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a/docs_md/articles/abusive-head-trauma_c57982e3-fa8f-4fd6-9184-04fd1d37a906.md b/docs_md/articles/abusive-head-trauma_c57982e3-fa8f-4fd6-9184-04fd1d37a906.md new file mode 100644 index 0000000..8a83da4 --- /dev/null +++ b/docs_md/articles/abusive-head-trauma_c57982e3-fa8f-4fd6-9184-04fd1d37a906.md @@ -0,0 +1,513 @@ +--- +title: "Abusive Head Trauma" +docid: "c57982e3-fa8f-4fd6-9184-04fd1d37a906" +authors: + - key: "47381de4-c9fd-4999-8dd0-1808cd72db6b" + value: "Luke L. Linscott, 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: "Trauma" + slug: "trauma" + treeNodeId: "871c3647-7ecf-43b1-bdb0-062c7daa3c63" + - + name: "Abusive Head Trauma" + slug: "abusive-head-trauma" + treeNodeId: null +category: "Pediatrics" +cmeTopicId: "f065dff4-f104-46b4-a247-3dc20ba04d24" +documentVersionId: "c6f1cf6d-e4c9-4d6f-b5ea-df5d6fb5ab1c" +imageCount: 32 +lastUpdated: "01/31/24" +pageDescription: "Abusive Head Trauma" +pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Trauma, Abusive Head Trauma" +pageTitle: "Abusive Head Trauma | STATdx" +enhancedTitle: "Abusive Head Trauma" +type: "DX" +references: true +ddx: true +anatomy: + - "{'authors': 'Jeffrey S. 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"{'authors': 'Anne Kennedy, MD, FSRU, FAIUM', 'bookmarked': False, 'bookmarkUrl': '/document/bookmark/8fb2f541-799d-479f-b000-ab54f216199a', 'category': 'Ultrasound', 'compareUrl': '/compare/document/8fb2f541-799d-479f-b000-ab54f216199a/related-anatomy/treeNode?subContext=Embryology and Anatomy of Brain', 'documentId': '8fb2f541-799d-479f-b000-ab54f216199a', 'documentType': 'ANATOMY', 'documentUrl': '/document/embryology-and-anatomy-of-brain/8fb2f541-799d-479f-b000-ab54f216199a', 'enhancedTitle': 'Embryology and Anatomy of Brain', 'entryDate': '10/20/20', 'imageCount': 42, 'imageUrl': '/image/thumbnail/032e2d4b-7e55-4ba5-acf8-9a86b3586148?size=174&quality=85', 'inCompareCart': False, 'rank': 3, 'referenceCount': 7, 'showCompareButton': False, 'title': 'Embryology and Anatomy of Brain'}" +cases: 2 +breadcrumbs: + - "Pediatrics" + - "Diagnosis" + - "Pediatric Neuroradiology" + - "Brain" + - "Pathology-Based Diagnoses" + - "Trauma" + - "Abusive Head Trauma" +--- +# KEY FACTS + +- ## Terminology + + + - Abusive head trauma (AHT) + - Traumatic injury inflicted on infants & children by adults +- ## Imaging + + + - Direct impact injury: Direct blow to cranium + - Calvarial (often complex) & skull base fractures + - Focal brain injury deep to impact + - Shaking injury: Result of violent to-&-fro motion of head + - Subdural hematomas (SDHs) in 90-98% + - Generalized parenchymal injuries (cytotoxic edema, lacerations, axonal injury) + - Bridging vein injury & thrombosis common + - CT primary imaging tool in initial evaluation of AHT + - Multiplanar reconstructions improve detection of + - Small intracranial hemorrhages (ICHs) + - Fractures (with bone algorithm & 3D reformats) + - MR best for determining full extent of injury + - DWI paramount for parenchymal injury + - SWI/T2* GRE for hemorrhage + - T2/T1/FLAIR for detection of subdural collections + - T1 C+ for chronic SDH membranes + - Avoid speculation regarding timing & specific source of injury in report +- ## Top Differential Diagnoses + + + - Accidental trauma + - Benign macrocrania of infancy + - Mitochondrial encephalopathies + - Bleeding disorders +- ## Clinical Issues + + + - Discordance between stated history & degree of injury + - "Killer couch": Injuries blamed on infant rolling off couch + - Retinal hemorrhages in ~ 75% + - #1 cause of brain injury death in children < 2 years of age + - 17-25:100,000 annual incidence + - Cause of death in 80% of fatalities is brain swelling + - Severe hypoxic ischemic encephalopathy > diffuse axonal injury +- ## Diagnostic Checklist + + + - Reporting tips + - Avoid temptation to precisely time ICH + - Avoid vague, oblique, obscuring language in reports + - Avoid speculation