34742a8c9f88_annotated_true_size_900_quality_90_8dc210af_20251018T165041Z.jpg) +*Sagittal T2WI MR of a 5 day old with prenatal diagnosis of aqueductal stenosis demonstrates effacement of the cerebral aqueduct with thickening of the tectum .* + +![Axial T1WI MR in the same patient demonstrates rhombencephalosynapsis and bilateral choanal atresia . Other anomalies in this patient included bilateral microphthalmia and tracheoesophageal fistula. This patient had a partial deletion of chromosome 3q and SOX2 gene mutation.](images/app.statdx.com_image_thumbnail_60101105-9030-4352-ac33-e49762486e93_annotated_true_size_900_quality_90_117f5060_20251018T165041Z.jpg) +*Axial T1WI MR in the same patient demonstrates rhombencephalosynapsis and bilateral choanal atresia . Other anomalies in this patient included bilateral microphthalmia and tracheoesophageal fistula. This patient had a partial deletion of chromosome 3q and SOX2 gene mutation.* + +![Sagittal T1WI MR in a 2 day old with aqueductal stenosis with effacement of the aqueduct is shown. This patient also has diencephalic-mesencephalic dysplasia with incomplete separation of an enlarged massa intermedia from the midbrain with thickening of the 3rd ventricular floor .](images/app.statdx.com_image_thumbnail_7dec61fd-7d33-473d-a72a-826dbfe86d05_annotated_true_size_900_quality_90_6e98cdde_20251018T165041Z.jpg) +*Sagittal T1WI MR in a 2 day old with aqueductal stenosis with effacement of the aqueduct is shown. This patient also has diencephalic-mesencephalic dysplasia with incomplete separation of an enlarged massa intermedia from the midbrain with thickening of the 3rd ventricular floor .* + +![Axial T2WI MR in the same patient demonstrates dilation of the lateral and 3rd ventricles with right ventricular diverticulum and multiple subependymal gray matter heterotopias .](images/app.statdx.com_image_thumbnail_89ddd8bc-0a66-4a10-ba6f-582e15706b98_annotated_true_size_900_quality_90_f90d73d0_20251018T165041Z.jpg) +*Axial T2WI MR in the same patient demonstrates dilation of the lateral and 3rd ventricles with right ventricular diverticulum and multiple subependymal gray matter heterotopias .* + +![Sagittal T1WI MR in a patient with Walker-Warburg syndrome shows severe tectal dysgenesis with aqueductal occlusion. Marked enlargement of the lateral ventricles more than the 3rd ventricle is present. A "zigzag" brainstem and very small cerebellum are characteristic of this syndrome.](images/app.statdx.com_image_thumbnail_803672c1-c4ea-4b63-9503-c1496c7e60c7_annotated_true_size_900_quality_90_ad19ef8f_20251018T165041Z.jpg) +*Sagittal T1WI MR in a patient with Walker-Warburg syndrome shows severe tectal dysgenesis with aqueductal occlusion. Marked enlargement of the lateral ventricles more than the 3rd ventricle is present. A "zigzag" brainstem and very small cerebellum are characteristic of this syndrome.* + +![Coronal T2WI MR in the same patient confirms marked ventriculomegaly, funnel-shaped cerebral aqueductal stenosis , fused fornices , and classic cobblestone lissencephaly.](images/app.statdx.com_image_thumbnail_3f80b454-0197-4bfd-a775-7b7c846e46a4_annotated_true_size_900_quality_90_9ac2b5aa_20251018T160013Z.jpg) +*Coronal T2WI MR in the same patient confirms marked ventriculomegaly, funnel-shaped cerebral aqueductal stenosis , fused fornices , and classic cobblestone lissencephaly.* + + +### Additional Images + +![Coronal T2WI MR of the same neonate, on the 1st day of life, shows marked ventriculomegaly with asymmetric bilateral subdural hygromas following spontaneous ventricular decompression into the bilateral subdural spaces. This patient also has the additional midline congenital anomaly of rhombencephalosynapsis with characteristic incomplete dentate gyrus separation correlating with clinical truncal ataxia.](images/app.statdx.com_image_thumbnail_8cd11d10-23ae-4a94-96b5-f990d00e6278_annotated_true_size_900_quality_90_0f7423b8_20251018T160013Z.jpg) +*Coronal T2WI MR of the same neonate, on the 1st day of life, shows marked ventriculomegaly with asymmetric bilateral subdural hygromas following spontaneous ventricular decompression into the bilateral subdural spaces. This patient also has the additional midline congenital anomaly of rhombencephalosynapsis with characteristic incomplete dentate gyrus separation correlating with clinical truncal ataxia.* + +![Sagittal T2WI MR of a neonate with severe congenital hydrocephalus, imaged on the 1st day of life, shows severe aqueductal stenosis and abnormal dysplastic tectal thickening . Severe congenital hydrocephalus has resulted in spontaneous decompression into the subdural spaces .](images/app.statdx.com_image_thumbnail_0163a4f7-18e5-4811-8e10-7cbfc0576f14_annotated_true_size_900_quality_90_d4c7a9c8_20251018T160014Z.jpg) +*Sagittal T2WI MR of a neonate with severe congenital hydrocephalus, imaged on the 1st day of life, shows severe aqueductal stenosis and abnormal dysplastic tectal thickening . Severe congenital hydrocephalus has resulted in spontaneous decompression into the subdural spaces .* + +![Coronal T2WI MR shows "funneling" of the aqueduct in the coronal plane , with a markedly distended ventricular system proximal to the stenotic aqueduct.](e30bbdfe-503f-454d-a570-d0d071adf442) +*Coronal T2WI MR shows "funneling" of the aqueduct in the coronal plane , with a markedly distended ventricular system proximal to the stenotic aqueduct.* + +![Sagittal T2WI MR shows massively distended 3rd and lateral ventricles with distal aqueductal stenosis . Note the severe stretching of the corpus callosum and depression of the fornices .](39652cf8-1cab-4160-a593-4f0796871390) +*Sagittal T2WI MR shows massively distended 3rd and lateral ventricles with distal aqueductal stenosis . Note the severe stretching of the corpus callosum and depression of the fornices .* + +![Sagittal T2WI MR reveals distal aqueductal stenosis with an enlarged, funnel-shaped cerebral aqueduct and mild abnormal tectal thickening. Note the lateral and 3rd ventriculomegaly with normal size of the 4th ventricle.](891e5fdf-9bb6-4085-b168-4e5738367176) +*Sagittal T2WI MR reveals distal aqueductal stenosis with an enlarged, funnel-shaped cerebral aqueduct and mild abnormal tectal thickening. Note the lateral and 3rd ventriculomegaly with normal size of the 4th ventricle.* + diff --git a/docs_md/articles/asymmetric-lateral-ventricles_87387f0d-9b20-4288-a250-aa3ec83520c4.md b/docs_md/articles/asymmetric-lateral-ventricles_87387f0d-9b20-4288-a250-aa3ec83520c4.md new file mode 100644 index 0000000..2170f64 --- /dev/null +++ b/docs_md/articles/asymmetric-lateral-ventricles_87387f0d-9b20-4288-a250-aa3ec83520c4.md @@ -0,0 +1,413 @@ +--- +title: "Asymmetric Lateral Ventricles" +docid: "87387f0d-9b20-4288-a250-aa3ec83520c4" +authors: + - key: "1fa14dfd-71ea-4960-908e-e720313bc63a" + value: "Santhosh Gaddikeri, MD" + - key: "30ce27b2-237f-4aff-a88f-65ead356335b" + value: "Marinos Kontzialis, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60" + - + name: "Ventricles, Periventricular Regions" + slug: "ventricles-periventricular-regions" + treeNodeId: "353c434a-a6fc-4ef1-8786-d30a1988a4dc" + - + name: "Generic Imaging Patterns" + slug: "generic-imaging-patterns" + treeNodeId: "969c31a2-ef56-4fc3-9125-05857cf9aac3" + - + name: "Asymmetric Lateral Ventricles" + slug: "asymmetric-lateral-ventricles" + treeNodeId: null +category: "Brain" +cmeTopicId: "5536eb32-54f9-4eb3-83c6-15950dc4efe6" +documentVersionId: "7b24399c-3ad6-47c3-9521-b6125b27d26d" +imageCount: 54 +lastUpdated: "01/25/23" +pageDescription: "Asymmetric Lateral Ventricles" +pageKeywords: "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Asymmetric Lateral Ventricles" +pageTitle: "Asymmetric Lateral Ventricles | STATdx" +enhancedTitle: "Asymmetric Lateral Ventricles" +type: "DDX" +references: true +breadcrumbs: + - "Brain" + - "Differential Diagnosis" + - "Ventricles, Periventricular Regions" + - "Generic Imaging Patterns" + - "Asymmetric Lateral Ventricles" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Asymmetric lateral ventricles are most commonly seen as normal variant +- ## Helpful Clues for Common Diagnoses + + + - **Normal Variant** + - Asymmetric lateral ventricles seen in 5-10% of normal population + - Asymmetry mild to moderate, left > right + - Septum may be displaced across midline + - No associated mass effect, herniation, or parenchymal atrophy + - Must exclude parenchymal or intraventricular abnormality + - **Extrinsic Mass Effect** + - Etiologies include mass, hemorrhage, infarct, infection + - Mass can cause ventricular deformity, subfalcine herniation + - **Encephalomalacia, General** + - Parenchymal loss results in compensatory ventricular enlargement + - Common etiologies include chronic infarct, trauma, surgery + - **Intraventricular Hemorrhage** + - Involved ventricle may dilate early from mass effect + - Chronic dilation may be due to scarring and adhesions + - Etiologies include trauma, arteriovenous malformation (AVM), basal ganglia hemorrhage + - **Herniation Syndromes, Intracranial** + - Subfalcine herniation: Cingulate gyrus herniates under falx + - Ipsilateral lateral ventricle compressed + - Foramen of Monro obstructs and causes contralateral lateral ventricle enlargement + - Unilateral descending transtentorial herniation (uncal): Herniation of medial temporal lobe inferiorly + - Contralateral temporal horn becomes entrapped and enlarges + - Entrapped ventricle: Typically temporal horn, by extrinsic mass effect + - **Surgical Defects** + - Look for calvarial defect or "tract" + - Typically related to resection of mass + - Ventricle enlarged unilateral to defect + - **Obstructive Hydrocephalus** + - Typically acquired and bilateral + - May be unilateral if shunt complication or obstructing tumor is cause + - Rare: Colloid cyst may obstruct unilateral foramen of Monro and cause unilateral ventriculomegaly + - **Choroid Plexus Cyst** + - a.k.a. choroid plexus xanthogranuloma + - Most common choroid plexus mass in adults + - Nonneoplastic, noninflammatory cyst of choroid plexus + - Common incidental finding in older patients (40% prevalence) + - Typically bilateral; may be unilateral and enlarge lateral ventricle + - Most in lateral ventricular atria + - 60-80% bright on DWI +- ## Helpful Clues for Less Common Diagnoses + + + - **Ventriculitis** + - Ventriculomegaly with debris level, enhancing ependyma, periventricular T2/FLAIR hyperintensities + - May affect lateral ventricles asymmetrically, particularly if related to shunt placement or abscess rupture + - Restricted diffusion of layering debris is characteristic + - Bacterial ventriculitis may occur in healthy individuals after trauma or neurosurgical procedure + - Fungal or viral ventriculitis occurs most commonly in immunosuppressed patients + - **CSF Shunts and Complications** + - Common complications include shunt obstruction or breakage, infection, overdrainage + - Asymmetric ventricles may result from overdrainage or underdrainage of "isolated" ventricle + - **Meningioma** + - Although rare, still one of more common intraventricular neoplasms in adults + - More common in females (F:M = 2:1) + - Most common location is ventricular atrium with slight majority on left + - Smooth, avidly enhancing intraventricular mass + - 50% calcified; cystic changes may be present + - **Choroid Plexus Papilloma** + - Enhancing, lobulated intraventricular mass in child + - 50% in lateral ventricle atrium, left > right + - 40% in 4th ventricle + - Increased CSF production in most cases, CSF obstruction in some cases + - May have CSF spread of tumor + - **Neurocytoma, Central** + - Neurocytoma arising from ventricular system, usually septum pellucidum or lateral ventricle + - Well-circumscribed, lobulated, "bubbly" lesions + - 50% calcifications, cyst-like areas, enhancement, prominent associated flow voids + - MRS: Glycine peak (3.55 ppm) may help differentiation from other intraventricular neoplasms + - **Neurocysticercosis** + - May involve cisterns > parenchyma > ventricles + - Intraventricular disease anywhere from 0.7-33% of cases + - May lead to obstructive hydrocephalus, ventriculitis, adhesions + - Intraventricular cyst signal intensity may differ slightly from CSF on T1, T2, FLAIR +- ## Helpful Clues for Rare Diagnoses + + + - **Intraventricular Synechiae/Adhesions** + - May be congenital or acquired (prior bleed, infection, tumor) + - Look for enhancing septa, intraventricular cysts within ventricle + - **Choroid Plexus Carcinoma** + - Enhancing intraventricular mass and ependymal invasion in young child + - CSF seeding common + - Imaging does not allow reliable distinction between choroid plexus papilloma and carcinoma + - May be more heterogeneous than choroid plexus papilloma in part reflecting areas of necrosis + - May demonstrate elevation of lactate level + - **Ependymal Cyst** + - Nonenhancing, thin-walled cyst with CSF density/intensity + - Lateral ventricle most common location + - Most are incidental + - Best diagnostic clue: Nonenhancing, thin-walled CSF density/intensity cyst in lateral ventricle + - **Dyke-Davidoff-Masson** + - Antenatal unilateral hemispheric insult causes cerebral hemiatrophy + - Compensatory ipsilateral calvarial thickening, hyperpneumatized frontal sinuses and temporal bones due to longstanding cerebral hemiatrophy + - Dilated ventricle from volume loss is ipsilateral to small hemisphere + - **Hemimegalencephaly** + - Unilateral hemispheric cortical thickening + - Dilated, usually dysmorphic ventricle ipsilateral to enlarged hemisphere + - Ipsilateral extracalvarial soft tissues may be larger + - Pachygyria, polymicrogyria, heterotopias, abnormal white matter signal, blurring of gray-white matter junction + - **Rasmussen Encephalitis** + - Chronic unilateral brain inflammation of uncertain etiology leading to progressive hemispheric atrophy + - Early focal swelling of gyri + - Unilateral, predominantly frontoinsular cortical and subcortical T2/FLAIR hyperintensity progress to atrophy +- ## Other Essential Information + + + - High-resolution MR cisternography: CISS, balanced FFE, FIESTA + - May detect small septations or arachnoid membranes causing obstruction + - Cine CSF flow study may help detect physiologic flow obstruction from arachnoid webs or membranes + - May assess adequacy of drainage procedures + +## References + +# Selected References + +1. [Balasubramaniam C: Shunt complications - staying out of trouble. Neurol India. 69(Supplement):S495-501, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=35103008%5Bpmid%5D) +1. [Lanska DJ: Cruveilhier's unrecognized case (c1831) of Dyke-Davidoff-Masson syndrome. Eur Neurol. 84(4):300-6, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33965957%5Bpmid%5D) +1. [Cay-Martinez KC et al: Rasmussen encephalitis: an update. Semin Neurol. 40(2):201-10, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32185790%5Bpmid%5D) +1. [Crawford JR et al: Perinatal (fetal and neonatal) choroid plexus tumors: a review. Childs Nerv Syst. 35(6):937-44, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30953158%5Bpmid%5D) +1. [Tan LA et al: Obstructive hydrocephalus due to intraventricular hemorrhage after incidental durotomy during lumbar spine surgery. Spine (Phila Pa 1976). 43(5):E316-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=26208226%5Bpmid%5D) +1. [Zamora CA et al: Teaching neuroImages: Dyke-Davidoff-Masson in Sturge-Weber syndrome. Neurology. 85(16):e128, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26481933%5Bpmid%5D) +1. [Smith AB et al: From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 33(1):21-43, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23322825%5Bpmid%5D) +1. [Kimura-Hayama ET et al: Neurocysticercosis: radiologic-pathologic correlation. Radiographics. 30(6):1705-19, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=21071384%5Bpmid%5D) +1. [Kiroğlu Y et al: Cerebral lateral ventricular asymmetry on CT: how much asymmetry is representing pathology? Surg Radiol Anat. 30(3):249-55, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18253688%5Bpmid%5D) +1. [Rastogi S et al: Neuroimaging in pediatric epilepsy: a multimodality approach. Radiographics. 28(4):1079-95, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18635630%5Bpmid%5D) +1. [Osborn AG, Preece MT. Intracranial cysts: radiologic-pathologic correlation and imaging approach. Radiology. 2006 Jun;239(3):650-64.](http://www.ncbi.nlm.nih.gov/pubmed/?term=16714456%5Bpmid%5D) +1. [Koeller KK et al: Cerebral intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 22(6):1473-505, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12432118%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T2 MR demonstrates normal variant anatomy with mild asymmetric prominence of the right lateral ventricle when compared to the left. Note the septum is slightly deviated to the left .](images/app.statdx.com_image_thumbnail_74e87ea4-3c2a-400b-b714-f9fa2db4e66a_annotated_true_size_900_quality_90_2466c18c_20251018T165124Z.jpg) +**Normal Variant** +*Axial T2 MR demonstrates normal variant anatomy with mild asymmetric prominence of the right lateral ventricle when compared to the left. Note the septum is slightly deviated to the left .* + +![Axial T2 MR demonstrates normal variant anatomy with mild asymmetric prominence of the right lateral ventricle when compared to the left. Note the septum is slightly deviated to the left .](images/app.statdx.com_image_thumbnail_74e87ea4-3c2a-400b-b714-f9fa2db4e66a_size_174_quality_85_592b5076_20251018T165117Z.jpg) +**Normal Variant** +*Axial T2 MR demonstrates normal variant anatomy with mild asymmetric prominence of the right lateral ventricle when compared to the left. Note the septum is slightly deviated to the left .* + +![Axial FLAIR MR demonstrates a large, heterogeneous mass in the left frontal lobe with surrounding edema and mass effect with compression of the left lateral ventricle and moderate dilation of the right lateral ventricle .](images/app.statdx.com_image_thumbnail_ddc74acb-a5fe-4147-bc17-3e3862305da7_annotated_true_size_900_quality_90_d599964a_20251018T165124Z.jpg) +**Extrinsic Mass Effect** +*Axial FLAIR MR demonstrates a large, heterogeneous mass in the left frontal lobe with surrounding edema and mass effect with compression of the left lateral ventricle and moderate dilation of the right lateral ventricle .* + +![Axial T2 MR demonstrates a large, cystic encephalomalacia in the left frontal and parietal lobes due to chronic infarction with ex-vacuo dilation of the left lateral ventricle .](images/app.statdx.com_image_thumbnail_f987a5f2-594d-4b3a-84d0-640a0b70c03b_annotated_true_size_900_quality_90_e62de461_20251018T165124Z.jpg) +**Encephalomalacia, General** +*Axial T2 MR demonstrates a large, cystic encephalomalacia in the left frontal and parietal lobes due to chronic infarction with ex-vacuo dilation of the left lateral ventricle .* + +![Axial T2 MR demonstrates a large amount of acute intraventricular hemorrhage with mild dilation of the right lateral ventricle . Note CSF seepage in the peritrigonal region .](1ce39627-5994-4f0b-a77e-c02fcf1d1c66) +**Intraventricular Hemorrhage** +*Axial T2 MR demonstrates a large amount of acute intraventricular hemorrhage with mild dilation of the right lateral ventricle . Note CSF seepage in the peritrigonal region .* + +![Axial NECT demonstrates a large left subdural hematoma and a small parenchymal hemorrhage , resulting in rightward subfalcine herniation , compression of the left lateral ventricle , and mild dilation of the right lateral ventricle .](b9992ffd-4f31-44e2-8c92-83ab8fad30c1) +**Herniation Syndromes, Intracranial** +*Axial NECT demonstrates a large left subdural hematoma and a small parenchymal hemorrhage , resulting in rightward subfalcine herniation , compression of the left lateral ventricle , and mild dilation of the right lateral ventricle .* + +![Axial FLAIR MR demonstrates a large surgical defect in the left frontal lobe due to a tumor resection with resultant mild prominence of the left lateral ventricle .](a77eef3e-29e3-44c5-88bc-d67eff223e44) +**Surgical Defects** +*Axial FLAIR MR demonstrates a large surgical defect in the left frontal lobe due to a tumor resection with resultant mild prominence of the left lateral ventricle .* + +![Axial T1 C+ MR demonstrates enhancing intraventricular mass with obstructive dilation of the left temporal horn .](181895c1-e6ec-4317-a0d8-d85acb1e681a) +**Obstructive Hydrocephalus** +*Axial T1 C+ MR demonstrates enhancing intraventricular mass with obstructive dilation of the left temporal horn .* + +![Axial T2 MR demonstrates a shunt catheter in the right lateral ventricle with asymmetric lateral ventricles due to overdrainage of the right and mild underdrainage of the left . Note susceptibility artifact due to the shunt reservoir.](6a5a42a2-43a2-4fe6-8dd5-b2b1fc3e83fd) +**CSF Shunts and Complications** +*Axial T2 MR demonstrates a shunt catheter in the right lateral ventricle with asymmetric lateral ventricles due to overdrainage of the right and mild underdrainage of the left . Note susceptibility artifact due to the shunt reservoir.* + +![Axial T2 MR demonstrates a left lateral intraventricular isointense mass in the trigone with dilatation of the left trigone . Note periventricular edema . Biopsy revealed meningioma.](c60f5671-0335-43af-8efa-e151dbc51c78) +**Meningioma** +*Axial T2 MR demonstrates a left lateral intraventricular isointense mass in the trigone with dilatation of the left trigone . Note periventricular edema . Biopsy revealed meningioma.* + +![Axial T2 MR demonstrates a well-circumscribed, isointense, left lateral ventricular mass attached to the septum pellucidum with peripheral tiny cysts . Note moderate dilation of the left lateral ventricle . Biopsy revealed neurocytoma.](ebb2adb7-6287-41d0-8a41-de9563c420d8) +**Neurocytoma, Central** +*Axial T2 MR demonstrates a well-circumscribed, isointense, left lateral ventricular mass attached to the septum pellucidum with peripheral tiny cysts . Note moderate dilation of the left lateral ventricle . Biopsy revealed neurocytoma.* + +![Coronal T2 MR demonstrates asymmetric mild enlargement of the left lateral ventricle due to synechiae obstructing the foramen of Monro.](a22d935b-ac47-4f41-8b5d-57d599e0d11f) +**Intraventricular Synechiae/Adhesions** +*Coronal T2 MR demonstrates asymmetric mild enlargement of the left lateral ventricle due to synechiae obstructing the foramen of Monro.* + +![Axial T2 MR demonstrates a poorly circumscribed heterogeneous left trigonal mass with internal cysts/necrosis. Note extensive periventricular edema . Biopsy revealed choroid plexus carcinoma.](77fdb2fd-452d-4226-b341-62bf87689ec5) +**Choroid Plexus Carcinoma** +*Axial T2 MR demonstrates a poorly circumscribed heterogeneous left trigonal mass with internal cysts/necrosis. Note extensive periventricular edema . Biopsy revealed choroid plexus carcinoma.* + +![Axial NECT demonstrates atrophy of the left cerebral hemisphere with encephalomalacia due to antenatal vascular insult. Note the dilated left lateral ventricle and mild overlying calvarial thickening .](71a10f6b-7ad7-4e54-98ae-1784130732b9) +**Dyke-Davidoff-Masson** +*Axial NECT demonstrates atrophy of the left cerebral hemisphere with encephalomalacia due to antenatal vascular insult. Note the dilated left lateral ventricle and mild overlying calvarial thickening .* + +![Axial T2 MR in a patient with refractory epilepsy due to Rasmussen encephalitis demonstrates left cerebral hemispheric atrophy and mild ex-vacuo dilation of the left lateral ventricle .](71509045-9597-44be-b81f-44e0bc95be4e) +**Rasmussen Encephalitis** +*Axial T2 MR in a patient with refractory epilepsy due to Rasmussen encephalitis demonstrates left cerebral hemispheric atrophy and mild ex-vacuo dilation of the left lateral ventricle .* + + +### Additional Images + +![Axial T1WI C+ MR shows compression of the left frontal horn by a large, periventricular, enhancing mass , primary CNS lymphoma. Extrinsic mass effect is a common cause of ventricular asymmetry.](images/app.statdx.com_image_thumbnail_9c7a2c27-fa74-474a-a33e-3cfbf2329f76_annotated_true_size_900_quality_90_8cafe5b4_20251018T165124Z.jpg) +**Extrinsic Mass Effect** +*Axial T1WI C+ MR shows compression of the left frontal horn by a large, periventricular, enhancing mass , primary CNS lymphoma. Extrinsic mass effect is a common cause of ventricular asymmetry.* + +![Axial FLAIR MR demonstrates a left posterior MCA encephalomalacia resulting in mild ex-vacuo dilatation of the left atrium .](images/app.statdx.com_image_thumbnail_d875dfa4-5133-4dd6-9471-a019de504b4a_annotated_true_size_900_quality_90_e15566aa_20251018T165124Z.jpg) +**Encephalomalacia, General** +*Axial FLAIR MR demonstrates a left posterior MCA encephalomalacia resulting in mild ex-vacuo dilatation of the left atrium .* + +![Axial T2 MR shows right hemiatrophy in Sturge-Weber syndrome. Chronic venous ischemia leads to progressive hemiatrophy. Note the ipsilateral large right ventricle due to volume loss.](images/app.statdx.com_image_thumbnail_370d0b50-9617-4697-8005-d759cf146130_annotated_true_size_900_quality_90_83c359ec_20251018T165124Z.jpg) +**Encephalomalacia, General** +*Axial T2 MR shows right hemiatrophy in Sturge-Weber syndrome. Chronic venous ischemia leads to progressive hemiatrophy. Note the ipsilateral large right ventricle due to volume loss.* + +![Axial NECT shows a basal ganglia hypertensive hemorrhage with intraventricular extension . Associated midline shift results in dilation of the contralateral ventricles from foramen of Monro obstruction.](4897b7a6-5a0f-49c0-8a96-26f7653455b6) +**Intraventricular Hemorrhage** +*Axial NECT shows a basal ganglia hypertensive hemorrhage with intraventricular extension . Associated midline shift results in dilation of the contralateral ventricles from foramen of Monro obstruction.* + +![Coronal FLAIR MR shows a diffusely enlarged hyperintense supratentorial cortex compared to the cerebellum in this patient in longstanding status epilepticus. Disproportionate left hemisphere involvement has resulted in left ventricular compression , right foramen of Monro outlet obstruction, and dilation of the right ventricular system .](b73d4ba3-3f44-41f0-8763-5a8ef431e21a) +**Herniation Syndromes, Intracranial** +*Coronal FLAIR MR shows a diffusely enlarged hyperintense supratentorial cortex compared to the cerebellum in this patient in longstanding status epilepticus. Disproportionate left hemisphere involvement has resulted in left ventricular compression , right foramen of Monro outlet obstruction, and dilation of the right ventricular system .* + +![Coronal T1WI C+ MR shows a right hemispheric, subacute, subdural hematoma causing subfalcine and uncal herniation. Mass effect compresses the right frontal horn. The left ventricle is enlarged from foramen of Monro obstruction.](babb7bb2-a880-4e0d-84e4-a4a72ba57ee1) +**Herniation Syndromes, Intracranial** +*Coronal T1WI C+ MR shows a right hemispheric, subacute, subdural hematoma causing subfalcine and uncal herniation. Mass effect compresses the right frontal horn. The left ventricle is enlarged from foramen of Monro obstruction.* + +![Coronal T2WI MR shows right temporal viral encephalitis causing local mass effect . The left lateral ventricle is larger from foramen of Monro obstruction due to midline shift.](ac578239-383c-479c-9fd1-ec8b74c4c90d) +**Herniation Syndromes, Intracranial** +*Coronal T2WI MR shows right temporal viral encephalitis causing local mass effect . The left lateral ventricle is larger from foramen of Monro obstruction due to midline shift.* + +![Axial T2WI MR shows widening of right foramen of Monro , septum pellucidum deviation , and an enlarged right lateral ventricle in this tuberous sclerosis patient with remote tumor resection.](166a8b5f-51ff-4bff-a262-2f54eae8dec6) +**Surgical Defects** +*Axial T2WI MR shows widening of right foramen of Monro , septum pellucidum deviation , and an enlarged right lateral ventricle in this tuberous sclerosis patient with remote tumor resection.* + +![Axial NECT shows marked enlargement of the left lateral ventricle with bowing of septum pellucidum across midline and transependymal CSF migration indicating acute obstruction. Findings were related to a small atrial diverticulum.](111d8d26-88f6-43d3-8ddb-407a85e2707e) +**Obstructive Hydrocephalus** +*Axial NECT shows marked enlargement of the left lateral ventricle with bowing of septum pellucidum across midline and transependymal CSF migration indicating acute obstruction. Findings were related to a small atrial diverticulum.* + +![Axial T1WI C+ MR shows a colloid cyst believed to be complicated by inflammatory changes. Obstruction of the left foramen of Monro causes unilateral left lateral ventricle dilation .](f99e392c-9132-4bdd-b2fb-665f8ff10dd3) +**Obstructive Hydrocephalus** +*Axial T1WI C+ MR shows a colloid cyst believed to be complicated by inflammatory changes. Obstruction of the left foramen of Monro causes unilateral left lateral ventricle dilation .* + +![Axial T2WI MR shows a medial atrial diverticulum , a rare complication of severe hydrocephalus. CSF pouch herniates inferomedially through tentorial incisura.](51902a9b-bfb3-4df5-b937-6d3eb682f0f6) +**Obstructive Hydrocephalus** +*Axial T2WI MR shows a medial atrial diverticulum , a rare complication of severe hydrocephalus. CSF pouch herniates inferomedially through tentorial incisura.* + +![Coronal T1WI C+ MR shows typical case of coccidioidomycosis meningitis. Note marked enhancement of basal cisterns and asymmetric ventricular enlargement from CSF obstruction.](c4b489b1-dc1a-4d23-84fc-a62cd35c8b12) +**Obstructive Hydrocephalus** +*Coronal T1WI C+ MR shows typical case of coccidioidomycosis meningitis. Note marked enhancement of basal cisterns and asymmetric ventricular enlargement from CSF obstruction.* + +![Axial T1WI C+ MR shows a lobulated, nonenhancing mass in the lateral ventricle atrium, a choroid plexus xanthogranuloma. This degenerative cyst of the choroid plexus is often found incidentally in older patients.](0f9bad8c-233c-480f-adc5-7eb1ab83f856) +**Choroid Plexus Cyst** +*Axial T1WI C+ MR shows a lobulated, nonenhancing mass in the lateral ventricle atrium, a choroid plexus xanthogranuloma. This degenerative cyst of the choroid plexus is often found incidentally in older patients.* + +![Axial T1WI C+ MR shows ventriculitis with asymmetric lateral ventricles related to a temporal lobe abscess rupture and meningitis . Note ventriculomegaly and ventricular wall enhancement characteristic of ventriculitis.](792dc081-566e-4513-987f-ec30914d3bcf) +**Ventriculitis** +*Axial T1WI C+ MR shows ventriculitis with asymmetric lateral ventricles related to a temporal lobe abscess rupture and meningitis . Note ventriculomegaly and ventricular wall enhancement characteristic of ventriculitis.* + +![Axial CECT shows marked ventriculomegaly and ependymal enhancement . A dependent debris level is noted in both lateral ventricles. Ventriculitis has resulted from abscess rupture.](288f6dcf-fe0a-4dbf-809a-c0654c4a32c8) +**Ventriculitis** +*Axial CECT shows marked ventriculomegaly and ependymal enhancement . A dependent debris level is noted in both lateral ventricles. Ventriculitis has resulted from abscess rupture.* + +![Axial NECT shows an infant with hydrocephalus after placement of a right frontal ventricular drain . The shunt did not cross midline. The left lateral ventricle remained enlarged, and the right became slit-like.](5fc74921-60fe-4ec5-b99e-8667a5b6d288) +**CSF Shunts and Complications** +*Axial NECT shows an infant with hydrocephalus after placement of a right frontal ventricular drain . The shunt did not cross midline. The left lateral ventricle remained enlarged, and the right became slit-like.* + +![Axial T2WI MR shows marked enlargement of the isolated right lateral ventricle with transependymal flow of CSF indicating acute obstruction. Note left shunt and completely decompressed left lateral ventricle.](ce67f32e-2900-499f-8122-83108e8cb3f9) +**CSF Shunts and Complications** +*Axial T2WI MR shows marked enlargement of the isolated right lateral ventricle with transependymal flow of CSF indicating acute obstruction. Note left shunt and completely decompressed left lateral ventricle.* + +![Axial NECT shows asymmetric left ventricular dilation post shunting . Shunt tip may be occluded from clot or from imperforate septum, preventing drainage of the left ventricular system.](d7b146cb-1f10-4d4a-a067-85779240fbfb) +**CSF Shunts and Complications** +*Axial NECT shows asymmetric left ventricular dilation post shunting . Shunt tip may be occluded from clot or from imperforate septum, preventing drainage of the left ventricular system.* + +![Axial T2WI FS MR shows a hypointense choroid plexus mass in the atrium of the left lateral ventricle that enhanced intensely (not shown). Note striking surrounding vasogenic edema in adjacent brain parenchyma, thought due to locally obstructed CSF.](d39a3243-21fc-4d93-9a3e-85e6a329c81d) +**Meningioma** +*Axial T2WI FS MR shows a hypointense choroid plexus mass in the atrium of the left lateral ventricle that enhanced intensely (not shown). Note striking surrounding vasogenic edema in adjacent brain parenchyma, thought due to locally obstructed CSF.* + +![Axial T1WI C+ MR demonstrates a mildly enhancing and heterogeneous mass arising from the septum pellucidum . Note asymmetric dilatation of the right lateral ventricle .](1c64172a-f7fe-40df-8387-08f484c5cbad) +**Neurocytoma, Central** +*Axial T1WI C+ MR demonstrates a mildly enhancing and heterogeneous mass arising from the septum pellucidum . Note asymmetric dilatation of the right lateral ventricle .* + +![Axial T1WI C+ MR shows enhancement within the mass in the atrium of the lateral ventricle with an encysted asymmetrically larger left lateral ventricle.](4df1735f-b3a7-4ab4-9e73-ce5d331abb62) +**Choroid Plexus Papilloma** +*Axial T1WI C+ MR shows enhancement within the mass in the atrium of the lateral ventricle with an encysted asymmetrically larger left lateral ventricle.* + +![Axial T1WI C+ MR shows asymmetric lateral ventricles caused by a giant neurocysticercosis cyst in the body of the left lateral ventricle.](fcfcfeb1-c938-47be-ba9e-a639c55bbdd3) +**Neurocysticercosis** +*Axial T1WI C+ MR shows asymmetric lateral ventricles caused by a giant neurocysticercosis cyst in the body of the left lateral ventricle.* + +![Axial T1WI C+ FS MR shows a large mass in the atrium of the right lateral ventricle with a trapped, encysted occipital horn . Ependymal enhancement represents tumor spread from choroidal metastasis.](2bb8e963-0d65-4974-ae8d-429abcee49df) +**Intraventricular Synechiae/Adhesions** +*Axial T1WI C+ FS MR shows a large mass in the atrium of the right lateral ventricle with a trapped, encysted occipital horn . Ependymal enhancement represents tumor spread from choroidal metastasis.* + +![Axial T1WI C+ MR shows an asymmetrically larger right atrium in this patient with Sturge-Weber syndrome. Note associated ipsilateral enlarged frontal sinus and calvarial thickening .](29370a1b-3c42-4629-8032-765ef4ce7c56) +**Dyke-Davidoff-Masson** +*Axial T1WI C+ MR shows an asymmetrically larger right atrium in this patient with Sturge-Weber syndrome. Note associated ipsilateral enlarged frontal sinus and calvarial thickening .* + +![Axial CECT shows enlargement of the right hemisphere and lateral ventricle compared to the left side. Expansion of the hemisphere is mostly due to increased white matter. An enlarged, often deformed, lateral ventricle on the abnormal side is typical.](9a0ef691-0fe7-474a-86d7-121a23736b79) +**Hemimegalencephaly** +*Axial CECT shows enlargement of the right hemisphere and lateral ventricle compared to the left side. Expansion of the hemisphere is mostly due to increased white matter. An enlarged, often deformed, lateral ventricle on the abnormal side is typical.* + +![Axial FLAIR MR shows a cyst enlarging the left lateral ventricle with signal intensity isointense to CSF . There was no enhancement of the cyst wall, typical of ependymal cyst.](5dc6448b-9e90-480c-92c4-b59914acd3c2) +**Ependymal Cyst** +*Axial FLAIR MR shows a cyst enlarging the left lateral ventricle with signal intensity isointense to CSF . There was no enhancement of the cyst wall, typical of ependymal cyst.* + +![Axial FLAIR MR shows a cystic lesion arising from the ependymal lining of the left temporal horn , consistent with an ependymal cyst. The lesion followed CSF signal on every sequence.](6b71914c-51db-4512-973b-d4b49427919c) +**Ependymal Cyst** +*Axial FLAIR MR shows a cystic lesion arising from the ependymal lining of the left temporal horn , consistent with an ependymal cyst. The lesion followed CSF signal on every sequence.* + +![Axial T1WI MR demonstrates a right parietal shunt catheter with its tip in the right frontal horn in a patient with congenital aqueductal stenosis. The right lateral ventricle is collapsed, while the 3rd and left lateral ventricles are moderately dilated.](a93b23ad-da88-4c04-9b31-d3a582cad7c2) +**CSF Shunts and Complications** +*Axial T1WI MR demonstrates a right parietal shunt catheter with its tip in the right frontal horn in a patient with congenital aqueductal stenosis. The right lateral ventricle is collapsed, while the 3rd and left lateral ventricles are moderately dilated.* + +![Axial T1WI C+ MR shows ependymal enhancement and mild asymmetric dilatation of the left occipital horn in ventriculitis. There is also asymmetric enhancement of the adjacent choroid plexus consistent with choroid plexitis .](00e493e3-51d9-420f-be10-b731a98a2fc1) +**Ventriculitis** +*Axial T1WI C+ MR shows ependymal enhancement and mild asymmetric dilatation of the left occipital horn in ventriculitis. There is also asymmetric enhancement of the adjacent choroid plexus consistent with choroid plexitis .* + +![Axial T2WI MR shows a large, hyperintense choroid plexus cyst in a newborn. The lesion increased in size on sequential imaging, requiring endoscopic fenestration.](273e2721-a959-4b65-82dd-32f8f9fa8623) +**Choroid Plexus Cyst** +*Axial T2WI MR shows a large, hyperintense choroid plexus cyst in a newborn. The lesion increased in size on sequential imaging, requiring endoscopic fenestration.* + +![Axial FLAIR MR shows asymmetric dilatation of the left frontal horn and surrounding gliosis following the resection of an intraaxial metastasis. Part of the surgical tract is seen . Note multiple additional metastatic lesions in the right cerebral hemisphere.](53f0bb77-3081-483a-ba27-25e293db386b) +**Surgical Defects** +*Axial FLAIR MR shows asymmetric dilatation of the left frontal horn and surrounding gliosis following the resection of an intraaxial metastasis. Part of the surgical tract is seen . Note multiple additional metastatic lesions in the right cerebral hemisphere.* + +![Axial NECT demonstrates a large left frontal intraparenchymal hematoma causing subfalcine herniation to the right . The left lateral ventricle is effaced. The posterior right lateral ventricle is mildly dilated .](2ed18dfe-fece-449e-806e-9274ebd2cdd6) +**Herniation Syndromes, Intracranial** +*Axial NECT demonstrates a large left frontal intraparenchymal hematoma causing subfalcine herniation to the right . The left lateral ventricle is effaced. The posterior right lateral ventricle is mildly dilated .* + +![Axial NECT shows a large amount of intraventricular hemorrhage resulting in moderate asymmetric expansion of the left lateral ventricle. A smaller amount of layering blood products is seen in the right lateral ventricle .](3e87fdc0-f72c-4519-8fa8-407d3ca749a8) +**Intraventricular Hemorrhage** +*Axial NECT shows a large amount of intraventricular hemorrhage resulting in moderate asymmetric expansion of the left lateral ventricle. A smaller amount of layering blood products is seen in the right lateral ventricle .* + +![Axial T2WI MR shows mild, asymmetric, right hemispheric volume loss with prominent sulci and mild, asymmetric, right lateral ventricle dilatation in a pediatric patient with Rasmussen encephalitis.](de0627e1-81c9-498e-9a0e-9f525bf8351e) +**Rasmussen Encephalitis** +*Axial T2WI MR shows mild, asymmetric, right hemispheric volume loss with prominent sulci and mild, asymmetric, right lateral ventricle dilatation in a pediatric patient with Rasmussen encephalitis.* + +![Axial FLAIR MR shows a round mass in the right occipital lobe (primary CNS lymphoma) that effaces the adjacent right occipital horn and atrium . Extrinsic mass effect is a common cause of ventricular asymmetry and compression.](images/app.statdx.com_image_thumbnail_e0671196-28db-4361-9b63-cdc32902585b_annotated_true_size_900_quality_90_9b78cd11_20251018T165124Z.jpg) +**Extrinsic Mass Effect** +*Axial FLAIR MR shows a round mass in the right occipital lobe (primary CNS lymphoma) that effaces the adjacent right occipital horn and atrium . Extrinsic mass effect is a common cause of ventricular asymmetry and compression.* + +![Axial T1WI C+ MR demonstrates a heterogeneous enhancing mass arising from the septum pellucidum, a pathologically proven central neurocytoma. Note moderate asymmetric dilatation of the right lateral ventricle .](ec4b2b8a-cd64-45e6-9430-1727c705ee35) +**Neurocytoma, Central** +*Axial T1WI C+ MR demonstrates a heterogeneous enhancing mass arising from the septum pellucidum, a pathologically proven central neurocytoma. Note moderate asymmetric dilatation of the right lateral ventricle .* + +![Axial FLAIR MR shows a left thalamic expansile mass , a glioblastoma, which demonstrated heterogeneous enhancement (not shown). It protrudes into and obstructs the left ventricular atrium, which is asymmetrically dilated .](cfe69151-f87a-4abc-aa1a-01fa9586fa5f) +**Obstructive Hydrocephalus** +*Axial FLAIR MR shows a left thalamic expansile mass , a glioblastoma, which demonstrated heterogeneous enhancement (not shown). It protrudes into and obstructs the left ventricular atrium, which is asymmetrically dilated .* + +![Axial T2WI MR demonstrates generalized left hemispheric encephalomalacia following necrotizing encephalitis of unknown origin. There is resultant mild ex-vacuo dilatation of the left lateral ventricle .](images/app.statdx.com_image_thumbnail_1c54ab83-8081-45a8-960e-a7f50c31cce6_annotated_true_size_900_quality_90_6554d649_20251018T165130Z.jpg) +**Encephalomalacia, General** +*Axial T2WI MR demonstrates generalized left hemispheric encephalomalacia following necrotizing encephalitis of unknown origin. There is resultant mild ex-vacuo dilatation of the left lateral ventricle .* + +![Axial T1WI C+ MR shows an avidly enhancing, lobulated mass arising from the right ventricular atrium , a meningioma. Note the mild asymmetric dilatation of the left lateral ventricle .](b426de7c-024c-4f25-ab33-1e7a567fe147) +**Meningioma** +*Axial T1WI C+ MR shows an avidly enhancing, lobulated mass arising from the right ventricular atrium , a meningioma. Note the mild asymmetric dilatation of the left lateral ventricle .* + +![Axial T2WI MR shows lateral ventricles with the right being larger than the left, representing a normal variant. Note mild bowing of the septum pellucidum across the midline .](images/app.statdx.com_image_thumbnail_7a290571-170f-4a98-8381-da762b17ab09_annotated_true_size_900_quality_90_97b6ac24_20251018T165124Z.jpg) +**Normal Variant** +*Axial T2WI MR shows lateral ventricles with the right being larger than the left, representing a normal variant. Note mild bowing of the septum pellucidum across the midline .* + diff --git a/docs_md/articles/benign-enlarged-subarachnoid-spaces_3da4fec0-6e87-4bcc-bd66-b4a5d1984f6e.md b/docs_md/articles/benign-enlarged-subarachnoid-spaces_3da4fec0-6e87-4bcc-bd66-b4a5d1984f6e.md new file mode 100644 index 0000000..a36b382 --- /dev/null +++ b/docs_md/articles/benign-enlarged-subarachnoid-spaces_3da4fec0-6e87-4bcc-bd66-b4a5d1984f6e.md @@ -0,0 +1,468 @@ +--- +title: "Benign Enlarged Subarachnoid Spaces" +docid: "3da4fec0-6e87-4bcc-bd66-b4a5d1984f6e" +authors: + - key: "47381de4-c9fd-4999-8dd0-1808cd72db6b" + value: "Luke L. Linscott, MD" + - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607" + value: "Surjith Vattoth, MD, FRCR" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Normal Variants" + slug: "normal-variants" + treeNodeId: "bf92256f-cdff-4bcd-8420-d876b9e4031a" + - + name: "Benign Enlarged Subarachnoid Spaces" + slug: "benign-enlarged-subarachnoid-spaces" + treeNodeId: null +category: "Brain" +documentVersionId: "223d884d-2cb4-42bf-b5ad-ed2ccbd89644" +imageCount: 20 +lastUpdated: "07/31/20" +pageDescription: "Benign Enlarged Subarachnoid Spaces" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Normal Variants, Benign Enlarged Subarachnoid Spaces" +pageTitle: "Benign Enlarged Subarachnoid Spaces | STATdx" +enhancedTitle: "Benign Enlarged Subarachnoid Spaces" +type: "DX" +references: true +ddx: true +cases: 2 +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Normal Variants" + - "Benign Enlarged Subarachnoid Spaces" +--- +# KEY FACTS + +- ## Terminology + + + - Idiopathic enlargement of subarachnoid spaces (SAS) during infancy +- ## Imaging + + + - Primary imaging modality: US + - CT/MR used if fontanelle closing or to further investigate atypical clinical/US findings + - Best clue: Enlarged SAS and ↑ orbitofrontal circumference (OFC) (> 95th percentile) + - Ventricles may be mildly enlarged + - Symmetric bifrontal and bitemporal SAS + - All modalities show veins coursing through SAS + - SAS follow CSF appearance on all modalities + - No compression of veins or gyri + - No inward displacement of arachnoid membrane by subdural fluid; small, nonhemorrhagic, subdural collections seen in ~ 4% of patients with enlarged SAS +- ## Top Differential Diagnoses + + + - Atrophy + - Acquired progressive communicating hydrocephalus + - Nonaccidental trauma (NAT) +- ## Pathology + + + - Etiology uncertain: Immature CSF drainage pathways likely + - Family history of macrocephaly > 80% +- ## Clinical Issues + + + - Mild developmental delay alone should not prompt further imaging or subspecialty evaluation + - Further evaluation required only in setting of focal neurologic signs &/or developmental regression + - Consider NAT if enlarged extraaxial spaces atypical + - Moderate/large/complex subdural collection → NAT work-up + - SAS enlargement and developmental delay typically resolve without therapy by 2 years of age + - No treatment necessary +- ## Diagnostic Checklist + + + - Further evaluation with brain MR or CT if US atypical + - Even small/simple subdural collections should be discussed with referring clinician to identify any concerns for NAT that merit further work-up + +# TERMINOLOGY + +- ## Synonyms + + + - Benign enlargement of subarachnoid spaces of infancy (BESSI) + - Benign external hydrocephalus + - Benign extracerebral fluid collections of infancy + - Benign communicating hydrocephalus + - Physiologic extraventricular obstructive hydrocephalus + - Benign macrocephaly of infancy +- ## Definitions + + + - Enlarged subarachnoid spaces (SAS) in patient < 1 year of age with macrocrania [head circumference (HC) > 95%] + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Symmetric bilateral SAS enlargement ± mild ventriculomegaly + - Patient with normal or mildly delayed development + - ### Location + + + - Enlarged SAS in infant with macrocrania + - Symmetric at bifrontal and bitemporal SAS + - ### Size + + + - Normal SAS values differ significantly between studies + - Normal maximum width peaks at 28 postnatal weeks (7 months of age) + - Interhemispheric width: 95th percentile: ~ 8 mm + - Widest distance between hemispheres + - Craniocortical width: 95th percentile: ~ 10 mm + - Widest vertical distance between brain and inner table of calvarium + - Sinocortical width: 95th percentile: ~ 7 mm + - Widest distance between cortex and superior sagittal sinus + - ### Morphology + + + - CSF space follows (not flattens) gyral contour +- ## Radiographic Findings + + + - Radiography + - ↑ craniofacial ratio +- ## CT Findings + + + - ### NECT + + + - Enlarged SAS with normal sulci; no hemorrhage + - Enlarged cisterns (especially suprasellar/chiasmatic) + - ### CECT + + + - Demonstrates veins traversing SAS + - No abnormal meningeal enhancement +- ## MR Findings + + + - ### T1WI + + + - Normal brain parenchyma without edema + - Small subdural collections sometimes visible + - ### T2WI + + + - Arachnoid membranes + - Small nonhemorrhagic subdural collections in ~ 4% + - ### FLAIR + + + - SAS fluid follows CSF signal on all sequences + - Incomplete signal suppression in subdural collections + - ### DWI + + + - ↑ fractional anisotropy and mean diffusivity in brain of patients with enlarged SAS compared to controls + - Normalizes over time with resolution of SAS enlargement + - ### T1WI C+ + + + - Demonstrates veins traversing SAS + - SSFSE + - May be used for follow-up to avoid sedation in children +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Primary modality used whenever possible + - Symmetric enlargement of bifrontal SAS + - ± mild ventricular enlargement + - ### Color Doppler + + + - Cortical veins seen within subarachnoid fluid space + - No mass effect displacing veins against pia + - No inward displacement of arachnoid membrane by subdural fluid + - Subdural collections lack traversing veins +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - US if acoustic window available + - CT/MR if no acoustic window available + - ### Protocol advice + + + - Doppler sonography: Documents veins traversing SAS + - Linear high-resolution US most sensitive for detection of associated subdural fluid + - After diagnosis, best follow-up: Clinical monitoring of HC and development of any neurologic findings + - Follow-up with MR/CT typically not necessary, unless + - Focal neurologic signs/symptoms + - Suspicion for subdural collection on US + +# DIFFERENTIAL DIAGNOSIS + +- ## Atrophy + + + - Small HC; sulcal prominence out of proportion + - Forehead "pointed" due to metopic fusion +- ## Incidental Bilateral Subdural Fluid Collections + + + - Subdural fluid not normally visualized + - Small, nonhemorrhagic subdural collections seen in 4% of benign macrocrania patients + - Characterized by crescentic fluid collection separating dura from arachnoid + - No cortical veins traversing subdural space + - Discrete arachnoid membrane displaced toward cortex; may be compressing SAS veins + - May have different signal intensity on PD and other MR sequences compared to CSF + - Discuss need for further work-up with referring clinician + - Close clinical follow-up at minimum; work-up for nonaccidental trauma (NAT) at discretion of clinician +- ## Nonaccidental Trauma + + + - Moderate/large or hemorrhagic subdurals or unusual clinical findings should raise concern +- [Glutaric Aciduria Type 1](/document/glutaric-acidemia-type-1/55db6f3e-1d78-4bc6-b366-68b2d37e5d80) + - Enlarged sylvian fissures with delayed myelination + - Subdural collections may be present + - T2-hyperintense basal ganglia +- ## Elevated Venous Pressures + + + - Causes: Cardiac disease, internal jugular vein sacrifice for ECMO, arteriovenous fistula, or sinus venous thrombosis +- ## Communicating Hydrocephalus + + + - Often post hemorrhagic/post inflammatory/neoplastic + - Density of extraaxial collection does not = CSF + - Achondroplasia and other skull base anomalies + - Coarctation of foramen magnum (narrow) + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Remains incompletely understood + - Immature CSF drainage pathways: Most accepted theory + - CSF primarily drained via extracellular space → capillaries + - Pacchionian granulations (PGs) do not mature until 18 months + - PGs then displaced into veins (as Starling-type resistors) + - PGs regulate venous drainage of CSF when fontanels close + - Benign SAS enlargement usually resolves at that time + - Disproportionate growth of skull and brain + - Faster growth of skull results in ↑ SAS ± ↑ ventricles + - This theory helps to explain frequent identification of subdural fluid collections + - Family history of macrocephaly > 80% + - ### Associated abnormalities + + + - Subdural collections (typically small and incidental) in ~ 4% + - Predisposition to bleed with minor trauma: Controversial + - Possibility of ↑ risk for bridging vein injury and subdural collection/hematoma in absence of major trauma + - Venous "stretching" implicated + - May ↑ risk of arachnoid cyst development compared to normal population +- ## Gross Pathologic & Surgical Features + + + - Deep/prominent but otherwise normal-appearing SAS + - No pathologic membranes +- ## Microscopic Features + + + - Ependymal damage not seen in benign SAS enlargement + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Macrocrania: HC > 95th percentile + - Frontal bossing + - No signs of elevated intracranial pressure (ICP); normal pressure on lumbar puncture + - Danger signs + - Elevated ICP + - Persistent or rapid deviation of HC from normal curve + - Developmental regression, focal neurologic signs, vomiting, bruising + - ### Other signs/symptoms + + + - Mild developmental delay common (20-50%) and usually resolves over time + - Should not necessarily prompt further evaluation + - ### Clinical profile + + + - Family history of benign macrocephaly common + - Male infants, ± late to walk +- ## Demographics + + + - Most common imaging diagnosis for macrocrania in patients < 1 year of age  + - Usually presents at 3-9 months + - Sex: M:F = 2:1 +- ## Natural History & Prognosis + + + - Enlarged SAS → ↑ suture/calvarial malleability/compliance → predisposes to posterior plagiocephaly + - Self-limited; resolves without therapy by 12-24 months + - Spontaneous resolution of spaces and symptoms + - Macrocephaly may persist +- ## Treatment + + + - No treatment necessary + - Normal outcome (developmental delay usually resolves as prominent SAS resolves) + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - Crucial to know HC + - Further evaluation with brain MR or CT if US atypical + - Moderate/large/complex subdural collection → NAT work-up + - Even small/simple subdural collections should be discussed with referring clinician to identify any concerns for NAT that merit further work-up + + 17f5e314-01d9-44b0-81da-6468019ad492 + +## References + +# Selected References + +1. [Yum SK et al: Enlarged subarachnoid space on cranial ultrasound in preterm infants: Neurodevelopmental implication. Sci Rep. 9(1):19072, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31836837%5Bpmid%5D) +1. [Zahl SM et al: Clinical, radiological, and demographic details of benign external hydrocephalus: a population-based study. Pediatr Neurol. 96:53-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30808532%5Bpmid%5D) +1. [Zahl SM et al: Quality of life and physician-reported developmental, cognitive, and social problems in children with benign external hydrocephalus-long-term follow-up. Childs Nerv Syst. 35(2):245-50, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30523438%5Bpmid%5D) +1. [Hansen JB et al: Evaluations for abuse in young children with subdural hemorrhages: findings based on symptom severity and benign enlargement of the subarachnoid spaces. J Neurosurg Pediatr. 21(1):31-7, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29099352%5Bpmid%5D) +1. [Haws ME et al: A retrospective analysis of the utility of head computed tomography and/or magnetic resonance imaging in the management of benign macrocrania. J Pediatr. 182:283-9.e1, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27989412%5Bpmid%5D) +1. [Hussain ZB et al: Extra-axial cerebrospinal fluid spaces in children with benign external hydrocephalus: a case-control study. Neuroradiol J. 30(5):410-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28691570%5Bpmid%5D) +1. [Naffaa L et al: The diagnostic yield of ultrasound of the head in healthy infants presenting with the clinical diagnosis of benign macrocrania. Clin Radiol. 72(1):94.e7-94.e11, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27756452%5Bpmid%5D) +1. [Whitehead MT et al: Reduced subarachnoid fluid diffusion in enlarged subarachnoid spaces of infancy. Neuroradiol J. 30(5):418-24, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28195509%5Bpmid%5D) +1. [Tucker J et al: Macrocephaly in infancy: benign enlargement of the subarachnoid spaces and subdural collections. J Neurosurg Pediatr. 1-5, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26942270%5Bpmid%5D) +1. [Halevy A et al: Development of infants with idiopathic external hydrocephalus. J Child Neurol. 30(8):1044-7, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25348416%5Bpmid%5D) +1. [Marino MA et al: Benign external hydrocephalus in infants. A single centre experience and literature review. Neuroradiol J. 27(2):245-50, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24750715%5Bpmid%5D) +1. [Greiner MV et al: Prevalence of subdural collections in children with macrocrania. AJNR Am J Neuroradiol. 34(12):2373-8, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23868166%5Bpmid%5D) +1. [Mattei TA et al: Benign extracerebral fluid collection in infancy as a risk factor for the development of de novo intracranial arachnoid cysts. J Neurosurg Pediatr. 12(6):555-64, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24093592%5Bpmid%5D) +1. [Schulz M et al: Intracranial pressure measurement in infants presenting with progressive macrocephaly and enlarged subarachnoid spaces. Acta Neurochir Suppl. 114:261-6, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22327705%5Bpmid%5D) +1. [Sun M et al: Diffusion tensor imaging findings in young children with benign external hydrocephalus differ from the normal population. Childs Nerv Syst. 28(2):199-208, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22167268%5Bpmid%5D) +1. [Bateman GA et al: External hydrocephalus in infants: six cases with MR venogram and flow quantification correlation. Childs Nerv Syst. 27(12):2087-96, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21833725%5Bpmid%5D) +1. [Yew AY et al: Long-term health status in benign external hydrocephalus. Pediatr Neurosurg. 47(1):1-6, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21778677%5Bpmid%5D) +1. [Zahl SM et al: Benign external hydrocephalus: a review, with emphasis on management. Neurosurg Rev. 34(4):417-32, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21647596%5Bpmid%5D) +1. [Fernando S et al: Neuroimaging of nonaccidental head trauma: pitfalls and controversies. Pediatr Radiol. 38(8):827-38, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18176805%5Bpmid%5D) +1. [Hellbusch LC: Benign extracerebral fluid collections in infancy: clinical presentation and long-term follow-up. J Neurosurg. 107(2 Suppl):119-25, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=18459883%5Bpmid%5D) +1. [Paciorkowski AR et al: When is enlargement of the subarachnoid spaces not benign? A genetic perspective. Pediatr Neurol. 37(1):1-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17628215%5Bpmid%5D) +1. [Muenchberger H et al: Idiopathic macrocephaly in the infant: long-term neurological and neuropsychological outcome. Childs Nerv Syst. 22(10):1242-8, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16544148%5Bpmid%5D) +1. [Lam WW et al: Ultrasonographic measurement of subarachnoid space in normal infants and children. Pediatr Neurol. 25(5):380-4, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11744312%5Bpmid%5D) + +## Differential diagnosis + +### Cistern, Subarachnoid Space Normal Variant +DDX:167a514e-0b18-4a16-9474-41a1d760607b + +### Subarachnoid Space Normal Variants +DDX:558a9979-3a38-473f-a5f8-bf6b6d6538e2 + +## Cases + +- {'cases': [{'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': 'bd82b696-6c64-439d-a4e9-417829b23517', 'description': 'T2W axial images (#1-4) demonstrate "squaring" of the forehead and prominent pericerebral spaces. Small linear flow voids (arrows #2-4) represent veins traversing the subarachnoid space. The CSF spaces are at their widest at approximately 7 months of life. This process has also been called benign macrocephaly of infancy, physiologic extraventricular obstructive hydrocephalus, and external hydrocephalus. It is self-limited, usually resolving without therapy by 1 to 2 years of age.', 'history': 'Presented with macrocrania and frontal bossing.', 'imagePoolId': 'fd5ba87e-b9d9-48f7-aa78-96eac3fc925e', 'name': 'Crossing vessels', 'teachingPoint': None, 'demographics': '7 Months old male'}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': '9e524b89-ebd0-4d81-9776-61b08302b2ec', 'description': 'NECT (#1, 2) demonstrate marked enlargement of the frontal pericerebral CSF spaces (arrow). Without the use of IV contrast material, traversing venous structures cannot be assessed. Similar findings are seen on sagittal and axial T1WIs (#3, 4) and axial FLAIR (#5). Axial (#6, 7) and coronal (#8) T2W images, however, are extremely useful. The T2W images reveal fine linear flow-voids due to traversing veins (#6-8, curved arrows), confirming that these enlarged spaces are in fact the subarachnoid space. \n\nComment: Enlarged pericerebral spaces in infancy are often a transient and benign condition. They resolve between 8 and 12 months of age, usually when the infant is able to be in the upright position for longer periods of time. They are slower to resolve in late walkers. The traversing veins may bleed with trauma, simulating non-accidental injury. Sequential follow-up of head-circumference (tape-measure, not imaging) is suggested. Repeat imaging is suggested when the macrocrania rapidly progresses, or when there are neurological symptoms.', 'history': 'Typically present with macrocrania between the ages of 3 and 8 months of age.', 'imagePoolId': 'ad479e42-534c-4d0e-9c71-416160f544a5', 'name': 'Marked', 'teachingPoint': None, 'demographics': '8 Months old male'}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': '94dfc51a-38fa-4d6c-98ea-9215b294dad3', 'description': 'Axial NECT reveals an asymmetric prominence of pericerebral spaces (arrow, #1). T2W images obtained 5 months later demonstrate persistence of the asymmetry (arrows, #2, 3). No membranes or unequal signal intensity are seen on FLAIR (#4). Enhancing veins traverse the dilated subarachnoid space (curved arrows, #5). Enlargement of the subarachnoid space is common during infancy; danger signs requiring imaging evaluation would include rapid enlargement of head circumference, marked prominence of subarachnoid space, increased intracranial pressure and persistence or onset after 1 year of age. Asymmetry is also concerning, raising the suspicion for underlying subdural collection in non-accidental trauma.', 'history': 'Presented with macrocrania and possible seizures.', 'imagePoolId': 'd78c443b-8aae-4312-ab47-bfae3322a312', 'name': 'Asymmetric', 'teachingPoint': None, 'demographics': '3 Months old male'}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}, {'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'c76edba4-99cf-4471-99a3-e58bc8676bab', 'description': 'There is marked asymmetry of subarachnoid space enlargement (#1-8). After contrast administration, however, veins are seen to traverse the subarachnoid space (arrow, #5-8). Compression of the right posterior subarachnoid space by positional lambdoid flattening (open arrow, #1, 2, 4) and calvarial deformation accounts, in part, for the prominence of the contralateral spaces. Asymmetry is of concern and should prompt a search for an underlying subdural component or of underlying brain dysgenesis. In this child, however, traversing veins confirm involvement only of the subarachnoid space.', 'history': None, 'imagePoolId': '91d9adcc-19f5-46a3-b1c1-2ceff2c37812', 'name': 'Asymmetric', 'teachingPoint': None, 'demographics': '3 Months old male'}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': 'ddc8c96d-0330-4a2a-8065-24b477e33f11', 'description': 'Enlarged pericerebral spaces are identified on coronal sonography. Distance between the surface of the brain and the dura is 1.5 cm, or 3 times the maximum allowable measurement of 5 mm. High-resolution view reveals multiple linear veins (arrows) traversing the subarachnoid space, confirming the diagnosis. \n\nNECT demonstrates huge pericerebral spaces over the frontal convexities and widening the interhemispheric and Sylvian fissures. CECT confirms a plethora of veins (arrows) traversing the subarachnoid space. No subdural membrane is identified.\n\nEnlargement of the subarachnoid spaces, also known as physiologic extraventricular obstructive hydrocephalus (EVOH), external hydrocephalus or benign macrocephaly of infancy is usually a transient phenomenon peaking between 3 and 8 months of age. Surgical intervention is needed only when spaces reach massive size, as in this case. \n\nBe careful not to mistake enlarged arachnoid spaces (in the presence of macrocephaly) for atrophy. Always determine and document the head circumference.', 'history': 'Patient was noted to have macrocephaly at 2 months of age. Subarachnoid spaces enlarged dramatically over time. Patient required shunt at 7 months.', 'imagePoolId': 'e88a10c0-feca-4740-8c77-7996345f28ae', 'name': 'Massive', 'teachingPoint': None, 'demographics': '7 Months old male'}], 'caseType': 'variant', 'name': 'VARIANT'} + + +## Images + + +### Selected Images + +![Axial graphic shows classic enlarged subarachnoid spaces (SAS) in a macrocephalic infant (head circumference > 95%). Note the symmetric enlargement with idiopathic enlargement of SAS during the 1st year of life.](images/app.statdx.com_image_thumbnail_625c002a-cbdb-4b3b-82b8-c174380b8cab_annotated_true_size_900_quality_90_0217a31f_20251018T164822Z.jpg) +*Axial graphic shows classic enlarged subarachnoid spaces (SAS) in a macrocephalic infant (head circumference > 95%). Note the symmetric enlargement with idiopathic enlargement of SAS during the 1st year of life.* + +![Axial graphic shows classic enlarged subarachnoid spaces (SAS) in a macrocephalic infant (head circumference > 95%). Note the symmetric enlargement with idiopathic enlargement of SAS during the 1st year of life.](images/app.statdx.com_image_thumbnail_625c002a-cbdb-4b3b-82b8-c174380b8cab_size_174_quality_85_c1b69f4c_20251018T164756Z.jpg) +*Axial graphic shows classic enlarged subarachnoid spaces (SAS) in a macrocephalic infant (head circumference > 95%). Note the symmetric enlargement with idiopathic enlargement of SAS during the 1st year of life.* + +![Axial T2 MR shows enlarged frontal & anterior interhemispheric pericerebral fluid spaces , mild ventriculomegaly, & right-sided posterior plagiocephaly in a 7-month-old boy with macrocephaly.](images/app.statdx.com_image_thumbnail_999bae79-c259-4e1e-99f7-df0f6d6080e6_annotated_true_size_900_quality_90_99a2989a_20251018T164822Z.jpg) +*Axial T2 MR shows enlarged frontal & anterior interhemispheric pericerebral fluid spaces , mild ventriculomegaly, & right-sided posterior plagiocephaly in a 7-month-old boy with macrocephaly.* + +![Coronal US in a 7-month-old boy with macrocrania shows enlarged SAS & normal ventricular size. Note the normal size of the sulci. This is a typical clinical history & imaging appearance for benign enlargement of the SAS.](images/app.statdx.com_image_thumbnail_e4adf66f-2a85-49d4-81d1-b5a27e609e47_annotated_true_size_900_quality_90_697cf19c_20251018T164822Z.jpg) +*Coronal US in a 7-month-old boy with macrocrania shows enlarged SAS & normal ventricular size. Note the normal size of the sulci. This is a typical clinical history & imaging appearance for benign enlargement of the SAS.* + +![Coronal color Doppler US in a 4-month-old girl shows vessels traversing the enlarged SAS . Doppler US can be helpful to exclude subdural collections by demonstrating normal veins in the SAS.](images/app.statdx.com_image_thumbnail_2378a15b-8f1c-47cf-bf91-5ab9181ca721_annotated_true_size_900_quality_90_ad41edb4_20251018T164822Z.jpg) +*Coronal color Doppler US in a 4-month-old girl shows vessels traversing the enlarged SAS . Doppler US can be helpful to exclude subdural collections by demonstrating normal veins in the SAS.* + +![Coronal US in a 3 month old with macrocephaly shows prominent SAS as well as mild enlargement of the lateral ventricles . Mild lateral ventricular enlargement is common in benign enlargement of subarachnoid spaces (BESSI).](images/app.statdx.com_image_thumbnail_7ed3d5fd-ca66-4f3f-b16a-def63fa152be_annotated_true_size_900_quality_90_0bbc9592_20251018T164822Z.jpg) +*Coronal US in a 3 month old with macrocephaly shows prominent SAS as well as mild enlargement of the lateral ventricles . Mild lateral ventricular enlargement is common in benign enlargement of subarachnoid spaces (BESSI).* + +![Coronal T2 MR in a 6 month old with macrocephaly shows symmetrically prominent bifrontal SAS with mild enlargement of the lateral ventricles . Mild enlargement of the lateral ventricles should not dissuade one from suggesting BESSI.](images/app.statdx.com_image_thumbnail_82a63fe6-8800-487d-a506-2f4b3399fd2d_annotated_true_size_900_quality_90_fef6728f_20251018T164822Z.jpg) +*Coronal T2 MR in a 6 month old with macrocephaly shows symmetrically prominent bifrontal SAS with mild enlargement of the lateral ventricles . Mild enlargement of the lateral ventricles should not dissuade one from suggesting BESSI.* + +![Coronal T2 MR at 13 months (left) & NECT at 5 years (right) of age show expected resolution of the enlarged SAS over a 4-year period. Enlarged SAS typically resolve by 24 months of age.](images/app.statdx.com_image_thumbnail_6d4772cf-8e5a-46f7-b768-d7df54bae831_annotated_true_size_900_quality_90_1842bb3c_20251018T164822Z.jpg) +*Coronal T2 MR at 13 months (left) & NECT at 5 years (right) of age show expected resolution of the enlarged SAS over a 4-year period. Enlarged SAS typically resolve by 24 months of age.* + +![Coronal high-resolution US in a 4-month-old girl with macrocrania shows bilateral enlargement of the SAS . Also present are small, bilateral, subdural collections , which are anechoic compared to the SAS. Note the separation of the arachnoid membrane .](images/app.statdx.com_image_thumbnail_e1ad2f29-ee6d-4acb-9009-60ab93f84ebb_annotated_true_size_900_quality_90_5094ced0_20251018T164822Z.jpg) +*Coronal high-resolution US in a 4-month-old girl with macrocrania shows bilateral enlargement of the SAS . Also present are small, bilateral, subdural collections , which are anechoic compared to the SAS. Note the separation of the arachnoid membrane .* + +![Axial PD MR in a 4-month-old girl with macrocrania shows enlarged SAS , which are isointense to the brain. Also note the small, bilateral, nonhemorrhagic, hyperintense subdural fluid collections .](images/app.statdx.com_image_thumbnail_c10a685b-5eda-415a-a0a7-c9ba71989dbc_annotated_true_size_900_quality_90_5ff1fb6d_20251018T164822Z.jpg) +*Axial PD MR in a 4-month-old girl with macrocrania shows enlarged SAS , which are isointense to the brain. Also note the small, bilateral, nonhemorrhagic, hyperintense subdural fluid collections .* + +![Coronal T2 MR in the same patient shows symmetrically enlarged SAS as well as small, bilateral, nonhemorrhagic subdural fluid collections . Small, subdural fluid collections are seen in ~ 4% of patients with enlarged SAS.](images/app.statdx.com_image_thumbnail_dadaa55c-ec98-4fde-8e1a-f4ff02313e31_annotated_true_size_900_quality_90_8c00f0a2_20251018T164802Z.jpg) +*Coronal T2 MR in the same patient shows symmetrically enlarged SAS as well as small, bilateral, nonhemorrhagic subdural fluid collections . Small, subdural fluid collections are seen in ~ 4% of patients with enlarged SAS.* + + +### Additional Images + +![Axial graphic shows classic enlargement of the subarachnoid spaces (SAS) in a macrocephalic infant. There is symmetric bifrontal enlargement of the SAS, which contain multiple bridging veins . Mild ventriculomegaly is present.](images/app.statdx.com_image_thumbnail_970df207-9c92-40e4-83d2-71d303b99906_annotated_true_size_900_quality_90_013d624a_20251018T164803Z.jpg) +*Axial graphic shows classic enlargement of the subarachnoid spaces (SAS) in a macrocephalic infant. There is symmetric bifrontal enlargement of the SAS, which contain multiple bridging veins . Mild ventriculomegaly is present.* + +![Axial T2 MR in a 6-month-old boy with enlarged SAS shows vessels coursing through the SAS. Note the lack of mass effect on the underlying brain parenchyma. There is mild enlargement of the lateral ventricles , a common finding in benign enlargement of the SAS.](images/app.statdx.com_image_thumbnail_853ffc4b-e3ff-4876-a50a-9fd8233de3af_annotated_true_size_900_quality_90_7726ca40_20251018T164803Z.jpg) +*Axial T2 MR in a 6-month-old boy with enlarged SAS shows vessels coursing through the SAS. Note the lack of mass effect on the underlying brain parenchyma. There is mild enlargement of the lateral ventricles , a common finding in benign enlargement of the SAS.* + +![Coronal T2 MR in the same 4-month-old girl with macrocrania shows symmetrically enlarged SAS as well as small to moderate, bilateral subdural fluid collections . The subdural collections are slightly hyperintense to the SAS. Small subdural fluid collections are seen in ~ 4% of patients with enlarged SAS.](images/app.statdx.com_image_thumbnail_954b835b-dd1b-4ebc-922e-0e238b1c9468_annotated_true_size_900_quality_90_d9a0b852_20251018T164803Z.jpg) +*Coronal T2 MR in the same 4-month-old girl with macrocrania shows symmetrically enlarged SAS as well as small to moderate, bilateral subdural fluid collections . The subdural collections are slightly hyperintense to the SAS. Small subdural fluid collections are seen in ~ 4% of patients with enlarged SAS.* + +![Axial CECT shows enlarged SAS with enhancing traversing veins in a macrocephalic infant. This benign condition usually peaks at 7 months of age & resolves spontaneously by 12-24 months of age.](images/app.statdx.com_image_thumbnail_6853ee67-afda-4c9b-86f1-275ecc5b5521_annotated_true_size_900_quality_90_9bff7ce2_20251018T164803Z.jpg) +*Axial CECT shows enlarged SAS with enhancing traversing veins in a macrocephalic infant. This benign condition usually peaks at 7 months of age & resolves spontaneously by 12-24 months of age.* + +![Axial T2 MR shows prominent frontal CSF spaces (craniocortical & interhemispheric) with mildly prominent ventricles in this macrocephalic infant. Note the squaring of the forehead, seen clinically as "frontal bossing." About 20-50% of cases have mild developmental delay (motor > > language), which nearly always resolves without therapy.](images/app.statdx.com_image_thumbnail_e08c6654-1cb2-4a96-bab2-26736e44e4d7_annotated_true_size_900_quality_90_85e3f2a6_20251018T164803Z.jpg) +*Axial T2 MR shows prominent frontal CSF spaces (craniocortical & interhemispheric) with mildly prominent ventricles in this macrocephalic infant. Note the squaring of the forehead, seen clinically as "frontal bossing." About 20-50% of cases have mild developmental delay (motor > > language), which nearly always resolves without therapy.* + +![Axial NECT shows classic enlargement of SAS in this macrocephalic 5-month-old patient. Note the > 5-mm widening of the bifrontal craniocortical & anterior interhemispheric SAS.](images/app.statdx.com_image_thumbnail_d638e4e6-f964-4d41-808f-5de842763d53_annotated_true_size_900_quality_90_8f53a129_20251018T164803Z.jpg) +*Axial NECT shows classic enlargement of SAS in this macrocephalic 5-month-old patient. Note the > 5-mm widening of the bifrontal craniocortical & anterior interhemispheric SAS.* + +![Axial CECT shows veins traversing the enlarged SAS.](images/app.statdx.com_image_thumbnail_d39a9497-890e-46b1-96c0-be6b83826f7c_annotated_true_size_900_quality_90_c8e6f3da_20251018T164803Z.jpg) +*Axial CECT shows veins traversing the enlarged SAS.* + +![Axial T2 MR shows veins, represented by linear flow voids , traversing the enlarged SAS.](images/app.statdx.com_image_thumbnail_227b79c7-dac6-4e19-b936-bb18a562b566_annotated_true_size_900_quality_90_750c0054_20251018T164804Z.jpg) +*Axial T2 MR shows veins, represented by linear flow voids , traversing the enlarged SAS.* + +![Coronal US shows dilated craniocortical SAS (note the space between the 2 markers) with veins traversing the SAS.](8b6b881a-7f98-409c-bb74-a593ce3bcc3c) +*Coronal US shows dilated craniocortical SAS (note the space between the 2 markers) with veins traversing the SAS.* + +![Coronal T2 MR shows markedly enlarged SAS with prominent ventricles & traversing bridging veins . Tiny, bilateral subdural collections are present .](82b8f025-0258-42d9-992c-faa46158c0e2) +*Coronal T2 MR shows markedly enlarged SAS with prominent ventricles & traversing bridging veins . Tiny, bilateral subdural collections are present .* + diff --git a/docs_md/articles/cavum-septi-pellucidi-csp_02127bd4-1efa-4056-925e-f1a1bbadf154.md b/docs_md/articles/cavum-septi-pellucidi-csp_02127bd4-1efa-4056-925e-f1a1bbadf154.md new file mode 100644 index 0000000..ef3d2df --- /dev/null +++ b/docs_md/articles/cavum-septi-pellucidi-csp_02127bd4-1efa-4056-925e-f1a1bbadf154.md @@ -0,0 +1,319 @@ +--- +title: "Cavum Septi Pellucidi (CSP)" +docid: "02127bd4-1efa-4056-925e-f1a1bbadf154" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" + - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850" + value: "Anne G. Osborn, MD, FACR" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Normal Variants" + slug: "normal-variants" + treeNodeId: "bf92256f-cdff-4bcd-8420-d876b9e4031a" + - + name: "Cavum Septi Pellucidi (CSP)" + slug: "cavum-septi-pellucidi-csp" + treeNodeId: null +category: "Brain" +documentVersionId: "1d576faa-488e-43a7-b6d4-2479d199a187" +imageCount: 11 +lastUpdated: "05/08/20" +pageDescription: "Cavum Septi Pellucidi (CSP)" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Normal Variants, Cavum Septi Pellucidi (CSP)" +pageTitle: "Cavum Septi Pellucidi (CSP) | STATdx" +enhancedTitle: "Cavum Septi Pellucidi (CSP)" +type: "DX" +references: true +cases: 2 +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Normal Variants" + - "Cavum Septi Pellucidi (CSP)" +--- +# KEY FACTS + +- ## Terminology + + + - Cystic CSF cavity of septum pellucidum (SP) + - Occurs ± cavum vergae (CV) +- ## Imaging + + + - Elongated finger-shaped CSF collection between lateral ventricles + - Cavum septi pellucidi (CSP): Between frontal horns of lateral ventricles + - CV: Posterior extension between fornices + - Size varies from slit like to several mm, occasionally > 1 cm + - SP invariably cystic in fetus + - Width of fetal CSP increases between 19-27 weeks + - Plateaus at 28 weeks + - Gradually closes in rostral direction between 28 weeks and term + - CSP present in 100% of premature, 85% of term infants + - CSP seen in up to 15-20% of adults +- ## Top Differential Diagnoses + + + - Asymmetric lateral ventricles + - Cavum velum interpositum + - Ependymal cyst + - Absent SP +- ## Pathology + + + - CSP forms if fetal SP fails to obliterate + - Precise etiology of fluid accumulation unknown + - CSP is not "5th ventricle" + - CV is not "6th ventricle" +- ## Clinical Issues + + + - Usually asymptomatic, incidental + - Headache (relationship to cyst unclear) + - CSP frequent among athletes with history of repeated traumatic brain injury, such as boxers +- ## Diagnostic Checklist + + + - CV almost never occurs without CSP + +# TERMINOLOGY + +- ## Abbreviations + + + - Cavum septi pellucidi (CSP) + - Cavum vergae (CV) +- ## Definitions + + + - Cystic CSF cavity of septum pellucidum (CSP) ± posterior continuation (CV) + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Elongated finger-shaped CSF collection between lateral ventricles + - ### Location + + + - CSP: Between frontal horns of lateral ventricles + - CV: Posterior extension between fornices + - ### Size + + + - From slit-like to several mm, occasionally > 1 cm + - ### Morphology + + + - Elongated, finger like +- ## CT Findings + + + - ### NECT + + + - CSF collection in septum pellucidum + - ### CECT + + + - Does not enhance +- ## MR Findings + + + - ### T1WI + + + - Axial: Finger-like CSF space between lateral ventricles + - Sagittal: Extends posteriorly from rostrum to splenium of corpus callosum (CC) above, ICVs below + - ### T2WI + + + - Isointense with CSF + - ### FLAIR + + + - Suppresses completely + - ### DWI + + + - Does not restrict +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Septum pellucidum invariably present in normal fetus + - Width of fetal CSP increases between 19-27 weeks + - Plateaus, then gradually closes in rostral direction between 28 weeks and term + - Inability to find CSP on fetal US requires search for CC + +# DIFFERENTIAL DIAGNOSIS + +- [Asymmetric Lateral Ventricles](/document/arachnoid-cyst/7d63f0d3-6999-4d8c-a41d-953f738c43a6) + - Septum pellucidum bowed but intact +- [Cavum Velum Interpositum](/document/arachnoid-cyst/7d63f0d3-6999-4d8c-a41d-953f738c43a6) + - Triangular shaped; no extension anterior to foramen of Monro +- [Ependymal Cyst](/document/ependymal-cyst/910da375-2150-49b5-8e0c-bc93487239d1) + - In body/atrium of lateral ventricle +- [Septooptic Dysplasia](/document/septo-optic-dysplasia/df4653b9-6e80-4f77-b737-29280f00d1ad) + - SP absent, "squared off" frontal horns + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - CSP forms if fetal septum pellucidum fails to obliterate + - Precise etiology of fluid accumulation unknown + - CSP is not "5th ventricle," nor is CV "6th ventricle" + - ### Associated abnormalities + + + - Rare: Hydrocephalus +- ## Staging, Grading, & Classification + + + - Shaw and Ellsworth classification for CSP, CV + - Asymptomatic, incidental cavum (communicating or not) + - Symptomatic, pathological, noncommunicating cavum + - Simple and uncomplicated + - Complicated by other lesions + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Usually asymptomatic, incidental + - May remain asymptomatic even if mass effect present + - Headache (relationship to cyst unclear) +- ## Demographics + + + - ### Age + + + - CSP + - Present in 100% of premature, 85% of term infants + - From 1% up to 15-20% of adults + - CV + - 100% at fetal age of 6 months, 30% at term + - < 1% of adults +- ## Natural History & Prognosis + + + - Normally regresses + - May persist as normal variant + - Rare: Enlarges, may cause mass effect +- ## Treatment + + + - Usually none + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - CV almost never occurs without CSP + + 1f464af2-e137-4e40-827c-7f61f0623628 + +## References + +# Selected References + +1. [Krejčí T et al: Symptomatic cysts of the cavum septi pellucidi, cavum vergae and cavum veli interpositi: A retrospective duocentric study of 10 patients. Clin Neurol Neurosurg. 185:105494, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31472394%5Bpmid%5D) +1. [Lee JK et al: Association of cavum septum pellucidum and cavum vergae with cognition, mood, and brain volumes in professional fighters. JAMA Neurol. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31498371%5Bpmid%5D) +1. [M Das J et al: Cavum septum pellucidum 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30725733%5Bpmid%5D) +1. [Nagaraj UD et al: Abnormalities associated with the cavum septi pellucidi on fetal MRI: What radiologists need to know. AJR Am J Roentgenol. 210(5):989-97, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29489402%5Bpmid%5D) +1. [Tsutsumi S et al: Visualization of the cavum septi pellucidi, cavum Vergae, and cavum veli interpositi using magnetic resonance imaging. Surg Radiol Anat. 40(2):159-64, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29094193%5Bpmid%5D) +1. [Gardner RC et al: Cavum septum pellucidum in retired American pro-football players. J Neurotrauma. ePub, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25970145%5Bpmid%5D) +1. [Toivonen P et al: Cavum septum pellucidum and psychopathy. Br J Psychiatry. 203(2):152-3, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23908342%5Bpmid%5D) +1. [Santo S et al: Counseling in fetal medicine: agenesis of the corpus callosum. Ultrasound Obstet Gynecol. 40(5):513-21, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23024003%5Bpmid%5D) +1. [Winter TC et al: The cavum septi pellucidi: why is it important? J Ultrasound Med. 29(3):427-44, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20194938%5Bpmid%5D) +1. [Callen PW et al: Columns of the fornix, not to be mistaken for the cavum septi pellucidi on prenatal sonography. J Ultrasound Med. 27(1):25-31, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18096727%5Bpmid%5D) +1. [Takahashi T et al: Prevalence of large cavum septi pellucidi in ultra high-risk individuals and patients with psychotic disorders. Schizophr Res. 105(1-3):236-44, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18693084%5Bpmid%5D) +1. [Needelman H et al: Postterm closure of the cavum septi pellucidi and developmental outcome in premature infants. J Child Neurol. 22(3):314-6, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17621502%5Bpmid%5D) +1. [Sencer A et al: Cerebrospinal fluid dynamics of the cava septi pellucidi and vergae. Case report. J Neurosurg. 94(1):127-9, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11147881%5Bpmid%5D) + +## Cases + +- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '66e58cd7-5239-4b71-8bd5-88312583a976', 'description': 'Axial (image 1) and sagittal (image 2) T1WIs show classic cavum septi pellucidi (CSP) and Vergae. The CSP lies in between the frontal horns of the lateral ventricles (open arrows) and the cavum Vergae (CV) (curved arrows) extends posteriorly in between the bodies of the lateral ventricles. The CSP + CV form a "cigar-shaped" CSF-containing structure that lies between the lateral ventricles. Note that on the sagittal view, the cavum occupies the entire space under the corpus callosum, flattening the fornix (arrows). This contrasts with cavum velum interpositum, which usually displaces the fornix anterosuperiorly.', 'history': 'Middle-aged patient with headaches, no neurologic findings.', 'imagePoolId': 'fbd519fb-6ab7-498c-8474-51dbaa25dd2b', 'name': 'With cavum vergae', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '15afabe5-824c-46be-9f9b-58cded699493', 'description': 'Axial T1-weighted MR scans show the classic appearance of cavum septi pellucidi (arrows) with posterior extension into a cavum Vergae (open arrows), seen here as a CSF-signal collection that lies between the bodies of the lateral ventricles.', 'history': 'Asymptomatic patient, incidental finding.', 'imagePoolId': '8d51f828-9478-4e8d-945d-09d8496552d5', 'name': 'CSP, vergae', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'c38954f4-6732-402d-ad20-5bf751eefb80', 'description': 'Sagittal T1-weighted MR scan (#1) shows the classic finding of a cavum septi pellucidi (CSP), seen here as a CSF-filled collection under the corpus callosum (open arrows) that flattens and displaces the thinned fornix (arrows). Axial image (#2) and coronal image (#3) T2WIs show the mass follows CSF in signal intensity. On the coronal scan, the thinned fornices (arrows) are barely visible. Coronal FLAIR scan (#4) shows the rounded contours of the CSP. Fluid in the CSP suppresses completely on FLAIR.', 'history': None, 'imagePoolId': 'ae79975a-0e41-4b33-ad5f-5a209538cd81', 'name': 'CSP cyst, fornix dysplasia', 'teachingPoint': None}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'ce9dcc88-81a6-4e2b-b127-03d2d2cbefe8', 'description': 'Axial NECT scans (#1, 2) show a rounded CSF-density mass in the septum pellucidum that is contiguous with an elongated CSF mass lying in-between the bodies of the lateral ventricles (arrows). This is a cavum septi pellucidi with cavum Vergae. Contrast-enhanced scans (source images from the CTA performed in this patient) show no enhancement (#3-5). \n\nThis case is a slight variant on the typical CSP + Vergae as it is somewhat rounder than usually seen. This is considered a normal variant.', 'history': 'Incidental finding.', 'imagePoolId': 'b7937706-cf34-4252-a8e0-4e96b937e7c9', 'name': 'Very round', 'teachingPoint': None, 'demographics': '30 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}, {'key': '789e84ce-9fde-4452-b27a-21d45423ac32', 'value': 'Anne Kennedy, MD, FSRU, FAIUM'}], 'caseVersionId': 'a5a4b3e1-aaa0-4d47-ae95-c458d17afb1f', 'description': 'Fetal MR (#1) and ultrasound (# 2) show absent septum pellucidum in corpus callosum agenesis. Note "Viking helmet" appearance on the coronal T2WI through the frontal and temporal horns (#1), with widely spaced parallel lateral ventricles and a high-riding third ventricle that is continuous superiorly with the interhemispheric fissure. Colpocephaly (enlarged occipital horns) is common in callosal agenesis and is seen on image #2 (arrow).', 'history': 'Routine prenatal imaging.', 'imagePoolId': '53203fa9-945d-44e0-8723-bab3d8cf207c', 'name': 'Absent CSP, CC agenesis', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'b793cb29-03cb-45cd-a54d-1805f7544044', 'description': 'A classic cavum septi pellucidi (CSP) with cavum Vergae (CV) is seen (arrows). This case is unusual because the CSP and CV are very small.', 'history': 'Incidental finding. Patient being imaged for possible intracranial metastases.', 'imagePoolId': '77f1b3ce-a427-4b7e-b075-8278f3c4e3ce', 'name': 'Very small', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'ede02258-1779-427d-9e2e-f9aeb96dfac9', 'description': 'An unusually large cavum septi pellucidi with cavum Vergae is present. Note mass effect with lateral bowing of the leaves of the septum pellucidum on the coronal scans (#3-4, arrows). Lateral/inferior displacement of the internal cerebral veins on the sagittal image (#1, open arrows) and coronal views (#3-4) (#3, open arrows) is seen. Axial scan (#2) shows lateral bowing of the fornices posteriorly (arrows). Image 4 also shows lateral displacement of the foramen of Monro (curved arrows). No obstructive hydrocephalus is present.', 'history': 'Asymptomatic patient, incidental finding.', 'imagePoolId': '80daa008-e840-43a4-82e4-b9d25b8696f1', 'name': 'Large with mass effect', 'teachingPoint': None, 'demographics': '20 Years old female'}], 'caseType': 'variant', 'name': 'VARIANT'} + + +## Images + + +### Selected Images + +![Coronal graphic with axial insert shows classic cavum septi pellucidi (CSP) with cavum vergae (CV) . Note the finger-like CSF collection between the lateral ventricles.](images/app.statdx.com_image_thumbnail_1fb3ac06-849c-4812-b789-388045765af2_annotated_true_size_900_quality_90_99777374_20251018T164843Z.jpg) +*Coronal graphic with axial insert shows classic cavum septi pellucidi (CSP) with cavum vergae (CV) . Note the finger-like CSF collection between the lateral ventricles.* + +![Coronal graphic with axial insert shows classic cavum septi pellucidi (CSP) with cavum vergae (CV) . Note the finger-like CSF collection between the lateral ventricles.](images/app.statdx.com_image_thumbnail_1fb3ac06-849c-4812-b789-388045765af2_size_174_quality_85_bf1f1833_20251018T164756Z.jpg) +*Coronal graphic with axial insert shows classic cavum septi pellucidi (CSP) with cavum vergae (CV) . Note the finger-like CSF collection between the lateral ventricles.* + +![Coronal T1 C+ SPGR MR shows a classic large CSP between the frontal horns . There is lateral bowing of the leaves of the septum pellucidum .](images/app.statdx.com_image_thumbnail_977baa81-1d68-456f-b6bc-4f9d4aceed84_annotated_true_size_900_quality_90_a6318635_20251018T164843Z.jpg) +*Coronal T1 C+ SPGR MR shows a classic large CSP between the frontal horns . There is lateral bowing of the leaves of the septum pellucidum .* + +![Axial T2 MR shows cavum septi pellucidi between the leaves of the septum pellucidum . Although seen incidentally, some studies have reported that CSP is frequent among athletes with a history of repeated traumatic brain injury (TBI), such as boxers and American professional football players.](images/app.statdx.com_image_thumbnail_a03300a7-6e69-481b-9a3b-1537f7202ca1_annotated_true_size_900_quality_90_9abe7b83_20251018T164843Z.jpg) +*Axial T2 MR shows cavum septi pellucidi between the leaves of the septum pellucidum . Although seen incidentally, some studies have reported that CSP is frequent among athletes with a history of repeated traumatic brain injury (TBI), such as boxers and American professional football players.* + +![Axial FLAIR MR shows a large CSP with CV as a large CSF collection between the leaves of the septum pellucidum continuing directly posteriorly with the CSF collection, splaying the fornices laterally .](images/app.statdx.com_image_thumbnail_9995e3f8-eb09-40bd-8ff6-c7065bb93d40_annotated_true_size_900_quality_90_fbf42288_20251018T164843Z.jpg) +*Axial FLAIR MR shows a large CSP with CV as a large CSF collection between the leaves of the septum pellucidum continuing directly posteriorly with the CSF collection, splaying the fornices laterally .* + + +### Additional Images + +![Axial NECT shows a variant of cavum septi pellucidi. Here, the CSP appears almost round .](images/app.statdx.com_image_thumbnail_4dd3811b-29d3-488c-bdd1-f8a58e630508_annotated_true_size_900_quality_90_d1b1d220_20251018T164843Z.jpg) +*Axial NECT shows a variant of cavum septi pellucidi. Here, the CSP appears almost round .* + +![Sagittal T1 C+ MR shows a large CSP/CV that extends from just behind the corpus callosum genu all the way posteriorly to the splenium. The fornices are not visible, and the internal cerebral vein is flattened .](images/app.statdx.com_image_thumbnail_effbc5cf-b18b-47ed-917e-e16bceb020a6_annotated_true_size_900_quality_90_440f151e_20251018T164843Z.jpg) +*Sagittal T1 C+ MR shows a large CSP/CV that extends from just behind the corpus callosum genu all the way posteriorly to the splenium. The fornices are not visible, and the internal cerebral vein is flattened .* + +![Coronal T1 C+ MR in the same patient shows the large CSP bowing the leaves of the septum pellucidum laterally .](images/app.statdx.com_image_thumbnail_86ce8855-4e01-4fd1-bd27-4ddfdd11bea3_annotated_true_size_900_quality_90_96ab0ca1_20251018T164843Z.jpg) +*Coronal T1 C+ MR in the same patient shows the large CSP bowing the leaves of the septum pellucidum laterally .* + +![Axial T1 MR shows a small cavum septi pellucidi with cavum vergae . Note the finger-like appearance of the CSF collection that lies between the frontal horns and bodies of the lateral ventricle.](images/app.statdx.com_image_thumbnail_80289a63-e206-480d-8e27-773285024d3e_annotated_true_size_900_quality_90_50066962_20251018T164843Z.jpg) +*Axial T1 MR shows a small cavum septi pellucidi with cavum vergae . Note the finger-like appearance of the CSF collection that lies between the frontal horns and bodies of the lateral ventricle.* + +![Axial T2 MR shows a variant of a cavum septi pellucidi with cavum vergae. Note the large CSF collection between leaves of septum pellucidum continuing directly posteriorly with the CSF collection, splaying the fornices laterally .](a9f16e39-5902-4fff-ba93-4c486599b232) +*Axial T2 MR shows a variant of a cavum septi pellucidi with cavum vergae. Note the large CSF collection between leaves of septum pellucidum continuing directly posteriorly with the CSF collection, splaying the fornices laterally .* + +![Axial T2 MR shows cavum septi pellucidi as a CSF collection between the leaves of the septum pellucidum . Although seen incidentally, some studies have reported that CSP is frequent among athletes with a history of repeated TBI, such as boxers and American professional football players.](1fd75692-6b22-492c-916e-20211f0706ea) +*Axial T2 MR shows cavum septi pellucidi as a CSF collection between the leaves of the septum pellucidum . Although seen incidentally, some studies have reported that CSP is frequent among athletes with a history of repeated TBI, such as boxers and American professional football players.* + +![Coronal T1 MR shows a classic large CSP between the frontal horns , bowing the leaves of the septum pellucidum laterally .](6e082f2e-afc6-402e-b55e-9e70eb8461d2) +*Coronal T1 MR shows a classic large CSP between the frontal horns , bowing the leaves of the septum pellucidum laterally .* + diff --git a/docs_md/articles/cavum-velum-interpositum-cvi_849ee468-35c4-46e3-9297-96196109cdb8.md b/docs_md/articles/cavum-velum-interpositum-cvi_849ee468-35c4-46e3-9297-96196109cdb8.md new file mode 100644 index 0000000..eb5e6c2 --- /dev/null +++ b/docs_md/articles/cavum-velum-interpositum-cvi_849ee468-35c4-46e3-9297-96196109cdb8.md @@ -0,0 +1,315 @@ +--- +title: "Cavum Velum Interpositum (CVI)" +docid: "849ee468-35c4-46e3-9297-96196109cdb8" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" + - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850" + value: "Anne G. Osborn, MD, FACR" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Normal Variants" + slug: "normal-variants" + treeNodeId: "bf92256f-cdff-4bcd-8420-d876b9e4031a" + - + name: "Cavum Velum Interpositum (CVI)" + slug: "cavum-velum-interpositum-cvi" + treeNodeId: null +category: "Brain" +documentVersionId: "75312185-3ddd-4300-a4fb-d824b606effc" +imageCount: 12 +lastUpdated: "05/08/20" +pageDescription: "Cavum Velum Interpositum (CVI)" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Normal Variants, Cavum Velum Interpositum (CVI)" +pageTitle: "Cavum Velum Interpositum (CVI) | STATdx" +enhancedTitle: "Cavum Velum Interpositum (CVI)" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Normal Variants" + - "Cavum Velum Interpositum (CVI)" +--- +# KEY FACTS + +- ## Terminology + + + - Cavum velum interpositum (CVI), cyst of velum interpositum (VI) +- ## Imaging + + + - Triangular CSF space + - Between lateral ventricles, over thalami + - Apex points toward foramen of Monro + - Elevates, splays fornices + - Flattens, displaces internal cerebral veins inferiorly + - Size varies from slit-like linear to triangular to round/ovoid CSF collection + - Isodense/isointense with CSF + - Suppresses completely on FLAIR + - Does not restrict on DWI + - Does not enhance + - US shows hypoechoic midline interhemispheric cyst +- ## Top Differential Diagnoses + + + - Normal cistern of velum interpositum + - Cavum septi pellucidi, cavum vergae + - Arachnoid cyst + - Epidermoid cyst +- ## Clinical Issues + + + - Can be found at any age + - Common in infants, rare in adults + - Symptoms + - Usually asymptomatic, found incidentally + - Headache (relationship to cyst unclear) + - Large CVI can obstruct normal CSF flow; treated by endoscopic fenestration +- ## Diagnostic Checklist + + + - CSF-like "cyst" could be epidermoid + - Include FLAIR and DWI to distinguish between CVI, epidermoid cyst + +# TERMINOLOGY + +- ## Abbreviations + + + - Cavum velum interpositum (CVI), cyst of velum interpositum (VI) +- ## Definitions + + + - Cystic dilation of VI + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Triangular-shaped CSF space + - Between lateral ventricles, over thalami + - Apex points toward but does not extend anteriorly beyond foramen of Monro + - Base contiguous with quadrigeminal cistern + - ### Location + + + - Midline between lateral ventricles, below fornices + - Continuous with choroid plexus subependymally + - ### Size + + + - Varies (few mm to several cm) +- ## CT Findings + + + - ### NECT + + + - Triangle of CSF between lateral ventricles + - ### CECT + + + - Does not enhance +- ## MR Findings + + + - ### T1WI + + + - Sagittal: Varies from slit-like linear to round/ovoid + - Elevates fornices + - Flattens, displaces internal cerebral veins inferiorly + - Axial: Triangle of CSF between lateral ventricles + - ### T2WI + + + - Isointense with CSF + - ### FLAIR + + + - Suppresses completely + - ### DWI + + + - Does not restrict +- ## Ultrasonographic Findings + + + - ### Color Doppler + + + - Hypoechoic midline interhemispheric cyst +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR ± contrast + - ### Protocol advice + + + - FLAIR/DWI (distinguish between CVI and epidermoid) + +# DIFFERENTIAL DIAGNOSIS + +- [Normal Cistern of Velum Interpositum](/document/pineal-cyst/eda99e5c-c992-4798-aa35-21437eb505ea) + - Usually small (slit-like or oval) + - Does not elevate fornices or depress internal cerebral veins +- ## Cavum Septi Pellucidi, Vergae + + + - CSP + CV elongated, finger-like CSF space +- [Arachnoid Cyst](/document/arachnoid-cyst/7d63f0d3-6999-4d8c-a41d-953f738c43a6) + - Lined with arachnoid (may be indistinguishable) +- [Epidermoid Cyst](/document/epidermoid-cyst/704c5ddf-e1f7-4a5d-a1b8-5b0e603170d9) + - Lobulated, insinuating mass + - Does not suppress with FLAIR; DWI shows restriction + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Pia infolds along transverse fissure, forms CSF cistern (VI) + - Cystic dilatation of VI may occur (precise etiology unknown) + - ### Associated abnormalities + + + - Usually none (large CVIs may cause hydrocephalus) +- ## Gross Pathologic & Surgical Features + + + - Normal cistern of VI is small to inapparent at autopsy + - Pial-lined CSF-filled space +- ## Microscopic Features + + + - Occasionally cysts of midline CSF spaces contain glial cells, scattered neurons + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Usually asymptomatic, found incidentally + - Headache (relationship to cyst unclear) +- ## Demographics + + + - ### Age + + + - Can be found at any age + - Common in infants, rare in adults + - ### Gender + + + - M = F +- ## Treatment + + + - Usually none + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - CSF-like "cyst" could be epidermoid + - Use DWI, FLAIR to differentiate from other cysts + + 806ab3b4-9c9a-4887-a52d-d5809cd5a417 + +## References + +# Selected References + +1. [Krejčí T et al: Symptomatic cysts of the cavum septi pellucidi, cavum vergae and cavum veli interpositi: A retrospective duocentric study of 10 patients. Clin Neurol Neurosurg. 185:105494, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31472394%5Bpmid%5D) +1. [Akinola RA et al: Caval variations in neurologically diseased patients. Acta Radiol Short Rep. 3(5):2047981614530288, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25298867%5Bpmid%5D) +1. [Tong CK et al: Endoscopic fenestration of cavum velum interpositum cysts: a case study of two symptomatic patients. Childs Nerv Syst. 28(8):1261-4, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22543434%5Bpmid%5D) +1. [Tubbs RS et al: Cavum velum interpositum, cavum septum pellucidum, and cavum vergae: a review. Childs Nerv Syst. 27(11):1927-30, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21687999%5Bpmid%5D) +1. [Tubbs RS et al: The velum interpositum revisited and redefined. Surg Radiol Anat. 30(2):131-5, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18094919%5Bpmid%5D) +1. [Osborn AG et al: Intracranial cysts: radiologic-pathologic correlation and imaging approach. Radiology. 239(3):650-64, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16714456%5Bpmid%5D) +1. [Eisenberg VH et al: Prenatal diagnosis of cavum velum interpositum cysts: significance and outcome. Prenat Diagn. 23(10):779-83, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14558018%5Bpmid%5D) +1. [Vergani P et al: Ultrasonographic differential diagnosis of fetal intracranial interhemispheric cysts. Am J Obstet Gynecol. 180(2 Pt 1):423-8, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=9988813%5Bpmid%5D) +1. [Chen CY et al: Sonographic characteristics of the cavum velum interpositum. AJNR Am J Neuroradiol. 19(9):1631-5, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9802483%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal graphic with axial insert shows a cavum velum interpositum (CVI). Note the elevation and splaying of the fornices . Also noted is the inferior displacement of the internal cerebral veins and 3rd ventricle .](images/app.statdx.com_image_thumbnail_2df127e7-04ef-413c-b33e-46703bf26fc3_annotated_true_size_900_quality_90_43c5eb31_20251018T164844Z.jpg) +*Sagittal graphic with axial insert shows a cavum velum interpositum (CVI). Note the elevation and splaying of the fornices . Also noted is the inferior displacement of the internal cerebral veins and 3rd ventricle .* + +![Sagittal graphic with axial insert shows a cavum velum interpositum (CVI). Note the elevation and splaying of the fornices . Also noted is the inferior displacement of the internal cerebral veins and 3rd ventricle .](images/app.statdx.com_image_thumbnail_2df127e7-04ef-413c-b33e-46703bf26fc3_size_174_quality_85_6f6a77c4_20251018T164756Z.jpg) +*Sagittal graphic with axial insert shows a cavum velum interpositum (CVI). Note the elevation and splaying of the fornices . Also noted is the inferior displacement of the internal cerebral veins and 3rd ventricle .* + +![Sagittal T1 MR shows a classic CVI as a CSF-like enlargement that elevates the fornix and flattens and displaces the internal cerebral vein inferiorly. These are usually asymptomatic; however, large ones can cause CSF obstruction and can be treated by fenestration.](images/app.statdx.com_image_thumbnail_bc8c67a9-f3e5-4673-a4f6-3febba3eccd9_annotated_true_size_900_quality_90_39cc402c_20251018T164845Z.jpg) +*Sagittal T1 MR shows a classic CVI as a CSF-like enlargement that elevates the fornix and flattens and displaces the internal cerebral vein inferiorly. These are usually asymptomatic; however, large ones can cause CSF obstruction and can be treated by fenestration.* + +![Axial T2 MR in a 37-year-old man with headaches shows a triangular-shaped CSF collection between the lateral ventricles, spreading the fornices laterally .](images/app.statdx.com_image_thumbnail_e9fcc6c7-e349-4a26-8cd1-1585a0da8e2e_annotated_true_size_900_quality_90_0de56902_20251018T164845Z.jpg) +*Axial T2 MR in a 37-year-old man with headaches shows a triangular-shaped CSF collection between the lateral ventricles, spreading the fornices laterally .* + +![Axial FLAIR MR in the same patient shows complete suppression of the CSF signal within the cyst similar to the lateral ventricles. Findings are consistent with a classic cavum velum interpositum. FLAIR and DWI distinguish between cavum velum interpositum and an epidermoid cyst.](images/app.statdx.com_image_thumbnail_a2c60186-2347-4c48-b827-1bae6f61ff3d_annotated_true_size_900_quality_90_f9c0e3e7_20251018T164845Z.jpg) +*Axial FLAIR MR in the same patient shows complete suppression of the CSF signal within the cyst similar to the lateral ventricles. Findings are consistent with a classic cavum velum interpositum. FLAIR and DWI distinguish between cavum velum interpositum and an epidermoid cyst.* + + +### Additional Images + +![Axial T2 MR shows a cavum septi pellucidi (CSP) along with a very small cavum velum interpositum . Note that these 2 unrelated lesions do not communicate with each other. It is common to see a CSP with a cavum vergae; it is rare to see a CSP and a CVI in the same patient.](images/app.statdx.com_image_thumbnail_8f807265-867d-45c4-a0e0-d4b5ed77ec48_annotated_true_size_900_quality_90_14754121_20251018T164844Z.jpg) +*Axial T2 MR shows a cavum septi pellucidi (CSP) along with a very small cavum velum interpositum . Note that these 2 unrelated lesions do not communicate with each other. It is common to see a CSP with a cavum vergae; it is rare to see a CSP and a CVI in the same patient.* + +![Coronal T1 C+ MR shows classic cavum velum interpositum that spreads the fornices apart.](images/app.statdx.com_image_thumbnail_52a1d3d4-3d12-4d0c-99df-2eea486bbc3b_annotated_true_size_900_quality_90_8a9e912a_20251018T164845Z.jpg) +*Coronal T1 C+ MR shows classic cavum velum interpositum that spreads the fornices apart.* + +![Axial T2 MR shows a small, triangular-shaped CSF space. Note the cavum velum interpositum interposed between the fornices and lateral ventricles. The CVI ends at the foramen of Monro.](images/app.statdx.com_image_thumbnail_1d26fd47-cf3a-49e9-b019-149ab6aa9d95_annotated_true_size_900_quality_90_3b864288_20251018T164844Z.jpg) +*Axial T2 MR shows a small, triangular-shaped CSF space. Note the cavum velum interpositum interposed between the fornices and lateral ventricles. The CVI ends at the foramen of Monro.* + +![Axial T1 MR shows a very large cavum velum interpositum. Note the splaying of fornices and anterior displacement of the septum pellucidum . Mild enlargement of the lateral ventricles is seen.](images/app.statdx.com_image_thumbnail_21237160-fb5d-46fc-8708-36027df8182c_annotated_true_size_900_quality_90_227e0506_20251018T164845Z.jpg) +*Axial T1 MR shows a very large cavum velum interpositum. Note the splaying of fornices and anterior displacement of the septum pellucidum . Mild enlargement of the lateral ventricles is seen.* + +![Sagittal T1 MR in the same patient shows anterior/superior displacement of the fornix and inferior displacement of the 3rd ventricle . The corpus callosum is elevated and thinned.](570ac479-0220-4e7c-95cf-b05fa1d08c4f) +*Sagittal T1 MR in the same patient shows anterior/superior displacement of the fornix and inferior displacement of the 3rd ventricle . The corpus callosum is elevated and thinned.* + +![Sagittal T1 MR in a 40-year-old woman with headaches shows CSF-like enlargement of the velum interpositum that elevates the fornix and flattens and displaces the internal cerebral vein inferiorly . This large CVI is probably unrelated to the patient's symptoms.](15050721-daf0-49a6-a3f3-45a4a2b17b85) +*Sagittal T1 MR in a 40-year-old woman with headaches shows CSF-like enlargement of the velum interpositum that elevates the fornix and flattens and displaces the internal cerebral vein inferiorly . This large CVI is probably unrelated to the patient's symptoms.* + +![Axial T2 MR in a 46-year-old woman with headaches shows a classic CVI with a triangular-shaped CSF collection , spreading the fornices laterally . The posterior location between the lateral ventricles is typical.](368270d4-dee5-4f8e-8b6e-47b96373ed43) +*Axial T2 MR in a 46-year-old woman with headaches shows a classic CVI with a triangular-shaped CSF collection , spreading the fornices laterally . The posterior location between the lateral ventricles is typical.* + +![Sagittal T1 MR shows a variant CVI that elevates the fornix , flattens the internal cerebral vein , and extends into the quadrigeminal and suprasellar cisterns . This case probably represents an arachnoid cyst of the cavum velum interpositum.](b6d129c7-1434-422d-91d7-83b43bc72494) +*Sagittal T1 MR shows a variant CVI that elevates the fornix , flattens the internal cerebral vein , and extends into the quadrigeminal and suprasellar cisterns . This case probably represents an arachnoid cyst of the cavum velum interpositum.* + diff --git a/docs_md/articles/corpus-callosum-impingement-syndrome_e84adf32-bae3-47d5-b368-489f413f6aea.md b/docs_md/articles/corpus-callosum-impingement-syndrome_e84adf32-bae3-47d5-b368-489f413f6aea.md new file mode 100644 index 0000000..3f2d2e5 --- /dev/null +++ b/docs_md/articles/corpus-callosum-impingement-syndrome_e84adf32-bae3-47d5-b368-489f413f6aea.md @@ -0,0 +1,305 @@ +--- +title: "Corpus Callosum Impingement Syndrome" +docid: "e84adf32-bae3-47d5-b368-489f413f6aea" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "Corpus Callosum Impingement Syndrome" + slug: "corpus-callosum-impingement-syndro-" + treeNodeId: null +category: "Brain" +documentVersionId: "2188842b-0f05-4580-9c01-5ee89586a08f" +imageCount: 4 +lastUpdated: "06/09/20" +pageDescription: "Corpus Callosum Impingement Syndrome" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Corpus Callosum Impingement Syndrome" +pageTitle: "Corpus Callosum Impingement Syndrome | STATdx" +enhancedTitle: "Corpus Callosum Impingement Syndrome" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "Corpus Callosum Impingement Syndrome" +--- +# KEY FACTS + +- ## Terminology + + + - Corpus callosum impingement syndrome (CCIS) + - Corpus callosum (CC) + - Callosal injury from longstanding obstructive hydrocephalus +- ## Imaging + + + - Acute + - Following ventricular decompression in longstanding obstructive hydrocephalus + - Swollen and hyperintense body of CC + - Diffuse or focal hyperintense areas in CC + - Chronic + - Encephalomalacic foci, shrunken and atrophic-appearing CC + - No hemorrhage, restricted diffusion, or enhancement +- ## Top Differential Diagnoses + + + - **Acute phase: CC swollen with T2/FLAIR hyperintensity** + - Tumors involving CC, tumefactive demyelination, transient cytotoxic splenial lesion, ischemia/infarct, diffuse axonal injury + - **Chronic phase: CC atrophy with cystic changes** + - Multiple sclerosis, postsurgical, enlarged perivascular spaces, Marchiafava-Bignami disease, Susac syndrome +- ## Pathology + + + - Exact mechanism for callosal lesions unknown +- ## Clinical Issues + + + - CC injury does not appear to produce any clinically recognizable symptomatology + - CCIS is uncommon sequel of severe chronic hydrocephalus + - Longstanding cases: Atrophic CC and signal abnormality, may persist after ventricular decompression +- ## Diagnostic Checklist + + + - Consider CCIS in patient treated with ventricular decompression for longstanding obstructive hydrocephalus + - CC signal change, although dramatic, should not be mistaken for other pathologies + +# TERMINOLOGY + +- ## Abbreviations + + + - Corpus callosum impingement syndrome (CCIS) + - Corpus callosum (CC) +- ## Definitions + + + - Callosal injury from longstanding obstructive hydrocephalus + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Diffuse &/or focal T2/FLAIR hyperintensity in CC + - ### Location + + + - Isthmus, body of CC, splenium generally spared + - ### Size + + + - Variable + - ### Morphology + + + - Ill-defined or focal lesions +- ## CT Findings + + + - Thinning and upward displacement of CC + - Postventricular decompression or shunting + - Acute: Swollen, hypodense body of CC + - Chronic: Atrophy, cystic changes in body of CC +- ## MR Findings + + + - ### T1WI + + + - Severe hydrocephalus with upward bowing and thinning of CC + - Following ventricular decompression, swollen hypointense body of CC + - ### T2WI + + + - Acute + - Following ventricular decompression + - Swollen, hyperintense body of CC + - Diffuse or focal hyperintense areas + - May see periventricular white matter (WM) hyperintensities + - Chronic + - Encephalomalacic foci, shrunken and atrophic-appearing CC + - ### FLAIR + + + - More sensitive to evaluate CC and periventricular WM + - ### T2* GRE + + + - No hemorrhage + - ### DWI + + + - No restricted diffusion + - ### T1WI C+ + + + - No enhancement + - DTI: Fiber disruption in body of CC +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR + - ### Protocol advice + + + - Add sagittal T2/FLAIR + +# DIFFERENTIAL DIAGNOSIS + +- ## Acute Phase: Corpus Callosum Swollen With T2/FLAIR Hyperintensity + + + - **Tumors involving CC** + - Lymphoma: Uniform enhancement, restricted diffusion + - Glioblastoma: Central necrosis, irregular enhancement + - [Tumefactive demyelination](/document/demyelinating-diseases/e3ba880e-d924-4594-a6f4-c21c5f1f0ae7) + - Often incomplete, horseshoe-shaped enhancement + - **Transient cytotoxic splenial lesion** + - Round or boomerang-shaped diffusion restriction in CC splenium, diverse etiologies + - [Ischemia/infarct](/document/acute-cerebral-ischemiainfarction/a405285f-aaea-43ca-8dc4-6f8120eaabc1) + - Diffusion restriction in CC likely due to "acute wallerian degeneration" + - [Diffuse axonal injury (DAI)](/document/diffuse-axonal-injury/c67bbb31-aea3-420f-b65f-de49bb26fcfc) + - Signal loss on SWI, ↑ T2, diffusion restriction + - **Interstitial edema (obstructive hydrocephalus)** + - ↑ T2/FLAIR signal along CC ventricular surface + - Look for additional abnormal signal of frontal and occipital periventricular WM +- ## Chronic Phase: Corpus Callosum Atrophy With Cystic Changes + + + - [Multiple sclerosis](/document/multiple-sclerosis/7892b2a2-f52a-4d7f-9858-a326f2b7ab04) + - "Burned-out" chronic lesions + - **Postsurgical** + - Small CC "holes" common after shunt + - Corpus callosotomy + - [Enlarged perivascular spaces](/document/enlarged-perivascular-spaces/58fff1ec-50b7-4caf-ab31-3341ab7044c9) + - Follow CSF on all sequences + - When CC involved, adjacent brain often involved + - [Marchiafava-Bignami disease](/document/alcoholic-encephalopathy/88021852-b73d-4cdf-a719-dd4ae3231e45) + - Rare complication of chronic alcoholism + - T2-hyperintense CC (middle layers) + - **Susac syndrome** + - Encephalopathy, visual changes, hearing loss + - Multifocal supratentorial WM lesions + CC + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Exact mechanism for callosal lesions unknown + - Mechanical compression + - Severe obstructive hydrocephalus + - CC compressed against free inferior margin of falx + - Pressure necrosis + - Ventricular decompression associated edema + - Compromised venous drainage + - Traction-induced arterial compromise, demyelination +- ## Gross Pathologic & Surgical Features + + + - Chronic impingement of CC associated with callosal thinning, cystic changes +- ## Microscopic Features + + + - Loss of callosal axons + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Signs/symptoms related to obstructive hydrocephalus + - CCIS does not appear to produce any clinically recognizable symptoms +- ## Demographics + + + - CCIS uncommon sequela of severe chronic hydrocephalus + - MR CC signal change in 8.3% of patients following shunt insertion for obstructive hydrocephalus +- ## Natural History & Prognosis + + + - Ventricular decompression may reverse CC signal change + - Longstanding cases: Atrophic CC and signal abnormality, may persist after ventricular decompression + - No long-term neurologic sequel from callosal damage +- ## Treatment + + + - None for CC signal abnormality + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - CCIS in patient treated with ventricular decompression for longstanding obstructive hydrocephalus +- ## Image Interpretation Pearls + + + - CC signal change, although dramatic, should not be mistaken for other pathologies + + ef393b1c-c6e2-4c4a-b4db-ada4a6ccd7cb + +## References + +# Selected References + +1. [Su S et al: Post-shunting corpus callosal signal change and review of the literature. J Clin Neurosci. 72:466-8, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31874812%5Bpmid%5D) +1. [Oon SF et al: Corpus callosum impingement syndrome: a callosal or colossal problem? Can J Neurol Sci. 44(6):728-9, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29391083%5Bpmid%5D) +1. [Ferrara JM: Signal hyperintensity of the callosum after ventriculoperitoneal shunting. Neurology. 84(15):1609-10, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25870451%5Bpmid%5D) +1. [Lane JI et al: Corpus callosal signal changes in patients with obstructive hydrocephalus after ventriculoperitoneal shunting. AJNR Am J Neuroradiol. 22(1):158-62, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11158902%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal T1 MR in a patient with longstanding severe obstructive hydrocephalus demonstrates markedly dilated lateral ventricle with upward displacement and thinning of the corpus callosum (CC) . Note the shunt catheter , which was placed immediately before the scan.](images/app.statdx.com_image_thumbnail_190b394f-861b-42e4-909b-125648b68eed_annotated_true_size_900_quality_90_06683e2f_20251018T160022Z.jpg) +*Sagittal T1 MR in a patient with longstanding severe obstructive hydrocephalus demonstrates markedly dilated lateral ventricle with upward displacement and thinning of the corpus callosum (CC) . Note the shunt catheter , which was placed immediately before the scan.* + +![Sagittal T1 MR in the same patient 7 days after placement of the shunt catheter shows patchy areas of low signal in the CC . Lateral ventricles are now decompressed, and there is no mass effect on the CC.](images/app.statdx.com_image_thumbnail_a662615a-810a-4a2d-830f-573e159d170a_annotated_true_size_900_quality_90_1d3bc703_20251018T160022Z.jpg) +*Sagittal T1 MR in the same patient 7 days after placement of the shunt catheter shows patchy areas of low signal in the CC . Lateral ventricles are now decompressed, and there is no mass effect on the CC.* + +![Axial FLAIR MR in the same patient 7 days following placement of the shunt catheter demonstrates ill-defined hyperintensities in the body of the CC as well as in the periventricular white matter . Note decompressed lateral and 3rd ventricles.](images/app.statdx.com_image_thumbnail_8a95800b-56d9-4311-bf1b-3fd3a6302496_annotated_true_size_900_quality_90_e12da5be_20251018T160022Z.jpg) +*Axial FLAIR MR in the same patient 7 days following placement of the shunt catheter demonstrates ill-defined hyperintensities in the body of the CC as well as in the periventricular white matter . Note decompressed lateral and 3rd ventricles.* + +![Axial FLAIR MR in the same patient 1 month after placement of the shunt shows mild decrease in the hyperintensities in the CC as well as the periventricular white matter with further decompression of the lateral and 3rd ventricles.](images/app.statdx.com_image_thumbnail_f93749c2-5c2a-43fb-9cc6-55a6f3b93079_annotated_true_size_900_quality_90_f97b7f6a_20251018T160022Z.jpg) +*Axial FLAIR MR in the same patient 1 month after placement of the shunt shows mild decrease in the hyperintensities in the CC as well as the periventricular white matter with further decompression of the lateral and 3rd ventricles.* + diff --git a/docs_md/articles/csf-shunts-and-complications_1027d634-92ff-47c1-8266-a7fc3acd1529.md b/docs_md/articles/csf-shunts-and-complications_1027d634-92ff-47c1-8266-a7fc3acd1529.md new file mode 100644 index 0000000..522d4ca --- /dev/null +++ b/docs_md/articles/csf-shunts-and-complications_1027d634-92ff-47c1-8266-a7fc3acd1529.md @@ -0,0 +1,531 @@ +--- +title: "CSF Shunts and Complications" +docid: "1027d634-92ff-47c1-8266-a7fc3acd1529" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" + - key: "99e1aff7-f42c-43a0-95ae-d89c8551aa01" + value: "Kevin R. Moore, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "CSF Shunts and Complications" + slug: "csf-shunts-and-complications" + treeNodeId: null +category: "Brain" +documentVersionId: "201dad63-bfb4-4f05-ade4-1140c667d000" +imageCount: 23 +lastUpdated: "09/24/20" +pageDescription: "CSF Shunts and Complications" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, CSF Shunts and Complications" +pageTitle: "CSF Shunts and Complications | STATdx" +enhancedTitle: "CSF Shunts and Complications" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "CSF Shunts and Complications" +--- +# KEY FACTS + +- ## Terminology + + + - Hydrocephalus (HCP) + - Enlargement of cerebral ventricles secondary to abnormal CSF formation, flow, or absorption resulting in ↑ CSF volume +- ## Imaging + + + - Shunt failure → dilated ventricles + edema around ventricles, along catheter and reservoir + - Use CT or MR to evaluate ventricle size, plain radiograph shunt series to identify mechanical shunt failure + - Baseline CT/MR following shunt insertion, follow-up at 1 year and as clinically needed + - Shunt radionuclide studies: Used to confirm distal obstruction +- ## Top Differential Diagnoses + + + - Shunt failure with normal ventricle size or lack of interstitial edema + - Noncompliant (slit) ventricle syndrome + - Acquired Chiari 1 malformation/tonsillar ectopia +- ## Pathology + + + - Obstructive HCP: Secondary to physical blockage by tumor, adhesions, cyst + - Communicating HCP: Secondary to ↓ CSF absorption across arachnoid granulations +- ## Clinical Issues + + + - Older children/adults: Headache, vomiting, lethargy, seizure, neurocognitive symptoms + - Infants: Bulging fontanelle, ↑ head circumference, irritability, lethargy +- ## Diagnostic Checklist + + + - Shunt + headache not always shunt failure + - Confirm programmable shunt valve setting after MR + - Compare current CT with prior studies to detect subtle changes in ventricle size + +# TERMINOLOGY + +- ## Abbreviations + + + - Shunt types: Ventriculoperitoneal (VP), ventriculoatrial (VA), ventriculopleural (VPL), lumboperitoneal (LP) +- ## Definitions + + + - Ventriculomegaly + - General term for enlargement of cerebral ventricles + - Hydrocephalus (HCP) + - Enlargement of cerebral ventricles secondary to abnormal CSF formation, flow, or absorption resulting in ↑ CSF volume + - Subset of ventriculomegaly + - Onset over days (acute), weeks (subacute), or months to years (chronic) + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Shunt failure: Dilated ventricles + edema ("blurring") around ventricles and along catheter, reservoir + - ### Location + + + - VP shunt common; VA and VPL used rarely unless VP contraindicated + - ### Size + + + - Ventricular size is relative → ventriculomegaly, may indicate shunt failure in one patient and be stable finding in another + - Change in ventricular size in individual patient probably significant + - Conversely, some patients manifest shunt failure with minimal to no change in ventricular size + - Distal catheter must be sized long enough to permit somatic growth, prevent retraction out of abdomen or chest + - ### Morphology + + + - Shunt system components + - Proximal catheter in ventricles, subarachnoid space, syrinx cavity, or thecal sac + - Unidirectional valve prevents reflux into ventricles + - Reservoir used to sample CSF, acutely relieve pressure + - Distal catheter tunneled through subcutaneous tissues → tip in peritoneal cavity, cardiac atrium, or pleural cavity +- ## Radiographic Findings + + + - ### Radiography + + + - Evaluate shunt catheter system integrity + - Shunt fracture, separation, migration + - Distal catheter may retract out of abdomen if significant somatic growth since shunt placement +- ## Fluoroscopic Findings + + + - Contrast shuntogram to define site of obstruction (historical interest) +- ## CT Findings + + + - ### NECT + + + - Ventricular dilatation (diffuse or loculated) + - Isolated ventricle after infection, hemorrhage → interventricular synechia + - Periventricular interstitial edema ("blurred" ventricle margins) → acute HCP + - Small, slit ventricles → noncompliant ventricle syndrome, chronic overdrainage + - ± subdural hematoma (CSF overdrainage) + - ### CECT + + + - ± ependymal enhancement (chemical or infectious ventriculitis) + - Detection of intracranial abscess or empyema as complication of shunt infection +- ## MR Findings + + + - ### T1WI + + + - Assess ventricular size, characterize brain anatomy + - ### T2WI + + + - ± interstitial periventricular edema → acute shunt failure + - ### FLAIR + + + - Interstitial edema more conspicuous than on T1WI or T2WI + - ### T2* GRE + + + - Assess hemorrhagic shunt tracts, interventricular hemorrhage + - ### DWI + + + - ↑ diffusivity with interstitial edema + - ### T1WI C+ + + + - ± enhancement with ventriculitis, abscess, neoplasm + - Pachymeningeal enhancement due to low intracranial pressure (ICP) + - ### MRA + + + - Stretched, displaced arteries around dilated ventricles secondary to ventriculomegaly + - ### MRV + + + - Venous thrombosis may precede HCP or follow shunting + - Leads to ↑ intraventricular/ICP + - ### MR cine + + + - Evaluate patency of normal CSF pathways, 3rd ventriculostomy + - ### MRS + + + - Small lactate resonances detected in CSF of up to 20% of HCP, even if HCP absent +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Useful in neonates for serial assessment of ventricular size (requires open fontanelle) + - ### Pulsed Doppler + + + - Resistive indices increase with shunt obstruction, ↑ ICP + - ### Color Doppler + + + - Research studies document flow within shunt tubing, aqueduct +- ## Nonvascular Interventions + + + - Interventricular contrast injection through shunt + NECT→ detect ventricular isolation needing additional catheter +- ## Nuclear Medicine Findings + + + - ### PET + + + - Cerebral vascular reserve (CVR) measurement may aid selection of shunt candidates + - Shunt radionuclide studies + - Radiotracer injected into shunt reservoir; serial imaging to document timing of radiotracer egress from distal catheter tip + - Used to confirm distal obstruction +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Brain NECT to assess for acute ventricular size change + - Fast MR protocols (HASTE, SSFSE) used in many centers with 24-hour MR availability to avoid cumulative radiation exposure + - ### Protocol advice + + + - Brain CT or MR to evaluate ventricle size + - Baseline CT/MR following shunt insertion, follow-up at 1 year and as clinically needed + - Plain film shunt series to identify mechanical shunt fracture or disconnection + +# DIFFERENTIAL DIAGNOSIS + +- ## Shunt Failure With Normal Ventricle Size or Lack of Interstitial Edema + + + - Look for fluid along shunt catheter or reservoir as only sign of malfunction + - May require diagnosis on clinical grounds +- ## Noncompliant (Slit) Ventricle Syndrome + + + - Usually older child (shunted in infancy) + - Small ventricles + intermittent signs of shunt obstruction + - Ventricles normal/small, even if shunt malfunctioning + - May be caused by shunt-induced sutural ossification or poor ventricular compliance +- ## Acquired Chiari 1 Malformation/Tonsillar Ectopia + + + - Functioning LP shunt produces tonsillar descent through foramen magnum + - More common with valveless systems + - Not always symptomatic + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Normal CSF production = 0.2-0.7 mL/minute; 250 (child) to 500 mL (adult) per 24-hour period + - Majority of CSF produced by choroid plexus, resorbed by arachnoid granulations + - Capacity of ventricles in healthy adult = 25 mL + - Total CSF volume (adult) = 125 mL + - Impairment of CSF circulation + - Obstructive + - Usually at narrowest points in CSF circulation (aqueduct, foramina of Monro) + - Tumor, web/synechia, congenital aqueductal stenosis + - Inadequate reabsorption across arachnoid granulations into venous sinuses + - Arachnoid granulations "clogged" after hemorrhage, inflammation + - Diminished pressure gradient from subarachnoid space to venous sinuses secondary to venous hypertension + - Impaired CSF absorption → CSF accumulation, ↑ ICP + - CSF shunt establishes accessory drainage pathway to bypass obstructed natural CSF flow pathways + - Restores or maintains normal ICP + - Each shunt, valve, device carries its own set of complications + - All types → material degradation/fatigue, mechanical stress (especially craniocervical junction, inferior ribs) + - VP → abdominal complications (CSF pseudocyst, ascites, bowel perforation) + - VPL → symptomatic pleural effusion + - VA → shunt nephritis, cor pulmonale, pulmonary embolus + - LP → arachnoiditis, cerebellar tonsillar herniation, high catheter migration rate + - Programmable shunt → unintentional reprogram during MR + - Shuntless CSF diversion → 3rd ventriculostomy, 4th ventricle outlet fenestration + - Silicone allergy → allergic response, catheter occlusion with debris + - Antisiphon devices → obstruction by capsule formation + - 1-piece shunt → ↓ catheter obstruction rate, ↑ slit ventricle/subdural hemorrhage rate + - Flanged catheters → ↑ incidence of proximal occlusion + - Internal 3rd ventricle to spinal SAS (Lapras catheter) → no external access, no method to check flow + - Flow vs. pressure regulation + - Pressure-regulating shunts prone to overdrainage + - Flow-regulating valves prone to obstruction + - Magnetic valves commonly used but cause artifact on MR and require resetting after MR + - Cerebral atrophy, focal destructive lesions also produce ↑ CSF spaces, but these are not HCP + - Loss of cerebral tissue → vacant space passively filled with CSF + - Not result of hydrodynamic disorder → not HCP + - ### Associated abnormalities + + + - Shunts placed with CSF blood/protein > 1 g/dL prone to early blockage, failure + - Shunt infection + - Ventricular loculation or isolation + - Overshunting +- ## Gross Pathologic & Surgical Features + + + - Ventricular ependymal adhesions (scar) + - Extracranial shunt tubing calcification +- ## Microscopic Features + + + - Gliosis along intracranial shunt tract + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Children, adults + - Headache, vomiting, lethargy, seizure + - Neuropsychologic, cognitive, or behavioral + - Infants + - Bulging fontanelle, ↑ head circumference, irritability, lethargy + - ### Clinical profile + + + - Depends on underlying clinical diagnosis necessitating CSF diversion, number of previous shunts, complications +- ## Demographics + + + - ### Age + + + - 1st weeks of life for myelomeningocele, congenital HCP + - Older age at 1st shunting for HCP following trauma, meningitis, tumor + - ### Epidemiology + + + - 160,000 shunts implanted each year worldwide + - CSF shunts in USA ~ 125,000 total + - 33,000 placed per year (~ 45-50% revisions) +- ## Natural History & Prognosis + + + - After shunting, 70% either normal or relatively normal intelligence (if no complications or associated anomalies) + - Epilepsy incidence up to 47% if shunt follows meningitis, hemorrhage + - HCP mortality depends on shunt complication: Malfunction (30%), infection (20%), pulmonary embolus (7%) + - Acute shunt obstruction in shunt-dependent patients may lead to death + - Majority of shunt revisions occur during first 6 months after shunt placement + - Age at time of shunt surgery, previous shunt surgery, etiology of HCP, and HCP type independently associated with incidence of shunt revision + - 50% of patients need multiple revisions, progressively shorter time interval to next failure +- ## Treatment + + + - Shunt revision + - Replace intraventricular component/valve for proximal obstruction + - Alter valve pressure setting/type if over- or underdraining + - Programmable shunt valves permit transcutaneous adjustment of pressure setting + - Lengthen distal shunt as child grows + - 3rd ventriculostomy to avoid indwelling shunt if blockage is distal to 3rd ventricle + - Subtemporal decompression or 3rd ventriculostomy for noncompliant ventricle syndrome + - Laparoscopic or open abdominal procedure for distal obstruction related to CSF pseudocyst + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Shunt + headache does not always mean shunt failure + - Consider sinusitis, trauma, sinovenous thrombosis, viral infection + - Confirm programmable shunt valve setting after MR + - Plain film shunt series has extremely low yield in absence of clinical evidence for mechanical shunt failure +- ## Image Interpretation Pearls + + + - Compare with prior studies to detect subtle ventricular size changes + - Poor ventricular compliance may prevent change in ventricular size despite florid clinical shunt failure + - Fluid tracking along shunt may be only sign of failure; possible even if ventricles normal or unchanged size + + 5eea2e07-bb80-48db-ad5d-a0c14176d17f + +## References + +# Selected References + +1. [Fowler JB et al: Ventriculoperitoneal shunt, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=29083724%5Bpmid%5D) +1. [Kaestner S et al: Revision surgery following CSF shunt insertion: how often could it be avoided? Acta Neurochir (Wien). 162(1):9-14, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31667581%5Bpmid%5D) +1. [Mohammad SA et al: The value of CSF flow studies in the management of CSF disorders in children: a pictorial review. Insights Imaging. 10(1):3, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30689061%5Bpmid%5D) +1. [Ezzat AAM et al: Migration of the distal catheter of ventriculoperitoneal shunts in pediatric age group: case series. World Neurosurg. 119:e131-7, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30031953%5Bpmid%5D) +1. [Kraemer MR et al: Overdrainage-related ependymal bands: a postulated cause of proximal shunt obstruction. J Neurosurg Pediatr. 1-11, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30117791%5Bpmid%5D) +1. [Ros B et al: Shunt overdrainage syndrome: review of the literature. Neurosurg Rev. 41(4):969-81, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=28352945%5Bpmid%5D) +1. [Hanak BW et al: Cerebrospinal fluid shunting complications in children. Pediatr Neurosurg. 52(6):381-400, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28249297%5Bpmid%5D) +1. [Rinker EK et al: CSF shunt complications: what the abdominal imager needs to know. Abdom Imaging. 40(6):2030-40, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25652954%5Bpmid%5D) +1. [Symss NP et al: Is there an ideal shunt? A panoramic view of 110 years in CSF diversions and shunt systems used for the treatment of hydrocephalus: from historical events to current trends. Childs Nerv Syst. 31(2):191-202, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25547875%5Bpmid%5D) +1. [Koktekir E et al: Resolution of papilledema after endoscopic third ventriculostomy versus cerebrospinal fluid shunting in hydrocephalus: a comparative study. J Neurosurg. 120(6):1465-70, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24678778%5Bpmid%5D) +1. [Bateman GA: Hypertensive slit ventricle syndrome: pseudotumor cerebri with a malfunctioning shunt? J Neurosurg. 119(6):1503-10, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23991845%5Bpmid%5D) +1. [Rasul FT et al: Is endoscopic third ventriculostomy superior to shunts in patients with non-communicating hydrocephalus? A systematic review and meta-analysis of the evidence. Acta Neurochir (Wien). 155(5):883-9, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23456239%5Bpmid%5D) +1. [Symss NP et al: Theories of cerebrospinal fluid dynamics and hydrocephalus: historical trend. J Neurosurg Pediatr. 11(2):170-7, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23215851%5Bpmid%5D) +1. [Robinson S: Neonatal posthemorrhagic hydrocephalus from prematurity: pathophysiology and current treatment concepts. J Neurosurg Pediatr. 9(3):242-58, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22380952%5Bpmid%5D) +1. [Sivaganesan A et al: Neuroimaging of ventriculoperitoneal shunt complications in children. Pediatr Radiol. 42(9):1029-46, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22740019%5Bpmid%5D) +1. [Chan M et al: Prediction of ventriculoperitoneal shunt dependency in patients with aneurysmal subarachnoid hemorrhage. J Neurosurg. 110(1):44-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18950263%5Bpmid%5D) +1. [Peraud A et al: Decompensated hydrocephalus causing syringomyelia and tetraparesis: a case report. Childs Nerv Syst. 25(2):263-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18979102%5Bpmid%5D) +1. [Willis B et al: Ventricular reservoirs and ventriculoperitoneal shunts for premature infants with posthemorrhagic hydrocephalus: an institutional experience. J Neurosurg Pediatr. 3(2):94-100, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19278306%5Bpmid%5D) +1. [Dusick JR et al: Success and complication rates of endoscopic third ventriculostomy for adult hydrocephalus: a series of 108 patients. Surg Neurol. 69(1):5-15, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18054606%5Bpmid%5D) +1. [Ellis MJ et al: Treatment of recurrent ventriculoperitoneal shunt failure associated with persistent cerebrospinal fluid eosinophilia and latex allergy by use of an "extracted" shunt. J Neurosurg Pediatr. 1(3):237-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18352769%5Bpmid%5D) +1. [James HE et al: Management of complicated shunt infections: a clinical report. J Neurosurg Pediatr. 1(3):223-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18352767%5Bpmid%5D) +1. [Martínez-Lage JF et al: Acute cholecystitis complicating ventriculo-peritoneal shunting: report of a case and review of the literature. Childs Nerv Syst. 24(6):777-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18365208%5Bpmid%5D) +1. [Nfonsam V et al: Laparoscopic management of distal ventriculoperitoneal shunt complications. Surg Endosc. 22(8):1866-70, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18175181%5Bpmid%5D) +1. [Riffaud L et al: Acquired Chiari I malformation and syringomyelia after valveless lumboperitoneal shunt in infancy. Pediatr Neurosurg. 44(3):229-33, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18354264%5Bpmid%5D) +1. [Liao YJ et al: Intracranial hypotension caused by leakage of cerebrospinal fluid from the thecal sac after lumboperitoneal shunt placement. Case report. J Neurosurg. 107(1):173-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17639890%5Bpmid%5D) +1. [Tseng JS et al: Motor neuron disease-like syndrome secondary to trapped fourth ventricle and obstruction of cerebrospinal fluid pathway. Clin Neurol Neurosurg. 109(4):383-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17275996%5Bpmid%5D) +1. [Woodworth G et al: Prior CSF shunting increases the risk of endoscopic third ventriculostomy failure in the treatment of obstructive hydrocephalus in adults. Neurol Res. 29(1):27-31, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17427271%5Bpmid%5D) +1. [Di Rocco C et al: Shunts vs endoscopic third ventriculostomy in infants: are there different types and/or rates of complications? A review. Childs Nerv Syst. 22(12):1573-89, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17053941%5Bpmid%5D) +1. [Winston KR et al: CSF shunt failure with stable normal ventricular size. Pediatr Neurosurg. 42(3):151-5, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16636615%5Bpmid%5D) +1. [Zamponi N et al: Bobble head doll syndrome in a child with a third ventricular cyst and hydrocephalus. Childs Nerv Syst. 21(5):350-4, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15449088%5Bpmid%5D) +1. [Arnell K et al: Distal catheter obstruction from non-infectious cause in ventriculo-peritoneal shunted children. Eur J Pediatr Surg. 14(4):245-9, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15343464%5Bpmid%5D) +1. [Blount JP et al: Sports and pediatric cerebrospinal fluid shunts: who can play? Neurosurgery. 54(5):1190-6; discussion 1196-8, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15113475%5Bpmid%5D) +1. [Fewel ME et al: Migration of distal ventriculoperitoneal shunt catheter into the heart. Case report and review of the literature. J Neurosurg. 100(2 Suppl Pediatrics):206-11, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14758952%5Bpmid%5D) +1. [Hashimoto M et al: A case of abdominal CSF pseudocyst associated with silicone allergy. Childs Nerv Syst. 20(10):761-4, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14999512%5Bpmid%5D) +1. [Sgouros S et al: An investigation of structural degradation of cerebrospinal fluid shunt valves performed using scanning electron microscopy and energy-dispersive x-ray microanalysis. J Neurosurg. 100(3):534-40, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15035291%5Bpmid%5D) +1. [Tuli S et al: Predictors of death in pediatric patients requiring cerebrospinal fluid shunts. J Neurosurg. 100(5 Suppl Pediatrics):442-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15287452%5Bpmid%5D) +1. [Villarejo FJ et al: Cerebral fluid edema: an unusual complication of ventriculoperitoneal shunts. Childs Nerv Syst. 20(3):195-8, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14749945%5Bpmid%5D) +1. [Braun KP et al: 1H magnetic resonance spectroscopy in human hydrocephalus. J Magn Reson Imaging. 17(3):291-9, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12594718%5Bpmid%5D) +1. [Krassoudakis A et al: Ventriculoperitoneal shunting complicated with cerebrospinal fluid pseudocyst and acute appendicitis. Minerva Pediatr. 54(4):321-3, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12131868%5Bpmid%5D) +1. [Oh A et al: Laparoscopic repositioning of a ventriculo-peritoneal catheter tip for a sterile abdominal cerebrospinal fluid (CSF) pseudocyst. Surg Endosc. 15(5):518, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11353974%5Bpmid%5D) +1. [Drake JM et al: CSF shunts 50 years on--past, present and future. Childs Nerv Syst. 16(10-11):800-4, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11151733%5Bpmid%5D) +1. [Tuli S et al: Risk factors for repeated cerebrospinal shunt failures in pediatric patients with hydrocephalus. J Neurosurg. 92(1):31-8, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10616079%5Bpmid%5D) +1. [Lee TT et al: Unique clinical presentation of pediatric shunt malfunction. Pediatr Neurosurg. 30(3):122-6, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10352413%5Bpmid%5D) +1. [Salomão JF et al: Abdominal pseudocysts complicating CSF shunting in infants and children. Report of 18 cases. Pediatr Neurosurg. 31(5):274-8, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10681683%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Lateral skull radiograph of acute ventriculoperitoneal (VP) shunt failure from a plain radiograph shunt series demonstrates a mechanical shunt catheter disconnection between the programmable valve and the reservoir.](images/app.statdx.com_image_thumbnail_da023baa-4d46-4fb6-8717-a85638b77ef6_annotated_true_size_900_quality_90_971d1d2d_20251018T164445Z.jpg) +*Lateral skull radiograph of acute ventriculoperitoneal (VP) shunt failure from a plain radiograph shunt series demonstrates a mechanical shunt catheter disconnection between the programmable valve and the reservoir.* + +![Lateral skull radiograph of acute ventriculoperitoneal (VP) shunt failure from a plain radiograph shunt series demonstrates a mechanical shunt catheter disconnection between the programmable valve and the reservoir.](images/app.statdx.com_image_thumbnail_da023baa-4d46-4fb6-8717-a85638b77ef6_size_174_quality_85_e8d9943b_20251018T165055Z.jpg) +*Lateral skull radiograph of acute ventriculoperitoneal (VP) shunt failure from a plain radiograph shunt series demonstrates a mechanical shunt catheter disconnection between the programmable valve and the reservoir.* + +![Axial bone CT in the same patient reveals the mechanical catheter disconnection between the reservoir and the programmable shunt valve. This finding had not appeared on the most recent comparison CT scan (not shown).](images/app.statdx.com_image_thumbnail_87340bd2-1fa0-4463-95b4-8a835e5b6f25_annotated_true_size_900_quality_90_48f77c00_20251018T164445Z.jpg) +*Axial bone CT in the same patient reveals the mechanical catheter disconnection between the reservoir and the programmable shunt valve. This finding had not appeared on the most recent comparison CT scan (not shown).* + +![AP radiograph from a shunt series demonstrates intracardiac migration of the VP shunt catheter with the tip residing in the right interlobar pulmonary artery.](images/app.statdx.com_image_thumbnail_2c7ef1d1-3464-480a-8b31-bd93676ca33f_annotated_true_size_900_quality_90_4b19d26a_20251018T164445Z.jpg) +*AP radiograph from a shunt series demonstrates intracardiac migration of the VP shunt catheter with the tip residing in the right interlobar pulmonary artery.* + +![AP radiograph of the pelvis in a 4 year old with a VP shunt who presented with left scrotal swelling demonstrates a coiled distal shunt catheter in the left scrotum. The migration of the catheter to the scrotum is due to a patent processus vaginalis.](images/app.statdx.com_image_thumbnail_54dcd3b6-9e3a-4edb-8a6d-4aa25c8efc4f_annotated_true_size_900_quality_90_b264cf48_20251018T164445Z.jpg) +*AP radiograph of the pelvis in a 4 year old with a VP shunt who presented with left scrotal swelling demonstrates a coiled distal shunt catheter in the left scrotum. The migration of the catheter to the scrotum is due to a patent processus vaginalis.* + +![Axial NECT depicts symmetric interstitial edema within the periventricular white matter. Ventricular size is significantly larger than demonstrated on a prior CT (not shown), supporting the diagnosis of acute shunt failure.](images/app.statdx.com_image_thumbnail_55b2c8c6-2eb1-42ee-b735-b6c0f9cb116d_annotated_true_size_900_quality_90_2a57efea_20251018T164445Z.jpg) +*Axial NECT depicts symmetric interstitial edema within the periventricular white matter. Ventricular size is significantly larger than demonstrated on a prior CT (not shown), supporting the diagnosis of acute shunt failure.* + +![Axial NECT in a patient with VP shunt who presented with severe headaches shows collapsed lateral ventricles . Slit ventricle syndrome presents as severe headaches due to noncompliant ventricles and should not be confused with radiologic slit ventricles.](images/app.statdx.com_image_thumbnail_650743ea-956f-46a3-93a0-873aa0cbddd1_annotated_true_size_900_quality_90_dbd0eceb_20251018T164445Z.jpg) +*Axial NECT in a patient with VP shunt who presented with severe headaches shows collapsed lateral ventricles . Slit ventricle syndrome presents as severe headaches due to noncompliant ventricles and should not be confused with radiologic slit ventricles.* + +![Coronal bone CT demonstrates fracture or disconnection of the ventricular catheter from the reservoir resulting in clinical shunt failure (larger ventricles on NECT).](images/app.statdx.com_image_thumbnail_b7a01263-8d5d-4bcd-a819-2e6b04c35eef_annotated_true_size_900_quality_90_e668546f_20251018T164445Z.jpg) +*Coronal bone CT demonstrates fracture or disconnection of the ventricular catheter from the reservoir resulting in clinical shunt failure (larger ventricles on NECT).* + +![Axial NECT following bilateral ventricular catheter placement in a patient with severe hydrocephalus (HCP) and brain atrophy reveals development of a large left subdural hematoma following VP shunting.](images/app.statdx.com_image_thumbnail_763808e4-56c2-4987-afc2-ab975e4d6708_annotated_true_size_900_quality_90_269bc444_20251018T164445Z.jpg) +*Axial NECT following bilateral ventricular catheter placement in a patient with severe hydrocephalus (HCP) and brain atrophy reveals development of a large left subdural hematoma following VP shunting.* + +![Axial NECT in a patient with HCP presenting with distal VP shunt failure shows the peritoneal catheter tip within a loculated pelvic fluid collection (CSF pseudocyst ).](images/app.statdx.com_image_thumbnail_8a8dd95c-3f84-4e93-a845-7b63366fa374_annotated_true_size_900_quality_90_0d3b647c_20251018T164445Z.jpg) +*Axial NECT in a patient with HCP presenting with distal VP shunt failure shows the peritoneal catheter tip within a loculated pelvic fluid collection (CSF pseudocyst ).* + +![Frontal cisternogram-radionuclide shuntogram examination performed after injecting the shunt valve reservoir reveals no spillage from the distal catheter after 10 minutes. Further delayed imaging (not shown) confirmed absence of spillage from the catheter, substantiating distal shunt obstruction.](images/app.statdx.com_image_thumbnail_9399b93d-ebc2-488c-a1cd-7360eecb3d04_annotated_true_size_900_quality_90_cbfc04fc_20251018T164452Z.jpg) +*Frontal cisternogram-radionuclide shuntogram examination performed after injecting the shunt valve reservoir reveals no spillage from the distal catheter after 10 minutes. Further delayed imaging (not shown) confirmed absence of spillage from the catheter, substantiating distal shunt obstruction.* + + +### Additional Images + +![Lateral radiograph from a plain film shunt series in an infant with acute shunt failure demonstrates that the ventricular catheter has pulled out of the head and is lying along the distal catheter within the scalp (tip ).](images/app.statdx.com_image_thumbnail_7fa2b6f1-07c9-45fc-9a29-d79045ac436a_annotated_true_size_900_quality_90_5a7ac107_20251018T164455Z.jpg) +*Lateral radiograph from a plain film shunt series in an infant with acute shunt failure demonstrates that the ventricular catheter has pulled out of the head and is lying along the distal catheter within the scalp (tip ).* + +![AP radiograph in a patient with chest pain after ventriculopleural (VPL) shunt placement depicts a right pneumothorax related to the shunt placement. Note the abandoned catheter fragment from a prior VP shunt system.](images/app.statdx.com_image_thumbnail_5448724e-86c6-4f45-80dc-1f1856863864_annotated_true_size_900_quality_90_1a2e5733_20251018T164455Z.jpg) +*AP radiograph in a patient with chest pain after ventriculopleural (VPL) shunt placement depicts a right pneumothorax related to the shunt placement. Note the abandoned catheter fragment from a prior VP shunt system.* + +![AP radiography indicates a large left pleural effusion in a symptomatic child with a left VPL shunt catheter .](images/app.statdx.com_image_thumbnail_2e0678e8-e16c-4859-b94f-b85ec399af77_annotated_true_size_900_quality_90_a2293a04_20251018T164458Z.jpg) +*AP radiography indicates a large left pleural effusion in a symptomatic child with a left VPL shunt catheter .* + +![Axial CECT of the pelvis in a shunted patient with HCP presenting with acute shunt failure, fever, and abdominal pain shows the peritoneal VP shunt catheter residing within a rim-enhancing pelvic fluid collection that represents a pelvic abscess secondary to perforated appendicitis.](images/app.statdx.com_image_thumbnail_fbf68223-80c5-46c7-957f-ca81995ba5d9_annotated_true_size_900_quality_90_1980ef02_20251018T164458Z.jpg) +*Axial CECT of the pelvis in a shunted patient with HCP presenting with acute shunt failure, fever, and abdominal pain shows the peritoneal VP shunt catheter residing within a rim-enhancing pelvic fluid collection that represents a pelvic abscess secondary to perforated appendicitis.* + +![Axial NECT in a patient with HCP presenting with shunt failure shows the peritoneal VP shunt catheter tip residing within a large loculated pelvic fluid collection (CSF pseudocyst).](images/app.statdx.com_image_thumbnail_d9b08852-2e8c-4bef-adcb-04e10e0e34c3_annotated_true_size_900_quality_90_3b3e0372_20251018T164459Z.jpg) +*Axial NECT in a patient with HCP presenting with shunt failure shows the peritoneal VP shunt catheter tip residing within a large loculated pelvic fluid collection (CSF pseudocyst).* + +![Axial T2 MR depicts reservoir , shunt tubing , collapsed left lateral ventricle, and dilated isolated right lateral ventricle with associated interstitial transependymal edema .](images/app.statdx.com_image_thumbnail_f7ebef35-a2fe-4035-a785-7ff1d2c028a3_annotated_true_size_900_quality_90_5f628e23_20251018T164459Z.jpg) +*Axial T2 MR depicts reservoir , shunt tubing , collapsed left lateral ventricle, and dilated isolated right lateral ventricle with associated interstitial transependymal edema .* + +![Axial NECT in a patient with posthemorrhagic HCP following contrast injection through the right ventricular catheter shows contrast within the isolated right ventricle but no contrast transit into either the left lateral or 3rd ventricle .](images/app.statdx.com_image_thumbnail_3f761b63-67ef-4fd4-9e41-cf419010a054_annotated_true_size_900_quality_90_f0a5195a_20251018T164459Z.jpg) +*Axial NECT in a patient with posthemorrhagic HCP following contrast injection through the right ventricular catheter shows contrast within the isolated right ventricle but no contrast transit into either the left lateral or 3rd ventricle .* + +![Axial FLAIR MR shows the sequelae of CSF overdrainage leading to bilateral subdural hematomas and ventricular collapse following shunt placement.](images/app.statdx.com_image_thumbnail_b3dfe4c0-3da8-447c-8f47-97a19528e184_annotated_true_size_900_quality_90_5a3db1f4_20251018T164503Z.jpg) +*Axial FLAIR MR shows the sequelae of CSF overdrainage leading to bilateral subdural hematomas and ventricular collapse following shunt placement.* + +![Axial NECT of the brain shows development of bilateral subdural hematohygromata following shunting of severe obstructive HCP.](images/app.statdx.com_image_thumbnail_9b51a0ae-8574-43c8-ad66-c9d88ace4ebb_annotated_true_size_900_quality_90_135d3f47_20251018T164503Z.jpg) +*Axial NECT of the brain shows development of bilateral subdural hematohygromata following shunting of severe obstructive HCP.* + +![Sagittal T1 C+ MR of a patient with intracranial hypotension shows obliteration of the suprasellar cistern, sagging fat midbrain with a closed angle between peduncles/pons , dural enhancement, and tonsillar descent.](images/app.statdx.com_image_thumbnail_fe076dec-96fa-4fe3-97cd-3c71abd81c09_annotated_true_size_900_quality_90_db1ac179_20251018T164503Z.jpg) +*Sagittal T1 C+ MR of a patient with intracranial hypotension shows obliteration of the suprasellar cistern, sagging fat midbrain with a closed angle between peduncles/pons , dural enhancement, and tonsillar descent.* + +![AP radiography in patient with a VP shunt and acute shunt failure reveals fractured shunt tubing .](images/app.statdx.com_image_thumbnail_32aa4174-806c-4239-819d-49f88f817fa0_annotated_true_size_900_quality_90_8ab2e3f8_20251018T164503Z.jpg) +*AP radiography in patient with a VP shunt and acute shunt failure reveals fractured shunt tubing .* + +![AP radiography shows a disconnected and caudally migrated peritoneal shunt catheter fragment looped within the pelvis.](images/app.statdx.com_image_thumbnail_0680af81-ef70-4c08-b3a5-c025ba031a43_annotated_true_size_900_quality_90_128e6046_20251018T164503Z.jpg) +*AP radiography shows a disconnected and caudally migrated peritoneal shunt catheter fragment looped within the pelvis.* + +![AP radiography shows knotted abdominal shunt tubing. The tightly coiled appearance suggests an abnormal extraperitoneal placement of the shunt. Two shunts are present due to isolated ventricles.](images/app.statdx.com_image_thumbnail_0567b9fb-c5c2-4c12-8a0d-5af29bc02aaa_annotated_true_size_900_quality_90_d05e5b19_20251018T164504Z.jpg) +*AP radiography shows knotted abdominal shunt tubing. The tightly coiled appearance suggests an abnormal extraperitoneal placement of the shunt. Two shunts are present due to isolated ventricles.* + diff --git a/docs_md/articles/demyelinating-diseases_e3ba880e-d924-4594-a6f4-c21c5f1f0ae7.md b/docs_md/articles/demyelinating-diseases_e3ba880e-d924-4594-a6f4-c21c5f1f0ae7.md new file mode 100644 index 0000000..4edeec3 --- /dev/null +++ b/docs_md/articles/demyelinating-diseases_e3ba880e-d924-4594-a6f4-c21c5f1f0ae7.md @@ -0,0 +1,399 @@ +--- +title: "Demyelinating Diseases" +docid: "e3ba880e-d924-4594-a6f4-c21c5f1f0ae7" +authors: + - key: "838e1722-2479-4fbd-a5fe-d965980a1a2c" + value: "Blaise V. Jones, MD" +breadcrumbs: + - + name: "Pediatrics" + slug: "pediatrics" + treeNodeId: "a915965c-d436-44cf-ae65-2f22e7246ea4" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "2b5cea64-a083-489e-ac0c-ec14ba059026" + - + name: "Brain" + slug: "brain" + treeNodeId: "95caa0da-bc4f-4103-8551-f58d6e415781" + - + name: "Metabolic, Infectious, and Inflammatory Disorders" + slug: "metabolic-infectious-and-inflammat-" + treeNodeId: "ed178695-cb39-40bc-981d-d0031c835a98" + - + name: "Demyelinating Diseases" + slug: "demyelinating-diseases" + treeNodeId: null +category: "Pediatrics" +cmeTopicId: "273337e9-da4e-4caa-8a31-95fa5c11a1b9" +documentVersionId: "5557de59-59d3-4508-b2dd-6913988c047d" +imageCount: 23 +lastUpdated: "11/04/21" +pageDescription: "Demyelinating Diseases" +pageKeywords: "Pediatrics, Diagnosis, Brain, Metabolic, Infectious, and Inflammatory Disorders, Demyelinating Diseases" +pageTitle: "Demyelinating Diseases | STATdx" +enhancedTitle: "Demyelinating Diseases" +type: "DX" +references: true +breadcrumbs: + - "Pediatrics" + - "Diagnosis" + - "Brain" + - "Metabolic, Infectious, and Inflammatory Disorders" + - "Demyelinating Diseases" +--- +# KEY FACTS + +- ## Diagnostic Checklist + + + - Multiple sclerosis (MS) + - Demyelinating disease characterized by multiple lesions disseminated in time & space + - Brain lesions: Multiple T2- & FLAIR MR hyperintense foci, typically small (5-10 mm), ovoid, discrete, periventricular, & perpendicular to ventricular margins + - Optic neuritis (ON): Unilateral, short segment, intraorbital; myelitis: < 2 vertebrae in length, < 50% of cross-sectional area, typically peripheral + - Acute disseminated encephalomyelitis (ADEM) + - Acute demyelinating disease with encephalopathy, without NMOSD or anti-MOG associated antibodies + - Characteristically arises subsequent to infection (viral respiratory) or vaccination + - Brain: Ill-defined, larger T2-/FLAIR hyperintense lesions + - Neuromyelitis optica spectrum disorders (NMOSD) + - Inflammatory CNS disease caused by antibodies to aquaporin-4 (AQP-4) on astrocytic end feet + - ON & transverse myelitis predominate clinically + - Brain: Commonly periventricular but parallel to ependymal lining; area postrema involvement is classic + - ON: Bilateral, posterior predominant (including chiasm) + - Myelitis: Longitudinally extensive transverse myelitis (LETM) (> 3 vertebrae), > 50% of cord cross section, central + - Anti-MOG syndromes + - Acute demyelinating disease caused by antibodies to myelin oligodendrocyte glycoprotein (MOG) + - Extensive clinical overlap with ADEM & NMOSD; encephalopathy in younger patients, ON in older + - Brain: Similar to ADEM + - ON: Bilateral anterior predominant (including optic disc) with perineural enhancement + - Myelitis: LETM vs. short segment; conus involvement + - Lyme disease + - 11% develop neurologic manifestations + - May be accompanied by ON or other CN inflammation + +# TERMINOLOGY + +- ## Definitions + + + - Acquired demyelinating processes characterized by inflammation + - Multiple sclerosis (MS) + - Demyelinating disease characterized by multiple lesions disseminated in time & space + - Acute disseminated encephalomyelitis (ADEM) + - Acute demyelinating disease with encephalopathy, without NMOSD or anti-MOG-associated antibodies + - Neuromyelitis optica spectrum disorders (NMOSD) + - Inflammatory CNS disease caused by antibodies to aquaporin-4 (AQP-4) on astrocytic end feet + - Clinically characterized by optic neuritis & transverse myelitis + - Antimyelin oligodendrocyte glycoprotein (MOG) syndromes + - Acute demyelinating disease caused by antibodies to MOG + - Extensive clinical overlap with ADEM & NMOSD + - Lyme disease + - CNS inflammation associated with *Borrelia burgdorferi* infection + +# IMAGING + +- ## General Features + + + - MS + - Brain: Multiple T2- & FLAIR MR hyperintense lesions, typically small (5-10 mm), ovoid, discrete + - > 85% are periventricular: Callosal involvement, hemispheric white matter; perpendicular to ventricle margin in perivenular distribution + - Variable enhancement: Presumed to reflect active demyelination + - Nodular, diffuse, or ring-like + - Can be mass-like: Tumefactive MS + - Diffusion restriction in acute lesions + - Diffusely abnormal ADC values + - "Black holes" (due to axonal destruction) on T1 are much more likely to be seen in MS than ADEM + - Optic neuritis (ON): Unilateral, short length, intraorbital + - Myelitis: < 2 vertebral lengths, < 50% of cord cross section, cervical > thoracic + - ADEM + - Brain: Ill-defined larger T2-/FLAIR hyperintense lesions + - Lesions are more likely to be diffuse & bilateral + - Frequent brainstem & thalamic involvement + - ON: Less common; myelitis: Less common + - NMOSD + - Brain: May have extensive lesions + - Commonly periventricular but parallel + - Dorsal brainstem (especially area postrema) + - ON: Bilateral long segment + - Posterior predominant, including chiasm + - Myelitis: Longitudinally extensive transverse myelitis (LETM) (> 3 segments), typically central & > 50% circumference + - Anti-MOG syndromes + - Brain: Similar in appearance to ADEM + - Much less likely to involve corpus callosum + - ON: Bilateral long length with perineural enhancement + - Anterior predominant, including optic disc + - Myelitis: LETM vs. short segment; conus often affected + - Lyme disease + - Presents as meningoencephalitis + - May be accompanied by ON or other cranial nerve inflammation; Bell palsy is characteristic +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR + - ### Protocol advice + + + - FLAIR MR imaging for detection + - Postcontrast FLAIR may ↑ detection of meningeal disease + - Fat-saturated, high-resolution postcontrast orbital MR for assessment of ON + - Spine imaging with contrast & axial T2-weighted sequences + +# DIFFERENTIAL DIAGNOSIS + +- [Posterior Reversible Encephalopathy Syndrome](/document/acute-hypertensive-encephalopathy--/efc6f9c2-dad9-4eb8-bad2-421bfaf1ec57) + - Subcortical vasogenic edema associated with hypertension +- [Viral Encephalitis](/document/acute-encephalitis/a45f63bb-c25b-481d-a001-9c520c58060b) + - Widely variable, but often affects white matter & deep gray nuclei +- [Autoimmune-Mediated Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0) + - Enhancing lesions spare callososeptal interface + - Reported in COVID-19 infection + - Beaded angiogram appearance +- [Leukodystrophies](/document/leukodystrophies/f4ff3738-131c-46bf-be71-1811f2c1776c) + - Patterns vary by metabolic defect + - Metachromatic leukodystrophy, Alexander disease, X-linked adrenoleukodystrophy +- ## Toxin-Induced Brain Injury + + + - Carbon monoxide or methanol poisoning + - Bilateral symmetric basal ganglia lesions + - Accompanying subcortical lesions in methanol poisoning + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - MS + - Possibly viral-incited autoimmune reaction in genetically susceptible individuals + - No "trigger" identified + - Activated T cells attack myelinated axons + - B cells, antibodies, macrophages, & microglia all contribute to lesions + - Cox-2, iNOS may cause excitotoxic death of oligodendrocytes + - ADEM + - Autoimmune-mediated demyelination + - Characteristically arises subsequent to infection (viral respiratory) or vaccination + - NMOSD + - Antibodies to aquaporin-4 (AQP-4) channels on astrocytic end feet + - Technically not demyelinating + - AQP-4 is dispersed throughout CNS + - Highly expressed in optic nerves & spinal cord + - AQP-4 antibodies are more highly expressed in peripheral blood than CSF + - Anti-MOG syndromes: Antibodies to MOG + - MOG is CNS specific protein expressed on outer surface of myelin sheath + - Lyme disease + - Caused by spirochete *Borrelia burgdorferi* + - Tick-borne disease; deer tick (Ixodes scapularis) or Western black-legged tick (Ixodes pacificus) +- ## Staging, Grading, & Classification + + + - Major clinical subtypes of MS + - Relapsing-remitting**** (85% initial presentation) + - Primary-progressive****, a.k.a. chronic progressive (5-10%) + - Progressive from start + - Secondary-progressive****, a.k.a. relapsing progressive + - By 10 years 50% & by 25 years 90% of relapsing-remitting patients enter secondary-progressive phase + - Progressive-relapsing**** + - Rare; defined as progressive disease with clear acute relapses ± full recovery + - Periods between relapses are characterized by continuing disease progression + - Clinically isolated syndrome (CIS): Single episode > 24 hours; vast majority progress to MS after number of years + - MS variants/subtypes + - Malignant****: Younger patients, febrile prodrome, clinically fulminant, death in months + - Schilder ****type ("diffuse sclerosis"): Extensive, confluent, asymmetric demyelination in bilateral supra-/infratentorial parenchyma + - Baló ****type ("concentric sclerosis"): Large lesions with alternating zones of demyelinated/myelinated white matter +- ## Gross Pathologic & Surgical Features + + + - Acute MS: Poorly delineated, yellowish-white, periventricular plaques + - Chronic MS: Gray, granular, well-demarcated plaques ± generalized volume loss +- ## Microscopic Features + + + - MS + - Perivenous demyelination & oligodendrocyte loss + - Active: Foamy macrophages with myelin fragments, lipids; reactive astrocytes + perivascular inflammation; some are hypercellular with atypical reactive astrocytes & mitoses (mimics tumor) + - Chronic: Marked loss of myelin & oligodendrocytes; dense astrogliosis; minimal/no perivascular inflammation + - Axonal transection + - CSF positive for oligoclonal bands + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - MS + - Variable + - Initially impaired/double vision of acute ON (50% with positive MR develop MS) + - Weakness, numbness, tingling, gait disturbances + - ↓ sphincter control, blindness, paralysis, dementia + - Cranial nerve palsies; usually multiple, 1-5% isolated (CNV & VI are most common) + - Spinal cord symptoms in 80% + - ADEM + - Cranial nerve palsies, encephalopathy, headache 2 days to 4 weeks after prodrome + - Seizures in 10-35% + - Monophasic illness + - Can recur in small percentage of cases (controversial) + - NMO + - Rapid onset of vision loss + - Subsequent spinal cord symptoms, paralysis + - Lyme disease + - Stereotypical expanding rash around tick bite: Erythema chronicum migrans + - Infrequently recognized + - Bell palsy, meningitis, arthralgias +- ## Demographics + + + - Estimated 2,500,000 have MS worldwide + - MS most often occurs in temperate climates + - Most common disabling CNS disease of young adults: 1 in 1,000 in Western world + - 3-5% of MS is diagnosed before age 15 years + - 20% of childhood MS is initially diagnosed as ADEM + - 1/3 of acute demyelinating disease in children is anti-MOG +- ## Natural History & Prognosis + + + - MS: 45% of MS patients are not severely affected & are nearly normal + - > 80% with "probable" MS & positive MR progress to clinically definite MS + - ADEM: Characteristically monophasic + - Recurrence suggests anti-MOG + - Anti-MOG syndromes are more frequently seen in young + - Encephalopathy is more common in younger patients, ON in older + - > 90% of NMOSD in pediatrics have relapsing disease + - Lyme disease: 11% develop neurologic manifestations +- ## Treatment + + + - MS & NMOSD are both treated with immune-modulating therapy + - ADEM is typically treated with high-dose steroids + - Alternative treatments include IVIg & plasma exchange + - Anti-MOG syndromes respond quickly to steroid & IVIg treatment + - Lyme is treated with antibiotics + - No evidence that antibiotic therapy alters natural history + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - 95% with definite MS clinically have positive MR + + 12c5cb99-14a6-4793-9a7d-a6f1c3511f06 + +## References + +# Selected References + +1. [Chhabda S et al: Relapsing demyelinating syndromes in children: a practical review of neuroradiological mimics. Front Neurol. 11:627, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32849169%5Bpmid%5D) +1. [Padilha IG et al: Pediatric multiple sclerosis: from clinical basis to imaging spectrum and differential diagnosis. Pediatr Radiol. 50(6):776-92, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31925460%5Bpmid%5D) +1. [Bulut E et al: Brain MRI findings in pediatric-onset neuromyelitis optica spectrum disorder: challenges in differentiation from acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 40(4):726-31, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30846436%5Bpmid%5D) +1. [Galardi MM et al: Differential diagnosis of pediatric multiple sclerosis. Children (Basel). 6(6), 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31163654%5Bpmid%5D) +1. [Lana-Peixoto MA et al: Neuromyelitis optica spectrum disorder and anti-MOG syndromes. Biomedicines. 7(2), 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31212763%5Bpmid%5D) +1. [Troxell RM et al: Atypical pediatric demyelinating diseases of the central nervous system. Curr Neurol Neurosci Rep. 19(12):95, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31773416%5Bpmid%5D) +1. [Reich DS et al: Multiple sclerosis. N Engl J Med. 378(2):169-80, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29320652%5Bpmid%5D) +1. [Berzero G et al: Diagnosis and therapy of acute disseminated encephalomyelitis and its variants. Expert Rev Neurother. 16(1):83-101, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26620160%5Bpmid%5D) +1. [Faguy K: Multiple sclerosis: an update. Radiol Technol. 87(5):529-50, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27146176%5Bpmid%5D) +1. [Filippi M et al: MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines. Lancet Neurol. 15(3):292-303, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26822746%5Bpmid%5D) +1. [Borchers AT et al: Lyme disease: a rigorous review of diagnostic criteria and treatment. J Autoimmun. 57:82-115, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25451629%5Bpmid%5D) +1. [Koelman DL et al: Acute disseminated encephalomyelitis: current controversies in diagnosis and outcome. J Neurol. 262(9):2013-24, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25761377%5Bpmid%5D) +1. [Wingerchuk DM et al: International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 85(2):177-89, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26092914%5Bpmid%5D) +1. [Flanagan EP et al: Neuromyelitis optica spectrum disorders. Curr Neurol Neurosci Rep. 14(9):483, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25027264%5Bpmid%5D) +1. [Ketelslegers IA et al: A comparison of MRI criteria for diagnosing pediatric ADEM and MS. Neurology. 74(18):1412-5, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20335562%5Bpmid%5D) +1. [VanLandingham M et al: An uncommon illness with a rare presentation: neurosurgical management of ADEM with tumefactive demyelination in children. Childs Nerv Syst. 26(5):655-61, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19949803%5Bpmid%5D) +1. [Calabrese M et al: Cortical lesions in primary progressive multiple sclerosis: a 2-year longitudinal MR study. Neurology. 72(15):1330-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19365054%5Bpmid%5D) +1. [Callen DJ et al: Role of MRI in the differentiation of ADEM from MS in children. Neurology. 72(11):968-73, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19038851%5Bpmid%5D) +1. [Filippi M et al: Conventional MRI in multiple sclerosis. J Neuroimaging. 17 Suppl 1:3S-9S, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17425730%5Bpmid%5D) +1. [Janardhan V et al: Multiple sclerosis: hyperintense lesions in the brain on nonenhanced T1-weighted MR images evidenced as areas of T1 shortening. Radiology. 244(3):823-31, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17690319%5Bpmid%5D) +1. [Traboulsee AL et al: The role of MRI in the diagnosis of multiple sclerosis. Adv Neurol. 98:125-46, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16400831%5Bpmid%5D) +1. [Polman CH et al: Diagnostic criteria for multiple sclerosis: 2005 revisions to the "McDonald Criteria". Ann Neurol. 58(6):840-6, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16283615%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal T2 MR in a 9-year-old with optic neuritis shows multiple ill-defined hyperintensities in the medulla & cervical cord. Subsequent serum testing revealed antibodies to aquaporin 4, confirming a diagnosis of neuromyelitis optica spectrum disorders (NMOSD).](images/app.statdx.com_image_thumbnail_9dd9f6f3-8e49-4e37-8adb-1a1245b14d28_annotated_true_size_900_quality_90_6a4e150f_20251018T152401Z.jpg) +*Sagittal T2 MR in a 9-year-old with optic neuritis shows multiple ill-defined hyperintensities in the medulla & cervical cord. Subsequent serum testing revealed antibodies to aquaporin 4, confirming a diagnosis of neuromyelitis optica spectrum disorders (NMOSD).* + +![Sagittal T2 MR in a 9-year-old with optic neuritis shows multiple ill-defined hyperintensities in the medulla & cervical cord. Subsequent serum testing revealed antibodies to aquaporin 4, confirming a diagnosis of neuromyelitis optica spectrum disorders (NMOSD).](images/app.statdx.com_image_thumbnail_9dd9f6f3-8e49-4e37-8adb-1a1245b14d28_size_174_quality_85_a98eac63_20251018T152348Z.jpg) +*Sagittal T2 MR in a 9-year-old with optic neuritis shows multiple ill-defined hyperintensities in the medulla & cervical cord. Subsequent serum testing revealed antibodies to aquaporin 4, confirming a diagnosis of neuromyelitis optica spectrum disorders (NMOSD).* + +![Axial T1 C+ FS MR through the orbits shows diffuse bilateral optic nerve enhancement in this 9-year-old with vision loss. Clinical features were suggestive of NMOSD, but CSF analysis confirmed anti-myelin oligodendrocyte glycoprotein (MOG) disease.](images/app.statdx.com_image_thumbnail_0644d2a0-6cc0-4d76-aa55-0dc02b971f87_annotated_true_size_900_quality_90_b85e5c5c_20251018T152401Z.jpg) +*Axial T1 C+ FS MR through the orbits shows diffuse bilateral optic nerve enhancement in this 9-year-old with vision loss. Clinical features were suggestive of NMOSD, but CSF analysis confirmed anti-myelin oligodendrocyte glycoprotein (MOG) disease.* + +![Axial NECT in a 16-year-old with progressive left-sided weakness after minor trauma shows a large, low-attenuation white matter lesion in the anterior right frontal lobe & a smaller one near the right motor strip .](images/app.statdx.com_image_thumbnail_be1040b7-52b7-4198-85c6-7c794b869ca0_annotated_true_size_900_quality_90_40619fe9_20251018T152401Z.jpg) +*Axial NECT in a 16-year-old with progressive left-sided weakness after minor trauma shows a large, low-attenuation white matter lesion in the anterior right frontal lobe & a smaller one near the right motor strip .* + +![Sagittal T1 C+ MR in the same patient shows the borders of the large lesion nearest to the cortex to be nonenhancing as compared to the other margins . This open ring appearance can help distinguish tumefactive MS from abscess or neoplasm (which more typically have complete ring enhancement).](images/app.statdx.com_image_thumbnail_f6c3b95c-7708-493e-adf2-d530a89cf1b3_annotated_true_size_900_quality_90_9839b7e5_20251018T152401Z.jpg) +*Sagittal T1 C+ MR in the same patient shows the borders of the large lesion nearest to the cortex to be nonenhancing as compared to the other margins . This open ring appearance can help distinguish tumefactive MS from abscess or neoplasm (which more typically have complete ring enhancement).