regarding source of injury + +# TERMINOLOGY + +- ## Abbreviations + + + - Abusive head trauma (AHT) +- ## Synonyms + + + - Battered child syndrome, whiplash shaken infant syndrome, trauma-X, Caffey-Kempe syndrome, shaken-baby syndrome, nonaccidental head injury + - Multiple alternate titles have been suggested in attempt to minimize accusatory labeling in clinical setting + - May do more harm than good by causing confusion & hampering communication +- ## Definitions + + + - Traumatic injury inflicted on infants & children by adults + - This discussion centers on head injury + +# IMAGING + +- ## General Features + + + - Multiple brain injuries disproportionately severe relative to offered history + - 2 major groupings of injuries (but can occur together) + - Direct impact injury: Result of direct blow to cranium or impact of skull on object + - Shaking injury: Result of violent to-&-fro head motion + - Direct impact injury typified by skull fractures & injury to immediately underlying brain + - Scalp laceration, hematoma, swelling strongly associated + - High association with injuries to other organs + - Shaking injury typified by subdural hemorrhage (SDH) & generalized brain parenchymal injury + - Cytotoxic brain injury not conforming to arterial territories + - Exact etiology of parenchymal injury uncertain but usually permanent + - Hypoxic-ischemic vs. direct traumatic brain injury + - May see bridging vein injury ± thrombosis + - Imaging findings may suggest injuries of differing ages +- ## Radiographic Findings + + + - Sensitive in detection of linear skull fractures + - Fracture detection key component in forensic evaluation of suspected nonaccidental trauma (NAT) + - CT (with appropriate techniques) better characterizes fractures; often being obtained to evaluate for intracranial hemorrhage (ICH) + - Some fractures considered more suspicious for NAT + - Evidence does not support this + - Multiple compound, diastatic fractures, & fractures crossing sutures imply significant trauma but are not specific for NAT + - Discordance with provided history best indicator +- ## CT Findings + + + - NECT primary imaging tool for initial evaluation of AHT + - ICH + - SDH most common (90-98%) + - Dominant feature of shaking injury + - Overlying cerebral convexities, in interhemispheric fissure, overlying tentorium + - Normal density of subarachnoid space (SAS) stands out next to ↑ density of SDH + - Subarachnoid hemorrhage common (> 50%) + - Epidural hemorrhage uncommon but may occur + - More characteristic of accidental trauma + - Use great caution if attempting to estimate "age" of ICH + - Blood density based upon multiple factors: CSF dilution, hematocrit, coagulation status + - SDHs of same age can have significantly different densities + - Acute SDHs likely to have hyperdense component but usually have associated low-attenuation component from CSF admixture (i.e., hematohygroma) + - Most specific findings for chronic SDHs are compartmentalization/loculation & membrane formation + - Bridging vein injury ± thrombosis common (40-50%) + - Areas of ↑ density in paramedian high convexities + - Tadpole & lollipop signs + - May occur in accidental injury but more common in AHT + - Parenchymal ischemic injury often seen in shaking injuries + - Areas of ↓ density (with loss of gray matter-white matter differentiation) & sulcal effacement not confined to arterial territories; may be diffuse + - ↓ density of cerebrum vs. cerebellum: "Bright cerebellum" + - May be evident within several hours after injury + - Parenchymal laceration in 10-15% + - More commonly seen in AHT vs. accidental injury + - Shear injury (axonal injury) in ~ 15% + - Retinal hemorrhages uncommonly visualized on CT + - **CECT**:**** Enhancing membranes best sign of chronic SDH + - **CTA**: Detectable vascular injury relatively uncommon in child abuse + - Posttraumatic aneurysm, dissection can be demonstrated by CTA +- ## MR Findings + + + - **DWI**: Key for parenchymal injury + - Patterns of parenchymal injury are variable, including diffuse symmetric, multifocal, & focal + - **T1WI**: Bright foci of hemorrhage or evolving cortical injury + - **T2WI**: Loss of cortical ribbon & deep nuclei in neonates + - Coronal T2 often helpful to identify bridging vein injury + - Lacerations are characterized by fluid clefts with hematocrit levels + - **FLAIR**: Very sensitive for detection of small subdural collections + - **SWI/T2* GRE**: Detects small ICH ± retinal hemorrhages + - SWI is ~ 50% sensitive & 100% specific for retinal hemorrhage + - Best sequence for detecting & characterizing bridging vein injury + - **T1WI C+**: Enhancing membranes best sign of chronic SDH + - **MRA**: Proximal vascular correlate (dissection, spasm) rarely shown in association with parenchymal injury + - **MRS**: Will show ↓ NAA, ↑ lactate in regions of parenchymal injury; may detect injury in normal-appearing regions +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - NECT for acute evaluation + - Sensitive in detection & characterization of fractures + - Sensitive in detection & characterization of most ICH, but small low attenuation subdurals can be missed + - MR after 48-72 hours + - Best exam for characterizing brain injury + - Helpful to further characterize subdural collections (CT & MR are complementary) + - ### Protocol advice + + + - NECT: Multiplanar & 3D reconstructions improve detection of + - Small ICHs + - Fractures (especially with bone algorithm & 3D) + - Consider imaging down to C2 to detect atlantooccipital injuries + - MR: DWI to assess parenchymal injury; FLAIR/SWI sequence to detect subtle SDH/subdural hygroma + - MRA or CTA to evaluate suspected pseudoaneurysm or dissection + +# DIFFERENTIAL DIAGNOSIS + +- ## Accidental Trauma + + + - Appropriate history for degree of injury +- [Benign Macrocrania of Infancy](/document/benign-enlarged-subarachnoid-spaces/3da4fec0-6e87-4bcc-bd66-b4a5d1984f6e) + - Self-limited communicating hydrocephalus + - Prominence of extraaxial spaces → isodense to CSF +- [Mitochondrial Encephalopathies](/document/mitochondrial-encephalopathies/40004435-b768-4baf-a31e-651f8a174fe2) + - May cause atrophy with subdural collections + - Glutaric acidurias (types I & II), Menkes syndrome + - Rare diseases with preexisting neurologic symptoms +- [Overshunting](/document/csf-shunts-and-complications/1027d634-92ff-47c1-8266-a7fc3acd1529) + - "Passive" subdurals can develop from ↓ volume associated with CSF shunting +- [Subdural Empyema](/document/empyema/30b1f367-f047-4664-b34b-69b2d13867e0) + - Febrile, sinusitis, meningitis +- ## Bleeding Disorders + + + - von Willebrand, thrombocytopenia + - Intracranial bleeding with minor trauma + +# PATHOLOGY + +- ## General Features + + + - ↑ vulnerability in infants due to + - Large head:body ratio + weak neck muscles + - Developing brain has less structural integrity prior to myelination, greater susceptibility to injury + - 85% of fatal child abuse victims have evidence of impact head injury at postmortem examination + - Retinal hemorrhage in ~ 75% (50-100% in literature) + - Much less common in accidental head trauma (~ 6%) + - Retroclival collections can be seen in ~ 30% of AHT victims + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Discordance between stated history & degree of injury + - Attempt by perpetrator to minimize suspicion + - "Killer couch": Severe injuries blamed on infant rolling off couch onto floor by perpetrator + - Frequently in infants too young to roll over at all + - Unprovoked seizures & apnea raise suspicion for AHT + - ### Other signs/symptoms + + + - Poor feeding, vomiting, irritability, seizures, lethargy, coma, apnea + - Retinal hemorrhage + - Can be missed on cursory exam + - Can be seen in glutaric acidurias + - Cause of death in 80% of fatalities is brain swelling + - Severe hypoxic ischemic encephalopathy > diffuse axonal injury + - ### Clinical profile + + + - Perpetrators