* + + +### Additional Images + +![Sagittal graphic illustrates MS plaques involving the corpus callosum, pons, & spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.](images/app.statdx.com_image_thumbnail_5982d841-bf3c-48bf-981b-2b113c170f77_annotated_true_size_900_quality_90_2f597c2e_20251018T152401Z.jpg) +*Sagittal graphic illustrates MS plaques involving the corpus callosum, pons, & spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.* + +![Sagittal FLAIR MR shows numerous MS plaques with typical perpendicular orientation at the callososeptal interface along penetrating venules ("Dawson fingers") as well as in the subcortical white matter.](images/app.statdx.com_image_thumbnail_61781d1c-c076-4c21-b20e-880cfd4f55d0_annotated_true_size_900_quality_90_db0af7fe_20251018T152401Z.jpg) +*Sagittal FLAIR MR shows numerous MS plaques with typical perpendicular orientation at the callososeptal interface along penetrating venules ("Dawson fingers") as well as in the subcortical white matter.* + +![Sagittal FLAIR MR shows perpendicular callosal/pericallosal MS plaques with hyperintense rims & hypointense centers (with corresponding hypointensities also demonstrated on T1 as "black holes," not shown). Note an additional posterior fossa lesion .](images/app.statdx.com_image_thumbnail_401faf53-5b53-457b-a4cf-e082df97355b_annotated_true_size_900_quality_90_5f2d28b5_20251018T152401Z.jpg) +*Sagittal FLAIR MR shows perpendicular callosal/pericallosal MS plaques with hyperintense rims & hypointense centers (with corresponding hypointensities also demonstrated on T1 as "black holes," not shown). Note an additional posterior fossa lesion .* + +![Axial T1 C+ MR demonstrates multiple nodular, enhancing multiple sclerosis plaques . Note the common periventricular location with perpendicular orientation as well as the involvement of subcortical white matter.](images/app.statdx.com_image_thumbnail_189e1205-c396-4b72-b951-2dbe76e9e36e_annotated_true_size_900_quality_90_c8c44a6e_20251018T152401Z.jpg) +*Axial T1 C+ MR demonstrates multiple nodular, enhancing multiple sclerosis plaques . Note the common periventricular location with perpendicular orientation as well as the involvement of subcortical white matter.* + +![Axial FLAIR MR shows confluent multiple sclerosis plaques in commonly seen periventricular locations.](35652553-b36f-4763-b184-1ab3ab11db8d) +*Axial FLAIR MR shows confluent multiple sclerosis plaques in commonly seen periventricular locations.* + +![Axial FLAIR MR in a 9-year-old patient with altered mental status & hyperreflexia shows ill-defined, hyperintense lesions in the thalami , basal ganglia , & insula . Involvement of the deep nuclei is a relatively common feature of acute disseminated encephalomyelitis.](0e99452e-0b58-4f4c-bfac-641ed551772f) +*Axial FLAIR MR in a 9-year-old patient with altered mental status & hyperreflexia shows ill-defined, hyperintense lesions in the thalami , basal ganglia , & insula . Involvement of the deep nuclei is a relatively common feature of acute disseminated encephalomyelitis.* + +![Axial FLAIR MR shows large lesions in the thalamus & basal ganglia in this 16-year-old with a headache & weakness 2 weeks after a viral illness. Acute disseminated encephalomyelitis will frequently affect deep gray matter structures.](33c4db71-7819-4951-82d5-6179d57c46ca) +*Axial FLAIR MR shows large lesions in the thalamus & basal ganglia in this 16-year-old with a headache & weakness 2 weeks after a viral illness. Acute disseminated encephalomyelitis will frequently affect deep gray matter structures.* + +![Coronal FLAIR MR in a 12-year-old patient with neuromyelitis optica & bladder dysfunction shows large lesions extending across the corpus callosum & left cerebral peduncle .](ef83ab0b-2e3d-42bf-93c7-bca7c44ab4a8) +*Coronal FLAIR MR in a 12-year-old patient with neuromyelitis optica & bladder dysfunction shows large lesions extending across the corpus callosum & left cerebral peduncle .* + +![Axial NECT in a 14-year-old patient with vomiting shows a nonspecific, low-attenuation lesion in the left posterior frontal subcortical white matter.](df4278d1-a558-4b35-8d57-4eb498198844) +*Axial NECT in a 14-year-old patient with vomiting shows a nonspecific, low-attenuation lesion in the left posterior frontal subcortical white matter.* + +![Axial FLAIR MR in the same patient acquired the next day shows several ovoid MS plaques . Active lesions will also show contrast enhancement & restricted diffusion.](98da69ab-68a3-4e4e-a449-cc1d3d5b039c) +*Axial FLAIR MR in the same patient acquired the next day shows several ovoid MS plaques . Active lesions will also show contrast enhancement & restricted diffusion.* + +![Axial FLAIR MR in a 14-year-old with MS shows multiple ovoid lesions oriented perpendicular to the long axis of the lateral ventricles with hazy ↑ signal intensity in the white matter between them.](535bc8f9-9d5a-4a71-ace3-2096aed4a3bb) +*Axial FLAIR MR in a 14-year-old with MS shows multiple ovoid lesions oriented perpendicular to the long axis of the lateral ventricles with hazy ↑ signal intensity in the white matter between them.* + +![Axial T2 MR in a 17-year-old with Baló concentric sclerosis .](1917d677-6c61-4ff0-9e4b-e361b7735747) +*Axial T2 MR in a 17-year-old with Baló concentric sclerosis .* + +![Sagittal T1 C+ FS MR shows an enhancing MS lesion in the dorsal aspect of the cervical cord . Approximately 2/3 of spinal cord MS lesions are found in the cervical cord. Typical features include a dorsal intramedullary lesion spanning < 2 vertebral segments in length.](ea063d1c-a56a-4f8d-b816-59c489f886d3) +*Sagittal T1 C+ FS MR shows an enhancing MS lesion in the dorsal aspect of the cervical cord . Approximately 2/3 of spinal cord MS lesions are found in the cervical cord. Typical features include a dorsal intramedullary lesion spanning < 2 vertebral segments in length.* + +![Axial FLAIR MR shows numerous peripheral white matter & cortical lesions that exhibited robust contrast enhancement (not shown) in an 18-year-old woman with malignant (Marburg) MS. The patient presented with a 2-week history of behavioral changes & leg pain & died 3 weeks after presentation. The autopsy showed typical demyelinating pathology.](6037cba2-b41b-4f70-924c-eff9b9fa2adb) +*Axial FLAIR MR shows numerous peripheral white matter & cortical lesions that exhibited robust contrast enhancement (not shown) in an 18-year-old woman with malignant (Marburg) MS. The patient presented with a 2-week history of behavioral changes & leg pain & died 3 weeks after presentation. The autopsy showed typical demyelinating pathology.* + +![Axial T1 C+ FS MR in a patient with MS shows ring-enhancing masses of active demyelination. The rings of enhancement are incomplete with each ring defect pointing towards an adjacent cortex.](8bd36d87-e22a-4ecd-9c56-0c05b7d8b5e9) +*Axial T1 C+ FS MR in a patient with MS shows ring-enhancing masses of active demyelination. The rings of enhancement are incomplete with each ring defect pointing towards an adjacent cortex.* + +![Coronal T1 C+ MR shows a superficial hypointense mass in the left parasagittal posterior frontal region with a peripheral crescent of incomplete or "horseshoe" enhancement . This enhancement pattern is classic for tumefactive demyelinating disease, most commonly MS.](c0b8632c-e2e4-4a9b-8000-4d891f580d12) +*Coronal T1 C+ MR shows a superficial hypointense mass in the left parasagittal posterior frontal region with a peripheral crescent of incomplete or "horseshoe" enhancement . This enhancement pattern is classic for tumefactive demyelinating disease, most commonly MS.* + +![Axial FLAIR MR shows a case of proven tumefactive MS with extensive surrounding white matter edema . Note that the imaging features present in this case could also be seen with neoplasm.](29decf6b-f0d7-43f9-a4bf-788ed632fd56) +*Axial FLAIR MR shows a case of proven tumefactive MS with extensive surrounding white matter edema . Note that the imaging features present in this case could also be seen with neoplasm.* + +![Long TE MRS in a case of tumefactive MS reveals elevated choline , ↓ NAA , & a lactate doublet . These MRS findings could be consistent with acute demyelination & probably reflect a combination of membrane disruption, neuronal loss or dysfunction, & inflammation. Note that the MRS findings in MS are not specific. The spectral pattern of demyelination & low-grade neoplasms can be similar & should therefore be interpreted cautiously.](c16341cd-e0de-42ba-943c-f51e0344ca54) +*Long TE MRS in a case of tumefactive MS reveals elevated choline , ↓ NAA , & a lactate doublet . These MRS findings could be consistent with acute demyelination & probably reflect a combination of membrane disruption, neuronal loss or dysfunction, & inflammation. Note that the MRS findings in MS are not specific. The spectral pattern of demyelination & low-grade neoplasms can be similar & should therefore be interpreted cautiously.* + +![Axial T1 C+ MR shows numerous enhancing MS plaques that were present throughout the infratentorial & supratentorial brain. MS lesions may show homogeneous enhancement but may also exhibit ring or incomplete ring patterns of enhancement.](9818c56a-8f4e-4e75-8d5e-86a6bdb62f40) +*Axial T1 C+ MR shows numerous enhancing MS plaques that were present throughout the infratentorial & supratentorial brain. MS lesions may show homogeneous enhancement but may also exhibit ring or incomplete ring patterns of enhancement.* + diff --git a/docs_md/articles/extraventricular-obstructive-hydrocephalus_a0886d4c-f504-4165-bb52-2400e2385f68.md b/docs_md/articles/extraventricular-obstructive-hydrocephalus_a0886d4c-f504-4165-bb52-2400e2385f68.md new file mode 100644 index 0000000..d772c58 --- /dev/null +++ b/docs_md/articles/extraventricular-obstructive-hydrocephalus_a0886d4c-f504-4165-bb52-2400e2385f68.md @@ -0,0 +1,301 @@ +--- +title: "Extraventricular Obstructive Hydrocephalus" +docid: "a0886d4c-f504-4165-bb52-2400e2385f68" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "Extraventricular Obstructive Hydrocephalus" + slug: "extraventricular-obstructive-hydro-" + treeNodeId: null +category: "Brain" +documentVersionId: "244d0107-0a80-48b5-96b6-e095c226c02a" +imageCount: 9 +lastUpdated: "08/20/20" +pageDescription: "Extraventricular Obstructive Hydrocephalus" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Extraventricular Obstructive Hydrocephalus" +pageTitle: "Extraventricular Obstructive Hydrocephalus | STATdx" +enhancedTitle: "Extraventricular Obstructive Hydrocephalus" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "Extraventricular Obstructive Hydrocephalus" +--- +# KEY FACTS + +- ## Terminology + + + - Extraventricular obstructive hydrocephalus (EVOH): Enlarged ventricles due to obstruction located outside ventricular system + - Synonym: "Communicating" hydrocephalus +- ## Imaging + + + - Impaired absorption of CSF distal to 4th ventricle outlet foramina + - Ventricular size varies with duration of obstruction + - All ventricles enlarged with no intraventricular obstructive cause + - Lateral, 3rd, and 4th ventricles dilated + - ± periventricular white matter interstitial edema + - ± abnormal density/intensity of cisternal CSF ± leptomeningeal enhancement +- ## Top Differential Diagnoses + + + - Intraventricular obstructive hydrocephalus + - Ventricular enlargement 2° to parenchymal loss + - Normal-pressure hydrocephalus +- ## Pathology + + + - Hemorrhage → fibrosis/obstruction of subarachnoid space + - Most common cause of EVOH + - Other etiologies include suppurative meningitis, neoplastic or inflammatory exudates + - Subarachnoid hemorrhage (SAH), exudates may fibrose/occlude subarachnoid space, reduce CSF pulsations +- ## Clinical Issues + + + - Headache, papilledema + - Nausea, vomiting, diplopia (cranial nerve palsy) +- ## Diagnostic Checklist + + + - EVOH: Generalized ventricular enlargement with abnormal density/intensity in basal cisterns ± leptomeningeal enhancement + +# TERMINOLOGY + +- ## Abbreviations + + + - Extraventricular obstructive hydrocephalus (EVOH) +- ## Synonyms + + + - "Communicating" hydrocephalus +- ## Definitions + + + - Enlarged ventricles due to obstruction located outside ventricular system + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Lateral, 3rd, and 4th ventricles all dilated + - ± abnormal density/intensity of cisternal CSF ± leptomeningeal enhancement + - ### Location + + + - Obstruction distal to 4th ventricle outlet foramina + - ### Size + + + - Bifrontal horn to intracranial diameter ratio > 0.3 + - Temporal horn width > 3 mm + - ### Morphology + + + - All ventricles enlarged + - Generally proportionate, symmetrical increase + - No intraventricular obstructive cause +- ## CT Findings + + + - ### NECT + + + - Variable ventricular dilatation ± basal cisterns effaced + - If subarachnoid hemorrhage (SAH), look for hyperdense CSF + - ### CECT + + + - Look for sulcal/cisternal enhancement +- ## MR Findings + + + - ### T1WI + + + - "Dirty" CSF, ventricular dilatation + - ### T2WI + + + - Dilated ventricles ± periventricular white matter interstitial edema + - Effacement of cortical sulci + - Hypointense CSF-SAH, exudates + - ### FLAIR + + + - ± periventricular white matter interstitial edema better delineated than T2 + - ### T1WI C+ + + + - ± enhancement of basal cisterns/sulci + - Meningitis, carcinomatosis, etc. + - 3D CISS/FIESTA + - Exquisitely delineates CSF spaces and helps to exclude intraventricular obstruction +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR with T1WI C+ + - 3D CISS/FIESTA +- ## Other Modality Findings + + + - Isotope cisternography may show ventricular reflux, stasis (EVOH) + +# DIFFERENTIAL DIAGNOSIS + +- ## Intraventricular Obstructive Hydrocephalus + + + - Global/focal enlarged ventricles due to obstruction proximal to 4th ventricle outflow +- ## Ventricular Enlargement Secondary to Parenchymal Loss + + + - Neurodegenerative disease, cerebritis, hypoxia/ischemia + - Diffuse/focal enlargement of sulci, cisterns +- [Normal-Pressure Hydrocephalus](/document/normal-pressure-hydrocephalus/ba3f857d-58de-4f21-8463-1631b4cb9972) + - Ventricular enlargement with normal CSF pressure + - Sulci normal/minimally enlarged + - Progressive dementia, gait disturbance, incontinence + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Obstruction to CSF flow at level of basal cisterns or arachnoid villi + - Also reduced CSF pulsations reduce venous resorption of CSF + - SAH: Most common cause of EVOH + - Other etiologies include suppurative meningitis, neoplastic inflammatory exudates + - All lead to subarachnoid scarring, reduced CSF pulsations +- ## Gross Pathologic & Surgical Features + + + - Generalized ventricular dilatation + - SAH, exudates in basal cisterns & convexity sulci + - Meningeal fibrosis, arachnoid adhesions + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Headache, papilledema + - Nausea, vomiting, diplopia (cranial nerve palsy) +- ## Natural History & Prognosis + + + - Usually progressive unless shunted and primary cause treated +- ## Treatment + + + - CSF diversion (shunt); directed to primary cause + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - EVOH: Generalized ventricular enlargement with abnormal density/intensity in basal cisterns ± leptomeningeal enhancement + + 94b03593-3810-49dd-a698-ad9f46f7ba1f + +## References + +# Selected References + +1. [Capone PM et al: Neuroimaging of normal pressure hydrocephalus and hydrocephalus. Neurol Clin. 38(1):171-83, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31761057%5Bpmid%5D) +1. [Farb R et al: Hydrocephalus and CSF disorders. Diseases of the brain, head and neck, spine 2020-3: Diagnostic Imaging. Cham (CH): Springer; 11-24, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32119247%5Bpmid%5D) +1. [Fowler JB et al: Ventriculoperitoneal shunt. Treasure Island (FL): StatPearls Publishing, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=29083724%5Bpmid%5D) +1. [Roth J et al: The added value of magnetic resonance imaging cisternography and ventriculography as a diagnostic aid in pediatric Hydrocephalus. Pediatr Neurosurg. 54(3):165-72, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30870836%5Bpmid%5D) +1. [Fink KR et al: Imaging of nontraumatic neuroradiology emergencies. Radiol Clin North Am. 53(4):871-90, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26046515%5Bpmid%5D) +1. [Flannery AM et al: Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 1: introduction and methodology. J Neurosurg Pediatr. 14 Suppl 1:3-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25988777%5Bpmid%5D) +1. [Grunwald IQ et al: Aneurysmal SAH: current management and complications associated with treatment and disease. J Invasive Cardiol. 26(1):30-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24402809%5Bpmid%5D) +1. [Krähenbühl AK et al: Endoscopic temporal ventriculocisternostomy: an option for the treatment of trapped temporal horns. J Neurosurg Pediatr. 11(5):568-74, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23521153%5Bpmid%5D) +1. [McAllister JP 2nd: Pathophysiology of congenital and neonatal hydrocephalus. Semin Fetal Neonatal Med. 17(5):285-94, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22800608%5Bpmid%5D) +1. [Feng F et al: Evaluation of radionuclide cerebrospinal fluid scintigraphy as a guide in the management of patients with hydrocephalus. Clin Imaging. 33(2):85-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19237049%5Bpmid%5D) +1. [Yamada S et al: Visualization of cerebrospinal fluid movement with spin labeling at MR imaging: preliminary results in normal and pathophysiologic conditions. Radiology. 249(2):644-52, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18936318%5Bpmid%5D) +1. [Greitz D: Paradigm shift in hydrocephalus research in legacy of Dandy's pioneering work: rationale for third ventriculostomy in communicating hydrocephalus. Childs Nerv Syst. 23(5):487-9, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=-1%5Bpmid%5D) +1. [ter Laan M et al: Improvement after treatment of hydrocephalus in aneurysmal subarachnoid haemorrhage: implications for grading and prognosis. Acta Neurochir (Wien). 148(3):325-8; discussion 328, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16328775%5Bpmid%5D) +1. [Greitz D: Radiological assessment of hydrocephalus: new theories and implications for therapy. Neurosurg Rev. 27(3):145-65; discussion 166-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15164255%5Bpmid%5D) +1. [Kehler U et al: Extraventricular intracisternal obstructive hydrocephalus--a hypothesis to explain successful 3rd ventriculostomy in communicating hydrocephalus. Pediatr Neurosurg. 38(2):98-101, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12566844%5Bpmid%5D) +1. [Biedert S et al: [Extraventricular obstructive hydrocephalus.] Fortschr Neurol Psychiatr. 62(11):405-8, 1994](http://www.ncbi.nlm.nih.gov/pubmed/?term=7829027%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and early extraventricular obstructive hydrocephalus with dilatation of all the ventricles and subtle periventricular hypodensity due to interstitial edema.](images/app.statdx.com_image_thumbnail_5111b0b3-10b0-4451-b80f-787287add3e4_annotated_true_size_900_quality_90_52e5b3da_20251018T164521Z.jpg) +*Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and early extraventricular obstructive hydrocephalus with dilatation of all the ventricles and subtle periventricular hypodensity due to interstitial edema.* + +![Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and early extraventricular obstructive hydrocephalus with dilatation of all the ventricles and subtle periventricular hypodensity due to interstitial edema.](images/app.statdx.com_image_thumbnail_5111b0b3-10b0-4451-b80f-787287add3e4_size_174_quality_85_07f2e641_20251018T155131Z.jpg) +*Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and early extraventricular obstructive hydrocephalus with dilatation of all the ventricles and subtle periventricular hypodensity due to interstitial edema.* + +![Axial FLAIR MR images (top) in a patient with breast carcinoma leptomeningeal metastasis shows dilatation of the ventricles with mild periventricular interstitial edema . Axial T1 C+ MR (bottom) shows extensive leptomeningeal enhancement along the cerebellar folia.](images/app.statdx.com_image_thumbnail_d998cd4d-9969-40a5-af8f-4530c1fa1cf0_annotated_true_size_900_quality_90_19eb7d4c_20251018T164521Z.jpg) +*Axial FLAIR MR images (top) in a patient with breast carcinoma leptomeningeal metastasis shows dilatation of the ventricles with mild periventricular interstitial edema . Axial T1 C+ MR (bottom) shows extensive leptomeningeal enhancement along the cerebellar folia.* + +![Axial T2 MR in a 21-year-old patient with a remote history of meningitis shows chronic "compensated" extraventricular communicating hydrocephalus with marked dilatation of the lateral and 3rd ventricles .](images/app.statdx.com_image_thumbnail_99b1acc6-b089-48c5-bc97-c72d4bd09eb0_annotated_true_size_900_quality_90_dc39d287_20251018T164521Z.jpg) +*Axial T2 MR in a 21-year-old patient with a remote history of meningitis shows chronic "compensated" extraventricular communicating hydrocephalus with marked dilatation of the lateral and 3rd ventricles .* + +![Sagittal T1 MR in the same patient shows a patent widened cerebral aqueduct and foramen of Magendie with dilatation of the 4th ventricle . In longstanding "compensated" hydrocephalus, there is no periventricular interstitial edema around the ventricles, as in this case.](images/app.statdx.com_image_thumbnail_92b29be1-65a5-4179-9175-265ac3a16e7b_annotated_true_size_900_quality_90_3b9fecbf_20251018T164521Z.jpg) +*Sagittal T1 MR in the same patient shows a patent widened cerebral aqueduct and foramen of Magendie with dilatation of the 4th ventricle . In longstanding "compensated" hydrocephalus, there is no periventricular interstitial edema around the ventricles, as in this case.* + + +### Additional Images + +![Axial T1WI C+ MR demonstrates subtle leptomeningeal enhancement in the left sylvian fissure in this patient with tuberculous meningitis. There is mild dilatation of the lateral ventricles due to extraventricular obstructive hydrocephalus.](images/app.statdx.com_image_thumbnail_2a43dda8-e5ae-43ec-94c6-0f57c803a451_annotated_true_size_900_quality_90_1b106673_20251018T164521Z.jpg) +*Axial T1WI C+ MR demonstrates subtle leptomeningeal enhancement in the left sylvian fissure in this patient with tuberculous meningitis. There is mild dilatation of the lateral ventricles due to extraventricular obstructive hydrocephalus.* + +![Coronal T1WI MR in a toddler with rapid head growth for 4 months shows enlarged ventricular trigone on the left and enlarging subarachnoid spaces at an age when they should be shrinking. MR venography showed occlusion of both transverse sinuses.](images/app.statdx.com_image_thumbnail_ebff970c-aa3e-4179-bbd9-c97acd72f056_annotated_true_size_900_quality_90_c2d1d8f1_20251018T164521Z.jpg) +*Coronal T1WI MR in a toddler with rapid head growth for 4 months shows enlarged ventricular trigone on the left and enlarging subarachnoid spaces at an age when they should be shrinking. MR venography showed occlusion of both transverse sinuses.* + +![Axial NECT shows hyperdense material in the basal cisterns and sylvian fissures in acute subarachnoid hemorrhage. There is early dilatation of the ventricles with mild periventricular edema due to interstitial edema.](images/app.statdx.com_image_thumbnail_8ec2b8fa-6b76-4d89-b2ab-6f1f7e0a4d3c_annotated_true_size_900_quality_90_9e79e2bf_20251018T164521Z.jpg) +*Axial NECT shows hyperdense material in the basal cisterns and sylvian fissures in acute subarachnoid hemorrhage. There is early dilatation of the ventricles with mild periventricular edema due to interstitial edema.* + +![Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and sylvian fissures . There is early extraventricular obstructive hydrocephalus with mild periventricular hypodensity due to interstitial edema.](images/app.statdx.com_image_thumbnail_da3cea4c-f283-4f12-b661-5b1c2df2b201_annotated_true_size_900_quality_90_8c48b911_20251018T164521Z.jpg) +*Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and sylvian fissures . There is early extraventricular obstructive hydrocephalus with mild periventricular hypodensity due to interstitial edema.* + +![Axial T1WI C+ MR shows extensive leptomeningeal enhancement of the basal cisterns in neurosarcoidosis . Notice the early communicating hydrocephalus with the dilated 3rd ventricle and temporal horns .](f314f067-37d3-4bfe-b551-1dd1cd36f1e3) +*Axial T1WI C+ MR shows extensive leptomeningeal enhancement of the basal cisterns in neurosarcoidosis . Notice the early communicating hydrocephalus with the dilated 3rd ventricle and temporal horns .* + diff --git a/docs_md/articles/hydrocephalus_e9481739-278e-4682-ab1e-4326a77c3d0c.md b/docs_md/articles/hydrocephalus_e9481739-278e-4682-ab1e-4326a77c3d0c.md new file mode 100644 index 0000000..5a1c664 --- /dev/null +++ b/docs_md/articles/hydrocephalus_e9481739-278e-4682-ab1e-4326a77c3d0c.md @@ -0,0 +1,480 @@ +--- +title: "Hydrocephalus" +docid: "e9481739-278e-4682-ab1e-4326a77c3d0c" +authors: + - key: "838e1722-2479-4fbd-a5fe-d965980a1a2c" + value: "Blaise V. Jones, MD" +breadcrumbs: + - + name: "Pediatrics" + slug: "pediatrics" + treeNodeId: "a915965c-d436-44cf-ae65-2f22e7246ea4" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "2b5cea64-a083-489e-ac0c-ec14ba059026" + - + name: "Pediatric Neuroradiology" + slug: "pediatric-neuroradiology" + treeNodeId: "d0eb8f4a-e769-43dd-896c-8c9c27ce8759" + - + name: "Brain" + slug: "brain" + treeNodeId: "feaaadba-649b-4f0a-9aad-9188a8f9926a" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "0c0853dc-8217-425b-86d1-2d958e17f1f9" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "8b535c75-9cd3-445e-a4c7-345b2e444f03" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: null +category: "Pediatrics" +cmeTopicId: "bddbd7cd-39c8-4b51-98d3-3e314108c4d1" +documentVersionId: "47d2ee1e-6049-4ddc-8280-fc06e5d281da" +imageCount: 25 +lastUpdated: "02/06/24" +pageDescription: "Hydrocephalus" +pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus" +pageTitle: "Hydrocephalus | STATdx" +enhancedTitle: "Hydrocephalus" +type: "DX" +references: true +breadcrumbs: + - "Pediatrics" + - "Diagnosis" + - "Pediatric Neuroradiology" + - "Brain" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" +--- +# KEY FACTS + +- ## Terminology + + + - Ventriculomegaly caused by + - Obstruction of CSF egress from ventricles: Intraventricular obstructive hydrocephalus + - Decreased CSF resorption from subarachnoid space: Extraventricular obstructive hydrocephalus + - Increased CSF production + - Use FOHR to track ventricle size over time + - (Transverse diameter of frontal horns + transverse diameter of occipital horns) ÷ 2x transverse diameter of cranium +- ## Imaging + + + - 3D FIESTA/CISS + - Acquire in sagittal plane to outline aqueduct + - Limited shunt MR protocol + - 30-second acquisition in each plane + - Useful substitute for CT + - Insensitive for parenchymal abnormalities +- ## Pathology + + + - Extracellular, extravascular fluid in brain is managed by glymphatic system + - CSF circulates from within ventricles → subarachnoid space → perivascular spaces ↔ interstitium ↔ perivenular spaces → subarachnoid space + - CSF is resorbed from subarachnoid space at multiple sites + - Along sheaths of cranial nerves (especially olfactory bulb) into head and neck lymphatics + - Along sheaths of spinal nerves into perispinal lymphatics + - Into meningeal (dural) lymphatics + - Into dural sinuses via arachnoid granulations + - Previously thought to be major site of resorption + - Hydrocephalus results from obstruction of CSF egress from ventricular system or reduced resorption from subarachnoid space + +# TERMINOLOGY + +- ## Abbreviations + + + - Intraventricular obstructive hydrocephalus (IVOH) + - Acute IVOH (aIVOH) + - Chronic "compensated" IVOH (cIVOH) + - Extraventricular obstructive hydrocephalus (EVOH) + - Frontal occipital horn ratio (FOHR) + - (Transverse diameter of frontal horns + transverse diameter of occipital horns) ÷ 2x transverse diameter of cranium + - Subarachnoid lymphatic-like membrane (SLYM) + - Recently discovered 4th meningeal membrane that divides subarachnoid space into inner and outer compartments +- ## Definitions + + + - Ventriculomegaly caused by + - Obstruction of CSF egress from ventricles: IVOH + - Decreased CSF resorption from subarachnoid space: EVOH + - Increased CSF production + - Ventriculomegaly secondary to loss of parenchyma, a.k.a. ex vacuo dilation, is **not**hydrocephalus + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Enlarged ventricles + - With decreased extraaxial spaces (EAS): IVOH + - With periventricular (transependymal) edema: Acute IVOH + - With enlarged EAS: EVOH + - ### Size + + + - FOHR > 0.33 + - Temporal horn width > 3 mm + - ### Morphology + + + - Ventricles proximal to obstruction enlarge, appear more rounded + - Trigones and occipital horns typically enlarge most + - Wall pressure is proportional to diameter: Laplace law + - Optic and infundibular recesses of 3rd ventricle may preferentially enlarge + - Optic nerve sheaths may enlarge → papilledema +- ## CT Findings + + + - ### NECT + + + - Large ventricles proximal to obstruction + - aIVOH + - "Ballooned" ventricles with periventricular low-density "halo" + - cIVOH + - "Ballooned" ventricles, periventricular "halo" + - Basal cisterns, sulci compressed/obliterated +- ## MR Findings + + + - ### T1WI + + + - Lateral ventricles enlarged + - Corpus callosum (CC) thinned, stretched upward + - May be impinged against falx + - Impaction may cause pressure necrosis + - Fornix, internal cerebral veins (ICV) displaced downward + - Enlarged 3rd ventricle often herniated into expanded sella + - Funnel-shaped aqueduct of Sylvius in aqueductal stenosis + - ### T2WI + + + - Acute obstruction + - Transependymal edema extends into periventricular white matter (WM) + - Accentuated around frontal and occipital horns + - Chronic obstruction + - Large ventricles without transependymal edema + - CC may show hyperintensity after decompression (15% of shunted IVOH cases) + - ### T1WI C+ + + + - Diffuse leptomeningeal disease can cause EVOH + - May only be apparent on postcontrast imaging + - ### MRS + + + - Small lactate resonances can be detected in up to 20% of CSF spaces, even if no hydrocephalus +- ## Other Modality Findings + + + - Contrast-enhanced ventriculography + - MR/CT used to identify site of obstruction, status of 3rd ventriculostomies + - MR can be used for assessing CSF flow + - Cardiac-gated phase-contrast MR + - May show loss of CSF flow through aqueduct + - Useful for assessing status of endoscopic 3rd ventriculostomy (ETV) +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Sagittal T2WI MR + - Administer contrast if pattern suggests EVOH and no etiology is apparent + - ### Protocol advice + + + - 3D FIESTA/CISS + - Acquire in sagittal plane to outline aqueduct + - Decreases CSF flow artifact + - Allows better delineation of ventricular contour, septa + - SWI to assess for superficial siderosis in posthemorrhagic hydrocephalus + - Limited shunt MR protocol + - Single-shot rapid technique with heavy T2 weighting + - 30-second acquisition in each plane + - Useful substitute for CT + - Reduce radiation exposure in frequently imaged population + - Rapid aquisition reduces need for sedation + - Insensitive for parenchymal abnormalities + - Use FOHR to track ventricle size over time + - Especially in younger children with open sutures + - Accounts for increase in head circumference with increase in ventricular size + +# DIFFERENTIAL DIAGNOSIS + +- ## Ventricular Enlargement Secondary to Parenchymal Loss + + + - a.k.a. ex vacuo ventriculomegaly + - Age related (ventricular volume increases 1.2-1.4 mL after 60 years) + - Ischemia/infarction, trauma, infection, toxic + - Obtuse frontal angle (> 110°) + - Associated enlargement of sulci, cisterns + - Normal lateral ventricles can be asymmetric +- ## Benign Enlargement of Subarachnoid Spaces and Ventricles + + + - a.k.a. benign macrocrania + - Seen in association with macrocephaly in infants + - Transient and self-limited + - Not associated with developmental delay + - Does not require CSF diversion + - Likely reflects relative immaturity of glymphatic system + - Diminished ability to resorb CSF as interstitial fluid compartment is shrunk by progressive myelination + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Extracellular, extravascular fluid in brain is managed by glymphatic system + - Maintains homeostatic balance between interstitial fluid compartment, cellular compartment, and intravascular compartment + - Analogous to lymphatic system in other organs + - While brain parenchyma does not have lymphatic vessels, they are present in dura mater and along sheaths of cranial nerves + - CSF circulates from within ventricles → subarachnoid space → perivascular spaces ↔ interstitium ↔ perivenular spaces → subarachnoid space + - CSF is resorbed from subarachnoid space at multiple sites + - Along sheaths of cranial nerves (especially olfactory bulb) into head and neck lymphatics + - Along sheaths of spinal nerves into perispinal lymphatics + - Into meningeal (dural) lymphatics + - Into dural sinuses via arachnoid granulations + - Previously thought to be major site of resorption + - SLYM is thought to facilitate CSF flow + - Disruption after trauma may explain reduced glymphatic flow and posttraumatic cerebral edema + - Normal CSF production = 0.2-0.35 mL/min + - Capacity of lateral, 3rd ventricles in adult = 20 mL + - Total volume of CSF in adult = 120 mL + - Hydrocephalus results from obstruction of CSF egress from ventricular system or reduced resorption from subarachnoid space + - Obstruction within ventricular system results in IVOH + - Ventricles expand, compress adjacent parenchyma; stretching may rupture/open ependymal cell junctions + - Periventricular interstitial fluid increases → myelin destruction + - Etiology depends on site + - Foramen of Monro + - Colloid cyst + - Subependymal giant cell astrocytoma in tuberous sclerosis + - 3rd ventricle + - Craniopharyngioma, hypothalamic glioma, arachnoid cyst + - Aqueduct of Sylvius + - Aqueductal stenosis, tectal glioma, pineal region tumor + - Obstruction by hemorrhage or inflammatory debris + - Enlarged vein of Galen due to arteriovenous fistula + - 4th ventricle + - Medulloblastoma, ependymoma, pilocytic astrocytoma + - Chiari, Dandy-Walker malformation, Blake pouch cyst + - Metastasis, neurocysticercosis, or meningioma can occur at multiple intraventricular locations + - Reduced resorption from subarachnoid space results in EVOH + - Subarachnoid pathology may reduce absorptive capacity + - Hemorrhage or inflammation (acute or chronic) + - Metabolic disorders may reduce resorptive capacity + - Overproduction of CSF may overwhelm ability of glymphatic system to manage and resorb CSF + - Choroid plexus papilloma or carcinoma + - Focally enlarged and hyperenhancing choroidal mass + - Choroid plexus villous hyperplasia + - Diffusely enlarged choroid + - ### Genetics + + + - Cell adhesion molecule L1 (*L1CAM*) recognized as cause of X-linked aqueductal stenosis + - Located on X chromosome (Xq28) +- ## Gross Pathologic & Surgical Features + + + - Focal/generalized ventricular enlargement + - Ependyma, adjacent WM are secondarily injured + - Variable pathology depending on causative factor + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Headache, papilledema (aIVOH) + - Nausea, vomiting + - Diplopia + - 6th nerve palsy caused by compression of cisternal segment + - ### Clinical profile + + + - Varies with etiology, severity, age of onset +- ## Demographics + + + - ### Age + + + - May be any age from in utero (congenital hydrocephalus) to adult + - ### Epidemiology + + + - Epidemiological data varies widely, depending upon etiology and type of hydrocephalus +- ## Natural History & Prognosis + + + - Usually progressive unless treated +- ## Treatment + + + - Obstructive hydrocephalus is managed surgically + - CSF diversion (shunt or ETV) + - Resection of obstructing or hypersecreting lesion + - Most common neurosurgical procedure in children = CSF shunting for hydrocephalus + - CSF diversion is typically delayed in EVOH + - Tendency to be much more shunt dependent + - Less tolerant of minor pressure changes + - Lesser degree of ventriculomegaly increases difficulty of surgery + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Longstanding aqueductal stenosis can be caused by slow-growing tectal tumor + - Compensated IVOH + - CSF function and homeostasis are far more complex than previously thought + - Free communication among anatomic/functional compartments is necessary for proper brain health +- ## Image Interpretation Pearls + + + - Size of ventricles generally correlates poorly with intracranial pressure + - Pulsatile CSF may create confusing signal intensity, even mimic intraventricular mass + + cf76a2a7-da6e-419b-9e56-481a10e5b803 + +## References + +# Selected References + +1. [Møllgård K et al: A mesothelium divides the subarachnoid space into functional compartments. Science. 379(6627):84-8, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36603070%5Bpmid%5D) +1. [Krishnan P et al: Neuroimaging in pediatric hydrocephalus. Indian J Pediatr. 86(10):952-60, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31077004%5Bpmid%5D) +1. [Ha SK et al: Magnetic resonance imaging and histopathological visualization of human dural lymphatic vessels. Bio Protoc. 8(8), 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29780855%5Bpmid%5D) +1. [Patel SK et al: Advanced neuroimaging techniques in pediatric hydrocephalus. Pediatr Neurosurg. 52(6):436-45, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28095378%5Bpmid%5D) +1. [Algin O et al: Assessment of third ventriculostomy patency with the 3D-SPACE technique: a preliminary multicenter research study. J Neurosurg. 122(6):1347-55, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25859808%5Bpmid%5D) +1. [Jessen NA et al: The glymphatic system: a beginner's guide. Neurochem Res. 40(12):2583-99, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25947369%5Bpmid%5D) +1. [Russo N et al: Endoscopic approaches to intraventricular lesions. J Neurol Surg A Cent Eur Neurosurg. 76(5):353-60, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26008954%5Bpmid%5D) +1. [Flannery AM et al: Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 1: introduction and methodology. J Neurosurg Pediatr. 14 Suppl 1:3-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25988777%5Bpmid%5D) +1. [Mazzola CA et al: Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 2: management of posthemorrhagic hydrocephalus in premature infants. J Neurosurg Pediatr. 14 Suppl 1:8-23, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25988778%5Bpmid%5D) +1. [Nikas DC et al: Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 10: change in ventricle size as a measurement of effective treatment of hydrocephalus. J Neurosurg Pediatr. 14 Suppl 1:77-81, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25988786%5Bpmid%5D) +1. [Dinçer A et al: Radiologic evaluation of pediatric hydrocephalus. Childs Nerv Syst. 27(10):1543-62, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928020%5Bpmid%5D) +1. [Mirone G et al: Hydrocephalus and spinal cord tumors: a review. Childs Nerv Syst. 27(10):1741-9, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928038%5Bpmid%5D) +1. [Oi S: Classification of hydrocephalus: critical analysis of classification categories and advantages of "Multi-categorical Hydrocephalus Classification" (Mc HC). Childs Nerv Syst. 27(10):1523-33, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928018%5Bpmid%5D) +1. [Dinçer A et al: Is all "communicating" hydrocephalus really communicating? Prospective study on the value of 3D-constructive interference in steady state sequence at 3T. AJNR Am J Neuroradiol. 30(10):1898-906, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19643921%5Bpmid%5D) +1. [Feng F et al: Evaluation of radionuclide cerebrospinal fluid scintigraphy as a guide in the management of patients with hydrocephalus. Clin Imaging. 33(2):85-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19237049%5Bpmid%5D) +1. [Linninger AA et al: Normal and hydrocephalic brain dynamics: the role of reduced cerebrospinal fluid reabsorption in ventricular enlargement. Ann Biomed Eng. 37(7):1434-47, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19373558%5Bpmid%5D) +1. [Oertel JM et al: Endoscopic third ventriculostomy in obstructive hydrocephalus due to giant basilar artery aneurysm. J Neurosurg. 110(1):14-8, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18991498%5Bpmid%5D) +1. [Stoquart-El Sankari S et al: Phase-contrast MR imaging support for the diagnosis of aqueductal stenosis. AJNR Am J Neuroradiol. 30(1):209-14, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18832663%5Bpmid%5D) +1. [Sekula RF Jr et al: A case of an elderly adult presenting with obstructive hydrocephalus secondary to a rare hemorrhagic suprasellar pilocytic astrocytoma. Clin Neuropathol. 27(6):396-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19130737%5Bpmid%5D) +1. [Yamada S et al: Visualization of cerebrospinal fluid movement with spin labeling at MR imaging: preliminary results in normal and pathophysiologic conditions. Radiology. 249(2):644-52, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18936318%5Bpmid%5D) +1. [Erdogan AR et al: Sex and handedness differences in size of cerebral ventricles of normal subjects. Int J Neurosci. 114(1):67-73, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14660068%5Bpmid%5D) +1. [Gaser C et al: Ventricular enlargement in schizophrenia related to volume reduction of the thalamus, striatum, and superior temporal cortex. Am J Psychiatry. 161(1):154-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14702264%5Bpmid%5D) +1. [Wyldes M et al: Isolated mild fetal ventriculomegaly. Arch Dis Child Fetal Neonatal Ed. 89(1):F9-13, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14711845%5Bpmid%5D) +1. [Akhondi H et al: Hydrocephalus as a presenting manifestation of neurosarcoidosis. South Med J. 96(4):403-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12916562%5Bpmid%5D) +1. [Bhattacharyya KB et al: Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature. Acta Neurol Scand. 108(3):216-20, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12911467%5Bpmid%5D) +1. Brown KP et al: 1H MRS in human hydrocephalus. J MRI. 14:291-9, 2003 +1. [Grunert P et al: The role of third ventriculostomy in the management of obstructive hydrocephalus. Minim Invasive Neurosurg. 46(1):16-21, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12640578%5Bpmid%5D) +1. [Joseph VB et al: MR ventriculography for the study of CSF flow. AJNR Am J Neuroradiol. 24(3):373-81, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12637285%5Bpmid%5D) +1. [Sener RN: Callosal changes in obstructive hydrocephalus: observations with FLAIR imaging, and diffusion MRI. Comput Med Imaging Graph. 26(5):333-7, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12204238%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Depiction of normal CSF flow through the glymphatic system. CSF descends along periarterial perivascular spaces (PVS) and through the interstitium before exiting along perivenular PVS, clearing macromolecules (black particles). The exchange between interstitium and PVS is modulated by astrocytic endfeet expressing AQP4 (pink channels).](images/app.statdx.com_image_thumbnail_e84cc4eb-f65b-49c8-8023-d157ef9155ba_annotated_true_size_900_quality_90_1b92eb51_20251018T164957Z.jpg) +*Depiction of normal CSF flow through the glymphatic system. CSF descends along periarterial perivascular spaces (PVS) and through the interstitium before exiting along perivenular PVS, clearing macromolecules (black particles). The exchange between interstitium and PVS is modulated by astrocytic endfeet expressing AQP4 (pink channels).* + +![Depiction of normal CSF flow through the glymphatic system. CSF descends along periarterial perivascular spaces (PVS) and through the interstitium before exiting along perivenular PVS, clearing macromolecules (black particles). The exchange between interstitium and PVS is modulated by astrocytic endfeet expressing AQP4 (pink channels).](images/app.statdx.com_image_thumbnail_e84cc4eb-f65b-49c8-8023-d157ef9155ba_size_174_quality_85_184aa51d_20251018T155131Z.jpg) +*Depiction of normal CSF flow through the glymphatic system. CSF descends along periarterial perivascular spaces (PVS) and through the interstitium before exiting along perivenular PVS, clearing macromolecules (black particles). The exchange between interstitium and PVS is modulated by astrocytic endfeet expressing AQP4 (pink channels).* + +![An isoattenuating colloid cyst obstructs the foramina of Monro in this 7-year-old, causing obstructive hydrocephalus.](images/app.statdx.com_image_thumbnail_dfa6cf57-4225-40b1-8037-f7f2557ee773_annotated_true_size_900_quality_90_b05cdf7d_20251018T164957Z.jpg) +*An isoattenuating colloid cyst obstructs the foramina of Monro in this 7-year-old, causing obstructive hydrocephalus.* + +![A large suprasellar arachnoid cyst balloons upward and obstructs the foramina of Monro in this 1-year-old with macrocrania.](images/app.statdx.com_image_thumbnail_70070ccc-c977-4c05-a903-81616fbbea63_annotated_true_size_900_quality_90_dddf27ae_20251018T164957Z.jpg) +*A large suprasellar arachnoid cyst balloons upward and obstructs the foramina of Monro in this 1-year-old with macrocrania.* + +![This 3-month-old with hydrocephalus has a Blake pouch cyst obstructing outflow of CSF from the 4th ventricle. Note the membrane across the posterior foramen magnum and the uplifting of the vermis .](images/app.statdx.com_image_thumbnail_310d1a62-4dbc-479a-9e9e-423205955735_annotated_true_size_900_quality_90_b75c461d_20251018T164957Z.jpg) +*This 3-month-old with hydrocephalus has a Blake pouch cyst obstructing outflow of CSF from the 4th ventricle. Note the membrane across the posterior foramen magnum and the uplifting of the vermis .* + +![A medulloblastoma fills and obstructs the 4th ventricle in this 10-year-old, leading to supratentorial ventriculomegaly and papilledema . Papilledema visible on MR typically correlates to grade 3 on the Frisen scale (moderate edema).](images/app.statdx.com_image_thumbnail_9a9c7b4f-95d2-4e4d-8b5d-68161defab53_annotated_true_size_900_quality_90_a79dce92_20251018T164957Z.jpg) +*A medulloblastoma fills and obstructs the 4th ventricle in this 10-year-old, leading to supratentorial ventriculomegaly and papilledema . Papilledema visible on MR typically correlates to grade 3 on the Frisen scale (moderate edema).* + +![Axial FLAIR MR through the lateral ventricles in the same child shows transependymal edema capping the frontal and occipital horns , reflecting the increased pressure in the ventricular system.](images/app.statdx.com_image_thumbnail_d9d39656-21b9-48e2-82d3-86f3cabd705a_annotated_true_size_900_quality_90_91b55c48_20251018T164957Z.jpg) +*Axial FLAIR MR through the lateral ventricles in the same child shows transependymal edema capping the frontal and occipital horns , reflecting the increased pressure in the ventricular system.* + +![Bacterial meningitis (group A Streptococcus in this example) can restrict resorption of CSF but can also obstruct at the cerebral aqueduct and 4th ventricular outlets when complicated by ventriculitis, evident on this image by abnormal enhancement of the ependyma .](images/app.statdx.com_image_thumbnail_e5af07e5-9045-454e-919f-bb1cfd505bdc_annotated_true_size_900_quality_90_bee3db67_20251018T164957Z.jpg) +*Bacterial meningitis (group A Streptococcus in this example) can restrict resorption of CSF but can also obstruct at the cerebral aqueduct and 4th ventricular outlets when complicated by ventriculitis, evident on this image by abnormal enhancement of the ependyma .* + +![An infiltrating tectal glioma obstructs the cerebral aqueduct in this 10-year-old. Absence of transependymal edema suggests a compensated hydrocephalus.](images/app.statdx.com_image_thumbnail_0e5ef294-c697-4d00-9852-0ad6f730f900_annotated_true_size_900_quality_90_68db5118_20251018T164957Z.jpg) +*An infiltrating tectal glioma obstructs the cerebral aqueduct in this 10-year-old. Absence of transependymal edema suggests a compensated hydrocephalus.* + +![A papilloma of the choroid plexus in the occipital horn of the left lateral ventricle causes moderate hydrocephalus by excessive CSF production in this 2-month-old.](images/app.statdx.com_image_thumbnail_3404043a-6569-4de0-b4a3-a0cc0bd2b6dc_annotated_true_size_900_quality_90_aa2614f3_20251018T164957Z.jpg) +*A papilloma of the choroid plexus in the occipital horn of the left lateral ventricle causes moderate hydrocephalus by excessive CSF production in this 2-month-old.* + +![CSF overproduction can rarely be nonneoplastic in nature, as in this 6-month-old with villous hyperplasia of the choroid plexus in each lateral ventricle . Note the preservation of peripheral sulci, as the unfused sutures of the infant can widen in response to increased intracranial volume.](images/app.statdx.com_image_thumbnail_e380d6ae-5d60-44e2-bf15-30137880085f_annotated_true_size_900_quality_90_1fe1546a_20251018T164959Z.jpg) +*CSF overproduction can rarely be nonneoplastic in nature, as in this 6-month-old with villous hyperplasia of the choroid plexus in each lateral ventricle . Note the preservation of peripheral sulci, as the unfused sutures of the infant can widen in response to increased intracranial volume.* + + +### Additional Images + +![Sagittal T1 MR shows a large mass within the 4th ventricle causing intraventricular obstructive hydrocephalus or noncommunicating hydrocephalus.](images/app.statdx.com_image_thumbnail_c48f3e2e-092c-4ce3-881e-9a269ec15ea6_annotated_true_size_900_quality_90_78b02641_20251018T165000Z.jpg) +*Sagittal T1 MR shows a large mass within the 4th ventricle causing intraventricular obstructive hydrocephalus or noncommunicating hydrocephalus.* + +![Sagittal T2 MR in the same patient shows transependymal CSF flow, seen here as "fingers" extending into white matter around the enlarged lateral ventricle. The case was medulloblastoma with acute IVOH.](images/app.statdx.com_image_thumbnail_15897f20-57e8-4b17-9ac4-30cab6467abb_annotated_true_size_900_quality_90_9fed6b74_20251018T165000Z.jpg) +*Sagittal T2 MR in the same patient shows transependymal CSF flow, seen here as "fingers" extending into white matter around the enlarged lateral ventricle. The case was medulloblastoma with acute IVOH.* + +![Coronal T1 C+ MR shows IVOH with a large, enhancing intraventricular mass causing marked enlargement of the lateral ventricles .](655689cf-c7a3-44eb-85b1-9c92f7dc0cb1) +*Coronal T1 C+ MR shows IVOH with a large, enhancing intraventricular mass causing marked enlargement of the lateral ventricles .* + +![Axial NECT in the same patient shows the large intraventricular mass within the 4th ventricle. Note the dilated temporal horns .](images/app.statdx.com_image_thumbnail_1d873379-77f2-4ba4-85c9-43715aedaedc_annotated_true_size_900_quality_90_092a91f4_20251018T165000Z.jpg) +*Axial NECT in the same patient shows the large intraventricular mass within the 4th ventricle. Note the dilated temporal horns .* + +![Sagittal T1 MR shows IVOH secondary to aqueductal stenosis and distal stenosis of cerebral aqueduct . Note the enlarged lateral and 3rd ventricles.](images/app.statdx.com_image_thumbnail_e2d6cb01-32da-4284-85fb-e1d0b11218b1_annotated_true_size_900_quality_90_168d3b76_20251018T165000Z.jpg) +*Sagittal T1 MR shows IVOH secondary to aqueductal stenosis and distal stenosis of cerebral aqueduct . Note the enlarged lateral and 3rd ventricles.* + +![Axial FLAIR MR shows neurosarcoidosis and EVOH secondary to diffuse meningeal disease. Periventricular white matter hyperintensities are also present, as well as choroid involvement .](images/app.statdx.com_image_thumbnail_f9368bc3-dfdf-4912-bb77-5097bb5043bb_annotated_true_size_900_quality_90_45550aa6_20251018T165000Z.jpg) +*Axial FLAIR MR shows neurosarcoidosis and EVOH secondary to diffuse meningeal disease. Periventricular white matter hyperintensities are also present, as well as choroid involvement .* + +![Coronal T1 C+ MR shows neurocysticercosis involvement within the 3rd ventricle and aqueduct , causing IVOH. The lateral ventricles are dilated.](images/app.statdx.com_image_thumbnail_be2f5692-eeeb-4ea0-aae2-9ececaf9072b_annotated_true_size_900_quality_90_31f0b627_20251018T165000Z.jpg) +*Coronal T1 C+ MR shows neurocysticercosis involvement within the 3rd ventricle and aqueduct , causing IVOH. The lateral ventricles are dilated.* + +![Axial FLAIR MR shows neurocysticercosis resulting in IVOH. Large intraventricular cysts are present in the lateral vents , obstructing the foramina of Monro.](images/app.statdx.com_image_thumbnail_d4fbed45-ded4-4557-8a60-ffa2c5a76b7b_annotated_true_size_900_quality_90_b7584229_20251018T165001Z.jpg) +*Axial FLAIR MR shows neurocysticercosis resulting in IVOH. Large intraventricular cysts are present in the lateral vents , obstructing the foramina of Monro.* + +![Axial T1 MR shows a well-defined, hyperintense lesion at the foramen of Monro in a patient with headaches, most consistent with a colloid cyst. Note the enlargement of the lateral ventricles due to obstruction at the foramen of Monro.](images/app.statdx.com_image_thumbnail_709fe152-548b-43c9-a72d-d13abe331723_annotated_true_size_900_quality_90_9f3616ed_20251018T165001Z.jpg) +*Axial T1 MR shows a well-defined, hyperintense lesion at the foramen of Monro in a patient with headaches, most consistent with a colloid cyst. Note the enlargement of the lateral ventricles due to obstruction at the foramen of Monro.* + +![Sagittal T1 C+ MR shows a homogeneously enhancing mass in the posterior 3rd ventricle , which causes obstruction and dilatation of the lateral and 3rd ventricles. On pathology, this was an astrocytoma.](images/app.statdx.com_image_thumbnail_f26e26ab-4622-4975-b3b8-c0241bd59f6e_annotated_true_size_900_quality_90_214d2580_20251018T165001Z.jpg) +*Sagittal T1 C+ MR shows a homogeneously enhancing mass in the posterior 3rd ventricle , which causes obstruction and dilatation of the lateral and 3rd ventricles. On pathology, this was an astrocytoma.* + +![Coronal T2 MR shows a pilocytic astrocytoma centered in the right thalamus , causing severe mass effect on the 3rd ventricle and resultant obstructive hydrocephalus .](39ae17e4-a50a-4f11-b6c5-42b47b2c293a) +*Coronal T2 MR shows a pilocytic astrocytoma centered in the right thalamus , causing severe mass effect on the 3rd ventricle and resultant obstructive hydrocephalus .* + +![Axial T2 MR demonstrates a well-defined CSF intensity cyst with the left temporal horn most consistent with an ependymal cyst . Note the dilated and trapped left temporal horn .](c1b16bae-b36a-4bf1-8de9-ca410a8c3f96) +*Axial T2 MR demonstrates a well-defined CSF intensity cyst with the left temporal horn most consistent with an ependymal cyst . Note the dilated and trapped left temporal horn .* + +![Sagittal T1 C+ MR shows an enhancing mass in the pineal region causing mass effect on the tectal plate and aqueductal obstruction. Note the extensive leptomeningeal enhancement due to CSF spread of tumor. CSF cytology showed a primitive neuroectodermal tumor.](8096e72c-52bb-4372-a469-ed9b3c222bd4) +*Sagittal T1 C+ MR shows an enhancing mass in the pineal region causing mass effect on the tectal plate and aqueductal obstruction. Note the extensive leptomeningeal enhancement due to CSF spread of tumor. CSF cytology showed a primitive neuroectodermal tumor.* + +![A medulloblastoma fills and obstructs the 4th ventricle in this 10-year-old, leading to supratentorial ventriculomegaly and papilledema.](a7643e46-4a5e-47f0-b843-87e9f35fca31) +*A medulloblastoma fills and obstructs the 4th ventricle in this 10-year-old, leading to supratentorial ventriculomegaly and papilledema.* + +![FIESTA shows pineal parenchymal tumor of intermediate differentiation obstructing the cerebral aqueduct in this 8-year-old boy.](229bee91-b7b6-4584-97a3-e6f656b0000c) +*FIESTA shows pineal parenchymal tumor of intermediate differentiation obstructing the cerebral aqueduct in this 8-year-old boy.* + diff --git a/docs_md/articles/idiopathic-intracranial-hypertension_d7a0a1b6-1d94-473c-9fe9-021443969f9f.md b/docs_md/articles/idiopathic-intracranial-hypertension_d7a0a1b6-1d94-473c-9fe9-021443969f9f.md new file mode 100644 index 0000000..e199f31 --- /dev/null +++ b/docs_md/articles/idiopathic-intracranial-hypertension_d7a0a1b6-1d94-473c-9fe9-021443969f9f.md @@ -0,0 +1,428 @@ +--- +title: "Idiopathic Intracranial Hypertension" +docid: "d7a0a1b6-1d94-473c-9fe9-021443969f9f" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "CSF Disorders" + slug: "csf-disorders" + treeNodeId: "d305bd95-7cca-4888-80b9-fabe45d84ee5" + - + name: "Idiopathic Intracranial Hypertension" + slug: "idiopathic-intracranial-hypertensi-" + treeNodeId: null +category: "Brain" +documentVersionId: "8907806d-0770-46fa-b129-78e393ad4038" +imageCount: 22 +lastUpdated: "10/08/20" +pageDescription: "Idiopathic Intracranial Hypertension" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, CSF Disorders, Idiopathic Intracranial Hypertension" +pageTitle: "Idiopathic Intracranial Hypertension | STATdx" +enhancedTitle: "Idiopathic Intracranial Hypertension" +type: "DX" +references: true +tables: 1 +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "CSF Disorders" + - "Idiopathic Intracranial Hypertension" +--- +# KEY FACTS + +- ## Terminology + + + - Idiopathic intracranial hypertension (IIH) + - Pseudotumor cerebri + - Benign intracranial hypertension + - ↑ intracranial pressure (ICP) without identifiable cause +- ## Imaging + + + - Empty or partially empty sella + - Posterior globe flattening + - Intraocular protrusion of optic nerve head + - Optic nerve sheath enlargement ± tortuosity + - Optic nerve head DWI hyperintensity, ± enhancement + - Slit-like ventricles, rare: Poor neuroimaging sign of IIH + - MRV: Often shows transverse sinus stenosis and flow gaps + - Whether this is cause or consequence of raised ICP is controversial + - Best imaging tool: MR brain + T2 coronal fat-saturated orbit + MRV +- ## Top Differential Diagnoses + + + - Secondary pseudotumor syndromes + - Idiopathic or postinflammatory (i.e., multiple sclerosis) optic nerve atrophy + - Idiopathic empty sella (normal variant) + - Chiari 1 malformation +- ## Clinical Issues + + + - Obese woman age 20-44 years with headache and papilledema most common presentation + - Headache in 75-94% + - Papilledema (bilateral optic nerve head swelling) virtually universal + - Progressive visual loss ± CNVI paresis, diplopia + - Chief hazard: Vision loss from chronic papilledema + - Treatment: Medical or surgical (lumbar puncture, shunt, optic nerve sheath fenestration) + - Stent placement in transverse sinus stenosis with significant pressure differentials across stenosis (controversial) + +# TERMINOLOGY + +- ## Abbreviations + + + - Idiopathic intracranial hypertension (IIH) +- ## Synonyms + + + - Pseudotumor cerebri + - Benign intracranial hypertension +- ## Definitions + + + - ↑ intracranial pressure (ICP) without identifiable cause + - Association of any medication or condition with IIH better termed "secondary intracranial hypertension" + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Flattening of posterior sclera, intraocular optic nerve protrusion, enlarged optic nerve sheath, ↑ tortuosity of optic nerve, partially empty sella, and venous sinus stenosis in patient with clinical findings of IIH + - Imaging in IIH + - Exclude identifiable causes of ↑ ICP + - Detect findings associated with IIH +- ## CT Findings + + + - ### NECT + + + - Usually normal + - Enlarged optic nerve sheaths ± empty sella + - Less common: Slit ventricles + - ### Bone CT + + + - Solitary or multiple skull base osseous-dural defects + - May see skull base foramina enlargement +- ## MR Findings + + + - ### T1WI + + + - Partially empty sella turcica + - Enlarged/tortuous optic nerve sheaths + - Posterior sclera flattened + - Small "pinched" ventricles + - Midline sagittal: Cerebellar tonsillar ectopia may mimic Chiari malformation type 1 + - ### T2WI + + + - Empty or partially empty sella + - Posterior globe flattening + - Intraocular protrusion of optic nerve head + - Optic nerve sheath enlargement: Widened ring of CSF around optic nerve + - Optic nerve tortuosity + - Slit-like ventricles, rare: Poor neuroimaging sign of IIH + - "Tight" subarachnoid spaces + - Meningoencephaloceles + - ### FLAIR + + + - Contrast-enhanced 3D-FLAIR: Hyperintensity of optic nerve head sensitive for detection of papilledema in IIH + - ### DWI + + + - DWI hyperintensity of optic nerve head with papilledema + - DTI: Optic disc fractional anisotropy (FA) low & mean diffusivity (MD) high in IIH + - ### T1WI C+ + + + - Enhancement of optic nerve head + - ### MRV + + + - Often shows transverse sinus stenosis and flow gaps + - Controversial whether this is cause or consequence of raised ICP + - CTV helpful to differentiate hypoplastic sinus segment from thrombosis +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR brain + T2 coronal fat-saturated orbit + MRV + +# DIFFERENTIAL DIAGNOSIS + +- ## Secondary Intracranial Hypertension + + + - Cerebral venous abnormalities + - [Dural venous sinus thrombosis, bilateral jugular vein thrombosis, superior vena cava syndrome, arteriovenous fistula](/document/dural-sinus-thrombosis/4e81a1de-df92-4172-99ec-1377b0d9d188) + - ↓ CSF absorption from previous intracranial infection or subarachnoid hemorrhage, hypercoagulable states + - Ventriculomegaly more common + - Medications and exposures + - Tetracycline, minocycline, vitamin A, lithium, retinoids, anabolic steroids, withdrawal from chronic corticosteroids + - Medical conditions + - Endocrine disorders (Addison disease, hypoparathyroidism), hypercapnia, sleep apnea, SLE +- [Idiopathic or Postinflammatory Optic Nerve Atrophy](/document/optic-neuritis/ac9c8fc9-33cd-4716-a509-2542ec5579ca) + - Small optic nerves without scleral flattening +- [Idiopathic Empty Sella](/document/empty-sella/39a0d2d1-1439-4558-8f5d-86a2a6d93e3a) + - Normal variant; normal optic nerve sheaths +- [Chiari 1 Malformation](/document/chiari-1-malformation/97837e15-0d39-4c87-8af0-028652b399a6) + - Peg-like tonsils ≥ 5 mm below foramen magnum + - Low cerebellar tonsils in IIH may mimic Chiari 1 + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Precise etiology of IIH unknown + - 5 different proposed mechanisms resulting in ↑ ICP + - ↑ cerebral volume + - Possible etiology: ↑ interstitial fluid, ↑ blood volume, ↑ tissue volume + - ↑ CSF volume + - Possible etiology: ↑ CSF production rate, ↑ CSF outflow resistance + - ↑ cerebral arterial pressure + - Possible etiology: Loss of cerebral autoregulation + - ↑ venous blood volume and interstitial fluid + - Possible etiology: ↑ cerebral venous pressure + - ↓ CSF outflow and ↑ CSF volume +- ## Gross Pathologic & Surgical Features + + + - Bilateral papilledema +- ## Microscopic Features + + + - Normal CSF cytology, chemistry + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Headache in 75-94% + - Generalized, episodic, throbbing, aggravated by Valsalva + - Transient vision loss, other visual complaints + - Fulminant IIH, severe vision loss < 4 weeks from onset of symptoms + - Papilledema (bilateral optic nerve head swelling) virtually universal + - Progressive visual loss ± CNVI paresis, diplopia + - Vertigo, tinnitus (52-60%), occasional pituitary dysfunction + - May present with spontaneous CSF leak + - Some patient with spontaneous CSF leak may not exhibit typical symptoms of IIH + - May develop symptoms of IIH after CSF leak repair + - Temporal lobe epilepsy caused by anteroinferior temporal lobe meningoencephaloceles in IIH + - ### Clinical profile + + + - Obese, young to middle-aged woman with headache, papilledema +- ## Demographics + + + - ### Age + + + - Peak: 15-40 years (occasionally seen in children) + - ### Epidemiology + + + - 0.9 cases per 100,000 population in USA + - More common in overweight, reproductive-aged women + - Incidence in females aged 2-44 years & 20% above ideal body weight: ~ 19.3 cases per 100,000 population + - ### Sex + + + - M:F = 1:8 + - Epidemiology: ↑ prevalence with obesity +- ## Natural History & Prognosis + + + - Chief hazard: Vision loss from chronic papilledema, severe visual acuity deficits in 25% of patients without treatment +- ## Treatment + + + - Goal: Prevent visual loss, improve associated symptoms + - Options + - Medical: Weight loss, carbonic anhydrase inhibitors: Acetazolamide + - Therapeutic lumbar puncture + - Surgical: Reserved for patients who continue to experience vision loss despite conservative management and those initially presenting with rapid vision loss + - Lumboperitoneal shunt, optic nerve sheath fenestration + - Venous stent placement + - Stent placement in transverse sinus stenosis with significant pressure differentials across stenosis has shown to improve symptoms and ↓ papilledema + - ↓ cerebral venous pressure, improve CSF resorption in venous system: ↓ intracranial (CSF) pressure, improving symptoms of IIH, and ↓ papilledema + - **Venous stent placement is controversial** + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - Must exclude venous thrombosis/space-occupying lesion + + 58e13d74-efc9-4630-9014-3c28122c7470 + +## References + +# Selected References + +1. [Nagarajan E et al: Is magnetic resonance imaging diffusion restriction of the optic disc head a new marker for idiopathic intracranial hypertension? J Neurosci Rural Pract. 11(1):170-4, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32140023%5Bpmid%5D) +1. [Boyter E: Idiopathic intracranial hypertension. JAAPA. 32(5):30-5, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30969189%5Bpmid%5D) +1. [Golden E et al: Contrast-enhanced 3D-FLAIR imaging of the optic nerve and optic nerve head: novel neuroimaging findings of idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 40(2):334-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30679213%5Bpmid%5D) +1. [Rehder D: Idiopathic intracranial hypertension: review of clinical syndrome, imaging findings, and treatment. Curr Probl Diagn Radiol. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31056359%5Bpmid%5D) +1. [Thurtell MJ: Idiopathic intracranial hypertension. Continuum (Minneap Minn). 25(5):1289-309, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31584538%5Bpmid%5D) +1. [Madriz Peralta G et al: An update of idiopathic intracranial hypertension. Curr Opin Ophthalmol. 29(6):495-502, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30169466%5Bpmid%5D) +1. [Stevens SM et al: Idiopathic intracranial hypertension: contemporary review and implications for the otolaryngologist. Laryngoscope. 128(1):248-56, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=28349571%5Bpmid%5D) +1. [Wall M: Update on idiopathic intracranial hypertension. Neurol Clin. 35(1):45-57, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27886895%5Bpmid%5D) +1. [Görkem SB et al: MR imaging findings in children with pseudotumor cerebri and comparison with healthy controls. Childs Nerv Syst. 31(3):373-80, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25358812%5Bpmid%5D) +1. [Masri A et al: Intracranial hypertension in children: etiologies, clinical features, and outcome. J Child Neurol. 30(12):1562-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25762586%5Bpmid%5D) +1. [Sivasankar R et al: Imaging and interventions in idiopathic intracranial hypertension: a pictorial essay. Indian J Radiol Imaging. 25(4):439-44, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26752823%5Bpmid%5D) +1. [Ahmed RM et al: Transverse sinus stenting for pseudotumor cerebri: a cost comparison with CSF shunting. AJNR Am J Neuroradiol. 35(5):952-8, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24287092%5Bpmid%5D) +1. [Dave SB et al: Pseudotumor cerebri: an update on treatment options. Indian J Ophthalmol. 62(10):996-8, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25449933%5Bpmid%5D) +1. [Liguori C et al: Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 82(19):1752-3, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24821936%5Bpmid%5D) +1. [Friedman DI et al: Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 81(13):1159-65, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23966248%5Bpmid%5D) +1. [Passi N et al: MR imaging of papilledema and visual pathways: effects of increased intracranial pressure and pathophysiologic mechanisms. AJNR Am J Neuroradiol. 34(5):919-24, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=22422187%5Bpmid%5D) +1. [Aiken AH et al: Incidence of cerebellar tonsillar ectopia in idiopathic intracranial hypertension: a mimic of the Chiari I malformation. AJNR Am J Neuroradiol. 33(10):1901-6, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22723059%5Bpmid%5D) +1. [Ahmed RM et al: Transverse sinus stenting for idiopathic intracranial hypertension: a review of 52 patients and of model predictions. AJNR Am J Neuroradiol. 32(8):1408-14, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21799038%5Bpmid%5D) +1. [Degnan AJ et al: Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol. 32(11):1986-93, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21680652%5Bpmid%5D) +1. [Furtado SV et al: Pseudotumor cerebri: as a cause for early deterioration after Chiari I malformation surgery. Childs Nerv Syst. 25(8):1007-12, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19296114%5Bpmid%5D) +1. [Hershko AY et al: Increased intracranial pressure related to systemic lupus erythematosus: a 26-year experience. Semin Arthritis Rheum. 38(2):110-5, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18221986%5Bpmid%5D) +1. [Randhawa S et al: Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Opin Ophthalmol. 19(6):445-53, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18854688%5Bpmid%5D) +1. [Agarwal MR et al: Optic nerve sheath fenestration for vision preservation in idiopathic intracranial hypertension. Neurosurg Focus. 23(5):E7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=18004969%5Bpmid%5D) +1. [Binder DK et al: Idiopathic intracranial hypertension. Neurosurgery. 54(3):538-51; discussion 551-2, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15028127%5Bpmid%5D) +1. [Bastin ME et al: Diffuse brain oedema in idiopathic intracranial hypertension: a quantitative magnetic resonance imaging study. J Neurol Neurosurg Psychiatry. 74(12):1693-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14638893%5Bpmid%5D) +1. [Bandyopadhyay S: Pseudotumor cerebri. Arch Neurol. 58(10):1699-701, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11594936%5Bpmid%5D) +1. [Suzuki H et al: MR imaging of idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 22(1):196-9, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11158909%5Bpmid%5D) + +## Tables + +# Original Modified Dandy Criteria and Criteria Utilized in IIH Treatment Trial + +| A: Modified Dandy Criteria for IIH | +| --- | +| (1) Signs and symptoms of ↑ ICP (headaches, nausea, vomiting, transient visual obscurations, or papilledema) | +| (2) Absence of localized findings in neurologic examination (except for false localizing signs, such as abducens nerve palsy) | +| (3) Normal CT/MR findings without evidence of hydrocephalus or mass lesion | +| (4) CSF opening pressure > 25 cm with normal CSF cytologic and chemical findings | +| (5) No other causes of ↑ ICP identified | +| B: Idiopathic IIH Treatment Trial: Modified Dandy Criteria | +| (1) Signs and symptoms of ↑ ICP | +| (2) Absence of localized findings in neurologic examination | +| (3) ↑ CSF pressure > 20 cm; normal neuroimaging except for empty sella, flattening optic nerve head, distention of perioptic subarachnoid space ± tortuous optic nerve, transverse venous sinus stenosis | +| (4) Awake and alert | +| (5) No other causes of ↑ ICP | +| If CSF opening pressure was 20-25 cm, at least 1 of following was also required | +| Pulse synchronous tinnitus, CNVI palsy, Frisen grade II papilledema, no disc anomalies mimicking disc edema, MRV with lateral sinus collapse/stenosis, partially empty sella, dilated optic nerve sheaths | + + +## Images + + +### Selected Images + +![Axial T2 FS MR in a young obese female with headaches and visual symptoms shows flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe. Note mild prominence of the CSF along the optic nerve sheaths.](images/app.statdx.com_image_thumbnail_24d4b975-7325-44c1-915b-9a6d28bfe436_annotated_true_size_900_quality_90_3a507534_20251018T164622Z.jpg) +*Axial T2 FS MR in a young obese female with headaches and visual symptoms shows flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe. Note mild prominence of the CSF along the optic nerve sheaths.* + +![Axial T2 FS MR in a young obese female with headaches and visual symptoms shows flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe. Note mild prominence of the CSF along the optic nerve sheaths.](images/app.statdx.com_image_thumbnail_24d4b975-7325-44c1-915b-9a6d28bfe436_size_174_quality_85_5e6fc32c_20251018T164552Z.jpg) +*Axial T2 FS MR in a young obese female with headaches and visual symptoms shows flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe. Note mild prominence of the CSF along the optic nerve sheaths.* + +![Axial DWI in the same patient demonstrates subtle high signal in the region of the optic nerve heads bilaterally. Hyperintensity of the optic nerve heads on DWI can serve as a useful imaging marker for papilledema, especially if bilateral.](images/app.statdx.com_image_thumbnail_ae0e2ddf-eb4d-4442-85d4-ab2b80819b1f_annotated_true_size_900_quality_90_ae9539e6_20251018T164622Z.jpg) +*Axial DWI in the same patient demonstrates subtle high signal in the region of the optic nerve heads bilaterally. Hyperintensity of the optic nerve heads on DWI can serve as a useful imaging marker for papilledema, especially if bilateral.* + +![Axial T1 C+ MR in the same patient shows subtle enhancement , as well as protrusion of prelaminar optic nerves bilaterally.](images/app.statdx.com_image_thumbnail_9a3de7d7-cac0-453f-8762-b1acc1059c51_annotated_true_size_900_quality_90_fbbf5704_20251018T164622Z.jpg) +*Axial T1 C+ MR in the same patient shows subtle enhancement , as well as protrusion of prelaminar optic nerves bilaterally.* + +![Coronal T2 FS MR in the same patient shows a partially empty sella with the pituitary gland flattened along the floor of the sella. Idiopathic intracranial hypertension is more commonly observed in overweight women of reproductive age. Treatment includes weight loss and medications, as well as lumbar punctures, shunt, and optic nerve fenestration.](images/app.statdx.com_image_thumbnail_6033189e-a7cc-4e6f-a8f7-4c3b312359f7_annotated_true_size_900_quality_90_a2d89787_20251018T164622Z.jpg) +*Coronal T2 FS MR in the same patient shows a partially empty sella with the pituitary gland flattened along the floor of the sella. Idiopathic intracranial hypertension is more commonly observed in overweight women of reproductive age. Treatment includes weight loss and medications, as well as lumbar punctures, shunt, and optic nerve fenestration.* + +![Coronal T2 FS MR of orbits in a 6 year old with papilledema and opening CSF pressure of 32 cm of H₂O shows dilated optic nerve sheaths bilaterally .](images/app.statdx.com_image_thumbnail_5e42b718-df6f-41f7-86cd-35fbe1dbee62_annotated_true_size_900_quality_90_29305ac9_20251018T164622Z.jpg) +*Coronal T2 FS MR of orbits in a 6 year old with papilledema and opening CSF pressure of 32 cm of H₂O shows dilated optic nerve sheaths bilaterally .* + +![Sagittal T2 FS MR in the same patient shows tortuosity of the optic nerve, dilated optic nerve sheath , flattening of the posterior sclera , and mild bulging of the optic nerve disc head due to papilledema. Findings are typical of idiopathic intracranial hypertension (IIH). Childhood obesity has a strong association with ↑ risk of pediatric IIH.](images/app.statdx.com_image_thumbnail_2bd694d1-0915-4b68-9b90-85c463dfe179_annotated_true_size_900_quality_90_7a482e85_20251018T164622Z.jpg) +*Sagittal T2 FS MR in the same patient shows tortuosity of the optic nerve, dilated optic nerve sheath , flattening of the posterior sclera , and mild bulging of the optic nerve disc head due to papilledema. Findings are typical of idiopathic intracranial hypertension (IIH). Childhood obesity has a strong association with ↑ risk of pediatric IIH.* + +![Sagittal T2 MR in a young female with IIH and temporal lobe epilepsy shows a defect along the floor of the middle cranial fossa with herniation of the anteroinferior temporal lobe .](images/app.statdx.com_image_thumbnail_53799f83-fd6b-42ac-b8a4-1041a19ec160_annotated_true_size_900_quality_90_fe5d009a_20251018T164622Z.jpg) +*Sagittal T2 MR in a young female with IIH and temporal lobe epilepsy shows a defect along the floor of the middle cranial fossa with herniation of the anteroinferior temporal lobe .* + +![Coronal CT cisternogram in a patient with IIH shows an osteodural defect along the great wing of the sphenoid. Defect along the lateral wall of the sphenoid sinus with a meningocele and contrast in the lateral sphenoid sinus due to CSF leak is shown. Patients with IIH can present with spontaneous CSF leaks.](images/app.statdx.com_image_thumbnail_66502da6-e3f7-4a1b-b5de-afe2df76f8d9_annotated_true_size_900_quality_90_ee7dc6dd_20251018T164622Z.jpg) +*Coronal CT cisternogram in a patient with IIH shows an osteodural defect along the great wing of the sphenoid. Defect along the lateral wall of the sphenoid sinus with a meningocele and contrast in the lateral sphenoid sinus due to CSF leak is shown. Patients with IIH can present with spontaneous CSF leaks.* + +![MIP image of a postcontrast MR venogram study in a patient with IIH shows stenosis of the distal transverse sinuses bilaterally , right > left.](images/app.statdx.com_image_thumbnail_610e2c15-7dd5-42a8-9642-3d8fb1089582_annotated_true_size_900_quality_90_6b91fa11_20251018T164622Z.jpg) +*MIP image of a postcontrast MR venogram study in a patient with IIH shows stenosis of the distal transverse sinuses bilaterally , right > left.* + +![3D VRT MR in the same patient shows transverse sinus stenosis , right > left. Phase contrast and postcontrast MR venogram techniques are preferred over TOF-MR venogram to evaluate for transverse sinus stenosis. Stenting of transverse sinus stenosis in patients with IIH is a controversial treatment option.](164ece26-20b8-4cae-b030-9b72efe9a987) +*3D VRT MR in the same patient shows transverse sinus stenosis , right > left. Phase contrast and postcontrast MR venogram techniques are preferred over TOF-MR venogram to evaluate for transverse sinus stenosis. Stenting of transverse sinus stenosis in patients with IIH is a controversial treatment option.* + + +### Additional Images + +![Coronal T1WI MR in the same patient shows unusually small lateral ventricles with a "pinched" appearance. These findings in an obese female with headaches and papilledema are consistent with IIH.](970fd4dc-11be-4478-9403-460a22cd3552) +*Coronal T1WI MR in the same patient shows unusually small lateral ventricles with a "pinched" appearance. These findings in an obese female with headaches and papilledema are consistent with IIH.* + +![Axial T2WI MR shows ↑ fluid in bilateral optic nerve sheaths with mild flattening of the globes at optic nerve insertion. Also note the CSF-filled and expanded empty sella .](3bf70f24-e2cf-411f-afde-5d27b9d05741) +*Axial T2WI MR shows ↑ fluid in bilateral optic nerve sheaths with mild flattening of the globes at optic nerve insertion. Also note the CSF-filled and expanded empty sella .* + +![Sagittal T1WI MR in another patient with IIH ("pseudotumor cerebri") shows empty sella . The ventricular size is normal.](390c2ec4-5e56-423f-bffb-2414615987cd) +*Sagittal T1WI MR in another patient with IIH ("pseudotumor cerebri") shows empty sella . The ventricular size is normal.* + +![Axial T2WI MR shows ↑ fluid in the sheaths surrounding the optic nerves , associated with severe scleral flattening .](df25df16-6b53-4850-a23d-304bb0de9fb4) +*Axial T2WI MR shows ↑ fluid in the sheaths surrounding the optic nerves , associated with severe scleral flattening .* + +![Axial T2WI MR shows dilated CSF spaces around the optic nerves and protrusion of the optic nerve papilla into the posterior globes . Opening CSF pressure in this 32-year-old woman was 45 cm of H₂O. Prominent CSF space in the suprasellar cistern represents an empty sella . Note the tortuosity of the left optic nerve.](56e575d8-16f3-4e95-95f8-2d3cbb5caea6) +*Axial T2WI MR shows dilated CSF spaces around the optic nerves and protrusion of the optic nerve papilla into the posterior globes . Opening CSF pressure in this 32-year-old woman was 45 cm of H₂O. Prominent CSF space in the suprasellar cistern represents an empty sella . Note the tortuosity of the left optic nerve.* + +![Sagittal T1WI MR in the same patient shows a partially empty sella , suggesting high CSF pressure in this young obese woman with headaches.](2b283afb-fa49-4301-af75-c08cd47d46e6) +*Sagittal T1WI MR in the same patient shows a partially empty sella , suggesting high CSF pressure in this young obese woman with headaches.* + +![Axial T1WI C+ MR in the same patient demonstrates enhancement, as well as protrusion of prelaminar optic nerves bilaterally . Mild diffuse optic nerve sheath enhancement is also present.](5520270b-2fc2-4233-8468-bb37f0efdc21) +*Axial T1WI C+ MR in the same patient demonstrates enhancement, as well as protrusion of prelaminar optic nerves bilaterally . Mild diffuse optic nerve sheath enhancement is also present.* + +![Coronal T1WI C+ FS MR in the same patient shows diffuse enhancement of the optic nerve sheaths associated with prominent subarachnoid spaces along the optic nerves. Treatment for pseudotumor cerebri includes weight loss and medications, as well as lumbar punctures, shunt, and optic nerve fenestration.](0a5bed97-d570-4eeb-b531-590bbaca6290) +*Coronal T1WI C+ FS MR in the same patient shows diffuse enhancement of the optic nerve sheaths associated with prominent subarachnoid spaces along the optic nerves. Treatment for pseudotumor cerebri includes weight loss and medications, as well as lumbar punctures, shunt, and optic nerve fenestration.* + +![Sagittal T1 MR in a patient with IIH shows a partially empty sella with the pituitary gland flattened along the floor of the sella.](72675faf-c940-4f75-9867-fd6a13d4baca) +*Sagittal T1 MR in a patient with IIH shows a partially empty sella with the pituitary gland flattened along the floor of the sella.* + +![MIP image of a postcontrast MR venogram study in the same patient shows stenosis of the distal transverse sinuses bilaterally . Transverse sinus stenosis is common in patients with IIH.](a5ccfa9c-e717-4afd-9745-28dfcf0d1498) +*MIP image of a postcontrast MR venogram study in the same patient shows stenosis of the distal transverse sinuses bilaterally . Transverse sinus stenosis is common in patients with IIH.* + +![Axial T2WI MR in a young obese female with headaches and visual symptoms shows mild dilatation of the CSF spaces around the optic nerves. There is mild flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe.](84a7ddc8-9ad0-4905-8866-acb2c4b60502) +*Axial T2WI MR in a young obese female with headaches and visual symptoms shows mild dilatation of the CSF spaces around the optic nerves. There is mild flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe.* + +![3D TOF-MR venogram image in the same patient shows stenosis in the distal transverse sinuses bilaterally . Stent placement in sinus stenosis with significant pressure differentials has shown to reduce papilledema.](21466142-7771-4e31-ac8a-4b6fd8d008d5) +*3D TOF-MR venogram image in the same patient shows stenosis in the distal transverse sinuses bilaterally . 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a/docs_md/articles/intracranial-hypotension_818a7972-1032-4d3e-a65a-97c494334aac.md b/docs_md/articles/intracranial-hypotension_818a7972-1032-4d3e-a65a-97c494334aac.md new file mode 100644 index 0000000..2964b29 --- /dev/null +++ b/docs_md/articles/intracranial-hypotension_818a7972-1032-4d3e-a65a-97c494334aac.md @@ -0,0 +1,480 @@ +--- +title: "Intracranial Hypotension" +docid: "818a7972-1032-4d3e-a65a-97c494334aac" +authors: + - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850" + value: "Anne G. Osborn, MD, FACR" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "CSF Disorders" + slug: "csf-disorders" + treeNodeId: "d305bd95-7cca-4888-80b9-fabe45d84ee5" + - + name: "Intracranial Hypotension" + slug: "intracranial-hypotension" + treeNodeId: null +category: "Brain" +documentVersionId: "5af54552-b0b6-49ed-a983-ba5e31060fe7" +imageCount: 20 +lastUpdated: "06/30/25" +pageDescription: "Intracranial Hypotension" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, CSF Disorders, Intracranial Hypotension" +pageTitle: "Intracranial Hypotension | STATdx" +enhancedTitle: "Intracranial Hypotension" +type: "DX" +references: true +cases: 2 +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "CSF Disorders" + - "Intracranial Hypotension" +--- +# KEY FACTS + +- ## Terminology + + + - Headache caused by ↓ intracranial CSF pressure +- ## Imaging + + + - Classic imaging quintet + - Downward displacement of brain through incisura (brain "sagging") + - Diffuse dural thickening/enhancement + - Veins, dural sinuses distended + - Engorged pituitary gland + - Subdural hygromas/hematomas + - Lack of 1 or more of 5 classic findings does not preclude diagnosis + - Cranial MR diagnostic in 90% but 10% normal +- ## Top Differential Diagnoses + + + - Meningitis + - Meningeal metastases + - Chronic subdural hematoma + - Dural sinus thrombosis + - Postsurgical dural thickening + - Idiopathic hypertrophic cranial pachymeningitis +- ## Clinical Issues + + + - Severe headache (orthostatic, persistent, pulsatile, or even associated with nuchal rigidity) + - Uncommon: Cranial nerve (CN) palsy (e.g., abducens), visual disturbances + - Rare: Severe encephalopathy with disturbances of consciousness + - Profile: Young/middle-aged adult with orthostatic headache + - Opening pressure often < 6 cm H₂O but normal pressure common and should not exclude diagnosis +- ## Diagnostic Checklist + + + - Frequently misdiagnosed clinically; imaging is key to diagnosis + - Do not misdiagnose intracranial hypotension as Chiari 1! + - Surgery can exacerbate symptoms; in rare cases can be fatal + - Only rarely are **all** classic clinical and imaging findings of spontaneous intracranial hemorrhage present in same patient + +# TERMINOLOGY + +- ## Abbreviations + + + - Intracranial hypotension (IH) +- ## Definitions + + + - Headache caused by ↓ intracranial cerebrospinal fluid (CSF) pressure + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Classic imaging quintet + - Downward displacement of brain through incisura (brain "sagging") + - Diffuse dural thickening/enhancement + - Veins, dural sinuses distended + - Subdural hygromas/hematomas + - Engorged pituitary gland + - Lack of 1 or more of 5 classic findings does not preclude diagnosis + - Cranial MR diagnostic in 90% of cases but 10% normal + - ### Location + + + - Pachymeninges (dura) + - Both supra- and infratentorial + - May extend into internal auditory canals + - Spinal dura, epidural venous plexuses may be involved + - ### Morphology + + + - Dural enhancement is smooth, not nodular or "lumpy-bumpy" +- ## CT Findings + + + - ### NECT + + + - Relatively insensitive; may appear normal + - Look for effaced suprasellar/basilar cisterns, "fat" midbrain/pons + - ± thick dura + - ± subdural fluid collections + - Usually bilateral + - CSF (hygroma) or blood (hematoma) + - Atria of lateral ventricles may appear deviated medially, abnormally close ("tethered") to midline + - Skull base defect on bone CT rarely causes spontaneous IH + - ### CECT + + + - Diffuse dural thickening, enhancement +- ## MR Findings + + + - ### T1WI + + + - Sagittal view shows brain descent in 40-50% of cases + - "Sagging" midbrain + - Midbrain displaced inferiorly below level of dorsum sellae + - Pons may be compressed against clivus + - ↓ angle between peduncles, pons + - Caudal displacement of tonsils in 25-75% + - Caution: Do not misdiagnose as Chiari 1 malformation; look for other imaging features of brain "sagging" + - Optic chiasm, hypothalamus draped over sella + - Pituitary enlarged above sella in 50% + - ↓ angle between internal cerebral veins (ICVs), vein of Galen + - ↓ vertical distance between pons, mammillary bodies + - Axial + - Suprasellar cistern crowded/effaced + - Midbrain, pons appear elongated ("fat" midbrain) + - Interpeduncular angle ↓ (avg = 25⁰) + - Temporal lobes herniated over tentorium, into incisura + - Lateral ventricles small, often distorted + - Atria pulled medially by downward displacement of midbrain + - Coronal + - Severe cases show ↓ venous angle (< 120°) between roofs of lateral ventricles + - Bilateral subdural fluid collections in 35% + - 70% hygromas (clear fluid collects within dural border cell layer) + - 10% hematomas (blood of variable signal intensity) + - ### T2WI + + + - Thickened dura usually hyperintense + - Subdural fluid (variable signal) + - ### FLAIR + + + - Hyperintense dura, subdural fluid + - Hyperintense midbrain corticospinal tracts in 55% + - ### T2* GRE + + + - May bloom if hemorrhage present + - Rare: Superficial siderosis + - ### T1WI C+ + + + - Diffuse, smooth intense dural enhancement in 85% + - Often extends into cerebellopontine angles (CPAs) + - Veins/dural sinuses distended (convex margins) +- ## Ultrasonographic Findings + + + - ### Color Doppler + + + - Enlarged superior ophthalmic veins with higher mean maximum flow velocity +- ## Angiographic Findings + + + - Cortical, medullary veins may be diffusely enlarged +- ## Nonvascular Interventions + + + - Spinal imaging (site of leak identified in ≈ 50%) + - Digital subtraction myelography > dynamic CT myelography + - Type 1 leaks = dural tear, extradural CSF collection + - Almost always associated with extradural CSF collection + - Adjacent bony abnormality (osteophyte, calcification) common + - 1a leak = ventral + - 1b = posterolateral + - Type 2 leaks = meningeal diverticula > 8 mm + - 2a = single or multiple diverticula + - 2b = complex meningeal diverticula or dural ectasia + - Type 3 leaks = direct CSF-venous fistulas + - Never show extradural CSF collection + - Single vein or network of dilated veins around root sleeve + - Type 4 leaks = without confirmed source on dedicated spinal imaging + - Extradural CSF in 50%; 50% have no evidence of extradural CSF +- ## Nuclear Medicine Findings + + + - Radionuclide cisternography (RNC) + - Direct findings: Focal accumulation of radioactivity outside of subarachnoid space at leakage site + - Indirect findings + - Rapid washout from CSF space + - Early appearance of activity in kidneys, urinary bladder + - Poor migration of isotope over convexities +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Contrast-enhanced cranial MR for diagnosis + - Dynamic CT myelography for leak localization + - ### Protocol advice + + + - Search for actual leakage site only if + - 2 technically adequate blood patches fail + - Posttraumatic leak is suspected + +# DIFFERENTIAL DIAGNOSIS + +- [Meningitis](/document/meningitis/7fdf69fa-c171-4e6b-b6d7-4e8fc94fdc53) + - Pia-subarachnoid enhancement > dura-arachnoid +- ## Meningeal Metastases + + + - Enhancement usually thicker, irregular ("bumpy") +- [Chronic Subdural Hematoma](/document/chronic-subdural-hematoma/cc7b52b4-c6a0-4b4e-ae8c-f05bfc5c5cb2) + - Look for enhancing membranes with blood products +- [Dural Sinus Thrombosis](/document/dural-sinus-thrombosis/4e81a1de-df92-4172-99ec-1377b0d9d188) + - Look for thrombosed sinus (empty delta sign, etc.) +- ## Postsurgical Dural Thickening + + + - Look for other postoperative findings (e.g., burr holes) + - May occur almost immediately after surgery, persist for months/years +- ## Idiopathic Hypertrophic Cranial Pachymeningitis + + + - Headache usually not orthostatic + - May cause bone invasion + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Dural thickening, enhancement due to venous engorgement + - Common cause of IH = spontaneous spinal CSF leak + - Weak dura ± arachnoid diverticulae common + - Aberrant extracellular matrix with abnormalities of fibrillin-containing microfibrils + - Most (but not all) cases caused by reduced CSF pressure precipitated by + - Surgery (CSF overshunting) or trauma (including trivial fall) + - Vigorous exercise or violent coughing + - Diagnostic lumbar puncture + - Spontaneous dural tear, ruptured arachnoid diverticulum + - Severe dehydration + - Disc herniation or osteophyte (rare) + - Pathophysiology = Monro-Kellie doctrine + - CSF, intracranial blood volume vary inversely + - In face of low CSF pressure, dural venous plexuses dilate + - ### Associated abnormalities + + + - Dilated cervical epidural venous plexus, spinal hygromas, retrospinal fluid collections + - Typical + - Low opening pressure, < 6 cm H₂O + - CSF frequently shows pleocytosis, ↑ protein + - Variant + - Opening pressure occasionally normal (CSF hypovolemia rather than hypotension) + - Stigmata of systemic connective tissue disorder found in up to 2/3 of patients + - Marfan, Ehlers-Danlos type 2 + - Clinical findings = minor skeletal features, small joint hypermobility, etc.; may be subtle +- ## Gross Pathologic & Surgical Features + + + - Surgical specimen generally unremarkable with grossly normal-appearing dura + - Spinal meningeal diverticula (often multiple), dural holes/rents common + - No specific leakage site identified at surgery in at least 50% +- ## Microscopic Features + + + - Meningeal surface normal + - No evidence for inflammation or neoplasia + - Inner surface + - Layer of numerous delicate, thin-walled dilated, vessels often attached to inner surface + - Nests of meningothelial cells may be prominent, should not be misinterpreted as meningioma + - May show marked arachnoidal, dural fibrosis if longstanding + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Severe headache (orthostatic, persistent, pulsatile, or even associated with nuchal rigidity) + - Uncommon: Cranial nerve (CN) palsy (e.g., abducens), visual disturbances + - Rare: Severe encephalopathy with disturbances of consciousness + - ### Clinical profile + + + - Young/middle-aged adult with orthostatic headache + - Lack of orthostatic headache should not exclude spontaneous IH! + - Opening pressure often < 6 cm H₂O but normal pressure common and should not exclude diagnosis +- ## Demographics + + + - ### Age + + + - Peak in 3rd, 4th decades +- ## Natural History & Prognosis + + + - Most IH cases resolve spontaneously + - Dural thickening, enhancement disappears; midline structures return (ascend) to normal position + - Rare: Coma, death from severe intracranial herniation +- ## Treatment + + + - "Blind" (nontargeted) epidural blood patch (EBP) + - Immediate: Replaces fluid (tamponade effect) + - Emergent intrathecal saline infusion if patient severely encephalopathic, obtunded + - Negative pressure gradient within epidural space + - Causes rostral flow of injected blood + - Proximal flow of blood products has "plug" effect + - Underreported complication = rebound intracranial **hyper**tension + - Targeted vs. blind EBP + - Targeted EBP 87% successful; 52% nontargeted + - 2-site EBP may be as efficacious as targeted EBP + - Surgery if blood patch fails (usually large dural tear) or subdural hematomas with acute clinical deterioration + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Frequently misdiagnosed clinically; imaging is key to diagnosis + - Do not misdiagnose IH as Chiari 1! + - Surgery can exacerbate symptoms; in rare cases can be fatal +- ## Image Interpretation Pearls + + + - Only rarely are **all** classic findings of IH present in the same patient + - Look for enlarged spinal epidural venous plexuses + + 88645d02-a7df-4c9f-a878-7d8e8140c877 + +## References + +# Selected References + +1. [Davies MJ et al: Epidural blood patch as a diagnostic and therapeutic intervention in spontaneous intracranial hypotension: a novel approach to management. World Neurosurg. 137:e242-50, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32004736%5Bpmid%5D) +1. [Gandhi J et al: Cerebrospinal fluid leaks secondary to dural tears: a review of etiology, clinical evaluation, and management. Int J Neurosci. 1-13, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32242448%5Bpmid%5D) +1. [Martineau P et al: Imaging of the spontaneous low cerebrospinal fluid pressure headache: a review. Can Assoc Radiol J. 71(2):174-85, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32063004%5Bpmid%5D) +1. [Ahn C et al: Two-site blind epidural blood patch versus targeted epidural blood patch in spontaneous intracranial hypotension. J Clin Neurosci. 62:147-54, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30497854%5Bpmid%5D) +1. [Amrhein TJ et al: Spontaneous intracranial hypotension: imaging in diagnosis and treatment. Radiol Clin North Am. 57(2):439-51, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30709479%5Bpmid%5D) +1. [Chan SM et al: Intracranial hypotension and cerebrospinal fluid leak. Neuroimaging Clin N Am. 29(2):213-26, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30926112%5Bpmid%5D) +1. [Farb RI et al: Spontaneous intracranial hypotension: a systematic imaging approach for CSF leak localization and management based on MRI and digital subtraction myelography. AJNR Am J Neuroradiol. 40(4):745-53, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30923083%5Bpmid%5D) +1. [Guryildirim M et al: Acute headache in the emergency setting. Radiographics. 39(6):1739-59, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31589569%5Bpmid%5D) +1. [Kim JH et al: Clinical Features of patients with spontaneous intracranial hypotension complicated with bilateral subdural fluid collections. Headache. 59(5):775-86, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30985923%5Bpmid%5D) +1. [Kim SC et al: MRI findings of spontaneous intracranial hypotension: usefulness of straight sinus distention. AJR Am J Roentgenol. 1-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30807225%5Bpmid%5D) +1. [Kranz PG et al: Spontaneous intracranial hypotension: pathogenesis, diagnosis, and treatment. Neuroimaging Clin N Am. 29(4):581-94, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31677732%5Bpmid%5D) +1. [Wang DJ et al: The interpeduncular angle: a practical and objective marker for the detection and diagnosis of intracranial hypotension on brain MRI. AJNR Am J Neuroradiol. 40(8):1299-1303, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31296521%5Bpmid%5D) +1. [Kranz PG et al: Spontaneous intracranial hypotension: 10 myths and misperceptions. Headache. 58(7):948-59, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29797515%5Bpmid%5D) + +## Cases + +- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '08037059-66e8-40f3-97d5-33a03f1f61ec', 'description': 'C4-5 level CSF leaks along roots sleeves.\n\nMR of the brain (#1-3) shows typical diffuse dural enhancement of intracranial hypotension.\n\nPost myelographic CT images (#4-6) show contrast extending along the roots sleeves bilaterally at C4-6 out into the cervical plexus (arrows).\n\nPatient was treated with multiple blood patches.\n\nPatients with persistent and "occult" CSF leaks do not infrequently have multiple sources involving the cervical-thoracic junction or thoracic spine. Myelography, myelo CT, nuclear cisternography and heavily T2-weighted MR imaging may all be used to try and localize the source of leak.', 'history': 'Severe positional headache.', 'imagePoolId': '4434b392-889b-4575-8190-0b401b9ae3c1', 'name': 'Bilateral cervical root sleeve CSF leaks', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '7fc99261-e3c8-4d04-af7d-99044309e2fe', 'description': "Sagittal T1WI (#1) shows several interesting findings. The midbrain appears "slumped", and the angle between it and the pons (usually almost 90 degrees) appears much more "closed" than normal (open arrow). The cerebellar tonsil lies well below the foramen magnum (white arrow). The pituitary gland is convex upwards, and the optic chiasm is draped over the dorsum sellae (curved arrow). Axial T1WI (#2) through the suprasellar cistern shows tissue- not CSF- within the suprasellar area (arrows). The temporal lobes appear herniated medially (curved arrows), and the surface sulci are obliterated. The axial T2WI (#3) shows that the "flow void" of the superior sagittal sinus (arrow) is unusually large and has convex margins.\n\nThe post-contrast axial (#4-7) and coronal (#8-9) T1WIs are very interesting. They show diffuse dural thickening and enhancement (open arrows), and all the dural sinuses are enlarged, with convex-appearing margins (arrows). Even the superior ophthalmic veins (curved arrows, #5) appear unusually prominent.\n\nSagittal T2WI (#10) through the cervical spine shows very prominent epidural spaces (arrows), probably secondary to engorgement of the epidural venous plexuses.\n\nComment: The low tonsils are round, not pointed (as would be seen in a Chiari 1 malformation). The "fat" pituitary, "slumped" midbrain, diffuse dural thickening and enhancement, and engorged venous sinuses with convex (not concave) margins all indicate intracranial hypotension.\n\nThe patient's symptoms resolved after epidural blood patch, and follow-up MR (not shown) appeared normal.", 'history': 'Worsening postural headaches.', 'imagePoolId': 'a6aae8a1-3c99-4ca6-8c55-239c1fad6ec7', 'name': 'Amazing enlargement of venous sinuses', 'teachingPoint': None, 'demographics': '32 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'f806495a-4edd-416b-9d5d-b395d9a5b417', 'description': 'Intracranial hypotension causes venous engorgement of the dura. Occasionally, it may cause pituitary enlargement and mimic adenoma or pituitary hyperplasia, as happened in this case.\n\nSagittal T1WI MR (#1), demonstrates sagging midbrain. Note upward bulging pituitary gland with draping of the optic chiasm over the gland (arrow). Coronal T1 C+ MR through the sella (#2), shows an enlarged pituitary gland (open arrow) with mild dural thickening (arrows). Coronal post-contrast T1 weighted MR (#3), shows the typical dural thickening and venous engorgement of classic intracranial hypotension.', 'history': 'Intractable headaches relieved by lying down.', 'imagePoolId': '7252d1cc-cefb-47fd-a08a-3a54861f14cd', 'name': 'Enlarged pituitary gland', 'teachingPoint': None, 'demographics': '48 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'f0e41a00-8504-4ea8-a1f1-09123ac2ce47', 'description': 'Sagittal T1WI (#1) shows slight inferior displacement of the tonsils below the foramen magnum (arrow) and decreased distance between the optic chiasm and sella (curved arrow). The midbrain is not obviously "slumping." FLAIR scan (#2) shows hyperintense thickened dura (arrows). Axial (#3) and coronal (#4, 5) show diffusely enhancing, smoothly thickened dura. \n\nThis is a case where the central inferior midbrain displacement is minimal but the dural engorgement and thickening is relatively striking. Intracranial hypotension (in this case, spontaneous) may display a spectrum of findings, not all of which are present in every case.', 'history': 'Headaches. No history of trauma.', 'imagePoolId': 'c4de09d4-8e1a-456e-9b77-e057efe38740', 'name': 'Mild slumping midbrain, striking dural enhancement', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '98e82d35-9959-47fa-95dc-2ba5e8500712', 'description': "Intracranial hypotension is typically caused by CSF leak. History of trauma or other predisposing condition is often lacking and the diagnosis is established when patients are imaged for headache. Contrast-enhanced scans should be done when sagittal T1WI MR suggests "slumping midbrain" (as was seen in this case).\n\nSagittal T1 weighted MR scan shows the classic appearance of "slumping midbrain," with the junction between the midbrain and pons displaced inferiorly below the level of the dorsum sellae (#1, arrow). The suprasellar cistern may be effaced, with the optic chiasm and hypothalamus draped over the dorsum (#1, open arrow). Tonsillar herniation is common but is minimal in this case. Axial pre-contrast T1WI shows the suprasellar cistern is effaced and both temporal lobes have herniated medially over the tentorial incisura (#2, arrows). Axial post-contrast T1WIs (#3-4) show diffuse dural enhancement that extends into the internal auditory canals. Diagnosis was intracranial hypotension and a blood patch was performed. Patient's symptoms resolved.\n\nFollow-up scan (#5-6) performed 12 months later was normal. Note normal position of the pons and optic chiasm (#5) and resolution of abnormal dural thickening and enhancement (#6) as compared to the original examination.", 'history': 'Intractable chronic headaches, relieved in supine position.', 'imagePoolId': '1df83528-3d6c-470c-8e8b-e1be7a4e6567', 'name': 'Classic with resolution s/p blood patch', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '7eff3cb8-762c-48f0-872a-5825f9f21379', 'description': "Intracranial hypotension can be life-threatening if the downwards herniation becomes severe or if subdural hematomas develop.\n\nCoronal T2WI MR (#1), shows the fluid collections are SDHs of different ages. Drainage of the SDHs without recognizing the underlying diagnosis of spontaneous IH caused worsening of the patient's symptoms. Sagittal T1 C+ MR (#2), shows obliteration of suprasellar cistern, sagging and fat midbrain with closed angle between peduncles/pons (arrow), dural enhancement, tonsillar descent. Axial T1 C+ MR (#3), shows diffuse dural enhancement, hypodense extra-axial fluid collections, small ventricles with medial deviation of choroid and ICVs (arrows) caused by midbrain descent. Coronal T1 C+ MR (#4), shows subdural fluid (open arrows) with diffuse dural thickening extending into both IACs (arrows). Lateral ventricles are pulled towards the midline.", 'history': 'Severe postural headaches for several weeks, presented with drowsiness and decreased mental status.', 'imagePoolId': '173f8ea3-1bd0-4b35-a298-bb631d7829bb', 'name': 'Classic MR, severe', 'teachingPoint': None}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': 'a3fd8b37-12fd-4db2-9cee-66847267a893', 'description': 'Brain study (#1, 2) show typical appearance of intracranial hypotension with diffuse dural enhancement both above and below the tentorium. Radionuclide cisternography (anterior view) (#3) show a large amount of activity spilling from the spinal thecal sac into the right chest (arrows). CT following myelogram (#4-12) shows leakage of contrast from the thoracic thecal sac through the right T4-5 neural foramen into the right pleural space (arrows). More inferior axial slice (#8, 12) shows prominent epidural soft tissue due to distended epidural veins (curved arrows). MR study (#13-17) shows diffuse dural thickening and enhancement (arrows, #13-14), and the site of CSF leak extending from the right neural foramen (#15-17). Patient required right laminectomy at T4 to repair the leak.', 'history': 'Recent right thoracotomy for lung carcinoma, now with unremitting severe postural headache.', 'imagePoolId': 'abe41d93-65e8-42fe-b525-1714847527e8', 'name': 'CSF leak following thoracotomy', 'teachingPoint': None, 'demographics': '50 Years old female'}, {'authors': [{'key': '33151213-01b2-4542-9105-342e006b3915', 'value': 'H. Ric Harnsberger, MD'}], 'caseVersionId': '57d36d6f-00c4-4fb7-887c-4c81c842cc8a', 'description': 'Variant CT-MR case of arachnoid granulation in the area of the petromastoid canal (subarcuate canaliculus) with associated CSF leak, intracranial hypotension, and multiple episodes of meningitis.\n\nAxial bone CT images (#1-2) reveal a large arachnoid granulation in the roof of the petrous apex (arrow, #1) and opacification of the middle ear and mastoid (curved arrows, #1-2). CT with intrathecal contrast shows opacified CSF traversing the arachnoid granulation (arrows, #3-4) into temporal bone air cells and into the middle ear cavity (curved arrow, #4).\n \nAn axial T2 MR image (#5) demonstrates fluid signal in the middle ear and mastoid (curved arrows) from CSF leak into these areas. Axial (#6) and coronal (#7-9) T1 C+ fat-saturated MR images show the non-enhancing arachnoid granulation in the petrous apex (arrows, #6-9). Diffuse dural enhancement (open arrows, #7-9) most likely is secondary to intracranial hypotension from the CSF leak. Pia-arachnoid enhancement seen along the nerves of the IAC (curved arrows, #9) results from the recurrent meningitis episodes.', 'history': 'Patient presents with recurrent episodes of meningitis; skull base CT shows an abnormality in the right petrous apex. CT with intrathecal contrast and MR completed for further evaluation.', 'imagePoolId': 'c602dfeb-e877-4a87-871c-c610cb8b21a6', 'name': 'Variant AG with CSF leak and intracranial hypotension', 'teachingPoint': None, 'demographics': '75 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '81585ec2-bd1f-4481-8632-ff6b10bb2e7d', 'description': 'Sagittal T1WI (#1) shows a slightly "slumping" brainstem with the hypothalamus and optic chiasm draped over the dorsum sellae (curved arrow). Note absence of tonsillar herniation. Axial T1WI (#2) shows both temporal lobes are herniated medially over the tentorium (arrows) and there is complete effacement of the suprasellar subarachnoid cistern, seen here as absence of CSF. Axial T2WI through the posterior fossa (#3) shows a hyperintense band (arrows) around both cerebellar hemispheres (arrows). Post-contrast axial (#4-5) T1 C+ scans show diffuse dural enhancement that surrounds the cerebral and cerebellar hemispheres and even extends into both internal auditory canals (#4, arrows).\n\nNot all findings of intracranial hypotension are present in every case. This example is unusual because it lacks the downwards tonsillar herniation (sometimes misnamed "acquired Chiari I") that is seen in most cases.', 'history': 'Severe headaches relieved by supine position.', 'imagePoolId': '00a36dac-2e02-40ae-9c43-499956f81a67', 'name': 'Desc transtentorial herniation', 'teachingPoint': None}], 'caseType': 'variant', 'name': 'VARIANT'} + + +## Images + + +### Selected Images + +![Graphic shows IH with distended dural sinuses and enlarged pituitary and herniated tonsils . Central brain descent causes midbrain slumping, inferiorly displaced pons, closed pons-midbrain angle , and splenium depressing ICV/vein of Galen junction .](images/app.statdx.com_image_thumbnail_3f47f189-81ba-42ab-a5f7-5b241e621ca1_annotated_true_size_900_quality_90_d63c8d90_20251018T164727Z.jpg) +*Graphic shows IH with distended dural sinuses and enlarged pituitary and herniated tonsils . Central brain descent causes midbrain slumping, inferiorly displaced pons, closed pons-midbrain angle , and splenium depressing ICV/vein of Galen junction .* + +![Graphic shows IH with distended dural sinuses and enlarged pituitary and herniated tonsils . Central brain descent causes midbrain slumping, inferiorly displaced pons, closed pons-midbrain angle , and splenium depressing ICV/vein of Galen junction .](images/app.statdx.com_image_thumbnail_3f47f189-81ba-42ab-a5f7-5b241e621ca1_size_174_quality_85_38151f0c_20251018T164552Z.jpg) +*Graphic shows IH with distended dural sinuses and enlarged pituitary and herniated tonsils . Central brain descent causes midbrain slumping, inferiorly displaced pons, closed pons-midbrain angle , and splenium depressing ICV/vein of Galen junction .* + +![T2 MR in a 57-year-old man treated for migraine headaches shows severe midbrain slumping , downwardly displaced cerebellar tonsils , and "draping" of the hypothalamus over the dorsum sellae with mammillary bodies below the dorsum.](images/app.statdx.com_image_thumbnail_bbafbf07-9d0c-4fe0-8e81-fcbe63a510fc_annotated_true_size_900_quality_90_2000dafd_20251018T164727Z.jpg) +*T2 MR in a 57-year-old man treated for migraine headaches shows severe midbrain slumping , downwardly displaced cerebellar tonsils , and "draping" of the hypothalamus over the dorsum sellae with mammillary bodies below the dorsum.* + +![Sagittal T1 C+ FS MR in the same patient shows the severe midbrain slumping and inferiorly displaced tonsils. In addition, the pituitary gland appears "fat" and the dural venous sinuses are engorged .](images/app.statdx.com_image_thumbnail_e7a59de5-ab4b-48bb-9500-1858e5026fbc_annotated_true_size_900_quality_90_1e0d484e_20251018T164727Z.jpg) +*Sagittal T1 C+ FS MR in the same patient shows the severe midbrain slumping and inferiorly displaced tonsils. In addition, the pituitary gland appears "fat" and the dural venous sinuses are engorged .* + +![Axial T1 C+ FS MR in the same patient shows a "fat" midbrain/pons , prominent superior ophthalmic veins , and engorged, outwardly convex transverse/sigmoid sinuses . No subdural hematomas were identified. Severe IH was treated successfully with a blood patch.](images/app.statdx.com_image_thumbnail_0325d630-656f-4d97-b5a9-0ab52423cdfd_annotated_true_size_900_quality_90_89e7c2fe_20251018T164727Z.jpg) +*Axial T1 C+ FS MR in the same patient shows a "fat" midbrain/pons , prominent superior ophthalmic veins , and engorged, outwardly convex transverse/sigmoid sinuses . No subdural hematomas were identified. Severe IH was treated successfully with a blood patch.* + +![Axial NECT in a 55-year-old man with a severe headache in the ER shows downward herniation of both cerebellar tonsils through the foramen magnum.](images/app.statdx.com_image_thumbnail_f21667f8-6e56-4344-99f0-01881e854752_annotated_true_size_900_quality_90_4924f2e1_20251018T164727Z.jpg) +*Axial NECT in a 55-year-old man with a severe headache in the ER shows downward herniation of both cerebellar tonsils through the foramen magnum.* + +![More cephalad NECT in the same patient shows effacement of all basal cisterns, especially the suprasellar cistern . The midbrain appears "fat" . The imaging findings are suggestive of IH.](images/app.statdx.com_image_thumbnail_5fd9a6e2-7068-49f5-8e86-556f3db7c42e_annotated_true_size_900_quality_90_127dfa34_20251018T164727Z.jpg) +*More cephalad NECT in the same patient shows effacement of all basal cisterns, especially the suprasellar cistern . The midbrain appears "fat" . The imaging findings are suggestive of IH.* + +![Sagittal T1WI MR in the same patient shows changes of severe IH with midbrain slumping , downward tonsillar displacement , "fat" pituitary gland , and "draping" of the optic chiasm/hypothalamus over the dorsum sellae .](images/app.statdx.com_image_thumbnail_580c9dda-241e-459c-9d69-df0d5191353d_annotated_true_size_900_quality_90_00072ee4_20251018T164727Z.jpg) +*Sagittal T1WI MR in the same patient shows changes of severe IH with midbrain slumping , downward tonsillar displacement , "fat" pituitary gland , and "draping" of the optic chiasm/hypothalamus over the dorsum sellae .* + +![Axial T2WI MR in the same patient shows the inferiorly displaced hypothalamus and 3rd ventricle obliterating the suprasellar cistern . The midbrain appears "fat" and elongated.](images/app.statdx.com_image_thumbnail_455ec428-a8ce-408e-94c3-7b2facee16e4_annotated_true_size_900_quality_90_b287112d_20251018T164727Z.jpg) +*Axial T2WI MR in the same patient shows the inferiorly displaced hypothalamus and 3rd ventricle obliterating the suprasellar cistern . The midbrain appears "fat" and elongated.* + +![Axial T1 C+ FS MR in the same patient shows smooth, diffuse dura-arachnoid enhancement , and an engorged, outwardly convex superior sagittal sinus .](images/app.statdx.com_image_thumbnail_f63a1d44-5412-4dd6-ab9d-dd2091c16f6b_annotated_true_size_900_quality_90_eb95d811_20251018T164727Z.jpg) +*Axial T1 C+ FS MR in the same patient shows smooth, diffuse dura-arachnoid enhancement , and an engorged, outwardly convex superior sagittal sinus .* + +![Coronal T1 C+ FS MR in the same patient shows the diffuse dura-arachnoid enhancement extends into both internal auditory canals . The lateral ventricles have a more acute angle and appear "pulled down" toward the incisura. An epidural blood patch relieved the symptoms.](images/app.statdx.com_image_thumbnail_5276ba18-7d76-4d61-8a2b-6058ab8f226b_annotated_true_size_900_quality_90_07652c9d_20251018T164731Z.jpg) +*Coronal T1 C+ FS MR in the same patient shows the diffuse dura-arachnoid enhancement extends into both internal auditory canals . The lateral ventricles have a more acute angle and appear "pulled down" toward the incisura. An epidural blood patch relieved the symptoms.* + + +### Additional Images + +![Sagittal T1WI C+ MR in a patient with severe IH shows obliteration of the suprasellar cistern, "sagging" and "fat" midbrain with closed angle between the peduncles/pons , dural enhancement, and tonsillar descent.](images/app.statdx.com_image_thumbnail_853d940e-4247-482c-8fbb-cea42f02bc4e_annotated_true_size_900_quality_90_2c33b49b_20251018T164731Z.jpg) +*Sagittal T1WI C+ MR in a patient with severe IH shows obliteration of the suprasellar cistern, "sagging" and "fat" midbrain with closed angle between the peduncles/pons , dural enhancement, and tonsillar descent.* + +![Axial T1WI C+ MR in the same patient shows diffuse dural enhancement, hypodense extraaxial fluid collections, and small ventricles with medial deviation of the choroid and internal cerebral veins caused by midbrain descent.](images/app.statdx.com_image_thumbnail_9e92d281-2a10-418d-bb72-522547caf29b_annotated_true_size_900_quality_90_e8be1938_20251018T164731Z.jpg) +*Axial T1WI C+ MR in the same patient shows diffuse dural enhancement, hypodense extraaxial fluid collections, and small ventricles with medial deviation of the choroid and internal cerebral veins caused by midbrain descent.* + +![Coronal T1WI C+ MR in the same patient shows subdural fluid with diffuse dural thickening extending into both internal auditory canals . The lateral ventricles are pulled toward the midline.](images/app.statdx.com_image_thumbnail_4bc5f106-f884-480a-bbc6-1d456488613e_annotated_true_size_900_quality_90_de26b61d_20251018T164731Z.jpg) +*Coronal T1WI C+ MR in the same patient shows subdural fluid with diffuse dural thickening extending into both internal auditory canals . The lateral ventricles are pulled toward the midline.* + +![Coronal T2WI MR shows that the fluid collections are subdural hematomas of different ages. Drainage of the subdural hematomas without recognizing the underlying diagnosis of spontaneous IH caused worsening of the patient's symptoms.](images/app.statdx.com_image_thumbnail_1c0fc05e-ae2a-4271-a79d-cfe79cb4cbaf_annotated_true_size_900_quality_90_7a7da97d_20251018T164731Z.jpg) +*Coronal T2WI MR shows that the fluid collections are subdural hematomas of different ages. Drainage of the subdural hematomas without recognizing the underlying diagnosis of spontaneous IH caused worsening of the patient's symptoms.* + +![Axial T1WI C+ MR at the C2 level in a patient with spontaneous IH shows the draped curtain appearance of the markedly engorged epidural venous plexus . Brain MR (not shown) showed only mild dural enhancement.](images/app.statdx.com_image_thumbnail_7b81ece7-66b8-483a-84be-c9d986b4f22c_annotated_true_size_900_quality_90_24b3411c_20251018T164731Z.jpg) +*Axial T1WI C+ MR at the C2 level in a patient with spontaneous IH shows the draped curtain appearance of the markedly engorged epidural venous plexus . Brain MR (not shown) showed only mild dural enhancement.* + +![Coronal T1WI C+ MR in the same patient shows an enlarged pituitary gland with mild dural thickening .](images/app.statdx.com_image_thumbnail_feba1690-6fec-4c28-b9e4-0599b470158e_annotated_true_size_900_quality_90_ad0019d8_20251018T164732Z.jpg) +*Coronal T1WI C+ MR in the same patient shows an enlarged pituitary gland with mild dural thickening .* + +![Sagittal T1WI MR shows a rounded, "plump" pituitary gland , often seen in IH. Note the effaced suprasellar cistern with optic chiasm draped over the pituitary gland, classic "slumping" midbrain, with decreased angle between the pons and midbrain .](images/app.statdx.com_image_thumbnail_9d45611b-ca6b-412d-b18e-3e28ed55daa7_annotated_true_size_900_quality_90_8502c539_20251018T164732Z.jpg) +*Sagittal T1WI MR shows a rounded, "plump" pituitary gland , often seen in IH. Note the effaced suprasellar cistern with optic chiasm draped over the pituitary gland, classic "slumping" midbrain, with decreased angle between the pons and midbrain .* + +![Sagittal T1WI MR shows "sagging" midbrain, tonsillar herniation, and the optic chiasm "draped" over the dorsum sellae. T1WI C+ MR (not shown) demonstrated diffuse dural enhancement in this classic case of spontaneous IH.](images/app.statdx.com_image_thumbnail_df286399-1c32-47a3-919c-d093c5e1699b_annotated_true_size_900_quality_90_81f62a20_20251018T164732Z.jpg) +*Sagittal T1WI MR shows "sagging" midbrain, tonsillar herniation, and the optic chiasm "draped" over the dorsum sellae. T1WI C+ MR (not shown) demonstrated diffuse dural enhancement in this classic case of spontaneous IH.* + +![Axial T1WI C+ FS MR in a patient with spontaneous IH shows diffuse dura-arachnoid thickening from venous engorgement. Note extension into cerebellopontine angles .](images/app.statdx.com_image_thumbnail_00b6b708-959a-4d66-ae4a-958137970aad_annotated_true_size_900_quality_90_d535a53a_20251018T164732Z.jpg) +*Axial T1WI C+ FS MR in a patient with spontaneous IH shows diffuse dura-arachnoid thickening from venous engorgement. Note extension into cerebellopontine angles .* + +![Axial T1WI C+ MR in the same patient after the blood patch shows complete resolution of dura-arachnoid enhancement.](images/app.statdx.com_image_thumbnail_d9028720-92df-433e-8d90-e962df59b5ef_annotated_true_size_900_quality_90_21f8c1e2_20251018T164732Z.jpg) +*Axial T1WI C+ MR in the same patient after the blood patch shows complete resolution of dura-arachnoid enhancement.* + diff --git a/docs_md/articles/intraventricular-obstructive-hydrocephalus_eeac8d9b-1fdc-432e-8e09-11589611f7a8.md b/docs_md/articles/intraventricular-obstructive-hydrocephalus_eeac8d9b-1fdc-432e-8e09-11589611f7a8.md new file mode 100644 index 0000000..016feb6 --- /dev/null +++ b/docs_md/articles/intraventricular-obstructive-hydrocephalus_eeac8d9b-1fdc-432e-8e09-11589611f7a8.md @@ -0,0 +1,492 @@ +--- +title: "Intraventricular Obstructive Hydrocephalus" +docid: "eeac8d9b-1fdc-432e-8e09-11589611f7a8" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "Intraventricular Obstructive Hydrocephalus" + slug: "intraventricular-obstructive-hydro-" + treeNodeId: null +category: "Brain" +cmeTopicId: "7f17d5cc-6470-403d-8f99-cbb3d3ea610d" +documentVersionId: "102c8265-55ef-4963-964e-d8ed6d766992" +imageCount: 30 +lastUpdated: "09/24/20" +pageDescription: "Intraventricular Obstructive Hydrocephalus" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Intraventricular Obstructive Hydrocephalus" +pageTitle: "Intraventricular Obstructive Hydrocephalus | STATdx" +enhancedTitle: "Intraventricular Obstructive Hydrocephalus" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "Intraventricular Obstructive Hydrocephalus" +--- +# KEY FACTS + +- ## Terminology + + + - Intraventricular obstructive hydrocephalus (IVOH) = obstruction proximal to foramina of Luschka, Magendie + - Acute IVOH (aIVOH) + - Chronic compensated IVOH (cIVOH) +- ## Imaging + + + - aIVOH = ballooned ventricles plus indistinct (blurred) margins + - "Fingers" of CSF extend into periventricular WM + - Most striking around ventricular horns (periventricular halos) + - After decompression, corpus callosum may show hyperintensity + - cIVOH = ballooned ventricles without periventricular halo +- ## Top Differential Diagnoses + + + - Ventricular enlargement secondary to parenchymal loss + - Normal-pressure hydrocephalus + - Extraventricular obstructive hydrocephalus + - Choroid plexus papilloma +- ## Pathology + + + - Intraventricular obstruction to CSF flow + - CSF production continues, ventricular pressure ↑ + - Ventricles expand, compress adjacent parenchyma + - Periventricular interstitial fluid ↑ + - Leads to myelin vacuolization, destruction + - Pathology varies depending on obstruction etiology +- ## Clinical Issues + + + - Varies with acuity, severity + - Headache, papilledema (aIVOH) + - Nausea, vomiting, diplopia (6th nerve palsy) +- ## Diagnostic Checklist + + + - Size of ventricles generally correlates poorly with intracranial pressure + +# TERMINOLOGY + +- ## Abbreviations + + + - Intraventricular obstructive hydrocephalus (IVOH) + - Acute IVOH (aIVOH) + - Chronic compensated IVOH (cIVOH) +- ## Synonyms + + + - Noncommunicating hydrocephalus +- ## Definitions + + + - Enlarged ventricles caused by physical obstruction at or proximal to 4th ventricular outflow foramina (of Luschka, Magendie) + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - aIVOH + - Ballooned ventricles with indistinct (blurred) margins + - cIVOH + - Ballooned ventricles without periventricular halo + - ### Size + + + - Bifrontal horn:intracranial diameter ratio > 0.3 + - Temporal horn width > 3 mm + - ### Morphology + + + - Varies with site, duration of blockage + - Global/focally enlarged ventricle(s) ± ↑ intracranial pressure (ICP) + - Ventricles proximal to obstruction enlarge, appear more rounded + - Look for enlarged anterior recesses of 3rd ventricle +- ## CT Findings + + + - ### NECT + + + - Large ventricles proximal to obstruction + - aIVOH + - Ballooned ventricles with periventricular low-density halo + - cIVOH + - Ballooned ventricles without thick periventricular halo + - Basal cisterns, sulci compressed/obliterated +- ## MR Findings + + + - ### T1WI + + + - Lateral ventricles enlarged + - Corpus callosum (CC) thinned, stretched upward + - May be impinged against falx + - Impaction may cause pressure necrosis + - Fornix, internal cerebral veins (ICV) displaced downward + - Enlarged 3rd ventricle often herniated into expanded sella + - Funnel-shaped aqueduct of Sylvius in aqueductal stenosis + - ### T2WI + + + - aIVOH + - "Fingers" of CSF-like hyperintensity extend into periventricular white matter (WM), most striking around ventricular horns (periventricular halos) + - Disturbed/turbulent CSF flow + - Absent aqueductal flow void common + - CC may appear hyperintense + - cIVOH + - Large ventricles, normal CSF pressure + - No periventricular halo + - CC may show hyperintensity after decompression (15% of shunted IVOH cases) + - Thin-section T2WI, FIESTA, or CISS sequences + - Exquisitely delineate CSF spaces + - May demonstrate subtle abnormalities not detected on standard images + - ### FLAIR + + + - Fluid in periventricular halo does not suppress + - ### T1WI C+ + + + - Neoplasm causing IVOH may enhance + - aIVOH may cause leptomeningeal vascular stasis, enhancement + - Can mimic meningitis, metastases + - ### MRS + + + - Small lactate resonances can be detected in up to 20% of CSF spaces, even if no hydrocephalus +- ## Other Modality Findings + + + - Contrast-enhanced ventriculography + - MR/CT used to identify site of obstruction, status of 3rd ventriculostomies + - MR can be used for assessing CSF flow + - Cardiac gated-phase contrast MR + - May show absent aqueductal CSF flow +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR with contrast to evaluate cause of CSF obstruction + - ### Protocol advice + + + - 3D FEISTA/CISS + - ↓ CSF flow artifact + - Allows better delineation of ventricular contour, septa + - Sagittal high-resolution T2-weighted images + +# DIFFERENTIAL DIAGNOSIS + +- ## Ventricular Enlargement Secondary to Parenchymal Loss + + + - Old term: Ex vacuo hydrocephalus (not used) + - Age related (ventricular volume ↑ 1.2-1.4 mL after 60 years) + - Ischemia/infarction, trauma, infection, toxic + - Obtuse frontal angle (> 110°) + - Diffuse/focal enlargement of sulci, cisterns + - Normal lateral ventricles can be asymmetric (related to handedness, not sex) + - May correlate with some psychiatric disorders (e.g., schizophrenia) +- [Normal-Pressure Hydrocephalus](/document/normal-pressure-hydrocephalus/ba3f857d-58de-4f21-8463-1631b4cb9972) + - Progressive dementia, gait disturbance, incontinence + - Ventricular dilation with normal CSF pressure + - Sulci normal/minimally enlarged + - ↑ CSF displacement through aqueduct + - MRS shows lactate peak +- [Extraventricular Obstructive Hydrocephalus](/document/extraventricular-obstructive-hydro-/a0886d4c-f504-4165-bb52-2400e2385f68) + - Dilated ventricles due to mismatch between CSF formation, absorption + - ↓ CSF absorption through arachnoid villi + - Subarachnoid hemorrhage most common cause + - Others: Meningitis, carcinomatosis, granulomatous disease +- [Choroid Plexus Papilloma](/document/choroid-plexus-papilloma/18e712f5-8553-487d-a939-044336cbf0ad) + - Accounts for 2-5% of childhood intracranial tumors + - Child < 5 years with ↑ ICP + - Most common lateral ventricle trigone + - May overproduce CSF + - Hemorrhage, tumor spread may cause IVOH +- ## Longstanding Overt Ventriculomegaly in Adults + + + - Early childhood onset or longstanding progression of hydrocephalus into adulthood + - Markedly enlarged ventricles, high ICP +- ## Benign Enlargement of Subarachnoid Spaces and Ventricles + + + - Seen in association with macrocephaly in infants + - Not associated with developmental delay + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Normal CSF production = ~ 0.4 mL/min, 500-600 mL/24 hrs + - Total volume of CSF in adult = 125-150 mL + - Intraventricular obstruction to CSF flow; as CSF production continues, ventricular fluid pressure ↑ + - Ventricles expand, compress adjacent parenchyma; stretching may rupture/open ependymal cell junctions + - Periventricular interstitial fluid ↑ → myelin destruction + - Etiology depends on site + - Foramen of Monro + - Colloid cyst + - Subependymal nodule, tuberous sclerosis complex + - Subependymal giant cell astrocytoma + - 3rd ventricle + - Pituitary macroadenoma + - Craniopharyngioma + - Aqueduct of Sylvius + - Aqueductal stenosis + - Tectal glioma + - Pineal region tumors + - 4th ventricle + - Medulloblastoma, ependymoma + - Glioma, pilocytic astrocytoma, hemangioblastoma + - Cerebellar infarct + - Congenital anomalies (Chiari malformations, Dandy-Walker malformations, rhombencephalosynapsis) + - Metastasis, neurocysticercosis, or meningioma can occur at multiple intraventricular locations + - ### Genetics + + + - Cell adhesion molecule L1 (*L1CAM*) only gene recognized to cause congenital hydrocephalus + - Located on X chromosome (Xq28) + - Complications of hydrocephalus + - CC impingement syndrome + - Medial atrial diverticula +- ## Gross Pathologic & Surgical Features + + + - Focal/generalized ventricular enlargement + - Ependyma, adjacent WM are secondarily injured + - Variable pathology depending on causative factor +- ## Microscopic Features + + + - ↑ periventricular extracellular space + - Ependymal lining damaged or lost; surrounding WM becomes pale and rarefied + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Varies with acuity, severity + - Headache, papilledema (aIVOH) + - Nausea, vomiting, diplopia (6th nerve palsy) + - ### Clinical profile + + + - Varies with etiology, severity, age of onset +- ## Demographics + + + - ### Age + + + - May be any age from in utero (congenital hydrocephalus) to adult + - ### Epidemiology + + + - Epidemiologic data varies widely, depending upon etiology and type of hydrocephalus +- ## Natural History & Prognosis + + + - Usually progressive unless treated +- ## Treatment + + + - Medical management to delay surgical intervention + - CSF diversion (shunt), endoscopic intervention, and ventriculostomy + - Surgery to alleviate primary cause of obstruction + - Most common neurosurgical procedure in children = CSF shunting for hydrocephalus + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Longstanding aqueductal stenosis can be caused by slow-growing tectal tumor + - Rarely, hydrocephalus caused by spinal tumor +- ## Image Interpretation Pearls + + + - Size of ventricles generally correlates poorly with ICP + - Pulsatile CSF may create confusing signal intensity, even mimic intraventricular mass + - Ventricular asymmetry can be normal variant + - If seen with absent septum pellucidum in fetus/neonate, look for vermis (r/o rhombencephalosynapsis) + + 5b1dd5dd-62fc-4446-8e5d-a43b8e65444e + +## References + +# Selected References + +1. [Gholampour S et al: Comparing the efficiency of two treatment methods of hydrocephalus: shunt implantation and endoscopic third ventriculostomy. Basic Clin Neurosci. 10(3):185-98, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31462974%5Bpmid%5D) +1. [Santiago-Dieppa DR et al: Obstructive hydrocephalus. N Engl J Med. 381(5):e10, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31365804%5Bpmid%5D) +1. [Chellathurai A et al: Role of 3D SPACE sequence and susceptibility weighted imaging in the evaluation of hydrocephalus and treatment-oriented refined classification of hydrocephalus. Indian J Radiol Imaging. 28(4):385-94, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30662197%5Bpmid%5D) +1. [Hurni Y et al: Arrested hydrocephalus in childhood: case series and review of the literature. Neuropediatrics. 49(5):302-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29933461%5Bpmid%5D) +1. [Langner S et al: Diagnosis and differential diagnosis of hydrocephalus in adults. 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Childs Nerv Syst. 27(10):1543-62, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928020%5Bpmid%5D) +1. [Mirone G et al: Hydrocephalus and spinal cord tumors: a review. Childs Nerv Syst. 27(10):1741-9, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928038%5Bpmid%5D) +1. [Oi S: Classification of hydrocephalus: critical analysis of classification categories and advantages of "multi-categorical hydrocephalus classification" (Mc HC). Childs Nerv Syst. 27(10):1523-33, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928018%5Bpmid%5D) +1. [Dinçer A et al: Is all "communicating" hydrocephalus really communicating? Prospective study on the value of 3D-constructive interference in steady state sequence at 3T. AJNR Am J Neuroradiol. 30(10):1898-906, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19643921%5Bpmid%5D) +1. [Feng F et al: Evaluation of radionuclide cerebrospinal fluid scintigraphy as a guide in the management of patients with hydrocephalus. Clin Imaging. 33(2):85-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19237049%5Bpmid%5D) +1. [Linninger AA et al: Normal and hydrocephalic brain dynamics: the role of reduced cerebrospinal fluid reabsorption in ventricular enlargement. Ann Biomed Eng. 37(7):1434-47, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19373558%5Bpmid%5D) +1. [Oertel JM et al: Endoscopic third ventriculostomy in obstructive hydrocephalus due to giant basilar artery aneurysm. J Neurosurg. 110(1):14-8, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18991498%5Bpmid%5D) +1. [Stoquart-El Sankari S et al: Phase-contrast MR imaging support for the diagnosis of aqueductal stenosis. AJNR Am J Neuroradiol. 30(1):209-14, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18832663%5Bpmid%5D) +1. [Sekula RF Jr et al: A case of an elderly adult presenting with obstructive hydrocephalus secondary to a rare hemorrhagic suprasellar pilocytic astrocytoma. Clin Neuropathol. 27(6):396-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19130737%5Bpmid%5D) +1. [Yamada S et al: Visualization of cerebrospinal fluid movement with spin labeling at MR imaging: preliminary results in normal and pathophysiologic conditions. Radiology. 249(2):644-52, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18936318%5Bpmid%5D) +1. [Erdogan AR et al: Sex and handedness differences in size of cerebral ventricles of normal subjects. Int J Neurosci. 114(1):67-73, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14660068%5Bpmid%5D) +1. [Gaser C et al: Ventricular enlargement in schizophrenia related to volume reduction of the thalamus, striatum, and superior temporal cortex. Am J Psychiatry. 161(1):154-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14702264%5Bpmid%5D) +1. [Wyldes M et al: Isolated mild fetal ventriculomegaly. Arch Dis Child Fetal Neonatal Ed. 89(1):F9-13, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14711845%5Bpmid%5D) +1. [Akhondi H et al: Hydrocephalus as a presenting manifestation of neurosarcoidosis. South Med J. 96(4):403-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12916562%5Bpmid%5D) +1. [Bhattacharyya KB et al: Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature. Acta Neurol Scand. 108(3):216-20, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12911467%5Bpmid%5D) +1. Brown KP et al: 1H MRS in human hydrocephalus. J MRI. 14:291-9, 2003 +1. [Grunert P et al: The role of third ventriculostomy in the management of obstructive hydrocephalus. Minim Invasive Neurosurg. 46(1):16-21, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12640578%5Bpmid%5D) +1. [Joseph VB et al: MR ventriculography for the study of CSF flow. AJNR Am J Neuroradiol. 24(3):373-81, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12637285%5Bpmid%5D) +1. [Sener RN: Callosal changes in obstructive hydrocephalus: observations with FLAIR imaging, and diffusion MRI. Comput Med Imaging Graph. 26(5):333-7, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12204238%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial FLAIR MR in a patient with headache and vomiting demonstrates a colloid cyst at the foramen of Monro causing intraventricular obstructive hydrocephalus (IVOH) with dilatation of both lateral ventricles . Note thin rim of periventricular hyperintensity due to interstitial edema.](images/app.statdx.com_image_thumbnail_e5c174f6-9b00-41ca-b8e0-52124d6d5d33_annotated_true_size_900_quality_90_3aa6f8d6_20251018T164538Z.jpg) +*Axial FLAIR MR in a patient with headache and vomiting demonstrates a colloid cyst at the foramen of Monro causing intraventricular obstructive hydrocephalus (IVOH) with dilatation of both lateral ventricles . Note thin rim of periventricular hyperintensity due to interstitial edema.* + +![Axial FLAIR MR in a patient with headache and vomiting demonstrates a colloid cyst at the foramen of Monro causing intraventricular obstructive hydrocephalus (IVOH) with dilatation of both lateral ventricles . Note thin rim of periventricular hyperintensity due to interstitial edema.](images/app.statdx.com_image_thumbnail_e5c174f6-9b00-41ca-b8e0-52124d6d5d33_size_174_quality_85_febe2249_20251018T155131Z.jpg) +*Axial FLAIR MR in a patient with headache and vomiting demonstrates a colloid cyst at the foramen of Monro causing intraventricular obstructive hydrocephalus (IVOH) with dilatation of both lateral ventricles . Note thin rim of periventricular hyperintensity due to interstitial edema.* + +![Axial NECT in a patient with pineal region germinoma shows marked dilatation of the lateral ventricles and anterior 3rd ventricle with periventricular halo and diffuse effacement of the cortical sulci.](images/app.statdx.com_image_thumbnail_bb072fec-1cdc-4da0-8dfc-36cfc2a5a110_annotated_true_size_900_quality_90_6685e5f7_20251018T164538Z.jpg) +*Axial NECT in a patient with pineal region germinoma shows marked dilatation of the lateral ventricles and anterior 3rd ventricle with periventricular halo and diffuse effacement of the cortical sulci.* + +![Coronal T1 C+ MR in a patient presenting with headache and ataxia demonstrates a large heterogeneously enhancing mass in the left cerebellum with mass effect and effacement of the 4th ventricle . Biopsy revealed a glioblastoma.](images/app.statdx.com_image_thumbnail_a4d9bbda-78d1-493c-baaf-b7ab31b10da6_annotated_true_size_900_quality_90_3651409b_20251018T164538Z.jpg) +*Coronal T1 C+ MR in a patient presenting with headache and ataxia demonstrates a large heterogeneously enhancing mass in the left cerebellum with mass effect and effacement of the 4th ventricle . Biopsy revealed a glioblastoma.* + +![Axial FLAIR MR in the same patient shows marked dilatation of the lateral ventricles with extensive periventricular interstitial edema caused by compromised drainage of interstitial fluid or transependymal CSF migration.](images/app.statdx.com_image_thumbnail_2d8f3e24-47a1-4c45-9ecc-cebc09885e9d_annotated_true_size_900_quality_90_e373c7b5_20251018T164538Z.jpg) +*Axial FLAIR MR in the same patient shows marked dilatation of the lateral ventricles with extensive periventricular interstitial edema caused by compromised drainage of interstitial fluid or transependymal CSF migration.* + +![Sagittal CISS MR in a patient with obstruction at the 4th ventricular outlet due to adhesions shows ballooning of the 4th ventricle , widening of the aqueduct of Sylvius , dilated 3rd and lateral ventricles with downward sloping of 3rd ventricular floor .](images/app.statdx.com_image_thumbnail_91c986f1-b192-4ee0-86ad-ed4014373565_annotated_true_size_900_quality_90_efdb7ec4_20251018T164538Z.jpg) +*Sagittal CISS MR in a patient with obstruction at the 4th ventricular outlet due to adhesions shows ballooning of the 4th ventricle , widening of the aqueduct of Sylvius , dilated 3rd and lateral ventricles with downward sloping of 3rd ventricular floor .* + +![Axial FLAIR MR in a patient with tuberous sclerosis shows large subependymal giant cell astrocytoma causing obstructive hydrocephalus with mild periventricular edema . Note the subtle hyperintensity in the occipital lobe tuber .](images/app.statdx.com_image_thumbnail_e8bd350a-0dbd-4b07-a628-7ba634965a46_annotated_true_size_900_quality_90_49d18706_20251018T164538Z.jpg) +*Axial FLAIR MR in a patient with tuberous sclerosis shows large subependymal giant cell astrocytoma causing obstructive hydrocephalus with mild periventricular edema . Note the subtle hyperintensity in the occipital lobe tuber .* + +![Sagittal CISS MR in a patient with aqueductal stenosis due to a thin web causing obstructive hydrocephalus is shown. High resolution thin-section T2 MR exquisitely delineates the CSF spaces and may demonstrate subtle abnormalities not detected on standard sequences.](images/app.statdx.com_image_thumbnail_ef27fe2f-6eef-4306-8752-89fecd5fc331_annotated_true_size_900_quality_90_4217aa29_20251018T164538Z.jpg) +*Sagittal CISS MR in a patient with aqueductal stenosis due to a thin web causing obstructive hydrocephalus is shown. High resolution thin-section T2 MR exquisitely delineates the CSF spaces and may demonstrate subtle abnormalities not detected on standard sequences.* + +![Axial FLAIR MR in the same patient shows marked enlarged lateral ventricles with a very thin periventricular hyperintense rim and no sulcal effacement due to chronic compensated IVOH.](images/app.statdx.com_image_thumbnail_ec7e804b-5c09-4235-8429-ee0dc4fc2a7f_annotated_true_size_900_quality_90_cf502530_20251018T164538Z.jpg) +*Axial FLAIR MR in the same patient shows marked enlarged lateral ventricles with a very thin periventricular hyperintense rim and no sulcal effacement due to chronic compensated IVOH.* + +![Axial FLAIR MR in a patient with IVOH shows an ependymal cyst at the foramen of Monro with asymmetric dilatation of the lateral ventricles, L > R. There is marked bulging of the medial wall of the left lateral ventricle.](images/app.statdx.com_image_thumbnail_cb3411d0-41c7-433a-97e3-f121306cf24f_annotated_true_size_900_quality_90_069d90f2_20251018T164538Z.jpg) +*Axial FLAIR MR in a patient with IVOH shows an ependymal cyst at the foramen of Monro with asymmetric dilatation of the lateral ventricles, L > R. There is marked bulging of the medial wall of the left lateral ventricle.* + +![Sagittal T1 MR in the same patient demonstrates the large medial atrial diverticula , which herniates inferiorly through the tentorial incisura into the posterior fossa, compressing the vermis , tectal plate , aqueduct, and 4th ventricle .](images/app.statdx.com_image_thumbnail_9d4aeb38-2745-4513-9b2b-e51d38e60ec2_annotated_true_size_900_quality_90_efd2d55e_20251018T165016Z.jpg) +*Sagittal T1 MR in the same patient demonstrates the large medial atrial diverticula , which herniates inferiorly through the tentorial incisura into the posterior fossa, compressing the vermis , tectal plate , aqueduct, and 4th ventricle .* + + +### Additional Images + +![Sagittal T1WI MR shows large mass within the 4th ventricle causing IVOH or noncommunicating hydrocephalus.](images/app.statdx.com_image_thumbnail_111173b4-2ccd-4d7a-9423-8ea7a67d3faa_annotated_true_size_900_quality_90_db7227b6_20251018T165016Z.jpg) +*Sagittal T1WI MR shows large mass within the 4th ventricle causing IVOH or noncommunicating hydrocephalus.* + +![Sagittal T2WI MR in the same patient shows transependymal CSF flow, seen here as "fingers" extending into white matter around the enlarged lateral ventricle. The case was medulloblastoma with acute IVOH.](images/app.statdx.com_image_thumbnail_0096a55e-9e96-409e-ac0f-01c95f3d26bb_annotated_true_size_900_quality_90_b7750d59_20251018T165017Z.jpg) +*Sagittal T2WI MR in the same patient shows transependymal CSF flow, seen here as "fingers" extending into white matter around the enlarged lateral ventricle. The case was medulloblastoma with acute IVOH.* + +![Coronal T1 C+ MR shows IVOH with a large enhancing intraventricular mass causing marked enlargement of the lateral ventricles .](images/app.statdx.com_image_thumbnail_df9fd7e5-2974-4621-9d50-efceebb95d70_annotated_true_size_900_quality_90_ada0f1a6_20251018T165016Z.jpg) +*Coronal T1 C+ MR shows IVOH with a large enhancing intraventricular mass causing marked enlargement of the lateral ventricles .* + +![Axial NECT in the same patient shows the large intraventricular mass within the 4th ventricle. Note the dilated temporal horns .](images/app.statdx.com_image_thumbnail_5e46b562-c694-4b32-a46c-af0d64e8c289_annotated_true_size_900_quality_90_9842d1b3_20251018T165016Z.jpg) +*Axial NECT in the same patient shows the large intraventricular mass within the 4th ventricle. Note the dilated temporal horns .* + +![Sagittal T1WI MR shows IVOH secondary to aqueductal stenosis and distal stenosis of cerebral aqueduct . Note the enlarged lateral and 3rd ventricles.](images/app.statdx.com_image_thumbnail_59c0d666-8b8b-4f34-881e-7578f851fa7f_annotated_true_size_900_quality_90_ac9b7114_20251018T165016Z.jpg) +*Sagittal T1WI MR shows IVOH secondary to aqueductal stenosis and distal stenosis of cerebral aqueduct . Note the enlarged lateral and 3rd ventricles.* + +![Axial FLAIR MR shows neurosarcoidosis and IVOH secondary to diffuse meningeal disease. Periventricular white matter hyperintensities are also present, as well as choroid involvement .](images/app.statdx.com_image_thumbnail_32fe8687-87ee-4698-81ba-308f84e4409a_annotated_true_size_900_quality_90_6ebf3bdd_20251018T165017Z.jpg) +*Axial FLAIR MR shows neurosarcoidosis and IVOH secondary to diffuse meningeal disease. Periventricular white matter hyperintensities are also present, as well as choroid involvement .* + +![Coronal T1 C+ MR shows neurocysticercosis involvement within the 3rd ventricle and aqueduct , causing IVOH. The lateral ventricles are dilated.](images/app.statdx.com_image_thumbnail_30290860-d589-4878-ab88-f2e5114f4116_annotated_true_size_900_quality_90_d81df3a5_20251018T165017Z.jpg) +*Coronal T1 C+ MR shows neurocysticercosis involvement within the 3rd ventricle and aqueduct , causing IVOH. The lateral ventricles are dilated.* + +![Axial FLAIR MR shows neurocysticercosis resulting in IVOH. Large intraventricular cysts are present in the lateral vents , obstructing the foramina of Monro.](images/app.statdx.com_image_thumbnail_7bbd2516-73b7-45fb-b5ff-9f306d6d3a80_annotated_true_size_900_quality_90_09e03021_20251018T165016Z.jpg) +*Axial FLAIR MR shows neurocysticercosis resulting in IVOH. Large intraventricular cysts are present in the lateral vents , obstructing the foramina of Monro.* + +![Axial T1WI MR shows a well-defined, hyperintense lesion at the foramen of Monro in a patient with headaches, most consistent with a colloid cyst. Note the enlargement of the lateral ventricles due to obstruction at the foramen of Monro.](images/app.statdx.com_image_thumbnail_7ae9f42a-db38-4524-b7c0-79d52b798e21_annotated_true_size_900_quality_90_67db5fbc_20251018T165016Z.jpg) +*Axial T1WI MR shows a well-defined, hyperintense lesion at the foramen of Monro in a patient with headaches, most consistent with a colloid cyst. Note the enlargement of the lateral ventricles due to obstruction at the foramen of Monro.* + +![Sagittal T1WI C+ MR shows a homogeneously enhancing mass in the posterior 3rd ventricle , which causes obstruction and dilatation of the lateral and 3rd ventricles. On pathology, this was an astrocytoma.](images/app.statdx.com_image_thumbnail_a6d2a9a8-b74a-495d-8445-9c79b3030f4d_annotated_true_size_900_quality_90_54985e0c_20251018T165017Z.jpg) +*Sagittal T1WI C+ MR shows a homogeneously enhancing mass in the posterior 3rd ventricle , which causes obstruction and dilatation of the lateral and 3rd ventricles. On pathology, this was an astrocytoma.* + +![Coronal T2WI MR shows a pilocytic astrocytoma centered in the right thalamus causing severe mass effect on the 3rd ventricle and resultant obstructive hydrocephalus .](images/app.statdx.com_image_thumbnail_80232788-db60-4f4f-895b-3180811f62b7_annotated_true_size_900_quality_90_9f55a6f6_20251018T165017Z.jpg) +*Coronal T2WI MR shows a pilocytic astrocytoma centered in the right thalamus causing severe mass effect on the 3rd ventricle and resultant obstructive hydrocephalus .* + +![Axial T2WI MR demonstrates a well-defined CSF intensity cyst with the left temporal horn most consistent with an ependymal cyst . Note the dilated and trapped left temporal horn .](images/app.statdx.com_image_thumbnail_66d7ab24-8ae0-472b-9313-305394ec4c03_annotated_true_size_900_quality_90_29d87168_20251018T165017Z.jpg) +*Axial T2WI MR demonstrates a well-defined CSF intensity cyst with the left temporal horn most consistent with an ependymal cyst . Note the dilated and trapped left temporal horn .* + +![Sagittal T1WI C+ MR shows an enhancing mass in the pineal region causing mass effect on the tectal plate and aqueductal obstruction. Note the extensive leptomeningeal enhancement due to CSF spread of tumor. CSF cytology showed a primitive neuroectodermal tumor.](images/app.statdx.com_image_thumbnail_13465a11-c0bb-4055-8c14-056ea999ea83_annotated_true_size_900_quality_90_d6a81efb_20251018T165017Z.jpg) +*Sagittal T1WI C+ MR shows an enhancing mass in the pineal region causing mass effect on the tectal plate and aqueductal obstruction. Note the extensive leptomeningeal enhancement due to CSF spread of tumor. CSF cytology showed a primitive neuroectodermal tumor.* + +![Axial T2WI MR in a patient with corpus callosum impingement syndrome, after shunting for severe IVOH, shows a shunt tube , bilateral subdural fluid collections, and striated hyperintensity in the corpus callosum with somewhat less striking changes in the periventricular white matter . (Courtesy S. Candy, MD.)](images/app.statdx.com_image_thumbnail_e3c1a91c-c335-42df-baed-81b3ef83e867_annotated_true_size_900_quality_90_90e63be0_20251018T165017Z.jpg) +*Axial T2WI MR in a patient with corpus callosum impingement syndrome, after shunting for severe IVOH, shows a shunt tube , bilateral subdural fluid collections, and striated hyperintensity in the corpus callosum with somewhat less striking changes in the periventricular white matter . (Courtesy S. Candy, MD.)* + +![Axial NECT in a patient with headache demonstrates a classic colloid cyst at the foramen of Monro causing IVOH with dilatation of both lateral ventricles . Note the periventricular hypodensities due to transependymal leakage of CSF.](images/app.statdx.com_image_thumbnail_5272a7c9-5ae5-4498-bff6-256abf29b99a_annotated_true_size_900_quality_90_d97a420a_20251018T165017Z.jpg) +*Axial NECT in a patient with headache demonstrates a classic colloid cyst at the foramen of Monro causing IVOH with dilatation of both lateral ventricles . Note the periventricular hypodensities due to transependymal leakage of CSF.* + +![Axial CECT demonstrates a subacute left posterior inferior cerebellar infarct causing mass effect on the 4th ventricle and resulting in obstructive hydrocephalus .](images/app.statdx.com_image_thumbnail_7b359f58-dd44-4486-b7e0-8d172de6aece_annotated_true_size_900_quality_90_baccaab2_20251018T165017Z.jpg) +*Axial CECT demonstrates a subacute left posterior inferior cerebellar infarct causing mass effect on the 4th ventricle and resulting in obstructive hydrocephalus .* + +![Axial FLAIR MR shows massive enlargement of the 3rd and lateral ventricles by a CSF-like mass within the 3rd ventricle . There is periventricular interstitial edema . At surgery, an ependymal cyst of the 3rd ventricle was found and fenestrated.](images/app.statdx.com_image_thumbnail_5623057c-c423-4d4c-9801-45283be9a3dd_annotated_true_size_900_quality_90_b5086e29_20251018T165017Z.jpg) +*Axial FLAIR MR shows massive enlargement of the 3rd and lateral ventricles by a CSF-like mass within the 3rd ventricle . There is periventricular interstitial edema . At surgery, an ependymal cyst of the 3rd ventricle was found and fenestrated.* + +![Sagittal T1 MR shows a large arachnoid cyst in the superior cerebellar cistern causing severe mass effect on the tectal plate and aqueduct .There is dilatation of the 3rd and lateral ventricles with thinning of the corpus callosum .](images/app.statdx.com_image_thumbnail_4805b47b-9b84-4da7-9efa-e16c8cefdcf8_annotated_true_size_900_quality_90_039b4f28_20251018T165017Z.jpg) +*Sagittal T1 MR shows a large arachnoid cyst in the superior cerebellar cistern causing severe mass effect on the tectal plate and aqueduct .There is dilatation of the 3rd and lateral ventricles with thinning of the corpus callosum .* + +![Sagittal T1WI C+ MR shows a cyst with an enhancing mural nodule of hemangioblastoma in the vermis, causing severe effacement of the 4th ventricle and obstructive hydrocephalus.](images/app.statdx.com_image_thumbnail_519a5b7e-6869-4b93-98f6-a6edefac58a8_annotated_true_size_900_quality_90_d8758d1c_20251018T165017Z.jpg) +*Sagittal T1WI C+ MR shows a cyst with an enhancing mural nodule of hemangioblastoma in the vermis, causing severe effacement of the 4th ventricle and obstructive hydrocephalus.* + +![Sagittal T2 MR demonstrates an enlarged T2 hyperintense tectal plate glioma , which causes obstruction at the aqueduct and dilatation of the lateral and 3rd ventricles .](images/app.statdx.com_image_thumbnail_7ca87971-568b-4324-bd66-b820c656d469_annotated_true_size_900_quality_90_0c6bf4c7_20251018T165017Z.jpg) +*Sagittal T2 MR demonstrates an enlarged T2 hyperintense tectal plate glioma , which causes obstruction at the aqueduct and dilatation of the lateral and 3rd ventricles .* + diff --git a/docs_md/articles/irregular-lateral-ventricles_f42ce651-9877-480b-90d8-665be656b33f.md b/docs_md/articles/irregular-lateral-ventricles_f42ce651-9877-480b-90d8-665be656b33f.md new file mode 100644 index 0000000..efe4a32 --- /dev/null +++ b/docs_md/articles/irregular-lateral-ventricles_f42ce651-9877-480b-90d8-665be656b33f.md @@ -0,0 +1,359 @@ +--- +title: "Irregular Lateral Ventricles" +docid: "f42ce651-9877-480b-90d8-665be656b33f" +authors: + - key: "1fa14dfd-71ea-4960-908e-e720313bc63a" + value: "Santhosh Gaddikeri, MD" + - key: "30ce27b2-237f-4aff-a88f-65ead356335b" + value: "Marinos Kontzialis, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60" + - + name: "Ventricles, Periventricular Regions" + slug: "ventricles-periventricular-regions" + treeNodeId: "353c434a-a6fc-4ef1-8786-d30a1988a4dc" + - + name: "Generic Imaging Patterns" + slug: "generic-imaging-patterns" + treeNodeId: "969c31a2-ef56-4fc3-9125-05857cf9aac3" + - + name: "Irregular Lateral Ventricles" + slug: "irregular-lateral-ventricles" + treeNodeId: null +category: "Brain" +documentVersionId: "2594bf27-49d6-4896-8859-f9eee7b4228c" +imageCount: 42 +lastUpdated: "02/15/23" +pageDescription: "Irregular Lateral Ventricles" +pageKeywords: "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Irregular Lateral Ventricles" +pageTitle: "Irregular Lateral Ventricles | STATdx" +enhancedTitle: "Irregular Lateral Ventricles" +type: "DDX" +references: true +breadcrumbs: + - "Brain" + - "Differential Diagnosis" + - "Ventricles, Periventricular Regions" + - "Generic Imaging Patterns" + - "Irregular Lateral Ventricles" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Irregular ventricles may be result of obstruction, chronic volume loss, &/or congenital deformities + - Obstruction: Mass effect, ballooned-appearing ventricles, and transependymal CSF migration + - Volume loss: Ventricle irregularity with brain parenchymal loss + - Congenital: Look for associated findings (colpocephaly, subependymal nodules) + - Ventricular deformities may become permanent despite relief of obstruction due to parenchymal atrophy or acquired ventricular noncompliance + - Enhancement may help differentiate etiologies +- ## Helpful Clues for Common Diagnoses + + + - **CSF Shunts and Complications** + - Common complications include shunt obstruction/breakage, infection, overdrainage + - Acquired ventricular noncompliance may result in ventricle deformity + - Small, "slit" ventricles → noncompliant ventricle syndrome, chronic overdrainage + - **Surgical Defects** + - Often evident from prior shunt tract or burr hole + - Overlying skull or scalp may show defect + - Deformity is chronic + - **Periventricular Leukomalacia** + - Selective vulnerability of periventricular white matter of preterm neonate + - Injury in late 2nd or early 3rd trimester + - Thinning of posterior body of corpus callosum, enlargement and irregularity of lateral ventricular walls + - Colpocephaly: Atrium/occipital horns dilated + - Related to spastic cerebral palsy + - **Cerebral Infarction, Chronic** + - Vascular territory wedge-shaped area of encephalomalacia + - Results in compensatory or ex vacuo dilation of regional ventricle due to volume loss + - **Multiple Sclerosis** + - Multiple periventricular/perivenular and callososeptal T2 hyperintensities + - Confluent lesions in severe disease lead to atrophy and irregular ventricular margins + - **Porencephalic Cyst** + - Congenital porencephalic cysts result from intrauterine vascular or infectious injury + - Acquired cysts are secondary to injury later in life from trauma, surgery, ischemia, or infection + - CSF intensity cysts with smooth walls and T2-hyperintense surrounding gliotic changes (no gray matter lining walls as in open-lip schizencephaly) + - Usually communicate directly with ventricular system +- ## Helpful Clues for Less Common Diagnoses + + + - **Chiari 2** + - Pointed anterior horns, colpocephaly + - Small, crowded posterior fossa, widening of tentorial incisura, towering cerebellum, tectal beaking, downward herniation of cerebellar vermian tissue through foramen magnum + - Associated with lumbar myelomeningocele + - Hydrocephalus and other midline malformations + - **Heterotopic Gray Matter** + - Subependymal heterotopia: Subependymal nodules follow gray matter signal and protrude into ventricles; no enhancement + - Focal/multifocal asymmetric gray matter indentation of ventricle + - **Tuberous Sclerosis Complex** + - Subependymal nodules lining ventricles characteristic + - Mostly along striothalamic groove + - Calcify with increasing age + - < 1.3 cm + - Cortical and subcortical tubers are usually multifocal ± mild mass effect + - Tubers most easily seen on FLAIR + - Rarely, tubers may calcify or enhance + - White matter radial migration lines + - Cyst-like white matter lesions (cystoid brain degeneration) + - Enhancing mass with rapid growth at foramen of Monro = subependymal giant cell astrocytoma + - **Metastases, Intracranial, Other** + - CSF seeding of primary CNS tumors, lymphoma, or systemic malignancy may cause irregular ventricles + - Intraventricular metastases = 0.9-4.6% of cerebral metastases + - Adults: Renal, colon, lung + - Children: Neuroblastoma, Wilms tumor, retinoblastoma + - May result in ventricular nodules, which can deform ventricles + - Lateral ventricles most common location + - Avid enhancement ± vasogenic edema in adjacent parenchyma + - **Intraventricular Webs or Adhesions** + - May be congenital or acquired (prior hemorrhage, infection, or tumor) + - Contours of ventricles may be rounded or balloon-like due to obstructive symptoms + - Contrast ventriculography or cine CSF can be helpful to assess for evidence of physiological flow obstruction + - Heavily weighted T2 sequences, such as FIESTA/CISS, helpful + - **CMV, Congenital** + - Microcephaly + - Periventricular calcifications 40-70% + - Migrational abnormalities: Lissencephaly, pachygyria, polymicrogyria, schizencephaly + - Delayed myelination, dysmyelination + - Cerebral + cerebellar volume loss + - Periventricular cysts, ventriculomegaly, ventricular adhesions, lenticulostriate vasculopathy + - **Schizencephaly** + - Transmantle cleft lined by gray matter + - Open lip (large defect), closed lip (small defect) + - Dimple in wall of ventricle when defect small/closed + - Up to 50% bilateral + - When bilateral, 60% are open lipped on both sides +- ## Helpful Clues for Rare Diagnoses + + + - **Hemimegalencephaly** + - Hamartomatous overgrowth of part/all of hemisphere + - Lateral ventricle ipsilateral to enlarged hemisphere is usually bizarre-shaped and typically enlarged + - Involved hemisphere may eventually atrophy from chronic seizures + - **Holoprosencephaly** + - Congenital structural forebrain anomalies defined by degree of frontal lobe fusion + - All types have absent septum pellucidum and frontal lobe fusion anomaly + - Alobar + - Single midline forebrain + - Single primitive monoventricle, often incompletely covered posteriorly by brain (dorsal cyst) + - Semilobar + - Frontal lobes > 50% fused + - Thalami and hypothalamus may be fused + - Interhemispheric fissure and falx cerebri may be present posteriorly + - Facial malformations mild or absent + - Lobar: Anterior lateral ventricle may be deficient + - Interhemispheric fissure present along most of midline + - Only most inferior frontal lobes fused + - Thalami almost/completely separated + - Callosal dysgenesis (genu, rostrum) + - **Holoprosencephaly Variants** + - Middle interhemispheric variant of holoprosencephaly + - Lack of separation in posterior frontal and parietal areas + - Dorsal cyst in 40% + - Callosal dysgenesis (body) +- ## Alternative Differential Approaches + + + - Gadolinium studies can differentiate among causes of ependymal nodules + - Nonenhancing subependymal nodules may represent gray matter heterotopia or tuberous sclerosis nodules + - Gray matter heterotopias follow gray matter signal/density + - Tuberous sclerosis nodules follow white matter signal or are calcified + - Enhancing nodules suggest ependymal tumor seeding + +## References + +# Selected References + +1. [Balasubramaniam C: Shunt complications - staying out of trouble. Neurol India. 69(Supplement):S495-501, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=35103008%5Bpmid%5D) +1. [Society for Maternal-Fetal Medicine (SMFM) et al: Holoprosencephaly. Am J Obstet Gynecol. 223(6):B13-6, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=33168217%5Bpmid%5D) +1. [Gotardo JW et al: Impact of peri-intraventricular haemorrhage and periventricular leukomalacia in the neurodevelopment of preterms: a systematic review and meta-analysis. PLoS One. 14(10):e0223427, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31600248%5Bpmid%5D) +1. [Winter TC et al: Holoprosencephaly: a survey of the entity, with embryology and fetal imaging. Radiographics. 35(1):275-90, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25590404%5Bpmid%5D) +1. [Smith AB et al: From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 33(1):21-43, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23322825%5Bpmid%5D) +1. [Osborn AG et al: Intracranial cysts: radiologic-pathologic correlation and imaging approach. Radiology. 239(3):650-64, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16714456%5Bpmid%5D) +1. [Melhem ER et al: Periventricular leukomalacia: relationship between lateral ventricular volume on brain MR images and severity of cognitive and motor impairment. Radiology. 214(1):199-204, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10644124%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T2 MR in a patient with chronic shunting demonstrates slit-like irregular lateral ventricles due to noncompliance from chronic drainage.](images/app.statdx.com_image_thumbnail_d27bb8c7-8ff5-46dc-a0ef-68850de500d4_annotated_true_size_900_quality_90_20b437c9_20251018T165133Z.jpg) +**CSF Shunts and Complications** +*Axial T2 MR in a patient with chronic shunting demonstrates slit-like irregular lateral ventricles due to noncompliance from chronic drainage.* + +![Axial FLAIR MR demonstrates a large surgical defect in the left frontal lobe due to prior tumor resection communicating with the left lateral ventricle , which appears irregular.](images/app.statdx.com_image_thumbnail_8880f9eb-b099-4000-9e1e-48961ce97d43_annotated_true_size_900_quality_90_2ce848f4_20251018T165133Z.jpg) +**Surgical Defects** +*Axial FLAIR MR demonstrates a large surgical defect in the left frontal lobe due to prior tumor resection communicating with the left lateral ventricle , which appears irregular.* + +![Axial T2 MR in a 5-year-old boy with spastic cerebral palsy demonstrates irregular lateral ventricles with paucity of white matter and periventricular hyperintensities , consistent with periventricular leukomalacia.](images/app.statdx.com_image_thumbnail_9c9a7a2f-9e6b-4a73-8333-221f3fe6ed9c_annotated_true_size_900_quality_90_fc040ce9_20251018T165133Z.jpg) +**Periventricular Leukomalacia** +*Axial T2 MR in a 5-year-old boy with spastic cerebral palsy demonstrates irregular lateral ventricles with paucity of white matter and periventricular hyperintensities , consistent with periventricular leukomalacia.* + +![Axial T2 MR demonstrates encephalomalacia in the left occipital lobe with ex vacuo dilation of left occipital horn due to PCA territory chronic infarct.](1e04b9f4-77c2-4080-86ac-47fcf2378c38) +**Cerebral Infarction, Chronic** +*Axial T2 MR demonstrates encephalomalacia in the left occipital lobe with ex vacuo dilation of left occipital horn due to PCA territory chronic infarct.* + +![Axial T2 MR in a patient with primary progressive MS demonstrates extensive white matter hyperintensities with asymmetric parenchymal volume loss and ex vacuo dilation of lateral ventricles .](696938c6-acff-4981-a198-bb3859a4b58d) +**Multiple Sclerosis** +*Axial T2 MR in a patient with primary progressive MS demonstrates extensive white matter hyperintensities with asymmetric parenchymal volume loss and ex vacuo dilation of lateral ventricles .* + +![Axial T2 MR demonstrates a right occipital lobe, smooth-walled, cystic encephalomalacia lined by white matter and communicating with the lateral ventricle, consistent with porencephalic cyst.](2a419e56-b484-4c51-91b1-c0157549c29a) +**Porencephalic Cyst** +*Axial T2 MR demonstrates a right occipital lobe, smooth-walled, cystic encephalomalacia lined by white matter and communicating with the lateral ventricle, consistent with porencephalic cyst.* + +![Axial NECT demonstrates irregular lateral ventricles with a right frontal lobe shunt catheter . Note diffuse calvarial thickening due to chronic shunting. Images of posterior fossa revealed small posterior fossa and other stigmata of Chiari 2 malformation (not shown).](918faead-f7fc-49d0-b0db-ad6d896501a9) +**Chiari 2** +*Axial NECT demonstrates irregular lateral ventricles with a right frontal lobe shunt catheter . Note diffuse calvarial thickening due to chronic shunting. Images of posterior fossa revealed small posterior fossa and other stigmata of Chiari 2 malformation (not shown).* + +![Axial T2 MR demonstrates nodular gray matter heterotopia along the ependymal lining of bilateral occipital horns.](8eb6070b-371d-4980-af73-d3f71628661a) +**Heterotopic Gray Matter** +*Axial T2 MR demonstrates nodular gray matter heterotopia along the ependymal lining of bilateral occipital horns.* + +![Axial 3D T1 MPRAGE in a patient with known tuberous sclerosis demonstrates multiple subependymal nodules . Also note tiny cysts in white matter . Cortical/ subcortical tubers and white matter radial migration lines were seen (not shown).](92d8e135-15c5-4c7f-8210-fdcd830ae6b8) +**Tuberous Sclerosis Complex** +*Axial 3D T1 MPRAGE in a patient with known tuberous sclerosis demonstrates multiple subependymal nodules . Also note tiny cysts in white matter . Cortical/ subcortical tubers and white matter radial migration lines were seen (not shown).* + +![Axial T1 C+ MR in a patient with metastatic lung cancer demonstrates multiple heterogeneously enhancing metastatic lesions in bilateral periventricular regions .](cb26dd77-411b-4e6c-848b-269ad104eac2) +**Metastases, Intracranial, Other** +*Axial T1 C+ MR in a patient with metastatic lung cancer demonstrates multiple heterogeneously enhancing metastatic lesions in bilateral periventricular regions .* + +![Axial 3D T2 HASTE MR in a neonate demonstrates multiple septa/webs in both lateral ventricles . Also note asymmetrically dilated, irregular lateral ventricles . Encephalomalacia in the right parietooccipital region is due to antenatal insult.](4670b1bf-fbaf-4453-9587-496db01a5658) +**Intraventricular Webs or Adhesions** +*Axial 3D T2 HASTE MR in a neonate demonstrates multiple septa/webs in both lateral ventricles . Also note asymmetrically dilated, irregular lateral ventricles . Encephalomalacia in the right parietooccipital region is due to antenatal insult.* + +![Axial NECT in a 2-year-old with a known congenital CMV infection demonstrates moderately dilated irregular lateral ventricles as well as periventricular and deep white matter calcifications . Note lissencephalic gyral pattern .](a2c56b9b-f5ae-439d-a188-f63b50a7c07a) +**CMV, Congenital** +*Axial NECT in a 2-year-old with a known congenital CMV infection demonstrates moderately dilated irregular lateral ventricles as well as periventricular and deep white matter calcifications . Note lissencephalic gyral pattern .* + +![Axial 3D T1 MR demonstrates open-lip schizencephaly with a seam connecting ependymal to pial surface . Note gray matter lining the cystic area , differentiating it from a porencephalic cyst.](2946249f-8a43-47bd-8ae2-c72415856ca0) +**Schizencephaly** +*Axial 3D T1 MR demonstrates open-lip schizencephaly with a seam connecting ependymal to pial surface . Note gray matter lining the cystic area , differentiating it from a porencephalic cyst.* + +![Axial T2 MR demonstrates absent septum with absent posterior body of corpus callosum. Also seen was abnormal bilateral sylvian fissure with ventricle orientation and midline fusion (not shown), consistent with syntelencephaly, a.k.a. middle interhemispheric variant holoprosencephaly.](8bbc4ba5-5a4f-43f5-ba8b-69dd4fa8af36) +**Holoprosencephaly Variants** +*Axial T2 MR demonstrates absent septum with absent posterior body of corpus callosum. Also seen was abnormal bilateral sylvian fissure with ventricle orientation and midline fusion (not shown), consistent with syntelencephaly, a.k.a. middle interhemispheric variant holoprosencephaly.* + + +### Additional Images + +![Axial NECT shows a right frontal ventricular drain that traverses the right ventricle but is not decompressing the left lateral ventricle, which remains irregularly enlarged .](images/app.statdx.com_image_thumbnail_2926513c-8a66-47ff-9091-463f9a854993_annotated_true_size_900_quality_90_63045a79_20251018T165133Z.jpg) +**CSF Shunts and Complications** +*Axial NECT shows a right frontal ventricular drain that traverses the right ventricle but is not decompressing the left lateral ventricle, which remains irregularly enlarged .* + +![Axial T2 MR shows irregular enlargement of the left occipital horn due to left temporal and occipital surgical defect and encephalomalacia from tumor removal in this location.](images/app.statdx.com_image_thumbnail_81d90381-93ef-4fd9-804f-98f39faf22d8_annotated_true_size_900_quality_90_3f67ffb9_20251018T165133Z.jpg) +**Surgical Defects** +*Axial T2 MR shows irregular enlargement of the left occipital horn due to left temporal and occipital surgical defect and encephalomalacia from tumor removal in this location.* + +![Axial T2 MR shows classic "wavy" or undulating contours of the lateral ventricles in addition to colpocephaly (enlargement of the posterior portions of lateral ventricles). Colpocephaly reflects the predominantly posterior volume loss.](images/app.statdx.com_image_thumbnail_c7d04410-a691-42e3-9428-d55060a26d68_annotated_true_size_900_quality_90_5996778f_20251018T165133Z.jpg) +**Periventricular Leukomalacia** +*Axial T2 MR shows classic "wavy" or undulating contours of the lateral ventricles in addition to colpocephaly (enlargement of the posterior portions of lateral ventricles). Colpocephaly reflects the predominantly posterior volume loss.* + +![Axial NECT shows irregular enlargement of the left frontal horn due to focal regional parenchymal volume loss in this patient with remote MCA infarct.](2f456604-123a-498e-a877-660381509108) +**Cerebral Infarction, Chronic** +*Axial NECT shows irregular enlargement of the left frontal horn due to focal regional parenchymal volume loss in this patient with remote MCA infarct.* + +![Axial T1 FS MR shows multifocal nodularity along ependymal margins of both lateral ventricles . These nodules follow gray matter signal on all sequences and do not enhance or change over time.](f1496ad0-85d2-45aa-a05b-4f49fd882c3b) +**Heterotopic Gray Matter** +*Axial T1 FS MR shows multifocal nodularity along ependymal margins of both lateral ventricles . These nodules follow gray matter signal on all sequences and do not enhance or change over time.* + +![Axial NECT shows irregularly dilated occipital horns with interdigitation of parietal and occipital parenchyma across midline due to a falx deficiency.](5a2506e9-70d3-46f0-a725-26e1b13fa20f) +**Chiari 2** +*Axial NECT shows irregularly dilated occipital horns with interdigitation of parietal and occipital parenchyma across midline due to a falx deficiency.* + +![Coronal T2 MR shows dysgenetic corpus callosum, small posterior fossa, and interdigitation of gyri from deficient falx, best seen post shunting. Cerebellum "towers" through the tentorial notch.](d3885609-d163-4bb5-8179-ba8901e78c3a) +**Chiari 2** +*Coronal T2 MR shows dysgenetic corpus callosum, small posterior fossa, and interdigitation of gyri from deficient falx, best seen post shunting. Cerebellum "towers" through the tentorial notch.* + +![Axial T2 MR shows multiple calcified subependymal nodules (SEN) lining ventricles. Note also subcortical tubers . SEN calcify much more commonly than cortical/subcortical tubers. ~ 50% of SEN are calcified by 10 years.](cd3b3a2b-1a19-4a9b-a8db-db235f939c8c) +**Tuberous Sclerosis Complex** +*Axial T2 MR shows multiple calcified subependymal nodules (SEN) lining ventricles. Note also subcortical tubers . SEN calcify much more commonly than cortical/subcortical tubers. ~ 50% of SEN are calcified by 10 years.* + +![Axial T2 MR shows small, subependymal nodules , which indent lateral ventricle margins. Unlike gray matter heterotopia, these follow WM signal or are calcified.](009fab60-a9d4-4283-aba1-b86b3717cb0a) +**Tuberous Sclerosis Complex** +*Axial T2 MR shows small, subependymal nodules , which indent lateral ventricle margins. Unlike gray matter heterotopia, these follow WM signal or are calcified.* + +![Axial T1 MR shows nodular ependymal thickening with an enhancing rind of tissue along the entire ventricular ependyma . While infection & primary malignant brain neoplasms such as GBM, germinoma, and lymphoma commonly spread along ventricular ependyma, this is a recognized but uncommon site for tumor deposits from extracranial primary tumors (melanoma in this case).](3602f90f-46e4-4ebe-b9a2-8f72def57b1f) +**Metastases, Intracranial, Other** +*Axial T1 MR shows nodular ependymal thickening with an enhancing rind of tissue along the entire ventricular ependyma . While infection & primary malignant brain neoplasms such as GBM, germinoma, and lymphoma commonly spread along ventricular ependyma, this is a recognized but uncommon site for tumor deposits from extracranial primary tumors (melanoma in this case).