are often direct caretakers: Parents, babysitters, mother's boyfriend + - Developmentally delayed, "colicky," premature or low-birth-weight infants at higher risk + - Psychosocial stressors & poor coping mechanisms in family environment +- ## Demographics + + + - Most common from 1-6 months of age + - 17-25:100,000 annual incidence + - Almost certainly underreported + - #1 cause of brain injury death in children < 2 years of age + - M > F +- ## Natural History & Prognosis + + + - Mortality rate: 20-25% + - High rates of impairment for survivors + - Psychomotor delay, epilepsy, visual impairment, cognitive/behavioral disorders +- ## Treatment + + + - Notification of local Child Protection Agency + - Mandated in USA/Canada/Australia/some European countries + - Multidisciplinary child abuse & neglect team intervention + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - Avoid temptation to precisely time ICH + - Accurate descriptions & detailed report are most helpful +- ## Reporting Tips + + + - Avoid use of vague, oblique, obscuring language in reports + - Can hamper care of child & legal investigation + - May ↑ likelihood of interpretation being challenged in legal proceedings + - Avoid speculation regarding timing & specific source of injury in report + - Appropriate to raise concern for abusive head injury + - More can always be said about timing & mechanism in legal setting, but once statement is made in report, it is difficult to retract + + cf586261-2109-4438-b175-9f1754ef6b56 + +## References + +# Selected References + +1. [Ferguson NM et al: Magnetic resonance imaging findings in infants with severe traumatic brain injury and associations with abusive head trauma. Children (Basel). 9(7), 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35884076%5Bpmid%5D) +1. [Orman G et al: An in-depth analysis of brain and spine neuroimaging in children with abusive head trauma: beyond the classic imaging findings. AJNR Am J Neuroradiol. 43(5):764-8, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35393363%5Bpmid%5D) +1. [Bhatia A et al: Neuroimaging of retinal hemorrhage utilizing adjunct orbital susceptibility-weighted imaging. Pediatr Radiol. 51(6):991-6, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33710408%5Bpmid%5D) +1. [Dias MS et al: Neuroradiologic timing of intracranial hemorrhage in abusive head trauma. Pediatr Radiol. 51(6):911-7, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33999236%5Bpmid%5D) +1. [Oates AJ et al: Parenchymal brain injuries in abusive head trauma. Pediatr Radiol. 51(6):898-910, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33638693%5Bpmid%5D) +1. [Sidpra J et al: Abusive head trauma: neuroimaging mimics and diagnostic complexities. Pediatr Radiol. 51(6):947-65, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33999237%5Bpmid%5D) +1. [Vilanilam GK et al: Venous injury in pediatric abusive head trauma: a pictorial review. Pediatr Radiol. 51(6):918-26, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33884464%5Bpmid%5D) +1. [Kralik SF et al: Black bone MRI with 3D reconstruction for the detection of skull fractures in children with suspected abusive head trauma. Neuroradiology. 61(1):81-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30406272%5Bpmid%5D) +1. [Mankad K et al: The neuroimaging mimics of abusive head trauma. Eur J Paediatr Neurol. 23(1):19-30, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30527893%5Bpmid%5D) +1. [Silverman LB et al: Cytotoxic edema in pediatric abusive head trauma: adopting a common nomenclature. J Neuroimaging. 29(2):272-3, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30623511%5Bpmid%5D) +1. [Thamburaj K et al: Susceptibility-weighted imaging of retinal hemorrhages in abusive head trauma. Pediatr Radiol. 49(2):210-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30392163%5Bpmid%5D) +1. [Wittschieber D et al: Understanding subdural collections in pediatric abusive head trauma. AJNR Am J Neuroradiol. 40(3):388-95, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30523144%5Bpmid%5D) +1. [Choudhary AK et al: Consensus statement on abusive head trauma in infants and young children. Pediatr Radiol. 48(8):1048-65, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29796797%5Bpmid%5D) +1. [Gencturk M et al: Various cranial and orbital imaging findings in pediatric abusive and non-abusive head trauma, and relation to outcomes. Clin Neuroradiol. 29(2):253-61, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29362831%5Bpmid%5D) +1. [Ronning MM et al: Parasagittal vertex clots on head CT in infants with subdural hemorrhage as a predictor for abusive head trauma. Pediatr Radiol. 48(13):1915-23, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30187091%5Bpmid%5D) +1. [Teixeira SR et al: Ocular and intracranial MR imaging findings in abusive head trauma. Top Magn Reson Imaging. 27(6):503-14, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30516697%5Bpmid%5D) +1. [Kralik SF et al: Radiologic head CT interpretation errors in pediatric abusive and non-abusive head trauma patients. Pediatr Radiol. 47(8):942-51, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28497263%5Bpmid%5D) +1. [Wong AM et al: Arterial spin-labeling perfusion imaging of children with subdural hemorrhage: perfusion abnormalities in abusive head trauma. J Neuroradiol. 44(4):281-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28341000%5Bpmid%5D) +1. [Wright JN: CNS Injuries in abusive head trauma. AJR Am J Roentgenol. 28:1-11, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28245144%5Bpmid%5D) +1. [Cramer JA et al: Limitations of T2*-gradient recalled-echo and susceptibility-weighted imaging in characterizing chronic subdural hemorrhage in infant survivors of abusive head trauma. AJNR Am J Neuroradiol. 37(9):1752-6, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27032973%5Bpmid%5D) +1. [Girard N et al: Neuroimaging differential diagnoses to abusive head trauma. Pediatr Radiol. 46(5):603-14, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26718196%5Bpmid%5D) +1. [Palifka LA et al: Parenchymal brain laceration as a predictor of abusive head trauma. AJNR Am J Neuroradiol. 37(1):163-8, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26471745%5Bpmid%5D) +1. [Choudhary AK et al: Venous injury in abusive head trauma. Pediatr Radiol. 45(12):1803-13, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26150078%5Bpmid%5D) +1. [Cowley LE et al: Validation of a prediction tool for abusive head trauma. Pediatrics. 136(2):290-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26216332%5Bpmid%5D) +1. [Hahnemann ML et al: Imaging of bridging vein thrombosis in infants with abusive head trauma: the "tadpole sign". Eur Radiol. 25(2):299-305, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25274619%5Bpmid%5D) +1. [Rambaud C: Bridging veins and autopsy findings in abusive head trauma. Pediatr Radiol. 45(8):1126-31, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25698365%5Bpmid%5D) +1. [Wittschieber D et al: Subdural hygromas in abusive head trauma: pathogenesis, diagnosis, and forensic implications. AJNR Am J Neuroradiol. 36(3):432-9, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=24948499%5Bpmid%5D) +1. [Adamsbaum C et al: Dating the abusive head trauma episode and perpetrator statements: key points for imaging. Pediatr Radiol. 44 Suppl 4:S578-88, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25501730%5Bpmid%5D) +1. [Binenbaum G et al: The eye in child abuse: key points on retinal hemorrhages and abusive head trauma. Pediatr Radiol. 44 Suppl 4:S571-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25501729%5Bpmid%5D) +1. [Case ME: Distinguishing accidental from inflicted head trauma at autopsy. Pediatr Radiol. 44 Suppl 4:S632-40, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25501735%5Bpmid%5D) +1. [Nadarasa J et al: Update on injury mechanisms in abusive head trauma--shaken baby syndrome. Pediatr Radiol. 