* + +![Axial T2 MR shows a small dimple on the lateral ventricular wall, which "points" to the site of a fused pial-ependymal seam . The aperture of the cleft is lined by gray matter in this closed-lip schizencephaly.](05bee719-bf55-496d-8865-8bae802bbf01) +**Schizencephaly** +*Axial T2 MR shows a small dimple on the lateral ventricular wall, which "points" to the site of a fused pial-ependymal seam . The aperture of the cleft is lined by gray matter in this closed-lip schizencephaly.* + +![Axial T2 MR shows cortical dysplasia and open-lip schizencephaly . Schizencephaly is closed-lip with a fused, gray matter-lined pial-ependymal seam or open-lip with large, gray matter-lined and fluid-filled CSF clefts.](544584c2-74ba-4f36-8701-7b6f8d26db3c) +**Schizencephaly** +*Axial T2 MR shows cortical dysplasia and open-lip schizencephaly . Schizencephaly is closed-lip with a fused, gray matter-lined pial-ependymal seam or open-lip with large, gray matter-lined and fluid-filled CSF clefts.* + +![Axial NECT shows focal outpouchings of CSF from both lateral ventricles with a CSF cleft extending from lateral ventricles to the subpial surface. The pial-ependymal seam is lined by gray matter.](2bc44bf5-bc94-467d-84f9-8de71b93d08b) +**Schizencephaly** +*Axial NECT shows focal outpouchings of CSF from both lateral ventricles with a CSF cleft extending from lateral ventricles to the subpial surface. The pial-ependymal seam is lined by gray matter.* + +![Axial NECT shows septum pellucidum and anterior falx absence. Frontal horns are hypoplastic. A band of parenchyma crosses midline . Mild frontal lobe fusion anomalies, as seen here, are typical of lobar holoprosencephaly.](fd55a6d3-31b8-4659-8163-ae8f4b2a3fae) +**Holoprosencephaly** +*Axial NECT shows septum pellucidum and anterior falx absence. Frontal horns are hypoplastic. A band of parenchyma crosses midline . Mild frontal lobe fusion anomalies, as seen here, are typical of lobar holoprosencephaly.* + +![Axial T1 MR shows open-lip schizencephaly with large, gray matter-lined and a fluid-filled CSF cleft. In addition, there is ventricular wall irregularity due to subependymal gray matter heterotopia bilaterally .](b7d119f1-d67e-42cd-bbfa-21c225e8cb7d) +**Schizencephaly** +*Axial T1 MR shows open-lip schizencephaly with large, gray matter-lined and a fluid-filled CSF cleft. In addition, there is ventricular wall irregularity due to subependymal gray matter heterotopia bilaterally .* + +![Coronal T2 MR demonstrates closed-lip schizencephaly. Abnormal, thick gray matter lines the cleft extending to a dimple in the wall of the right lateral ventricle .](55272009-ca22-4b9f-9162-af69d8a49014) +**Schizencephaly** +*Coronal T2 MR demonstrates closed-lip schizencephaly. Abnormal, thick gray matter lines the cleft extending to a dimple in the wall of the right lateral ventricle .* + +![Axial CT shows multiple calcified subependymal nodules lining the ventricles in a patient with tuberous sclerosis. The nodules calcify much more commonly than cortical/subcortical tubers. Note traumatic subarachnoid hemorrhage in the left Sylvian fissure.](1253b6d1-a526-4e7f-8b3e-2c2744b25924) +**Tuberous Sclerosis Complex** +*Axial CT shows multiple calcified subependymal nodules lining the ventricles in a patient with tuberous sclerosis. The nodules calcify much more commonly than cortical/subcortical tubers. Note traumatic subarachnoid hemorrhage in the left Sylvian fissure.* + +![Axial T2 MR shows multiple bilateral subependymal nodules of heterotopic gray matter along the lateral ventricular margins. These nodules follow gray matter signal on all sequences.](8a3bcced-e3f1-4a17-8cc5-b9d76bb8e2d7) +**Heterotopic Gray Matter** +*Axial T2 MR shows multiple bilateral subependymal nodules of heterotopic gray matter along the lateral ventricular margins. These nodules follow gray matter signal on all sequences.* + +![Axial CT in a Chiari 2 patient shows typical irregular appearance of the ventricles. Note the left posterior shunt catheter .](fefed345-c959-4896-b9b2-5ad4f10bae9a) +**Chiari 2** +*Axial CT in a Chiari 2 patient shows typical irregular appearance of the ventricles. Note the left posterior shunt catheter .* + +![Axial FLAIR MR demonstrates left posterior middle cerebral artery encephalomalacia with mild ex vacuo dilatation of the left occipital horn and atrium .](9f0d6726-27e8-4c3e-92a9-9aa52067d6cb) +**Cerebral Infarction, Chronic** +*Axial FLAIR MR demonstrates left posterior middle cerebral artery encephalomalacia with mild ex vacuo dilatation of the left occipital horn and atrium .* + +![Axial T2 MR in periventricular leukomalacia shows asymmetric, posterior, periventricular white matter (WM) volume loss with irregular ventricular margins . Periventricular leukomalacia, a.k.a. WM injury of prematurity, is a result of brain injury occurring before 33 weeks gestation and resulting in loss of periventricular WM.](c36e4c1d-b34f-41e9-a31c-aab2cb0df8bf) +**Periventricular Leukomalacia** +*Axial T2 MR in periventricular leukomalacia shows asymmetric, posterior, periventricular white matter (WM) volume loss with irregular ventricular margins . Periventricular leukomalacia, a.k.a. WM injury of prematurity, is a result of brain injury occurring before 33 weeks gestation and resulting in loss of periventricular WM.* + +![Axial FIESTA MR in a patient following left temporal bone surgery shows skull defect , underlying encephalomalacia , and ex vacuo dilatation of the left lateral ventricle .](images/app.statdx.com_image_thumbnail_c51882c7-eec5-40d6-9cfa-982849a82c22_annotated_true_size_900_quality_90_c7bab1c9_20251018T165133Z.jpg) +**Surgical Defects** +*Axial FIESTA MR in a patient following left temporal bone surgery shows skull defect , underlying encephalomalacia , and ex vacuo dilatation of the left lateral ventricle .* + +![Axial T1 MR demonstrates a right parietal shunt catheter with its tip in the right frontal horn in a patient with congenital aqueductal stenosis. The right lateral ventricle is collapsed, while the 3rd and left lateral ventricles are moderately dilated.](images/app.statdx.com_image_thumbnail_b0b4ac10-a5f5-45c0-8e06-16b37ce8e9e0_annotated_true_size_900_quality_90_abb03b0b_20251018T165133Z.jpg) +**CSF Shunts and Complications** +*Axial T1 MR demonstrates a right parietal shunt catheter with its tip in the right frontal horn in a patient with congenital aqueductal stenosis. The right lateral ventricle is collapsed, while the 3rd and left lateral ventricles are moderately dilated.* + +![Axial CECT shows a low-density outpouching from the right lateral ventricle . While a thin rim of cortex seems intact, the cyst nearly reaches brain surface and can be considered a porencephalic dilation or porencephalic lateral ventricle cyst.](90e2763f-d574-42bb-b173-33d8362e631b) +**Porencephalic Cyst** +*Axial CECT shows a low-density outpouching from the right lateral ventricle . While a thin rim of cortex seems intact, the cyst nearly reaches brain surface and can be considered a porencephalic dilation or porencephalic lateral ventricle cyst.* + +![Axial T2 MR shows near-complete coating of the ependymal lining of both lateral ventricles with tumor nodules due to metastatic seeding of an anaplastic oligodendroglioma.](66f6ca25-e3b3-437c-8531-4faa19f2e26c) +**Metastases, Intracranial, Other** +*Axial T2 MR shows near-complete coating of the ependymal lining of both lateral ventricles with tumor nodules due to metastatic seeding of an anaplastic oligodendroglioma.* + +![Axial NECT shows periventricular calcification , particularly along the caudostriatal groove, in the context of microcephaly and developmental delay. This strongly suggests congenital CMV infection. Note smooth ventricular margins, unlike calcified nodules in tuberous sclerosis complex.](27976562-e322-411d-b649-4edfd868c0da) +**CMV, Congenital** +*Axial NECT shows periventricular calcification , particularly along the caudostriatal groove, in the context of microcephaly and developmental delay. This strongly suggests congenital CMV infection. Note smooth ventricular margins, unlike calcified nodules in tuberous sclerosis complex.* + +![Axial T2 MR shows enlargement of left cerebral hemisphere accompanied by an irregular ipsilateral ventricle . The body of the left hemispheric WM is bulky. Note left fornix overgrowth.](6cf5c025-6a72-4fc7-8343-15d1240eec7b) +**Hemimegalencephaly** +*Axial T2 MR shows enlargement of left cerebral hemisphere accompanied by an irregular ipsilateral ventricle . The body of the left hemispheric WM is bulky. Note left fornix overgrowth.* + +![Axial T1 MR shows a large, horseshoe-shaped monoventricle with fused basal ganglia . There is no interhemispheric fissure and no identifiable lobulation or formation of ventricular horns in this alobar holoprosencephaly.](88c3bc23-d2eb-443c-832b-37829eed0963) +**Holoprosencephaly** +*Axial T1 MR shows a large, horseshoe-shaped monoventricle with fused basal ganglia . There is no interhemispheric fissure and no identifiable lobulation or formation of ventricular horns in this alobar holoprosencephaly.* + diff --git a/docs_md/articles/multiple-sclerosis_7892b2a2-f52a-4d7f-9858-a326f2b7ab04.md b/docs_md/articles/multiple-sclerosis_7892b2a2-f52a-4d7f-9858-a326f2b7ab04.md index 3dc80b9..9473d42 100644 --- a/docs_md/articles/multiple-sclerosis_7892b2a2-f52a-4d7f-9858-a326f2b7ab04.md +++ b/docs_md/articles/multiple-sclerosis_7892b2a2-f52a-4d7f-9858-a326f2b7ab04.md @@ -39,6 +39,7 @@ pageTitle: "Multiple Sclerosis | STATdx" enhancedTitle: "Multiple Sclerosis" type: "DX" references: true +tables: 1 breadcrumbs: - "Brain" - "Diagnosis" @@ -375,15 +376,23 @@ breadcrumbs: 1. [Klawiter EC: Current and new directions in MRI in multiple sclerosis. Continuum (Minneap Minn). 19(4 Multiple Sclerosis):1058-73, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23917101%5Bpmid%5D) 1. [Polman CH et al: Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol. 69(2):292-302, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21387374%5Bpmid%5D) +## Tables + +# 2017 McDonald Criteria for Diagnosis of Multiple Sclerosis + +| Clinical Presentation | Additional Criteria to Make Multiple Sclerosis Diagnosis | +| --- | --- | +| ≥ 2 attacks & clinical evidence of ≥ 2 lesions; or ≥ 2 attacks & clinical evidence of 1 lesion with historical evidence of prior attack involving lesion in different location | None ; DIS and DIT have been met | +| ≥ 2 attacks and clinical evidence of 1 lesion | DIS shown by 1 of these criteria - Additional clinical attack implicating different CNS site - ≥ 1 MS-typical T2 lesions in ≥ 2 areas of CNS: Periventricular, cortical, juxtacortical, infratentorial, or spinal cord | +| 1 attack and clinical evidence of ≥ 2 lesions | DIT shown by 1 of these criteria - Additional clinical attack - Simultaneous presence of enhancing & nonenhancing MS-typical MR lesions or new T2/enhancing MR lesion compared to baseline - CSF oligoclonal bands | +| 1 attack and clinical evidence of 1 lesion | DIS + DIT criteria - CSF oligoclonal bands | + ## Images ### Selected Images -![Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.](images/app.statdx.com_image_thumbnail_298cc9db-f7e6-4904-a92c-b4014d263b26_annotated_true_size_900_quality_90_3f552f53_20251018T122505Z.jpg) -*Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.* - ![Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.](images/app.statdx.com_image_thumbnail_298cc9db-f7e6-4904-a92c-b4014d263b26_size_168_quality_85_89ae47ce_20251018T095337Z.jpg) *Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.* diff --git a/docs_md/articles/multiple-sclerosis_89599954-599e-4410-a517-eb22125cedfb.md b/docs_md/articles/multiple-sclerosis_89599954-599e-4410-a517-eb22125cedfb.md index d665c80..6da3aec 100644 --- a/docs_md/articles/multiple-sclerosis_89599954-599e-4410-a517-eb22125cedfb.md +++ b/docs_md/articles/multiple-sclerosis_89599954-599e-4410-a517-eb22125cedfb.md @@ -445,12 +445,16 @@ breadcrumbs: ## Differential diagnosis -- {'authors': 'Bernadette L. Koch, MD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/56579195-7385-4918-872c-c25d965e8486', 'category': 'Pediatrics', 'compareUrl': '/compare/document/56579195-7385-4918-872c-c25d965e8486/related-ddx/treeNode?subContext=Intramedullary Spinal Cord Lesion', 'documentId': '56579195-7385-4918-872c-c25d965e8486', 'documentType': 'DDX', 'documentUrl': '/document/intramedullary-spinal-cord-lesion/56579195-7385-4918-872c-c25d965e8486', 'enhancedTitle': 'Intramedullary Spinal Cord Lesion', 'entryDate': '07/10/19', 'imageCount': 21, 'imageUrl': '/image/thumbnail/d58903d8-1977-44cd-b85d-f87d4e999f1a?size=174&quality=85', 'inCompareCart': False, 'rank': 1, 'referenceCount': 4, 'showCompareButton': False, 'title': 'Intramedullary Spinal Cord Lesion'} +### Intramedullary Spinal Cord Lesion +DDX:56579195-7385-4918-872c-c25d965e8486 ## Anatomy -- {'authors': 'Kevin R. Moore, MD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/7bc73286-326e-4017-a9d8-eabc87b88ac9', 'category': 'Spine', 'compareUrl': '/compare/document/7bc73286-326e-4017-a9d8-eabc87b88ac9/related-anatomy/treeNode?subContext=Spinal Cord and Cauda Equina', 'documentId': '7bc73286-326e-4017-a9d8-eabc87b88ac9', 'documentType': 'ANATOMY', 'documentUrl': '/document/spinal-cord-and-cauda-equina/7bc73286-326e-4017-a9d8-eabc87b88ac9', 'enhancedTitle': 'Spinal Cord and Cauda Equina', 'entryDate': '10/20/20', 'imageCount': 24, 'imageUrl': '/image/thumbnail/e2e75362-7958-443d-aa70-f465fad198dd?size=174&quality=85', 'inCompareCart': False, 'rank': 1, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Spinal Cord and Cauda Equina'} -- {'authors': 'Paula J. Woodward, MD, FSRU', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/daf8e6c7-c462-456a-ae66-ba4c913c42d3', 'category': 'Ultrasound', 'compareUrl': '/compare/document/daf8e6c7-c462-456a-ae66-ba4c913c42d3/related-anatomy/treeNode?subContext=Vertebral Column and Spinal Cord', 'documentId': 'daf8e6c7-c462-456a-ae66-ba4c913c42d3', 'documentType': 'ANATOMY', 'documentUrl': '/document/vertebral-column-and-spinal-cord/daf8e6c7-c462-456a-ae66-ba4c913c42d3', 'enhancedTitle': 'Vertebral Column and Spinal Cord', 'entryDate': '12/20/17', 'imageCount': 24, 'imageUrl': '/image/thumbnail/2a4b84c1-8e69-43c0-8bad-e08e8f3d0991?size=174&quality=85', 'inCompareCart': False, 'rank': 2, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Vertebral Column and Spinal Cord'} +### Spinal Cord and Cauda Equina +Spine/ANATOMY:7bc73286-326e-4017-a9d8-eabc87b88ac9 + +### Vertebral Column and Spinal Cord +Ultrasound/ANATOMY:daf8e6c7-c462-456a-ae66-ba4c913c42d3 ## Cases @@ -463,7 +467,7 @@ breadcrumbs: ### Selected Images -![Sagittal graphic depicts multiple demyelinating plaques within the cervical spinal cord, which are < 2 vertebral bodies in length.](images/app.statdx.com_image_thumbnail_cdd1d309-5f88-4517-8434-da8f015ecf81_annotated_true_size_900_quality_90_d1dfab0c_20251018T142350Z.jpg) +![Sagittal graphic depicts multiple demyelinating plaques within the cervical spinal cord, which are < 2 vertebral bodies in length.](cdd1d309-5f88-4517-8434-da8f015ecf81) *Sagittal graphic depicts multiple demyelinating plaques within the cervical spinal cord, which are < 2 vertebral bodies in length.* ![Sagittal T2 (left) & T1 C+ MR (right) show an active plaque at the C6-C7 level with ring enhancement & focal T2 hyperintensity .](images/app.statdx.com_image_thumbnail_f7c6cfd4-7a27-4173-b60d-f813b61cf506_annotated_true_size_900_quality_90_94245c62_20251018T142350Z.jpg) diff --git a/docs_md/articles/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-brain_a5b155b3-03ee-4934-8023-e681ed9e8296.md b/docs_md/articles/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-brain_a5b155b3-03ee-4934-8023-e681ed9e8296.md new file mode 100644 index 0000000..6c7dd02 --- /dev/null +++ b/docs_md/articles/myelin-oligodendrocyte-glycoprotein-antibody-associated-disease-brain_a5b155b3-03ee-4934-8023-e681ed9e8296.md @@ -0,0 +1,473 @@ +--- +title: "Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease, Brain" +docid: "a5b155b3-03ee-4934-8023-e681ed9e8296" +authors: + - key: "99e1aff7-f42c-43a0-95ae-d89c8551aa01" + value: "Kevin R. Moore, MD" +breadcrumbs: + - + name: "Pediatrics" + slug: "pediatrics" + treeNodeId: "a915965c-d436-44cf-ae65-2f22e7246ea4" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "2b5cea64-a083-489e-ac0c-ec14ba059026" + - + name: "Pediatric Neuroradiology" + slug: "pediatric-neuroradiology" + treeNodeId: "d0eb8f4a-e769-43dd-896c-8c9c27ce8759" + - + name: "Brain" + slug: "brain" + treeNodeId: "feaaadba-649b-4f0a-9aad-9188a8f9926a" + - + name: "Pathology-Based Diagnoses" + slug: "pathology-based-diagnoses" + treeNodeId: "2d26053f-23a7-4062-bf35-a93775ae1209" + - + name: "Inflammatory and Demyelinating Disease" + slug: "inflammatory-and-demyelinating-dis-" + treeNodeId: "cb319228-da96-4d29-8276-c72388a57656" + - + name: "Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease, Brain" + slug: "myelin-oligodendrocyte-glycoprotei-" + treeNodeId: null +category: "Pediatrics" +cmeTopicId: "5358b581-ed5c-44dc-8b93-3d6dcccbe83e" +documentVersionId: "74a3bf99-9d92-4e81-8b98-044d1cee70ba" +imageCount: 14 +lastUpdated: "02/07/24" +pageDescription: "Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease, Brain" +pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Inflammatory and Demyelinating Disease, Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease, Brain" +pageTitle: "Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease, Brain | STATdx" +enhancedTitle: "Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease, Brain" +type: "DX" +references: true +breadcrumbs: + - "Pediatrics" + - "Diagnosis" + - "Pediatric Neuroradiology" + - "Brain" + - "Pathology-Based Diagnoses" + - "Inflammatory and Demyelinating Disease" + - "Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease, Brain" +--- +# KEY FACTS + +- ## Terminology + + + - Myelin oligodendrocyte glycoprotein (MOG), MOG antibody-associated disease (MOGAD) +- ## Imaging + + + - Orbits + - Abnormal bilateral long-segment hyperintensity of optic nerve **anterior** segments + - Usually spares optic chiasm, retrochiasmatic pathways + - Brain + - T2-hyperintense, fluffy, poorly demarcated lesions + - Bilateral, large brainstem and deep gray nuclei lesions more common in children +- ## Top Differential Diagnoses + + + - Multiple sclerosis + - Acute disseminated encephalomyelitis (ADEM) + - Neuromyelitis optica spectrum disorder (NMOSD) + - Brain neoplasms + - Collagen vascular disorders + - Hemophagocytic lymphohistiocytosis (HLH) + - Mitochondrial diseases + - Leukodystrophies +- ## Pathology + + + - MOG protein expressed on surface of oligodendrocytes, in myelin sheath + - Cortical demyelination common, topographically adjacent meningeal inflammation +- ## Clinical Issues + + + - Unilateral or bilateral optic neuritis (ON) + - Most common + - Acute disseminated encephalomyelitis-like presentation with altered mental status, focal neurologic deficits + - Transverse myelitis + - Less common: Cerebral cortical encephalitis + - Progressive clinical decline with leukodystrophy-like MR pattern + - Infectious prodrome precedes initial symptom onset ≤ 60% of patients +- ## Diagnostic Checklist + + + - Best diagnostic clue: Bilateral **anterior** ON + longitudinally extensive transverse myelitis (LETM) + - Evaluate optic nerves on brain imaging to identify concurrent ON + - Ovoid periventricular (Dawson fingers) lesions **not**typical of MOGAD + +# TERMINOLOGY + +- ## Abbreviations + + + - Myelin oligodendrocyte glycoprotein (MOG), MOG antibody-associated disease (MOGAD) +- ## Definitions + + + - Inflammatory process of CNS characterized by attacks of immune-mediated demyelination + - Clinically and laboratory distinguishable from multiple sclerosis (MS) and AQP4-IgG (+) neuromyelitis optica spectrum disorder (NMOSD) + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Bilateral **anterior** optic pathway neuritis + longitudinally extensive transverse myelitis (**LETM**) + - Bilateral involvement during 1st optic neuritis (ON) presentation in 84% of MOGAD patients + - Brain imaging findings dependent on age at diagnosis (adult vs. child) + - ### Location + + + - Orbits, cerebral hemispheres, spinal cord + - Cortical gray matter (GM)/paracortical white matter (WM) most common location for MOGAD lesions + - ### Size + + + - Variable +- ## CT Findings + + + - ### NECT + + + - Hypodense lesions in areas of acute demyelination + - ### CECT + + + - Variable enhancement pattern, similar to MR +- ## MR Findings + + + - ### T1WI + + + - ± hypointensity within brain lesions + - Not typical of "black holes" seen with MS + - ### T2WI + + + - Orbits + - Abnormal bilateral long-segment hyperintensity of optic nerve **anterior** segments + - Best seen on fat-saturated imaging + - Usually spares optic chiasm, retrochiasmatic pathways + - Edematous, enlarged, tortuous optic nerve or nerves + - ± optic nerve head elevation + - Brain + - Hyperintensity in cortical GM, subcortical WM, deep WM, and deep GM + - Perivenous confluent pattern around small veins, lacking radiologic central vein sign (as opposed to MS) + - Bilateral hyperintense, fluffy, poorly demarcated lesions + - Brain lesions present at onset < 1/2 of adults, more common in children + - 1/3 infratentorial, predominantly in brainstem and cerebellar peduncles (children) + - Ovoid periventricular (Dawson fingers) lesions **not** typical + - Bilateral, large brainstem and deep gray nuclei lesions more common in children + - ### STIR + + + - Orbits + - Abnormal bilateral long-segment hyperintensity of anterior optic nerve segments + - Spares optic chiasm, retrochiasmatic pathways + - ± optic nerve head elevation + - Brain + - Same as FLAIR + - ### FLAIR + + + - Orbits + - Same as STIR + - Brain + - Same as T2WI, lesions usually better seen + - ± T2-hypointense subcortical WM lesions + - Unilateral cortical **FLA**IR-hyperintense lesions in anti-**M**OG-associated encephalitis with **s**eizures (**FLAMES**) + - ### T1WI C+ + + + - Orbits + - Avid optic nerve enhancement + - Perioptic nerve sheath and surrounding orbital fat enhancement + - In 50-80% of MOGAD patients + - Brain + - Variable; absent, faint, or patchy + - Enhancement in ≈ 12% of lesions + - ± leptomeningeal enhancement, ± adjacent cortical lesions +- ## Imaging Recommendations + + + - ### Protocol advice + + + - MR ± contrast of orbits, brain, and spinal cord + +# DIFFERENTIAL DIAGNOSIS + +- [Multiple Sclerosis](/document/pediatric-multiple-sclerosis-brain/f2592b04-f800-4235-9eea-a43f2bf4adfe) + - Optic nerves: Typically unilateral, short-segment, less extensive enhancement than MOGAD, NMOSD + - ± restricted diffusion + - Bilateral ON during initial presentation ≈ 23% of patients with MS-associated ON + - Brain: Presence of perpendicular periventricular lesions (Dawson fingers), "black holes" + - (+) CSF oligoclonal bands, (-) MOG antibodies, (-) AQP4 antibodies +- [Acute Disseminated Encephalomyelitis](/document/adem-brain/ed94b660-cf20-4ebb-8d6f-2b93505f2928) + - Polyfocal neurologic deficits and encephalopathy not explainable by fever + - Diffuse T2 signal abnormality in cortical GM, subcortical WM, deep WM, deep GM (or combination) + - ± linear, patchy, nodular enhancement + - May clinically mimic NMOSD and MOGAD + - Clinical presentation similar to NMOSD, but AQP4 IgG (-) + - Prolonged MOGAD IgG positivity suggests MOGAD +- [Neuromyelitis Optica Spectrum Disorder](/document/neuromyelitis-optica/11d42d8d-e7bb-4ecf-85ce-d96f0afeb076) + - Most patients (+) for AQP4-IgG antibodies (seropositive NMOSD) + - Preceding infectious prodrome in ≈ 10% patients + - Optic nerves: Favors **posterior** optic pathway segments (optic chiasm, retrochiasmatic optic tract) + - Bilateral involvement during 1st ON presentation in 82% of patients with AQP4-IgG (+) NMOSD + - Brain: Medulla oblongata (45.5%) and area postrema (31.8%) lesions most common +- ## Brain Neoplasms + + + - Intracranial mass lesion + - May mimic tumefactive MS on imaging + - Distinguish on clinical and imaging features +- ## Collagen Vascular Disorders + + + - Systemic lupus erythematosus, Sjögren syndrome neuro-Behçet disease, neurosarcoidosis + - Look for elevated markers of systemic inflammation + - Abnormal antinuclear antibody profile, (+) double-stranded DNA antibodies, elevated angiotensin converting enzyme, and (+) antiphospholipid antibodies +- ## Hemophagocytic Lymphohistiocytosis + + + - Primary involvement of CNS (rarely) precedes systemic manifestations of hemophagocytic lymphohistiocytosis (HLH) + - Pediatric CNS-isolated HLH can mimic acute disseminated encephalomyelitis (ADEM) clinically and on imaging +- ## Mitochondrial Diseases + + + - Typical MR features include abnormal T2 hyperintensity in basal ganglia or parietooccipital regions + - (Mitochondrial) **m**yopathy, **e**ncephalopathy, **l**actic**a**cidosis, and **s**troke-like episodes syndrome (MELAS), Leigh disease, others + - Elevation of serum and CSF lactate levels +- ## Leukodystrophies + + + - Metachromatic leukodystrophy (MLD), X-linked adrenoleukodystrophy (ALD), Alexander disease (AD), Krabbe disease (globoid cell leukodystrophy) + - Look for characteristic imaging or laboratory abnormalities + +# PATHOLOGY + +- ## General Features + + + - MOG is myelin protein expressed on outer surface of oligodendrocytes and myelin sheath + - Function not known; may act as cell adhesion molecule, regulate microtubule stability, and modulate myelin immune interactions + - Cortical demyelination common, topographically associated with adjacent meningeal inflammation + - Intracortical > subcortical WM demyelinated lesions predominate (compared to MS) + - MOG-IgG present in ~ 1/3 of pediatric acute acquired demyelinating syndromes +- ## Staging, Grading, & Classification + + + - **New diagnostic criteria for MOGAD (March 2023)** + - Intended to improve MOGAD identification, distinguish it from other disorders with overlapping clinical features, such as NMOSD, MS + - 3 primary diagnostic criteria + - 1 of 6 core clinical demyelinating events [i.e., ON, transverse myelitis (TM), ADEM-like encephalopathy, cerebral monofocal or polyfocal deficits, brainstem or cerebellar deficits, or cerebral cortical encephalitis] + - MOG-immunoglobulin G (IgG) antibody test (+) + - In absence of serum positivity for MOG-immunoglobulin G (IgG) by cell-based assay, CSF positivity for MOG-IgG fulfills criteria + - Exclusion of better diagnosis +- ## Microscopic Features + + + - Initial peripheral T-cell activation of T cells → reactivation in subarachnoid/perivascular spaces by MOG-laden antigen-presenting cells and inflammatory CSF milieu + - T cells, granulocytes cross blood brain barrier, infiltrate CNS parenchyma + - Macrophages/microglia appear in lesions + - Anti-MOG antibodies opsonize MOG, activate complement- and antibody-dependent cellular cytotoxicity + - CD4 (+) T-cell and granulocytic inflammation typical + - CD8 (+) T-cell infiltrate typical in MS + - Perivenous confluent pattern around small veins + - Coexisting perivenous and confluent demyelination + cortical demyelination + - Intracortical demyelinating lesions predominate + - Extensive subpial cortical demyelination with microglial activation, inflammatory infiltrate + - Affects oligodendrocytes but spares astrocytes (unlike NMOSD) + - Oligodendroglial progenitor cells spared because they do not yet express MOG + - Chronic active lesions absent +- ## Laboratory Evaluation + + + - Serum MOG-IgG antibody testing by cell-based assay + - Serum AQP4-IgG testing to exclude NMOSD + - CSF testing for oligoclonal bands to evaluate for MS + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Unilateral or bilateral ON + - Varying degrees of vision loss + eye pain worsened with eye movement + - Most common clinical MOGAD manifestation at onset + - Visual loss typically central and severe (more severe in MOGAD than MS, similar in severity to NMOSD + - Opticospinal phenotype increases in frequency as children get older + - ADEM-like presentation with altered mental status, focal neurologic deficits + - Up to 68% of cases at initial presentation + - Some MOGAD patients have clinical and MR features of ADEM but not encephalopathy + - Recurrence of ADEM features after recovery suggests MOGAD + - TM + - Limb weakness, sensory loss, and bowel, bladder, or sexual dysfunction + - Cerebral cortical encephalitis + - Headache, seizures, stroke-like episodes, and focal neurologic deficits + - Seizures more common than with NMOSD and MS + - ### Other signs/symptoms + + + - Rare: Progressive clinical decline with leukodystrophy-like MR pattern + - ### Clinical profile + + + - Most common pediatric clinical phenotype is ADEM-like encephalopathy, ON (or both), followed by TM +- ## Demographics + + + - ### Age + + + - Predilection for children but can affect any age + - Median age of MOGAD onset: 20-30 years + - Children: ≈ 50% of MOGAD cases + - ### Sex + + + - M = F + - ### Epidemiology + + + - Incidence and prevalence largely unknown + - European studies suggest incidence 1.6-3.4 per 1,000,000 person-years +- ## Natural History & Prognosis + + + - Infectious prodrome precedes initial symptom onset ≤ 60% of patients + - Monophasic or relapsing course (≈ 50/50) + - ON is most common relapse manifestation (followed by TM) + - Attacks develop over days, plateau with variable recovery over weeks to months + - Most T2 lesions resolve completely over months to few years + - Useful discriminator from MS and AQP4-IgG NMOSD, in which majority have residual T2-hyperintense lesions + - MOG-associated ON generally responds well to steroid administration + - Optic nerve edema and enlargement may resolve with residual T2 hyperintensity and optic nerve atrophy + - Visual outcomes after MOG-associated ON better than NMOSD + - Long-term disability rates lower than with NMOSD or MS + - MOGAD not associated with primary or secondary progressive course + - Low mortality +- ## Treatment + + + - Glucocorticoid therapy 1st line + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Simultaneous bilateral ON involving anterior optic pathway with associated optic disc edema strongly implies MOGAD +- ## Image Interpretation Pearls + + + - Evaluate optic apparatus carefully on brain imaging to identify concurrent ON + + 2d7cd594-5de1-4c79-a3d7-f6a7c5e45a2a + +## References + +# Selected References + +1. [Banwell B et al: Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria. Lancet Neurol. 22(3):268-82, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36706773%5Bpmid%5D) +1. [Gilani A et al: Childhood small-vessel primary angiitis of the central nervous system: overlap with MOG-associated disease. Pediatr Dev Pathol. 26(1):18-29, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36377607%5Bpmid%5D) +1. [Bells S et al: Patterns of white and gray structural abnormality associated with paediatric demyelinating disorders. Neuroimage Clin. 34:103001, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35381508%5Bpmid%5D) +1. [Lasrado A et al: Optic chiasm, optic tract and deep white demyelination: an unusual distribution of myelin oligodendrocyte glycoprotein-associated demyelination (MOGAD), case report and review of literature. BMJ Case Rep. 15(7):e249398, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35798498%5Bpmid%5D) +1. [Lin S et al: Myelin oligodendrocyte glycoprotein antibody-associated aseptic meningitis without neurological parenchymal lesions: a novel phenotype. Mult Scler Relat Disord. 68:104126, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36115288%5Bpmid%5D) +1. [Cobo-Calvo A et al: Clinical features and risk of relapse in children and adults with myelin oligodendrocyte glycoprotein antibody-associated disease. Ann Neurol. 89(1):30-41, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=32959427%5Bpmid%5D) +1. [Gadde JA et al: Rate of leptomeningeal enhancement in pediatric myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis. J Child Neurol. 36(11):1042-6, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34547933%5Bpmid%5D) +1. [Li Y et al: Clinical and radiological characteristics of children and adults with first-attack myelin oligodendrocyte glycoprotein antibody disease and analysis of risk factors for predicting the severity at disease onset in central China. Front Immunol. 12:752557, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34975841%5Bpmid%5D) +1. [Lopez Chiriboga S et al: Myelitis and other autoimmune myelopathies. Continuum (Minneap Minn). 27(1):62-92, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33522737%5Bpmid%5D) +1. [Shahriari M et al: MOGAD: How it differs from and resembles other neuroinflammatory disorders. AJR Am J Roentgenol. 216(4):1031-9, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=32755221%5Bpmid%5D) +1. [Ambrosius W et al: Myelin oligodendrocyte glycoprotein antibody-associated disease: current insights into the disease pathophysiology, diagnosis and management. Int J Mol Sci. 22(1):100, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=33374173%5Bpmid%5D) +1. [Armangue T et al: Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study. Lancet Neurol. 19(3):234-46, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32057303%5Bpmid%5D) +1. [Banks SA et al: Brainstem and cerebellar involvement in MOG-IgG-associated disorder versus aquaporin-4-IgG and MS. J Neurol Neurosurg Psychiatry. jnnp-2020-325121, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=33372052%5Bpmid%5D) +1. [Chen JJ et al: Clinical phenotype, radiological features, and treatment of myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) optic neuritis. Curr Opin Neurol. 33(1):47-54, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31743235%5Bpmid%5D) +1. [Höftberger R et al: The pathology of central nervous system inflammatory demyelinating disease accompanying myelin oligodendrocyte glycoprotein autoantibody. Acta Neuropathol. 139(5):875-92, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32048003%5Bpmid%5D) +1. [O'Connell K et al: Prevalence and incidence of neuromyelitis optica spectrum disorder, aquaporin-4 antibody-positive NMOSD and MOG antibody-positive disease in Oxfordshire, UK. J Neurol Neurosurg Psychiatry. 91(10):1126-8, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32576617%5Bpmid%5D) +1. [Takai Y et al: Myelin oligodendrocyte glycoprotein antibody-associated disease: an immunopathological study. Brain. 143(5):1431-46, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32412053%5Bpmid%5D) +1. [Waters P et al: Serial anti-myelin oligodendrocyte glycoprotein antibody analyses and outcomes in children with demyelinating syndromes. JAMA Neurol. 77(1):82-93, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31545352%5Bpmid%5D) +1. [Dubey D et al: Clinical, radiologic, and prognostic features of myelitis associated with myelin oligodendrocyte glycoprotein autoantibody. JAMA Neurol. 76(3):301-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30575890%5Bpmid%5D) +1. [Mariano R et al: Comparison of clinical outcomes of transverse myelitis among adults with myelin oligodendrocyte glycoprotein antibody vs aquaporin-4 antibody disease. JAMA Netw Open. 2(10):e1912732, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31596489%5Bpmid%5D) +1. [Salama S et al: MOG antibody-associated encephalomyelitis/encephalitis. Mult Scler. 25(11):1427-33, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30907249%5Bpmid%5D) +1. [Cobo-Calvo A et al: Clinical spectrum and prognostic value of CNS MOG autoimmunity in adults: the MOGADOR study. Neurology. 90(21):e1858-69, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29695592%5Bpmid%5D) +1. [Hamid SHM et al: Seizures and encephalitis in myelin oligodendrocyte glycoprotein IgG disease vs aquaporin 4 IgG disease. JAMA Neurol. 75(1):65-71, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29131884%5Bpmid%5D) +1. [López-Chiriboga AS et al: Association of MOG-IgG serostatus with relapse after acute disseminated encephalomyelitis and proposed diagnostic criteria for MOG-IgG-associated disorders. JAMA Neurol. 75(11):1355-63, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30014148%5Bpmid%5D) +1. [Ramanathan S et al: Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination. J Neurol Neurosurg Psychiatry. 89(2):127-37, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29142145%5Bpmid%5D) +1. [Fujimori J et al: Bilateral frontal cortex encephalitis and paraparesis in a patient with anti-MOG antibodies. J Neurol Neurosurg Psychiatry. 88(6):534-6, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28209651%5Bpmid%5D) +1. [Hennes EM et al: Prognostic relevance of MOG antibodies in children with an acquired demyelinating syndrome. Neurology. 89(9):900-8, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28768844%5Bpmid%5D) +1. [Jurynczyk M et al: Clinical presentation and prognosis in MOG-antibody disease: a UK study. Brain. 140(12):3128-38, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29136091%5Bpmid%5D) +1. [Ogawa R et al: MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy. Neurol Neuroimmunol Neuroinflamm. 4(2):e322, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28105459%5Bpmid%5D) +1. [Ramanathan S et al: Radiological differentiation of optic neuritis with myelin oligodendrocyte glycoprotein antibodies, aquaporin-4 antibodies, and multiple sclerosis. Mult Scler. 22(4):470-82, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26163068%5Bpmid%5D) +1. [Krupp LB et al: International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions. Mult Scler. 19(10):1261-7, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23572237%5Bpmid%5D) +1. [Wolf VL et al: Pediatric acute transverse myelitis overview and differential diagnosis. J Child Neurol. 27(11):1426-36, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22914370%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial FLAIR MR in a 10-year-old with acute myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) shows characteristic thalamic , basal ganglia , hypothalamic , and insular cortex hyperintense lesions. The hypothalamic lesions are relatively subtle but correlated clinically with lethargy and hypothalamic symptoms and signs.](images/app.statdx.com_image_thumbnail_39c8eda2-a1dc-4da7-985e-c058983bd32a_annotated_true_size_900_quality_90_447d176f_20251018T151317Z.jpg) +*Axial FLAIR MR in a 10-year-old with acute myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) shows characteristic thalamic , basal ganglia , hypothalamic , and insular cortex hyperintense lesions. The hypothalamic lesions are relatively subtle but correlated clinically with lethargy and hypothalamic symptoms and signs.* + +![Axial FLAIR MR in a 10-year-old with acute myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) shows characteristic thalamic , basal ganglia , hypothalamic , and insular cortex hyperintense lesions. The hypothalamic lesions are relatively subtle but correlated clinically with lethargy and hypothalamic symptoms and signs.](images/app.statdx.com_image_thumbnail_39c8eda2-a1dc-4da7-985e-c058983bd32a_size_174_quality_85_8075a57d_20251018T152348Z.jpg) +*Axial FLAIR MR in a 10-year-old with acute myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) shows characteristic thalamic , basal ganglia , hypothalamic , and insular cortex hyperintense lesions. The hypothalamic lesions are relatively subtle but correlated clinically with lethargy and hypothalamic symptoms and signs.* + +![Axial FLAIR MR in the same patient additionally reveals brainstem/cerebellar peduncle , deep cerebellar white matter , and cerebellar hemisphere lesions.](images/app.statdx.com_image_thumbnail_2d03fd97-7b4d-48ac-be0f-ff4a4c16b743_annotated_true_size_900_quality_90_b0ab4b76_20251018T151317Z.jpg) +*Axial FLAIR MR in the same patient additionally reveals brainstem/cerebellar peduncle , deep cerebellar white matter , and cerebellar hemisphere lesions.* + +![Axial T1 C+ FS MR in the same patient shows patchy enhancement of the thalamic, basal ganglia, and hypothalamic lesions. In this patient, the hypothalamic lesions are conspicuous on contrast imaging but subtle on noncontrast FLAIR MR.](images/app.statdx.com_image_thumbnail_07907bcb-564a-46eb-a133-d70b1e4efeed_annotated_true_size_900_quality_90_8aec5f98_20251018T151317Z.jpg) +*Axial T1 C+ FS MR in the same patient shows patchy enhancement of the thalamic, basal ganglia, and hypothalamic lesions. In this patient, the hypothalamic lesions are conspicuous on contrast imaging but subtle on noncontrast FLAIR MR.* + +![Axial T1 C+ FS MR in the same patient 3 months prior reveals avid contrast enhancement of the left optic nerve (anterior segment) . Laboratory assessment at that time confirmed MOG antibodies, but brain imaging was normal (not shown).](images/app.statdx.com_image_thumbnail_ed40238a-37a6-44eb-bd0e-96d2c6d0a27d_annotated_true_size_900_quality_90_f38886da_20251018T151317Z.jpg) +*Axial T1 C+ FS MR in the same patient 3 months prior reveals avid contrast enhancement of the left optic nerve (anterior segment) . Laboratory assessment at that time confirmed MOG antibodies, but brain imaging was normal (not shown).* + +![Axial FLAIR MR in a 4-year-old with florid optic neuritis and encephalopathy with positive anti-MOG antibody titer reveals abnormal hyperintensity and swelling within the bilateral basal ganglia , insular cortex , perventricular white matter, and posterior thalami.](images/app.statdx.com_image_thumbnail_02d881d3-77c4-494f-931a-734929316600_annotated_true_size_900_quality_90_eb67523c_20251018T151317Z.jpg) +*Axial FLAIR MR in a 4-year-old with florid optic neuritis and encephalopathy with positive anti-MOG antibody titer reveals abnormal hyperintensity and swelling within the bilateral basal ganglia , insular cortex , perventricular white matter, and posterior thalami.* + +![Axial T1 C+ FS MR in the same patient reveals minimal, if any, enhancement within the same abnormal brain regions.](images/app.statdx.com_image_thumbnail_57f986b6-8d58-423e-94f3-1fe232fe5243_annotated_true_size_900_quality_90_e4f08619_20251018T151317Z.jpg) +*Axial T1 C+ FS MR in the same patient reveals minimal, if any, enhancement within the same abnormal brain regions.* + +![Axial FLAIR MR in a 4-year-old with severe onset of encephalopathy with positive MOG antibody titer depicts several areas of abnormal cortical hyperintensity and edema as well as subcortical white matter signal abnormalities.](images/app.statdx.com_image_thumbnail_a6165758-1867-43aa-b3d6-338c799188fc_annotated_true_size_900_quality_90_b6a638c0_20251018T151317Z.jpg) +*Axial FLAIR MR in a 4-year-old with severe onset of encephalopathy with positive MOG antibody titer depicts several areas of abnormal cortical hyperintensity and edema as well as subcortical white matter signal abnormalities.* + +![Axial T1 C+ FS MR in the same patient reveals subtle leptomeningeal enhancement over the right parietal lobe. This constellation of findings represents cerebral cortical encephalitis, a finding unique to MOGAD and potentially predisposing to seizures.](images/app.statdx.com_image_thumbnail_f91cd62c-4b35-4234-af05-bff81550fce1_annotated_true_size_900_quality_90_e47a8829_20251018T151317Z.jpg) +*Axial T1 C+ FS MR in the same patient reveals subtle leptomeningeal enhancement over the right parietal lobe. This constellation of findings represents cerebral cortical encephalitis, a finding unique to MOGAD and potentially predisposing to seizures.* + +![Axial FLAIR MR in a 5-year-old patient with florid encephalopathy and emesis reveals multifocal T2-hyperintense lesions predominantly localizing to the brainstem and posterior fossa. Lesions are noted in the middle cerebellar peduncles, deep cerebellar white matter, and dorsal brainstem near the facial colliculus (area postrema, explaining vomiting).](images/app.statdx.com_image_thumbnail_2dbd89cf-d204-4c45-b035-96a76b225584_annotated_true_size_900_quality_90_20590ffb_20251018T151317Z.jpg) +*Axial FLAIR MR in a 5-year-old patient with florid encephalopathy and emesis reveals multifocal T2-hyperintense lesions predominantly localizing to the brainstem and posterior fossa. Lesions are noted in the middle cerebellar peduncles, deep cerebellar white matter, and dorsal brainstem near the facial colliculus (area postrema, explaining vomiting).* + +![Axial T1 C+ FS MR in the same patient reveals patchy partial ring and nodular enhancement in these abnormal areas.](images/app.statdx.com_image_thumbnail_25d3f494-9eff-4136-ae2f-41cd50e01442_annotated_true_size_900_quality_90_84b9d90f_20251018T151358Z.jpg) +*Axial T1 C+ FS MR in the same patient reveals patchy partial ring and nodular enhancement in these abnormal areas.* + + +### Additional Images + +![Axial FLAIR MR in a 7-year-old patient with MOGAD reveals subcortical white matter and deep nuclear gray matter involvement. This is a fairly typical appearance for MOGAD MR abnormalities.](images/app.statdx.com_image_thumbnail_5e346d9c-850c-4dcf-bd4d-1fd87e6882c0_annotated_true_size_900_quality_90_9ba04802_20251018T151359Z.jpg) +*Axial FLAIR MR in a 7-year-old patient with MOGAD reveals subcortical white matter and deep nuclear gray matter involvement. This is a fairly typical appearance for MOGAD MR abnormalities.* + +![Axial FLAIR MR in the same patient confirms patchy brainstem signal abnormality as well. This patient also presented with severe transverse myelopathy with longitudinally extensive transverse myelitis (LETM) pattern on spine MR (not shown).](images/app.statdx.com_image_thumbnail_041f5ec5-5083-4aa9-bfe3-1d17bf71105a_annotated_true_size_900_quality_90_ed8e4748_20251018T151359Z.jpg) +*Axial FLAIR MR in the same patient confirms patchy brainstem signal abnormality as well. This patient also presented with severe transverse myelopathy with longitudinally extensive transverse myelitis (LETM) pattern on spine MR (not shown).* + +![Coronal T2 MR through the frontal lobes of a pediatric patient with encephalopathy, optic neuritis, and confirmed MOGAD reveals abnormal hyperintensity within the left basal ganglia as well as mild hyperintensity in the left cisternal optic nerve .](images/app.statdx.com_image_thumbnail_5750a103-97cb-46c3-a22e-9ed93f090f99_annotated_true_size_900_quality_90_99611241_20251018T151359Z.jpg) +*Coronal T2 MR through the frontal lobes of a pediatric patient with encephalopathy, optic neuritis, and confirmed MOGAD reveals abnormal hyperintensity within the left basal ganglia as well as mild hyperintensity in the left cisternal optic nerve .* + +![Coronal T1 C+ FS MR in the same patient shows no enhancement in the slightly hypointense basal ganglia lesion , but avid enhancement of both cisternal optic nerves (bilateral anterior segment optic neuritis). Contrast is essential for assessing the optic nerves for signs of neuritis.](images/app.statdx.com_image_thumbnail_9da82dee-4a03-47fb-98d4-c52d939c6d46_annotated_true_size_900_quality_90_35a902b9_20251018T151400Z.jpg) +*Coronal T1 C+ FS MR in the same patient shows no enhancement in the slightly hypointense basal ganglia lesion , but avid enhancement of both cisternal optic nerves (bilateral anterior segment optic neuritis). Contrast is essential for assessing the optic nerves for signs of neuritis.* + diff --git a/docs_md/articles/neuromyelitis-optica-spectrum-disorders_54d4a8bc-9267-4df6-98c1-f22aae051d01.md b/docs_md/articles/neuromyelitis-optica-spectrum-disorders_54d4a8bc-9267-4df6-98c1-f22aae051d01.md index 91d3123..988fff4 100644 --- a/docs_md/articles/neuromyelitis-optica-spectrum-disorders_54d4a8bc-9267-4df6-98c1-f22aae051d01.md +++ b/docs_md/articles/neuromyelitis-optica-spectrum-disorders_54d4a8bc-9267-4df6-98c1-f22aae051d01.md @@ -39,6 +39,7 @@ pageTitle: "Neuromyelitis Optica Spectrum Disorders | STATdx" enhancedTitle: "Neuromyelitis Optica Spectrum Disorders" type: "DX" references: true +tables: 1 breadcrumbs: - "Brain" - "Diagnosis" @@ -335,6 +336,18 @@ breadcrumbs: 1. [Pittock SJ et al: Brain abnormalities in neuromyelitis optica. Arch Neurol. 63(3):390-6, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16533966%5Bpmid%5D) 1. [Wingerchuk DM et al: Revised diagnostic criteria for neuromyelitis optica. Neurology. 66(10):1485-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16717206%5Bpmid%5D) +## Tables + +# 2015 IPND Criteria for NMOSD + +| AQP4-IgG (+) Status | AQP4-IgG (-) or Unknown Status | +| --- | --- | +| AQP4-IgG positivity + 1 core clinical characteristic | At least 2 core clinical characteristics; 1 must be optic neuritis, LETM, or area postrema syndrome | +| - Optic neuritis - Acute myelitis - Area postrema syndrome Unexplained hiccups, or nausea and vomiting | If optic neuritis, MR with - Normal brain or nonspecific white matter lesions or - T2-hyperintense or T1 C+ enhancing optic nerve involving chiasm or > 50% of optic nerve | +| - Acute brainstem syndrome | If acute myelitis, MR with - Intramedullary lesion over 3 contiguous segments, or - Focal atrophy of at least 3 contiguous segments | +| - Symptomatic narcolepsy or diencephalic syndrome With NMOSD-typical diencephalic MR lesions | If area postrema syndrome, MR with - Dorsal medullary/area postrema lesion(s) | +| - Symptomatic cerebral syndrome With NMOSD-typical brain lesions | If acute brainstem syndrome, MR with - Periependymal brainstem lesions | + ## Images @@ -347,7 +360,7 @@ breadcrumbs: ![Axial T1 C+ MR in a 22-year-old woman with right vision loss demonstrates swelling with enhancement of the intraorbital segment of the right optic nerve.](images/app.statdx.com_image_thumbnail_e8ba18d2-7677-4bf1-8a89-ea3f7610a1dd_size_168_quality_85_e6e9aa1a_20251018T095348Z.jpg) *Axial T1 C+ MR in a 22-year-old woman with right vision loss demonstrates swelling with enhancement of the intraorbital segment of the right optic nerve.* -![Axial T1 C+ MR in a 22-year-old woman with right vision loss demonstrates swelling with enhancement of the intraorbital segment of the right optic nerve.](images/app.statdx.com_image_thumbnail_e8ba18d2-7677-4bf1-8a89-ea3f7610a1dd_size_174_quality_85_7d69b1cc_20251018T122441Z.jpg) +![Axial T1 C+ MR in a 22-year-old woman with right vision loss demonstrates swelling with enhancement of the intraorbital segment of the right optic nerve.](images/app.statdx.com_image_thumbnail_e8ba18d2-7677-4bf1-8a89-ea3f7610a1dd_size_174_quality_85_db2e719d_20251018T152348Z.jpg) *Axial T1 C+ MR in a 22-year-old woman with right vision loss demonstrates swelling with enhancement of the intraorbital segment of the right optic nerve.* ![Axial FLAIR MR (top) and T1 C+ MR (bottom) images in the same patient show hyperintense enhancing lesions along the cerebral peduncles. AQP4 antibody was positive in this patient with NMOSD. Brain lesions in NMOSD typically involve the deep white matter, periependymal regions, corpus callosum, corticospinal tracts, brainstem, and cerebellum.](images/app.statdx.com_image_thumbnail_d26008df-62a0-4116-a5a3-ebf9f767936e_annotated_true_size_900_quality_90_95e09ab2_20251018T122510Z.jpg) diff --git a/docs_md/articles/normal-pressure-hydrocephalus_ba3f857d-58de-4f21-8463-1631b4cb9972.md b/docs_md/articles/normal-pressure-hydrocephalus_ba3f857d-58de-4f21-8463-1631b4cb9972.md new file mode 100644 index 0000000..529baae --- /dev/null +++ b/docs_md/articles/normal-pressure-hydrocephalus_ba3f857d-58de-4f21-8463-1631b4cb9972.md @@ -0,0 +1,438 @@ +--- +title: "Normal-Pressure Hydrocephalus" +docid: "ba3f857d-58de-4f21-8463-1631b4cb9972" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "Normal-Pressure Hydrocephalus" + slug: "normal-pressure-hydrocephalus" + treeNodeId: null +category: "Brain" +cmeTopicId: "fbb5e19b-9cf4-446b-b7c7-f02091bc7b0f" +documentVersionId: "87d30aa9-d11d-4e89-b66b-ec30c687ba56" +imageCount: 25 +lastUpdated: "08/20/20" +pageDescription: "Normal-Pressure Hydrocephalus" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Normal-Pressure Hydrocephalus" +pageTitle: "Normal-Pressure Hydrocephalus | STATdx" +enhancedTitle: "Normal-Pressure Hydrocephalus" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "Normal-Pressure Hydrocephalus" +--- +# KEY FACTS + +- ## Terminology + + + - Ventriculomegaly with normal CSF pressure, altered CSF dynamics + - Idiopathic normal-pressure hydrocephalus (iNPH) +- ## Imaging + + + - Enlarged lateral & 3rd ventricles, 4th ventricle relatively normal + - **Evans index**(ratio of widest diameter of frontal horns to widest diameter of brain on same axial slice) ≥ 0.3 + - **Callosal angle** (angle between lateral ventricles on coronal image at level of posterior commissure) < 90° + - Disproportionately enlarged subarachnoid space hydrocephalus (**DESH**) (particularly sylvian fissures & basal cisterns) + - **Cingulate sulcus sign**: Narrowing of posterior cingulate sulcus compared with anterior + - Tight high convexity with effacement of parafalcine sulci + - Aqueductal flow void + - Aqueduct stroke volume (ASV) > 42 μL +- ## Top Differential Diagnoses + + + - Normal aging brain + - Alzheimer disease + - Multiinfarct dementia (MID) + - Subcortical arteriosclerotic encephalopathy +- ## Pathology + + + - Pathogenesis of **iNPH**poorly understood & controversial + - Secondary NPH (**sNPH**): Subarachnoid hemorrhage, meningitis, neurosurgery, or head trauma) +- ## Clinical Issues + + + - Heterogeneous syndrome (classic clinical triad = dementia, gait apraxia, urinary incontinence) +- ## Diagnostic Checklist + + + - Is ventricular dilation solely due to atrophy? + - Diagnostic challenge = identify shunt-responsive NPH + +# TERMINOLOGY + +- ## Abbreviations + + + - Normal-pressure hydrocephalus (NPH) +- ## Synonyms + + + - Idiopathic adult hydrocephalus syndrome +- ## Definitions + + + - Ventriculomegaly with normal CSF pressure, altered CSF dynamics + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Enlarged lateral & 3rd ventricles, 4th ventricle relatively normal + - Ventricular enlargement out of proportion to generalized sulcal enlargement + - Disproportionately enlarged subarachnoid space hydrocephalus (**DESH**) (particularly sylvian fissures & basal cisterns) + - High tight convexity with effacement of sulci at vertex + - ### Location + + + - Ventriculomegaly involves all 3 horns of lateral ventricles, plus 3rd ventricle + - 4th ventricle relatively spared + - ### Size + + + - **Evans index**(ratio of widest diameter of frontal horns to widest diameter of brain on same axial slice) ≥ 0.3 + - **Callosal angle** (angle between lateral ventricles on coronal image at level of posterior commissure) < 90° + - Widening of temporal horn > 6 mm + - ### Morphology + + + - Diffuse expansion of ventricles +- ## CT Findings + + + - ### NECT + + + - Ventriculomegaly with rounded frontal horns, out of proportion to sulcal atrophy (ventriculosulcal disproportion) + - Frontal/occipital periventricular hypodensities may be present + - Corpus callosal thinning (nonspecific) + - Prominent basal cisterns & sylvian fissures +- ## MR Findings + + + - Lateral ventricles enlarged with rounded frontal horns + - Focal bulging of roof of lateral ventricles + - Moderately dilated 3rd ventricle, relatively normal 4th ventricle + - Dilatation of optic & infundibular recesses of anterior 3rd ventricle + - **Cingulate sulcus sign**: Narrowing of posterior cingulate sulcus compared with anterior + - DESH + - High tight convexity with effacement of parafalcine sulci + - Corpus callosum bowed upward (may be impinged by falx) + - **Aqueductal flow void sign** + - Reflects increased CSF velocity through cerebral aqueduct + - May be reduced if flow compensation, fast spin-echo techniques used + - Periventricular "halo" high signal, primarily anterior to frontal horns or posterior to occipital horns of lateral ventricles + - 50-60% have periventricular & deep white matter (WM) lesions + - More frequent, severe compared to age-matched controls + - **Diffusion tensor imaging (DTI)** + - Increased fractional anisotropy (FA) values in posterior limb of internal capsule + - **MR elastography (MRE)** + - Increased stiffness in cerebral, occipital, parietal lobes + - Decreased stiffness in periventricular WM + - **MR perfusion** + - ASL: Reduced CBF in periventricular WM, basal ganglia, & thalamus +- ## Nuclear Medicine Findings + + + - ### PET + + + - F-18 FDG PET shows decreased regional cerebral metabolism + - SPECT: Cerebral blood flow decreased in patients with NPH + - **In-111 DTPA cisternography** + - Prominent ventricular activity with no flow over convexity at 24-48 hours + - High false-positive rate +- ## Other Modality Findings + + + - **Phase-contrast cine MR** + - Cardiac-gated CSF flow studies through aqueduct + - Hyperdynamic CSF flow in both systole & diastole + - Net CSF flow direction caudocranial, reverse of normal + - Aqueduct stroke volume (ASV) > 42 μL + - Lack of correlation between ASV & symptom severity + - ICP monitoring: Wave amplitude > 9-mm Hg correlates with post shunt cognitive improvement +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR with CSF flow studies + - CT helpful + +# DIFFERENTIAL DIAGNOSIS + +- [Normal Aging Brain](/document/normal-aging-brain/2a315550-b2ea-4afe-a2ef-f93a2209f276) + - Thin, periventricular high-signal rim is normal + - Few/no WM hyperintensities + - Proportionately enlarged ventricles & subarachnoid spaces +- [Alzheimer Disease](/document/alzheimer-disease/f71f5cf5-b1af-4c6d-b145-b4c10eec7b58) + - Dementia out of proportion to gait disturbance + - Large parahippocampal fissures, small hippocampi, sulcal enlargement +- [Multiinfarct Dementia](/document/vascular-dementia/f59dab57-c511-4369-8fcc-592421a4b8d1) + - Multiple infarcts on imaging +- [Subcortical Arteriosclerotic Encephalopathy (Binswanger Disease)](/document/chronic-hypertensive-encephalopathy/1afc1f3f-203d-4cdf-8d49-2283cb13d6db) + - Irreversible ischemic degeneration of periventricular & deep WM + - MR shows extensive periventricular & deep WM hyperintensities, enlarged ventricles + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Pathogenesis of idiopathic NPH (iNPH)****poorly understood & controversial + - Reduced CBF, altered CSF resorption without increased CSF pressure + - Brain expands in systole, causes CSF displacement + - Loss of parenchymal compliance, altered viscoelastic properties of ventricular wall + - Increased interstitial fluid (ISF), pulsation pressure directed toward ventricles, water hammer effect + - May be further complicated by microangiopathy (including venous compromise), atrophy + - Vascular etiology + - Altered arterial hemodynamics + - Cortical venous dysfunction, impairs CSF absorption + - Imbalance between CSF & ISF + - Excess ISF, reversing ISF flow, impaired removal of neurotoxic compounds (β-amyloid & tau) + - Glymphatic system + - Facilitates brain fluid clearance & waste removal during sleep + - Studies implicated impaired glymphatic function in both Alzheimer disease (AD) & iNPH + - Secondary NPH (**sNPH**): Subarachnoid hemorrhage, meningitis, neurosurgery, or head trauma +- ## Microscopic Features + + + - Arachnoid fibrosis (50%) + - Periventricular tissue: Disruption of ependyma, edema, neuronal degeneration, & gliosis + - Cerebral parenchyma: Almost 50% show no significant pathology + - 20% neurofibrillary tangles, other AD changes + - 10% arteriosclerosis, ischemic encephalomalacia + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Heterogeneous syndrome (classic clinical triad = dementia, gait disorder, urinary incontinence) + - Gait disorder: Disturbed postural & locomotor reflexes in absence of primary sensorimotor deficits + - Bladder dysfunction: Urinary urgency with difficulty inhibiting bladder emptying + - Dementia: Apathy or amotivation, daytime sleepiness, psychomotor slowing + - ### Clinical profile + + + - Reversible cause of dementia + - Confirmed iNPH, possible iNPH & probable iNPH based on clinical findings, imaging, response to high-volume lumbar tap +- ## Demographics + + + - ### Age + + + - Average onset: 70 years + - Idiopathic form of NPH tends to present in elderly + - Patients with chronic communicating hydrocephalus due to prior known insult tend to present at earlier age + - ### Sex + + + - M = F + - ### Ethnicity + + + - No ethnic predilection + - ### Epidemiology + + + - Accounts for ~ 5-6% of dementias + - Reported prevalence 0.5-3.0% in elderly population +- ## Natural History & Prognosis + + + - Continuing cognitive & motor decline, akinetic mutism, & eventual death + - Potentially reversible cause of dementia when shunted +- ## Treatment + + + - **Presurgical confirmatory tests** + - Tap test (large-volume lumbar puncture) + - To assess patient's response to CSF removal + - Remove 30-50 mL of CSF + - Gait & cognition evaluated before LP & 3-4 hours after LP + - High positive predictive value (73-100%) + - Drainage of CSF via spinal catheter (external lumbar drainage) + - Lumbar catheter placed, CSF removal (10 mL/h) for 2-3 days + - Gait & cognition evaluated before & after + - Infusion testing + - 2 LP needles in lumbar subarachnoid space: 1 connected to infusion pump to infuse artificial CSF, 1 to closed-pressure recording device + - CSF outflow resistance (Rουτ) & conductance calculated + - Rουτ 8-18 mm Hg/mL/min associated with favorable shunt outcomes + - 1-2 hours to complete, needs specialized equipment + - **CSF diversion surgery** + - Shunt surgery indicated for patients who respond to CSF drainage or have CSF hydrodynamic variables consistent with NPH + - Endoscopic third ventriculostomy (ETV) + - Has not been favorable for treatment of iNPH + - Predictors of positive response to shunting remains elusive + - Gait disturbance is clinical symptom most likely to respond to surgery + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Whether ventricular dilation is solely due to atrophy + - Diagnostic challenge = identify shunt-responsive NPH + + e10a5877-65d6-43e9-a9ce-75d0ebe5fd92 + +## References + +# Selected References + +1. [Capone PM et al: Neuroimaging of normal pressure hydrocephalus and hydrocephalus. Neurol Clin. 38(1):171-83, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31761057%5Bpmid%5D) +1. [Reeves BC et al: Glymphatic system impairment in Alzheimer's disease and idiopathic normal pressure hydrocephalus. Trends Mol Med. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31959516%5Bpmid%5D) +1. [Graff-Radford NR et al: Normal pressure hydrocephalus. Continuum (Minneap Minn). 25(1):165-86, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30707192%5Bpmid%5D) +1. [Isaacs AM et al: Current update on treatment strategies for idiopathic normal pressure hydrocephalus. Curr Treat Options Neurol. 21(12):65, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31792620%5Bpmid%5D) +1. [Liew BS et al: Current updates on idiopathic normal pressure hydrocephalus. Asian J Neurosurg. 14(3):648-56, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31497081%5Bpmid%5D) +1. [Skalický P et al: Normal pressure hydrocephalus-an overview of pathophysiological mechanisms and diagnostic procedures. Neurosurg Rev. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31705404%5Bpmid%5D) +1. [Bradley WG Jr: CSF Flow in the brain in the context of normal pressure hydrocephalus. AJNR Am J Neuroradiol. 36(5):831-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25355813%5Bpmid%5D) +1. [Hoza D et al: DTI-MRI biomarkers in the search for normal pressure hydrocephalus aetiology: a review. Neurosurg Rev. 38(2):239-44; discussion 244, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25345377%5Bpmid%5D) +1. [Ringstad G et al: Aqueductal stroke volume: comparisons with intracranial pressure scores in idiopathic normal pressure hydrocephalus. AJNR Am J Neuroradiol. 36(9):1623-30, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25977480%5Bpmid%5D) +1. [Chotai S et al: External lumbar drain: a pragmatic test for prediction of shunt outcomes in idiopathic normal pressure hydrocephalus. Surg Neurol Int. 5:12, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24678428%5Bpmid%5D) +1. [Reddy GK et al: Long-term outcomes of ventriculoperitoneal shunt surgery in patients with hydrocephalus. World Neurosurg. 81(2):404-10, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=23380280%5Bpmid%5D) +1. [Torsnes L et al: Treatment and clinical outcome in patients with idiopathic normal pressure hydrocephalus--a systematic review. Dan Med J. 61(10):A4911, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25283616%5Bpmid%5D) +1. [Virhammar J et al: Preoperative prognostic value of MRI findings in 108 patients with idiopathic normal pressure hydrocephalus. AJNR Am J Neuroradiol. 35(12):2311-8, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25012669%5Bpmid%5D) +1. [Williams MA et al: Diagnosis and management of idiopathic normal-pressure hydrocephalus. Neurol Clin Pract. 3(5):375-85, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24175154%5Bpmid%5D) +1. [Kim MJ et al: Differential diagnosis of idiopathic normal pressure hydrocephalus from other dementias using diffusion tensor imaging. AJNR Am J Neuroradiol. 32(8):1496-503, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21700790%5Bpmid%5D) +1. [Linninger AA et al: Normal and hydrocephalic brain dynamics: the role of reduced cerebrospinal fluid reabsorption in ventricular enlargement. Ann Biomed Eng. 37(7):1434-47, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19373558%5Bpmid%5D) +1. [Scollato A et al: Changes in aqueductal CSF stroke volume in shunted patients with idiopathic normal-pressure hydrocephalus. AJNR Am J Neuroradiol. 30(8):1580-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19461060%5Bpmid%5D) +1. [Woodworth GF et al: Cerebrospinal fluid drainage and dynamics in the diagnosis of normal pressure hydrocephalus. Neurosurgery. 64(5):919-25; discussion 925-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19404152%5Bpmid%5D) +1. [Shprecher D et al: Normal pressure hydrocephalus: diagnosis and treatment. Curr Neurol Neurosci Rep. 8(5):371-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18713572%5Bpmid%5D) +1. [Owler BK et al: Normal pressure hydrocephalus and cerebral blood flow: a PET study of baseline values. J Cereb Blood Flow Metab. 24(1):17-23, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14688613%5Bpmid%5D) +1. [Czosnyka M et al: Age dependence of cerebrospinal pressure-volume compensation in patients with hydrocephalus. J Neurosurg. 94(3):482-6, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11235954%5Bpmid%5D) +1. [Kizu O et al: Proton chemical shift imaging in normal pressure hydrocephalus. AJNR Am J Neuroradiol. 22(9):1659-64, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11673158%5Bpmid%5D) +1. [Tullberg M et al: Normal pressure hydrocephalus: vascular white matter changes on MR images must not exclude patients from shunt surgery. AJNR Am J Neuroradiol. 22(9):1665-73, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11673159%5Bpmid%5D) +1. [Parkkola RK et al: Cerebrospinal fluid flow in patients with dilated ventricles studied with MR imaging. Eur Radiol. 10(9):1442-6, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10997433%5Bpmid%5D) +1. [Bech RA et al: Frontal brain and leptomeningeal biopsy specimens correlated with cerebrospinal fluid outflow resistance and B-wave activity in patients suspected of normal-pressure hydrocephalus. Neurosurgery. 40(3):497-502, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9055288%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal T1 MR shows large lateral ventricles , thinning of the corpus callosum , and a relatively normal 4th ventricle in a patient with iNPH.](images/app.statdx.com_image_thumbnail_a2b16904-c08e-4fc6-a3fc-276862837b89_annotated_true_size_900_quality_90_53b3054c_20251018T155136Z.jpg) +*Sagittal T1 MR shows large lateral ventricles , thinning of the corpus callosum , and a relatively normal 4th ventricle in a patient with iNPH.* + +![Sagittal T1 MR shows large lateral ventricles , thinning of the corpus callosum , and a relatively normal 4th ventricle in a patient with iNPH.](images/app.statdx.com_image_thumbnail_a2b16904-c08e-4fc6-a3fc-276862837b89_size_174_quality_85_31388a23_20251018T155131Z.jpg) +*Sagittal T1 MR shows large lateral ventricles , thinning of the corpus callosum , and a relatively normal 4th ventricle in a patient with iNPH.* + +![Axial CECT demonstrates typical findings suggestive of iNPH. There is enlargement of the lateral ventricles and sylvian fissures out of proportion to the amount of general sulcal enlargement. The frontal horns show a characteristic rounded appearance. Periventricular hypodensities could reflect interstitial migration of CSF.](images/app.statdx.com_image_thumbnail_85c645de-d929-40ae-a6fe-1be12714e0f0_annotated_true_size_900_quality_90_a8c68531_20251018T155136Z.jpg) +*Axial CECT demonstrates typical findings suggestive of iNPH. There is enlargement of the lateral ventricles and sylvian fissures out of proportion to the amount of general sulcal enlargement. The frontal horns show a characteristic rounded appearance. Periventricular hypodensities could reflect interstitial migration of CSF.* + +![Axial T2 MR in a patient with NPH demonstrates lateral ventricular enlargement and disproportionately enlarged sylvian fissure (DESH). Evans index, which is the ratio of the maximum width of the frontal horns to the maximum internal diameter of the skull at the same level, measures 0.38. Normal Evans index is < 0.3.](images/app.statdx.com_image_thumbnail_ebc7d0a3-c2f8-464c-ac29-5c68c19bb84b_annotated_true_size_900_quality_90_71d36abb_20251018T155136Z.jpg) +*Axial T2 MR in a patient with NPH demonstrates lateral ventricular enlargement and disproportionately enlarged sylvian fissure (DESH). Evans index, which is the ratio of the maximum width of the frontal horns to the maximum internal diameter of the skull at the same level, measures 0.38. Normal Evans index is < 0.3.* + +![Coronal T2 MR in a patient with NPH shows reduced callosal angle (71°). The callosal angle is measured at the level of the posterior commissure and a normal value is between 100-120°.](images/app.statdx.com_image_thumbnail_aeb64c28-c3d9-458e-a6bd-ad656d9af047_annotated_true_size_900_quality_90_00409dd4_20251018T155136Z.jpg) +*Coronal T2 MR in a patient with NPH shows reduced callosal angle (71°). The callosal angle is measured at the level of the posterior commissure and a normal value is between 100-120°.* + +![Sagittal T1 MR demonstrates the cingulate sulcus sign in a patient with NPH with narrowing of the posterior 1/2 of the cingulate sulcus as compared with the anterior .](images/app.statdx.com_image_thumbnail_2339b846-aea2-4877-97a9-233ee3ebe4ee_annotated_true_size_900_quality_90_3a2e725a_20251018T155136Z.jpg) +*Sagittal T1 MR demonstrates the cingulate sulcus sign in a patient with NPH with narrowing of the posterior 1/2 of the cingulate sulcus as compared with the anterior .* + +![Axial FLAIR MR in the same patient demonstrates disproportionately enlarged subarachnoid spaces , consistent with DESH, and narrowing of the sulci and subarachnoid spaces over the high convexity parasagittal frontoparietal regions with a tight interhemispheric fissure .](images/app.statdx.com_image_thumbnail_9cdff4ef-acfa-4c07-ae32-9fa89770c6a0_annotated_true_size_900_quality_90_7f514e45_20251018T155136Z.jpg) +*Axial FLAIR MR in the same patient demonstrates disproportionately enlarged subarachnoid spaces , consistent with DESH, and narrowing of the sulci and subarachnoid spaces over the high convexity parasagittal frontoparietal regions with a tight interhemispheric fissure .* + +![Sagittal T1 MR in a patient with NPH demonstrates focal bulging of the roof of the lateral ventricles , which has been recently described.](images/app.statdx.com_image_thumbnail_5f38cad8-ece0-458b-9d75-babe49ca034e_annotated_true_size_900_quality_90_b94e3d2e_20251018T155136Z.jpg) +*Sagittal T1 MR in a patient with NPH demonstrates focal bulging of the roof of the lateral ventricles , which has been recently described.* + +![Twenty-four hour multiplanar In-111 DTPA cisternography in a patient with NPH shows radiotracer in the lateral ventricles with lack of activity over the convexity . Normally, there should be radiotracer movement over the convexities at 24 hours. (Courtesy C. Singh, MD and A. Ali, MD.)](images/app.statdx.com_image_thumbnail_ff9b7855-f13a-4176-883e-c4e89f0f7dd1_annotated_true_size_900_quality_90_5f1a4d63_20251018T155136Z.jpg) +*Twenty-four hour multiplanar In-111 DTPA cisternography in a patient with NPH shows radiotracer in the lateral ventricles with lack of activity over the convexity . Normally, there should be radiotracer movement over the convexities at 24 hours. (Courtesy C. Singh, MD and A. Ali, MD.)* + +![Axial T2 MR in 65 year old with NPH shows dilated temporal horns and low-signal flow void in the aqueduct caused by hyperdynamic flow of CSF.](images/app.statdx.com_image_thumbnail_47b09bc1-bb20-4e14-8e81-59f6cfdf7cf8_annotated_true_size_900_quality_90_81a16de8_20251018T155136Z.jpg) +*Axial T2 MR in 65 year old with NPH shows dilated temporal horns and low-signal flow void in the aqueduct caused by hyperdynamic flow of CSF.* + +![Axial phase-contrast cine MR CSF flow study shows increased velocity of CSF through the dilated aqueduct . There is more hyperdynamic flow through the aqueduct than the cisterns, where no high-velocity signal change is seen. Flow is incidentally noted in the posterior cerebral arteries .](images/app.statdx.com_image_thumbnail_f9fbb916-dc1a-462b-a2c3-a618a645ff44_annotated_true_size_900_quality_90_c77dcbbf_20251018T155139Z.jpg) +*Axial phase-contrast cine MR CSF flow study shows increased velocity of CSF through the dilated aqueduct . There is more hyperdynamic flow through the aqueduct than the cisterns, where no high-velocity signal change is seen. Flow is incidentally noted in the posterior cerebral arteries .* + + +### Additional Images + +![Axial NECT shows ventriculomegaly with rounded frontal horns out of proportion to sulcal enlargement.](images/app.statdx.com_image_thumbnail_d3a96325-6e69-4daf-8c92-172394deeef7_annotated_true_size_900_quality_90_3f001b22_20251018T155143Z.jpg) +*Axial NECT shows ventriculomegaly with rounded frontal horns out of proportion to sulcal enlargement.* + +![Axial T2WI MR shows ventricles dilated out of proportion to sulcal enlargement. Periventricular deep white matter lesions are also present.](images/app.statdx.com_image_thumbnail_3bcdbf6c-f961-4b53-86da-721bb8b6081c_annotated_true_size_900_quality_90_c7bfeb2c_20251018T155143Z.jpg) +*Axial T2WI MR shows ventricles dilated out of proportion to sulcal enlargement. Periventricular deep white matter lesions are also present.* + +![Axial T2WI MR shows ventriculomegaly.](images/app.statdx.com_image_thumbnail_f5f5e9ca-fa1d-4e95-8a27-08852c110848_annotated_true_size_900_quality_90_9dad0c1b_20251018T155145Z.jpg) +*Axial T2WI MR shows ventriculomegaly.* + +![Sagittal T1WI MR in the same patient shows an accentuated aqueductal flow void .](images/app.statdx.com_image_thumbnail_6adeb8c6-9016-42c7-9aaf-2f86ff10ba70_annotated_true_size_900_quality_90_96ea3ea4_20251018T155146Z.jpg) +*Sagittal T1WI MR in the same patient shows an accentuated aqueductal flow void .* + +![Axial T2WI MR shows enlarged ventricles with rounded frontal horns.](0c4cea37-93ae-487c-85f3-c8a7d71d9aa6) +*Axial T2WI MR shows enlarged ventricles with rounded frontal horns.* + +![Axial FLAIR MR in the same patient shows ventriculomegaly out of proportion to sulcal enlargement.](2d75e2a1-bb3d-4bb4-beb9-a0b7cd9ac87d) +*Axial FLAIR MR in the same patient shows ventriculomegaly out of proportion to sulcal enlargement.* + +![Axial CECT shows symmetric dilatation of the ventricles and sylvian fissures out of proportion to sulcal enlargement. Frontal and occipital periventricular hypodensities suggest transependymal CSF flow.](c6e7ae11-efe8-47e7-840b-1f5b32a0f39e) +*Axial CECT shows symmetric dilatation of the ventricles and sylvian fissures out of proportion to sulcal enlargement. Frontal and occipital periventricular hypodensities suggest transependymal CSF flow.* + +![Axial CECT in the same patient shows symmetric dilatation of the ventricles and sylvian fissures out of proportion to sulcal enlargement. Frontal and occipital periventricular hypodensities are also present.](9edb3ebd-4157-434a-81dd-15f7ac5596a9) +*Axial CECT in the same patient shows symmetric dilatation of the ventricles and sylvian fissures out of proportion to sulcal enlargement. Frontal and occipital periventricular hypodensities are also present.* + +![Axial T2WI MR shows a typical case of normal pressure hydrocephalus. There is enlargement of the lateral ventricles with no sulcal enlargement. The frontal horns show a typical rounded configuration.](feb58db2-dbcf-48ca-9ecf-10d820a2f460) +*Axial T2WI MR shows a typical case of normal pressure hydrocephalus. There is enlargement of the lateral ventricles with no sulcal enlargement. The frontal horns show a typical rounded configuration.* + +![Axial T2WI MR shows dilated temporal horns out of proportion to the sulcal prominence. Notice the low-signal flow void in the aqueduct caused by hyperdynamic flow of CSF.](d9961a29-b001-45f8-8299-4d0d582d3b1b) +*Axial T2WI MR shows dilated temporal horns out of proportion to the sulcal prominence. Notice the low-signal flow void in the aqueduct caused by hyperdynamic flow of CSF.* + +![Sagittal T1WI MR shows enlargement of the 3rd and lateral ventricles. The infundibular recess is enlarged and bulges downward. Note mild thinning of the corpus callosum .](d7ea7a3a-0439-4962-863a-eaa68f1e3090) +*Sagittal T1WI MR shows enlargement of the 3rd and lateral ventricles. The infundibular recess is enlarged and bulges downward. Note mild thinning of the corpus callosum .* + +![Axial FLAIR MR shows enlarged ventricles out of proportion to the sulcal enlargement. Notice that periventricular hyperintensity is also present .](ba993dae-1031-4377-8266-a63c7401e6e3) +*Axial FLAIR MR shows enlarged ventricles out of proportion to the sulcal enlargement. Notice that periventricular hyperintensity is also present .* + +![Axial T2WI MR in the same patient shows dilated ventricles. Normal pressure hydrocephalus accounts for ~ 5-6% of all dementias. The classic Hakim triad of dementia, gait apraxia, and urinary incontinence is present in a minority of patients.](f12e817a-78e3-4e95-82cd-a1015b08a31b) +*Axial T2WI MR in the same patient shows dilated ventricles. Normal pressure hydrocephalus accounts for ~ 5-6% of all dementias. The classic Hakim triad of dementia, gait apraxia, and urinary incontinence is present in a minority of patients.* + +![Axial NECT shows large ventricles out of proportion to the sulcal prominence with a rounded appearance of the frontal horns .](155bb56c-c65a-499e-a6ff-c17c446f1209) +*Axial NECT shows large ventricles out of proportion to the sulcal prominence with a rounded appearance of the frontal horns .* + +![Axial T2WI MR in the same patient shows ventriculomegaly. The patient presented with the classic clinical triad of NPH: Dementia, gait apraxia, and urinary incontinence. One treatment option is ventricular shunting. The response to shunting is variable.](f855cf16-59b3-438f-bdd1-282537eb5091) +*Axial T2WI MR in the same patient shows ventriculomegaly. The patient presented with the classic clinical triad of NPH: Dementia, gait apraxia, and urinary incontinence. One treatment option is ventricular shunting. The response to shunting is variable.* + diff --git a/docs_md/articles/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md b/docs_md/articles/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md index 0b23caa..5e920e1 100644 --- a/docs_md/articles/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md +++ b/docs_md/articles/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md @@ -44,6 +44,39 @@ pageTitle: "Pediatric Multiple Sclerosis, Brain | STATdx" enhancedTitle: "Pediatric Multiple Sclerosis, Brain" type: "DX" references: true +ddx: true +anatomy: + - "{'authors': 'Karen L. Salzman, MD, FACR; Ryan P. 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Ric Harnsberger, MD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/2e74a767-3f28-49be-a50f-1dbcc10ce90f', 'category': 'Head and Neck', 'compareUrl': '/compare/document/2e74a767-3f28-49be-a50f-1dbcc10ce90f/related-anatomy/treeNode?subContext=CNIX (Glossopharyngeal Nerve)', 'documentId': '2e74a767-3f28-49be-a50f-1dbcc10ce90f', 'documentType': 'ANATOMY', 'documentUrl': '/document/cnix-glossopharyngeal-nerve/2e74a767-3f28-49be-a50f-1dbcc10ce90f', 'enhancedTitle': 'CNIX (Glossopharyngeal Nerve)', 'entryDate': '12/20/23', 'imageCount': 17, 'imageUrl': '/image/thumbnail/0568113e-ccf1-462c-a4f2-6c6f3b17a483?size=174&quality=85', 'inCompareCart': False, 'rank': 26, 'referenceCount': 0, 'showCompareButton': False, 'title': 'CNIX (Glossopharyngeal Nerve)'}" + - "{'authors': 'Aparna Singhal, MD; H. Ric Harnsberger, MD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/83868689-c995-4608-bed3-f59664cbd586', 'category': 'Head and Neck', 'compareUrl': '/compare/document/83868689-c995-4608-bed3-f59664cbd586/related-anatomy/treeNode?subContext=CNX (Vagus Nerve)', 'documentId': '83868689-c995-4608-bed3-f59664cbd586', 'documentType': 'ANATOMY', 'documentUrl': '/document/cnx-vagus-nerve/83868689-c995-4608-bed3-f59664cbd586', 'enhancedTitle': 'CNX (Vagus Nerve)', 'entryDate': '02/01/24', 'imageCount': 15, 'imageUrl': '/image/thumbnail/49808556-8ea5-4fdf-8a80-170f28e0213f?size=174&quality=85', 'inCompareCart': False, 'rank': 27, 'referenceCount': 0, 'showCompareButton': False, 'title': 'CNX (Vagus Nerve)'}" + - "{'authors': 'Paula J. Woodward, MD, FSRU', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/080771c2-02f3-408d-ad70-04a80d849500', 'category': 'Ultrasound', 'compareUrl': '/compare/document/080771c2-02f3-408d-ad70-04a80d849500/related-anatomy/treeNode?subContext=Brain', 'documentId': '080771c2-02f3-408d-ad70-04a80d849500', 'documentType': 'ANATOMY', 'documentUrl': '/document/brain/080771c2-02f3-408d-ad70-04a80d849500', 'enhancedTitle': 'Brain', 'entryDate': '12/20/17', 'imageCount': 77, 'imageUrl': '/image/thumbnail/244b60fe-e15f-4ffb-8a39-cdbfe64fb3c4?size=174&quality=85', 'inCompareCart': False, 'rank': 28, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Brain'}" + - "{'authors': 'Aparna Singhal, MD; Ryan P. Cabeen, PhD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/71012f02-fab7-42ed-bc60-e584dc229ccb', 'category': 'Brain', 'compareUrl': '/compare/document/71012f02-fab7-42ed-bc60-e584dc229ccb/related-anatomy/treeNode?subContext=Hypoglossal Nerve (CNXII)', 'documentId': '71012f02-fab7-42ed-bc60-e584dc229ccb', 'documentType': 'ANATOMY', 'documentUrl': '/document/hypoglossal-nerve-cnxii/71012f02-fab7-42ed-bc60-e584dc229ccb', 'enhancedTitle': 'Hypoglossal Nerve (CNXII)', 'entryDate': '10/20/20', 'imageCount': 18, 'imageUrl': '/image/thumbnail/b997f8dc-536c-4480-8778-bf6949b83a18?size=174&quality=85', 'inCompareCart': False, 'rank': 29, 'referenceCount': 0, 'showCompareButton': False, 'title': 'Hypoglossal Nerve (CNXII)'}" + - "{'authors': 'Thomas Jose Eluvathingal Muttikkal, MD; H. Ric Harnsberger, MD', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/18e60151-70bc-40a1-9b4f-4b86f8fd65c2', 'category': 'Head and Neck', 'compareUrl': '/compare/document/18e60151-70bc-40a1-9b4f-4b86f8fd65c2/related-anatomy/treeNode?subContext=CNXI (Accessory Nerve)', 'documentId': '18e60151-70bc-40a1-9b4f-4b86f8fd65c2', 'documentType': 'ANATOMY', 'documentUrl': '/document/cnxi-accessory-nerve/18e60151-70bc-40a1-9b4f-4b86f8fd65c2', 'enhancedTitle': 'CNXI (Accessory Nerve)', 'entryDate': '11/28/23', 'imageCount': 10, 'imageUrl': '/image/thumbnail/f43628f4-42a9-4331-8508-079cb8abf634?size=174&quality=85', 'inCompareCart': False, 'rank': 30, 'referenceCount': 0, 'showCompareButton': False, 'title': 'CNXI (Accessory Nerve)'}" +cases: 2 breadcrumbs: - "Pediatrics" - "Diagnosis" @@ -376,7 +409,7 @@ breadcrumbs: - 95% with clinically definite MS have positive MR findings - 4f257a7a-e9b9-4b8e-81ab-2922fcef8a3b + 4f9b413c-409d-4c27-ad17-6de8aa9ba6d3 ## References @@ -409,6 +442,144 @@ breadcrumbs: 1. [Janardhan V et al: Multiple sclerosis: hyperintense lesions in the brain on nonenhanced T1-weighted MR images evidenced as areas of T1 shortening. Radiology. 244(3):823-31, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17690319%5Bpmid%5D) 1. [Traboulsee AL et al: The role of MRI in the diagnosis of multiple sclerosis. Adv Neurol. 98:125-46, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16400831%5Bpmid%5D) +## Differential diagnosis + +### Abnormal Shape/Configuration of Corpus Callosum +DDX:238ca32d-6bc6-4f5a-81b1-6601dd605856 + +### Complex Cranial Nerve IX-XII Neuropathy +DDX:2b17f148-bf71-4b13-8373-d3c65459dd67 + +### Enhancing Cranial Nerve(s) +DDX:6471fb1c-d46d-47cd-8322-1671e82335c3 + +### Hemifacial Spasm +DDX:1b390143-1212-4447-beb3-ed9e85ef34e4 + +### Homonymous Hemianopsia +DDX:1cdca8f4-95f8-4d19-b28a-19a433d1a624 + +### Oculomotor, Trochlear, or Abducens Neuropathy +DDX:58b32fb2-78a3-48c1-9aaf-5222667fe59f + +### Peripheral Facial Nerve Paralysis +DDX:4da52ac4-c03c-4711-ae7e-bb4f2f7c5ab8 + +### Ring-Enhancing Lesions +DDX:3463ac7a-c216-4fbd-aa73-03166a6ebc52 + +### Signal/Attenuation Abnormalities of Corpus Callosum +DDX:578bd09b-da74-43a2-b068-2d4854a61254 + +### Trigeminal Neuralgia +DDX:294f4a67-30d6-4488-bd1a-931d1f8c7609 + +### Trigeminal Neuropathy +DDX:6d668284-4c8e-4a14-90b6-c7abf86065e6 + +### Vocal Cord Paralysis (Left) +DDX:50022134-773c-4771-b07d-4d64c6c2dbe5 + +### Vocal Cord Paralysis (Right) +DDX:b8a07cab-5427-4efe-b55d-2460fec053db + +## Anatomy + +### White Matter Tracts +Brain/ANATOMY:846101a2-e892-4c70-9a32-c9fa887d073a + +### Language Overview +Brain/ANATOMY:40f2ed79-0d31-4943-aaa2-7c3244a7e87b + +### Cranial Nerves Overview +Brain/ANATOMY:1edc42e3-5c31-42b9-b8b8-44cc3bd38432 + +### Olfactory Nerve (CNI) +Brain/ANATOMY:c4a6920b-a112-4a51-921f-60d2f0a479ef + +### Optic Nerve (CNII) +Brain/ANATOMY:54712d62-1940-4028-8161-4cfd3c4dbb20 + +### Oculomotor Nerve (CNIII) +Brain/ANATOMY:495aba11-106f-439d-8516-6a445b085919 + +### Trochlear Nerve (CNIV) +Brain/ANATOMY:cc69e654-b9cd-465b-8437-fba44f3c034b + +### Trigeminal Nerve (CNV) +Brain/ANATOMY:06e99da6-48fc-45f4-a571-97d12a15f365 + +### Abducens Nerve (CNVI) +Brain/ANATOMY:655e63da-d744-4267-9d90-07f52493bbc0 + +### Facial Nerve (CNVII) +Brain/ANATOMY:2f4818dd-6438-405b-8561-5cbbb9c91562 + +### Vestibulocochlear Nerve (CNVIII) +Brain/ANATOMY:498e844d-faca-4c6a-bff1-9c6ad4993e62 + +### Glossopharyngeal Nerve (CNIX) +Brain/ANATOMY:172680f5-d290-4a02-b07e-63c1da75e148 + +### Vagus Nerve (CNX) +Brain/ANATOMY:68b6ede4-c797-4d55-8d29-aed3df441741 + +### Accessory Nerve (CNXI) +Brain/ANATOMY:9d50453e-c26a-46a3-826e-265736e43174 + +### CNXII (Hypoglossal Nerve) +Head and Neck/ANATOMY:f734d678-561c-47fd-afb5-dab6afacc1a8 + +### Optic Nerve/Sheath Complex +Head and Neck/ANATOMY:fdb933e1-ce0c-4f99-9ddb-cbe5f3b0f015 + +### Cranial Nerves Overview +Head and Neck/ANATOMY:170ad135-ca16-497a-80de-5a24b9ca2f47 + +### CNI (Olfactory Nerve) +Head and Neck/ANATOMY:54c329dd-8363-4b61-9af3-bf78909ea790 + +### CNII (Optic Nerve) +Head and Neck/ANATOMY:1b5322bb-bdca-4605-9ab5-43598b5c322f + +### CNIII (Oculomotor Nerve) +Head and Neck/ANATOMY:ad21daaf-1f28-4f9c-bc74-3d0142c167ab + +### CNIV (Trochlear Nerve) +Head and Neck/ANATOMY:52486223-9cb5-43f5-b9af-c46431ae6637 + +### CNV (Trigeminal Nerve) +Head and Neck/ANATOMY:9db87cfa-58ff-45fd-a7fd-fe8c73eb9770 + +### CNVI (Abducens Nerve) +Head and Neck/ANATOMY:923d7c4a-93a3-42ba-b8db-c7b15348e473 + +### CNVII (Facial Nerve) +Head and Neck/ANATOMY:98cb2d45-e64c-4295-9662-3470cd46513a + +### CNVIII (Vestibulocochlear Nerve) +Head and Neck/ANATOMY:e9917c41-94c9-46aa-b9d8-b196c375d35b + +### CNIX (Glossopharyngeal Nerve) +Head and Neck/ANATOMY:2e74a767-3f28-49be-a50f-1dbcc10ce90f + +### CNX (Vagus Nerve) +Head and Neck/ANATOMY:83868689-c995-4608-bed3-f59664cbd586 + +### Brain +Ultrasound/ANATOMY:080771c2-02f3-408d-ad70-04a80d849500 + +### Hypoglossal Nerve (CNXII) +Brain/ANATOMY:71012f02-fab7-42ed-bc60-e584dc229ccb + +### CNXI (Accessory Nerve) +Head and Neck/ANATOMY:18e60151-70bc-40a1-9b4f-4b86f8fd65c2 + +## Cases + +- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': 'a9ec521a-8d2e-4d27-9e87-79eaf424d678', 'description': 'Classic appearance of Devic disease, involving the optic nerves and spinal cord, with no brain parenchymal abnormalities.\n\nBrain examination (#1-4) shows normal FLAIR (#1), and markedly enhancing right optic nerve and chiasm (arrows, #3, 4). There is also abnormal T2 hyperintensity in the left optic nerve on the STIR image (open arrow, #2).\n\nEvaluation of the spinal cord (#5-10) shows a long segment of cord enlargement with T2 hyperintensity, and ill-defined enhancement (arrows).', 'history': 'Myelopathic and blind in both eyes.', 'imagePoolId': '98716ff1-9b49-4826-be9e-8d2b4073814e', 'name': 'Devic Disease (neuromyelitis optica)', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'de2e7c22-1bbb-4ca4-94ea-5a530e24b723', 'description': 'Axial T1WI (#1) with close-up view cropped and adjusted to emphasize the subtle signal abnormalities in this case (#2) show multiple hypointense lesions in the deep cerebral and periventricular white matter. Note slight, hazy "rings" of subtle T1 shortening surrounding many of the lesions (arrows). These lesions are sometimes termed "lesions within a lesion" or "ghostlike rings" of T1 shortening, presumably due to coagulative necrosis in the periphery of chronic MS plaques. The T2WI (#3) and FLAIR scans (#4-7) in this patient show the classic periventricular lesions of MS.', 'history': 'Known MS.', 'imagePoolId': '454e8800-33fe-41df-9e79-25cbb3d8e068', 'name': '3T', 'teachingPoint': None, 'demographics': '39 Years old male'}, {'authors': [{'key': '7cc3ba75-2642-4233-b9f6-0ce69ffe28f3', 'value': 'Sheri L. Harder, MD, FRCPC'}, {'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '7de44e74-cd6f-4f8b-9658-e92b7eca7903', 'description': "This is a typical MR case of severe multiple sclerosis. \nAxial T2 MR images (Figs. 1-3) demonstrate very hypointense bilateral basal ganglia (, Figs. 2-3) atrophy (evidenced by ventricular prominence), and confluent periventricular/subcortical hyperintense plaques in keeping with severe multiple sclerosis.", 'history': None, 'imagePoolId': 'c9f6813d-c72f-401f-887c-0c914467d34b', 'name': 'Severe', 'teachingPoint': None, 'demographics': '42 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '019e8634-5336-4c86-b8e5-1668535b67f8', 'description': 'Axial T2WI with fat saturation (#1) shows swelling and hyperintensity of the left optic nerve (open arrow). Note protrusion of the swollen optic nerve head in the posterior segment (curved arrow). Axial (#2) and coronal (#3) scans performed after contrast administration show intense enhancement of the enlarged left optic nerve (open arrows). Mild enhancement of the right optic nerve is present (arrow). \n\nComment: The majority of patients with optic neuritis eventually develop frank multiple sclerosis.', 'history': 'Young woman with first episode of optic neuritis. Funduscopic examination disclosed a swollen, elevated optic nerve head with blurred margins.', 'imagePoolId': '083ccf16-2fdb-4ae6-88a8-255ca6366036', 'name': 'Optic Neuritis', 'teachingPoint': None}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '0f768485-6b53-4896-a247-ecfa60c89422', 'description': 'Classic MS within both infra- and supratentorial brain.\n\nNumerous MS T2 hyperintense plaques (only a few annotated by arrows) are seen throughout the infra- and supratentorium. A few show a classic perpendicular orientation at the periventricular interface (open arrows). Several demonstrate contrast-enhancement (curved arrows). Note lesions are more conspicuous on FLAIR than T2.', 'history': None, 'imagePoolId': '3e8a21e3-3148-45ff-ab88-72af75fbb13e', 'name': 'Classic', 'teachingPoint': None}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': '5a4b8198-a988-494f-85eb-861a1321481c', 'description': "Figures 1-4 represent typical 3 tesla MR imaging, which exquisitely shows nonenhancing, perivenular, MS lesions oriented perpendicular to the callosomarginal interface .", 'history': None, 'imagePoolId': '880ed8f1-e963-46d6-96d6-344d27ca4f1f', 'name': 'Classic, 3T', 'teachingPoint': '3 tesla has the capability to better image the anatomic relationship of MS plaques.'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'c5bb2f4d-9f63-4ba6-903d-69152d897168', 'description': "Sagittal T1WI (#1) shows multiple hypointense lesions in the deep white matter perpendicular to the lateral ventricle (arrows). Note moderate ventricular and sulcal enlargement for patient's age. Axial T2WIs (#2-4) show round/ovoid lesions in pons, periventricular and deep white matter (arrows). The ovoid configuration along white matter veins (open arrow, #4) represents typical perivenous demyelination or "Dawson fingers." Lesions do not suppress on FLAIR (#5,6).", 'history': 'Longstanding diagnosis of multiple sclerosis.', 'imagePoolId': '908a1f3e-65ff-4730-a2c3-6dd959797e8c', 'name': 'Perivenous demyelination, Dawson fingers', 'teachingPoint': None, 'demographics': '47 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': '33151213-01b2-4542-9105-342e006b3915', 'value': 'H. Ric Harnsberger, MD'}], 'caseVersionId': '6485c987-8966-40d8-82b9-f2b6efcb5519', 'description': "Variant MR case of multiple sclerosis where midbrain, pontine and medullary plaques are prevalent. Axial and sagittal FLAIR images (Figs. 1-8) show extensive suprasellar white matter high-signal lesions typical of severe multiple sclerosis in association with midbrain (, Fig. 4), pontine (, Figs. 2-3, 6), and medullary (F , Figs. 1, 7) plaques. Axial T2 MR images (Figs. 9-12) even more clearly delineate the midbrain (, Fig. 12), pontine (, Figs. 10-12), and medullary (, Fig. 9) plaques.", 'history': 'Patient presents with known multiple sclerosis with recent onset of multiple brainstem associated symptoms.', 'imagePoolId': '17600538-654b-44fe-83d3-71a36871a3ab', 'name': 'Pontine and medullary plaques', 'teachingPoint': None, 'demographics': '26 Years old female'}, {'authors': [{'key': '33151213-01b2-4542-9105-342e006b3915', 'value': 'H. Ric Harnsberger, MD'}], 'caseVersionId': '71128702-fdcf-40c1-9c2a-fa8f81b00236', 'description': "This is a variant case of MS with both supratentorial and posterior fossa white matter lesions.\n\nAxial T2 MR images (Figs. 1-2) reveal a prominent MS plaque in the lateral pons in the region of the root exit zone of the facial nerve. Axial (Fig. 3) and sagittal (Fig. 4) FLAIR images in this area also show this hyperintense lesion . The typical corpus callosum plaque (, Fig. 5) and supratentorial white matter plaque (, Fig. 6) help confirm the imaging diagnosis of MS.", 'history': 'Patient presents with intermittent paresthesias and headache over a 2-year period. Facial spasms were reported in the 6 months before MR imaging completed.', 'imagePoolId': '8c7dcb70-b7e4-45bf-835f-b9249d50da5f', 'name': 'Pontine plaque', 'teachingPoint': 'Patients with multiple sclerosis (MS) rarely present with hemifacial spasm. Even when they, do < 1/2 the time a pontine plaque is visible.', 'demographics': '36 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}, {'key': '8d0c0f3b-13c2-45ac-8116-3725810235ec', 'value': 'Gary L. Hedlund, DO'}], 'caseVersionId': '61481d0e-8c44-4a25-aebb-9e79dd861ed8', 'description': 'Sagittal (#1) and axial (#2) T1WIs show a hypodense mass in the left posterior frontal/anterior parietal area (arrows). PD (#3), T2WIs (#4, 5) and FLAIR (#6) show the mass is hyperintense (arrows). The lesion is well-circumscribed and shows no surrounding edema. On the coronal FLAIR (#6) the lesion shows a peripheral crescent of very hyperintense signal (open arrow). Sagittal (#7), axial (#8, 9) and coronal (#10) T1C+ scans show an incomplete "horseshoe" area of peripheral enhancement (arrows). The nonenhancing part of the mass is adjacent to the cortex.\n\nThis finding is classic for "tumefactive" demyelinating disease, most commonly MS. Frozen section of tissue removed at biopsy was initially read as low grade astrocytoma but subsequent histologic examination disclosed tumefactive demyelination.\n\nMS is unusual in pediatric cases but this is a classic imaging presentation of a solitary "tumefactive" focus of demyelination.', 'history': '10 day history of right foot weakness. ', 'imagePoolId': '3d995088-1e68-4ec3-931f-a10016aeaaba', 'name': 'classic tumefactive', 'teachingPoint': None, 'demographics': '12 Years old female'}, {'authors': [{'key': '33151213-01b2-4542-9105-342e006b3915', 'value': 'H. Ric Harnsberger, MD'}], 'caseVersionId': '9de83fe1-bca7-4bfe-9603-7b69db3df097', 'description': "This is a variant MR case of multiples sclerosis that involves the medulla of the brainstem.\n\nSagittal and axial FLAIR images (Figs. 1-2) show a large dorsal medullary plaque that extends somewhat asymmetrically into the left medullary parenchyma. On axial T2 images (Figs. 3-4) the large plaque is visible (, Fig. 3) as are multiple more inferior and right-sided plaques (, Fig. 4).", 'history': 'Patient presents with history of multiple sclerosis and recent onset of right body numbness and hoarseness.', 'imagePoolId': '62a5e4f3-a927-4530-acc0-2682a7b341d1', 'name': 'Medullary plaques', 'teachingPoint': None, 'demographics': '37 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '3fe0582d-9996-41e9-9319-47b2a7a69f18', 'description': 'Axial T1WI (#1) shows a cystic-appearing mass in the right parietal lobe (open arrow). The lesion (open arrows) is very hyperintense on T2WI (#2) and does not suppress on FLAIR (#3). Note additional white matter lesions in the corpus callosum and frontal lobe (arrows). Axial T1C+ scan (#4) shows a thin rim of enhancement around the lesion (open arrow) and a very subtle enhancing lesion in the corpus callosum (arrow). The coronal T1C+ scan (#5) shows a classic incomplete rim ("horseshoe") of enhancement. The lesion does not restrict on DWI (#6) and is hyperintense on ADC (#7).\n\nClassic multiple sclerosis with a large "tumefactive" demyelinating lesions as well as a few scattered white matter foci near the ventricles and corpus callosum.', 'history': 'Clinically suspicious for MS.', 'imagePoolId': 'c39d220a-c6f3-4b6b-9f62-9da32c86c428', 'name': 'Ring-enhancing', 'teachingPoint': None, 'demographics': '23 Years old female'}, {'authors': [{'key': '40294e37-1dd3-4403-961c-b944b04e62bd', 'value': 'Richard Hewlett, FRCPath'}], 'caseVersionId': 'b9aa851f-7563-4650-8f74-3bdf0d653a87', 'description': 'Variant case of a large solitary demyelinative lesion with brain swelling, in a patient in the sixth decade.\n\nMR images (#1-5) show a near-round mass lesion in the R centrum, with a thin, conspicuous isointense rim on the T2WI (open arrow, #1). This image distinguishes the different external (parenchymal edema) and internal signal (lesion) hyperintensities. The lesion exhibits homogeneous, although discontinuous enhancement after contrast administration (#3, 5); note, moderate suppression of homogeneous lesion contents on the FLAIR sequence (#2). \nFindings considered most suggestive of tumefactive demyelinating disease.\n\nMicroscopy: Intraoperative cytology (#6, H&E x400) shows foamy macrophages (arrow) within a meshwork of axons. Paraffin processed tissue shows complete loss of myelin sheaths with residual myelin debris in macrophages (open arrows, #7, H&E-LB x400), macrophages (#8, CD68 x400), and axons widely separated by inflammatory cells (#9, Neurofilament x400). Morphologic findings consistent with inflammatory demyelination, presumably idiopathic.\n\nComment: Despite the radiologic diagnosis, the patient was subjected to stereotactic biopsy on the suspicion of glioma, yielding a single core of clearly discolored, softened tissue, part of which was squashed for cytologic examination in theater, the rest of the sample being fixed in formalin (UCT frame, needle bore 3 mm). Intra-operative cytology showing hordes of large round cells was initially diagnosed as being likely to represent oligodendroglioma, but intact axons with adherent macrophages most suggestive of demyelination. Histologic proof of macrophages with immunohistochemical (anti-NF antibody) demonstration of axonal conspicuity with interspersed inflammatory infiltrate confirms the diagnosis, and distinguishes demyelination from ischemic injury. \nSolitary demyelinative lesions with associated brain swelling are understandably suspected of being neoplastic, especially on CT, and when appropriate to age. MR features, particularly the pattern of contrast uptake, are now considered characteristic in their way, and are particularly important in the context of pediatric disease. Examination of autopsy material suggests that vascular proliferation/leakage evolves on the lesion periphery, consistent with enhancement, whilst the greatly widened interstitial space forming the demyelinated core is reflected as perturbed water on the FLAIR sequence.', 'history': 'Previously well, normotensive woman. Presented acutely (days) with onset of severe headache followed by L hemiparesis. Apart from weakness, the neurological examination was normal, including funduscopy.', 'imagePoolId': '3d48b4c6-3ebe-48e6-a44b-b7748c6c457a', 'name': 'Solitary, tumefactive', 'teachingPoint': None, 'demographics': '46 Years old female'}, {'authors': [{'key': '7cc3ba75-2642-4233-b9f6-0ce69ffe28f3', 'value': 'Sheri L. Harder, MD, FRCPC'}, {'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'fd0cac8a-dcb8-4f24-b400-d8f125491f7a', 'description': 'Variant CT, MRI and MRS case of tumefactive multiple sclerosis.\n\nAxial NECT (#1) demonstrates extensive right parieto-occipital white matter edema (arrow). Axial FLAIR MR images (#2-5) also demonstrate marked white matter edema (arrow) with extension into the corpus callosum. There is a central hypointense mass (curved arrow) which causes mass effect on the adjacent lateral ventricle and effaces the regional sulci. Axial (#6) and coronal (#7) T1 C+ MR images demonstrate ill-defined enhancement at the margin of the white matter edema (open arrows). Long TE MRS (#8) reveals elevated choline (arrow), decreased NAA (open arrow), and a lactate doublet (curved arrow). These MRS findings could be consistent with acute demyelination and probably reflect a combination of membrane disruption, neuronal loss or dysfunction and inflammation.\n\nComment: Although MR spectroscopy can be helpful in evaluation, the MRS findings in multiple sclerosis are not specific. The spectral pattern of demyelination and low grade neoplasms can be similar, and should therefore be interpreted cautiously.', 'history': None, 'imagePoolId': 'be36e86d-23a4-498a-b614-f6b91c73cb95', 'name': 'Tumefactive, Balo type', 'teachingPoint': None, 'demographics': '8 Years old female'}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '37f29d50-e59d-4bd3-893c-6b86eb49257a', 'description': 'Classic pattern of tumefactive demyelination disease, with partial ring enhancement.\n\nMR study (#1-7) shows large T2 hyperintense lesion in right frontal lobe with mild mass effect, and diffusion restriction along the periphery. Following contrast, there is intense enhancement of a portion of the periphery of the lesion. \n\nComment: This pattern of enhancement makes the diagnosis of demyelinating disease highly likely. Tumor and abscess would all show a complete ring of enhancement.', 'history': 'Presented with weakness. Followup study 2 months later showed marked resolution of the lesion.', 'imagePoolId': '920fd052-87a5-41d5-840e-4a78e3d07715', 'name': 'Tumefactive', 'teachingPoint': None, 'demographics': '65 Years old female'}, {'authors': [{'key': '33151213-01b2-4542-9105-342e006b3915', 'value': 'H. Ric Harnsberger, MD'}], 'caseVersionId': 'fb51616f-a24f-4509-9cbb-f03109749515', 'description': 'Variant MR case of multiple sclerosis (MS) involving both supratentorial white matter and pons-medulla. Lateral pontine plaque is seen associated with enhancing preganglionic segment of the trigeminal nerve.\n\nAxial T2 MR images (#1-4) best delineate the large left lateral pontine MS plaque (curved arrow) with involvement of the root entry zone of CN5 (open arrow, #4). The left middle cerebellar peduncle (open arrow, #2) and bilateral inferior cerebellar peduncles (open arrows, #1) are also affected. Sagittal FLAIR image (#5) shows both the typical supratentorial white matter plaques and the lateral pontine lesion (curved arrow) pointing into the root entry zone (open arrow) and preganglionic segment CN5 (arrow). \n\nEnhance axial (#6-7) and coronal (#8-10) T1 MR images reveal an enhancing preganglionic segment of CN5 on the left (arrow). Interestingly, the root entry zone area of CN5 on the left does not enhance (open arrow) nor does the lateral pontine MS plaque (curved arrow).', 'history': 'Patient presents with previous history of multiple sclerosis and new onset of left trigeminal neuropathy.', 'imagePoolId': '5ce11fd3-2fe9-4356-ab61-259c2a213f78', 'name': 'Pons, medulla plaques; CN5 involvement', 'teachingPoint': None, 'demographics': '48 Years old female'}, {'authors': [{'key': '83f867a5-a183-4396-82ea-384015da4d2f', 'value': 'Gregory L. Katzman, MD, MBA'}], 'caseVersionId': 'd99b0dc5-cbd0-42ce-805c-c629eda08e60', 'description': 'Variant tumefactive MS plaque; biopsy proven.\n\nSagittal T1 imaging reveals a mass-like lesion which is hypointense (image 1, arrows). Axial MRI shows corresponding FLAIR hyperintensity (image 2, open arrows) and irregular, thick, partial ring-enhancement (images 3 & 4, arrows) about this mass-like lesion in a patient not previously diagnosed with MS. Note the lesion crosses the splenium (images 2 & 4, curved arrows).', 'history': 'No prior history of MS. Biopsy confirmed this as a tumefactive MS lesion.', 'imagePoolId': '64fdb676-adb6-4c62-8a45-87bbd2940cd9', 'name': 'Tumefactive', 'teachingPoint': None}], 'caseType': 'variant', 'name': 'VARIANT'} + ## Images @@ -418,7 +589,7 @@ breadcrumbs: ![Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.](images/app.statdx.com_image_thumbnail_276cdca2-d11b-40a4-a1b9-c1e6f9e2755e_annotated_true_size_900_quality_90_1bd1fcce_20251018T122619Z.jpg) *Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.* -![Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.](images/app.statdx.com_image_thumbnail_276cdca2-d11b-40a4-a1b9-c1e6f9e2755e_size_174_quality_85_f12fe06c_20251018T122603Z.jpg) +![Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.](images/app.statdx.com_image_thumbnail_276cdca2-d11b-40a4-a1b9-c1e6f9e2755e_size_174_quality_85_924b074d_20251018T152348Z.jpg) *Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.* ![Sagittal T1 MR in a 14-year-old presenting with gait instability and facial numbness demonstrates T1-hypointense corpus callosum lesions that represent "black holes" of chronic demyelination. This was the initial clinical MS presentation for this patient.](images/app.statdx.com_image_thumbnail_ac69502e-a0c1-4199-995e-2c6e9a7c3086_annotated_true_size_900_quality_90_b2ce42e7_20251018T122619Z.jpg) diff --git a/docs_md/articles/pediatric-multiple-sclerosis-spine_59786b97-2a4d-4706-a6fe-fe2dcd476b5e.md b/docs_md/articles/pediatric-multiple-sclerosis-spine_59786b97-2a4d-4706-a6fe-fe2dcd476b5e.md new file mode 100644 index 0000000..59e6256 --- /dev/null +++ b/docs_md/articles/pediatric-multiple-sclerosis-spine_59786b97-2a4d-4706-a6fe-fe2dcd476b5e.md @@ -0,0 +1,525 @@ +--- +title: "Pediatric Multiple Sclerosis, Spine" +docid: "59786b97-2a4d-4706-a6fe-fe2dcd476b5e" +authors: + - key: "99e1aff7-f42c-43a0-95ae-d89c8551aa01" + value: "Kevin R. Moore, MD" +breadcrumbs: + - + name: "Pediatrics" + slug: "pediatrics" + treeNodeId: "a915965c-d436-44cf-ae65-2f22e7246ea4" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "2b5cea64-a083-489e-ac0c-ec14ba059026" + - + name: "Pediatric Neuroradiology" + slug: "pediatric-neuroradiology" + treeNodeId: "d0eb8f4a-e769-43dd-896c-8c9c27ce8759" + - + name: "Spine" + slug: "spine" + treeNodeId: "b9e72e11-010d-4dd1-a609-2072db6047b2" + - + name: "Infection and Inflammatory Disorders" + slug: "infection-and-inflammatory-disorde-" + treeNodeId: "f2c25be2-4696-46d8-be79-383a6f15e826" + - + name: "Inflammatory and Autoimmune Disorders" + slug: "inflammatory-and-autoimmune-disord-" + treeNodeId: "dbd53cba-1e54-40fc-b62f-f7a8e8e78b53" + - + name: "Pediatric Multiple Sclerosis, Spine" + slug: "pediatric-multiple-sclerosis-spine" + treeNodeId: null +category: "Pediatrics" +cmeTopicId: "54a46ea3-7b26-4525-964c-6aa99ac50564" +documentVersionId: "28aeb597-125a-4f17-91b9-738a90cdd336" +imageCount: 19 +lastUpdated: "02/09/24" +pageDescription: "Pediatric Multiple Sclerosis, Spine" +pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Spine, Infection and Inflammatory Disorders, Inflammatory and Autoimmune Disorders, Pediatric Multiple Sclerosis, Spine" +pageTitle: "Pediatric Multiple Sclerosis, Spine | STATdx" +enhancedTitle: "Pediatric Multiple Sclerosis, Spine" +type: "DX" +references: true +breadcrumbs: + - "Pediatrics" + - "Diagnosis" + - "Pediatric Neuroradiology" + - "Spine" + - "Infection and Inflammatory Disorders" + - "Inflammatory and Autoimmune Disorders" + - "Pediatric Multiple Sclerosis, Spine" +--- +# KEY FACTS + +- ## Terminology + + + - Primary demyelinating disease of CNS with multiple lesions disseminated over time & space + - Concomitant intracranial lesions in periventricular, subcallosal, brainstem, or cerebellar white matter +- ## Imaging + + + - Isolated spinal cord disease (10-20%) + - Cervical segment is most commonly affected + - Dorsolateral aspect of cord + - < 1/2 of cross-sectional area of spinal cord + - < 2 vertebral segments in length + - Sagittal & axial T1WI/T2WI sequences with gadolinium + - Lesions typically oval, peripheral, & asymmetric + - Discrete vs. vague hyperintense lesions + - Enhancement lasts 1-2 months but does not reflect disease progression +- ## Top Differential Diagnoses + + + - Acute disseminated encephalomyelitis + - Neuromyelitis optica spectrum disorders + - Myelin oligodendrocyte glycoprotein antibody disorder + - Idiopathic transverse myelitis + - Intramedullary neoplasm + - Spinal cord infarct +- ## Pathology + + + - Autoimmune, cell-mediated inflammatory process focused on CNS myelin +- ## Clinical Issues + + + - Peak onset: 20-40 years + - Adult females more susceptible than males (1.7:1) + - Multiple clinical presentations + - Relapsing remitting (RR) + - Secondary progressive (SP) + - Primary progressive (PP) + - Progressive relapsing (PR) +- ## Diagnostic Checklist + + + - Imaging findings must be correlated with clinical & laboratory features to confirm diagnosis + +# TERMINOLOGY + +- ## Abbreviations + + + - Spinal cord multiple sclerosis (MS) +- ## Definitions + + + - Primary demyelinating disease of CNS with multiple lesions disseminated over time & space + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Concomitant T2-hyperintense lesions in ≥ 2 of 4 areas of CNS: Periventricular, cortical or juxtacortical, infratentorial, & spinal cord + - ### Location + + + - Lesions solitary or multifocal + - Isolated spinal cord disease in 10-20% + - Cervical segment is most commonly affected (2/3 of cord lesions) + - Lesions frequently in dorsolateral cord + - Does not respect gray-white boundary + - ### Size + + + - < 1/2 of cross-sectional area of spinal cord + - < 2 vertebral segments in length + - ### Morphology + + + - Wedge-shaped on axial MR + - Apex directed centrally +- ## MR Findings + + + - ### T1WI + + + - Iso- to hypointense lesions + - In cord (unlike brain), rarely visible as hypointense + - 30% of brain lesions are dark, "black holes" + - Normal or mild focal cord expansion + - Cord edema + - Resolves after 6-8 weeks + - ### T2WI + + + - Discrete or ill-defined hyperintense lesions + - May be related to extent of demyelination + - Lesions ↑ in size due to edema associated with inflammatory infiltrates → reach max size at 4 weeks + - Slow ↓ in size over 6-8 weeks as edema resolves ± remyelination + - Lesions typically oval, peripheral, & asymmetric + - ### PD/intermediate + + + - Hyperintense lesions + - ### STIR + + + - Improved lesion detection, more artifact + - ### FLAIR + + + - Lower lesion sensitivity compared to STIR + - ### DWI + + + - ↑ mean diffusivity, ↓ fractional anisotropy in plaques & areas without T2 abnormality + - ### T1WI C+ + + + - Variable + - Homogeneous, nodular, or ring enhancement during acute or subacute phase + - Enhancement lasts 1-2 months + - Does not reflect disease progression + - No enhancement during chronic phase + - ### MRS + + + - ↓ N-acetylaspartate level + - ↑ choline levels, even in normal-appearing white matter + - Cord atrophy + - Usually in late stage + - May be seen in early disease course + - Useful for monitoring disease progression & therapeutic efficacy + - Correlates with clinical disability + - fMRI + - Tactile-associated cervical cord fMRI activity ↑ in relapse-onset MS patients + - Overactivation more prominent in patients with more severe locomotor disability + - Suggests abnormality of cord functional properties may be among factors associated with clinical status of MS patient +- ## Nonvascular Interventions + + + - ### Myelography + + + - Nonspecific mild cord expansion +- ## Other Modality Findings + + + - Magnetization transfer (MT) imaging + - ↓ MT ratio in spinal cord + - Better correlation with disability & axonal loss + - ↓ MT ratios in enhancement patterns in which myelin known to be ↓ histopathologically +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - T1WI/T2WI spinal cord MR in sagittal & axial planes with gadolinium + +# DIFFERENTIAL DIAGNOSIS + +- [Acute Disseminated Encephalomyelitis](/document/adem-spine/d2226d10-f582-4cdf-8f32-71718e6c494d) + - Para-/postinfectious immune-mediated inflammatory disorder of spinal cord white matter + - Frequently preceding infection 4-12 days before onset + - Usually monophasic +- [Syringohydromyelia](/document/syringomyelia/fb362df4-5033-4f7c-9f4d-01d701ebab84) + - Central cystic lesion + - CSF intensity on all sequences + - No abnormal enhancement +- [Neuromyelitis Optica Spectrum Disorders](/document/neuromyelitis-optica/11d42d8d-e7bb-4ecf-85ce-d96f0afeb076) + - Autoimmune inflammatory disorder involving myelin of neurons of optic nerves & spinal cord + - Longitudinally extensive cord T2 hyperintensity + optic nerve enhancement + - T2 abnormality involves entire cross section of cord + - Limited brain involvement +- ## Myelin Oligodendrocyte Glycoprotein Antibody Disease + + + - Autoimmune inflammatory disorder involving neuronal myelin of brain, optic nerves, &/or spinal cord + - Often longitudinally extensive + - Predilection for conus +- [Idiopathic Transverse Myelitis](/document/idiopathic-acute-transverse-myelit-/6e82fa47-19b7-45ac-b195-3c21687fa648) + - Longitudinally extensive, > 2/3 of cord cross-sectional area + - Lesion centrally located, variable enhancement + - No associated intracranial lesions + - Diagnosis of exclusion +- [Intramedullary Neoplasm](/document/spinal-cord-astrocytoma/43d5efa2-7a6d-4972-bfc6-c300cc31f9af) + - Cord expansion, peritumoral edema, cystic ± hemorrhagic components + - Entire cross section of spinal cord + - Diffuse or partial enhancement +- [Spinal Cord Infarction](/document/spinal-cord-infarction/5afcaea7-09a0-49b7-8f23-f93734d627fb) + - Sudden onset of symptoms + - Positive diffusion restriction + - Posterior columns typically spared in anterior spinal infarct + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Autoimmune, cell-mediated inflammatory process focused on CNS myelin + - Infectious agents may play primary or secondary role + - Humoral mechanism: Cross reactivity between infectious & self-epitopes + - May be association between MS & altered venous return due to multiple extracranial venous strictures + - Hampered cerebrospinal venous drainage in patients with MS determines complex hemodynamic picture → chronic cerebrospinal venous insufficiency (CCSVI) + - Multiple substitute circles with very high incidence of reflux in both intra-/extracranial venous segments + - Loss of postural regulation of cerebral venous outflow + - Primary progressive (PP) course related to CCSVI pattern differs significantly from relapsing remitting (RR) & secondary progressive (SP) → location of venous obstruction plays role in clinical course + - Obstruction at several levels of azygous vein & of lumbar plexuses + - → venous blood of cord can be drained only in upward direction & is shunted toward venous plexuses inside spine + - ↑ cerebral blood flow/volume & ↓ mean transit time (compared with baseline values before relapse) precede development of plaques + - Susceptibility-weighted imaging → venous blood in cerebral veins of patients with MS is less deoxygenated compared with healthy controls + - Previously, these findings interpreted as sign of local flow disturbances mediated by inflammatory & neurodegenerative processes + - However, may be attributable to recent findings of significant stenoses in extracranial veins draining brain & spinal cord + - ### Genetics + + + - May be inherited as complex multifactorial disorder resulting from interaction of genetic & environmental factors + - Estimated risk to siblings of proband ~ 3.0-5.0%, ↑ to 29.5% if 1 or both parents have MS + - Risk to offspring of person with MS is 2.0-3.0% & higher if both parents have MS + - ### Associated abnormalities + + + - 90% incidence of associated intracranial lesions + - Neurofibromatosis type 1 + - Different serum thyroid hormone & complement C3, C4, & CH50 levels in neuromyelitis optica vs. MS + - Thyroid hormones may play different role in modulating complement activation in MS & neuromyelitis optica + - Focal regions of demyelination of varying size & age scattered throughout CNS white matter +- ## Staging, Grading, & Classification + + + - McDonald criteria (2001, revised in 2005 & 2017) widely used for adult MS diagnosis + - Has also been shown to be useful for pediatric MS diagnosis + - Relies on lesion dissemination in both time & space + - Dissemination in space + - Demonstrated by ≥ 1 T2-hyperintense lesions that are characteristic of MS in ≥ 2 of 4 areas of CNS + - Periventricular, cortical or juxtacortical, infratentorial brain regions, & spinal cord + - Dissemination in time + - Simultaneous presence of gadolinium-enhancing & nonenhancing lesions at any time + - New T2-hyperintense or gadolinium-enhancing lesion on follow-up MR with reference to baseline scan, irrespective of timing of baseline MR +- ## Microscopic Features + + + - Discrete lesions of myelin destruction + - Active lesions with macrophages & lymphocytes + - Chronic lesions with gliosis & cavitation + - Perivascular cuffs of lymphocytes & mononuclear cells + - Involvement of dorsal horns common + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Cord lesions asymptomatic + - Paresthesia + - ### Other signs/symptoms + + + - Muscle weakness, hyperreflexia, gait disturbance + - Bladder/bowel dysfunction + - Surveillance includes periodic neurologic examination to track disease progression & periodic brain & spinal cord MRs to monitor disease activity + - Additional examination techniques → ambulation index, 25-foot timed walk, & 25-foot walk combined with 9-hole peg test & paced serial auditory addition test +- ## Demographics + + + - ### Age + + + - Peak onset: 20-40 years + - Onset < 18 years in 3-5% of MS cases + - ### Sex + + + - Women more susceptible than men (1.7:1) + - Men more likely to have progressive relapsing (PR) & SP MS + - Women more likely to have RR MS + - Both sexes equally affected in PP MS + - ### Ethnicity + + + - Western Europeans have higher risk + - ### Epidemiology + + + - ↑ prevalence farther north from equator + - 30-80 per 100,000 in northern USA & Europe + - 6-14 per 100,000 in southern USA & Europe + - 1 per 100,000 in equatorial regions +- ## Natural History & Prognosis + + + - Benign: 20% + - Complete recovery after 1-2 attacks + - Some may experience progressive MS after 10-15 years + - RR: 25% + - Distinct periods of new or worsening symptoms alternating with complete or partial recovery + - 90% will evolve into progressive MS after 25 years + - SP: 40% + - From RR MS + - Worsening deficits & disabilities + - Incomplete & infrequent remission + - PP: 12% + - Steady progression of symptoms + - Motor dysfunction common + - Primary cord involvement; no distinct attacks + - PR: 3% + - Similar to PP MS + - Distinct periods of exacerbation but without recovery + - High mortality rate +- ## Treatment + + + - Multiple approved medications include interferon, chemotherapy agents, monoclonal antibodies + - Several preparations available of interferon β (interferon β-1b, interferon β-1a) + - Inhibition of immune cells + - Glatiramer acetate + - Synthetic protein similar to myelin protein + - Serves as substrate for T cells + - Mitoxantrone (chemotherapeutic agent) + - Suppression of T lymphocytes & B lymphocytes + - Monoclonal antibodies + - Natalizumab, alemtuzumab, daclizumab, ocrelizumab + - Small-molecule oral agents + - Fingolimod, dimethyl fumarate, teriflunomide + - Supportive therapy + - Symptomatic treatment of pain, muscle spasms, fatigue, depression, sexual/bladder/bowel dysfunction + - Anticholinergics, smooth muscle relaxants + - Physical therapy + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Multiplanar spine contrast MR ± contrast, including STIR MR + - Brain MR, including high-resolution fast spin-echo T2 through corpus callosum + - Gray matter atrophy correlates with disability + - Periventricular, subcallosal, brainstem, or cerebellar white matter lesions suggest MS +- ## Image Interpretation Pearls + + + - Imaging findings must be correlated with clinical & laboratory features to confirm diagnosis + - Acute MS can mimic cord neoplasm + + ec82b351-fbdc-43cf-8899-7d0ffed659d4 + +## References + +# Selected References + +1. [Bower A et al: Radiologically isolated syndrome and the multiple sclerosis prodrome in pediatrics: early features of the spectrum of demyelination. Semin Pediatr Neurol. 46:101053, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37451751%5Bpmid%5D) +1. [Malani Shukla N et al: Demographic features and clinical course of patients with pediatric-onset multiple sclerosis on newer disease-modifying treatments. Pediatr Neurol. 145:125-31, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37348193%5Bpmid%5D) +1. [Fadda G et al: Comparison of spinal cord magnetic resonance imaging features among children with acquired demyelinating syndromes. JAMA Netw Open. 4(10):e2128871, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34643718%5Bpmid%5D) +1. [Ciccarelli O et al: Spinal cord involvement in multiple sclerosis and neuromyelitis optica spectrum disorders. Lancet Neurol. 18(2):185-97, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30663608%5Bpmid%5D) +1. [Thompson AJ et al: Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol. 17(2):162-73, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29275977%5Bpmid%5D) +1. [Bigi S et al: Outcomes after early administration of plasma exchange in pediatric central nervous system inflammatory demyelination. J Child Neurol. 30(7):874-80, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25246301%5Bpmid%5D) +1. [Kearney H et al: Spinal cord grey matter abnormalities are associated with secondary progression and physical disability in multiple sclerosis. J Neurol Neurosurg Psychiatry. 86(6):608-14, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25097217%5Bpmid%5D) +1. [Riederer I et al: Double inversion recovery sequence of the cervical spinal cord in multiple sclerosis and related inflammatory diseases. AJNR Am J Neuroradiol. 36(1):219-25, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25169924%5Bpmid%5D) +1. [Russi AE et al: The meninges: new therapeutic targets for multiple sclerosis. Transl Res. 165(2):255-69, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25241937%5Bpmid%5D) +1. [De Stefano N et al: Spinal cord imaging in multiple sclerosis: filling the gap with the brain. Neurology. 83(15):1306-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25186859%5Bpmid%5D) +1. [Makary MS et al: Tumefactive demyelinating disease with isolated spinal cord involvement. Acta Radiol Short Rep. 3(5):2047981614539324, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25298871%5Bpmid%5D) +1. [Schlaeger R et al: Spinal cord gray matter atrophy correlates with multiple sclerosis disability. Ann Neurol. 76(4):568-80, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25087920%5Bpmid%5D) +1. [Toosy AT et al: Voxel-based cervical spinal cord mapping of diffusion abnormalities in MS-related myelitis. Neurology. 83(15):1321-5, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25186861%5Bpmid%5D) +1. [Simka M et al: Reinterpreting the magnetic resonance signs of hemodynamic impairment in the brains of multiple sclerosis patients from the perspective of a recent discovery of outflow block in the extracranial veins. J Neurosci Res. 88(9):1841-5, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20127806%5Bpmid%5D) +1. [Tallantyre EC et al: Clinico-pathological evidence that axonal loss underlies disability in progressive multiple sclerosis. Mult Scler. 16(4):406-11, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20215480%5Bpmid%5D) +1. [Valsasina P et al: Cervical cord functional MRI changes in relapse-onset MS patients. J Neurol Neurosurg Psychiatry. 81(4):405-8, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19965858%5Bpmid%5D) +1. [Zamboni P et al: Chronic cerebrospinal venous insufficiency in patients with multiple sclerosis. J Neurol Neurosurg Psychiatry. 80(4):392-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19060024%5Bpmid%5D) +1. [Zhang B et al: Correlation between serum thyroxine and complements in patients with multiple sclerosis and neuromyelitis optica. Neuro Endocrinol Lett. 29(2):256-60, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18404143%5Bpmid%5D) +1. [Yukawa Y et al: MR T2 image classification in cervical compression myelopathy: predictor of surgical outcomes. Spine (Phila Pa 1976). 32(15):1675-8; discussion 1679, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17621217%5Bpmid%5D) +1. [Stüve O, Oksenberg J. Multiple sclerosis overview. 1993-, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=20301492%5Bpmid%5D) +1. [International Working Group for Treatment Optimization in MS: Treatment optimization in multiple sclerosis: report of an international consensus meeting. Eur J Neurol. 11(1):43-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14692887%5Bpmid%5D) +1. [Pretorius PM et al: The role of MRI in the diagnosis of MS. Clin Radiol. 58(6):434-48, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12788312%5Bpmid%5D) +1. [Filippi M et al: Overview of diffusion-weighted magnetic resonance studies in multiple sclerosis. J Neurol Sci. 186 Suppl 1:S37-43, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11334988%5Bpmid%5D) +1. [Institute of Medicine (US) Committee on Multiple Sclerosis: current status and strategies for the future et al: 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=25057543%5Bpmid%5D) +1. [Poser CM et al: Diagnostic criteria for multiple sclerosis. Clin Neurol Neurosurg. 103(1):1-11, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11311469%5Bpmid%5D) +1. [Steiner I et al: Infection and the etiology and pathogenesis of multiple sclerosis. Curr Neurol Neurosci Rep. 1(3):271-6, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11898529%5Bpmid%5D) +1. [Bastianello S et al: MRI of spinal cord in MS. J Neurovirol. 6 Suppl 2:S130-3, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10871800%5Bpmid%5D) +1. [Hickman SJ et al: Imaging of the spine in multiple sclerosis. Neuroimaging Clin N Am. 10(4):689-704 ,viii, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11359719%5Bpmid%5D) +1. [Simon JH: Brain and spinal cord atrophy in multiple sclerosis. Neuroimaging Clin N Am. 10(4):753-70 ,ix, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11359723%5Bpmid%5D) +1. [Simon JH: The contribution of spinal cord MRI to the diagnosis and differential diagnosis of multiple sclerosis. J Neurol Sci. 172 Suppl 1:S32-5, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10606803%5Bpmid%5D) +1. [van Waesberghe JH et al: Magnetization transfer imaging of the spinal cord and the optic nerve in patients with multiple sclerosis. Neurology. 53(5 Suppl 3):S46-8, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10496211%5Bpmid%5D) +1. [McFarland HF: The lesion in multiple sclerosis: clinical, pathological, and magnetic resonance imaging considerations. J Neurol Neurosurg Psychiatry. 64 Suppl 1:S26-30, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9647281%5Bpmid%5D) +1. [Campi A et al: Acute transverse myelopathy: spinal and cranial MR study with clinical follow-up. AJNR Am J Neuroradiol. 16(1):115-23, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7900579%5Bpmid%5D) +1. [Miller DH: Magnetic resonance imaging and spectroscopy in multiple sclerosis. Curr Opin Neurol. 8(3):210-5, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7551120%5Bpmid%5D) +1. [Tartaglino LM et al: Multiple sclerosis in the spinal cord: MR appearance and correlation with clinical parameters. Radiology. 195(3):725-32, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7754002%5Bpmid%5D) +1. [Jeffery DR et al: Transverse myelitis. Retrospective analysis of 33 cases, with differentiation of cases associated with multiple sclerosis and parainfectious events. Arch Neurol. 50(5):532-5, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8489410%5Bpmid%5D) +1. [Thomas DJ et al: Magnetic resonance imaging of spinal cord in multiple sclerosis by fluid-attenuated inversion recovery. Lancet. 341(8845):593-4, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8094830%5Bpmid%5D) +1. [Maravilla KR et al: Magnetic resonance demonstration of multiple sclerosis plaques in the cervical cord. AJR Am J Roentgenol. 144(2):381-5, 1985](http://www.ncbi.nlm.nih.gov/pubmed/?term=3871287%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal graphic depicts multiple sclerosis (MS) demyelinating plaques within the cervical spinal cord. Lesions are focal and < 2 vertebral bodies in length, typical of MS.](images/app.statdx.com_image_thumbnail_cb4ae1bc-6382-4116-850f-ff27cb4cbaed_annotated_true_size_900_quality_90_8496d08e_20251018T152352Z.jpg) +*Sagittal graphic depicts multiple sclerosis (MS) demyelinating plaques within the cervical spinal cord. Lesions are focal and < 2 vertebral bodies in length, typical of MS.* + +![Sagittal graphic depicts multiple sclerosis (MS) demyelinating plaques within the cervical spinal cord. Lesions are focal and < 2 vertebral bodies in length, typical of MS.](images/app.statdx.com_image_thumbnail_cb4ae1bc-6382-4116-850f-ff27cb4cbaed_size_174_quality_85_b9226ca2_20251018T152348Z.jpg) +*Sagittal graphic depicts multiple sclerosis (MS) demyelinating plaques within the cervical spinal cord. Lesions are focal and < 2 vertebral bodies in length, typical of MS.* + +![Sagittal T2WI MR (left) demonstrates a solitary active MS plaque at the C6-C7 level with focal T2 hyperintensity but without significant cord enlargement. Sagittal T1WI C+ FS MR (right) confirms ring enhancement of the focal lesion, consistent with an active MS plaque.](images/app.statdx.com_image_thumbnail_c31c5226-cb26-43a1-8576-48d04418c273_annotated_true_size_900_quality_90_8ee82cd5_20251018T152352Z.jpg) +*Sagittal T2WI MR (left) demonstrates a solitary active MS plaque at the C6-C7 level with focal T2 hyperintensity but without significant cord enlargement. Sagittal T1WI C+ FS MR (right) confirms ring enhancement of the focal lesion, consistent with an active MS plaque.* + +![Sagittal T2WI (left), PD (middle), and STIR (right) MR images show multiple short-segment MS plaques within the thoracic spinal cord . Note the relatively improved conspicuity of the plaques on PD and STIR relative to the routine T2 sequence.](images/app.statdx.com_image_thumbnail_31cc6d5c-7839-48b3-b51a-fc81328fda8b_annotated_true_size_900_quality_90_7373ff66_20251018T152352Z.jpg) +*Sagittal T2WI (left), PD (middle), and STIR (right) MR images show multiple short-segment MS plaques within the thoracic spinal cord . Note the relatively improved conspicuity of the plaques on PD and STIR relative to the routine T2 sequence.* + +![Sagittal STIR (left), T2WI (middle), and T1WI C+ FS (right) MR images of the thoracic spine show multiple short-segment foci of T2 hyperintensity in a different patient with MS. Multiple lesions show solid enhancement.](images/app.statdx.com_image_thumbnail_41b3ffe4-cf4a-4907-883c-a1fd68e31818_annotated_true_size_900_quality_90_5e88242a_20251018T152352Z.jpg) +*Sagittal STIR (left), T2WI (middle), and T1WI C+ FS (right) MR images of the thoracic spine show multiple short-segment foci of T2 hyperintensity in a different patient with MS. Multiple lesions show solid enhancement.* + + +### Additional Images + +![Sagittal T2WI MR in a patient with MS and characteristic brain lesions (not shown) reveals a focal lesion centered at C7 with minimal if any cord enlargement.](images/app.statdx.com_image_thumbnail_67cd3d94-6fe9-49f5-b800-d416a2f69352_annotated_true_size_900_quality_90_9aa1f9ea_20251018T152352Z.jpg) +*Sagittal T2WI MR in a patient with MS and characteristic brain lesions (not shown) reveals a focal lesion centered at C7 with minimal if any cord enlargement.* + +![Axial T2WI MR in the same patient reveals a lesion in the left hemicord that is focal and does not involve the entire cord diameter, features favoring MS.](images/app.statdx.com_image_thumbnail_84284388-9d82-4c35-bbad-b3f0c617e209_annotated_true_size_900_quality_90_8ace282c_20251018T152352Z.jpg) +*Axial T2WI MR in the same patient reveals a lesion in the left hemicord that is focal and does not involve the entire cord diameter, features favoring MS.* + +![Sagittal T2WI (left) and T1WI C+ FS (right) MR images show several T2-hyperintense foci in the cervical cord in this patient with MS. Two of the lesions enhance, reflecting active demyelination .](images/app.statdx.com_image_thumbnail_85483f4f-a34e-47f3-907e-a993c3f55aaa_annotated_true_size_900_quality_90_db4a6a37_20251018T152352Z.jpg) +*Sagittal T2WI (left) and T1WI C+ FS (right) MR images show several T2-hyperintense foci in the cervical cord in this patient with MS. Two of the lesions enhance, reflecting active demyelination .* + +![Sagittal T2WI (left) and T1WI C+ (right) MR images show active enhancing plaque at the C2 level with both focal, well-defined (enhancing) T2 focus and a small amount of surrounding nonenhancing edema .](images/app.statdx.com_image_thumbnail_3c1da632-78ca-4603-a25b-eb368bd85c4a_annotated_true_size_900_quality_90_fadc4036_20251018T152352Z.jpg) +*Sagittal T2WI (left) and T1WI C+ (right) MR images show active enhancing plaque at the C2 level with both focal, well-defined (enhancing) T2 focus and a small amount of surrounding nonenhancing edema .* + +![Sagittal T2WI MR of the cervical cord shows a more discrete demyelinating focus at C3-C4.](5bdf1734-0516-4201-8b49-b36d8ab7f043) +*Sagittal T2WI MR of the cervical cord shows a more discrete demyelinating focus at C3-C4.* + +![Axial T2WI MR of the cervical cord in a different patient shows a poorly defined, wedge-shaped, mildly hyperintense plaque within the right lateral aspect of the cord.](b9d34c09-e1fd-4a0b-b616-d899db88cd10) +*Axial T2WI MR of the cervical cord in a different patient shows a poorly defined, wedge-shaped, mildly hyperintense plaque within the right lateral aspect of the cord.* + +![Axial T1WI C+ MR with fat suppression of the cervical cord in a different patient shows right peripheral nodular enhancement.](1d72e7e4-afc1-4b6e-bb55-107df83d15c0) +*Axial T1WI C+ MR with fat suppression of the cervical cord in a different patient shows right peripheral nodular enhancement.* + +![Sagittal T2WI MR of the cervical cord shows an ill-defined, hyperintense intramedullary lesion at C5-C6.](599b6814-a970-4c6c-a5a1-b6f4937037ac) +*Sagittal T2WI MR of the cervical cord shows an ill-defined, hyperintense intramedullary lesion at C5-C6.* + +![Sagittal T2WI MR of the cervical spinal cord demonstrates multiple T2-hyperintense foci , some well defined and others ill defined. The multiplicity of lesions and lack of edema or significant cord expansion is typical for demyelinating disease.](c7ebc209-64e3-431b-b02f-c133d763dfd1) +*Sagittal T2WI MR of the cervical spinal cord demonstrates multiple T2-hyperintense foci , some well defined and others ill defined. The multiplicity of lesions and lack of edema or significant cord expansion is typical for demyelinating disease.* + +![Sagittal T1WI C+ MR shows multiple enhancing demyelinating lesions within the cervical spinal cord. Enhancement varies from focal to ill defined . The enhancement pattern changes with evolution of inflammation.](89d4add6-eae5-4f1e-9e67-1bf79b4235aa) +*Sagittal T1WI C+ MR shows multiple enhancing demyelinating lesions within the cervical spinal cord. Enhancement varies from focal to ill defined . The enhancement pattern changes with evolution of inflammation.* + +![T1WI C+ MR (sagittal on top, axial on bottom) illustrates an incomplete rim-enhancing lesion in the dorsal cervical cord at the C3-C4 level. A 2nd small enhancing focus is noted in the ventral cord at the C6 level .](dbc296db-6e58-4d47-b30c-4e8d1e65903c) +*T1WI C+ MR (sagittal on top, axial on bottom) illustrates an incomplete rim-enhancing lesion in the dorsal cervical cord at the C3-C4 level. A 2nd small enhancing focus is noted in the ventral cord at the C6 level .* + +![Sagittal PD FSE MR of the cervical spinal cord demonstrates characteristic ovoid hyperintense intramedullary demyelinating lesions without significant cord expansion.](1eb015e3-b95e-46b0-84c6-791e2f1cd2be) +*Sagittal PD FSE MR of the cervical spinal cord demonstrates characteristic ovoid hyperintense intramedullary demyelinating lesions without significant cord expansion.* + +![Axial T1WI C+ MR of the cervical spinal cord depicts focal ring enhancement within an active MS demyelinating lesion.](7fd64d38-5f6e-4d25-97d4-e7a3d9378652) +*Axial T1WI C+ MR of the cervical spinal cord depicts focal ring enhancement within an active MS demyelinating lesion.* + +![Sagittal STIR MR shows a focal hyperintense demyelinating plaque within the thoracic cord without significant cord expansion. STIR MR is more sensitive for lesion depiction than T2WI MR at the price of more artifacts.](b1b6855b-8a2b-441d-90fd-919a3e24778c) +*Sagittal STIR MR shows a focal hyperintense demyelinating plaque within the thoracic cord without significant cord expansion. STIR MR is more sensitive for lesion depiction than T2WI MR at the price of more artifacts.* + +![Sagittal high-resolution GRE MR of the thoracic cord shows multiple areas of ↑ signal in this patient with MS. All lesions are ≤ 2 vertebral bodies in length, typical for MS.](9e0b4310-22ec-4437-b08b-182518fd85dd) +*Sagittal high-resolution GRE MR of the thoracic cord shows multiple areas of ↑ signal in this patient with MS. All lesions are ≤ 2 vertebral bodies in length, typical for MS.* + diff --git a/docs_md/articles/periventricular-enhancing-lesions_0edb9603-ea97-4f3b-be82-21d53c42be32.md b/docs_md/articles/periventricular-enhancing-lesions_0edb9603-ea97-4f3b-be82-21d53c42be32.md new file mode 100644 index 0000000..072b878 --- /dev/null +++ b/docs_md/articles/periventricular-enhancing-lesions_0edb9603-ea97-4f3b-be82-21d53c42be32.md @@ -0,0 +1,409 @@ +--- +title: "Periventricular Enhancing Lesions" +docid: "0edb9603-ea97-4f3b-be82-21d53c42be32" +authors: + - key: "1fa14dfd-71ea-4960-908e-e720313bc63a" + value: "Santhosh Gaddikeri, MD" + - key: "30ce27b2-237f-4aff-a88f-65ead356335b" + value: "Marinos Kontzialis, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60" + - + name: "Ventricles, Periventricular Regions" + slug: "ventricles-periventricular-regions" + treeNodeId: "353c434a-a6fc-4ef1-8786-d30a1988a4dc" + - + name: "Generic Imaging Patterns" + slug: "generic-imaging-patterns" + treeNodeId: "969c31a2-ef56-4fc3-9125-05857cf9aac3" + - + name: "Periventricular Enhancing Lesions" + slug: "periventricular-enhancing-lesions" + treeNodeId: null +category: "Brain" +documentVersionId: "d7a3032c-c0c2-4516-837e-59885e1d3d24" +imageCount: 54 +lastUpdated: "02/14/23" +pageDescription: "Periventricular Enhancing Lesions" +pageKeywords: "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Periventricular Enhancing Lesions" +pageTitle: "Periventricular Enhancing Lesions | STATdx" +enhancedTitle: "Periventricular Enhancing Lesions" +type: "DDX" +references: true +breadcrumbs: + - "Brain" + - "Differential Diagnosis" + - "Ventricles, Periventricular Regions" + - "Generic Imaging Patterns" + - "Periventricular Enhancing Lesions" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - DWI MR may help differentiate various etiologies +- ## Helpful Clues for Common Diagnoses + + + - **Multiple Sclerosis** + - Most common acquired CNS autoimmune demyelinating disease + - Callososeptal interface involvement + - Subcallosal striations + - Ovoid lesions radiating from ventricular surface of corpus callosum (CC) into pericallosal white matter (WM) corresponding to perivenular inflammation + - Juxtacortical lesions + - Acute demyelinating lesions = may show restricted diffusion, enhancement (nodular, ring, or incomplete rim) + - **Tumefactive demyelination** + - Demyelinating lesions > 2 cm; may be mistaken for tumor when edema & mass effect + - May occur in neuroinflammatory conditions, including multiple sclerosis (MS) & ADEM + - Most closely linked with MS often at time of 1st presentation + - Look for characteristic incomplete ring of enhancement + - **ADEM** + - Monophasic demyelination triggered by febrile illness or vaccination; impaired consciousness + - Multifocal WM + deep gray matter (GM) lesions; periventricular + CC lesions larger than in MS + - Complete or partial resolution of lesions without development of new lesions + - Enhancement typical + - **Glioblastoma, IDH-Wildtype** + - Most common primary intracranial neoplasm + - Rapid enlargement, thick irregular enhancement, necrotic core + - Heterogeneous, hyperintense T2 FLAIR mass + surrounding vasogenic edema/tumor infiltration + - Tumor extends beyond visible signal changes + - **Lymphoma, Primary CNS** + - Immunocompetent: May be T2 hypointense related to high nuclear:cytoplasmic ratio, solid enhancement + - Immunocompromised: May be heterogeneous from hemorrhage, necrosis, rim enhancement + - Often crosses CC + - Mild surrounding edema is typical + - Enhancing lesion(s) within basal ganglia (BG) &/or periventricular WM + - **Metastases, Parenchymal** + - Round enhancing lesion(s) at GM-WM interface + - May be punctate to massive with variable surrounding edema, mass effect + - 50% solitary + - Primary tumor often known +- ## Helpful Clues for Less Common Diagnoses + + + - **Abscess** + - Central restricted diffusion, enhancing T2-hypointense rim, surrounding vasogenic edema + - **Septic Emboli** + - Scattered, small juxtacortical hyperintensities + - Develop into small, ring-enhancing microabscesses + - **Toxoplasmosis, Acquired** + - Multiple WM & BG ring-enhancing masses + - May show target sign + - DWI restriction variable + - Typically seen in HIV patients + - **Neurocysticercosis** + - Vesicular phase: Small 10-mm cysts with central scolex, no edema, follows CSF + - Colloidal phase: Cyst may enlarge, may be hyperintense to CSF, surrounding edema, enhancement + - Granular nodular & calcified phase: Cyst retracts, wall thickens, edema resolves, calcifies + - **Germinoma** + - Enhancing midline mass (pineal, suprasellar) typical + - Occurs in BG or thalamus 5-10% + - Hyperdense on CT + - CSF spread common + - **Vasculitis** + - Irregularities, stenosis, & vascular occlusions + - Multifocal cortical/subcortical & BG T2 hyperintensities; DWI restriction if acute + - Patchy enhancement typical + - High-resolution MR may show vessel wall enhancement + - **Lyme Disease** + - MS-like WM lesions (may enhance) + - ± multiple enhancing cranial nerves + - ± cauda equina, meningeal enhancement + - **Ependymoma** + - Majority (2/3) infratentorial + - 4th ventricle in child + - ± extension through lateral recesses into cerebellopontine angle cisterns + - 1/3 are supratentorial + - Most are extraventricular + - Typically periventricular WM + - Heterogeneous enhancing mass + - 50% are calcified + - Cysts, hemorrhage common + - **Neurosarcoid** + - Periventricular T2-hyperintense lesions + - Enhancing parenchymal lesions + - Leptomeningeal disease + - Hypothalamus/pituitary involvement + - Cranial nerve involvement + - Dural involvement + - Vasculitis + - **Neuromyelitis Optica Spectrum Disorders** + - Recurrent, often bilateral optic neuritis &/or longitudinally extensive cord lesions > 3 vertebral segments + - Periventricular/periependymal hyperintensities following distribution of aquaporin-4 + - Patchy enhancement with blurred margins (cloud-like enhancement) in cerebral lesions + - "Pencil-thin" ependymal enhancement + - Rarely, well-marginated nodular enhancement or meningeal enhancement +- ## Helpful Clues for Rare Diagnoses + + + - **Leukemia** + - Typically involves dura + - May see along penetrating vessels or ependyma + - Enhancing mass(es) in child + - **Susac Syndrome** + - Triad of encephalopathy, branch retinal artery occlusions, hearing loss + - Endotheliopathy of cerebral precapillary arterioles + - Characteristic multifocal lesions involving central fibers of CC = small infarcts + - **Alexander Disease** + - Diffuse symmetric bifrontal WM signal abnormality & enhancement + - Near-total lack of myelin + - Infant with macrocephaly, seizures, developmental delay + - **Ependymal/Subependymal Veins (Mimic)** + - Normal periventricular venous structures may become engorged with various pathologies + - Venous thrombosis, vascular malformations (arteriovenous malformation, developmental venous anomaly) + - **CLIPPERS** + - Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids + - Recently described inflammatory CNS disorder + - Predominant brainstem & pons involvement + - Punctate perivascular enhancement with surrounding T2 FLAIR hyperintensities + - Lesions typically clustered in pons, & adjacent rhombencephalic structures may extend into spinal cord & supratentorial GM & WM + - **Immune Reconstitution Inflammatory Syndrome (IRIS)** + - Atypical/worsening imaging appearance of infection in HIV/AIDS following initiation of HAART + - Immune reconstitution leading to inflammatory response to preexisting infection + - Pathogens: JC virus (progressive multifocal leukoencephalopathy), TB, CMV, *Cryptococcus*, others + - **Behçet Disease** + - Vasculitis with venous predominance + - Asymmetric mesodiencephalic junction hyperintense lesion with cranial & caudal extension ± enhancement + - Midbrain involvement with sparing of red nucleus suggestive of vasogenic edema + - Findings likely secondary to venous thrombosis with reversible edema + - Associated enhancement in acute lesions may mimic tumor + +## References + +# Selected References + +1. [Clarke L et al: Magnetic resonance imaging in neuromyelitis optica spectrum disorder. Clin Exp Immunol. 206(3):251-65, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34080180%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T1 C+ MR demonstrates multiple lesions with nodular, broken ring, and curvilinear enhancement in juxtacortical , deep and periventricular white matter (WM), consistent with active demyelinating lesions in a patient with multiple sclerosis.](images/app.statdx.com_image_thumbnail_cec9f7fa-cc33-4fb0-bc41-7c94d60c9f66_annotated_true_size_900_quality_90_1a0a2332_20251018T165158Z.jpg) +**Multiple Sclerosis** +*Axial T1 C+ MR demonstrates multiple lesions with nodular, broken ring, and curvilinear enhancement in juxtacortical , deep and periventricular white matter (WM), consistent with active demyelinating lesions in a patient with multiple sclerosis.* + +![Axial T1 C+ 3D SPGR MR demonstrates a large, enhancing lesion involving bifrontal periventricular WM and corpus callosum . Biopsy revealed demyelination. There is encephalomalacia in the bilateral parietal lobes .](images/app.statdx.com_image_thumbnail_96da31e5-9034-45be-ba03-4e0dce782335_annotated_true_size_900_quality_90_2e9667a2_20251018T165158Z.jpg) +**Tumefactive Demyelination** +*Axial T1 C+ 3D SPGR MR demonstrates a large, enhancing lesion involving bifrontal periventricular WM and corpus callosum . Biopsy revealed demyelination. There is encephalomalacia in the bilateral parietal lobes .* + +![Coronal T1 C+ MR shows multiple patchy/confluent, avidly enhancing lesions involving gray matter , WM , and along periventricular region , findings consistent with ADEM. Patient had upper respiratory tract infection 1 week before neurologic symptom onset.](images/app.statdx.com_image_thumbnail_2fe022b0-01ed-42cc-85b2-bc1b1b4ea50a_annotated_true_size_900_quality_90_9bb597d6_20251018T165158Z.jpg) +**ADEM** +*Coronal T1 C+ MR shows multiple patchy/confluent, avidly enhancing lesions involving gray matter , WM , and along periventricular region , findings consistent with ADEM. Patient had upper respiratory tract infection 1 week before neurologic symptom onset.* + +![Coronal T1 C+ SPGR MR shows a necrotic enhancing mass in the right temporal lobe periventricular region . There is additional nodular foci along the septum and ependyma of of the 3rd ventricle . Biopsy revealed glioblastoma, IDH-wildtype.](d8681e69-3087-48d8-b436-b2d58e5188be) +**Glioblastoma, IDH-Wildtype** +*Coronal T1 C+ SPGR MR shows a necrotic enhancing mass in the right temporal lobe periventricular region . There is additional nodular foci along the septum and ependyma of of the 3rd ventricle . Biopsy revealed glioblastoma, IDH-wildtype.* + +![Axial T1 C+ SPGR MR demonstrates a large, avidly enhancing mass in the right cerebellum adjacent to the 4th ventricle. Biopsy revealed lymphoma. Note mild mass effect on the 4th ventricle and surrounding hypointensity due to edema .](99c1f5b7-c21a-4f02-be42-d35376dfe337) +**Lymphoma, Primary CNS** +*Axial T1 C+ SPGR MR demonstrates a large, avidly enhancing mass in the right cerebellum adjacent to the 4th ventricle. Biopsy revealed lymphoma. Note mild mass effect on the 4th ventricle and surrounding hypointensity due to edema .* + +![Axial CECT shows rim-enhancing, periventricular metastatic lesions adjacent to the right ventricular atrium in a patient with lung carcinoma. An additional enhancing metastasis involves the left choroid plexus.](60dd2571-7af1-4d3e-978c-c7daac3c4bac) +**Metastases, Parenchymal** +*Axial CECT shows rim-enhancing, periventricular metastatic lesions adjacent to the right ventricular atrium in a patient with lung carcinoma. An additional enhancing metastasis involves the left choroid plexus.* + +![Axial T1 C+ MR demonstrates a rim-enhancing lesion in the left occipital lobe , which showed restricted diffusion and was consistent with an abscess. The abscess shows intraventricular rupture with loculated intraventricular enhancement .](3502bc4b-4b2a-4b7a-8b54-51bb7ea2033e) +**Abscess** +*Axial T1 C+ MR demonstrates a rim-enhancing lesion in the left occipital lobe , which showed restricted diffusion and was consistent with an abscess. The abscess shows intraventricular rupture with loculated intraventricular enhancement .* + +![Coronal T1 C+ SPGR MR demonstrates a right ring-enhancing lesion with an eccentric target sign , consistent with toxoplasmosis (acquired) in this patient with HIV/AIDS. Note surrounding edema and mass effect .](c5cd9ab8-7660-4df6-b178-7b01fe72c274) +**Toxoplasmosis, Acquired** +*Coronal T1 C+ SPGR MR demonstrates a right ring-enhancing lesion with an eccentric target sign , consistent with toxoplasmosis (acquired) in this patient with HIV/AIDS. Note surrounding edema and mass effect .* + +![Axial T1 C+ MR demonstrates linear periventricular and perivascular enhancement along the medullary veins. Meningeal biopsy revealed primary CNS vasculitis.](fbd95e5a-166a-4d24-a58f-cd57d4c192b9) +**Vasculitis** +*Axial T1 C+ MR demonstrates linear periventricular and perivascular enhancement along the medullary veins. Meningeal biopsy revealed primary CNS vasculitis.* + +![Axial T1 C+ MR demonstrates periventricular , ependymal , and perivascular enhancement . Biopsy of enlarged lung hilar lymph nodes was consistent with sarcoidosis.](4f7a0402-398c-48e9-a6d5-afc9c1039288) +**Neurosarcoid** +*Axial T1 C+ MR demonstrates periventricular , ependymal , and perivascular enhancement . Biopsy of enlarged lung hilar lymph nodes was consistent with sarcoidosis.* + +![Axial T1 C+ 3D SPGR MR demonstrates periventricular edema and enhancement around the 3rd ventricle. CSF was positive for antiaquaporin-4 IgG. These findings are consistent with neuromyelitis optica.](59ecb478-a253-40b0-b9ec-892043208caf) +**Neuromyelitis Optica Spectrum Disorders** +*Axial T1 C+ 3D SPGR MR demonstrates periventricular edema and enhancement around the 3rd ventricle. CSF was positive for antiaquaporin-4 IgG. These findings are consistent with neuromyelitis optica.* + +![Axial T1 C+ MR in a 9-year-old boy with acute myeloid leukemia demonstrates multiple foci of nodular enhancement in the periventricular and perivascular regions, suggesting leukemic infiltration.](c811fe46-bd19-4426-b9fb-1345ab0eb3e4) +**Leukemia** +*Axial T1 C+ MR in a 9-year-old boy with acute myeloid leukemia demonstrates multiple foci of nodular enhancement in the periventricular and perivascular regions, suggesting leukemic infiltration.* + +![Axial T1 C+ MR shows multiple foci of perivascular enhancement in the pons and bilateral middle cerebellar peduncles around the 4th ventricle. This completely resolved after steroids (not shown), suggestive of CLIPPERS.](546f7c81-009c-4d06-89eb-8803ea8e6901) +**CLIPPERS** +*Axial T1 C+ MR shows multiple foci of perivascular enhancement in the pons and bilateral middle cerebellar peduncles around the 4th ventricle. This completely resolved after steroids (not shown), suggestive of CLIPPERS.* + +![Axial T1 C+ MR shows a hypointense lesion in the right temporooccipital region with periventricular extension with marginal enhancement . In this patient with HIV/AIDS and progressive multifocal leucoencephalopathy (PML) (on HAART therapy), this is suggestive of PML-IRIS.](2957aa33-fada-47a9-b65b-fcf4730e1b2a) +**Immune Reconstitution Inflammatory Syndrome (IRIS)** +*Axial T1 C+ MR shows a hypointense lesion in the right temporooccipital region with periventricular extension with marginal enhancement . In this patient with HIV/AIDS and progressive multifocal leucoencephalopathy (PML) (on HAART therapy), this is suggestive of PML-IRIS.* + + +### Additional Images + +![Axial T1 C+ MR shows a characteristic tumefactive multiple sclerosis (MS) plaque with irregular, thick partial ring enhancement and mass effect . These lesions may cross the corpus callosum and mimic tumors.](images/app.statdx.com_image_thumbnail_8c4f66bc-64d0-4be3-936d-557116cd6718_annotated_true_size_900_quality_90_c8ffa373_20251018T165158Z.jpg) +**Multiple Sclerosis** +*Axial T1 C+ MR shows a characteristic tumefactive multiple sclerosis (MS) plaque with irregular, thick partial ring enhancement and mass effect . These lesions may cross the corpus callosum and mimic tumors.* + +![Axial T1 C+ MR shows numerous enhancing MS plaques in the periventricular and subcortical WM. Note the typical lack of mass effect. ADEM and Lyme disease may be identical.](images/app.statdx.com_image_thumbnail_6ec86b92-b92a-4ce9-9fa6-e489ec38ab24_annotated_true_size_900_quality_90_456d38ed_20251018T165158Z.jpg) +**Multiple Sclerosis** +*Axial T1 C+ MR shows numerous enhancing MS plaques in the periventricular and subcortical WM. Note the typical lack of mass effect. ADEM and Lyme disease may be identical.* + +![Coronal T1 C+ MR shows classic incomplete ring-enhancing focus in the right frontal WM with minimal mass effect. No other enhancing foci were seen.](images/app.statdx.com_image_thumbnail_67f3d4fe-0f8e-4ceb-9996-304fbc6b4f2e_annotated_true_size_900_quality_90_af6683b6_20251018T165158Z.jpg) +**Multiple Sclerosis** +*Coronal T1 C+ MR shows classic incomplete ring-enhancing focus in the right frontal WM with minimal mass effect. No other enhancing foci were seen.* + +![Axial T1 C+ MR shows characteristic imaging findings of glioblastoma that include a large, heterogeneous mass with thick, aggressive rim enhancement and central necrosis. There is mass effect and effacement of the anterior lateral ventricles.](e9c87bda-3609-4850-9608-6e3eaf0ba198) +**Glioblastoma, IDH-Wildtype** +*Axial T1 C+ MR shows characteristic imaging findings of glioblastoma that include a large, heterogeneous mass with thick, aggressive rim enhancement and central necrosis. There is mass effect and effacement of the anterior lateral ventricles.* + +![Axial T1 C+ FS MR shows a large, heterogeneously enhancing occipital lobe mass with central necrosis. Note extension across the splenium of the corpus callosum , characteristic of glioblastoma multiforme.](807e9108-c225-4863-96f0-33581213538a) +**Glioblastoma, IDH-Wildtype** +*Axial T1 C+ FS MR shows a large, heterogeneously enhancing occipital lobe mass with central necrosis. Note extension across the splenium of the corpus callosum , characteristic of glioblastoma multiforme.* + +![Axial T1 C+ FS MR shows multifocal enhancement in periventricular WM, fornices , and septum pellucidum . Noncontiguous regional involvement (satellite lesions) is a less common pattern in glioblastoma multiforme.](c7715f85-b899-4c9c-a604-d61affd63ef7) +**Glioblastoma, IDH-Wildtype** +*Axial T1 C+ FS MR shows multifocal enhancement in periventricular WM, fornices , and septum pellucidum . Noncontiguous regional involvement (satellite lesions) is a less common pattern in glioblastoma multiforme.* + +![Axial T1 C+ MR demonstrates a solidly enhancing mass with mild surrounding vasogenic edema, consistent with primary CNS lymphoma in an immunocompetent patient. In immunocompromised lymphoma, the lesions tend to present with ring enhancement.](d79a1356-b5f4-4dc5-ab1c-65b7291ae583) +**Lymphoma, Primary CNS** +*Axial T1 C+ MR demonstrates a solidly enhancing mass with mild surrounding vasogenic edema, consistent with primary CNS lymphoma in an immunocompetent patient. In immunocompromised lymphoma, the lesions tend to present with ring enhancement.* + +![Axial T1 C+ MR shows homogeneous enhancement within multiple periventricular WM foci. Lack of significant surrounding T2 abnormality (not shown) and mild mass and corpus callosum involvement is common.](e298292d-2258-4d61-8e9e-1d173c42e8ac) +**Lymphoma, Primary CNS** +*Axial T1 C+ MR shows homogeneous enhancement within multiple periventricular WM foci. Lack of significant surrounding T2 abnormality (not shown) and mild mass and corpus callosum involvement is common.* + +![Axial T1 MR shows the neoplasm is an uncommon cause of vasculitis in this case of intravascular (angiocentric) lymphoma. Note numerous foci of punctate, linear, and confluent enhancement .](2482a3b6-bf22-467c-b4f1-378e803e1ac0) +**Lymphoma, Primary CNS** +*Axial T1 MR shows the neoplasm is an uncommon cause of vasculitis in this case of intravascular (angiocentric) lymphoma. Note numerous foci of punctate, linear, and confluent enhancement .* + +![Axial T1 C+ MR shows multiple periventricular and subcortical metastatic enhancing lesions in a patient with lung adenocarcinoma.](fd0cf8be-9b1a-47b7-9200-a1f4bf8be499) +**Metastases, Parenchymal** +*Axial T1 C+ MR shows multiple periventricular and subcortical metastatic enhancing lesions in a patient with lung adenocarcinoma.* + +![Axial T1 C+ MR shows enhancing lesions in the periventricular WM in this patient with a history of breast cancer.](a1a2c677-d180-48c5-8cde-6f554452bbf4) +**Metastases, Parenchymal** +*Axial T1 C+ MR shows enhancing lesions in the periventricular WM in this patient with a history of breast cancer.* + +![Axial T1 C+ MR demonstrates a large, ring-enhancing lesion in the left frontal lobe with mild surrounding vasogenic edema. The lesion was drained surgically, yielding Streptococcus anginosus cerebral abscess.](4b227bca-6604-429e-952f-d8cac23f6382) +**Abscess** +*Axial T1 C+ MR demonstrates a large, ring-enhancing lesion in the left frontal lobe with mild surrounding vasogenic edema. The lesion was drained surgically, yielding Streptococcus anginosus cerebral abscess.* + +![Axial T1 C+ FS MR shows a ring-enhancing mass in the left frontal lobe. Thin-walled enhancement is typical of an abscess. Note the impending intraventricular rupture .](ed2bf437-1286-4b66-9979-fc68e1400ee5) +**Abscess** +*Axial T1 C+ FS MR shows a ring-enhancing mass in the left frontal lobe. Thin-walled enhancement is typical of an abscess. Note the impending intraventricular rupture .* + +![Axial T1 C+ MR shows bilateral rim- enhancing lesions with a targetoid appearance , characteristic of CNS toxoplasmosis. This was the initial presentation of a patient who suffered from undiagnosed HIV/AIDS.](52b0f1cb-1330-4ff6-95a2-3aa2d6b8236e) +**Toxoplasmosis, Acquired** +*Axial T1 C+ MR shows bilateral rim- enhancing lesions with a targetoid appearance , characteristic of CNS toxoplasmosis. This was the initial presentation of a patient who suffered from undiagnosed HIV/AIDS.* + +![Coronal T1 C+ MR shows multifocal masses with ring enhancement . Nodular enhancement is also frequently seen. Toxoplasmosis often lacks restricted diffusion on MR, unlike most abscesses.](e575c943-d5f9-49f9-8f94-08f45b5f0aa4) +**Toxoplasmosis, Acquired** +*Coronal T1 C+ MR shows multifocal masses with ring enhancement . Nodular enhancement is also frequently seen. Toxoplasmosis often lacks restricted diffusion on MR, unlike most abscesses.* + +![Coronal T1 C+ MR shows a large, mixed solid and cystic heterogeneously enhancing mass involving the right basal ganglia . Up to 10% of CNS germinomas arise within the basal ganglia.](aa807232-7be8-4d3c-a03b-c14c4e9ada9f) +**Germinoma** +*Coronal T1 C+ MR shows a large, mixed solid and cystic heterogeneously enhancing mass involving the right basal ganglia . Up to 10% of CNS germinomas arise within the basal ganglia.* + +![Axial T1 C+ MR shows patchy, multifocal enhancement consistent with subacute infarcts in this patient with lupus vasculitis. Vasculitis is often in the cortical and subcortical WM, although basal ganglia involvement is common. Associated DWI restriction may be seen.](4a81c19b-0b3f-4a7e-9d3f-3721e00f8674) +**Vasculitis** +*Axial T1 C+ MR shows patchy, multifocal enhancement consistent with subacute infarcts in this patient with lupus vasculitis. Vasculitis is often in the cortical and subcortical WM, although basal ganglia involvement is common. Associated DWI restriction may be seen.* + +![Axial T1 C+ MR shows multifocal punctate foci of periventricular enhancement with associated T2 hyperintensity (not shown) without significant mass effect. The pattern of involvement in Lyme disease mimics MS lesions.](06bbddf8-e16d-49c4-b015-d2a05c47ffaf) +**Lyme Disease** +*Axial T1 C+ MR shows multifocal punctate foci of periventricular enhancement with associated T2 hyperintensity (not shown) without significant mass effect. The pattern of involvement in Lyme disease mimics MS lesions.* + +![Axial NECT shows a left periventricular enhancing mass with small cystic areas that are commonly present. Ependymomas more commonly are in or near the 4th ventricle but may be supratentorial (1/3 of cases). Calcifications are seen in 50%.](29ce194a-6225-4704-b8dd-a74ec26f735a) +**Ependymoma** +*Axial NECT shows a left periventricular enhancing mass with small cystic areas that are commonly present. Ependymomas more commonly are in or near the 4th ventricle but may be supratentorial (1/3 of cases). Calcifications are seen in 50%.* + +![Axial NECT shows a periventricular, supratentorial ependymoma containing coarse calcifications . There is marked peritumoral edema. Note the subfalcine shift and obstructed ventricles.](c79b6aa9-22a5-4782-af8a-f94b62ebcc29) +**Ependymoma** +*Axial NECT shows a periventricular, supratentorial ependymoma containing coarse calcifications . There is marked peritumoral edema. Note the subfalcine shift and obstructed ventricles.* + +![Axial T1 C+ MR shows bilateral periventricular linear perivascular enhancement in sarcoidosis. Note additional leptomeningeal enhancement .](e6bd4bde-4060-42b2-974a-d05bf3c89019) +**Neurosarcoid** +*Axial T1 C+ MR shows bilateral periventricular linear perivascular enhancement in sarcoidosis. Note additional leptomeningeal enhancement .* + +![Axial T1 C+ MR shows linear enhancing foci in deep and periventricular WM that parallel the course of the cerebral microvasculature. This form of "carcinomatous encephalitis" is a rare intracranial manifestation of systemic leukemia.](ea07d0f0-efc4-458e-afaa-6ae4fc61ea72) +**Leukemia** +*Axial T1 C+ MR shows linear enhancing foci in deep and periventricular WM that parallel the course of the cerebral microvasculature. This form of "carcinomatous encephalitis" is a rare intracranial manifestation of systemic leukemia.* + +![Sagittal FLAIR MR shows multiple hyperintense lesions in the corpus callosum, typical for Susac syndrome and MS. Enhanced scans typically show leptomeningeal enhancement.](20e9c61e-0280-4fbf-a151-c2bcbc38bcb8) +**Susac Syndrome** +*Sagittal FLAIR MR shows multiple hyperintense lesions in the corpus callosum, typical for Susac syndrome and MS. Enhanced scans typically show leptomeningeal enhancement.* + +![Axial T1 C+ MR shows characteristic near-total lack of WM myelination and striking enhancement of the deep periventricular white matter . These patients usually present with a large head.](20f8df48-30cb-4c40-a8a2-94b8cc8c1d28) +**Alexander Disease** +*Axial T1 C+ MR shows characteristic near-total lack of WM myelination and striking enhancement of the deep periventricular white matter . These patients usually present with a large head.* + +![Axial CECT shows dilated ependymal veins due to venous congestion in an infant with a large vein of Galen malformation.](8dd8f348-1c4c-46fa-9187-3381e43ad096) +**Ependymal/Subependymal Veins (Mimic)** +*Axial CECT shows dilated ependymal veins due to venous congestion in an infant with a large vein of Galen malformation.* + +![Axial T1 C+ MR shows marked enhancement of the deep nuclei in the setting of subacute venous infarction due to deep venous thrombosis. Note subependymal venous congestion .](4bd485f1-6a73-4b41-b317-1510ca8e7e63) +**Ependymal/Subependymal Veins (Mimic)** +*Axial T1 C+ MR shows marked enhancement of the deep nuclei in the setting of subacute venous infarction due to deep venous thrombosis. Note subependymal venous congestion .* + +![Axial T1 C+ MR shows punctate and confluent enhancement in bilateral subcortical and periventricular white matter in PML-IRIS in an AIDS patient following initiation of HAART. There was associated hyperintense T2 FLAIR signal and mild mass effect.](e8938ebb-f3be-4cc0-a793-490f961ba82b) +**Immune Reconstitution Inflammatory Syndrome (IRIS)** +*Axial T1 C+ MR shows punctate and confluent enhancement in bilateral subcortical and periventricular white matter in PML-IRIS in an AIDS patient following initiation of HAART. There was associated hyperintense T2 FLAIR signal and mild mass effect.* + +![Axial T1 C+ MR demonstrates hypointense lesions centered in the bilateral thalami around the 3rd ventricle in a patient with neuromyelitis optica. Note faint peripheral enhancement on the right representing active demyelination. Typical neuromyelitis optica lesions are periventricular/periependymal following the distribution of aquaporin-4.](7c2aad0e-0760-41d9-aef0-585ada50424e) +**Neuromyelitis Optica Spectrum Disorders** +*Axial T1 C+ MR demonstrates hypointense lesions centered in the bilateral thalami around the 3rd ventricle in a patient with neuromyelitis optica. Note faint peripheral enhancement on the right representing active demyelination. Typical neuromyelitis optica lesions are periventricular/periependymal following the distribution of aquaporin-4.* + +![Axial T1 C+ MR demonstrates periventricular enhancement around the 4th ventricle in a patient with neurosarcoid.](516b084a-3a47-43e6-bd9e-90210655baab) +**Neurosarcoid** +*Axial T1 C+ MR demonstrates periventricular enhancement around the 4th ventricle in a patient with neurosarcoid.* + +![Axial T1 C+ MR demonstrates a large periventricular enhancing mass, a glioblastoma, centered in the genu and body of the corpus callosum. The main differential consideration for a mass involving the corpus callosum and crossing the midline is lymphoma.](64b4c649-57ce-4a66-bf09-8a4e692af554) +**Glioblastoma, IDH-Wildtype** +*Axial T1 C+ MR demonstrates a large periventricular enhancing mass, a glioblastoma, centered in the genu and body of the corpus callosum. The main differential consideration for a mass involving the corpus callosum and crossing the midline is lymphoma.* + +![Axial T1 C+ MR demonstrates periventricular and smaller subcortical enhancing lesions in a patient with primary CNS lymphoma. The larger periventricular lesions show solid enhancement, which is the norm in immunocompetent lymphoma patients.](748cf6c4-a9be-44bd-8091-033f89ebafa3) +**Lymphoma, Primary CNS** +*Axial T1 C+ MR demonstrates periventricular and smaller subcortical enhancing lesions in a patient with primary CNS lymphoma. The larger periventricular lesions show solid enhancement, which is the norm in immunocompetent lymphoma patients.* + +![Axial T1 C+ MR shows bilateral multiple periventricular enhancing demyelinating lesions in a patient with ADEM. ADEM typically follows an infection or vaccination and can involve gray matter.](images/app.statdx.com_image_thumbnail_8199b423-692d-444a-a9e2-6c6a0b9e4c54_annotated_true_size_900_quality_90_cf8ead5b_20251018T165200Z.jpg) +**ADEM** +*Axial T1 C+ MR shows bilateral multiple periventricular enhancing demyelinating lesions in a patient with ADEM. ADEM typically follows an infection or vaccination and can involve gray matter.* + +![Axial T1 C+ MR shows extensive bilateral periventricular and subcortical enhancing lesions in a patient with PML-IRIS. Unlike PML, PML-IRIS demonstrates mass effect and enhancement.](997613b8-e42e-4c39-a700-d36abd78ee3a) +**Immune Reconstitution Inflammatory Syndrome (IRIS)** +*Axial T1 C+ MR shows extensive bilateral periventricular and subcortical enhancing lesions in a patient with PML-IRIS. Unlike PML, PML-IRIS demonstrates mass effect and enhancement.* + +![Axial C+ MR demonstrates a rim-enhancing periventricular lesion , which was consistent with a toxoplasmosis abscess. Note local mass effect and surrounding hypointense vasogenic edema . The main differential consideration in an immunocompromised HIV patient would be lymphoma.](9e58cb1b-6c77-4f2a-9ba6-0559b63d1aa4) +**Toxoplasmosis, Acquired** +*Axial C+ MR demonstrates a rim-enhancing periventricular lesion , which was consistent with a toxoplasmosis abscess. Note local mass effect and surrounding hypointense vasogenic edema . The main differential consideration in an immunocompromised HIV patient would be lymphoma.* + +![Axial T1 C+ MR demonstrates punctate stippled enhancement in the pons and adjacent middle cerebellar peduncles . This is the typical perivascular enhancement pattern in CLIPPERS, and it can extend to involve the supratentorial brain.](c088034a-4d78-48e8-8bba-403a1470434a) +**CLIPPERS** +*Axial T1 C+ MR demonstrates punctate stippled enhancement in the pons and adjacent middle cerebellar peduncles . This is the typical perivascular enhancement pattern in CLIPPERS, and it can extend to involve the supratentorial brain.* + +![Axial T1 C+ MR demonstrates bilateral periventricular punctate and patchy linear enhancement in a patient with pathologically confirmed primary CNS vasculitis.](91ac979e-39ff-45b8-8f31-83f8d73f3231) +**Vasculitis** +*Axial T1 C+ MR demonstrates bilateral periventricular punctate and patchy linear enhancement in a patient with pathologically confirmed primary CNS vasculitis.* + +![Axial T1 C+ MR demonstrates multiple bilateral enhancing subcortical and periventricular septic emboli of variable size and appearance in a patient with Escherichia coli septicemia.](53e343e1-4044-4e50-ad42-5f52d1e503ec) +**Septic Emboli** +*Axial T1 C+ MR demonstrates multiple bilateral enhancing subcortical and periventricular septic emboli of variable size and appearance in a patient with Escherichia coli septicemia.* + +![Axial T1 C+ MR demonstrates a typical incomplete ring of enhancement in tumefactive demyelination . Such lesions can present with variable degrees of mass effect. The incomplete ring of enhancement allows differentiation from neoplastic causes.](images/app.statdx.com_image_thumbnail_9cc153d1-8efa-437e-b056-8fb7d5682d4b_annotated_true_size_900_quality_90_0ce8b76a_20251018T165158Z.jpg) +**Tumefactive Demyelination** +*Axial T1 C+ MR demonstrates a typical incomplete ring of enhancement in tumefactive demyelination . Such lesions can present with variable degrees of mass effect. The incomplete ring of enhancement allows differentiation from neoplastic causes.* + +![Axial T1 C+ MR shows several periventricular and juxtacortical enhancing lesions in a patient with relapsing/remitting multiple sclerosis. Note no associated mass effect.](images/app.statdx.com_image_thumbnail_3f3b258d-f5d6-468f-a67c-3bde583caee1_annotated_true_size_900_quality_90_5949d05d_20251018T165158Z.jpg) +**Multiple Sclerosis** +*Axial T1 C+ MR shows several periventricular and juxtacortical enhancing lesions in a patient with relapsing/remitting multiple sclerosis. Note no associated mass effect.* + +![Coronal T1 C+ MR shows numerous foci of enhancement in the subcortical and periventricular WM. Fuzzy enhancing margins are typical for demyelination. ADEM typically follows an infection or vaccination.](9145a408-0c29-4a73-9c3e-fbdcf613b141) +**ADEM** +*Coronal T1 C+ MR shows numerous foci of enhancement in the subcortical and periventricular WM. Fuzzy enhancing margins are typical for demyelination. ADEM typically follows an infection or vaccination.* + diff --git a/docs_md/articles/small-ventricles_2f99bc62-163e-41aa-b190-0da8a4de6d11.md b/docs_md/articles/small-ventricles_2f99bc62-163e-41aa-b190-0da8a4de6d11.md new file mode 100644 index 0000000..36a24a5 --- /dev/null +++ b/docs_md/articles/small-ventricles_2f99bc62-163e-41aa-b190-0da8a4de6d11.md @@ -0,0 +1,227 @@ +--- +title: "Small Ventricles" +docid: "2f99bc62-163e-41aa-b190-0da8a4de6d11" +authors: + - key: "d19354f3-7ff2-495a-ad3f-064122e45602" + value: "Bernadette L. Koch, MD" + - key: "f184750a-90b4-47a7-907b-23b05d70357a" + value: "Chang Yueh Ho, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60" + - + name: "Ventricles, Periventricular Regions" + slug: "ventricles-periventricular-regions" + treeNodeId: "353c434a-a6fc-4ef1-8786-d30a1988a4dc" + - + name: "Generic Imaging Patterns" + slug: "generic-imaging-patterns" + treeNodeId: "969c31a2-ef56-4fc3-9125-05857cf9aac3" + - + name: "Small Ventricles" + slug: "small-ventricles" + treeNodeId: null +category: "Brain" +documentVersionId: "5b4a0ab0-ffd6-4209-a606-7d38fb6ec442" +imageCount: 27 +lastUpdated: "01/26/23" +pageDescription: "Small Ventricles" +pageKeywords: "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Small Ventricles" +pageTitle: "Small Ventricles | STATdx" +enhancedTitle: "Small Ventricles" +type: "DDX" +references: true +breadcrumbs: + - "Brain" + - "Differential Diagnosis" + - "Ventricles, Periventricular Regions" + - "Generic Imaging Patterns" + - "Small Ventricles" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Clinical features usually help define cause +- ## Helpful Clues for Common Diagnoses + + + - **Normal Variant (Young Brain)** + - Small ventricles in children & young adults may be normal + - Look for visualization of sulci at vertex & sylvian fissures to exclude diffuse cerebral edema + - **CSF Shunts & Complications** + - **Slit ventricle syndrome**: Symptoms of ↑ ICP with collapsed shunted ventricles + - Noncompliant ventricles, stiff brain, or overshunting + - Anti-siphon devices (gravitational shunt valves) to prevent overshunting + - **Posttraumatic Brain Swelling** + - Diffuse edema with sulcal & ventricular effacement + - Frequently with associated diffuse axonal injury + - Focal edema from contusions may cause ventricular effacement & herniation + - **Herniation Syndromes, Intracranial** + - Ventricular effacement from herniation due to hematoma & tumors + - Increasing subfalcine herniation → ipsilateral ventricular effacement & contralateral ventricular dilatation + - **Adult Hypoxic-Ischemic Injury** + - Hypoxic-ischemic injury (HII) = global HII, global anoxic injury, & cerebral hypoperfusion injury or cerebral hypoperfusion → diffuse cytotoxic edema + - CT: Diffuse cerebral edema + small ventricles & sulci + - ↓ gray-white differentiation, hypodense basal ganglia, bright cerebellar reversal sign + - MR: Diffuse cortical ↑ T2 signal & ↓ diffusion + - ± pattern of central gray ↓ diffusion + - **Neonatal Hypoxic-Ischemic Injury** + - Hypoxic-ischemic encephalopathy from perinatal hypoxia or hypoperfusion + - Deep or central pattern: Deep gray ± brainstem injury & loss of cortical ribbon on T2WI + - Most likely to cause small ventricles + - Peripheral pattern = injury to watershed zones, less likely to cause diffuse cerebral swelling +- ## Helpful Clues for Less Common Diagnoses + + + - **Hyperglycemia** + - Diabetic ketoacidosis (DKA): Diffuse cerebral edema + - ± osmotic demyelination + - **Cerebral Infection** + - Gray & white matter T2 hyperintensity & edema + - Mild restriction on DWI common in viral encephalitis + - **Intracranial Hypotension** + - "Slumping" midbrain, acquired tonsillar herniation, diffusely enhancing dura, veins/dural sinuses distended + - Interpeduncular cistern lower than dorsum sella + - Best evaluated on sagittal images + - **Idiopathic Intracranial Hypertension** + - Dilated optic nerve sheaths, posterior globe flattening, empty sella, slit-like ventricles rare +- ## Helpful Clues for Rare Diagnoses + + + - **Inborn Errors of Metabolism (Acute Presentation)** + - Acute exacerbation + diffuse edema & brain injury → mass effect on ventricles + +## References + +# Selected References + +1. [Panagopoulos D et al: Shunt over-drainage, slit ventricle syndrome, programmable valves and anti-siphon devices. a narrative review of a multifactorial and intractable problem. J Integr Neurosci. 