44 Suppl 4:S565-70, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25501728%5Bpmid%5D) +1. [Roach JP et al: Head injury pattern in children can help differentiate accidental from non-accidental trauma. Pediatr Surg Int. 30(11):1103-6, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25252922%5Bpmid%5D) +1. [Vázquez E et al: Imaging abusive head trauma: why use both computed tomography and magnetic resonance imaging? Pediatr Radiol. 44 Suppl 4:S589-603, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25501731%5Bpmid%5D) +1. [Piteau SJ et al: Clinical and radiographic characteristics associated with abusive and nonabusive head trauma: a systematic review. Pediatrics. 130(2):315-23, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22778309%5Bpmid%5D) +1. [Barnes PD: Imaging of nonaccidental injury and the mimics: issues and controversies in the era of evidence-based medicine. Radiol Clin North Am. 49(1):205-29, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21111136%5Bpmid%5D) +1. [Ashwal S et al: Advanced neuroimaging in children with nonaccidental trauma. Dev Neurosci. 32(5-6):343-60, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20938158%5Bpmid%5D) +1. [Hedlund GL et al: Neuroimaging of abusive head trauma. Forensic Sci Med Pathol. 5(4):280-90, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=20012715%5Bpmid%5D) + +## Differential diagnosis + +### Macrocrania/Macrocephaly +DDX:f2d6806c-a267-4f64-ba16-b0fa89e229b6 + +### Microcephaly +DDX:6083739e-ec7b-48ad-9b34-80434c3142f2 + +### Microcephaly +DDX:17ae265f-b471-4ca0-bba4-75b73a9d76f6 + +### Ventriculomegaly +DDX:da3780c6-b627-47c3-912d-6f4c452a1acd + +## Anatomy + +### Visual Network +Brain/ANATOMY:404625d9-3125-4923-9f9d-53d0f81c3542 + +### Embryology and Anatomy of the Brain +Obstetrics/ANATOMY:bf20450d-2629-4795-98e7-7788b665ad3c + +### Embryology and Anatomy of Brain +Ultrasound/ANATOMY:8fb2f541-799d-479f-b000-ab54f216199a + +## Cases + +- {'cases': [{'authors': [{'key': '838e1722-2479-4fbd-a5fe-d965980a1a2c', 'value': 'Blaise V. Jones, MD'}], 'caseVersionId': 'fade88d5-75e7-42b2-8495-32157314e199', 'description': "Initial CT images (#1, 2) show loss of gray-white differentiation and swelling throughout the right cerebral hemisphere, along with subdural blood (arrows, #1,2). No fractures were identified. \n\nFollow-up CT study less than 24 hours later (#3) showed progression of right hemisphere swelling, with transtentorial, subfalcine, and uncal herniation. Gray-white differentiation is accentuated on this exam, but in an inverted configuration, with the white matter being more dense than the edematous gray matter. This is in contrast to the normal relationship demonstrated in the left hemisphere. This is the "reversal sign", indicating a severe and irreversible insult.\n\n"Killer couch" is a term sardonically used by health care professionals in reference to the frequent explanation of the injury leading an abused infant to medical care. The child is frequently said to have rolled off a sofa onto a carpeted or wooden floor, typically unwitnessed. The abuser's attempt to minimize the trauma is often the first clue to the correct diagnosis of inflicted injury, as it is so disproportionate to the documented degree of injury.", 'history': 'Fell off a sofa. Now unresponsive.', 'imagePoolId': 'dafd3e2b-c935-4e57-bc98-7d7273efa5ac', 'name': 'Killer couch, reversal sign', 'teachingPoint': None, 'demographics': '18 Months old female'}, {'authors': [{'key': '815f3e98-b5da-43c7-8f99-d3db52947320', 'value': 'Hank Baskin, MD'}], 'caseVersionId': '224f3ea3-1e84-4150-a090-b28e8e096129', 'description': 'Axial NECT images (#1-3) show abundant extraaxial hemorrhage (straight arrows) scattered over the bilateral hemispheres and within the interhemispheric fissure. There is also diffuse low attenuation of the cerebral hemispheres from generalized cerebral edema (black open arrows) which, juxtaposed by the more normal attenuation