21(3):84, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35633165%5Bpmid%5D) +1. [Bond KM et al: Spontaneous intracranial hypotension: atypical radiologic ppearances, imaging mimickers, and clinical look-alikes. AJNR Am J Neuroradiol. 41(8):1339-47, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32646948%5Bpmid%5D) +1. [Kranz PG et al: Spontaneous intracranial hypotension: pathogenesis, diagnosis, and treatment. Neuroimaging Clin N Am. 29(4):581-94, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31677732%5Bpmid%5D) +1. [Rekate HL: Shunt-related headaches: the slit ventricle syndromes. Childs Nerv Syst. 24(4):423-30, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18259760%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial NECT shows completely collapsed lateral ventricles with a shunt catheter . Slit ventricle syndrome must have clinical symptoms of ↑ intracranial pressure with slit appearance of the lateral ventricles due to shunt.](0728b805-3262-47ee-8be6-1021849a885b) +**CSF Shunts & Complications** +*Axial NECT shows completely collapsed lateral ventricles with a shunt catheter . Slit ventricle syndrome must have clinical symptoms of ↑ intracranial pressure with slit appearance of the lateral ventricles due to shunt.* + +![Axial CT in a 9-year-old injured by a horse shows bilateral scalp hematomas, effacement of sulci, loss of gray-white matter differentiation, and small lateral ventricles. Also note small foci of hemorrhagic shear-type injury at the gray-white junction in the frontal lobes.](images/app.statdx.com_image_thumbnail_c09bfc46-3236-46b4-a14e-ee747ac8ace8_annotated_true_size_900_quality_90_ae8ac602_20251018T165215Z.jpg) +**Posttraumatic Brain Swelling** +*Axial CT in a 9-year-old injured by a horse shows bilateral scalp hematomas, effacement of sulci, loss of gray-white matter differentiation, and small lateral ventricles. Also note small foci of hemorrhagic shear-type injury at the gray-white junction in the frontal lobes.* + +![Axial NECT shows complete loss of the left middle cerebral artery gray-white distinction with cytotoxic edema and left-to-right subfalcine herniation . Note the compression of the ipsilateral left lateral ventricle and enlargement of the right lateral ventricle from entrapment.](images/app.statdx.com_image_thumbnail_59079b5e-7d2d-4336-a5ad-aa23a5785112_annotated_true_size_900_quality_90_39d39744_20251018T165215Z.jpg) +**Herniation Syndromes, Intracranial** +*Axial NECT shows complete loss of the left middle cerebral artery gray-white distinction with cytotoxic edema and left-to-right subfalcine herniation . Note the compression of the ipsilateral left lateral ventricle and enlargement of the right lateral ventricle from entrapment.* + +![Axial NECT shows diffuse effacement of ventricles and sulci from diffuse cerebral edema. There is focal symmetric hypodensity of the globi pallidi in this patient with a history of cardiogenic shock.](images/app.statdx.com_image_thumbnail_9c410bc5-2a93-4247-924c-e0d8c4cf1015_annotated_true_size_900_quality_90_c898a7ba_20251018T165215Z.jpg) +**Adult Hypoxic-Ischemic Injury** +*Axial NECT shows diffuse effacement of ventricles and sulci from diffuse cerebral edema. There is focal symmetric hypodensity of the globi pallidi in this patient with a history of cardiogenic shock.* + +![Axial PD MR in a 2-day-old with severe deep/central pattern of hypoxic-ischemic injury demonstrates small lateral ventricles and cerebral sulci, as well as symmetric, hyperintense signal in the bilateral globus pallidus, putamen, and thalamus .](images/app.statdx.com_image_thumbnail_93f3b7e9-aa77-4429-9f1f-eebcc59aa0e9_annotated_true_size_900_quality_90_193f00bb_20251018T165216Z.jpg) +**Neonatal Hypoxic-Ischemic Injury** +*Axial PD MR in a 2-day-old with severe deep/central pattern of hypoxic-ischemic injury demonstrates small lateral ventricles and cerebral sulci, as well as symmetric, hyperintense signal in the bilateral globus pallidus, putamen, and thalamus .* + +![Axial NECT in a 12-year-old with altered mental status and diabetic ketoacidosis shows near-complete effacement of the lateral ventricles, perimesencephalic cisterns, and cerebral sulci related to diffuse cerebral edema.](images/app.statdx.com_image_thumbnail_2210a160-876a-42ce-bc87-c3dbbed69388_annotated_true_size_900_quality_90_4abbe33c_20251018T165217Z.jpg) +**Hyperglycemia** +*Axial NECT in a 12-year-old with altered mental status and diabetic ketoacidosis shows near-complete effacement of the lateral ventricles, perimesencephalic cisterns, and cerebral sulci related to diffuse cerebral edema.* + +![Axial FLAIR MR shows diffuse gray matter swelling and hyperintensity with resulting effacement of the cerebral sulci and right lateral ventricle. This patient eventually succumbed to rabies encephalitis.](images/app.statdx.com_image_thumbnail_344f9e7e-6aa2-4ddc-a054-c77a38728a66_annotated_true_size_900_quality_90_7004467a_20251018T165217Z.jpg) +**Cerebral Infection** +*Axial FLAIR MR shows diffuse gray matter swelling and hyperintensity with resulting effacement of the cerebral sulci and right lateral ventricle. This patient eventually succumbed to rabies encephalitis.* + +![Sagittal T1 MR shows downward displacement of the cerebellar tonsils and brainstem. There is effacement of the suprasellar cistern . Note effacement of the interpeduncular cistern , which has moved below the level of the dorsum sella, related to "brainstem sagging."](6e228d55-4868-4c43-95d8-556d5988bb6d) +**Intracranial Hypotension** +*Sagittal T1 MR shows downward displacement of the cerebellar tonsils and brainstem. There is effacement of the suprasellar cistern . Note effacement of the interpeduncular cistern , which has moved below the level of the dorsum sella, related to "brainstem sagging."* + + +### Additional Images + +![Axial NECT shows small ventricles and indeterminate shunt position . Symptomatic ventricular collapse is known as slit-like ventricle syndrome and suggests overshunting.](8cb4ffa1-9769-4a57-8cea-8cb068beeeaa) +**CSF Shunts & Complications** +*Axial NECT shows small ventricles and indeterminate shunt position . Symptomatic ventricular collapse is known as slit-like ventricle syndrome and suggests overshunting.* + +![Axial GRE MR in an 4-year-old pedestrian struck by a motor vehicle shows scalp swelling, small ventricles and sulci, and multifocal areas of hemorrhagic shear type injury .](images/app.statdx.com_image_thumbnail_40bf8fe2-f04d-43dc-bfdc-f5aa59a7acd4_annotated_true_size_900_quality_90_133dfd0c_20251018T165215Z.jpg) +**Posttraumatic Brain Swelling** +*Axial GRE MR in an 4-year-old pedestrian struck by a motor vehicle shows scalp swelling, small ventricles and sulci, and multifocal areas of hemorrhagic shear type injury .* + +![Axial NECT shows hyperdense foci of diffuse axonal injury , which is commonly associated with traumatic cerebral edema. Note sulcal and ventricular effacement . Loss of gray-white differentiation is common.](images/app.statdx.com_image_thumbnail_38e45bd4-1af7-4ecc-9abc-c1746abd57a4_annotated_true_size_900_quality_90_0f71d8b2_20251018T165215Z.jpg) +**Posttraumatic Brain Swelling** +*Axial NECT shows hyperdense foci of diffuse axonal injury , which is commonly associated with traumatic cerebral edema. Note sulcal and ventricular effacement . Loss of gray-white differentiation is common.* + +![Axial GRE MR shows multiple punctate areas of susceptibility in the subcortical frontal white matter but also in the splenium of the corpus callosum from diffuse axonal injury. Less well appreciated on this sequence are small lateral ventricles and cerebral edema.](images/app.statdx.com_image_thumbnail_360ffa4d-494f-496c-bbd8-c5f86b31d309_annotated_true_size_900_quality_90_0f4ae828_20251018T165215Z.jpg) +**Posttraumatic Brain Swelling** +*Axial GRE MR shows multiple punctate areas of susceptibility in the subcortical frontal white matter but also in the splenium of the corpus callosum from diffuse axonal injury. Less well appreciated on this sequence are small lateral ventricles and cerebral edema.* + +![Axial NECT shows low-density subacute infarcts in the cerebellar hemispheres. Basal cisterns are effaced , as is the 4th ventricle in this patient with transtentorial herniation. Herniation syndromes typically result from trauma, ischemia, or mass.](images/app.statdx.com_image_thumbnail_e1b30e49-cf00-4604-b50a-e783eed16b3f_annotated_true_size_900_quality_90_35ec3262_20251018T165215Z.jpg) +**Herniation Syndromes, Intracranial** +*Axial NECT shows low-density subacute infarcts in the cerebellar hemispheres. Basal cisterns are effaced , as is the 4th ventricle in this patient with transtentorial herniation. Herniation syndromes typically result from trauma, ischemia, or mass.* + +![Axial NECT shows small ventricles and right occipital and vermian low density due to infarct. Basal cisterns are effaced. Vermis is herniated upward through the tentorial incisura , displacing midbrain anteriorly and superiorly.](images/app.statdx.com_image_thumbnail_378afa91-9f62-471d-bfab-5a895cb3a502_annotated_true_size_900_quality_90_e93cc46e_20251018T165215Z.jpg) +**Herniation Syndromes, Intracranial** +*Axial NECT shows small ventricles and right occipital and vermian low density due to infarct. Basal cisterns are effaced. Vermis is herniated upward through the tentorial incisura , displacing midbrain anteriorly and superiorly.* + +![Axial NECT shows a typical case of impending brain death with diffuse cerebral edema. Note diffuse sulcal effacement, small ventricles , and decreased gray-white matter differentiation within the cerebral hemispheres.](images/app.statdx.com_image_thumbnail_6f7bdf82-ce21-42e7-b457-9f7ff5483ab4_annotated_true_size_900_quality_90_322edb6c_20251018T165216Z.jpg) +**Adult Hypoxic-Ischemic Injury** +*Axial NECT shows a typical case of impending brain death with diffuse cerebral edema. Note diffuse sulcal effacement, small ventricles , and decreased gray-white matter differentiation within the cerebral hemispheres.* + +![Axial PD FSE MR shows enlarged, bilateral, hyperintense deep nuclei and small ventricles from mass effect in hypoxic-ischemic encephalopathy. Cortical hyperintensity is less prominent than on DWI (not shown) except for more advanced bilateral occipital involvement .](images/app.statdx.com_image_thumbnail_9f391b27-1dc1-4c50-b042-f1697cebd49c_annotated_true_size_900_quality_90_7f7b09b9_20251018T165216Z.jpg) +**Adult Hypoxic-Ischemic Injury** +*Axial PD FSE MR shows enlarged, bilateral, hyperintense deep nuclei and small ventricles from mass effect in hypoxic-ischemic encephalopathy. Cortical hyperintensity is less prominent than on DWI (not shown) except for more advanced bilateral occipital involvement .* + +![Axial FLAIR MR shows near-confluent T2 hyperintensity in the deep white matter and small ventricles related to mild mass effect from encephalitis.](c5657e5a-dbfc-49d1-af9b-909ebc180469) +**Cerebral Infection** +*Axial FLAIR MR shows near-confluent T2 hyperintensity in the deep white matter and small ventricles related to mild mass effect from encephalitis.* + +![Axial CECT shows thick, enhancing frontal leptomeninges with adjacent frontal lobe hypodensity from edema and mass effect on the frontal horns. This was pyogenic meningitis with adjacent cerebritis.](5c5ce154-c80c-41b3-919e-e93471bee87a) +**Cerebral Infection** +*Axial CECT shows thick, enhancing frontal leptomeninges with adjacent frontal lobe hypodensity from edema and mass effect on the frontal horns. This was pyogenic meningitis with adjacent cerebritis.* + +![Axial T1 C+ FS MR shows subtle enhancement of the pial surface of the brain . Note associated ependymitis and choroid plexitis , which may complicate meningitis. Ventricles are small due to diffuse brain edema, although when pial exudates obstruct CSF flow, hydrocephalus is more common.](d0e3444e-a542-4be2-8ff1-366016be79a4) +**Cerebral Infection** +*Axial T1 C+ FS MR shows subtle enhancement of the pial surface of the brain . Note associated ependymitis and choroid plexitis , which may complicate meningitis. Ventricles are small due to diffuse brain edema, although when pial exudates obstruct CSF flow, hydrocephalus is more common.* + +![Coronal T1 C+ MR shows meningitis complicated by local cerebritis, as evidenced in this patient with focal mass effect, small ventricles, and ill-defined enhancement .](a1be6ac0-cc98-4376-a5c3-17f2281a9597) +**Cerebral Infection** +*Coronal T1 C+ MR shows meningitis complicated by local cerebritis, as evidenced in this patient with focal mass effect, small ventricles, and ill-defined enhancement .* + +![Sagittal T1 MR shows an empty sella and small ventricles in a patient with idiopathic intracranial hypertension or "pseudotumor cerebri."](05572786-4d3d-47f0-b423-86d77738848c) +**Idiopathic Intracranial Hypertension** +*Sagittal T1 MR shows an empty sella and small ventricles in a patient with idiopathic intracranial hypertension or "pseudotumor cerebri."* + +![Coronal T1 MR in a young woman with headaches and papilledema shows very small lateral ventricles . Superficial sulci also look somewhat less prominent than normal. Pituitary gland is normal for a young, menstruating female.](71426456-b87a-4901-a5e7-29b8fb07649c) +**Idiopathic Intracranial Hypertension** +*Coronal T1 MR in a young woman with headaches and papilledema shows very small lateral ventricles . Superficial sulci also look somewhat less prominent than normal. Pituitary gland is normal for a young, menstruating female.* + +![Axial T2 MR shows increased fluid in the optic nerve sheaths and mild flattening of the globes at the optic nerve heads . Note the partly empty sella . Findings suggest idiopathic intracranial hypertension that can be confirmed with CSF opening pressures.](77d06d43-62be-4662-bba1-60f6070ef6d0) +**Idiopathic Intracranial Hypertension** +*Axial T2 MR shows increased fluid in the optic nerve sheaths and mild flattening of the globes at the optic nerve heads . Note the partly empty sella . Findings suggest idiopathic intracranial hypertension that can be confirmed with CSF opening pressures.* + +![Sagittal T1 MR shows a "slumping midbrain," where the midbrain is displaced below the dorsum sellae . Ventricles are commonly small . The cerebellar tonsils are typically low-lying or herniated. Diffuse dural enhancement is characteristic. Subdural collections are uncommon (15%).](c9cc0066-c38f-443b-b84b-dd28c83aecca) +**Intracranial Hypotension** +*Sagittal T1 MR shows a "slumping midbrain," where the midbrain is displaced below the dorsum sellae . Ventricles are commonly small . The cerebellar tonsils are typically low-lying or herniated. Diffuse dural enhancement is characteristic. Subdural collections are uncommon (15%).* + +![Axial T1 C+ MR shows symmetric, small ventricles and smooth, diffuse, linear pachymeningeal thickening and enhancement .](b6acf477-3b41-4a08-a6a4-8c299968b6e7) +**Intracranial Hypotension** +*Axial T1 C+ MR shows symmetric, small ventricles and smooth, diffuse, linear pachymeningeal thickening and enhancement .* + +![Axial T2 MR shows diffuse, acute brain swelling in maple syrup urine disease and small ventricles due to subacute edema of deep white matter , thalami , and internal capsules.](f48cacf7-0b37-4f80-8db2-c843bc0ffa63) +**Inborn Errors of Metabolism (Acute Presentation)** +*Axial T2 MR shows diffuse, acute brain swelling in maple syrup urine disease and small ventricles due to subacute edema of deep white matter , thalami , and internal capsules.* + +![Axial T1 C+ MR shows diffuse dural enhancement and leptomeningeal enhancement from venous distention. There are small lateral ventricles. Intracranial hypotension is commonly from CSF leak through a dural defect or from LP.](87f394c3-7c19-4b36-b0cd-74b740fda4a4) +**Intracranial Hypotension** +*Axial T1 C+ MR shows diffuse dural enhancement and leptomeningeal enhancement from venous distention. There are small lateral ventricles. Intracranial hypotension is commonly from CSF leak through a dural defect or from LP.* + diff --git a/docs_md/articles/ventricles-and-cisterns-overview_ad860c4f-fe9a-4469-8eca-a7ccd5cff70f.md b/docs_md/articles/ventricles-and-cisterns-overview_ad860c4f-fe9a-4469-8eca-a7ccd5cff70f.md new file mode 100644 index 0000000..6d2597b --- /dev/null +++ b/docs_md/articles/ventricles-and-cisterns-overview_ad860c4f-fe9a-4469-8eca-a7ccd5cff70f.md @@ -0,0 +1,229 @@ +--- +title: "Ventricles and Cisterns Overview" +docid: "ad860c4f-fe9a-4469-8eca-a7ccd5cff70f" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Ventricles and Cisterns Overview" + slug: "ventricles-and-cisterns-overview" + treeNodeId: null +category: "Brain" +documentVersionId: "a4de8cd1-51b7-439f-b4c8-707218ae4252" +imageCount: 32 +lastUpdated: "08/10/20" +pageDescription: "Ventricles and Cisterns Overview" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Ventricles and Cisterns Overview" +pageTitle: "Ventricles and Cisterns Overview | STATdx" +enhancedTitle: "Ventricles and Cisterns Overview" +type: "INTRO" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Ventricles and Cisterns Overview" +--- +# Gross and Imaging Anatomy + +- ## Ventricles and Choroid Plexus +- **Basic embryology**: Early in embryonic development, the forebrain cavity divides into 2 lateral ventricles, which develop as outpouchings from the rostral 3rd ventricle and are connected to it by the interventricular foramen (a.k.a. foramen of Monro). In the coronal plane, these form a central H-shaped "monoventricle." The cerebral aqueduct develops from the midbrain vesicle. The 4th ventricle develops from a cavity within the hindbrain and merges caudally with the central canal of the spinal cord. +- **Anatomic overview**: The brain CSF spaces include both the ventricular system and subarachnoid spaces (SASs). The ventricular system is comprised of 4 interconnected CSF-filled, ependymal-lined cavities that lie deep within the brain. The paired **lateral ventricles** communicate with the 3rd ventricle via the Y-shaped **foramen of Monro**. The **3rd********ventricle** communicates with the 4th ventricle via the **cerebral aqueduct** (of Sylvius). In turn, the **4th********ventricle** communicates with the SAS via its outlet foramina (the midline **foramen of Magendie**and the 2 lateral **foramina of Luschka**). +- **Lateral ventricles**: Each lateral ventricle has a body, atrium, and 3 projections ("horns"). The roof of the **frontal horn**is formed by the corpus callosum genu. It is bordered laterally and inferiorly by the head of the caudate nucleus. The septi pellucidi is a thin, bilayered membrane that extends from the corpus callosum genu anteriorly to the foramen of Monro posteriorly and forms the medial borders of both frontal horns. +- The **body**of the lateral ventricle passes posteriorly under the corpus callosum. Its floor is formed by the dorsal thalamus and its medial wall is bordered by the fornix. Laterally, it curves around the body and tail of the caudate nucleus. +- The **atrium**contains the choroid plexus glomus and is formed by the confluence of the body with the temporal and occipital horns. The **temporal horn**extends anteroinferiorly from the atrium and is bordered on its floor and medial wall by the hippocampus. Its roof is formed by the tail of the caudate nucleus. The **occipital horn**is surrounded entirely by white matter fiber tracts, principally the geniculocalcarine tract and the forceps major of the corpus callosum. +- **Foramen of Monro** is a Y-shaped structure with 2 long arms extending towards each lateral ventricle and a short inferior common stem that connects with the roof of the 3rd ventricle. +- **3rd ventricle**: The 3rd ventricle is a single, slit-like, midline, vertically oriented cavity that lies between the thalami. Its roof is formed by the tela choroidea, a double layer of invaginated pia. The lamina terminalis and anterior commissure lie along the anterior border of the 3rd ventricle. The floor of the 3rd ventricle is formed from front to back by the optic chiasm, the hypothalamus with the tuber cinereum and infundibular stalk, mammillary bodies, and the roof of the midbrain tegmentum. +- The 3rd ventricle has 2 inferiorly located CSF-filled projections: The slightly rounded **optic recess** and the more pointed **infundibular recess**. Two small recesses, the **suprapineal and pineal recesses***,* form the posterior border of the 3rd ventricle. A variably sized interthalamic adhesion (also called the **massa intermedia**) lies between the lateral walls of the 3rd ventricle. The massa intermedia is not a true commissure. +- **Cerebral aqueduct** is an elongated tubular conduit that lies between the midbrain tegmentum and the quadrigeminal plate. It connects the 3rd ventricle with the 4th ventricle. +- **4th ventricle**: The 4th ventricle is a roughly diamond-shaped cavity that lies between the pons anteriorly and the cerebellar vermis posteriorly. Its roof is covered by the anterior (superior) medullary velum above and the inferior medullary velum below. +- The 4th ventricle has 5 distinctly shaped recesses. The **posterior superior recesses** are paired, thin, flat, CSF-filled pouches that cap the cerebellar tonsils. The **lateral recesses**curve anterolaterally from the 4th ventricle, extending under the brachium pontis (major cerebellar peduncle) into the lower cerebellopontine angle cisterns (CPAs). The lateral recesses transmit choroid plexus through the foramina of Luschka into the adjacent SAS. The **fastigium**is a triangular, blind-ending, dorsal midline outpouching that points towards the cerebellar vermis. The 4th ventricle gradually narrows as it courses inferiorly, forming the **obex**. Near the cervicomedullary junction, the obex becomes continuous with the central canal of the spinal cord. +- **Choroid plexus, CSF, and brain interstitial fluid (ISF)**: The choroid plexus is comprised of highly vascular papillary excrescences with a central connective tissue core coated by an ependyma-derived secretory epithelium. The embryonic choroid plexus forms where the infolded tela choroidea contacts the ependymal lining of the ventricles, thus developing along the entire choroidal fissure. +- The largest mass of choroid plexus, the**glomus**, is located in the atrium of the lateral ventricles. The choroid plexus extends anteriorly along the floor of the lateral ventricle, lying between the fornix and thalamus. It extends anteroinferiorly from the glomus into the temporal horn, where it fills the choroidal fissure and lies superomedial to the hippocampus. It also dives through the interventricular foramen (of Monro) and curves posteriorly along the roof of the 3rd ventricle. +- The choroid plexus has 2 major functions: CSF production and maintenance of the blood-CSF barrier. +- CSF is predominantly, but not exclusively, secreted by the choroid plexuses. **Brain ISF**, ependyma, and capillaries all play a potential role in CSF secretion. Drainage of brain ISF is a significant extrachoroidal source of CSF. The choroid plexus epithelium secretes CSF at the rate of ~ 0.4 mL/minute or 500-600 mL/day. In adult humans, there are 280 mL of ISF and 140 mL of CSF, of which 30 mL are in the ventricle, 80 mL in the cerebral SAS, and 30 mL in the spinal SAS. +- CSF plays an essential role in the maintenance of brain ISF homeostasis and regulation of neuronal functioning. +- **Traditional model of CSF****homeostasis**:****CSF flows through the ventricular system and passes through the exit foramina of the 4th ventricle into the SAS. The bulk of CSF resorption is through the arachnoid villi along the superior sagittal sinus. CSF also drains into lymphatic vessels around the cranial cavity and spinal canal. +- **Updated model of CSF and ISF****homeostasi****s**: Brain perivascular spaces and paravascular spaces play a critical role in CSF homeostasis. The perivascular spaces from a key component of the brain's "protolymphatic" or "glymphatic" system. ISF circulation likely occurs through the water-selective aquaporin (AQP) channels of the glymphatic system, a key factor in regulating extracellular space water homeostasis. AQP4 is highly expressed in the atrocytic end-feet. +- ## Cisterns and SASs +- **Overview**: The SASs lie between the pia and arachnoid. The sulci are CSF-filled spaces between the gyral folds. Focal expansions of the SASs form the brain CSF cisterns. These cisterns are found at the base of the brain around the brainstem, tentorial incisura, and foramen magnum. Numerous pial-covered septa cross the SAS from the brain to the arachnoid. All SAS cisterns communicate with each other and the ventricular system, providing natural pathways for disease spread (e.g., meningitis, neoplasms). The brain cisterns are conveniently grouped into supra-, peri-, and infratentorial cisterns. All contain numerous important critical structures, such as vessels and cranial nerves. +- **Supratentorial/peritentorial cisterns**: The **suprasellar cistern** lies between the diaphragma sellae and the hypothalamus. Critical contents include the infundibulum, optic chiasm, and circle of Willis. +- The **interpeduncular cistern**is the posterior continuation of the suprasellar cistern. Lying between the cerebral peduncles, it contains the oculomotor nerves as well as the distal basilar artery and proximal segments of the posterior cerebral arteries (PCAs). Important perforating arteries, the thalamoperforating and thalamogeniculate arteries, arise from the top of the basilar artery and cross the interpeduncular cistern to enter the midbrain. +- The**perimesencephalic****(ambient) cisterns**are thin wings of CSF that extend posterosuperiorly from the suprasellar cistern to the quadrigeminal cistern. They wrap around the midbrain and contain the trochlear nerves, P2 PCA segments, superior cerebellar arteries, and the basal vein of Rosenthal. +- The **quadrigeminal cistern** lies under the corpus callosum splenium, behind the pineal gland and tectal plate. It connects with the ambient cisterns laterally and the superior cerebellar cistern inferiorly. The quadrigeminal cistern contains the pineal gland, trochlear nerves, P3 PCA segments, proximal choroidal arteries, and the vein of Galen. An anterior extension, the **velum interpositum**, lies below the fornix and above the 3rd ventricle. The velum interpositum contains the internal cerebral veins and medial posterior choroidal arteries. +- **Infratentorial cisterns**: The unpaired posterior fossa cisterns that lie in the midline are the prepontine, premedullary, and superior cerebellar cisterns, as well as the cisterna magna. The lateral cisterns are paired and include the cerebellopontine and cerebellomedullary cisterns. +- The **prepontine cistern** lies between the upper clivus and the "belly" of the pons. It contains numerous important structures, including the basilar artery, anterior inferior cerebellar arteries (AICAs), and trigeminal and abducens nerves (CNV and CNVI). +- The **premedullary cistern** is the inferior continuation of the prepontine cistern. It lies between the lower clivus in front and the medulla behind. It extends inferiorly to the foramen magnum and contains the vertebral arteries and branches [e.g., posterior inferior cerebellar arteries (PICAs)] and the hypoglossal nerve (CNXII). +- The **superior cerebellar cistern** lies between the straight sinus above and the vermis below. It contains the superior cerebellar arteries and veins. It connects superiorly through the tentorial incisura with the quadrigeminal cistern and inferiorly with the cisterna magna. The **cisterna magna** lies below the inferior vermis between the medulla and the occiput. It contains the cerebellar tonsils and the tonsillohemispheric branches of the PICA. The cisterna magna merges imperceptibly with the SAS of the upper cervical spinal canal. +- The **CPAs** lie between the pons/cerebellum and the petrous temporal bone. Their most important contents are the trigeminal, facial, and vestibulocochlear nerves (CNV, CNVII, and CNVIII). Other structures found here include the petrosal veins and AICAs. The CPA cisterns are contiguous inferiorly with the cerebellomedullary cisterns. +- The **cerebellomedullary cisterns** extend laterally around the medulla and are continuous with the cisterna magna below and the CPAs above. They contain the vagus, glossopharyngeal, and spinal accessory nerves (CNIX, CNX, and CNXI). A tuft of the choroid plexus exits each foramen of Luschka into the cerebellomedullary cistern. The flocculus of the cerebellum that projects into this cistern can appear very prominent. The flocculus and choroid plexus are normal contents of the cerebellomedullary cisterns and should not be mistaken for pathology. + +# Imaging Recommendations + +- **MR**: Thin-section 3D T2WI or FIESTA/CISS best detail CSF within the ventricular system, SASs, and basal cisterns, and exquisitely delineates their contents. FLAIR MR is especially useful for evaluating potential abnormalities in the SASs. Spin dephasing with pulsatile CSF flow is common and can mimic pathology, especially in the basal cisterns and around the interventricular foramen. Incomplete CSF suppression with "bright" CSF can mimic pathologic SASs. + +# Differential Diagnosis Approach + +- ## Ventricles and Choroid Plexus +- **Overview**: Approximately 10% of intracranial neoplasms involve the cerebral ventricles, either primarily or by extension. An anatomy-based approach is most effective as there is a distinct predilection for certain lesions to occur in one ventricle or cistern and not others. Age is also a helpful consideration. Specific imaging findings, such as signal intensity, enhancement, and the presence or absence of calcification are relatively less important than location and age. +- **Normal variants**: Asymmetry of the lateral ventricles is a common normal variant, as is flow-related CSF pulsation artifact. A cavum septi pellucidi (CSP) is a common normal variant, seen as a CSF cleft between the 2 leaves of the septum pellucidum. An elongated, finger-like posterior continuation of the CSP between the fornices, a cavum vergae (CV), may be associated with a CSP. +- **Lateral ventricle mass**: Choroid plexus cysts (xanthogranulomas) are a common, generally age-related, degenerative finding with no clinical significance. They are nonneoplastic noninflammatory cysts, usually bilateral with rim calcification, may be hyperintense on FLAIR MR, and 60-80% appear bright on DWI. A strongly enhancing choroid plexus mass in a child is most likely a choroid plexus papilloma. With the exception of the 4th ventricle, a choroid plexus mass in an adult is usually meningioma or metastasis, not a choroid plexus papilloma. +- Some lateral ventricle lesions display a distinct predilection for specific sublocations within the lateral ventricles. An innocent-appearing frontal horn mass in a middle-aged or older adult is most often a subependymoma. A "bubbly" mass in the body of the lateral ventricle is usually a central neurocytoma. Neurocysticercosis cysts can occur in all ages and in virtually every CSF space. +- **Foramen of Monro mass**: The most common "abnormality" here is a pseudolesion caused by CSF pulsation artifact. A colloid cyst is the only relatively common pathology here. It is rare in children and is typically a lesion of adults. Flow artifact can mimic a colloid cyst, but mass effect is absent. In a child with an enhancing mass in the interventricular foramen, tuberous sclerosis with subependymal nodule &/or giant cell astrocytoma should be a consideration. Masses such as an ependymoma, papilloma, and metastasis are rare. +- **3rd ventricle mass**: Again, the most common "lesion" in this location is either CSF flow artifact or a normal structure (the massa intermedia). A colloid cyst is the only common lesion that occurs in the 3rd ventricle; 99% are wedged into the foramen of Monro. Extreme vertebrobasilar dolichoectasia can indent the 3rd ventricle, sometimes projecting upward as high as the interventricular foramen, and should not be mistaken for colloid cyst. +- Primary neoplasms in children are uncommon here but include choroid plexus papilloma, germinoma, craniopharyngioma, and a sessile-type tuber cinereum hamartoma. Primary neoplasms of the 3rd ventricle in adults are also uncommon, though an intraventricular macroadenoma and chordoid glioma are examples. +- **Cerebral aqueduct**: Other than aqueductal stenosis, intrinsic lesions of the cerebral aqueduct are rare. Most are related to masses in adjacent structures (e.g., tectal plate glioma). +- **4th ventricle mass**: Pediatric masses are the most common intrinsic abnormalities of the 4th ventricle. Medulloblastoma, ependymoma, and astrocytoma predominate. Atypical teratoid/rhabdoid tumor (AT/RT) is a less common neoplasm that may occur here. It usually occurs in children under the age of 3 and can mimic medulloblastoma. +- Metastases to the choroid or ependyma are probably the most common 4th ventricle neoplasm of adults. Choroid plexus papilloma does occur here as well as in the CPA cistern. Subependymoma is a lesion of middle-aged adults that is found in the inferior 4th ventricle, lying behind the pontomedullary junction. A newly described rare neoplasm, rosette-forming glioneuronal tumor, is a midline mass of the 4th ventricle. It has no particular distinguishing imaging features and, although it may appear aggressive, it is a benign (WHO grade 1) lesion. Hemangioblastomas are intraaxial masses but may project into the 4th ventricle. Epidermoid cysts and neurocysticercosis cysts can be found in all ages. +- ## SASs and Cisterns +- **Overview**: The SASs are a common site of pathology that varies from benign congenital lesions (such as arachnoid cyst) to infection (meningitis) and neoplastic involvement ("carcinomatous meningitis"). Anatomic location is key to the differential diagnosis, as imaging findings, such as enhancement and hyperintensity on FLAIR MR, are often nonspecific. +- **Normal variants**: CSF flow-related artifacts are common, especially in the basal cisterns on FLAIR MR. Mega cisterna magna may be considered a normal variant, as is a cavum velum interpositum (CVI). A CVI is a thin, triangular-shaped CSF space between the lateral ventricles that lies below the fornices and above the 3rd ventricle. Occasionally, a CVI may become quite large. +- **Suprasellar cistern mass**: Common masses in adults are upward extensions of macroadenoma, meningioma, and aneurysm. The 2 most common suprasellar masses in children are astrocytoma of the optic chiasm/hypothalamus and craniopharyngioma. +- **Cerebellopontine angle mass**: In adults, vestibular schwannoma accounts for almost 90% of all CPA-internal auditory canal (IAC) masses. A meningioma, epidermoid cyst, aneurysm, and arachnoid cyst **together** represent ~ 8% of lesions in this location. All other less common entities, such as lipoma, schwannomas of other cranial nerves, metastases, neurenteric cysts, etc., account for ~ 2%. +- In the absence of neurofibromatosis type 2, vestibular schwannomas are very rare in children. CPA epidermoid and arachnoid cysts may occur in children. Extension of ependymoma laterally through the foramina of Luschka may involve the CPA. +- Cystic-appearing CPA masses comprise their own special differential diagnosis. While vestibular schwannoma with intramural cysts can occur, it is less common than epidermoid and arachnoid cysts. Neurocysticercosis may occasionally involve the CPA. Large endolymphatic sac anomaly (IP-2) shows a CSF-like mass within the posterior wall of the temporal bone. Hemangioblastoma and neurenteric cysts are other less common cystic masses that occur in the CPA. +- **Cisterna magna mass**: Tonsillar herniation, whether congenital (Chiari 1) or secondary to posterior fossa mass effect or intracranial hypotension, is the most common "mass" in this location. Nonneoplastic cysts (arachnoid, epidermoid, dermoid, neurenteric) may also occur here. +- Neoplasms in and around the cisterna magna, such as meningioma and metastasis, are typically anterior to the medulla. Subependymoma of the 4th ventricle originates in the obex and lies behind the medulla. +- **FLAIR MR hyperintensity**: Hyperintense sulci and SASs are seen with MR artifacts as well as a variety of lesions. Pathologic FLAIR MR hyperintensity is typically related to blood (e.g., subarachnoid hemorrhage), protein (meningitis), or cells (pia-SAS metastases). Less commonly, gadolinium-based contrast agents in patients with blood-brain barrier leakage or renal failure can cause FLAIR MR hyperintensity. +- Rare causes of FLAIR MR hyperintensity include a ruptured dermoid cyst, moyamoya (ivy sign), and acute cerebral ischemia. Contrast enhancement helps distinguish meningitis and metastases from subarachnoid hemorrhage and CSF artifacts. + + 91dc714e-682b-4d02-a8c5-c6decb8bc1ba + +## References + +# Selected References + +1. [Adigun OO et al: Anatomy, head and neck, cerebrospinal fluid, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=29083815%5Bpmid%5D) +1. [Altafulla J et al: The basal subarachnoid cisterns: surgical and anatomical considerations. World Neurosurg. 129:190-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31136838%5Bpmid%5D) +1. [Korzh V: Development of brain ventricular system. Cell Mol Life Sci. 75(3):375-83, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=28780589%5Bpmid%5D) +1. [Tumani H et al: The cerebrospinal fluid and barriers - anatomic and physiologic considerations. Handb Clin Neurol. 146:21-32, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29110772%5Bpmid%5D) +1. [Sakka L et al: Anatomy and physiology of cerebrospinal fluid. Eur Ann Otorhinolaryngol Head Neck Dis. 128(6):309-16, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=22100360%5Bpmid%5D) +1. [Lowery LA et al: Totally tubular: the mystery behind function and origin of the brain ventricular system. Bioessays. 31(4):446-58, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19274662%5Bpmid%5D) +1. [Barshes N et al: Anatomy and physiology of the leptomeninges and CSF space. Cancer Treat Res. 125:1-16, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16211880%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Schematic 3D representation of the ventricular system, viewed in the sagittal plane, demonstrates the normal appearance and communicating pathways of the cerebral ventricles.](images/app.statdx.com_image_thumbnail_18da13b4-cc47-4475-9b99-987ece9e9550_annotated_false_size_900_quality_90_aaad2f37_20251018T164921Z.jpg) +*Schematic 3D representation of the ventricular system, viewed in the sagittal plane, demonstrates the normal appearance and communicating pathways of the cerebral ventricles.* + +![Schematic 3D representation of the ventricular system, viewed in the sagittal plane, demonstrates the normal appearance and communicating pathways of the cerebral ventricles.](images/app.statdx.com_image_thumbnail_18da13b4-cc47-4475-9b99-987ece9e9550_size_174_quality_85_a814c085_20251018T165117Z.jpg) +*Schematic 3D representation of the ventricular system, viewed in the sagittal plane, demonstrates the normal appearance and communicating pathways of the cerebral ventricles.* + +![Sagittal midline graphic through the interhemispheric fissure depicts the subarachnoid spaces (SASs) with CSF (blue) between the arachnoid (purple) and pia (orange). The central sulcus separates the frontal lobe (anterior) from the parietal lobe (posterior). The pia mater is closely applied to the brain surface, whereas the arachnoid is adherent to the dura. The ventricles communicate with the cisterns and SAS via the foramina of Luschka and Magendie. The cisterns normally communicate freely with each other.](images/app.statdx.com_image_thumbnail_d0fd3b0a-6e5b-4d8d-8ff9-018a2701429a_annotated_false_size_900_quality_90_2677fbfc_20251018T164921Z.jpg) +*Sagittal midline graphic through the interhemispheric fissure depicts the subarachnoid spaces (SASs) with CSF (blue) between the arachnoid (purple) and pia (orange). The central sulcus separates the frontal lobe (anterior) from the parietal lobe (posterior). The pia mater is closely applied to the brain surface, whereas the arachnoid is adherent to the dura. The ventricles communicate with the cisterns and SAS via the foramina of Luschka and Magendie. The cisterns normally communicate freely with each other.* + +![Axial T2 MR demonstrates normal anatomy at the level of the lateral ventricles. The frontal horns of the lateral ventricle are separated by a tiny septi pellucidi . Note the foramen of Monro connecting the lateral ventricles to the 3rd ventricle .](images/app.statdx.com_image_thumbnail_4288ccd4-2baa-4fad-a804-777de6cf9758_annotated_true_size_900_quality_90_6b61e4ac_20251018T164921Z.jpg) +*Axial T2 MR demonstrates normal anatomy at the level of the lateral ventricles. The frontal horns of the lateral ventricle are separated by a tiny septi pellucidi . Note the foramen of Monro connecting the lateral ventricles to the 3rd ventricle .* + +![Axial T2 MR at the level of the cerebral aqueduct demonstrates the infundibular recess of the 3rd ventricle , mammillary bodies , interpeduncular cistern , and the quadrigeminal cisterns .](images/app.statdx.com_image_thumbnail_9ffc389c-d960-42d5-8503-304234193f7a_annotated_true_size_900_quality_90_0a8a0203_20251018T164921Z.jpg) +*Axial T2 MR at the level of the cerebral aqueduct demonstrates the infundibular recess of the 3rd ventricle , mammillary bodies , interpeduncular cistern , and the quadrigeminal cisterns .* + +![Axial T2 MR at the level of the 4th ventricular outlet shows the foramen of Magendie and foramina of Luschka .](images/app.statdx.com_image_thumbnail_240fe702-9e39-4bb4-8b55-39c0a4dd83a7_annotated_true_size_900_quality_90_924b302b_20251018T164921Z.jpg) +*Axial T2 MR at the level of the 4th ventricular outlet shows the foramen of Magendie and foramina of Luschka .* + +![Sagittal FEISTA MR demonstrates the lateral ventricle , chiasmatic and infundibular recesses of the 3rd ventricle, the cerebral aqueduct , and the fastigium of the 4th ventricle. The floor of the 3rd ventricle in formed from front to back by the optic chiasm, hypothalamus, mammillary bodies, and roof of the midbrain tegmentum.](images/app.statdx.com_image_thumbnail_fc362fbe-b32d-422b-b185-247deeb14c6e_annotated_true_size_900_quality_90_d4614ba6_20251018T164921Z.jpg) +*Sagittal FEISTA MR demonstrates the lateral ventricle , chiasmatic and infundibular recesses of the 3rd ventricle, the cerebral aqueduct , and the fastigium of the 4th ventricle. The floor of the 3rd ventricle in formed from front to back by the optic chiasm, hypothalamus, mammillary bodies, and roof of the midbrain tegmentum.* + +![Axial NECT (L) and axial T2 MR (R) shows normal asymmetry of the lateral ventricles with the right being larger than the left. The septi pellucidi are slightly bowed and displaced across the midline. When there is lateral ventricular asymmetry, it is important to scrutinize the region of foramen of Monro to exclude any obstructing pathology.](images/app.statdx.com_image_thumbnail_4941a673-d28c-47c0-bea9-92d4cd99f431_annotated_true_size_900_quality_90_935169e1_20251018T164921Z.jpg) +*Axial NECT (L) and axial T2 MR (R) shows normal asymmetry of the lateral ventricles with the right being larger than the left. The septi pellucidi are slightly bowed and displaced across the midline. When there is lateral ventricular asymmetry, it is important to scrutinize the region of foramen of Monro to exclude any obstructing pathology.* + +![Axial FLAIR MR in a patient with hydrocephalus demonstrates focal hyperintensity in the 3rd ventricle due to a pseudomasses caused by pulsatile CSF.](images/app.statdx.com_image_thumbnail_5710a471-d62a-4c03-af96-60870e2354c1_annotated_true_size_900_quality_90_f4c0b8b2_20251018T164921Z.jpg) +*Axial FLAIR MR in a patient with hydrocephalus demonstrates focal hyperintensity in the 3rd ventricle due to a pseudomasses caused by pulsatile CSF.* + +![Axial T2 MR shows a large ventricular mass in the frontal horn and anterior body of the right lateral ventricle. There is dilatation of the posterior body of the right lateral ventricle and displacement of the septi pellucidi to the left. On histopathology, this was a central neurocytoma.](images/app.statdx.com_image_thumbnail_524494cb-37a1-444b-83cb-e4ffd7d6d101_annotated_true_size_900_quality_90_930f6a47_20251018T164921Z.jpg) +*Axial T2 MR shows a large ventricular mass in the frontal horn and anterior body of the right lateral ventricle. There is dilatation of the posterior body of the right lateral ventricle and displacement of the septi pellucidi to the left. On histopathology, this was a central neurocytoma.* + +![Axial FLAIR MR demonstrates an intraventricular neurocysticercosis in the posterior 3rd ventricle with dilatation of the anterior 1/3 and lateral ventricles. Note the mild periventricular interstitial edema .](images/app.statdx.com_image_thumbnail_657cbe5a-415a-418b-a61b-7a0b96284a65_annotated_true_size_900_quality_90_801c6a60_20251018T164926Z.jpg) +*Axial FLAIR MR demonstrates an intraventricular neurocysticercosis in the posterior 3rd ventricle with dilatation of the anterior 1/3 and lateral ventricles. Note the mild periventricular interstitial edema .* + +![Axial DWI (L) and axial FLAIR MR (R) shows characteristic large choroid plexus cysts in the atria of both lateral ventricles. Choroid plexus cysts, often called choroid plexus xanthogranuloma, are nonneoplastic noninflammatory cysts and are often bright on DWI.](images/app.statdx.com_image_thumbnail_63bee34f-4b15-4df5-9184-f331532989da_annotated_true_size_900_quality_90_7af97812_20251018T164926Z.jpg) +*Axial DWI (L) and axial FLAIR MR (R) shows characteristic large choroid plexus cysts in the atria of both lateral ventricles. Choroid plexus cysts, often called choroid plexus xanthogranuloma, are nonneoplastic noninflammatory cysts and are often bright on DWI.* + +![Sagittal T1 C+ MR in a 8-year-old child with ataxia demonstrates a large, heterogeneously enhancing mass centered in the 4th ventricle. Pathology revealed a classic medulloblastoma, Wnt-activated.](images/app.statdx.com_image_thumbnail_a24ea68a-9b86-47df-91c0-fe5cb9736415_annotated_true_size_900_quality_90_1f86202e_20251018T164928Z.jpg) +*Sagittal T1 C+ MR in a 8-year-old child with ataxia demonstrates a large, heterogeneously enhancing mass centered in the 4th ventricle. Pathology revealed a classic medulloblastoma, Wnt-activated.* + +![Axial FLAIR MR in a patient with acute cortical subarachnoid hemorrhage due to cortical venous thrombosis shows high signal in the left frontal sulci.](images/app.statdx.com_image_thumbnail_bbab6561-190c-4c36-83c8-32eda9785a39_annotated_true_size_900_quality_90_2e85ab8a_20251018T164928Z.jpg) +*Axial FLAIR MR in a patient with acute cortical subarachnoid hemorrhage due to cortical venous thrombosis shows high signal in the left frontal sulci.* + +![Axial FLAIR MR in a patient with chronic renal disease who received IV gadolinium 48 hours prior shows marked FLAIR hyperintensity in the cortical sulci. Sulcal hyperintensity on FLAIR can be caused by pia-subarachnoid metastases, blood, protein (meningitis), high oxygen content, and retained contrast (renal failure, as in this case).](images/app.statdx.com_image_thumbnail_90af95ee-2e00-4f2a-a832-2fec510f8150_annotated_true_size_900_quality_90_950a63d1_20251018T164929Z.jpg) +*Axial FLAIR MR in a patient with chronic renal disease who received IV gadolinium 48 hours prior shows marked FLAIR hyperintensity in the cortical sulci. Sulcal hyperintensity on FLAIR can be caused by pia-subarachnoid metastases, blood, protein (meningitis), high oxygen content, and retained contrast (renal failure, as in this case).* + + +### Additional Images + +![Axial T1 C+ MR in an 18-month-old child with severe hydrocephalus shows a choroid plexus papilloma (CPP). The intensely enhancing frond-like projections and location in the atrium of the left lateral ventricle are both classic findings.](images/app.statdx.com_image_thumbnail_d844bf7c-09b7-49ce-8ee8-e7cf2890dfc4_annotated_true_size_900_quality_90_f57411b5_20251018T164929Z.jpg) +*Axial T1 C+ MR in an 18-month-old child with severe hydrocephalus shows a choroid plexus papilloma (CPP). The intensely enhancing frond-like projections and location in the atrium of the left lateral ventricle are both classic findings.* + +![Axial T1 C+ MR in a middle-aged woman shows a smoothly lobulated, intensely enhancing choroid plexus mass . CPP in adults is rare, except for the 4th ventricle. Meningioma was found at surgery.](images/app.statdx.com_image_thumbnail_fa650ed7-2e34-45bb-ab32-d3e7cf96dd81_annotated_true_size_900_quality_90_dbd4ffd2_20251018T164929Z.jpg) +*Axial T1 C+ MR in a middle-aged woman shows a smoothly lobulated, intensely enhancing choroid plexus mass . CPP in adults is rare, except for the 4th ventricle. Meningioma was found at surgery.* + +![Axial T1 C+ FS MR in a 72-year-old man with declining mental state shows a nonenhancing mass in the frontal horn of the left lateral ventricle. This is an incidental finding, most likely a subependymoma.](images/app.statdx.com_image_thumbnail_8f87abeb-662f-4047-9f74-66034afca89f_annotated_true_size_900_quality_90_714f2c9a_20251018T164929Z.jpg) +*Axial T1 C+ FS MR in a 72-year-old man with declining mental state shows a nonenhancing mass in the frontal horn of the left lateral ventricle. This is an incidental finding, most likely a subependymoma.* + +![Axial T1 C+ MR in a 33 year old with headaches shows an inhomogeneously enhancing bubbly lesion in the body of the left lateral ventricle. The appearance and location distinguish a central neurocytoma from subependymoma and other possible lateral ventricular masses, such as a meningioma.](images/app.statdx.com_image_thumbnail_5b8a7998-bb55-4ff4-a940-66e19336b5df_annotated_true_size_900_quality_90_47653a8b_20251018T164929Z.jpg) +*Axial T1 C+ MR in a 33 year old with headaches shows an inhomogeneously enhancing bubbly lesion in the body of the left lateral ventricle. The appearance and location distinguish a central neurocytoma from subependymoma and other possible lateral ventricular masses, such as a meningioma.* + +![Axial FLAIR MR in a 3 year old with seizures shows a hyperintense mass at the interventricular foramen. Note the flame-shaped subcortical hyperintensities . This is tuberous sclerosis with a subependymal giant cell astrocytoma.](images/app.statdx.com_image_thumbnail_d3712d46-4bd1-4dfa-acf0-d8c5a5f5e99a_annotated_true_size_900_quality_90_e6d92377_20251018T164929Z.jpg) +*Axial FLAIR MR in a 3 year old with seizures shows a hyperintense mass at the interventricular foramen. Note the flame-shaped subcortical hyperintensities . This is tuberous sclerosis with a subependymal giant cell astrocytoma.* + +![Axial FLAIR MR in a 65 year old with a "thunderclap" headache shows a foramen of Monro mass that is a colloid cyst. Other than CSF flow artifact, colloid cysts are the most common lesion found in this location. They are common in adults but relatively rare in children.](images/app.statdx.com_image_thumbnail_cf06d949-a6c8-48ed-a03d-fcb179f922ae_annotated_true_size_900_quality_90_70746469_20251018T164929Z.jpg) +*Axial FLAIR MR in a 65 year old with a "thunderclap" headache shows a foramen of Monro mass that is a colloid cyst. Other than CSF flow artifact, colloid cysts are the most common lesion found in this location. They are common in adults but relatively rare in children.* + +![Coronal T1WI MR shows prominent pseudomasses of the 3rd and lateral ventricles caused by pulsatile CSF in and around the interventricular foramen (of Monro). Note the propagation of phase artifact across the image.](images/app.statdx.com_image_thumbnail_d7cf19d1-466f-4c2a-9466-cfab49680886_annotated_true_size_900_quality_90_298e5a3c_20251018T164929Z.jpg) +*Coronal T1WI MR shows prominent pseudomasses of the 3rd and lateral ventricles caused by pulsatile CSF in and around the interventricular foramen (of Monro). Note the propagation of phase artifact across the image.* + +![Axial T1WI C+ MR shows large lateral 3rd ventricles with "blurred" margins from transependymal CSF flow. A cysticercus cyst with scolex causes obstructive hydrocephalus. Intrinsic 3rd ventricle masses are less common than lateral or 4th ventricular lesions.](images/app.statdx.com_image_thumbnail_7164036e-4620-4279-92d5-d9aac20687cb_annotated_true_size_900_quality_90_33feec68_20251018T164930Z.jpg) +*Axial T1WI C+ MR shows large lateral 3rd ventricles with "blurred" margins from transependymal CSF flow. A cysticercus cyst with scolex causes obstructive hydrocephalus. Intrinsic 3rd ventricle masses are less common than lateral or 4th ventricular lesions.* + +![Sagittal T1WI C+ MR in a 2 year old with ataxia, nausea, and vomiting shows a lobulated enhancing mass in the 4th ventricle . Fourth ventricle masses in children are usually primitive neuroectodermal tumor or ependymoma, less often atypical teratoid-rhabdoid tumor which was found at surgery.](images/app.statdx.com_image_thumbnail_4f5f99a5-2e74-441d-aebf-641717636700_annotated_true_size_900_quality_90_7b85c1c4_20251018T164930Z.jpg) +*Sagittal T1WI C+ MR in a 2 year old with ataxia, nausea, and vomiting shows a lobulated enhancing mass in the 4th ventricle . Fourth ventricle masses in children are usually primitive neuroectodermal tumor or ependymoma, less often atypical teratoid-rhabdoid tumor which was found at surgery.* + +![Sagittal T1WI C+ MR in a 52-year-old woman with episodic headaches, nausea, and vomiting shows an intensely enhancing 4th ventricle mass. This proved to be choroid plexus papilloma.](images/app.statdx.com_image_thumbnail_3be18417-401b-4732-9fcb-68d0389a6a9e_annotated_true_size_900_quality_90_4d8f9d0b_20251018T164930Z.jpg) +*Sagittal T1WI C+ MR in a 52-year-old woman with episodic headaches, nausea, and vomiting shows an intensely enhancing 4th ventricle mass. This proved to be choroid plexus papilloma.* + +![Axial FLAIR MR shows multifocal sulcal hyperintensities caused by aneurysmal subarachnoid hemorrhage.](images/app.statdx.com_image_thumbnail_93e84051-219a-4618-950d-097de4af3dfc_annotated_true_size_900_quality_90_d3383237_20251018T164931Z.jpg) +*Axial FLAIR MR shows multifocal sulcal hyperintensities caused by aneurysmal subarachnoid hemorrhage.* + +![Axial FLAIR MR shows artifactual hyperintensity in the occipital sulci secondary to incomplete CSF suppression. A repeat scan (not shown) was normal. Sulcal hyperintensity on FLAIR is nonspecific and can be caused by pia-subarachnoid metastases, blood, protein (meningitis), high oxygen content, retained contrast (renal failure), and artifact (as in this case).](images/app.statdx.com_image_thumbnail_29e5ed04-7404-4c85-8879-6934b62d9243_annotated_true_size_900_quality_90_73fe8c83_20251018T164931Z.jpg) +*Axial FLAIR MR shows artifactual hyperintensity in the occipital sulci secondary to incomplete CSF suppression. A repeat scan (not shown) was normal. Sulcal hyperintensity on FLAIR is nonspecific and can be caused by pia-subarachnoid metastases, blood, protein (meningitis), high oxygen content, retained contrast (renal failure), and artifact (as in this case).* + +![Sagittal T2 SPACE MR shows the normal flow void due to CSF flow at the cerebral aqueduct and foramen of Magendie . Note the chiasmatic and infundibular recesses of the 3rd ventricle and fastigium of the 4th ventricle.](images/app.statdx.com_image_thumbnail_fa347901-c1cf-4049-a7fb-9cd96a14a1c0_annotated_true_size_900_quality_90_e67cc834_20251018T164931Z.jpg) +*Sagittal T2 SPACE MR shows the normal flow void due to CSF flow at the cerebral aqueduct and foramen of Magendie . Note the chiasmatic and infundibular recesses of the 3rd ventricle and fastigium of the 4th ventricle.* + +![Axial T2 MR shows normal asymmetry of the lateral ventricles with the right being larger than the left. The septi pellucidi are slightly bowed and displaced across the midline. When there is lateral ventricular asymmetry, it is important to scrutinize the region of foramen of Monro to exclude any obstructing pathology.](images/app.statdx.com_image_thumbnail_5bd78d24-30b7-4af5-bd73-be49ef2afcff_annotated_true_size_900_quality_90_fc43cd81_20251018T164931Z.jpg) +*Axial T2 MR shows normal asymmetry of the lateral ventricles with the right being larger than the left. The septi pellucidi are slightly bowed and displaced across the midline. When there is lateral ventricular asymmetry, it is important to scrutinize the region of foramen of Monro to exclude any obstructing pathology.* + +![Axial FLAIR MR in a patient with hydrocephalus demonstrates a prominent pseudomasses of the 3rd ventricle caused by pulsatile CSF.](images/app.statdx.com_image_thumbnail_e6176e42-b6e2-492b-a896-ce2fc19efdd4_annotated_true_size_900_quality_90_c16dc82b_20251018T164931Z.jpg) +*Axial FLAIR MR in a patient with hydrocephalus demonstrates a prominent pseudomasses of the 3rd ventricle caused by pulsatile CSF.* + +![Sagittal T1 C+ MR shows a large homogenously enhancing 4th ventricular mass , which on pathology was a meningioma. There is dilatation of the ventricular system proximal to the mass.](images/app.statdx.com_image_thumbnail_530cce92-dd05-4a8e-af12-e451a270b370_annotated_true_size_900_quality_90_b1421f8a_20251018T164931Z.jpg) +*Sagittal T1 C+ MR shows a large homogenously enhancing 4th ventricular mass , which on pathology was a meningioma. There is dilatation of the ventricular system proximal to the mass.* + +![Axial DWI MR shows characteristic large choroid plexus cysts in the atria of both lateral ventricles within the choroid plexus glomi. Choroid plexus cysts, often called choroid plexus xanthogranuloma, are nonneoplastic noninflammatory cysts. Between 60-80% appear quite bright on DWI, as in this case.](images/app.statdx.com_image_thumbnail_82e0f8d0-567d-469d-b620-d1d2c95866bd_annotated_true_size_900_quality_90_852aa16e_20251018T164931Z.jpg) +*Axial DWI MR shows characteristic large choroid plexus cysts in the atria of both lateral ventricles within the choroid plexus glomi. Choroid plexus cysts, often called choroid plexus xanthogranuloma, are nonneoplastic noninflammatory cysts. Between 60-80% appear quite bright on DWI, as in this case.* + +![Axial FLAIR MR in a patient with acute subarachnoid hemorrhage due to aneurysm rupture shows high signal in the left sylvian fissure and posterior cortical sulci .](images/app.statdx.com_image_thumbnail_3957aec8-23c1-4e4f-8bd2-8527c8bc9a6a_annotated_true_size_900_quality_90_9747d5ed_20251018T164931Z.jpg) +*Axial FLAIR MR in a patient with acute subarachnoid hemorrhage due to aneurysm rupture shows high signal in the left sylvian fissure and posterior cortical sulci .* + diff --git a/docs_md/articles/ventriculomegaly_f40bd6eb-e7e5-498a-8bde-ad6bcd546f21.md b/docs_md/articles/ventriculomegaly_f40bd6eb-e7e5-498a-8bde-ad6bcd546f21.md new file mode 100644 index 0000000..38b6c69 --- /dev/null +++ b/docs_md/articles/ventriculomegaly_f40bd6eb-e7e5-498a-8bde-ad6bcd546f21.md @@ -0,0 +1,317 @@ +--- +title: "Ventriculomegaly" +docid: "f40bd6eb-e7e5-498a-8bde-ad6bcd546f21" +authors: + - key: "47381de4-c9fd-4999-8dd0-1808cd72db6b" + value: "Luke L. Linscott, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60" + - + name: "Ventricles, Periventricular Regions" + slug: "ventricles-periventricular-regions" + treeNodeId: "353c434a-a6fc-4ef1-8786-d30a1988a4dc" + - + name: "Generic Imaging Patterns" + slug: "generic-imaging-patterns" + treeNodeId: "969c31a2-ef56-4fc3-9125-05857cf9aac3" + - + name: "Ventriculomegaly" + slug: "ventriculomegaly" + treeNodeId: null +category: "Brain" +documentVersionId: "69dd2446-06ad-4f37-bba3-f59d4c5d40b8" +imageCount: 26 +lastUpdated: "01/23/23" +pageDescription: "Ventriculomegaly" +pageKeywords: "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Ventriculomegaly" +pageTitle: "Ventriculomegaly | STATdx" +enhancedTitle: "Ventriculomegaly" +type: "DDX" +references: true +breadcrumbs: + - "Brain" + - "Differential Diagnosis" + - "Ventricles, Periventricular Regions" + - "Generic Imaging Patterns" + - "Ventriculomegaly" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - 2 main causes of ventriculomegaly + - ↑ CSF pressures → hydrocephalus + - ↓ brain volume → passive ventricular enlargement + - Clinical features to consider + - Head circumference is critical to interpreting significance of enlarged ventricles + - ↑ head size: Hydrocephalus + - ↓ head size: Brain parenchymal volume loss + - Patients may come to clinical attention during prenatal screening or well-child check-ups + - Imaging features of hydrocephalus + - Etiologies to consider + - Obstruction usually occurs at cerebral aqueduct, foramen of Monro, or 4th ventricular outlets + - e.g., aqueductal stenosis, obstructing tumor + - Communicating hydrocephalus is due to CSF overproduction or poor CSF resorption + - e.g., choroid plexus papilloma, benign enlargement of subarachnoid spaces + - Some etiologies can cause either or mixed forms + - e.g., posthemorrhagic, postinfectious, vein of Galen malformation (VGAM) + - Imaging signs of hydrocephalus + - Macrocephaly: ↑ craniofacial ratio + - Rounded margins of lateral ventricles, depressed floor of 3rd ventricle + - Transependymal edema: Periventricular white matter (WM) ↑ T2/FLAIR signal, greatest at frontal & occipital horns; effacement of cerebral sulci & basilar cisterns + - Imaging features of volume loss + - Etiologies to consider + - Diffuse vs. focal cortical ischemia + - e.g., hypoxic-ischemic encephalopathy (HIE) vs. arterial ischemic stroke + - Symmetric vs. focal WM volume loss + - e.g., periventricular leukomalacia vs. porencephaly + - Imaging signs of brain parenchymal volume loss + - Microcephaly: ↓ craniofacial ratio + - Angular margins of lateral ventricles + - Prominent sulci & basilar cisterns + - Modality considerations + - Fetal US for detection of in utero ventriculomegaly + - Fetal MR is excellent for underlying/associated anomalies + - US is important tool for initial evaluation of neonates/infants with suspected ventriculomegaly + - Requires open anterior fontanelle + - CT is often 1st-line modality in acute presentations + - MR is most definitive test for determination of underlying cause of ventriculomegaly + - Best test for determining cause of hydrocephalus + - Best evaluation of brain parenchyma to detect possible volume loss +- ## Helpful Clues for Common Diagnoses + + + - **Fetal Ventriculomegaly** + - Likelihood of normal neurologic development based on lateral ventricular size in utero + - Mild (10-12 mm): > 90% + - Moderate (13-15 mm): 80-93% + - Severe (> 15 mm): ~ 62% + - Underlying causes of fetal ventriculomegaly + - Infectious (e.g., CMV), genetic (e.g., trisomy), malformations (e.g., Chiari 2) + - CMV testing & amniocentesis typically performed + - Obstructive causes considered separately + - **Benign Enlargement of Subarachnoid Spaces** + - a.k.a. benign hydrocephalus of infancy, benign macrocrania of infancy, external hydrocephalus + - Enlarged subarachnoid spaces (SAS) ± mild ventriculomegaly + - Findings develop in 1st few months of life & usually resolve by ~ 2 years of age + - Often family history of macrocephaly + - Normal development or mild developmental delay + - Imaging: Prominent SAS over frontal & parietal convexities with normal vessels traversing SAS + - No displacement of arachnoid membrane from dura (i.e., no subdural fluid) + - **Chiari 2 Malformation** + - Brain malformation due to open neural tube defect (most commonly lumbosacral myelomeningocele) + - Hydrocephalus requiring shunting occurs almost universally without in utero surgical repair + - Imaging: Small posterior fossa, inferior migration of cerebellum/brainstem, beaked tectum, dysplastic corpus callosum, scalloping of dorsal clivus + - **Aqueductal Stenosis** + - Congenital atresia/stenosis of cerebral aqueduct + - Imaging: Lateral & 3rd ventricle dilation with normal 4th ventricle + - Narrowed/absent cerebral aqueduct on 3D SSFP MR + - Lack of flow void within cerebral aqueduct on T2 + - **Acquired Hydrocephalus** + - 2 main types: Communicating & noncommunicating + - Communicating: ↑ production or ↓ resorption of CSF + - Obstructive: Blockage of ventricular outlets + - Depending on cause, may present enlarging head or signs of ↑ intracranial pressure + - May have signs of ↑ intracranial pressure + - Treatment: Shunting or 3rd ventriculostomy + - **Posthemorrhagic hydrocephalus** + - Most common in premature infants with germinal matrix/intraventricular hemorrhage (IVH) + - Look for hemosiderin along ventricles/brainstem + - SWI/GRE is most sensitive for detection of prior IVH + - **Acute infectious hydrocephalus** + - During acute bacterial infection, ↑ head size may occur due to ↑ extraaxial spaces from meningitis & subdural/epidural empyema + - **Postinfectious hydrocephalus** + - Obstruction may occur due to synechiae + - Communicating hydrocephalus may occur due to arachnoid granulation dysfunction + - **Obstructing tumor** + - Smaller tumors in critical locations may obstruct + - e.g., subependymal giant cell astrocytoma (SEGA) at foramen of Monro, tectal plate glioma at cerebral aqueduct + - Larger tumors anywhere in brain may obstruct, particularly in posterior fossa + - **CSF overproduction (choroid plexus tumors)** + - Choroid plexus papilloma/carcinoma causes CSF overproduction + - Inability to resorb excess CSF → ↑ ventricular size + - **Brain Volume Loss** + - Results in ventricular enlargement + - Focal (e.g., porencephaly, stroke) + - Symmetric (e.g., periventricular leukomalacia, metabolic disease) + - **Hypoxic-ischemic injury** + - Often results in symmetric or asymmetric brain volume loss & associated ventricular enlargement + - Look for characteristic patterns of injury (e.g., perirolandic or watershed) + - **Arterial ischemic stroke** + - Most often occurs in perinatal period or related to embolic phenomenon + - Encephalomalacia develops in arterial territory with focal ventricular enlargement + - **Periventricular leukomalacia** + - Sequelae of WM injury of prematurity + - Usually symmetric WM volume loss with little or no associated gliosis (↑ FLAIR signal) + - **Porencephaly** + - Most often results from grade 4 IVH, but any insult early in development may cause porencephaly + - Porencephaly develops rather than encephalomalacia as no astrocytic response occurs in very young brains + - **Metabolic brain disease** + - Numerous diseases cause significant brain volume loss, resulting in ventriculomegaly + - Usually symmetric; may be + - Frontal predominant (e.g., Alexander disease) + - Posterior predominant (e.g., X-linked adrenoleukodystrophy) + - Diffuse (e.g., metachromatic leukodystrophy) +- ## Helpful Clues for Less Common Diagnoses + + + - **Hemimegalencephaly** + - Unilateral abnormal neuronal proliferation & migration + - Neonate/infant: ↑ T1 & ↓ T2 in WM with blurred gray matter/WM junctions + - Often shows enlarged ipsilateral ventricle + - **Vein of Galen Aneurysmal Malformation** + - Ectatic vascular structure is median prosencephalic vein, not vein of Galen, which never forms + - High-flow vascular malformation due to direct communication between arteries & median prosencephalic vein + - Ventricular enlargement may occur due to + - Mass effect of malformation on cerebral aqueduct + - ↑ venous pressure & poor CSF resorption + - Parenchymal insult with volume loss + - After birth, ↑ in blood flow through malformation + - Up to 80% of left ventricular output may supply brain + - Leads to ↑ cardiac output & heart failure + - **Dandy-Walker Malformation** + - Vermian hypoplasia with large posterior fossa cyst that communicates with 4th ventricle but not SAS + - Lambdoid-torcular inversion + - Often associated with hydrocephalus + - **Hydranencephaly** + - In utero destruction of brain in internal carotid artery territories + - May cause poor CSF regulation & hydrocephalus, requiring shunting + - **Holoprosencephaly** + - Holoprosencephaly (alobar form) often develops ↑ size of monoventricle over time + - Typically treated with CSF shunting + +## References + +# Selected References + +1. [Adiyaman D et al: Contribution of fetal magnetic resonance imaging in the evaluation of neurosonographically detected cases of isolated mild and moderate cerebral ventriculomegaly. J Obstet Gynaecol Res. 48(9):2314-24, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35778980%5Bpmid%5D) +1. [D'Addario V: Diagnostic approach to fetal ventriculomegaly. J Perinat Med. ePub, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36005554%5Bpmid%5D) +1. [Bauer DF et al: Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on the Treatment of Pediatric Hydrocephalus: Update of the 2014 Guidelines. Neurosurgery. 87(6):1071-75, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=34791462%5Bpmid%5D) +1. [Di Mascio D et al: Systematic review and meta-analysis on the role of prenatal magnetic resonance imaging in the era of fetal neurosonography: mild and moderate ventriculomegaly. Ultrasound Obstet Gynecol. 54(2):164-71, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30549340%5Bpmid%5D) +1. [Katz JA et al: Utility of prenatal MRI in the evaluation and management of fetal ventriculomegaly. J Perinatol. 38(11):1444-52, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30158676%5Bpmid%5D) +1. [Orrù E et al: The child with macrocephaly: differential diagnosis and neuroimaging findings. AJR Am J Roentgenol. 210(4):848-59, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29470161%5Bpmid%5D) +1. [Rashid QT et al: Time trends and age-related etiologies of pediatric hydrocephalus: results of a groupwise analysis in a clinical cohort. Childs Nerv Syst. 28(2):221-7, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=21818584%5Bpmid%5D) +1. [Kurihara Y et al: MR imaging of ventriculomegaly--a qualitative and quantitative comparison of communicating hydrocephalus, central atrophy, and normal studies. J Magn Reson Imaging. 5(4):451-6, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7549210%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Transverse oblique US in an 18-week fetus shows mild enlargement of the lateral ventricles (11 mm). If the ventriculomegaly worsens later in pregnancy, fetal or postnatal MR imaging should be obtained.](images/app.statdx.com_image_thumbnail_70468ab8-1e6a-4667-8825-a704fdbf86ce_annotated_true_size_900_quality_90_cfed8e44_20251018T165228Z.jpg) +**Fetal Ventriculomegaly** +*Transverse oblique US in an 18-week fetus shows mild enlargement of the lateral ventricles (11 mm). If the ventriculomegaly worsens later in pregnancy, fetal or postnatal MR imaging should be obtained.* + +![Transverse oblique US in an 18-week fetus shows mild enlargement of the lateral ventricles (11 mm). If the ventriculomegaly worsens later in pregnancy, fetal or postnatal MR imaging should be obtained.](images/app.statdx.com_image_thumbnail_70468ab8-1e6a-4667-8825-a704fdbf86ce_size_174_quality_85_c708c1a0_20251018T155131Z.jpg) +**Fetal Ventriculomegaly** +*Transverse oblique US in an 18-week fetus shows mild enlargement of the lateral ventricles (11 mm). If the ventriculomegaly worsens later in pregnancy, fetal or postnatal MR imaging should be obtained.* + +![Axial T2 SSFSE MR in a fetus shows marked enlargement (> 15 mm) of the lateral ventricles & thinning of the cerebrum . With severe fetal ventriculomegaly (particularly in the setting of aqueductal stenosis), the septum pellucidum (& even the cerebral mantle) may become disrupted.](images/app.statdx.com_image_thumbnail_82f99506-7e41-4801-843b-30dd71594c0b_annotated_true_size_900_quality_90_45cd65ce_20251018T165228Z.jpg) +**Fetal Ventriculomegaly** +*Axial T2 SSFSE MR in a fetus shows marked enlargement (> 15 mm) of the lateral ventricles & thinning of the cerebrum . With severe fetal ventriculomegaly (particularly in the setting of aqueductal stenosis), the septum pellucidum (& even the cerebral mantle) may become disrupted.* + +![Coronal T2 MR in a 1-year-old with macrocephaly shows mild enlargement of the lateral & 3rd ventricles with moderate enlargement of the bifrontal subarachnoid spaces (SAS), which are traversed by normal veins . Mild ventriculomegaly is often seen in benign enlargement of subarachnoid spaces (BESS).](images/app.statdx.com_image_thumbnail_0db6c91f-cab8-4b5c-ac67-4fb4207fefb1_annotated_true_size_900_quality_90_37c9b115_20251018T165228Z.jpg) +**Benign Enlargement of Subarachnoid Spaces** +*Coronal T2 MR in a 1-year-old with macrocephaly shows mild enlargement of the lateral & 3rd ventricles with moderate enlargement of the bifrontal subarachnoid spaces (SAS), which are traversed by normal veins . Mild ventriculomegaly is often seen in benign enlargement of subarachnoid spaces (BESS).* + +![Coronal color Doppler US in a 9-month-old with BESS shows normal vessels coursing through prominent fluid , an expected finding that helps differentiate the SAS from subdural collections.](images/app.statdx.com_image_thumbnail_037fb32e-f70d-4868-8c48-ac7cc17c756d_annotated_true_size_900_quality_90_3febc017_20251018T165228Z.jpg) +**Benign Enlargement of Subarachnoid Spaces** +*Coronal color Doppler US in a 9-month-old with BESS shows normal vessels coursing through prominent fluid , an expected finding that helps differentiate the SAS from subdural collections.* + +![Sagittal 3D SSFP MR in a neonate with a myelomeningocele shows marked enlargement of the lateral ventricles & characteristic features of Chiari 2 malformation: Small posterior fossa, brainstem & cerebellar descent , tectal beaking , & scalloped clivus .](images/app.statdx.com_image_thumbnail_ccc8cc54-8a5d-4831-a355-77f332dd3317_annotated_true_size_900_quality_90_97654240_20251018T165228Z.jpg) +**Chiari 2 Malformation** +*Sagittal 3D SSFP MR in a neonate with a myelomeningocele shows marked enlargement of the lateral ventricles & characteristic features of Chiari 2 malformation: Small posterior fossa, brainstem & cerebellar descent , tectal beaking , & scalloped clivus .* + +![Sagittal T2 MR in a neonate shows massive enlargement of the lateral ventricles due to obstruction at the level of the cerebral aqueduct . The 3rd ventricle is also enlarged , but the 4th ventricle is normal, typical of this disorder.](images/app.statdx.com_image_thumbnail_bba6ec86-c9f5-4828-93d3-e66a48d1f5e7_annotated_true_size_900_quality_90_544f92ca_20251018T165228Z.jpg) +**Aqueductal Stenosis** +*Sagittal T2 MR in a neonate shows massive enlargement of the lateral ventricles due to obstruction at the level of the cerebral aqueduct . The 3rd ventricle is also enlarged , but the 4th ventricle is normal, typical of this disorder.* + +![Sagittal T2 MR in a 1-month-old former premature infant with posthemorrhagic hydrocephalus shows marked enlargement of the lateral , 3rd , & 4th ventricles. Note the dark hemosiderin lining the pial surface of the brainstem from prior intraventricular hemorrhage (IVH).](images/app.statdx.com_image_thumbnail_1507c0c1-9157-4c19-89cf-df575996cf50_annotated_true_size_900_quality_90_5c58dea8_20251018T165228Z.jpg) +**Posthemorrhagic Hydrocephalus** +*Sagittal T2 MR in a 1-month-old former premature infant with posthemorrhagic hydrocephalus shows marked enlargement of the lateral , 3rd , & 4th ventricles. Note the dark hemosiderin lining the pial surface of the brainstem from prior intraventricular hemorrhage (IVH).* + +![Axial SWI MR in a 3-month-old former premature infant with posthemorrhagic hydrocephalus shows signal loss along the ependymal margins of the ventricles & choroid plexus , consistent with prior IVH.](images/app.statdx.com_image_thumbnail_7e222e3d-c62e-4387-84b2-eb4f8647e09b_annotated_true_size_900_quality_90_d4a8ce99_20251018T165228Z.jpg) +**Posthemorrhagic Hydrocephalus** +*Axial SWI MR in a 3-month-old former premature infant with posthemorrhagic hydrocephalus shows signal loss along the ependymal margins of the ventricles & choroid plexus , consistent with prior IVH.* + +![Axial T1 C+ MR in a 7-year-old with Haemophilus influenzae meningitis shows marked expansion of the bifrontal SAS & mild enlargement of the lateral ventricles .](images/app.statdx.com_image_thumbnail_9a7bb1d1-b81f-4369-93ad-95c530aed370_annotated_true_size_900_quality_90_f7aa2f43_20251018T165234Z.jpg) +**Acute Infectious Hydrocephalus** +*Axial T1 C+ MR in a 7-year-old with Haemophilus influenzae meningitis shows marked expansion of the bifrontal SAS & mild enlargement of the lateral ventricles .* + +![Axial T1 C+ FS MR in an 8-month-old with tuberculous meningitis shows extensive basilar leptomeningeal enhancement & enlargement of the lateral ventricles , resulting in macrocephaly. Granulomatous infections are more likely to result in hydrocephalus compared to other bacterial meningitis.](images/app.statdx.com_image_thumbnail_5303411a-ca31-4d2c-b74c-0cf2a8730339_annotated_true_size_900_quality_90_c6cc9e06_20251018T165234Z.jpg) +**Postinfectious Hydrocephalus** +*Axial T1 C+ FS MR in an 8-month-old with tuberculous meningitis shows extensive basilar leptomeningeal enhancement & enlargement of the lateral ventricles , resulting in macrocephaly. Granulomatous infections are more likely to result in hydrocephalus compared to other bacterial meningitis.* + +![Sagittal FLAIR MR in a neonate with a large, obstructing, hemorrhagic posterior fossa mass shows enlargement of the lateral ventricles & posterior fossa. Note the ↑ craniofacial ratio.](images/app.statdx.com_image_thumbnail_3ff8c828-9d09-46d7-b1a1-d19d6bd5fb8d_annotated_true_size_900_quality_90_9f72e3d5_20251018T165234Z.jpg) +**Obstructing Tumor** +*Sagittal FLAIR MR in a neonate with a large, obstructing, hemorrhagic posterior fossa mass shows enlargement of the lateral ventricles & posterior fossa. Note the ↑ craniofacial ratio.* + +![Coronal T1 C+ MR in a 1-year-old with a choroid plexus papilloma shows an enhancing mass in the right choroid plexus. The lateral ventricles are enlarged without evidence of obstruction. Hydrocephalus in this case is due to overproduction of CSF by the tumor.](images/app.statdx.com_image_thumbnail_87a6356e-2c5c-44af-9516-f54896957a41_annotated_true_size_900_quality_90_b9f6d0f2_20251018T165234Z.jpg) +**CSF Overproduction (Choroid Plexus Tumor)** +*Coronal T1 C+ MR in a 1-year-old with a choroid plexus papilloma shows an enhancing mass in the right choroid plexus. The lateral ventricles are enlarged without evidence of obstruction. Hydrocephalus in this case is due to overproduction of CSF by the tumor.* + +![Axial FLAIR MR in a 4-year-old with a history of perinatal hypoxic-ischemic injury (HII) shows extensive areas of cortical encephalomalacia . Note the localized areas of ventriculomegaly due to overlying brain volume loss.](images/app.statdx.com_image_thumbnail_85b3abc9-edc3-48ec-9e40-734d0f72d297_annotated_true_size_900_quality_90_72ffe6d4_20251018T165234Z.jpg) +**Hypoxic-Ischemic Injury** +*Axial FLAIR MR in a 4-year-old with a history of perinatal hypoxic-ischemic injury (HII) shows extensive areas of cortical encephalomalacia . Note the localized areas of ventriculomegaly due to overlying brain volume loss.* + +![Axial T2 MR in a 2-year-old with a history of perinatal HII shows symmetric areas of signal abnormality & volume loss involving the thalami , putamina , & periventricular white matter , resulting in enlargement of the lateral & 3rd ventricles.](images/app.statdx.com_image_thumbnail_10667680-dfdd-4ced-a40d-43372e509021_annotated_true_size_900_quality_90_e1abf64c_20251018T165234Z.jpg) +**Hypoxic-Ischemic Injury** +*Axial T2 MR in a 2-year-old with a history of perinatal HII shows symmetric areas of signal abnormality & volume loss involving the thalami , putamina , & periventricular white matter , resulting in enlargement of the lateral & 3rd ventricles.* + +![Axial T2 MR in a 9-month-old with previous infarction shows extensive cystic encephalomalacia in right middle cerebral artery (MCA) territory with resultant asymmetric enlargement of the right lateral ventricle .](6accf05b-0e43-4a70-a966-67794ba8f8dd) +**Arterial Ischemic Stroke** +*Axial T2 MR in a 9-month-old with previous infarction shows extensive cystic encephalomalacia in right middle cerebral artery (MCA) territory with resultant asymmetric enlargement of the right lateral ventricle .* + +![Axial FLAIR MR in a 7-year-old with a history of extreme prematurity & periventricular leukomalacia (PVL) shows symmetric focal enlargement of the atria with adjacent white matter volume loss. The relative lack of abnormal FLAIR signal compared to the degree of volume loss is typical of PVL.](cd6af741-e6fa-46aa-bd6e-a2be4fa84b23) +**Periventricular Leukomalacia** +*Axial FLAIR MR in a 7-year-old with a history of extreme prematurity & periventricular leukomalacia (PVL) shows symmetric focal enlargement of the atria with adjacent white matter volume loss. The relative lack of abnormal FLAIR signal compared to the degree of volume loss is typical of PVL.* + +![Coronal US at 2 days of life in an extremely premature (23-week) infant shows a large right germinal matrix hemorrhage with associated hemorrhagic venous infarction in the right frontoparietal white matter.](2acd2437-e908-4dc8-8834-2946efa2858f) +**Porencephaly** +*Coronal US at 2 days of life in an extremely premature (23-week) infant shows a large right germinal matrix hemorrhage with associated hemorrhagic venous infarction in the right frontoparietal white matter.* + +![Coronal T2 MR in the same patient 3 months later shows the expected development of right parietal porencephaly & focal ventricular enlargement. Note the rim of T2 hypointensity , related to hemosiderin, which will eventually resolve.](f0f97ad6-303c-43fc-b9d1-3cc3eba46780) +**Porencephaly** +*Coronal T2 MR in the same patient 3 months later shows the expected development of right parietal porencephaly & focal ventricular enlargement. Note the rim of T2 hypointensity , related to hemosiderin, which will eventually resolve.* + +![Axial T2 MR in a teenager with metachromatic leukodystrophy shows ↑ signal & volume loss in the periventricular & deep white matter with sparing of the subcortical white matter, characteristic of this disease. Note the enlargement of the lateral ventricles & sulci due to the brain volume loss.](94c86422-ef68-4680-8877-d188e7f59890) +**Metabolic Brain Disease** +*Axial T2 MR in a teenager with metachromatic leukodystrophy shows ↑ signal & volume loss in the periventricular & deep white matter with sparing of the subcortical white matter, characteristic of this disease. Note the enlargement of the lateral ventricles & sulci due to the brain volume loss.* + +![Axial T2 MR in a teenager with vanishing white matter disease shows extensive ↑ signal intensity & volume loss in the white matter with associated enlargement of the lateral & 3rd ventricles & sulci .](efb42805-a92c-4737-a01b-753b53b8cc79) +**Metabolic Brain Disease** +*Axial T2 MR in a teenager with vanishing white matter disease shows extensive ↑ signal intensity & volume loss in the white matter with associated enlargement of the lateral & 3rd ventricles & sulci .* + +![Axial T1 MR in a neonate with seizures & hemimegalencephaly shows ↑ size of the left parietooccipital hemisphere with loss of normal sulcation & markedly abnormal neuronal organization . Also note enlargement of the ipsilateral occipital horn .](95b7dcd5-6ed3-4d5f-b06e-9a9c8071382b) +**Hemimegalencephaly** +*Axial T1 MR in a neonate with seizures & hemimegalencephaly shows ↑ size of the left parietooccipital hemisphere with loss of normal sulcation & markedly abnormal neuronal organization . Also note enlargement of the ipsilateral occipital horn .* + +![Axial T1 MR in a 3-day-old with left hemimegalencephaly shows marked occipital horn enlargement . Note the abnormal white matter in the left frontal lobe. Enlargement of the ipsilateral occipital horn is common in this disease.](df838971-ddb2-478e-bd8e-61844e1598ea) +**Hemimegalencephaly** +*Axial T1 MR in a 3-day-old with left hemimegalencephaly shows marked occipital horn enlargement . Note the abnormal white matter in the left frontal lobe. Enlargement of the ipsilateral occipital horn is common in this disease.* + +![Coronal T2 MR in a neonate with VGAM shows a markedly enlarged central vein with numerous enlarged choroidal & pericallosal feeding arteries. Note the enlarged ventricles , which are likely due to ↓ resorption of CSF due to ↑ venous pressures.](c5d3d1dc-0041-4021-9ee0-f19c99cc4d0e) +**Vein of Galen Aneurysmal Malformation** +*Coronal T2 MR in a neonate with VGAM shows a markedly enlarged central vein with numerous enlarged choroidal & pericallosal feeding arteries. Note the enlarged ventricles , which are likely due to ↓ resorption of CSF due to ↑ venous pressures.* + +![Sagittal 3D SSFP MR in a 2-month-old with Dandy-Walker malformation shows a small cerebellar vermis & large posterior fossa cyst that is continuous with the 4th ventricle. There is elevation of the tentorium & torcular Herophili .](fd790334-ffcb-435d-afe1-06cd9fb5581d) +**Dandy-Walker Malformation** +*Sagittal 3D SSFP MR in a 2-month-old with Dandy-Walker malformation shows a small cerebellar vermis & large posterior fossa cyst that is continuous with the 4th ventricle. There is elevation of the tentorium & torcular Herophili .* + +![Axial NECT in a 4-year-old with hydranencephaly shows porencephaly in the bilateral MCA & left anterior cerebral artery (ACA) territories in continuity with the lateral ventricles. Note the intact falx . Patients with hydranencephaly typically become macrocephalic due to poor CSF regulation.](0bce95e0-fa1b-4963-b4ec-ca1c53bd22f7) +**Hydranencephaly** +*Axial NECT in a 4-year-old with hydranencephaly shows porencephaly in the bilateral MCA & left anterior cerebral artery (ACA) territories in continuity with the lateral ventricles. Note the intact falx . Patients with hydranencephaly typically become macrocephalic due to poor CSF regulation.* + +![Sagittal T2 MR in a neonate with holoprosencephaly shows an enlarged monoventricle . However, the patient is microcephalic overall due to the ↓ brain parenchymal volume.](a0fe9fac-0d38-4324-a508-85ddc944e911) +**Holoprosencephaly** +*Sagittal T2 MR in a neonate with holoprosencephaly shows an enlarged monoventricle . However, the patient is microcephalic overall due to the ↓ brain parenchymal volume.* + diff --git a/out/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md b/out/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md deleted file mode 100644 index 8fb7912..0000000 --- a/out/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md +++ /dev/null @@ -1,543 +0,0 @@ ---- -title: "ADEM" -docid: "a3fafeb7-5861-4364-beb8-c0e30220564e" -authors: - - key: "99e1aff7-f42c-43a0-95ae-d89c8551aa01" - value: "Kevin R. Moore, MD" - - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" - value: "Miral D. Jhaveri, MD, MBA" - - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607" - value: "Surjith Vattoth, MD, FRCR" -breadcrumbs: - - - name: "Brain" - slug: "brain" - treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" - - - name: "Diagnosis" - slug: "diagnosis" - treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" - - - name: "Pathology-Based Diagnoses" - slug: "pathology-based-diagnoses" - treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" - - - name: "Infectious, Inflammatory, and Demyelinating Disease" - slug: "infectious-inflammatory-and-demyel-" - treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607" - - - name: "Inflammatory and Demyelinating Disease" - slug: "inflammatory-and-demyelinating-dis-" - treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc" - - - name: "ADEM" - slug: "adem" - treeNodeId: null -category: "Brain" -documentVersionId: "c2a39730-fd89-4f20-9447-d7fb297710c6" -imageCount: 22 -lastUpdated: "08/07/20" -pageDescription: "ADEM" -pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, ADEM" -pageTitle: "ADEM | STATdx" -enhancedTitle: "ADEM" -type: "DX" -references: true -breadcrumbs: - - "Brain" - - "Diagnosis" - - "Pathology-Based Diagnoses" - - "Infectious, Inflammatory, and Demyelinating Disease" - - "Inflammatory and Demyelinating Disease" - - "ADEM" ---- -# KEY FACTS - -- ## Terminology - - - - Autoimmune-mediated white matter (WM) demyelination of brain &/or spinal cord, usually with remyelination -- ## Imaging - - - - Best diagnostic clue: Multifocal WM and deep gray lesions days to weeks following infection/vaccination - - May involve both brain and spinal cord; WM > gray matter, but usually both affected - - Both supratentorial and infratentorial lesions - - Multifocal punctate to large flocculent FLAIR hyperintensities - - Deep/juxtacortical WM > periventricular WM - - Do not usually involve callososeptal interface - - Most lesions show increased signal on DWI (T2 shine-through) - - Punctate, ring, incomplete ring, peripheral enhancement - - Absence of enhancement does not exclude diagnosis - - MRS: ↓ NAA within lesions; may see ↑ Cho, ↑ lactate -- ## Top Differential Diagnoses - - - - Multiple sclerosis - - Autoimmune-mediated vasculitis - - Acute hypertensive encephalopathy, PRES - - Fabry disease - - Behçet disease -- ## Pathology - - - - > 30 different infectious agents and immunizations reported - - Anti-MOG (myelin oligodendrocyte glycoprotein) IgG antibodies found more commonly in younger patients -- ## Clinical Issues - - - - Mean age 5-8 years, but can occur at any age - - Male predominance (M:F = 1:0.6-0.8), unlike MS - - Usually monophasic, self-limited - - Complete recovery within 1 month: 50-60% - - Mortality: 10-30% -- ## Diagnostic Checklist - - - - Imaging findings often lag behind symptom onset, resolution - -# TERMINOLOGY - -- ## Abbreviations - - - - Acute disseminated encephalomyelitis (ADEM) -- ## Definitions - - - - Autoimmune-mediated white matter (WM) demyelination of brain &/or spinal cord, usually with remyelination - -# IMAGING - -- ## General Features - - - - ### Best diagnostic clue - - - - Multifocal WM/basal ganglia lesions days to weeks following infection/vaccination - - 93% within 3 weeks of infection, 5% within 1 month of vaccination - - ### Location - - - - May involve both brain and spinal cord; WM > gray matter, but usually both affected - - Deep/juxtacortical WM > periventricular WM - - Both supratentorial and infratentorial lesions - - ### Size - - - - Tumefactive lesions may be large, but with less mass effect than expected from tumor size - - ### Morphology - - - - Punctate to flocculent - - Tumefactive, mass-like lesions possible -- ## CT Findings - - - - ### NECT - - - - Initial CT normal in 40% - - ### CECT - - - - Multifocal punctate or ring-enhancing lesions -- ## MR Findings - - - - ### T2WI - - - - Hyperintensities may be better visualized in brainstem and posterior fossa on T2 - - ### FLAIR - - - - Multifocal punctate to large, flocculent FLAIR hyperintensities - - Bilateral but asymmetric - - Involve peripheral WM-gray matter junction subcortical WM - - Thalami and basal ganglia frequently involved, typically symmetric - - Can involve brainstem and posterior fossa - - Do not usually involve callososeptal interface - - ### DWI - - - - Variably hyperintense lesions on DWI (trace) images - - Apparent diffusion coefficient (ADC) may be increased or decreased - - Most lesions show increased signal (T2 shine-through) - - Diffusion restriction uncommon, suggests worse prognosis - - Diffusivity normal within normal-appearing WM (NAWM), unlike MS - - ### T1WI C+ - - - - Punctate, ring, incomplete ring, peripheral enhancement - - Cranial nerve(s) may enhance - - Absence does not exclude diagnosis - - ### MRS - - - - NAA low within lesions; lactate may be elevated - - Choline often elevated in acute lesions - - NAA normalizes with resolution of symptoms/MR abnormalities - - Magnetization transfer ratio (MTR) - - ADEM MTR normal within NAWM, unlike MS -- ## Imaging Recommendations - - - - ### Best imaging tool - - - - Contrast-enhanced MR - - Initial imaging often normal but more sensitive than CT - - May appear identical to MS; repeat MR necessary to distinguish with certainty - - ### Protocol advice - - - - Limited rapid interval follow-up may be provided by FLAIR alone -- ## Nuclear Medicine Findings - - - - Tc-99m-HMPAO SPECT shows more extensive hypoperfusion than T2 lesions - -# DIFFERENTIAL DIAGNOSIS - -- [Multiple Sclerosis](/document/pediatric-multiple-sclerosis-brain/f2592b04-f800-4235-9eea-a43f2bf4adfe) - - Predilection for periventricular WM (callososeptal interface), involves subcortical U fibers, commonly in posterior fossa - - Lesions often more symmetric than ADEM - - Relapsing-remitting course common -- [Autoimmune-Mediated Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0) - - Multifocal gray matter-WM lesions - - Bilateral, usually cortical/subcortical, basal ganglia/thalami - - Ring-enhancing lesions may mimic infection -- [Acute Hypertensive Encephalopathy, PRES](/document/acute-hypertensive-encephalopathy--/efc6f9c2-dad9-4eb8-bad2-421bfaf1ec57) - - Typically posterior circulation in cortex/subcortical WM - - May affect deep gray nuclei -- [Aging Brain With Hyperintense WM Lesions](/document/normal-aging-brain/2a315550-b2ea-4afe-a2ef-f93a2209f276) - - Atherosclerotic brain changes in 50% patients > 50 years old - - Found in normotensive patients; more common in hypertensives patients - - Present in 10-30% of cognitively normal elderly patients - - MR: Scattered, asymmetric WM lesions, without enhancement - - Often periatrial; posterior fossa uncommon - - Spares callososeptal interface, subcortical U fibers -- [Fabry Disease](/document/fabry-disease/83fd222a-9b37-4087-afab-34ba74525887) - - Synonym: Angiokeratoma corporis diffusum universalis - - X-linked recessive; incidence 1/40,000 - - Deficiency α-galactosidase A; overaccumulation of glycosphingolipids within lysosomes - - MR: Scattered, asymmetric WM lesions without enhancement - - May involve brainstem and posterior fossa - - Spares callososeptal interface and subcortical U fibers - - Cranial MR sensitive to identify neurologic involvement in asymptomatic patients - - Present with renal failure/heart disease -- [Behçet Disease](/document/behet-disease/4e447bb6-0f14-40e1-929a-4c1465feec0a) - - MR: Scattered, asymmetric, subcortical WM lesions without cortical involvement - - Nodular enhancement in acute phase - - Predilection for midbrain - - ADC ↑, similar to ADEM - - Classic triad: Oral and genital ulcerations with uveitis - -# PATHOLOGY - -- ## General Features - - - - ### Etiology - - - - Autoimmune-mediated severe acute demyelination - - Following nonspecific upper respiratory tract infection, often viral - - > 30 different infectious agents and immunizations reported - - After specific viral illness: Epstein-Barr, influenza A, mumps, coronavirus - - Especially after exanthematous diseases of childhood (chickenpox, measles) - - After vaccination: Diphtheria, influenza, rabies, smallpox, tetanus, typhoid - - Spontaneous (no known cause) - - ### Genetics - - - - ADEM associated with DRB1*01 and DRB1*017(03) in Russian population - - ### Associated abnormalities - - - - Acute hemorrhagic leukoencephalopathy variant associated with ulcerative colitis and asthma - - Anti-MOG (myelin oligodendrocyte glycoprotein) IgG antibodies found more commonly in younger patients -- ## Gross Pathologic & Surgical Features - - - - None, unless hemorrhage (rare) or tumefactive edema -- ## Microscopic Features - - - - Acute myelin breakdown - - Perivenous inflammation; lymphocytic infiltrates - - Relative axonal preservation; atypical astrogliosis - - Virus generally not found, unlike viral encephalitides - - Similar to experimental allergic encephalomyelitis, supporting autoimmune-related etiology - -# CLINICAL ISSUES - -- ## Presentation - - - - ### Most common signs/symptoms - - - - Usually preceded by prodromal phase: Fever, malaise, myalgia - - Multifocal neurologic symptoms, 2 days to 4 weeks after viral illness/immunization - - Initial symptoms: Headache, fever, drowsiness - - Cranial nerve palsies, seizures, hemiparesis - - Decreased consciousness (from lethargy to coma) - - Behavioral changes - - ### Other signs/symptoms - - - - Seizures in 10-35% - - ### Clinical profile - - - - Cerebrospinal fluid (CSF) normal in 60% - - If abnormal (lymphocyte pleocytosis, elevated protein) - - Usually lacks CSF oligoclonal bands -- ## Demographics - - - - ### Age - - - - Children > adults - - Mean age 5-8 years, but can occur at any age - - ### Sex - - - - Male predominance (M:F = 1.0:0.6-0.8), unlike MS - - ### Epidemiology - - - - Rare, yet most common para-/postinfectious disorder - - Most common in winter and spring - - Exact epidemiology unknown, but increasingly reported -- ## Natural History & Prognosis - - - - Usually monophasic, self-limited - - Variable prognosis - - Complete recovery within 1 month (50-60%) - - Neurologic sequelae (most commonly seizures) (20-30%) - - Mortality (10-30%) - - Relapses are rare - - "Relapsing disseminated encephalomyelitis" - - May not be separate entity from relapsing-remitting MS - - Typically delay between symptom onset and imaging findings - - Varicella and rubella ADEM have preferential patterns - - Varicella ADEM characterized by cerebellar ataxia and mild pyramidal dysfunction - - Rubella ADEM characterized by acute explosive onset, seizures, coma, and moderate pyramidal signs - - Rare manifestations of ADEM - - Acute hemorrhagic leukoencephalopathy (2%) - - Young patients with abrupt symptom onset - - Fulminant, often ending in death - - Bilateral striatal necrosis (usually in infants, may be reversible) -- ## Treatment - - - - Immunosuppressive/immunomodulatory therapy - - MR may show prompt improvement after therapy - - Plasma exchange therapy - - 40% of patients failing steroid treatment may show marked improvement - -# DIAGNOSTIC CHECKLIST - -- ## Image Interpretation Pearls - - - - Imaging findings often lag behind symptom onset, resolution - - 3aec74c8-de53-4430-a00a-d8165ad210c7 - -## References - -# Selected References - -1. [Carvalho K et al: Acute disseminated encephalomyelitis (ADEM) associated with mosquito-borne diseases: Chikungunya virus X yellow fever immunization. Rev Soc Bras Med Trop. 53:e20190160, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31994659%5Bpmid%5D) -1. [Molero-Senosiain M et al: Neuro-ophthalmological manifestations as complication of an infection with Mycoplasma pneumoniae and subsequent development of disseminated acute encephalitis. Arch Soc Esp Oftalmol. 95(5(:254-8, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32147128%5Bpmid%5D) -1. [Stokes Brackett AC et al: Multiphasic acute disseminated encephalomyelitis and differential with early onset multiple sclerosis. Intractable Rare Dis Res. 9(1):61-3, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32201679%5Bpmid%5D) -1. [Baumann M et al: MRI of the first event in pediatric acquired demyelinating syndromes with antibodies to myelin oligodendrocyte glycoprotein. J Neurol. 265(4):845-55, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29423614%5Bpmid%5D) -1. [Aubert-Broche B et al: Monophasic demyelination reduces brain growth in children. Neurology. 88(18):1744-50, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28381515%5Bpmid%5D) -1. [Bester M et al: Neuroimaging of multiple sclerosis, acute disseminated encephalomyelitis, and other demyelinating diseases. Semin Roentgenol. 49(1):76-85, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24342677%5Bpmid%5D) -1. [Longoni G et al: White matter changes in paediatric multiple sclerosis and monophasic demyelinating disorders. Brain. 140(5):1300-15, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28334875%5Bpmid%5D) -1. [Koelman DLH et al: Acute disseminated encephalomyelitis: prognostic value of early follow-up brain MRI. J Neurol. 264(8):1754-62, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28695361%5Bpmid%5D) -1. [Marziali S et al: Acute disseminated encephalomyelitis following Campylobacter jejuni gastroenteritis: Case report and review of the literature. Neuroradiol J. 30(1):65-70, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27888275%5Bpmid%5D) -1. [Kanekar S et al: A pattern approach to focal white matter hyperintensities on magnetic resonance imaging. Radiol Clin North Am. 52(2):241-61, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24582339%5Bpmid%5D) -1. [Daida K et al: Cytomegalovirus-associated encephalomyelitis in an immunocompetent adult: a two-stage attack of direct viral and delayed immune-mediated invasions. case report. BMC Neurol. 16(1):223, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27855658%5Bpmid%5D) -1. [Pohl D et al: Acute disseminated encephalomyelitis: updates on an inflammatory CNS syndrome. Neurology. 87(9 Suppl 2):S38-45, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27572859%5Bpmid%5D) -1. [Yuan JL et al: Acute Disseminated Encephalomyelitis following Vaccination against Hepatitis B in a Child: A Case Report and Literature Review. Case Rep Neurol Med. 2016:2401809, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27478662%5Bpmid%5D) -1. [Baumann M et al: Clinical and neuroradiological differences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein. J Neurol Neurosurg Psychiatry. 86(3):265-72, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25121570%5Bpmid%5D) -1. [Karussis D: The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review. J Autoimmun. 48-49:134-42, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24524923%5Bpmid%5D) -1. [Mariotto S et al: Clinical spectrum and IgG subclass analysis of anti-myelin oligodendrocyte glycoprotein antibody-associated syndromes: a multicenter study. J Neurol. 264(12):2420-30, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29063242%5Bpmid%5D) -1. [Nakamura Y et al: Anti-MOG antibody-positive ADEM following infectious mononucleosis due to a primary EBV infection: a case report. BMC Neurol. 17(1):76, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28420330%5Bpmid%5D) -1. [Tenembaum SN: Acute disseminated encephalomyelitis. Handb Clin Neurol. 112:1253-62, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23622336%5Bpmid%5D) -1. [Wingerchuk DM et al: Acute disseminated encephalomyelitis, transverse myelitis, and neuromyelitis optica. Continuum (Minneap Minn). 19(4 Multiple Sclerosis):944-67, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23917095%5Bpmid%5D) -1. [Callen DJ et al: Role of MRI in the differentiation of ADEM from MS in children. Neurology. 72(11):968-73, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19038851%5Bpmid%5D) -1. [Noorbakhsh F et al: Acute disseminated encephalomyelitis: clinical and pathogenesis features. Neurol Clin. 26(3):759-80, ix, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18657725%5Bpmid%5D) -1. [Rossi A: Imaging of acute disseminated encephalomyelitis. Neuroimaging Clin N Am. 18(1):149-61; ix, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18319160%5Bpmid%5D) -1. [Tenembaum S et al: Acute disseminated encephalomyelitis. Neurology. 68(16 Suppl 2):S23-36, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17438235%5Bpmid%5D) -1. [Menge T et al: Acute disseminated encephalomyelitis: an update. Arch Neurol. 62(11):1673-80, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16286539%5Bpmid%5D) -1. [Yeh EA et al: Detection of coronavirus in the central nervous system of a child with acute disseminated encephalomyelitis. Pediatrics. 113(1 Pt 1):e73-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14702500%5Bpmid%5D) -1. [Dale RC: Acute disseminated encephalomyelitis. Semin Pediatr Infect Dis. 14(2):90-5, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12881796%5Bpmid%5D) -1. [Garg RK: Acute disseminated encephalomyelitis. Postgrad Med J. 79(927):11-17, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12566545%5Bpmid%5D) -1. [Idrissova ZhR et al: Acute disseminated encephalomyelitis in children: clinical features and HLA-DR linkage. Eur J Neurol. 10(5):537-46, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12940836%5Bpmid%5D) -1. [Okamoto K et al: MR features of diseases involving bilateral middle cerebellar peduncles. AJNR Am J Neuroradiol. 24(10):1946-54, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14625215%5Bpmid%5D) -1. [Sener RN: Neuro-Behcet's disease: diffusion MR imaging and proton MR spectroscopy. AJNR Am J Neuroradiol. 24(8):1612-4, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=13679280%5Bpmid%5D) -1. [Stonehouse M et al: Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians. Arch Dis Child. 88(2):122-4, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12538312%5Bpmid%5D) -1. [Inglese M et al: Magnetization transfer and diffusion tensor MR imaging of acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 23(2):267-72, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11847052%5Bpmid%5D) -1. [Murthy JM: Acute disseminated encephalomyelitis. Neurol India. 50(3):238-43, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12391446%5Bpmid%5D) -1. [Tenembaum S et al: Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology. 59(8):1224-31, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12391351%5Bpmid%5D) -1. [Bizzi A et al: Quantitative proton MR spectroscopic imaging in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 22(6):1125-30, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11415908%5Bpmid%5D) -1. [Honkaniemi J et al: Delayed MR imaging changes in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 22(6):1117-24, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11415907%5Bpmid%5D) -1. [Straussberg R et al: Improvement of atypical acute disseminated encephalomyelitis with steroids and intravenous immunoglobulins. Pediatr Neurol. 24(2):139-43, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11275464%5Bpmid%5D) -1. [Dale RC et al: Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain. 123 Pt 12:2407-22, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11099444%5Bpmid%5D) -1. [Rust RS: Multiple sclerosis, acute disseminated encephalomyelitis, and related conditions. Semin Pediatr Neurol. 7(2):66-90, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10914409%5Bpmid%5D) -1. [Schaefer PW et al: Diffusion-weighted MR imaging of the brain. Radiology. 217(2):331-45, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11058626%5Bpmid%5D) -1. [Kocer N et al: CNS involvement in neuro-Behcet syndrome: an MR study. AJNR Am J Neuroradiol. 20(6):1015-24, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10445437%5Bpmid%5D) - - -## Images - - -### Selected Images - -![Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.](images/app.statdx.com_image_deba3310-8e09-466c-97d2-1e6bccb28edf_dafbadc1_20251018T064947Z.jpg) -*Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.* - -![Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.](images/app.statdx.com_image_thumbnail_deba3310-8e09-466c-97d2-1e6bccb28edf_size_168_quality_85_3dfef960_20251018T064936Z.jpg) -*Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.* - -![Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.](images/app.statdx.com_image_thumbnail_deba3310-8e09-466c-97d2-1e6bccb28edf_size_174_quality_85_6f9ba8b5_20251018T122441Z.jpg) -*Axial FLAIR MR shows peripheral, confluent areas of hyperintensity predominantly involving the subcortical white matter (WM) in this child with ADEM. The bilateral but asymmetric pattern is typical of ADEM.* - -![Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of the majority of lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis (MS) lesions is more variable.](images/app.statdx.com_image_e18458d3-5f6e-43fd-ab6d-72c7a86dca92_0f3893d6_20251018T064949Z.jpg) -*Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of the majority of lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis (MS) lesions is more variable.* - -![Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of the majority of lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis (MS) lesions is more variable.](images/app.statdx.com_image_thumbnail_e18458d3-5f6e-43fd-ab6d-72c7a86dca92_size_168_quality_85_70272c40_20251018T064936Z.jpg) -*Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of the majority of lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis (MS) lesions is more variable.* - -![Axial T1 C+ MR shows an incomplete ring of peripheral enhancement, typical of a demyelinating process. Other contrast enhancement patterns include ovoid or punctate homogeneous enhancement.](images/app.statdx.com_image_53135c0f-4c9e-4fb9-ab09-df46c5b40f70_c986514a_20251018T064950Z.jpg) -*Axial T1 C+ MR shows an incomplete ring of peripheral enhancement, typical of a demyelinating process. Other contrast enhancement patterns include ovoid or punctate homogeneous enhancement.* - -![Axial T1 C+ MR shows an incomplete ring of peripheral enhancement, typical of a demyelinating process. Other contrast enhancement patterns include ovoid or punctate homogeneous enhancement.](images/app.statdx.com_image_thumbnail_53135c0f-4c9e-4fb9-ab09-df46c5b40f70_size_168_quality_85_3f185a62_20251018T064936Z.jpg) -*Axial T1 C+ MR shows an incomplete ring of peripheral enhancement, typical of a demyelinating process. Other contrast enhancement patterns include ovoid or punctate homogeneous enhancement.* - -![Axial DWI MR shows increased signal in areas of FLAIR hyperintensity. The foci were hypointense on ADC images, indicating diffusion restriction. Both WM and gray matter involvement is present. Diffusion restriction is an uncommon imaging finding and is associated with a worse prognosis.](images/app.statdx.com_image_b79995c4-6bff-49f1-af71-cb56fe4b4aa1_119a9c33_20251018T064951Z.jpg) -*Axial DWI MR shows increased signal in areas of FLAIR hyperintensity. The foci were hypointense on ADC images, indicating diffusion restriction. Both WM and gray matter involvement is present. Diffusion restriction is an uncommon imaging finding and is associated with a worse prognosis.* - -![Axial DWI MR shows increased signal in areas of FLAIR hyperintensity. The foci were hypointense on ADC images, indicating diffusion restriction. Both WM and gray matter involvement is present. Diffusion restriction is an uncommon imaging finding and is associated with a worse prognosis.](images/app.statdx.com_image_thumbnail_b79995c4-6bff-49f1-af71-cb56fe4b4aa1_size_168_quality_85_03275787_20251018T064936Z.jpg) -*Axial DWI MR shows increased signal in areas of FLAIR hyperintensity. The foci were hypointense on ADC images, indicating diffusion restriction. Both WM and gray matter involvement is present. Diffusion restriction is an uncommon imaging finding and is associated with a worse prognosis.* - -![Axial T2 MR shows hyperintense lesions in the brachium pontis bilaterally, typical for demyelination. The right-sided lesion shows a targetoid appearance. Enhancement of several lesions was present on postcontrast T1 images (not shown).](images/app.statdx.com_image_f74d958e-f845-47e7-884e-331bf3dcb299_89b1781b_20251018T064952Z.jpg) -*Axial T2 MR shows hyperintense lesions in the brachium pontis bilaterally, typical for demyelination. The right-sided lesion shows a targetoid appearance. Enhancement of several lesions was present on postcontrast T1 images (not shown).* - -![Axial T2 MR shows hyperintense lesions in the brachium pontis bilaterally, typical for demyelination. The right-sided lesion shows a targetoid appearance. Enhancement of several lesions was present on postcontrast T1 images (not shown).](images/app.statdx.com_image_thumbnail_f74d958e-f845-47e7-884e-331bf3dcb299_size_168_quality_85_10b7c767_20251018T064936Z.jpg) -*Axial T2 MR shows hyperintense lesions in the brachium pontis bilaterally, typical for demyelination. The right-sided lesion shows a targetoid appearance. Enhancement of several lesions was present on postcontrast T1 images (not shown).* - -![Axial FLAIR MR shows large, confluent regions of hyperintense signal in the periventricular and subcortical WM in a 14 year old who presented with neck stiffness, fatigue, and seizures.](images/app.statdx.com_image_3e8a9c1e-560e-4c5c-93bc-dda9b3773622_eda9eb67_20251018T064954Z.jpg) -*Axial FLAIR MR shows large, confluent regions of hyperintense signal in the periventricular and subcortical WM in a 14 year old who presented with neck stiffness, fatigue, and seizures.* - -![Axial FLAIR MR shows large, confluent regions of hyperintense signal in the periventricular and subcortical WM in a 14 year old who presented with neck stiffness, fatigue, and seizures.](images/app.statdx.com_image_thumbnail_3e8a9c1e-560e-4c5c-93bc-dda9b3773622_size_168_quality_85_caf40da9_20251018T064936Z.jpg) -*Axial FLAIR MR shows large, confluent regions of hyperintense signal in the periventricular and subcortical WM in a 14 year old who presented with neck stiffness, fatigue, and seizures.* - -![Axial SWI MR in the same patient shows petechial hemorrhages in regions of FLAIR signal abnormality.](images/app.statdx.com_image_fa96c2d2-8153-44c2-bd76-be0296382093_e2505a5f_20251018T064955Z.jpg) -*Axial SWI MR in the same patient shows petechial hemorrhages in regions of FLAIR signal abnormality.* - -![Axial SWI MR in the same patient shows petechial hemorrhages in regions of FLAIR signal abnormality.](images/app.statdx.com_image_thumbnail_fa96c2d2-8153-44c2-bd76-be0296382093_size_168_quality_85_93dd4c72_20251018T064936Z.jpg) -*Axial SWI MR in the same patient shows petechial hemorrhages in regions of FLAIR signal abnormality.* - -![Sagittal T1 C+ MR in the same patient shows extensive irregular ring enhancement in multiple subcortical WM lesions. Acute hemorrhagic leukoencephalopathy (AHL) is a rare manifestation of ADEM occurring in 2% of cases. AHL is associated with a very poor prognosis. Aggressive therapeutic management is a prerequisite to avoid usual disease course with fatal outcome.](images/app.statdx.com_image_b9452b6b-6772-4d91-8ca4-47ceaafa25f4_ddfedaec_20251018T064957Z.jpg) -*Sagittal T1 C+ MR in the same patient shows extensive irregular ring enhancement in multiple subcortical WM lesions. Acute hemorrhagic leukoencephalopathy (AHL) is a rare manifestation of ADEM occurring in 2% of cases. AHL is associated with a very poor prognosis. Aggressive therapeutic management is a prerequisite to avoid usual disease course with fatal outcome.* - -![Sagittal T1 C+ MR in the same patient shows extensive irregular ring enhancement in multiple subcortical WM lesions. Acute hemorrhagic leukoencephalopathy (AHL) is a rare manifestation of ADEM occurring in 2% of cases. AHL is associated with a very poor prognosis. Aggressive therapeutic management is a prerequisite to avoid usual disease course with fatal outcome.](images/app.statdx.com_image_thumbnail_b9452b6b-6772-4d91-8ca4-47ceaafa25f4_size_168_quality_85_3dcd4458_20251018T064936Z.jpg) -*Sagittal T1 C+ MR in the same patient shows extensive irregular ring enhancement in multiple subcortical WM lesions. Acute hemorrhagic leukoencephalopathy (AHL) is a rare manifestation of ADEM occurring in 2% of cases. AHL is associated with a very poor prognosis. Aggressive therapeutic management is a prerequisite to avoid usual disease course with fatal outcome.* - -![Coronal T2 MR shows large, confluent regions of hyperintense signal in the WM and deep gray nuclei of a child with ADEM. Although ADEM predominantly involves WM, gray matter is often affected.](images/app.statdx.com_image_113e918b-8d8f-46b8-8664-e0f617904251_b9bf7b86_20251018T064958Z.jpg) -*Coronal T2 MR shows large, confluent regions of hyperintense signal in the WM and deep gray nuclei of a child with ADEM. Although ADEM predominantly involves WM, gray matter is often affected.* - -![Coronal T2 MR shows large, confluent regions of hyperintense signal in the WM and deep gray nuclei of a child with ADEM. Although ADEM predominantly involves WM, gray matter is often affected.](images/app.statdx.com_image_thumbnail_113e918b-8d8f-46b8-8664-e0f617904251_size_168_quality_85_1b06d2b7_20251018T064936Z.jpg) -*Coronal T2 MR shows large, confluent regions of hyperintense signal in the WM and deep gray nuclei of a child with ADEM. Although ADEM predominantly involves WM, gray matter is often affected.* - -![MRS at long TE in a patient with acute lesions in ADEM demonstrates ↑ choline , ↓ NAA , and the presence of a lactate doublet . Increase in choline with corresponding reductions in NAA normalize as the clinical and conventional neuroimaging abnormalities resolve.](images/app.statdx.com_image_6364dfb4-c43c-4ad6-afe6-c2abed27d385_15c7b525_20251018T065000Z.jpg) -*MRS at long TE in a patient with acute lesions in ADEM demonstrates ↑ choline , ↓ NAA , and the presence of a lactate doublet . Increase in choline with corresponding reductions in NAA normalize as the clinical and conventional neuroimaging abnormalities resolve.* - -![MRS at long TE in a patient with acute lesions in ADEM demonstrates ↑ choline , ↓ NAA , and the presence of a lactate doublet . Increase in choline with corresponding reductions in NAA normalize as the clinical and conventional neuroimaging abnormalities resolve.](images/app.statdx.com_image_thumbnail_6364dfb4-c43c-4ad6-afe6-c2abed27d385_size_168_quality_85_a0acf936_20251018T064936Z.jpg) -*MRS at long TE in a patient with acute lesions in ADEM demonstrates ↑ choline , ↓ NAA , and the presence of a lactate doublet . Increase in choline with corresponding reductions in NAA normalize as the clinical and conventional neuroimaging abnormalities resolve.* - - -### Additional Images - -![Axial FLAIR MR shows multiple bilateral, asymmetric, flocculent, hyperintense lesions of acute disseminated encephalomyelitis.](images/app.statdx.com_image_bac2d338-fffe-48e4-be97-bb8258970076_425b9699_20251018T065002Z.jpg) -*Axial FLAIR MR shows multiple bilateral, asymmetric, flocculent, hyperintense lesions of acute disseminated encephalomyelitis.* - -![Axial FLAIR MR shows multiple bilateral, asymmetric, flocculent, hyperintense lesions of acute disseminated encephalomyelitis.](images/app.statdx.com_image_thumbnail_bac2d338-fffe-48e4-be97-bb8258970076_size_168_quality_85_4b79e54c_20251018T064936Z.jpg) -*Axial FLAIR MR shows multiple bilateral, asymmetric, flocculent, hyperintense lesions of acute disseminated encephalomyelitis.* - -![Coronal T1 C+ MR demonstrates partial peripheral enhancement around multiple asymmetric, flocculent lesions of acute disseminated encephalomyelitis. Note the supra- and infratentorial lesions.](images/app.statdx.com_image_674fcfe9-2db8-4d36-a55c-9ca0a0672252_4fcbd413_20251018T065003Z.jpg) -*Coronal T1 C+ MR demonstrates partial peripheral enhancement around multiple asymmetric, flocculent lesions of acute disseminated encephalomyelitis. Note the supra- and infratentorial lesions.* - -![Coronal T1 C+ MR demonstrates partial peripheral enhancement around multiple asymmetric, flocculent lesions of acute disseminated encephalomyelitis. Note the supra- and infratentorial lesions.](images/app.statdx.com_image_thumbnail_674fcfe9-2db8-4d36-a55c-9ca0a0672252_size_168_quality_85_05b2865d_20251018T064936Z.jpg) -*Coronal T1 C+ MR demonstrates partial peripheral enhancement around multiple asymmetric, flocculent lesions of acute disseminated encephalomyelitis. Note the supra- and infratentorial lesions.* - -![Axial FLAIR MR shows asymmetric, flocculent, nearly confluent, hyperintense lesions of acute disseminated encephalomyelitis within posterior fossa structures.](images/app.statdx.com_image_09b3df53-221b-4806-9896-be6e908b87e5_07de590e_20251018T065005Z.jpg) -*Axial FLAIR MR shows asymmetric, flocculent, nearly confluent, hyperintense lesions of acute disseminated encephalomyelitis within posterior fossa structures.* - -![Axial FLAIR MR shows asymmetric, flocculent, nearly confluent, hyperintense lesions of acute disseminated encephalomyelitis within posterior fossa structures.](images/app.statdx.com_image_thumbnail_09b3df53-221b-4806-9896-be6e908b87e5_size_168_quality_85_c4b5370a_20251018T064936Z.jpg) -*Axial FLAIR MR shows asymmetric, flocculent, nearly confluent, hyperintense lesions of acute disseminated encephalomyelitis within posterior fossa structures.* - -![Axial FLAIR MR reveals multiple asymmetric, primarily punctate, hyperintense lesions of acute disseminated encephalomyelitis.](images/app.statdx.com_image_4544554f-59e5-452b-a6b3-42d96236d4b0_9de028d1_20251018T065007Z.jpg) -*Axial FLAIR MR reveals multiple asymmetric, primarily punctate, hyperintense lesions of acute disseminated encephalomyelitis.* - -![Axial FLAIR MR reveals multiple asymmetric, primarily punctate, hyperintense lesions of acute disseminated encephalomyelitis.](images/app.statdx.com_image_thumbnail_4544554f-59e5-452b-a6b3-42d96236d4b0_size_168_quality_85_d15beb2e_20251018T064936Z.jpg) -*Axial FLAIR MR reveals multiple asymmetric, primarily punctate, hyperintense lesions of acute disseminated encephalomyelitis.* - -![Axial FLAIR MR demonstrates a large, tumefactive, hyperintense lesion. Less mass effect is present than expected for lesion size. Smaller lesions were also present at other locations.](images/app.statdx.com_image_25db98bd-b92d-407c-b5f8-796cf53ee71f_b0040522_20251018T065008Z.jpg) -*Axial FLAIR MR demonstrates a large, tumefactive, hyperintense lesion. Less mass effect is present than expected for lesion size. Smaller lesions were also present at other locations.* - -![Axial FLAIR MR demonstrates a large, tumefactive, hyperintense lesion. Less mass effect is present than expected for lesion size. Smaller lesions were also present at other locations.](images/app.statdx.com_image_thumbnail_25db98bd-b92d-407c-b5f8-796cf53ee71f_size_168_quality_85_861400ad_20251018T064936Z.jpg) -*Axial FLAIR MR demonstrates a large, tumefactive, hyperintense lesion. Less mass effect is present than expected for lesion size. Smaller lesions were also present at other locations.* - -![Axial T1 C+ MR demonstrates a large, tumefactive, hypointense lesion with minimal partial peripheral enhancement. Less mass effect is present than expected for lesion size. More lesions were seen elsewhere.](images/app.statdx.com_image_a985da5d-914a-4071-8be7-5090530a9b69_9ef143d1_20251018T065009Z.jpg) -*Axial T1 C+ MR demonstrates a large, tumefactive, hypointense lesion with minimal partial peripheral enhancement. Less mass effect is present than expected for lesion size. More lesions were seen elsewhere.* - -![Axial T1 C+ MR demonstrates a large, tumefactive, hypointense lesion with minimal partial peripheral enhancement. Less mass effect is present than expected for lesion size. More lesions were seen elsewhere.](images/app.statdx.com_image_thumbnail_a985da5d-914a-4071-8be7-5090530a9b69_size_168_quality_85_72123be9_20251018T064936Z.jpg) -*Axial T1 C+ MR demonstrates a large, tumefactive, hypointense lesion with minimal partial peripheral enhancement. Less mass effect is present than expected for lesion size. More lesions were seen elsewhere.* - -![Axial FLAIR MR demonstrates a rare manifestation of ADEM: Bilateral striatal necrosis, evidenced by asymmetric confluent hyperintensity involving the gray and white matter of bilateral corpus striatum.](images/app.statdx.com_image_9115564d-21e2-45af-8636-0e4a32bd55e3_504d56ec_20251018T065011Z.jpg) -*Axial FLAIR MR demonstrates a rare manifestation of ADEM: Bilateral striatal necrosis, evidenced by asymmetric confluent hyperintensity involving the gray and white matter of bilateral corpus striatum.* - -![Axial FLAIR MR demonstrates a rare manifestation of ADEM: Bilateral striatal necrosis, evidenced by asymmetric confluent hyperintensity involving the gray and white matter of bilateral corpus striatum.](images/app.statdx.com_image_thumbnail_9115564d-21e2-45af-8636-0e4a32bd55e3_size_168_quality_85_184601eb_20251018T064936Z.jpg) -*Axial FLAIR MR demonstrates a rare manifestation of ADEM: Bilateral striatal necrosis, evidenced by asymmetric confluent hyperintensity involving the gray and white matter of bilateral corpus striatum.* - -![Axial DWI MR confirms the rare manifestation of ADEM, displaying bilateral striatal necrosis, as evidenced by asymmetric confluent restricted diffusion involving gray and white matter of bilateral corpus striatum.](images/app.statdx.com_image_f2308cec-0c77-4d95-ae67-c7db7930d83e_b6945539_20251018T065012Z.jpg) -*Axial DWI MR confirms the rare manifestation of ADEM, displaying bilateral striatal necrosis, as evidenced by asymmetric confluent restricted diffusion involving gray and white matter of bilateral corpus striatum.* - -![Axial DWI MR confirms the rare manifestation of ADEM, displaying bilateral striatal necrosis, as evidenced by asymmetric confluent restricted diffusion involving gray and white matter of bilateral corpus striatum.](images/app.statdx.com_image_thumbnail_f2308cec-0c77-4d95-ae67-c7db7930d83e_size_168_quality_85_a2613b2e_20251018T064936Z.jpg) -*Axial DWI MR confirms the rare manifestation of ADEM, displaying bilateral striatal necrosis, as evidenced by asymmetric confluent restricted diffusion involving gray and white matter of bilateral corpus striatum.* - -![Axial T2 MR shows multiple bilateral, but asymmetric, T2-hyperintense foci . None of the lesions demonstrate significant mass effect in this adult patient with ADEM. Imaging mimics multiple sclerosis, vasculitis, and microvascular ischemia.](images/app.statdx.com_image_92293bff-8c2c-40f0-83da-bafe77cc24c6_50085203_20251018T065013Z.jpg) -*Axial T2 MR shows multiple bilateral, but asymmetric, T2-hyperintense foci . None of the lesions demonstrate significant mass effect in this adult patient with ADEM. Imaging mimics multiple sclerosis, vasculitis, and microvascular ischemia.* - -![Axial T2 MR shows multiple bilateral, but asymmetric, T2-hyperintense foci . None of the lesions demonstrate significant mass effect in this adult patient with ADEM. Imaging mimics multiple sclerosis, vasculitis, and microvascular ischemia.](images/app.statdx.com_image_thumbnail_92293bff-8c2c-40f0-83da-bafe77cc24c6_size_168_quality_85_8c339a53_20251018T064936Z.jpg) -*Axial T2 MR shows multiple bilateral, but asymmetric, T2-hyperintense foci . None of the lesions demonstrate significant mass effect in this adult patient with ADEM. Imaging mimics multiple sclerosis, vasculitis, and microvascular ischemia.* - -![Axial FLAIR MR shows a large, tumefactive, hyperintense ADEM lesion with mass effect less than expected for the size of the lesion. Another clue to its nonneoplastic nature is the right-sided lesion .](images/app.statdx.com_image_f2086d11-e747-4b89-99d4-34c16ebd985c_cd1a99bd_20251018T065014Z.jpg) -*Axial FLAIR MR shows a large, tumefactive, hyperintense ADEM lesion with mass effect less than expected for the size of the lesion. Another clue to its nonneoplastic nature is the right-sided lesion .* - -![Axial FLAIR MR shows a large, tumefactive, hyperintense ADEM lesion with mass effect less than expected for the size of the lesion. Another clue to its nonneoplastic nature is the right-sided lesion .](images/app.statdx.com_image_thumbnail_f2086d11-e747-4b89-99d4-34c16ebd985c_size_168_quality_85_726f0ff0_20251018T064936Z.jpg) -*Axial FLAIR MR shows a large, tumefactive, hyperintense ADEM lesion with mass effect less than expected for the size of the lesion. Another clue to its nonneoplastic nature is the right-sided lesion .* - -![MRS at a long TE in the same patient shows the tumefactive lesion has a depressed choline and NAA metabolites in the presence of a large lactate doublet . This MRS helps distinguish this lesion from a neoplasm. MRS of ADEM may show elevated choline acutely.](images/app.statdx.com_image_cae7da61-ff92-4736-8487-7596beca115c_60dd87b3_20251018T065015Z.jpg) -*MRS at a long TE in the same patient shows the tumefactive lesion has a depressed choline and NAA metabolites in the presence of a large lactate doublet . This MRS helps distinguish this lesion from a neoplasm. MRS of ADEM may show elevated choline acutely.* - -![MRS at a long TE in the same patient shows the tumefactive lesion has a depressed choline and NAA metabolites in the presence of a large lactate doublet . This MRS helps distinguish this lesion from a neoplasm. MRS of ADEM may show elevated choline acutely.](images/app.statdx.com_image_thumbnail_cae7da61-ff92-4736-8487-7596beca115c_size_168_quality_85_e460a925_20251018T064936Z.jpg) -*MRS at a long TE in the same patient shows the tumefactive lesion has a depressed choline and NAA metabolites in the presence of a large lactate doublet . This MRS helps distinguish this lesion from a neoplasm. MRS of ADEM may show elevated choline acutely.* - -![Axial FLAIR MR shows typical findings of ADEM with peripheral, subcortical hyperintense foci . Bilateral insular involvement is seen . Periventricular and callososeptal lesions, which are typical of multiple sclerosis, are not commonly seen in ADEM.](images/app.statdx.com_image_21d4f966-efa4-4a49-84b0-70be248001f8_461342d2_20251018T065016Z.jpg) -*Axial FLAIR MR shows typical findings of ADEM with peripheral, subcortical hyperintense foci . Bilateral insular involvement is seen . Periventricular and callososeptal lesions, which are typical of multiple sclerosis, are not commonly seen in ADEM.* - -![Axial FLAIR MR shows typical findings of ADEM with peripheral, subcortical hyperintense foci . Bilateral insular involvement is seen . Periventricular and callososeptal lesions, which are typical of multiple sclerosis, are not commonly seen in ADEM.](images/app.statdx.com_image_thumbnail_21d4f966-efa4-4a49-84b0-70be248001f8_size_168_quality_85_5f6fc3a6_20251018T064936Z.jpg) -*Axial FLAIR MR shows typical findings of ADEM with peripheral, subcortical hyperintense foci . Bilateral insular involvement is seen . Periventricular and callososeptal lesions, which are typical of multiple sclerosis, are not commonly seen in ADEM.* - diff --git a/out/ahle_0ec0bca6-abee-4931-a6ed-43541b626261.md b/out/ahle_0ec0bca6-abee-4931-a6ed-43541b626261.md deleted file mode 100644 index abfc76d..0000000 --- a/out/ahle_0ec0bca6-abee-4931-a6ed-43541b626261.md +++ /dev/null @@ -1,326 +0,0 @@ ---- -title: "AHLE" -docid: "0ec0bca6-abee-4931-a6ed-43541b626261" -authors: - - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850" - value: "Anne G. Osborn, MD, FACR" -breadcrumbs: - - - name: "Brain" - slug: "brain" - treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" - - - name: "Diagnosis" - slug: "diagnosis" - treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" - - - name: "Pathology-Based Diagnoses" - slug: "pathology-based-diagnoses" - treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" - - - name: "Infectious, Inflammatory, and Demyelinating Disease" - slug: "infectious-inflammatory-and-demyel-" - treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607" - - - name: "Inflammatory and Demyelinating Disease" - slug: "inflammatory-and-demyelinating-dis-" - treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc" - - - name: "AHLE" - slug: "ahle" - treeNodeId: null -category: "Brain" -documentVersionId: "05d076c7-6110-4f03-952a-c22726d85e4d" -imageCount: 12 -lastUpdated: "08/05/20" -pageDescription: "AHLE" -pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, AHLE" -pageTitle: "AHLE | STATdx" -enhancedTitle: "AHLE" -type: "DX" -references: true -breadcrumbs: - - "Brain" - - "Diagnosis" - - "Pathology-Based Diagnoses" - - "Infectious, Inflammatory, and Demyelinating Disease" - - "Inflammatory and Demyelinating Disease" - - "AHLE" ---- -# KEY FACTS - -- ## Terminology - - - - Hyperacute, fulminant hemorrhagic perivascular demyelinating disorder - - Usually viral or immunization related -- ## Imaging - - - - Best imaging: MR with T2* GRE, SWI - - CT: May be normal if only microbleeds present - - MR - - Multifocal scattered or confluent WM hyperintensities on T2/FLAIR - - T2* shows multifocal blooming microbleeds in WM (often striking sparing of cortex) - - Most striking in corpus callosum - - Less common: Large, lobar, confluent hemorrhages - - SWI significantly more sensitive than GRE -- ## Top Differential Diagnoses - - - - Acute disseminated encephalomyelitis (ADEM) - - Multiple sclerosis - - Acute necrotizing encephalopathy - - Other brain "microbleeds" - - Critical illness associated (e.g., ARDS ± on ECMO) - - Trauma (DAI), fat emboli, HUS/TTP - - Sepsis, vasculitis, hemorrhagic viral fevers - - High-altitude cerebral edema -- ## Clinical Issues - - - - Demographics - - AHLE represents ~ 2% of ADEM cases - - All ages but children, young adults most common - - Presentation and course - - Fever, then rapid neurologic deterioration - - 60-80% mortality without treatment -- ## Diagnostic Checklist - - - - T2* (GRE or SWI) MR in all febrile CNS illnesses with rapid clinical deterioration - -# TERMINOLOGY - -- ## Abbreviations - - - - Acute hemorrhagic leukoencephalitis (AHLE) - - Acute disseminated encephalomyelopathy (ADEM) -- ## Synonyms - - - - Acute hemorrhagic encephalomyelitis (AHEM) - - Weston-Hurst disease -- ## Definitions - - - - Hyperacute, fulminant, hemorrhagic perivascular demyelinating disorder - - Usually post viral or immunization related - - May be exceptionally severe, fulminant form of ADEM - -# IMAGING - -- ## General Features - - - - ### Best diagnostic clue - - - - T2* shows multifocal petechial white matter (WM) microhemorrhages with striking sparing of cortex - - Less common: Large, lobar, confluent hemorrhages - - ### Location - - - - WM - - Corpus callosum, subcortical WM (U fibers) - - Less common: Basal ganglia, midbrain, pons, cerebellum - - ### Size - - - - Punctate microbleeds > large, lobar hemorrhages -- ## CT Findings - - - - ### NECT - - - - May be normal if only microbleeds present - - ± WM edema with hypodensity -- ## MR Findings - - - - ### T1WI - - - - Often normal - - ### T2WI - - - - Multifocal scattered or confluent WM hyperintensities - - Most striking in corpus callosum - - May have reversible corpus callosum splenium lesion - - ### T2* GRE - - - - Multifocal blooming microbleeds - - SWI significantly more sensitive than GRE -- ## Imaging Recommendations - - - - ### Best imaging tool - - - - MR with T2* GRE, SWI - -# DIFFERENTIAL DIAGNOSIS - -- [Acute Disseminated Encephalomyelitis](/document/adem/a3fafeb7-5861-4364-beb8-c0e30220564e) - - AHLE may be most severe form of ADEM - - ADEM usually less fulminant - - ADEM lacks lobar or perivascular hemorrhages of AHLE -- [Multiple Sclerosis](/document/multiple-sclerosis/7892b2a2-f52a-4d7f-9858-a326f2b7ab04) - - Acute fulminant multiple sclerosis - - Lacks hemorrhage, high fever, marked leukocytosis -- ## Acute Necrotizing Encephalopathy - - - - Rare complication of acute viral infection, such as influenza A - - Children < 4 years most common - - Bilateral, symmetric lesions in thalami typical - - WM predominance rare -- ## Other Etiologies of Brain Microbleeds - - - - Diffuse, traumatic vascular injury - - Critical illness-associated (e.g., ARDS ± on ECMO) - - Sepsis, vasculitis - - Fat emboli - - HUS/TTP - - Hemorrhagic infections - - Viral (H1N1, coronavirus), malaria, rickettsia - - High-altitude cerebral edema - -# PATHOLOGY - -- ## Gross Pathologic & Surgical Features - - - - Predominantly involves WM of brain ± spinal cord - - May affect basal ganglia but usually spares cortical gray matter - - Focal confluent &/or multifocal petechial WM hemorrhages -- ## Microscopic Features - - - - Fibrinoid necrosis of vessel walls - - Perivascular demyelination, hemorrhages - -# CLINICAL ISSUES - -- ## Presentation - - - - ### Most common signs/symptoms - - - - Fever, then rapid neurologic deterioration - - Somnolence, impaired consciousness - - ### Other signs/symptoms - - - - Long-tract signs -- ## Demographics - - - - AHLE represents ~ 2% of ADEM cases - - All ages but children, young adults most common -- ## Natural History & Prognosis - - - - Rapid clinical deterioration; death within days typical course - - Mortality: 60-80% -- ## Treatment - - - - Aggressive IV steroids, immunoglobulin, plasmapheresis - -# DIAGNOSTIC CHECKLIST - -- ## Consider - - - - T2* (GRE or SWI) MR in all febrile CNS illnesses with rapid clinical deterioration -- ## Image Interpretation Pearls - - - - Corpus callosum, subcortical WM microbleeds with striking sparing of overlying cortex typical for AHLE - - 8f325204-5ce7-4da8-b816-cb545fa5b054 - -## References - -# Selected References - -1. [Mondia MWL et al: Acute hemorrhagic leukoencephalitis of Weston Hurst secondary to herpes encephalitis presenting as status epilepticus: a case report and review of literature. J Clin Neurosci. 67:265-70, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31239199%5Bpmid%5D) -1. [Yae Y et al: Fulminant acute disseminated encephalomyelitis in children. Brain Dev. 41(4):373-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30522797%5Bpmid%5D) -1. [Bonduelle T et al: Weston-Hurst syndrome with acute hemorrhagic cerebellitis. Clin Neurol Neurosurg. 173:118-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30121019%5Bpmid%5D) -1. [Fanou EM et al: Critical illness-associated cerebral microbleeds. Stroke. 48(4):1085-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28235962%5Bpmid%5D) -1. [Nabi S et al: Weston-Hurst syndrome: a rare fulminant form of acute disseminated encephalomyelitis (ADEM). BMJ Case Rep. 2016, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27797801%5Bpmid%5D) -1. [Jeganathan N et al: Acute hemorrhagic leukoencephalopathy associated with influenza A (H1N1) virus. Neurocrit Care. 19(2):218-21, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23943349%5Bpmid%5D) - - -## Images - - -### Selected Images - -![Close-up view of autopsied brain in a patient with acute hemorrhagic leukoencephalitis (AHLE) shows innumerable tiny microbleeds in the subcortical and deep white matter (WM) and corpus callosum . Note the overlying cortex is almost completely spared . (Courtesy E. Rushing, MD.)](images/app.statdx.com_image_93f46c03-6a13-429c-9a24-57e552795482_70fac743_20251018T070801Z.jpg) -*Close-up view of autopsied brain in a patient with acute hemorrhagic leukoencephalitis (AHLE) shows innumerable tiny microbleeds in the subcortical and deep white matter (WM) and corpus callosum . Note the overlying cortex is almost completely spared . (Courtesy E. Rushing, MD.)* - -![Close-up view of autopsied brain in a patient with acute hemorrhagic leukoencephalitis (AHLE) shows innumerable tiny microbleeds in the subcortical and deep white matter (WM) and corpus callosum . Note the overlying cortex is almost completely spared . (Courtesy E. Rushing, MD.)](images/app.statdx.com_image_thumbnail_93f46c03-6a13-429c-9a24-57e552795482_size_168_quality_85_3867679d_20251018T070800Z.jpg) -*Close-up view of autopsied brain in a patient with acute hemorrhagic leukoencephalitis (AHLE) shows innumerable tiny microbleeds in the subcortical and deep white matter (WM) and corpus callosum . Note the overlying cortex is almost completely spared . (Courtesy E. Rushing, MD.)* - -![Close-up view of autopsied brain in a patient with acute hemorrhagic leukoencephalitis (AHLE) shows innumerable tiny microbleeds in the subcortical and deep white matter (WM) and corpus callosum . Note the overlying cortex is almost completely spared . (Courtesy E. Rushing, MD.)](images/app.statdx.com_image_thumbnail_93f46c03-6a13-429c-9a24-57e552795482_size_174_quality_85_88ff12ab_20251018T122441Z.jpg) -*Close-up view of autopsied brain in a patient with acute hemorrhagic leukoencephalitis (AHLE) shows innumerable tiny microbleeds in the subcortical and deep white matter (WM) and corpus callosum . Note the overlying cortex is almost completely spared . (Courtesy E. Rushing, MD.)* - -![Axial FLAIR MR in a 28-year-old man with rapidly declining mental status after a flu-like illness shows patchy hyperintensities in the corpus callosum and deep/subcortical WM .](images/app.statdx.com_image_bc85bdf7-9d59-41a2-aa1c-4936434aeb8a_bb2e3243_20251018T070813Z.jpg) -*Axial FLAIR MR in a 28-year-old man with rapidly declining mental status after a flu-like illness shows patchy hyperintensities in the corpus callosum and deep/subcortical WM .* - -![Axial FLAIR MR in a 28-year-old man with rapidly declining mental status after a flu-like illness shows patchy hyperintensities in the corpus callosum and deep/subcortical WM .](images/app.statdx.com_image_thumbnail_bc85bdf7-9d59-41a2-aa1c-4936434aeb8a_size_168_quality_85_ef2bd8af_20251018T070800Z.jpg) -*Axial FLAIR MR in a 28-year-old man with rapidly declining mental status after a flu-like illness shows patchy hyperintensities in the corpus callosum and deep/subcortical WM .* - -![Axial T2* GRE MR in the same patient shows multiple tiny hypointensities in the corpus callosum and deep/subcortical WM . The cortex is largely spared.](images/app.statdx.com_image_57828b57-0c4e-4be9-80d0-971f2d79ffb4_b3737dfb_20251018T070817Z.jpg) -*Axial T2* GRE MR in the same patient shows multiple tiny hypointensities in the corpus callosum and deep/subcortical WM . The cortex is largely spared.* - -![Axial T2* GRE MR in the same patient shows multiple tiny hypointensities in the corpus callosum and deep/subcortical WM . The cortex is largely spared.](images/app.statdx.com_image_thumbnail_57828b57-0c4e-4be9-80d0-971f2d79ffb4_size_168_quality_85_e0d7750b_20251018T070800Z.jpg) -*Axial T2* GRE MR in the same patient shows multiple tiny hypointensities in the corpus callosum and deep/subcortical WM . The cortex is largely spared.* - -![Axial T2* SWI MR MIP shows the innumerable microbleeds in the corpus callosum with diffuse involvement of the hemispheric WM in this patient with AHLE.](images/app.statdx.com_image_9228cf7b-7628-4416-aff0-05b17a828cbc_2992bc89_20251018T070821Z.jpg) -*Axial T2* SWI MR MIP shows the innumerable microbleeds in the corpus callosum with diffuse involvement of the hemispheric WM in this patient with AHLE.* - -![Axial T2* SWI MR MIP shows the innumerable microbleeds in the corpus callosum with diffuse involvement of the hemispheric WM in this patient with AHLE.](images/app.statdx.com_image_thumbnail_9228cf7b-7628-4416-aff0-05b17a828cbc_size_168_quality_85_ee5c235b_20251018T070800Z.jpg) -*Axial T2* SWI MR MIP shows the innumerable microbleeds in the corpus callosum with diffuse involvement of the hemispheric WM in this patient with AHLE.* - - -### Additional Images - -![Axial FLAIR MR in a 25-year-old man with fever and rapidly decreasing mental status shows no definite abnormalities.](images/app.statdx.com_image_328245bb-e479-4018-a04e-d266db0c951b_77fc58cc_20251018T070823Z.jpg) -*Axial FLAIR MR in a 25-year-old man with fever and rapidly decreasing mental status shows no definite abnormalities.* - -![Axial FLAIR MR in a 25-year-old man with fever and rapidly decreasing mental status shows no definite abnormalities.](images/app.statdx.com_image_thumbnail_328245bb-e479-4018-a04e-d266db0c951b_size_168_quality_85_f1c882c4_20251018T070800Z.jpg) -*Axial FLAIR MR in a 25-year-old man with fever and rapidly decreasing mental status shows no definite abnormalities.* - -![Axial T2* GRE MR in the same patient shows a few punctate "blooming" foci in the corpus callosum genu and splenium . The remainder of the WM appears normal.](images/app.statdx.com_image_cb9da6f4-8eb1-4e0f-8f9b-b3bc86522b86_9f11d750_20251018T070825Z.jpg) -*Axial T2* GRE MR in the same patient shows a few punctate "blooming" foci in the corpus callosum genu and splenium . The remainder of the WM appears normal.* - -![Axial T2* GRE MR in the same patient shows a few punctate "blooming" foci in the corpus callosum genu and splenium . The remainder of the WM appears normal.](images/app.statdx.com_image_thumbnail_cb9da6f4-8eb1-4e0f-8f9b-b3bc86522b86_size_168_quality_85_5644deca_20251018T070800Z.jpg) -*Axial T2* GRE MR in the same patient shows a few punctate "blooming" foci in the corpus callosum genu and splenium . The remainder of the WM appears normal.* - -![Axial SWI MR n the same patient shows innumerable tiny "blooming" microbleeds in the corpus callosum and subcortical and deep WM . The cortex is largely spared.](images/app.statdx.com_image_thumbnail_7dcafdbc-3901-40ca-96dd-74eccc11d791_size_168_quality_85_fcdd0bea_20251018T070800Z.jpg) -*Axial SWI MR n the same patient shows innumerable tiny "blooming" microbleeds in the corpus callosum and subcortical and deep WM . The cortex is largely spared.* - -![More cephalad axial T2* SWI MR shows innumerable tiny "blooming" foci in the WM, especially in the corpus callosum . These imaging findings are characteristic of AHLE.](images/app.statdx.com_image_thumbnail_a68ad5c0-2643-4b64-9fb3-faea8207fe58_size_168_quality_85_11cb2b05_20251018T070800Z.jpg) -*More cephalad axial T2* SWI MR shows innumerable tiny "blooming" foci in the WM, especially in the corpus callosum . These imaging findings are characteristic of AHLE.* - -![Autopsy shows 2 areas of gross hemorrhagic necrosis . These findings and clinical history of prior flu-like illness with rapidly progressive fatal clinical course are characteristic of AHLE. (Courtesy R. Hewlett, MD).](images/app.statdx.com_image_thumbnail_782f752f-48ad-404d-8836-0f634954e747_size_168_quality_85_9d3d21e2_20251018T070800Z.jpg) -*Autopsy shows 2 areas of gross hemorrhagic necrosis . These findings and clinical history of prior flu-like illness with rapidly progressive fatal clinical course are characteristic of AHLE. (Courtesy R. Hewlett, MD).* - -![Axial T2* GRE MR in a patient with rapid decline after a flu-like illness shows a large left frontal hemorrhage with numerous "blooming" foci in multiple WM lesions . Diagnosis was AHLE. (Courtesy R. Ramakantan, MD).](images/app.statdx.com_image_thumbnail_4c0a24f1-bc15-49c3-bf38-536daaefccb4_size_168_quality_85_1ae5e76e_20251018T070800Z.jpg) -*Axial T2* GRE MR in a patient with rapid decline after a flu-like illness shows a large left frontal hemorrhage with numerous "blooming" foci in multiple WM lesions . Diagnosis was AHLE. (Courtesy R. Ramakantan, MD).* - -![Axial NECT in a patient with AHLE shows patchy hemorrhages in the corpus callosum splenium .](images/app.statdx.com_image_thumbnail_bfa72b67-3004-4561-a0e0-e8111f33394e_size_168_quality_85_6e30ce1d_20251018T070800Z.jpg) -*Axial NECT in a patient with AHLE shows patchy hemorrhages in the corpus callosum splenium .* - -![Axial T2* SWI MR MIP in the same patient shows the microbleeds are heavily concentrated in the corpus callosum and hemispheric WM . Note involvement of the internal capsules and relative sparing of the basal ganglia and cortex.](images/app.statdx.com_image_thumbnail_ee366227-5d90-4e0d-bde0-f550da761ab8_size_168_quality_85_daba2c9c_20251018T070800Z.jpg) -*Axial T2* SWI MR MIP in the same patient shows the microbleeds are heavily concentrated in the corpus callosum and hemispheric WM . Note involvement of the internal capsules and relative sparing of the basal ganglia and cortex.* - diff --git a/out/aqueductal-stenosis_6dfa6261-3945-4606-850b-51484d05e70c.md b/out/aqueductal-stenosis_6dfa6261-3945-4606-850b-51484d05e70c.md new file mode 100644 index 0000000..bbf73bf --- /dev/null +++ b/out/aqueductal-stenosis_6dfa6261-3945-4606-850b-51484d05e70c.md @@ -0,0 +1,430 @@ +--- +title: "Aqueductal Stenosis" +docid: "6dfa6261-3945-4606-850b-51484d05e70c" +authors: + - key: "2c9d2e67-05db-4d26-b8cb-02e0f7566179" + value: "Usha D. Nagaraj, MD" + - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607" + value: "Surjith Vattoth, MD, FRCR" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "Aqueductal Stenosis" + slug: "aqueductal-stenosis" + treeNodeId: null +category: "Brain" +cmeTopicId: "22225b65-7fc8-4415-b55f-20fb85f6ecf5" +documentVersionId: "f14f3356-bf6d-4b97-a2e5-77751c20492b" +imageCount: 15 +lastUpdated: "07/16/20" +pageDescription: "Aqueductal Stenosis" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Aqueductal Stenosis" +pageTitle: "Aqueductal Stenosis | STATdx" +enhancedTitle: "Aqueductal Stenosis" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "Aqueductal Stenosis" +--- +# KEY FACTS + +- ## Terminology + + + - Aqueductal stenosis (AS) +- ## Imaging + + + - Ventriculomegaly of lateral and 3rd ventricles with normal-sized 4th ventricle + - Obstruction of cerebral aqueduct ± tectal thickening + - Macrocephaly in fetus and infant + - Multiplanar MR with sagittal 3D True FISP/bFFE sequence to evaluate aqueduct +- ## Top Differential Diagnoses + + + - Supratentorial volume loss + - Benign enlargement of subarachnoid fluid spaces of infancy + - Communicating hydrocephalus + - Secondary obstructive hydrocephalus +- ## Pathology + + + - Congenital AS is common cause of fetal hydrocephalus + - Can be acquired (isolated) or associated with genetic disorder + - Subsets include stenosis from tectal thickening, obstructing web/gliotic tissue, or forking +- ## Clinical Issues + + + - Though may present at any time from birth to adulthood, bimodal distribution in 1st year of life and adolescence + - Headache, papilledema, 6th nerve palsy, macrocephaly, bulging fontanelle + - C-section may be required for prenatally diagnosed cases due to macrocephaly +- ## Diagnostic Checklist + + + - Look for coexisting brain anomalies, such as rhombencephalosynapsis or diencephalic-mesencephalic dysplasia + +# TERMINOLOGY + +- ## Abbreviations + + + - Aqueductal stenosis (AS) +- ## Definitions + + + - Ventriculomegaly involving lateral and 3rd ventricles as result of complete or partial obstruction to CSF flow within cerebral aqueduct + - AS diagnosis generally refers to congenital form characterized by varying degree of obstruction at level of cerebral aqueduct + - However, tumors, germinal matrix hemorrhage, or vascular lesions can obstruct aqueduct as well + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Ventriculomegaly of lateral and 3rd ventricles with normal-sized 4th ventricle + - Macrocephaly (typical of AS) can help differentiate from supratentorial volume loss (usually normal or decreased head circumference) + - ### Location + + + - Cerebral aqueduct + - Most commonly at superior colliculi or intercollicular sulcus level + - ### Size + + + - Normal mean aqueductal cross-sectional area at birth is 0.2-1.8 mm² + - ### Morphology + + + - Funnel-shaped enlargement of proximal cerebral aqueduct or diffuse ↓ caliber of entire aqueduct +- ## CT Findings + + + - ### NECT + + + - Ventriculomegaly of lateral and 3rd ventricles, normal-sized 4th ventricle + - ± periventricular interstitial edema from uncompensated hydrocephalus +- ## MR Findings + + + - ### T1WI + + + - Ventriculomegaly of lateral and 3rd ventricles, foramina of Monro + - Corpus callosum (CC) thinned, stretched upward + - Often limits evaluation for coexisting callosal dysgenesis + - ± lateral ventricular diverticulum (a.k.a. ventricular rupture or dehiscence) + - Extraaxial CSF effacement + - Limits ability to evaluate gyral-sulcal pattern + - Normal size of 4th ventricle, basilar foramina + - Aqueductal web: Thin tissue membrane separating dilated aqueduct from normal-sized 4th ventricle + - Look for coexisting brain anomalies, such as rhombencephalosynapsis or diencephalic-mesencephalic dysplasia (incomplete segmentation between diencephalon and mesencephalon) + - ### T2WI + + + - Presence of dephasing jet or flow void through aqueduct may suggest that AS is less likely, though does not completely exclude diagnosis + - ± periventricular interstitial edema + - Tectal plate thickening + - Loss of differentiation between superior and inferior colliculi + - May be difficult to differentiate from tectal plate glioma in certain cases + - AS should **not** have T2-/FLAIR hyperintense signal or enhancement in tectal plate (tectum should be isointense to rest of midbrain on all pulse sequences) + - Unlikely to be tectal plate glioma < 3 years of age + - Septum pellucidum often absent secondary to perforation + - ### T2* GRE + + + - May have trace amounts of blood products in aqueduct and ventricular system but no frank germinal matrix hemorrhage or other cause for bleeding + - ### T1WI C+ + + + - Presence of tumor enhancement excludes congenital AS + - Hydrocephalus may induce leptomeningeal venous stasis → mimics meningitis or CSF metastases + - ### MRA + + + - Upward displacement of anterior cerebral artery branches secondary to hydrocephalus + - ### MRV + + + - Downward displacement of internal cerebral veins secondary to hydrocephalus + - ### MR cine + + + - Phase-contrast imaging may demonstrate absent or diminished CSF flow in aqueduct +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Ventriculomegaly of lateral and 3rd ventricles with normal-sized 4th ventricle in newborn with macrocephaly is highly suggestive of AS + - Obstetrical ultrasound may permit prenatal diagnosis + - Usually severe lateral ventriculomegaly (> 15 mm) + - Decreased transverse cerebellar diameter with coexisting rhombencephalosynapsis; fetal MR can help confirm + - Adducted thumbs in male fetus raise possibility of X-linked hydrocephalus +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Multiplanar MR with sagittal 3D True FISP/bFFE sequence to evaluate aqueduct + +# DIFFERENTIAL DIAGNOSIS + +- ## Supratentorial Volume Loss + + + - Should have normal or decreased head circumference +- ## Benign Enlargement of Subarachnoid Spaces in Infancy + + + - a.k.a "benign macrocrania," thought to be mild form of communicating hydrocephalus from immaturity of CSF absorption mechanisms + - Patients have normal neurologic exam + - Ventricles are normal in size or mildly enlarged +- ## Communicating Hydrocephalus + + + - Secondary to impaired absorption of CSF in subarachnoid spaces rather than anatomic obstruction + - Causes include meningitis, leptomeningeal metastatic disease, venous hypertension +- ## Secondary Obstructive Hydrocephalus + + + - Cause of obstruction is extrinsic to aqueduct: Tumor, germinal matrix hemorrhage, vascular lesion, etc. + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - AS + - Aqueductal lumen normally decreases in size beginning in 2nd month of fetal life and continuing until birth + - Normal mean cross-sectional area of aqueduct is 0.5 mm² (range: 0.2-1.8 mm²) + - Narrowing caused by growth pressures upon aqueduct from adjacent mesencephalic structures + - AS pathologically obstructs CSF flow into 4th ventricle + - CSF production in choroid plexus continues → lateral/3rd ventricular fluid ↑ pressure, ventriculomegaly + - Ventricles expand, compress adjacent parenchyma, stretch CC + - May rupture/open ependymal cell junctions → periventricular edema, ventricular diverticulum + - May be acquired or genetic + - Isolated acquired forms from prior injury/insult, such as trace hemorrhage or infection, which results in webs or gliotic tissue obstructing aqueduct + - Genetic forms often have other associated anomalies + - ### Genetics + + + - X-linked hydrocephalus + - One of most common inherited causes of AS + - Caused by mutation of *L1CAM*gene + - Gene located on X chromosome (Xq28) + - *L1CAM* expression is essential during normal embryonic development of nervous system + - Codes for neural cell adhesion molecule transmembrane glycoprotein in immunoglobulin superfamily of cell adhesion molecules + - Site of mutation within L1 protein correlates with disease severity + - Patients have poor prognosis despite early shunting + - Associated syndromes + - MASA syndrome: **Mental disability**, **a**phasia, **s**huffling gait, and **a**dducted thumbs + - CRASH syndrome: **C**allosal hypoplasia, mental disability, **a**dducted thumbs, **s**pastic paraplegia, and X-linked **h**ydrocephalus + - ### Associated abnormalities + + + - Rhombencephalosynapsis + - Up to 65% of patients with rhombencephalosynapsis have coexisting AS + - CRASH syndrome + - Absence/diminution of corticospinal tracts, thalamic fusion, collicular fusion, absence of septum pellucidum, CC dysgenesis + - Thin cerebral mantle, malformations of cortical development, hypoplastic white matter + - Dystroglycanopathy (a.k.a. congenital muscular dystrophies, such as Walker-Warburg) + - Usually associated with cerebellar dysplasia and abnormally small brainstem + - Chiari 2 malformation + - Not well described, but coexisting AS suspected in cases with increased head circumference (majority of Chiari 2 patients have decreased head circumference) +- ## Microscopic Features + + + - Can have associated malformations of cortical development with poor differentiation and maturation of cortical neurons on histology + - Aqueductal fork shows branching of aqueduct into dorsal and ventral channels + - Dorsal channel usually divided into several ductules + - These channels cannot be resolved on imaging due to microscopic size + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Symptoms depend upon patient age at time of diagnosis + - Onset can be insidious, may occur from birth to adulthood though typically bimodal distribution + - ### Other signs/symptoms + + + - Headache, papilledema, 6th nerve palsy, bulging fontanelles + - Macrocrania, especially if sutures open + - Parinaud syndrome + - Sun-setting eyes + - Lid retraction + - Tonic downgaze + - Bobble-head doll syndrome (rare) +- ## Demographics + + + - ### Age + + + - Presentation, 2 peaks of distribution: 1 in 1st year of life (more common), other in adolescence + - ### Sex + + + - M:F = 2:1 + - ### Epidemiology + + + - 0.5-1 per 1,000 births, recurrence rate of 1-4.5% in siblings + - AS responsible for ~ 20% of congenital hydrocephalus + - Most common cause of prenatal obstructive hydrocephalus +- ## Natural History & Prognosis + + + - Hydrocephalus usually progressive unless treated + - May stabilize as "arrested" or compensated hydrocephalus + - While isolated congenital AS has much better prognosis than AS with genetic disorder or other brain anomalies, only ~ 1/3 of patients with isolated AS have normal neurodevelopmental outcomes +- ## Treatment + + + - CSF shunt diversion + - Endoscopic 3rd ventriculostomy + - Cerebral aqueductoplasty for membranous and short-segment aqueductal stenoses (selected cases) + - Prenatally diagnosed cases may require C-section due to macrocephaly + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Look for coexisting brain anomalies as they make difference in prognosis +- ## Image Interpretation Pearls + + + - Use thin-section 3D True FISP/bFFE to better delineate aqueduct + + 51b0f452-75a6-4416-baed-960891a1f404 + +## References + +# Selected References + +1. [Guo D et al: A novel nonsense mutation in the L1CAM gene responsible for X-linked congenital hydrocephalus. J Gene Med. e3180, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32128973%5Bpmid%5D) +1. [Alhousseini A et al: Familial hydrocephalus and dysgenesis of the corpus callosum associated with Xp22.33 duplication and stenosis of the aqueduct of sylvius with X-linked recessive inheritance pattern. Gynecol Obstet Invest. 84(4):412-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30965333%5Bpmid%5D) +1. [Heaphy-Henault KJ et al: Congenital aqueductal stenosis: findings at fetal mri that accurately predict a postnatal diagnosis. AJNR Am J Neuroradiol. 39(5):942-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29519789%5Bpmid%5D) +1. [Kline-Fath BM et al: Congenital aqueduct stenosis: progressive brain findings in utero to birth in the presence of severe hydrocephalus. Prenat Diagn. 38(9):706-12, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29927492%5Bpmid%5D) +1. [Tonetti DA et al: Clinical outcomes of isolated congenital aqueductal stenosis. World Neurosurg. 114:e976-81, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29588243%5Bpmid%5D) +1. [Yamada S et al: Current and emerging MR imaging techniques for the diagnosis and management of CSF flow disorders: a review of phase-contrast and time-spatial labeling inversion pulse. AJNR Am J Neuroradiol. 36(4):623-30, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25012672%5Bpmid%5D) +1. [Griessenauer CJ et al: Pediatric tectal plate gliomas: clinical and radiological progression, MR imaging characteristics, and management of hydrocephalus. J Neurosurg Pediatr. 13(1):13-20, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24180680%5Bpmid%5D) +1. [Kartal MG et al: Evaluation of hydrocephalus and other cerebrospinal fluid disorders with MRI: an update. Insights Imaging. 5(4):531-41, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24903254%5Bpmid%5D) +1. [Tully HM et al: Infantile hydrocephalus: a review of epidemiology, classification and causes. Eur J Med Genet. 57(8):359-68, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24932902%5Bpmid%5D) +1. [Ucar M et al: Evaluation of aqueductal patency in patients with hydrocephalus: three-dimensional high-sampling-efficiency technique (SPACE) versus two-dimensional turbo spin echo at 3 Tesla. Korean J Radiol. 15(6):827-35, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25469096%5Bpmid%5D) +1. [Whitehead MT et al: Rhombencephalosynapsis as a cause of aqueductal stenosis: an under-recognized association in hydrocephalic children. Pediatr Radiol. 44(7):849-56, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24633306%5Bpmid%5D) +1. [Muehlmann M et al: Magnetic resonance-based estimation of intracranial pressure correlates with ventriculoperitoneal shunt valve opening pressure setting in children with hydrocephalus. Invest Radiol. 48(7):543-7, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23695081%5Bpmid%5D) +1. [O'Neill BR et al: Rapid sequence magnetic resonance imaging in the assessment of children with hydrocephalus. World Neurosurg. 80(6):e307-12, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23111234%5Bpmid%5D) +1. [Rush ET et al: Four new patients with Gomez-Lopez-Hernandez syndrome and proposed diagnostic criteria. Am J Med Genet A. 161A(2):320-6, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23292994%5Bpmid%5D) +1. [Gallo P et al: The endoscopic trans-fourth ventricle aqueductoplasty and stent placement for the treatment of trapped fourth ventricle: long-term results in a series of 18 consecutive patients. Neurol India. 60(3):271-7, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22824682%5Bpmid%5D) +1. [Ishak GE et al: Rhombencephalosynapsis: a hindbrain malformation associated with incomplete separation of midbrain and forebrain, hydrocephalus and a broad spectrum of severity. Brain. 135(Pt 5):1370-86, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22451504%5Bpmid%5D) +1. [Schroeder C et al: Why does endoscopic aqueductoplasty fail so frequently? Analysis of cerebrospinal fluid flow after endoscopic third ventriculostomy and aqueductoplasty using cine phase-contrast magnetic resonance imaging. J Neurosurg. 117(1):141-9, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=-1%5Bpmid%5D) +1. [Cinalli G et al: Hydrocephalus in aqueductal stenosis. Childs Nerv Syst. 27(10):1621-42, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928028%5Bpmid%5D) +1. [Algin O et al: Phase-contrast MRI and 3D-CISS versus contrast-enhanced MR cisternography on the evaluation of the aqueductal stenosis. Neuroradiology. 52(2):99-108, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19756563%5Bpmid%5D) +1. [Stoquart-El Sankari S et al: Phase-contrast MR imaging support for the diagnosis of aqueductal stenosis. AJNR Am J Neuroradiol. 30(1):209-14, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18832663%5Bpmid%5D) +1. [Bateman GA: Magnetic resonance imaging quantification of compliance and collateral flow in late-onset idiopathic aqueductal stenosis: venous pathophysiology revisited. J Neurosurg. 107(5):951-8, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17977266%5Bpmid%5D) +1. [da Silva LR et al: Endoscopic aqueductoplasty in the treatment of aqueductal stenosis. Childs Nerv Syst. 23(11):1263-8, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17676325%5Bpmid%5D) +1. [Koch-Wiewrodt D et al: Success and failure of endoscopic third ventriculostomy in young infants: are there different age distributions?. Childs Nerv Syst. 22(12):1537-41, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16944172%5Bpmid%5D) +1. [Sansone JM et al: Endoscopic cerebral aqueductoplasty: a trans-fourth ventricle approach. J Neurosurg. 103(5 Suppl):388-92, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16302609%5Bpmid%5D) +1. [Bhattacharyya KB et al: Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature. Acta Neurol Scand. 108(3):216-20, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12911467%5Bpmid%5D) +1. [Tisell M et al: Neurological symptoms and signs in adult aqueductal stenosis. Acta Neurol Scand. 107(5):311-7, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12713521%5Bpmid%5D) +1. [Fukuhara T et al: Clinical features of late-onset idiopathic aqueductal stenosis. Surg Neurol. 55(3):132-6; discussion 136-7, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11311904%5Bpmid%5D) +1. [Partington MD: Congenital hydrocephalus. Neurosurg Clin N Am. 12(4):737-42, ix, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11524294%5Bpmid%5D) +1. [Schroeder HW et al: Endoscopic aqueductoplasty: technique and results. Neurosurgery. 45(3):508-15; discussion 515-8, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10493373%5Bpmid%5D) +1. [Graf WD et al: The pachygyria-polymicrogyria spectrum of cortical dysplasia in X-linked hydrocephalus. Eur J Pediatr Surg. 8 Suppl 1:10-4, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9926316%5Bpmid%5D) +1. [Castro-Gago M et al: Autosomal recessive hydrocephalus with aqueductal stenosis. Childs Nerv Syst. 12(4):188-91, 1996](http://www.ncbi.nlm.nih.gov/pubmed/?term=8739404%5Bpmid%5D) +1. [Kadowaki C et al: Cine magnetic resonance imaging of aqueductal stenosis. Childs Nerv Syst. 11(2):107-11, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7758008%5Bpmid%5D) +1. [Villani R et al: Long-term outcome in aqueductal stenosis. Childs Nerv Syst. 11(3):180-5, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7773981%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal graphic shows obstructive hydrocephalus with markedly enlarged lateral and 3rd ventricles, a stretched (thinned) corpus callosum, and a funnel-shaped cerebral aqueduct related to distal obstruction. Note the normal size of the 4th ventricle and depression of the floor of the 3rd ventricle from the hydrocephalus.](images/app.statdx.com_image_thumbnail_b8037593-f2c6-448f-890c-33e3c832cfb8_annotated_true_size_900_quality_90_d08fbaed_20251018T165041Z.jpg) +*Sagittal graphic shows obstructive hydrocephalus with markedly enlarged lateral and 3rd ventricles, a stretched (thinned) corpus callosum, and a funnel-shaped cerebral aqueduct related to distal obstruction. Note the normal size of the 4th ventricle and depression of the floor of the 3rd ventricle from the hydrocephalus.* + +![Sagittal T2WI from a fetal MR at 25 weeks gestational age with aqueductal stenosis shows macrocephaly, lateral and 3rd ventriculomegaly, and no CSF in the cerebral aqueduct .](images/app.statdx.com_image_thumbnail_cfcc833d-ed65-4463-8b21-0a16f998b61a_annotated_true_size_900_quality_90_0f9aeb64_20251018T165041Z.jpg) +*Sagittal T2WI from a fetal MR at 25 weeks gestational age with aqueductal stenosis shows macrocephaly, lateral and 3rd ventriculomegaly, and no CSF in the cerebral aqueduct .* + +![Sagittal T1WI MR depicts proximal aqueductal stenosis producing enlargement of the lateral and 3rd ventricles with depression of the fornices in conjunction with normal 4th ventricle size. The tectum is dysplastic and thickened with collicular fusion .](images/app.statdx.com_image_thumbnail_691b95bc-abde-45e4-9c41-f468bff7e576_annotated_true_size_900_quality_90_5ca20c30_20251018T165041Z.jpg) +*Sagittal T1WI MR depicts proximal aqueductal stenosis producing enlargement of the lateral and 3rd ventricles with depression of the fornices in conjunction with normal 4th ventricle size. The tectum is dysplastic and thickened with collicular fusion .* + +![Sagittal FIESTA of a 5 year old with aqueductal stenosis secondary to a small obstructing web is shown. This patient underwent a 3rd ventriculostomy and is doing well. There are no other brain anomalies.](images/app.statdx.com_image_thumbnail_7e7c9735-6651-40b7-92d2-9ba4c6ba4eb9_annotated_true_size_900_quality_90_58d84b56_20251018T165041Z.jpg) +*Sagittal FIESTA of a 5 year old with aqueductal stenosis secondary to a small obstructing web is shown. This patient underwent a 3rd ventriculostomy and is doing well. There are no other brain anomalies.* + +![Sagittal T2WI MR of a 5 day old with prenatal diagnosis of aqueductal stenosis demonstrates effacement of the cerebral aqueduct with thickening of the tectum .](images/app.statdx.com_image_thumbnail_6e7dc665-e001-4483-982c-34742a8c9f88_annotated_true_size_900_quality_90_8dc210af_20251018T165041Z.jpg) +*Sagittal T2WI MR of a 5 day old with prenatal diagnosis of aqueductal stenosis demonstrates effacement of the cerebral aqueduct with thickening of the tectum .* + +![Axial T1WI MR in the same patient demonstrates rhombencephalosynapsis and bilateral choanal atresia . Other anomalies in this patient included bilateral microphthalmia and tracheoesophageal fistula. This patient had a partial deletion of chromosome 3q and SOX2 gene mutation.](images/app.statdx.com_image_thumbnail_60101105-9030-4352-ac33-e49762486e93_annotated_true_size_900_quality_90_117f5060_20251018T165041Z.jpg) +*Axial T1WI MR in the same patient demonstrates rhombencephalosynapsis and bilateral choanal atresia . Other anomalies in this patient included bilateral microphthalmia and tracheoesophageal fistula. This patient had a partial deletion of chromosome 3q and SOX2 gene mutation.* + +![Sagittal T1WI MR in a 2 day old with aqueductal stenosis with effacement of the aqueduct is shown. This patient also has diencephalic-mesencephalic dysplasia with incomplete separation of an enlarged massa intermedia from the midbrain with thickening of the 3rd ventricular floor .](images/app.statdx.com_image_thumbnail_7dec61fd-7d33-473d-a72a-826dbfe86d05_annotated_true_size_900_quality_90_6e98cdde_20251018T165041Z.jpg) +*Sagittal T1WI MR in a 2 day old with aqueductal stenosis with effacement of the aqueduct is shown. This patient also has diencephalic-mesencephalic dysplasia with incomplete separation of an enlarged massa intermedia from the midbrain with thickening of the 3rd ventricular floor .* + +![Axial T2WI MR in the same patient demonstrates dilation of the lateral and 3rd ventricles with right ventricular diverticulum and multiple subependymal gray matter heterotopias .](images/app.statdx.com_image_thumbnail_89ddd8bc-0a66-4a10-ba6f-582e15706b98_annotated_true_size_900_quality_90_f90d73d0_20251018T165041Z.jpg) +*Axial T2WI MR in the same patient demonstrates dilation of the lateral and 3rd ventricles with right ventricular diverticulum and multiple subependymal gray matter heterotopias .* + +![Sagittal T1WI MR in a patient with Walker-Warburg syndrome shows severe tectal dysgenesis with aqueductal occlusion. Marked enlargement of the lateral ventricles more than the 3rd ventricle is present. A "zigzag" brainstem and very small cerebellum are characteristic of this syndrome.](images/app.statdx.com_image_thumbnail_803672c1-c4ea-4b63-9503-c1496c7e60c7_annotated_true_size_900_quality_90_ad19ef8f_20251018T165041Z.jpg) +*Sagittal T1WI MR in a patient with Walker-Warburg syndrome shows severe tectal dysgenesis with aqueductal occlusion. Marked enlargement of the lateral ventricles more than the 3rd ventricle is present. A "zigzag" brainstem and very small cerebellum are characteristic of this syndrome.* + +![Coronal T2WI MR in the same patient confirms marked ventriculomegaly, funnel-shaped cerebral aqueductal stenosis , fused fornices , and classic cobblestone lissencephaly.](images/app.statdx.com_image_thumbnail_3f80b454-0197-4bfd-a775-7b7c846e46a4_annotated_true_size_900_quality_90_9ac2b5aa_20251018T160013Z.jpg) +*Coronal T2WI MR in the same patient confirms marked ventriculomegaly, funnel-shaped cerebral aqueductal stenosis , fused fornices , and classic cobblestone lissencephaly.* + + +### Additional Images + +![Coronal T2WI MR of the same neonate, on the 1st day of life, shows marked ventriculomegaly with asymmetric bilateral subdural hygromas following spontaneous ventricular decompression into the bilateral subdural spaces. This patient also has the additional midline congenital anomaly of rhombencephalosynapsis with characteristic incomplete dentate gyrus separation correlating with clinical truncal ataxia.](images/app.statdx.com_image_thumbnail_8cd11d10-23ae-4a94-96b5-f990d00e6278_annotated_true_size_900_quality_90_0f7423b8_20251018T160013Z.jpg) +*Coronal T2WI MR of the same neonate, on the 1st day of life, shows marked ventriculomegaly with asymmetric bilateral subdural hygromas following spontaneous ventricular decompression into the bilateral subdural spaces. This patient also has the additional midline congenital anomaly of rhombencephalosynapsis with characteristic incomplete dentate gyrus separation correlating with clinical truncal ataxia.* + +![Sagittal T2WI MR of a neonate with severe congenital hydrocephalus, imaged on the 1st day of life, shows severe aqueductal stenosis and abnormal dysplastic tectal thickening . Severe congenital hydrocephalus has resulted in spontaneous decompression into the subdural spaces .](images/app.statdx.com_image_thumbnail_0163a4f7-18e5-4811-8e10-7cbfc0576f14_annotated_true_size_900_quality_90_d4c7a9c8_20251018T160014Z.jpg) +*Sagittal T2WI MR of a neonate with severe congenital hydrocephalus, imaged on the 1st day of life, shows severe aqueductal stenosis and abnormal dysplastic tectal thickening . Severe congenital hydrocephalus has resulted in spontaneous decompression into the subdural spaces .* + +![Coronal T2WI MR shows "funneling" of the aqueduct in the coronal plane , with a markedly distended ventricular system proximal to the stenotic aqueduct.](e30bbdfe-503f-454d-a570-d0d071adf442) +*Coronal T2WI MR shows "funneling" of the aqueduct in the coronal plane , with a markedly distended ventricular system proximal to the stenotic aqueduct.* + +![Sagittal T2WI MR shows massively distended 3rd and lateral ventricles with distal aqueductal stenosis . Note the severe stretching of the corpus callosum and depression of the fornices .](39652cf8-1cab-4160-a593-4f0796871390) +*Sagittal T2WI MR shows massively distended 3rd and lateral ventricles with distal aqueductal stenosis . Note the severe stretching of the corpus callosum and depression of the fornices .* + +![Sagittal T2WI MR reveals distal aqueductal stenosis with an enlarged, funnel-shaped cerebral aqueduct and mild abnormal tectal thickening. Note the lateral and 3rd ventriculomegaly with normal size of the 4th ventricle.](891e5fdf-9bb6-4085-b168-4e5738367176) +*Sagittal T2WI MR reveals distal aqueductal stenosis with an enlarged, funnel-shaped cerebral aqueduct and mild abnormal tectal thickening. Note the lateral and 3rd ventriculomegaly with normal size of the 4th ventricle.* + diff --git a/out/asymmetric-lateral-ventricles_87387f0d-9b20-4288-a250-aa3ec83520c4.md b/out/asymmetric-lateral-ventricles_87387f0d-9b20-4288-a250-aa3ec83520c4.md new file mode 100644 index 0000000..2170f64 --- /dev/null +++ b/out/asymmetric-lateral-ventricles_87387f0d-9b20-4288-a250-aa3ec83520c4.md @@ -0,0 +1,413 @@ +--- +title: "Asymmetric Lateral Ventricles" +docid: "87387f0d-9b20-4288-a250-aa3ec83520c4" +authors: + - key: "1fa14dfd-71ea-4960-908e-e720313bc63a" + value: "Santhosh Gaddikeri, MD" + - key: "30ce27b2-237f-4aff-a88f-65ead356335b" + value: "Marinos Kontzialis, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60" + - + name: "Ventricles, Periventricular Regions" + slug: "ventricles-periventricular-regions" + treeNodeId: "353c434a-a6fc-4ef1-8786-d30a1988a4dc" + - + name: "Generic Imaging Patterns" + slug: "generic-imaging-patterns" + treeNodeId: "969c31a2-ef56-4fc3-9125-05857cf9aac3" + - + name: "Asymmetric Lateral Ventricles" + slug: "asymmetric-lateral-ventricles" + treeNodeId: null +category: "Brain" +cmeTopicId: "5536eb32-54f9-4eb3-83c6-15950dc4efe6" +documentVersionId: "7b24399c-3ad6-47c3-9521-b6125b27d26d" +imageCount: 54 +lastUpdated: "01/25/23" +pageDescription: "Asymmetric Lateral Ventricles" +pageKeywords: "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Asymmetric Lateral Ventricles" +pageTitle: "Asymmetric Lateral Ventricles | STATdx" +enhancedTitle: "Asymmetric Lateral Ventricles" +type: "DDX" +references: true +breadcrumbs: + - "Brain" + - "Differential Diagnosis" + - "Ventricles, Periventricular Regions" + - "Generic Imaging Patterns" + - "Asymmetric Lateral Ventricles" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Asymmetric lateral ventricles are most commonly seen as normal variant +- ## Helpful Clues for Common Diagnoses + + + - **Normal Variant** + - Asymmetric lateral ventricles seen in 5-10% of normal population + - Asymmetry mild to moderate, left > right + - Septum may be displaced across midline + - No associated mass effect, herniation, or parenchymal atrophy + - Must exclude parenchymal or intraventricular abnormality + - **Extrinsic Mass Effect** + - Etiologies include mass, hemorrhage, infarct, infection + - Mass can cause ventricular deformity, subfalcine herniation + - **Encephalomalacia, General** + - Parenchymal loss results in compensatory ventricular enlargement + - Common etiologies include chronic infarct, trauma, surgery + - **Intraventricular Hemorrhage** + - Involved ventricle may dilate early from mass effect + - Chronic dilation may be due to scarring and adhesions + - Etiologies include trauma, arteriovenous malformation (AVM), basal ganglia hemorrhage + - **Herniation Syndromes, Intracranial** + - Subfalcine herniation: Cingulate gyrus herniates under falx + - Ipsilateral lateral ventricle compressed + - Foramen of Monro obstructs and causes contralateral lateral ventricle enlargement + - Unilateral descending transtentorial herniation (uncal): Herniation of medial temporal lobe inferiorly + - Contralateral temporal horn becomes entrapped and enlarges + - Entrapped ventricle: Typically temporal horn, by extrinsic mass effect + - **Surgical Defects** + - Look for calvarial defect or "tract" + - Typically related to resection of mass + - Ventricle enlarged unilateral to defect + - **Obstructive Hydrocephalus** + - Typically acquired and bilateral + - May be unilateral if shunt complication or obstructing tumor is cause + - Rare: Colloid cyst may obstruct unilateral foramen of Monro and cause unilateral ventriculomegaly + - **Choroid Plexus Cyst** + - a.k.a. choroid plexus xanthogranuloma + - Most common choroid plexus mass in adults + - Nonneoplastic, noninflammatory cyst of choroid plexus + - Common incidental finding in older patients (40% prevalence) + - Typically bilateral; may be unilateral and enlarge lateral ventricle + - Most in lateral ventricular atria + - 60-80% bright on DWI +- ## Helpful Clues for Less Common Diagnoses + + + - **Ventriculitis** + - Ventriculomegaly with debris level, enhancing ependyma, periventricular T2/FLAIR hyperintensities + - May affect lateral ventricles asymmetrically, particularly if related to shunt placement or abscess rupture + - Restricted diffusion of layering debris is characteristic + - Bacterial ventriculitis may occur in healthy individuals after trauma or neurosurgical procedure + - Fungal or viral ventriculitis occurs most commonly in immunosuppressed patients + - **CSF Shunts and Complications** + - Common complications include shunt obstruction or breakage, infection, overdrainage + - Asymmetric ventricles may result from overdrainage or underdrainage of "isolated" ventricle + - **Meningioma** + - Although rare, still one of more common intraventricular neoplasms in adults + - More common in females (F:M = 2:1) + - Most common location is ventricular atrium with slight majority on left + - Smooth, avidly enhancing intraventricular mass + - 50% calcified; cystic changes may be present + - **Choroid Plexus Papilloma** + - Enhancing, lobulated intraventricular mass in child + - 50% in lateral ventricle atrium, left > right + - 40% in 4th ventricle + - Increased CSF production in most cases, CSF obstruction in some cases + - May have CSF spread of tumor + - **Neurocytoma, Central** + - Neurocytoma arising from ventricular system, usually septum pellucidum or lateral ventricle + - Well-circumscribed, lobulated, "bubbly" lesions + - 50% calcifications, cyst-like areas, enhancement, prominent associated flow voids + - MRS: Glycine peak (3.55 ppm) may help differentiation from other intraventricular neoplasms + - **Neurocysticercosis** + - May involve cisterns > parenchyma > ventricles + - Intraventricular disease anywhere from 0.7-33% of cases + - May lead to obstructive hydrocephalus, ventriculitis, adhesions + - Intraventricular cyst signal intensity may differ slightly from CSF on T1, T2, FLAIR +- ## Helpful Clues for Rare Diagnoses + + + - **Intraventricular Synechiae/Adhesions** + - May be congenital or acquired (prior bleed, infection, tumor) + - Look for enhancing septa, intraventricular cysts within ventricle + - **Choroid Plexus Carcinoma** + - Enhancing intraventricular mass and ependymal invasion in young child + - CSF seeding common + - Imaging does not allow reliable distinction between choroid plexus papilloma and carcinoma + - May be more heterogeneous than choroid plexus papilloma in part reflecting areas of necrosis + - May demonstrate elevation of lactate level + - **Ependymal Cyst** + - Nonenhancing, thin-walled cyst with CSF density/intensity + - Lateral ventricle most common location + - Most are incidental + - Best diagnostic clue: Nonenhancing, thin-walled CSF density/intensity cyst in lateral ventricle + - **Dyke-Davidoff-Masson** + - Antenatal unilateral hemispheric insult causes cerebral hemiatrophy + - Compensatory ipsilateral calvarial thickening, hyperpneumatized frontal sinuses and temporal bones due to longstanding cerebral hemiatrophy + - Dilated ventricle from volume loss is ipsilateral to small hemisphere + - **Hemimegalencephaly** + - Unilateral hemispheric cortical thickening + - Dilated, usually dysmorphic ventricle ipsilateral to enlarged hemisphere + - Ipsilateral extracalvarial soft tissues may be larger + - Pachygyria, polymicrogyria, heterotopias, abnormal white matter signal, blurring of gray-white matter junction + - **Rasmussen Encephalitis** + - Chronic unilateral brain inflammation of uncertain etiology leading to progressive hemispheric atrophy + - Early focal swelling of gyri + - Unilateral, predominantly frontoinsular cortical and subcortical T2/FLAIR hyperintensity progress to atrophy +- ## Other Essential Information + + + - High-resolution MR cisternography: CISS, balanced FFE, FIESTA + - May detect small septations or arachnoid membranes causing obstruction + - Cine CSF flow study may help detect physiologic flow obstruction from arachnoid webs or membranes + - May assess adequacy of drainage procedures + +## References + +# Selected References + +1. [Balasubramaniam C: Shunt complications - staying out of trouble. Neurol India. 69(Supplement):S495-501, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=35103008%5Bpmid%5D) +1. [Lanska DJ: Cruveilhier's unrecognized case (c1831) of Dyke-Davidoff-Masson syndrome. Eur Neurol. 84(4):300-6, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33965957%5Bpmid%5D) +1. [Cay-Martinez KC et al: Rasmussen encephalitis: an update. Semin Neurol. 40(2):201-10, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32185790%5Bpmid%5D) +1. [Crawford JR et al: Perinatal (fetal and neonatal) choroid plexus tumors: a review. Childs Nerv Syst. 35(6):937-44, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30953158%5Bpmid%5D) +1. [Tan LA et al: Obstructive hydrocephalus due to intraventricular hemorrhage after incidental durotomy during lumbar spine surgery. Spine (Phila Pa 1976). 43(5):E316-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=26208226%5Bpmid%5D) +1. [Zamora CA et al: Teaching neuroImages: Dyke-Davidoff-Masson in Sturge-Weber syndrome. Neurology. 85(16):e128, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26481933%5Bpmid%5D) +1. [Smith AB et al: From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 33(1):21-43, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23322825%5Bpmid%5D) +1. [Kimura-Hayama ET et al: Neurocysticercosis: radiologic-pathologic correlation. Radiographics. 30(6):1705-19, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=21071384%5Bpmid%5D) +1. [Kiroğlu Y et al: Cerebral lateral ventricular asymmetry on CT: how much asymmetry is representing pathology? Surg Radiol Anat. 30(3):249-55, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18253688%5Bpmid%5D) +1. [Rastogi S et al: Neuroimaging in pediatric epilepsy: a multimodality approach. Radiographics. 28(4):1079-95, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18635630%5Bpmid%5D) +1. [Osborn AG, Preece MT. Intracranial cysts: radiologic-pathologic correlation and imaging approach. Radiology. 2006 Jun;239(3):650-64.](http://www.ncbi.nlm.nih.gov/pubmed/?term=16714456%5Bpmid%5D) +1. [Koeller KK et al: Cerebral intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 22(6):1473-505, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12432118%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T2 MR demonstrates normal variant anatomy with mild asymmetric prominence of the right lateral ventricle when compared to the left. Note the septum is slightly deviated to the left .](images/app.statdx.com_image_thumbnail_74e87ea4-3c2a-400b-b714-f9fa2db4e66a_annotated_true_size_900_quality_90_2466c18c_20251018T165124Z.jpg) +**Normal Variant** +*Axial T2 MR demonstrates normal variant anatomy with mild asymmetric prominence of the right lateral ventricle when compared to the left. Note the septum is slightly deviated to the left .* + +![Axial T2 MR demonstrates normal variant anatomy with mild asymmetric prominence of the right lateral ventricle when compared to the left. Note the septum is slightly deviated to the left .](images/app.statdx.com_image_thumbnail_74e87ea4-3c2a-400b-b714-f9fa2db4e66a_size_174_quality_85_592b5076_20251018T165117Z.jpg) +**Normal Variant** +*Axial T2 MR demonstrates normal variant anatomy with mild asymmetric prominence of the right lateral ventricle when compared to the left. Note the septum is slightly deviated to the left .* + +![Axial FLAIR MR demonstrates a large, heterogeneous mass in the left frontal lobe with surrounding edema and mass effect with compression of the left lateral ventricle and moderate dilation of the right lateral ventricle .](images/app.statdx.com_image_thumbnail_ddc74acb-a5fe-4147-bc17-3e3862305da7_annotated_true_size_900_quality_90_d599964a_20251018T165124Z.jpg) +**Extrinsic Mass Effect** +*Axial FLAIR MR demonstrates a large, heterogeneous mass in the left frontal lobe with surrounding edema and mass effect with compression of the left lateral ventricle and moderate dilation of the right lateral ventricle .* + +![Axial T2 MR demonstrates a large, cystic encephalomalacia in the left frontal and parietal lobes due to chronic infarction with ex-vacuo dilation of the left lateral ventricle .](images/app.statdx.com_image_thumbnail_f987a5f2-594d-4b3a-84d0-640a0b70c03b_annotated_true_size_900_quality_90_e62de461_20251018T165124Z.jpg) +**Encephalomalacia, General** +*Axial T2 MR demonstrates a large, cystic encephalomalacia in the left frontal and parietal lobes due to chronic infarction with ex-vacuo dilation of the left lateral ventricle .* + +![Axial T2 MR demonstrates a large amount of acute intraventricular hemorrhage with mild dilation of the right lateral ventricle . Note CSF seepage in the peritrigonal region .](1ce39627-5994-4f0b-a77e-c02fcf1d1c66) +**Intraventricular Hemorrhage** +*Axial T2 MR demonstrates a large amount of acute intraventricular hemorrhage with mild dilation of the right lateral ventricle . Note CSF seepage in the peritrigonal region .* + +![Axial NECT demonstrates a large left subdural hematoma and a small parenchymal hemorrhage , resulting in rightward subfalcine herniation , compression of the left lateral ventricle , and mild dilation of the right lateral ventricle .](b9992ffd-4f31-44e2-8c92-83ab8fad30c1) +**Herniation Syndromes, Intracranial** +*Axial NECT demonstrates a large left subdural hematoma and a small parenchymal hemorrhage , resulting in rightward subfalcine herniation , compression of the left lateral ventricle , and mild dilation of the right lateral ventricle .* + +![Axial FLAIR MR demonstrates a large surgical defect in the left frontal lobe due to a tumor resection with resultant mild prominence of the left lateral ventricle .](a77eef3e-29e3-44c5-88bc-d67eff223e44) +**Surgical Defects** +*Axial FLAIR MR demonstrates a large surgical defect in the left frontal lobe due to a tumor resection with resultant mild prominence of the left lateral ventricle .* + +![Axial T1 C+ MR demonstrates enhancing intraventricular mass with obstructive dilation of the left temporal horn .](181895c1-e6ec-4317-a0d8-d85acb1e681a) +**Obstructive Hydrocephalus** +*Axial T1 C+ MR demonstrates enhancing intraventricular mass with obstructive dilation of the left temporal horn .* + +![Axial T2 MR demonstrates a shunt catheter in the right lateral ventricle with asymmetric lateral ventricles due to overdrainage of the right and mild underdrainage of the left . Note susceptibility artifact due to the shunt reservoir.](6a5a42a2-43a2-4fe6-8dd5-b2b1fc3e83fd) +**CSF Shunts and Complications** +*Axial T2 MR demonstrates a shunt catheter in the right lateral ventricle with asymmetric lateral ventricles due to overdrainage of the right and mild underdrainage of the left . Note susceptibility artifact due to the shunt reservoir.* + +![Axial T2 MR demonstrates a left lateral intraventricular isointense mass in the trigone with dilatation of the left trigone . Note periventricular edema . Biopsy revealed meningioma.](c60f5671-0335-43af-8efa-e151dbc51c78) +**Meningioma** +*Axial T2 MR demonstrates a left lateral intraventricular isointense mass in the trigone with dilatation of the left trigone . Note periventricular edema . Biopsy revealed meningioma.* + +![Axial T2 MR demonstrates a well-circumscribed, isointense, left lateral ventricular mass attached to the septum pellucidum with peripheral tiny cysts . Note moderate dilation of the left lateral ventricle . Biopsy revealed neurocytoma.](ebb2adb7-6287-41d0-8a41-de9563c420d8) +**Neurocytoma, Central** +*Axial T2 MR demonstrates a well-circumscribed, isointense, left lateral ventricular mass attached to the septum pellucidum with peripheral tiny cysts . Note moderate dilation of the left lateral ventricle . Biopsy revealed neurocytoma.* + +![Coronal T2 MR demonstrates asymmetric mild enlargement of the left lateral ventricle due to synechiae obstructing the foramen of Monro.](a22d935b-ac47-4f41-8b5d-57d599e0d11f) +**Intraventricular Synechiae/Adhesions** +*Coronal T2 MR demonstrates asymmetric mild enlargement of the left lateral ventricle due to synechiae obstructing the foramen of Monro.* + +![Axial T2 MR demonstrates a poorly circumscribed heterogeneous left trigonal mass with internal cysts/necrosis. Note extensive periventricular edema . Biopsy revealed choroid plexus carcinoma.](77fdb2fd-452d-4226-b341-62bf87689ec5) +**Choroid Plexus Carcinoma** +*Axial T2 MR demonstrates a poorly circumscribed heterogeneous left trigonal mass with internal cysts/necrosis. Note extensive periventricular edema . Biopsy revealed choroid plexus carcinoma.* + +![Axial NECT demonstrates atrophy of the left cerebral hemisphere with encephalomalacia due to antenatal vascular insult. Note the dilated left lateral ventricle and mild overlying calvarial thickening .](71a10f6b-7ad7-4e54-98ae-1784130732b9) +**Dyke-Davidoff-Masson** +*Axial NECT demonstrates atrophy of the left cerebral hemisphere with encephalomalacia due to antenatal vascular insult. Note the dilated left lateral ventricle and mild overlying calvarial thickening .* + +![Axial T2 MR in a patient with refractory epilepsy due to Rasmussen encephalitis demonstrates left cerebral hemispheric atrophy and mild ex-vacuo dilation of the left lateral ventricle .](71509045-9597-44be-b81f-44e0bc95be4e) +**Rasmussen Encephalitis** +*Axial T2 MR in a patient with refractory epilepsy due to Rasmussen encephalitis demonstrates left cerebral hemispheric atrophy and mild ex-vacuo dilation of the left lateral ventricle .* + + +### Additional Images + +![Axial T1WI C+ MR shows compression of the left frontal horn by a large, periventricular, enhancing mass , primary CNS lymphoma. Extrinsic mass effect is a common cause of ventricular asymmetry.](images/app.statdx.com_image_thumbnail_9c7a2c27-fa74-474a-a33e-3cfbf2329f76_annotated_true_size_900_quality_90_8cafe5b4_20251018T165124Z.jpg) +**Extrinsic Mass Effect** +*Axial T1WI C+ MR shows compression of the left frontal horn by a large, periventricular, enhancing mass , primary CNS lymphoma. Extrinsic mass effect is a common cause of ventricular asymmetry.* + +![Axial FLAIR MR demonstrates a left posterior MCA encephalomalacia resulting in mild ex-vacuo dilatation of the left atrium .](images/app.statdx.com_image_thumbnail_d875dfa4-5133-4dd6-9471-a019de504b4a_annotated_true_size_900_quality_90_e15566aa_20251018T165124Z.jpg) +**Encephalomalacia, General** +*Axial FLAIR MR demonstrates a left posterior MCA encephalomalacia resulting in mild ex-vacuo dilatation of the left atrium .* + +![Axial T2 MR shows right hemiatrophy in Sturge-Weber syndrome. Chronic venous ischemia leads to progressive hemiatrophy. Note the ipsilateral large right ventricle due to volume loss.](images/app.statdx.com_image_thumbnail_370d0b50-9617-4697-8005-d759cf146130_annotated_true_size_900_quality_90_83c359ec_20251018T165124Z.jpg) +**Encephalomalacia, General** +*Axial T2 MR shows right hemiatrophy in Sturge-Weber syndrome. Chronic venous ischemia leads to progressive hemiatrophy. Note the ipsilateral large right ventricle due to volume loss.* + +![Axial NECT shows a basal ganglia hypertensive hemorrhage with intraventricular extension . Associated midline shift results in dilation of the contralateral ventricles from foramen of Monro obstruction.](4897b7a6-5a0f-49c0-8a96-26f7653455b6) +**Intraventricular Hemorrhage** +*Axial NECT shows a basal ganglia hypertensive hemorrhage with intraventricular extension . Associated midline shift results in dilation of the contralateral ventricles from foramen of Monro obstruction.* + +![Coronal FLAIR MR shows a diffusely enlarged hyperintense supratentorial cortex compared to the cerebellum in this patient in longstanding status epilepticus. Disproportionate left hemisphere involvement has resulted in left ventricular compression , right foramen of Monro outlet obstruction, and dilation of the right ventricular system .](b73d4ba3-3f44-41f0-8763-5a8ef431e21a) +**Herniation Syndromes, Intracranial** +*Coronal FLAIR MR shows a diffusely enlarged hyperintense supratentorial cortex compared to the cerebellum in this patient in longstanding status epilepticus. Disproportionate left hemisphere involvement has resulted in left ventricular compression , right foramen of Monro outlet obstruction, and dilation of the right ventricular system .* + +![Coronal T1WI C+ MR shows a right hemispheric, subacute, subdural hematoma causing subfalcine and uncal herniation. Mass effect compresses the right frontal horn. The left ventricle is enlarged from foramen of Monro obstruction.](babb7bb2-a880-4e0d-84e4-a4a72ba57ee1) +**Herniation Syndromes, Intracranial** +*Coronal T1WI C+ MR shows a right hemispheric, subacute, subdural hematoma causing subfalcine and uncal herniation. Mass effect compresses the right frontal horn. The left ventricle is enlarged from foramen of Monro obstruction.* + +![Coronal T2WI MR shows right temporal viral encephalitis causing local mass effect . The left lateral ventricle is larger from foramen of Monro obstruction due to midline shift.](ac578239-383c-479c-9fd1-ec8b74c4c90d) +**Herniation Syndromes, Intracranial** +*Coronal T2WI MR shows right temporal viral encephalitis causing local mass effect . The left lateral ventricle is larger from foramen of Monro obstruction due to midline shift.* + +![Axial T2WI MR shows widening of right foramen of Monro , septum pellucidum deviation , and an enlarged right lateral ventricle in this tuberous sclerosis patient with remote tumor resection.](166a8b5f-51ff-4bff-a262-2f54eae8dec6) +**Surgical Defects** +*Axial T2WI MR shows widening of right foramen of Monro , septum pellucidum deviation , and an enlarged right lateral ventricle in this tuberous sclerosis patient with remote tumor resection.* + +![Axial NECT shows marked enlargement of the left lateral ventricle with bowing of septum pellucidum across midline and transependymal CSF migration indicating acute obstruction. Findings were related to a small atrial diverticulum.](111d8d26-88f6-43d3-8ddb-407a85e2707e) +**Obstructive Hydrocephalus** +*Axial NECT shows marked enlargement of the left lateral ventricle with bowing of septum pellucidum across midline and transependymal CSF migration indicating acute obstruction. Findings were related to a small atrial diverticulum.* + +![Axial T1WI C+ MR shows a colloid cyst believed to be complicated by inflammatory changes. Obstruction of the left foramen of Monro causes unilateral left lateral ventricle dilation .](f99e392c-9132-4bdd-b2fb-665f8ff10dd3) +**Obstructive Hydrocephalus** +*Axial T1WI C+ MR shows a colloid cyst believed to be complicated by inflammatory changes. Obstruction of the left foramen of Monro causes unilateral left lateral ventricle dilation .* + +![Axial T2WI MR shows a medial atrial diverticulum , a rare complication of severe hydrocephalus. CSF pouch herniates inferomedially through tentorial incisura.](51902a9b-bfb3-4df5-b937-6d3eb682f0f6) +**Obstructive Hydrocephalus** +*Axial T2WI MR shows a medial atrial diverticulum , a rare complication of severe hydrocephalus. CSF pouch herniates inferomedially through tentorial incisura.* + +![Coronal T1WI C+ MR shows typical case of coccidioidomycosis meningitis. Note marked enhancement of basal cisterns and asymmetric ventricular enlargement from CSF obstruction.](c4b489b1-dc1a-4d23-84fc-a62cd35c8b12) +**Obstructive Hydrocephalus** +*Coronal T1WI C+ MR shows typical case of coccidioidomycosis meningitis. Note marked enhancement of basal cisterns and asymmetric ventricular enlargement from CSF obstruction.* + +![Axial T1WI C+ MR shows a lobulated, nonenhancing mass in the lateral ventricle atrium, a choroid plexus xanthogranuloma. This degenerative cyst of the choroid plexus is often found incidentally in older patients.](0f9bad8c-233c-480f-adc5-7eb1ab83f856) +**Choroid Plexus Cyst** +*Axial T1WI C+ MR shows a lobulated, nonenhancing mass in the lateral ventricle atrium, a choroid plexus xanthogranuloma. This degenerative cyst of the choroid plexus is often found incidentally in older patients.* + +![Axial T1WI C+ MR shows ventriculitis with asymmetric lateral ventricles related to a temporal lobe abscess rupture and meningitis . Note ventriculomegaly and ventricular wall enhancement characteristic of ventriculitis.](792dc081-566e-4513-987f-ec30914d3bcf) +**Ventriculitis** +*Axial T1WI C+ MR shows ventriculitis with asymmetric lateral ventricles related to a temporal lobe abscess rupture and meningitis . Note ventriculomegaly and ventricular wall enhancement characteristic of ventriculitis.* + +![Axial CECT shows marked ventriculomegaly and ependymal enhancement . A dependent debris level is noted in both lateral ventricles. Ventriculitis has resulted from abscess rupture.](288f6dcf-fe0a-4dbf-809a-c0654c4a32c8) +**Ventriculitis** +*Axial CECT shows marked ventriculomegaly and ependymal enhancement . A dependent debris level is noted in both lateral ventricles. Ventriculitis has resulted from abscess rupture.* + +![Axial NECT shows an infant with hydrocephalus after placement of a right frontal ventricular drain . The shunt did not cross midline. The left lateral ventricle remained enlarged, and the right became slit-like.](5fc74921-60fe-4ec5-b99e-8667a5b6d288) +**CSF Shunts and Complications** +*Axial NECT shows an infant with hydrocephalus after placement of a right frontal ventricular drain . The shunt did not cross midline. The left lateral ventricle remained enlarged, and the right became slit-like.* + +![Axial T2WI MR shows marked enlargement of the isolated right lateral ventricle with transependymal flow of CSF indicating acute obstruction. Note left shunt and completely decompressed left lateral ventricle.](ce67f32e-2900-499f-8122-83108e8cb3f9) +**CSF Shunts and Complications** +*Axial T2WI MR shows marked enlargement of the isolated right lateral ventricle with transependymal flow of CSF indicating acute obstruction. Note left shunt and completely decompressed left lateral ventricle.* + +![Axial NECT shows asymmetric left ventricular dilation post shunting . Shunt tip may be occluded from clot or from imperforate septum, preventing drainage of the left ventricular system.](d7b146cb-1f10-4d4a-a067-85779240fbfb) +**CSF Shunts and Complications** +*Axial NECT shows asymmetric left ventricular dilation post shunting . Shunt tip may be occluded from clot or from imperforate septum, preventing drainage of the left ventricular system.* + +![Axial T2WI FS MR shows a hypointense choroid plexus mass in the atrium of the left lateral ventricle that enhanced intensely (not shown). Note striking surrounding vasogenic edema in adjacent brain parenchyma, thought due to locally obstructed CSF.](d39a3243-21fc-4d93-9a3e-85e6a329c81d) +**Meningioma** +*Axial T2WI FS MR shows a hypointense choroid plexus mass in the atrium of the left lateral ventricle that enhanced intensely (not shown). Note striking surrounding vasogenic edema in adjacent brain parenchyma, thought due to locally obstructed CSF.* + +![Axial T1WI C+ MR demonstrates a mildly enhancing and heterogeneous mass arising from the septum pellucidum . Note asymmetric dilatation of the right lateral ventricle .](1c64172a-f7fe-40df-8387-08f484c5cbad) +**Neurocytoma, Central** +*Axial T1WI C+ MR demonstrates a mildly enhancing and heterogeneous mass arising from the septum pellucidum . Note asymmetric dilatation of the right lateral ventricle .* + +![Axial T1WI C+ MR shows enhancement within the mass in the atrium of the lateral ventricle with an encysted asymmetrically larger left lateral ventricle.](4df1735f-b3a7-4ab4-9e73-ce5d331abb62) +**Choroid Plexus Papilloma** +*Axial T1WI C+ MR shows enhancement within the mass in the atrium of the lateral ventricle with an encysted asymmetrically larger left lateral ventricle.* + +![Axial T1WI C+ MR shows asymmetric lateral ventricles caused by a giant neurocysticercosis cyst in the body of the left lateral ventricle.](fcfcfeb1-c938-47be-ba9e-a639c55bbdd3) +**Neurocysticercosis** +*Axial T1WI C+ MR shows asymmetric lateral ventricles caused by a giant neurocysticercosis cyst in the body of the left lateral ventricle.* + +![Axial T1WI C+ FS MR shows a large mass in the atrium of the right lateral ventricle with a trapped, encysted occipital horn . Ependymal enhancement represents tumor spread from choroidal metastasis.](2bb8e963-0d65-4974-ae8d-429abcee49df) +**Intraventricular Synechiae/Adhesions** +*Axial T1WI C+ FS MR shows a large mass in the atrium of the right lateral ventricle with a trapped, encysted occipital horn . Ependymal enhancement represents tumor spread from choroidal metastasis.* + +![Axial T1WI C+ MR shows an asymmetrically larger right atrium in this patient with Sturge-Weber syndrome. Note associated ipsilateral enlarged frontal sinus and calvarial thickening .](29370a1b-3c42-4629-8032-765ef4ce7c56) +**Dyke-Davidoff-Masson** +*Axial T1WI C+ MR shows an asymmetrically larger right atrium in this patient with Sturge-Weber syndrome. Note associated ipsilateral enlarged frontal sinus and calvarial thickening .* + +![Axial CECT shows enlargement of the right hemisphere and lateral ventricle compared to the left side. Expansion of the hemisphere is mostly due to increased white matter. An enlarged, often deformed, lateral ventricle on the abnormal side is typical.](9a0ef691-0fe7-474a-86d7-121a23736b79) +**Hemimegalencephaly** +*Axial CECT shows enlargement of the right hemisphere and lateral ventricle compared to the left side. Expansion of the hemisphere is mostly due to increased white matter. An enlarged, often deformed, lateral ventricle on the abnormal side is typical.* + +![Axial FLAIR MR shows a cyst enlarging the left lateral ventricle with signal intensity isointense to CSF . There was no enhancement of the cyst wall, typical of ependymal cyst.](5dc6448b-9e90-480c-92c4-b59914acd3c2) +**Ependymal Cyst** +*Axial FLAIR MR shows a cyst enlarging the left lateral ventricle with signal intensity isointense to CSF . There was no enhancement of the cyst wall, typical of ependymal cyst.* + +![Axial FLAIR MR shows a cystic lesion arising from the ependymal lining of the left temporal horn , consistent with an ependymal cyst. The lesion followed CSF signal on every sequence.](6b71914c-51db-4512-973b-d4b49427919c) +**Ependymal Cyst** +*Axial FLAIR MR shows a cystic lesion arising from the ependymal lining of the left temporal horn , consistent with an ependymal cyst. The lesion followed CSF signal on every sequence.* + +![Axial T1WI MR demonstrates a right parietal shunt catheter with its tip in the right frontal horn in a patient with congenital aqueductal stenosis. The right lateral ventricle is collapsed, while the 3rd and left lateral ventricles are moderately dilated.](a93b23ad-da88-4c04-9b31-d3a582cad7c2) +**CSF Shunts and Complications** +*Axial T1WI MR demonstrates a right parietal shunt catheter with its tip in the right frontal horn in a patient with congenital aqueductal stenosis. The right lateral ventricle is collapsed, while the 3rd and left lateral ventricles are moderately dilated.* + +![Axial T1WI C+ MR shows ependymal enhancement and mild asymmetric dilatation of the left occipital horn in ventriculitis. There is also asymmetric enhancement of the adjacent choroid plexus consistent with choroid plexitis .](00e493e3-51d9-420f-be10-b731a98a2fc1) +**Ventriculitis** +*Axial T1WI C+ MR shows ependymal enhancement and mild asymmetric dilatation of the left occipital horn in ventriculitis. There is also asymmetric enhancement of the adjacent choroid plexus consistent with choroid plexitis .* + +![Axial T2WI MR shows a large, hyperintense choroid plexus cyst in a newborn. The lesion increased in size on sequential imaging, requiring endoscopic fenestration.](273e2721-a959-4b65-82dd-32f8f9fa8623) +**Choroid Plexus Cyst** +*Axial T2WI MR shows a large, hyperintense choroid plexus cyst in a newborn. The lesion increased in size on sequential imaging, requiring endoscopic fenestration.* + +![Axial FLAIR MR shows asymmetric dilatation of the left frontal horn and surrounding gliosis following the resection of an intraaxial metastasis. Part of the surgical tract is seen . Note multiple additional metastatic lesions in the right cerebral hemisphere.](53f0bb77-3081-483a-ba27-25e293db386b) +**Surgical Defects** +*Axial FLAIR MR shows asymmetric dilatation of the left frontal horn and surrounding gliosis following the resection of an intraaxial metastasis. Part of the surgical tract is seen . Note multiple additional metastatic lesions in the right cerebral hemisphere.* + +![Axial NECT demonstrates a large left frontal intraparenchymal hematoma causing subfalcine herniation to the right . The left lateral ventricle is effaced. The posterior right lateral ventricle is mildly dilated .](2ed18dfe-fece-449e-806e-9274ebd2cdd6) +**Herniation Syndromes, Intracranial** +*Axial NECT demonstrates a large left frontal intraparenchymal hematoma causing subfalcine herniation to the right . The left lateral ventricle is effaced. The posterior right lateral ventricle is mildly dilated .* + +![Axial NECT shows a large amount of intraventricular hemorrhage resulting in moderate asymmetric expansion of the left lateral ventricle. A smaller amount of layering blood products is seen in the right lateral ventricle .](3e87fdc0-f72c-4519-8fa8-407d3ca749a8) +**Intraventricular Hemorrhage** +*Axial NECT shows a large amount of intraventricular hemorrhage resulting in moderate asymmetric expansion of the left lateral ventricle. A smaller amount of layering blood products is seen in the right lateral ventricle .* + +![Axial T2WI MR shows mild, asymmetric, right hemispheric volume loss with prominent sulci and mild, asymmetric, right lateral ventricle dilatation in a pediatric patient with Rasmussen encephalitis.](de0627e1-81c9-498e-9a0e-9f525bf8351e) +**Rasmussen Encephalitis** +*Axial T2WI MR shows mild, asymmetric, right hemispheric volume loss with prominent sulci and mild, asymmetric, right lateral ventricle dilatation in a pediatric patient with Rasmussen encephalitis.* + +![Axial FLAIR MR shows a round mass in the right occipital lobe (primary CNS lymphoma) that effaces the adjacent right occipital horn and atrium . Extrinsic mass effect is a common cause of ventricular asymmetry and compression.](images/app.statdx.com_image_thumbnail_e0671196-28db-4361-9b63-cdc32902585b_annotated_true_size_900_quality_90_9b78cd11_20251018T165124Z.jpg) +**Extrinsic Mass Effect** +*Axial FLAIR MR shows a round mass in the right occipital lobe (primary CNS lymphoma) that effaces the adjacent right occipital horn and atrium . Extrinsic mass effect is a common cause of ventricular asymmetry and compression.* + +![Axial T1WI C+ MR demonstrates a heterogeneous enhancing mass arising from the septum pellucidum, a pathologically proven central neurocytoma. Note moderate asymmetric dilatation of the right lateral ventricle .](ec4b2b8a-cd64-45e6-9430-1727c705ee35) +**Neurocytoma, Central** +*Axial T1WI C+ MR demonstrates a heterogeneous enhancing mass arising from the septum pellucidum, a pathologically proven central neurocytoma. Note moderate asymmetric dilatation of the right lateral ventricle .* + +![Axial FLAIR MR shows a left thalamic expansile mass , a glioblastoma, which demonstrated heterogeneous enhancement (not shown). It protrudes into and obstructs the left ventricular atrium, which is asymmetrically dilated .](cfe69151-f87a-4abc-aa1a-01fa9586fa5f) +**Obstructive Hydrocephalus** +*Axial FLAIR MR shows a left thalamic expansile mass , a glioblastoma, which demonstrated heterogeneous enhancement (not shown). It protrudes into and obstructs the left ventricular atrium, which is asymmetrically dilated .* + +![Axial T2WI MR demonstrates generalized left hemispheric encephalomalacia following necrotizing encephalitis of unknown origin. There is resultant mild ex-vacuo dilatation of the left lateral ventricle .](images/app.statdx.com_image_thumbnail_1c54ab83-8081-45a8-960e-a7f50c31cce6_annotated_true_size_900_quality_90_6554d649_20251018T165130Z.jpg) +**Encephalomalacia, General** +*Axial T2WI MR demonstrates generalized left hemispheric encephalomalacia following necrotizing encephalitis of unknown origin. There is resultant mild ex-vacuo dilatation of the left lateral ventricle .* + +![Axial T1WI C+ MR shows an avidly enhancing, lobulated mass arising from the right ventricular atrium , a meningioma. Note the mild asymmetric dilatation of the left lateral ventricle .](b426de7c-024c-4f25-ab33-1e7a567fe147) +**Meningioma** +*Axial T1WI C+ MR shows an avidly enhancing, lobulated mass arising from the right ventricular atrium , a meningioma. Note the mild asymmetric dilatation of the left lateral ventricle .* + +![Axial T2WI MR shows lateral ventricles with the right being larger than the left, representing a normal variant. Note mild bowing of the septum pellucidum across the midline .](images/app.statdx.com_image_thumbnail_7a290571-170f-4a98-8381-da762b17ab09_annotated_true_size_900_quality_90_97b6ac24_20251018T165124Z.jpg) +**Normal Variant** +*Axial T2WI MR shows lateral ventricles with the right being larger than the left, representing a normal variant. Note mild bowing of the septum pellucidum across the midline .* + diff --git a/out/autoimmune-encephalitis_6eb3d5d6-7f6a-4367-a792-b5d4b19675da.md b/out/autoimmune-encephalitis_6eb3d5d6-7f6a-4367-a792-b5d4b19675da.md deleted file mode 100644 index 7c4dbc8..0000000 --- a/out/autoimmune-encephalitis_6eb3d5d6-7f6a-4367-a792-b5d4b19675da.md +++ /dev/null @@ -1,516 +0,0 @@ ---- -title: "Autoimmune Encephalitis" -docid: "6eb3d5d6-7f6a-4367-a792-b5d4b19675da" -authors: - - key: "8d5254e9-8dda-478b-8f08-bdee97a32c79" - value: "Karen L. Salzman, MD, FACR" -breadcrumbs: - - - name: "Brain" - slug: "brain" - treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" - - - name: "Diagnosis" - slug: "diagnosis" - treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" - - - name: "Pathology-Based Diagnoses" - slug: "pathology-based-diagnoses" - treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" - - - name: "Infectious, Inflammatory, and Demyelinating Disease" - slug: "infectious-inflammatory-and-demyel-" - treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607" - - - name: "Inflammatory and Demyelinating Disease" - slug: "inflammatory-and-demyelinating-dis-" - treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc" - - - name: "Autoimmune Encephalitis" - slug: "autoimmune-encephalitis" - treeNodeId: null -category: "Brain" -documentVersionId: "cc8c3891-88b7-44bb-9e2a-3a321d092f4c" -imageCount: 22 -lastUpdated: "08/05/20" -pageDescription: "Autoimmune Encephalitis" -pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Autoimmune Encephalitis" -pageTitle: "Autoimmune Encephalitis | STATdx" -enhancedTitle: "Autoimmune Encephalitis" -type: "DX" -references: true -breadcrumbs: - - "Brain" - - "Diagnosis" - - "Pathology-Based Diagnoses" - - "Infectious, Inflammatory, and Demyelinating Disease" - - "Inflammatory and Demyelinating Disease" - - "Autoimmune Encephalitis" ---- -# KEY FACTS - -- ## Terminology - - - - Autoimmune encephalitis (AE): Immune-mediated disease with antibody-mediated inflammation of brain - - May be immune mediated or paraneoplastic - - Paraneoplastic: Remote neurologic effects of cancer, associated with extra-CNS tumors - - Most common tumor: Small cell lung carcinoma - - Limbic encephalitis (LE) is most common clinical paraneoplastic syndrome -- ## Imaging - - - - AE: T2/FLAIR hyperintensities in cortex/subcortical white matter, basal ganglia (BG), thalami, brainstem - - AE: May have no associated imaging findings - - LE: Hyperintensity in mesial temporal lobes/limbic system - - Mimics herpes encephalitis but subacute/chronic - - Paraneoplastic cerebellar degeneration (PCD): Cerebellar atrophy - - Brainstem encephalitis: T2 hyperintensity in midbrain, pons, cerebellar peduncles, BG -- ## Top Differential Diagnoses - - - - AE: Viral encephalitis, ADEM, vasculitis - - LE: Herpes encephalitis, diffuse astrocytoma, status epilepticus, gliomatosis cerebri imaging pattern -- ## Clinical Issues - - - - AE: 2 distinct groups (group I and group II) have overlapping clinical and imaging features - - Group I (intracellular) or group II (cell surface) antigens - - < 1% of patients with systemic cancers develop paraneoplastic syndrome - - LE: Memory loss, cognitive dysfunction, dementia, psychological features, seizures - - PCD: Ataxia, incoordination, dysarthria, nystagmus - - Brainstem encephalitis: Brainstem dysfunction - - Treatment of primary tumor may improve symptoms - - AE treated with immunosuppressive therapy - - AE may occur as complication of cancer treatment (immune checkpoint inhibitors) - -# TERMINOLOGY - -- ## Synonyms - - - - Paraneoplastic syndromes (PS), paraneoplastic disease -- ## Definitions - - - - Autoimmune encephalitis (AE): Immune-mediated disease with antibody-mediated inflammation of brain - - Includes autoimmune and paraneoplastic encephalitis - - Paraneoplastic: Remote neurologic effects of cancer, associated with extra-CNS tumors - - Most common tumor: Small cell lung carcinoma - - Limbic encephalitis (LE) is most common clinical paraneoplastic syndrome - - Only PS with clearly defined imaging features - -# IMAGING - -- ## General Features - - - - ### Best diagnostic clue - - - - AE: Hyperintensity in mesial temporal lobes, limbic system - - Often mimics herpes encephalitis but has different clinical course (subacute vs. chronic) - - Initial study normal in 20-40% - - Most PS do not have associated imaging findings - - ### Location - - - - AE: Limbic structures, deep gray nuclei, brainstem, cortex and subcortical white matter (WM) - - LE: Hippocampus, amygdala, cingulate gyrus, pyriform cortex, subfrontal cortex, insula -- ## CT Findings - - - - NECT: Initial CT scan normal in > 95% - - Rare: Low density within mesial temporal lobes - - CECT: Usually no visible enhancement -- ## MR Findings - - - - ### T1WI - - - - AE: Hypointensity in cortex, WM, deep gray, or brainstem - - LE: Hypointensity in mesial temporal lobes (hippocampus, amygdala), insula, cingulate gyrus, subfrontal cortex, inferior frontal WM - - May see minimal mass effect - - May see atrophy in chronic cases - - No hemorrhage - - ### DWI - - - - Diffusion restriction rare - - T2WI: Hyperintensity in mesial temporal lobes &/or cortex, WM, deep gray, or brainstem - - May see minimal mass effect - - FLAIR: Hyperintensity - - T2* GRE: No hemorrhage - - If blood products seen, consider herpes encephalitis - - T1WI C+: Patchy enhancement may be seen - - Brainstem encephalitis: T2 hyperintensity in midbrain, pons, cerebellar peduncles, basal ganglia - - Paraneoplastic cerebellar degeneration (PCD): Cerebellar atrophy -- ## Nuclear Medicine Findings - - - - FDG PET: Increased glucose metabolism in medial temporal lobes in LE patients -- ## Imaging Recommendations - - - - ### Best imaging tool - - - - MR is most sensitive - - ### Protocol advice - - - - Contrast-enhanced MR with coronal T2 or FLAIR - - Consider repeat MR if initial scan normal with high clinical suspicion - -# DIFFERENTIAL DIAGNOSIS - -- [Herpes Encephalitis](/document/herpes-encephalitis/6bbe5645-2178-411e-871e-8878d244f482) - - T2 hyperintensity in temporal lobes, limbic system - - Mass effect common; restricted DWI common - - Rapid onset, febrile illness - - HSV titers (CSF, serum) may be negative early - - Late acute/subacute may hemorrhage - - May be indistinguishable from LE -- ## Diffuse Astrocytoma (WHO Grade 2) - - - - Unilateral T2-hyperintense mass - - May involve medial temporal lobe - - No enhancement typical -- [Status Epilepticus](/document/status-epilepticus/a058b733-4b80-46a1-8097-d68685ecf921) - - Seizures related to abnormal T2/FLAIR of mesial temporal lobes - - Cortical enhancement with DWI is typical - - Clinical history of seizures - - Follow-up imaging may be necessary -- ## Gliomatosis Cerebri Imaging Pattern - - - - Diffuse process; no predilection for limbic system - - T2 hyperintensity in multiple contiguous lobes - - Enlarges affected area -- ## Vasculitis - - - - Multiple T2/FLAIR hyperintensities - - Blood products common - - Small infarcts common -- ## Acute Disseminated Encephalomyelitis - - - - T2/FLAIR hyperintensities in WM and deep gray nuclei - - Enhancement typical - - Often 1-2 weeks following infection/vaccination -- ## Parenchymal Metastases - - - - Typically multifocal, enhancing lesions - - Primary tumor often known - - No predilection for limbic system - -# PATHOLOGY - -- ## General Features - - - - ### Etiology - - - - AE may be characterized as either **group I**or **group II** according to location of their neuronal antigens - - **Group I**antibodies targeting **intracellular antigens** - - **Group II**antibodies targeting **antigens on cell surface**:1, 2, 6, 7, 9, 27 - - Distinction clinically relevant: Implications for treatment response, association with underlying malignancy, and prognosis - - **Group I**antibodies are more closely associated with underlying malignancy - - Immune mediated by **autoantibodies** or cytotoxic T cell-related mechanisms - - 60% of patients have circulating serum **autoantibodies** - - **Anti-Hu** (75% small cell lung cancer): LE pattern - - **Anti-Ma/Ta** (testicular germ cell tumors > > lung cancer > breast cancer): LE, brainstem encephalitis - - **Anti-Yo** (breast and ovarian): PCD pattern - - **Anti-Tr** (Hodgkin disease): PCD pattern - - **Anti-Ri**(lung, breast, ovarian): Opsoclonus myoclonus, brainstem encephalitis - - **Anti-CV2**(small-cell lung cancer, malignant thymoma): T2/FLAIR-hyperintense lesions in striatum - - **Anti-glutamic acid decarboxylase (GAD65)**(not typically associated with malignancy): LE pattern - - Stiff man syndrome, cerebellar ataxia, seizures - - Reversible extralimbic paraneoplastic encephalopathy - - Associated with breast cancer and lung cancer - - Reversible when primary tumors controlled - - **Group II**cell surface antigens - - **Voltage-gated potassium channels (VGKC)** and N-methyl-D-aspartate receptor (**NMDAR**) - - Appear to be antibody mediated and respond better to immunotherapy (90%) - - Associated with other tumors (thymoma, teratoma, Hodgkin lymphoma) - - Patients may present with LE; more frequently manifest severe psychiatric symptoms, seizures, dyskinesias, autonomic instability, or hypoventilation - - Antibody to α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (**AMPAr**) - - Associated with LE pattern, T2 hyperintensities in hippocampus - - May occur with other antibodies - - Often associated with lung, breast, or thymic tumors - - γ aminobutyric acid encephalitis (**GABAr**) - - LE pattern ± other MR findings, seizures -- ## Staging, Grading, & Classification - - - - 2 distinct groups (group I and group II) have overlapping clinical and imaging features - - PSs divided into disorders of CNS, peripheral NS (PNS), CNS/PNS, neuromuscular junction - - CNS: PCD, opsoclonus myoclonus, retinopathy - - PNS: Sensory-motor neuropathy, autonomic neuropathy - - Both CNS/PNS: Encephalomyelitis (LE, brainstem encephalitis, myelitis, motor neuron disease) - - Neuromuscular junction: Lambert-Eaton myasthenic syndrome - - LE is most common AE (paraneoplastic, group I) - - Nonparaneoplastic LE reported - - PCD is 2nd most common PS - - Multiple PSs may occur in same patient - - NMDAR and VGKC encephalitis are most common group II subtypes of AE -- ## Gross Pathologic & Surgical Features - - - - AE: Ill-defined softening, discoloration of gray matter - - LE: Hippocampus, cingulate gyrus, pyriform cortex, frontal orbital surface of temporal lobe, insula, amygdala; typically bilateral - - PCD: Cerebellar atrophy, gyral thinning - - Brainstem encephalitis: Brainstem softening -- ## Microscopic Features - - - - AE: Neuronal loss, reactive gliosis, perivascular infiltration of lymphocytes, microglial nodules - - No neoplasm and no viral inclusions - - PCD: Purkinje cell loss, microglial proliferation, Bergmann glia hyperplasia, decrease in granule cells - - Brainstem encephalitis: Perivascular inflammatory infiltrates, glial nodules, neuronophagia - -# CLINICAL ISSUES - -- ## Presentation - - - - ### Most common signs/symptoms - - - - AE: Memory loss, cognitive dysfunction, dementia, psychological features (anxiety, depression, hallucinations), seizures; subacute presentation - - PCD: Ataxia, incoordination, dysarthria, nystagmus - - In patients > 50 years, cerebellar degeneration is paraneoplastic in 50% of cases, often precedes remote malignancy - - Brainstem encephalitis: Brainstem dysfunction including cranial nerve palsies, visual changes - - PSs represent spectrum of neurologic manifestations - - In patients with known primary tumor, must exclude other complications - - Metastases, infection, metabolic disorder, chemotherapy effects - - ### Clinical profile - - - - Up to 60% of LE patients have no known primary tumor at presentation, many have no tumor found at work-up - - Identification of antineuronal antibodies in serum or cerebrospinal fluid (CSF) facilitates diagnosis of PS and primary cancer - - AE may occur as complication of cancer treatment (immune checkpoint inhibitors) - - Primary neoplasms - - LE - - Most common: Small cell lung carcinoma - - Other: Gastrointestinal, genitourinary (ovary > renal > uterus), lymphoma, breast, testicular, thymus, neuroblastoma (pediatric) - - 90% have positive CSF (pleocytosis, increased protein, oligoclonal bands) - - EEG reveals involvement of temporal lobes - - PCD - - Genitourinary (ovary), breast, lung, lymphoma - - Opsoclonus myoclonus - - Neuroblastoma, lung cancer - - Lambert-Eaton myasthenic syndrome - - Small cell lung cancer -- ## Demographics - - - - ### Sex - - - - No sex predominance - - Age: Occurs at all ages, most commonly adults - - Epidemiology: < 1% of patients with systemic cancers develop paraneoplastic syndrome -- ## Natural History & Prognosis - - - - AE has variable prognosis - - Prognosis of PS may relate to primary neoplasm - - Relates to type of paraneoplastic syndrome - - Slow, long-term cognitive decline (LE) - - Progressive ataxia, weakness (PCD, spinal cord degeneration) - - Some reports suggest patients with PSs have more indolent primary tumor growth than those without -- ## Treatment - - - - AE often treated with immunotherapy (IV steroids, plasma exchange, IVIg) ± rituximab or cyclophosphamide - - Treatment of primary malignancy may improve neurologic symptoms of PS - - Primary neoplasm resected, ± chemoradiation - - Treatment of PS is variable - - Treatment of primary tumor is best therapy - - ± steroids, immunoglobulins, plasmapheresis - -# DIAGNOSTIC CHECKLIST - -- ## Consider - - - - LE is only PS with defined imaging features - - PS are often clinically evident before diagnosis of primary tumor - - Repeat MR if initial scan normal and high clinical suspicion, as initial MR often normal in LE -- ## Image Interpretation Pearls - - - - Herpes encephalitis mimics LE on imaging but has acute presentation - - Patients often initially treated with antiviral therapy until HSV titers final - - Hemorrhage suggests herpes rather than LE - - 89d11c00-03ef-4d5a-aea9-2d9c2669fa58 - -## References - -# Selected References - -1. [Iorio R et al: Clinical characteristics and outcome of patients with autoimmune encephalitis: clues for paraneoplastic etiology. Eur J Neurol. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32412135%5Bpmid%5D) -1. [Mongay-Ochoa N et al: Anti-Hu-associated paraneoplastic syndromes triggered by immune-checkpoint inhibitor treatment. J Neurol. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32451614%5Bpmid%5D) -1. [Vogrig A et al: Central nervous system complications associated with immune checkpoint inhibitors. J Neurol Neurosurg Psychiatry. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32312871%5Bpmid%5D) -1. [Bradshaw MJ et al: An overview of autoimmune and paraneoplastic encephalitides. Semin Neurol. 38(3):330-43, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30011413%5Bpmid%5D) -1. [Long JM et al: Autoimmune dementia. Semin Neurol. 38(3):303-15, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30011411%5Bpmid%5D) -1. 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[Modoni A et al: Successful treatment of acute autoimmune limbic encephalitis with negative VGKC and NMDAR antibodies. Cogn Behav Neurol. 22(1):63-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19372772%5Bpmid%5D) -1. [Saiz A et al: Anti-Hu-associated brainstem encephalitis. J Neurol Neurosurg Psychiatry. 80(4):404-7, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19015226%5Bpmid%5D) -1. [Uribe-Uribe NO et al: Paraneoplastic sensory neuropathy associated with small cell carcinoma of the gallbladder. Ann Diagn Pathol. 13(2):124-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19302962%5Bpmid%5D) -1. [Anderson NE et al: Limbic encephalitis - a review. J Clin Neurosci. 15(9):961-71, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18411052%5Bpmid%5D) -1. [Basu S et al: Role of FDG-PET in the clinical management of paraneoplastic neurological syndrome: detection of the underlying malignancy and the brain PET-MRI correlates. Mol Imaging Biol. 10(3):131-7, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18297363%5Bpmid%5D) -1. [Blaes F et al: Autoantibodies in childhood opsoclonus-myoclonus syndrome. J Neuroimmunol. 201-202:221-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18687475%5Bpmid%5D) -1. [Corapcioglu F et al: Response to rituximab and prednisolone for opsoclonus-myoclonus-ataxia syndrome in a child with ganglioneuroblastoma. Pediatr Hematol Oncol. 25(8):756-61, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19065442%5Bpmid%5D) -1. [Dalakas MC: Invited article: inhibition of B cell functions: implications for neurology. Neurology. 70(23):2252-60, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18519875%5Bpmid%5D) -1. [Eker A et al: Testicular teratoma and anti-N-methyl-D-aspartate receptor-associated encephalitis. J Neurol Neurosurg Psychiatry. 79(9):1082-3, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18708569%5Bpmid%5D) -1. [Fitzpatrick AS et al: Opsoclonus-myoclonus syndrome associated with benign ovarian teratoma. Neurology. 70(15):1292-3, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18391162%5Bpmid%5D) -1. [Geschwind MD et al: Voltage-gated potassium channel autoimmunity mimicking creutzfeldt-jakob disease. Arch Neurol. 65(10):1341-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18852349%5Bpmid%5D) -1. [Hassan KA et al: Long-term survival in paraneoplastic opsoclonus-myoclonus syndrome associated with small cell lung cancer. J Neuroophthalmol. 28(1):27-30, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18347455%5Bpmid%5D) -1. [Jarius S et al: Relative frequency of VGKC and 'classical' paraneoplastic antibodies in patients with limbic encephalitis. J Neurol. 255(7):1100-1, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18574619%5Bpmid%5D) -1. [Johnson V et al: Immune mediated neurologic dysfunction as a paraneoplastic syndrome in renal cell carcinoma. J Neurooncol. 90(3):279-81, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18726185%5Bpmid%5D) -1. [Ko MW et al: Neuro-ophthalmologic manifestations of paraneoplastic syndromes. J Neuroophthalmol. 28(1):58-68, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18347462%5Bpmid%5D) -1. [Musunuru K et al: Paraneoplastic opsoclonus-myoclonus ataxia associated with non-small-cell lung carcinoma. J Neurooncol. 90(2):213-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18618225%5Bpmid%5D) -1. [Novillo-López ME et al: Treatment-responsive subacute limbic encephalitis and NMDA receptor antibodies in a man. Neurology. 70(9):728-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18299525%5Bpmid%5D) -1. [Pellkofer HL et al: Lambert-eaton myasthenic syndrome differential reactivity of tumor versus non-tumor patients to subunits of the voltage-gated calcium channel. J Neuroimmunol. 204(1-2):136-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18809213%5Bpmid%5D) -1. [Sabater L et al: SOX1 antibodies are markers of paraneoplastic Lambert-Eaton myasthenic syndrome. Neurology. 70(12):924-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18032743%5Bpmid%5D) -1. [Sekiguchi Y et al: Potassium channel antibody-associated encephalitis with hypothalamic lesions and intestinal pseudo-obstruction. J Neurol Sci. 269(1-2):176-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18378260%5Bpmid%5D) -1. [Tan KM et al: Clinical spectrum of voltage-gated potassium channel autoimmunity. Neurology. 70(20):1883-90, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18474843%5Bpmid%5D) -1. [Darnell RB et al: Paraneoplastic syndromes affecting the nervous system. Semin Oncol. 33(3):270-98, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16769417%5Bpmid%5D) -1. [Dropcho EJ: Update on paraneoplastic syndromes. Curr Opin Neurol. 18(3):331-6, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15891421%5Bpmid%5D) -1. [Dadparvar S et al: Paraneoplastic encephalitis associated with cystic teratoma is detected by fluorodeoxyglucose positron emission tomography with negative magnetic resonance image findings. Clin Nucl Med. 28(11):893-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14578703%5Bpmid%5D) -1. [Messori A et al: Resolution of limbic encephalitis with detection and treatment of lung cancer: clinical-radiological correlation. Eur J Radiol. 45(1): 78-80, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12499067%5Bpmid%5D) -1. [Barnett M et al: Paraneoplastic brain stem encephalitis in a woman with anti-Ma2 antibody. J Neurol Neurosurg Psychiatry. 70(2):222-5, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11160472%5Bpmid%5D) -1. [Gultekin SH et al: Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 123 ( Pt 7):1481-94, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10869059%5Bpmid%5D) -1. [Dalmau J et al: Paraneoplastic neurologic syndromes: pathogenesis and physiopathology. Brain Pathol. 9(2):275-84, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10219745%5Bpmid%5D) -1. [Scaravilli F et al: The neuropathology of paraneoplastic syndromes. Brain Pathol. 9(2):251-60, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10219743%5Bpmid%5D) -1. [Voltz R et al: A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer. N Engl J Med. 340(23): 1788-95, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10362822%5Bpmid%5D) - - -## Images - - -### Selected Images - -![Axial FLAIR MR shows abnormal hyperintensity in the bilateral medial temporal lobes , characteristic of limbic encephalitis (LE), the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.](images/app.statdx.com_image_thumbnail_55b572ea-97f1-4aef-96e8-2d4953c437bb_annotated_true_size_900_quality_90_e8e0e41a_20251018T122445Z.jpg) -*Axial FLAIR MR shows abnormal hyperintensity in the bilateral medial temporal lobes , characteristic of limbic encephalitis (LE), the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.* - -![Axial FLAIR MR shows abnormal hyperintensity in the bilateral medial temporal lobes , characteristic of limbic encephalitis (LE), the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.](images/app.statdx.com_image_thumbnail_55b572ea-97f1-4aef-96e8-2d4953c437bb_size_168_quality_85_8c5e5586_20251018T095220Z.jpg) -*Axial FLAIR MR shows abnormal hyperintensity in the bilateral medial temporal lobes , characteristic of limbic encephalitis (LE), the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.* - -![Axial FLAIR MR shows abnormal hyperintensity in the bilateral medial temporal lobes , characteristic of limbic encephalitis (LE), the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.](images/app.statdx.com_image_thumbnail_55b572ea-97f1-4aef-96e8-2d4953c437bb_size_174_quality_85_614503aa_20251018T122441Z.jpg) -*Axial FLAIR MR shows abnormal hyperintensity in the bilateral medial temporal lobes , characteristic of limbic encephalitis (LE), the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.* - -![Axial T1 C+ MR in the same patient shows no significant enhancement in the medial temporal lobes. Enhancement is often present in limbic encephalitis. The patient's symptoms often improve after treatment of the primary tumor.](images/app.statdx.com_image_thumbnail_f19048d1-415f-4f2a-8236-6c1c9c263ade_annotated_true_size_900_quality_90_520f6cc2_20251018T122445Z.jpg) -*Axial T1 C+ MR in the same patient shows no significant enhancement in the medial temporal lobes. Enhancement is often present in limbic encephalitis. The patient's symptoms often improve after treatment of the primary tumor.* - -![Axial T1 C+ MR in the same patient shows no significant enhancement in the medial temporal lobes. Enhancement is often present in limbic encephalitis. The patient's symptoms often improve after treatment of the primary tumor.](images/app.statdx.com_image_thumbnail_f19048d1-415f-4f2a-8236-6c1c9c263ade_size_168_quality_85_afbb7b9e_20251018T095220Z.jpg) -*Axial T1 C+ MR in the same patient shows no significant enhancement in the medial temporal lobes. Enhancement is often present in limbic encephalitis. The patient's symptoms often improve after treatment of the primary tumor.* - -![Axial FLAIR MR in a 61 year old with multiple myeloma who presented with seizures shows abnormal hyperintensity in the cortex and subcortical white matter of the temporal lobes.](images/app.statdx.com_image_thumbnail_bb9be3cc-b23c-4941-a7a4-16f0a1736308_annotated_true_size_900_quality_90_52d58654_20251018T122445Z.jpg) -*Axial FLAIR MR in a 61 year old with multiple myeloma who presented with seizures shows abnormal hyperintensity in the cortex and subcortical white matter of the temporal lobes.* - -![Axial FLAIR MR in a 61 year old with multiple myeloma who presented with seizures shows abnormal hyperintensity in the cortex and subcortical white matter of the temporal lobes.](images/app.statdx.com_image_thumbnail_bb9be3cc-b23c-4941-a7a4-16f0a1736308_size_168_quality_85_73cf1488_20251018T095220Z.jpg) -*Axial FLAIR MR in a 61 year old with multiple myeloma who presented with seizures shows abnormal hyperintensity in the cortex and subcortical white matter of the temporal lobes.* - -![Axial T1 C+ FS MR in the same patient shows no enhancement. Differential considerations include autoimmune encephalitis (AE), acute demyelinating encephalomyelitis (ADEM), viral encephalitis, and vasculitis in this case. AE related to GABAr was diagnosed by CSF and serum markers.](images/app.statdx.com_image_thumbnail_b830356c-f148-4519-ba8d-65c67dbb5380_annotated_true_size_900_quality_90_b9c5d4d4_20251018T122445Z.jpg) -*Axial T1 C+ FS MR in the same patient shows no enhancement. Differential considerations include autoimmune encephalitis (AE), acute demyelinating encephalomyelitis (ADEM), viral encephalitis, and vasculitis in this case. AE related to GABAr was diagnosed by CSF and serum markers.* - -![Axial T1 C+ FS MR in the same patient shows no enhancement. Differential considerations include autoimmune encephalitis (AE), acute demyelinating encephalomyelitis (ADEM), viral encephalitis, and vasculitis in this case. AE related to GABAr was diagnosed by CSF and serum markers.](images/app.statdx.com_image_thumbnail_b830356c-f148-4519-ba8d-65c67dbb5380_size_168_quality_85_1c0c9901_20251018T095220Z.jpg) -*Axial T1 C+ FS MR in the same patient shows no enhancement. Differential considerations include autoimmune encephalitis (AE), acute demyelinating encephalomyelitis (ADEM), viral encephalitis, and vasculitis in this case. AE related to GABAr was diagnosed by CSF and serum markers.* - -![Axial FLAIR MR in a 71 year old with altered mental status shows abnormal hyperintensity in the left temporal lobe . Differential considerations include infectious, inflammatory, and neoplastic etiologies. GAD65 AE was diagnosed at brain biopsy.](images/app.statdx.com_image_thumbnail_931ed2de-80d8-4747-a34c-d2a0a05f526e_annotated_true_size_900_quality_90_b03e40a4_20251018T122445Z.jpg) -*Axial FLAIR MR in a 71 year old with altered mental status shows abnormal hyperintensity in the left temporal lobe . Differential considerations include infectious, inflammatory, and neoplastic etiologies. GAD65 AE was diagnosed at brain biopsy.* - -![Axial FLAIR MR in a 71 year old with altered mental status shows abnormal hyperintensity in the left temporal lobe . Differential considerations include infectious, inflammatory, and neoplastic etiologies. GAD65 AE was diagnosed at brain biopsy.](images/app.statdx.com_image_thumbnail_931ed2de-80d8-4747-a34c-d2a0a05f526e_size_168_quality_85_9bd3c62a_20251018T095220Z.jpg) -*Axial FLAIR MR in a 71 year old with altered mental status shows abnormal hyperintensity in the left temporal lobe . Differential considerations include infectious, inflammatory, and neoplastic etiologies. GAD65 AE was diagnosed at brain biopsy.* - -![Axial FLAIR MR shows hyperintensity in the medial temporal lobes in this patient with subacute dementia and voltage-gated potassium channel (VGKC) autoimmunity. VGKC may occur with or without a primary neoplasm and may have an LE pattern.](images/app.statdx.com_image_thumbnail_75dce6be-287e-4d7e-be14-8d0e424db77f_annotated_true_size_900_quality_90_d7c99a83_20251018T122445Z.jpg) -*Axial FLAIR MR shows hyperintensity in the medial temporal lobes in this patient with subacute dementia and voltage-gated potassium channel (VGKC) autoimmunity. VGKC may occur with or without a primary neoplasm and may have an LE pattern.* - -![Axial FLAIR MR shows hyperintensity in the medial temporal lobes in this patient with subacute dementia and voltage-gated potassium channel (VGKC) autoimmunity. VGKC may occur with or without a primary neoplasm and may have an LE pattern.](images/app.statdx.com_image_thumbnail_75dce6be-287e-4d7e-be14-8d0e424db77f_size_168_quality_85_5458e01b_20251018T095220Z.jpg) -*Axial FLAIR MR shows hyperintensity in the medial temporal lobes in this patient with subacute dementia and voltage-gated potassium channel (VGKC) autoimmunity. VGKC may occur with or without a primary neoplasm and may have an LE pattern.* - -![Axial T2 MR shows midbrain hyperintensity related to brainstem encephalitis, which is characterized by hyperintensity in the midbrain, pons, cerebellar peduncle, and basal ganglia.](images/app.statdx.com_image_thumbnail_b8522ce7-c8be-4ed7-a7f9-4788abe7a535_annotated_true_size_900_quality_90_a11bc1a5_20251018T122445Z.jpg) -*Axial T2 MR shows midbrain hyperintensity related to brainstem encephalitis, which is characterized by hyperintensity in the midbrain, pons, cerebellar peduncle, and basal ganglia.* - -![Axial T2 MR shows midbrain hyperintensity related to brainstem encephalitis, which is characterized by hyperintensity in the midbrain, pons, cerebellar peduncle, and basal ganglia.](images/app.statdx.com_image_thumbnail_b8522ce7-c8be-4ed7-a7f9-4788abe7a535_size_168_quality_85_810aceae_20251018T095220Z.jpg) -*Axial T2 MR shows midbrain hyperintensity related to brainstem encephalitis, which is characterized by hyperintensity in the midbrain, pons, cerebellar peduncle, and basal ganglia.* - -![Axial T1 C+ MR in the same patient shows patchy enhancement of the midbrain lesions and the medial temporal lobe . This patient was diagnosed with LE with new brainstem symptoms. Multiple paraneoplastic syndromes may occur in the same patient.](images/app.statdx.com_image_thumbnail_fd5e962c-ebfb-4724-930d-dbda5d025098_annotated_true_size_900_quality_90_fa9ee197_20251018T122445Z.jpg) -*Axial T1 C+ MR in the same patient shows patchy enhancement of the midbrain lesions and the medial temporal lobe . This patient was diagnosed with LE with new brainstem symptoms. Multiple paraneoplastic syndromes may occur in the same patient.* - -![Axial T1 C+ MR in the same patient shows patchy enhancement of the midbrain lesions and the medial temporal lobe . This patient was diagnosed with LE with new brainstem symptoms. Multiple paraneoplastic syndromes may occur in the same patient.](images/app.statdx.com_image_thumbnail_fd5e962c-ebfb-4724-930d-dbda5d025098_size_168_quality_85_91e4482d_20251018T095220Z.jpg) -*Axial T1 C+ MR in the same patient shows patchy enhancement of the midbrain lesions and the medial temporal lobe . This patient was diagnosed with LE with new brainstem symptoms. Multiple paraneoplastic syndromes may occur in the same patient.* - -![Axial FLAIR MR shows abnormal hyperintensity in the medial temporal lobes bilaterally, related to LE. As imaging mimics herpes encephalitis, most patients are initially treated with antiviral therapy until HSV titers are found to be negative. < 1% of cancer patients develop a paraneoplastic syndrome.](images/app.statdx.com_image_thumbnail_dda402f8-e60a-451f-a558-d44f1038c882_annotated_true_size_900_quality_90_28e2bbdb_20251018T122445Z.jpg) -*Axial FLAIR MR shows abnormal hyperintensity in the medial temporal lobes bilaterally, related to LE. As imaging mimics herpes encephalitis, most patients are initially treated with antiviral therapy until HSV titers are found to be negative. < 1% of cancer patients develop a paraneoplastic syndrome.* - -![Axial FLAIR MR shows abnormal hyperintensity in the medial temporal lobes bilaterally, related to LE. As imaging mimics herpes encephalitis, most patients are initially treated with antiviral therapy until HSV titers are found to be negative. < 1% of cancer patients develop a paraneoplastic syndrome.](images/app.statdx.com_image_thumbnail_dda402f8-e60a-451f-a558-d44f1038c882_size_168_quality_85_41c962cf_20251018T095220Z.jpg) -*Axial FLAIR MR shows abnormal hyperintensity in the medial temporal lobes bilaterally, related to LE. As imaging mimics herpes encephalitis, most patients are initially treated with antiviral therapy until HSV titers are found to be negative. < 1% of cancer patients develop a paraneoplastic syndrome.* - -![Axial T1 C+ MR in the same patient shows patchy enhancement of the medial temporal lobes. LE is the only paraneoplastic syndrome with defined imaging features.](images/app.statdx.com_image_thumbnail_adfb6224-fa92-44a6-8fb9-71bb22dd6eb3_size_168_quality_85_611d6d6f_20251018T095220Z.jpg) -*Axial T1 C+ MR in the same patient shows patchy enhancement of the medial temporal lobes. LE is the only paraneoplastic syndrome with defined imaging features.* - - -### Additional Images - -![Axial T2 MR shows abnormal hyperintensity in the right medial temporal lobe , typical of limbic encephalitis. Note the abnormal hyperintensity in the midbrain , indicative of brainstem encephalitis. Brainstem encephalitis is a very uncommon paraneoplastic syndrome. Multiple paraneoplastic syndromes can occur in the same patient.](images/app.statdx.com_image_thumbnail_f2540964-3ae6-4b6b-a7dc-f128f8e94c29_size_168_quality_85_6e806d79_20251018T095220Z.jpg) -*Axial T2 MR shows abnormal hyperintensity in the right medial temporal lobe , typical of limbic encephalitis. Note the abnormal hyperintensity in the midbrain , indicative of brainstem encephalitis. Brainstem encephalitis is a very uncommon paraneoplastic syndrome. Multiple paraneoplastic syndromes can occur in the same patient.* - -![Axial FLAIR MR in a patient with subacute dementia and lung cancer shows hyperintensity within the medial temporal lobes, classic for limbic encephalitis. Imaging mimics herpes encephalitis.](images/app.statdx.com_image_thumbnail_a4b705df-d7f3-498c-8ed1-a3f989922ee3_size_168_quality_85_7905ee41_20251018T095220Z.jpg) -*Axial FLAIR MR in a patient with subacute dementia and lung cancer shows hyperintensity within the medial temporal lobes, classic for limbic encephalitis. Imaging mimics herpes encephalitis.* - -![Axial T1 C+ MR shows subtle patchy enhancement of the medial temporal lobes bilaterally , which is a typical enhancement pattern for limbic encephalitis. Bilateral involvement is common.](images/app.statdx.com_image_thumbnail_72659a12-1bfa-4a0a-87f1-973e53c6b558_size_168_quality_85_aa2d61f2_20251018T095220Z.jpg) -*Axial T1 C+ MR shows subtle patchy enhancement of the medial temporal lobes bilaterally , which is a typical enhancement pattern for limbic encephalitis. Bilateral involvement is common.* - -![Coronal T2 MR in a patient with limbic encephalitis shows abnormal hyperintensity in the medial temporal lobes and right insula in this patient with severe memory loss and dementia. Symptoms improved after the removal of the primary tumor.](images/app.statdx.com_image_thumbnail_63d4e718-6ee6-437c-a23b-bd2a56f8c106_size_168_quality_85_4a3881a2_20251018T095220Z.jpg) -*Coronal T2 MR in a patient with limbic encephalitis shows abnormal hyperintensity in the medial temporal lobes and right insula in this patient with severe memory loss and dementia. Symptoms improved after the removal of the primary tumor.* - -![Coronal T1 C+ MR shows abnormal gyriform enhancement in the medial temporal lobes and left insula . The more typical patchy enhancement pattern of limbic encephalitis is seen in the hippocampi bilaterally.](images/app.statdx.com_image_thumbnail_88093894-f2b4-4a12-aa75-1ae5ec313f44_size_168_quality_85_6a739671_20251018T095220Z.jpg) -*Coronal T1 C+ MR shows abnormal gyriform enhancement in the medial temporal lobes and left insula . The more typical patchy enhancement pattern of limbic encephalitis is seen in the hippocampi bilaterally.* - -![Axial T1 MR shows hyperintensity representing blood products in the medial temporal lobes in this patient with treated lung cancer and limbic encephalitis. Blood products are rare in limbic encephalitis, in which imaging mimics herpes encephalitis.](images/app.statdx.com_image_thumbnail_2871f764-a9a3-42a8-8368-0e308b38f69b_size_168_quality_85_7e80eab5_20251018T095220Z.jpg) -*Axial T1 MR shows hyperintensity representing blood products in the medial temporal lobes in this patient with treated lung cancer and limbic encephalitis. Blood products are rare in limbic encephalitis, in which imaging mimics herpes encephalitis.* - -![Axial FLAIR MR shows abnormal hyperintensity in the right medial temporal lobe and midbrain in this patient with a history of limbic encephalitis and new brainstem symptoms positive for anti-Hu autoantibody.](images/app.statdx.com_image_thumbnail_70b9a9d9-e630-45d3-854d-91ace5229992_size_168_quality_85_28b0318f_20251018T095220Z.jpg) -*Axial FLAIR MR shows abnormal hyperintensity in the right medial temporal lobe and midbrain in this patient with a history of limbic encephalitis and new brainstem symptoms positive for anti-Hu autoantibody.* - -![Axial T1 C+ MR shows enhancement in the medial temporal lobes and left anterior temporal lobe related to limbic encephalitis. As imaging mimics herpes encephalitis, most patients are initially treated with antiviral therapy until HSV titers are found to be negative.](images/app.statdx.com_image_thumbnail_cc4230df-5bec-43d5-bc3b-9df86787806b_size_168_quality_85_98755dd1_20251018T095220Z.jpg) -*Axial T1 C+ MR shows enhancement in the medial temporal lobes and left anterior temporal lobe related to limbic encephalitis. As imaging mimics herpes encephalitis, most patients are initially treated with antiviral therapy until HSV titers are found to be negative.* - -![Axial FLAIR MR shows abnormal hyperintensity in the medial temporal lobes bilaterally, characteristic of limbic encephalitis, the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.](images/app.statdx.com_image_thumbnail_34910713-5aa7-45ab-a7fe-44681c2acd17_size_168_quality_85_9229bcc4_20251018T095220Z.jpg) -*Axial FLAIR MR shows abnormal hyperintensity in the medial temporal lobes bilaterally, characteristic of limbic encephalitis, the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.* - -![Axial FLAIR MR in an older adult with small cell lung cancer and subacute dementia shows striking hyperintensity in the right insula .](images/app.statdx.com_image_thumbnail_eff199ae-0e2e-4db5-8473-f78aa2acb8e7_size_168_quality_85_dba11f26_20251018T095220Z.jpg) -*Axial FLAIR MR in an older adult with small cell lung cancer and subacute dementia shows striking hyperintensity in the right insula .* - -![Coronal T2 MR in the same patient shows abnormal hyperintensity in both medial temporal lobes and right insular cortex . Imaging of limbic encephalitis mimics that of herpes encephalitis; however, patients with limbic encephalitis have a subacute presentation. Hemorrhage suggests herpes rather than limbic encephalitis.](images/app.statdx.com_image_thumbnail_8ba47b07-a617-46ff-8081-bf3ee120aacf_size_168_quality_85_37a386d3_20251018T095220Z.jpg) -*Coronal T2 MR in the same patient shows abnormal hyperintensity in both medial temporal lobes and right insular cortex . Imaging of limbic encephalitis mimics that of herpes encephalitis; however, patients with limbic encephalitis have a subacute presentation. Hemorrhage suggests herpes rather than limbic encephalitis.* - -![Axial FLAIR MR shows abnormal hyperintensity in the medial temporal lobes bilaterally, characteristic of limbic encephalitis, the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.](images/app.statdx.com_image_thumbnail_1b9bf79c-994c-4fa6-81d7-5a5964383bfe_size_168_quality_85_be3dda6b_20251018T095220Z.jpg) -*Axial FLAIR MR shows abnormal hyperintensity in the medial temporal lobes bilaterally, characteristic of limbic encephalitis, the most common paraneoplastic syndrome. Bilateral involvement is typical of limbic encephalitis.* - diff --git a/out/benign-enlarged-subarachnoid-spaces_3da4fec0-6e87-4bcc-bd66-b4a5d1984f6e.md b/out/benign-enlarged-subarachnoid-spaces_3da4fec0-6e87-4bcc-bd66-b4a5d1984f6e.md new file mode 100644 index 0000000..a36b382 --- /dev/null +++ b/out/benign-enlarged-subarachnoid-spaces_3da4fec0-6e87-4bcc-bd66-b4a5d1984f6e.md @@ -0,0 +1,468 @@ +--- +title: "Benign Enlarged Subarachnoid Spaces" +docid: "3da4fec0-6e87-4bcc-bd66-b4a5d1984f6e" +authors: + - key: "47381de4-c9fd-4999-8dd0-1808cd72db6b" + value: "Luke L. Linscott, MD" + - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607" + value: "Surjith Vattoth, MD, FRCR" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Normal Variants" + slug: "normal-variants" + treeNodeId: "bf92256f-cdff-4bcd-8420-d876b9e4031a" + - + name: "Benign Enlarged Subarachnoid Spaces" + slug: "benign-enlarged-subarachnoid-spaces" + treeNodeId: null +category: "Brain" +documentVersionId: "223d884d-2cb4-42bf-b5ad-ed2ccbd89644" +imageCount: 20 +lastUpdated: "07/31/20" +pageDescription: "Benign Enlarged Subarachnoid Spaces" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Normal Variants, Benign Enlarged Subarachnoid Spaces" +pageTitle: "Benign Enlarged Subarachnoid Spaces | STATdx" +enhancedTitle: "Benign Enlarged Subarachnoid Spaces" +type: "DX" +references: true +ddx: true +cases: 2 +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Normal Variants" + - "Benign Enlarged Subarachnoid Spaces" +--- +# KEY FACTS + +- ## Terminology + + + - Idiopathic enlargement of subarachnoid spaces (SAS) during infancy +- ## Imaging + + + - Primary imaging modality: US + - CT/MR used if fontanelle closing or to further investigate atypical clinical/US findings + - Best clue: Enlarged SAS and ↑ orbitofrontal circumference (OFC) (> 95th percentile) + - Ventricles may be mildly enlarged + - Symmetric bifrontal and bitemporal SAS + - All modalities show veins coursing through SAS + - SAS follow CSF appearance on all modalities + - No compression of veins or gyri + - No inward displacement of arachnoid membrane by subdural fluid; small, nonhemorrhagic, subdural collections seen in ~ 4% of patients with enlarged SAS +- ## Top Differential Diagnoses + + + - Atrophy + - Acquired progressive communicating hydrocephalus + - Nonaccidental trauma (NAT) +- ## Pathology + + + - Etiology uncertain: Immature CSF drainage pathways likely + - Family history of macrocephaly > 80% +- ## Clinical Issues + + + - Mild developmental delay alone should not prompt further imaging or subspecialty evaluation + - Further evaluation required only in setting of focal neurologic signs &/or developmental regression + - Consider NAT if enlarged extraaxial spaces atypical + - Moderate/large/complex subdural collection → NAT work-up + - SAS enlargement and developmental delay typically resolve without therapy by 2 years of age + - No treatment necessary +- ## Diagnostic Checklist + + + - Further evaluation with brain MR or CT if US atypical + - Even small/simple subdural collections should be discussed with referring clinician to identify any concerns for NAT that merit further work-up + +# TERMINOLOGY + +- ## Synonyms + + + - Benign enlargement of subarachnoid spaces of infancy (BESSI) + - Benign external hydrocephalus + - Benign extracerebral fluid collections of infancy + - Benign communicating hydrocephalus + - Physiologic extraventricular obstructive hydrocephalus + - Benign macrocephaly of infancy +- ## Definitions + + + - Enlarged subarachnoid spaces (SAS) in patient < 1 year of age with macrocrania [head circumference (HC) > 95%] + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Symmetric bilateral SAS enlargement ± mild ventriculomegaly + - Patient with normal or mildly delayed development + - ### Location + + + - Enlarged SAS in infant with macrocrania + - Symmetric at bifrontal and bitemporal SAS + - ### Size + + + - Normal SAS values differ significantly between studies + - Normal maximum width peaks at 28 postnatal weeks (7 months of age) + - Interhemispheric width: 95th percentile: ~ 8 mm + - Widest distance between hemispheres + - Craniocortical width: 95th percentile: ~ 10 mm + - Widest vertical distance between brain and inner table of calvarium + - Sinocortical width: 95th percentile: ~ 7 mm + - Widest distance between cortex and superior sagittal sinus + - ### Morphology + + + - CSF space follows (not flattens) gyral contour +- ## Radiographic Findings + + + - Radiography + - ↑ craniofacial ratio +- ## CT Findings + + + - ### NECT + + + - Enlarged SAS with normal sulci; no hemorrhage + - Enlarged cisterns (especially suprasellar/chiasmatic) + - ### CECT + + + - Demonstrates veins traversing SAS + - No abnormal meningeal enhancement +- ## MR Findings + + + - ### T1WI + + + - Normal brain parenchyma without edema + - Small subdural collections sometimes visible + - ### T2WI + + + - Arachnoid membranes + - Small nonhemorrhagic subdural collections in ~ 4% + - ### FLAIR + + + - SAS fluid follows CSF signal on all sequences + - Incomplete signal suppression in subdural collections + - ### DWI + + + - ↑ fractional anisotropy and mean diffusivity in brain of patients with enlarged SAS compared to controls + - Normalizes over time with resolution of SAS enlargement + - ### T1WI C+ + + + - Demonstrates veins traversing SAS + - SSFSE + - May be used for follow-up to avoid sedation in children +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Primary modality used whenever possible + - Symmetric enlargement of bifrontal SAS + - ± mild ventricular enlargement + - ### Color Doppler + + + - Cortical veins seen within subarachnoid fluid space + - No mass effect displacing veins against pia + - No inward displacement of arachnoid membrane by subdural fluid + - Subdural collections lack traversing veins +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - US if acoustic window available + - CT/MR if no acoustic window available + - ### Protocol advice + + + - Doppler sonography: Documents veins traversing SAS + - Linear high-resolution US most sensitive for detection of associated subdural fluid + - After diagnosis, best follow-up: Clinical monitoring of HC and development of any neurologic findings + - Follow-up with MR/CT typically not necessary, unless + - Focal neurologic signs/symptoms + - Suspicion for subdural collection on US + +# DIFFERENTIAL DIAGNOSIS + +- ## Atrophy + + + - Small HC; sulcal prominence out of proportion + - Forehead "pointed" due to metopic fusion +- ## Incidental Bilateral Subdural Fluid Collections + + + - Subdural fluid not normally visualized + - Small, nonhemorrhagic subdural collections seen in 4% of benign macrocrania patients + - Characterized by crescentic fluid collection separating dura from arachnoid + - No cortical veins traversing subdural space + - Discrete arachnoid membrane displaced toward cortex; may be compressing SAS veins + - May have different signal intensity on PD and other MR sequences compared to CSF + - Discuss need for further work-up with referring clinician + - Close clinical follow-up at minimum; work-up for nonaccidental trauma (NAT) at discretion of clinician +- ## Nonaccidental Trauma + + + - Moderate/large or hemorrhagic subdurals or unusual clinical findings should raise concern +- [Glutaric Aciduria Type 1](/document/glutaric-acidemia-type-1/55db6f3e-1d78-4bc6-b366-68b2d37e5d80) + - Enlarged sylvian fissures with delayed myelination + - Subdural collections may be present + - T2-hyperintense basal ganglia +- ## Elevated Venous Pressures + + + - Causes: Cardiac disease, internal jugular vein sacrifice for ECMO, arteriovenous fistula, or sinus venous thrombosis +- ## Communicating Hydrocephalus + + + - Often post hemorrhagic/post inflammatory/neoplastic + - Density of extraaxial collection does not = CSF + - Achondroplasia and other skull base anomalies + - Coarctation of foramen magnum (narrow) + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Remains incompletely understood + - Immature CSF drainage pathways: Most accepted theory + - CSF primarily drained via extracellular space → capillaries + - Pacchionian granulations (PGs) do not mature until 18 months + - PGs then displaced into veins (as Starling-type resistors) + - PGs regulate venous drainage of CSF when fontanels close + - Benign SAS enlargement usually resolves at that time + - Disproportionate growth of skull and brain + - Faster growth of skull results in ↑ SAS ± ↑ ventricles + - This theory helps to explain frequent identification of subdural fluid collections + - Family history of macrocephaly > 80% + - ### Associated abnormalities + + + - Subdural collections (typically small and incidental) in ~ 4% + - Predisposition to bleed with minor trauma: Controversial + - Possibility of ↑ risk for bridging vein injury and subdural collection/hematoma in absence of major trauma + - Venous "stretching" implicated + - May ↑ risk of arachnoid cyst development compared to normal population +- ## Gross Pathologic & Surgical Features + + + - Deep/prominent but otherwise normal-appearing SAS + - No pathologic membranes +- ## Microscopic Features + + + - Ependymal damage not seen in benign SAS enlargement + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Macrocrania: HC > 95th percentile + - Frontal bossing + - No signs of elevated intracranial pressure (ICP); normal pressure on lumbar puncture + - Danger signs + - Elevated ICP + - Persistent or rapid deviation of HC from normal curve + - Developmental regression, focal neurologic signs, vomiting, bruising + - ### Other signs/symptoms + + + - Mild developmental delay common (20-50%) and usually resolves over time + - Should not necessarily prompt further evaluation + - ### Clinical profile + + + - Family history of benign macrocephaly common + - Male infants, ± late to walk +- ## Demographics + + + - Most common imaging diagnosis for macrocrania in patients < 1 year of age  + - Usually presents at 3-9 months + - Sex: M:F = 2:1 +- ## Natural History & Prognosis + + + - Enlarged SAS → ↑ suture/calvarial malleability/compliance → predisposes to posterior plagiocephaly + - Self-limited; resolves without therapy by 12-24 months + - Spontaneous resolution of spaces and symptoms + - Macrocephaly may persist +- ## Treatment + + + - No treatment necessary + - Normal outcome (developmental delay usually resolves as prominent SAS resolves) + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - Crucial to know HC + - Further evaluation with brain MR or CT if US atypical + - Moderate/large/complex subdural collection → NAT work-up + - Even small/simple subdural collections should be discussed with referring clinician to identify any concerns for NAT that merit further work-up + + 17f5e314-01d9-44b0-81da-6468019ad492 + +## References + +# Selected References + +1. [Yum SK et al: Enlarged subarachnoid space on cranial ultrasound in preterm infants: Neurodevelopmental implication. Sci Rep. 9(1):19072, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31836837%5Bpmid%5D) +1. [Zahl SM et al: Clinical, radiological, and demographic details of benign external hydrocephalus: a population-based study. Pediatr Neurol. 96:53-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30808532%5Bpmid%5D) +1. [Zahl SM et al: Quality of life and physician-reported developmental, cognitive, and social problems in children with benign external hydrocephalus-long-term follow-up. Childs Nerv Syst. 35(2):245-50, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30523438%5Bpmid%5D) +1. [Hansen JB et al: Evaluations for abuse in young children with subdural hemorrhages: findings based on symptom severity and benign enlargement of the subarachnoid spaces. J Neurosurg Pediatr. 21(1):31-7, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29099352%5Bpmid%5D) +1. [Haws ME et al: A retrospective analysis of the utility of head computed tomography and/or magnetic resonance imaging in the management of benign macrocrania. J Pediatr. 182:283-9.e1, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27989412%5Bpmid%5D) +1. [Hussain ZB et al: Extra-axial cerebrospinal fluid spaces in children with benign external hydrocephalus: a case-control study. Neuroradiol J. 30(5):410-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28691570%5Bpmid%5D) +1. [Naffaa L et al: The diagnostic yield of ultrasound of the head in healthy infants presenting with the clinical diagnosis of benign macrocrania. Clin Radiol. 72(1):94.e7-94.e11, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27756452%5Bpmid%5D) +1. [Whitehead MT et al: Reduced subarachnoid fluid diffusion in enlarged subarachnoid spaces of infancy. Neuroradiol J. 30(5):418-24, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28195509%5Bpmid%5D) +1. [Tucker J et al: Macrocephaly in infancy: benign enlargement of the subarachnoid spaces and subdural collections. J Neurosurg Pediatr. 1-5, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26942270%5Bpmid%5D) +1. [Halevy A et al: Development of infants with idiopathic external hydrocephalus. J Child Neurol. 30(8):1044-7, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25348416%5Bpmid%5D) +1. [Marino MA et al: Benign external hydrocephalus in infants. A single centre experience and literature review. Neuroradiol J. 27(2):245-50, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24750715%5Bpmid%5D) +1. [Greiner MV et al: Prevalence of subdural collections in children with macrocrania. AJNR Am J Neuroradiol. 34(12):2373-8, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23868166%5Bpmid%5D) +1. [Mattei TA et al: Benign extracerebral fluid collection in infancy as a risk factor for the development of de novo intracranial arachnoid cysts. J Neurosurg Pediatr. 12(6):555-64, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24093592%5Bpmid%5D) +1. [Schulz M et al: Intracranial pressure measurement in infants presenting with progressive macrocephaly and enlarged subarachnoid spaces. Acta Neurochir Suppl. 114:261-6, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22327705%5Bpmid%5D) +1. [Sun M et al: Diffusion tensor imaging findings in young children with benign external hydrocephalus differ from the normal population. Childs Nerv Syst. 28(2):199-208, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22167268%5Bpmid%5D) +1. [Bateman GA et al: External hydrocephalus in infants: six cases with MR venogram and flow quantification correlation. Childs Nerv Syst. 27(12):2087-96, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21833725%5Bpmid%5D) +1. [Yew AY et al: Long-term health status in benign external hydrocephalus. Pediatr Neurosurg. 47(1):1-6, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21778677%5Bpmid%5D) +1. [Zahl SM et al: Benign external hydrocephalus: a review, with emphasis on management. Neurosurg Rev. 34(4):417-32, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21647596%5Bpmid%5D) +1. [Fernando S et al: Neuroimaging of nonaccidental head trauma: pitfalls and controversies. Pediatr Radiol. 38(8):827-38, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18176805%5Bpmid%5D) +1. [Hellbusch LC: Benign extracerebral fluid collections in infancy: clinical presentation and long-term follow-up. J Neurosurg. 107(2 Suppl):119-25, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=18459883%5Bpmid%5D) +1. [Paciorkowski AR et al: When is enlargement of the subarachnoid spaces not benign? A genetic perspective. Pediatr Neurol. 37(1):1-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17628215%5Bpmid%5D) +1. [Muenchberger H et al: Idiopathic macrocephaly in the infant: long-term neurological and neuropsychological outcome. Childs Nerv Syst. 22(10):1242-8, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16544148%5Bpmid%5D) +1. [Lam WW et al: Ultrasonographic measurement of subarachnoid space in normal infants and children. Pediatr Neurol. 25(5):380-4, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11744312%5Bpmid%5D) + +## Differential diagnosis + +### Cistern, Subarachnoid Space Normal Variant +DDX:167a514e-0b18-4a16-9474-41a1d760607b + +### Subarachnoid Space Normal Variants +DDX:558a9979-3a38-473f-a5f8-bf6b6d6538e2 + +## Cases + +- {'cases': [{'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': 'bd82b696-6c64-439d-a4e9-417829b23517', 'description': 'T2W axial images (#1-4) demonstrate "squaring" of the forehead and prominent pericerebral spaces. Small linear flow voids (arrows #2-4) represent veins traversing the subarachnoid space. The CSF spaces are at their widest at approximately 7 months of life. This process has also been called benign macrocephaly of infancy, physiologic extraventricular obstructive hydrocephalus, and external hydrocephalus. It is self-limited, usually resolving without therapy by 1 to 2 years of age.', 'history': 'Presented with macrocrania and frontal bossing.', 'imagePoolId': 'fd5ba87e-b9d9-48f7-aa78-96eac3fc925e', 'name': 'Crossing vessels', 'teachingPoint': None, 'demographics': '7 Months old male'}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': '9e524b89-ebd0-4d81-9776-61b08302b2ec', 'description': 'NECT (#1, 2) demonstrate marked enlargement of the frontal pericerebral CSF spaces (arrow). Without the use of IV contrast material, traversing venous structures cannot be assessed. Similar findings are seen on sagittal and axial T1WIs (#3, 4) and axial FLAIR (#5). Axial (#6, 7) and coronal (#8) T2W images, however, are extremely useful. The T2W images reveal fine linear flow-voids due to traversing veins (#6-8, curved arrows), confirming that these enlarged spaces are in fact the subarachnoid space. \n\nComment: Enlarged pericerebral spaces in infancy are often a transient and benign condition. They resolve between 8 and 12 months of age, usually when the infant is able to be in the upright position for longer periods of time. They are slower to resolve in late walkers. The traversing veins may bleed with trauma, simulating non-accidental injury. Sequential follow-up of head-circumference (tape-measure, not imaging) is suggested. Repeat imaging is suggested when the macrocrania rapidly progresses, or when there are neurological symptoms.', 'history': 'Typically present with macrocrania between the ages of 3 and 8 months of age.', 'imagePoolId': 'ad479e42-534c-4d0e-9c71-416160f544a5', 'name': 'Marked', 'teachingPoint': None, 'demographics': '8 Months old male'}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': '94dfc51a-38fa-4d6c-98ea-9215b294dad3', 'description': 'Axial NECT reveals an asymmetric prominence of pericerebral spaces (arrow, #1). T2W images obtained 5 months later demonstrate persistence of the asymmetry (arrows, #2, 3). No membranes or unequal signal intensity are seen on FLAIR (#4). Enhancing veins traverse the dilated subarachnoid space (curved arrows, #5). Enlargement of the subarachnoid space is common during infancy; danger signs requiring imaging evaluation would include rapid enlargement of head circumference, marked prominence of subarachnoid space, increased intracranial pressure and persistence or onset after 1 year of age. Asymmetry is also concerning, raising the suspicion for underlying subdural collection in non-accidental trauma.', 'history': 'Presented with macrocrania and possible seizures.', 'imagePoolId': 'd78c443b-8aae-4312-ab47-bfae3322a312', 'name': 'Asymmetric', 'teachingPoint': None, 'demographics': '3 Months old male'}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}, {'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'c76edba4-99cf-4471-99a3-e58bc8676bab', 'description': 'There is marked asymmetry of subarachnoid space enlargement (#1-8). After contrast administration, however, veins are seen to traverse the subarachnoid space (arrow, #5-8). Compression of the right posterior subarachnoid space by positional lambdoid flattening (open arrow, #1, 2, 4) and calvarial deformation accounts, in part, for the prominence of the contralateral spaces. Asymmetry is of concern and should prompt a search for an underlying subdural component or of underlying brain dysgenesis. In this child, however, traversing veins confirm involvement only of the subarachnoid space.', 'history': None, 'imagePoolId': '91d9adcc-19f5-46a3-b1c1-2ceff2c37812', 'name': 'Asymmetric', 'teachingPoint': None, 'demographics': '3 Months old male'}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': 'ddc8c96d-0330-4a2a-8065-24b477e33f11', 'description': 'Enlarged pericerebral spaces are identified on coronal sonography. Distance between the surface of the brain and the dura is 1.5 cm, or 3 times the maximum allowable measurement of 5 mm. High-resolution view reveals multiple linear veins (arrows) traversing the subarachnoid space, confirming the diagnosis. \n\nNECT demonstrates huge pericerebral spaces over the frontal convexities and widening the interhemispheric and Sylvian fissures. CECT confirms a plethora of veins (arrows) traversing the subarachnoid space. No subdural membrane is identified.\n\nEnlargement of the subarachnoid spaces, also known as physiologic extraventricular obstructive hydrocephalus (EVOH), external hydrocephalus or benign macrocephaly of infancy is usually a transient phenomenon peaking between 3 and 8 months of age. Surgical intervention is needed only when spaces reach massive size, as in this case. \n\nBe careful not to mistake enlarged arachnoid spaces (in the presence of macrocephaly) for atrophy. Always determine and document the head circumference.', 'history': 'Patient was noted to have macrocephaly at 2 months of age. Subarachnoid spaces enlarged dramatically over time. Patient required shunt at 7 months.', 'imagePoolId': 'e88a10c0-feca-4740-8c77-7996345f28ae', 'name': 'Massive', 'teachingPoint': None, 'demographics': '7 Months old male'}], 'caseType': 'variant', 'name': 'VARIANT'} + + +## Images + + +### Selected Images + +![Axial graphic shows classic enlarged subarachnoid spaces (SAS) in a macrocephalic infant (head circumference > 95%). Note the symmetric enlargement with idiopathic enlargement of SAS during the 1st year of life.](images/app.statdx.com_image_thumbnail_625c002a-cbdb-4b3b-82b8-c174380b8cab_annotated_true_size_900_quality_90_0217a31f_20251018T164822Z.jpg) +*Axial graphic shows classic enlarged subarachnoid spaces (SAS) in a macrocephalic infant (head circumference > 95%). Note the symmetric enlargement with idiopathic enlargement of SAS during the 1st year of life.* + +![Axial graphic shows classic enlarged subarachnoid spaces (SAS) in a macrocephalic infant (head circumference > 95%). Note the symmetric enlargement with idiopathic enlargement of SAS during the 1st year of life.](images/app.statdx.com_image_thumbnail_625c002a-cbdb-4b3b-82b8-c174380b8cab_size_174_quality_85_c1b69f4c_20251018T164756Z.jpg) +*Axial graphic shows classic enlarged subarachnoid spaces (SAS) in a macrocephalic infant (head circumference > 95%). Note the symmetric enlargement with idiopathic enlargement of SAS during the 1st year of life.* + +![Axial T2 MR shows enlarged frontal & anterior interhemispheric pericerebral fluid spaces , mild ventriculomegaly, & right-sided posterior plagiocephaly in a 7-month-old boy with macrocephaly.](images/app.statdx.com_image_thumbnail_999bae79-c259-4e1e-99f7-df0f6d6080e6_annotated_true_size_900_quality_90_99a2989a_20251018T164822Z.jpg) +*Axial T2 MR shows enlarged frontal & anterior interhemispheric pericerebral fluid spaces , mild ventriculomegaly, & right-sided posterior plagiocephaly in a 7-month-old boy with macrocephaly.* + +![Coronal US in a 7-month-old boy with macrocrania shows enlarged SAS & normal ventricular size. Note the normal size of the sulci. This is a typical clinical history & imaging appearance for benign enlargement of the SAS.](images/app.statdx.com_image_thumbnail_e4adf66f-2a85-49d4-81d1-b5a27e609e47_annotated_true_size_900_quality_90_697cf19c_20251018T164822Z.jpg) +*Coronal US in a 7-month-old boy with macrocrania shows enlarged SAS & normal ventricular size. Note the normal size of the sulci. This is a typical clinical history & imaging appearance for benign enlargement of the SAS.* + +![Coronal color Doppler US in a 4-month-old girl shows vessels traversing the enlarged SAS . Doppler US can be helpful to exclude subdural collections by demonstrating normal veins in the SAS.](images/app.statdx.com_image_thumbnail_2378a15b-8f1c-47cf-bf91-5ab9181ca721_annotated_true_size_900_quality_90_ad41edb4_20251018T164822Z.jpg) +*Coronal color Doppler US in a 4-month-old girl shows vessels traversing the enlarged SAS . Doppler US can be helpful to exclude subdural collections by demonstrating normal veins in the SAS.* + +![Coronal US in a 3 month old with macrocephaly shows prominent SAS as well as mild enlargement of the lateral ventricles . Mild lateral ventricular enlargement is common in benign enlargement of subarachnoid spaces (BESSI).](images/app.statdx.com_image_thumbnail_7ed3d5fd-ca66-4f3f-b16a-def63fa152be_annotated_true_size_900_quality_90_0bbc9592_20251018T164822Z.jpg) +*Coronal US in a 3 month old with macrocephaly shows prominent SAS as well as mild enlargement of the lateral ventricles . Mild lateral ventricular enlargement is common in benign enlargement of subarachnoid spaces (BESSI).* + +![Coronal T2 MR in a 6 month old with macrocephaly shows symmetrically prominent bifrontal SAS with mild enlargement of the lateral ventricles . Mild enlargement of the lateral ventricles should not dissuade one from suggesting BESSI.](images/app.statdx.com_image_thumbnail_82a63fe6-8800-487d-a506-2f4b3399fd2d_annotated_true_size_900_quality_90_fef6728f_20251018T164822Z.jpg) +*Coronal T2 MR in a 6 month old with macrocephaly shows symmetrically prominent bifrontal SAS with mild enlargement of the lateral ventricles . Mild enlargement of the lateral ventricles should not dissuade one from suggesting BESSI.* + +![Coronal T2 MR at 13 months (left) & NECT at 5 years (right) of age show expected resolution of the enlarged SAS over a 4-year period. Enlarged SAS typically resolve by 24 months of age.](images/app.statdx.com_image_thumbnail_6d4772cf-8e5a-46f7-b768-d7df54bae831_annotated_true_size_900_quality_90_1842bb3c_20251018T164822Z.jpg) +*Coronal T2 MR at 13 months (left) & NECT at 5 years (right) of age show expected resolution of the enlarged SAS over a 4-year period. Enlarged SAS typically resolve by 24 months of age.* + +![Coronal high-resolution US in a 4-month-old girl with macrocrania shows bilateral enlargement of the SAS . Also present are small, bilateral, subdural collections , which are anechoic compared to the SAS. Note the separation of the arachnoid membrane .](images/app.statdx.com_image_thumbnail_e1ad2f29-ee6d-4acb-9009-60ab93f84ebb_annotated_true_size_900_quality_90_5094ced0_20251018T164822Z.jpg) +*Coronal high-resolution US in a 4-month-old girl with macrocrania shows bilateral enlargement of the SAS . Also present are small, bilateral, subdural collections , which are anechoic compared to the SAS. Note the separation of the arachnoid membrane .* + +![Axial PD MR in a 4-month-old girl with macrocrania shows enlarged SAS , which are isointense to the brain. Also note the small, bilateral, nonhemorrhagic, hyperintense subdural fluid collections .](images/app.statdx.com_image_thumbnail_c10a685b-5eda-415a-a0a7-c9ba71989dbc_annotated_true_size_900_quality_90_5ff1fb6d_20251018T164822Z.jpg) +*Axial PD MR in a 4-month-old girl with macrocrania shows enlarged SAS , which are isointense to the brain. Also note the small, bilateral, nonhemorrhagic, hyperintense subdural fluid collections .* + +![Coronal T2 MR in the same patient shows symmetrically enlarged SAS as well as small, bilateral, nonhemorrhagic subdural fluid collections . Small, subdural fluid collections are seen in ~ 4% of patients with enlarged SAS.](images/app.statdx.com_image_thumbnail_dadaa55c-ec98-4fde-8e1a-f4ff02313e31_annotated_true_size_900_quality_90_8c00f0a2_20251018T164802Z.jpg) +*Coronal T2 MR in the same patient shows symmetrically enlarged SAS as well as small, bilateral, nonhemorrhagic subdural fluid collections . Small, subdural fluid collections are seen in ~ 4% of patients with enlarged SAS.* + + +### Additional Images + +![Axial graphic shows classic enlargement of the subarachnoid spaces (SAS) in a macrocephalic infant. There is symmetric bifrontal enlargement of the SAS, which contain multiple bridging veins . Mild ventriculomegaly is present.](images/app.statdx.com_image_thumbnail_970df207-9c92-40e4-83d2-71d303b99906_annotated_true_size_900_quality_90_013d624a_20251018T164803Z.jpg) +*Axial graphic shows classic enlargement of the subarachnoid spaces (SAS) in a macrocephalic infant. There is symmetric bifrontal enlargement of the SAS, which contain multiple bridging veins . Mild ventriculomegaly is present.* + +![Axial T2 MR in a 6-month-old boy with enlarged SAS shows vessels coursing through the SAS. Note the lack of mass effect on the underlying brain parenchyma. There is mild enlargement of the lateral ventricles , a common finding in benign enlargement of the SAS.](images/app.statdx.com_image_thumbnail_853ffc4b-e3ff-4876-a50a-9fd8233de3af_annotated_true_size_900_quality_90_7726ca40_20251018T164803Z.jpg) +*Axial T2 MR in a 6-month-old boy with enlarged SAS shows vessels coursing through the SAS. Note the lack of mass effect on the underlying brain parenchyma. There is mild enlargement of the lateral ventricles , a common finding in benign enlargement of the SAS.* + +![Coronal T2 MR in the same 4-month-old girl with macrocrania shows symmetrically enlarged SAS as well as small to moderate, bilateral subdural fluid collections . The subdural collections are slightly hyperintense to the SAS. Small subdural fluid collections are seen in ~ 4% of patients with enlarged SAS.](images/app.statdx.com_image_thumbnail_954b835b-dd1b-4ebc-922e-0e238b1c9468_annotated_true_size_900_quality_90_d9a0b852_20251018T164803Z.jpg) +*Coronal T2 MR in the same 4-month-old girl with macrocrania shows symmetrically enlarged SAS as well as small to moderate, bilateral subdural fluid collections . The subdural collections are slightly hyperintense to the SAS. Small subdural fluid collections are seen in ~ 4% of patients with enlarged SAS.* + +![Axial CECT shows enlarged SAS with enhancing traversing veins in a macrocephalic infant. This benign condition usually peaks at 7 months of age & resolves spontaneously by 12-24 months of age.](images/app.statdx.com_image_thumbnail_6853ee67-afda-4c9b-86f1-275ecc5b5521_annotated_true_size_900_quality_90_9bff7ce2_20251018T164803Z.jpg) +*Axial CECT shows enlarged SAS with enhancing traversing veins in a macrocephalic infant. This benign condition usually peaks at 7 months of age & resolves spontaneously by 12-24 months of age.* + +![Axial T2 MR shows prominent frontal CSF spaces (craniocortical & interhemispheric) with mildly prominent ventricles in this macrocephalic infant. Note the squaring of the forehead, seen clinically as "frontal bossing." About 20-50% of cases have mild developmental delay (motor > > language), which nearly always resolves without therapy.](images/app.statdx.com_image_thumbnail_e08c6654-1cb2-4a96-bab2-26736e44e4d7_annotated_true_size_900_quality_90_85e3f2a6_20251018T164803Z.jpg) +*Axial T2 MR shows prominent frontal CSF spaces (craniocortical & interhemispheric) with mildly prominent ventricles in this macrocephalic infant. Note the squaring of the forehead, seen clinically as "frontal bossing." About 20-50% of cases have mild developmental delay (motor > > language), which nearly always resolves without therapy.* + +![Axial NECT shows classic enlargement of SAS in this macrocephalic 5-month-old patient. Note the > 5-mm widening of the bifrontal craniocortical & anterior interhemispheric SAS.](images/app.statdx.com_image_thumbnail_d638e4e6-f964-4d41-808f-5de842763d53_annotated_true_size_900_quality_90_8f53a129_20251018T164803Z.jpg) +*Axial NECT shows classic enlargement of SAS in this macrocephalic 5-month-old patient. Note the > 5-mm widening of the bifrontal craniocortical & anterior interhemispheric SAS.* + +![Axial CECT shows veins traversing the enlarged SAS.](images/app.statdx.com_image_thumbnail_d39a9497-890e-46b1-96c0-be6b83826f7c_annotated_true_size_900_quality_90_c8e6f3da_20251018T164803Z.jpg) +*Axial CECT shows veins traversing the enlarged SAS.* + +![Axial T2 MR shows veins, represented by linear flow voids , traversing the enlarged SAS.](images/app.statdx.com_image_thumbnail_227b79c7-dac6-4e19-b936-bb18a562b566_annotated_true_size_900_quality_90_750c0054_20251018T164804Z.jpg) +*Axial T2 MR shows veins, represented by linear flow voids , traversing the enlarged SAS.* + +![Coronal US shows dilated craniocortical SAS (note the space between the 2 markers) with veins traversing the SAS.](8b6b881a-7f98-409c-bb74-a593ce3bcc3c) +*Coronal US shows dilated craniocortical SAS (note the space between the 2 markers) with veins traversing the SAS.* + +![Coronal T2 MR shows markedly enlarged SAS with prominent ventricles & traversing bridging veins . Tiny, bilateral subdural collections are present .](82b8f025-0258-42d9-992c-faa46158c0e2) +*Coronal T2 MR shows markedly enlarged SAS with prominent ventricles & traversing bridging veins . Tiny, bilateral subdural collections are present .* + diff --git a/out/cavum-septi-pellucidi-csp_02127bd4-1efa-4056-925e-f1a1bbadf154.md b/out/cavum-septi-pellucidi-csp_02127bd4-1efa-4056-925e-f1a1bbadf154.md new file mode 100644 index 0000000..ef3d2df --- /dev/null +++ b/out/cavum-septi-pellucidi-csp_02127bd4-1efa-4056-925e-f1a1bbadf154.md @@ -0,0 +1,319 @@ +--- +title: "Cavum Septi Pellucidi (CSP)" +docid: "02127bd4-1efa-4056-925e-f1a1bbadf154" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" + - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850" + value: "Anne G. Osborn, MD, FACR" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Normal Variants" + slug: "normal-variants" + treeNodeId: "bf92256f-cdff-4bcd-8420-d876b9e4031a" + - + name: "Cavum Septi Pellucidi (CSP)" + slug: "cavum-septi-pellucidi-csp" + treeNodeId: null +category: "Brain" +documentVersionId: "1d576faa-488e-43a7-b6d4-2479d199a187" +imageCount: 11 +lastUpdated: "05/08/20" +pageDescription: "Cavum Septi Pellucidi (CSP)" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Normal Variants, Cavum Septi Pellucidi (CSP)" +pageTitle: "Cavum Septi Pellucidi (CSP) | STATdx" +enhancedTitle: "Cavum Septi Pellucidi (CSP)" +type: "DX" +references: true +cases: 2 +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Normal Variants" + - "Cavum Septi Pellucidi (CSP)" +--- +# KEY FACTS + +- ## Terminology + + + - Cystic CSF cavity of septum pellucidum (SP) + - Occurs ± cavum vergae (CV) +- ## Imaging + + + - Elongated finger-shaped CSF collection between lateral ventricles + - Cavum septi pellucidi (CSP): Between frontal horns of lateral ventricles + - CV: Posterior extension between fornices + - Size varies from slit like to several mm, occasionally > 1 cm + - SP invariably cystic in fetus + - Width of fetal CSP increases between 19-27 weeks + - Plateaus at 28 weeks + - Gradually closes in rostral direction between 28 weeks and term + - CSP present in 100% of premature, 85% of term infants + - CSP seen in up to 15-20% of adults +- ## Top Differential Diagnoses + + + - Asymmetric lateral ventricles + - Cavum velum interpositum + - Ependymal cyst + - Absent SP +- ## Pathology + + + - CSP forms if fetal SP fails to obliterate + - Precise etiology of fluid accumulation unknown + - CSP is not "5th ventricle" + - CV is not "6th ventricle" +- ## Clinical Issues + + + - Usually asymptomatic, incidental + - Headache (relationship to cyst unclear) + - CSP frequent among athletes with history of repeated traumatic brain injury, such as boxers +- ## Diagnostic Checklist + + + - CV almost never occurs without CSP + +# TERMINOLOGY + +- ## Abbreviations + + + - Cavum septi pellucidi (CSP) + - Cavum vergae (CV) +- ## Definitions + + + - Cystic CSF cavity of septum pellucidum (CSP) ± posterior continuation (CV) + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Elongated finger-shaped CSF collection between lateral ventricles + - ### Location + + + - CSP: Between frontal horns of lateral ventricles + - CV: Posterior extension between fornices + - ### Size + + + - From slit-like to several mm, occasionally > 1 cm + - ### Morphology + + + - Elongated, finger like +- ## CT Findings + + + - ### NECT + + + - CSF collection in septum pellucidum + - ### CECT + + + - Does not enhance +- ## MR Findings + + + - ### T1WI + + + - Axial: Finger-like CSF space between lateral ventricles + - Sagittal: Extends posteriorly from rostrum to splenium of corpus callosum (CC) above, ICVs below + - ### T2WI + + + - Isointense with CSF + - ### FLAIR + + + - Suppresses completely + - ### DWI + + + - Does not restrict +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Septum pellucidum invariably present in normal fetus + - Width of fetal CSP increases between 19-27 weeks + - Plateaus, then gradually closes in rostral direction between 28 weeks and term + - Inability to find CSP on fetal US requires search for CC + +# DIFFERENTIAL DIAGNOSIS + +- [Asymmetric Lateral Ventricles](/document/arachnoid-cyst/7d63f0d3-6999-4d8c-a41d-953f738c43a6) + - Septum pellucidum bowed but intact +- [Cavum Velum Interpositum](/document/arachnoid-cyst/7d63f0d3-6999-4d8c-a41d-953f738c43a6) + - Triangular shaped; no extension anterior to foramen of Monro +- [Ependymal Cyst](/document/ependymal-cyst/910da375-2150-49b5-8e0c-bc93487239d1) + - In body/atrium of lateral ventricle +- [Septooptic Dysplasia](/document/septo-optic-dysplasia/df4653b9-6e80-4f77-b737-29280f00d1ad) + - SP absent, "squared off" frontal horns + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - CSP forms if fetal septum pellucidum fails to obliterate + - Precise etiology of fluid accumulation unknown + - CSP is not "5th ventricle," nor is CV "6th ventricle" + - ### Associated abnormalities + + + - Rare: Hydrocephalus +- ## Staging, Grading, & Classification + + + - Shaw and Ellsworth classification for CSP, CV + - Asymptomatic, incidental cavum (communicating or not) + - Symptomatic, pathological, noncommunicating cavum + - Simple and uncomplicated + - Complicated by other lesions + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Usually asymptomatic, incidental + - May remain asymptomatic even if mass effect present + - Headache (relationship to cyst unclear) +- ## Demographics + + + - ### Age + + + - CSP + - Present in 100% of premature, 85% of term infants + - From 1% up to 15-20% of adults + - CV + - 100% at fetal age of 6 months, 30% at term + - < 1% of adults +- ## Natural History & Prognosis + + + - Normally regresses + - May persist as normal variant + - Rare: Enlarges, may cause mass effect +- ## Treatment + + + - Usually none + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - CV almost never occurs without CSP + + 1f464af2-e137-4e40-827c-7f61f0623628 + +## References + +# Selected References + +1. [Krejčí T et al: Symptomatic cysts of the cavum septi pellucidi, cavum vergae and cavum veli interpositi: A retrospective duocentric study of 10 patients. Clin Neurol Neurosurg. 185:105494, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31472394%5Bpmid%5D) +1. [Lee JK et al: Association of cavum septum pellucidum and cavum vergae with cognition, mood, and brain volumes in professional fighters. JAMA Neurol. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31498371%5Bpmid%5D) +1. [M Das J et al: Cavum septum pellucidum 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30725733%5Bpmid%5D) +1. [Nagaraj UD et al: Abnormalities associated with the cavum septi pellucidi on fetal MRI: What radiologists need to know. AJR Am J Roentgenol. 210(5):989-97, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29489402%5Bpmid%5D) +1. [Tsutsumi S et al: Visualization of the cavum septi pellucidi, cavum Vergae, and cavum veli interpositi using magnetic resonance imaging. Surg Radiol Anat. 40(2):159-64, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29094193%5Bpmid%5D) +1. [Gardner RC et al: Cavum septum pellucidum in retired American pro-football players. J Neurotrauma. ePub, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25970145%5Bpmid%5D) +1. [Toivonen P et al: Cavum septum pellucidum and psychopathy. Br J Psychiatry. 203(2):152-3, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23908342%5Bpmid%5D) +1. [Santo S et al: Counseling in fetal medicine: agenesis of the corpus callosum. Ultrasound Obstet Gynecol. 40(5):513-21, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23024003%5Bpmid%5D) +1. [Winter TC et al: The cavum septi pellucidi: why is it important? J Ultrasound Med. 29(3):427-44, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20194938%5Bpmid%5D) +1. [Callen PW et al: Columns of the fornix, not to be mistaken for the cavum septi pellucidi on prenatal sonography. J Ultrasound Med. 27(1):25-31, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18096727%5Bpmid%5D) +1. [Takahashi T et al: Prevalence of large cavum septi pellucidi in ultra high-risk individuals and patients with psychotic disorders. Schizophr Res. 105(1-3):236-44, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18693084%5Bpmid%5D) +1. [Needelman H et al: Postterm closure of the cavum septi pellucidi and developmental outcome in premature infants. J Child Neurol. 22(3):314-6, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17621502%5Bpmid%5D) +1. [Sencer A et al: Cerebrospinal fluid dynamics of the cava septi pellucidi and vergae. Case report. J Neurosurg. 94(1):127-9, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11147881%5Bpmid%5D) + +## Cases + +- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '66e58cd7-5239-4b71-8bd5-88312583a976', 'description': 'Axial (image 1) and sagittal (image 2) T1WIs show classic cavum septi pellucidi (CSP) and Vergae. The CSP lies in between the frontal horns of the lateral ventricles (open arrows) and the cavum Vergae (CV) (curved arrows) extends posteriorly in between the bodies of the lateral ventricles. The CSP + CV form a "cigar-shaped" CSF-containing structure that lies between the lateral ventricles. Note that on the sagittal view, the cavum occupies the entire space under the corpus callosum, flattening the fornix (arrows). This contrasts with cavum velum interpositum, which usually displaces the fornix anterosuperiorly.', 'history': 'Middle-aged patient with headaches, no neurologic findings.', 'imagePoolId': 'fbd519fb-6ab7-498c-8474-51dbaa25dd2b', 'name': 'With cavum vergae', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '15afabe5-824c-46be-9f9b-58cded699493', 'description': 'Axial T1-weighted MR scans show the classic appearance of cavum septi pellucidi (arrows) with posterior extension into a cavum Vergae (open arrows), seen here as a CSF-signal collection that lies between the bodies of the lateral ventricles.', 'history': 'Asymptomatic patient, incidental finding.', 'imagePoolId': '8d51f828-9478-4e8d-945d-09d8496552d5', 'name': 'CSP, vergae', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'c38954f4-6732-402d-ad20-5bf751eefb80', 'description': 'Sagittal T1-weighted MR scan (#1) shows the classic finding of a cavum septi pellucidi (CSP), seen here as a CSF-filled collection under the corpus callosum (open arrows) that flattens and displaces the thinned fornix (arrows). Axial image (#2) and coronal image (#3) T2WIs show the mass follows CSF in signal intensity. On the coronal scan, the thinned fornices (arrows) are barely visible. Coronal FLAIR scan (#4) shows the rounded contours of the CSP. Fluid in the CSP suppresses completely on FLAIR.', 'history': None, 'imagePoolId': 'ae79975a-0e41-4b33-ad5f-5a209538cd81', 'name': 'CSP cyst, fornix dysplasia', 'teachingPoint': None}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'ce9dcc88-81a6-4e2b-b127-03d2d2cbefe8', 'description': 'Axial NECT scans (#1, 2) show a rounded CSF-density mass in the septum pellucidum that is contiguous with an elongated CSF mass lying in-between the bodies of the lateral ventricles (arrows). This is a cavum septi pellucidi with cavum Vergae. Contrast-enhanced scans (source images from the CTA performed in this patient) show no enhancement (#3-5). \n\nThis case is a slight variant on the typical CSP + Vergae as it is somewhat rounder than usually seen. This is considered a normal variant.', 'history': 'Incidental finding.', 'imagePoolId': 'b7937706-cf34-4252-a8e0-4e96b937e7c9', 'name': 'Very round', 'teachingPoint': None, 'demographics': '30 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}, {'key': '789e84ce-9fde-4452-b27a-21d45423ac32', 'value': 'Anne Kennedy, MD, FSRU, FAIUM'}], 'caseVersionId': 'a5a4b3e1-aaa0-4d47-ae95-c458d17afb1f', 'description': 'Fetal MR (#1) and ultrasound (# 2) show absent septum pellucidum in corpus callosum agenesis. Note "Viking helmet" appearance on the coronal T2WI through the frontal and temporal horns (#1), with widely spaced parallel lateral ventricles and a high-riding third ventricle that is continuous superiorly with the interhemispheric fissure. Colpocephaly (enlarged occipital horns) is common in callosal agenesis and is seen on image #2 (arrow).', 'history': 'Routine prenatal imaging.', 'imagePoolId': '53203fa9-945d-44e0-8723-bab3d8cf207c', 'name': 'Absent CSP, CC agenesis', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'b793cb29-03cb-45cd-a54d-1805f7544044', 'description': 'A classic cavum septi pellucidi (CSP) with cavum Vergae (CV) is seen (arrows). This case is unusual because the CSP and CV are very small.', 'history': 'Incidental finding. Patient being imaged for possible intracranial metastases.', 'imagePoolId': '77f1b3ce-a427-4b7e-b075-8278f3c4e3ce', 'name': 'Very small', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'ede02258-1779-427d-9e2e-f9aeb96dfac9', 'description': 'An unusually large cavum septi pellucidi with cavum Vergae is present. Note mass effect with lateral bowing of the leaves of the septum pellucidum on the coronal scans (#3-4, arrows). Lateral/inferior displacement of the internal cerebral veins on the sagittal image (#1, open arrows) and coronal views (#3-4) (#3, open arrows) is seen. Axial scan (#2) shows lateral bowing of the fornices posteriorly (arrows). Image 4 also shows lateral displacement of the foramen of Monro (curved arrows). No obstructive hydrocephalus is present.', 'history': 'Asymptomatic patient, incidental finding.', 'imagePoolId': '80daa008-e840-43a4-82e4-b9d25b8696f1', 'name': 'Large with mass effect', 'teachingPoint': None, 'demographics': '20 Years old female'}], 'caseType': 'variant', 'name': 'VARIANT'} + + +## Images + + +### Selected Images + +![Coronal graphic with axial insert shows classic cavum septi pellucidi (CSP) with cavum vergae (CV) . Note the finger-like CSF collection between the lateral ventricles.](images/app.statdx.com_image_thumbnail_1fb3ac06-849c-4812-b789-388045765af2_annotated_true_size_900_quality_90_99777374_20251018T164843Z.jpg) +*Coronal graphic with axial insert shows classic cavum septi pellucidi (CSP) with cavum vergae (CV) . Note the finger-like CSF collection between the lateral ventricles.* + +![Coronal graphic with axial insert shows classic cavum septi pellucidi (CSP) with cavum vergae (CV) . Note the finger-like CSF collection between the lateral ventricles.](images/app.statdx.com_image_thumbnail_1fb3ac06-849c-4812-b789-388045765af2_size_174_quality_85_bf1f1833_20251018T164756Z.jpg) +*Coronal graphic with axial insert shows classic cavum septi pellucidi (CSP) with cavum vergae (CV) . Note the finger-like CSF collection between the lateral ventricles.* + +![Coronal T1 C+ SPGR MR shows a classic large CSP between the frontal horns . There is lateral bowing of the leaves of the septum pellucidum .](images/app.statdx.com_image_thumbnail_977baa81-1d68-456f-b6bc-4f9d4aceed84_annotated_true_size_900_quality_90_a6318635_20251018T164843Z.jpg) +*Coronal T1 C+ SPGR MR shows a classic large CSP between the frontal horns . There is lateral bowing of the leaves of the septum pellucidum .* + +![Axial T2 MR shows cavum septi pellucidi between the leaves of the septum pellucidum . Although seen incidentally, some studies have reported that CSP is frequent among athletes with a history of repeated traumatic brain injury (TBI), such as boxers and American professional football players.](images/app.statdx.com_image_thumbnail_a03300a7-6e69-481b-9a3b-1537f7202ca1_annotated_true_size_900_quality_90_9abe7b83_20251018T164843Z.jpg) +*Axial T2 MR shows cavum septi pellucidi between the leaves of the septum pellucidum . Although seen incidentally, some studies have reported that CSP is frequent among athletes with a history of repeated traumatic brain injury (TBI), such as boxers and American professional football players.* + +![Axial FLAIR MR shows a large CSP with CV as a large CSF collection between the leaves of the septum pellucidum continuing directly posteriorly with the CSF collection, splaying the fornices laterally .](images/app.statdx.com_image_thumbnail_9995e3f8-eb09-40bd-8ff6-c7065bb93d40_annotated_true_size_900_quality_90_fbf42288_20251018T164843Z.jpg) +*Axial FLAIR MR shows a large CSP with CV as a large CSF collection between the leaves of the septum pellucidum continuing directly posteriorly with the CSF collection, splaying the fornices laterally .* + + +### Additional Images + +![Axial NECT shows a variant of cavum septi pellucidi. Here, the CSP appears almost round .](images/app.statdx.com_image_thumbnail_4dd3811b-29d3-488c-bdd1-f8a58e630508_annotated_true_size_900_quality_90_d1b1d220_20251018T164843Z.jpg) +*Axial NECT shows a variant of cavum septi pellucidi. Here, the CSP appears almost round .* + +![Sagittal T1 C+ MR shows a large CSP/CV that extends from just behind the corpus callosum genu all the way posteriorly to the splenium. The fornices are not visible, and the internal cerebral vein is flattened .](images/app.statdx.com_image_thumbnail_effbc5cf-b18b-47ed-917e-e16bceb020a6_annotated_true_size_900_quality_90_440f151e_20251018T164843Z.jpg) +*Sagittal T1 C+ MR shows a large CSP/CV that extends from just behind the corpus callosum genu all the way posteriorly to the splenium. The fornices are not visible, and the internal cerebral vein is flattened .* + +![Coronal T1 C+ MR in the same patient shows the large CSP bowing the leaves of the septum pellucidum laterally .](images/app.statdx.com_image_thumbnail_86ce8855-4e01-4fd1-bd27-4ddfdd11bea3_annotated_true_size_900_quality_90_96ab0ca1_20251018T164843Z.jpg) +*Coronal T1 C+ MR in the same patient shows the large CSP bowing the leaves of the septum pellucidum laterally .* + +![Axial T1 MR shows a small cavum septi pellucidi with cavum vergae . Note the finger-like appearance of the CSF collection that lies between the frontal horns and bodies of the lateral ventricle.](images/app.statdx.com_image_thumbnail_80289a63-e206-480d-8e27-773285024d3e_annotated_true_size_900_quality_90_50066962_20251018T164843Z.jpg) +*Axial T1 MR shows a small cavum septi pellucidi with cavum vergae . Note the finger-like appearance of the CSF collection that lies between the frontal horns and bodies of the lateral ventricle.* + +![Axial T2 MR shows a variant of a cavum septi pellucidi with cavum vergae. Note the large CSF collection between leaves of septum pellucidum continuing directly posteriorly with the CSF collection, splaying the fornices laterally .](a9f16e39-5902-4fff-ba93-4c486599b232) +*Axial T2 MR shows a variant of a cavum septi pellucidi with cavum vergae. Note the large CSF collection between leaves of septum pellucidum continuing directly posteriorly with the CSF collection, splaying the fornices laterally .* + +![Axial T2 MR shows cavum septi pellucidi as a CSF collection between the leaves of the septum pellucidum . Although seen incidentally, some studies have reported that CSP is frequent among athletes with a history of repeated TBI, such as boxers and American professional football players.](1fd75692-6b22-492c-916e-20211f0706ea) +*Axial T2 MR shows cavum septi pellucidi as a CSF collection between the leaves of the septum pellucidum . Although seen incidentally, some studies have reported that CSP is frequent among athletes with a history of repeated TBI, such as boxers and American professional football players.* + +![Coronal T1 MR shows a classic large CSP between the frontal horns , bowing the leaves of the septum pellucidum laterally .](6e082f2e-afc6-402e-b55e-9e70eb8461d2) +*Coronal T1 MR shows a classic large CSP between the frontal horns , bowing the leaves of the septum pellucidum laterally .* + diff --git a/out/cavum-velum-interpositum-cvi_849ee468-35c4-46e3-9297-96196109cdb8.md b/out/cavum-velum-interpositum-cvi_849ee468-35c4-46e3-9297-96196109cdb8.md new file mode 100644 index 0000000..eb5e6c2 --- /dev/null +++ b/out/cavum-velum-interpositum-cvi_849ee468-35c4-46e3-9297-96196109cdb8.md @@ -0,0 +1,315 @@ +--- +title: "Cavum Velum Interpositum (CVI)" +docid: "849ee468-35c4-46e3-9297-96196109cdb8" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" + - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850" + value: "Anne G. Osborn, MD, FACR" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Normal Variants" + slug: "normal-variants" + treeNodeId: "bf92256f-cdff-4bcd-8420-d876b9e4031a" + - + name: "Cavum Velum Interpositum (CVI)" + slug: "cavum-velum-interpositum-cvi" + treeNodeId: null +category: "Brain" +documentVersionId: "75312185-3ddd-4300-a4fb-d824b606effc" +imageCount: 12 +lastUpdated: "05/08/20" +pageDescription: "Cavum Velum Interpositum (CVI)" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Normal Variants, Cavum Velum Interpositum (CVI)" +pageTitle: "Cavum Velum Interpositum (CVI) | STATdx" +enhancedTitle: "Cavum Velum Interpositum (CVI)" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Normal Variants" + - "Cavum Velum Interpositum (CVI)" +--- +# KEY FACTS + +- ## Terminology + + + - Cavum velum interpositum (CVI), cyst of velum interpositum (VI) +- ## Imaging + + + - Triangular CSF space + - Between lateral ventricles, over thalami + - Apex points toward foramen of Monro + - Elevates, splays fornices + - Flattens, displaces internal cerebral veins inferiorly + - Size varies from slit-like linear to triangular to round/ovoid CSF collection + - Isodense/isointense with CSF + - Suppresses completely on FLAIR + - Does not restrict on DWI + - Does not enhance + - US shows hypoechoic midline interhemispheric cyst +- ## Top Differential Diagnoses + + + - Normal cistern of velum interpositum + - Cavum septi pellucidi, cavum vergae + - Arachnoid cyst + - Epidermoid cyst +- ## Clinical Issues + + + - Can be found at any age + - Common in infants, rare in adults + - Symptoms + - Usually asymptomatic, found incidentally + - Headache (relationship to cyst unclear) + - Large CVI can obstruct normal CSF flow; treated by endoscopic fenestration +- ## Diagnostic Checklist + + + - CSF-like "cyst" could be epidermoid + - Include FLAIR and DWI to distinguish between CVI, epidermoid cyst + +# TERMINOLOGY + +- ## Abbreviations + + + - Cavum velum interpositum (CVI), cyst of velum interpositum (VI) +- ## Definitions + + + - Cystic dilation of VI + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Triangular-shaped CSF space + - Between lateral ventricles, over thalami + - Apex points toward but does not extend anteriorly beyond foramen of Monro + - Base contiguous with quadrigeminal cistern + - ### Location + + + - Midline between lateral ventricles, below fornices + - Continuous with choroid plexus subependymally + - ### Size + + + - Varies (few mm to several cm) +- ## CT Findings + + + - ### NECT + + + - Triangle of CSF between lateral ventricles + - ### CECT + + + - Does not enhance +- ## MR Findings + + + - ### T1WI + + + - Sagittal: Varies from slit-like linear to round/ovoid + - Elevates fornices + - Flattens, displaces internal cerebral veins inferiorly + - Axial: Triangle of CSF between lateral ventricles + - ### T2WI + + + - Isointense with CSF + - ### FLAIR + + + - Suppresses completely + - ### DWI + + + - Does not restrict +- ## Ultrasonographic Findings + + + - ### Color Doppler + + + - Hypoechoic midline interhemispheric cyst +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR ± contrast + - ### Protocol advice + + + - FLAIR/DWI (distinguish between CVI and epidermoid) + +# DIFFERENTIAL DIAGNOSIS + +- [Normal Cistern of Velum Interpositum](/document/pineal-cyst/eda99e5c-c992-4798-aa35-21437eb505ea) + - Usually small (slit-like or oval) + - Does not elevate fornices or depress internal cerebral veins +- ## Cavum Septi Pellucidi, Vergae + + + - CSP + CV elongated, finger-like CSF space +- [Arachnoid Cyst](/document/arachnoid-cyst/7d63f0d3-6999-4d8c-a41d-953f738c43a6) + - Lined with arachnoid (may be indistinguishable) +- [Epidermoid Cyst](/document/epidermoid-cyst/704c5ddf-e1f7-4a5d-a1b8-5b0e603170d9) + - Lobulated, insinuating mass + - Does not suppress with FLAIR; DWI shows restriction + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Pia infolds along transverse fissure, forms CSF cistern (VI) + - Cystic dilatation of VI may occur (precise etiology unknown) + - ### Associated abnormalities + + + - Usually none (large CVIs may cause hydrocephalus) +- ## Gross Pathologic & Surgical Features + + + - Normal cistern of VI is small to inapparent at autopsy + - Pial-lined CSF-filled space +- ## Microscopic Features + + + - Occasionally cysts of midline CSF spaces contain glial cells, scattered neurons + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Usually asymptomatic, found incidentally + - Headache (relationship to cyst unclear) +- ## Demographics + + + - ### Age + + + - Can be found at any age + - Common in infants, rare in adults + - ### Gender + + + - M = F +- ## Treatment + + + - Usually none + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - CSF-like "cyst" could be epidermoid + - Use DWI, FLAIR to differentiate from other cysts + + 806ab3b4-9c9a-4887-a52d-d5809cd5a417 + +## References + +# Selected References + +1. [Krejčí T et al: Symptomatic cysts of the cavum septi pellucidi, cavum vergae and cavum veli interpositi: A retrospective duocentric study of 10 patients. Clin Neurol Neurosurg. 185:105494, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31472394%5Bpmid%5D) +1. [Akinola RA et al: Caval variations in neurologically diseased patients. Acta Radiol Short Rep. 3(5):2047981614530288, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25298867%5Bpmid%5D) +1. [Tong CK et al: Endoscopic fenestration of cavum velum interpositum cysts: a case study of two symptomatic patients. Childs Nerv Syst. 28(8):1261-4, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22543434%5Bpmid%5D) +1. [Tubbs RS et al: Cavum velum interpositum, cavum septum pellucidum, and cavum vergae: a review. Childs Nerv Syst. 27(11):1927-30, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21687999%5Bpmid%5D) +1. [Tubbs RS et al: The velum interpositum revisited and redefined. Surg Radiol Anat. 30(2):131-5, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18094919%5Bpmid%5D) +1. [Osborn AG et al: Intracranial cysts: radiologic-pathologic correlation and imaging approach. Radiology. 239(3):650-64, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16714456%5Bpmid%5D) +1. [Eisenberg VH et al: Prenatal diagnosis of cavum velum interpositum cysts: significance and outcome. Prenat Diagn. 23(10):779-83, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14558018%5Bpmid%5D) +1. [Vergani P et al: Ultrasonographic differential diagnosis of fetal intracranial interhemispheric cysts. Am J Obstet Gynecol. 180(2 Pt 1):423-8, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=9988813%5Bpmid%5D) +1. [Chen CY et al: Sonographic characteristics of the cavum velum interpositum. AJNR Am J Neuroradiol. 19(9):1631-5, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9802483%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal graphic with axial insert shows a cavum velum interpositum (CVI). Note the elevation and splaying of the fornices . Also noted is the inferior displacement of the internal cerebral veins and 3rd ventricle .](images/app.statdx.com_image_thumbnail_2df127e7-04ef-413c-b33e-46703bf26fc3_annotated_true_size_900_quality_90_43c5eb31_20251018T164844Z.jpg) +*Sagittal graphic with axial insert shows a cavum velum interpositum (CVI). Note the elevation and splaying of the fornices . Also noted is the inferior displacement of the internal cerebral veins and 3rd ventricle .* + +![Sagittal graphic with axial insert shows a cavum velum interpositum (CVI). Note the elevation and splaying of the fornices . Also noted is the inferior displacement of the internal cerebral veins and 3rd ventricle .](images/app.statdx.com_image_thumbnail_2df127e7-04ef-413c-b33e-46703bf26fc3_size_174_quality_85_6f6a77c4_20251018T164756Z.jpg) +*Sagittal graphic with axial insert shows a cavum velum interpositum (CVI). Note the elevation and splaying of the fornices . Also noted is the inferior displacement of the internal cerebral veins and 3rd ventricle .* + +![Sagittal T1 MR shows a classic CVI as a CSF-like enlargement that elevates the fornix and flattens and displaces the internal cerebral vein inferiorly. These are usually asymptomatic; however, large ones can cause CSF obstruction and can be treated by fenestration.](images/app.statdx.com_image_thumbnail_bc8c67a9-f3e5-4673-a4f6-3febba3eccd9_annotated_true_size_900_quality_90_39cc402c_20251018T164845Z.jpg) +*Sagittal T1 MR shows a classic CVI as a CSF-like enlargement that elevates the fornix and flattens and displaces the internal cerebral vein inferiorly. These are usually asymptomatic; however, large ones can cause CSF obstruction and can be treated by fenestration.* + +![Axial T2 MR in a 37-year-old man with headaches shows a triangular-shaped CSF collection between the lateral ventricles, spreading the fornices laterally .](images/app.statdx.com_image_thumbnail_e9fcc6c7-e349-4a26-8cd1-1585a0da8e2e_annotated_true_size_900_quality_90_0de56902_20251018T164845Z.jpg) +*Axial T2 MR in a 37-year-old man with headaches shows a triangular-shaped CSF collection between the lateral ventricles, spreading the fornices laterally .* + +![Axial FLAIR MR in the same patient shows complete suppression of the CSF signal within the cyst similar to the lateral ventricles. Findings are consistent with a classic cavum velum interpositum. FLAIR and DWI distinguish between cavum velum interpositum and an epidermoid cyst.](images/app.statdx.com_image_thumbnail_a2c60186-2347-4c48-b827-1bae6f61ff3d_annotated_true_size_900_quality_90_f9c0e3e7_20251018T164845Z.jpg) +*Axial FLAIR MR in the same patient shows complete suppression of the CSF signal within the cyst similar to the lateral ventricles. Findings are consistent with a classic cavum velum interpositum. FLAIR and DWI distinguish between cavum velum interpositum and an epidermoid cyst.* + + +### Additional Images + +![Axial T2 MR shows a cavum septi pellucidi (CSP) along with a very small cavum velum interpositum . Note that these 2 unrelated lesions do not communicate with each other. It is common to see a CSP with a cavum vergae; it is rare to see a CSP and a CVI in the same patient.](images/app.statdx.com_image_thumbnail_8f807265-867d-45c4-a0e0-d4b5ed77ec48_annotated_true_size_900_quality_90_14754121_20251018T164844Z.jpg) +*Axial T2 MR shows a cavum septi pellucidi (CSP) along with a very small cavum velum interpositum . Note that these 2 unrelated lesions do not communicate with each other. It is common to see a CSP with a cavum vergae; it is rare to see a CSP and a CVI in the same patient.* + +![Coronal T1 C+ MR shows classic cavum velum interpositum that spreads the fornices apart.](images/app.statdx.com_image_thumbnail_52a1d3d4-3d12-4d0c-99df-2eea486bbc3b_annotated_true_size_900_quality_90_8a9e912a_20251018T164845Z.jpg) +*Coronal T1 C+ MR shows classic cavum velum interpositum that spreads the fornices apart.* + +![Axial T2 MR shows a small, triangular-shaped CSF space. Note the cavum velum interpositum interposed between the fornices and lateral ventricles. The CVI ends at the foramen of Monro.](images/app.statdx.com_image_thumbnail_1d26fd47-cf3a-49e9-b019-149ab6aa9d95_annotated_true_size_900_quality_90_3b864288_20251018T164844Z.jpg) +*Axial T2 MR shows a small, triangular-shaped CSF space. Note the cavum velum interpositum interposed between the fornices and lateral ventricles. The CVI ends at the foramen of Monro.* + +![Axial T1 MR shows a very large cavum velum interpositum. Note the splaying of fornices and anterior displacement of the septum pellucidum . Mild enlargement of the lateral ventricles is seen.](images/app.statdx.com_image_thumbnail_21237160-fb5d-46fc-8708-36027df8182c_annotated_true_size_900_quality_90_227e0506_20251018T164845Z.jpg) +*Axial T1 MR shows a very large cavum velum interpositum. Note the splaying of fornices and anterior displacement of the septum pellucidum . Mild enlargement of the lateral ventricles is seen.* + +![Sagittal T1 MR in the same patient shows anterior/superior displacement of the fornix and inferior displacement of the 3rd ventricle . The corpus callosum is elevated and thinned.](570ac479-0220-4e7c-95cf-b05fa1d08c4f) +*Sagittal T1 MR in the same patient shows anterior/superior displacement of the fornix and inferior displacement of the 3rd ventricle . The corpus callosum is elevated and thinned.* + +![Sagittal T1 MR in a 40-year-old woman with headaches shows CSF-like enlargement of the velum interpositum that elevates the fornix and flattens and displaces the internal cerebral vein inferiorly . This large CVI is probably unrelated to the patient's symptoms.](15050721-daf0-49a6-a3f3-45a4a2b17b85) +*Sagittal T1 MR in a 40-year-old woman with headaches shows CSF-like enlargement of the velum interpositum that elevates the fornix and flattens and displaces the internal cerebral vein inferiorly . This large CVI is probably unrelated to the patient's symptoms.* + +![Axial T2 MR in a 46-year-old woman with headaches shows a classic CVI with a triangular-shaped CSF collection , spreading the fornices laterally . The posterior location between the lateral ventricles is typical.](368270d4-dee5-4f8e-8b6e-47b96373ed43) +*Axial T2 MR in a 46-year-old woman with headaches shows a classic CVI with a triangular-shaped CSF collection , spreading the fornices laterally . The posterior location between the lateral ventricles is typical.* + +![Sagittal T1 MR shows a variant CVI that elevates the fornix , flattens the internal cerebral vein , and extends into the quadrigeminal and suprasellar cisterns . This case probably represents an arachnoid cyst of the cavum velum interpositum.](b6d129c7-1434-422d-91d7-83b43bc72494) +*Sagittal T1 MR shows a variant CVI that elevates the fornix , flattens the internal cerebral vein , and extends into the quadrigeminal and suprasellar cisterns . This case probably represents an arachnoid cyst of the cavum velum interpositum.* + diff --git a/out/cidp_12e4033c-edc8-46ff-8081-3acc433cda78.md b/out/cidp_12e4033c-edc8-46ff-8081-3acc433cda78.md deleted file mode 100644 index fa7877e..0000000 --- a/out/cidp_12e4033c-edc8-46ff-8081-3acc433cda78.md +++ /dev/null @@ -1,395 +0,0 @@ ---- -title: "CIDP" -docid: "12e4033c-edc8-46ff-8081-3acc433cda78" -authors: - - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607" - value: "Surjith Vattoth, MD, FRCR" -breadcrumbs: - - - name: "Brain" - slug: "brain" - treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" - - - name: "Diagnosis" - slug: "diagnosis" - treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" - - - name: "Pathology-Based Diagnoses" - slug: "pathology-based-diagnoses" - treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" - - - name: "Infectious, Inflammatory, and Demyelinating Disease" - slug: "infectious-inflammatory-and-demyel-" - treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607" - - - name: "Inflammatory and Demyelinating Disease" - slug: "inflammatory-and-demyelinating-dis-" - treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc" - - - name: "CIDP" - slug: "cidp" - treeNodeId: null -category: "Brain" -documentVersionId: "96729e13-6c4b-4fd3-be3e-4e1a940566fd" -imageCount: 12 -lastUpdated: "06/08/20" -pageDescription: "CIDP" -pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, CIDP" -pageTitle: "CIDP | STATdx" -enhancedTitle: "CIDP" -type: "DX" -references: true -breadcrumbs: - - "Brain" - - "Diagnosis" - - "Pathology-Based Diagnoses" - - "Infectious, Inflammatory, and Demyelinating Disease" - - "Inflammatory and Demyelinating Disease" - - "CIDP" ---- -# KEY FACTS - -- ## Terminology - - - - Clinically heterogeneous, grossly symmetric, sensory & motor neuropathy evolving as monophasic, relapsing, or progressive disorder - - Develops over > 8 weeks -- ## Imaging - - - - Sagittal FLAIR may reveal hyperintense brain lesions similar to multiple sclerosis - - Enlargement & abnormal T2 hyperintensity of nerve roots, plexi, or peripheral nerves - - ↑ nerve root diameter, cross-sectional area (CSA), & volume - - Spinal nerve roots & peripheral nerves (extraforaminal > intradural) - - Lumbar > cervical, brachial plexus, thoracic/intercostal > cranial nerve - - Fair degree of CSA correlation between high-resonance nerve ultrasound (HRUS) & MR neurography (MRN) -- ## Top Differential Diagnoses - - - - Guillain-Barré (AIDP) - - Inherited demyelinating neuropathy (Charcot-Marie-Tooth) - - Neurofibromatosis type 1, schwannomatosis -- ## Pathology - - - - Autoimmune disease of cellular & humoral immunity - - Hallmarks of CIDP: Enlarged nerves with onion bulb formations, demyelination -- ## Clinical Issues - - - - Usually **clinical**diagnosis based on progressive weakness/sensory loss & response to steroids - - Typical: Symmetric proximal & distal weakness, sensory loss - - Abnormal EMG/NCV: Key electrophysiologic features → nerve conduction block, slowed conduction velocities suggestive of demyelination - - Diagnosis relies primarily on clinical, electrophysiologic examination supplemented by nerve biopsy - -# TERMINOLOGY - -- ## Abbreviations - - - - Chronic inflammatory demyelinating polyneuropathy (CIDP) -- ## Synonyms - - - - Chronic inflammatory demyelinating polyradiculoneuropathy -- ## Definitions - - - - Chronic acquired, immune-mediated demyelinating neuropathy characterized by relapsing or progressive muscle weakness ± sensory loss - -# IMAGING - -- ## General Features - - - - ### Best diagnostic clue - - - - Enlargement & abnormal T2 hyperintensity of nerve roots, plexi, or peripheral nerves - - Spinal nerve roots & peripheral nerves (extraforaminal > intradural) - - Lumbar > cervical, brachial plexus, thoracic/intercostal > cranial nerves (CNs) - - ### Size - - - - Nerve size varies; small → very large - - Mean diameter of spinal nerve roots in CIDP: Cervical 6-6.8 mm; lumbosacral 7.3-10.4 mm - - 5-mm best cut-off value of C6, C7, C8 nerve root diameters to distinguish CIDP patients from controls - - CIDP nerves larger volumes, which positively correlate with disease duration - - Recent MR neurography (MRN) of L3-S1 nerve roots of lumbosacral plexus using 3D multiple echo recalled gradient-echo (3D MERGE) sequence showed - - ↑ mean cross-sectional area (CSA): 28.04 ± 8.55 mm² in CIDP (14.91 ± 2.36 square mm² in normal); optimal cut-off value 19.20 mm² - - ### Morphology - - - - Focal or diffuse fusiform enlargement of cauda equina, nerve roots/plexi, & peripheral nerves -- ## CT Findings - - - - ### NECT - - - - Isodense nerve enlargement - - ### CECT - - - - Mild to moderate nerve enhancement -- ## MR Findings - - - - ### T2WI - - - - Enlargement, abnormal hyperintensity of intradural & extradural spinal nerves/branches - - ### FLAIR - - - - Sagittal FLAIR may reveal hyperintense brain lesions similar to multiple sclerosis (MS) - - ### DWI - - - - Diffusion-weighted MRN - - DTI: ↓ nerve fractional anisotropy (FA) (mean 0.42 ± 0.08) in CIDP compared to healthy controls (0.52 ± 0.04) - - ↓ FA due to ↑ radial diffusivity (RD); axial diffusivity (AD) not significant - - FA & RD correlate strongly with electrophysiological markers of demyelination - - ### T1WI C+ - - - - Mild to moderate nerve enhancement -- ## Ultrasonographic Findings - - - - ### Grayscale ultrasound - - - - Hypoechoic, hypertrophic nerves - - Fair degree of CSA correlation in high resonance nerve US (HRUS) & MRN of cervical plexus, & peripheral nerves in CIDP - - CSA in HRUS correlate well with markers of nerve integrity, such as ↓ FA in DTI & with ↑ T2 signal - - HRUS-CSA of interscalene brachial plexus correlated significantly with MRN-CSA & T2 signal of L5 & S1 lumbar plexus roots -- ## Imaging Recommendations - - - - ### Best imaging tool - - - - MRN, T2WI, enhanced coronal & axial T1WI sequences with fat suppression best delineate nerve lesions - - Brain MR to detect subclinical CNS demyelination - -# DIFFERENTIAL DIAGNOSIS - -- ## Conditions Recently Proposed to be Included Under CIDP Syndrome - - - - Antimyelin associated glycoprotein (MAG) neuropathy - - Chronic neuropathies associated with IgG4 antibodies against paranodal/nodal proteins; chronic immune sensory polyradiculopathy (CISP); multifocal motor neuropathy -- [Guillain-Barré (Acute Inflammatory Demyelinating Polyneuropathy)](/document/guillain-barr-spectrum-disorders/c1f52a65-920e-4e28-8a75-07dfa208f290) - - Pial, nerve root enhancement similar to CIDP - - Differs from CIDP in onset duration, clinical course - - Acute onset of ascending paralysis with relative sensory preservation -- [Hereditary Motor and Sensory Neuropathy](/document/hypertrophic-neuropathy/e246f4d1-0262-4ca7-b8e1-6f2a4bd67c06) - - Also called Charcot-Marie-Tooth (CMT) disease - - CMT1, CMT 3 (Dejerine-Sottas disease) CMT4, CMTX1 - - Genetic testing, clinical phenotype distinguish from CIDP -- [Neurofibromatosis Type 1](/document/neurofibromatosis-type-1-spine/89236653-e750-4fa7-b2b1-0a3c4ed31a87) - - Diffuse nerve root enlargement, enhancement - - Genetic testing & distinctive clinical stigmata to distinguish -- ## Lateral Meningocele - - - - CSF density/signal intensity (not solid) ± foraminal enlargement, dural ectasia - - Usually coexisting NF1 or connective tissue disorder (Marfan syndrome) -- ## Schwannomatosis - - - - Multiple schwannomas of peripheral nerves & CNs [nonvestibular schwannomas (nVS)] - - However, unilateral VS described with germline mutations of Schwannomatosis in SMARCB1 & LZTR1 -- ## Other Clinical Differential Diagnosis - - - - Diabetic neuropathy, amyloid neuropathy due to TTR mutations, vasculitic neuropathy, POEMS syndrome - -# PATHOLOGY - -- ## General Features - - - - ### Etiology - - - - Exact pathogenesis of CIDP unclear; involves both cellular & humoral immune factors - - Polyneuropathies co-occurring with MS: Underdiagnosed; extra disability burden; includes CIDP - - 1/3 of MS-CIDP cases with serum testing show IgG4 autoantibodies to neurofascin-155 -- ## Gross Pathologic & Surgical Features - - - - Extensive fusiform nerve enlargement ± gross onion bulb formations -- ## Microscopic Features - - - - Large nerve, onion bulb formations, demyelination - - Macrophage, T-cell infiltration → perivascular inflammatory infiltrates, nerve demyelination & remyelination - - Onion bulb formation: Excessive Schwann cell process proliferation → repetitive demyelination/remyelination - -# CLINICAL ISSUES - -- ## Presentation - - - - ### Most common signs/symptoms - - - - Mixed sensorimotor neuropathy; typical form: Symmetric proximal & distal weakness, sensory loss - - Rarer atypical form (Lewis-Sumner syndrome) - - Predominantly uni- or multifocal as well as distal - - CNs are occasionally affected, with particular tropism for CNVII, but ophthalmoplegia or bulbar weakness can be present - - ### Other signs/symptoms - - - - Chronic progressive: Progressively deteriorate until treatment is given -- ## Demographics - - - - ### Sex - - - - M = F -- ## Natural History & Prognosis - - - - Average disease duration: 7.5 years -- ## Treatment - - - - European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy; immunomodulation or immunosuppression therapy - -# DIAGNOSTIC CHECKLIST - -- ## Consider - - - - Consider CIDP in differential of nerve root/peripheral nerve enlargement -- ## Image Interpretation Pearls - - - - MR findings imperfectly correlate with clinical disease activity/severity, laboratory findings - - 0f953548-b230-4137-9147-51d6ed147c6c - -## References - -# Selected References - -1. 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Intern Med. 47(23):2019-24, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19043253%5Bpmid%5D) -1. [Tsuchiya K et al: Demonstration of spinal cord and nerve root abnormalities by diffusion neurography. J Comput Assist Tomogr. 32(2):286-90, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18379319%5Bpmid%5D) -1. [Said G: Chronic inflammatory demyelinating polyneuropathy. Neuromuscul Disord. 16(5):293-303, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16631367%5Bpmid%5D) -1. [Köller H et al: Chronic inflammatory demyelinating polyneuropathy--update on pathogenesis, diagnostic criteria and therapy. Curr Opin Neurol. 18(3):273-8, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15891411%5Bpmid%5D) -1. [Matsuoka N et al: Detection of cervical nerve root hypertrophy by ultrasonography in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Sci. 219(1-2):15-21, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15050432%5Bpmid%5D) -1. 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[Midroni G et al: MRI of the cauda equina in CIDP: clinical correlations. J Neurol Sci. 170(1):36-44, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10540034%5Bpmid%5D) -1. [Mizuno K et al: Chronic inflammatory demyelinating polyradiculoneuropathy with diffuse and massive peripheral nerve hypertrophy: distinctive clinical and magnetic resonance imaging features. Muscle Nerve. 21(6):805-8, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9585338%5Bpmid%5D) -1. [Kuwabara S et al: Magnetic resonance imaging at the demyelinative foci in chronic inflammatory demyelinating polyneuropathy. Neurology. 48(4):874-7, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9109870%5Bpmid%5D) -1. [Simmons Z et al: Chronic inflammatory demyelinating polyradiculoneuropathy in children: I. Presentation, electrodiagnostic studies, and initial clinical course, with comparison to adults. 20(12):1569-75, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9390670%5Bpmid%5D) -1. [Van Es HW et al: Magnetic resonance imaging of the brachial plexus in patients with multifocal motor neuropathy. Neurology. 48(5):1218-24, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9153446%5Bpmid%5D) - - -## Images - - -### Selected Images - -![Sagittal T1 C+ MR of the cervical spine shows marked hypertrophy and enhancement of all exiting cervical nerve roots . 5 mm is considered an adequate cut-off value of cervical spinal nerve root diameter, discriminating CIDP from controls. Mean diameter of spinal nerve roots in CIDP: Cervical 6-6.8 mm; lumbosacral 7.3-10.4 mm.](images/app.statdx.com_image_thumbnail_e0d1598d-4a92-4d78-9124-87f27a196230_annotated_true_size_900_quality_90_8ebe0b28_20251018T122453Z.jpg) -*Sagittal T1 C+ MR of the cervical spine shows marked hypertrophy and enhancement of all exiting cervical nerve roots . 5 mm is considered an adequate cut-off value of cervical spinal nerve root diameter, discriminating CIDP from controls. Mean diameter of spinal nerve roots in CIDP: Cervical 6-6.8 mm; lumbosacral 7.3-10.4 mm.* - -![Sagittal T1 C+ MR of the cervical spine shows marked hypertrophy and enhancement of all exiting cervical nerve roots . 5 mm is considered an adequate cut-off value of cervical spinal nerve root diameter, discriminating CIDP from controls. Mean diameter of spinal nerve roots in CIDP: Cervical 6-6.8 mm; lumbosacral 7.3-10.4 mm.](images/app.statdx.com_image_thumbnail_e0d1598d-4a92-4d78-9124-87f27a196230_size_168_quality_85_41f53f54_20251018T095234Z.jpg) -*Sagittal T1 C+ MR of the cervical spine shows marked hypertrophy and enhancement of all exiting cervical nerve roots . 5 mm is considered an adequate cut-off value of cervical spinal nerve root diameter, discriminating CIDP from controls. Mean diameter of spinal nerve roots in CIDP: Cervical 6-6.8 mm; lumbosacral 7.3-10.4 mm.* - -![Sagittal T1 C+ MR of the cervical spine shows marked hypertrophy and enhancement of all exiting cervical nerve roots . 5 mm is considered an adequate cut-off value of cervical spinal nerve root diameter, discriminating CIDP from controls. Mean diameter of spinal nerve roots in CIDP: Cervical 6-6.8 mm; lumbosacral 7.3-10.4 mm.](images/app.statdx.com_image_thumbnail_e0d1598d-4a92-4d78-9124-87f27a196230_size_174_quality_85_90acc783_20251018T122441Z.jpg) -*Sagittal T1 C+ MR of the cervical spine shows marked hypertrophy and enhancement of all exiting cervical nerve roots . 5 mm is considered an adequate cut-off value of cervical spinal nerve root diameter, discriminating CIDP from controls. Mean diameter of spinal nerve roots in CIDP: Cervical 6-6.8 mm; lumbosacral 7.3-10.4 mm.* - -![Sagittal T2WI MR reveals enlargement and T2 hyperintensity of exiting extradural lumbosacral nerves . High signal of CSF should be excluded while measuring nerve root size/area in T2 MR.](images/app.statdx.com_image_thumbnail_c27b3469-6c6e-4d3c-8cc8-a93671c5bf09_annotated_true_size_900_quality_90_ac6a09e4_20251018T122453Z.jpg) -*Sagittal T2WI MR reveals enlargement and T2 hyperintensity of exiting extradural lumbosacral nerves . High signal of CSF should be excluded while measuring nerve root size/area in T2 MR.* - -![Sagittal T2WI MR reveals enlargement and T2 hyperintensity of exiting extradural lumbosacral nerves . High signal of CSF should be excluded while measuring nerve root size/area in T2 MR.](images/app.statdx.com_image_thumbnail_c27b3469-6c6e-4d3c-8cc8-a93671c5bf09_size_168_quality_85_8455ce81_20251018T095234Z.jpg) -*Sagittal T2WI MR reveals enlargement and T2 hyperintensity of exiting extradural lumbosacral nerves . High signal of CSF should be excluded while measuring nerve root size/area in T2 MR.* - -![Axial T1WI C+ MR depicts enlargement and abnormal enhancement of exiting extradural lumbosacral nerves . Blood-nerve barrier breakdown can cause contrast enhancement. Axon loss associated with demyelination is the most important factor of disability and resistance to treatment. Root hypertrophy also may cause stenosis symptoms.](images/app.statdx.com_image_thumbnail_f40f3c68-4a6c-4e61-a1d0-818ea614c071_annotated_true_size_900_quality_90_d99899db_20251018T122453Z.jpg) -*Axial T1WI C+ MR depicts enlargement and abnormal enhancement of exiting extradural lumbosacral nerves . Blood-nerve barrier breakdown can cause contrast enhancement. Axon loss associated with demyelination is the most important factor of disability and resistance to treatment. Root hypertrophy also may cause stenosis symptoms.* - -![Axial T1WI C+ MR depicts enlargement and abnormal enhancement of exiting extradural lumbosacral nerves . Blood-nerve barrier breakdown can cause contrast enhancement. Axon loss associated with demyelination is the most important factor of disability and resistance to treatment. Root hypertrophy also may cause stenosis symptoms.](images/app.statdx.com_image_thumbnail_f40f3c68-4a6c-4e61-a1d0-818ea614c071_size_168_quality_85_b4a51382_20251018T095234Z.jpg) -*Axial T1WI C+ MR depicts enlargement and abnormal enhancement of exiting extradural lumbosacral nerves . Blood-nerve barrier breakdown can cause contrast enhancement. Axon loss associated with demyelination is the most important factor of disability and resistance to treatment. Root hypertrophy also may cause stenosis symptoms.* - -![Sagittal FLAIR MR demonstrates periventricular ovoid hyperintensities in a typical case of marked fusiform CIDP nerve enlargement with brain demyelination.](images/app.statdx.com_image_thumbnail_8ef8ec72-8984-4f90-8380-953114da6604_annotated_true_size_900_quality_90_c59fe9eb_20251018T122453Z.jpg) -*Sagittal FLAIR MR demonstrates periventricular ovoid hyperintensities in a typical case of marked fusiform CIDP nerve enlargement with brain demyelination.* - -![Sagittal FLAIR MR demonstrates periventricular ovoid hyperintensities in a typical case of marked fusiform CIDP nerve enlargement with brain demyelination.](images/app.statdx.com_image_thumbnail_8ef8ec72-8984-4f90-8380-953114da6604_size_168_quality_85_e847b484_20251018T095234Z.jpg) -*Sagittal FLAIR MR demonstrates periventricular ovoid hyperintensities in a typical case of marked fusiform CIDP nerve enlargement with brain demyelination.* - - -### Additional Images - -![Axial T1WI C+ MR shows thickening and enhancement of ventral and dorsal cauda equina nerve roots .](images/app.statdx.com_image_thumbnail_385d96c2-5ef1-466a-bbf7-bcfbf8fb9433_annotated_true_size_900_quality_90_b76815ce_20251018T122453Z.jpg) -*Axial T1WI C+ MR shows thickening and enhancement of ventral and dorsal cauda equina nerve roots .* - -![Axial T1WI C+ MR shows thickening and enhancement of ventral and dorsal cauda equina nerve roots .](images/app.statdx.com_image_thumbnail_385d96c2-5ef1-466a-bbf7-bcfbf8fb9433_size_168_quality_85_3828ef00_20251018T095234Z.jpg) -*Axial T1WI C+ MR shows thickening and enhancement of ventral and dorsal cauda equina nerve roots .* - -![Sagittal T2WI MR demonstrates diffuse thickening of the intradural cauda equina nerve roots.](images/app.statdx.com_image_thumbnail_e85adcc5-c5d2-4c04-b676-83773765bd8e_annotated_true_size_900_quality_90_6f96bf2d_20251018T122453Z.jpg) -*Sagittal T2WI MR demonstrates diffuse thickening of the intradural cauda equina nerve roots.* - -![Sagittal T2WI MR demonstrates diffuse thickening of the intradural cauda equina nerve roots.](images/app.statdx.com_image_thumbnail_e85adcc5-c5d2-4c04-b676-83773765bd8e_size_168_quality_85_0a98e931_20251018T095234Z.jpg) -*Sagittal T2WI MR demonstrates diffuse thickening of the intradural cauda equina nerve roots.* - -![Sagittal FLAIR MR of the brain in a CIDP patient shows a typical paraventricular demyelinating lesion similar to those seen in multiple sclerosis patients.](images/app.statdx.com_image_thumbnail_4161f150-8dc2-4c83-94b9-4ee9d01c70f7_annotated_true_size_900_quality_90_b058499e_20251018T122453Z.jpg) -*Sagittal FLAIR MR of the brain in a CIDP patient shows a typical paraventricular demyelinating lesion similar to those seen in multiple sclerosis patients.* - -![Sagittal FLAIR MR of the brain in a CIDP patient shows a typical paraventricular demyelinating lesion similar to those seen in multiple sclerosis patients.](images/app.statdx.com_image_thumbnail_4161f150-8dc2-4c83-94b9-4ee9d01c70f7_size_168_quality_85_efb486ac_20251018T095234Z.jpg) -*Sagittal FLAIR MR of the brain in a CIDP patient shows a typical paraventricular demyelinating lesion similar to those seen in multiple sclerosis patients.* - -![Sagittal T2WI MR depicts enlarged lumbar nerve roots extending into extraforaminal ventral primary rami .](images/app.statdx.com_image_thumbnail_4683fb7b-747f-4882-8f72-0a9b82b28723_annotated_true_size_900_quality_90_af319ef1_20251018T122453Z.jpg) -*Sagittal T2WI MR depicts enlarged lumbar nerve roots extending into extraforaminal ventral primary rami .* - -![Sagittal T2WI MR depicts enlarged lumbar nerve roots extending into extraforaminal ventral primary rami .](images/app.statdx.com_image_thumbnail_4683fb7b-747f-4882-8f72-0a9b82b28723_size_168_quality_85_b2a08acd_20251018T095234Z.jpg) -*Sagittal T2WI MR depicts enlarged lumbar nerve roots extending into extraforaminal ventral primary rami .* - -![Axial T2WI MR shows diffuse thickening and hyperintensity of thoracic nerve roots and paraspinal intercostal nerves.](images/app.statdx.com_image_thumbnail_7443f593-4ded-4c77-b1e2-b2d61ecea64a_size_168_quality_85_52da947b_20251018T095234Z.jpg) -*Axial T2WI MR shows diffuse thickening and hyperintensity of thoracic nerve roots and paraspinal intercostal nerves.* - -![Axial T2WI MR reveals bilateral symmetric enlargement, hyperintensity of cervical nerve roots and brachial plexus .](images/app.statdx.com_image_thumbnail_d53c481c-3aa4-4771-8aa3-b7081202b269_size_168_quality_85_93e086b6_20251018T095234Z.jpg) -*Axial T2WI MR reveals bilateral symmetric enlargement, hyperintensity of cervical nerve roots and brachial plexus .* - -![Sagittal T1WI C+ MR demonstrates diffuse pial thickening and enhancement extending into the cauda equina nerve roots. Clinical course distinguished from Guillain-Barré (AIDP).](images/app.statdx.com_image_thumbnail_f2bfe031-78d4-4f16-baa0-c95a54f6e565_size_168_quality_85_0fab86e2_20251018T095234Z.jpg) -*Sagittal T1WI C+ MR demonstrates diffuse pial thickening and enhancement extending into the cauda equina nerve roots. Clinical course distinguished from Guillain-Barré (AIDP).* - -![Axial T2WI MR shows marked enlargement of the lumbar/sacral nerve roots and lumbosacral trunk .](images/app.statdx.com_image_thumbnail_7776240a-5af6-404a-bc66-83b9ee89150e_size_168_quality_85_e3313250_20251018T095234Z.jpg) -*Axial T2WI MR shows marked enlargement of the lumbar/sacral nerve roots and lumbosacral trunk .* - diff --git a/out/clippers_ba394f3b-bbff-4128-90b5-3e1c07564c5f.md b/out/clippers_ba394f3b-bbff-4128-90b5-3e1c07564c5f.md deleted file mode 100644 index bd2043a..0000000 --- a/out/clippers_ba394f3b-bbff-4128-90b5-3e1c07564c5f.md +++ /dev/null @@ -1,346 +0,0 @@ ---- -title: "CLIPPERS" -docid: "ba394f3b-bbff-4128-90b5-3e1c07564c5f" -authors: - - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850" - value: "Anne G. Osborn, MD, FACR" -breadcrumbs: - - - name: "Brain" - slug: "brain" - treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" - - - name: "Diagnosis" - slug: "diagnosis" - treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" - - - name: "Pathology-Based Diagnoses" - slug: "pathology-based-diagnoses" - treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" - - - name: "Infectious, Inflammatory, and Demyelinating Disease" - slug: "infectious-inflammatory-and-demyel-" - treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607" - - - name: "Inflammatory and Demyelinating Disease" - slug: "inflammatory-and-demyelinating-dis-" - treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc" - - - name: "CLIPPERS" - slug: "clippers" - treeNodeId: null -category: "Brain" -documentVersionId: "259b8c88-93cc-45d6-93d8-75d279e9ead2" -imageCount: 12 -lastUpdated: "08/05/20" -pageDescription: "CLIPPERS" -pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, CLIPPERS" -pageTitle: "CLIPPERS | STATdx" -enhancedTitle: "CLIPPERS" -type: "DX" -references: true -breadcrumbs: - - "Brain" - - "Diagnosis" - - "Pathology-Based Diagnoses" - - "Infectious, Inflammatory, and Demyelinating Disease" - - "Inflammatory and Demyelinating Disease" - - "CLIPPERS" ---- -# KEY FACTS - -- ## Terminology - - - - **C**hronic **l**ymphocytic **i**nflammation with**p**ontine **p**erivascular **e**nhancement **r**esponsive to **s**teroids (CLIPPERS) -- ## Imaging - - - - Location predominantly pons/cerebellum **but****may extend - - Laterally into cerebellar peduncles or hemispheres - - Rostrally into midbrain - - Caudally into medulla, spinal cord - - ≈ 60% have CNS lesions **outside**pontocerebellar region - - Basal ganglia, hemispheric white matter, cortex - - MR - - Punctate/curvilinear enhancing foci "peppering" pons - - May exhibit subtle radiating pattern - - No ring or patchy enhancement - - Patchy/"speckled" punctate hyperintensities on T2/FLAIR - - Does not significantly exceed areas of T1 C+ enhancement - - Caution: May begin as isolated, enhancing mass in pons/cerebellar peduncle before exhibiting typical pattern of multifocal punctate pontine lesions -- ## Top Differential Diagnoses - - - - Angiocentric (intravascular) lymphoma - - Neurosarcoidosis - - Demyelinating disease (multiple sclerosis, MOG spectrum) - - Vasculitis (primary, secondary, Behçet) - - Lymphomatoid granulomatosis - - Histiocytosis (e.g., hemophagocytic lymphohistiocytosis) -- ## Pathology - - - - Perivascular predominance lymphohistiocytic infiltrates + diffuse adjacent parenchymal inflammatory infiltrate - - May be type of macrophage activation syndrome (secondary hemophagocytic lymphohistiocytosis) -- ## Clinical Issues - - - - Mean age at onset 50 years (range: 13-86 years) - - Subacute pontocerebeller dysfunction - - Often relapsing-remitting course (without treatment) - - Keep in mind: CLIPPERS is diagnosis of exclusion! - -# TERMINOLOGY - -- ## Abbreviations - - - - **C**hronic **l**ymphocytic **i**nflammation with**p**ontine **p**erivascular **e**nhancement responsive to **s**teroids (CLIPPERS) -- ## Definitions - - - - Recently described inflammatory CNS disorder - - Distinct form of nonneoplastic encephalitis with predominant T-cell pathology - - Predominantly involves brainstem, adjacent rhombencephalic structures - - Striking clinical, imaging response to glucocorticosteroids - -# IMAGING - -- ## General Features - - - - ### Best diagnostic clue - - - - Enhancing punctate/curvilinear lesions "peppering" pons - - ### Location - - - - Predominantly pons/cerebellum **but**may extend - - Caudally into medulla, spinal cord - - Rostrally into midbrain - - ≈ 60% have CNS lesions **outside** pontocerebellar region - - Basal ganglia, hemispheric white matter, cortex - - Meningeal inflammation - - Caution: May begin as isolated, enhancing mass in pons/cerebellar peduncle before exhibiting typical pattern of multifocal punctate pontine lesions - - ### Size - - - - Usually (but not always) ≤ 3mm - - ### Morphology - - - - Typically small, punctate or curvilinear -- ## CT Findings - - - - Usually normal -- ## MR Findings - - - - ### T1WI - - - - Usually normal - - Pontocerebellar/cerebellar, cord, cerebral atrophy may be late changes - - ### T2WI - - - - ± faint, patchy or "speckled" punctate hyperintensities on T2/FLAIR - - Minimal or no mass effect, vasogenic edema - - Homogeneous hyperintensity; does not significantly exceed T1 C+ enhancement - - ### T2* GRE - - - - Usually negative - - ### DWI - - - - Usually absent - - ### T1WI C+ - - - - Punctate &/or curvilinear enhancing foci ("peppering" pons) - - No ring or patchy enhancement - - ↓ to absence of enhancement following steroids -- ## Angiographic Findings - - - - DSA normal without evidence for vasculitis -- ## Imaging Recommendations - - - - ### Best imaging tool - - - - MR ± contrast (include coronal T1C+), DWI, T2* - -# DIFFERENTIAL DIAGNOSIS - -- ## Lymphoma, Angiocentric (Intravascular) - - - - Stroke-like symptoms, dementia - - Hemorrhage, foci of restricted diffusion common -- [Neurosarcoidosis](/document/neurosarcoid/fef69139-0019-4be3-9bdc-e26bc3644251) - - Dura, leptomeningeal lesions common - - Pituitary-hypothalamus often affected -- [Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0) - - Primary angiitis of CNS (PACNS), systemic vasculitides - - Neuro-Behçet -- [Demyelinating Disease](/document/multiple-sclerosis/7892b2a2-f52a-4d7f-9858-a326f2b7ab04) - - Multiple sclerosis (MS), MOG antibody-associated disease - - Seropositive autoimmune encephalitides -- ## Lymphomatoid Granulomatosis - - - - Brain often more diffusely involved -- [CNS Histiocytosis](/document/langerhans-cell-histiocytosis-skul-/6515bdbb-ce3d-47ef-9930-2dbb1949f807) - - Hemophagocytic lymphohistiocytosis (HLH) resembles CLIPPERS on brain biopsy - -# PATHOLOGY - -- ## General Features - - - - ### Etiology - - - - May be type of macrophage activation syndrome (secondary HLH) -- ## Microscopic Features - - - - Perivascular predominance lymphohistiocytic infiltrates + diffuse adjacent parenchymal inflammatory infiltrate - - Marked CD3-positive T lymphocytes, variable macrophage components - - Variable tissue destruction, astrogliosis, myelin loss - -# CLINICAL ISSUES - -- ## Presentation - - - - ### Most common signs/symptoms - - - - Subacute pontocerebeller dysfunction - - ± other CNS symptoms (e.g., cognitive dysfunction, myelopathy) -- ## Demographics - - - - ### Age - - - - Mean age at onset 50 years (range: 13-86 years) - - ### Sex - - - - M:F = 3:1 -- ## Natural History & Prognosis - - - - Generally subacute presentation - - Often relapsing-remitting course (without treatment) - - Relapse rare when daily glucocorticoids ≥ 30 mg - - Diagnosis of exclusion - - Requires careful exclusion of alternative diagnoses -- ## Treatment - - - - Corticosteroids (marked clinical, imaging response key to diagnosis) - - Corticosteroid responsiveness also common but not universal in non-CLIPPERS diagnoses - - Relapse off treatment common - - Hydroxychloroquine has been reported to induce, maintain remission of symptoms - - 0e17b374-1564-4020-a6e2-552480332e98 - -## References - -# Selected References - -1. [Li Z et al: CLIPPERS, a syndrome of lymphohistiocytic disorders. Mult Scler Relat Disord. 42:102063, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32234602%5Bpmid%5D) -1. [Turnquist C et al: CLIPPERS: a case report with radiology, three serial biopsies and a literature review. Clin Neuropathol. 39(1):19-24, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31661071%5Bpmid%5D) -1. [Taieb G et al: CLIPPERS and its mimics: evaluation of new criteria for the diagnosis of CLIPPERS. J Neurol Neurosurg Psychiatry. 90(9):1027-38, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31072955%5Bpmid%5D) -1. [Berzero G et al: CLIPPERS mimickers: relapsing brainstem encephalitis associated with anti-MOG antibodies. Eur J Neurol. 25(2):e16-7, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29356261%5Bpmid%5D) -1. [Tian D et al: Case 259: Primary central nervous system lymphomatoid granulomatosis mimicking chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Radiology. 289(2):572-7, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30332362%5Bpmid%5D) -1. [Tobin WO et al: Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. 140(9):2415-25, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29050399%5Bpmid%5D) -1. [Taieb G et al: Punctate and curvilinear gadolinium enhancing lesions in the brain: a practical approach. Neuroradiology. 58(3):221-35, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26700824%5Bpmid%5D) -1. [Gul M et al: Atypical presentation of CLIPPERS syndrome: a new entity in the differential diagnosis of central nervous system rheumatologic diseases. J Clin Rheumatol. 21(3):144-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25807094%5Bpmid%5D) -1. [Dudesek A et al: CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders. Clin Exp Immunol. 175(3):385-96, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24028073%5Bpmid%5D) -1. [Pittock SJ et al: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. 133(9):2626-34, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20639547%5Bpmid%5D) - - -## Images - - -### Selected Images - -![Sagittal FLAIR MR in a 56-year-old woman with weight loss and a 3-week history of diplopia and disequilibrium shows confluent and punctate hyperintensities in the pons and medulla .](images/app.statdx.com_image_thumbnail_3b810ab1-5bad-4ec7-936d-bc3cf0683b4d_annotated_true_size_900_quality_90_665175a0_20251018T122457Z.jpg) -*Sagittal FLAIR MR in a 56-year-old woman with weight loss and a 3-week history of diplopia and disequilibrium shows confluent and punctate hyperintensities in the pons and medulla .* - -![Sagittal FLAIR MR in a 56-year-old woman with weight loss and a 3-week history of diplopia and disequilibrium shows confluent and punctate hyperintensities in the pons and medulla .](images/app.statdx.com_image_thumbnail_3b810ab1-5bad-4ec7-936d-bc3cf0683b4d_size_168_quality_85_8ebb6805_20251018T095255Z.jpg) -*Sagittal FLAIR MR in a 56-year-old woman with weight loss and a 3-week history of diplopia and disequilibrium shows confluent and punctate hyperintensities in the pons and medulla .* - -![Sagittal FLAIR MR in a 56-year-old woman with weight loss and a 3-week history of diplopia and disequilibrium shows confluent and punctate hyperintensities in the pons and medulla .](images/app.statdx.com_image_thumbnail_3b810ab1-5bad-4ec7-936d-bc3cf0683b4d_size_174_quality_85_03245c01_20251018T122441Z.jpg) -*Sagittal FLAIR MR in a 56-year-old woman with weight loss and a 3-week history of diplopia and disequilibrium shows confluent and punctate hyperintensities in the pons and medulla .* - -![Axial T1 C+ MR in the same patient shows multiple punctate and curvilinear enhancing foci "peppering" the pons . Additional lesions are present in both cerebellar peduncles, vermis, and the left cerebellar hemisphere.](images/app.statdx.com_image_thumbnail_0412a98f-922e-4e0e-8106-5bf10bac5e68_annotated_true_size_900_quality_90_1e116da8_20251018T122457Z.jpg) -*Axial T1 C+ MR in the same patient shows multiple punctate and curvilinear enhancing foci "peppering" the pons . Additional lesions are present in both cerebellar peduncles, vermis, and the left cerebellar hemisphere.* - -![Axial T1 C+ MR in the same patient shows multiple punctate and curvilinear enhancing foci "peppering" the pons . Additional lesions are present in both cerebellar peduncles, vermis, and the left cerebellar hemisphere.](images/app.statdx.com_image_thumbnail_0412a98f-922e-4e0e-8106-5bf10bac5e68_size_168_quality_85_3093054b_20251018T095255Z.jpg) -*Axial T1 C+ MR in the same patient shows multiple punctate and curvilinear enhancing foci "peppering" the pons . Additional lesions are present in both cerebellar peduncles, vermis, and the left cerebellar hemisphere.* - -![More cephalad T1 C+ MR scan in the same patient shows the punctate and curvilinear lesions involving the upper pons.](images/app.statdx.com_image_thumbnail_bbe0d150-9cfd-4e4c-b8ca-2d1adceadb6b_annotated_true_size_900_quality_90_8ea0b444_20251018T122457Z.jpg) -*More cephalad T1 C+ MR scan in the same patient shows the punctate and curvilinear lesions involving the upper pons.* - -![More cephalad T1 C+ MR scan in the same patient shows the punctate and curvilinear lesions involving the upper pons.](images/app.statdx.com_image_thumbnail_bbe0d150-9cfd-4e4c-b8ca-2d1adceadb6b_size_168_quality_85_d66f7fff_20251018T095255Z.jpg) -*More cephalad T1 C+ MR scan in the same patient shows the punctate and curvilinear lesions involving the upper pons.* - -![Coronal T1 C+ FS MR in the same patient shows the lesions "peppering" the pons. Note cephalad extension into the cerebral peduncles and inferior extension into the medulla and upper cervical cord . DSA (not shown) was negative. The lesions resolved with corticosteroids, so this is a presumed case of CLIPPERS.](images/app.statdx.com_image_thumbnail_014b1c32-e926-4656-987e-d8311c674576_annotated_true_size_900_quality_90_8455b9a1_20251018T122457Z.jpg) -*Coronal T1 C+ FS MR in the same patient shows the lesions "peppering" the pons. Note cephalad extension into the cerebral peduncles and inferior extension into the medulla and upper cervical cord . DSA (not shown) was negative. The lesions resolved with corticosteroids, so this is a presumed case of CLIPPERS.* - -![Coronal T1 C+ FS MR in the same patient shows the lesions "peppering" the pons. Note cephalad extension into the cerebral peduncles and inferior extension into the medulla and upper cervical cord . DSA (not shown) was negative. The lesions resolved with corticosteroids, so this is a presumed case of CLIPPERS.](images/app.statdx.com_image_thumbnail_014b1c32-e926-4656-987e-d8311c674576_size_168_quality_85_e2ebbeb7_20251018T095255Z.jpg) -*Coronal T1 C+ FS MR in the same patient shows the lesions "peppering" the pons. Note cephalad extension into the cerebral peduncles and inferior extension into the medulla and upper cervical cord . DSA (not shown) was negative. The lesions resolved with corticosteroids, so this is a presumed case of CLIPPERS.* - - -### Additional Images - -![Sagittal FLAIR in a 52-year-old man with diplopia, dysarthria, and facial numbness shows confluent hyperintensity in the pons .](images/app.statdx.com_image_thumbnail_b7924d60-22a9-44a8-8eab-a122a8fabee9_annotated_true_size_900_quality_90_e520a898_20251018T122457Z.jpg) -*Sagittal FLAIR in a 52-year-old man with diplopia, dysarthria, and facial numbness shows confluent hyperintensity in the pons .* - -![Sagittal FLAIR in a 52-year-old man with diplopia, dysarthria, and facial numbness shows confluent hyperintensity in the pons .](images/app.statdx.com_image_thumbnail_b7924d60-22a9-44a8-8eab-a122a8fabee9_size_168_quality_85_afe982f1_20251018T095255Z.jpg) -*Sagittal FLAIR in a 52-year-old man with diplopia, dysarthria, and facial numbness shows confluent hyperintensity in the pons .* - -![Axial T1 C+ MR shows scattered, faint, punctate enhancing foci as well as larger confluent, nodular , and partial ring-enhancing lesions in the pons.](images/app.statdx.com_image_thumbnail_54794f9e-0cff-4b09-8514-3f0c0f658cbd_annotated_true_size_900_quality_90_3d84ec59_20251018T122457Z.jpg) -*Axial T1 C+ MR shows scattered, faint, punctate enhancing foci as well as larger confluent, nodular , and partial ring-enhancing lesions in the pons.* - -![Axial T1 C+ MR shows scattered, faint, punctate enhancing foci as well as larger confluent, nodular , and partial ring-enhancing lesions in the pons.](images/app.statdx.com_image_thumbnail_54794f9e-0cff-4b09-8514-3f0c0f658cbd_size_168_quality_85_ca6dda0c_20251018T095255Z.jpg) -*Axial T1 C+ MR shows scattered, faint, punctate enhancing foci as well as larger confluent, nodular , and partial ring-enhancing lesions in the pons.* - -![Coronal T1 C+ MR in the same patient shows large, confluent, patchy enhancing lesions in the pons. Differential diagnosis included lymphoma, lymphomatoid granulomatosis, vasculitis, and CLIPPERS. The patient improved on steroids.](images/app.statdx.com_image_thumbnail_179e89fa-d5c6-4f10-b298-b1179723c303_annotated_true_size_900_quality_90_df3edd4b_20251018T122457Z.jpg) -*Coronal T1 C+ MR in the same patient shows large, confluent, patchy enhancing lesions in the pons. Differential diagnosis included lymphoma, lymphomatoid granulomatosis, vasculitis, and CLIPPERS. The patient improved on steroids.* - -![Coronal T1 C+ MR in the same patient shows large, confluent, patchy enhancing lesions in the pons. Differential diagnosis included lymphoma, lymphomatoid granulomatosis, vasculitis, and CLIPPERS. The patient improved on steroids.](images/app.statdx.com_image_thumbnail_179e89fa-d5c6-4f10-b298-b1179723c303_size_168_quality_85_f7ef21fc_20251018T095255Z.jpg) -*Coronal T1 C+ MR in the same patient shows large, confluent, patchy enhancing lesions in the pons. Differential diagnosis included lymphoma, lymphomatoid granulomatosis, vasculitis, and CLIPPERS. The patient improved on steroids.* - -![Sagittal FLAIR in the same patient obtained a year later when symptoms relapsed off steroids shows multiple punctate hyperintensities "peppering" the pons and medulla . Note extension into upper spinal cord .](images/app.statdx.com_image_thumbnail_6b5d2b9c-b124-4a6a-beba-fe65179bd0b6_annotated_true_size_900_quality_90_fc0ab4e1_20251018T122457Z.jpg) -*Sagittal FLAIR in the same patient obtained a year later when symptoms relapsed off steroids shows multiple punctate hyperintensities "peppering" the pons and medulla . Note extension into upper spinal cord .* - -![Sagittal FLAIR in the same patient obtained a year later when symptoms relapsed off steroids shows multiple punctate hyperintensities "peppering" the pons and medulla . Note extension into upper spinal cord .](images/app.statdx.com_image_thumbnail_6b5d2b9c-b124-4a6a-beba-fe65179bd0b6_size_168_quality_85_07b4c70d_20251018T095255Z.jpg) -*Sagittal FLAIR in the same patient obtained a year later when symptoms relapsed off steroids shows multiple punctate hyperintensities "peppering" the pons and medulla . Note extension into upper spinal cord .* - -![Axial T1 C + FS MR in the same patient shows small, punctate foci of enhancement "peppering" the pons, cerebellar peduncles.](images/app.statdx.com_image_thumbnail_f849c125-5528-4f44-943a-db3c921f3a9b_annotated_true_size_900_quality_90_1b2921c8_20251018T122500Z.jpg) -*Axial T1 C + FS MR in the same patient shows small, punctate foci of enhancement "peppering" the pons, cerebellar peduncles.* - -![Axial T1 C + FS MR in the same patient shows small, punctate foci of enhancement "peppering" the pons, cerebellar peduncles.](images/app.statdx.com_image_thumbnail_f849c125-5528-4f44-943a-db3c921f3a9b_size_168_quality_85_7fa9d5c0_20251018T095255Z.jpg) -*Axial T1 C + FS MR in the same patient shows small, punctate foci of enhancement "peppering" the pons, cerebellar peduncles.* - -![More inferior T1 C+ FS MR in the same patient shows additional small enhancing foci in the medulla .](images/app.statdx.com_image_thumbnail_b1bf1bf1-9d90-4fe9-905b-3b85debd861f_size_168_quality_85_2931bc9a_20251018T095255Z.jpg) -*More inferior T1 C+ FS MR in the same patient shows additional small enhancing foci in the medulla .* - -![More cephalad T1 C+ FS MR in the same patient shows additional lesions in the midbrain and medial temporal lobe .](images/app.statdx.com_image_thumbnail_56b83112-4e12-4510-beff-f833b9599c27_size_168_quality_85_dab3c1ff_20251018T095255Z.jpg) -*More cephalad T1 C+ FS MR in the same patient shows additional lesions in the midbrain and medial temporal lobe .* - -![More cephalad T1 C+ FS MR in the same patient shows a solitary enhancing lesion in the subcortical white matter of the "hand knob." One of the cerebellar lesions was biopsied and disclosed CD4+ T-cell perivascular infiltrates, consistent with CLIPPERS. In rare cases, CLIPPERS initially manifests as a more mass-like confluent pontine lesion before the typical peppering pattern emerges.](images/app.statdx.com_image_thumbnail_784cb251-3014-4189-b444-0f63b0f2a125_size_168_quality_85_1dac933c_20251018T095255Z.jpg) -*More cephalad T1 C+ FS MR in the same patient shows a solitary enhancing lesion in the subcortical white matter of the "hand knob." One of the cerebellar lesions was biopsied and disclosed CD4+ T-cell perivascular infiltrates, consistent with CLIPPERS. In rare cases, CLIPPERS initially manifests as a more mass-like confluent pontine lesion before the typical peppering pattern emerges.* - diff --git a/out/corpus-callosum-impingement-syndrome_e84adf32-bae3-47d5-b368-489f413f6aea.md b/out/corpus-callosum-impingement-syndrome_e84adf32-bae3-47d5-b368-489f413f6aea.md new file mode 100644 index 0000000..3f2d2e5 --- /dev/null +++ b/out/corpus-callosum-impingement-syndrome_e84adf32-bae3-47d5-b368-489f413f6aea.md @@ -0,0 +1,305 @@ +--- +title: "Corpus Callosum Impingement Syndrome" +docid: "e84adf32-bae3-47d5-b368-489f413f6aea" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "Corpus Callosum Impingement Syndrome" + slug: "corpus-callosum-impingement-syndro-" + treeNodeId: null +category: "Brain" +documentVersionId: "2188842b-0f05-4580-9c01-5ee89586a08f" +imageCount: 4 +lastUpdated: "06/09/20" +pageDescription: "Corpus Callosum Impingement Syndrome" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Corpus Callosum Impingement Syndrome" +pageTitle: "Corpus Callosum Impingement Syndrome | STATdx" +enhancedTitle: "Corpus Callosum Impingement Syndrome" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "Corpus Callosum Impingement Syndrome" +--- +# KEY FACTS + +- ## Terminology + + + - Corpus callosum impingement syndrome (CCIS) + - Corpus callosum (CC) + - Callosal injury from longstanding obstructive hydrocephalus +- ## Imaging + + + - Acute + - Following ventricular decompression in longstanding obstructive hydrocephalus + - Swollen and hyperintense body of CC + - Diffuse or focal hyperintense areas in CC + - Chronic + - Encephalomalacic foci, shrunken and atrophic-appearing CC + - No hemorrhage, restricted diffusion, or enhancement +- ## Top Differential Diagnoses + + + - **Acute phase: CC swollen with T2/FLAIR hyperintensity** + - Tumors involving CC, tumefactive demyelination, transient cytotoxic splenial lesion, ischemia/infarct, diffuse axonal injury + - **Chronic phase: CC atrophy with cystic changes** + - Multiple sclerosis, postsurgical, enlarged perivascular spaces, Marchiafava-Bignami disease, Susac syndrome +- ## Pathology + + + - Exact mechanism for callosal lesions unknown +- ## Clinical Issues + + + - CC injury does not appear to produce any clinically recognizable symptomatology + - CCIS is uncommon sequel of severe chronic hydrocephalus + - Longstanding cases: Atrophic CC and signal abnormality, may persist after ventricular decompression +- ## Diagnostic Checklist + + + - Consider CCIS in patient treated with ventricular decompression for longstanding obstructive hydrocephalus + - CC signal change, although dramatic, should not be mistaken for other pathologies + +# TERMINOLOGY + +- ## Abbreviations + + + - Corpus callosum impingement syndrome (CCIS) + - Corpus callosum (CC) +- ## Definitions + + + - Callosal injury from longstanding obstructive hydrocephalus + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Diffuse &/or focal T2/FLAIR hyperintensity in CC + - ### Location + + + - Isthmus, body of CC, splenium generally spared + - ### Size + + + - Variable + - ### Morphology + + + - Ill-defined or focal lesions +- ## CT Findings + + + - Thinning and upward displacement of CC + - Postventricular decompression or shunting + - Acute: Swollen, hypodense body of CC + - Chronic: Atrophy, cystic changes in body of CC +- ## MR Findings + + + - ### T1WI + + + - Severe hydrocephalus with upward bowing and thinning of CC + - Following ventricular decompression, swollen hypointense body of CC + - ### T2WI + + + - Acute + - Following ventricular decompression + - Swollen, hyperintense body of CC + - Diffuse or focal hyperintense areas + - May see periventricular white matter (WM) hyperintensities + - Chronic + - Encephalomalacic foci, shrunken and atrophic-appearing CC + - ### FLAIR + + + - More sensitive to evaluate CC and periventricular WM + - ### T2* GRE + + + - No hemorrhage + - ### DWI + + + - No restricted diffusion + - ### T1WI C+ + + + - No enhancement + - DTI: Fiber disruption in body of CC +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR + - ### Protocol advice + + + - Add sagittal T2/FLAIR + +# DIFFERENTIAL DIAGNOSIS + +- ## Acute Phase: Corpus Callosum Swollen With T2/FLAIR Hyperintensity + + + - **Tumors involving CC** + - Lymphoma: Uniform enhancement, restricted diffusion + - Glioblastoma: Central necrosis, irregular enhancement + - [Tumefactive demyelination](/document/demyelinating-diseases/e3ba880e-d924-4594-a6f4-c21c5f1f0ae7) + - Often incomplete, horseshoe-shaped enhancement + - **Transient cytotoxic splenial lesion** + - Round or boomerang-shaped diffusion restriction in CC splenium, diverse etiologies + - [Ischemia/infarct](/document/acute-cerebral-ischemiainfarction/a405285f-aaea-43ca-8dc4-6f8120eaabc1) + - Diffusion restriction in CC likely due to "acute wallerian degeneration" + - [Diffuse axonal injury (DAI)](/document/diffuse-axonal-injury/c67bbb31-aea3-420f-b65f-de49bb26fcfc) + - Signal loss on SWI, ↑ T2, diffusion restriction + - **Interstitial edema (obstructive hydrocephalus)** + - ↑ T2/FLAIR signal along CC ventricular surface + - Look for additional abnormal signal of frontal and occipital periventricular WM +- ## Chronic Phase: Corpus Callosum Atrophy With Cystic Changes + + + - [Multiple sclerosis](/document/multiple-sclerosis/7892b2a2-f52a-4d7f-9858-a326f2b7ab04) + - "Burned-out" chronic lesions + - **Postsurgical** + - Small CC "holes" common after shunt + - Corpus callosotomy + - [Enlarged perivascular spaces](/document/enlarged-perivascular-spaces/58fff1ec-50b7-4caf-ab31-3341ab7044c9) + - Follow CSF on all sequences + - When CC involved, adjacent brain often involved + - [Marchiafava-Bignami disease](/document/alcoholic-encephalopathy/88021852-b73d-4cdf-a719-dd4ae3231e45) + - Rare complication of chronic alcoholism + - T2-hyperintense CC (middle layers) + - **Susac syndrome** + - Encephalopathy, visual changes, hearing loss + - Multifocal supratentorial WM lesions + CC + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Exact mechanism for callosal lesions unknown + - Mechanical compression + - Severe obstructive hydrocephalus + - CC compressed against free inferior margin of falx + - Pressure necrosis + - Ventricular decompression associated edema + - Compromised venous drainage + - Traction-induced arterial compromise, demyelination +- ## Gross Pathologic & Surgical Features + + + - Chronic impingement of CC associated with callosal thinning, cystic changes +- ## Microscopic Features + + + - Loss of callosal axons + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Signs/symptoms related to obstructive hydrocephalus + - CCIS does not appear to produce any clinically recognizable symptoms +- ## Demographics + + + - CCIS uncommon sequela of severe chronic hydrocephalus + - MR CC signal change in 8.3% of patients following shunt insertion for obstructive hydrocephalus +- ## Natural History & Prognosis + + + - Ventricular decompression may reverse CC signal change + - Longstanding cases: Atrophic CC and signal abnormality, may persist after ventricular decompression + - No long-term neurologic sequel from callosal damage +- ## Treatment + + + - None for CC signal abnormality + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - CCIS in patient treated with ventricular decompression for longstanding obstructive hydrocephalus +- ## Image Interpretation Pearls + + + - CC signal change, although dramatic, should not be mistaken for other pathologies + + ef393b1c-c6e2-4c4a-b4db-ada4a6ccd7cb + +## References + +# Selected References + +1. [Su S et al: Post-shunting corpus callosal signal change and review of the literature. J Clin Neurosci. 72:466-8, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31874812%5Bpmid%5D) +1. [Oon SF et al: Corpus callosum impingement syndrome: a callosal or colossal problem? Can J Neurol Sci. 44(6):728-9, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29391083%5Bpmid%5D) +1. [Ferrara JM: Signal hyperintensity of the callosum after ventriculoperitoneal shunting. Neurology. 84(15):1609-10, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25870451%5Bpmid%5D) +1. [Lane JI et al: Corpus callosal signal changes in patients with obstructive hydrocephalus after ventriculoperitoneal shunting. AJNR Am J Neuroradiol. 22(1):158-62, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11158902%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal T1 MR in a patient with longstanding severe obstructive hydrocephalus demonstrates markedly dilated lateral ventricle with upward displacement and thinning of the corpus callosum (CC) . Note the shunt catheter , which was placed immediately before the scan.](images/app.statdx.com_image_thumbnail_190b394f-861b-42e4-909b-125648b68eed_annotated_true_size_900_quality_90_06683e2f_20251018T160022Z.jpg) +*Sagittal T1 MR in a patient with longstanding severe obstructive hydrocephalus demonstrates markedly dilated lateral ventricle with upward displacement and thinning of the corpus callosum (CC) . Note the shunt catheter , which was placed immediately before the scan.* + +![Sagittal T1 MR in the same patient 7 days after placement of the shunt catheter shows patchy areas of low signal in the CC . Lateral ventricles are now decompressed, and there is no mass effect on the CC.](images/app.statdx.com_image_thumbnail_a662615a-810a-4a2d-830f-573e159d170a_annotated_true_size_900_quality_90_1d3bc703_20251018T160022Z.jpg) +*Sagittal T1 MR in the same patient 7 days after placement of the shunt catheter shows patchy areas of low signal in the CC . Lateral ventricles are now decompressed, and there is no mass effect on the CC.* + +![Axial FLAIR MR in the same patient 7 days following placement of the shunt catheter demonstrates ill-defined hyperintensities in the body of the CC as well as in the periventricular white matter . Note decompressed lateral and 3rd ventricles.](images/app.statdx.com_image_thumbnail_8a95800b-56d9-4311-bf1b-3fd3a6302496_annotated_true_size_900_quality_90_e12da5be_20251018T160022Z.jpg) +*Axial FLAIR MR in the same patient 7 days following placement of the shunt catheter demonstrates ill-defined hyperintensities in the body of the CC as well as in the periventricular white matter . Note decompressed lateral and 3rd ventricles.* + +![Axial FLAIR MR in the same patient 1 month after placement of the shunt shows mild decrease in the hyperintensities in the CC as well as the periventricular white matter with further decompression of the lateral and 3rd ventricles.](images/app.statdx.com_image_thumbnail_f93749c2-5c2a-43fb-9cc6-55a6f3b93079_annotated_true_size_900_quality_90_f97b7f6a_20251018T160022Z.jpg) +*Axial FLAIR MR in the same patient 1 month after placement of the shunt shows mild decrease in the hyperintensities in the CC as well as the periventricular white matter with further decompression of the lateral and 3rd ventricles.* + diff --git a/out/csf-shunts-and-complications_1027d634-92ff-47c1-8266-a7fc3acd1529.md b/out/csf-shunts-and-complications_1027d634-92ff-47c1-8266-a7fc3acd1529.md new file mode 100644 index 0000000..522d4ca --- /dev/null +++ b/out/csf-shunts-and-complications_1027d634-92ff-47c1-8266-a7fc3acd1529.md @@ -0,0 +1,531 @@ +--- +title: "CSF Shunts and Complications" +docid: "1027d634-92ff-47c1-8266-a7fc3acd1529" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" + - key: "99e1aff7-f42c-43a0-95ae-d89c8551aa01" + value: "Kevin R. Moore, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "CSF Shunts and Complications" + slug: "csf-shunts-and-complications" + treeNodeId: null +category: "Brain" +documentVersionId: "201dad63-bfb4-4f05-ade4-1140c667d000" +imageCount: 23 +lastUpdated: "09/24/20" +pageDescription: "CSF Shunts and Complications" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, CSF Shunts and Complications" +pageTitle: "CSF Shunts and Complications | STATdx" +enhancedTitle: "CSF Shunts and Complications" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "CSF Shunts and Complications" +--- +# KEY FACTS + +- ## Terminology + + + - Hydrocephalus (HCP) + - Enlargement of cerebral ventricles secondary to abnormal CSF formation, flow, or absorption resulting in ↑ CSF volume +- ## Imaging + + + - Shunt failure → dilated ventricles + edema around ventricles, along catheter and reservoir + - Use CT or MR to evaluate ventricle size, plain radiograph shunt series to identify mechanical shunt failure + - Baseline CT/MR following shunt insertion, follow-up at 1 year and as clinically needed + - Shunt radionuclide studies: Used to confirm distal obstruction +- ## Top Differential Diagnoses + + + - Shunt failure with normal ventricle size or lack of interstitial edema + - Noncompliant (slit) ventricle syndrome + - Acquired Chiari 1 malformation/tonsillar ectopia +- ## Pathology + + + - Obstructive HCP: Secondary to physical blockage by tumor, adhesions, cyst + - Communicating HCP: Secondary to ↓ CSF absorption across arachnoid granulations +- ## Clinical Issues + + + - Older children/adults: Headache, vomiting, lethargy, seizure, neurocognitive symptoms + - Infants: Bulging fontanelle, ↑ head circumference, irritability, lethargy +- ## Diagnostic Checklist + + + - Shunt + headache not always shunt failure + - Confirm programmable shunt valve setting after MR + - Compare current CT with prior studies to detect subtle changes in ventricle size + +# TERMINOLOGY + +- ## Abbreviations + + + - Shunt types: Ventriculoperitoneal (VP), ventriculoatrial (VA), ventriculopleural (VPL), lumboperitoneal (LP) +- ## Definitions + + + - Ventriculomegaly + - General term for enlargement of cerebral ventricles + - Hydrocephalus (HCP) + - Enlargement of cerebral ventricles secondary to abnormal CSF formation, flow, or absorption resulting in ↑ CSF volume + - Subset of ventriculomegaly + - Onset over days (acute), weeks (subacute), or months to years (chronic) + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Shunt failure: Dilated ventricles + edema ("blurring") around ventricles and along catheter, reservoir + - ### Location + + + - VP shunt common; VA and VPL used rarely unless VP contraindicated + - ### Size + + + - Ventricular size is relative → ventriculomegaly, may indicate shunt failure in one patient and be stable finding in another + - Change in ventricular size in individual patient probably significant + - Conversely, some patients manifest shunt failure with minimal to no change in ventricular size + - Distal catheter must be sized long enough to permit somatic growth, prevent retraction out of abdomen or chest + - ### Morphology + + + - Shunt system components + - Proximal catheter in ventricles, subarachnoid space, syrinx cavity, or thecal sac + - Unidirectional valve prevents reflux into ventricles + - Reservoir used to sample CSF, acutely relieve pressure + - Distal catheter tunneled through subcutaneous tissues → tip in peritoneal cavity, cardiac atrium, or pleural cavity +- ## Radiographic Findings + + + - ### Radiography + + + - Evaluate shunt catheter system integrity + - Shunt fracture, separation, migration + - Distal catheter may retract out of abdomen if significant somatic growth since shunt placement +- ## Fluoroscopic Findings + + + - Contrast shuntogram to define site of obstruction (historical interest) +- ## CT Findings + + + - ### NECT + + + - Ventricular dilatation (diffuse or loculated) + - Isolated ventricle after infection, hemorrhage → interventricular synechia + - Periventricular interstitial edema ("blurred" ventricle margins) → acute HCP + - Small, slit ventricles → noncompliant ventricle syndrome, chronic overdrainage + - ± subdural hematoma (CSF overdrainage) + - ### CECT + + + - ± ependymal enhancement (chemical or infectious ventriculitis) + - Detection of intracranial abscess or empyema as complication of shunt infection +- ## MR Findings + + + - ### T1WI + + + - Assess ventricular size, characterize brain anatomy + - ### T2WI + + + - ± interstitial periventricular edema → acute shunt failure + - ### FLAIR + + + - Interstitial edema more conspicuous than on T1WI or T2WI + - ### T2* GRE + + + - Assess hemorrhagic shunt tracts, interventricular hemorrhage + - ### DWI + + + - ↑ diffusivity with interstitial edema + - ### T1WI C+ + + + - ± enhancement with ventriculitis, abscess, neoplasm + - Pachymeningeal enhancement due to low intracranial pressure (ICP) + - ### MRA + + + - Stretched, displaced arteries around dilated ventricles secondary to ventriculomegaly + - ### MRV + + + - Venous thrombosis may precede HCP or follow shunting + - Leads to ↑ intraventricular/ICP + - ### MR cine + + + - Evaluate patency of normal CSF pathways, 3rd ventriculostomy + - ### MRS + + + - Small lactate resonances detected in CSF of up to 20% of HCP, even if HCP absent +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Useful in neonates for serial assessment of ventricular size (requires open fontanelle) + - ### Pulsed Doppler + + + - Resistive indices increase with shunt obstruction, ↑ ICP + - ### Color Doppler + + + - Research studies document flow within shunt tubing, aqueduct +- ## Nonvascular Interventions + + + - Interventricular contrast injection through shunt + NECT→ detect ventricular isolation needing additional catheter +- ## Nuclear Medicine Findings + + + - ### PET + + + - Cerebral vascular reserve (CVR) measurement may aid selection of shunt candidates + - Shunt radionuclide studies + - Radiotracer injected into shunt reservoir; serial imaging to document timing of radiotracer egress from distal catheter tip + - Used to confirm distal obstruction +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Brain NECT to assess for acute ventricular size change + - Fast MR protocols (HASTE, SSFSE) used in many centers with 24-hour MR availability to avoid cumulative radiation exposure + - ### Protocol advice + + + - Brain CT or MR to evaluate ventricle size + - Baseline CT/MR following shunt insertion, follow-up at 1 year and as clinically needed + - Plain film shunt series to identify mechanical shunt fracture or disconnection + +# DIFFERENTIAL DIAGNOSIS + +- ## Shunt Failure With Normal Ventricle Size or Lack of Interstitial Edema + + + - Look for fluid along shunt catheter or reservoir as only sign of malfunction + - May require diagnosis on clinical grounds +- ## Noncompliant (Slit) Ventricle Syndrome + + + - Usually older child (shunted in infancy) + - Small ventricles + intermittent signs of shunt obstruction + - Ventricles normal/small, even if shunt malfunctioning + - May be caused by shunt-induced sutural ossification or poor ventricular compliance +- ## Acquired Chiari 1 Malformation/Tonsillar Ectopia + + + - Functioning LP shunt produces tonsillar descent through foramen magnum + - More common with valveless systems + - Not always symptomatic + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Normal CSF production = 0.2-0.7 mL/minute; 250 (child) to 500 mL (adult) per 24-hour period + - Majority of CSF produced by choroid plexus, resorbed by arachnoid granulations + - Capacity of ventricles in healthy adult = 25 mL + - Total CSF volume (adult) = 125 mL + - Impairment of CSF circulation + - Obstructive + - Usually at narrowest points in CSF circulation (aqueduct, foramina of Monro) + - Tumor, web/synechia, congenital aqueductal stenosis + - Inadequate reabsorption across arachnoid granulations into venous sinuses + - Arachnoid granulations "clogged" after hemorrhage, inflammation + - Diminished pressure gradient from subarachnoid space to venous sinuses secondary to venous hypertension + - Impaired CSF absorption → CSF accumulation, ↑ ICP + - CSF shunt establishes accessory drainage pathway to bypass obstructed natural CSF flow pathways + - Restores or maintains normal ICP + - Each shunt, valve, device carries its own set of complications + - All types → material degradation/fatigue, mechanical stress (especially craniocervical junction, inferior ribs) + - VP → abdominal complications (CSF pseudocyst, ascites, bowel perforation) + - VPL → symptomatic pleural effusion + - VA → shunt nephritis, cor pulmonale, pulmonary embolus + - LP → arachnoiditis, cerebellar tonsillar herniation, high catheter migration rate + - Programmable shunt → unintentional reprogram during MR + - Shuntless CSF diversion → 3rd ventriculostomy, 4th ventricle outlet fenestration + - Silicone allergy → allergic response, catheter occlusion with debris + - Antisiphon devices → obstruction by capsule formation + - 1-piece shunt → ↓ catheter obstruction rate, ↑ slit ventricle/subdural hemorrhage rate + - Flanged catheters → ↑ incidence of proximal occlusion + - Internal 3rd ventricle to spinal SAS (Lapras catheter) → no external access, no method to check flow + - Flow vs. pressure regulation + - Pressure-regulating shunts prone to overdrainage + - Flow-regulating valves prone to obstruction + - Magnetic valves commonly used but cause artifact on MR and require resetting after MR + - Cerebral atrophy, focal destructive lesions also produce ↑ CSF spaces, but these are not HCP + - Loss of cerebral tissue → vacant space passively filled with CSF + - Not result of hydrodynamic disorder → not HCP + - ### Associated abnormalities + + + - Shunts placed with CSF blood/protein > 1 g/dL prone to early blockage, failure + - Shunt infection + - Ventricular loculation or isolation + - Overshunting +- ## Gross Pathologic & Surgical Features + + + - Ventricular ependymal adhesions (scar) + - Extracranial shunt tubing calcification +- ## Microscopic Features + + + - Gliosis along intracranial shunt tract + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Children, adults + - Headache, vomiting, lethargy, seizure + - Neuropsychologic, cognitive, or behavioral + - Infants + - Bulging fontanelle, ↑ head circumference, irritability, lethargy + - ### Clinical profile + + + - Depends on underlying clinical diagnosis necessitating CSF diversion, number of previous shunts, complications +- ## Demographics + + + - ### Age + + + - 1st weeks of life for myelomeningocele, congenital HCP + - Older age at 1st shunting for HCP following trauma, meningitis, tumor + - ### Epidemiology + + + - 160,000 shunts implanted each year worldwide + - CSF shunts in USA ~ 125,000 total + - 33,000 placed per year (~ 45-50% revisions) +- ## Natural History & Prognosis + + + - After shunting, 70% either normal or relatively normal intelligence (if no complications or associated anomalies) + - Epilepsy incidence up to 47% if shunt follows meningitis, hemorrhage + - HCP mortality depends on shunt complication: Malfunction (30%), infection (20%), pulmonary embolus (7%) + - Acute shunt obstruction in shunt-dependent patients may lead to death + - Majority of shunt revisions occur during first 6 months after shunt placement + - Age at time of shunt surgery, previous shunt surgery, etiology of HCP, and HCP type independently associated with incidence of shunt revision + - 50% of patients need multiple revisions, progressively shorter time interval to next failure +- ## Treatment + + + - Shunt revision + - Replace intraventricular component/valve for proximal obstruction + - Alter valve pressure setting/type if over- or underdraining + - Programmable shunt valves permit transcutaneous adjustment of pressure setting + - Lengthen distal shunt as child grows + - 3rd ventriculostomy to avoid indwelling shunt if blockage is distal to 3rd ventricle + - Subtemporal decompression or 3rd ventriculostomy for noncompliant ventricle syndrome + - Laparoscopic or open abdominal procedure for distal obstruction related to CSF pseudocyst + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Shunt + headache does not always mean shunt failure + - Consider sinusitis, trauma, sinovenous thrombosis, viral infection + - Confirm programmable shunt valve setting after MR + - Plain film shunt series has extremely low yield in absence of clinical evidence for mechanical shunt failure +- ## Image Interpretation Pearls + + + - Compare with prior studies to detect subtle ventricular size changes + - Poor ventricular compliance may prevent change in ventricular size despite florid clinical shunt failure + - Fluid tracking along shunt may be only sign of failure; possible even if ventricles normal or unchanged size + + 5eea2e07-bb80-48db-ad5d-a0c14176d17f + +## References + +# Selected References + +1. 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[Dusick JR et al: Success and complication rates of endoscopic third ventriculostomy for adult hydrocephalus: a series of 108 patients. Surg Neurol. 69(1):5-15, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18054606%5Bpmid%5D) +1. [Ellis MJ et al: Treatment of recurrent ventriculoperitoneal shunt failure associated with persistent cerebrospinal fluid eosinophilia and latex allergy by use of an "extracted" shunt. J Neurosurg Pediatr. 1(3):237-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18352769%5Bpmid%5D) +1. [James HE et al: Management of complicated shunt infections: a clinical report. J Neurosurg Pediatr. 1(3):223-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18352767%5Bpmid%5D) +1. [Martínez-Lage JF et al: Acute cholecystitis complicating ventriculo-peritoneal shunting: report of a case and review of the literature. Childs Nerv Syst. 24(6):777-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18365208%5Bpmid%5D) +1. [Nfonsam V et al: Laparoscopic management of distal ventriculoperitoneal shunt complications. Surg Endosc. 22(8):1866-70, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18175181%5Bpmid%5D) +1. [Riffaud L et al: Acquired Chiari I malformation and syringomyelia after valveless lumboperitoneal shunt in infancy. Pediatr Neurosurg. 44(3):229-33, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18354264%5Bpmid%5D) +1. [Liao YJ et al: Intracranial hypotension caused by leakage of cerebrospinal fluid from the thecal sac after lumboperitoneal shunt placement. Case report. J Neurosurg. 107(1):173-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17639890%5Bpmid%5D) +1. [Tseng JS et al: Motor neuron disease-like syndrome secondary to trapped fourth ventricle and obstruction of cerebrospinal fluid pathway. Clin Neurol Neurosurg. 109(4):383-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17275996%5Bpmid%5D) +1. [Woodworth G et al: Prior CSF shunting increases the risk of endoscopic third ventriculostomy failure in the treatment of obstructive hydrocephalus in adults. Neurol Res. 29(1):27-31, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17427271%5Bpmid%5D) +1. [Di Rocco C et al: Shunts vs endoscopic third ventriculostomy in infants: are there different types and/or rates of complications? A review. Childs Nerv Syst. 22(12):1573-89, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17053941%5Bpmid%5D) +1. [Winston KR et al: CSF shunt failure with stable normal ventricular size. Pediatr Neurosurg. 42(3):151-5, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16636615%5Bpmid%5D) +1. [Zamponi N et al: Bobble head doll syndrome in a child with a third ventricular cyst and hydrocephalus. Childs Nerv Syst. 21(5):350-4, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15449088%5Bpmid%5D) +1. [Arnell K et al: Distal catheter obstruction from non-infectious cause in ventriculo-peritoneal shunted children. Eur J Pediatr Surg. 14(4):245-9, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15343464%5Bpmid%5D) +1. [Blount JP et al: Sports and pediatric cerebrospinal fluid shunts: who can play? Neurosurgery. 54(5):1190-6; discussion 1196-8, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15113475%5Bpmid%5D) +1. [Fewel ME et al: Migration of distal ventriculoperitoneal shunt catheter into the heart. Case report and review of the literature. J Neurosurg. 100(2 Suppl Pediatrics):206-11, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14758952%5Bpmid%5D) +1. [Hashimoto M et al: A case of abdominal CSF pseudocyst associated with silicone allergy. Childs Nerv Syst. 20(10):761-4, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14999512%5Bpmid%5D) +1. [Sgouros S et al: An investigation of structural degradation of cerebrospinal fluid shunt valves performed using scanning electron microscopy and energy-dispersive x-ray microanalysis. J Neurosurg. 100(3):534-40, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15035291%5Bpmid%5D) +1. [Tuli S et al: Predictors of death in pediatric patients requiring cerebrospinal fluid shunts. J Neurosurg. 100(5 Suppl Pediatrics):442-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15287452%5Bpmid%5D) +1. [Villarejo FJ et al: Cerebral fluid edema: an unusual complication of ventriculoperitoneal shunts. Childs Nerv Syst. 20(3):195-8, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14749945%5Bpmid%5D) +1. [Braun KP et al: 1H magnetic resonance spectroscopy in human hydrocephalus. J Magn Reson Imaging. 17(3):291-9, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12594718%5Bpmid%5D) +1. [Krassoudakis A et al: Ventriculoperitoneal shunting complicated with cerebrospinal fluid pseudocyst and acute appendicitis. Minerva Pediatr. 54(4):321-3, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12131868%5Bpmid%5D) +1. [Oh A et al: Laparoscopic repositioning of a ventriculo-peritoneal catheter tip for a sterile abdominal cerebrospinal fluid (CSF) pseudocyst. Surg Endosc. 15(5):518, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11353974%5Bpmid%5D) +1. [Drake JM et al: CSF shunts 50 years on--past, present and future. Childs Nerv Syst. 16(10-11):800-4, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11151733%5Bpmid%5D) +1. [Tuli S et al: Risk factors for repeated cerebrospinal shunt failures in pediatric patients with hydrocephalus. J Neurosurg. 92(1):31-8, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10616079%5Bpmid%5D) +1. [Lee TT et al: Unique clinical presentation of pediatric shunt malfunction. Pediatr Neurosurg. 30(3):122-6, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10352413%5Bpmid%5D) +1. [Salomão JF et al: Abdominal pseudocysts complicating CSF shunting in infants and children. Report of 18 cases. Pediatr Neurosurg. 31(5):274-8, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10681683%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Lateral skull radiograph of acute ventriculoperitoneal (VP) shunt failure from a plain radiograph shunt series demonstrates a mechanical shunt catheter disconnection between the programmable valve and the reservoir.](images/app.statdx.com_image_thumbnail_da023baa-4d46-4fb6-8717-a85638b77ef6_annotated_true_size_900_quality_90_971d1d2d_20251018T164445Z.jpg) +*Lateral skull radiograph of acute ventriculoperitoneal (VP) shunt failure from a plain radiograph shunt series demonstrates a mechanical shunt catheter disconnection between the programmable valve and the reservoir.* + +![Lateral skull radiograph of acute ventriculoperitoneal (VP) shunt failure from a plain radiograph shunt series demonstrates a mechanical shunt catheter disconnection between the programmable valve and the reservoir.](images/app.statdx.com_image_thumbnail_da023baa-4d46-4fb6-8717-a85638b77ef6_size_174_quality_85_e8d9943b_20251018T165055Z.jpg) +*Lateral skull radiograph of acute ventriculoperitoneal (VP) shunt failure from a plain radiograph shunt series demonstrates a mechanical shunt catheter disconnection between the programmable valve and the reservoir.* + +![Axial bone CT in the same patient reveals the mechanical catheter disconnection between the reservoir and the programmable shunt valve. This finding had not appeared on the most recent comparison CT scan (not shown).](images/app.statdx.com_image_thumbnail_87340bd2-1fa0-4463-95b4-8a835e5b6f25_annotated_true_size_900_quality_90_48f77c00_20251018T164445Z.jpg) +*Axial bone CT in the same patient reveals the mechanical catheter disconnection between the reservoir and the programmable shunt valve. This finding had not appeared on the most recent comparison CT scan (not shown).* + +![AP radiograph from a shunt series demonstrates intracardiac migration of the VP shunt catheter with the tip residing in the right interlobar pulmonary artery.](images/app.statdx.com_image_thumbnail_2c7ef1d1-3464-480a-8b31-bd93676ca33f_annotated_true_size_900_quality_90_4b19d26a_20251018T164445Z.jpg) +*AP radiograph from a shunt series demonstrates intracardiac migration of the VP shunt catheter with the tip residing in the right interlobar pulmonary artery.* + +![AP radiograph of the pelvis in a 4 year old with a VP shunt who presented with left scrotal swelling demonstrates a coiled distal shunt catheter in the left scrotum. The migration of the catheter to the scrotum is due to a patent processus vaginalis.](images/app.statdx.com_image_thumbnail_54dcd3b6-9e3a-4edb-8a6d-4aa25c8efc4f_annotated_true_size_900_quality_90_b264cf48_20251018T164445Z.jpg) +*AP radiograph of the pelvis in a 4 year old with a VP shunt who presented with left scrotal swelling demonstrates a coiled distal shunt catheter in the left scrotum. The migration of the catheter to the scrotum is due to a patent processus vaginalis.* + +![Axial NECT depicts symmetric interstitial edema within the periventricular white matter. Ventricular size is significantly larger than demonstrated on a prior CT (not shown), supporting the diagnosis of acute shunt failure.](images/app.statdx.com_image_thumbnail_55b2c8c6-2eb1-42ee-b735-b6c0f9cb116d_annotated_true_size_900_quality_90_2a57efea_20251018T164445Z.jpg) +*Axial NECT depicts symmetric interstitial edema within the periventricular white matter. Ventricular size is significantly larger than demonstrated on a prior CT (not shown), supporting the diagnosis of acute shunt failure.* + +![Axial NECT in a patient with VP shunt who presented with severe headaches shows collapsed lateral ventricles . Slit ventricle syndrome presents as severe headaches due to noncompliant ventricles and should not be confused with radiologic slit ventricles.](images/app.statdx.com_image_thumbnail_650743ea-956f-46a3-93a0-873aa0cbddd1_annotated_true_size_900_quality_90_dbd0eceb_20251018T164445Z.jpg) +*Axial NECT in a patient with VP shunt who presented with severe headaches shows collapsed lateral ventricles . Slit ventricle syndrome presents as severe headaches due to noncompliant ventricles and should not be confused with radiologic slit ventricles.* + +![Coronal bone CT demonstrates fracture or disconnection of the ventricular catheter from the reservoir resulting in clinical shunt failure (larger ventricles on NECT).](images/app.statdx.com_image_thumbnail_b7a01263-8d5d-4bcd-a819-2e6b04c35eef_annotated_true_size_900_quality_90_e668546f_20251018T164445Z.jpg) +*Coronal bone CT demonstrates fracture or disconnection of the ventricular catheter from the reservoir resulting in clinical shunt failure (larger ventricles on NECT).* + +![Axial NECT following bilateral ventricular catheter placement in a patient with severe hydrocephalus (HCP) and brain atrophy reveals development of a large left subdural hematoma following VP shunting.](images/app.statdx.com_image_thumbnail_763808e4-56c2-4987-afc2-ab975e4d6708_annotated_true_size_900_quality_90_269bc444_20251018T164445Z.jpg) +*Axial NECT following bilateral ventricular catheter placement in a patient with severe hydrocephalus (HCP) and brain atrophy reveals development of a large left subdural hematoma following VP shunting.* + +![Axial NECT in a patient with HCP presenting with distal VP shunt failure shows the peritoneal catheter tip within a loculated pelvic fluid collection (CSF pseudocyst ).](images/app.statdx.com_image_thumbnail_8a8dd95c-3f84-4e93-a845-7b63366fa374_annotated_true_size_900_quality_90_0d3b647c_20251018T164445Z.jpg) +*Axial NECT in a patient with HCP presenting with distal VP shunt failure shows the peritoneal catheter tip within a loculated pelvic fluid collection (CSF pseudocyst ).* + +![Frontal cisternogram-radionuclide shuntogram examination performed after injecting the shunt valve reservoir reveals no spillage from the distal catheter after 10 minutes. Further delayed imaging (not shown) confirmed absence of spillage from the catheter, substantiating distal shunt obstruction.](images/app.statdx.com_image_thumbnail_9399b93d-ebc2-488c-a1cd-7360eecb3d04_annotated_true_size_900_quality_90_cbfc04fc_20251018T164452Z.jpg) +*Frontal cisternogram-radionuclide shuntogram examination performed after injecting the shunt valve reservoir reveals no spillage from the distal catheter after 10 minutes. Further delayed imaging (not shown) confirmed absence of spillage from the catheter, substantiating distal shunt obstruction.* + + +### Additional Images + +![Lateral radiograph from a plain film shunt series in an infant with acute shunt failure demonstrates that the ventricular catheter has pulled out of the head and is lying along the distal catheter within the scalp (tip ).](images/app.statdx.com_image_thumbnail_7fa2b6f1-07c9-45fc-9a29-d79045ac436a_annotated_true_size_900_quality_90_5a7ac107_20251018T164455Z.jpg) +*Lateral radiograph from a plain film shunt series in an infant with acute shunt failure demonstrates that the ventricular catheter has pulled out of the head and is lying along the distal catheter within the scalp (tip ).* + +![AP radiograph in a patient with chest pain after ventriculopleural (VPL) shunt placement depicts a right pneumothorax related to the shunt placement. Note the abandoned catheter fragment from a prior VP shunt system.](images/app.statdx.com_image_thumbnail_5448724e-86c6-4f45-80dc-1f1856863864_annotated_true_size_900_quality_90_1a2e5733_20251018T164455Z.jpg) +*AP radiograph in a patient with chest pain after ventriculopleural (VPL) shunt placement depicts a right pneumothorax related to the shunt placement. Note the abandoned catheter fragment from a prior VP shunt system.* + +![AP radiography indicates a large left pleural effusion in a symptomatic child with a left VPL shunt catheter .](images/app.statdx.com_image_thumbnail_2e0678e8-e16c-4859-b94f-b85ec399af77_annotated_true_size_900_quality_90_a2293a04_20251018T164458Z.jpg) +*AP radiography indicates a large left pleural effusion in a symptomatic child with a left VPL shunt catheter .* + +![Axial CECT of the pelvis in a shunted patient with HCP presenting with acute shunt failure, fever, and abdominal pain shows the peritoneal VP shunt catheter residing within a rim-enhancing pelvic fluid collection that represents a pelvic abscess secondary to perforated appendicitis.](images/app.statdx.com_image_thumbnail_fbf68223-80c5-46c7-957f-ca81995ba5d9_annotated_true_size_900_quality_90_1980ef02_20251018T164458Z.jpg) +*Axial CECT of the pelvis in a shunted patient with HCP presenting with acute shunt failure, fever, and abdominal pain shows the peritoneal VP shunt catheter residing within a rim-enhancing pelvic fluid collection that represents a pelvic abscess secondary to perforated appendicitis.* + +![Axial NECT in a patient with HCP presenting with shunt failure shows the peritoneal VP shunt catheter tip residing within a large loculated pelvic fluid collection (CSF pseudocyst).](images/app.statdx.com_image_thumbnail_d9b08852-2e8c-4bef-adcb-04e10e0e34c3_annotated_true_size_900_quality_90_3b3e0372_20251018T164459Z.jpg) +*Axial NECT in a patient with HCP presenting with shunt failure shows the peritoneal VP shunt catheter tip residing within a large loculated pelvic fluid collection (CSF pseudocyst).* + +![Axial T2 MR depicts reservoir , shunt tubing , collapsed left lateral ventricle, and dilated isolated right lateral ventricle with associated interstitial transependymal edema .](images/app.statdx.com_image_thumbnail_f7ebef35-a2fe-4035-a785-7ff1d2c028a3_annotated_true_size_900_quality_90_5f628e23_20251018T164459Z.jpg) +*Axial T2 MR depicts reservoir , shunt tubing , collapsed left lateral ventricle, and dilated isolated right lateral ventricle with associated interstitial transependymal edema .* + +![Axial NECT in a patient with posthemorrhagic HCP following contrast injection through the right ventricular catheter shows contrast within the isolated right ventricle but no contrast transit into either the left lateral or 3rd ventricle .](images/app.statdx.com_image_thumbnail_3f761b63-67ef-4fd4-9e41-cf419010a054_annotated_true_size_900_quality_90_f0a5195a_20251018T164459Z.jpg) +*Axial NECT in a patient with posthemorrhagic HCP following contrast injection through the right ventricular catheter shows contrast within the isolated right ventricle but no contrast transit into either the left lateral or 3rd ventricle .* + +![Axial FLAIR MR shows the sequelae of CSF overdrainage leading to bilateral subdural hematomas and ventricular collapse following shunt placement.](images/app.statdx.com_image_thumbnail_b3dfe4c0-3da8-447c-8f47-97a19528e184_annotated_true_size_900_quality_90_5a3db1f4_20251018T164503Z.jpg) +*Axial FLAIR MR shows the sequelae of CSF overdrainage leading to bilateral subdural hematomas and ventricular collapse following shunt placement.* + +![Axial NECT of the brain shows development of bilateral subdural hematohygromata following shunting of severe obstructive HCP.](images/app.statdx.com_image_thumbnail_9b51a0ae-8574-43c8-ad66-c9d88ace4ebb_annotated_true_size_900_quality_90_135d3f47_20251018T164503Z.jpg) +*Axial NECT of the brain shows development of bilateral subdural hematohygromata following shunting of severe obstructive HCP.* + +![Sagittal T1 C+ MR of a patient with intracranial hypotension shows obliteration of the suprasellar cistern, sagging fat midbrain with a closed angle between peduncles/pons , dural enhancement, and tonsillar descent.](images/app.statdx.com_image_thumbnail_fe076dec-96fa-4fe3-97cd-3c71abd81c09_annotated_true_size_900_quality_90_db1ac179_20251018T164503Z.jpg) +*Sagittal T1 C+ MR of a patient with intracranial hypotension shows obliteration of the suprasellar cistern, sagging fat midbrain with a closed angle between peduncles/pons , dural enhancement, and tonsillar descent.* + +![AP radiography in patient with a VP shunt and acute shunt failure reveals fractured shunt tubing .](images/app.statdx.com_image_thumbnail_32aa4174-806c-4239-819d-49f88f817fa0_annotated_true_size_900_quality_90_8ab2e3f8_20251018T164503Z.jpg) +*AP radiography in patient with a VP shunt and acute shunt failure reveals fractured shunt tubing .* + +![AP radiography shows a disconnected and caudally migrated peritoneal shunt catheter fragment looped within the pelvis.](images/app.statdx.com_image_thumbnail_0680af81-ef70-4c08-b3a5-c025ba031a43_annotated_true_size_900_quality_90_128e6046_20251018T164503Z.jpg) +*AP radiography shows a disconnected and caudally migrated peritoneal shunt catheter fragment looped within the pelvis.* + +![AP radiography shows knotted abdominal shunt tubing. The tightly coiled appearance suggests an abnormal extraperitoneal placement of the shunt. Two shunts are present due to isolated ventricles.](images/app.statdx.com_image_thumbnail_0567b9fb-c5c2-4c12-8a0d-5af29bc02aaa_annotated_true_size_900_quality_90_d05e5b19_20251018T164504Z.jpg) +*AP radiography shows knotted abdominal shunt tubing. The tightly coiled appearance suggests an abnormal extraperitoneal placement of the shunt. Two shunts are present due to isolated ventricles.* + diff --git a/out/extraventricular-obstructive-hydrocephalus_a0886d4c-f504-4165-bb52-2400e2385f68.md b/out/extraventricular-obstructive-hydrocephalus_a0886d4c-f504-4165-bb52-2400e2385f68.md new file mode 100644 index 0000000..d772c58 --- /dev/null +++ b/out/extraventricular-obstructive-hydrocephalus_a0886d4c-f504-4165-bb52-2400e2385f68.md @@ -0,0 +1,301 @@ +--- +title: "Extraventricular Obstructive Hydrocephalus" +docid: "a0886d4c-f504-4165-bb52-2400e2385f68" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "Extraventricular Obstructive Hydrocephalus" + slug: "extraventricular-obstructive-hydro-" + treeNodeId: null +category: "Brain" +documentVersionId: "244d0107-0a80-48b5-96b6-e095c226c02a" +imageCount: 9 +lastUpdated: "08/20/20" +pageDescription: "Extraventricular Obstructive Hydrocephalus" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Extraventricular Obstructive Hydrocephalus" +pageTitle: "Extraventricular Obstructive Hydrocephalus | STATdx" +enhancedTitle: "Extraventricular Obstructive Hydrocephalus" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "Extraventricular Obstructive Hydrocephalus" +--- +# KEY FACTS + +- ## Terminology + + + - Extraventricular obstructive hydrocephalus (EVOH): Enlarged ventricles due to obstruction located outside ventricular system + - Synonym: "Communicating" hydrocephalus +- ## Imaging + + + - Impaired absorption of CSF distal to 4th ventricle outlet foramina + - Ventricular size varies with duration of obstruction + - All ventricles enlarged with no intraventricular obstructive cause + - Lateral, 3rd, and 4th ventricles dilated + - ± periventricular white matter interstitial edema + - ± abnormal density/intensity of cisternal CSF ± leptomeningeal enhancement +- ## Top Differential Diagnoses + + + - Intraventricular obstructive hydrocephalus + - Ventricular enlargement 2° to parenchymal loss + - Normal-pressure hydrocephalus +- ## Pathology + + + - Hemorrhage → fibrosis/obstruction of subarachnoid space + - Most common cause of EVOH + - Other etiologies include suppurative meningitis, neoplastic or inflammatory exudates + - Subarachnoid hemorrhage (SAH), exudates may fibrose/occlude subarachnoid space, reduce CSF pulsations +- ## Clinical Issues + + + - Headache, papilledema + - Nausea, vomiting, diplopia (cranial nerve palsy) +- ## Diagnostic Checklist + + + - EVOH: Generalized ventricular enlargement with abnormal density/intensity in basal cisterns ± leptomeningeal enhancement + +# TERMINOLOGY + +- ## Abbreviations + + + - Extraventricular obstructive hydrocephalus (EVOH) +- ## Synonyms + + + - "Communicating" hydrocephalus +- ## Definitions + + + - Enlarged ventricles due to obstruction located outside ventricular system + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Lateral, 3rd, and 4th ventricles all dilated + - ± abnormal density/intensity of cisternal CSF ± leptomeningeal enhancement + - ### Location + + + - Obstruction distal to 4th ventricle outlet foramina + - ### Size + + + - Bifrontal horn to intracranial diameter ratio > 0.3 + - Temporal horn width > 3 mm + - ### Morphology + + + - All ventricles enlarged + - Generally proportionate, symmetrical increase + - No intraventricular obstructive cause +- ## CT Findings + + + - ### NECT + + + - Variable ventricular dilatation ± basal cisterns effaced + - If subarachnoid hemorrhage (SAH), look for hyperdense CSF + - ### CECT + + + - Look for sulcal/cisternal enhancement +- ## MR Findings + + + - ### T1WI + + + - "Dirty" CSF, ventricular dilatation + - ### T2WI + + + - Dilated ventricles ± periventricular white matter interstitial edema + - Effacement of cortical sulci + - Hypointense CSF-SAH, exudates + - ### FLAIR + + + - ± periventricular white matter interstitial edema better delineated than T2 + - ### T1WI C+ + + + - ± enhancement of basal cisterns/sulci + - Meningitis, carcinomatosis, etc. + - 3D CISS/FIESTA + - Exquisitely delineates CSF spaces and helps to exclude intraventricular obstruction +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR with T1WI C+ + - 3D CISS/FIESTA +- ## Other Modality Findings + + + - Isotope cisternography may show ventricular reflux, stasis (EVOH) + +# DIFFERENTIAL DIAGNOSIS + +- ## Intraventricular Obstructive Hydrocephalus + + + - Global/focal enlarged ventricles due to obstruction proximal to 4th ventricle outflow +- ## Ventricular Enlargement Secondary to Parenchymal Loss + + + - Neurodegenerative disease, cerebritis, hypoxia/ischemia + - Diffuse/focal enlargement of sulci, cisterns +- [Normal-Pressure Hydrocephalus](/document/normal-pressure-hydrocephalus/ba3f857d-58de-4f21-8463-1631b4cb9972) + - Ventricular enlargement with normal CSF pressure + - Sulci normal/minimally enlarged + - Progressive dementia, gait disturbance, incontinence + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Obstruction to CSF flow at level of basal cisterns or arachnoid villi + - Also reduced CSF pulsations reduce venous resorption of CSF + - SAH: Most common cause of EVOH + - Other etiologies include suppurative meningitis, neoplastic inflammatory exudates + - All lead to subarachnoid scarring, reduced CSF pulsations +- ## Gross Pathologic & Surgical Features + + + - Generalized ventricular dilatation + - SAH, exudates in basal cisterns & convexity sulci + - Meningeal fibrosis, arachnoid adhesions + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Headache, papilledema + - Nausea, vomiting, diplopia (cranial nerve palsy) +- ## Natural History & Prognosis + + + - Usually progressive unless shunted and primary cause treated +- ## Treatment + + + - CSF diversion (shunt); directed to primary cause + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - EVOH: Generalized ventricular enlargement with abnormal density/intensity in basal cisterns ± leptomeningeal enhancement + + 94b03593-3810-49dd-a698-ad9f46f7ba1f + +## References + +# Selected References + +1. [Capone PM et al: Neuroimaging of normal pressure hydrocephalus and hydrocephalus. Neurol Clin. 38(1):171-83, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31761057%5Bpmid%5D) +1. [Farb R et al: Hydrocephalus and CSF disorders. Diseases of the brain, head and neck, spine 2020-3: Diagnostic Imaging. Cham (CH): Springer; 11-24, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32119247%5Bpmid%5D) +1. [Fowler JB et al: Ventriculoperitoneal shunt. Treasure Island (FL): StatPearls Publishing, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=29083724%5Bpmid%5D) +1. [Roth J et al: The added value of magnetic resonance imaging cisternography and ventriculography as a diagnostic aid in pediatric Hydrocephalus. Pediatr Neurosurg. 54(3):165-72, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30870836%5Bpmid%5D) +1. [Fink KR et al: Imaging of nontraumatic neuroradiology emergencies. Radiol Clin North Am. 53(4):871-90, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26046515%5Bpmid%5D) +1. [Flannery AM et al: Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 1: introduction and methodology. J Neurosurg Pediatr. 14 Suppl 1:3-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25988777%5Bpmid%5D) +1. [Grunwald IQ et al: Aneurysmal SAH: current management and complications associated with treatment and disease. J Invasive Cardiol. 26(1):30-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24402809%5Bpmid%5D) +1. [Krähenbühl AK et al: Endoscopic temporal ventriculocisternostomy: an option for the treatment of trapped temporal horns. J Neurosurg Pediatr. 11(5):568-74, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23521153%5Bpmid%5D) +1. [McAllister JP 2nd: Pathophysiology of congenital and neonatal hydrocephalus. Semin Fetal Neonatal Med. 17(5):285-94, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22800608%5Bpmid%5D) +1. [Feng F et al: Evaluation of radionuclide cerebrospinal fluid scintigraphy as a guide in the management of patients with hydrocephalus. Clin Imaging. 33(2):85-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19237049%5Bpmid%5D) +1. [Yamada S et al: Visualization of cerebrospinal fluid movement with spin labeling at MR imaging: preliminary results in normal and pathophysiologic conditions. Radiology. 249(2):644-52, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18936318%5Bpmid%5D) +1. [Greitz D: Paradigm shift in hydrocephalus research in legacy of Dandy's pioneering work: rationale for third ventriculostomy in communicating hydrocephalus. Childs Nerv Syst. 23(5):487-9, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=-1%5Bpmid%5D) +1. [ter Laan M et al: Improvement after treatment of hydrocephalus in aneurysmal subarachnoid haemorrhage: implications for grading and prognosis. Acta Neurochir (Wien). 148(3):325-8; discussion 328, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16328775%5Bpmid%5D) +1. [Greitz D: Radiological assessment of hydrocephalus: new theories and implications for therapy. Neurosurg Rev. 27(3):145-65; discussion 166-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15164255%5Bpmid%5D) +1. [Kehler U et al: Extraventricular intracisternal obstructive hydrocephalus--a hypothesis to explain successful 3rd ventriculostomy in communicating hydrocephalus. Pediatr Neurosurg. 38(2):98-101, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12566844%5Bpmid%5D) +1. [Biedert S et al: [Extraventricular obstructive hydrocephalus.] Fortschr Neurol Psychiatr. 62(11):405-8, 1994](http://www.ncbi.nlm.nih.gov/pubmed/?term=7829027%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and early extraventricular obstructive hydrocephalus with dilatation of all the ventricles and subtle periventricular hypodensity due to interstitial edema.](images/app.statdx.com_image_thumbnail_5111b0b3-10b0-4451-b80f-787287add3e4_annotated_true_size_900_quality_90_52e5b3da_20251018T164521Z.jpg) +*Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and early extraventricular obstructive hydrocephalus with dilatation of all the ventricles and subtle periventricular hypodensity due to interstitial edema.* + +![Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and early extraventricular obstructive hydrocephalus with dilatation of all the ventricles and subtle periventricular hypodensity due to interstitial edema.](images/app.statdx.com_image_thumbnail_5111b0b3-10b0-4451-b80f-787287add3e4_size_174_quality_85_07f2e641_20251018T155131Z.jpg) +*Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and early extraventricular obstructive hydrocephalus with dilatation of all the ventricles and subtle periventricular hypodensity due to interstitial edema.* + +![Axial FLAIR MR images (top) in a patient with breast carcinoma leptomeningeal metastasis shows dilatation of the ventricles with mild periventricular interstitial edema . Axial T1 C+ MR (bottom) shows extensive leptomeningeal enhancement along the cerebellar folia.](images/app.statdx.com_image_thumbnail_d998cd4d-9969-40a5-af8f-4530c1fa1cf0_annotated_true_size_900_quality_90_19eb7d4c_20251018T164521Z.jpg) +*Axial FLAIR MR images (top) in a patient with breast carcinoma leptomeningeal metastasis shows dilatation of the ventricles with mild periventricular interstitial edema . Axial T1 C+ MR (bottom) shows extensive leptomeningeal enhancement along the cerebellar folia.* + +![Axial T2 MR in a 21-year-old patient with a remote history of meningitis shows chronic "compensated" extraventricular communicating hydrocephalus with marked dilatation of the lateral and 3rd ventricles .](images/app.statdx.com_image_thumbnail_99b1acc6-b089-48c5-bc97-c72d4bd09eb0_annotated_true_size_900_quality_90_dc39d287_20251018T164521Z.jpg) +*Axial T2 MR in a 21-year-old patient with a remote history of meningitis shows chronic "compensated" extraventricular communicating hydrocephalus with marked dilatation of the lateral and 3rd ventricles .* + +![Sagittal T1 MR in the same patient shows a patent widened cerebral aqueduct and foramen of Magendie with dilatation of the 4th ventricle . In longstanding "compensated" hydrocephalus, there is no periventricular interstitial edema around the ventricles, as in this case.](images/app.statdx.com_image_thumbnail_92b29be1-65a5-4179-9175-265ac3a16e7b_annotated_true_size_900_quality_90_3b9fecbf_20251018T164521Z.jpg) +*Sagittal T1 MR in the same patient shows a patent widened cerebral aqueduct and foramen of Magendie with dilatation of the 4th ventricle . In longstanding "compensated" hydrocephalus, there is no periventricular interstitial edema around the ventricles, as in this case.* + + +### Additional Images + +![Axial T1WI C+ MR demonstrates subtle leptomeningeal enhancement in the left sylvian fissure in this patient with tuberculous meningitis. There is mild dilatation of the lateral ventricles due to extraventricular obstructive hydrocephalus.](images/app.statdx.com_image_thumbnail_2a43dda8-e5ae-43ec-94c6-0f57c803a451_annotated_true_size_900_quality_90_1b106673_20251018T164521Z.jpg) +*Axial T1WI C+ MR demonstrates subtle leptomeningeal enhancement in the left sylvian fissure in this patient with tuberculous meningitis. There is mild dilatation of the lateral ventricles due to extraventricular obstructive hydrocephalus.* + +![Coronal T1WI MR in a toddler with rapid head growth for 4 months shows enlarged ventricular trigone on the left and enlarging subarachnoid spaces at an age when they should be shrinking. MR venography showed occlusion of both transverse sinuses.](images/app.statdx.com_image_thumbnail_ebff970c-aa3e-4179-bbd9-c97acd72f056_annotated_true_size_900_quality_90_c2d1d8f1_20251018T164521Z.jpg) +*Coronal T1WI MR in a toddler with rapid head growth for 4 months shows enlarged ventricular trigone on the left and enlarging subarachnoid spaces at an age when they should be shrinking. MR venography showed occlusion of both transverse sinuses.* + +![Axial NECT shows hyperdense material in the basal cisterns and sylvian fissures in acute subarachnoid hemorrhage. There is early dilatation of the ventricles with mild periventricular edema due to interstitial edema.](images/app.statdx.com_image_thumbnail_8ec2b8fa-6b76-4d89-b2ab-6f1f7e0a4d3c_annotated_true_size_900_quality_90_9e79e2bf_20251018T164521Z.jpg) +*Axial NECT shows hyperdense material in the basal cisterns and sylvian fissures in acute subarachnoid hemorrhage. There is early dilatation of the ventricles with mild periventricular edema due to interstitial edema.* + +![Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and sylvian fissures . There is early extraventricular obstructive hydrocephalus with mild periventricular hypodensity due to interstitial edema.](images/app.statdx.com_image_thumbnail_da3cea4c-f283-4f12-b661-5b1c2df2b201_annotated_true_size_900_quality_90_8c48b911_20251018T164521Z.jpg) +*Axial NECT shows acute subarachnoid hemorrhage in the basal cisterns and sylvian fissures . There is early extraventricular obstructive hydrocephalus with mild periventricular hypodensity due to interstitial edema.* + +![Axial T1WI C+ MR shows extensive leptomeningeal enhancement of the basal cisterns in neurosarcoidosis . Notice the early communicating hydrocephalus with the dilated 3rd ventricle and temporal horns .](f314f067-37d3-4bfe-b551-1dd1cd36f1e3) +*Axial T1WI C+ MR shows extensive leptomeningeal enhancement of the basal cisterns in neurosarcoidosis . Notice the early communicating hydrocephalus with the dilated 3rd ventricle and temporal horns .* + diff --git a/out/guillain-barr-spectrum-disorders_c1f52a65-920e-4e28-8a75-07dfa208f290.md b/out/guillain-barr-spectrum-disorders_c1f52a65-920e-4e28-8a75-07dfa208f290.md deleted file mode 100644 index f533a82..0000000 --- a/out/guillain-barr-spectrum-disorders_c1f52a65-920e-4e28-8a75-07dfa208f290.md +++ /dev/null @@ -1,320 +0,0 @@ ---- -title: "Guillain-Barr\u00e9 Spectrum Disorders" -docid: "c1f52a65-920e-4e28-8a75-07dfa208f290" -authors: - - key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607" - value: "Surjith Vattoth, MD, FRCR" -breadcrumbs: - - - name: "Brain" - slug: "brain" - treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" - - - name: "Diagnosis" - slug: "diagnosis" - treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" - - - name: "Pathology-Based Diagnoses" - slug: "pathology-based-diagnoses" - treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" - - - name: "Infectious, Inflammatory, and Demyelinating Disease" - slug: "infectious-inflammatory-and-demyel-" - treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607" - - - name: "Inflammatory and Demyelinating Disease" - slug: "inflammatory-and-demyelinating-dis-" - treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc" - - - name: "Guillain-Barr\u00e9 Spectrum Disorders" - slug: "guillain-barr-spectrum-disorders" - treeNodeId: null -category: "Brain" -documentVersionId: "52016b28-7710-43a4-8cca-e659ab8227cf" -imageCount: 5 -lastUpdated: "06/12/20" -pageDescription: "Guillain-Barr\u00e9 Spectrum Disorders" -pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Guillain-Barr\u00e9 Spectrum Disorders" -pageTitle: "Guillain-Barr\u00e9 Spectrum Disorders | STATdx" -enhancedTitle: "Guillain-Barr\u00e9 Spectrum Disorders" -type: "DX" -references: true -breadcrumbs: - - "Brain" - - "Diagnosis" - - "Pathology-Based Diagnoses" - - "Infectious, Inflammatory, and Demyelinating Disease" - - "Inflammatory and Demyelinating Disease" - - "Guillain-Barr\u00e9 Spectrum Disorders" ---- -# KEY FACTS - -- ## Terminology - - - - Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS), Bickerstaff brainstem encephalitis (BBE) - - **Classic GBS**: Acute-onset, rapidly progressive, ascending sensorimotor neuropathy; absent or ↓ tendon reflexes - - **Clinical variants of GBS**: Pure motor, paraparetic, bilateral facial palsy with paraesthesias, pharyngeal-cervical-brachial (PCB) variants and MFS - - **Other disorders often included in GBS spectrum** (controversial): Pure sensory variant, pure sensory ataxia, BBE -- ## Imaging - - - - Cauda equina (CE) nerve root enhancement in > 80% - - Isolated ventral CE in 17%, both ventral and dorsal in 65% - - Enhancement intensity of ventral CE ≥ dorsal CE - - Pial surface of distal cord/conus variable enhancement - - Pediatric GBS and MFS: CN enhancement in > 80%  - - CNIII, CNVI, CNVII most common; others like CNII (and optic chiasm), CNV, CNXI, CNXII - - BBE: Brainstem T2 hyperintensities, may involve cerebellar peduncles and cerebellum -- ## Top Differential Diagnoses - - - - Subacute or chronic demyelinating polyneuropathies (SIDP/CIDP) - - CSF metastatic seeding, infections, arachnoiditis -- ## Pathology - - - - 6 temporally associated pathogens: *Campylobacter jejuni*, Cytomegalovirus (CMV), Hepatitis E virus, *Mycoplasma pneumoniae*, Epstein Barr virus (EBV), and Zika virus -- ## Clinical Issues - - - - Motor weakness and sensory signs in legs progressing to upper limbs and cranial muscles - - GBS spectrum/clinical variants with various findings - - Serum anti-GQ1b antibodies in up to 90% of MFS, 70% of BBE, and 8% of classic GBS - - Anti-GM1 antibodies more characteristic of classic GBS - - Intravenous immunoglobulins and plasma exchange therapy -- ## Diagnostic Checklist - - - - Consider GBS, variants or overlap syndromes in MR with ventral > dorsal cauda equina nerve root enhancement - - Look for cranial nerve root enhancement in MFS - - Look for brainstem signal abnormalities in BBE - -# TERMINOLOGY - -- ## Abbreviations - - - - Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS), Bickerstaff brainstem encephalitis (BBE) - - Acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN) -- ## Definitions - - - - Immune-mediated peripheral nerves and nerve roots disorder, usually triggered by infections - - **Classic GBS**: Acute-onset, rapidly progressive, ascending sensorimotor neuropathy; absent or ↓ tendon reflexes - - Initially in legs, progressing to arms and cranial muscles - - Usually reach maximum disability within 2 weeks - - **Electrophysiological subtypes**: **AIDP, AMAN, AMSAN** - - **Clinical variants of GBS**: Do not characteristically progress to classic GBS pattern of sensory loss and weakness - - Usually not "pure"; often partial overlap with classic GBS or other variants - - **Pure motor variant**: Motor weakness; no sensory signs - - **Paraparetic variant**: Paresis in lower limbs only - - **Bilateral facial palsy with paraesthesias variant**: Weakness limited to cranial nerves; ↓ reflexes - - **Pharyngeal-cervical-brachial (PCB) variant**: PCB muscle weakness, no lower limb weakness - - **MFS**: Ophthalmoplegia, areflexia, ataxia - - 15% overlap classic GBS - - Incomplete forms: **Acute ataxic neuropathy** (isolated ataxia); **acute ophthalmoplegia** - - **Other disorders often included in GBS spectrum**:****Due to similar clinical or pathophysiological features - - Inclusion in GBS spectrum debatable as these do not fulfil GBS diagnostic criteria - - **Pure sensory variant**: Overlapping clinical features of classic GBS, except motor signs and symptoms - - **Pure sensory ataxia**: Overlap with MFS - - **BBE**: Initially ophthalmoplegia, ataxia, areflexia (like MFS), later brainstem dysfunction, e.g., ↓ consciousness and pyramidal tract signs; often overlap classic GBS - -# IMAGING - -- ## General Features - - - - ### Best diagnostic clue - - - - Cauda equina (CE) nerve root enhancement > 80% - - Isolated ventral CE 17%, both ventral and dorsal 65% - - Enhancement intensity of ventral CE ≥ dorsal CE - - Pial surface of distal cord/conus variable enhancement - - Pediatric GBS and MFS: Cranial nerve enhancement > 80%  - - CNIII, CNVI, CNVII most common; others like CNII (and optic chiasm), CNV, CNXI, CNXII - - Entire spinal cord T2-hyperintense posterior column described as delayed-onset finding in MFS case - - BBE: Brainstem T2 hyperintensities, may involve cerebellar peduncles and cerebellum - - Enhance only rarely; extremely rare, patchy spinal cord involvement described -- ## MR Findings - - - - ### T2WI - - - - May see slight nerve root thickening - - Brainstem hyperintensities in BBE - - ### T1WI C+ - - - - Ventral > dorsal nerve root enhancement - - Cranial nerve enhancement -- ## Ultrasonographic Findings - - - - Enlarged nerves/roots during first 3 weeks, improve later -- ## Imaging Recommendations - - - - ### Best imaging tool - - - - MR of lumbar spine with contrast - - MR of brain with contrast if MFS or BBE suspected - -# DIFFERENTIAL DIAGNOSIS - -- ## Infections - - - - Bacterial or granulomatous meningitis - - Nerve root enhancement in minority of enterovirus D68 acute flaccid myelitis -- ## CSF Metastatic Seeding - - - - Nodular nerve root thickening and enhancement -- ## Subacute or Chronic Demyelinating Polyneuropathies - - - - Slower onset, protracted course - - CIDP: Nadir 2 months; SIDP: 4-8 weeks -- ## Arachnoiditis - - - - Secondary to hemorrhage, surgery, chemicals, chemotherapy (like vincristine), radiation -- ## Vasculitic Neuropathy - - - - Polyarteritis nodosa, Churg-Strauss syndrome - -# PATHOLOGY - -- ## General Features - - - - Infection in 6 weeks prior to onset in 2/3; triggering immune response causing GBS - - 6 temporally associated pathogens: *Campylobacter jejuni*, Cytomegalovirus (CMV), Hepatitis E virus, *Mycoplasma pneumoniae*, Epstein Barr virus (EBV), and Zika virus - - Absence of preceding illness does not exclude GBS  - - Infections and other immunological stimuli (immunobiologicals like tumour necrosis factor antagonists, immune checkpoint inhibitors or type I interferons, surgery, amlgnancy) may be subclinical - - Serum antibodies against gangliosides in axolemma and other peripheral nerve components -- ## Gross Pathologic & Surgical Features - - - - Peripheral nerve and nerve root complement activation, macrophage infiltration, and edema - -# CLINICAL ISSUES - -- ## Presentation - - - - ### Most common signs/symptoms - - - - Motor weakness and sensory signs in legs progressing to upper limbs and cranial muscles - - Low back pain common symptom - - GBS spectrum/clinical variants with various findings - - Respiratory failure and mechanical ventilation in 20% - - Autonomic nervous system involvement with cardiac arrhythmias and unstable blood pressure - - ### Other signs/symptoms - - - - Serum **anti-GQ1b** antibodies in up to 90% of MFS, 70% of BBE, and 8% of classic GBS - - **Anti-GM1** antibodies more characteristic of classic GBS - - Should not wait for antibody test results to start treatment when GBS is suspected - - Albumino-cytological dissociation: Classic GBS finding  - - ↑ CSF protein and normal CSF cell count - - Protein levels normal in 30-50% during 1st week and 10-30% during 2nd week - - Mild CSF pleocytosis (10-50 cells/μl) can be seen in GBS, but should exclude infectious polyradiculitis - - Electrodiagnostic studies not required to diagnose GBS; might be normal during 1st week, mild or variant disease, initial proximal weakness, or slow progression - - GBS: Sensorimotor polyradiculoneuropathy or polyneuropathy: ↓ conduction velocities, sensory and motor evoked amplitudes, abnormal temporal dispersion &/or partial motor conduction blocks - - GBS: Typical "sural sparing" pattern: Normal sural sensory nerve action potential, but abnormal or absent median and ulnar sensory nerve action potentials - - MFS: Normal or only ↓ amplitude of sensory nerve action potentials -- ## Demographics - - - - 1-2 per 100,000 person-years; M > F; ↑ with age -- ## Natural History & Prognosis - - - - Rapid progression, nadir within 2 weeks; mortality 3-10% - - GBS plateau phase (days to weeks or months); then recovery - - 60-80% walk independently after 6 months - - GBS typically monophasic illness, but relapses in 2-5% - - Treatment-related fluctuation (TRF): Deterioration after intial stability/improvement on therapy -- ## Treatment - - - - Intravenous immunoglobulin for 5 days (0.4 g/kg daily) - - Plasma exchange for 5 sessions  (200-250 mL/kg) - - If TRF, repeat same treatment - - If no initial response or incomplete recovery, currently no evidence to support repeat treatment - -# DIAGNOSTIC CHECKLIST - -- ## Consider - - - - GBS, variants or overlap syndromes in MR showing ventral > dorsal cauda equina nerve root enhancement -- ## Image Interpretation Pearls - - - - Look for cranial nerve root enhancement in MFS - - Look for brainstem signal abnormalities in BBE - - 5e867f2a-de7e-44dd-83cf-67453b9125d2 - -## References - -# Selected References - -1. [Al Othman B et al: Update: the Miller Fisher variants of Guillain-Barré syndrome. Curr Opin Ophthalmol. 30(6):462-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31567467%5Bpmid%5D) -1. [Leonhard SE et al: Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 15(11):671-83, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31541214%5Bpmid%5D) -1. [Malhotra A et al: MRI findings of optic pathway involvement in Miller Fisher syndrome in 3 pediatric patients and a review of the literature. J Clin Neurosci. 39:63-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28209311%5Bpmid%5D) -1. [Cuneo GL et al: An atypical Bickerstaff's brainstem encephalitis with involvement of spinal cord. Neuroradiol J. 29(5):396-9, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27540012%5Bpmid%5D) -1. [Tyrakowska Z et al: Relapsing-Remitting Severe Bickerstaff's Brainstem Encephalitis - Case Report and Literature Review. Pol J Radiol. 81:622-628, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=28096906%5Bpmid%5D) -1. [Zuccoli G et al: Redefining the Guillain-Barr√© spectrum in children: neuroimaging findings of cranial nerve involvement. AJNR Am J Neuroradiol. 32(4):639-42, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21292802%5Bpmid%5D) -1. [Inoue N et al: MR imaging findings of spinal posterior column involvement in a case of Miller Fisher syndrome. AJNR Am J Neuroradiol. 25(4):645-8, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15090361%5Bpmid%5D) - - -## Images - - -### Selected Images - -![Axial T1 C+ MR of the lumbar spine in a patient with Guillain-Barré syndrome (GBS) shows the characteristic ventral cauda equina (CE) nerve root enhancement and slight thickening.](images/app.statdx.com_image_thumbnail_437a7388-6761-4ae6-bbe8-a94c64172505_annotated_true_size_900_quality_90_f3459dbb_20251018T122501Z.jpg) -*Axial T1 C+ MR of the lumbar spine in a patient with Guillain-Barré syndrome (GBS) shows the characteristic ventral cauda equina (CE) nerve root enhancement and slight thickening.* - -![Axial T1 C+ MR of the lumbar spine in a patient with Guillain-Barré syndrome (GBS) shows the characteristic ventral cauda equina (CE) nerve root enhancement and slight thickening.](images/app.statdx.com_image_thumbnail_437a7388-6761-4ae6-bbe8-a94c64172505_size_168_quality_85_3b1ee2d9_20251018T095312Z.jpg) -*Axial T1 C+ MR of the lumbar spine in a patient with Guillain-Barré syndrome (GBS) shows the characteristic ventral cauda equina (CE) nerve root enhancement and slight thickening.* - -![Axial T1 C+ MR of the lumbar spine in a patient with Guillain-Barré syndrome (GBS) shows the characteristic ventral cauda equina (CE) nerve root enhancement and slight thickening.](images/app.statdx.com_image_thumbnail_437a7388-6761-4ae6-bbe8-a94c64172505_size_174_quality_85_f5af7cdc_20251018T122441Z.jpg) -*Axial T1 C+ MR of the lumbar spine in a patient with Guillain-Barré syndrome (GBS) shows the characteristic ventral cauda equina (CE) nerve root enhancement and slight thickening.* - -![Sagittal T1 C+ FS MR of the lumbar spine in a patient with GBS shows CE nerve root enhancement, more intense in ventral than dorsal CE. Also note enhancement of the pial surface of the distal cord/conus . GBS is an immune-mediated peripheral nerves and nerve roots disorder, usually triggered by infections.](images/app.statdx.com_image_thumbnail_5d50656f-8e56-40a4-9ad8-54778d94348f_annotated_true_size_900_quality_90_e8431c7b_20251018T122501Z.jpg) -*Sagittal T1 C+ FS MR of the lumbar spine in a patient with GBS shows CE nerve root enhancement, more intense in ventral than dorsal CE. Also note enhancement of the pial surface of the distal cord/conus . GBS is an immune-mediated peripheral nerves and nerve roots disorder, usually triggered by infections.* - -![Sagittal T1 C+ FS MR of the lumbar spine in a patient with GBS shows CE nerve root enhancement, more intense in ventral than dorsal CE. Also note enhancement of the pial surface of the distal cord/conus . GBS is an immune-mediated peripheral nerves and nerve roots disorder, usually triggered by infections.](images/app.statdx.com_image_thumbnail_5d50656f-8e56-40a4-9ad8-54778d94348f_size_168_quality_85_f640ff58_20251018T095312Z.jpg) -*Sagittal T1 C+ FS MR of the lumbar spine in a patient with GBS shows CE nerve root enhancement, more intense in ventral than dorsal CE. Also note enhancement of the pial surface of the distal cord/conus . GBS is an immune-mediated peripheral nerves and nerve roots disorder, usually triggered by infections.* - -![Coronal angled T1 C+ MPRAGE MR reformat in a patient with Miller Fisher syndrome (MFS) shows mild thickening and enhancement of right facial (CNVII) and bilateral trigeminal (CNV) nerves.](images/app.statdx.com_image_thumbnail_121ccea5-3b17-4960-8fad-5e03c2574112_annotated_true_size_900_quality_90_e49fc831_20251018T122501Z.jpg) -*Coronal angled T1 C+ MPRAGE MR reformat in a patient with Miller Fisher syndrome (MFS) shows mild thickening and enhancement of right facial (CNVII) and bilateral trigeminal (CNV) nerves.* - -![Coronal angled T1 C+ MPRAGE MR reformat in a patient with Miller Fisher syndrome (MFS) shows mild thickening and enhancement of right facial (CNVII) and bilateral trigeminal (CNV) nerves.](images/app.statdx.com_image_thumbnail_121ccea5-3b17-4960-8fad-5e03c2574112_size_168_quality_85_55630496_20251018T095312Z.jpg) -*Coronal angled T1 C+ MPRAGE MR reformat in a patient with Miller Fisher syndrome (MFS) shows mild thickening and enhancement of right facial (CNVII) and bilateral trigeminal (CNV) nerves.* - -![Axial FLAIR MR in a patient with BBE shows hyperintense signal in the pons , middle cerebellar peduncles , and cerebellum . Both MFS and BBE may initially show ophthalmoplegia, ataxia, and areflexia, the differentiating feature being reduced consciousness and other brainstem signs in BBE later.](images/app.statdx.com_image_thumbnail_c7553e93-a863-4cb6-b052-a0e2437db982_annotated_true_size_900_quality_90_d0dfcf0d_20251018T122501Z.jpg) -*Axial FLAIR MR in a patient with BBE shows hyperintense signal in the pons , middle cerebellar peduncles , and cerebellum . Both MFS and BBE may initially show ophthalmoplegia, ataxia, and areflexia, the differentiating feature being reduced consciousness and other brainstem signs in BBE later.* - -![Axial FLAIR MR in a patient with BBE shows hyperintense signal in the pons , middle cerebellar peduncles , and cerebellum . Both MFS and BBE may initially show ophthalmoplegia, ataxia, and areflexia, the differentiating feature being reduced consciousness and other brainstem signs in BBE later.](images/app.statdx.com_image_thumbnail_c7553e93-a863-4cb6-b052-a0e2437db982_size_168_quality_85_2af9fcb7_20251018T095312Z.jpg) -*Axial FLAIR MR in a patient with BBE shows hyperintense signal in the pons , middle cerebellar peduncles , and cerebellum . Both MFS and BBE may initially show ophthalmoplegia, ataxia, and areflexia, the differentiating feature being reduced consciousness and other brainstem signs in BBE later.* - - -### Additional Images - -![Axial FLAIR MR in a patient with Bickerstaff brainstem encephalitis (BBE) shows abnormal hyperintense signal in the pons . Both MFS and BBE initially present with ophthalmoplegia, ataxia and areflexia; the differentiating feature being development of brainstem dysfunction in BBE later (reduced consciousness and pyramidal tract signs).](images/app.statdx.com_image_thumbnail_62a77d2a-4a78-448b-891c-9f5482c408c9_annotated_true_size_900_quality_90_ee755e68_20251018T122501Z.jpg) -*Axial FLAIR MR in a patient with Bickerstaff brainstem encephalitis (BBE) shows abnormal hyperintense signal in the pons . Both MFS and BBE initially present with ophthalmoplegia, ataxia and areflexia; the differentiating feature being development of brainstem dysfunction in BBE later (reduced consciousness and pyramidal tract signs).* - -![Axial FLAIR MR in a patient with Bickerstaff brainstem encephalitis (BBE) shows abnormal hyperintense signal in the pons . Both MFS and BBE initially present with ophthalmoplegia, ataxia and areflexia; the differentiating feature being development of brainstem dysfunction in BBE later (reduced consciousness and pyramidal tract signs).](images/app.statdx.com_image_thumbnail_62a77d2a-4a78-448b-891c-9f5482c408c9_size_168_quality_85_09feb623_20251018T095312Z.jpg) -*Axial FLAIR MR in a patient with Bickerstaff brainstem encephalitis (BBE) shows abnormal hyperintense signal in the pons . Both MFS and BBE initially present with ophthalmoplegia, ataxia and areflexia; the differentiating feature being development of brainstem dysfunction in BBE later (reduced consciousness and pyramidal tract signs).* - diff --git a/out/hydrocephalus_e9481739-278e-4682-ab1e-4326a77c3d0c.md b/out/hydrocephalus_e9481739-278e-4682-ab1e-4326a77c3d0c.md new file mode 100644 index 0000000..5a1c664 --- /dev/null +++ b/out/hydrocephalus_e9481739-278e-4682-ab1e-4326a77c3d0c.md @@ -0,0 +1,480 @@ +--- +title: "Hydrocephalus" +docid: "e9481739-278e-4682-ab1e-4326a77c3d0c" +authors: + - key: "838e1722-2479-4fbd-a5fe-d965980a1a2c" + value: "Blaise V. Jones, MD" +breadcrumbs: + - + name: "Pediatrics" + slug: "pediatrics" + treeNodeId: "a915965c-d436-44cf-ae65-2f22e7246ea4" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "2b5cea64-a083-489e-ac0c-ec14ba059026" + - + name: "Pediatric Neuroradiology" + slug: "pediatric-neuroradiology" + treeNodeId: "d0eb8f4a-e769-43dd-896c-8c9c27ce8759" + - + name: "Brain" + slug: "brain" + treeNodeId: "feaaadba-649b-4f0a-9aad-9188a8f9926a" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "0c0853dc-8217-425b-86d1-2d958e17f1f9" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "8b535c75-9cd3-445e-a4c7-345b2e444f03" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: null +category: "Pediatrics" +cmeTopicId: "bddbd7cd-39c8-4b51-98d3-3e314108c4d1" +documentVersionId: "47d2ee1e-6049-4ddc-8280-fc06e5d281da" +imageCount: 25 +lastUpdated: "02/06/24" +pageDescription: "Hydrocephalus" +pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus" +pageTitle: "Hydrocephalus | STATdx" +enhancedTitle: "Hydrocephalus" +type: "DX" +references: true +breadcrumbs: + - "Pediatrics" + - "Diagnosis" + - "Pediatric Neuroradiology" + - "Brain" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" +--- +# KEY FACTS + +- ## Terminology + + + - Ventriculomegaly caused by + - Obstruction of CSF egress from ventricles: Intraventricular obstructive hydrocephalus + - Decreased CSF resorption from subarachnoid space: Extraventricular obstructive hydrocephalus + - Increased CSF production + - Use FOHR to track ventricle size over time + - (Transverse diameter of frontal horns + transverse diameter of occipital horns) ÷ 2x transverse diameter of cranium +- ## Imaging + + + - 3D FIESTA/CISS + - Acquire in sagittal plane to outline aqueduct + - Limited shunt MR protocol + - 30-second acquisition in each plane + - Useful substitute for CT + - Insensitive for parenchymal abnormalities +- ## Pathology + + + - Extracellular, extravascular fluid in brain is managed by glymphatic system + - CSF circulates from within ventricles → subarachnoid space → perivascular spaces ↔ interstitium ↔ perivenular spaces → subarachnoid space + - CSF is resorbed from subarachnoid space at multiple sites + - Along sheaths of cranial nerves (especially olfactory bulb) into head and neck lymphatics + - Along sheaths of spinal nerves into perispinal lymphatics + - Into meningeal (dural) lymphatics + - Into dural sinuses via arachnoid granulations + - Previously thought to be major site of resorption + - Hydrocephalus results from obstruction of CSF egress from ventricular system or reduced resorption from subarachnoid space + +# TERMINOLOGY + +- ## Abbreviations + + + - Intraventricular obstructive hydrocephalus (IVOH) + - Acute IVOH (aIVOH) + - Chronic "compensated" IVOH (cIVOH) + - Extraventricular obstructive hydrocephalus (EVOH) + - Frontal occipital horn ratio (FOHR) + - (Transverse diameter of frontal horns + transverse diameter of occipital horns) ÷ 2x transverse diameter of cranium + - Subarachnoid lymphatic-like membrane (SLYM) + - Recently discovered 4th meningeal membrane that divides subarachnoid space into inner and outer compartments +- ## Definitions + + + - Ventriculomegaly caused by + - Obstruction of CSF egress from ventricles: IVOH + - Decreased CSF resorption from subarachnoid space: EVOH + - Increased CSF production + - Ventriculomegaly secondary to loss of parenchyma, a.k.a. ex vacuo dilation, is **not**hydrocephalus + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Enlarged ventricles + - With decreased extraaxial spaces (EAS): IVOH + - With periventricular (transependymal) edema: Acute IVOH + - With enlarged EAS: EVOH + - ### Size + + + - FOHR > 0.33 + - Temporal horn width > 3 mm + - ### Morphology + + + - Ventricles proximal to obstruction enlarge, appear more rounded + - Trigones and occipital horns typically enlarge most + - Wall pressure is proportional to diameter: Laplace law + - Optic and infundibular recesses of 3rd ventricle may preferentially enlarge + - Optic nerve sheaths may enlarge → papilledema +- ## CT Findings + + + - ### NECT + + + - Large ventricles proximal to obstruction + - aIVOH + - "Ballooned" ventricles with periventricular low-density "halo" + - cIVOH + - "Ballooned" ventricles, periventricular "halo" + - Basal cisterns, sulci compressed/obliterated +- ## MR Findings + + + - ### T1WI + + + - Lateral ventricles enlarged + - Corpus callosum (CC) thinned, stretched upward + - May be impinged against falx + - Impaction may cause pressure necrosis + - Fornix, internal cerebral veins (ICV) displaced downward + - Enlarged 3rd ventricle often herniated into expanded sella + - Funnel-shaped aqueduct of Sylvius in aqueductal stenosis + - ### T2WI + + + - Acute obstruction + - Transependymal edema extends into periventricular white matter (WM) + - Accentuated around frontal and occipital horns + - Chronic obstruction + - Large ventricles without transependymal edema + - CC may show hyperintensity after decompression (15% of shunted IVOH cases) + - ### T1WI C+ + + + - Diffuse leptomeningeal disease can cause EVOH + - May only be apparent on postcontrast imaging + - ### MRS + + + - Small lactate resonances can be detected in up to 20% of CSF spaces, even if no hydrocephalus +- ## Other Modality Findings + + + - Contrast-enhanced ventriculography + - MR/CT used to identify site of obstruction, status of 3rd ventriculostomies + - MR can be used for assessing CSF flow + - Cardiac-gated phase-contrast MR + - May show loss of CSF flow through aqueduct + - Useful for assessing status of endoscopic 3rd ventriculostomy (ETV) +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Sagittal T2WI MR + - Administer contrast if pattern suggests EVOH and no etiology is apparent + - ### Protocol advice + + + - 3D FIESTA/CISS + - Acquire in sagittal plane to outline aqueduct + - Decreases CSF flow artifact + - Allows better delineation of ventricular contour, septa + - SWI to assess for superficial siderosis in posthemorrhagic hydrocephalus + - Limited shunt MR protocol + - Single-shot rapid technique with heavy T2 weighting + - 30-second acquisition in each plane + - Useful substitute for CT + - Reduce radiation exposure in frequently imaged population + - Rapid aquisition reduces need for sedation + - Insensitive for parenchymal abnormalities + - Use FOHR to track ventricle size over time + - Especially in younger children with open sutures + - Accounts for increase in head circumference with increase in ventricular size + +# DIFFERENTIAL DIAGNOSIS + +- ## Ventricular Enlargement Secondary to Parenchymal Loss + + + - a.k.a. ex vacuo ventriculomegaly + - Age related (ventricular volume increases 1.2-1.4 mL after 60 years) + - Ischemia/infarction, trauma, infection, toxic + - Obtuse frontal angle (> 110°) + - Associated enlargement of sulci, cisterns + - Normal lateral ventricles can be asymmetric +- ## Benign Enlargement of Subarachnoid Spaces and Ventricles + + + - a.k.a. benign macrocrania + - Seen in association with macrocephaly in infants + - Transient and self-limited + - Not associated with developmental delay + - Does not require CSF diversion + - Likely reflects relative immaturity of glymphatic system + - Diminished ability to resorb CSF as interstitial fluid compartment is shrunk by progressive myelination + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Extracellular, extravascular fluid in brain is managed by glymphatic system + - Maintains homeostatic balance between interstitial fluid compartment, cellular compartment, and intravascular compartment + - Analogous to lymphatic system in other organs + - While brain parenchyma does not have lymphatic vessels, they are present in dura mater and along sheaths of cranial nerves + - CSF circulates from within ventricles → subarachnoid space → perivascular spaces ↔ interstitium ↔ perivenular spaces → subarachnoid space + - CSF is resorbed from subarachnoid space at multiple sites + - Along sheaths of cranial nerves (especially olfactory bulb) into head and neck lymphatics + - Along sheaths of spinal nerves into perispinal lymphatics + - Into meningeal (dural) lymphatics + - Into dural sinuses via arachnoid granulations + - Previously thought to be major site of resorption + - SLYM is thought to facilitate CSF flow + - Disruption after trauma may explain reduced glymphatic flow and posttraumatic cerebral edema + - Normal CSF production = 0.2-0.35 mL/min + - Capacity of lateral, 3rd ventricles in adult = 20 mL + - Total volume of CSF in adult = 120 mL + - Hydrocephalus results from obstruction of CSF egress from ventricular system or reduced resorption from subarachnoid space + - Obstruction within ventricular system results in IVOH + - Ventricles expand, compress adjacent parenchyma; stretching may rupture/open ependymal cell junctions + - Periventricular interstitial fluid increases → myelin destruction + - Etiology depends on site + - Foramen of Monro + - Colloid cyst + - Subependymal giant cell astrocytoma in tuberous sclerosis + - 3rd ventricle + - Craniopharyngioma, hypothalamic glioma, arachnoid cyst + - Aqueduct of Sylvius + - Aqueductal stenosis, tectal glioma, pineal region tumor + - Obstruction by hemorrhage or inflammatory debris + - Enlarged vein of Galen due to arteriovenous fistula + - 4th ventricle + - Medulloblastoma, ependymoma, pilocytic astrocytoma + - Chiari, Dandy-Walker malformation, Blake pouch cyst + - Metastasis, neurocysticercosis, or meningioma can occur at multiple intraventricular locations + - Reduced resorption from subarachnoid space results in EVOH + - Subarachnoid pathology may reduce absorptive capacity + - Hemorrhage or inflammation (acute or chronic) + - Metabolic disorders may reduce resorptive capacity + - Overproduction of CSF may overwhelm ability of glymphatic system to manage and resorb CSF + - Choroid plexus papilloma or carcinoma + - Focally enlarged and hyperenhancing choroidal mass + - Choroid plexus villous hyperplasia + - Diffusely enlarged choroid + - ### Genetics + + + - Cell adhesion molecule L1 (*L1CAM*) recognized as cause of X-linked aqueductal stenosis + - Located on X chromosome (Xq28) +- ## Gross Pathologic & Surgical Features + + + - Focal/generalized ventricular enlargement + - Ependyma, adjacent WM are secondarily injured + - Variable pathology depending on causative factor + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Headache, papilledema (aIVOH) + - Nausea, vomiting + - Diplopia + - 6th nerve palsy caused by compression of cisternal segment + - ### Clinical profile + + + - Varies with etiology, severity, age of onset +- ## Demographics + + + - ### Age + + + - May be any age from in utero (congenital hydrocephalus) to adult + - ### Epidemiology + + + - Epidemiological data varies widely, depending upon etiology and type of hydrocephalus +- ## Natural History & Prognosis + + + - Usually progressive unless treated +- ## Treatment + + + - Obstructive hydrocephalus is managed surgically + - CSF diversion (shunt or ETV) + - Resection of obstructing or hypersecreting lesion + - Most common neurosurgical procedure in children = CSF shunting for hydrocephalus + - CSF diversion is typically delayed in EVOH + - Tendency to be much more shunt dependent + - Less tolerant of minor pressure changes + - Lesser degree of ventriculomegaly increases difficulty of surgery + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Longstanding aqueductal stenosis can be caused by slow-growing tectal tumor + - Compensated IVOH + - CSF function and homeostasis are far more complex than previously thought + - Free communication among anatomic/functional compartments is necessary for proper brain health +- ## Image Interpretation Pearls + + + - Size of ventricles generally correlates poorly with intracranial pressure + - Pulsatile CSF may create confusing signal intensity, even mimic intraventricular mass + + cf76a2a7-da6e-419b-9e56-481a10e5b803 + +## References + +# Selected References + +1. [Møllgård K et al: A mesothelium divides the subarachnoid space into functional compartments. Science. 379(6627):84-8, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36603070%5Bpmid%5D) +1. [Krishnan P et al: Neuroimaging in pediatric hydrocephalus. Indian J Pediatr. 86(10):952-60, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31077004%5Bpmid%5D) +1. [Ha SK et al: Magnetic resonance imaging and histopathological visualization of human dural lymphatic vessels. Bio Protoc. 8(8), 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29780855%5Bpmid%5D) +1. [Patel SK et al: Advanced neuroimaging techniques in pediatric hydrocephalus. Pediatr Neurosurg. 52(6):436-45, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28095378%5Bpmid%5D) +1. [Algin O et al: Assessment of third ventriculostomy patency with the 3D-SPACE technique: a preliminary multicenter research study. J Neurosurg. 122(6):1347-55, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25859808%5Bpmid%5D) +1. [Jessen NA et al: The glymphatic system: a beginner's guide. Neurochem Res. 40(12):2583-99, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25947369%5Bpmid%5D) +1. [Russo N et al: Endoscopic approaches to intraventricular lesions. J Neurol Surg A Cent Eur Neurosurg. 76(5):353-60, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26008954%5Bpmid%5D) +1. [Flannery AM et al: Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 1: introduction and methodology. J Neurosurg Pediatr. 14 Suppl 1:3-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25988777%5Bpmid%5D) +1. [Mazzola CA et al: Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 2: management of posthemorrhagic hydrocephalus in premature infants. J Neurosurg Pediatr. 14 Suppl 1:8-23, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25988778%5Bpmid%5D) +1. [Nikas DC et al: Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 10: change in ventricle size as a measurement of effective treatment of hydrocephalus. J Neurosurg Pediatr. 14 Suppl 1:77-81, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25988786%5Bpmid%5D) +1. [Dinçer A et al: Radiologic evaluation of pediatric hydrocephalus. Childs Nerv Syst. 27(10):1543-62, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928020%5Bpmid%5D) +1. [Mirone G et al: Hydrocephalus and spinal cord tumors: a review. Childs Nerv Syst. 27(10):1741-9, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928038%5Bpmid%5D) +1. [Oi S: Classification of hydrocephalus: critical analysis of classification categories and advantages of "Multi-categorical Hydrocephalus Classification" (Mc HC). Childs Nerv Syst. 27(10):1523-33, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928018%5Bpmid%5D) +1. [Dinçer A et al: Is all "communicating" hydrocephalus really communicating? Prospective study on the value of 3D-constructive interference in steady state sequence at 3T. AJNR Am J Neuroradiol. 30(10):1898-906, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19643921%5Bpmid%5D) +1. [Feng F et al: Evaluation of radionuclide cerebrospinal fluid scintigraphy as a guide in the management of patients with hydrocephalus. Clin Imaging. 33(2):85-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19237049%5Bpmid%5D) +1. [Linninger AA et al: Normal and hydrocephalic brain dynamics: the role of reduced cerebrospinal fluid reabsorption in ventricular enlargement. Ann Biomed Eng. 37(7):1434-47, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19373558%5Bpmid%5D) +1. [Oertel JM et al: Endoscopic third ventriculostomy in obstructive hydrocephalus due to giant basilar artery aneurysm. J Neurosurg. 110(1):14-8, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18991498%5Bpmid%5D) +1. [Stoquart-El Sankari S et al: Phase-contrast MR imaging support for the diagnosis of aqueductal stenosis. AJNR Am J Neuroradiol. 30(1):209-14, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18832663%5Bpmid%5D) +1. [Sekula RF Jr et al: A case of an elderly adult presenting with obstructive hydrocephalus secondary to a rare hemorrhagic suprasellar pilocytic astrocytoma. Clin Neuropathol. 27(6):396-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19130737%5Bpmid%5D) +1. [Yamada S et al: Visualization of cerebrospinal fluid movement with spin labeling at MR imaging: preliminary results in normal and pathophysiologic conditions. Radiology. 249(2):644-52, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18936318%5Bpmid%5D) +1. [Erdogan AR et al: Sex and handedness differences in size of cerebral ventricles of normal subjects. Int J Neurosci. 114(1):67-73, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14660068%5Bpmid%5D) +1. [Gaser C et al: Ventricular enlargement in schizophrenia related to volume reduction of the thalamus, striatum, and superior temporal cortex. Am J Psychiatry. 161(1):154-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14702264%5Bpmid%5D) +1. [Wyldes M et al: Isolated mild fetal ventriculomegaly. Arch Dis Child Fetal Neonatal Ed. 89(1):F9-13, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14711845%5Bpmid%5D) +1. [Akhondi H et al: Hydrocephalus as a presenting manifestation of neurosarcoidosis. South Med J. 96(4):403-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12916562%5Bpmid%5D) +1. [Bhattacharyya KB et al: Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature. Acta Neurol Scand. 108(3):216-20, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12911467%5Bpmid%5D) +1. Brown KP et al: 1H MRS in human hydrocephalus. J MRI. 14:291-9, 2003 +1. [Grunert P et al: The role of third ventriculostomy in the management of obstructive hydrocephalus. Minim Invasive Neurosurg. 46(1):16-21, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12640578%5Bpmid%5D) +1. [Joseph VB et al: MR ventriculography for the study of CSF flow. AJNR Am J Neuroradiol. 24(3):373-81, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12637285%5Bpmid%5D) +1. [Sener RN: Callosal changes in obstructive hydrocephalus: observations with FLAIR imaging, and diffusion MRI. Comput Med Imaging Graph. 26(5):333-7, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12204238%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Depiction of normal CSF flow through the glymphatic system. CSF descends along periarterial perivascular spaces (PVS) and through the interstitium before exiting along perivenular PVS, clearing macromolecules (black particles). The exchange between interstitium and PVS is modulated by astrocytic endfeet expressing AQP4 (pink channels).](images/app.statdx.com_image_thumbnail_e84cc4eb-f65b-49c8-8023-d157ef9155ba_annotated_true_size_900_quality_90_1b92eb51_20251018T164957Z.jpg) +*Depiction of normal CSF flow through the glymphatic system. CSF descends along periarterial perivascular spaces (PVS) and through the interstitium before exiting along perivenular PVS, clearing macromolecules (black particles). The exchange between interstitium and PVS is modulated by astrocytic endfeet expressing AQP4 (pink channels).* + +![Depiction of normal CSF flow through the glymphatic system. CSF descends along periarterial perivascular spaces (PVS) and through the interstitium before exiting along perivenular PVS, clearing macromolecules (black particles). The exchange between interstitium and PVS is modulated by astrocytic endfeet expressing AQP4 (pink channels).](images/app.statdx.com_image_thumbnail_e84cc4eb-f65b-49c8-8023-d157ef9155ba_size_174_quality_85_184aa51d_20251018T155131Z.jpg) +*Depiction of normal CSF flow through the glymphatic system. CSF descends along periarterial perivascular spaces (PVS) and through the interstitium before exiting along perivenular PVS, clearing macromolecules (black particles). The exchange between interstitium and PVS is modulated by astrocytic endfeet expressing AQP4 (pink channels).* + +![An isoattenuating colloid cyst obstructs the foramina of Monro in this 7-year-old, causing obstructive hydrocephalus.](images/app.statdx.com_image_thumbnail_dfa6cf57-4225-40b1-8037-f7f2557ee773_annotated_true_size_900_quality_90_b05cdf7d_20251018T164957Z.jpg) +*An isoattenuating colloid cyst obstructs the foramina of Monro in this 7-year-old, causing obstructive hydrocephalus.* + +![A large suprasellar arachnoid cyst balloons upward and obstructs the foramina of Monro in this 1-year-old with macrocrania.](images/app.statdx.com_image_thumbnail_70070ccc-c977-4c05-a903-81616fbbea63_annotated_true_size_900_quality_90_dddf27ae_20251018T164957Z.jpg) +*A large suprasellar arachnoid cyst balloons upward and obstructs the foramina of Monro in this 1-year-old with macrocrania.* + +![This 3-month-old with hydrocephalus has a Blake pouch cyst obstructing outflow of CSF from the 4th ventricle. Note the membrane across the posterior foramen magnum and the uplifting of the vermis .](images/app.statdx.com_image_thumbnail_310d1a62-4dbc-479a-9e9e-423205955735_annotated_true_size_900_quality_90_b75c461d_20251018T164957Z.jpg) +*This 3-month-old with hydrocephalus has a Blake pouch cyst obstructing outflow of CSF from the 4th ventricle. Note the membrane across the posterior foramen magnum and the uplifting of the vermis .* + +![A medulloblastoma fills and obstructs the 4th ventricle in this 10-year-old, leading to supratentorial ventriculomegaly and papilledema . Papilledema visible on MR typically correlates to grade 3 on the Frisen scale (moderate edema).](images/app.statdx.com_image_thumbnail_9a9c7b4f-95d2-4e4d-8b5d-68161defab53_annotated_true_size_900_quality_90_a79dce92_20251018T164957Z.jpg) +*A medulloblastoma fills and obstructs the 4th ventricle in this 10-year-old, leading to supratentorial ventriculomegaly and papilledema . Papilledema visible on MR typically correlates to grade 3 on the Frisen scale (moderate edema).* + +![Axial FLAIR MR through the lateral ventricles in the same child shows transependymal edema capping the frontal and occipital horns , reflecting the increased pressure in the ventricular system.](images/app.statdx.com_image_thumbnail_d9d39656-21b9-48e2-82d3-86f3cabd705a_annotated_true_size_900_quality_90_91b55c48_20251018T164957Z.jpg) +*Axial FLAIR MR through the lateral ventricles in the same child shows transependymal edema capping the frontal and occipital horns , reflecting the increased pressure in the ventricular system.* + +![Bacterial meningitis (group A Streptococcus in this example) can restrict resorption of CSF but can also obstruct at the cerebral aqueduct and 4th ventricular outlets when complicated by ventriculitis, evident on this image by abnormal enhancement of the ependyma .](images/app.statdx.com_image_thumbnail_e5af07e5-9045-454e-919f-bb1cfd505bdc_annotated_true_size_900_quality_90_bee3db67_20251018T164957Z.jpg) +*Bacterial meningitis (group A Streptococcus in this example) can restrict resorption of CSF but can also obstruct at the cerebral aqueduct and 4th ventricular outlets when complicated by ventriculitis, evident on this image by abnormal enhancement of the ependyma .* + +![An infiltrating tectal glioma obstructs the cerebral aqueduct in this 10-year-old. Absence of transependymal edema suggests a compensated hydrocephalus.](images/app.statdx.com_image_thumbnail_0e5ef294-c697-4d00-9852-0ad6f730f900_annotated_true_size_900_quality_90_68db5118_20251018T164957Z.jpg) +*An infiltrating tectal glioma obstructs the cerebral aqueduct in this 10-year-old. Absence of transependymal edema suggests a compensated hydrocephalus.* + +![A papilloma of the choroid plexus in the occipital horn of the left lateral ventricle causes moderate hydrocephalus by excessive CSF production in this 2-month-old.](images/app.statdx.com_image_thumbnail_3404043a-6569-4de0-b4a3-a0cc0bd2b6dc_annotated_true_size_900_quality_90_aa2614f3_20251018T164957Z.jpg) +*A papilloma of the choroid plexus in the occipital horn of the left lateral ventricle causes moderate hydrocephalus by excessive CSF production in this 2-month-old.* + +![CSF overproduction can rarely be nonneoplastic in nature, as in this 6-month-old with villous hyperplasia of the choroid plexus in each lateral ventricle . Note the preservation of peripheral sulci, as the unfused sutures of the infant can widen in response to increased intracranial volume.](images/app.statdx.com_image_thumbnail_e380d6ae-5d60-44e2-bf15-30137880085f_annotated_true_size_900_quality_90_1fe1546a_20251018T164959Z.jpg) +*CSF overproduction can rarely be nonneoplastic in nature, as in this 6-month-old with villous hyperplasia of the choroid plexus in each lateral ventricle . Note the preservation of peripheral sulci, as the unfused sutures of the infant can widen in response to increased intracranial volume.* + + +### Additional Images + +![Sagittal T1 MR shows a large mass within the 4th ventricle causing intraventricular obstructive hydrocephalus or noncommunicating hydrocephalus.](images/app.statdx.com_image_thumbnail_c48f3e2e-092c-4ce3-881e-9a269ec15ea6_annotated_true_size_900_quality_90_78b02641_20251018T165000Z.jpg) +*Sagittal T1 MR shows a large mass within the 4th ventricle causing intraventricular obstructive hydrocephalus or noncommunicating hydrocephalus.* + +![Sagittal T2 MR in the same patient shows transependymal CSF flow, seen here as "fingers" extending into white matter around the enlarged lateral ventricle. The case was medulloblastoma with acute IVOH.](images/app.statdx.com_image_thumbnail_15897f20-57e8-4b17-9ac4-30cab6467abb_annotated_true_size_900_quality_90_9fed6b74_20251018T165000Z.jpg) +*Sagittal T2 MR in the same patient shows transependymal CSF flow, seen here as "fingers" extending into white matter around the enlarged lateral ventricle. The case was medulloblastoma with acute IVOH.* + +![Coronal T1 C+ MR shows IVOH with a large, enhancing intraventricular mass causing marked enlargement of the lateral ventricles .](655689cf-c7a3-44eb-85b1-9c92f7dc0cb1) +*Coronal T1 C+ MR shows IVOH with a large, enhancing intraventricular mass causing marked enlargement of the lateral ventricles .* + +![Axial NECT in the same patient shows the large intraventricular mass within the 4th ventricle. Note the dilated temporal horns .](images/app.statdx.com_image_thumbnail_1d873379-77f2-4ba4-85c9-43715aedaedc_annotated_true_size_900_quality_90_092a91f4_20251018T165000Z.jpg) +*Axial NECT in the same patient shows the large intraventricular mass within the 4th ventricle. Note the dilated temporal horns .* + +![Sagittal T1 MR shows IVOH secondary to aqueductal stenosis and distal stenosis of cerebral aqueduct . Note the enlarged lateral and 3rd ventricles.](images/app.statdx.com_image_thumbnail_e2d6cb01-32da-4284-85fb-e1d0b11218b1_annotated_true_size_900_quality_90_168d3b76_20251018T165000Z.jpg) +*Sagittal T1 MR shows IVOH secondary to aqueductal stenosis and distal stenosis of cerebral aqueduct . Note the enlarged lateral and 3rd ventricles.* + +![Axial FLAIR MR shows neurosarcoidosis and EVOH secondary to diffuse meningeal disease. Periventricular white matter hyperintensities are also present, as well as choroid involvement .](images/app.statdx.com_image_thumbnail_f9368bc3-dfdf-4912-bb77-5097bb5043bb_annotated_true_size_900_quality_90_45550aa6_20251018T165000Z.jpg) +*Axial FLAIR MR shows neurosarcoidosis and EVOH secondary to diffuse meningeal disease. Periventricular white matter hyperintensities are also present, as well as choroid involvement .* + +![Coronal T1 C+ MR shows neurocysticercosis involvement within the 3rd ventricle and aqueduct , causing IVOH. The lateral ventricles are dilated.](images/app.statdx.com_image_thumbnail_be2f5692-eeeb-4ea0-aae2-9ececaf9072b_annotated_true_size_900_quality_90_31f0b627_20251018T165000Z.jpg) +*Coronal T1 C+ MR shows neurocysticercosis involvement within the 3rd ventricle and aqueduct , causing IVOH. The lateral ventricles are dilated.* + +![Axial FLAIR MR shows neurocysticercosis resulting in IVOH. Large intraventricular cysts are present in the lateral vents , obstructing the foramina of Monro.](images/app.statdx.com_image_thumbnail_d4fbed45-ded4-4557-8a60-ffa2c5a76b7b_annotated_true_size_900_quality_90_b7584229_20251018T165001Z.jpg) +*Axial FLAIR MR shows neurocysticercosis resulting in IVOH. Large intraventricular cysts are present in the lateral vents , obstructing the foramina of Monro.* + +![Axial T1 MR shows a well-defined, hyperintense lesion at the foramen of Monro in a patient with headaches, most consistent with a colloid cyst. Note the enlargement of the lateral ventricles due to obstruction at the foramen of Monro.](images/app.statdx.com_image_thumbnail_709fe152-548b-43c9-a72d-d13abe331723_annotated_true_size_900_quality_90_9f3616ed_20251018T165001Z.jpg) +*Axial T1 MR shows a well-defined, hyperintense lesion at the foramen of Monro in a patient with headaches, most consistent with a colloid cyst. Note the enlargement of the lateral ventricles due to obstruction at the foramen of Monro.* + +![Sagittal T1 C+ MR shows a homogeneously enhancing mass in the posterior 3rd ventricle , which causes obstruction and dilatation of the lateral and 3rd ventricles. On pathology, this was an astrocytoma.](images/app.statdx.com_image_thumbnail_f26e26ab-4622-4975-b3b8-c0241bd59f6e_annotated_true_size_900_quality_90_214d2580_20251018T165001Z.jpg) +*Sagittal T1 C+ MR shows a homogeneously enhancing mass in the posterior 3rd ventricle , which causes obstruction and dilatation of the lateral and 3rd ventricles. On pathology, this was an astrocytoma.* + +![Coronal T2 MR shows a pilocytic astrocytoma centered in the right thalamus , causing severe mass effect on the 3rd ventricle and resultant obstructive hydrocephalus .](39ae17e4-a50a-4f11-b6c5-42b47b2c293a) +*Coronal T2 MR shows a pilocytic astrocytoma centered in the right thalamus , causing severe mass effect on the 3rd ventricle and resultant obstructive hydrocephalus .* + +![Axial T2 MR demonstrates a well-defined CSF intensity cyst with the left temporal horn most consistent with an ependymal cyst . Note the dilated and trapped left temporal horn .](c1b16bae-b36a-4bf1-8de9-ca410a8c3f96) +*Axial T2 MR demonstrates a well-defined CSF intensity cyst with the left temporal horn most consistent with an ependymal cyst . Note the dilated and trapped left temporal horn .* + +![Sagittal T1 C+ MR shows an enhancing mass in the pineal region causing mass effect on the tectal plate and aqueductal obstruction. Note the extensive leptomeningeal enhancement due to CSF spread of tumor. CSF cytology showed a primitive neuroectodermal tumor.](8096e72c-52bb-4372-a469-ed9b3c222bd4) +*Sagittal T1 C+ MR shows an enhancing mass in the pineal region causing mass effect on the tectal plate and aqueductal obstruction. Note the extensive leptomeningeal enhancement due to CSF spread of tumor. CSF cytology showed a primitive neuroectodermal tumor.* + +![A medulloblastoma fills and obstructs the 4th ventricle in this 10-year-old, leading to supratentorial ventriculomegaly and papilledema.](a7643e46-4a5e-47f0-b843-87e9f35fca31) +*A medulloblastoma fills and obstructs the 4th ventricle in this 10-year-old, leading to supratentorial ventriculomegaly and papilledema.* + +![FIESTA shows pineal parenchymal tumor of intermediate differentiation obstructing the cerebral aqueduct in this 8-year-old boy.](229bee91-b7b6-4584-97a3-e6f656b0000c) +*FIESTA shows pineal parenchymal tumor of intermediate differentiation obstructing the cerebral aqueduct in this 8-year-old boy.* + diff --git a/out/idiopathic-intracranial-hypertension_d7a0a1b6-1d94-473c-9fe9-021443969f9f.md b/out/idiopathic-intracranial-hypertension_d7a0a1b6-1d94-473c-9fe9-021443969f9f.md new file mode 100644 index 0000000..e199f31 --- /dev/null +++ b/out/idiopathic-intracranial-hypertension_d7a0a1b6-1d94-473c-9fe9-021443969f9f.md @@ -0,0 +1,428 @@ +--- +title: "Idiopathic Intracranial Hypertension" +docid: "d7a0a1b6-1d94-473c-9fe9-021443969f9f" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "CSF Disorders" + slug: "csf-disorders" + treeNodeId: "d305bd95-7cca-4888-80b9-fabe45d84ee5" + - + name: "Idiopathic Intracranial Hypertension" + slug: "idiopathic-intracranial-hypertensi-" + treeNodeId: null +category: "Brain" +documentVersionId: "8907806d-0770-46fa-b129-78e393ad4038" +imageCount: 22 +lastUpdated: "10/08/20" +pageDescription: "Idiopathic Intracranial Hypertension" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, CSF Disorders, Idiopathic Intracranial Hypertension" +pageTitle: "Idiopathic Intracranial Hypertension | STATdx" +enhancedTitle: "Idiopathic Intracranial Hypertension" +type: "DX" +references: true +tables: 1 +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "CSF Disorders" + - "Idiopathic Intracranial Hypertension" +--- +# KEY FACTS + +- ## Terminology + + + - Idiopathic intracranial hypertension (IIH) + - Pseudotumor cerebri + - Benign intracranial hypertension + - ↑ intracranial pressure (ICP) without identifiable cause +- ## Imaging + + + - Empty or partially empty sella + - Posterior globe flattening + - Intraocular protrusion of optic nerve head + - Optic nerve sheath enlargement ± tortuosity + - Optic nerve head DWI hyperintensity, ± enhancement + - Slit-like ventricles, rare: Poor neuroimaging sign of IIH + - MRV: Often shows transverse sinus stenosis and flow gaps + - Whether this is cause or consequence of raised ICP is controversial + - Best imaging tool: MR brain + T2 coronal fat-saturated orbit + MRV +- ## Top Differential Diagnoses + + + - Secondary pseudotumor syndromes + - Idiopathic or postinflammatory (i.e., multiple sclerosis) optic nerve atrophy + - Idiopathic empty sella (normal variant) + - Chiari 1 malformation +- ## Clinical Issues + + + - Obese woman age 20-44 years with headache and papilledema most common presentation + - Headache in 75-94% + - Papilledema (bilateral optic nerve head swelling) virtually universal + - Progressive visual loss ± CNVI paresis, diplopia + - Chief hazard: Vision loss from chronic papilledema + - Treatment: Medical or surgical (lumbar puncture, shunt, optic nerve sheath fenestration) + - Stent placement in transverse sinus stenosis with significant pressure differentials across stenosis (controversial) + +# TERMINOLOGY + +- ## Abbreviations + + + - Idiopathic intracranial hypertension (IIH) +- ## Synonyms + + + - Pseudotumor cerebri + - Benign intracranial hypertension +- ## Definitions + + + - ↑ intracranial pressure (ICP) without identifiable cause + - Association of any medication or condition with IIH better termed "secondary intracranial hypertension" + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Flattening of posterior sclera, intraocular optic nerve protrusion, enlarged optic nerve sheath, ↑ tortuosity of optic nerve, partially empty sella, and venous sinus stenosis in patient with clinical findings of IIH + - Imaging in IIH + - Exclude identifiable causes of ↑ ICP + - Detect findings associated with IIH +- ## CT Findings + + + - ### NECT + + + - Usually normal + - Enlarged optic nerve sheaths ± empty sella + - Less common: Slit ventricles + - ### Bone CT + + + - Solitary or multiple skull base osseous-dural defects + - May see skull base foramina enlargement +- ## MR Findings + + + - ### T1WI + + + - Partially empty sella turcica + - Enlarged/tortuous optic nerve sheaths + - Posterior sclera flattened + - Small "pinched" ventricles + - Midline sagittal: Cerebellar tonsillar ectopia may mimic Chiari malformation type 1 + - ### T2WI + + + - Empty or partially empty sella + - Posterior globe flattening + - Intraocular protrusion of optic nerve head + - Optic nerve sheath enlargement: Widened ring of CSF around optic nerve + - Optic nerve tortuosity + - Slit-like ventricles, rare: Poor neuroimaging sign of IIH + - "Tight" subarachnoid spaces + - Meningoencephaloceles + - ### FLAIR + + + - Contrast-enhanced 3D-FLAIR: Hyperintensity of optic nerve head sensitive for detection of papilledema in IIH + - ### DWI + + + - DWI hyperintensity of optic nerve head with papilledema + - DTI: Optic disc fractional anisotropy (FA) low & mean diffusivity (MD) high in IIH + - ### T1WI C+ + + + - Enhancement of optic nerve head + - ### MRV + + + - Often shows transverse sinus stenosis and flow gaps + - Controversial whether this is cause or consequence of raised ICP + - CTV helpful to differentiate hypoplastic sinus segment from thrombosis +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR brain + T2 coronal fat-saturated orbit + MRV + +# DIFFERENTIAL DIAGNOSIS + +- ## Secondary Intracranial Hypertension + + + - Cerebral venous abnormalities + - [Dural venous sinus thrombosis, bilateral jugular vein thrombosis, superior vena cava syndrome, arteriovenous fistula](/document/dural-sinus-thrombosis/4e81a1de-df92-4172-99ec-1377b0d9d188) + - ↓ CSF absorption from previous intracranial infection or subarachnoid hemorrhage, hypercoagulable states + - Ventriculomegaly more common + - Medications and exposures + - Tetracycline, minocycline, vitamin A, lithium, retinoids, anabolic steroids, withdrawal from chronic corticosteroids + - Medical conditions + - Endocrine disorders (Addison disease, hypoparathyroidism), hypercapnia, sleep apnea, SLE +- [Idiopathic or Postinflammatory Optic Nerve Atrophy](/document/optic-neuritis/ac9c8fc9-33cd-4716-a509-2542ec5579ca) + - Small optic nerves without scleral flattening +- [Idiopathic Empty Sella](/document/empty-sella/39a0d2d1-1439-4558-8f5d-86a2a6d93e3a) + - Normal variant; normal optic nerve sheaths +- [Chiari 1 Malformation](/document/chiari-1-malformation/97837e15-0d39-4c87-8af0-028652b399a6) + - Peg-like tonsils ≥ 5 mm below foramen magnum + - Low cerebellar tonsils in IIH may mimic Chiari 1 + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Precise etiology of IIH unknown + - 5 different proposed mechanisms resulting in ↑ ICP + - ↑ cerebral volume + - Possible etiology: ↑ interstitial fluid, ↑ blood volume, ↑ tissue volume + - ↑ CSF volume + - Possible etiology: ↑ CSF production rate, ↑ CSF outflow resistance + - ↑ cerebral arterial pressure + - Possible etiology: Loss of cerebral autoregulation + - ↑ venous blood volume and interstitial fluid + - Possible etiology: ↑ cerebral venous pressure + - ↓ CSF outflow and ↑ CSF volume +- ## Gross Pathologic & Surgical Features + + + - Bilateral papilledema +- ## Microscopic Features + + + - Normal CSF cytology, chemistry + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Headache in 75-94% + - Generalized, episodic, throbbing, aggravated by Valsalva + - Transient vision loss, other visual complaints + - Fulminant IIH, severe vision loss < 4 weeks from onset of symptoms + - Papilledema (bilateral optic nerve head swelling) virtually universal + - Progressive visual loss ± CNVI paresis, diplopia + - Vertigo, tinnitus (52-60%), occasional pituitary dysfunction + - May present with spontaneous CSF leak + - Some patient with spontaneous CSF leak may not exhibit typical symptoms of IIH + - May develop symptoms of IIH after CSF leak repair + - Temporal lobe epilepsy caused by anteroinferior temporal lobe meningoencephaloceles in IIH + - ### Clinical profile + + + - Obese, young to middle-aged woman with headache, papilledema +- ## Demographics + + + - ### Age + + + - Peak: 15-40 years (occasionally seen in children) + - ### Epidemiology + + + - 0.9 cases per 100,000 population in USA + - More common in overweight, reproductive-aged women + - Incidence in females aged 2-44 years & 20% above ideal body weight: ~ 19.3 cases per 100,000 population + - ### Sex + + + - M:F = 1:8 + - Epidemiology: ↑ prevalence with obesity +- ## Natural History & Prognosis + + + - Chief hazard: Vision loss from chronic papilledema, severe visual acuity deficits in 25% of patients without treatment +- ## Treatment + + + - Goal: Prevent visual loss, improve associated symptoms + - Options + - Medical: Weight loss, carbonic anhydrase inhibitors: Acetazolamide + - Therapeutic lumbar puncture + - Surgical: Reserved for patients who continue to experience vision loss despite conservative management and those initially presenting with rapid vision loss + - Lumboperitoneal shunt, optic nerve sheath fenestration + - Venous stent placement + - Stent placement in transverse sinus stenosis with significant pressure differentials across stenosis has shown to improve symptoms and ↓ papilledema + - ↓ cerebral venous pressure, improve CSF resorption in venous system: ↓ intracranial (CSF) pressure, improving symptoms of IIH, and ↓ papilledema + - **Venous stent placement is controversial** + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - Must exclude venous thrombosis/space-occupying lesion + + 58e13d74-efc9-4630-9014-3c28122c7470 + +## References + +# Selected References + +1. [Nagarajan E et al: Is magnetic resonance imaging diffusion restriction of the optic disc head a new marker for idiopathic intracranial hypertension? J Neurosci Rural Pract. 11(1):170-4, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32140023%5Bpmid%5D) +1. [Boyter E: Idiopathic intracranial hypertension. JAAPA. 32(5):30-5, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30969189%5Bpmid%5D) +1. [Golden E et al: Contrast-enhanced 3D-FLAIR imaging of the optic nerve and optic nerve head: novel neuroimaging findings of idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 40(2):334-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30679213%5Bpmid%5D) +1. [Rehder D: Idiopathic intracranial hypertension: review of clinical syndrome, imaging findings, and treatment. Curr Probl Diagn Radiol. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31056359%5Bpmid%5D) +1. [Thurtell MJ: Idiopathic intracranial hypertension. Continuum (Minneap Minn). 25(5):1289-309, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31584538%5Bpmid%5D) +1. [Madriz Peralta G et al: An update of idiopathic intracranial hypertension. Curr Opin Ophthalmol. 29(6):495-502, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30169466%5Bpmid%5D) +1. [Stevens SM et al: Idiopathic intracranial hypertension: contemporary review and implications for the otolaryngologist. Laryngoscope. 128(1):248-56, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=28349571%5Bpmid%5D) +1. [Wall M: Update on idiopathic intracranial hypertension. Neurol Clin. 35(1):45-57, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27886895%5Bpmid%5D) +1. [Görkem SB et al: MR imaging findings in children with pseudotumor cerebri and comparison with healthy controls. Childs Nerv Syst. 31(3):373-80, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25358812%5Bpmid%5D) +1. [Masri A et al: Intracranial hypertension in children: etiologies, clinical features, and outcome. J Child Neurol. 30(12):1562-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25762586%5Bpmid%5D) +1. [Sivasankar R et al: Imaging and interventions in idiopathic intracranial hypertension: a pictorial essay. Indian J Radiol Imaging. 25(4):439-44, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26752823%5Bpmid%5D) +1. [Ahmed RM et al: Transverse sinus stenting for pseudotumor cerebri: a cost comparison with CSF shunting. AJNR Am J Neuroradiol. 35(5):952-8, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24287092%5Bpmid%5D) +1. [Dave SB et al: Pseudotumor cerebri: an update on treatment options. Indian J Ophthalmol. 62(10):996-8, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25449933%5Bpmid%5D) +1. [Liguori C et al: Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 82(19):1752-3, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24821936%5Bpmid%5D) +1. [Friedman DI et al: Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 81(13):1159-65, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23966248%5Bpmid%5D) +1. [Passi N et al: MR imaging of papilledema and visual pathways: effects of increased intracranial pressure and pathophysiologic mechanisms. AJNR Am J Neuroradiol. 34(5):919-24, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=22422187%5Bpmid%5D) +1. [Aiken AH et al: Incidence of cerebellar tonsillar ectopia in idiopathic intracranial hypertension: a mimic of the Chiari I malformation. AJNR Am J Neuroradiol. 33(10):1901-6, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22723059%5Bpmid%5D) +1. [Ahmed RM et al: Transverse sinus stenting for idiopathic intracranial hypertension: a review of 52 patients and of model predictions. AJNR Am J Neuroradiol. 32(8):1408-14, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21799038%5Bpmid%5D) +1. [Degnan AJ et al: Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol. 32(11):1986-93, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21680652%5Bpmid%5D) +1. [Furtado SV et al: Pseudotumor cerebri: as a cause for early deterioration after Chiari I malformation surgery. Childs Nerv Syst. 25(8):1007-12, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19296114%5Bpmid%5D) +1. [Hershko AY et al: Increased intracranial pressure related to systemic lupus erythematosus: a 26-year experience. Semin Arthritis Rheum. 38(2):110-5, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18221986%5Bpmid%5D) +1. [Randhawa S et al: Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Opin Ophthalmol. 19(6):445-53, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18854688%5Bpmid%5D) +1. [Agarwal MR et al: Optic nerve sheath fenestration for vision preservation in idiopathic intracranial hypertension. Neurosurg Focus. 23(5):E7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=18004969%5Bpmid%5D) +1. [Binder DK et al: Idiopathic intracranial hypertension. Neurosurgery. 54(3):538-51; discussion 551-2, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15028127%5Bpmid%5D) +1. [Bastin ME et al: Diffuse brain oedema in idiopathic intracranial hypertension: a quantitative magnetic resonance imaging study. J Neurol Neurosurg Psychiatry. 74(12):1693-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14638893%5Bpmid%5D) +1. [Bandyopadhyay S: Pseudotumor cerebri. Arch Neurol. 58(10):1699-701, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11594936%5Bpmid%5D) +1. [Suzuki H et al: MR imaging of idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 22(1):196-9, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11158909%5Bpmid%5D) + +## Tables + +# Original Modified Dandy Criteria and Criteria Utilized in IIH Treatment Trial + +| A: Modified Dandy Criteria for IIH | +| --- | +| (1) Signs and symptoms of ↑ ICP (headaches, nausea, vomiting, transient visual obscurations, or papilledema) | +| (2) Absence of localized findings in neurologic examination (except for false localizing signs, such as abducens nerve palsy) | +| (3) Normal CT/MR findings without evidence of hydrocephalus or mass lesion | +| (4) CSF opening pressure > 25 cm with normal CSF cytologic and chemical findings | +| (5) No other causes of ↑ ICP identified | +| B: Idiopathic IIH Treatment Trial: Modified Dandy Criteria | +| (1) Signs and symptoms of ↑ ICP | +| (2) Absence of localized findings in neurologic examination | +| (3) ↑ CSF pressure > 20 cm; normal neuroimaging except for empty sella, flattening optic nerve head, distention of perioptic subarachnoid space ± tortuous optic nerve, transverse venous sinus stenosis | +| (4) Awake and alert | +| (5) No other causes of ↑ ICP | +| If CSF opening pressure was 20-25 cm, at least 1 of following was also required | +| Pulse synchronous tinnitus, CNVI palsy, Frisen grade II papilledema, no disc anomalies mimicking disc edema, MRV with lateral sinus collapse/stenosis, partially empty sella, dilated optic nerve sheaths | + + +## Images + + +### Selected Images + +![Axial T2 FS MR in a young obese female with headaches and visual symptoms shows flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe. Note mild prominence of the CSF along the optic nerve sheaths.](images/app.statdx.com_image_thumbnail_24d4b975-7325-44c1-915b-9a6d28bfe436_annotated_true_size_900_quality_90_3a507534_20251018T164622Z.jpg) +*Axial T2 FS MR in a young obese female with headaches and visual symptoms shows flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe. Note mild prominence of the CSF along the optic nerve sheaths.* + +![Axial T2 FS MR in a young obese female with headaches and visual symptoms shows flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe. Note mild prominence of the CSF along the optic nerve sheaths.](images/app.statdx.com_image_thumbnail_24d4b975-7325-44c1-915b-9a6d28bfe436_size_174_quality_85_5e6fc32c_20251018T164552Z.jpg) +*Axial T2 FS MR in a young obese female with headaches and visual symptoms shows flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe. Note mild prominence of the CSF along the optic nerve sheaths.* + +![Axial DWI in the same patient demonstrates subtle high signal in the region of the optic nerve heads bilaterally. Hyperintensity of the optic nerve heads on DWI can serve as a useful imaging marker for papilledema, especially if bilateral.](images/app.statdx.com_image_thumbnail_ae0e2ddf-eb4d-4442-85d4-ab2b80819b1f_annotated_true_size_900_quality_90_ae9539e6_20251018T164622Z.jpg) +*Axial DWI in the same patient demonstrates subtle high signal in the region of the optic nerve heads bilaterally. Hyperintensity of the optic nerve heads on DWI can serve as a useful imaging marker for papilledema, especially if bilateral.* + +![Axial T1 C+ MR in the same patient shows subtle enhancement , as well as protrusion of prelaminar optic nerves bilaterally.](images/app.statdx.com_image_thumbnail_9a3de7d7-cac0-453f-8762-b1acc1059c51_annotated_true_size_900_quality_90_fbbf5704_20251018T164622Z.jpg) +*Axial T1 C+ MR in the same patient shows subtle enhancement , as well as protrusion of prelaminar optic nerves bilaterally.* + +![Coronal T2 FS MR in the same patient shows a partially empty sella with the pituitary gland flattened along the floor of the sella. Idiopathic intracranial hypertension is more commonly observed in overweight women of reproductive age. Treatment includes weight loss and medications, as well as lumbar punctures, shunt, and optic nerve fenestration.](images/app.statdx.com_image_thumbnail_6033189e-a7cc-4e6f-a8f7-4c3b312359f7_annotated_true_size_900_quality_90_a2d89787_20251018T164622Z.jpg) +*Coronal T2 FS MR in the same patient shows a partially empty sella with the pituitary gland flattened along the floor of the sella. Idiopathic intracranial hypertension is more commonly observed in overweight women of reproductive age. Treatment includes weight loss and medications, as well as lumbar punctures, shunt, and optic nerve fenestration.* + +![Coronal T2 FS MR of orbits in a 6 year old with papilledema and opening CSF pressure of 32 cm of H₂O shows dilated optic nerve sheaths bilaterally .](images/app.statdx.com_image_thumbnail_5e42b718-df6f-41f7-86cd-35fbe1dbee62_annotated_true_size_900_quality_90_29305ac9_20251018T164622Z.jpg) +*Coronal T2 FS MR of orbits in a 6 year old with papilledema and opening CSF pressure of 32 cm of H₂O shows dilated optic nerve sheaths bilaterally .* + +![Sagittal T2 FS MR in the same patient shows tortuosity of the optic nerve, dilated optic nerve sheath , flattening of the posterior sclera , and mild bulging of the optic nerve disc head due to papilledema. Findings are typical of idiopathic intracranial hypertension (IIH). Childhood obesity has a strong association with ↑ risk of pediatric IIH.](images/app.statdx.com_image_thumbnail_2bd694d1-0915-4b68-9b90-85c463dfe179_annotated_true_size_900_quality_90_7a482e85_20251018T164622Z.jpg) +*Sagittal T2 FS MR in the same patient shows tortuosity of the optic nerve, dilated optic nerve sheath , flattening of the posterior sclera , and mild bulging of the optic nerve disc head due to papilledema. Findings are typical of idiopathic intracranial hypertension (IIH). Childhood obesity has a strong association with ↑ risk of pediatric IIH.* + +![Sagittal T2 MR in a young female with IIH and temporal lobe epilepsy shows a defect along the floor of the middle cranial fossa with herniation of the anteroinferior temporal lobe .](images/app.statdx.com_image_thumbnail_53799f83-fd6b-42ac-b8a4-1041a19ec160_annotated_true_size_900_quality_90_fe5d009a_20251018T164622Z.jpg) +*Sagittal T2 MR in a young female with IIH and temporal lobe epilepsy shows a defect along the floor of the middle cranial fossa with herniation of the anteroinferior temporal lobe .* + +![Coronal CT cisternogram in a patient with IIH shows an osteodural defect along the great wing of the sphenoid. Defect along the lateral wall of the sphenoid sinus with a meningocele and contrast in the lateral sphenoid sinus due to CSF leak is shown. Patients with IIH can present with spontaneous CSF leaks.](images/app.statdx.com_image_thumbnail_66502da6-e3f7-4a1b-b5de-afe2df76f8d9_annotated_true_size_900_quality_90_ee7dc6dd_20251018T164622Z.jpg) +*Coronal CT cisternogram in a patient with IIH shows an osteodural defect along the great wing of the sphenoid. Defect along the lateral wall of the sphenoid sinus with a meningocele and contrast in the lateral sphenoid sinus due to CSF leak is shown. Patients with IIH can present with spontaneous CSF leaks.* + +![MIP image of a postcontrast MR venogram study in a patient with IIH shows stenosis of the distal transverse sinuses bilaterally , right > left.](images/app.statdx.com_image_thumbnail_610e2c15-7dd5-42a8-9642-3d8fb1089582_annotated_true_size_900_quality_90_6b91fa11_20251018T164622Z.jpg) +*MIP image of a postcontrast MR venogram study in a patient with IIH shows stenosis of the distal transverse sinuses bilaterally , right > left.* + +![3D VRT MR in the same patient shows transverse sinus stenosis , right > left. Phase contrast and postcontrast MR venogram techniques are preferred over TOF-MR venogram to evaluate for transverse sinus stenosis. Stenting of transverse sinus stenosis in patients with IIH is a controversial treatment option.](164ece26-20b8-4cae-b030-9b72efe9a987) +*3D VRT MR in the same patient shows transverse sinus stenosis , right > left. Phase contrast and postcontrast MR venogram techniques are preferred over TOF-MR venogram to evaluate for transverse sinus stenosis. Stenting of transverse sinus stenosis in patients with IIH is a controversial treatment option.* + + +### Additional Images + +![Coronal T1WI MR in the same patient shows unusually small lateral ventricles with a "pinched" appearance. These findings in an obese female with headaches and papilledema are consistent with IIH.](970fd4dc-11be-4478-9403-460a22cd3552) +*Coronal T1WI MR in the same patient shows unusually small lateral ventricles with a "pinched" appearance. These findings in an obese female with headaches and papilledema are consistent with IIH.* + +![Axial T2WI MR shows ↑ fluid in bilateral optic nerve sheaths with mild flattening of the globes at optic nerve insertion. Also note the CSF-filled and expanded empty sella .](3bf70f24-e2cf-411f-afde-5d27b9d05741) +*Axial T2WI MR shows ↑ fluid in bilateral optic nerve sheaths with mild flattening of the globes at optic nerve insertion. Also note the CSF-filled and expanded empty sella .* + +![Sagittal T1WI MR in another patient with IIH ("pseudotumor cerebri") shows empty sella . The ventricular size is normal.](390c2ec4-5e56-423f-bffb-2414615987cd) +*Sagittal T1WI MR in another patient with IIH ("pseudotumor cerebri") shows empty sella . The ventricular size is normal.* + +![Axial T2WI MR shows ↑ fluid in the sheaths surrounding the optic nerves , associated with severe scleral flattening .](df25df16-6b53-4850-a23d-304bb0de9fb4) +*Axial T2WI MR shows ↑ fluid in the sheaths surrounding the optic nerves , associated with severe scleral flattening .* + +![Axial T2WI MR shows dilated CSF spaces around the optic nerves and protrusion of the optic nerve papilla into the posterior globes . Opening CSF pressure in this 32-year-old woman was 45 cm of H₂O. Prominent CSF space in the suprasellar cistern represents an empty sella . Note the tortuosity of the left optic nerve.](56e575d8-16f3-4e95-95f8-2d3cbb5caea6) +*Axial T2WI MR shows dilated CSF spaces around the optic nerves and protrusion of the optic nerve papilla into the posterior globes . Opening CSF pressure in this 32-year-old woman was 45 cm of H₂O. Prominent CSF space in the suprasellar cistern represents an empty sella . Note the tortuosity of the left optic nerve.* + +![Sagittal T1WI MR in the same patient shows a partially empty sella , suggesting high CSF pressure in this young obese woman with headaches.](2b283afb-fa49-4301-af75-c08cd47d46e6) +*Sagittal T1WI MR in the same patient shows a partially empty sella , suggesting high CSF pressure in this young obese woman with headaches.* + +![Axial T1WI C+ MR in the same patient demonstrates enhancement, as well as protrusion of prelaminar optic nerves bilaterally . Mild diffuse optic nerve sheath enhancement is also present.](5520270b-2fc2-4233-8468-bb37f0efdc21) +*Axial T1WI C+ MR in the same patient demonstrates enhancement, as well as protrusion of prelaminar optic nerves bilaterally . Mild diffuse optic nerve sheath enhancement is also present.* + +![Coronal T1WI C+ FS MR in the same patient shows diffuse enhancement of the optic nerve sheaths associated with prominent subarachnoid spaces along the optic nerves. Treatment for pseudotumor cerebri includes weight loss and medications, as well as lumbar punctures, shunt, and optic nerve fenestration.](0a5bed97-d570-4eeb-b531-590bbaca6290) +*Coronal T1WI C+ FS MR in the same patient shows diffuse enhancement of the optic nerve sheaths associated with prominent subarachnoid spaces along the optic nerves. Treatment for pseudotumor cerebri includes weight loss and medications, as well as lumbar punctures, shunt, and optic nerve fenestration.* + +![Sagittal T1 MR in a patient with IIH shows a partially empty sella with the pituitary gland flattened along the floor of the sella.](72675faf-c940-4f75-9867-fd6a13d4baca) +*Sagittal T1 MR in a patient with IIH shows a partially empty sella with the pituitary gland flattened along the floor of the sella.* + +![MIP image of a postcontrast MR venogram study in the same patient shows stenosis of the distal transverse sinuses bilaterally . Transverse sinus stenosis is common in patients with IIH.](a5ccfa9c-e717-4afd-9745-28dfcf0d1498) +*MIP image of a postcontrast MR venogram study in the same patient shows stenosis of the distal transverse sinuses bilaterally . Transverse sinus stenosis is common in patients with IIH.* + +![Axial T2WI MR in a young obese female with headaches and visual symptoms shows mild dilatation of the CSF spaces around the optic nerves. There is mild flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe.](84a7ddc8-9ad0-4905-8866-acb2c4b60502) +*Axial T2WI MR in a young obese female with headaches and visual symptoms shows mild dilatation of the CSF spaces around the optic nerves. There is mild flattening of the posterior sclera and minimal protrusion of the optic nerve papilla into the posterior globe.* + +![3D TOF-MR venogram image in the same patient shows stenosis in the distal transverse sinuses bilaterally . Stent placement in sinus stenosis with significant pressure differentials has shown to reduce papilledema.](21466142-7771-4e31-ac8a-4b6fd8d008d5) +*3D TOF-MR venogram image in the same patient shows stenosis in the distal transverse sinuses bilaterally . Stent placement in sinus stenosis with significant pressure differentials has shown to reduce papilledema.* + diff --git a/out/intracranial-hypotension_818a7972-1032-4d3e-a65a-97c494334aac.md b/out/intracranial-hypotension_818a7972-1032-4d3e-a65a-97c494334aac.md new file mode 100644 index 0000000..2964b29 --- /dev/null +++ b/out/intracranial-hypotension_818a7972-1032-4d3e-a65a-97c494334aac.md @@ -0,0 +1,480 @@ +--- +title: "Intracranial Hypotension" +docid: "818a7972-1032-4d3e-a65a-97c494334aac" +authors: + - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850" + value: "Anne G. Osborn, MD, FACR" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "CSF Disorders" + slug: "csf-disorders" + treeNodeId: "d305bd95-7cca-4888-80b9-fabe45d84ee5" + - + name: "Intracranial Hypotension" + slug: "intracranial-hypotension" + treeNodeId: null +category: "Brain" +documentVersionId: "5af54552-b0b6-49ed-a983-ba5e31060fe7" +imageCount: 20 +lastUpdated: "06/30/25" +pageDescription: "Intracranial Hypotension" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, CSF Disorders, Intracranial Hypotension" +pageTitle: "Intracranial Hypotension | STATdx" +enhancedTitle: "Intracranial Hypotension" +type: "DX" +references: true +cases: 2 +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "CSF Disorders" + - "Intracranial Hypotension" +--- +# KEY FACTS + +- ## Terminology + + + - Headache caused by ↓ intracranial CSF pressure +- ## Imaging + + + - Classic imaging quintet + - Downward displacement of brain through incisura (brain "sagging") + - Diffuse dural thickening/enhancement + - Veins, dural sinuses distended + - Engorged pituitary gland + - Subdural hygromas/hematomas + - Lack of 1 or more of 5 classic findings does not preclude diagnosis + - Cranial MR diagnostic in 90% but 10% normal +- ## Top Differential Diagnoses + + + - Meningitis + - Meningeal metastases + - Chronic subdural hematoma + - Dural sinus thrombosis + - Postsurgical dural thickening + - Idiopathic hypertrophic cranial pachymeningitis +- ## Clinical Issues + + + - Severe headache (orthostatic, persistent, pulsatile, or even associated with nuchal rigidity) + - Uncommon: Cranial nerve (CN) palsy (e.g., abducens), visual disturbances + - Rare: Severe encephalopathy with disturbances of consciousness + - Profile: Young/middle-aged adult with orthostatic headache + - Opening pressure often < 6 cm H₂O but normal pressure common and should not exclude diagnosis +- ## Diagnostic Checklist + + + - Frequently misdiagnosed clinically; imaging is key to diagnosis + - Do not misdiagnose intracranial hypotension as Chiari 1! + - Surgery can exacerbate symptoms; in rare cases can be fatal + - Only rarely are **all** classic clinical and imaging findings of spontaneous intracranial hemorrhage present in same patient + +# TERMINOLOGY + +- ## Abbreviations + + + - Intracranial hypotension (IH) +- ## Definitions + + + - Headache caused by ↓ intracranial cerebrospinal fluid (CSF) pressure + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Classic imaging quintet + - Downward displacement of brain through incisura (brain "sagging") + - Diffuse dural thickening/enhancement + - Veins, dural sinuses distended + - Subdural hygromas/hematomas + - Engorged pituitary gland + - Lack of 1 or more of 5 classic findings does not preclude diagnosis + - Cranial MR diagnostic in 90% of cases but 10% normal + - ### Location + + + - Pachymeninges (dura) + - Both supra- and infratentorial + - May extend into internal auditory canals + - Spinal dura, epidural venous plexuses may be involved + - ### Morphology + + + - Dural enhancement is smooth, not nodular or "lumpy-bumpy" +- ## CT Findings + + + - ### NECT + + + - Relatively insensitive; may appear normal + - Look for effaced suprasellar/basilar cisterns, "fat" midbrain/pons + - ± thick dura + - ± subdural fluid collections + - Usually bilateral + - CSF (hygroma) or blood (hematoma) + - Atria of lateral ventricles may appear deviated medially, abnormally close ("tethered") to midline + - Skull base defect on bone CT rarely causes spontaneous IH + - ### CECT + + + - Diffuse dural thickening, enhancement +- ## MR Findings + + + - ### T1WI + + + - Sagittal view shows brain descent in 40-50% of cases + - "Sagging" midbrain + - Midbrain displaced inferiorly below level of dorsum sellae + - Pons may be compressed against clivus + - ↓ angle between peduncles, pons + - Caudal displacement of tonsils in 25-75% + - Caution: Do not misdiagnose as Chiari 1 malformation; look for other imaging features of brain "sagging" + - Optic chiasm, hypothalamus draped over sella + - Pituitary enlarged above sella in 50% + - ↓ angle between internal cerebral veins (ICVs), vein of Galen + - ↓ vertical distance between pons, mammillary bodies + - Axial + - Suprasellar cistern crowded/effaced + - Midbrain, pons appear elongated ("fat" midbrain) + - Interpeduncular angle ↓ (avg = 25⁰) + - Temporal lobes herniated over tentorium, into incisura + - Lateral ventricles small, often distorted + - Atria pulled medially by downward displacement of midbrain + - Coronal + - Severe cases show ↓ venous angle (< 120°) between roofs of lateral ventricles + - Bilateral subdural fluid collections in 35% + - 70% hygromas (clear fluid collects within dural border cell layer) + - 10% hematomas (blood of variable signal intensity) + - ### T2WI + + + - Thickened dura usually hyperintense + - Subdural fluid (variable signal) + - ### FLAIR + + + - Hyperintense dura, subdural fluid + - Hyperintense midbrain corticospinal tracts in 55% + - ### T2* GRE + + + - May bloom if hemorrhage present + - Rare: Superficial siderosis + - ### T1WI C+ + + + - Diffuse, smooth intense dural enhancement in 85% + - Often extends into cerebellopontine angles (CPAs) + - Veins/dural sinuses distended (convex margins) +- ## Ultrasonographic Findings + + + - ### Color Doppler + + + - Enlarged superior ophthalmic veins with higher mean maximum flow velocity +- ## Angiographic Findings + + + - Cortical, medullary veins may be diffusely enlarged +- ## Nonvascular Interventions + + + - Spinal imaging (site of leak identified in ≈ 50%) + - Digital subtraction myelography > dynamic CT myelography + - Type 1 leaks = dural tear, extradural CSF collection + - Almost always associated with extradural CSF collection + - Adjacent bony abnormality (osteophyte, calcification) common + - 1a leak = ventral + - 1b = posterolateral + - Type 2 leaks = meningeal diverticula > 8 mm + - 2a = single or multiple diverticula + - 2b = complex meningeal diverticula or dural ectasia + - Type 3 leaks = direct CSF-venous fistulas + - Never show extradural CSF collection + - Single vein or network of dilated veins around root sleeve + - Type 4 leaks = without confirmed source on dedicated spinal imaging + - Extradural CSF in 50%; 50% have no evidence of extradural CSF +- ## Nuclear Medicine Findings + + + - Radionuclide cisternography (RNC) + - Direct findings: Focal accumulation of radioactivity outside of subarachnoid space at leakage site + - Indirect findings + - Rapid washout from CSF space + - Early appearance of activity in kidneys, urinary bladder + - Poor migration of isotope over convexities +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Contrast-enhanced cranial MR for diagnosis + - Dynamic CT myelography for leak localization + - ### Protocol advice + + + - Search for actual leakage site only if + - 2 technically adequate blood patches fail + - Posttraumatic leak is suspected + +# DIFFERENTIAL DIAGNOSIS + +- [Meningitis](/document/meningitis/7fdf69fa-c171-4e6b-b6d7-4e8fc94fdc53) + - Pia-subarachnoid enhancement > dura-arachnoid +- ## Meningeal Metastases + + + - Enhancement usually thicker, irregular ("bumpy") +- [Chronic Subdural Hematoma](/document/chronic-subdural-hematoma/cc7b52b4-c6a0-4b4e-ae8c-f05bfc5c5cb2) + - Look for enhancing membranes with blood products +- [Dural Sinus Thrombosis](/document/dural-sinus-thrombosis/4e81a1de-df92-4172-99ec-1377b0d9d188) + - Look for thrombosed sinus (empty delta sign, etc.) +- ## Postsurgical Dural Thickening + + + - Look for other postoperative findings (e.g., burr holes) + - May occur almost immediately after surgery, persist for months/years +- ## Idiopathic Hypertrophic Cranial Pachymeningitis + + + - Headache usually not orthostatic + - May cause bone invasion + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Dural thickening, enhancement due to venous engorgement + - Common cause of IH = spontaneous spinal CSF leak + - Weak dura ± arachnoid diverticulae common + - Aberrant extracellular matrix with abnormalities of fibrillin-containing microfibrils + - Most (but not all) cases caused by reduced CSF pressure precipitated by + - Surgery (CSF overshunting) or trauma (including trivial fall) + - Vigorous exercise or violent coughing + - Diagnostic lumbar puncture + - Spontaneous dural tear, ruptured arachnoid diverticulum + - Severe dehydration + - Disc herniation or osteophyte (rare) + - Pathophysiology = Monro-Kellie doctrine + - CSF, intracranial blood volume vary inversely + - In face of low CSF pressure, dural venous plexuses dilate + - ### Associated abnormalities + + + - Dilated cervical epidural venous plexus, spinal hygromas, retrospinal fluid collections + - Typical + - Low opening pressure, < 6 cm H₂O + - CSF frequently shows pleocytosis, ↑ protein + - Variant + - Opening pressure occasionally normal (CSF hypovolemia rather than hypotension) + - Stigmata of systemic connective tissue disorder found in up to 2/3 of patients + - Marfan, Ehlers-Danlos type 2 + - Clinical findings = minor skeletal features, small joint hypermobility, etc.; may be subtle +- ## Gross Pathologic & Surgical Features + + + - Surgical specimen generally unremarkable with grossly normal-appearing dura + - Spinal meningeal diverticula (often multiple), dural holes/rents common + - No specific leakage site identified at surgery in at least 50% +- ## Microscopic Features + + + - Meningeal surface normal + - No evidence for inflammation or neoplasia + - Inner surface + - Layer of numerous delicate, thin-walled dilated, vessels often attached to inner surface + - Nests of meningothelial cells may be prominent, should not be misinterpreted as meningioma + - May show marked arachnoidal, dural fibrosis if longstanding + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Severe headache (orthostatic, persistent, pulsatile, or even associated with nuchal rigidity) + - Uncommon: Cranial nerve (CN) palsy (e.g., abducens), visual disturbances + - Rare: Severe encephalopathy with disturbances of consciousness + - ### Clinical profile + + + - Young/middle-aged adult with orthostatic headache + - Lack of orthostatic headache should not exclude spontaneous IH! + - Opening pressure often < 6 cm H₂O but normal pressure common and should not exclude diagnosis +- ## Demographics + + + - ### Age + + + - Peak in 3rd, 4th decades +- ## Natural History & Prognosis + + + - Most IH cases resolve spontaneously + - Dural thickening, enhancement disappears; midline structures return (ascend) to normal position + - Rare: Coma, death from severe intracranial herniation +- ## Treatment + + + - "Blind" (nontargeted) epidural blood patch (EBP) + - Immediate: Replaces fluid (tamponade effect) + - Emergent intrathecal saline infusion if patient severely encephalopathic, obtunded + - Negative pressure gradient within epidural space + - Causes rostral flow of injected blood + - Proximal flow of blood products has "plug" effect + - Underreported complication = rebound intracranial **hyper**tension + - Targeted vs. blind EBP + - Targeted EBP 87% successful; 52% nontargeted + - 2-site EBP may be as efficacious as targeted EBP + - Surgery if blood patch fails (usually large dural tear) or subdural hematomas with acute clinical deterioration + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Frequently misdiagnosed clinically; imaging is key to diagnosis + - Do not misdiagnose IH as Chiari 1! + - Surgery can exacerbate symptoms; in rare cases can be fatal +- ## Image Interpretation Pearls + + + - Only rarely are **all** classic findings of IH present in the same patient + - Look for enlarged spinal epidural venous plexuses + + 88645d02-a7df-4c9f-a878-7d8e8140c877 + +## References + +# Selected References + +1. [Davies MJ et al: Epidural blood patch as a diagnostic and therapeutic intervention in spontaneous intracranial hypotension: a novel approach to management. World Neurosurg. 137:e242-50, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32004736%5Bpmid%5D) +1. [Gandhi J et al: Cerebrospinal fluid leaks secondary to dural tears: a review of etiology, clinical evaluation, and management. Int J Neurosci. 1-13, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32242448%5Bpmid%5D) +1. [Martineau P et al: Imaging of the spontaneous low cerebrospinal fluid pressure headache: a review. Can Assoc Radiol J. 71(2):174-85, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32063004%5Bpmid%5D) +1. [Ahn C et al: Two-site blind epidural blood patch versus targeted epidural blood patch in spontaneous intracranial hypotension. J Clin Neurosci. 62:147-54, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30497854%5Bpmid%5D) +1. [Amrhein TJ et al: Spontaneous intracranial hypotension: imaging in diagnosis and treatment. Radiol Clin North Am. 57(2):439-51, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30709479%5Bpmid%5D) +1. [Chan SM et al: Intracranial hypotension and cerebrospinal fluid leak. Neuroimaging Clin N Am. 29(2):213-26, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30926112%5Bpmid%5D) +1. [Farb RI et al: Spontaneous intracranial hypotension: a systematic imaging approach for CSF leak localization and management based on MRI and digital subtraction myelography. AJNR Am J Neuroradiol. 40(4):745-53, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30923083%5Bpmid%5D) +1. [Guryildirim M et al: Acute headache in the emergency setting. Radiographics. 39(6):1739-59, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31589569%5Bpmid%5D) +1. [Kim JH et al: Clinical Features of patients with spontaneous intracranial hypotension complicated with bilateral subdural fluid collections. Headache. 59(5):775-86, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30985923%5Bpmid%5D) +1. [Kim SC et al: MRI findings of spontaneous intracranial hypotension: usefulness of straight sinus distention. AJR Am J Roentgenol. 1-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30807225%5Bpmid%5D) +1. [Kranz PG et al: Spontaneous intracranial hypotension: pathogenesis, diagnosis, and treatment. Neuroimaging Clin N Am. 29(4):581-94, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31677732%5Bpmid%5D) +1. [Wang DJ et al: The interpeduncular angle: a practical and objective marker for the detection and diagnosis of intracranial hypotension on brain MRI. AJNR Am J Neuroradiol. 40(8):1299-1303, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31296521%5Bpmid%5D) +1. [Kranz PG et al: Spontaneous intracranial hypotension: 10 myths and misperceptions. Headache. 58(7):948-59, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29797515%5Bpmid%5D) + +## Cases + +- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '08037059-66e8-40f3-97d5-33a03f1f61ec', 'description': 'C4-5 level CSF leaks along roots sleeves.\n\nMR of the brain (#1-3) shows typical diffuse dural enhancement of intracranial hypotension.\n\nPost myelographic CT images (#4-6) show contrast extending along the roots sleeves bilaterally at C4-6 out into the cervical plexus (arrows).\n\nPatient was treated with multiple blood patches.\n\nPatients with persistent and "occult" CSF leaks do not infrequently have multiple sources involving the cervical-thoracic junction or thoracic spine. Myelography, myelo CT, nuclear cisternography and heavily T2-weighted MR imaging may all be used to try and localize the source of leak.', 'history': 'Severe positional headache.', 'imagePoolId': '4434b392-889b-4575-8190-0b401b9ae3c1', 'name': 'Bilateral cervical root sleeve CSF leaks', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '7fc99261-e3c8-4d04-af7d-99044309e2fe', 'description': "Sagittal T1WI (#1) shows several interesting findings. The midbrain appears "slumped", and the angle between it and the pons (usually almost 90 degrees) appears much more "closed" than normal (open arrow). The cerebellar tonsil lies well below the foramen magnum (white arrow). The pituitary gland is convex upwards, and the optic chiasm is draped over the dorsum sellae (curved arrow). Axial T1WI (#2) through the suprasellar cistern shows tissue- not CSF- within the suprasellar area (arrows). The temporal lobes appear herniated medially (curved arrows), and the surface sulci are obliterated. The axial T2WI (#3) shows that the "flow void" of the superior sagittal sinus (arrow) is unusually large and has convex margins.\n\nThe post-contrast axial (#4-7) and coronal (#8-9) T1WIs are very interesting. They show diffuse dural thickening and enhancement (open arrows), and all the dural sinuses are enlarged, with convex-appearing margins (arrows). Even the superior ophthalmic veins (curved arrows, #5) appear unusually prominent.\n\nSagittal T2WI (#10) through the cervical spine shows very prominent epidural spaces (arrows), probably secondary to engorgement of the epidural venous plexuses.\n\nComment: The low tonsils are round, not pointed (as would be seen in a Chiari 1 malformation). The "fat" pituitary, "slumped" midbrain, diffuse dural thickening and enhancement, and engorged venous sinuses with convex (not concave) margins all indicate intracranial hypotension.\n\nThe patient's symptoms resolved after epidural blood patch, and follow-up MR (not shown) appeared normal.", 'history': 'Worsening postural headaches.', 'imagePoolId': 'a6aae8a1-3c99-4ca6-8c55-239c1fad6ec7', 'name': 'Amazing enlargement of venous sinuses', 'teachingPoint': None, 'demographics': '32 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'f806495a-4edd-416b-9d5d-b395d9a5b417', 'description': 'Intracranial hypotension causes venous engorgement of the dura. Occasionally, it may cause pituitary enlargement and mimic adenoma or pituitary hyperplasia, as happened in this case.\n\nSagittal T1WI MR (#1), demonstrates sagging midbrain. Note upward bulging pituitary gland with draping of the optic chiasm over the gland (arrow). Coronal T1 C+ MR through the sella (#2), shows an enlarged pituitary gland (open arrow) with mild dural thickening (arrows). Coronal post-contrast T1 weighted MR (#3), shows the typical dural thickening and venous engorgement of classic intracranial hypotension.', 'history': 'Intractable headaches relieved by lying down.', 'imagePoolId': '7252d1cc-cefb-47fd-a08a-3a54861f14cd', 'name': 'Enlarged pituitary gland', 'teachingPoint': None, 'demographics': '48 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'f0e41a00-8504-4ea8-a1f1-09123ac2ce47', 'description': 'Sagittal T1WI (#1) shows slight inferior displacement of the tonsils below the foramen magnum (arrow) and decreased distance between the optic chiasm and sella (curved arrow). The midbrain is not obviously "slumping." FLAIR scan (#2) shows hyperintense thickened dura (arrows). Axial (#3) and coronal (#4, 5) show diffusely enhancing, smoothly thickened dura. \n\nThis is a case where the central inferior midbrain displacement is minimal but the dural engorgement and thickening is relatively striking. Intracranial hypotension (in this case, spontaneous) may display a spectrum of findings, not all of which are present in every case.', 'history': 'Headaches. No history of trauma.', 'imagePoolId': 'c4de09d4-8e1a-456e-9b77-e057efe38740', 'name': 'Mild slumping midbrain, striking dural enhancement', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '98e82d35-9959-47fa-95dc-2ba5e8500712', 'description': "Intracranial hypotension is typically caused by CSF leak. History of trauma or other predisposing condition is often lacking and the diagnosis is established when patients are imaged for headache. Contrast-enhanced scans should be done when sagittal T1WI MR suggests "slumping midbrain" (as was seen in this case).\n\nSagittal T1 weighted MR scan shows the classic appearance of "slumping midbrain," with the junction between the midbrain and pons displaced inferiorly below the level of the dorsum sellae (#1, arrow). The suprasellar cistern may be effaced, with the optic chiasm and hypothalamus draped over the dorsum (#1, open arrow). Tonsillar herniation is common but is minimal in this case. Axial pre-contrast T1WI shows the suprasellar cistern is effaced and both temporal lobes have herniated medially over the tentorial incisura (#2, arrows). Axial post-contrast T1WIs (#3-4) show diffuse dural enhancement that extends into the internal auditory canals. Diagnosis was intracranial hypotension and a blood patch was performed. Patient's symptoms resolved.\n\nFollow-up scan (#5-6) performed 12 months later was normal. Note normal position of the pons and optic chiasm (#5) and resolution of abnormal dural thickening and enhancement (#6) as compared to the original examination.", 'history': 'Intractable chronic headaches, relieved in supine position.', 'imagePoolId': '1df83528-3d6c-470c-8e8b-e1be7a4e6567', 'name': 'Classic with resolution s/p blood patch', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '7eff3cb8-762c-48f0-872a-5825f9f21379', 'description': "Intracranial hypotension can be life-threatening if the downwards herniation becomes severe or if subdural hematomas develop.\n\nCoronal T2WI MR (#1), shows the fluid collections are SDHs of different ages. Drainage of the SDHs without recognizing the underlying diagnosis of spontaneous IH caused worsening of the patient's symptoms. Sagittal T1 C+ MR (#2), shows obliteration of suprasellar cistern, sagging and fat midbrain with closed angle between peduncles/pons (arrow), dural enhancement, tonsillar descent. Axial T1 C+ MR (#3), shows diffuse dural enhancement, hypodense extra-axial fluid collections, small ventricles with medial deviation of choroid and ICVs (arrows) caused by midbrain descent. Coronal T1 C+ MR (#4), shows subdural fluid (open arrows) with diffuse dural thickening extending into both IACs (arrows). Lateral ventricles are pulled towards the midline.", 'history': 'Severe postural headaches for several weeks, presented with drowsiness and decreased mental status.', 'imagePoolId': '173f8ea3-1bd0-4b35-a298-bb631d7829bb', 'name': 'Classic MR, severe', 'teachingPoint': None}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': 'a3fd8b37-12fd-4db2-9cee-66847267a893', 'description': 'Brain study (#1, 2) show typical appearance of intracranial hypotension with diffuse dural enhancement both above and below the tentorium. Radionuclide cisternography (anterior view) (#3) show a large amount of activity spilling from the spinal thecal sac into the right chest (arrows). CT following myelogram (#4-12) shows leakage of contrast from the thoracic thecal sac through the right T4-5 neural foramen into the right pleural space (arrows). More inferior axial slice (#8, 12) shows prominent epidural soft tissue due to distended epidural veins (curved arrows). MR study (#13-17) shows diffuse dural thickening and enhancement (arrows, #13-14), and the site of CSF leak extending from the right neural foramen (#15-17). Patient required right laminectomy at T4 to repair the leak.', 'history': 'Recent right thoracotomy for lung carcinoma, now with unremitting severe postural headache.', 'imagePoolId': 'abe41d93-65e8-42fe-b525-1714847527e8', 'name': 'CSF leak following thoracotomy', 'teachingPoint': None, 'demographics': '50 Years old female'}, {'authors': [{'key': '33151213-01b2-4542-9105-342e006b3915', 'value': 'H. Ric Harnsberger, MD'}], 'caseVersionId': '57d36d6f-00c4-4fb7-887c-4c81c842cc8a', 'description': 'Variant CT-MR case of arachnoid granulation in the area of the petromastoid canal (subarcuate canaliculus) with associated CSF leak, intracranial hypotension, and multiple episodes of meningitis.\n\nAxial bone CT images (#1-2) reveal a large arachnoid granulation in the roof of the petrous apex (arrow, #1) and opacification of the middle ear and mastoid (curved arrows, #1-2). CT with intrathecal contrast shows opacified CSF traversing the arachnoid granulation (arrows, #3-4) into temporal bone air cells and into the middle ear cavity (curved arrow, #4).\n \nAn axial T2 MR image (#5) demonstrates fluid signal in the middle ear and mastoid (curved arrows) from CSF leak into these areas. Axial (#6) and coronal (#7-9) T1 C+ fat-saturated MR images show the non-enhancing arachnoid granulation in the petrous apex (arrows, #6-9). Diffuse dural enhancement (open arrows, #7-9) most likely is secondary to intracranial hypotension from the CSF leak. Pia-arachnoid enhancement seen along the nerves of the IAC (curved arrows, #9) results from the recurrent meningitis episodes.', 'history': 'Patient presents with recurrent episodes of meningitis; skull base CT shows an abnormality in the right petrous apex. CT with intrathecal contrast and MR completed for further evaluation.', 'imagePoolId': 'c602dfeb-e877-4a87-871c-c610cb8b21a6', 'name': 'Variant AG with CSF leak and intracranial hypotension', 'teachingPoint': None, 'demographics': '75 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '81585ec2-bd1f-4481-8632-ff6b10bb2e7d', 'description': 'Sagittal T1WI (#1) shows a slightly "slumping" brainstem with the hypothalamus and optic chiasm draped over the dorsum sellae (curved arrow). Note absence of tonsillar herniation. Axial T1WI (#2) shows both temporal lobes are herniated medially over the tentorium (arrows) and there is complete effacement of the suprasellar subarachnoid cistern, seen here as absence of CSF. Axial T2WI through the posterior fossa (#3) shows a hyperintense band (arrows) around both cerebellar hemispheres (arrows). Post-contrast axial (#4-5) T1 C+ scans show diffuse dural enhancement that surrounds the cerebral and cerebellar hemispheres and even extends into both internal auditory canals (#4, arrows).\n\nNot all findings of intracranial hypotension are present in every case. This example is unusual because it lacks the downwards tonsillar herniation (sometimes misnamed "acquired Chiari I") that is seen in most cases.', 'history': 'Severe headaches relieved by supine position.', 'imagePoolId': '00a36dac-2e02-40ae-9c43-499956f81a67', 'name': 'Desc transtentorial herniation', 'teachingPoint': None}], 'caseType': 'variant', 'name': 'VARIANT'} + + +## Images + + +### Selected Images + +![Graphic shows IH with distended dural sinuses and enlarged pituitary and herniated tonsils . Central brain descent causes midbrain slumping, inferiorly displaced pons, closed pons-midbrain angle , and splenium depressing ICV/vein of Galen junction .](images/app.statdx.com_image_thumbnail_3f47f189-81ba-42ab-a5f7-5b241e621ca1_annotated_true_size_900_quality_90_d63c8d90_20251018T164727Z.jpg) +*Graphic shows IH with distended dural sinuses and enlarged pituitary and herniated tonsils . Central brain descent causes midbrain slumping, inferiorly displaced pons, closed pons-midbrain angle , and splenium depressing ICV/vein of Galen junction .* + +![Graphic shows IH with distended dural sinuses and enlarged pituitary and herniated tonsils . Central brain descent causes midbrain slumping, inferiorly displaced pons, closed pons-midbrain angle , and splenium depressing ICV/vein of Galen junction .](images/app.statdx.com_image_thumbnail_3f47f189-81ba-42ab-a5f7-5b241e621ca1_size_174_quality_85_38151f0c_20251018T164552Z.jpg) +*Graphic shows IH with distended dural sinuses and enlarged pituitary and herniated tonsils . Central brain descent causes midbrain slumping, inferiorly displaced pons, closed pons-midbrain angle , and splenium depressing ICV/vein of Galen junction .* + +![T2 MR in a 57-year-old man treated for migraine headaches shows severe midbrain slumping , downwardly displaced cerebellar tonsils , and "draping" of the hypothalamus over the dorsum sellae with mammillary bodies below the dorsum.](images/app.statdx.com_image_thumbnail_bbafbf07-9d0c-4fe0-8e81-fcbe63a510fc_annotated_true_size_900_quality_90_2000dafd_20251018T164727Z.jpg) +*T2 MR in a 57-year-old man treated for migraine headaches shows severe midbrain slumping , downwardly displaced cerebellar tonsils , and "draping" of the hypothalamus over the dorsum sellae with mammillary bodies below the dorsum.* + +![Sagittal T1 C+ FS MR in the same patient shows the severe midbrain slumping and inferiorly displaced tonsils. In addition, the pituitary gland appears "fat" and the dural venous sinuses are engorged .](images/app.statdx.com_image_thumbnail_e7a59de5-ab4b-48bb-9500-1858e5026fbc_annotated_true_size_900_quality_90_1e0d484e_20251018T164727Z.jpg) +*Sagittal T1 C+ FS MR in the same patient shows the severe midbrain slumping and inferiorly displaced tonsils. In addition, the pituitary gland appears "fat" and the dural venous sinuses are engorged .* + +![Axial T1 C+ FS MR in the same patient shows a "fat" midbrain/pons , prominent superior ophthalmic veins , and engorged, outwardly convex transverse/sigmoid sinuses . No subdural hematomas were identified. Severe IH was treated successfully with a blood patch.](images/app.statdx.com_image_thumbnail_0325d630-656f-4d97-b5a9-0ab52423cdfd_annotated_true_size_900_quality_90_89e7c2fe_20251018T164727Z.jpg) +*Axial T1 C+ FS MR in the same patient shows a "fat" midbrain/pons , prominent superior ophthalmic veins , and engorged, outwardly convex transverse/sigmoid sinuses . No subdural hematomas were identified. Severe IH was treated successfully with a blood patch.* + +![Axial NECT in a 55-year-old man with a severe headache in the ER shows downward herniation of both cerebellar tonsils through the foramen magnum.](images/app.statdx.com_image_thumbnail_f21667f8-6e56-4344-99f0-01881e854752_annotated_true_size_900_quality_90_4924f2e1_20251018T164727Z.jpg) +*Axial NECT in a 55-year-old man with a severe headache in the ER shows downward herniation of both cerebellar tonsils through the foramen magnum.* + +![More cephalad NECT in the same patient shows effacement of all basal cisterns, especially the suprasellar cistern . The midbrain appears "fat" . The imaging findings are suggestive of IH.](images/app.statdx.com_image_thumbnail_5fd9a6e2-7068-49f5-8e86-556f3db7c42e_annotated_true_size_900_quality_90_127dfa34_20251018T164727Z.jpg) +*More cephalad NECT in the same patient shows effacement of all basal cisterns, especially the suprasellar cistern . The midbrain appears "fat" . The imaging findings are suggestive of IH.* + +![Sagittal T1WI MR in the same patient shows changes of severe IH with midbrain slumping , downward tonsillar displacement , "fat" pituitary gland , and "draping" of the optic chiasm/hypothalamus over the dorsum sellae .](images/app.statdx.com_image_thumbnail_580c9dda-241e-459c-9d69-df0d5191353d_annotated_true_size_900_quality_90_00072ee4_20251018T164727Z.jpg) +*Sagittal T1WI MR in the same patient shows changes of severe IH with midbrain slumping , downward tonsillar displacement , "fat" pituitary gland , and "draping" of the optic chiasm/hypothalamus over the dorsum sellae .* + +![Axial T2WI MR in the same patient shows the inferiorly displaced hypothalamus and 3rd ventricle obliterating the suprasellar cistern . The midbrain appears "fat" and elongated.](images/app.statdx.com_image_thumbnail_455ec428-a8ce-408e-94c3-7b2facee16e4_annotated_true_size_900_quality_90_b287112d_20251018T164727Z.jpg) +*Axial T2WI MR in the same patient shows the inferiorly displaced hypothalamus and 3rd ventricle obliterating the suprasellar cistern . The midbrain appears "fat" and elongated.* + +![Axial T1 C+ FS MR in the same patient shows smooth, diffuse dura-arachnoid enhancement , and an engorged, outwardly convex superior sagittal sinus .](images/app.statdx.com_image_thumbnail_f63a1d44-5412-4dd6-ab9d-dd2091c16f6b_annotated_true_size_900_quality_90_eb95d811_20251018T164727Z.jpg) +*Axial T1 C+ FS MR in the same patient shows smooth, diffuse dura-arachnoid enhancement , and an engorged, outwardly convex superior sagittal sinus .* + +![Coronal T1 C+ FS MR in the same patient shows the diffuse dura-arachnoid enhancement extends into both internal auditory canals . The lateral ventricles have a more acute angle and appear "pulled down" toward the incisura. An epidural blood patch relieved the symptoms.](images/app.statdx.com_image_thumbnail_5276ba18-7d76-4d61-8a2b-6058ab8f226b_annotated_true_size_900_quality_90_07652c9d_20251018T164731Z.jpg) +*Coronal T1 C+ FS MR in the same patient shows the diffuse dura-arachnoid enhancement extends into both internal auditory canals . The lateral ventricles have a more acute angle and appear "pulled down" toward the incisura. An epidural blood patch relieved the symptoms.* + + +### Additional Images + +![Sagittal T1WI C+ MR in a patient with severe IH shows obliteration of the suprasellar cistern, "sagging" and "fat" midbrain with closed angle between the peduncles/pons , dural enhancement, and tonsillar descent.](images/app.statdx.com_image_thumbnail_853d940e-4247-482c-8fbb-cea42f02bc4e_annotated_true_size_900_quality_90_2c33b49b_20251018T164731Z.jpg) +*Sagittal T1WI C+ MR in a patient with severe IH shows obliteration of the suprasellar cistern, "sagging" and "fat" midbrain with closed angle between the peduncles/pons , dural enhancement, and tonsillar descent.* + +![Axial T1WI C+ MR in the same patient shows diffuse dural enhancement, hypodense extraaxial fluid collections, and small ventricles with medial deviation of the choroid and internal cerebral veins caused by midbrain descent.](images/app.statdx.com_image_thumbnail_9e92d281-2a10-418d-bb72-522547caf29b_annotated_true_size_900_quality_90_e8be1938_20251018T164731Z.jpg) +*Axial T1WI C+ MR in the same patient shows diffuse dural enhancement, hypodense extraaxial fluid collections, and small ventricles with medial deviation of the choroid and internal cerebral veins caused by midbrain descent.* + +![Coronal T1WI C+ MR in the same patient shows subdural fluid with diffuse dural thickening extending into both internal auditory canals . The lateral ventricles are pulled toward the midline.](images/app.statdx.com_image_thumbnail_4bc5f106-f884-480a-bbc6-1d456488613e_annotated_true_size_900_quality_90_de26b61d_20251018T164731Z.jpg) +*Coronal T1WI C+ MR in the same patient shows subdural fluid with diffuse dural thickening extending into both internal auditory canals . The lateral ventricles are pulled toward the midline.* + +![Coronal T2WI MR shows that the fluid collections are subdural hematomas of different ages. Drainage of the subdural hematomas without recognizing the underlying diagnosis of spontaneous IH caused worsening of the patient's symptoms.](images/app.statdx.com_image_thumbnail_1c0fc05e-ae2a-4271-a79d-cfe79cb4cbaf_annotated_true_size_900_quality_90_7a7da97d_20251018T164731Z.jpg) +*Coronal T2WI MR shows that the fluid collections are subdural hematomas of different ages. Drainage of the subdural hematomas without recognizing the underlying diagnosis of spontaneous IH caused worsening of the patient's symptoms.* + +![Axial T1WI C+ MR at the C2 level in a patient with spontaneous IH shows the draped curtain appearance of the markedly engorged epidural venous plexus . Brain MR (not shown) showed only mild dural enhancement.](images/app.statdx.com_image_thumbnail_7b81ece7-66b8-483a-84be-c9d986b4f22c_annotated_true_size_900_quality_90_24b3411c_20251018T164731Z.jpg) +*Axial T1WI C+ MR at the C2 level in a patient with spontaneous IH shows the draped curtain appearance of the markedly engorged epidural venous plexus . Brain MR (not shown) showed only mild dural enhancement.* + +![Coronal T1WI C+ MR in the same patient shows an enlarged pituitary gland with mild dural thickening .](images/app.statdx.com_image_thumbnail_feba1690-6fec-4c28-b9e4-0599b470158e_annotated_true_size_900_quality_90_ad0019d8_20251018T164732Z.jpg) +*Coronal T1WI C+ MR in the same patient shows an enlarged pituitary gland with mild dural thickening .* + +![Sagittal T1WI MR shows a rounded, "plump" pituitary gland , often seen in IH. Note the effaced suprasellar cistern with optic chiasm draped over the pituitary gland, classic "slumping" midbrain, with decreased angle between the pons and midbrain .](images/app.statdx.com_image_thumbnail_9d45611b-ca6b-412d-b18e-3e28ed55daa7_annotated_true_size_900_quality_90_8502c539_20251018T164732Z.jpg) +*Sagittal T1WI MR shows a rounded, "plump" pituitary gland , often seen in IH. Note the effaced suprasellar cistern with optic chiasm draped over the pituitary gland, classic "slumping" midbrain, with decreased angle between the pons and midbrain .* + +![Sagittal T1WI MR shows "sagging" midbrain, tonsillar herniation, and the optic chiasm "draped" over the dorsum sellae. T1WI C+ MR (not shown) demonstrated diffuse dural enhancement in this classic case of spontaneous IH.](images/app.statdx.com_image_thumbnail_df286399-1c32-47a3-919c-d093c5e1699b_annotated_true_size_900_quality_90_81f62a20_20251018T164732Z.jpg) +*Sagittal T1WI MR shows "sagging" midbrain, tonsillar herniation, and the optic chiasm "draped" over the dorsum sellae. T1WI C+ MR (not shown) demonstrated diffuse dural enhancement in this classic case of spontaneous IH.* + +![Axial T1WI C+ FS MR in a patient with spontaneous IH shows diffuse dura-arachnoid thickening from venous engorgement. Note extension into cerebellopontine angles .](images/app.statdx.com_image_thumbnail_00b6b708-959a-4d66-ae4a-958137970aad_annotated_true_size_900_quality_90_d535a53a_20251018T164732Z.jpg) +*Axial T1WI C+ FS MR in a patient with spontaneous IH shows diffuse dura-arachnoid thickening from venous engorgement. Note extension into cerebellopontine angles .* + +![Axial T1WI C+ MR in the same patient after the blood patch shows complete resolution of dura-arachnoid enhancement.](images/app.statdx.com_image_thumbnail_d9028720-92df-433e-8d90-e962df59b5ef_annotated_true_size_900_quality_90_21f8c1e2_20251018T164732Z.jpg) +*Axial T1WI C+ MR in the same patient after the blood patch shows complete resolution of dura-arachnoid enhancement.* + diff --git a/out/intraventricular-obstructive-hydrocephalus_eeac8d9b-1fdc-432e-8e09-11589611f7a8.md b/out/intraventricular-obstructive-hydrocephalus_eeac8d9b-1fdc-432e-8e09-11589611f7a8.md new file mode 100644 index 0000000..016feb6 --- /dev/null +++ b/out/intraventricular-obstructive-hydrocephalus_eeac8d9b-1fdc-432e-8e09-11589611f7a8.md @@ -0,0 +1,492 @@ +--- +title: "Intraventricular Obstructive Hydrocephalus" +docid: "eeac8d9b-1fdc-432e-8e09-11589611f7a8" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "Intraventricular Obstructive Hydrocephalus" + slug: "intraventricular-obstructive-hydro-" + treeNodeId: null +category: "Brain" +cmeTopicId: "7f17d5cc-6470-403d-8f99-cbb3d3ea610d" +documentVersionId: "102c8265-55ef-4963-964e-d8ed6d766992" +imageCount: 30 +lastUpdated: "09/24/20" +pageDescription: "Intraventricular Obstructive Hydrocephalus" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Intraventricular Obstructive Hydrocephalus" +pageTitle: "Intraventricular Obstructive Hydrocephalus | STATdx" +enhancedTitle: "Intraventricular Obstructive Hydrocephalus" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "Intraventricular Obstructive Hydrocephalus" +--- +# KEY FACTS + +- ## Terminology + + + - Intraventricular obstructive hydrocephalus (IVOH) = obstruction proximal to foramina of Luschka, Magendie + - Acute IVOH (aIVOH) + - Chronic compensated IVOH (cIVOH) +- ## Imaging + + + - aIVOH = ballooned ventricles plus indistinct (blurred) margins + - "Fingers" of CSF extend into periventricular WM + - Most striking around ventricular horns (periventricular halos) + - After decompression, corpus callosum may show hyperintensity + - cIVOH = ballooned ventricles without periventricular halo +- ## Top Differential Diagnoses + + + - Ventricular enlargement secondary to parenchymal loss + - Normal-pressure hydrocephalus + - Extraventricular obstructive hydrocephalus + - Choroid plexus papilloma +- ## Pathology + + + - Intraventricular obstruction to CSF flow + - CSF production continues, ventricular pressure ↑ + - Ventricles expand, compress adjacent parenchyma + - Periventricular interstitial fluid ↑ + - Leads to myelin vacuolization, destruction + - Pathology varies depending on obstruction etiology +- ## Clinical Issues + + + - Varies with acuity, severity + - Headache, papilledema (aIVOH) + - Nausea, vomiting, diplopia (6th nerve palsy) +- ## Diagnostic Checklist + + + - Size of ventricles generally correlates poorly with intracranial pressure + +# TERMINOLOGY + +- ## Abbreviations + + + - Intraventricular obstructive hydrocephalus (IVOH) + - Acute IVOH (aIVOH) + - Chronic compensated IVOH (cIVOH) +- ## Synonyms + + + - Noncommunicating hydrocephalus +- ## Definitions + + + - Enlarged ventricles caused by physical obstruction at or proximal to 4th ventricular outflow foramina (of Luschka, Magendie) + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - aIVOH + - Ballooned ventricles with indistinct (blurred) margins + - cIVOH + - Ballooned ventricles without periventricular halo + - ### Size + + + - Bifrontal horn:intracranial diameter ratio > 0.3 + - Temporal horn width > 3 mm + - ### Morphology + + + - Varies with site, duration of blockage + - Global/focally enlarged ventricle(s) ± ↑ intracranial pressure (ICP) + - Ventricles proximal to obstruction enlarge, appear more rounded + - Look for enlarged anterior recesses of 3rd ventricle +- ## CT Findings + + + - ### NECT + + + - Large ventricles proximal to obstruction + - aIVOH + - Ballooned ventricles with periventricular low-density halo + - cIVOH + - Ballooned ventricles without thick periventricular halo + - Basal cisterns, sulci compressed/obliterated +- ## MR Findings + + + - ### T1WI + + + - Lateral ventricles enlarged + - Corpus callosum (CC) thinned, stretched upward + - May be impinged against falx + - Impaction may cause pressure necrosis + - Fornix, internal cerebral veins (ICV) displaced downward + - Enlarged 3rd ventricle often herniated into expanded sella + - Funnel-shaped aqueduct of Sylvius in aqueductal stenosis + - ### T2WI + + + - aIVOH + - "Fingers" of CSF-like hyperintensity extend into periventricular white matter (WM), most striking around ventricular horns (periventricular halos) + - Disturbed/turbulent CSF flow + - Absent aqueductal flow void common + - CC may appear hyperintense + - cIVOH + - Large ventricles, normal CSF pressure + - No periventricular halo + - CC may show hyperintensity after decompression (15% of shunted IVOH cases) + - Thin-section T2WI, FIESTA, or CISS sequences + - Exquisitely delineate CSF spaces + - May demonstrate subtle abnormalities not detected on standard images + - ### FLAIR + + + - Fluid in periventricular halo does not suppress + - ### T1WI C+ + + + - Neoplasm causing IVOH may enhance + - aIVOH may cause leptomeningeal vascular stasis, enhancement + - Can mimic meningitis, metastases + - ### MRS + + + - Small lactate resonances can be detected in up to 20% of CSF spaces, even if no hydrocephalus +- ## Other Modality Findings + + + - Contrast-enhanced ventriculography + - MR/CT used to identify site of obstruction, status of 3rd ventriculostomies + - MR can be used for assessing CSF flow + - Cardiac gated-phase contrast MR + - May show absent aqueductal CSF flow +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR with contrast to evaluate cause of CSF obstruction + - ### Protocol advice + + + - 3D FEISTA/CISS + - ↓ CSF flow artifact + - Allows better delineation of ventricular contour, septa + - Sagittal high-resolution T2-weighted images + +# DIFFERENTIAL DIAGNOSIS + +- ## Ventricular Enlargement Secondary to Parenchymal Loss + + + - Old term: Ex vacuo hydrocephalus (not used) + - Age related (ventricular volume ↑ 1.2-1.4 mL after 60 years) + - Ischemia/infarction, trauma, infection, toxic + - Obtuse frontal angle (> 110°) + - Diffuse/focal enlargement of sulci, cisterns + - Normal lateral ventricles can be asymmetric (related to handedness, not sex) + - May correlate with some psychiatric disorders (e.g., schizophrenia) +- [Normal-Pressure Hydrocephalus](/document/normal-pressure-hydrocephalus/ba3f857d-58de-4f21-8463-1631b4cb9972) + - Progressive dementia, gait disturbance, incontinence + - Ventricular dilation with normal CSF pressure + - Sulci normal/minimally enlarged + - ↑ CSF displacement through aqueduct + - MRS shows lactate peak +- [Extraventricular Obstructive Hydrocephalus](/document/extraventricular-obstructive-hydro-/a0886d4c-f504-4165-bb52-2400e2385f68) + - Dilated ventricles due to mismatch between CSF formation, absorption + - ↓ CSF absorption through arachnoid villi + - Subarachnoid hemorrhage most common cause + - Others: Meningitis, carcinomatosis, granulomatous disease +- [Choroid Plexus Papilloma](/document/choroid-plexus-papilloma/18e712f5-8553-487d-a939-044336cbf0ad) + - Accounts for 2-5% of childhood intracranial tumors + - Child < 5 years with ↑ ICP + - Most common lateral ventricle trigone + - May overproduce CSF + - Hemorrhage, tumor spread may cause IVOH +- ## Longstanding Overt Ventriculomegaly in Adults + + + - Early childhood onset or longstanding progression of hydrocephalus into adulthood + - Markedly enlarged ventricles, high ICP +- ## Benign Enlargement of Subarachnoid Spaces and Ventricles + + + - Seen in association with macrocephaly in infants + - Not associated with developmental delay + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Normal CSF production = ~ 0.4 mL/min, 500-600 mL/24 hrs + - Total volume of CSF in adult = 125-150 mL + - Intraventricular obstruction to CSF flow; as CSF production continues, ventricular fluid pressure ↑ + - Ventricles expand, compress adjacent parenchyma; stretching may rupture/open ependymal cell junctions + - Periventricular interstitial fluid ↑ → myelin destruction + - Etiology depends on site + - Foramen of Monro + - Colloid cyst + - Subependymal nodule, tuberous sclerosis complex + - Subependymal giant cell astrocytoma + - 3rd ventricle + - Pituitary macroadenoma + - Craniopharyngioma + - Aqueduct of Sylvius + - Aqueductal stenosis + - Tectal glioma + - Pineal region tumors + - 4th ventricle + - Medulloblastoma, ependymoma + - Glioma, pilocytic astrocytoma, hemangioblastoma + - Cerebellar infarct + - Congenital anomalies (Chiari malformations, Dandy-Walker malformations, rhombencephalosynapsis) + - Metastasis, neurocysticercosis, or meningioma can occur at multiple intraventricular locations + - ### Genetics + + + - Cell adhesion molecule L1 (*L1CAM*) only gene recognized to cause congenital hydrocephalus + - Located on X chromosome (Xq28) + - Complications of hydrocephalus + - CC impingement syndrome + - Medial atrial diverticula +- ## Gross Pathologic & Surgical Features + + + - Focal/generalized ventricular enlargement + - Ependyma, adjacent WM are secondarily injured + - Variable pathology depending on causative factor +- ## Microscopic Features + + + - ↑ periventricular extracellular space + - Ependymal lining damaged or lost; surrounding WM becomes pale and rarefied + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Varies with acuity, severity + - Headache, papilledema (aIVOH) + - Nausea, vomiting, diplopia (6th nerve palsy) + - ### Clinical profile + + + - Varies with etiology, severity, age of onset +- ## Demographics + + + - ### Age + + + - May be any age from in utero (congenital hydrocephalus) to adult + - ### Epidemiology + + + - Epidemiologic data varies widely, depending upon etiology and type of hydrocephalus +- ## Natural History & Prognosis + + + - Usually progressive unless treated +- ## Treatment + + + - Medical management to delay surgical intervention + - CSF diversion (shunt), endoscopic intervention, and ventriculostomy + - Surgery to alleviate primary cause of obstruction + - Most common neurosurgical procedure in children = CSF shunting for hydrocephalus + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Longstanding aqueductal stenosis can be caused by slow-growing tectal tumor + - Rarely, hydrocephalus caused by spinal tumor +- ## Image Interpretation Pearls + + + - Size of ventricles generally correlates poorly with ICP + - Pulsatile CSF may create confusing signal intensity, even mimic intraventricular mass + - Ventricular asymmetry can be normal variant + - If seen with absent septum pellucidum in fetus/neonate, look for vermis (r/o rhombencephalosynapsis) + + 5b1dd5dd-62fc-4446-8e5d-a43b8e65444e + +## References + +# Selected References + +1. [Gholampour S et al: Comparing the efficiency of two treatment methods of hydrocephalus: shunt implantation and endoscopic third ventriculostomy. Basic Clin Neurosci. 10(3):185-98, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31462974%5Bpmid%5D) +1. [Santiago-Dieppa DR et al: Obstructive hydrocephalus. N Engl J Med. 381(5):e10, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31365804%5Bpmid%5D) +1. [Chellathurai A et al: Role of 3D SPACE sequence and susceptibility weighted imaging in the evaluation of hydrocephalus and treatment-oriented refined classification of hydrocephalus. Indian J Radiol Imaging. 28(4):385-94, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30662197%5Bpmid%5D) +1. [Hurni Y et al: Arrested hydrocephalus in childhood: case series and review of the literature. Neuropediatrics. 49(5):302-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29933461%5Bpmid%5D) +1. [Langner S et al: Diagnosis and differential diagnosis of hydrocephalus in adults. Rofo. 189(8):728-39, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28511266%5Bpmid%5D) +1. [Leinonen V et al: Cerebrospinal fluid circulation and hydrocephalus. Handb Clin Neurol. 145:39-50, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28987185%5Bpmid%5D) +1. [Algin O et al: Assessment of third ventriculostomy patency with the 3D-SPACE technique: a preliminary multicenter research study. J Neurosurg. 122(6):1347-55, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25859808%5Bpmid%5D) +1. [Russo N et al: Endoscopic approaches to intraventricular lesions. J Neurol Surg A Cent Eur Neurosurg. 76(5):353-60, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26008954%5Bpmid%5D) +1. [Flannery AM et al: Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 1: Introduction and methodology. J Neurosurg Pediatr. 14 Suppl 1:3-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25988777%5Bpmid%5D) +1. [Dinçer A et al: Radiologic evaluation of pediatric hydrocephalus. Childs Nerv Syst. 27(10):1543-62, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928020%5Bpmid%5D) +1. [Mirone G et al: Hydrocephalus and spinal cord tumors: a review. Childs Nerv Syst. 27(10):1741-9, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928038%5Bpmid%5D) +1. [Oi S: Classification of hydrocephalus: critical analysis of classification categories and advantages of "multi-categorical hydrocephalus classification" (Mc HC). Childs Nerv Syst. 27(10):1523-33, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21928018%5Bpmid%5D) +1. [Dinçer A et al: Is all "communicating" hydrocephalus really communicating? Prospective study on the value of 3D-constructive interference in steady state sequence at 3T. AJNR Am J Neuroradiol. 30(10):1898-906, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19643921%5Bpmid%5D) +1. [Feng F et al: Evaluation of radionuclide cerebrospinal fluid scintigraphy as a guide in the management of patients with hydrocephalus. Clin Imaging. 33(2):85-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19237049%5Bpmid%5D) +1. [Linninger AA et al: Normal and hydrocephalic brain dynamics: the role of reduced cerebrospinal fluid reabsorption in ventricular enlargement. Ann Biomed Eng. 37(7):1434-47, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19373558%5Bpmid%5D) +1. [Oertel JM et al: Endoscopic third ventriculostomy in obstructive hydrocephalus due to giant basilar artery aneurysm. J Neurosurg. 110(1):14-8, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18991498%5Bpmid%5D) +1. [Stoquart-El Sankari S et al: Phase-contrast MR imaging support for the diagnosis of aqueductal stenosis. AJNR Am J Neuroradiol. 30(1):209-14, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18832663%5Bpmid%5D) +1. [Sekula RF Jr et al: A case of an elderly adult presenting with obstructive hydrocephalus secondary to a rare hemorrhagic suprasellar pilocytic astrocytoma. Clin Neuropathol. 27(6):396-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19130737%5Bpmid%5D) +1. [Yamada S et al: Visualization of cerebrospinal fluid movement with spin labeling at MR imaging: preliminary results in normal and pathophysiologic conditions. Radiology. 249(2):644-52, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18936318%5Bpmid%5D) +1. [Erdogan AR et al: Sex and handedness differences in size of cerebral ventricles of normal subjects. Int J Neurosci. 114(1):67-73, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14660068%5Bpmid%5D) +1. [Gaser C et al: Ventricular enlargement in schizophrenia related to volume reduction of the thalamus, striatum, and superior temporal cortex. Am J Psychiatry. 161(1):154-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14702264%5Bpmid%5D) +1. [Wyldes M et al: Isolated mild fetal ventriculomegaly. Arch Dis Child Fetal Neonatal Ed. 89(1):F9-13, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14711845%5Bpmid%5D) +1. [Akhondi H et al: Hydrocephalus as a presenting manifestation of neurosarcoidosis. South Med J. 96(4):403-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12916562%5Bpmid%5D) +1. [Bhattacharyya KB et al: Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature. Acta Neurol Scand. 108(3):216-20, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12911467%5Bpmid%5D) +1. Brown KP et al: 1H MRS in human hydrocephalus. J MRI. 14:291-9, 2003 +1. [Grunert P et al: The role of third ventriculostomy in the management of obstructive hydrocephalus. Minim Invasive Neurosurg. 46(1):16-21, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12640578%5Bpmid%5D) +1. [Joseph VB et al: MR ventriculography for the study of CSF flow. AJNR Am J Neuroradiol. 24(3):373-81, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12637285%5Bpmid%5D) +1. [Sener RN: Callosal changes in obstructive hydrocephalus: observations with FLAIR imaging, and diffusion MRI. Comput Med Imaging Graph. 26(5):333-7, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12204238%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial FLAIR MR in a patient with headache and vomiting demonstrates a colloid cyst at the foramen of Monro causing intraventricular obstructive hydrocephalus (IVOH) with dilatation of both lateral ventricles . Note thin rim of periventricular hyperintensity due to interstitial edema.](images/app.statdx.com_image_thumbnail_e5c174f6-9b00-41ca-b8e0-52124d6d5d33_annotated_true_size_900_quality_90_3aa6f8d6_20251018T164538Z.jpg) +*Axial FLAIR MR in a patient with headache and vomiting demonstrates a colloid cyst at the foramen of Monro causing intraventricular obstructive hydrocephalus (IVOH) with dilatation of both lateral ventricles . Note thin rim of periventricular hyperintensity due to interstitial edema.* + +![Axial FLAIR MR in a patient with headache and vomiting demonstrates a colloid cyst at the foramen of Monro causing intraventricular obstructive hydrocephalus (IVOH) with dilatation of both lateral ventricles . Note thin rim of periventricular hyperintensity due to interstitial edema.](images/app.statdx.com_image_thumbnail_e5c174f6-9b00-41ca-b8e0-52124d6d5d33_size_174_quality_85_febe2249_20251018T155131Z.jpg) +*Axial FLAIR MR in a patient with headache and vomiting demonstrates a colloid cyst at the foramen of Monro causing intraventricular obstructive hydrocephalus (IVOH) with dilatation of both lateral ventricles . Note thin rim of periventricular hyperintensity due to interstitial edema.* + +![Axial NECT in a patient with pineal region germinoma shows marked dilatation of the lateral ventricles and anterior 3rd ventricle with periventricular halo and diffuse effacement of the cortical sulci.](images/app.statdx.com_image_thumbnail_bb072fec-1cdc-4da0-8dfc-36cfc2a5a110_annotated_true_size_900_quality_90_6685e5f7_20251018T164538Z.jpg) +*Axial NECT in a patient with pineal region germinoma shows marked dilatation of the lateral ventricles and anterior 3rd ventricle with periventricular halo and diffuse effacement of the cortical sulci.* + +![Coronal T1 C+ MR in a patient presenting with headache and ataxia demonstrates a large heterogeneously enhancing mass in the left cerebellum with mass effect and effacement of the 4th ventricle . Biopsy revealed a glioblastoma.](images/app.statdx.com_image_thumbnail_a4d9bbda-78d1-493c-baaf-b7ab31b10da6_annotated_true_size_900_quality_90_3651409b_20251018T164538Z.jpg) +*Coronal T1 C+ MR in a patient presenting with headache and ataxia demonstrates a large heterogeneously enhancing mass in the left cerebellum with mass effect and effacement of the 4th ventricle . Biopsy revealed a glioblastoma.* + +![Axial FLAIR MR in the same patient shows marked dilatation of the lateral ventricles with extensive periventricular interstitial edema caused by compromised drainage of interstitial fluid or transependymal CSF migration.](images/app.statdx.com_image_thumbnail_2d8f3e24-47a1-4c45-9ecc-cebc09885e9d_annotated_true_size_900_quality_90_e373c7b5_20251018T164538Z.jpg) +*Axial FLAIR MR in the same patient shows marked dilatation of the lateral ventricles with extensive periventricular interstitial edema caused by compromised drainage of interstitial fluid or transependymal CSF migration.* + +![Sagittal CISS MR in a patient with obstruction at the 4th ventricular outlet due to adhesions shows ballooning of the 4th ventricle , widening of the aqueduct of Sylvius , dilated 3rd and lateral ventricles with downward sloping of 3rd ventricular floor .](images/app.statdx.com_image_thumbnail_91c986f1-b192-4ee0-86ad-ed4014373565_annotated_true_size_900_quality_90_efdb7ec4_20251018T164538Z.jpg) +*Sagittal CISS MR in a patient with obstruction at the 4th ventricular outlet due to adhesions shows ballooning of the 4th ventricle , widening of the aqueduct of Sylvius , dilated 3rd and lateral ventricles with downward sloping of 3rd ventricular floor .* + +![Axial FLAIR MR in a patient with tuberous sclerosis shows large subependymal giant cell astrocytoma causing obstructive hydrocephalus with mild periventricular edema . Note the subtle hyperintensity in the occipital lobe tuber .](images/app.statdx.com_image_thumbnail_e8bd350a-0dbd-4b07-a628-7ba634965a46_annotated_true_size_900_quality_90_49d18706_20251018T164538Z.jpg) +*Axial FLAIR MR in a patient with tuberous sclerosis shows large subependymal giant cell astrocytoma causing obstructive hydrocephalus with mild periventricular edema . Note the subtle hyperintensity in the occipital lobe tuber .* + +![Sagittal CISS MR in a patient with aqueductal stenosis due to a thin web causing obstructive hydrocephalus is shown. High resolution thin-section T2 MR exquisitely delineates the CSF spaces and may demonstrate subtle abnormalities not detected on standard sequences.](images/app.statdx.com_image_thumbnail_ef27fe2f-6eef-4306-8752-89fecd5fc331_annotated_true_size_900_quality_90_4217aa29_20251018T164538Z.jpg) +*Sagittal CISS MR in a patient with aqueductal stenosis due to a thin web causing obstructive hydrocephalus is shown. High resolution thin-section T2 MR exquisitely delineates the CSF spaces and may demonstrate subtle abnormalities not detected on standard sequences.* + +![Axial FLAIR MR in the same patient shows marked enlarged lateral ventricles with a very thin periventricular hyperintense rim and no sulcal effacement due to chronic compensated IVOH.](images/app.statdx.com_image_thumbnail_ec7e804b-5c09-4235-8429-ee0dc4fc2a7f_annotated_true_size_900_quality_90_cf502530_20251018T164538Z.jpg) +*Axial FLAIR MR in the same patient shows marked enlarged lateral ventricles with a very thin periventricular hyperintense rim and no sulcal effacement due to chronic compensated IVOH.* + +![Axial FLAIR MR in a patient with IVOH shows an ependymal cyst at the foramen of Monro with asymmetric dilatation of the lateral ventricles, L > R. There is marked bulging of the medial wall of the left lateral ventricle.](images/app.statdx.com_image_thumbnail_cb3411d0-41c7-433a-97e3-f121306cf24f_annotated_true_size_900_quality_90_069d90f2_20251018T164538Z.jpg) +*Axial FLAIR MR in a patient with IVOH shows an ependymal cyst at the foramen of Monro with asymmetric dilatation of the lateral ventricles, L > R. There is marked bulging of the medial wall of the left lateral ventricle.* + +![Sagittal T1 MR in the same patient demonstrates the large medial atrial diverticula , which herniates inferiorly through the tentorial incisura into the posterior fossa, compressing the vermis , tectal plate , aqueduct, and 4th ventricle .](images/app.statdx.com_image_thumbnail_9d4aeb38-2745-4513-9b2b-e51d38e60ec2_annotated_true_size_900_quality_90_efd2d55e_20251018T165016Z.jpg) +*Sagittal T1 MR in the same patient demonstrates the large medial atrial diverticula , which herniates inferiorly through the tentorial incisura into the posterior fossa, compressing the vermis , tectal plate , aqueduct, and 4th ventricle .* + + +### Additional Images + +![Sagittal T1WI MR shows large mass within the 4th ventricle causing IVOH or noncommunicating hydrocephalus.](images/app.statdx.com_image_thumbnail_111173b4-2ccd-4d7a-9423-8ea7a67d3faa_annotated_true_size_900_quality_90_db7227b6_20251018T165016Z.jpg) +*Sagittal T1WI MR shows large mass within the 4th ventricle causing IVOH or noncommunicating hydrocephalus.* + +![Sagittal T2WI MR in the same patient shows transependymal CSF flow, seen here as "fingers" extending into white matter around the enlarged lateral ventricle. The case was medulloblastoma with acute IVOH.](images/app.statdx.com_image_thumbnail_0096a55e-9e96-409e-ac0f-01c95f3d26bb_annotated_true_size_900_quality_90_b7750d59_20251018T165017Z.jpg) +*Sagittal T2WI MR in the same patient shows transependymal CSF flow, seen here as "fingers" extending into white matter around the enlarged lateral ventricle. The case was medulloblastoma with acute IVOH.* + +![Coronal T1 C+ MR shows IVOH with a large enhancing intraventricular mass causing marked enlargement of the lateral ventricles .](images/app.statdx.com_image_thumbnail_df9fd7e5-2974-4621-9d50-efceebb95d70_annotated_true_size_900_quality_90_ada0f1a6_20251018T165016Z.jpg) +*Coronal T1 C+ MR shows IVOH with a large enhancing intraventricular mass causing marked enlargement of the lateral ventricles .* + +![Axial NECT in the same patient shows the large intraventricular mass within the 4th ventricle. Note the dilated temporal horns .](images/app.statdx.com_image_thumbnail_5e46b562-c694-4b32-a46c-af0d64e8c289_annotated_true_size_900_quality_90_9842d1b3_20251018T165016Z.jpg) +*Axial NECT in the same patient shows the large intraventricular mass within the 4th ventricle. Note the dilated temporal horns .* + +![Sagittal T1WI MR shows IVOH secondary to aqueductal stenosis and distal stenosis of cerebral aqueduct . Note the enlarged lateral and 3rd ventricles.](images/app.statdx.com_image_thumbnail_59c0d666-8b8b-4f34-881e-7578f851fa7f_annotated_true_size_900_quality_90_ac9b7114_20251018T165016Z.jpg) +*Sagittal T1WI MR shows IVOH secondary to aqueductal stenosis and distal stenosis of cerebral aqueduct . Note the enlarged lateral and 3rd ventricles.* + +![Axial FLAIR MR shows neurosarcoidosis and IVOH secondary to diffuse meningeal disease. Periventricular white matter hyperintensities are also present, as well as choroid involvement .](images/app.statdx.com_image_thumbnail_32fe8687-87ee-4698-81ba-308f84e4409a_annotated_true_size_900_quality_90_6ebf3bdd_20251018T165017Z.jpg) +*Axial FLAIR MR shows neurosarcoidosis and IVOH secondary to diffuse meningeal disease. Periventricular white matter hyperintensities are also present, as well as choroid involvement .* + +![Coronal T1 C+ MR shows neurocysticercosis involvement within the 3rd ventricle and aqueduct , causing IVOH. The lateral ventricles are dilated.](images/app.statdx.com_image_thumbnail_30290860-d589-4878-ab88-f2e5114f4116_annotated_true_size_900_quality_90_d81df3a5_20251018T165017Z.jpg) +*Coronal T1 C+ MR shows neurocysticercosis involvement within the 3rd ventricle and aqueduct , causing IVOH. The lateral ventricles are dilated.* + +![Axial FLAIR MR shows neurocysticercosis resulting in IVOH. Large intraventricular cysts are present in the lateral vents , obstructing the foramina of Monro.](images/app.statdx.com_image_thumbnail_7bbd2516-73b7-45fb-b5ff-9f306d6d3a80_annotated_true_size_900_quality_90_09e03021_20251018T165016Z.jpg) +*Axial FLAIR MR shows neurocysticercosis resulting in IVOH. Large intraventricular cysts are present in the lateral vents , obstructing the foramina of Monro.* + +![Axial T1WI MR shows a well-defined, hyperintense lesion at the foramen of Monro in a patient with headaches, most consistent with a colloid cyst. Note the enlargement of the lateral ventricles due to obstruction at the foramen of Monro.](images/app.statdx.com_image_thumbnail_7ae9f42a-db38-4524-b7c0-79d52b798e21_annotated_true_size_900_quality_90_67db5fbc_20251018T165016Z.jpg) +*Axial T1WI MR shows a well-defined, hyperintense lesion at the foramen of Monro in a patient with headaches, most consistent with a colloid cyst. Note the enlargement of the lateral ventricles due to obstruction at the foramen of Monro.* + +![Sagittal T1WI C+ MR shows a homogeneously enhancing mass in the posterior 3rd ventricle , which causes obstruction and dilatation of the lateral and 3rd ventricles. On pathology, this was an astrocytoma.](images/app.statdx.com_image_thumbnail_a6d2a9a8-b74a-495d-8445-9c79b3030f4d_annotated_true_size_900_quality_90_54985e0c_20251018T165017Z.jpg) +*Sagittal T1WI C+ MR shows a homogeneously enhancing mass in the posterior 3rd ventricle , which causes obstruction and dilatation of the lateral and 3rd ventricles. On pathology, this was an astrocytoma.* + +![Coronal T2WI MR shows a pilocytic astrocytoma centered in the right thalamus causing severe mass effect on the 3rd ventricle and resultant obstructive hydrocephalus .](images/app.statdx.com_image_thumbnail_80232788-db60-4f4f-895b-3180811f62b7_annotated_true_size_900_quality_90_9f55a6f6_20251018T165017Z.jpg) +*Coronal T2WI MR shows a pilocytic astrocytoma centered in the right thalamus causing severe mass effect on the 3rd ventricle and resultant obstructive hydrocephalus .* + +![Axial T2WI MR demonstrates a well-defined CSF intensity cyst with the left temporal horn most consistent with an ependymal cyst . Note the dilated and trapped left temporal horn .](images/app.statdx.com_image_thumbnail_66d7ab24-8ae0-472b-9313-305394ec4c03_annotated_true_size_900_quality_90_29d87168_20251018T165017Z.jpg) +*Axial T2WI MR demonstrates a well-defined CSF intensity cyst with the left temporal horn most consistent with an ependymal cyst . Note the dilated and trapped left temporal horn .* + +![Sagittal T1WI C+ MR shows an enhancing mass in the pineal region causing mass effect on the tectal plate and aqueductal obstruction. Note the extensive leptomeningeal enhancement due to CSF spread of tumor. CSF cytology showed a primitive neuroectodermal tumor.](images/app.statdx.com_image_thumbnail_13465a11-c0bb-4055-8c14-056ea999ea83_annotated_true_size_900_quality_90_d6a81efb_20251018T165017Z.jpg) +*Sagittal T1WI C+ MR shows an enhancing mass in the pineal region causing mass effect on the tectal plate and aqueductal obstruction. Note the extensive leptomeningeal enhancement due to CSF spread of tumor. CSF cytology showed a primitive neuroectodermal tumor.* + +![Axial T2WI MR in a patient with corpus callosum impingement syndrome, after shunting for severe IVOH, shows a shunt tube , bilateral subdural fluid collections, and striated hyperintensity in the corpus callosum with somewhat less striking changes in the periventricular white matter . (Courtesy S. Candy, MD.)](images/app.statdx.com_image_thumbnail_e3c1a91c-c335-42df-baed-81b3ef83e867_annotated_true_size_900_quality_90_90e63be0_20251018T165017Z.jpg) +*Axial T2WI MR in a patient with corpus callosum impingement syndrome, after shunting for severe IVOH, shows a shunt tube , bilateral subdural fluid collections, and striated hyperintensity in the corpus callosum with somewhat less striking changes in the periventricular white matter . (Courtesy S. Candy, MD.)* + +![Axial NECT in a patient with headache demonstrates a classic colloid cyst at the foramen of Monro causing IVOH with dilatation of both lateral ventricles . Note the periventricular hypodensities due to transependymal leakage of CSF.](images/app.statdx.com_image_thumbnail_5272a7c9-5ae5-4498-bff6-256abf29b99a_annotated_true_size_900_quality_90_d97a420a_20251018T165017Z.jpg) +*Axial NECT in a patient with headache demonstrates a classic colloid cyst at the foramen of Monro causing IVOH with dilatation of both lateral ventricles . Note the periventricular hypodensities due to transependymal leakage of CSF.* + +![Axial CECT demonstrates a subacute left posterior inferior cerebellar infarct causing mass effect on the 4th ventricle and resulting in obstructive hydrocephalus .](images/app.statdx.com_image_thumbnail_7b359f58-dd44-4486-b7e0-8d172de6aece_annotated_true_size_900_quality_90_baccaab2_20251018T165017Z.jpg) +*Axial CECT demonstrates a subacute left posterior inferior cerebellar infarct causing mass effect on the 4th ventricle and resulting in obstructive hydrocephalus .* + +![Axial FLAIR MR shows massive enlargement of the 3rd and lateral ventricles by a CSF-like mass within the 3rd ventricle . There is periventricular interstitial edema . At surgery, an ependymal cyst of the 3rd ventricle was found and fenestrated.](images/app.statdx.com_image_thumbnail_5623057c-c423-4d4c-9801-45283be9a3dd_annotated_true_size_900_quality_90_b5086e29_20251018T165017Z.jpg) +*Axial FLAIR MR shows massive enlargement of the 3rd and lateral ventricles by a CSF-like mass within the 3rd ventricle . There is periventricular interstitial edema . At surgery, an ependymal cyst of the 3rd ventricle was found and fenestrated.* + +![Sagittal T1 MR shows a large arachnoid cyst in the superior cerebellar cistern causing severe mass effect on the tectal plate and aqueduct .There is dilatation of the 3rd and lateral ventricles with thinning of the corpus callosum .](images/app.statdx.com_image_thumbnail_4805b47b-9b84-4da7-9efa-e16c8cefdcf8_annotated_true_size_900_quality_90_039b4f28_20251018T165017Z.jpg) +*Sagittal T1 MR shows a large arachnoid cyst in the superior cerebellar cistern causing severe mass effect on the tectal plate and aqueduct .There is dilatation of the 3rd and lateral ventricles with thinning of the corpus callosum .* + +![Sagittal T1WI C+ MR shows a cyst with an enhancing mural nodule of hemangioblastoma in the vermis, causing severe effacement of the 4th ventricle and obstructive hydrocephalus.](images/app.statdx.com_image_thumbnail_519a5b7e-6869-4b93-98f6-a6edefac58a8_annotated_true_size_900_quality_90_d8758d1c_20251018T165017Z.jpg) +*Sagittal T1WI C+ MR shows a cyst with an enhancing mural nodule of hemangioblastoma in the vermis, causing severe effacement of the 4th ventricle and obstructive hydrocephalus.* + +![Sagittal T2 MR demonstrates an enlarged T2 hyperintense tectal plate glioma , which causes obstruction at the aqueduct and dilatation of the lateral and 3rd ventricles .](images/app.statdx.com_image_thumbnail_7ca87971-568b-4324-bd66-b820c656d469_annotated_true_size_900_quality_90_0c6bf4c7_20251018T165017Z.jpg) +*Sagittal T2 MR demonstrates an enlarged T2 hyperintense tectal plate glioma , which causes obstruction at the aqueduct and dilatation of the lateral and 3rd ventricles .* + diff --git a/out/irregular-lateral-ventricles_f42ce651-9877-480b-90d8-665be656b33f.md b/out/irregular-lateral-ventricles_f42ce651-9877-480b-90d8-665be656b33f.md new file mode 100644 index 0000000..efe4a32 --- /dev/null +++ b/out/irregular-lateral-ventricles_f42ce651-9877-480b-90d8-665be656b33f.md @@ -0,0 +1,359 @@ +--- +title: "Irregular Lateral Ventricles" +docid: "f42ce651-9877-480b-90d8-665be656b33f" +authors: + - key: "1fa14dfd-71ea-4960-908e-e720313bc63a" + value: "Santhosh Gaddikeri, MD" + - key: "30ce27b2-237f-4aff-a88f-65ead356335b" + value: "Marinos Kontzialis, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60" + - + name: "Ventricles, Periventricular Regions" + slug: "ventricles-periventricular-regions" + treeNodeId: "353c434a-a6fc-4ef1-8786-d30a1988a4dc" + - + name: "Generic Imaging Patterns" + slug: "generic-imaging-patterns" + treeNodeId: "969c31a2-ef56-4fc3-9125-05857cf9aac3" + - + name: "Irregular Lateral Ventricles" + slug: "irregular-lateral-ventricles" + treeNodeId: null +category: "Brain" +documentVersionId: "2594bf27-49d6-4896-8859-f9eee7b4228c" +imageCount: 42 +lastUpdated: "02/15/23" +pageDescription: "Irregular Lateral Ventricles" +pageKeywords: "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Irregular Lateral Ventricles" +pageTitle: "Irregular Lateral Ventricles | STATdx" +enhancedTitle: "Irregular Lateral Ventricles" +type: "DDX" +references: true +breadcrumbs: + - "Brain" + - "Differential Diagnosis" + - "Ventricles, Periventricular Regions" + - "Generic Imaging Patterns" + - "Irregular Lateral Ventricles" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Irregular ventricles may be result of obstruction, chronic volume loss, &/or congenital deformities + - Obstruction: Mass effect, ballooned-appearing ventricles, and transependymal CSF migration + - Volume loss: Ventricle irregularity with brain parenchymal loss + - Congenital: Look for associated findings (colpocephaly, subependymal nodules) + - Ventricular deformities may become permanent despite relief of obstruction due to parenchymal atrophy or acquired ventricular noncompliance + - Enhancement may help differentiate etiologies +- ## Helpful Clues for Common Diagnoses + + + - **CSF Shunts and Complications** + - Common complications include shunt obstruction/breakage, infection, overdrainage + - Acquired ventricular noncompliance may result in ventricle deformity + - Small, "slit" ventricles → noncompliant ventricle syndrome, chronic overdrainage + - **Surgical Defects** + - Often evident from prior shunt tract or burr hole + - Overlying skull or scalp may show defect + - Deformity is chronic + - **Periventricular Leukomalacia** + - Selective vulnerability of periventricular white matter of preterm neonate + - Injury in late 2nd or early 3rd trimester + - Thinning of posterior body of corpus callosum, enlargement and irregularity of lateral ventricular walls + - Colpocephaly: Atrium/occipital horns dilated + - Related to spastic cerebral palsy + - **Cerebral Infarction, Chronic** + - Vascular territory wedge-shaped area of encephalomalacia + - Results in compensatory or ex vacuo dilation of regional ventricle due to volume loss + - **Multiple Sclerosis** + - Multiple periventricular/perivenular and callososeptal T2 hyperintensities + - Confluent lesions in severe disease lead to atrophy and irregular ventricular margins + - **Porencephalic Cyst** + - Congenital porencephalic cysts result from intrauterine vascular or infectious injury + - Acquired cysts are secondary to injury later in life from trauma, surgery, ischemia, or infection + - CSF intensity cysts with smooth walls and T2-hyperintense surrounding gliotic changes (no gray matter lining walls as in open-lip schizencephaly) + - Usually communicate directly with ventricular system +- ## Helpful Clues for Less Common Diagnoses + + + - **Chiari 2** + - Pointed anterior horns, colpocephaly + - Small, crowded posterior fossa, widening of tentorial incisura, towering cerebellum, tectal beaking, downward herniation of cerebellar vermian tissue through foramen magnum + - Associated with lumbar myelomeningocele + - Hydrocephalus and other midline malformations + - **Heterotopic Gray Matter** + - Subependymal heterotopia: Subependymal nodules follow gray matter signal and protrude into ventricles; no enhancement + - Focal/multifocal asymmetric gray matter indentation of ventricle + - **Tuberous Sclerosis Complex** + - Subependymal nodules lining ventricles characteristic + - Mostly along striothalamic groove + - Calcify with increasing age + - < 1.3 cm + - Cortical and subcortical tubers are usually multifocal ± mild mass effect + - Tubers most easily seen on FLAIR + - Rarely, tubers may calcify or enhance + - White matter radial migration lines + - Cyst-like white matter lesions (cystoid brain degeneration) + - Enhancing mass with rapid growth at foramen of Monro = subependymal giant cell astrocytoma + - **Metastases, Intracranial, Other** + - CSF seeding of primary CNS tumors, lymphoma, or systemic malignancy may cause irregular ventricles + - Intraventricular metastases = 0.9-4.6% of cerebral metastases + - Adults: Renal, colon, lung + - Children: Neuroblastoma, Wilms tumor, retinoblastoma + - May result in ventricular nodules, which can deform ventricles + - Lateral ventricles most common location + - Avid enhancement ± vasogenic edema in adjacent parenchyma + - **Intraventricular Webs or Adhesions** + - May be congenital or acquired (prior hemorrhage, infection, or tumor) + - Contours of ventricles may be rounded or balloon-like due to obstructive symptoms + - Contrast ventriculography or cine CSF can be helpful to assess for evidence of physiological flow obstruction + - Heavily weighted T2 sequences, such as FIESTA/CISS, helpful + - **CMV, Congenital** + - Microcephaly + - Periventricular calcifications 40-70% + - Migrational abnormalities: Lissencephaly, pachygyria, polymicrogyria, schizencephaly + - Delayed myelination, dysmyelination + - Cerebral + cerebellar volume loss + - Periventricular cysts, ventriculomegaly, ventricular adhesions, lenticulostriate vasculopathy + - **Schizencephaly** + - Transmantle cleft lined by gray matter + - Open lip (large defect), closed lip (small defect) + - Dimple in wall of ventricle when defect small/closed + - Up to 50% bilateral + - When bilateral, 60% are open lipped on both sides +- ## Helpful Clues for Rare Diagnoses + + + - **Hemimegalencephaly** + - Hamartomatous overgrowth of part/all of hemisphere + - Lateral ventricle ipsilateral to enlarged hemisphere is usually bizarre-shaped and typically enlarged + - Involved hemisphere may eventually atrophy from chronic seizures + - **Holoprosencephaly** + - Congenital structural forebrain anomalies defined by degree of frontal lobe fusion + - All types have absent septum pellucidum and frontal lobe fusion anomaly + - Alobar + - Single midline forebrain + - Single primitive monoventricle, often incompletely covered posteriorly by brain (dorsal cyst) + - Semilobar + - Frontal lobes > 50% fused + - Thalami and hypothalamus may be fused + - Interhemispheric fissure and falx cerebri may be present posteriorly + - Facial malformations mild or absent + - Lobar: Anterior lateral ventricle may be deficient + - Interhemispheric fissure present along most of midline + - Only most inferior frontal lobes fused + - Thalami almost/completely separated + - Callosal dysgenesis (genu, rostrum) + - **Holoprosencephaly Variants** + - Middle interhemispheric variant of holoprosencephaly + - Lack of separation in posterior frontal and parietal areas + - Dorsal cyst in 40% + - Callosal dysgenesis (body) +- ## Alternative Differential Approaches + + + - Gadolinium studies can differentiate among causes of ependymal nodules + - Nonenhancing subependymal nodules may represent gray matter heterotopia or tuberous sclerosis nodules + - Gray matter heterotopias follow gray matter signal/density + - Tuberous sclerosis nodules follow white matter signal or are calcified + - Enhancing nodules suggest ependymal tumor seeding + +## References + +# Selected References + +1. [Balasubramaniam C: Shunt complications - staying out of trouble. Neurol India. 69(Supplement):S495-501, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=35103008%5Bpmid%5D) +1. [Society for Maternal-Fetal Medicine (SMFM) et al: Holoprosencephaly. Am J Obstet Gynecol. 223(6):B13-6, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=33168217%5Bpmid%5D) +1. [Gotardo JW et al: Impact of peri-intraventricular haemorrhage and periventricular leukomalacia in the neurodevelopment of preterms: a systematic review and meta-analysis. PLoS One. 14(10):e0223427, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31600248%5Bpmid%5D) +1. [Winter TC et al: Holoprosencephaly: a survey of the entity, with embryology and fetal imaging. Radiographics. 35(1):275-90, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25590404%5Bpmid%5D) +1. [Smith AB et al: From the radiologic pathology archives: intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 33(1):21-43, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23322825%5Bpmid%5D) +1. [Osborn AG et al: Intracranial cysts: radiologic-pathologic correlation and imaging approach. Radiology. 239(3):650-64, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16714456%5Bpmid%5D) +1. [Melhem ER et al: Periventricular leukomalacia: relationship between lateral ventricular volume on brain MR images and severity of cognitive and motor impairment. Radiology. 214(1):199-204, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10644124%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T2 MR in a patient with chronic shunting demonstrates slit-like irregular lateral ventricles due to noncompliance from chronic drainage.](images/app.statdx.com_image_thumbnail_d27bb8c7-8ff5-46dc-a0ef-68850de500d4_annotated_true_size_900_quality_90_20b437c9_20251018T165133Z.jpg) +**CSF Shunts and Complications** +*Axial T2 MR in a patient with chronic shunting demonstrates slit-like irregular lateral ventricles due to noncompliance from chronic drainage.* + +![Axial FLAIR MR demonstrates a large surgical defect in the left frontal lobe due to prior tumor resection communicating with the left lateral ventricle , which appears irregular.](images/app.statdx.com_image_thumbnail_8880f9eb-b099-4000-9e1e-48961ce97d43_annotated_true_size_900_quality_90_2ce848f4_20251018T165133Z.jpg) +**Surgical Defects** +*Axial FLAIR MR demonstrates a large surgical defect in the left frontal lobe due to prior tumor resection communicating with the left lateral ventricle , which appears irregular.* + +![Axial T2 MR in a 5-year-old boy with spastic cerebral palsy demonstrates irregular lateral ventricles with paucity of white matter and periventricular hyperintensities , consistent with periventricular leukomalacia.](images/app.statdx.com_image_thumbnail_9c9a7a2f-9e6b-4a73-8333-221f3fe6ed9c_annotated_true_size_900_quality_90_fc040ce9_20251018T165133Z.jpg) +**Periventricular Leukomalacia** +*Axial T2 MR in a 5-year-old boy with spastic cerebral palsy demonstrates irregular lateral ventricles with paucity of white matter and periventricular hyperintensities , consistent with periventricular leukomalacia.* + +![Axial T2 MR demonstrates encephalomalacia in the left occipital lobe with ex vacuo dilation of left occipital horn due to PCA territory chronic infarct.](1e04b9f4-77c2-4080-86ac-47fcf2378c38) +**Cerebral Infarction, Chronic** +*Axial T2 MR demonstrates encephalomalacia in the left occipital lobe with ex vacuo dilation of left occipital horn due to PCA territory chronic infarct.* + +![Axial T2 MR in a patient with primary progressive MS demonstrates extensive white matter hyperintensities with asymmetric parenchymal volume loss and ex vacuo dilation of lateral ventricles .](696938c6-acff-4981-a198-bb3859a4b58d) +**Multiple Sclerosis** +*Axial T2 MR in a patient with primary progressive MS demonstrates extensive white matter hyperintensities with asymmetric parenchymal volume loss and ex vacuo dilation of lateral ventricles .* + +![Axial T2 MR demonstrates a right occipital lobe, smooth-walled, cystic encephalomalacia lined by white matter and communicating with the lateral ventricle, consistent with porencephalic cyst.](2a419e56-b484-4c51-91b1-c0157549c29a) +**Porencephalic Cyst** +*Axial T2 MR demonstrates a right occipital lobe, smooth-walled, cystic encephalomalacia lined by white matter and communicating with the lateral ventricle, consistent with porencephalic cyst.* + +![Axial NECT demonstrates irregular lateral ventricles with a right frontal lobe shunt catheter . Note diffuse calvarial thickening due to chronic shunting. Images of posterior fossa revealed small posterior fossa and other stigmata of Chiari 2 malformation (not shown).](918faead-f7fc-49d0-b0db-ad6d896501a9) +**Chiari 2** +*Axial NECT demonstrates irregular lateral ventricles with a right frontal lobe shunt catheter . Note diffuse calvarial thickening due to chronic shunting. Images of posterior fossa revealed small posterior fossa and other stigmata of Chiari 2 malformation (not shown).* + +![Axial T2 MR demonstrates nodular gray matter heterotopia along the ependymal lining of bilateral occipital horns.](8eb6070b-371d-4980-af73-d3f71628661a) +**Heterotopic Gray Matter** +*Axial T2 MR demonstrates nodular gray matter heterotopia along the ependymal lining of bilateral occipital horns.* + +![Axial 3D T1 MPRAGE in a patient with known tuberous sclerosis demonstrates multiple subependymal nodules . Also note tiny cysts in white matter . Cortical/ subcortical tubers and white matter radial migration lines were seen (not shown).](92d8e135-15c5-4c7f-8210-fdcd830ae6b8) +**Tuberous Sclerosis Complex** +*Axial 3D T1 MPRAGE in a patient with known tuberous sclerosis demonstrates multiple subependymal nodules . Also note tiny cysts in white matter . Cortical/ subcortical tubers and white matter radial migration lines were seen (not shown).* + +![Axial T1 C+ MR in a patient with metastatic lung cancer demonstrates multiple heterogeneously enhancing metastatic lesions in bilateral periventricular regions .](cb26dd77-411b-4e6c-848b-269ad104eac2) +**Metastases, Intracranial, Other** +*Axial T1 C+ MR in a patient with metastatic lung cancer demonstrates multiple heterogeneously enhancing metastatic lesions in bilateral periventricular regions .* + +![Axial 3D T2 HASTE MR in a neonate demonstrates multiple septa/webs in both lateral ventricles . Also note asymmetrically dilated, irregular lateral ventricles . Encephalomalacia in the right parietooccipital region is due to antenatal insult.](4670b1bf-fbaf-4453-9587-496db01a5658) +**Intraventricular Webs or Adhesions** +*Axial 3D T2 HASTE MR in a neonate demonstrates multiple septa/webs in both lateral ventricles . Also note asymmetrically dilated, irregular lateral ventricles . Encephalomalacia in the right parietooccipital region is due to antenatal insult.* + +![Axial NECT in a 2-year-old with a known congenital CMV infection demonstrates moderately dilated irregular lateral ventricles as well as periventricular and deep white matter calcifications . Note lissencephalic gyral pattern .](a2c56b9b-f5ae-439d-a188-f63b50a7c07a) +**CMV, Congenital** +*Axial NECT in a 2-year-old with a known congenital CMV infection demonstrates moderately dilated irregular lateral ventricles as well as periventricular and deep white matter calcifications . Note lissencephalic gyral pattern .* + +![Axial 3D T1 MR demonstrates open-lip schizencephaly with a seam connecting ependymal to pial surface . Note gray matter lining the cystic area , differentiating it from a porencephalic cyst.](2946249f-8a43-47bd-8ae2-c72415856ca0) +**Schizencephaly** +*Axial 3D T1 MR demonstrates open-lip schizencephaly with a seam connecting ependymal to pial surface . Note gray matter lining the cystic area , differentiating it from a porencephalic cyst.* + +![Axial T2 MR demonstrates absent septum with absent posterior body of corpus callosum. Also seen was abnormal bilateral sylvian fissure with ventricle orientation and midline fusion (not shown), consistent with syntelencephaly, a.k.a. middle interhemispheric variant holoprosencephaly.](8bbc4ba5-5a4f-43f5-ba8b-69dd4fa8af36) +**Holoprosencephaly Variants** +*Axial T2 MR demonstrates absent septum with absent posterior body of corpus callosum. Also seen was abnormal bilateral sylvian fissure with ventricle orientation and midline fusion (not shown), consistent with syntelencephaly, a.k.a. middle interhemispheric variant holoprosencephaly.* + + +### Additional Images + +![Axial NECT shows a right frontal ventricular drain that traverses the right ventricle but is not decompressing the left lateral ventricle, which remains irregularly enlarged .](images/app.statdx.com_image_thumbnail_2926513c-8a66-47ff-9091-463f9a854993_annotated_true_size_900_quality_90_63045a79_20251018T165133Z.jpg) +**CSF Shunts and Complications** +*Axial NECT shows a right frontal ventricular drain that traverses the right ventricle but is not decompressing the left lateral ventricle, which remains irregularly enlarged .* + +![Axial T2 MR shows irregular enlargement of the left occipital horn due to left temporal and occipital surgical defect and encephalomalacia from tumor removal in this location.](images/app.statdx.com_image_thumbnail_81d90381-93ef-4fd9-804f-98f39faf22d8_annotated_true_size_900_quality_90_3f67ffb9_20251018T165133Z.jpg) +**Surgical Defects** +*Axial T2 MR shows irregular enlargement of the left occipital horn due to left temporal and occipital surgical defect and encephalomalacia from tumor removal in this location.* + +![Axial T2 MR shows classic "wavy" or undulating contours of the lateral ventricles in addition to colpocephaly (enlargement of the posterior portions of lateral ventricles). Colpocephaly reflects the predominantly posterior volume loss.](images/app.statdx.com_image_thumbnail_c7d04410-a691-42e3-9428-d55060a26d68_annotated_true_size_900_quality_90_5996778f_20251018T165133Z.jpg) +**Periventricular Leukomalacia** +*Axial T2 MR shows classic "wavy" or undulating contours of the lateral ventricles in addition to colpocephaly (enlargement of the posterior portions of lateral ventricles). Colpocephaly reflects the predominantly posterior volume loss.* + +![Axial NECT shows irregular enlargement of the left frontal horn due to focal regional parenchymal volume loss in this patient with remote MCA infarct.](2f456604-123a-498e-a877-660381509108) +**Cerebral Infarction, Chronic** +*Axial NECT shows irregular enlargement of the left frontal horn due to focal regional parenchymal volume loss in this patient with remote MCA infarct.* + +![Axial T1 FS MR shows multifocal nodularity along ependymal margins of both lateral ventricles . These nodules follow gray matter signal on all sequences and do not enhance or change over time.](f1496ad0-85d2-45aa-a05b-4f49fd882c3b) +**Heterotopic Gray Matter** +*Axial T1 FS MR shows multifocal nodularity along ependymal margins of both lateral ventricles . These nodules follow gray matter signal on all sequences and do not enhance or change over time.* + +![Axial NECT shows irregularly dilated occipital horns with interdigitation of parietal and occipital parenchyma across midline due to a falx deficiency.](5a2506e9-70d3-46f0-a725-26e1b13fa20f) +**Chiari 2** +*Axial NECT shows irregularly dilated occipital horns with interdigitation of parietal and occipital parenchyma across midline due to a falx deficiency.* + +![Coronal T2 MR shows dysgenetic corpus callosum, small posterior fossa, and interdigitation of gyri from deficient falx, best seen post shunting. Cerebellum "towers" through the tentorial notch.](d3885609-d163-4bb5-8179-ba8901e78c3a) +**Chiari 2** +*Coronal T2 MR shows dysgenetic corpus callosum, small posterior fossa, and interdigitation of gyri from deficient falx, best seen post shunting. Cerebellum "towers" through the tentorial notch.* + +![Axial T2 MR shows multiple calcified subependymal nodules (SEN) lining ventricles. Note also subcortical tubers . SEN calcify much more commonly than cortical/subcortical tubers. ~ 50% of SEN are calcified by 10 years.](cd3b3a2b-1a19-4a9b-a8db-db235f939c8c) +**Tuberous Sclerosis Complex** +*Axial T2 MR shows multiple calcified subependymal nodules (SEN) lining ventricles. Note also subcortical tubers . SEN calcify much more commonly than cortical/subcortical tubers. ~ 50% of SEN are calcified by 10 years.* + +![Axial T2 MR shows small, subependymal nodules , which indent lateral ventricle margins. Unlike gray matter heterotopia, these follow WM signal or are calcified.](009fab60-a9d4-4283-aba1-b86b3717cb0a) +**Tuberous Sclerosis Complex** +*Axial T2 MR shows small, subependymal nodules , which indent lateral ventricle margins. Unlike gray matter heterotopia, these follow WM signal or are calcified.* + +![Axial T1 MR shows nodular ependymal thickening with an enhancing rind of tissue along the entire ventricular ependyma . While infection & primary malignant brain neoplasms such as GBM, germinoma, and lymphoma commonly spread along ventricular ependyma, this is a recognized but uncommon site for tumor deposits from extracranial primary tumors (melanoma in this case).](3602f90f-46e4-4ebe-b9a2-8f72def57b1f) +**Metastases, Intracranial, Other** +*Axial T1 MR shows nodular ependymal thickening with an enhancing rind of tissue along the entire ventricular ependyma . While infection & primary malignant brain neoplasms such as GBM, germinoma, and lymphoma commonly spread along ventricular ependyma, this is a recognized but uncommon site for tumor deposits from extracranial primary tumors (melanoma in this case).* + +![Axial T2 MR shows a small dimple on the lateral ventricular wall, which "points" to the site of a fused pial-ependymal seam . The aperture of the cleft is lined by gray matter in this closed-lip schizencephaly.](05bee719-bf55-496d-8865-8bae802bbf01) +**Schizencephaly** +*Axial T2 MR shows a small dimple on the lateral ventricular wall, which "points" to the site of a fused pial-ependymal seam . The aperture of the cleft is lined by gray matter in this closed-lip schizencephaly.* + +![Axial T2 MR shows cortical dysplasia and open-lip schizencephaly . Schizencephaly is closed-lip with a fused, gray matter-lined pial-ependymal seam or open-lip with large, gray matter-lined and fluid-filled CSF clefts.](544584c2-74ba-4f36-8701-7b6f8d26db3c) +**Schizencephaly** +*Axial T2 MR shows cortical dysplasia and open-lip schizencephaly . Schizencephaly is closed-lip with a fused, gray matter-lined pial-ependymal seam or open-lip with large, gray matter-lined and fluid-filled CSF clefts.* + +![Axial NECT shows focal outpouchings of CSF from both lateral ventricles with a CSF cleft extending from lateral ventricles to the subpial surface. The pial-ependymal seam is lined by gray matter.](2bc44bf5-bc94-467d-84f9-8de71b93d08b) +**Schizencephaly** +*Axial NECT shows focal outpouchings of CSF from both lateral ventricles with a CSF cleft extending from lateral ventricles to the subpial surface. The pial-ependymal seam is lined by gray matter.* + +![Axial NECT shows septum pellucidum and anterior falx absence. Frontal horns are hypoplastic. A band of parenchyma crosses midline . Mild frontal lobe fusion anomalies, as seen here, are typical of lobar holoprosencephaly.](fd55a6d3-31b8-4659-8163-ae8f4b2a3fae) +**Holoprosencephaly** +*Axial NECT shows septum pellucidum and anterior falx absence. Frontal horns are hypoplastic. A band of parenchyma crosses midline . Mild frontal lobe fusion anomalies, as seen here, are typical of lobar holoprosencephaly.* + +![Axial T1 MR shows open-lip schizencephaly with large, gray matter-lined and a fluid-filled CSF cleft. In addition, there is ventricular wall irregularity due to subependymal gray matter heterotopia bilaterally .](b7d119f1-d67e-42cd-bbfa-21c225e8cb7d) +**Schizencephaly** +*Axial T1 MR shows open-lip schizencephaly with large, gray matter-lined and a fluid-filled CSF cleft. In addition, there is ventricular wall irregularity due to subependymal gray matter heterotopia bilaterally .* + +![Coronal T2 MR demonstrates closed-lip schizencephaly. Abnormal, thick gray matter lines the cleft extending to a dimple in the wall of the right lateral ventricle .](55272009-ca22-4b9f-9162-af69d8a49014) +**Schizencephaly** +*Coronal T2 MR demonstrates closed-lip schizencephaly. Abnormal, thick gray matter lines the cleft extending to a dimple in the wall of the right lateral ventricle .* + +![Axial CT shows multiple calcified subependymal nodules lining the ventricles in a patient with tuberous sclerosis. The nodules calcify much more commonly than cortical/subcortical tubers. Note traumatic subarachnoid hemorrhage in the left Sylvian fissure.](1253b6d1-a526-4e7f-8b3e-2c2744b25924) +**Tuberous Sclerosis Complex** +*Axial CT shows multiple calcified subependymal nodules lining the ventricles in a patient with tuberous sclerosis. The nodules calcify much more commonly than cortical/subcortical tubers. Note traumatic subarachnoid hemorrhage in the left Sylvian fissure.* + +![Axial T2 MR shows multiple bilateral subependymal nodules of heterotopic gray matter along the lateral ventricular margins. These nodules follow gray matter signal on all sequences.](8a3bcced-e3f1-4a17-8cc5-b9d76bb8e2d7) +**Heterotopic Gray Matter** +*Axial T2 MR shows multiple bilateral subependymal nodules of heterotopic gray matter along the lateral ventricular margins. These nodules follow gray matter signal on all sequences.* + +![Axial CT in a Chiari 2 patient shows typical irregular appearance of the ventricles. Note the left posterior shunt catheter .](fefed345-c959-4896-b9b2-5ad4f10bae9a) +**Chiari 2** +*Axial CT in a Chiari 2 patient shows typical irregular appearance of the ventricles. Note the left posterior shunt catheter .* + +![Axial FLAIR MR demonstrates left posterior middle cerebral artery encephalomalacia with mild ex vacuo dilatation of the left occipital horn and atrium .](9f0d6726-27e8-4c3e-92a9-9aa52067d6cb) +**Cerebral Infarction, Chronic** +*Axial FLAIR MR demonstrates left posterior middle cerebral artery encephalomalacia with mild ex vacuo dilatation of the left occipital horn and atrium .* + +![Axial T2 MR in periventricular leukomalacia shows asymmetric, posterior, periventricular white matter (WM) volume loss with irregular ventricular margins . Periventricular leukomalacia, a.k.a. WM injury of prematurity, is a result of brain injury occurring before 33 weeks gestation and resulting in loss of periventricular WM.](c36e4c1d-b34f-41e9-a31c-aab2cb0df8bf) +**Periventricular Leukomalacia** +*Axial T2 MR in periventricular leukomalacia shows asymmetric, posterior, periventricular white matter (WM) volume loss with irregular ventricular margins . Periventricular leukomalacia, a.k.a. WM injury of prematurity, is a result of brain injury occurring before 33 weeks gestation and resulting in loss of periventricular WM.* + +![Axial FIESTA MR in a patient following left temporal bone surgery shows skull defect , underlying encephalomalacia , and ex vacuo dilatation of the left lateral ventricle .](images/app.statdx.com_image_thumbnail_c51882c7-eec5-40d6-9cfa-982849a82c22_annotated_true_size_900_quality_90_c7bab1c9_20251018T165133Z.jpg) +**Surgical Defects** +*Axial FIESTA MR in a patient following left temporal bone surgery shows skull defect , underlying encephalomalacia , and ex vacuo dilatation of the left lateral ventricle .* + +![Axial T1 MR demonstrates a right parietal shunt catheter with its tip in the right frontal horn in a patient with congenital aqueductal stenosis. The right lateral ventricle is collapsed, while the 3rd and left lateral ventricles are moderately dilated.](images/app.statdx.com_image_thumbnail_b0b4ac10-a5f5-45c0-8e06-16b37ce8e9e0_annotated_true_size_900_quality_90_abb03b0b_20251018T165133Z.jpg) +**CSF Shunts and Complications** +*Axial T1 MR demonstrates a right parietal shunt catheter with its tip in the right frontal horn in a patient with congenital aqueductal stenosis. The right lateral ventricle is collapsed, while the 3rd and left lateral ventricles are moderately dilated.* + +![Axial CECT shows a low-density outpouching from the right lateral ventricle . While a thin rim of cortex seems intact, the cyst nearly reaches brain surface and can be considered a porencephalic dilation or porencephalic lateral ventricle cyst.](90e2763f-d574-42bb-b173-33d8362e631b) +**Porencephalic Cyst** +*Axial CECT shows a low-density outpouching from the right lateral ventricle . While a thin rim of cortex seems intact, the cyst nearly reaches brain surface and can be considered a porencephalic dilation or porencephalic lateral ventricle cyst.* + +![Axial T2 MR shows near-complete coating of the ependymal lining of both lateral ventricles with tumor nodules due to metastatic seeding of an anaplastic oligodendroglioma.](66f6ca25-e3b3-437c-8531-4faa19f2e26c) +**Metastases, Intracranial, Other** +*Axial T2 MR shows near-complete coating of the ependymal lining of both lateral ventricles with tumor nodules due to metastatic seeding of an anaplastic oligodendroglioma.* + +![Axial NECT shows periventricular calcification , particularly along the caudostriatal groove, in the context of microcephaly and developmental delay. This strongly suggests congenital CMV infection. Note smooth ventricular margins, unlike calcified nodules in tuberous sclerosis complex.](27976562-e322-411d-b649-4edfd868c0da) +**CMV, Congenital** +*Axial NECT shows periventricular calcification , particularly along the caudostriatal groove, in the context of microcephaly and developmental delay. This strongly suggests congenital CMV infection. Note smooth ventricular margins, unlike calcified nodules in tuberous sclerosis complex.* + +![Axial T2 MR shows enlargement of left cerebral hemisphere accompanied by an irregular ipsilateral ventricle . The body of the left hemispheric WM is bulky. Note left fornix overgrowth.](6cf5c025-6a72-4fc7-8343-15d1240eec7b) +**Hemimegalencephaly** +*Axial T2 MR shows enlargement of left cerebral hemisphere accompanied by an irregular ipsilateral ventricle . The body of the left hemispheric WM is bulky. Note left fornix overgrowth.* + +![Axial T1 MR shows a large, horseshoe-shaped monoventricle with fused basal ganglia . There is no interhemispheric fissure and no identifiable lobulation or formation of ventricular horns in this alobar holoprosencephaly.](88c3bc23-d2eb-443c-832b-37829eed0963) +**Holoprosencephaly** +*Axial T1 MR shows a large, horseshoe-shaped monoventricle with fused basal ganglia . There is no interhemispheric fissure and no identifiable lobulation or formation of ventricular horns in this alobar holoprosencephaly.* + diff --git a/out/multiple-sclerosis_7892b2a2-f52a-4d7f-9858-a326f2b7ab04.md b/out/multiple-sclerosis_7892b2a2-f52a-4d7f-9858-a326f2b7ab04.md deleted file mode 100644 index 3dc80b9..0000000 --- a/out/multiple-sclerosis_7892b2a2-f52a-4d7f-9858-a326f2b7ab04.md +++ /dev/null @@ -1,497 +0,0 @@ ---- -title: "Multiple Sclerosis" -docid: "7892b2a2-f52a-4d7f-9858-a326f2b7ab04" -authors: - - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" - value: "Miral D. Jhaveri, MD, MBA" -breadcrumbs: - - - name: "Brain" - slug: "brain" - treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" - - - name: "Diagnosis" - slug: "diagnosis" - treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" - - - name: "Pathology-Based Diagnoses" - slug: "pathology-based-diagnoses" - treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" - - - name: "Infectious, Inflammatory, and Demyelinating Disease" - slug: "infectious-inflammatory-and-demyel-" - treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607" - - - name: "Inflammatory and Demyelinating Disease" - slug: "inflammatory-and-demyelinating-dis-" - treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc" - - - name: "Multiple Sclerosis" - slug: "multiple-sclerosis" - treeNodeId: null -category: "Brain" -documentVersionId: "7ced4781-f9a4-4a48-a5ed-954fa6ea87cf" -imageCount: 27 -lastUpdated: "10/08/20" -pageDescription: "Multiple Sclerosis" -pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Multiple Sclerosis" -pageTitle: "Multiple Sclerosis | STATdx" -enhancedTitle: "Multiple Sclerosis" -type: "DX" -references: true -breadcrumbs: - - "Brain" - - "Diagnosis" - - "Pathology-Based Diagnoses" - - "Infectious, Inflammatory, and Demyelinating Disease" - - "Inflammatory and Demyelinating Disease" - - "Multiple Sclerosis" ---- -# KEY FACTS - -- ## Terminology - - - - Idiopathic chronic inflammatory demyelinating disease of CNS -- ## Imaging - - - - Multiple perpendicular callososeptal T2 hyperintensities characteristic of multiple sclerosis (MS) - - Perivenular extension: "Dawson fingers" - - Bilateral, asymmetric linear/ovoid FLAIR hyperintensities - - Periventricular/perivenular, callososeptal interface - - May also commonly involve brachium pontis, brainstem, spinal cord, cortex/juxtacortical - - SWI central vein sign - - Transient enhancement during active demyelination - - Rare: Large tumefactive enhancing rings - - Advanced imaging techniques show disease in normal-appearing white matter -- ## Top Differential Diagnoses - - - - **Multifocal T2/FLAIR hyperintensities** - - Arteriolosclerosis, ADEM, NMOSD, vasculitis, Susac syndrome, Lyme disease - - **Mass-like ("tumefactive") lesion(s)** - - Neoplasms, PML/PML-IRIS -- ## Pathology - - - - Major clinical subtypes from least to most severe - - Radiologically isolated syndrome, clinically isolated syndrome, relapsing-remitting, relapsing-progressive, a.k.a. secondary progressive & primary progressive MS -- ## Clinical Issues - - - - ~ 2.5 million people in world have MS -- ## Diagnostic Checklist - - - - Requires dissemination in time & space in CNS for diagnosis - - McDonald criteria: Consensus statement for diagnostic criteria, last revised in 2017 - -# TERMINOLOGY - -- ## Abbreviations - - - - Multiple sclerosis (MS) -- ## Definitions - - - - Idiopathic chronic inflammatory demyelinating disease of CNS - -# IMAGING - -- ## General Features - - - - ### Best diagnostic clue - - - - Multiple perpendicular callososeptal T2 hyperintensities - - ### Location - - - - > 85% periventricular/perivenular, callososeptal interface - - Brachium pontis, brainstem, spinal cord - - Infratentorial (< 10% in adults, more common in children) - - Gray matter [cortex & basal ganglia (BG) ~ 10%] - - Juxtacortical: Lesion in cerebral white matter (WM) abutting cortex - - Cortical: Lesion within cerebral cortex - - Leukocortical (inner aspect ± involvement of juxtacortical WM) - - Intracortical (purely within cortex) - - Subpial (at its outer aspect) - - ### Size - - - - Small (5-10 mm); tumefactive lesions several cm - - ### Morphology - - - - Linear, round, or ovoid; beveled, target -- ## CT Findings - - - - NECT often normal early in disease course - - Solitary/multiple ill-defined WM hypodensities - - Mild/moderate punctate, patchy, ring enhancement in acute/subacute lesions -- ## MR Findings - - - - ### T1WI - - - - Typically hypo- or isointense - - Hypointensity correlates with axonal destruction ("black holes"), faint mildly hyperintense rim - - Chronic/severe: Volume loss, thin corpus callosum - - ### T2WI - - - - Hyperintense, linear foci radiating from ventricles - - ### FLAIR - - - - Early: Alternating linear hyperintensity along ependyma on sagittal FLAIR: Ependymal dot-dash sign - - Bilateral, asymmetric, linear/ovoid hyperintensities - - Perivenular extension; "Dawson fingers" - - Hyperintensities become confluent with severity - - Cortical lesions may precede classic WM lesions - - ### T2* GRE - - - - SWI central vein sign (CVS) = vein located centrally within MS lesion - - Magnetic resonance imaging in multiple sclerosis (MAGNIMS) study - - CVS sensitivity of 68.1% & specificity of 82.9% in distinguishing MS from radiologic mimics using 35% CVS proportion threshold - - Chronic lesions: Paramagnetic rims at lesion edge - - Iron accumulation in deep gray matter structures - - SWI C+ to better detect BBB dysfunction in MS plaques - - Natalizumab-associated progressive multifocal leukoencephalopathy (PML): Juxtacortical low signal - - ### DWI - - - - Majority of acute plaques: Normal or ↑ diffusivity - - May show restricted diffusion at plaque margins - - Subacute/chronic plaques show ↑ diffusivity - - DTI: Reduced longitudinal diffusivity due to axonal injury - - ### T1WI C+ - - - - Transient enhancement during active demyelination - - Punctate, nodular, linear, incomplete ring - - Semilunar, "open" nonenhancing segment facing cortex - - ± leptomeningeal enhancement on delayed FLAIR MR, marker for cortical demyelination - - ### MRS - - - - ↓ NAA , ↑ choline (Cho/Cr), ↑ myoinositol - - Abnormalities found in normal-appearing WM (NAWM) - - Secondary progressive MS may show ↓ NAA in normal-appearing gray matter (NAGM) - - May allow early distinction between relapsing-remitting & secondary progressive - - 3D double inversion recovery (DIR) sequence - - ↑ intracortical lesion detection - - Perfusion MR: Low rCBV, can help differentiate tumefactive MS from neoplasm - - Magnetization transfer (MT) - - ↓ MT ratio (MTR) in lesions/NAWM - - Functional connectivity MR (fcMR) - - ↓ functional connectivity between right/left hemisphere primary visual & motor cortices - - 3T vs. 1.5T: ↑ number of contrast-enhancing lesions, enhancing lesion volume, total lesion volume - - 7T: Improved detection of cortical lesions -- ## PET With Translocator Protein Radioligands - - - - In vivo quantification of microglial activation - - Presence of widespread inflammation in NAWM -- ## Imaging Recommendations - - - - ### Best imaging tool - - - - MR - - ### Protocol advice - - - - Contrast-enhanced MR with sagittal FLAIR - - Fat saturation to assess for optic neuritis - - **Consortium of Multiple Sclerosis Centers (CMSC) 2018 guidelines** - - Brain MR C+ recommended for diagnosis of MS - - Spinal cord MR if brain MR nondiagnostic or presenting symptoms referable to cord - - Follow-up brain MR recommended - - Demonstrate dissemination in time - - Detect clinically silent disease activity on Rx - - **PML**surveillance while on Rx - - Evaluate unexpected clinical worsening - - New baseline MR before starting/modifying Rx - - Every 6 months to 2 years for relapsing MS - - MR brain core sequences - - 2D/3D sagittal & axial FLAIR, 2D/3D axial T2, 2D axial DWI, 3D IR-prep GE T1 ± 2D/3D axial T1 C+ - -# DIFFERENTIAL DIAGNOSIS - -- ## Multifocal T2/FLAIR Hyperintensities - - - - [Arter io lo sc lerosis](/document/arteriolosclerosis/ce5a75ed-3a88-42a4-a0e8-4f339375c062) - - Patchy confluent & focal lesions; subcortical/deep WM & BG involved; ± cortical infarcts, vascular risk factors - - [Acute disseminated encephalomyelitis](/document/adem/a3fafeb7-5861-4364-beb8-c0e30220564e) - - Viral prodrome, monophasic, more common in children - - Can mimic MS; gray matter often involved - - Lesions tend to be larger & often symmetric - - [Neuromyelitis optica spectrum disorders](/document/neuromyelitis-optica-spectrum-diso-/54d4a8bc-9267-4df6-98c1-f22aae051d01) - - Optic neuritis & spinal cord lesions - - Tend to border midline CSF spaces - - [Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0) - - Enhancing lesions spare callososeptal interface - - Beaded angiogram appearance - - [Susac syndrome](/document/susac-syndrome/af240d31-5918-4981-971f-c6780f7b405f) - - Classic triad: Encephalopathy, branch retinal artery occlusions, hearing loss - - [Lyme Disease](/document/lyme-disease/ca3da7a8-0f21-4e06-9961-a43e6af1b0cc) - - Can be identical to MS (skin rash common) -- ## Mass-Like ("Tumefactive") Lesion(s) - - - - **Neoplasms**:****Glioblastoma, metastasis - - [PML/PML-IRIS : HIV/AIDS, Natalizumab-treated MS](/document/progressive-multifocal-leukoenceph-/fc94bcd6-17a1-4966-8e27-51f5c7df6537) - -# PATHOLOGY - -- ## General Features - - - - ### Etiology - - - - Multifactorial disease, precise pathogenesis unknown - - Autoimmune, environmental, & genetic factors - - ### Genetics - - - - Multifactorial; ↑ incidence in 1st-order relatives -- ## Staging, Grading, & Classification - - - - **Major clinical subtypes from least to most severe** - - **Radiologically isolated syndrome****(RIS)** - - Asymptomatic with routine MR findings highly suggestive of MS - - **Clinically isolated syndrome (CIS)** - - Single episode of neurologic symptoms suggestive of MS, chance of developing MS → CIS(+) MR(-) (20%) & if CIS(+) MR(+) (60-80%) - - **Relapsing-remitting** **(RR-MS)**85% initial presentation - - Relapses alternating with remission phases - - **Relapsing-progressive** **(RP-MS)** - - Secondary progressive MS, progressive worsening of neurologic function over time - - **Primary progressive (PP-MS)** - - 5-10% of patients progressive from start - - **MS variants/subtypes** - - **Malignant/Marburg disease**: Younger patients, febrile prodrome, clinically fulminant, death in months - - **Schilder** type ("diffuse sclerosis"): Extensive, confluent, asymmetric demyelination in bilateral supra-/infratentorial parenchyma - - **Baló**type ("concentric sclerosis"): Large lesions with alternating zones of demyelinated/myelinated WM -- ## Gross Pathologic & Surgical Features - - - - Acute: Poorly delineated, yellowish-white, periventricular plaques - - Chronic: Gray, granular, well-demarcated plaques ± generalized volume loss -- ## Microscopic Features - - - - Relatively sharp borders, macrophage infiltration (interstitial & perivascular), perivascular inflammation - - Acute: Foamy macrophages, perivascular T-cell lymphocytic cuffing, microglial activation - - Chronic: Marked loss of myelin, oligodendrocytes; dense astrogliosis; minimal/no perivascular inflammation - - CSF positive for oligoclonal bands - -# CLINICAL ISSUES - -- ## Presentation - - - - ### Most common signs/symptoms - - - - Weakness, numbness, tingling, gait disturbances, impaired vision, diplopia - - ↓ sphincter control, paralysis, dementia - - Cranial nerve palsy; usually multiple - - Spinal cord symptoms -- ## Demographics - - - - ### Age - - - - 20-40 years; median: 30 years - - ### Sex - - - - Adults: M:F = 1:1.77; adolescents: M:F = 1:3-5 - - ### Ethnicity - - - - All groups but most common in white patients - - ### Epidemiology - - - - ~ 2.5 million people worldwide - - Most often occurs in temperate zones -- ## Natural History & Prognosis - - - - 1/3 have initial episode followed by near-normal function - - Majority: Protracted course with progression of deficits - - Late: Severe disability, cognitive impairment -- ## Treatment - - - - Immunomodulators &/or immunosuppressants - -# DIAGNOSTIC CHECKLIST - -- ## Image Interpretation Pearls - - - - 95% with clinically definite MS have positive MR - - b58b4061-1b92-4b32-abf6-1debb49d180c - -## References - -# Selected References - -1. [Gaitán MI et al: SWAN-Venule: an optimized MRI technique to detect the central vein sign in MS plaques. AJNR Am J Neuroradiol. 41(3):456-60, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32054616%5Bpmid%5D) -1. [Sastre-Garriga J et al: MAGNIMS consensus recommendations on the use of brain and spinal cord atrophy measures in clinical practice. Nat Rev Neurol. 16(3):171-82, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32094485%5Bpmid%5D) -1. [do Amaral LLF et al: Gadolinium-enhanced susceptibility-weighted imaging in multiple sclerosis: optimizing the recognition of active plaques for different mr imaging sequences. AJNR Am J Neuroradiol. 40(4):614-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30846435%5Bpmid%5D) -1. [Filippi M et al: Association between pathological and MRI findings in multiple sclerosis. Lancet Neurol. 18(2):198-210, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30663609%5Bpmid%5D) -1. [Hartung HP et al: Diagnosis of multiple sclerosis: revisions of the McDonald criteria 2017 - continuity and change. Curr Opin Neurol. 32(3):327-37, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30985371%5Bpmid%5D) -1. [Maranzano J et al: Comparison of multiple sclerosis cortical lesion types detected by multicontrast 3T and 7T MRI. AJNR Am J Neuroradiol. 40(7):1162-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31221631%5Bpmid%5D) -1. [Suthiphosuwan S et al: The central vein sign in radiologically isolated syndrome. AJNR Am J Neuroradiol. 40(5):776-83, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31000526%5Bpmid%5D) -1. [Zipp F et al: Implementing the 2017 McDonald criteria for the diagnosis of multiple sclerosis. Nat Rev Neurol. 15(8):441-5, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31086264%5Bpmid%5D) -1. [Filippi M et al: MRI in multiple sclerosis: what is changing? Curr Opin Neurol. 31(4):386-95, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29952834%5Bpmid%5D) -1. [Inglese M et al: MRI in multiple sclerosis: clinical and research update. Curr Opin Neurol. 31(3):249-55, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29561520%5Bpmid%5D) -1. [Thompson AJ et al: Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol. 17(2):162-73, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29275977%5Bpmid%5D) -1. [Castellaro M et al: Heterogeneity of cortical lesion susceptibility mapping in multiple sclerosis. AJNR Am J Neuroradiol. 38(6):1087-95, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28408633%5Bpmid%5D) -1. [Filippi M et al: MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines. Lancet Neurol. 15(3):292-303, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26822746%5Bpmid%5D) -1. [Roosendaal SD et al: Imaging phenotypes in multiple sclerosis. Neuroimaging Clin N Am. 25(1):83-96, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25476514%5Bpmid%5D) -1. [Aliaga ES et al: MRI mimics of multiple sclerosis. Handb Clin Neurol. 122:291-316, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24507523%5Bpmid%5D) -1. [Ciccarelli O et al: Pathogenesis of multiple sclerosis: insights from molecular and metabolic imaging. Lancet Neurol. 13(8):807-22, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25008549%5Bpmid%5D) -1. [Fernandez O et al: Biomarkers in multiple sclerosis: an update for 2014. Rev Neurol. 58(12):553-70, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24915032%5Bpmid%5D) -1. [Hardy TA et al: Baló's concentric sclerosis. Lancet Neurol. 13(7):740-6, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24943346%5Bpmid%5D) -1. [Miller TR et al: Advances in multiple sclerosis and its variants: conventional and newer imaging techniques. Radiol Clin North Am. 52(2):321-36, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24582342%5Bpmid%5D) -1. [Steenwijk MD et al: What explains gray matter atrophy in long-standing multiple sclerosis? Radiology. 272(3):832-42, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24761837%5Bpmid%5D) -1. [Klawiter EC: Current and new directions in MRI in multiple sclerosis. Continuum (Minneap Minn). 19(4 Multiple Sclerosis):1058-73, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23917101%5Bpmid%5D) -1. [Polman CH et al: Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol. 69(2):292-302, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21387374%5Bpmid%5D) - - -## Images - - -### Selected Images - -![Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.](images/app.statdx.com_image_thumbnail_298cc9db-f7e6-4904-a92c-b4014d263b26_annotated_true_size_900_quality_90_3f552f53_20251018T122505Z.jpg) -*Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.* - -![Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.](images/app.statdx.com_image_thumbnail_298cc9db-f7e6-4904-a92c-b4014d263b26_size_168_quality_85_89ae47ce_20251018T095337Z.jpg) -*Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.* - -![Sagittal FLAIR demonstrates numerous well-defined and ill-defined callososeptal hyperintensities radiating from the lateral ventricular margin with a typical perpendicular orientation, characteristic of MS.](images/app.statdx.com_image_thumbnail_acb3f1b0-b500-47a5-9ed5-72dee0dd74dc_annotated_true_size_900_quality_90_53cf0096_20251018T122505Z.jpg) -*Sagittal FLAIR demonstrates numerous well-defined and ill-defined callososeptal hyperintensities radiating from the lateral ventricular margin with a typical perpendicular orientation, characteristic of MS.* - -![Sagittal FLAIR demonstrates numerous well-defined and ill-defined callososeptal hyperintensities radiating from the lateral ventricular margin with a typical perpendicular orientation, characteristic of MS.](images/app.statdx.com_image_thumbnail_acb3f1b0-b500-47a5-9ed5-72dee0dd74dc_size_168_quality_85_59b609a2_20251018T095337Z.jpg) -*Sagittal FLAIR demonstrates numerous well-defined and ill-defined callososeptal hyperintensities radiating from the lateral ventricular margin with a typical perpendicular orientation, characteristic of MS.* - -![Axial FLAIR MR shows subcortical and cortical demyelinating MS plaques. Cortical lesions are better seen at higher field strength MR and are classified as leukocortical [inner aspect of cortex ± involvement of juxtacortical white matter (WM)], intracortical (purely within cortex), and subpial (involving outer aspect of cortex).](images/app.statdx.com_image_thumbnail_8e1b38d3-c2c0-4128-80a1-f2e3640c3b91_annotated_true_size_900_quality_90_d73c35bf_20251018T122505Z.jpg) -*Axial FLAIR MR shows subcortical and cortical demyelinating MS plaques. Cortical lesions are better seen at higher field strength MR and are classified as leukocortical [inner aspect of cortex ± involvement of juxtacortical white matter (WM)], intracortical (purely within cortex), and subpial (involving outer aspect of cortex).* - -![Axial FLAIR MR shows subcortical and cortical demyelinating MS plaques. Cortical lesions are better seen at higher field strength MR and are classified as leukocortical [inner aspect of cortex ± involvement of juxtacortical white matter (WM)], intracortical (purely within cortex), and subpial (involving outer aspect of cortex).](images/app.statdx.com_image_thumbnail_8e1b38d3-c2c0-4128-80a1-f2e3640c3b91_size_168_quality_85_9844f252_20251018T095337Z.jpg) -*Axial FLAIR MR shows subcortical and cortical demyelinating MS plaques. Cortical lesions are better seen at higher field strength MR and are classified as leukocortical [inner aspect of cortex ± involvement of juxtacortical white matter (WM)], intracortical (purely within cortex), and subpial (involving outer aspect of cortex).* - -![Axial T1 C+ MR in a patient with MS demonstrate multiple enhancing plaques due to active demyelination. Punctate , nodular , and rim patterns are seen.](images/app.statdx.com_image_thumbnail_b8524003-2e1d-4d59-94d3-bf5d7634b01d_annotated_true_size_900_quality_90_08f44164_20251018T122505Z.jpg) -*Axial T1 C+ MR in a patient with MS demonstrate multiple enhancing plaques due to active demyelination. Punctate , nodular , and rim patterns are seen.* - -![Axial T1 C+ MR in a patient with MS demonstrate multiple enhancing plaques due to active demyelination. Punctate , nodular , and rim patterns are seen.](images/app.statdx.com_image_thumbnail_b8524003-2e1d-4d59-94d3-bf5d7634b01d_size_168_quality_85_160c28d1_20251018T095337Z.jpg) -*Axial T1 C+ MR in a patient with MS demonstrate multiple enhancing plaques due to active demyelination. Punctate , nodular , and rim patterns are seen.* - -![Sagittal T1 MR in a patient with longstanding MS shows ovoid lesions in the periventricular WM with ill-defined hyperintense rims surrounding the plaques, giving the distinct lesion within a lesion appearance.](images/app.statdx.com_image_thumbnail_5ab2519c-5653-43fe-b237-732e2fbc8b12_annotated_true_size_900_quality_90_d1b908ef_20251018T122505Z.jpg) -*Sagittal T1 MR in a patient with longstanding MS shows ovoid lesions in the periventricular WM with ill-defined hyperintense rims surrounding the plaques, giving the distinct lesion within a lesion appearance.* - -![Sagittal T1 MR in a patient with longstanding MS shows ovoid lesions in the periventricular WM with ill-defined hyperintense rims surrounding the plaques, giving the distinct lesion within a lesion appearance.](images/app.statdx.com_image_thumbnail_5ab2519c-5653-43fe-b237-732e2fbc8b12_size_168_quality_85_c124921d_20251018T095337Z.jpg) -*Sagittal T1 MR in a patient with longstanding MS shows ovoid lesions in the periventricular WM with ill-defined hyperintense rims surrounding the plaques, giving the distinct lesion within a lesion appearance.* - -![Axial SWI (R) demonstrates characteristic perivenular location of a demyelinating plaque with the medullary vein coursing through it. Axial SWI (L) in the same patient shows findings related to Natalizumab-associated PML with juxtacortical hypointense rim .](images/app.statdx.com_image_thumbnail_75e21646-c880-469e-850b-2caa2329b59b_annotated_true_size_900_quality_90_fa12f16f_20251018T122505Z.jpg) -*Axial SWI (R) demonstrates characteristic perivenular location of a demyelinating plaque with the medullary vein coursing through it. Axial SWI (L) in the same patient shows findings related to Natalizumab-associated PML with juxtacortical hypointense rim .* - -![Axial SWI (R) demonstrates characteristic perivenular location of a demyelinating plaque with the medullary vein coursing through it. Axial SWI (L) in the same patient shows findings related to Natalizumab-associated PML with juxtacortical hypointense rim .](images/app.statdx.com_image_thumbnail_75e21646-c880-469e-850b-2caa2329b59b_size_168_quality_85_97d150ff_20251018T095337Z.jpg) -*Axial SWI (R) demonstrates characteristic perivenular location of a demyelinating plaque with the medullary vein coursing through it. Axial SWI (L) in the same patient shows findings related to Natalizumab-associated PML with juxtacortical hypointense rim .* - -![Sagittal T1WI C+ MR shows a large hypointense mass with a peripheral crescent of incomplete or "open ring" enhancement . This enhancement pattern is classic for a tumefactive demyelinating disease, most commonly MS.](images/app.statdx.com_image_thumbnail_f8983790-81fa-4667-ada8-b38b6cd1f153_annotated_true_size_900_quality_90_db952b97_20251018T122505Z.jpg) -*Sagittal T1WI C+ MR shows a large hypointense mass with a peripheral crescent of incomplete or "open ring" enhancement . This enhancement pattern is classic for a tumefactive demyelinating disease, most commonly MS.* - -![Sagittal T1WI C+ MR shows a large hypointense mass with a peripheral crescent of incomplete or "open ring" enhancement . This enhancement pattern is classic for a tumefactive demyelinating disease, most commonly MS.](images/app.statdx.com_image_thumbnail_f8983790-81fa-4667-ada8-b38b6cd1f153_size_168_quality_85_c4b7db24_20251018T095337Z.jpg) -*Sagittal T1WI C+ MR shows a large hypointense mass with a peripheral crescent of incomplete or "open ring" enhancement . This enhancement pattern is classic for a tumefactive demyelinating disease, most commonly MS.* - -![MRS at 144 TE in the same patient demonstrates a large choline peak with ↓ in NAA . MRS in a tumefactive demyelinating lesion is not specific and can mimic a tumor profile. MR DSC perfusion (insert) shows marked ↓ rCBV , which goes more in favor of a demyelinating lesion.](images/app.statdx.com_image_thumbnail_52cb7d6b-f66d-4aa3-8c70-3058352b5bab_annotated_true_size_900_quality_90_64f6a076_20251018T122505Z.jpg) -*MRS at 144 TE in the same patient demonstrates a large choline peak with ↓ in NAA . MRS in a tumefactive demyelinating lesion is not specific and can mimic a tumor profile. MR DSC perfusion (insert) shows marked ↓ rCBV , which goes more in favor of a demyelinating lesion.* - -![MRS at 144 TE in the same patient demonstrates a large choline peak with ↓ in NAA . MRS in a tumefactive demyelinating lesion is not specific and can mimic a tumor profile. MR DSC perfusion (insert) shows marked ↓ rCBV , which goes more in favor of a demyelinating lesion.](images/app.statdx.com_image_thumbnail_52cb7d6b-f66d-4aa3-8c70-3058352b5bab_size_168_quality_85_5e322455_20251018T095337Z.jpg) -*MRS at 144 TE in the same patient demonstrates a large choline peak with ↓ in NAA . MRS in a tumefactive demyelinating lesion is not specific and can mimic a tumor profile. MR DSC perfusion (insert) shows marked ↓ rCBV , which goes more in favor of a demyelinating lesion.* - -![Axial T1 C+ MR demonstrates concentric laminated "onion bulb" enhancement , characteristic of acute Baló concentric sclerosis. Baló concentric sclerosis is a rare aggressive MS variant characterized by acute onset and rapid deterioration.](images/app.statdx.com_image_thumbnail_c73d1451-8702-40c9-a7d6-52f7ced3fb44_annotated_true_size_900_quality_90_f4006eb4_20251018T122505Z.jpg) -*Axial T1 C+ MR demonstrates concentric laminated "onion bulb" enhancement , characteristic of acute Baló concentric sclerosis. Baló concentric sclerosis is a rare aggressive MS variant characterized by acute onset and rapid deterioration.* - -![Axial T1 C+ MR demonstrates concentric laminated "onion bulb" enhancement , characteristic of acute Baló concentric sclerosis. Baló concentric sclerosis is a rare aggressive MS variant characterized by acute onset and rapid deterioration.](images/app.statdx.com_image_thumbnail_c73d1451-8702-40c9-a7d6-52f7ced3fb44_size_168_quality_85_70f447f9_20251018T095337Z.jpg) -*Axial T1 C+ MR demonstrates concentric laminated "onion bulb" enhancement , characteristic of acute Baló concentric sclerosis. Baló concentric sclerosis is a rare aggressive MS variant characterized by acute onset and rapid deterioration.* - -![Axial T1 C+ MR in a young male with rapid onset of visual disturbance demonstrates large enhancing demyelinating lesions in the deep and periventricular WM. Marburg disease is an acute fulminant MS variant.](images/app.statdx.com_image_thumbnail_0cb35959-c58d-42c3-89d6-4e1e83002315_annotated_true_size_900_quality_90_9538bc08_20251018T095333Z.jpg) -*Axial T1 C+ MR in a young male with rapid onset of visual disturbance demonstrates large enhancing demyelinating lesions in the deep and periventricular WM. Marburg disease is an acute fulminant MS variant.* - -![Axial T1 C+ MR in a young male with rapid onset of visual disturbance demonstrates large enhancing demyelinating lesions in the deep and periventricular WM. Marburg disease is an acute fulminant MS variant.](images/app.statdx.com_image_thumbnail_0cb35959-c58d-42c3-89d6-4e1e83002315_size_168_quality_85_9b6eff9b_20251018T095337Z.jpg) -*Axial T1 C+ MR in a young male with rapid onset of visual disturbance demonstrates large enhancing demyelinating lesions in the deep and periventricular WM. Marburg disease is an acute fulminant MS variant.* - - -### Additional Images - -![Sagittal FLAIR MR shows MS plaques with typical perpendicular orientation at the callososeptal interface along penetrating venules ("Dawson fingers"), as well as involving subcortical WM.](images/app.statdx.com_image_thumbnail_4429c9c8-59de-4763-965e-b51fdf048a3c_size_168_quality_85_1a582782_20251018T095337Z.jpg) -*Sagittal FLAIR MR shows MS plaques with typical perpendicular orientation at the callososeptal interface along penetrating venules ("Dawson fingers"), as well as involving subcortical WM.* - -![Sagittal FLAIR MR shows MS plaques with hyperintense rim and central hypointensity (latter also hypointense on T1WI; not shown). Note the characteristic posterior fossa lesion .](images/app.statdx.com_image_thumbnail_0da4da94-8e63-4d2c-931d-a09f0438166e_size_168_quality_85_13a1fd06_20251018T095337Z.jpg) -*Sagittal FLAIR MR shows MS plaques with hyperintense rim and central hypointensity (latter also hypointense on T1WI; not shown). Note the characteristic posterior fossa lesion .* - -![Axial T1WI C+ MR demonstrates nodular, enhancing MS plaques. Note the common periventricular location with perpendicular orientation, as well as the involvement of subcortical WM.](images/app.statdx.com_image_thumbnail_145b9fbf-434b-4db6-8c34-240875821d49_size_168_quality_85_59e955bb_20251018T095337Z.jpg) -*Axial T1WI C+ MR demonstrates nodular, enhancing MS plaques. Note the common periventricular location with perpendicular orientation, as well as the involvement of subcortical WM.* - -![Axial T2WI MR demonstrates very hypointense bilateral basal ganglia, atrophy (evidenced by ventricular prominence), and confluent periventricular/subcortical hyperintense plaques in this patient with advanced MS.](images/app.statdx.com_image_thumbnail_26c1f577-7d97-43b9-812e-4f4db88d8fce_size_168_quality_85_18fb923b_20251018T095337Z.jpg) -*Axial T2WI MR demonstrates very hypointense bilateral basal ganglia, atrophy (evidenced by ventricular prominence), and confluent periventricular/subcortical hyperintense plaques in this patient with advanced MS.* - -![Axial T1WI C+ MR shows irregular, thick, partial ring enhancement around a mass-like lesion in a patient not previously diagnosed with MS. This was biopsy-proven tumefactive MS. (Courtesy M. Mirfakharee, MD.)](images/app.statdx.com_image_thumbnail_0471add5-e1df-4d93-b336-ccbd0de9aec7_size_168_quality_85_6dfd081e_20251018T095337Z.jpg) -*Axial T1WI C+ MR shows irregular, thick, partial ring enhancement around a mass-like lesion in a patient not previously diagnosed with MS. This was biopsy-proven tumefactive MS. (Courtesy M. Mirfakharee, MD.)* - -![Sagittal FLAIR shows callososeptal hyperintensities radiating from the lateral ventricles with a typical perpendicular orientation, characteristic of MS.](images/app.statdx.com_image_thumbnail_d94ee5e0-f32e-4285-8795-d88b40cdd80a_size_168_quality_85_d2c1a91e_20251018T095337Z.jpg) -*Sagittal FLAIR shows callososeptal hyperintensities radiating from the lateral ventricles with a typical perpendicular orientation, characteristic of MS.* - -![Axial FLAIR MR 3T shows multiple nonenhancing, periventricular, hyperintense MS lesions oriented perpendicular to the callosomarginal interface. These lesions are perivenular, along the path of the deep medullary veins, and represent "Dawson fingers." Confluent lesions are also seen along the right periventricular margin.](images/app.statdx.com_image_thumbnail_12fc34cf-5a21-42fd-9489-1ad3b6572f03_size_168_quality_85_52dc3401_20251018T095337Z.jpg) -*Axial FLAIR MR 3T shows multiple nonenhancing, periventricular, hyperintense MS lesions oriented perpendicular to the callosomarginal interface. These lesions are perivenular, along the path of the deep medullary veins, and represent "Dawson fingers." Confluent lesions are also seen along the right periventricular margin.* - -![Axial FLAIR MR shows confluent periventricular WM hyperintensity typical of advanced, longstanding MS with loss of discrete, linear, periventricular lesions.](images/app.statdx.com_image_thumbnail_654a4004-229b-416c-a753-42d402b6b3ab_size_168_quality_85_cdd40c1f_20251018T095337Z.jpg) -*Axial FLAIR MR shows confluent periventricular WM hyperintensity typical of advanced, longstanding MS with loss of discrete, linear, periventricular lesions.* - -![Sagittal T1WI MR shows multiple hypointense lesions ("black holes") in the deep WM related to axonal destruction. Note the associated moderate ventricular and sulcal enlargement.](images/app.statdx.com_image_thumbnail_04cbe9a3-e39e-4fd3-865b-3f0d54163edf_size_168_quality_85_8932cd14_20251018T095337Z.jpg) -*Sagittal T1WI MR shows multiple hypointense lesions ("black holes") in the deep WM related to axonal destruction. Note the associated moderate ventricular and sulcal enlargement.* - -![Coronal T1WI C+ MR shows a hypointense mass in the left posterior frontal region with a peripheral crescent of incomplete or "horseshoe" enhancement . This enhancement pattern is classic for tumefactive demyelinating disease, most commonly MS.](images/app.statdx.com_image_thumbnail_03b47bd7-3474-492a-bff6-01abe9eb5c8a_size_168_quality_85_8d6e2a10_20251018T095337Z.jpg) -*Coronal T1WI C+ MR shows a hypointense mass in the left posterior frontal region with a peripheral crescent of incomplete or "horseshoe" enhancement . This enhancement pattern is classic for tumefactive demyelinating disease, most commonly MS.* - -![Axial T1WI C+ FS shows bright enhancement of the optic nerves , similar to the extraocular muscles, in a patient with MS and acute bilateral optic neuritis.](images/app.statdx.com_image_thumbnail_35a96664-28f8-4752-9dbb-8d5a8fb99276_size_168_quality_85_dc4e58c2_20251018T095337Z.jpg) -*Axial T1WI C+ FS shows bright enhancement of the optic nerves , similar to the extraocular muscles, in a patient with MS and acute bilateral optic neuritis.* - -![Axial FLAIR MR shows numerous peripheral WM and cortical lesions that exhibited robust contrast enhancement in an 18-year-old woman with malignant (Marburg) MS. The patient presented with a 2-week history of behavioral changes and leg pain and died 3 weeks after presentation. Autopsy showed typical demyelinating pathology.](images/app.statdx.com_image_thumbnail_c14ee6bd-8197-4368-81a7-8a12bdadf049_size_168_quality_85_8275dd1f_20251018T095337Z.jpg) -*Axial FLAIR MR shows numerous peripheral WM and cortical lesions that exhibited robust contrast enhancement in an 18-year-old woman with malignant (Marburg) MS. The patient presented with a 2-week history of behavioral changes and leg pain and died 3 weeks after presentation. Autopsy showed typical demyelinating pathology.* - -![Axial T1WI C+ MR shows numerous enhancing MS plaques that were present throughout the infratentorial and supratentorial brain. Lesions may show homogeneous enhancement but may also exhibit ring or an incomplete ring pattern of enhancement.](images/app.statdx.com_image_thumbnail_9197159e-317a-41bb-9ee4-ed7ba31454fc_size_168_quality_85_cf86065a_20251018T095337Z.jpg) -*Axial T1WI C+ MR shows numerous enhancing MS plaques that were present throughout the infratentorial and supratentorial brain. Lesions may show homogeneous enhancement but may also exhibit ring or an incomplete ring pattern of enhancement.* - -![Sagittal FLAIR shows callososeptal hyperintensities radiating from the lateral ventricles with a typical perpendicular orientation, characteristic of MS.](images/app.statdx.com_image_thumbnail_ea96d27e-42c1-429b-98a3-024bea525827_size_168_quality_85_08aa276d_20251018T095337Z.jpg) -*Sagittal FLAIR shows callososeptal hyperintensities radiating from the lateral ventricles with a typical perpendicular orientation, characteristic of MS.* - -![Axial FLAIR in a 35-year-old woman with MS shows extensive confluent periventricular hyperintense lesions , typical of advanced, longstanding MS with loss of discrete, linear, periventricular lesions. Note prominence of the ventricles and cortical sulci due to diffuse atrophy.](images/app.statdx.com_image_thumbnail_6a954445-52d5-4b02-9846-8e8f7a1d2d52_size_168_quality_85_3d20ae56_20251018T095337Z.jpg) -*Axial FLAIR in a 35-year-old woman with MS shows extensive confluent periventricular hyperintense lesions , typical of advanced, longstanding MS with loss of discrete, linear, periventricular lesions. Note prominence of the ventricles and cortical sulci due to diffuse atrophy.* - -![Sagittal T1WI MR shows multiple hypointense lesions ("black holes") in the periventricular WM related to axonal destruction. Note the associated moderate ventricular and sulcal enlargement. T1 "back holes" are correlated with greater tissue damage and ↑ axonal destruction on histopathology.](images/app.statdx.com_image_thumbnail_4158830a-642a-434a-9dd1-61c9321b67ee_size_168_quality_85_372f6946_20251018T095337Z.jpg) -*Sagittal T1WI MR shows multiple hypointense lesions ("black holes") in the periventricular WM related to axonal destruction. Note the associated moderate ventricular and sulcal enlargement. T1 "back holes" are correlated with greater tissue damage and ↑ axonal destruction on histopathology.* - -![Axial SWI demonstrates characteristic perivenular location of a demyelinating plaque with the medullary vein coursing through it.](images/app.statdx.com_image_thumbnail_408e2816-65c7-496c-b6d5-05a215d808ae_size_168_quality_85_de50003d_20251018T095337Z.jpg) -*Axial SWI demonstrates characteristic perivenular location of a demyelinating plaque with the medullary vein coursing through it.* - diff --git a/out/neuromyelitis-optica-spectrum-disorders_54d4a8bc-9267-4df6-98c1-f22aae051d01.md b/out/neuromyelitis-optica-spectrum-disorders_54d4a8bc-9267-4df6-98c1-f22aae051d01.md deleted file mode 100644 index 91d3123..0000000 --- a/out/neuromyelitis-optica-spectrum-disorders_54d4a8bc-9267-4df6-98c1-f22aae051d01.md +++ /dev/null @@ -1,445 +0,0 @@ ---- -title: "Neuromyelitis Optica Spectrum Disorders" -docid: "54d4a8bc-9267-4df6-98c1-f22aae051d01" -authors: - - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" - value: "Miral D. Jhaveri, MD, MBA" -breadcrumbs: - - - name: "Brain" - slug: "brain" - treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" - - - name: "Diagnosis" - slug: "diagnosis" - treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" - - - name: "Pathology-Based Diagnoses" - slug: "pathology-based-diagnoses" - treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" - - - name: "Infectious, Inflammatory, and Demyelinating Disease" - slug: "infectious-inflammatory-and-demyel-" - treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607" - - - name: "Inflammatory and Demyelinating Disease" - slug: "inflammatory-and-demyelinating-dis-" - treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc" - - - name: "Neuromyelitis Optica Spectrum Disorders" - slug: "neuromyelitis-optica-spectrum-diso-" - treeNodeId: null -category: "Brain" -documentVersionId: "e640534a-1c8b-4d12-970e-c0994fbfc239" -imageCount: 22 -lastUpdated: "07/28/20" -pageDescription: "Neuromyelitis Optica Spectrum Disorders" -pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Neuromyelitis Optica Spectrum Disorders" -pageTitle: "Neuromyelitis Optica Spectrum Disorders | STATdx" -enhancedTitle: "Neuromyelitis Optica Spectrum Disorders" -type: "DX" -references: true -breadcrumbs: - - "Brain" - - "Diagnosis" - - "Pathology-Based Diagnoses" - - "Infectious, Inflammatory, and Demyelinating Disease" - - "Inflammatory and Demyelinating Disease" - - "Neuromyelitis Optica Spectrum Disorders" ---- -# KEY FACTS - -- ## Terminology - - - - Neuromyelitis optica spectrum disorders (NMOSDs) - - MOG antibody-associated disease (MOG-AAD) - - Autoimmune inflammatory demyelinating disease of CNS with preferential involvement of optic nerves and spinal cord, AQP4 antibody (+/-) -- ## Imaging - - - - Hyperintense, mildly enlarged optic nerves + enhancement - - **AQP4 antibody (+)**:****More posterior involvement, including chiasm - - **MOG-AAD**:****More anterior optic nerve involvement - - Expansile, hyperintense cord signal, ≥ 3 segments - - Brain lesions in NMOSD are distinct from multiple sclerosis (MS) - - Acute cord lesions show patchy enhancement - - Patchy enhancement with blurred margins (cloud-like enhancement) in cerebral lesions - - "Pencil-thin" ependymal enhancement -- ## Top Differential Diagnoses - - - - MS, acute disseminated encephalomyelitis (ADEM) - - Optic neuritis, transverse myelitis - - Spinal cord neoplasm, neurosarcoid -- ## Pathology - - - - NMO antibody: IgG autoantibody that binds to AQP4 - - **AQP4-IgG** is 90% specific and 70-75% sensitive for NMOSD - - **AQP4-IgG (-) NMOSD** less common (10-25%) - - Minority of AQP4-IgG (-) patients with phenotype of NMOSD are MOG-IgG (+) - - 2015 International Panel for NMO Diagnosis (IPND) developed revised diagnostic criteria for NMOSD stratified by serologic testing (NMOSD with or without AQP4-IgG) -- ## Clinical Issues - - - - Impaired vision, myelopathy, brainstem syndrome - - Relapsing course in 85-90% of patients - - Acute exacerbation: Steroids, plasmapheresis - -# TERMINOLOGY - -- ## Abbreviations - - - - Neuromyelitis optica spectrum disorders (NMOSDs) - - Aquaporin-4 (AQP4) - - Myelin oligodendrocyte glycoprotein (MOG) - - MOG antibody-associated disease (MOG-AAD) -- ## Synonyms - - - - Devic syndrome -- ## Definitions - - - - Autoimmune inflammatory demyelinating disease of CNS with preferential involvement of optic nerves and spinal cord, AQP4 antibody (+/-) - -# IMAGING - -- ## General Features - - - - ### Best diagnostic clue - - - - Optic neuritis and myelitis - - Spectrum of lesions atypical for multiple sclerosis (MS) - - ### Location - - - - **AQP4 antibody (+)** - - Spinal cord: More common cervicomedullary junction - - Optic nerves: More posterior involvement of optic nerve, including chiasm, bilateral disease - - Brain lesions: Deep white matter, periependymal regions, corpus callosum, corticospinal tracts, brainstem, and cerebellum - - **MOG-AAD** - - Spinal cord: More likely thoracolumbar and conus - - Optic nerves: Long, bilateral, anterior optic nerve - - Brain lesions: Basal ganglia, thalamus, infratentorial (brainstem, cerebellar peduncles, around 4th ventricle) - - ### Size - - - - Spinal lesions typically expansile, multisegment - - Brain lesions punctate or small (< 3 mm) to large, confluent in cerebral white matter -- ## MR Findings - - - - ### T2WI - - - - Hyperintense, mildly enlarged optic nerves - - **AQP4 antibody (+)**:****More posterior involvement, including chiasm - - **MOG-AAD**: More anterior optic nerve involvement - - Expansile, hyperintense cord signal, ≥ 3 segments → longitudinally extensive transverse myelitis (LETM) - - May have heterogeneous areas of brighter T2 signal - - Central/central and peripheral, > 50% cord area - - ### STIR - - - - Hyperintense optic nerve - - LETM: Central gray matter/central and peripheral, > 50% cord area - - ### FLAIR - - - - Brain lesions are quite characteristic and distinct from lesions in MS - - Hyperintense brain lesions in AQP4 antibody (+) NMOSD correspond to sites of high AQP4 expression - - Adjacent to ventricular system at any level - - **Periependymal surrounding 3rd ventricle and cerebral aqueduct** - - Thalamus, hypothalamus, ventral midbrain - - **Dorsal brainstem adjacent to 4th ventricle** - - Including area postrema and nucleus tracts solitarius - - **Periventricular surrounding lateral ventricles** - - Follow ependymal lining in disseminated pattern - - Often edematous and heterogeneous, creating marbled pattern unlike MS - - Complete thickness of splenium in unique arch bridge pattern - - Chronic lesions reduce in size and intensity; may even disappear - - Cystic changes and atrophy of corpus callosum - - **Lesions involving corticospinal tracts** - - Unilateral or bilateral, posterior limb internal capsule, cerebral peduncle - - Contiguous, often longitudinally extensive - - **Extensive hemispheric lesions** - - Tumefactive (> 3 cm), long spindle-like, or radial - - Occasionally mimicking posterior reversible encephalopathy syndrome (PRES) - - **Nonspecific lesions: Not unique, but most common** - - Nonspecific punctate or small (< 3 mm) dots or patches of hyperintensities in subcortical or deep white matter - - **MOG-AAD** lesions tend to involve deep gray matter and infratentorial parenchyma - - ### DWI - - - - ↑ diffusivity, ↓ fractional anisotropy than MS patients or controls - - Patient with NMOSD and normal brain MR - - DTI shows ↑ mean diffusivity in corticospinal tract and optic radiation, but not in cingulum or corpus callosum - - ### T1WI C+ - - - - Long-segment optic nerve enhancement unilateral or bilateral, often extending posterior to optic chiasm - - Acute lesions in cord show patchy enhancement - - Most brain lesions do not show enhancement - - Patchy enhancement with blurred margins (cloud-like enhancement) in cerebral lesions - - Relatively specific for NMOSD - - "Pencil-thin" ependymal enhancement - - Rarely, well-marginated nodular enhancement, leptomeningeal enhancement - - ### MRS - - - - In NMOSD, metabolites are normal in normal-appearing white matter (NAWM) and normal-appearing gray matter (NAGM) compared to patients with MS - - In MS, NAA ↓ and choline ↑ in NAWM -- ## Imaging Recommendations - - - - ### Best imaging tool - - - - Contrast-enhanced MR of brain, spine, and orbits - - ### Protocol advice - - - - Orbits: Axial and coronal STIR/T2 FS and T1 C+ FS - - Spine: Sagittal STIR/T2, T1WI C+ - - Brain: Axial and sagittal FLAIR, T1WI C+ - -# DIFFERENTIAL DIAGNOSIS - -- [Multiple Sclerosis](/document/multiple-sclerosis/7892b2a2-f52a-4d7f-9858-a326f2b7ab04) - - Radially oriented, perivenular lesions (Dawson fingers) - - Optic neuritis, typically unilateral, shorter segment - - Myelitis, usually smaller longitudinal extension, peripheral -- [Acute Disseminated Encephalomyelitis](/document/adem/a3fafeb7-5861-4364-beb8-c0e30220564e) - - Bilateral, asymmetric involvement of supratentorial and infratentorial, white matter and gray matter lesions - - Common in children and young adults - - LETM similar to NMOSD -- [Optic Neuritis](/document/optic-neuritis/ac9c8fc9-33cd-4716-a509-2542ec5579ca) - - 1 component of NMOSD - - Consider NMOSD in case of 1st optic neuritis, but otherwise normal brain MR -- [Transverse Myelitis](/document/idiopathic-acute-transverse-myelit-/6e774628-d7ad-48f1-9847-e09a4fad168b) - - 1 component of NMOSD - - Idiopathic inflammatory or postinfectious myelitis - - Differentiation from other immune-mediated myelitis like Sjögren disease or SLE difficult -- [Spinal Cord Neoplasm](/document/spinal-cord-astrocytoma/43d5efa2-7a6d-4972-bfc6-c300cc31f9af) - - Astrocytoma or ependymoma can show similar multisegment cord T2 hyperintensity and enhancement -- [Neurosarcoid](/document/neurosarcoid/fef69139-0019-4be3-9bdc-e26bc3644251) - - Parenchymal granulomas near hypothalamus, visual pathway - - Optic nerve involvement bilateral, extensive, resemble NMOSD - - Nodular leptomeningeal, dural, and cranial nerve enhancement - - Systemic manifestations, (+) chest x-ray - -# PATHOLOGY - -- ## General Features - - - - NMO antibody: IgG autoantibody that binds to AQP4 - - AQP4 channel is most abundant water channel in CNS - - Located in foot processes of astrocytes - - Highly expressed in optic nerve, spinal cord, periventricular areas, hypothalamus, subpial regions, brainstem, area postrema - - **AQP4-IgG** can be identified in other autoimmune disorders, myasthenia gravis, SLE, Sjögren syndrome, RA - - Rare clinical coexistence of anti-*n*-methyl-*d*-aspartate (NMDA) receptor encephalitis and NMOSD - - **AQP4-IgG** is 90% specific and 70-75% sensitive for NMOSD - - **AQP4-IgG (-) NMOSD** less common (10-25%) - - Minority of AQP4-IgG (-) patients with phenotype of NMOSD are MOG-IgG (+) - - MOG antibody targets oligodendrocytes - - MOG-IgG also found in association with other demyelinating disorders, such as MS, acute disseminated encephalomyelitis (ADEM), optic neuritis, LETM - - Controversial if MOG-AAD distinct entity or part of NMOSD -- ## Microscopic Features - - - - Selective AQP4 immunoreactivity loss, vasculocentric complement, immunoglobulin deposition characteristic - -# CLINICAL ISSUES - -- ## Presentation - - - - ### Most common signs/symptoms - - - - Optic neuritis: Impaired or double vision - - Myelitis: Myelopathy - - Brainstem symptoms: Nystagmus, dysarthria, dysphagia, ataxia, ophthalmoplegia - - Area postrema syndrome: Hiccups, nausea, vomiting - - Symptomatic narcolepsy or acute diencephalic syndrome - - ### Clinical profile - - - - CSF: Pleocytosis, ↑ protein, (-) oligoclonal bands -- ## Demographics - - - - 1st symptoms commonly between 32-45 years of age - - 15-20% of patients over age 60 at onset - - NMOSD worldwide disease - - AQP4(+) NMOSD, F:M ratio is 8-9:1 - - MOG-AAD usually younger age at onset, F:M ratio is ~ 1:1 -- ## Natural History & Prognosis - - - - Relapsing course in 85-90% of patients - - Worse prognosis with more severe disability than MS - - Anti-AQP4 antibody (+): High risk of further relapses - - MOG-AAD: More likely to be monophasic, fewer relapses -- ## Treatment - - - - Acute exacerbation: Steroids - - Plasma exchange in relapses not responding to steroids - - Prevention of relapses: Immunosuppression, eculizumab - - Symptom management and rehabilitation - -# DIAGNOSTIC CHECKLIST - -- ## Consider - - - - NMOSD in patient with optic neuritis and myelitis - - Characteristic brain lesions involving periependymal regions, corpus callosum, corticospinal tracts, brainstem - - 9e0e24a9-6707-4e55-9895-fe13a49f7c8e - -## References - -# Selected References - -1. [Rosenthal JF et al: CNS inflammatory demyelinating disorders: MS, NMOSD and MOG antibody associated disease. J Investig Med. 68(2):321-30, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31582425%5Bpmid%5D) -1. [Salama S et al: MRI differences between MOG antibody disease and AQP4 NMOSD. Mult Scler. 1352458519893093, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31937191%5Bpmid%5D) -1. [Fujihara K: Neuromyelitis optica spectrum disorders: still evolving and broadening. Curr Opin Neurol. 32(3):385-94, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30893099%5Bpmid%5D) -1. [Huda S et al: Neuromyelitis optica spectrum disorders. Clin Med (Lond). 19(2):169-76, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30872305%5Bpmid%5D) -1. [Wynford-Thomas R et al: Neurological update: MOG antibody disease. J Neurol. 266(5):1280-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30569382%5Bpmid%5D) -1. [Dutra BG et al: Neuromyelitis optica spectrum disorders: spectrum of MR imaging findings and their differential diagnosis. Radiographics. 38(1):169-93, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29320331%5Bpmid%5D) -1. [Narayan R et al: MOG antibody disease: a review of MOG antibody seropositive neuromyelitis optica spectrum disorder. Mult Scler Relat Disord. 25:66-72, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30048919%5Bpmid%5D) -1. [Kim HJ et al: MRI characteristics of neuromyelitis optica spectrum disorder: an international update. Neurology. 84(11):1165-73, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25695963%5Bpmid%5D) -1. [Wingerchuk DM et al: International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 85(2):177-89, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26092914%5Bpmid%5D) -1. [Barnett Y et al: Conventional and advanced imaging in neuromyelitis optica. AJNR Am J Neuroradiol. 35(8):1458-66, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=23764723%5Bpmid%5D) -1. [Kawachi I et al: [Characteristic features of radiological findings in multiple sclerosis and neuromyelitis optica.] Nihon Rinsho. 72(11):1976-82, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25518380%5Bpmid%5D) -1. [Uzawa A et al: Neuromyelitis optica: concept, immunology and treatment. J Clin Neurosci. 21(1):12-21, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=23916471%5Bpmid%5D) -1. [Jacob A et al: Current concept of neuromyelitis optica (NMO) and NMO spectrum disorders. J Neurol Neurosurg Psychiatry. 84(8):922-30, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23142960%5Bpmid%5D) -1. [Sato DK et al: Clinical spectrum and treatment of neuromyelitis optica spectrum disorders: evolution and current status. Brain Pathol. 23(6):647-60, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24118482%5Bpmid%5D) -1. [Kim W et al: Brain abnormalities in neuromyelitis optica spectrum disorder. Mult Scler Int. 2012:735486, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23259063%5Bpmid%5D) -1. [Lana-Peixoto MA et al: The expanded spectrum of neuromyelitis optica: evidences for a new definition. Arq Neuropsiquiatr. 70(10):807-13, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23060108%5Bpmid%5D) -1. [Benavente E et al: Neuromyelitis optica-AQP4: an update. Curr Rheumatol Rep. 13(6):496-505, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21922173%5Bpmid%5D) -1. [Cree B: Neuromyelitis optica: diagnosis, pathogenesis, and treatment. Curr Neurol Neurosci Rep. 8(5):427-33, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18713580%5Bpmid%5D) -1. [Jacob A et al: Treatment of neuromyelitis optica with rituximab: retrospective analysis of 25 patients. Arch Neurol. 65(11):1443-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18779415%5Bpmid%5D) -1. [Li Y et al: Brain magnetic resonance imaging abnormalities in neuromyelitis optica. Acta Neurol Scand. 118(4):218-25, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18384459%5Bpmid%5D) -1. [Jarius S et al: NMO-IgG in the diagnosis of neuromyelitis optica. Neurology. 68(13):1076-7, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17287449%5Bpmid%5D) -1. [Matiello M et al: Neuromyelitis optica. Curr Opin Neurol. 20(3):255-60, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17495617%5Bpmid%5D) -1. [Benedetti B et al: Grading cervical cord damage in neuromyelitis optica and MS by diffusion tensor MRI. Neurology. 67(1):161-3, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16832101%5Bpmid%5D) -1. [Pittock SJ et al: Brain abnormalities in neuromyelitis optica. Arch Neurol. 63(3):390-6, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16533966%5Bpmid%5D) -1. [Wingerchuk DM et al: Revised diagnostic criteria for neuromyelitis optica. Neurology. 66(10):1485-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16717206%5Bpmid%5D) - - -## Images - - -### Selected Images - -![Axial T1 C+ MR in a 22-year-old woman with right vision loss demonstrates swelling with enhancement of the intraorbital segment of the right optic nerve.](images/app.statdx.com_image_thumbnail_e8ba18d2-7677-4bf1-8a89-ea3f7610a1dd_annotated_true_size_900_quality_90_503cf50a_20251018T122510Z.jpg) -*Axial T1 C+ MR in a 22-year-old woman with right vision loss demonstrates swelling with enhancement of the intraorbital segment of the right optic nerve.* - -![Axial T1 C+ MR in a 22-year-old woman with right vision loss demonstrates swelling with enhancement of the intraorbital segment of the right optic nerve.](images/app.statdx.com_image_thumbnail_e8ba18d2-7677-4bf1-8a89-ea3f7610a1dd_size_168_quality_85_e6e9aa1a_20251018T095348Z.jpg) -*Axial T1 C+ MR in a 22-year-old woman with right vision loss demonstrates swelling with enhancement of the intraorbital segment of the right optic nerve.* - -![Axial T1 C+ MR in a 22-year-old woman with right vision loss demonstrates swelling with enhancement of the intraorbital segment of the right optic nerve.](images/app.statdx.com_image_thumbnail_e8ba18d2-7677-4bf1-8a89-ea3f7610a1dd_size_174_quality_85_7d69b1cc_20251018T122441Z.jpg) -*Axial T1 C+ MR in a 22-year-old woman with right vision loss demonstrates swelling with enhancement of the intraorbital segment of the right optic nerve.* - -![Axial FLAIR MR (top) and T1 C+ MR (bottom) images in the same patient show hyperintense enhancing lesions along the cerebral peduncles. AQP4 antibody was positive in this patient with NMOSD. Brain lesions in NMOSD typically involve the deep white matter, periependymal regions, corpus callosum, corticospinal tracts, brainstem, and cerebellum.](images/app.statdx.com_image_thumbnail_d26008df-62a0-4116-a5a3-ebf9f767936e_annotated_true_size_900_quality_90_95e09ab2_20251018T122510Z.jpg) -*Axial FLAIR MR (top) and T1 C+ MR (bottom) images in the same patient show hyperintense enhancing lesions along the cerebral peduncles. AQP4 antibody was positive in this patient with NMOSD. Brain lesions in NMOSD typically involve the deep white matter, periependymal regions, corpus callosum, corticospinal tracts, brainstem, and cerebellum.* - -![Axial FLAIR MR (top) and T1 C+ MR (bottom) images in the same patient show hyperintense enhancing lesions along the cerebral peduncles. AQP4 antibody was positive in this patient with NMOSD. Brain lesions in NMOSD typically involve the deep white matter, periependymal regions, corpus callosum, corticospinal tracts, brainstem, and cerebellum.](images/app.statdx.com_image_thumbnail_d26008df-62a0-4116-a5a3-ebf9f767936e_size_168_quality_85_7d1f7816_20251018T095348Z.jpg) -*Axial FLAIR MR (top) and T1 C+ MR (bottom) images in the same patient show hyperintense enhancing lesions along the cerebral peduncles. AQP4 antibody was positive in this patient with NMOSD. Brain lesions in NMOSD typically involve the deep white matter, periependymal regions, corpus callosum, corticospinal tracts, brainstem, and cerebellum.* - -![Coronal T1 C+ MR images in a patient with AQP4 antibody (+) NMOSD show enhancement along the prechiasmatic right optic nerve and the optic chiasm .](images/app.statdx.com_image_thumbnail_18a3a92e-8b6e-4557-9a3e-308224813fb7_annotated_true_size_900_quality_90_79e1db98_20251018T122510Z.jpg) -*Coronal T1 C+ MR images in a patient with AQP4 antibody (+) NMOSD show enhancement along the prechiasmatic right optic nerve and the optic chiasm .* - -![Coronal T1 C+ MR images in a patient with AQP4 antibody (+) NMOSD show enhancement along the prechiasmatic right optic nerve and the optic chiasm .](images/app.statdx.com_image_thumbnail_18a3a92e-8b6e-4557-9a3e-308224813fb7_size_168_quality_85_b02969d9_20251018T095348Z.jpg) -*Coronal T1 C+ MR images in a patient with AQP4 antibody (+) NMOSD show enhancement along the prechiasmatic right optic nerve and the optic chiasm .* - -![Axial FLAIR MR (top) and T1 C+ MR (bottom) in the same patient show hyperintense lesions with patchy enhancement in the periependymal region surrounding the 3rd ventricle , and the hypothalamus . Brain lesions in NMOSD are typically localized in the periependymal regions where AQP4 is highly expressed.](images/app.statdx.com_image_thumbnail_68022be5-3461-4a7b-b463-bce85be372d7_annotated_true_size_900_quality_90_7f49ea45_20251018T122510Z.jpg) -*Axial FLAIR MR (top) and T1 C+ MR (bottom) in the same patient show hyperintense lesions with patchy enhancement in the periependymal region surrounding the 3rd ventricle , and the hypothalamus . Brain lesions in NMOSD are typically localized in the periependymal regions where AQP4 is highly expressed.* - -![Axial FLAIR MR (top) and T1 C+ MR (bottom) in the same patient show hyperintense lesions with patchy enhancement in the periependymal region surrounding the 3rd ventricle , and the hypothalamus . Brain lesions in NMOSD are typically localized in the periependymal regions where AQP4 is highly expressed.](images/app.statdx.com_image_thumbnail_68022be5-3461-4a7b-b463-bce85be372d7_size_168_quality_85_f3b0df93_20251018T095348Z.jpg) -*Axial FLAIR MR (top) and T1 C+ MR (bottom) in the same patient show hyperintense lesions with patchy enhancement in the periependymal region surrounding the 3rd ventricle , and the hypothalamus . Brain lesions in NMOSD are typically localized in the periependymal regions where AQP4 is highly expressed.* - -![Sagittal STIR MR in a patient presenting with myelopathy demonstrates a long segment of cord enlargement with hyperintensity .](images/app.statdx.com_image_thumbnail_f35b89c5-cc67-4525-a144-dce62ca75eb4_annotated_true_size_900_quality_90_9a7ebfe3_20251018T122510Z.jpg) -*Sagittal STIR MR in a patient presenting with myelopathy demonstrates a long segment of cord enlargement with hyperintensity .* - -![Sagittal STIR MR in a patient presenting with myelopathy demonstrates a long segment of cord enlargement with hyperintensity .](images/app.statdx.com_image_thumbnail_f35b89c5-cc67-4525-a144-dce62ca75eb4_size_168_quality_85_9dfd191d_20251018T095348Z.jpg) -*Sagittal STIR MR in a patient presenting with myelopathy demonstrates a long segment of cord enlargement with hyperintensity .* - -![Sagittal T1 C+ FS MR in the same patient demonstrates patchy enhancement in the cervical and upper thoracic cord. Cord lesions in NMOSD typically extend over 3 or more contiguous vertebral segments. There is involvement of the central gray matter or central and peripheral regions of the cord with > 50% cord area in the axial plane.](images/app.statdx.com_image_thumbnail_23f4c61a-3885-4def-aa58-ab1739b25892_annotated_true_size_900_quality_90_a4b7f5b0_20251018T122510Z.jpg) -*Sagittal T1 C+ FS MR in the same patient demonstrates patchy enhancement in the cervical and upper thoracic cord. Cord lesions in NMOSD typically extend over 3 or more contiguous vertebral segments. There is involvement of the central gray matter or central and peripheral regions of the cord with > 50% cord area in the axial plane.* - -![Sagittal T1 C+ FS MR in the same patient demonstrates patchy enhancement in the cervical and upper thoracic cord. Cord lesions in NMOSD typically extend over 3 or more contiguous vertebral segments. There is involvement of the central gray matter or central and peripheral regions of the cord with > 50% cord area in the axial plane.](images/app.statdx.com_image_thumbnail_23f4c61a-3885-4def-aa58-ab1739b25892_size_168_quality_85_e2eb51a4_20251018T095348Z.jpg) -*Sagittal T1 C+ FS MR in the same patient demonstrates patchy enhancement in the cervical and upper thoracic cord. Cord lesions in NMOSD typically extend over 3 or more contiguous vertebral segments. There is involvement of the central gray matter or central and peripheral regions of the cord with > 50% cord area in the axial plane.* - -![Axial FLAIR MR in a patient with AQP4-IgG (+) NMOSD demonstrates large confluent lesions in the right temporal and occipital regions with involvement of the splenium of the corpus callosum.](images/app.statdx.com_image_thumbnail_5cad442f-db96-4ae9-9274-e8133ea9ed78_annotated_true_size_900_quality_90_7a91accd_20251018T122510Z.jpg) -*Axial FLAIR MR in a patient with AQP4-IgG (+) NMOSD demonstrates large confluent lesions in the right temporal and occipital regions with involvement of the splenium of the corpus callosum.* - -![Axial FLAIR MR in a patient with AQP4-IgG (+) NMOSD demonstrates large confluent lesions in the right temporal and occipital regions with involvement of the splenium of the corpus callosum.](images/app.statdx.com_image_thumbnail_5cad442f-db96-4ae9-9274-e8133ea9ed78_size_168_quality_85_08023041_20251018T095342Z.jpg) -*Axial FLAIR MR in a patient with AQP4-IgG (+) NMOSD demonstrates large confluent lesions in the right temporal and occipital regions with involvement of the splenium of the corpus callosum.* - -![Axial T1 C+ MR in the same patient shows large patchy areas of enhancement , often called "cloud-like" enhancement. This is one of the most common patterns of enhancement in NMOSD. Other patterns of enhancement include "pencil-thin" periependymal, nodular, and leptomeningeal.](images/app.statdx.com_image_thumbnail_414b6d7b-239f-4fb1-bd21-074aa2d10de7_annotated_true_size_900_quality_90_8a83083d_20251018T122510Z.jpg) -*Axial T1 C+ MR in the same patient shows large patchy areas of enhancement , often called "cloud-like" enhancement. This is one of the most common patterns of enhancement in NMOSD. Other patterns of enhancement include "pencil-thin" periependymal, nodular, and leptomeningeal.* - -![Axial T1 C+ MR in the same patient shows large patchy areas of enhancement , often called "cloud-like" enhancement. This is one of the most common patterns of enhancement in NMOSD. Other patterns of enhancement include "pencil-thin" periependymal, nodular, and leptomeningeal.](images/app.statdx.com_image_thumbnail_414b6d7b-239f-4fb1-bd21-074aa2d10de7_size_168_quality_85_196c576a_20251018T095342Z.jpg) -*Axial T1 C+ MR in the same patient shows large patchy areas of enhancement , often called "cloud-like" enhancement. This is one of the most common patterns of enhancement in NMOSD. Other patterns of enhancement include "pencil-thin" periependymal, nodular, and leptomeningeal.* - -![Sagittal T2 MR (right) and T1 C+ MR (left) in a patient with NMOSD presenting with nausea and myelopathy demonstrate patchy enhancing lesion involving the dorsal medulla (area postrema region) and the upper cervical cord .](images/app.statdx.com_image_thumbnail_e41a4f6e-cc0e-4e88-9043-7449c91ff599_annotated_true_size_900_quality_90_9a269e45_20251018T122510Z.jpg) -*Sagittal T2 MR (right) and T1 C+ MR (left) in a patient with NMOSD presenting with nausea and myelopathy demonstrate patchy enhancing lesion involving the dorsal medulla (area postrema region) and the upper cervical cord .* - -![Sagittal T2 MR (right) and T1 C+ MR (left) in a patient with NMOSD presenting with nausea and myelopathy demonstrate patchy enhancing lesion involving the dorsal medulla (area postrema region) and the upper cervical cord .](images/app.statdx.com_image_thumbnail_e41a4f6e-cc0e-4e88-9043-7449c91ff599_size_168_quality_85_0cf202eb_20251018T095343Z.jpg) -*Sagittal T2 MR (right) and T1 C+ MR (left) in a patient with NMOSD presenting with nausea and myelopathy demonstrate patchy enhancing lesion involving the dorsal medulla (area postrema region) and the upper cervical cord .* - -![Axial FLAIR (top) and T1 C+ MR (bottom) show patchy hyperintensity involving the cortex , cingulate gyrus, and corpus callosum with leptomeningeal and nodular enhancement. MOG was (+) and AQP4-IgG was (-).](images/app.statdx.com_image_thumbnail_5abd7f5f-c219-4988-be56-6ab012cbd08c_annotated_true_size_900_quality_90_4488b678_20251018T095345Z.jpg) -*Axial FLAIR (top) and T1 C+ MR (bottom) show patchy hyperintensity involving the cortex , cingulate gyrus, and corpus callosum with leptomeningeal and nodular enhancement. MOG was (+) and AQP4-IgG was (-).* - -![Axial FLAIR (top) and T1 C+ MR (bottom) show patchy hyperintensity involving the cortex , cingulate gyrus, and corpus callosum with leptomeningeal and nodular enhancement. MOG was (+) and AQP4-IgG was (-).](images/app.statdx.com_image_thumbnail_5abd7f5f-c219-4988-be56-6ab012cbd08c_size_168_quality_85_abf644a3_20251018T095342Z.jpg) -*Axial FLAIR (top) and T1 C+ MR (bottom) show patchy hyperintensity involving the cortex , cingulate gyrus, and corpus callosum with leptomeningeal and nodular enhancement. MOG was (+) and AQP4-IgG was (-).* - - -### Additional Images - -![Axial FLAIR MR in a patient with NMO demonstrates an ill-defined hyperintense lesion involving the right cerebral peduncle and midbrain .](images/app.statdx.com_image_thumbnail_2c2321de-7889-40ce-8df8-04e4d3d797b4_size_168_quality_85_a4060e5c_20251018T095343Z.jpg) -*Axial FLAIR MR in a patient with NMO demonstrates an ill-defined hyperintense lesion involving the right cerebral peduncle and midbrain .* - -![Axial T1 C+ MR in the same patient shows a well-defined nodular area of enhancement in the region of FLAIR signal abnormality. Most reported NMO brain lesions do not show enhancement. The enhancement patterns reported include patchy enhancement with blurred margins ("cloud-like" enhancement), "pencil-thin" ependymal enhancement, and solid enhancement.](images/app.statdx.com_image_thumbnail_f366a54b-a153-482a-9a92-4cc470920912_size_168_quality_85_4afd1519_20251018T095342Z.jpg) -*Axial T1 C+ MR in the same patient shows a well-defined nodular area of enhancement in the region of FLAIR signal abnormality. Most reported NMO brain lesions do not show enhancement. The enhancement patterns reported include patchy enhancement with blurred margins ("cloud-like" enhancement), "pencil-thin" ependymal enhancement, and solid enhancement.* - -![Axial T1WI C+ FS MR shows a markedly enhancing prechiasmatic right optic nerve and chiasm , consistent with acute optic neuritis.](images/app.statdx.com_image_thumbnail_0c76b02a-afd8-4735-91b3-86054aaebc0b_size_168_quality_85_48167667_20251018T095343Z.jpg) -*Axial T1WI C+ FS MR shows a markedly enhancing prechiasmatic right optic nerve and chiasm , consistent with acute optic neuritis.* - -![Sagittal T2 DP FSE MR in the same patient shows a long segment of cord enlargement with hyperintensity. Ill-defined enhancement was also present in the cervical cord in this patient with myelopathy and vision loss. Patients with NMO have a worse prognosis with more severe disability than multiple sclerosis, despite lack of brain involvement.](images/app.statdx.com_image_thumbnail_e636432e-5135-43e6-a253-ca5aa5f3cb60_size_168_quality_85_6e526ceb_20251018T095342Z.jpg) -*Sagittal T2 DP FSE MR in the same patient shows a long segment of cord enlargement with hyperintensity. Ill-defined enhancement was also present in the cervical cord in this patient with myelopathy and vision loss. Patients with NMO have a worse prognosis with more severe disability than multiple sclerosis, despite lack of brain involvement.* - -![Sagittal T2 (left) and T1WI C+ FS (right) images show multilevel T2 hyperintensity with irregular posterior enhancement in the cervical cord in a patient with a previous history of optic neuritis.](images/app.statdx.com_image_thumbnail_8d9ecb2b-7375-4a5d-b755-00d2cf0f6a6e_size_168_quality_85_5b5fdacd_20251018T095343Z.jpg) -*Sagittal T2 (left) and T1WI C+ FS (right) images show multilevel T2 hyperintensity with irregular posterior enhancement in the cervical cord in a patient with a previous history of optic neuritis.* - -![Sagittal T2WI MR in the same patient 1 year after treatment shows near-complete resolution of the T2 signal abnormality. Enhancement was no longer seen in the cervical cord. The cord lesions seen in NMO typically extend over 3 or more segments.](images/app.statdx.com_image_thumbnail_82abad06-b739-4851-b986-c84ef3ee2600_size_168_quality_85_f32b39c9_20251018T095343Z.jpg) -*Sagittal T2WI MR in the same patient 1 year after treatment shows near-complete resolution of the T2 signal abnormality. Enhancement was no longer seen in the cervical cord. The cord lesions seen in NMO typically extend over 3 or more segments.* - -![Axial FLAIR MR in a patient who presented with altered sensorium demonstrates a large hyperintense lesion in the left medial temporal lobe .](images/app.statdx.com_image_thumbnail_06bed7ab-14a6-4703-b182-192335646122_size_168_quality_85_19338595_20251018T095342Z.jpg) -*Axial FLAIR MR in a patient who presented with altered sensorium demonstrates a large hyperintense lesion in the left medial temporal lobe .* - -![Axial T1 C+ MR in the same patient shows patchy enhancement with blurred margins ("cloud-like" enhancement). NMO- IgG antibody in serum was positive in this patient. This pattern of enhancement has been reported as relatively specific for NMO.](images/app.statdx.com_image_thumbnail_db3d5458-48ba-4f3d-ba25-faaac039a137_size_168_quality_85_5f5ffb06_20251018T095343Z.jpg) -*Axial T1 C+ MR in the same patient shows patchy enhancement with blurred margins ("cloud-like" enhancement). NMO- IgG antibody in serum was positive in this patient. This pattern of enhancement has been reported as relatively specific for NMO.* - -![Axial T1 C+ MR in the same patient 4 days after IV steroid therapy shows almost complete resolution of the enhancement .](images/app.statdx.com_image_thumbnail_9ef79e0d-9f98-4092-a887-e607fe1a5b8a_size_168_quality_85_49b31cf4_20251018T095343Z.jpg) -*Axial T1 C+ MR in the same patient 4 days after IV steroid therapy shows almost complete resolution of the enhancement .* - -![Axial FLAIR MR in a patient with NMO shows multiple characteristic brain lesions. A large tumefactive lesion with ill-defined borders is seen in the left frontal lobe . An ill-defined hyperintense lesion is seen involving the posterior limb of right internal capsule , and there is involvement of the splenium of corpus callosum in a unique "arch bridge" pattern.](images/app.statdx.com_image_thumbnail_edd8c34f-8077-4ed1-82a4-b1ecbfd4416e_size_168_quality_85_a2a9bd81_20251018T095342Z.jpg) -*Axial FLAIR MR in a patient with NMO shows multiple characteristic brain lesions. A large tumefactive lesion with ill-defined borders is seen in the left frontal lobe . An ill-defined hyperintense lesion is seen involving the posterior limb of right internal capsule , and there is involvement of the splenium of corpus callosum in a unique "arch bridge" pattern.* - -![Axial FLAIR MR in a patient with NMOSD shows characteristic periependymal lesions surrounding the 3rd ventricle, involving the thalamus and hypothalamus .](images/app.statdx.com_image_thumbnail_8e2f3575-7388-4b4f-8548-7e248b2eebc5_size_168_quality_85_838a7979_20251018T095343Z.jpg) -*Axial FLAIR MR in a patient with NMOSD shows characteristic periependymal lesions surrounding the 3rd ventricle, involving the thalamus and hypothalamus .* - -![Axial T1 C+ MR in the same patient shows subtle rim enhancement in the lesion in the right thalamus . Brain lesions in NMO/NMOSD are typically localized in the periependymal regions where AQP4 is highly expressed.](images/app.statdx.com_image_thumbnail_01660eb5-a786-4e07-86e9-89379547c8b8_size_168_quality_85_d91cf3d1_20251018T095343Z.jpg) -*Axial T1 C+ MR in the same patient shows subtle rim enhancement in the lesion in the right thalamus . Brain lesions in NMO/NMOSD are typically localized in the periependymal regions where AQP4 is highly expressed.* - diff --git a/out/normal-pressure-hydrocephalus_ba3f857d-58de-4f21-8463-1631b4cb9972.md b/out/normal-pressure-hydrocephalus_ba3f857d-58de-4f21-8463-1631b4cb9972.md new file mode 100644 index 0000000..529baae --- /dev/null +++ b/out/normal-pressure-hydrocephalus_ba3f857d-58de-4f21-8463-1631b4cb9972.md @@ -0,0 +1,438 @@ +--- +title: "Normal-Pressure Hydrocephalus" +docid: "ba3f857d-58de-4f21-8463-1631b4cb9972" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Hydrocephalus" + slug: "hydrocephalus" + treeNodeId: "9ce86e3b-fab6-4657-9e51-5f47bb1a51b5" + - + name: "Normal-Pressure Hydrocephalus" + slug: "normal-pressure-hydrocephalus" + treeNodeId: null +category: "Brain" +cmeTopicId: "fbb5e19b-9cf4-446b-b7c7-f02091bc7b0f" +documentVersionId: "87d30aa9-d11d-4e89-b66b-ec30c687ba56" +imageCount: 25 +lastUpdated: "08/20/20" +pageDescription: "Normal-Pressure Hydrocephalus" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Normal-Pressure Hydrocephalus" +pageTitle: "Normal-Pressure Hydrocephalus | STATdx" +enhancedTitle: "Normal-Pressure Hydrocephalus" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Hydrocephalus" + - "Normal-Pressure Hydrocephalus" +--- +# KEY FACTS + +- ## Terminology + + + - Ventriculomegaly with normal CSF pressure, altered CSF dynamics + - Idiopathic normal-pressure hydrocephalus (iNPH) +- ## Imaging + + + - Enlarged lateral & 3rd ventricles, 4th ventricle relatively normal + - **Evans index**(ratio of widest diameter of frontal horns to widest diameter of brain on same axial slice) ≥ 0.3 + - **Callosal angle** (angle between lateral ventricles on coronal image at level of posterior commissure) < 90° + - Disproportionately enlarged subarachnoid space hydrocephalus (**DESH**) (particularly sylvian fissures & basal cisterns) + - **Cingulate sulcus sign**: Narrowing of posterior cingulate sulcus compared with anterior + - Tight high convexity with effacement of parafalcine sulci + - Aqueductal flow void + - Aqueduct stroke volume (ASV) > 42 μL +- ## Top Differential Diagnoses + + + - Normal aging brain + - Alzheimer disease + - Multiinfarct dementia (MID) + - Subcortical arteriosclerotic encephalopathy +- ## Pathology + + + - Pathogenesis of **iNPH**poorly understood & controversial + - Secondary NPH (**sNPH**): Subarachnoid hemorrhage, meningitis, neurosurgery, or head trauma) +- ## Clinical Issues + + + - Heterogeneous syndrome (classic clinical triad = dementia, gait apraxia, urinary incontinence) +- ## Diagnostic Checklist + + + - Is ventricular dilation solely due to atrophy? + - Diagnostic challenge = identify shunt-responsive NPH + +# TERMINOLOGY + +- ## Abbreviations + + + - Normal-pressure hydrocephalus (NPH) +- ## Synonyms + + + - Idiopathic adult hydrocephalus syndrome +- ## Definitions + + + - Ventriculomegaly with normal CSF pressure, altered CSF dynamics + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Enlarged lateral & 3rd ventricles, 4th ventricle relatively normal + - Ventricular enlargement out of proportion to generalized sulcal enlargement + - Disproportionately enlarged subarachnoid space hydrocephalus (**DESH**) (particularly sylvian fissures & basal cisterns) + - High tight convexity with effacement of sulci at vertex + - ### Location + + + - Ventriculomegaly involves all 3 horns of lateral ventricles, plus 3rd ventricle + - 4th ventricle relatively spared + - ### Size + + + - **Evans index**(ratio of widest diameter of frontal horns to widest diameter of brain on same axial slice) ≥ 0.3 + - **Callosal angle** (angle between lateral ventricles on coronal image at level of posterior commissure) < 90° + - Widening of temporal horn > 6 mm + - ### Morphology + + + - Diffuse expansion of ventricles +- ## CT Findings + + + - ### NECT + + + - Ventriculomegaly with rounded frontal horns, out of proportion to sulcal atrophy (ventriculosulcal disproportion) + - Frontal/occipital periventricular hypodensities may be present + - Corpus callosal thinning (nonspecific) + - Prominent basal cisterns & sylvian fissures +- ## MR Findings + + + - Lateral ventricles enlarged with rounded frontal horns + - Focal bulging of roof of lateral ventricles + - Moderately dilated 3rd ventricle, relatively normal 4th ventricle + - Dilatation of optic & infundibular recesses of anterior 3rd ventricle + - **Cingulate sulcus sign**: Narrowing of posterior cingulate sulcus compared with anterior + - DESH + - High tight convexity with effacement of parafalcine sulci + - Corpus callosum bowed upward (may be impinged by falx) + - **Aqueductal flow void sign** + - Reflects increased CSF velocity through cerebral aqueduct + - May be reduced if flow compensation, fast spin-echo techniques used + - Periventricular "halo" high signal, primarily anterior to frontal horns or posterior to occipital horns of lateral ventricles + - 50-60% have periventricular & deep white matter (WM) lesions + - More frequent, severe compared to age-matched controls + - **Diffusion tensor imaging (DTI)** + - Increased fractional anisotropy (FA) values in posterior limb of internal capsule + - **MR elastography (MRE)** + - Increased stiffness in cerebral, occipital, parietal lobes + - Decreased stiffness in periventricular WM + - **MR perfusion** + - ASL: Reduced CBF in periventricular WM, basal ganglia, & thalamus +- ## Nuclear Medicine Findings + + + - ### PET + + + - F-18 FDG PET shows decreased regional cerebral metabolism + - SPECT: Cerebral blood flow decreased in patients with NPH + - **In-111 DTPA cisternography** + - Prominent ventricular activity with no flow over convexity at 24-48 hours + - High false-positive rate +- ## Other Modality Findings + + + - **Phase-contrast cine MR** + - Cardiac-gated CSF flow studies through aqueduct + - Hyperdynamic CSF flow in both systole & diastole + - Net CSF flow direction caudocranial, reverse of normal + - Aqueduct stroke volume (ASV) > 42 μL + - Lack of correlation between ASV & symptom severity + - ICP monitoring: Wave amplitude > 9-mm Hg correlates with post shunt cognitive improvement +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR with CSF flow studies + - CT helpful + +# DIFFERENTIAL DIAGNOSIS + +- [Normal Aging Brain](/document/normal-aging-brain/2a315550-b2ea-4afe-a2ef-f93a2209f276) + - Thin, periventricular high-signal rim is normal + - Few/no WM hyperintensities + - Proportionately enlarged ventricles & subarachnoid spaces +- [Alzheimer Disease](/document/alzheimer-disease/f71f5cf5-b1af-4c6d-b145-b4c10eec7b58) + - Dementia out of proportion to gait disturbance + - Large parahippocampal fissures, small hippocampi, sulcal enlargement +- [Multiinfarct Dementia](/document/vascular-dementia/f59dab57-c511-4369-8fcc-592421a4b8d1) + - Multiple infarcts on imaging +- [Subcortical Arteriosclerotic Encephalopathy (Binswanger Disease)](/document/chronic-hypertensive-encephalopathy/1afc1f3f-203d-4cdf-8d49-2283cb13d6db) + - Irreversible ischemic degeneration of periventricular & deep WM + - MR shows extensive periventricular & deep WM hyperintensities, enlarged ventricles + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Pathogenesis of idiopathic NPH (iNPH)****poorly understood & controversial + - Reduced CBF, altered CSF resorption without increased CSF pressure + - Brain expands in systole, causes CSF displacement + - Loss of parenchymal compliance, altered viscoelastic properties of ventricular wall + - Increased interstitial fluid (ISF), pulsation pressure directed toward ventricles, water hammer effect + - May be further complicated by microangiopathy (including venous compromise), atrophy + - Vascular etiology + - Altered arterial hemodynamics + - Cortical venous dysfunction, impairs CSF absorption + - Imbalance between CSF & ISF + - Excess ISF, reversing ISF flow, impaired removal of neurotoxic compounds (β-amyloid & tau) + - Glymphatic system + - Facilitates brain fluid clearance & waste removal during sleep + - Studies implicated impaired glymphatic function in both Alzheimer disease (AD) & iNPH + - Secondary NPH (**sNPH**): Subarachnoid hemorrhage, meningitis, neurosurgery, or head trauma +- ## Microscopic Features + + + - Arachnoid fibrosis (50%) + - Periventricular tissue: Disruption of ependyma, edema, neuronal degeneration, & gliosis + - Cerebral parenchyma: Almost 50% show no significant pathology + - 20% neurofibrillary tangles, other AD changes + - 10% arteriosclerosis, ischemic encephalomalacia + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Heterogeneous syndrome (classic clinical triad = dementia, gait disorder, urinary incontinence) + - Gait disorder: Disturbed postural & locomotor reflexes in absence of primary sensorimotor deficits + - Bladder dysfunction: Urinary urgency with difficulty inhibiting bladder emptying + - Dementia: Apathy or amotivation, daytime sleepiness, psychomotor slowing + - ### Clinical profile + + + - Reversible cause of dementia + - Confirmed iNPH, possible iNPH & probable iNPH based on clinical findings, imaging, response to high-volume lumbar tap +- ## Demographics + + + - ### Age + + + - Average onset: 70 years + - Idiopathic form of NPH tends to present in elderly + - Patients with chronic communicating hydrocephalus due to prior known insult tend to present at earlier age + - ### Sex + + + - M = F + - ### Ethnicity + + + - No ethnic predilection + - ### Epidemiology + + + - Accounts for ~ 5-6% of dementias + - Reported prevalence 0.5-3.0% in elderly population +- ## Natural History & Prognosis + + + - Continuing cognitive & motor decline, akinetic mutism, & eventual death + - Potentially reversible cause of dementia when shunted +- ## Treatment + + + - **Presurgical confirmatory tests** + - Tap test (large-volume lumbar puncture) + - To assess patient's response to CSF removal + - Remove 30-50 mL of CSF + - Gait & cognition evaluated before LP & 3-4 hours after LP + - High positive predictive value (73-100%) + - Drainage of CSF via spinal catheter (external lumbar drainage) + - Lumbar catheter placed, CSF removal (10 mL/h) for 2-3 days + - Gait & cognition evaluated before & after + - Infusion testing + - 2 LP needles in lumbar subarachnoid space: 1 connected to infusion pump to infuse artificial CSF, 1 to closed-pressure recording device + - CSF outflow resistance (Rουτ) & conductance calculated + - Rουτ 8-18 mm Hg/mL/min associated with favorable shunt outcomes + - 1-2 hours to complete, needs specialized equipment + - **CSF diversion surgery** + - Shunt surgery indicated for patients who respond to CSF drainage or have CSF hydrodynamic variables consistent with NPH + - Endoscopic third ventriculostomy (ETV) + - Has not been favorable for treatment of iNPH + - Predictors of positive response to shunting remains elusive + - Gait disturbance is clinical symptom most likely to respond to surgery + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Whether ventricular dilation is solely due to atrophy + - Diagnostic challenge = identify shunt-responsive NPH + + e10a5877-65d6-43e9-a9ce-75d0ebe5fd92 + +## References + +# Selected References + +1. 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[Skalický P et al: Normal pressure hydrocephalus-an overview of pathophysiological mechanisms and diagnostic procedures. Neurosurg Rev. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31705404%5Bpmid%5D) +1. [Bradley WG Jr: CSF Flow in the brain in the context of normal pressure hydrocephalus. AJNR Am J Neuroradiol. 36(5):831-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25355813%5Bpmid%5D) +1. [Hoza D et al: DTI-MRI biomarkers in the search for normal pressure hydrocephalus aetiology: a review. Neurosurg Rev. 38(2):239-44; discussion 244, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25345377%5Bpmid%5D) +1. [Ringstad G et al: Aqueductal stroke volume: comparisons with intracranial pressure scores in idiopathic normal pressure hydrocephalus. AJNR Am J Neuroradiol. 36(9):1623-30, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25977480%5Bpmid%5D) +1. [Chotai S et al: External lumbar drain: a pragmatic test for prediction of shunt outcomes in idiopathic normal pressure hydrocephalus. Surg Neurol Int. 5:12, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24678428%5Bpmid%5D) +1. [Reddy GK et al: Long-term outcomes of ventriculoperitoneal shunt surgery in patients with hydrocephalus. World Neurosurg. 81(2):404-10, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=23380280%5Bpmid%5D) +1. [Torsnes L et al: Treatment and clinical outcome in patients with idiopathic normal pressure hydrocephalus--a systematic review. Dan Med J. 61(10):A4911, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25283616%5Bpmid%5D) +1. [Virhammar J et al: Preoperative prognostic value of MRI findings in 108 patients with idiopathic normal pressure hydrocephalus. AJNR Am J Neuroradiol. 35(12):2311-8, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25012669%5Bpmid%5D) +1. [Williams MA et al: Diagnosis and management of idiopathic normal-pressure hydrocephalus. Neurol Clin Pract. 3(5):375-85, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24175154%5Bpmid%5D) +1. [Kim MJ et al: Differential diagnosis of idiopathic normal pressure hydrocephalus from other dementias using diffusion tensor imaging. AJNR Am J Neuroradiol. 32(8):1496-503, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21700790%5Bpmid%5D) +1. [Linninger AA et al: Normal and hydrocephalic brain dynamics: the role of reduced cerebrospinal fluid reabsorption in ventricular enlargement. Ann Biomed Eng. 37(7):1434-47, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19373558%5Bpmid%5D) +1. [Scollato A et al: Changes in aqueductal CSF stroke volume in shunted patients with idiopathic normal-pressure hydrocephalus. AJNR Am J Neuroradiol. 30(8):1580-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19461060%5Bpmid%5D) +1. [Woodworth GF et al: Cerebrospinal fluid drainage and dynamics in the diagnosis of normal pressure hydrocephalus. Neurosurgery. 64(5):919-25; discussion 925-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19404152%5Bpmid%5D) +1. [Shprecher D et al: Normal pressure hydrocephalus: diagnosis and treatment. Curr Neurol Neurosci Rep. 8(5):371-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18713572%5Bpmid%5D) +1. [Owler BK et al: Normal pressure hydrocephalus and cerebral blood flow: a PET study of baseline values. J Cereb Blood Flow Metab. 24(1):17-23, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14688613%5Bpmid%5D) +1. [Czosnyka M et al: Age dependence of cerebrospinal pressure-volume compensation in patients with hydrocephalus. J Neurosurg. 94(3):482-6, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11235954%5Bpmid%5D) +1. [Kizu O et al: Proton chemical shift imaging in normal pressure hydrocephalus. AJNR Am J Neuroradiol. 22(9):1659-64, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11673158%5Bpmid%5D) +1. [Tullberg M et al: Normal pressure hydrocephalus: vascular white matter changes on MR images must not exclude patients from shunt surgery. AJNR Am J Neuroradiol. 22(9):1665-73, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11673159%5Bpmid%5D) +1. [Parkkola RK et al: Cerebrospinal fluid flow in patients with dilated ventricles studied with MR imaging. Eur Radiol. 10(9):1442-6, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10997433%5Bpmid%5D) +1. [Bech RA et al: Frontal brain and leptomeningeal biopsy specimens correlated with cerebrospinal fluid outflow resistance and B-wave activity in patients suspected of normal-pressure hydrocephalus. Neurosurgery. 40(3):497-502, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9055288%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal T1 MR shows large lateral ventricles , thinning of the corpus callosum , and a relatively normal 4th ventricle in a patient with iNPH.](images/app.statdx.com_image_thumbnail_a2b16904-c08e-4fc6-a3fc-276862837b89_annotated_true_size_900_quality_90_53b3054c_20251018T155136Z.jpg) +*Sagittal T1 MR shows large lateral ventricles , thinning of the corpus callosum , and a relatively normal 4th ventricle in a patient with iNPH.* + +![Sagittal T1 MR shows large lateral ventricles , thinning of the corpus callosum , and a relatively normal 4th ventricle in a patient with iNPH.](images/app.statdx.com_image_thumbnail_a2b16904-c08e-4fc6-a3fc-276862837b89_size_174_quality_85_31388a23_20251018T155131Z.jpg) +*Sagittal T1 MR shows large lateral ventricles , thinning of the corpus callosum , and a relatively normal 4th ventricle in a patient with iNPH.* + +![Axial CECT demonstrates typical findings suggestive of iNPH. There is enlargement of the lateral ventricles and sylvian fissures out of proportion to the amount of general sulcal enlargement. The frontal horns show a characteristic rounded appearance. Periventricular hypodensities could reflect interstitial migration of CSF.](images/app.statdx.com_image_thumbnail_85c645de-d929-40ae-a6fe-1be12714e0f0_annotated_true_size_900_quality_90_a8c68531_20251018T155136Z.jpg) +*Axial CECT demonstrates typical findings suggestive of iNPH. There is enlargement of the lateral ventricles and sylvian fissures out of proportion to the amount of general sulcal enlargement. The frontal horns show a characteristic rounded appearance. Periventricular hypodensities could reflect interstitial migration of CSF.* + +![Axial T2 MR in a patient with NPH demonstrates lateral ventricular enlargement and disproportionately enlarged sylvian fissure (DESH). Evans index, which is the ratio of the maximum width of the frontal horns to the maximum internal diameter of the skull at the same level, measures 0.38. Normal Evans index is < 0.3.](images/app.statdx.com_image_thumbnail_ebc7d0a3-c2f8-464c-ac29-5c68c19bb84b_annotated_true_size_900_quality_90_71d36abb_20251018T155136Z.jpg) +*Axial T2 MR in a patient with NPH demonstrates lateral ventricular enlargement and disproportionately enlarged sylvian fissure (DESH). Evans index, which is the ratio of the maximum width of the frontal horns to the maximum internal diameter of the skull at the same level, measures 0.38. Normal Evans index is < 0.3.* + +![Coronal T2 MR in a patient with NPH shows reduced callosal angle (71°). The callosal angle is measured at the level of the posterior commissure and a normal value is between 100-120°.](images/app.statdx.com_image_thumbnail_aeb64c28-c3d9-458e-a6bd-ad656d9af047_annotated_true_size_900_quality_90_00409dd4_20251018T155136Z.jpg) +*Coronal T2 MR in a patient with NPH shows reduced callosal angle (71°). The callosal angle is measured at the level of the posterior commissure and a normal value is between 100-120°.* + +![Sagittal T1 MR demonstrates the cingulate sulcus sign in a patient with NPH with narrowing of the posterior 1/2 of the cingulate sulcus as compared with the anterior .](images/app.statdx.com_image_thumbnail_2339b846-aea2-4877-97a9-233ee3ebe4ee_annotated_true_size_900_quality_90_3a2e725a_20251018T155136Z.jpg) +*Sagittal T1 MR demonstrates the cingulate sulcus sign in a patient with NPH with narrowing of the posterior 1/2 of the cingulate sulcus as compared with the anterior .* + +![Axial FLAIR MR in the same patient demonstrates disproportionately enlarged subarachnoid spaces , consistent with DESH, and narrowing of the sulci and subarachnoid spaces over the high convexity parasagittal frontoparietal regions with a tight interhemispheric fissure .](images/app.statdx.com_image_thumbnail_9cdff4ef-acfa-4c07-ae32-9fa89770c6a0_annotated_true_size_900_quality_90_7f514e45_20251018T155136Z.jpg) +*Axial FLAIR MR in the same patient demonstrates disproportionately enlarged subarachnoid spaces , consistent with DESH, and narrowing of the sulci and subarachnoid spaces over the high convexity parasagittal frontoparietal regions with a tight interhemispheric fissure .* + +![Sagittal T1 MR in a patient with NPH demonstrates focal bulging of the roof of the lateral ventricles , which has been recently described.](images/app.statdx.com_image_thumbnail_5f38cad8-ece0-458b-9d75-babe49ca034e_annotated_true_size_900_quality_90_b94e3d2e_20251018T155136Z.jpg) +*Sagittal T1 MR in a patient with NPH demonstrates focal bulging of the roof of the lateral ventricles , which has been recently described.* + +![Twenty-four hour multiplanar In-111 DTPA cisternography in a patient with NPH shows radiotracer in the lateral ventricles with lack of activity over the convexity . Normally, there should be radiotracer movement over the convexities at 24 hours. (Courtesy C. Singh, MD and A. Ali, MD.)](images/app.statdx.com_image_thumbnail_ff9b7855-f13a-4176-883e-c4e89f0f7dd1_annotated_true_size_900_quality_90_5f1a4d63_20251018T155136Z.jpg) +*Twenty-four hour multiplanar In-111 DTPA cisternography in a patient with NPH shows radiotracer in the lateral ventricles with lack of activity over the convexity . Normally, there should be radiotracer movement over the convexities at 24 hours. (Courtesy C. Singh, MD and A. Ali, MD.)* + +![Axial T2 MR in 65 year old with NPH shows dilated temporal horns and low-signal flow void in the aqueduct caused by hyperdynamic flow of CSF.](images/app.statdx.com_image_thumbnail_47b09bc1-bb20-4e14-8e81-59f6cfdf7cf8_annotated_true_size_900_quality_90_81a16de8_20251018T155136Z.jpg) +*Axial T2 MR in 65 year old with NPH shows dilated temporal horns and low-signal flow void in the aqueduct caused by hyperdynamic flow of CSF.* + +![Axial phase-contrast cine MR CSF flow study shows increased velocity of CSF through the dilated aqueduct . There is more hyperdynamic flow through the aqueduct than the cisterns, where no high-velocity signal change is seen. Flow is incidentally noted in the posterior cerebral arteries .](images/app.statdx.com_image_thumbnail_f9fbb916-dc1a-462b-a2c3-a618a645ff44_annotated_true_size_900_quality_90_c77dcbbf_20251018T155139Z.jpg) +*Axial phase-contrast cine MR CSF flow study shows increased velocity of CSF through the dilated aqueduct . There is more hyperdynamic flow through the aqueduct than the cisterns, where no high-velocity signal change is seen. Flow is incidentally noted in the posterior cerebral arteries .* + + +### Additional Images + +![Axial NECT shows ventriculomegaly with rounded frontal horns out of proportion to sulcal enlargement.](images/app.statdx.com_image_thumbnail_d3a96325-6e69-4daf-8c92-172394deeef7_annotated_true_size_900_quality_90_3f001b22_20251018T155143Z.jpg) +*Axial NECT shows ventriculomegaly with rounded frontal horns out of proportion to sulcal enlargement.* + +![Axial T2WI MR shows ventricles dilated out of proportion to sulcal enlargement. Periventricular deep white matter lesions are also present.](images/app.statdx.com_image_thumbnail_3bcdbf6c-f961-4b53-86da-721bb8b6081c_annotated_true_size_900_quality_90_c7bfeb2c_20251018T155143Z.jpg) +*Axial T2WI MR shows ventricles dilated out of proportion to sulcal enlargement. Periventricular deep white matter lesions are also present.* + +![Axial T2WI MR shows ventriculomegaly.](images/app.statdx.com_image_thumbnail_f5f5e9ca-fa1d-4e95-8a27-08852c110848_annotated_true_size_900_quality_90_9dad0c1b_20251018T155145Z.jpg) +*Axial T2WI MR shows ventriculomegaly.* + +![Sagittal T1WI MR in the same patient shows an accentuated aqueductal flow void .](images/app.statdx.com_image_thumbnail_6adeb8c6-9016-42c7-9aaf-2f86ff10ba70_annotated_true_size_900_quality_90_96ea3ea4_20251018T155146Z.jpg) +*Sagittal T1WI MR in the same patient shows an accentuated aqueductal flow void .* + +![Axial T2WI MR shows enlarged ventricles with rounded frontal horns.](0c4cea37-93ae-487c-85f3-c8a7d71d9aa6) +*Axial T2WI MR shows enlarged ventricles with rounded frontal horns.* + +![Axial FLAIR MR in the same patient shows ventriculomegaly out of proportion to sulcal enlargement.](2d75e2a1-bb3d-4bb4-beb9-a0b7cd9ac87d) +*Axial FLAIR MR in the same patient shows ventriculomegaly out of proportion to sulcal enlargement.* + +![Axial CECT shows symmetric dilatation of the ventricles and sylvian fissures out of proportion to sulcal enlargement. Frontal and occipital periventricular hypodensities suggest transependymal CSF flow.](c6e7ae11-efe8-47e7-840b-1f5b32a0f39e) +*Axial CECT shows symmetric dilatation of the ventricles and sylvian fissures out of proportion to sulcal enlargement. Frontal and occipital periventricular hypodensities suggest transependymal CSF flow.* + +![Axial CECT in the same patient shows symmetric dilatation of the ventricles and sylvian fissures out of proportion to sulcal enlargement. Frontal and occipital periventricular hypodensities are also present.](9edb3ebd-4157-434a-81dd-15f7ac5596a9) +*Axial CECT in the same patient shows symmetric dilatation of the ventricles and sylvian fissures out of proportion to sulcal enlargement. Frontal and occipital periventricular hypodensities are also present.* + +![Axial T2WI MR shows a typical case of normal pressure hydrocephalus. There is enlargement of the lateral ventricles with no sulcal enlargement. The frontal horns show a typical rounded configuration.](feb58db2-dbcf-48ca-9ecf-10d820a2f460) +*Axial T2WI MR shows a typical case of normal pressure hydrocephalus. There is enlargement of the lateral ventricles with no sulcal enlargement. The frontal horns show a typical rounded configuration.* + +![Axial T2WI MR shows dilated temporal horns out of proportion to the sulcal prominence. Notice the low-signal flow void in the aqueduct caused by hyperdynamic flow of CSF.](d9961a29-b001-45f8-8299-4d0d582d3b1b) +*Axial T2WI MR shows dilated temporal horns out of proportion to the sulcal prominence. Notice the low-signal flow void in the aqueduct caused by hyperdynamic flow of CSF.* + +![Sagittal T1WI MR shows enlargement of the 3rd and lateral ventricles. The infundibular recess is enlarged and bulges downward. Note mild thinning of the corpus callosum .](d7ea7a3a-0439-4962-863a-eaa68f1e3090) +*Sagittal T1WI MR shows enlargement of the 3rd and lateral ventricles. The infundibular recess is enlarged and bulges downward. Note mild thinning of the corpus callosum .* + +![Axial FLAIR MR shows enlarged ventricles out of proportion to the sulcal enlargement. Notice that periventricular hyperintensity is also present .](ba993dae-1031-4377-8266-a63c7401e6e3) +*Axial FLAIR MR shows enlarged ventricles out of proportion to the sulcal enlargement. Notice that periventricular hyperintensity is also present .* + +![Axial T2WI MR in the same patient shows dilated ventricles. Normal pressure hydrocephalus accounts for ~ 5-6% of all dementias. The classic Hakim triad of dementia, gait apraxia, and urinary incontinence is present in a minority of patients.](f12e817a-78e3-4e95-82cd-a1015b08a31b) +*Axial T2WI MR in the same patient shows dilated ventricles. Normal pressure hydrocephalus accounts for ~ 5-6% of all dementias. The classic Hakim triad of dementia, gait apraxia, and urinary incontinence is present in a minority of patients.* + +![Axial NECT shows large ventricles out of proportion to the sulcal prominence with a rounded appearance of the frontal horns .](155bb56c-c65a-499e-a6ff-c17c446f1209) +*Axial NECT shows large ventricles out of proportion to the sulcal prominence with a rounded appearance of the frontal horns .* + +![Axial T2WI MR in the same patient shows ventriculomegaly. The patient presented with the classic clinical triad of NPH: Dementia, gait apraxia, and urinary incontinence. One treatment option is ventricular shunting. The response to shunting is variable.](f855cf16-59b3-438f-bdd1-282537eb5091) +*Axial T2WI MR in the same patient shows ventriculomegaly. The patient presented with the classic clinical triad of NPH: Dementia, gait apraxia, and urinary incontinence. One treatment option is ventricular shunting. The response to shunting is variable.* + diff --git a/out/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md b/out/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md deleted file mode 100644 index 26de533..0000000 --- a/out/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md +++ /dev/null @@ -1,504 +0,0 @@ ---- -title: "Pediatric Multiple Sclerosis, Brain" -docid: "f2592b04-f800-4235-9eea-a43f2bf4adfe" -authors: - - key: "99e1aff7-f42c-43a0-95ae-d89c8551aa01" - value: "Kevin R. Moore, MD" -breadcrumbs: - - - name: "Pediatrics" - slug: "pediatrics" - treeNodeId: "a915965c-d436-44cf-ae65-2f22e7246ea4" - - - name: "Diagnosis" - slug: "diagnosis" - treeNodeId: "2b5cea64-a083-489e-ac0c-ec14ba059026" - - - name: "Pediatric Neuroradiology" - slug: "pediatric-neuroradiology" - treeNodeId: "d0eb8f4a-e769-43dd-896c-8c9c27ce8759" - - - name: "Brain" - slug: "brain" - treeNodeId: "feaaadba-649b-4f0a-9aad-9188a8f9926a" - - - name: "Pathology-Based Diagnoses" - slug: "pathology-based-diagnoses" - treeNodeId: "2d26053f-23a7-4062-bf35-a93775ae1209" - - - name: "Inflammatory and Demyelinating Disease" - slug: "inflammatory-and-demyelinating-dis-" - treeNodeId: "cb319228-da96-4d29-8276-c72388a57656" - - - name: "Pediatric Multiple Sclerosis, Brain" - slug: "pediatric-multiple-sclerosis-brain" - treeNodeId: null -category: "Pediatrics" -cmeTopicId: "03b623c3-7d10-4a0f-88ec-e7830afde051" -documentVersionId: "caa247d6-e650-4954-8d11-33d96c2d0244" -imageCount: 27 -lastUpdated: "02/14/24" -pageDescription: "Pediatric Multiple Sclerosis, Brain" -pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Inflammatory and Demyelinating Disease, Pediatric Multiple Sclerosis, Brain" -pageTitle: "Pediatric Multiple Sclerosis, Brain | STATdx" -enhancedTitle: "Pediatric Multiple Sclerosis, Brain" -type: "DX" -references: true -breadcrumbs: - - "Pediatrics" - - "Diagnosis" - - "Pediatric Neuroradiology" - - "Brain" - - "Pathology-Based Diagnoses" - - "Inflammatory and Demyelinating Disease" - - "Pediatric Multiple Sclerosis, Brain" ---- -# KEY FACTS - -- ## Terminology - - - - Probable autoimmune-mediated demyelination in which environmental factors act upon genetically susceptible individuals -- ## Imaging - - - - Multiple perpendicular callososeptal T2 hyperintensities characteristic of multiple sclerosis (MS) - - Perivenular extension: Dawson fingers - - Bilateral, asymmetric, linear/ovoid FLAIR hyperintensities - - > 85% periventricular/perivenular - - 50-90% callososeptal interface - - May also commonly involve subcortical U fibers, brachium pontis, brainstem, spinal cord - - Transient enhancement during active demyelination - - > 90% disappear within 6 months - - Rare: Large, tumefactive enhancing rings - - T1-hypointense lesions suggest worse prognosis - - Correlate with disability, atrophy, progressive disease - - Advanced imaging techniques show disease in normal-appearing white matter -- ## Top Differential Diagnoses - - - - Acute disseminated encephalomyelitis - - Neuromyelitis optica - - Autoimmune-mediated vasculitis - - Leukodystrophies and mitochondrial diseases - - Lyme disease - - Susac syndrome -- ## Pathology - - - - Probable autoimmune-mediated demyelination in which environmental factors act upon genetically susceptible individuals - - Pathogenesis requires combination of genetically susceptible individual and specific environmental trigger -- ## Clinical Issues - - - - Estimated 2.5 million people in world have MS - - Pediatric MS defined as onset < 18 years - - ≈ 5% of MS patients - - < 1% of MS patients have onset < 10 years - - Most common disabling CNS disease of young adults; 1:1,000 in Western world - - Adults: M:F = 1:2; adolescents: M:F = 1:3 -- ## Diagnostic Checklist - - - - Requires dissemination in time and space within CNS for diagnosis - - McDonald criteria: Consensus statement for diagnostic criteria; last revised in 2010 - -# TERMINOLOGY - -- ## Abbreviations - - - - Multiple sclerosis (MS) -- ## Synonyms - - - - Previously known as early-onset MS (EOMS) or juvenile MS -- ## Definitions - - - - Chronic demyelinating disease characterized by recurrent episodes of CNS demyelination separated in space and time - - Pediatric MS defined as onset < 18 years - - ≈ 5% of MS patients - - < 1% of MS patients have onset < 10 years - -# IMAGING - -- ## General Features - - - - ### Best diagnostic clue - - - - Multiple perpendicular callososeptal T2 hyperintensities - - ### Location - - - - > 85% periventricular/perivenular - - 50-90% callososeptal interface - - Subcortical U fibers, brachium pontis, brainstem, spinal cord - - Infratentorial (< 10% in adults, more common in children) - - ### Size - - - - Small (5-10 mm); tumefactive lesions several cm - - ### Morphology - - - - Linear, round, or ovoid; beveled, target, lesion-in-lesion appearance - - Some children present with large, tumor-like demyelinating lesions [tumefactive demyelinating lesions (TDLs)] -- ## CT Findings - - - - ### CECT - - - - Iso-/hypodense ± mild/moderate enhancement - - Both solid and ring enhancement patterns -- ## MR Findings - - - - ### T1WI - - - - Typically hypo- or isointense - - Hypointensity correlates with axonal destruction (black holes) - - Complete tissue loss following inflammatory injury - - T1-hypointense lesions suggest worse prognosis - - Correlated with disability, atrophy, progressive disease - - Hyperintense dentate nuclei seen in secondary progressive form - - ### T2WI - - - - Hyperintense, linear foci radiating from ventricles - - Also prevalent in subcortical U fibers, brachium pontis, brainstem, and spinal cord - - Lesions tend to be well demarcated - - High cortical disease burden can be predictor of PPMS - - Hypointense basal ganglia 10-20% of chronic MS - - ### FLAIR - - - - Most sensitive sequence for brain lesions but relatively unhelpful in spinal cord - - Earliest finding: Alternating linear hyperintensity along ependyma on sagittal FLAIR - - Ependymal dot-dash sign - - Bilateral, asymmetric, linear/ovoid hyperintensities - - Perivenular extension; Dawson fingers - - Along path of deep medullary veins - - Hyperintensities become confluent with ↑ severity - - ### DWI - - - - Majority of acute plaques: Normal or ↑ diffusivity - - Few acute MS plaques may show restricted diffusion - - Often at margins of acute plaque - - Subacute/chronic plaques: ↑ diffusivity - - DTI: Reduced longitudinal diffusivity in areas of axonal injury - - ### T1WI C+ - - - - Transient enhancement during active demyelination (> 90% disappear within 6 months) - - Nodular (68%) or ring (23%) - - Semilunar, incomplete, horseshoe-shaped (9%) - - Rare: Large, tumefactive enhancing rings - - ### MRS - - - - ↓ NAA (NAA/Cr), ↑ choline (Cho/Cr), ↑ myoinositol - - MRS abnormalities found in normal-appearing white matter - - Only secondary progressive MS shows ↓ NAA in normal-appearing gray matter - - May allow early distinction between relapsing-remitting and secondary-progressive - - Perfusion MR (contrast-enhanced T2*): Low rCBV - - Can separate tumefactive MS from neoplasm - - Magnetization transfer (MT) - - ↓ MT ratio (MTR) in lesions/normal-appearing white matter - - Functional connectivity MR (fcMR) - - ↓ functional connectivity between right/left hemisphere primary visual and motor cortices - - 3.0 T vs. 1.5 T: 21% ↑ number of contrast-enhancing lesions, 30% ↑ enhancing lesion volume, 10% ↑ total lesion volume -- ## Imaging Recommendations - - - - ### Best imaging tool - - - - MR - - ### Protocol advice - - - - Contrast-enhanced brain MR with FLAIR - - Sagittal thin T2 FLAIR sequence in midline - - To identify callosal-septal interface demyelinating lesions - - Fat saturation to assess for optic neuritis (ON) - -# DIFFERENTIAL DIAGNOSIS - -- [Acute Disseminated Encephalomyelitis](/document/adem-brain/ed94b660-cf20-4ebb-8d6f-2b93505f2928) - - Viral prodrome, monophasic illness; more common in children - - Can mimic MS; gray matter often involved - - Lesions tend to be larger, more edematous, and often symmetric compared to MS -- [Neuromyelitis Optica](/document/neuromyelitis-optica-spectrum-diso-/54d4a8bc-9267-4df6-98c1-f22aae051d01) - - ON and spinal cord lesions - - Brain lesions look atypical for MS, tend to border midline CSF spaces -- [Autoimmune-Mediated Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0) - - Enhancing lesions spare callososeptal interface - - Beaded angiogram appearance -- ## Leukodystrophies and Mitochondrial Diseases - - - - Consider in younger patients and atypical presentations - - Genetic &/or laboratory confirmation -- [Lyme Disease](/document/lyme-disease/ca3da7a8-0f21-4e06-9961-a43e6af1b0cc) - - Can be identical to MS (skin rash common) -- [Susac Syndrome](/document/susac-syndrome/af240d31-5918-4981-971f-c6780f7b405f) - - Classic triad: Encephalopathy, branch retinal artery occlusions, hearing loss - -# PATHOLOGY - -- ## General Features - - - - ### Etiology - - - - Unknown; probably virus &/or autoimmune mediated in genetically susceptible individuals - - Proposed environmental triggers for MS include exposure to infectious agents (Epstein-Barr virus in particular), low serum vitamin D levels - - Higher MS prevalence in northern latitudes has prompted theories about role of vitamin D metabolites in MS pathogenesis - - Activated T cells attack myelinated axons - - Cox-2, iNOS may cause excitotoxic death of oligodendrocytes - - To date, causation has not been proved for any of proposed pathogenic etiologies - - ### Genetics - - - - Multifactorial; ↑ incidence in 1st-order relatives -- ## Staging, Grading, & Classification - - - - Major clinical subtypes - - **Relapsing-remitting** (RR) 85% initial presentation - - **Secondary-progressive** (SP), a.k.a. relapsing progressive - - By 10 years 50%; by 25 years 90% of RR patients enter SP phase - - **Primary-progressive** (PP), a.k.a. chronic progressive - - 5-10% of MS population progressive from start - - **Progressive-relapsing** (PR) - - Rare; defined as progressive disease with clear acute relapses ± full recovery - - Periods between relapses characterized by continuing disease progression - - **Radiologically isolated syndrome**(RIS) - - MR findings satisfy criteria for dissemination in space, but with no attributable MS signs and symptoms - - Increasingly recognized in pediatric population - - Presumed that some will transition to formal MS diagnosis over time - - MS variants/subtypes - - **Malignant/Marburg disease**: Younger patients, febrile prodrome, clinically fulminant, death in months - - **Schilder** type (diffuse sclerosis): Extensive, confluent, asymmetric demyelination in bilateral supra-/infratentorial parenchyma - - **Baló**type (concentric sclerosis): Large lesions with alternating zones of demyelinated/myelinated white matter - - **2017 revised McDonald criteria for MS diagnosis** - - **Dissemination in space** - - ≥ 1 T2-hyperintense lesion(s) characteristic of MS - - In at least 2 of following 4 areas - - Periventricular, juxtacortical, infratentorial, spinal cord - - **Dissemination in time** - - Simultaneous presence of gadolinium-enhancing and nonenhancing lesions at any time **or**by new T2-hyperintense or gadolinium-enhancing lesion on follow-up MR with reference to baseline scan, irrespective of timing of baseline MR - - Unlike 2010 McDonald criteria, no distinction between symptomatic and asymptomatic MR lesions is required -- ## Gross Pathologic & Surgical Features - - - - Acute: Poorly delineated, yellowish-white, periventricular plaques - - Chronic: Gray, granular, well-demarcated plaques ± generalized volume loss -- ## Microscopic Features - - - - Perivenous demyelination, oligodendrocyte loss - - Active: Foamy macrophages with myelin fragments, lipids; reactive astrocytes + perivascular inflammation; atypical reactive astrocytes, mitoses (mimic tumor) - - Chronic: Marked loss of myelin, oligodendrocytes; dense astrogliosis; minimal/no perivascular inflammation - - Axonal transection - - CSF positive for oligoclonal bands - -# CLINICAL ISSUES - -- ## Presentation - - - - ### Most common signs/symptoms - - - - Variable; initially impaired/double vision of acute ON - - ≈ 50% with positive MR develop MS - - Weakness, numbness, tingling, gait disturbances - - ↓ sphincter control, blindness, paralysis, dementia - - Cranial nerve palsy; usually multiple, 1-5% isolated (CNV, CNVI most common) - - Spinal cord symptoms in 80% - - Children may have similar clinical presentation to adults - - Clinically distinct episodes of ON, brainstem or cerebellar syndrome, or transverse myelitis followed by at least partial resolution - - More likely (than adults) to present with isolated ON, brainstem syndrome, or encephalitic symptoms, such as headache, vomiting, seizure, or altered consciousness -- ## Demographics - - - - ### Age - - - - 20-40 years; peak onset: 30 years, 3-5% < 15 years, 9% > 50 years - - ### Sex - - - - Adults: M:F = 1:2 - - Adolescents: M:F = 1:3 - - ### Ethnicity - - - - All groups, but White populations most common - - Most often occurs in temperate zones - - ### Epidemiology - - - - Estimated 2.5 million people in world have MS - - Rare disease; estimated frequency ~ 2.5 per 100,000 children - - Most common disabling CNS disease of young adults; 1:1,000 in Western world -- ## Natural History & Prognosis - - - - Prognostically, pediatric-onset MS (POMS) portends more inflammatory course and higher relapse rate compared with adult-onset MS (AOMS) - - Relapse recovery is faster and more complete compared to adults, disability progression is slower, and longer time elapses before transitioning to secondary progression - - 34% of patients have initial episode followed by normal or near-normal function - - Unfortunately, given younger age at diagnosis, POMS patients reach comparable level of handicap 10 years earlier than patients with AOMS - - Moreover, POMS can impact children's cognitive function and development - - > 50% of MS pediatric patients continue to accrue cognitive deficits within first 5 years after disease onset - - > 80% with probable MS, positive MR progress to clinically definite MS - - Majority: Protracted course with progression of deficits - - Late: Severe disability, cognitive impairment -- ## Treatment - - - - Immunomodulators &/or immunosuppressants - - Emerging trend toward earlier use of disease modifying therapies to achieve prompt immunomodulatory disease control - - Newer, more specific and effective treatments rapidly entering clinical realm - -# DIAGNOSTIC CHECKLIST - -- ## Image Interpretation Pearls - - - - 95% with clinically definite MS have positive MR findings - - a5089f2b-fbb1-4100-b013-c093925fe15e - -## References - -# Selected References - -1. [Bower A et al: Radiologically isolated syndrome and the multiple sclerosis prodrome in pediatrics: early features of the spectrum of demyelination. Semin Pediatr Neurol. 46:101053, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37451751%5Bpmid%5D) -1. [Kornbluh AB et al: Pediatric multiple sclerosis. Semin Pediatr Neurol. 46:101054, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37451754%5Bpmid%5D) -1. [Malani Shukla N et al: Demographic features and clinical course of patients with pediatric-onset multiple sclerosis on newer disease-modifying treatments. Pediatr Neurol. 145:125-31, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37348193%5Bpmid%5D) -1. [Teleanu RI et al: The state of the art of pediatric multiple sclerosis. Int J Mol Sci. 24(9), 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37175954%5Bpmid%5D) -1. [De Meo E et al: Dynamic gray matter volume changes in pediatric multiple sclerosis: a 3.5 year MRI study. Neurology. 92(15):e1709-23, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30867274%5Bpmid%5D) -1. [Makhani N et al: Oligoclonal bands increase the specificity of MRI criteria to predict multiple sclerosis in children with radiologically isolated syndrome. Mult Scler J Exp Transl Clin. 5(1):2055217319836664, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30915227%5Bpmid%5D) -1. [Fadda G et al: MRI and laboratory features and the performance of international criteria in the diagnosis of multiple sclerosis in children and adolescents: a prospective cohort study. Lancet Child Adolesc Health. 2(3):191-204, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30169254%5Bpmid%5D) -1. [Otallah S et al: Pediatric multiple sclerosis: an update. Curr Neurol Neurosci Rep. 18(11):76, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30229541%5Bpmid%5D) -1. [Ruet A: Update on pediatric-onset multiple sclerosis. Rev Neurol (Paris). 174(6):398-407, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29784250%5Bpmid%5D) -1. [Thompson AJ et al: Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol. 17(2):162-73, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29275977%5Bpmid%5D) -1. [Yeshokumar AK et al: Pediatric multiple sclerosis. Curr Opin Neurol. 30(3):216-21, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28323645%5Bpmid%5D) -1. [Waldman A et al: Pediatric multiple sclerosis: clinical features and outcome. Neurology. 87(9 Suppl 2):S74-81, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27572865%5Bpmid%5D) -1. [Roosendaal SD et al: Imaging phenotypes in multiple sclerosis. Neuroimaging Clin N Am. 25(1):83-96, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25476514%5Bpmid%5D) -1. [Aliaga ES et al: MRI mimics of multiple sclerosis. Handb Clin Neurol. 122:291-316, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24507523%5Bpmid%5D) -1. [Ciccarelli O et al: Pathogenesis of multiple sclerosis: insights from molecular and metabolic imaging. Lancet Neurol. 13(8):807-22, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25008549%5Bpmid%5D) -1. [Fernandez O et al: Biomarkers in multiple sclerosis: an update for 2014. Rev Neurol. 58(12):553-70, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24915032%5Bpmid%5D) -1. [Hardy TA et al: Baló's concentric sclerosis. Lancet Neurol. 13(7):740-6, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24943346%5Bpmid%5D) -1. [Miller TR et al: Advances in multiple sclerosis and its variants: conventional and newer imaging techniques. Radiol Clin North Am. 52(2):321-36, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24582342%5Bpmid%5D) -1. [Steenwijk MD et al: What explains gray matter atrophy in long-standing multiple sclerosis? Radiology. 272(3):832-42, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24761837%5Bpmid%5D) -1. [Klawiter EC: Current and new directions in MRI in multiple sclerosis. Continuum (Minneap Minn). 19(4 Multiple Sclerosis):1058-73, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23917101%5Bpmid%5D) -1. [Filippi M et al: MR imaging of multiple sclerosis. Radiology. 259(3):659-81, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21602503%5Bpmid%5D) -1. [Polman CH et al: Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol. 69(2):292-302, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21387374%5Bpmid%5D) -1. [Calabrese M et al: Cortical lesions in primary progressive multiple sclerosis: a 2-year longitudinal MR study. Neurology. 72(15):1330-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19365054%5Bpmid%5D) -1. [Filippi M et al: Conventional MRI in multiple sclerosis. J Neuroimaging. 17 Suppl 1:3S-9S, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17425730%5Bpmid%5D) -1. [Janardhan V et al: Multiple sclerosis: hyperintense lesions in the brain on nonenhanced T1-weighted MR images evidenced as areas of T1 shortening. Radiology. 244(3):823-31, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17690319%5Bpmid%5D) -1. [Traboulsee AL et al: The role of MRI in the diagnosis of multiple sclerosis. Adv Neurol. 98:125-46, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16400831%5Bpmid%5D) - - -## Images - - -### Selected Images - -![Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.](images/app.statdx.com_image_thumbnail_276cdca2-d11b-40a4-a1b9-c1e6f9e2755e_annotated_true_size_900_quality_90_1bd1fcce_20251018T122619Z.jpg) -*Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.* - -![Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.](images/app.statdx.com_image_thumbnail_276cdca2-d11b-40a4-a1b9-c1e6f9e2755e_size_174_quality_85_f12fe06c_20251018T122603Z.jpg) -*Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and spinal cord. Note the characteristic perpendicular orientation of the lesions at the callososeptal interface along penetrating venules.* - -![Sagittal T1 MR in a 14-year-old presenting with gait instability and facial numbness demonstrates T1-hypointense corpus callosum lesions that represent "black holes" of chronic demyelination. This was the initial clinical MS presentation for this patient.](images/app.statdx.com_image_thumbnail_ac69502e-a0c1-4199-995e-2c6e9a7c3086_annotated_true_size_900_quality_90_b2ce42e7_20251018T122619Z.jpg) -*Sagittal T1 MR in a 14-year-old presenting with gait instability and facial numbness demonstrates T1-hypointense corpus callosum lesions that represent "black holes" of chronic demyelination. This was the initial clinical MS presentation for this patient.* - -![Axial FLAIR MR in the same patient reveals larger lesions in the right frontal lobe and left periventricular white matter. Note surrounding edema in these more acute lesions.](images/app.statdx.com_image_thumbnail_3d4f4d9b-87e7-43ff-9987-ab6e2d403647_annotated_true_size_900_quality_90_e6595b25_20251018T122619Z.jpg) -*Axial FLAIR MR in the same patient reveals larger lesions in the right frontal lobe and left periventricular white matter. Note surrounding edema in these more acute lesions.* - -![Axial T1 C+ FS MR in the same patient reveals the characteristic heterogeneous enhancement pattern for a demyelinating process in these more recent lesions , meeting McDonald criteria for dissemination in space and time.](images/app.statdx.com_image_thumbnail_84d3f613-5fb0-49bc-9264-cd5d491c1dae_annotated_true_size_900_quality_90_d9864995_20251018T122619Z.jpg) -*Axial T1 C+ FS MR in the same patient reveals the characteristic heterogeneous enhancement pattern for a demyelinating process in these more recent lesions , meeting McDonald criteria for dissemination in space and time.* - -![Axial FLAIR MR in a teen patient with acute extremity sensory changes and visual disturbance shows a focal periventricular T2-hyperintense lesion . Imaging also showed additional periventricular and callosal-septal lesions (not shown).](images/app.statdx.com_image_thumbnail_16d916ea-0a7e-4e9f-a1c8-72d693cf0ed1_annotated_true_size_900_quality_90_1ca61f03_20251018T122619Z.jpg) -*Axial FLAIR MR in a teen patient with acute extremity sensory changes and visual disturbance shows a focal periventricular T2-hyperintense lesion . Imaging also showed additional periventricular and callosal-septal lesions (not shown).* - -![Axial T1 C+ FS MR in the same patient confirms abnormal peripheral lesion enhancement with an incomplete ring pattern .](images/app.statdx.com_image_thumbnail_eb4e4c93-3b75-4e29-8988-1bbd5f4dcc7e_annotated_true_size_900_quality_90_ed964a21_20251018T122619Z.jpg) -*Axial T1 C+ FS MR in the same patient confirms abnormal peripheral lesion enhancement with an incomplete ring pattern .* - -![Sagittal T2 MR in a teenager presenting with acute ataxia demonstrates numerous hyperintense corpus callosum lesions extending to the callosal-septal interface as well as brainstem, cervicomedullary , and cord lesions .](images/app.statdx.com_image_thumbnail_43966dd7-ca06-479c-b70f-ce96f8aafd66_annotated_true_size_900_quality_90_04ecb0ba_20251018T122619Z.jpg) -*Sagittal T2 MR in a teenager presenting with acute ataxia demonstrates numerous hyperintense corpus callosum lesions extending to the callosal-septal interface as well as brainstem, cervicomedullary , and cord lesions .* - -![Axial T1 C+ MR in the same patient confirms ring- and solid-enhancing demyelinating lesions, including a characteristic perpendicular periventricular lesion .](images/app.statdx.com_image_thumbnail_c072cfff-6c06-4844-b009-d262d798e8ae_annotated_true_size_900_quality_90_984edf32_20251018T122619Z.jpg) -*Axial T1 C+ MR in the same patient confirms ring- and solid-enhancing demyelinating lesions, including a characteristic perpendicular periventricular lesion .* - -![Axial FLAIR MR in a teen patient with acute left body weakness and sensory disturbance shows a tumefactive lesion with surrounding T2 hyperintensity extending into the corpus callosum. Differential considerations include neoplasm and abscess in addition to demyelinating disease.](images/app.statdx.com_image_thumbnail_50e0f229-c282-4d6a-9fef-b5791f6d67b6_annotated_true_size_900_quality_90_bc77028b_20251018T122619Z.jpg) -*Axial FLAIR MR in a teen patient with acute left body weakness and sensory disturbance shows a tumefactive lesion with surrounding T2 hyperintensity extending into the corpus callosum. Differential considerations include neoplasm and abscess in addition to demyelinating disease.* - -![Coronal T1 C+ MR in the same patient reveals an incomplete ring of enhancement surrounding the mildly hypointense lesion , permitting a diagnosis of tumefactive MS.](images/app.statdx.com_image_thumbnail_018f8f01-73b8-454e-8a37-5df91ff129a2_annotated_true_size_900_quality_90_2d52c50f_20251018T124348Z.jpg) -*Coronal T1 C+ MR in the same patient reveals an incomplete ring of enhancement surrounding the mildly hypointense lesion , permitting a diagnosis of tumefactive MS.* - - -### Additional Images - -![Sagittal FLAIR MR in a patient with chronic MS demonstrates diffuse thinning of the corpus callosum with extensive abnormal T2 hyperintensity along the callosal-septal interface, reflecting chronic demyelinating disease.](images/app.statdx.com_image_thumbnail_64f750dc-3224-43c5-90ba-8ddba6bdc43b_annotated_true_size_900_quality_90_62f650ea_20251018T124348Z.jpg) -*Sagittal FLAIR MR in a patient with chronic MS demonstrates diffuse thinning of the corpus callosum with extensive abnormal T2 hyperintensity along the callosal-septal interface, reflecting chronic demyelinating disease.* - -![Axial FLAIR MR in the same patient reveals extensive bilateral white matter demyelinating lesions, predominately periventricular but also within more peripheral white matter. Mild diffuse white matter volume loss with passive ventricular enlargement is present.](images/app.statdx.com_image_thumbnail_996cb677-acd1-4569-bfbf-ad2296ffcac2_annotated_true_size_900_quality_90_a7d0c2dd_20251018T124348Z.jpg) -*Axial FLAIR MR in the same patient reveals extensive bilateral white matter demyelinating lesions, predominately periventricular but also within more peripheral white matter. Mild diffuse white matter volume loss with passive ventricular enlargement is present.* - -![Axial T1 MR in the same patient reveals fairly extensive hypointensity within the demyelinating lesions, implying chronic disease with white matter axonal destruction (black holes).](images/app.statdx.com_image_thumbnail_3a657c80-7351-4082-95f3-595d893e949b_annotated_true_size_900_quality_90_9de59cd5_20251018T124348Z.jpg) -*Axial T1 MR in the same patient reveals fairly extensive hypointensity within the demyelinating lesions, implying chronic disease with white matter axonal destruction (black holes).* - -![Axial SWI demonstrates the characteristic perivenular location of a demyelinating plaque with the medullary vein coursing through it.](images/app.statdx.com_image_thumbnail_4c192524-a831-4d40-ba68-c5c02e83943a_annotated_true_size_900_quality_90_04c16f40_20251018T124348Z.jpg) -*Axial SWI demonstrates the characteristic perivenular location of a demyelinating plaque with the medullary vein coursing through it.* - -![Sagittal T1 C+ MR shows a large, hypointense mass with a peripheral crescent of incomplete or open ring enhancement . This enhancement pattern is classic for a tumefactive demyelinating disease, most commonly MS.](images/app.statdx.com_image_thumbnail_1837f9c5-8c83-4f6b-a1eb-2b908d9b06cc_annotated_true_size_900_quality_90_ee38f178_20251018T124348Z.jpg) -*Sagittal T1 C+ MR shows a large, hypointense mass with a peripheral crescent of incomplete or open ring enhancement . This enhancement pattern is classic for a tumefactive demyelinating disease, most commonly MS.* - -![Axial T1 C+ MR in a young male patient with rapid onset of visual disturbance demonstrates large enhancing demyelinating lesions in the deep and periventricular white matter. Marburg disease is an acute fulminant MS variant.](images/app.statdx.com_image_thumbnail_11f8e806-b63a-402c-818b-d09bb4292a9e_annotated_true_size_900_quality_90_e890f923_20251018T124348Z.jpg) -*Axial T1 C+ MR in a young male patient with rapid onset of visual disturbance demonstrates large enhancing demyelinating lesions in the deep and periventricular white matter. Marburg disease is an acute fulminant MS variant.* - -![Axial T1 C+ MR demonstrates concentric laminated "onion bulb" enhancement characteristic of acute Baló concentric sclerosis. Baló concentric sclerosis is a rare, aggressive MS variant characterized by acute onset and rapid deterioration.](images/app.statdx.com_image_thumbnail_4a75294e-51c4-4d89-b889-7acd68338eec_annotated_true_size_900_quality_90_da796d33_20251018T124348Z.jpg) -*Axial T1 C+ MR demonstrates concentric laminated "onion bulb" enhancement characteristic of acute Baló concentric sclerosis. Baló concentric sclerosis is a rare, aggressive MS variant characterized by acute onset and rapid deterioration.* - -![Sagittal FLAIR MR shows MS plaques with typical perpendicular orientation at the callososeptal interface along penetrating venules (Dawson fingers) as well as involving subcortical white matter.](images/app.statdx.com_image_thumbnail_c74fc30d-c463-45b9-b324-2a8cffd7d113_annotated_true_size_900_quality_90_57058fcd_20251018T124348Z.jpg) -*Sagittal FLAIR MR shows MS plaques with typical perpendicular orientation at the callososeptal interface along penetrating venules (Dawson fingers) as well as involving subcortical white matter.* - -![Sagittal FLAIR MR shows MS plaques with a hyperintense rim and central hypointensity (latter also hypointense on T1WI; not shown). Note the characteristic posterior fossa lesion .](images/app.statdx.com_image_thumbnail_4eb0a6d0-9539-4204-a570-926f106081ed_annotated_true_size_900_quality_90_42a0740d_20251018T124348Z.jpg) -*Sagittal FLAIR MR shows MS plaques with a hyperintense rim and central hypointensity (latter also hypointense on T1WI; not shown). Note the characteristic posterior fossa lesion .* - -![Axial T1 C+ MR demonstrates nodular enhancing MS plaques. Note the common periventricular location with perpendicular orientation as well as involvement of subcortical white matter.](images/app.statdx.com_image_thumbnail_7ddbe865-5df6-44ca-8781-de1475539664_annotated_true_size_900_quality_90_ddd6cd94_20251018T124348Z.jpg) -*Axial T1 C+ MR demonstrates nodular enhancing MS plaques. Note the common periventricular location with perpendicular orientation as well as involvement of subcortical white matter.* - -![Axial T1 C+ MR shows irregular, thick partial ring enhancement around a mass-like lesion in a patient not previously diagnosed with MS. This was biopsy-proven tumefactive MS. (Courtesy M. Mirfakharee, MD.)](images/app.statdx.com_image_thumbnail_0347cae0-486b-4334-b6d0-f328deff245f_annotated_true_size_900_quality_90_6d68bea0_20251018T124348Z.jpg) -*Axial T1 C+ MR shows irregular, thick partial ring enhancement around a mass-like lesion in a patient not previously diagnosed with MS. This was biopsy-proven tumefactive MS. (Courtesy M. Mirfakharee, MD.)* - -![Sagittal FLAIR MR shows callososeptal hyperintensities radiating from the lateral ventricles with a typical perpendicular orientation, characteristic of MS.](images/app.statdx.com_image_thumbnail_cd8f0f36-8545-4966-a39d-aef1443f29eb_annotated_true_size_900_quality_90_587568f9_20251018T124348Z.jpg) -*Sagittal FLAIR MR shows callososeptal hyperintensities radiating from the lateral ventricles with a typical perpendicular orientation, characteristic of MS.* - -![Axial FLAIR MR 3T shows multiple nonenhancing, periventricular, hyperintense MS lesions oriented perpendicular to the callosomarginal interface. These lesions are perivenular along the path of the deep medullary veins and represent Dawson fingers. Confluent lesions are also seen along the right periventricular margin.](images/app.statdx.com_image_thumbnail_29c6680a-93fb-4439-aa54-eecb2b27c0e2_annotated_true_size_900_quality_90_327daa0a_20251018T124348Z.jpg) -*Axial FLAIR MR 3T shows multiple nonenhancing, periventricular, hyperintense MS lesions oriented perpendicular to the callosomarginal interface. These lesions are perivenular along the path of the deep medullary veins and represent Dawson fingers. Confluent lesions are also seen along the right periventricular margin.* - -![Axial FLAIR MR shows confluent periventricular white matter hyperintensity typical of advanced, longstanding MS with loss of discrete, linear, periventricular lesions.](images/app.statdx.com_image_thumbnail_cd769470-4fee-4b98-8b2b-a237aa07dee2_annotated_true_size_900_quality_90_52149b0b_20251018T124348Z.jpg) -*Axial FLAIR MR shows confluent periventricular white matter hyperintensity typical of advanced, longstanding MS with loss of discrete, linear, periventricular lesions.* - -![Sagittal T1 MR shows multiple hypointense lesions ("black holes") in the deep white matter related to axonal destruction. Note the associated moderate ventricular and sulcal enlargement.](images/app.statdx.com_image_thumbnail_5a2a2ea2-f45b-4465-b4d2-ba5685cc617d_annotated_true_size_900_quality_90_40187927_20251018T124348Z.jpg) -*Sagittal T1 MR shows multiple hypointense lesions ("black holes") in the deep white matter related to axonal destruction. Note the associated moderate ventricular and sulcal enlargement.* - -![Coronal T1 C+ MR shows a hypointense mass in the left posterior frontal region with a peripheral crescent of incomplete or "horseshoe" enhancement . This enhancement pattern is classic for tumefactive demyelinating disease, most commonly MS.](images/app.statdx.com_image_thumbnail_b6035566-14cd-44af-95a0-de102374633d_annotated_true_size_900_quality_90_562d9ced_20251018T124348Z.jpg) -*Coronal T1 C+ MR shows a hypointense mass in the left posterior frontal region with a peripheral crescent of incomplete or "horseshoe" enhancement . This enhancement pattern is classic for tumefactive demyelinating disease, most commonly MS.* - -![Axial T1 C+ FS MR shows bright enhancement of the optic nerves similar to the extraocular muscles in a patient with MS with acute bilateral optic neuritis.](images/app.statdx.com_image_thumbnail_71506ec8-ddb5-4665-88cd-1a9bbbb6a4fd_annotated_true_size_900_quality_90_8e155fb2_20251018T124348Z.jpg) -*Axial T1 C+ FS MR shows bright enhancement of the optic nerves similar to the extraocular muscles in a patient with MS with acute bilateral optic neuritis.* - diff --git a/out/periventricular-enhancing-lesions_0edb9603-ea97-4f3b-be82-21d53c42be32.md b/out/periventricular-enhancing-lesions_0edb9603-ea97-4f3b-be82-21d53c42be32.md new file mode 100644 index 0000000..072b878 --- /dev/null +++ b/out/periventricular-enhancing-lesions_0edb9603-ea97-4f3b-be82-21d53c42be32.md @@ -0,0 +1,409 @@ +--- +title: "Periventricular Enhancing Lesions" +docid: "0edb9603-ea97-4f3b-be82-21d53c42be32" +authors: + - key: "1fa14dfd-71ea-4960-908e-e720313bc63a" + value: "Santhosh Gaddikeri, MD" + - key: "30ce27b2-237f-4aff-a88f-65ead356335b" + value: "Marinos Kontzialis, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60" + - + name: "Ventricles, Periventricular Regions" + slug: "ventricles-periventricular-regions" + treeNodeId: "353c434a-a6fc-4ef1-8786-d30a1988a4dc" + - + name: "Generic Imaging Patterns" + slug: "generic-imaging-patterns" + treeNodeId: "969c31a2-ef56-4fc3-9125-05857cf9aac3" + - + name: "Periventricular Enhancing Lesions" + slug: "periventricular-enhancing-lesions" + treeNodeId: null +category: "Brain" +documentVersionId: "d7a3032c-c0c2-4516-837e-59885e1d3d24" +imageCount: 54 +lastUpdated: "02/14/23" +pageDescription: "Periventricular Enhancing Lesions" +pageKeywords: "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Periventricular Enhancing Lesions" +pageTitle: "Periventricular Enhancing Lesions | STATdx" +enhancedTitle: "Periventricular Enhancing Lesions" +type: "DDX" +references: true +breadcrumbs: + - "Brain" + - "Differential Diagnosis" + - "Ventricles, Periventricular Regions" + - "Generic Imaging Patterns" + - "Periventricular Enhancing Lesions" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - DWI MR may help differentiate various etiologies +- ## Helpful Clues for Common Diagnoses + + + - **Multiple Sclerosis** + - Most common acquired CNS autoimmune demyelinating disease + - Callososeptal interface involvement + - Subcallosal striations + - Ovoid lesions radiating from ventricular surface of corpus callosum (CC) into pericallosal white matter (WM) corresponding to perivenular inflammation + - Juxtacortical lesions + - Acute demyelinating lesions = may show restricted diffusion, enhancement (nodular, ring, or incomplete rim) + - **Tumefactive demyelination** + - Demyelinating lesions > 2 cm; may be mistaken for tumor when edema & mass effect + - May occur in neuroinflammatory conditions, including multiple sclerosis (MS) & ADEM + - Most closely linked with MS often at time of 1st presentation + - Look for characteristic incomplete ring of enhancement + - **ADEM** + - Monophasic demyelination triggered by febrile illness or vaccination; impaired consciousness + - Multifocal WM + deep gray matter (GM) lesions; periventricular + CC lesions larger than in MS + - Complete or partial resolution of lesions without development of new lesions + - Enhancement typical + - **Glioblastoma, IDH-Wildtype** + - Most common primary intracranial neoplasm + - Rapid enlargement, thick irregular enhancement, necrotic core + - Heterogeneous, hyperintense T2 FLAIR mass + surrounding vasogenic edema/tumor infiltration + - Tumor extends beyond visible signal changes + - **Lymphoma, Primary CNS** + - Immunocompetent: May be T2 hypointense related to high nuclear:cytoplasmic ratio, solid enhancement + - Immunocompromised: May be heterogeneous from hemorrhage, necrosis, rim enhancement + - Often crosses CC + - Mild surrounding edema is typical + - Enhancing lesion(s) within basal ganglia (BG) &/or periventricular WM + - **Metastases, Parenchymal** + - Round enhancing lesion(s) at GM-WM interface + - May be punctate to massive with variable surrounding edema, mass effect + - 50% solitary + - Primary tumor often known +- ## Helpful Clues for Less Common Diagnoses + + + - **Abscess** + - Central restricted diffusion, enhancing T2-hypointense rim, surrounding vasogenic edema + - **Septic Emboli** + - Scattered, small juxtacortical hyperintensities + - Develop into small, ring-enhancing microabscesses + - **Toxoplasmosis, Acquired** + - Multiple WM & BG ring-enhancing masses + - May show target sign + - DWI restriction variable + - Typically seen in HIV patients + - **Neurocysticercosis** + - Vesicular phase: Small 10-mm cysts with central scolex, no edema, follows CSF + - Colloidal phase: Cyst may enlarge, may be hyperintense to CSF, surrounding edema, enhancement + - Granular nodular & calcified phase: Cyst retracts, wall thickens, edema resolves, calcifies + - **Germinoma** + - Enhancing midline mass (pineal, suprasellar) typical + - Occurs in BG or thalamus 5-10% + - Hyperdense on CT + - CSF spread common + - **Vasculitis** + - Irregularities, stenosis, & vascular occlusions + - Multifocal cortical/subcortical & BG T2 hyperintensities; DWI restriction if acute + - Patchy enhancement typical + - High-resolution MR may show vessel wall enhancement + - **Lyme Disease** + - MS-like WM lesions (may enhance) + - ± multiple enhancing cranial nerves + - ± cauda equina, meningeal enhancement + - **Ependymoma** + - Majority (2/3) infratentorial + - 4th ventricle in child + - ± extension through lateral recesses into cerebellopontine angle cisterns + - 1/3 are supratentorial + - Most are extraventricular + - Typically periventricular WM + - Heterogeneous enhancing mass + - 50% are calcified + - Cysts, hemorrhage common + - **Neurosarcoid** + - Periventricular T2-hyperintense lesions + - Enhancing parenchymal lesions + - Leptomeningeal disease + - Hypothalamus/pituitary involvement + - Cranial nerve involvement + - Dural involvement + - Vasculitis + - **Neuromyelitis Optica Spectrum Disorders** + - Recurrent, often bilateral optic neuritis &/or longitudinally extensive cord lesions > 3 vertebral segments + - Periventricular/periependymal hyperintensities following distribution of aquaporin-4 + - Patchy enhancement with blurred margins (cloud-like enhancement) in cerebral lesions + - "Pencil-thin" ependymal enhancement + - Rarely, well-marginated nodular enhancement or meningeal enhancement +- ## Helpful Clues for Rare Diagnoses + + + - **Leukemia** + - Typically involves dura + - May see along penetrating vessels or ependyma + - Enhancing mass(es) in child + - **Susac Syndrome** + - Triad of encephalopathy, branch retinal artery occlusions, hearing loss + - Endotheliopathy of cerebral precapillary arterioles + - Characteristic multifocal lesions involving central fibers of CC = small infarcts + - **Alexander Disease** + - Diffuse symmetric bifrontal WM signal abnormality & enhancement + - Near-total lack of myelin + - Infant with macrocephaly, seizures, developmental delay + - **Ependymal/Subependymal Veins (Mimic)** + - Normal periventricular venous structures may become engorged with various pathologies + - Venous thrombosis, vascular malformations (arteriovenous malformation, developmental venous anomaly) + - **CLIPPERS** + - Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids + - Recently described inflammatory CNS disorder + - Predominant brainstem & pons involvement + - Punctate perivascular enhancement with surrounding T2 FLAIR hyperintensities + - Lesions typically clustered in pons, & adjacent rhombencephalic structures may extend into spinal cord & supratentorial GM & WM + - **Immune Reconstitution Inflammatory Syndrome (IRIS)** + - Atypical/worsening imaging appearance of infection in HIV/AIDS following initiation of HAART + - Immune reconstitution leading to inflammatory response to preexisting infection + - Pathogens: JC virus (progressive multifocal leukoencephalopathy), TB, CMV, *Cryptococcus*, others + - **Behçet Disease** + - Vasculitis with venous predominance + - Asymmetric mesodiencephalic junction hyperintense lesion with cranial & caudal extension ± enhancement + - Midbrain involvement with sparing of red nucleus suggestive of vasogenic edema + - Findings likely secondary to venous thrombosis with reversible edema + - Associated enhancement in acute lesions may mimic tumor + +## References + +# Selected References + +1. [Clarke L et al: Magnetic resonance imaging in neuromyelitis optica spectrum disorder. Clin Exp Immunol. 206(3):251-65, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34080180%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T1 C+ MR demonstrates multiple lesions with nodular, broken ring, and curvilinear enhancement in juxtacortical , deep and periventricular white matter (WM), consistent with active demyelinating lesions in a patient with multiple sclerosis.](images/app.statdx.com_image_thumbnail_cec9f7fa-cc33-4fb0-bc41-7c94d60c9f66_annotated_true_size_900_quality_90_1a0a2332_20251018T165158Z.jpg) +**Multiple Sclerosis** +*Axial T1 C+ MR demonstrates multiple lesions with nodular, broken ring, and curvilinear enhancement in juxtacortical , deep and periventricular white matter (WM), consistent with active demyelinating lesions in a patient with multiple sclerosis.* + +![Axial T1 C+ 3D SPGR MR demonstrates a large, enhancing lesion involving bifrontal periventricular WM and corpus callosum . Biopsy revealed demyelination. There is encephalomalacia in the bilateral parietal lobes .](images/app.statdx.com_image_thumbnail_96da31e5-9034-45be-ba03-4e0dce782335_annotated_true_size_900_quality_90_2e9667a2_20251018T165158Z.jpg) +**Tumefactive Demyelination** +*Axial T1 C+ 3D SPGR MR demonstrates a large, enhancing lesion involving bifrontal periventricular WM and corpus callosum . Biopsy revealed demyelination. There is encephalomalacia in the bilateral parietal lobes .* + +![Coronal T1 C+ MR shows multiple patchy/confluent, avidly enhancing lesions involving gray matter , WM , and along periventricular region , findings consistent with ADEM. Patient had upper respiratory tract infection 1 week before neurologic symptom onset.](images/app.statdx.com_image_thumbnail_2fe022b0-01ed-42cc-85b2-bc1b1b4ea50a_annotated_true_size_900_quality_90_9bb597d6_20251018T165158Z.jpg) +**ADEM** +*Coronal T1 C+ MR shows multiple patchy/confluent, avidly enhancing lesions involving gray matter , WM , and along periventricular region , findings consistent with ADEM. Patient had upper respiratory tract infection 1 week before neurologic symptom onset.* + +![Coronal T1 C+ SPGR MR shows a necrotic enhancing mass in the right temporal lobe periventricular region . There is additional nodular foci along the septum and ependyma of of the 3rd ventricle . Biopsy revealed glioblastoma, IDH-wildtype.](d8681e69-3087-48d8-b436-b2d58e5188be) +**Glioblastoma, IDH-Wildtype** +*Coronal T1 C+ SPGR MR shows a necrotic enhancing mass in the right temporal lobe periventricular region . There is additional nodular foci along the septum and ependyma of of the 3rd ventricle . Biopsy revealed glioblastoma, IDH-wildtype.* + +![Axial T1 C+ SPGR MR demonstrates a large, avidly enhancing mass in the right cerebellum adjacent to the 4th ventricle. Biopsy revealed lymphoma. Note mild mass effect on the 4th ventricle and surrounding hypointensity due to edema .](99c1f5b7-c21a-4f02-be42-d35376dfe337) +**Lymphoma, Primary CNS** +*Axial T1 C+ SPGR MR demonstrates a large, avidly enhancing mass in the right cerebellum adjacent to the 4th ventricle. Biopsy revealed lymphoma. Note mild mass effect on the 4th ventricle and surrounding hypointensity due to edema .* + +![Axial CECT shows rim-enhancing, periventricular metastatic lesions adjacent to the right ventricular atrium in a patient with lung carcinoma. An additional enhancing metastasis involves the left choroid plexus.](60dd2571-7af1-4d3e-978c-c7daac3c4bac) +**Metastases, Parenchymal** +*Axial CECT shows rim-enhancing, periventricular metastatic lesions adjacent to the right ventricular atrium in a patient with lung carcinoma. An additional enhancing metastasis involves the left choroid plexus.* + +![Axial T1 C+ MR demonstrates a rim-enhancing lesion in the left occipital lobe , which showed restricted diffusion and was consistent with an abscess. The abscess shows intraventricular rupture with loculated intraventricular enhancement .](3502bc4b-4b2a-4b7a-8b54-51bb7ea2033e) +**Abscess** +*Axial T1 C+ MR demonstrates a rim-enhancing lesion in the left occipital lobe , which showed restricted diffusion and was consistent with an abscess. The abscess shows intraventricular rupture with loculated intraventricular enhancement .* + +![Coronal T1 C+ SPGR MR demonstrates a right ring-enhancing lesion with an eccentric target sign , consistent with toxoplasmosis (acquired) in this patient with HIV/AIDS. Note surrounding edema and mass effect .](c5cd9ab8-7660-4df6-b178-7b01fe72c274) +**Toxoplasmosis, Acquired** +*Coronal T1 C+ SPGR MR demonstrates a right ring-enhancing lesion with an eccentric target sign , consistent with toxoplasmosis (acquired) in this patient with HIV/AIDS. Note surrounding edema and mass effect .* + +![Axial T1 C+ MR demonstrates linear periventricular and perivascular enhancement along the medullary veins. Meningeal biopsy revealed primary CNS vasculitis.](fbd95e5a-166a-4d24-a58f-cd57d4c192b9) +**Vasculitis** +*Axial T1 C+ MR demonstrates linear periventricular and perivascular enhancement along the medullary veins. Meningeal biopsy revealed primary CNS vasculitis.* + +![Axial T1 C+ MR demonstrates periventricular , ependymal , and perivascular enhancement . Biopsy of enlarged lung hilar lymph nodes was consistent with sarcoidosis.](4f7a0402-398c-48e9-a6d5-afc9c1039288) +**Neurosarcoid** +*Axial T1 C+ MR demonstrates periventricular , ependymal , and perivascular enhancement . Biopsy of enlarged lung hilar lymph nodes was consistent with sarcoidosis.* + +![Axial T1 C+ 3D SPGR MR demonstrates periventricular edema and enhancement around the 3rd ventricle. CSF was positive for antiaquaporin-4 IgG. These findings are consistent with neuromyelitis optica.](59ecb478-a253-40b0-b9ec-892043208caf) +**Neuromyelitis Optica Spectrum Disorders** +*Axial T1 C+ 3D SPGR MR demonstrates periventricular edema and enhancement around the 3rd ventricle. CSF was positive for antiaquaporin-4 IgG. These findings are consistent with neuromyelitis optica.* + +![Axial T1 C+ MR in a 9-year-old boy with acute myeloid leukemia demonstrates multiple foci of nodular enhancement in the periventricular and perivascular regions, suggesting leukemic infiltration.](c811fe46-bd19-4426-b9fb-1345ab0eb3e4) +**Leukemia** +*Axial T1 C+ MR in a 9-year-old boy with acute myeloid leukemia demonstrates multiple foci of nodular enhancement in the periventricular and perivascular regions, suggesting leukemic infiltration.* + +![Axial T1 C+ MR shows multiple foci of perivascular enhancement in the pons and bilateral middle cerebellar peduncles around the 4th ventricle. This completely resolved after steroids (not shown), suggestive of CLIPPERS.](546f7c81-009c-4d06-89eb-8803ea8e6901) +**CLIPPERS** +*Axial T1 C+ MR shows multiple foci of perivascular enhancement in the pons and bilateral middle cerebellar peduncles around the 4th ventricle. This completely resolved after steroids (not shown), suggestive of CLIPPERS.* + +![Axial T1 C+ MR shows a hypointense lesion in the right temporooccipital region with periventricular extension with marginal enhancement . In this patient with HIV/AIDS and progressive multifocal leucoencephalopathy (PML) (on HAART therapy), this is suggestive of PML-IRIS.](2957aa33-fada-47a9-b65b-fcf4730e1b2a) +**Immune Reconstitution Inflammatory Syndrome (IRIS)** +*Axial T1 C+ MR shows a hypointense lesion in the right temporooccipital region with periventricular extension with marginal enhancement . In this patient with HIV/AIDS and progressive multifocal leucoencephalopathy (PML) (on HAART therapy), this is suggestive of PML-IRIS.* + + +### Additional Images + +![Axial T1 C+ MR shows a characteristic tumefactive multiple sclerosis (MS) plaque with irregular, thick partial ring enhancement and mass effect . These lesions may cross the corpus callosum and mimic tumors.](images/app.statdx.com_image_thumbnail_8c4f66bc-64d0-4be3-936d-557116cd6718_annotated_true_size_900_quality_90_c8ffa373_20251018T165158Z.jpg) +**Multiple Sclerosis** +*Axial T1 C+ MR shows a characteristic tumefactive multiple sclerosis (MS) plaque with irregular, thick partial ring enhancement and mass effect . These lesions may cross the corpus callosum and mimic tumors.* + +![Axial T1 C+ MR shows numerous enhancing MS plaques in the periventricular and subcortical WM. Note the typical lack of mass effect. ADEM and Lyme disease may be identical.](images/app.statdx.com_image_thumbnail_6ec86b92-b92a-4ce9-9fa6-e489ec38ab24_annotated_true_size_900_quality_90_456d38ed_20251018T165158Z.jpg) +**Multiple Sclerosis** +*Axial T1 C+ MR shows numerous enhancing MS plaques in the periventricular and subcortical WM. Note the typical lack of mass effect. ADEM and Lyme disease may be identical.* + +![Coronal T1 C+ MR shows classic incomplete ring-enhancing focus in the right frontal WM with minimal mass effect. No other enhancing foci were seen.](images/app.statdx.com_image_thumbnail_67f3d4fe-0f8e-4ceb-9996-304fbc6b4f2e_annotated_true_size_900_quality_90_af6683b6_20251018T165158Z.jpg) +**Multiple Sclerosis** +*Coronal T1 C+ MR shows classic incomplete ring-enhancing focus in the right frontal WM with minimal mass effect. No other enhancing foci were seen.* + +![Axial T1 C+ MR shows characteristic imaging findings of glioblastoma that include a large, heterogeneous mass with thick, aggressive rim enhancement and central necrosis. There is mass effect and effacement of the anterior lateral ventricles.](e9c87bda-3609-4850-9608-6e3eaf0ba198) +**Glioblastoma, IDH-Wildtype** +*Axial T1 C+ MR shows characteristic imaging findings of glioblastoma that include a large, heterogeneous mass with thick, aggressive rim enhancement and central necrosis. There is mass effect and effacement of the anterior lateral ventricles.* + +![Axial T1 C+ FS MR shows a large, heterogeneously enhancing occipital lobe mass with central necrosis. Note extension across the splenium of the corpus callosum , characteristic of glioblastoma multiforme.](807e9108-c225-4863-96f0-33581213538a) +**Glioblastoma, IDH-Wildtype** +*Axial T1 C+ FS MR shows a large, heterogeneously enhancing occipital lobe mass with central necrosis. Note extension across the splenium of the corpus callosum , characteristic of glioblastoma multiforme.* + +![Axial T1 C+ FS MR shows multifocal enhancement in periventricular WM, fornices , and septum pellucidum . Noncontiguous regional involvement (satellite lesions) is a less common pattern in glioblastoma multiforme.](c7715f85-b899-4c9c-a604-d61affd63ef7) +**Glioblastoma, IDH-Wildtype** +*Axial T1 C+ FS MR shows multifocal enhancement in periventricular WM, fornices , and septum pellucidum . Noncontiguous regional involvement (satellite lesions) is a less common pattern in glioblastoma multiforme.* + +![Axial T1 C+ MR demonstrates a solidly enhancing mass with mild surrounding vasogenic edema, consistent with primary CNS lymphoma in an immunocompetent patient. In immunocompromised lymphoma, the lesions tend to present with ring enhancement.](d79a1356-b5f4-4dc5-ab1c-65b7291ae583) +**Lymphoma, Primary CNS** +*Axial T1 C+ MR demonstrates a solidly enhancing mass with mild surrounding vasogenic edema, consistent with primary CNS lymphoma in an immunocompetent patient. In immunocompromised lymphoma, the lesions tend to present with ring enhancement.* + +![Axial T1 C+ MR shows homogeneous enhancement within multiple periventricular WM foci. Lack of significant surrounding T2 abnormality (not shown) and mild mass and corpus callosum involvement is common.](e298292d-2258-4d61-8e9e-1d173c42e8ac) +**Lymphoma, Primary CNS** +*Axial T1 C+ MR shows homogeneous enhancement within multiple periventricular WM foci. Lack of significant surrounding T2 abnormality (not shown) and mild mass and corpus callosum involvement is common.* + +![Axial T1 MR shows the neoplasm is an uncommon cause of vasculitis in this case of intravascular (angiocentric) lymphoma. Note numerous foci of punctate, linear, and confluent enhancement .](2482a3b6-bf22-467c-b4f1-378e803e1ac0) +**Lymphoma, Primary CNS** +*Axial T1 MR shows the neoplasm is an uncommon cause of vasculitis in this case of intravascular (angiocentric) lymphoma. Note numerous foci of punctate, linear, and confluent enhancement .* + +![Axial T1 C+ MR shows multiple periventricular and subcortical metastatic enhancing lesions in a patient with lung adenocarcinoma.](fd0cf8be-9b1a-47b7-9200-a1f4bf8be499) +**Metastases, Parenchymal** +*Axial T1 C+ MR shows multiple periventricular and subcortical metastatic enhancing lesions in a patient with lung adenocarcinoma.* + +![Axial T1 C+ MR shows enhancing lesions in the periventricular WM in this patient with a history of breast cancer.](a1a2c677-d180-48c5-8cde-6f554452bbf4) +**Metastases, Parenchymal** +*Axial T1 C+ MR shows enhancing lesions in the periventricular WM in this patient with a history of breast cancer.* + +![Axial T1 C+ MR demonstrates a large, ring-enhancing lesion in the left frontal lobe with mild surrounding vasogenic edema. The lesion was drained surgically, yielding Streptococcus anginosus cerebral abscess.](4b227bca-6604-429e-952f-d8cac23f6382) +**Abscess** +*Axial T1 C+ MR demonstrates a large, ring-enhancing lesion in the left frontal lobe with mild surrounding vasogenic edema. The lesion was drained surgically, yielding Streptococcus anginosus cerebral abscess.* + +![Axial T1 C+ FS MR shows a ring-enhancing mass in the left frontal lobe. Thin-walled enhancement is typical of an abscess. Note the impending intraventricular rupture .](ed2bf437-1286-4b66-9979-fc68e1400ee5) +**Abscess** +*Axial T1 C+ FS MR shows a ring-enhancing mass in the left frontal lobe. Thin-walled enhancement is typical of an abscess. Note the impending intraventricular rupture .* + +![Axial T1 C+ MR shows bilateral rim- enhancing lesions with a targetoid appearance , characteristic of CNS toxoplasmosis. This was the initial presentation of a patient who suffered from undiagnosed HIV/AIDS.](52b0f1cb-1330-4ff6-95a2-3aa2d6b8236e) +**Toxoplasmosis, Acquired** +*Axial T1 C+ MR shows bilateral rim- enhancing lesions with a targetoid appearance , characteristic of CNS toxoplasmosis. This was the initial presentation of a patient who suffered from undiagnosed HIV/AIDS.* + +![Coronal T1 C+ MR shows multifocal masses with ring enhancement . Nodular enhancement is also frequently seen. Toxoplasmosis often lacks restricted diffusion on MR, unlike most abscesses.](e575c943-d5f9-49f9-8f94-08f45b5f0aa4) +**Toxoplasmosis, Acquired** +*Coronal T1 C+ MR shows multifocal masses with ring enhancement . Nodular enhancement is also frequently seen. Toxoplasmosis often lacks restricted diffusion on MR, unlike most abscesses.* + +![Coronal T1 C+ MR shows a large, mixed solid and cystic heterogeneously enhancing mass involving the right basal ganglia . Up to 10% of CNS germinomas arise within the basal ganglia.](aa807232-7be8-4d3c-a03b-c14c4e9ada9f) +**Germinoma** +*Coronal T1 C+ MR shows a large, mixed solid and cystic heterogeneously enhancing mass involving the right basal ganglia . Up to 10% of CNS germinomas arise within the basal ganglia.* + +![Axial T1 C+ MR shows patchy, multifocal enhancement consistent with subacute infarcts in this patient with lupus vasculitis. Vasculitis is often in the cortical and subcortical WM, although basal ganglia involvement is common. Associated DWI restriction may be seen.](4a81c19b-0b3f-4a7e-9d3f-3721e00f8674) +**Vasculitis** +*Axial T1 C+ MR shows patchy, multifocal enhancement consistent with subacute infarcts in this patient with lupus vasculitis. Vasculitis is often in the cortical and subcortical WM, although basal ganglia involvement is common. Associated DWI restriction may be seen.* + +![Axial T1 C+ MR shows multifocal punctate foci of periventricular enhancement with associated T2 hyperintensity (not shown) without significant mass effect. The pattern of involvement in Lyme disease mimics MS lesions.](06bbddf8-e16d-49c4-b015-d2a05c47ffaf) +**Lyme Disease** +*Axial T1 C+ MR shows multifocal punctate foci of periventricular enhancement with associated T2 hyperintensity (not shown) without significant mass effect. The pattern of involvement in Lyme disease mimics MS lesions.* + +![Axial NECT shows a left periventricular enhancing mass with small cystic areas that are commonly present. Ependymomas more commonly are in or near the 4th ventricle but may be supratentorial (1/3 of cases). Calcifications are seen in 50%.](29ce194a-6225-4704-b8dd-a74ec26f735a) +**Ependymoma** +*Axial NECT shows a left periventricular enhancing mass with small cystic areas that are commonly present. Ependymomas more commonly are in or near the 4th ventricle but may be supratentorial (1/3 of cases). Calcifications are seen in 50%.* + +![Axial NECT shows a periventricular, supratentorial ependymoma containing coarse calcifications . There is marked peritumoral edema. Note the subfalcine shift and obstructed ventricles.](c79b6aa9-22a5-4782-af8a-f94b62ebcc29) +**Ependymoma** +*Axial NECT shows a periventricular, supratentorial ependymoma containing coarse calcifications . There is marked peritumoral edema. Note the subfalcine shift and obstructed ventricles.* + +![Axial T1 C+ MR shows bilateral periventricular linear perivascular enhancement in sarcoidosis. Note additional leptomeningeal enhancement .](e6bd4bde-4060-42b2-974a-d05bf3c89019) +**Neurosarcoid** +*Axial T1 C+ MR shows bilateral periventricular linear perivascular enhancement in sarcoidosis. Note additional leptomeningeal enhancement .* + +![Axial T1 C+ MR shows linear enhancing foci in deep and periventricular WM that parallel the course of the cerebral microvasculature. This form of "carcinomatous encephalitis" is a rare intracranial manifestation of systemic leukemia.](ea07d0f0-efc4-458e-afaa-6ae4fc61ea72) +**Leukemia** +*Axial T1 C+ MR shows linear enhancing foci in deep and periventricular WM that parallel the course of the cerebral microvasculature. This form of "carcinomatous encephalitis" is a rare intracranial manifestation of systemic leukemia.* + +![Sagittal FLAIR MR shows multiple hyperintense lesions in the corpus callosum, typical for Susac syndrome and MS. Enhanced scans typically show leptomeningeal enhancement.](20e9c61e-0280-4fbf-a151-c2bcbc38bcb8) +**Susac Syndrome** +*Sagittal FLAIR MR shows multiple hyperintense lesions in the corpus callosum, typical for Susac syndrome and MS. Enhanced scans typically show leptomeningeal enhancement.* + +![Axial T1 C+ MR shows characteristic near-total lack of WM myelination and striking enhancement of the deep periventricular white matter . These patients usually present with a large head.](20f8df48-30cb-4c40-a8a2-94b8cc8c1d28) +**Alexander Disease** +*Axial T1 C+ MR shows characteristic near-total lack of WM myelination and striking enhancement of the deep periventricular white matter . These patients usually present with a large head.* + +![Axial CECT shows dilated ependymal veins due to venous congestion in an infant with a large vein of Galen malformation.](8dd8f348-1c4c-46fa-9187-3381e43ad096) +**Ependymal/Subependymal Veins (Mimic)** +*Axial CECT shows dilated ependymal veins due to venous congestion in an infant with a large vein of Galen malformation.* + +![Axial T1 C+ MR shows marked enhancement of the deep nuclei in the setting of subacute venous infarction due to deep venous thrombosis. Note subependymal venous congestion .](4bd485f1-6a73-4b41-b317-1510ca8e7e63) +**Ependymal/Subependymal Veins (Mimic)** +*Axial T1 C+ MR shows marked enhancement of the deep nuclei in the setting of subacute venous infarction due to deep venous thrombosis. Note subependymal venous congestion .* + +![Axial T1 C+ MR shows punctate and confluent enhancement in bilateral subcortical and periventricular white matter in PML-IRIS in an AIDS patient following initiation of HAART. There was associated hyperintense T2 FLAIR signal and mild mass effect.](e8938ebb-f3be-4cc0-a793-490f961ba82b) +**Immune Reconstitution Inflammatory Syndrome (IRIS)** +*Axial T1 C+ MR shows punctate and confluent enhancement in bilateral subcortical and periventricular white matter in PML-IRIS in an AIDS patient following initiation of HAART. There was associated hyperintense T2 FLAIR signal and mild mass effect.* + +![Axial T1 C+ MR demonstrates hypointense lesions centered in the bilateral thalami around the 3rd ventricle in a patient with neuromyelitis optica. Note faint peripheral enhancement on the right representing active demyelination. Typical neuromyelitis optica lesions are periventricular/periependymal following the distribution of aquaporin-4.](7c2aad0e-0760-41d9-aef0-585ada50424e) +**Neuromyelitis Optica Spectrum Disorders** +*Axial T1 C+ MR demonstrates hypointense lesions centered in the bilateral thalami around the 3rd ventricle in a patient with neuromyelitis optica. Note faint peripheral enhancement on the right representing active demyelination. Typical neuromyelitis optica lesions are periventricular/periependymal following the distribution of aquaporin-4.* + +![Axial T1 C+ MR demonstrates periventricular enhancement around the 4th ventricle in a patient with neurosarcoid.](516b084a-3a47-43e6-bd9e-90210655baab) +**Neurosarcoid** +*Axial T1 C+ MR demonstrates periventricular enhancement around the 4th ventricle in a patient with neurosarcoid.* + +![Axial T1 C+ MR demonstrates a large periventricular enhancing mass, a glioblastoma, centered in the genu and body of the corpus callosum. The main differential consideration for a mass involving the corpus callosum and crossing the midline is lymphoma.](64b4c649-57ce-4a66-bf09-8a4e692af554) +**Glioblastoma, IDH-Wildtype** +*Axial T1 C+ MR demonstrates a large periventricular enhancing mass, a glioblastoma, centered in the genu and body of the corpus callosum. The main differential consideration for a mass involving the corpus callosum and crossing the midline is lymphoma.* + +![Axial T1 C+ MR demonstrates periventricular and smaller subcortical enhancing lesions in a patient with primary CNS lymphoma. The larger periventricular lesions show solid enhancement, which is the norm in immunocompetent lymphoma patients.](748cf6c4-a9be-44bd-8091-033f89ebafa3) +**Lymphoma, Primary CNS** +*Axial T1 C+ MR demonstrates periventricular and smaller subcortical enhancing lesions in a patient with primary CNS lymphoma. The larger periventricular lesions show solid enhancement, which is the norm in immunocompetent lymphoma patients.* + +![Axial T1 C+ MR shows bilateral multiple periventricular enhancing demyelinating lesions in a patient with ADEM. ADEM typically follows an infection or vaccination and can involve gray matter.](images/app.statdx.com_image_thumbnail_8199b423-692d-444a-a9e2-6c6a0b9e4c54_annotated_true_size_900_quality_90_cf8ead5b_20251018T165200Z.jpg) +**ADEM** +*Axial T1 C+ MR shows bilateral multiple periventricular enhancing demyelinating lesions in a patient with ADEM. ADEM typically follows an infection or vaccination and can involve gray matter.* + +![Axial T1 C+ MR shows extensive bilateral periventricular and subcortical enhancing lesions in a patient with PML-IRIS. Unlike PML, PML-IRIS demonstrates mass effect and enhancement.](997613b8-e42e-4c39-a700-d36abd78ee3a) +**Immune Reconstitution Inflammatory Syndrome (IRIS)** +*Axial T1 C+ MR shows extensive bilateral periventricular and subcortical enhancing lesions in a patient with PML-IRIS. Unlike PML, PML-IRIS demonstrates mass effect and enhancement.* + +![Axial C+ MR demonstrates a rim-enhancing periventricular lesion , which was consistent with a toxoplasmosis abscess. Note local mass effect and surrounding hypointense vasogenic edema . The main differential consideration in an immunocompromised HIV patient would be lymphoma.](9e58cb1b-6c77-4f2a-9ba6-0559b63d1aa4) +**Toxoplasmosis, Acquired** +*Axial C+ MR demonstrates a rim-enhancing periventricular lesion , which was consistent with a toxoplasmosis abscess. Note local mass effect and surrounding hypointense vasogenic edema . The main differential consideration in an immunocompromised HIV patient would be lymphoma.* + +![Axial T1 C+ MR demonstrates punctate stippled enhancement in the pons and adjacent middle cerebellar peduncles . This is the typical perivascular enhancement pattern in CLIPPERS, and it can extend to involve the supratentorial brain.](c088034a-4d78-48e8-8bba-403a1470434a) +**CLIPPERS** +*Axial T1 C+ MR demonstrates punctate stippled enhancement in the pons and adjacent middle cerebellar peduncles . This is the typical perivascular enhancement pattern in CLIPPERS, and it can extend to involve the supratentorial brain.* + +![Axial T1 C+ MR demonstrates bilateral periventricular punctate and patchy linear enhancement in a patient with pathologically confirmed primary CNS vasculitis.](91ac979e-39ff-45b8-8f31-83f8d73f3231) +**Vasculitis** +*Axial T1 C+ MR demonstrates bilateral periventricular punctate and patchy linear enhancement in a patient with pathologically confirmed primary CNS vasculitis.* + +![Axial T1 C+ MR demonstrates multiple bilateral enhancing subcortical and periventricular septic emboli of variable size and appearance in a patient with Escherichia coli septicemia.](53e343e1-4044-4e50-ad42-5f52d1e503ec) +**Septic Emboli** +*Axial T1 C+ MR demonstrates multiple bilateral enhancing subcortical and periventricular septic emboli of variable size and appearance in a patient with Escherichia coli septicemia.* + +![Axial T1 C+ MR demonstrates a typical incomplete ring of enhancement in tumefactive demyelination . Such lesions can present with variable degrees of mass effect. The incomplete ring of enhancement allows differentiation from neoplastic causes.](images/app.statdx.com_image_thumbnail_9cc153d1-8efa-437e-b056-8fb7d5682d4b_annotated_true_size_900_quality_90_0ce8b76a_20251018T165158Z.jpg) +**Tumefactive Demyelination** +*Axial T1 C+ MR demonstrates a typical incomplete ring of enhancement in tumefactive demyelination . Such lesions can present with variable degrees of mass effect. The incomplete ring of enhancement allows differentiation from neoplastic causes.* + +![Axial T1 C+ MR shows several periventricular and juxtacortical enhancing lesions in a patient with relapsing/remitting multiple sclerosis. Note no associated mass effect.](images/app.statdx.com_image_thumbnail_3f3b258d-f5d6-468f-a67c-3bde583caee1_annotated_true_size_900_quality_90_5949d05d_20251018T165158Z.jpg) +**Multiple Sclerosis** +*Axial T1 C+ MR shows several periventricular and juxtacortical enhancing lesions in a patient with relapsing/remitting multiple sclerosis. Note no associated mass effect.* + +![Coronal T1 C+ MR shows numerous foci of enhancement in the subcortical and periventricular WM. Fuzzy enhancing margins are typical for demyelination. ADEM typically follows an infection or vaccination.](9145a408-0c29-4a73-9c3e-fbdcf613b141) +**ADEM** +*Coronal T1 C+ MR shows numerous foci of enhancement in the subcortical and periventricular WM. Fuzzy enhancing margins are typical for demyelination. ADEM typically follows an infection or vaccination.* + diff --git a/out/small-ventricles_2f99bc62-163e-41aa-b190-0da8a4de6d11.md b/out/small-ventricles_2f99bc62-163e-41aa-b190-0da8a4de6d11.md new file mode 100644 index 0000000..36a24a5 --- /dev/null +++ b/out/small-ventricles_2f99bc62-163e-41aa-b190-0da8a4de6d11.md @@ -0,0 +1,227 @@ +--- +title: "Small Ventricles" +docid: "2f99bc62-163e-41aa-b190-0da8a4de6d11" +authors: + - key: "d19354f3-7ff2-495a-ad3f-064122e45602" + value: "Bernadette L. Koch, MD" + - key: "f184750a-90b4-47a7-907b-23b05d70357a" + value: "Chang Yueh Ho, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60" + - + name: "Ventricles, Periventricular Regions" + slug: "ventricles-periventricular-regions" + treeNodeId: "353c434a-a6fc-4ef1-8786-d30a1988a4dc" + - + name: "Generic Imaging Patterns" + slug: "generic-imaging-patterns" + treeNodeId: "969c31a2-ef56-4fc3-9125-05857cf9aac3" + - + name: "Small Ventricles" + slug: "small-ventricles" + treeNodeId: null +category: "Brain" +documentVersionId: "5b4a0ab0-ffd6-4209-a606-7d38fb6ec442" +imageCount: 27 +lastUpdated: "01/26/23" +pageDescription: "Small Ventricles" +pageKeywords: "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Small Ventricles" +pageTitle: "Small Ventricles | STATdx" +enhancedTitle: "Small Ventricles" +type: "DDX" +references: true +breadcrumbs: + - "Brain" + - "Differential Diagnosis" + - "Ventricles, Periventricular Regions" + - "Generic Imaging Patterns" + - "Small Ventricles" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Clinical features usually help define cause +- ## Helpful Clues for Common Diagnoses + + + - **Normal Variant (Young Brain)** + - Small ventricles in children & young adults may be normal + - Look for visualization of sulci at vertex & sylvian fissures to exclude diffuse cerebral edema + - **CSF Shunts & Complications** + - **Slit ventricle syndrome**: Symptoms of ↑ ICP with collapsed shunted ventricles + - Noncompliant ventricles, stiff brain, or overshunting + - Anti-siphon devices (gravitational shunt valves) to prevent overshunting + - **Posttraumatic Brain Swelling** + - Diffuse edema with sulcal & ventricular effacement + - Frequently with associated diffuse axonal injury + - Focal edema from contusions may cause ventricular effacement & herniation + - **Herniation Syndromes, Intracranial** + - Ventricular effacement from herniation due to hematoma & tumors + - Increasing subfalcine herniation → ipsilateral ventricular effacement & contralateral ventricular dilatation + - **Adult Hypoxic-Ischemic Injury** + - Hypoxic-ischemic injury (HII) = global HII, global anoxic injury, & cerebral hypoperfusion injury or cerebral hypoperfusion → diffuse cytotoxic edema + - CT: Diffuse cerebral edema + small ventricles & sulci + - ↓ gray-white differentiation, hypodense basal ganglia, bright cerebellar reversal sign + - MR: Diffuse cortical ↑ T2 signal & ↓ diffusion + - ± pattern of central gray ↓ diffusion + - **Neonatal Hypoxic-Ischemic Injury** + - Hypoxic-ischemic encephalopathy from perinatal hypoxia or hypoperfusion + - Deep or central pattern: Deep gray ± brainstem injury & loss of cortical ribbon on T2WI + - Most likely to cause small ventricles + - Peripheral pattern = injury to watershed zones, less likely to cause diffuse cerebral swelling +- ## Helpful Clues for Less Common Diagnoses + + + - **Hyperglycemia** + - Diabetic ketoacidosis (DKA): Diffuse cerebral edema + - ± osmotic demyelination + - **Cerebral Infection** + - Gray & white matter T2 hyperintensity & edema + - Mild restriction on DWI common in viral encephalitis + - **Intracranial Hypotension** + - "Slumping" midbrain, acquired tonsillar herniation, diffusely enhancing dura, veins/dural sinuses distended + - Interpeduncular cistern lower than dorsum sella + - Best evaluated on sagittal images + - **Idiopathic Intracranial Hypertension** + - Dilated optic nerve sheaths, posterior globe flattening, empty sella, slit-like ventricles rare +- ## Helpful Clues for Rare Diagnoses + + + - **Inborn Errors of Metabolism (Acute Presentation)** + - Acute exacerbation + diffuse edema & brain injury → mass effect on ventricles + +## References + +# Selected References + +1. [Panagopoulos D et al: Shunt over-drainage, slit ventricle syndrome, programmable valves and anti-siphon devices. a narrative review of a multifactorial and intractable problem. J Integr Neurosci. 21(3):84, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35633165%5Bpmid%5D) +1. [Bond KM et al: Spontaneous intracranial hypotension: atypical radiologic ppearances, imaging mimickers, and clinical look-alikes. AJNR Am J Neuroradiol. 41(8):1339-47, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32646948%5Bpmid%5D) +1. [Kranz PG et al: Spontaneous intracranial hypotension: pathogenesis, diagnosis, and treatment. Neuroimaging Clin N Am. 29(4):581-94, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31677732%5Bpmid%5D) +1. [Rekate HL: Shunt-related headaches: the slit ventricle syndromes. Childs Nerv Syst. 24(4):423-30, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18259760%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial NECT shows completely collapsed lateral ventricles with a shunt catheter . Slit ventricle syndrome must have clinical symptoms of ↑ intracranial pressure with slit appearance of the lateral ventricles due to shunt.](0728b805-3262-47ee-8be6-1021849a885b) +**CSF Shunts & Complications** +*Axial NECT shows completely collapsed lateral ventricles with a shunt catheter . Slit ventricle syndrome must have clinical symptoms of ↑ intracranial pressure with slit appearance of the lateral ventricles due to shunt.* + +![Axial CT in a 9-year-old injured by a horse shows bilateral scalp hematomas, effacement of sulci, loss of gray-white matter differentiation, and small lateral ventricles. Also note small foci of hemorrhagic shear-type injury at the gray-white junction in the frontal lobes.](images/app.statdx.com_image_thumbnail_c09bfc46-3236-46b4-a14e-ee747ac8ace8_annotated_true_size_900_quality_90_ae8ac602_20251018T165215Z.jpg) +**Posttraumatic Brain Swelling** +*Axial CT in a 9-year-old injured by a horse shows bilateral scalp hematomas, effacement of sulci, loss of gray-white matter differentiation, and small lateral ventricles. Also note small foci of hemorrhagic shear-type injury at the gray-white junction in the frontal lobes.* + +![Axial NECT shows complete loss of the left middle cerebral artery gray-white distinction with cytotoxic edema and left-to-right subfalcine herniation . Note the compression of the ipsilateral left lateral ventricle and enlargement of the right lateral ventricle from entrapment.](images/app.statdx.com_image_thumbnail_59079b5e-7d2d-4336-a5ad-aa23a5785112_annotated_true_size_900_quality_90_39d39744_20251018T165215Z.jpg) +**Herniation Syndromes, Intracranial** +*Axial NECT shows complete loss of the left middle cerebral artery gray-white distinction with cytotoxic edema and left-to-right subfalcine herniation . Note the compression of the ipsilateral left lateral ventricle and enlargement of the right lateral ventricle from entrapment.* + +![Axial NECT shows diffuse effacement of ventricles and sulci from diffuse cerebral edema. There is focal symmetric hypodensity of the globi pallidi in this patient with a history of cardiogenic shock.](images/app.statdx.com_image_thumbnail_9c410bc5-2a93-4247-924c-e0d8c4cf1015_annotated_true_size_900_quality_90_c898a7ba_20251018T165215Z.jpg) +**Adult Hypoxic-Ischemic Injury** +*Axial NECT shows diffuse effacement of ventricles and sulci from diffuse cerebral edema. There is focal symmetric hypodensity of the globi pallidi in this patient with a history of cardiogenic shock.* + +![Axial PD MR in a 2-day-old with severe deep/central pattern of hypoxic-ischemic injury demonstrates small lateral ventricles and cerebral sulci, as well as symmetric, hyperintense signal in the bilateral globus pallidus, putamen, and thalamus .](images/app.statdx.com_image_thumbnail_93f3b7e9-aa77-4429-9f1f-eebcc59aa0e9_annotated_true_size_900_quality_90_193f00bb_20251018T165216Z.jpg) +**Neonatal Hypoxic-Ischemic Injury** +*Axial PD MR in a 2-day-old with severe deep/central pattern of hypoxic-ischemic injury demonstrates small lateral ventricles and cerebral sulci, as well as symmetric, hyperintense signal in the bilateral globus pallidus, putamen, and thalamus .* + +![Axial NECT in a 12-year-old with altered mental status and diabetic ketoacidosis shows near-complete effacement of the lateral ventricles, perimesencephalic cisterns, and cerebral sulci related to diffuse cerebral edema.](images/app.statdx.com_image_thumbnail_2210a160-876a-42ce-bc87-c3dbbed69388_annotated_true_size_900_quality_90_4abbe33c_20251018T165217Z.jpg) +**Hyperglycemia** +*Axial NECT in a 12-year-old with altered mental status and diabetic ketoacidosis shows near-complete effacement of the lateral ventricles, perimesencephalic cisterns, and cerebral sulci related to diffuse cerebral edema.* + +![Axial FLAIR MR shows diffuse gray matter swelling and hyperintensity with resulting effacement of the cerebral sulci and right lateral ventricle. This patient eventually succumbed to rabies encephalitis.](images/app.statdx.com_image_thumbnail_344f9e7e-6aa2-4ddc-a054-c77a38728a66_annotated_true_size_900_quality_90_7004467a_20251018T165217Z.jpg) +**Cerebral Infection** +*Axial FLAIR MR shows diffuse gray matter swelling and hyperintensity with resulting effacement of the cerebral sulci and right lateral ventricle. This patient eventually succumbed to rabies encephalitis.* + +![Sagittal T1 MR shows downward displacement of the cerebellar tonsils and brainstem. There is effacement of the suprasellar cistern . Note effacement of the interpeduncular cistern , which has moved below the level of the dorsum sella, related to "brainstem sagging."](6e228d55-4868-4c43-95d8-556d5988bb6d) +**Intracranial Hypotension** +*Sagittal T1 MR shows downward displacement of the cerebellar tonsils and brainstem. There is effacement of the suprasellar cistern . Note effacement of the interpeduncular cistern , which has moved below the level of the dorsum sella, related to "brainstem sagging."* + + +### Additional Images + +![Axial NECT shows small ventricles and indeterminate shunt position . Symptomatic ventricular collapse is known as slit-like ventricle syndrome and suggests overshunting.](8cb4ffa1-9769-4a57-8cea-8cb068beeeaa) +**CSF Shunts & Complications** +*Axial NECT shows small ventricles and indeterminate shunt position . Symptomatic ventricular collapse is known as slit-like ventricle syndrome and suggests overshunting.* + +![Axial GRE MR in an 4-year-old pedestrian struck by a motor vehicle shows scalp swelling, small ventricles and sulci, and multifocal areas of hemorrhagic shear type injury .](images/app.statdx.com_image_thumbnail_40bf8fe2-f04d-43dc-bfdc-f5aa59a7acd4_annotated_true_size_900_quality_90_133dfd0c_20251018T165215Z.jpg) +**Posttraumatic Brain Swelling** +*Axial GRE MR in an 4-year-old pedestrian struck by a motor vehicle shows scalp swelling, small ventricles and sulci, and multifocal areas of hemorrhagic shear type injury .* + +![Axial NECT shows hyperdense foci of diffuse axonal injury , which is commonly associated with traumatic cerebral edema. Note sulcal and ventricular effacement . Loss of gray-white differentiation is common.](images/app.statdx.com_image_thumbnail_38e45bd4-1af7-4ecc-9abc-c1746abd57a4_annotated_true_size_900_quality_90_0f71d8b2_20251018T165215Z.jpg) +**Posttraumatic Brain Swelling** +*Axial NECT shows hyperdense foci of diffuse axonal injury , which is commonly associated with traumatic cerebral edema. Note sulcal and ventricular effacement . Loss of gray-white differentiation is common.* + +![Axial GRE MR shows multiple punctate areas of susceptibility in the subcortical frontal white matter but also in the splenium of the corpus callosum from diffuse axonal injury. Less well appreciated on this sequence are small lateral ventricles and cerebral edema.](images/app.statdx.com_image_thumbnail_360ffa4d-494f-496c-bbd8-c5f86b31d309_annotated_true_size_900_quality_90_0f4ae828_20251018T165215Z.jpg) +**Posttraumatic Brain Swelling** +*Axial GRE MR shows multiple punctate areas of susceptibility in the subcortical frontal white matter but also in the splenium of the corpus callosum from diffuse axonal injury. Less well appreciated on this sequence are small lateral ventricles and cerebral edema.* + +![Axial NECT shows low-density subacute infarcts in the cerebellar hemispheres. Basal cisterns are effaced , as is the 4th ventricle in this patient with transtentorial herniation. Herniation syndromes typically result from trauma, ischemia, or mass.](images/app.statdx.com_image_thumbnail_e1b30e49-cf00-4604-b50a-e783eed16b3f_annotated_true_size_900_quality_90_35ec3262_20251018T165215Z.jpg) +**Herniation Syndromes, Intracranial** +*Axial NECT shows low-density subacute infarcts in the cerebellar hemispheres. Basal cisterns are effaced , as is the 4th ventricle in this patient with transtentorial herniation. Herniation syndromes typically result from trauma, ischemia, or mass.* + +![Axial NECT shows small ventricles and right occipital and vermian low density due to infarct. Basal cisterns are effaced. Vermis is herniated upward through the tentorial incisura , displacing midbrain anteriorly and superiorly.](images/app.statdx.com_image_thumbnail_378afa91-9f62-471d-bfab-5a895cb3a502_annotated_true_size_900_quality_90_e93cc46e_20251018T165215Z.jpg) +**Herniation Syndromes, Intracranial** +*Axial NECT shows small ventricles and right occipital and vermian low density due to infarct. Basal cisterns are effaced. Vermis is herniated upward through the tentorial incisura , displacing midbrain anteriorly and superiorly.* + +![Axial NECT shows a typical case of impending brain death with diffuse cerebral edema. Note diffuse sulcal effacement, small ventricles , and decreased gray-white matter differentiation within the cerebral hemispheres.](images/app.statdx.com_image_thumbnail_6f7bdf82-ce21-42e7-b457-9f7ff5483ab4_annotated_true_size_900_quality_90_322edb6c_20251018T165216Z.jpg) +**Adult Hypoxic-Ischemic Injury** +*Axial NECT shows a typical case of impending brain death with diffuse cerebral edema. Note diffuse sulcal effacement, small ventricles , and decreased gray-white matter differentiation within the cerebral hemispheres.* + +![Axial PD FSE MR shows enlarged, bilateral, hyperintense deep nuclei and small ventricles from mass effect in hypoxic-ischemic encephalopathy. Cortical hyperintensity is less prominent than on DWI (not shown) except for more advanced bilateral occipital involvement .](images/app.statdx.com_image_thumbnail_9f391b27-1dc1-4c50-b042-f1697cebd49c_annotated_true_size_900_quality_90_7f7b09b9_20251018T165216Z.jpg) +**Adult Hypoxic-Ischemic Injury** +*Axial PD FSE MR shows enlarged, bilateral, hyperintense deep nuclei and small ventricles from mass effect in hypoxic-ischemic encephalopathy. Cortical hyperintensity is less prominent than on DWI (not shown) except for more advanced bilateral occipital involvement .* + +![Axial FLAIR MR shows near-confluent T2 hyperintensity in the deep white matter and small ventricles related to mild mass effect from encephalitis.](c5657e5a-dbfc-49d1-af9b-909ebc180469) +**Cerebral Infection** +*Axial FLAIR MR shows near-confluent T2 hyperintensity in the deep white matter and small ventricles related to mild mass effect from encephalitis.* + +![Axial CECT shows thick, enhancing frontal leptomeninges with adjacent frontal lobe hypodensity from edema and mass effect on the frontal horns. This was pyogenic meningitis with adjacent cerebritis.](5c5ce154-c80c-41b3-919e-e93471bee87a) +**Cerebral Infection** +*Axial CECT shows thick, enhancing frontal leptomeninges with adjacent frontal lobe hypodensity from edema and mass effect on the frontal horns. This was pyogenic meningitis with adjacent cerebritis.* + +![Axial T1 C+ FS MR shows subtle enhancement of the pial surface of the brain . Note associated ependymitis and choroid plexitis , which may complicate meningitis. Ventricles are small due to diffuse brain edema, although when pial exudates obstruct CSF flow, hydrocephalus is more common.](d0e3444e-a542-4be2-8ff1-366016be79a4) +**Cerebral Infection** +*Axial T1 C+ FS MR shows subtle enhancement of the pial surface of the brain . Note associated ependymitis and choroid plexitis , which may complicate meningitis. Ventricles are small due to diffuse brain edema, although when pial exudates obstruct CSF flow, hydrocephalus is more common.* + +![Coronal T1 C+ MR shows meningitis complicated by local cerebritis, as evidenced in this patient with focal mass effect, small ventricles, and ill-defined enhancement .](a1be6ac0-cc98-4376-a5c3-17f2281a9597) +**Cerebral Infection** +*Coronal T1 C+ MR shows meningitis complicated by local cerebritis, as evidenced in this patient with focal mass effect, small ventricles, and ill-defined enhancement .* + +![Sagittal T1 MR shows an empty sella and small ventricles in a patient with idiopathic intracranial hypertension or "pseudotumor cerebri."](05572786-4d3d-47f0-b423-86d77738848c) +**Idiopathic Intracranial Hypertension** +*Sagittal T1 MR shows an empty sella and small ventricles in a patient with idiopathic intracranial hypertension or "pseudotumor cerebri."* + +![Coronal T1 MR in a young woman with headaches and papilledema shows very small lateral ventricles . Superficial sulci also look somewhat less prominent than normal. Pituitary gland is normal for a young, menstruating female.](71426456-b87a-4901-a5e7-29b8fb07649c) +**Idiopathic Intracranial Hypertension** +*Coronal T1 MR in a young woman with headaches and papilledema shows very small lateral ventricles . Superficial sulci also look somewhat less prominent than normal. Pituitary gland is normal for a young, menstruating female.* + +![Axial T2 MR shows increased fluid in the optic nerve sheaths and mild flattening of the globes at the optic nerve heads . Note the partly empty sella . Findings suggest idiopathic intracranial hypertension that can be confirmed with CSF opening pressures.](77d06d43-62be-4662-bba1-60f6070ef6d0) +**Idiopathic Intracranial Hypertension** +*Axial T2 MR shows increased fluid in the optic nerve sheaths and mild flattening of the globes at the optic nerve heads . Note the partly empty sella . Findings suggest idiopathic intracranial hypertension that can be confirmed with CSF opening pressures.* + +![Sagittal T1 MR shows a "slumping midbrain," where the midbrain is displaced below the dorsum sellae . Ventricles are commonly small . The cerebellar tonsils are typically low-lying or herniated. Diffuse dural enhancement is characteristic. Subdural collections are uncommon (15%).](c9cc0066-c38f-443b-b84b-dd28c83aecca) +**Intracranial Hypotension** +*Sagittal T1 MR shows a "slumping midbrain," where the midbrain is displaced below the dorsum sellae . Ventricles are commonly small . The cerebellar tonsils are typically low-lying or herniated. Diffuse dural enhancement is characteristic. Subdural collections are uncommon (15%).* + +![Axial T1 C+ MR shows symmetric, small ventricles and smooth, diffuse, linear pachymeningeal thickening and enhancement .](b6acf477-3b41-4a08-a6a4-8c299968b6e7) +**Intracranial Hypotension** +*Axial T1 C+ MR shows symmetric, small ventricles and smooth, diffuse, linear pachymeningeal thickening and enhancement .* + +![Axial T2 MR shows diffuse, acute brain swelling in maple syrup urine disease and small ventricles due to subacute edema of deep white matter , thalami , and internal capsules.](f48cacf7-0b37-4f80-8db2-c843bc0ffa63) +**Inborn Errors of Metabolism (Acute Presentation)** +*Axial T2 MR shows diffuse, acute brain swelling in maple syrup urine disease and small ventricles due to subacute edema of deep white matter , thalami , and internal capsules.* + +![Axial T1 C+ MR shows diffuse dural enhancement and leptomeningeal enhancement from venous distention. There are small lateral ventricles. Intracranial hypotension is commonly from CSF leak through a dural defect or from LP.](87f394c3-7c19-4b36-b0cd-74b740fda4a4) +**Intracranial Hypotension** +*Axial T1 C+ MR shows diffuse dural enhancement and leptomeningeal enhancement from venous distention. There are small lateral ventricles. Intracranial hypotension is commonly from CSF leak through a dural defect or from LP.* + diff --git a/out/ventricles-and-cisterns-overview_ad860c4f-fe9a-4469-8eca-a7ccd5cff70f.md b/out/ventricles-and-cisterns-overview_ad860c4f-fe9a-4469-8eca-a7ccd5cff70f.md new file mode 100644 index 0000000..6d2597b --- /dev/null +++ b/out/ventricles-and-cisterns-overview_ad860c4f-fe9a-4469-8eca-a7ccd5cff70f.md @@ -0,0 +1,229 @@ +--- +title: "Ventricles and Cisterns Overview" +docid: "ad860c4f-fe9a-4469-8eca-a7ccd5cff70f" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" + - + name: "Anatomy-Based Diagnoses" + slug: "anatomy-based-diagnoses" + treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" + - + name: "Ventricles and Cisterns" + slug: "ventricles-and-cisterns" + treeNodeId: "33b267f0-908c-4c77-81f8-f6135d1bc592" + - + name: "Ventricles and Cisterns Overview" + slug: "ventricles-and-cisterns-overview" + treeNodeId: null +category: "Brain" +documentVersionId: "a4de8cd1-51b7-439f-b4c8-707218ae4252" +imageCount: 32 +lastUpdated: "08/10/20" +pageDescription: "Ventricles and Cisterns Overview" +pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Ventricles and Cisterns Overview" +pageTitle: "Ventricles and Cisterns Overview | STATdx" +enhancedTitle: "Ventricles and Cisterns Overview" +type: "INTRO" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Anatomy-Based Diagnoses" + - "Ventricles and Cisterns" + - "Ventricles and Cisterns Overview" +--- +# Gross and Imaging Anatomy + +- ## Ventricles and Choroid Plexus +- **Basic embryology**: Early in embryonic development, the forebrain cavity divides into 2 lateral ventricles, which develop as outpouchings from the rostral 3rd ventricle and are connected to it by the interventricular foramen (a.k.a. foramen of Monro). In the coronal plane, these form a central H-shaped "monoventricle." The cerebral aqueduct develops from the midbrain vesicle. The 4th ventricle develops from a cavity within the hindbrain and merges caudally with the central canal of the spinal cord. +- **Anatomic overview**: The brain CSF spaces include both the ventricular system and subarachnoid spaces (SASs). The ventricular system is comprised of 4 interconnected CSF-filled, ependymal-lined cavities that lie deep within the brain. The paired **lateral ventricles** communicate with the 3rd ventricle via the Y-shaped **foramen of Monro**. The **3rd********ventricle** communicates with the 4th ventricle via the **cerebral aqueduct** (of Sylvius). In turn, the **4th********ventricle** communicates with the SAS via its outlet foramina (the midline **foramen of Magendie**and the 2 lateral **foramina of Luschka**). +- **Lateral ventricles**: Each lateral ventricle has a body, atrium, and 3 projections ("horns"). The roof of the **frontal horn**is formed by the corpus callosum genu. It is bordered laterally and inferiorly by the head of the caudate nucleus. The septi pellucidi is a thin, bilayered membrane that extends from the corpus callosum genu anteriorly to the foramen of Monro posteriorly and forms the medial borders of both frontal horns. +- The **body**of the lateral ventricle passes posteriorly under the corpus callosum. Its floor is formed by the dorsal thalamus and its medial wall is bordered by the fornix. Laterally, it curves around the body and tail of the caudate nucleus. +- The **atrium**contains the choroid plexus glomus and is formed by the confluence of the body with the temporal and occipital horns. The **temporal horn**extends anteroinferiorly from the atrium and is bordered on its floor and medial wall by the hippocampus. Its roof is formed by the tail of the caudate nucleus. The **occipital horn**is surrounded entirely by white matter fiber tracts, principally the geniculocalcarine tract and the forceps major of the corpus callosum. +- **Foramen of Monro** is a Y-shaped structure with 2 long arms extending towards each lateral ventricle and a short inferior common stem that connects with the roof of the 3rd ventricle. +- **3rd ventricle**: The 3rd ventricle is a single, slit-like, midline, vertically oriented cavity that lies between the thalami. Its roof is formed by the tela choroidea, a double layer of invaginated pia. The lamina terminalis and anterior commissure lie along the anterior border of the 3rd ventricle. The floor of the 3rd ventricle is formed from front to back by the optic chiasm, the hypothalamus with the tuber cinereum and infundibular stalk, mammillary bodies, and the roof of the midbrain tegmentum. +- The 3rd ventricle has 2 inferiorly located CSF-filled projections: The slightly rounded **optic recess** and the more pointed **infundibular recess**. Two small recesses, the **suprapineal and pineal recesses***,* form the posterior border of the 3rd ventricle. A variably sized interthalamic adhesion (also called the **massa intermedia**) lies between the lateral walls of the 3rd ventricle. The massa intermedia is not a true commissure. +- **Cerebral aqueduct** is an elongated tubular conduit that lies between the midbrain tegmentum and the quadrigeminal plate. It connects the 3rd ventricle with the 4th ventricle. +- **4th ventricle**: The 4th ventricle is a roughly diamond-shaped cavity that lies between the pons anteriorly and the cerebellar vermis posteriorly. Its roof is covered by the anterior (superior) medullary velum above and the inferior medullary velum below. +- The 4th ventricle has 5 distinctly shaped recesses. The **posterior superior recesses** are paired, thin, flat, CSF-filled pouches that cap the cerebellar tonsils. The **lateral recesses**curve anterolaterally from the 4th ventricle, extending under the brachium pontis (major cerebellar peduncle) into the lower cerebellopontine angle cisterns (CPAs). The lateral recesses transmit choroid plexus through the foramina of Luschka into the adjacent SAS. The **fastigium**is a triangular, blind-ending, dorsal midline outpouching that points towards the cerebellar vermis. The 4th ventricle gradually narrows as it courses inferiorly, forming the **obex**. Near the cervicomedullary junction, the obex becomes continuous with the central canal of the spinal cord. +- **Choroid plexus, CSF, and brain interstitial fluid (ISF)**: The choroid plexus is comprised of highly vascular papillary excrescences with a central connective tissue core coated by an ependyma-derived secretory epithelium. The embryonic choroid plexus forms where the infolded tela choroidea contacts the ependymal lining of the ventricles, thus developing along the entire choroidal fissure. +- The largest mass of choroid plexus, the**glomus**, is located in the atrium of the lateral ventricles. The choroid plexus extends anteriorly along the floor of the lateral ventricle, lying between the fornix and thalamus. It extends anteroinferiorly from the glomus into the temporal horn, where it fills the choroidal fissure and lies superomedial to the hippocampus. It also dives through the interventricular foramen (of Monro) and curves posteriorly along the roof of the 3rd ventricle. +- The choroid plexus has 2 major functions: CSF production and maintenance of the blood-CSF barrier. +- CSF is predominantly, but not exclusively, secreted by the choroid plexuses. **Brain ISF**, ependyma, and capillaries all play a potential role in CSF secretion. Drainage of brain ISF is a significant extrachoroidal source of CSF. The choroid plexus epithelium secretes CSF at the rate of ~ 0.4 mL/minute or 500-600 mL/day. In adult humans, there are 280 mL of ISF and 140 mL of CSF, of which 30 mL are in the ventricle, 80 mL in the cerebral SAS, and 30 mL in the spinal SAS. +- CSF plays an essential role in the maintenance of brain ISF homeostasis and regulation of neuronal functioning. +- **Traditional model of CSF****homeostasis**:****CSF flows through the ventricular system and passes through the exit foramina of the 4th ventricle into the SAS. The bulk of CSF resorption is through the arachnoid villi along the superior sagittal sinus. CSF also drains into lymphatic vessels around the cranial cavity and spinal canal. +- **Updated model of CSF and ISF****homeostasi****s**: Brain perivascular spaces and paravascular spaces play a critical role in CSF homeostasis. The perivascular spaces from a key component of the brain's "protolymphatic" or "glymphatic" system. ISF circulation likely occurs through the water-selective aquaporin (AQP) channels of the glymphatic system, a key factor in regulating extracellular space water homeostasis. AQP4 is highly expressed in the atrocytic end-feet. +- ## Cisterns and SASs +- **Overview**: The SASs lie between the pia and arachnoid. The sulci are CSF-filled spaces between the gyral folds. Focal expansions of the SASs form the brain CSF cisterns. These cisterns are found at the base of the brain around the brainstem, tentorial incisura, and foramen magnum. Numerous pial-covered septa cross the SAS from the brain to the arachnoid. All SAS cisterns communicate with each other and the ventricular system, providing natural pathways for disease spread (e.g., meningitis, neoplasms). The brain cisterns are conveniently grouped into supra-, peri-, and infratentorial cisterns. All contain numerous important critical structures, such as vessels and cranial nerves. +- **Supratentorial/peritentorial cisterns**: The **suprasellar cistern** lies between the diaphragma sellae and the hypothalamus. Critical contents include the infundibulum, optic chiasm, and circle of Willis. +- The **interpeduncular cistern**is the posterior continuation of the suprasellar cistern. Lying between the cerebral peduncles, it contains the oculomotor nerves as well as the distal basilar artery and proximal segments of the posterior cerebral arteries (PCAs). Important perforating arteries, the thalamoperforating and thalamogeniculate arteries, arise from the top of the basilar artery and cross the interpeduncular cistern to enter the midbrain. +- The**perimesencephalic****(ambient) cisterns**are thin wings of CSF that extend posterosuperiorly from the suprasellar cistern to the quadrigeminal cistern. They wrap around the midbrain and contain the trochlear nerves, P2 PCA segments, superior cerebellar arteries, and the basal vein of Rosenthal. +- The **quadrigeminal cistern** lies under the corpus callosum splenium, behind the pineal gland and tectal plate. It connects with the ambient cisterns laterally and the superior cerebellar cistern inferiorly. The quadrigeminal cistern contains the pineal gland, trochlear nerves, P3 PCA segments, proximal choroidal arteries, and the vein of Galen. An anterior extension, the **velum interpositum**, lies below the fornix and above the 3rd ventricle. The velum interpositum contains the internal cerebral veins and medial posterior choroidal arteries. +- **Infratentorial cisterns**: The unpaired posterior fossa cisterns that lie in the midline are the prepontine, premedullary, and superior cerebellar cisterns, as well as the cisterna magna. The lateral cisterns are paired and include the cerebellopontine and cerebellomedullary cisterns. +- The **prepontine cistern** lies between the upper clivus and the "belly" of the pons. It contains numerous important structures, including the basilar artery, anterior inferior cerebellar arteries (AICAs), and trigeminal and abducens nerves (CNV and CNVI). +- The **premedullary cistern** is the inferior continuation of the prepontine cistern. It lies between the lower clivus in front and the medulla behind. It extends inferiorly to the foramen magnum and contains the vertebral arteries and branches [e.g., posterior inferior cerebellar arteries (PICAs)] and the hypoglossal nerve (CNXII). +- The **superior cerebellar cistern** lies between the straight sinus above and the vermis below. It contains the superior cerebellar arteries and veins. It connects superiorly through the tentorial incisura with the quadrigeminal cistern and inferiorly with the cisterna magna. The **cisterna magna** lies below the inferior vermis between the medulla and the occiput. It contains the cerebellar tonsils and the tonsillohemispheric branches of the PICA. The cisterna magna merges imperceptibly with the SAS of the upper cervical spinal canal. +- The **CPAs** lie between the pons/cerebellum and the petrous temporal bone. Their most important contents are the trigeminal, facial, and vestibulocochlear nerves (CNV, CNVII, and CNVIII). Other structures found here include the petrosal veins and AICAs. The CPA cisterns are contiguous inferiorly with the cerebellomedullary cisterns. +- The **cerebellomedullary cisterns** extend laterally around the medulla and are continuous with the cisterna magna below and the CPAs above. They contain the vagus, glossopharyngeal, and spinal accessory nerves (CNIX, CNX, and CNXI). A tuft of the choroid plexus exits each foramen of Luschka into the cerebellomedullary cistern. The flocculus of the cerebellum that projects into this cistern can appear very prominent. The flocculus and choroid plexus are normal contents of the cerebellomedullary cisterns and should not be mistaken for pathology. + +# Imaging Recommendations + +- **MR**: Thin-section 3D T2WI or FIESTA/CISS best detail CSF within the ventricular system, SASs, and basal cisterns, and exquisitely delineates their contents. FLAIR MR is especially useful for evaluating potential abnormalities in the SASs. Spin dephasing with pulsatile CSF flow is common and can mimic pathology, especially in the basal cisterns and around the interventricular foramen. Incomplete CSF suppression with "bright" CSF can mimic pathologic SASs. + +# Differential Diagnosis Approach + +- ## Ventricles and Choroid Plexus +- **Overview**: Approximately 10% of intracranial neoplasms involve the cerebral ventricles, either primarily or by extension. An anatomy-based approach is most effective as there is a distinct predilection for certain lesions to occur in one ventricle or cistern and not others. Age is also a helpful consideration. Specific imaging findings, such as signal intensity, enhancement, and the presence or absence of calcification are relatively less important than location and age. +- **Normal variants**: Asymmetry of the lateral ventricles is a common normal variant, as is flow-related CSF pulsation artifact. A cavum septi pellucidi (CSP) is a common normal variant, seen as a CSF cleft between the 2 leaves of the septum pellucidum. An elongated, finger-like posterior continuation of the CSP between the fornices, a cavum vergae (CV), may be associated with a CSP. +- **Lateral ventricle mass**: Choroid plexus cysts (xanthogranulomas) are a common, generally age-related, degenerative finding with no clinical significance. They are nonneoplastic noninflammatory cysts, usually bilateral with rim calcification, may be hyperintense on FLAIR MR, and 60-80% appear bright on DWI. A strongly enhancing choroid plexus mass in a child is most likely a choroid plexus papilloma. With the exception of the 4th ventricle, a choroid plexus mass in an adult is usually meningioma or metastasis, not a choroid plexus papilloma. +- Some lateral ventricle lesions display a distinct predilection for specific sublocations within the lateral ventricles. An innocent-appearing frontal horn mass in a middle-aged or older adult is most often a subependymoma. A "bubbly" mass in the body of the lateral ventricle is usually a central neurocytoma. Neurocysticercosis cysts can occur in all ages and in virtually every CSF space. +- **Foramen of Monro mass**: The most common "abnormality" here is a pseudolesion caused by CSF pulsation artifact. A colloid cyst is the only relatively common pathology here. It is rare in children and is typically a lesion of adults. Flow artifact can mimic a colloid cyst, but mass effect is absent. In a child with an enhancing mass in the interventricular foramen, tuberous sclerosis with subependymal nodule &/or giant cell astrocytoma should be a consideration. Masses such as an ependymoma, papilloma, and metastasis are rare. +- **3rd ventricle mass**: Again, the most common "lesion" in this location is either CSF flow artifact or a normal structure (the massa intermedia). A colloid cyst is the only common lesion that occurs in the 3rd ventricle; 99% are wedged into the foramen of Monro. Extreme vertebrobasilar dolichoectasia can indent the 3rd ventricle, sometimes projecting upward as high as the interventricular foramen, and should not be mistaken for colloid cyst. +- Primary neoplasms in children are uncommon here but include choroid plexus papilloma, germinoma, craniopharyngioma, and a sessile-type tuber cinereum hamartoma. Primary neoplasms of the 3rd ventricle in adults are also uncommon, though an intraventricular macroadenoma and chordoid glioma are examples. +- **Cerebral aqueduct**: Other than aqueductal stenosis, intrinsic lesions of the cerebral aqueduct are rare. Most are related to masses in adjacent structures (e.g., tectal plate glioma). +- **4th ventricle mass**: Pediatric masses are the most common intrinsic abnormalities of the 4th ventricle. Medulloblastoma, ependymoma, and astrocytoma predominate. Atypical teratoid/rhabdoid tumor (AT/RT) is a less common neoplasm that may occur here. It usually occurs in children under the age of 3 and can mimic medulloblastoma. +- Metastases to the choroid or ependyma are probably the most common 4th ventricle neoplasm of adults. Choroid plexus papilloma does occur here as well as in the CPA cistern. Subependymoma is a lesion of middle-aged adults that is found in the inferior 4th ventricle, lying behind the pontomedullary junction. A newly described rare neoplasm, rosette-forming glioneuronal tumor, is a midline mass of the 4th ventricle. It has no particular distinguishing imaging features and, although it may appear aggressive, it is a benign (WHO grade 1) lesion. Hemangioblastomas are intraaxial masses but may project into the 4th ventricle. Epidermoid cysts and neurocysticercosis cysts can be found in all ages. +- ## SASs and Cisterns +- **Overview**: The SASs are a common site of pathology that varies from benign congenital lesions (such as arachnoid cyst) to infection (meningitis) and neoplastic involvement ("carcinomatous meningitis"). Anatomic location is key to the differential diagnosis, as imaging findings, such as enhancement and hyperintensity on FLAIR MR, are often nonspecific. +- **Normal variants**: CSF flow-related artifacts are common, especially in the basal cisterns on FLAIR MR. Mega cisterna magna may be considered a normal variant, as is a cavum velum interpositum (CVI). A CVI is a thin, triangular-shaped CSF space between the lateral ventricles that lies below the fornices and above the 3rd ventricle. Occasionally, a CVI may become quite large. +- **Suprasellar cistern mass**: Common masses in adults are upward extensions of macroadenoma, meningioma, and aneurysm. The 2 most common suprasellar masses in children are astrocytoma of the optic chiasm/hypothalamus and craniopharyngioma. +- **Cerebellopontine angle mass**: In adults, vestibular schwannoma accounts for almost 90% of all CPA-internal auditory canal (IAC) masses. A meningioma, epidermoid cyst, aneurysm, and arachnoid cyst **together** represent ~ 8% of lesions in this location. All other less common entities, such as lipoma, schwannomas of other cranial nerves, metastases, neurenteric cysts, etc., account for ~ 2%. +- In the absence of neurofibromatosis type 2, vestibular schwannomas are very rare in children. CPA epidermoid and arachnoid cysts may occur in children. Extension of ependymoma laterally through the foramina of Luschka may involve the CPA. +- Cystic-appearing CPA masses comprise their own special differential diagnosis. While vestibular schwannoma with intramural cysts can occur, it is less common than epidermoid and arachnoid cysts. Neurocysticercosis may occasionally involve the CPA. Large endolymphatic sac anomaly (IP-2) shows a CSF-like mass within the posterior wall of the temporal bone. Hemangioblastoma and neurenteric cysts are other less common cystic masses that occur in the CPA. +- **Cisterna magna mass**: Tonsillar herniation, whether congenital (Chiari 1) or secondary to posterior fossa mass effect or intracranial hypotension, is the most common "mass" in this location. Nonneoplastic cysts (arachnoid, epidermoid, dermoid, neurenteric) may also occur here. +- Neoplasms in and around the cisterna magna, such as meningioma and metastasis, are typically anterior to the medulla. Subependymoma of the 4th ventricle originates in the obex and lies behind the medulla. +- **FLAIR MR hyperintensity**: Hyperintense sulci and SASs are seen with MR artifacts as well as a variety of lesions. Pathologic FLAIR MR hyperintensity is typically related to blood (e.g., subarachnoid hemorrhage), protein (meningitis), or cells (pia-SAS metastases). Less commonly, gadolinium-based contrast agents in patients with blood-brain barrier leakage or renal failure can cause FLAIR MR hyperintensity. +- Rare causes of FLAIR MR hyperintensity include a ruptured dermoid cyst, moyamoya (ivy sign), and acute cerebral ischemia. Contrast enhancement helps distinguish meningitis and metastases from subarachnoid hemorrhage and CSF artifacts. + + 91dc714e-682b-4d02-a8c5-c6decb8bc1ba + +## References + +# Selected References + +1. [Adigun OO et al: Anatomy, head and neck, cerebrospinal fluid, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=29083815%5Bpmid%5D) +1. [Altafulla J et al: The basal subarachnoid cisterns: surgical and anatomical considerations. World Neurosurg. 129:190-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31136838%5Bpmid%5D) +1. [Korzh V: Development of brain ventricular system. Cell Mol Life Sci. 75(3):375-83, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=28780589%5Bpmid%5D) +1. [Tumani H et al: The cerebrospinal fluid and barriers - anatomic and physiologic considerations. Handb Clin Neurol. 146:21-32, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29110772%5Bpmid%5D) +1. [Sakka L et al: Anatomy and physiology of cerebrospinal fluid. Eur Ann Otorhinolaryngol Head Neck Dis. 128(6):309-16, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=22100360%5Bpmid%5D) +1. [Lowery LA et al: Totally tubular: the mystery behind function and origin of the brain ventricular system. Bioessays. 31(4):446-58, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19274662%5Bpmid%5D) +1. [Barshes N et al: Anatomy and physiology of the leptomeninges and CSF space. Cancer Treat Res. 125:1-16, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16211880%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Schematic 3D representation of the ventricular system, viewed in the sagittal plane, demonstrates the normal appearance and communicating pathways of the cerebral ventricles.](images/app.statdx.com_image_thumbnail_18da13b4-cc47-4475-9b99-987ece9e9550_annotated_false_size_900_quality_90_aaad2f37_20251018T164921Z.jpg) +*Schematic 3D representation of the ventricular system, viewed in the sagittal plane, demonstrates the normal appearance and communicating pathways of the cerebral ventricles.* + +![Schematic 3D representation of the ventricular system, viewed in the sagittal plane, demonstrates the normal appearance and communicating pathways of the cerebral ventricles.](images/app.statdx.com_image_thumbnail_18da13b4-cc47-4475-9b99-987ece9e9550_size_174_quality_85_a814c085_20251018T165117Z.jpg) +*Schematic 3D representation of the ventricular system, viewed in the sagittal plane, demonstrates the normal appearance and communicating pathways of the cerebral ventricles.* + +![Sagittal midline graphic through the interhemispheric fissure depicts the subarachnoid spaces (SASs) with CSF (blue) between the arachnoid (purple) and pia (orange). The central sulcus separates the frontal lobe (anterior) from the parietal lobe (posterior). The pia mater is closely applied to the brain surface, whereas the arachnoid is adherent to the dura. The ventricles communicate with the cisterns and SAS via the foramina of Luschka and Magendie. The cisterns normally communicate freely with each other.](images/app.statdx.com_image_thumbnail_d0fd3b0a-6e5b-4d8d-8ff9-018a2701429a_annotated_false_size_900_quality_90_2677fbfc_20251018T164921Z.jpg) +*Sagittal midline graphic through the interhemispheric fissure depicts the subarachnoid spaces (SASs) with CSF (blue) between the arachnoid (purple) and pia (orange). The central sulcus separates the frontal lobe (anterior) from the parietal lobe (posterior). The pia mater is closely applied to the brain surface, whereas the arachnoid is adherent to the dura. The ventricles communicate with the cisterns and SAS via the foramina of Luschka and Magendie. The cisterns normally communicate freely with each other.* + +![Axial T2 MR demonstrates normal anatomy at the level of the lateral ventricles. The frontal horns of the lateral ventricle are separated by a tiny septi pellucidi . Note the foramen of Monro connecting the lateral ventricles to the 3rd ventricle .](images/app.statdx.com_image_thumbnail_4288ccd4-2baa-4fad-a804-777de6cf9758_annotated_true_size_900_quality_90_6b61e4ac_20251018T164921Z.jpg) +*Axial T2 MR demonstrates normal anatomy at the level of the lateral ventricles. The frontal horns of the lateral ventricle are separated by a tiny septi pellucidi . Note the foramen of Monro connecting the lateral ventricles to the 3rd ventricle .* + +![Axial T2 MR at the level of the cerebral aqueduct demonstrates the infundibular recess of the 3rd ventricle , mammillary bodies , interpeduncular cistern , and the quadrigeminal cisterns .](images/app.statdx.com_image_thumbnail_9ffc389c-d960-42d5-8503-304234193f7a_annotated_true_size_900_quality_90_0a8a0203_20251018T164921Z.jpg) +*Axial T2 MR at the level of the cerebral aqueduct demonstrates the infundibular recess of the 3rd ventricle , mammillary bodies , interpeduncular cistern , and the quadrigeminal cisterns .* + +![Axial T2 MR at the level of the 4th ventricular outlet shows the foramen of Magendie and foramina of Luschka .](images/app.statdx.com_image_thumbnail_240fe702-9e39-4bb4-8b55-39c0a4dd83a7_annotated_true_size_900_quality_90_924b302b_20251018T164921Z.jpg) +*Axial T2 MR at the level of the 4th ventricular outlet shows the foramen of Magendie and foramina of Luschka .* + +![Sagittal FEISTA MR demonstrates the lateral ventricle , chiasmatic and infundibular recesses of the 3rd ventricle, the cerebral aqueduct , and the fastigium of the 4th ventricle. The floor of the 3rd ventricle in formed from front to back by the optic chiasm, hypothalamus, mammillary bodies, and roof of the midbrain tegmentum.](images/app.statdx.com_image_thumbnail_fc362fbe-b32d-422b-b185-247deeb14c6e_annotated_true_size_900_quality_90_d4614ba6_20251018T164921Z.jpg) +*Sagittal FEISTA MR demonstrates the lateral ventricle , chiasmatic and infundibular recesses of the 3rd ventricle, the cerebral aqueduct , and the fastigium of the 4th ventricle. The floor of the 3rd ventricle in formed from front to back by the optic chiasm, hypothalamus, mammillary bodies, and roof of the midbrain tegmentum.* + +![Axial NECT (L) and axial T2 MR (R) shows normal asymmetry of the lateral ventricles with the right being larger than the left. The septi pellucidi are slightly bowed and displaced across the midline. When there is lateral ventricular asymmetry, it is important to scrutinize the region of foramen of Monro to exclude any obstructing pathology.](images/app.statdx.com_image_thumbnail_4941a673-d28c-47c0-bea9-92d4cd99f431_annotated_true_size_900_quality_90_935169e1_20251018T164921Z.jpg) +*Axial NECT (L) and axial T2 MR (R) shows normal asymmetry of the lateral ventricles with the right being larger than the left. The septi pellucidi are slightly bowed and displaced across the midline. When there is lateral ventricular asymmetry, it is important to scrutinize the region of foramen of Monro to exclude any obstructing pathology.* + +![Axial FLAIR MR in a patient with hydrocephalus demonstrates focal hyperintensity in the 3rd ventricle due to a pseudomasses caused by pulsatile CSF.](images/app.statdx.com_image_thumbnail_5710a471-d62a-4c03-af96-60870e2354c1_annotated_true_size_900_quality_90_f4c0b8b2_20251018T164921Z.jpg) +*Axial FLAIR MR in a patient with hydrocephalus demonstrates focal hyperintensity in the 3rd ventricle due to a pseudomasses caused by pulsatile CSF.* + +![Axial T2 MR shows a large ventricular mass in the frontal horn and anterior body of the right lateral ventricle. There is dilatation of the posterior body of the right lateral ventricle and displacement of the septi pellucidi to the left. On histopathology, this was a central neurocytoma.](images/app.statdx.com_image_thumbnail_524494cb-37a1-444b-83cb-e4ffd7d6d101_annotated_true_size_900_quality_90_930f6a47_20251018T164921Z.jpg) +*Axial T2 MR shows a large ventricular mass in the frontal horn and anterior body of the right lateral ventricle. There is dilatation of the posterior body of the right lateral ventricle and displacement of the septi pellucidi to the left. On histopathology, this was a central neurocytoma.* + +![Axial FLAIR MR demonstrates an intraventricular neurocysticercosis in the posterior 3rd ventricle with dilatation of the anterior 1/3 and lateral ventricles. Note the mild periventricular interstitial edema .](images/app.statdx.com_image_thumbnail_657cbe5a-415a-418b-a61b-7a0b96284a65_annotated_true_size_900_quality_90_801c6a60_20251018T164926Z.jpg) +*Axial FLAIR MR demonstrates an intraventricular neurocysticercosis in the posterior 3rd ventricle with dilatation of the anterior 1/3 and lateral ventricles. Note the mild periventricular interstitial edema .* + +![Axial DWI (L) and axial FLAIR MR (R) shows characteristic large choroid plexus cysts in the atria of both lateral ventricles. Choroid plexus cysts, often called choroid plexus xanthogranuloma, are nonneoplastic noninflammatory cysts and are often bright on DWI.](images/app.statdx.com_image_thumbnail_63bee34f-4b15-4df5-9184-f331532989da_annotated_true_size_900_quality_90_7af97812_20251018T164926Z.jpg) +*Axial DWI (L) and axial FLAIR MR (R) shows characteristic large choroid plexus cysts in the atria of both lateral ventricles. Choroid plexus cysts, often called choroid plexus xanthogranuloma, are nonneoplastic noninflammatory cysts and are often bright on DWI.* + +![Sagittal T1 C+ MR in a 8-year-old child with ataxia demonstrates a large, heterogeneously enhancing mass centered in the 4th ventricle. Pathology revealed a classic medulloblastoma, Wnt-activated.](images/app.statdx.com_image_thumbnail_a24ea68a-9b86-47df-91c0-fe5cb9736415_annotated_true_size_900_quality_90_1f86202e_20251018T164928Z.jpg) +*Sagittal T1 C+ MR in a 8-year-old child with ataxia demonstrates a large, heterogeneously enhancing mass centered in the 4th ventricle. Pathology revealed a classic medulloblastoma, Wnt-activated.* + +![Axial FLAIR MR in a patient with acute cortical subarachnoid hemorrhage due to cortical venous thrombosis shows high signal in the left frontal sulci.](images/app.statdx.com_image_thumbnail_bbab6561-190c-4c36-83c8-32eda9785a39_annotated_true_size_900_quality_90_2e85ab8a_20251018T164928Z.jpg) +*Axial FLAIR MR in a patient with acute cortical subarachnoid hemorrhage due to cortical venous thrombosis shows high signal in the left frontal sulci.* + +![Axial FLAIR MR in a patient with chronic renal disease who received IV gadolinium 48 hours prior shows marked FLAIR hyperintensity in the cortical sulci. Sulcal hyperintensity on FLAIR can be caused by pia-subarachnoid metastases, blood, protein (meningitis), high oxygen content, and retained contrast (renal failure, as in this case).](images/app.statdx.com_image_thumbnail_90af95ee-2e00-4f2a-a832-2fec510f8150_annotated_true_size_900_quality_90_950a63d1_20251018T164929Z.jpg) +*Axial FLAIR MR in a patient with chronic renal disease who received IV gadolinium 48 hours prior shows marked FLAIR hyperintensity in the cortical sulci. Sulcal hyperintensity on FLAIR can be caused by pia-subarachnoid metastases, blood, protein (meningitis), high oxygen content, and retained contrast (renal failure, as in this case).* + + +### Additional Images + +![Axial T1 C+ MR in an 18-month-old child with severe hydrocephalus shows a choroid plexus papilloma (CPP). The intensely enhancing frond-like projections and location in the atrium of the left lateral ventricle are both classic findings.](images/app.statdx.com_image_thumbnail_d844bf7c-09b7-49ce-8ee8-e7cf2890dfc4_annotated_true_size_900_quality_90_f57411b5_20251018T164929Z.jpg) +*Axial T1 C+ MR in an 18-month-old child with severe hydrocephalus shows a choroid plexus papilloma (CPP). The intensely enhancing frond-like projections and location in the atrium of the left lateral ventricle are both classic findings.* + +![Axial T1 C+ MR in a middle-aged woman shows a smoothly lobulated, intensely enhancing choroid plexus mass . CPP in adults is rare, except for the 4th ventricle. Meningioma was found at surgery.](images/app.statdx.com_image_thumbnail_fa650ed7-2e34-45bb-ab32-d3e7cf96dd81_annotated_true_size_900_quality_90_dbd4ffd2_20251018T164929Z.jpg) +*Axial T1 C+ MR in a middle-aged woman shows a smoothly lobulated, intensely enhancing choroid plexus mass . CPP in adults is rare, except for the 4th ventricle. Meningioma was found at surgery.* + +![Axial T1 C+ FS MR in a 72-year-old man with declining mental state shows a nonenhancing mass in the frontal horn of the left lateral ventricle. This is an incidental finding, most likely a subependymoma.](images/app.statdx.com_image_thumbnail_8f87abeb-662f-4047-9f74-66034afca89f_annotated_true_size_900_quality_90_714f2c9a_20251018T164929Z.jpg) +*Axial T1 C+ FS MR in a 72-year-old man with declining mental state shows a nonenhancing mass in the frontal horn of the left lateral ventricle. This is an incidental finding, most likely a subependymoma.* + +![Axial T1 C+ MR in a 33 year old with headaches shows an inhomogeneously enhancing bubbly lesion in the body of the left lateral ventricle. The appearance and location distinguish a central neurocytoma from subependymoma and other possible lateral ventricular masses, such as a meningioma.](images/app.statdx.com_image_thumbnail_5b8a7998-bb55-4ff4-a940-66e19336b5df_annotated_true_size_900_quality_90_47653a8b_20251018T164929Z.jpg) +*Axial T1 C+ MR in a 33 year old with headaches shows an inhomogeneously enhancing bubbly lesion in the body of the left lateral ventricle. The appearance and location distinguish a central neurocytoma from subependymoma and other possible lateral ventricular masses, such as a meningioma.* + +![Axial FLAIR MR in a 3 year old with seizures shows a hyperintense mass at the interventricular foramen. Note the flame-shaped subcortical hyperintensities . This is tuberous sclerosis with a subependymal giant cell astrocytoma.](images/app.statdx.com_image_thumbnail_d3712d46-4bd1-4dfa-acf0-d8c5a5f5e99a_annotated_true_size_900_quality_90_e6d92377_20251018T164929Z.jpg) +*Axial FLAIR MR in a 3 year old with seizures shows a hyperintense mass at the interventricular foramen. Note the flame-shaped subcortical hyperintensities . This is tuberous sclerosis with a subependymal giant cell astrocytoma.* + +![Axial FLAIR MR in a 65 year old with a "thunderclap" headache shows a foramen of Monro mass that is a colloid cyst. Other than CSF flow artifact, colloid cysts are the most common lesion found in this location. They are common in adults but relatively rare in children.](images/app.statdx.com_image_thumbnail_cf06d949-a6c8-48ed-a03d-fcb179f922ae_annotated_true_size_900_quality_90_70746469_20251018T164929Z.jpg) +*Axial FLAIR MR in a 65 year old with a "thunderclap" headache shows a foramen of Monro mass that is a colloid cyst. Other than CSF flow artifact, colloid cysts are the most common lesion found in this location. They are common in adults but relatively rare in children.* + +![Coronal T1WI MR shows prominent pseudomasses of the 3rd and lateral ventricles caused by pulsatile CSF in and around the interventricular foramen (of Monro). Note the propagation of phase artifact across the image.](images/app.statdx.com_image_thumbnail_d7cf19d1-466f-4c2a-9466-cfab49680886_annotated_true_size_900_quality_90_298e5a3c_20251018T164929Z.jpg) +*Coronal T1WI MR shows prominent pseudomasses of the 3rd and lateral ventricles caused by pulsatile CSF in and around the interventricular foramen (of Monro). Note the propagation of phase artifact across the image.* + +![Axial T1WI C+ MR shows large lateral 3rd ventricles with "blurred" margins from transependymal CSF flow. A cysticercus cyst with scolex causes obstructive hydrocephalus. Intrinsic 3rd ventricle masses are less common than lateral or 4th ventricular lesions.](images/app.statdx.com_image_thumbnail_7164036e-4620-4279-92d5-d9aac20687cb_annotated_true_size_900_quality_90_33feec68_20251018T164930Z.jpg) +*Axial T1WI C+ MR shows large lateral 3rd ventricles with "blurred" margins from transependymal CSF flow. A cysticercus cyst with scolex causes obstructive hydrocephalus. Intrinsic 3rd ventricle masses are less common than lateral or 4th ventricular lesions.* + +![Sagittal T1WI C+ MR in a 2 year old with ataxia, nausea, and vomiting shows a lobulated enhancing mass in the 4th ventricle . Fourth ventricle masses in children are usually primitive neuroectodermal tumor or ependymoma, less often atypical teratoid-rhabdoid tumor which was found at surgery.](images/app.statdx.com_image_thumbnail_4f5f99a5-2e74-441d-aebf-641717636700_annotated_true_size_900_quality_90_7b85c1c4_20251018T164930Z.jpg) +*Sagittal T1WI C+ MR in a 2 year old with ataxia, nausea, and vomiting shows a lobulated enhancing mass in the 4th ventricle . Fourth ventricle masses in children are usually primitive neuroectodermal tumor or ependymoma, less often atypical teratoid-rhabdoid tumor which was found at surgery.* + +![Sagittal T1WI C+ MR in a 52-year-old woman with episodic headaches, nausea, and vomiting shows an intensely enhancing 4th ventricle mass. This proved to be choroid plexus papilloma.](images/app.statdx.com_image_thumbnail_3be18417-401b-4732-9fcb-68d0389a6a9e_annotated_true_size_900_quality_90_4d8f9d0b_20251018T164930Z.jpg) +*Sagittal T1WI C+ MR in a 52-year-old woman with episodic headaches, nausea, and vomiting shows an intensely enhancing 4th ventricle mass. This proved to be choroid plexus papilloma.* + +![Axial FLAIR MR shows multifocal sulcal hyperintensities caused by aneurysmal subarachnoid hemorrhage.](images/app.statdx.com_image_thumbnail_93e84051-219a-4618-950d-097de4af3dfc_annotated_true_size_900_quality_90_d3383237_20251018T164931Z.jpg) +*Axial FLAIR MR shows multifocal sulcal hyperintensities caused by aneurysmal subarachnoid hemorrhage.* + +![Axial FLAIR MR shows artifactual hyperintensity in the occipital sulci secondary to incomplete CSF suppression. A repeat scan (not shown) was normal. Sulcal hyperintensity on FLAIR is nonspecific and can be caused by pia-subarachnoid metastases, blood, protein (meningitis), high oxygen content, retained contrast (renal failure), and artifact (as in this case).](images/app.statdx.com_image_thumbnail_29e5ed04-7404-4c85-8879-6934b62d9243_annotated_true_size_900_quality_90_73fe8c83_20251018T164931Z.jpg) +*Axial FLAIR MR shows artifactual hyperintensity in the occipital sulci secondary to incomplete CSF suppression. A repeat scan (not shown) was normal. Sulcal hyperintensity on FLAIR is nonspecific and can be caused by pia-subarachnoid metastases, blood, protein (meningitis), high oxygen content, retained contrast (renal failure), and artifact (as in this case).* + +![Sagittal T2 SPACE MR shows the normal flow void due to CSF flow at the cerebral aqueduct and foramen of Magendie . Note the chiasmatic and infundibular recesses of the 3rd ventricle and fastigium of the 4th ventricle.](images/app.statdx.com_image_thumbnail_fa347901-c1cf-4049-a7fb-9cd96a14a1c0_annotated_true_size_900_quality_90_e67cc834_20251018T164931Z.jpg) +*Sagittal T2 SPACE MR shows the normal flow void due to CSF flow at the cerebral aqueduct and foramen of Magendie . Note the chiasmatic and infundibular recesses of the 3rd ventricle and fastigium of the 4th ventricle.* + +![Axial T2 MR shows normal asymmetry of the lateral ventricles with the right being larger than the left. The septi pellucidi are slightly bowed and displaced across the midline. When there is lateral ventricular asymmetry, it is important to scrutinize the region of foramen of Monro to exclude any obstructing pathology.](images/app.statdx.com_image_thumbnail_5bd78d24-30b7-4af5-bd73-be49ef2afcff_annotated_true_size_900_quality_90_fc43cd81_20251018T164931Z.jpg) +*Axial T2 MR shows normal asymmetry of the lateral ventricles with the right being larger than the left. The septi pellucidi are slightly bowed and displaced across the midline. When there is lateral ventricular asymmetry, it is important to scrutinize the region of foramen of Monro to exclude any obstructing pathology.* + +![Axial FLAIR MR in a patient with hydrocephalus demonstrates a prominent pseudomasses of the 3rd ventricle caused by pulsatile CSF.](images/app.statdx.com_image_thumbnail_e6176e42-b6e2-492b-a896-ce2fc19efdd4_annotated_true_size_900_quality_90_c16dc82b_20251018T164931Z.jpg) +*Axial FLAIR MR in a patient with hydrocephalus demonstrates a prominent pseudomasses of the 3rd ventricle caused by pulsatile CSF.* + +![Sagittal T1 C+ MR shows a large homogenously enhancing 4th ventricular mass , which on pathology was a meningioma. There is dilatation of the ventricular system proximal to the mass.](images/app.statdx.com_image_thumbnail_530cce92-dd05-4a8e-af12-e451a270b370_annotated_true_size_900_quality_90_b1421f8a_20251018T164931Z.jpg) +*Sagittal T1 C+ MR shows a large homogenously enhancing 4th ventricular mass , which on pathology was a meningioma. There is dilatation of the ventricular system proximal to the mass.* + +![Axial DWI MR shows characteristic large choroid plexus cysts in the atria of both lateral ventricles within the choroid plexus glomi. Choroid plexus cysts, often called choroid plexus xanthogranuloma, are nonneoplastic noninflammatory cysts. Between 60-80% appear quite bright on DWI, as in this case.](images/app.statdx.com_image_thumbnail_82e0f8d0-567d-469d-b620-d1d2c95866bd_annotated_true_size_900_quality_90_852aa16e_20251018T164931Z.jpg) +*Axial DWI MR shows characteristic large choroid plexus cysts in the atria of both lateral ventricles within the choroid plexus glomi. Choroid plexus cysts, often called choroid plexus xanthogranuloma, are nonneoplastic noninflammatory cysts. Between 60-80% appear quite bright on DWI, as in this case.* + +![Axial FLAIR MR in a patient with acute subarachnoid hemorrhage due to aneurysm rupture shows high signal in the left sylvian fissure and posterior cortical sulci .](images/app.statdx.com_image_thumbnail_3957aec8-23c1-4e4f-8bd2-8527c8bc9a6a_annotated_true_size_900_quality_90_9747d5ed_20251018T164931Z.jpg) +*Axial FLAIR MR in a patient with acute subarachnoid hemorrhage due to aneurysm rupture shows high signal in the left sylvian fissure and posterior cortical sulci .* + diff --git a/out/ventriculomegaly_f40bd6eb-e7e5-498a-8bde-ad6bcd546f21.md b/out/ventriculomegaly_f40bd6eb-e7e5-498a-8bde-ad6bcd546f21.md new file mode 100644 index 0000000..38b6c69 --- /dev/null +++ b/out/ventriculomegaly_f40bd6eb-e7e5-498a-8bde-ad6bcd546f21.md @@ -0,0 +1,317 @@ +--- +title: "Ventriculomegaly" +docid: "f40bd6eb-e7e5-498a-8bde-ad6bcd546f21" +authors: + - key: "47381de4-c9fd-4999-8dd0-1808cd72db6b" + value: "Luke L. Linscott, MD" +breadcrumbs: + - + name: "Brain" + slug: "brain" + treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "a7fdd139-664e-4bb8-8d18-400e4733ff60" + - + name: "Ventricles, Periventricular Regions" + slug: "ventricles-periventricular-regions" + treeNodeId: "353c434a-a6fc-4ef1-8786-d30a1988a4dc" + - + name: "Generic Imaging Patterns" + slug: "generic-imaging-patterns" + treeNodeId: "969c31a2-ef56-4fc3-9125-05857cf9aac3" + - + name: "Ventriculomegaly" + slug: "ventriculomegaly" + treeNodeId: null +category: "Brain" +documentVersionId: "69dd2446-06ad-4f37-bba3-f59d4c5d40b8" +imageCount: 26 +lastUpdated: "01/23/23" +pageDescription: "Ventriculomegaly" +pageKeywords: "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Ventriculomegaly" +pageTitle: "Ventriculomegaly | STATdx" +enhancedTitle: "Ventriculomegaly" +type: "DDX" +references: true +breadcrumbs: + - "Brain" + - "Differential Diagnosis" + - "Ventricles, Periventricular Regions" + - "Generic Imaging Patterns" + - "Ventriculomegaly" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - 2 main causes of ventriculomegaly + - ↑ CSF pressures → hydrocephalus + - ↓ brain volume → passive ventricular enlargement + - Clinical features to consider + - Head circumference is critical to interpreting significance of enlarged ventricles + - ↑ head size: Hydrocephalus + - ↓ head size: Brain parenchymal volume loss + - Patients may come to clinical attention during prenatal screening or well-child check-ups + - Imaging features of hydrocephalus + - Etiologies to consider + - Obstruction usually occurs at cerebral aqueduct, foramen of Monro, or 4th ventricular outlets + - e.g., aqueductal stenosis, obstructing tumor + - Communicating hydrocephalus is due to CSF overproduction or poor CSF resorption + - e.g., choroid plexus papilloma, benign enlargement of subarachnoid spaces + - Some etiologies can cause either or mixed forms + - e.g., posthemorrhagic, postinfectious, vein of Galen malformation (VGAM) + - Imaging signs of hydrocephalus + - Macrocephaly: ↑ craniofacial ratio + - Rounded margins of lateral ventricles, depressed floor of 3rd ventricle + - Transependymal edema: Periventricular white matter (WM) ↑ T2/FLAIR signal, greatest at frontal & occipital horns; effacement of cerebral sulci & basilar cisterns + - Imaging features of volume loss + - Etiologies to consider + - Diffuse vs. focal cortical ischemia + - e.g., hypoxic-ischemic encephalopathy (HIE) vs. arterial ischemic stroke + - Symmetric vs. focal WM volume loss + - e.g., periventricular leukomalacia vs. porencephaly + - Imaging signs of brain parenchymal volume loss + - Microcephaly: ↓ craniofacial ratio + - Angular margins of lateral ventricles + - Prominent sulci & basilar cisterns + - Modality considerations + - Fetal US for detection of in utero ventriculomegaly + - Fetal MR is excellent for underlying/associated anomalies + - US is important tool for initial evaluation of neonates/infants with suspected ventriculomegaly + - Requires open anterior fontanelle + - CT is often 1st-line modality in acute presentations + - MR is most definitive test for determination of underlying cause of ventriculomegaly + - Best test for determining cause of hydrocephalus + - Best evaluation of brain parenchyma to detect possible volume loss +- ## Helpful Clues for Common Diagnoses + + + - **Fetal Ventriculomegaly** + - Likelihood of normal neurologic development based on lateral ventricular size in utero + - Mild (10-12 mm): > 90% + - Moderate (13-15 mm): 80-93% + - Severe (> 15 mm): ~ 62% + - Underlying causes of fetal ventriculomegaly + - Infectious (e.g., CMV), genetic (e.g., trisomy), malformations (e.g., Chiari 2) + - CMV testing & amniocentesis typically performed + - Obstructive causes considered separately + - **Benign Enlargement of Subarachnoid Spaces** + - a.k.a. benign hydrocephalus of infancy, benign macrocrania of infancy, external hydrocephalus + - Enlarged subarachnoid spaces (SAS) ± mild ventriculomegaly + - Findings develop in 1st few months of life & usually resolve by ~ 2 years of age + - Often family history of macrocephaly + - Normal development or mild developmental delay + - Imaging: Prominent SAS over frontal & parietal convexities with normal vessels traversing SAS + - No displacement of arachnoid membrane from dura (i.e., no subdural fluid) + - **Chiari 2 Malformation** + - Brain malformation due to open neural tube defect (most commonly lumbosacral myelomeningocele) + - Hydrocephalus requiring shunting occurs almost universally without in utero surgical repair + - Imaging: Small posterior fossa, inferior migration of cerebellum/brainstem, beaked tectum, dysplastic corpus callosum, scalloping of dorsal clivus + - **Aqueductal Stenosis** + - Congenital atresia/stenosis of cerebral aqueduct + - Imaging: Lateral & 3rd ventricle dilation with normal 4th ventricle + - Narrowed/absent cerebral aqueduct on 3D SSFP MR + - Lack of flow void within cerebral aqueduct on T2 + - **Acquired Hydrocephalus** + - 2 main types: Communicating & noncommunicating + - Communicating: ↑ production or ↓ resorption of CSF + - Obstructive: Blockage of ventricular outlets + - Depending on cause, may present enlarging head or signs of ↑ intracranial pressure + - May have signs of ↑ intracranial pressure + - Treatment: Shunting or 3rd ventriculostomy + - **Posthemorrhagic hydrocephalus** + - Most common in premature infants with germinal matrix/intraventricular hemorrhage (IVH) + - Look for hemosiderin along ventricles/brainstem + - SWI/GRE is most sensitive for detection of prior IVH + - **Acute infectious hydrocephalus** + - During acute bacterial infection, ↑ head size may occur due to ↑ extraaxial spaces from meningitis & subdural/epidural empyema + - **Postinfectious hydrocephalus** + - Obstruction may occur due to synechiae + - Communicating hydrocephalus may occur due to arachnoid granulation dysfunction + - **Obstructing tumor** + - Smaller tumors in critical locations may obstruct + - e.g., subependymal giant cell astrocytoma (SEGA) at foramen of Monro, tectal plate glioma at cerebral aqueduct + - Larger tumors anywhere in brain may obstruct, particularly in posterior fossa + - **CSF overproduction (choroid plexus tumors)** + - Choroid plexus papilloma/carcinoma causes CSF overproduction + - Inability to resorb excess CSF → ↑ ventricular size + - **Brain Volume Loss** + - Results in ventricular enlargement + - Focal (e.g., porencephaly, stroke) + - Symmetric (e.g., periventricular leukomalacia, metabolic disease) + - **Hypoxic-ischemic injury** + - Often results in symmetric or asymmetric brain volume loss & associated ventricular enlargement + - Look for characteristic patterns of injury (e.g., perirolandic or watershed) + - **Arterial ischemic stroke** + - Most often occurs in perinatal period or related to embolic phenomenon + - Encephalomalacia develops in arterial territory with focal ventricular enlargement + - **Periventricular leukomalacia** + - Sequelae of WM injury of prematurity + - Usually symmetric WM volume loss with little or no associated gliosis (↑ FLAIR signal) + - **Porencephaly** + - Most often results from grade 4 IVH, but any insult early in development may cause porencephaly + - Porencephaly develops rather than encephalomalacia as no astrocytic response occurs in very young brains + - **Metabolic brain disease** + - Numerous diseases cause significant brain volume loss, resulting in ventriculomegaly + - Usually symmetric; may be + - Frontal predominant (e.g., Alexander disease) + - Posterior predominant (e.g., X-linked adrenoleukodystrophy) + - Diffuse (e.g., metachromatic leukodystrophy) +- ## Helpful Clues for Less Common Diagnoses + + + - **Hemimegalencephaly** + - Unilateral abnormal neuronal proliferation & migration + - Neonate/infant: ↑ T1 & ↓ T2 in WM with blurred gray matter/WM junctions + - Often shows enlarged ipsilateral ventricle + - **Vein of Galen Aneurysmal Malformation** + - Ectatic vascular structure is median prosencephalic vein, not vein of Galen, which never forms + - High-flow vascular malformation due to direct communication between arteries & median prosencephalic vein + - Ventricular enlargement may occur due to + - Mass effect of malformation on cerebral aqueduct + - ↑ venous pressure & poor CSF resorption + - Parenchymal insult with volume loss + - After birth, ↑ in blood flow through malformation + - Up to 80% of left ventricular output may supply brain + - Leads to ↑ cardiac output & heart failure + - **Dandy-Walker Malformation** + - Vermian hypoplasia with large posterior fossa cyst that communicates with 4th ventricle but not SAS + - Lambdoid-torcular inversion + - Often associated with hydrocephalus + - **Hydranencephaly** + - In utero destruction of brain in internal carotid artery territories + - May cause poor CSF regulation & hydrocephalus, requiring shunting + - **Holoprosencephaly** + - Holoprosencephaly (alobar form) often develops ↑ size of monoventricle over time + - Typically treated with CSF shunting + +## References + +# Selected References + +1. [Adiyaman D et al: Contribution of fetal magnetic resonance imaging in the evaluation of neurosonographically detected cases of isolated mild and moderate cerebral ventriculomegaly. J Obstet Gynaecol Res. 48(9):2314-24, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35778980%5Bpmid%5D) +1. [D'Addario V: Diagnostic approach to fetal ventriculomegaly. J Perinat Med. ePub, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36005554%5Bpmid%5D) +1. [Bauer DF et al: Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on the Treatment of Pediatric Hydrocephalus: Update of the 2014 Guidelines. Neurosurgery. 87(6):1071-75, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=34791462%5Bpmid%5D) +1. [Di Mascio D et al: Systematic review and meta-analysis on the role of prenatal magnetic resonance imaging in the era of fetal neurosonography: mild and moderate ventriculomegaly. Ultrasound Obstet Gynecol. 54(2):164-71, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30549340%5Bpmid%5D) +1. [Katz JA et al: Utility of prenatal MRI in the evaluation and management of fetal ventriculomegaly. J Perinatol. 38(11):1444-52, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30158676%5Bpmid%5D) +1. [Orrù E et al: The child with macrocephaly: differential diagnosis and neuroimaging findings. AJR Am J Roentgenol. 210(4):848-59, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29470161%5Bpmid%5D) +1. [Rashid QT et al: Time trends and age-related etiologies of pediatric hydrocephalus: results of a groupwise analysis in a clinical cohort. Childs Nerv Syst. 28(2):221-7, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=21818584%5Bpmid%5D) +1. [Kurihara Y et al: MR imaging of ventriculomegaly--a qualitative and quantitative comparison of communicating hydrocephalus, central atrophy, and normal studies. J Magn Reson Imaging. 5(4):451-6, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7549210%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Transverse oblique US in an 18-week fetus shows mild enlargement of the lateral ventricles (11 mm). If the ventriculomegaly worsens later in pregnancy, fetal or postnatal MR imaging should be obtained.](images/app.statdx.com_image_thumbnail_70468ab8-1e6a-4667-8825-a704fdbf86ce_annotated_true_size_900_quality_90_cfed8e44_20251018T165228Z.jpg) +**Fetal Ventriculomegaly** +*Transverse oblique US in an 18-week fetus shows mild enlargement of the lateral ventricles (11 mm). If the ventriculomegaly worsens later in pregnancy, fetal or postnatal MR imaging should be obtained.* + +![Transverse oblique US in an 18-week fetus shows mild enlargement of the lateral ventricles (11 mm). If the ventriculomegaly worsens later in pregnancy, fetal or postnatal MR imaging should be obtained.](images/app.statdx.com_image_thumbnail_70468ab8-1e6a-4667-8825-a704fdbf86ce_size_174_quality_85_c708c1a0_20251018T155131Z.jpg) +**Fetal Ventriculomegaly** +*Transverse oblique US in an 18-week fetus shows mild enlargement of the lateral ventricles (11 mm). If the ventriculomegaly worsens later in pregnancy, fetal or postnatal MR imaging should be obtained.* + +![Axial T2 SSFSE MR in a fetus shows marked enlargement (> 15 mm) of the lateral ventricles & thinning of the cerebrum . With severe fetal ventriculomegaly (particularly in the setting of aqueductal stenosis), the septum pellucidum (& even the cerebral mantle) may become disrupted.](images/app.statdx.com_image_thumbnail_82f99506-7e41-4801-843b-30dd71594c0b_annotated_true_size_900_quality_90_45cd65ce_20251018T165228Z.jpg) +**Fetal Ventriculomegaly** +*Axial T2 SSFSE MR in a fetus shows marked enlargement (> 15 mm) of the lateral ventricles & thinning of the cerebrum . With severe fetal ventriculomegaly (particularly in the setting of aqueductal stenosis), the septum pellucidum (& even the cerebral mantle) may become disrupted.* + +![Coronal T2 MR in a 1-year-old with macrocephaly shows mild enlargement of the lateral & 3rd ventricles with moderate enlargement of the bifrontal subarachnoid spaces (SAS), which are traversed by normal veins . Mild ventriculomegaly is often seen in benign enlargement of subarachnoid spaces (BESS).](images/app.statdx.com_image_thumbnail_0db6c91f-cab8-4b5c-ac67-4fb4207fefb1_annotated_true_size_900_quality_90_37c9b115_20251018T165228Z.jpg) +**Benign Enlargement of Subarachnoid Spaces** +*Coronal T2 MR in a 1-year-old with macrocephaly shows mild enlargement of the lateral & 3rd ventricles with moderate enlargement of the bifrontal subarachnoid spaces (SAS), which are traversed by normal veins . Mild ventriculomegaly is often seen in benign enlargement of subarachnoid spaces (BESS).* + +![Coronal color Doppler US in a 9-month-old with BESS shows normal vessels coursing through prominent fluid , an expected finding that helps differentiate the SAS from subdural collections.](images/app.statdx.com_image_thumbnail_037fb32e-f70d-4868-8c48-ac7cc17c756d_annotated_true_size_900_quality_90_3febc017_20251018T165228Z.jpg) +**Benign Enlargement of Subarachnoid Spaces** +*Coronal color Doppler US in a 9-month-old with BESS shows normal vessels coursing through prominent fluid , an expected finding that helps differentiate the SAS from subdural collections.* + +![Sagittal 3D SSFP MR in a neonate with a myelomeningocele shows marked enlargement of the lateral ventricles & characteristic features of Chiari 2 malformation: Small posterior fossa, brainstem & cerebellar descent , tectal beaking , & scalloped clivus .](images/app.statdx.com_image_thumbnail_ccc8cc54-8a5d-4831-a355-77f332dd3317_annotated_true_size_900_quality_90_97654240_20251018T165228Z.jpg) +**Chiari 2 Malformation** +*Sagittal 3D SSFP MR in a neonate with a myelomeningocele shows marked enlargement of the lateral ventricles & characteristic features of Chiari 2 malformation: Small posterior fossa, brainstem & cerebellar descent , tectal beaking , & scalloped clivus .* + +![Sagittal T2 MR in a neonate shows massive enlargement of the lateral ventricles due to obstruction at the level of the cerebral aqueduct . The 3rd ventricle is also enlarged , but the 4th ventricle is normal, typical of this disorder.](images/app.statdx.com_image_thumbnail_bba6ec86-c9f5-4828-93d3-e66a48d1f5e7_annotated_true_size_900_quality_90_544f92ca_20251018T165228Z.jpg) +**Aqueductal Stenosis** +*Sagittal T2 MR in a neonate shows massive enlargement of the lateral ventricles due to obstruction at the level of the cerebral aqueduct . The 3rd ventricle is also enlarged , but the 4th ventricle is normal, typical of this disorder.* + +![Sagittal T2 MR in a 1-month-old former premature infant with posthemorrhagic hydrocephalus shows marked enlargement of the lateral , 3rd , & 4th ventricles. Note the dark hemosiderin lining the pial surface of the brainstem from prior intraventricular hemorrhage (IVH).](images/app.statdx.com_image_thumbnail_1507c0c1-9157-4c19-89cf-df575996cf50_annotated_true_size_900_quality_90_5c58dea8_20251018T165228Z.jpg) +**Posthemorrhagic Hydrocephalus** +*Sagittal T2 MR in a 1-month-old former premature infant with posthemorrhagic hydrocephalus shows marked enlargement of the lateral , 3rd , & 4th ventricles. Note the dark hemosiderin lining the pial surface of the brainstem from prior intraventricular hemorrhage (IVH).* + +![Axial SWI MR in a 3-month-old former premature infant with posthemorrhagic hydrocephalus shows signal loss along the ependymal margins of the ventricles & choroid plexus , consistent with prior IVH.](images/app.statdx.com_image_thumbnail_7e222e3d-c62e-4387-84b2-eb4f8647e09b_annotated_true_size_900_quality_90_d4a8ce99_20251018T165228Z.jpg) +**Posthemorrhagic Hydrocephalus** +*Axial SWI MR in a 3-month-old former premature infant with posthemorrhagic hydrocephalus shows signal loss along the ependymal margins of the ventricles & choroid plexus , consistent with prior IVH.* + +![Axial T1 C+ MR in a 7-year-old with Haemophilus influenzae meningitis shows marked expansion of the bifrontal SAS & mild enlargement of the lateral ventricles .](images/app.statdx.com_image_thumbnail_9a7bb1d1-b81f-4369-93ad-95c530aed370_annotated_true_size_900_quality_90_f7aa2f43_20251018T165234Z.jpg) +**Acute Infectious Hydrocephalus** +*Axial T1 C+ MR in a 7-year-old with Haemophilus influenzae meningitis shows marked expansion of the bifrontal SAS & mild enlargement of the lateral ventricles .* + +![Axial T1 C+ FS MR in an 8-month-old with tuberculous meningitis shows extensive basilar leptomeningeal enhancement & enlargement of the lateral ventricles , resulting in macrocephaly. Granulomatous infections are more likely to result in hydrocephalus compared to other bacterial meningitis.](images/app.statdx.com_image_thumbnail_5303411a-ca31-4d2c-b74c-0cf2a8730339_annotated_true_size_900_quality_90_c6cc9e06_20251018T165234Z.jpg) +**Postinfectious Hydrocephalus** +*Axial T1 C+ FS MR in an 8-month-old with tuberculous meningitis shows extensive basilar leptomeningeal enhancement & enlargement of the lateral ventricles , resulting in macrocephaly. Granulomatous infections are more likely to result in hydrocephalus compared to other bacterial meningitis.* + +![Sagittal FLAIR MR in a neonate with a large, obstructing, hemorrhagic posterior fossa mass shows enlargement of the lateral ventricles & posterior fossa. Note the ↑ craniofacial ratio.](images/app.statdx.com_image_thumbnail_3ff8c828-9d09-46d7-b1a1-d19d6bd5fb8d_annotated_true_size_900_quality_90_9f72e3d5_20251018T165234Z.jpg) +**Obstructing Tumor** +*Sagittal FLAIR MR in a neonate with a large, obstructing, hemorrhagic posterior fossa mass shows enlargement of the lateral ventricles & posterior fossa. Note the ↑ craniofacial ratio.* + +![Coronal T1 C+ MR in a 1-year-old with a choroid plexus papilloma shows an enhancing mass in the right choroid plexus. The lateral ventricles are enlarged without evidence of obstruction. Hydrocephalus in this case is due to overproduction of CSF by the tumor.](images/app.statdx.com_image_thumbnail_87a6356e-2c5c-44af-9516-f54896957a41_annotated_true_size_900_quality_90_b9f6d0f2_20251018T165234Z.jpg) +**CSF Overproduction (Choroid Plexus Tumor)** +*Coronal T1 C+ MR in a 1-year-old with a choroid plexus papilloma shows an enhancing mass in the right choroid plexus. The lateral ventricles are enlarged without evidence of obstruction. Hydrocephalus in this case is due to overproduction of CSF by the tumor.* + +![Axial FLAIR MR in a 4-year-old with a history of perinatal hypoxic-ischemic injury (HII) shows extensive areas of cortical encephalomalacia . Note the localized areas of ventriculomegaly due to overlying brain volume loss.](images/app.statdx.com_image_thumbnail_85b3abc9-edc3-48ec-9e40-734d0f72d297_annotated_true_size_900_quality_90_72ffe6d4_20251018T165234Z.jpg) +**Hypoxic-Ischemic Injury** +*Axial FLAIR MR in a 4-year-old with a history of perinatal hypoxic-ischemic injury (HII) shows extensive areas of cortical encephalomalacia . Note the localized areas of ventriculomegaly due to overlying brain volume loss.* + +![Axial T2 MR in a 2-year-old with a history of perinatal HII shows symmetric areas of signal abnormality & volume loss involving the thalami , putamina , & periventricular white matter , resulting in enlargement of the lateral & 3rd ventricles.](images/app.statdx.com_image_thumbnail_10667680-dfdd-4ced-a40d-43372e509021_annotated_true_size_900_quality_90_e1abf64c_20251018T165234Z.jpg) +**Hypoxic-Ischemic Injury** +*Axial T2 MR in a 2-year-old with a history of perinatal HII shows symmetric areas of signal abnormality & volume loss involving the thalami , putamina , & periventricular white matter , resulting in enlargement of the lateral & 3rd ventricles.* + +![Axial T2 MR in a 9-month-old with previous infarction shows extensive cystic encephalomalacia in right middle cerebral artery (MCA) territory with resultant asymmetric enlargement of the right lateral ventricle .](6accf05b-0e43-4a70-a966-67794ba8f8dd) +**Arterial Ischemic Stroke** +*Axial T2 MR in a 9-month-old with previous infarction shows extensive cystic encephalomalacia in right middle cerebral artery (MCA) territory with resultant asymmetric enlargement of the right lateral ventricle .* + +![Axial FLAIR MR in a 7-year-old with a history of extreme prematurity & periventricular leukomalacia (PVL) shows symmetric focal enlargement of the atria with adjacent white matter volume loss. The relative lack of abnormal FLAIR signal compared to the degree of volume loss is typical of PVL.](cd6af741-e6fa-46aa-bd6e-a2be4fa84b23) +**Periventricular Leukomalacia** +*Axial FLAIR MR in a 7-year-old with a history of extreme prematurity & periventricular leukomalacia (PVL) shows symmetric focal enlargement of the atria with adjacent white matter volume loss. The relative lack of abnormal FLAIR signal compared to the degree of volume loss is typical of PVL.* + +![Coronal US at 2 days of life in an extremely premature (23-week) infant shows a large right germinal matrix hemorrhage with associated hemorrhagic venous infarction in the right frontoparietal white matter.](2acd2437-e908-4dc8-8834-2946efa2858f) +**Porencephaly** +*Coronal US at 2 days of life in an extremely premature (23-week) infant shows a large right germinal matrix hemorrhage with associated hemorrhagic venous infarction in the right frontoparietal white matter.* + +![Coronal T2 MR in the same patient 3 months later shows the expected development of right parietal porencephaly & focal ventricular enlargement. Note the rim of T2 hypointensity , related to hemosiderin, which will eventually resolve.](f0f97ad6-303c-43fc-b9d1-3cc3eba46780) +**Porencephaly** +*Coronal T2 MR in the same patient 3 months later shows the expected development of right parietal porencephaly & focal ventricular enlargement. Note the rim of T2 hypointensity , related to hemosiderin, which will eventually resolve.* + +![Axial T2 MR in a teenager with metachromatic leukodystrophy shows ↑ signal & volume loss in the periventricular & deep white matter with sparing of the subcortical white matter, characteristic of this disease. Note the enlargement of the lateral ventricles & sulci due to the brain volume loss.](94c86422-ef68-4680-8877-d188e7f59890) +**Metabolic Brain Disease** +*Axial T2 MR in a teenager with metachromatic leukodystrophy shows ↑ signal & volume loss in the periventricular & deep white matter with sparing of the subcortical white matter, characteristic of this disease. Note the enlargement of the lateral ventricles & sulci due to the brain volume loss.* + +![Axial T2 MR in a teenager with vanishing white matter disease shows extensive ↑ signal intensity & volume loss in the white matter with associated enlargement of the lateral & 3rd ventricles & sulci .](efb42805-a92c-4737-a01b-753b53b8cc79) +**Metabolic Brain Disease** +*Axial T2 MR in a teenager with vanishing white matter disease shows extensive ↑ signal intensity & volume loss in the white matter with associated enlargement of the lateral & 3rd ventricles & sulci .* + +![Axial T1 MR in a neonate with seizures & hemimegalencephaly shows ↑ size of the left parietooccipital hemisphere with loss of normal sulcation & markedly abnormal neuronal organization . Also note enlargement of the ipsilateral occipital horn .](95b7dcd5-6ed3-4d5f-b06e-9a9c8071382b) +**Hemimegalencephaly** +*Axial T1 MR in a neonate with seizures & hemimegalencephaly shows ↑ size of the left parietooccipital hemisphere with loss of normal sulcation & markedly abnormal neuronal organization . Also note enlargement of the ipsilateral occipital horn .* + +![Axial T1 MR in a 3-day-old with left hemimegalencephaly shows marked occipital horn enlargement . Note the abnormal white matter in the left frontal lobe. Enlargement of the ipsilateral occipital horn is common in this disease.](df838971-ddb2-478e-bd8e-61844e1598ea) +**Hemimegalencephaly** +*Axial T1 MR in a 3-day-old with left hemimegalencephaly shows marked occipital horn enlargement . Note the abnormal white matter in the left frontal lobe. Enlargement of the ipsilateral occipital horn is common in this disease.* + +![Coronal T2 MR in a neonate with VGAM shows a markedly enlarged central vein with numerous enlarged choroidal & pericallosal feeding arteries. Note the enlarged ventricles , which are likely due to ↓ resorption of CSF due to ↑ venous pressures.](c5d3d1dc-0041-4021-9ee0-f19c99cc4d0e) +**Vein of Galen Aneurysmal Malformation** +*Coronal T2 MR in a neonate with VGAM shows a markedly enlarged central vein with numerous enlarged choroidal & pericallosal feeding arteries. Note the enlarged ventricles , which are likely due to ↓ resorption of CSF due to ↑ venous pressures.* + +![Sagittal 3D SSFP MR in a 2-month-old with Dandy-Walker malformation shows a small cerebellar vermis & large posterior fossa cyst that is continuous with the 4th ventricle. There is elevation of the tentorium & torcular Herophili .](fd790334-ffcb-435d-afe1-06cd9fb5581d) +**Dandy-Walker Malformation** +*Sagittal 3D SSFP MR in a 2-month-old with Dandy-Walker malformation shows a small cerebellar vermis & large posterior fossa cyst that is continuous with the 4th ventricle. There is elevation of the tentorium & torcular Herophili .* + +![Axial NECT in a 4-year-old with hydranencephaly shows porencephaly in the bilateral MCA & left anterior cerebral artery (ACA) territories in continuity with the lateral ventricles. Note the intact falx . Patients with hydranencephaly typically become macrocephalic due to poor CSF regulation.](0bce95e0-fa1b-4963-b4ec-ca1c53bd22f7) +**Hydranencephaly** +*Axial NECT in a 4-year-old with hydranencephaly shows porencephaly in the bilateral MCA & left anterior cerebral artery (ACA) territories in continuity with the lateral ventricles. Note the intact falx . Patients with hydranencephaly typically become macrocephalic due to poor CSF regulation.* + +![Sagittal T2 MR in a neonate with holoprosencephaly shows an enlarged monoventricle . However, the patient is microcephalic overall due to the ↓ brain parenchymal volume.](a0fe9fac-0d38-4324-a508-85ddc944e911) +**Holoprosencephaly** +*Sagittal T2 MR in a neonate with holoprosencephaly shows an enlarged monoventricle . However, the patient is microcephalic overall due to the ↓ brain parenchymal volume.* + diff --git a/results.json b/results.json index c679b38..e00b223 100644 --- a/results.json +++ b/results.json @@ -1,143 +1,157 @@ [ { - "path": "docs_md/articles/ahle_0ec0bca6-abee-4931-a6ed-43541b626261.md", - "title": "AHLE", - "docid": "0ec0bca6-abee-4931-a6ed-43541b626261", + "path": "docs_md/articles/cavum-septi-pellucidi-csp_02127bd4-1efa-4056-925e-f1a1bbadf154.md", + "title": "Cavum Septi Pellucidi (CSP)", + "docid": "02127bd4-1efa-4056-925e-f1a1bbadf154", "breadcrumbs": [ "Brain", "Diagnosis", - "Pathology-Based Diagnoses", - "Infectious, Inflammatory, and Demyelinating Disease", - "Inflammatory and Demyelinating Disease", - "AHLE" + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "Normal Variants", + "Cavum Septi Pellucidi (CSP)" ], "authors": [ + { + "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", + "value": "Miral D. 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Osborn, MD, FACR" } ], - "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, AHLE" + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Normal Variants, Cavum Septi Pellucidi (CSP)" }, { - "path": "docs_md/articles/adem_a3fafeb7-5861-4364-beb8-c0e30220564e.md", - "title": "ADEM", - "docid": "a3fafeb7-5861-4364-beb8-c0e30220564e", + "path": "docs_md/articles/periventricular-enhancing-lesions_0edb9603-ea97-4f3b-be82-21d53c42be32.md", + "title": "Periventricular Enhancing Lesions", + "docid": "0edb9603-ea97-4f3b-be82-21d53c42be32", + "breadcrumbs": [ + "Brain", + "Differential Diagnosis", + "Ventricles, Periventricular Regions", + "Generic Imaging Patterns", + "Periventricular Enhancing Lesions" + ], + "authors": [ + { + "key": "1fa14dfd-71ea-4960-908e-e720313bc63a", + "value": "Santhosh Gaddikeri, MD" + }, + { + "key": "30ce27b2-237f-4aff-a88f-65ead356335b", + "value": "Marinos Kontzialis, MD" + } + ], + "pageKeywords": "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Periventricular Enhancing Lesions" + }, + { + "path": "docs_md/articles/csf-shunts-and-complications_1027d634-92ff-47c1-8266-a7fc3acd1529.md", + "title": "CSF Shunts and Complications", + "docid": "1027d634-92ff-47c1-8266-a7fc3acd1529", "breadcrumbs": [ "Brain", "Diagnosis", - "Pathology-Based Diagnoses", - "Infectious, Inflammatory, and Demyelinating Disease", - "Inflammatory and Demyelinating Disease", - "ADEM" + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "Hydrocephalus", + "CSF Shunts and Complications" ], "authors": [ + { + "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", + "value": "Miral D. Jhaveri, MD, MBA" + }, { "key": "99e1aff7-f42c-43a0-95ae-d89c8551aa01", "value": "Kevin R. Moore, MD" + } + ], + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, CSF Shunts and Complications" + }, + { + "path": "docs_md/articles/small-ventricles_2f99bc62-163e-41aa-b190-0da8a4de6d11.md", + "title": "Small Ventricles", + "docid": "2f99bc62-163e-41aa-b190-0da8a4de6d11", + "breadcrumbs": [ + "Brain", + "Differential Diagnosis", + "Ventricles, Periventricular Regions", + "Generic Imaging Patterns", + "Small Ventricles" + ], + "authors": [ + { + "key": "d19354f3-7ff2-495a-ad3f-064122e45602", + "value": "Bernadette L. Koch, MD" }, { - "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", - "value": "Miral D. Jhaveri, MD, MBA" + "key": "f184750a-90b4-47a7-907b-23b05d70357a", + "value": "Chang Yueh Ho, MD" + } + ], + "pageKeywords": "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Small Ventricles" + }, + { + "path": "docs_md/articles/benign-enlarged-subarachnoid-spaces_3da4fec0-6e87-4bcc-bd66-b4a5d1984f6e.md", + "title": "Benign Enlarged Subarachnoid Spaces", + "docid": "3da4fec0-6e87-4bcc-bd66-b4a5d1984f6e", + "breadcrumbs": [ + "Brain", + "Diagnosis", + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "Normal Variants", + "Benign Enlarged Subarachnoid Spaces" + ], + "authors": [ + { + "key": "47381de4-c9fd-4999-8dd0-1808cd72db6b", + "value": "Luke L. Linscott, MD" }, { "key": "b2e6dabb-ee1c-42a4-a332-9f0814c1c607", "value": "Surjith Vattoth, MD, FRCR" } ], - "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, ADEM" + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Normal Variants, Benign Enlarged Subarachnoid Spaces" }, { - "path": "docs_md/articles/cidp_12e4033c-edc8-46ff-8081-3acc433cda78.md", - "title": "CIDP", - "docid": "12e4033c-edc8-46ff-8081-3acc433cda78", + "path": "docs_md/articles/aqueductal-stenosis_6dfa6261-3945-4606-850b-51484d05e70c.md", + "title": "Aqueductal Stenosis", + "docid": "6dfa6261-3945-4606-850b-51484d05e70c", "breadcrumbs": [ "Brain", "Diagnosis", - "Pathology-Based Diagnoses", - "Infectious, Inflammatory, and Demyelinating Disease", - "Inflammatory and Demyelinating Disease", - "CIDP" + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "Hydrocephalus", + "Aqueductal Stenosis" ], "authors": [ + { + "key": "2c9d2e67-05db-4d26-b8cb-02e0f7566179", + "value": "Usha D. Nagaraj, MD" + }, { "key": "b2e6dabb-ee1c-42a4-a332-9f0814c1c607", "value": "Surjith Vattoth, MD, FRCR" } ], - "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, CIDP" + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Aqueductal Stenosis" }, { - "path": "docs_md/articles/neuromyelitis-optica-spectrum-disorders_54d4a8bc-9267-4df6-98c1-f22aae051d01.md", - "title": "Neuromyelitis Optica Spectrum Disorders", - "docid": "54d4a8bc-9267-4df6-98c1-f22aae051d01", + "path": "docs_md/articles/intracranial-hypotension_818a7972-1032-4d3e-a65a-97c494334aac.md", + "title": "Intracranial Hypotension", + "docid": "818a7972-1032-4d3e-a65a-97c494334aac", "breadcrumbs": [ "Brain", "Diagnosis", - "Pathology-Based Diagnoses", - "Infectious, Inflammatory, and Demyelinating Disease", - "Inflammatory and Demyelinating Disease", - "Neuromyelitis Optica Spectrum Disorders" - ], - "authors": [ - { - "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", - "value": "Miral D. Jhaveri, MD, MBA" - } - ], - "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Neuromyelitis Optica Spectrum Disorders" - }, - { - "path": "docs_md/articles/autoimmune-encephalitis_6eb3d5d6-7f6a-4367-a792-b5d4b19675da.md", - "title": "Autoimmune Encephalitis", - "docid": "6eb3d5d6-7f6a-4367-a792-b5d4b19675da", - "breadcrumbs": [ - "Brain", - "Diagnosis", - "Pathology-Based Diagnoses", - "Infectious, Inflammatory, and Demyelinating Disease", - "Inflammatory and Demyelinating Disease", - "Autoimmune Encephalitis" - ], - "authors": [ - { - "key": "8d5254e9-8dda-478b-8f08-bdee97a32c79", - "value": "Karen L. Salzman, MD, FACR" - } - ], - "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Autoimmune Encephalitis" - }, - { - "path": "docs_md/articles/multiple-sclerosis_7892b2a2-f52a-4d7f-9858-a326f2b7ab04.md", - "title": "Multiple Sclerosis", - "docid": "7892b2a2-f52a-4d7f-9858-a326f2b7ab04", - "breadcrumbs": [ - "Brain", - "Diagnosis", - "Pathology-Based Diagnoses", - "Infectious, Inflammatory, and Demyelinating Disease", - "Inflammatory and Demyelinating Disease", - "Multiple Sclerosis" - ], - "authors": [ - { - "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", - "value": "Miral D. Jhaveri, MD, MBA" - } - ], - "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Multiple Sclerosis" - }, - { - "path": "docs_md/articles/clippers_ba394f3b-bbff-4128-90b5-3e1c07564c5f.md", - "title": "CLIPPERS", - "docid": "ba394f3b-bbff-4128-90b5-3e1c07564c5f", - "breadcrumbs": [ - "Brain", - "Diagnosis", - "Pathology-Based Diagnoses", - "Infectious, Inflammatory, and Demyelinating Disease", - "Inflammatory and Demyelinating Disease", - "CLIPPERS" + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "CSF Disorders", + "Intracranial Hypotension" ], "authors": [ { @@ -145,47 +159,235 @@ "value": "Anne G. Osborn, MD, FACR" } ], - "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, CLIPPERS" + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, CSF Disorders, Intracranial Hypotension" }, { - "path": "docs_md/articles/guillain-barr-spectrum-disorders_c1f52a65-920e-4e28-8a75-07dfa208f290.md", - "title": "Guillain-Barré Spectrum Disorders", - "docid": "c1f52a65-920e-4e28-8a75-07dfa208f290", + "path": "docs_md/articles/cavum-velum-interpositum-cvi_849ee468-35c4-46e3-9297-96196109cdb8.md", + "title": "Cavum Velum Interpositum (CVI)", + "docid": "849ee468-35c4-46e3-9297-96196109cdb8", "breadcrumbs": [ "Brain", "Diagnosis", - "Pathology-Based Diagnoses", - "Infectious, Inflammatory, and Demyelinating Disease", - "Inflammatory and Demyelinating Disease", - "Guillain-Barré Spectrum Disorders" + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "Normal Variants", + "Cavum Velum Interpositum (CVI)" ], "authors": [ { - "key": "b2e6dabb-ee1c-42a4-a332-9f0814c1c607", - "value": "Surjith Vattoth, MD, FRCR" + "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", + "value": "Miral D. Jhaveri, MD, MBA" + }, + { + "key": "5cff4116-3654-4b3a-bb75-5ebe0b8c9850", + "value": "Anne G. Osborn, MD, FACR" } ], - "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Guillain-Barré Spectrum Disorders" + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Normal Variants, Cavum Velum Interpositum (CVI)" }, { - "path": "docs_md/articles/pediatric-multiple-sclerosis-brain_f2592b04-f800-4235-9eea-a43f2bf4adfe.md", - "title": "Pediatric Multiple Sclerosis, Brain", - "docid": "f2592b04-f800-4235-9eea-a43f2bf4adfe", + "path": "docs_md/articles/asymmetric-lateral-ventricles_87387f0d-9b20-4288-a250-aa3ec83520c4.md", + "title": "Asymmetric Lateral Ventricles", + "docid": "87387f0d-9b20-4288-a250-aa3ec83520c4", + "breadcrumbs": [ + "Brain", + "Differential Diagnosis", + "Ventricles, Periventricular Regions", + "Generic Imaging Patterns", + "Asymmetric Lateral Ventricles" + ], + "authors": [ + { + "key": "1fa14dfd-71ea-4960-908e-e720313bc63a", + "value": "Santhosh Gaddikeri, MD" + }, + { + "key": "30ce27b2-237f-4aff-a88f-65ead356335b", + "value": "Marinos Kontzialis, MD" + } + ], + "pageKeywords": "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Asymmetric Lateral Ventricles" + }, + { + "path": "docs_md/articles/extraventricular-obstructive-hydrocephalus_a0886d4c-f504-4165-bb52-2400e2385f68.md", + "title": "Extraventricular Obstructive Hydrocephalus", + "docid": "a0886d4c-f504-4165-bb52-2400e2385f68", + "breadcrumbs": [ + "Brain", + "Diagnosis", + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "Hydrocephalus", + "Extraventricular Obstructive Hydrocephalus" + ], + "authors": [ + { + "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", + "value": "Miral D. Jhaveri, MD, MBA" + } + ], + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Extraventricular Obstructive Hydrocephalus" + }, + { + "path": "docs_md/articles/ventricles-and-cisterns-overview_ad860c4f-fe9a-4469-8eca-a7ccd5cff70f.md", + "title": "Ventricles and Cisterns Overview", + "docid": "ad860c4f-fe9a-4469-8eca-a7ccd5cff70f", + "breadcrumbs": [ + "Brain", + "Diagnosis", + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "Ventricles and Cisterns Overview" + ], + "authors": [ + { + "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", + "value": "Miral D. Jhaveri, MD, MBA" + } + ], + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Ventricles and Cisterns Overview" + }, + { + "path": "docs_md/articles/normal-pressure-hydrocephalus_ba3f857d-58de-4f21-8463-1631b4cb9972.md", + "title": "Normal-Pressure Hydrocephalus", + "docid": "ba3f857d-58de-4f21-8463-1631b4cb9972", + "breadcrumbs": [ + "Brain", + "Diagnosis", + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "Hydrocephalus", + "Normal-Pressure Hydrocephalus" + ], + "authors": [ + { + "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", + "value": "Miral D. Jhaveri, MD, MBA" + } + ], + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Normal-Pressure Hydrocephalus" + }, + { + "path": "docs_md/articles/idiopathic-intracranial-hypertension_d7a0a1b6-1d94-473c-9fe9-021443969f9f.md", + "title": "Idiopathic Intracranial Hypertension", + "docid": "d7a0a1b6-1d94-473c-9fe9-021443969f9f", + "breadcrumbs": [ + "Brain", + "Diagnosis", + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "CSF Disorders", + "Idiopathic Intracranial Hypertension" + ], + "authors": [ + { + "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", + "value": "Miral D. Jhaveri, MD, MBA" + } + ], + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, CSF Disorders, Idiopathic Intracranial Hypertension" + }, + { + "path": "docs_md/articles/corpus-callosum-impingement-syndrome_e84adf32-bae3-47d5-b368-489f413f6aea.md", + "title": "Corpus Callosum Impingement Syndrome", + "docid": "e84adf32-bae3-47d5-b368-489f413f6aea", + "breadcrumbs": [ + "Brain", + "Diagnosis", + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "Hydrocephalus", + "Corpus Callosum Impingement Syndrome" + ], + "authors": [ + { + "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", + "value": "Miral D. Jhaveri, MD, MBA" + } + ], + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Corpus Callosum Impingement Syndrome" + }, + { + "path": "docs_md/articles/hydrocephalus_e9481739-278e-4682-ab1e-4326a77c3d0c.md", + "title": "Hydrocephalus", + "docid": "e9481739-278e-4682-ab1e-4326a77c3d0c", "breadcrumbs": [ "Pediatrics", "Diagnosis", "Pediatric Neuroradiology", "Brain", - "Pathology-Based Diagnoses", - "Inflammatory and Demyelinating Disease", - "Pediatric Multiple Sclerosis, Brain" + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "Hydrocephalus" ], "authors": [ { - "key": "99e1aff7-f42c-43a0-95ae-d89c8551aa01", - "value": "Kevin R. Moore, MD" + "key": "838e1722-2479-4fbd-a5fe-d965980a1a2c", + "value": "Blaise V. Jones, MD" } ], - "pageKeywords": "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Inflammatory and Demyelinating Disease, Pediatric Multiple Sclerosis, Brain" + "pageKeywords": "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus" + }, + { + "path": "docs_md/articles/intraventricular-obstructive-hydrocephalus_eeac8d9b-1fdc-432e-8e09-11589611f7a8.md", + "title": "Intraventricular Obstructive Hydrocephalus", + "docid": "eeac8d9b-1fdc-432e-8e09-11589611f7a8", + "breadcrumbs": [ + "Brain", + "Diagnosis", + "Anatomy-Based Diagnoses", + "Ventricles and Cisterns", + "Hydrocephalus", + "Intraventricular Obstructive Hydrocephalus" + ], + "authors": [ + { + "key": "a25c450b-3d34-4f64-bba3-cc0834813df6", + "value": "Miral D. Jhaveri, MD, MBA" + } + ], + "pageKeywords": "Brain, Diagnosis, Anatomy-Based Diagnoses, Ventricles and Cisterns, Hydrocephalus, Intraventricular Obstructive Hydrocephalus" + }, + { + "path": "docs_md/articles/ventriculomegaly_f40bd6eb-e7e5-498a-8bde-ad6bcd546f21.md", + "title": "Ventriculomegaly", + "docid": "f40bd6eb-e7e5-498a-8bde-ad6bcd546f21", + "breadcrumbs": [ + "Brain", + "Differential Diagnosis", + "Ventricles, Periventricular Regions", + "Generic Imaging Patterns", + "Ventriculomegaly" + ], + "authors": [ + { + "key": "47381de4-c9fd-4999-8dd0-1808cd72db6b", + "value": "Luke L. Linscott, MD" + } + ], + "pageKeywords": "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Ventriculomegaly" + }, + { + "path": "docs_md/articles/irregular-lateral-ventricles_f42ce651-9877-480b-90d8-665be656b33f.md", + "title": "Irregular Lateral Ventricles", + "docid": "f42ce651-9877-480b-90d8-665be656b33f", + "breadcrumbs": [ + "Brain", + "Differential Diagnosis", + "Ventricles, Periventricular Regions", + "Generic Imaging Patterns", + "Irregular Lateral Ventricles" + ], + "authors": [ + { + "key": "1fa14dfd-71ea-4960-908e-e720313bc63a", + "value": "Santhosh Gaddikeri, MD" + }, + { + "key": "30ce27b2-237f-4aff-a88f-65ead356335b", + "value": "Marinos Kontzialis, MD" + } + ], + "pageKeywords": "Brain, Differential Diagnosis, Ventricles, Periventricular Regions, Generic Imaging Patterns, Irregular Lateral Ventricles" } ] \ No newline at end of file diff --git a/scrapers/document_to_markdown.py b/scrapers/document_to_markdown.py index 0a23ee8..1d42eb9 100644 --- a/scrapers/document_to_markdown.py +++ b/scrapers/document_to_markdown.py @@ -684,14 +684,11 @@ def process_file(path: str, out_dir: str, overwrite: bool = False) -> tuple[bool try: # DDX: only consider cached DDX[docid]; if not present, ddx is treated as not existing ddx_entry = None - ddx_html = None ddx_list = None if isinstance(DDX, dict) and docid and docid in DDX: ddx_entry = DDX.get(docid) - logger.debug(f"Found DDX entry for docid {docid}: {ddx_entry}") # ddx_entry may be HTML or structured list/dict if isinstance(ddx_entry, dict): - ddx_html = ddx_entry.get("ddxHtml") ddx_list = ddx_entry.get("ddx") or ddx_entry.get("differentialDiagnoses") or ddx_entry.get("differentials") else: # could be list or simple string @@ -700,29 +697,23 @@ def process_file(path: str, out_dir: str, overwrite: bool = False) -> tuple[bool logger.debug(f"No cached DDX entry for docid {docid}") logger.debug(f"DDX keys available: {list(DDX.keys())}") - logger.debug(f"DDX entry for docid {docid}: {ddx_entry}") - if ddx_html and isinstance(ddx_html, str) and ddx_html.strip(): - try: - ddx_md = html_to_markdown(ddx_html) - md = md.rstrip() + "\n\n" + "## Differential diagnosis\n\n" + ddx_md + "\n" - front_lines.append(f"ddx: {json.dumps(True)}") - except Exception: - logger.debug("Failed to convert ddxHtml for %s", out_path) - elif ddx_list: + if ddx_list: # render list representation try: front_lines.append(f"ddx: {json.dumps(True)}") md = md.rstrip() + "\n\n" + "## Differential diagnosis\n\n" if isinstance(ddx_list, list): for item in ddx_list: - md += "- " + str(item).strip() + "\n" + md += "### " + item.get("title") + "\n" + md += item.get("documentType") + ":" + item.get("documentId") + "\n\n" else: md += str(ddx_list).strip() + "\n" md += "\n" except Exception: + logger.debug(f"Failed to process DDX list for {out_path}") pass except Exception: - pass + logger.debug(f"Failed to process DDX for {out_path}") try: # Tables: only consider cached TABLES[docid] @@ -774,8 +765,9 @@ def process_file(path: str, out_dir: str, overwrite: bool = False) -> tuple[bool for a in anatomy_data: front_lines.append(f" - {json.dumps(str(a))}") md = md.rstrip() + "\n\n" + "## Anatomy\n\n" - for a in anatomy_data: - md += "- " + str(a).strip() + "\n" + for item in anatomy_data: + md += "### " + item.get("title") + "\n" + md += item.get("category", "").strip() + "/" + item.get("documentType").strip() + ":" + item.get("documentId") + "\n\n" md += "\n" else: front_lines.append(f"anatomy: {json.dumps(str(anatomy_data))}")