cerebellum (white open arrows, #1), creates the "CT reversal sign." Edema also causes effacement of the lateral ventricles (white curved arrows, #2) and perimesencephalic cisterns (black curved arrow, #2).', 'history': 'Patient was brought to emergency department because of seizure and listlessness; there is generalized cerebral edema and diffuse extraaxial hemorrhage from abusive head trauma; the child died 24 hours later.', 'imagePoolId': '00693687-d787-4c62-b446-88c55fe71d0d', 'name': 'Diffuse cerebral edema blood CT brain death', 'teachingPoint': None, 'demographics': '7 Weeks old male'}, {'authors': [{'key': '838e1722-2479-4fbd-a5fe-d965980a1a2c', 'value': 'Blaise V. Jones, MD'}], 'caseVersionId': '11b1d3de-688f-46d6-8eed-929c48dde54d', 'description': 'This case illustrates the CT and MR appearance of subacute to chronic subdural hematomas due to child abuse.\n\nInitial CT image (#1) shows subdural collections over both frontal lobes that are higher in attenuation that the underlying CSF in the subarachnoid space (arrows). This is further illustrated by the signal difference in the subdural collections on proton-density T2WI (#2) from an MR obtained the next day. Note the hyperintense signal in the subdural space posteriorly on T1WI and the hyperintense hemorrhagic staining in the left parietal cortex and T1WI (arrows, #3, 4). It is tempting to interpret these findings as reflecting more acute blood posteriorly and chronic subdurals anteriorly, but this is just as likely to represent pooling of blood products posteriorly such that the anterior portions of the collections only have xanthochromic CSF.\n\nDWI and ADC maps (#5, 6) show that there is significant injury to the posterior parietal and occipital lobes. \n\nMR is invaluable in showing the extent of brain injury in cases of child abuse.', 'history': '"Fell out of bed."', 'imagePoolId': '7d55e0da-c1df-4bd9-9be6-c9b0973a098a', 'name': 'Subdurals', 'teachingPoint': None, 'demographics': '3 Months old '}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '4e001e30-c901-4297-9ce6-e2406f4aceaa', 'description': 'MR can be very helpful in detecting mixed-age subdural hematomas. Here axial T1 weighted MR scans (#1-2) show high signal intensity extra-axial fluid over the right hemisphere. There is a hypointense collection on the left that may represent expanded subarachnoid space. T2 weighted scans (#3-4) show the right subdural collection is mostly hyperintense, but the more posterior portion (curved arrows) appears hypointense, indicated more recent hemorrhage. Other scattered foci of recent hemorrhage (open arrows) can be seen. At least two separate episodes of hemorrhage are present. Note scalp swelling (#4).', 'history': '"Fell off the couch." Nonaccidental trauma suspected.', 'imagePoolId': '4f28cfd1-8a22-4de3-a92b-469a0622f620', 'name': 'Classic mixed-age SDHs', 'teachingPoint': None, 'demographics': '3 Months old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '957a51bc-7a33-4558-9358-8cc3767a3392', 'description': 'Axial NECT scans show bifrontal low density extra-axial fluid collections (arrows). Note evidence for more recent, higher density hemorrhage (curved arrows) within the hypodense subdurals (SDHs). The more chronic, low density SDH extends along the interhemispheric fissure (open arrows). The presence of mixed-age SDHs, together with interhemispheric extension, strongly suggests nonaccidental trauma.', 'history': 'Suspected "shaken baby" syndrome.', 'imagePoolId': 'bc6acdb7-8626-404f-9894-1f693635b865', 'name': 'Mixed SDHs', 'teachingPoint': None}, {'authors': [{'key': 'e8af6d26-3aad-47c9-9083-5128aab09af2', 'value': 'Susan I. Blaser, MD, FRCPC'}], 'caseVersionId': 'd11c767b-2d5b-4a35-80d5-c8cf7739b3a2', 'description': 'Rib series demonstrates multiple healing rib fractures (#1). NECT obtained after fractures found (#2) demonstrates large subdural fluid collection (arrow), diffuse hypodensity of brain substance and significant shift of midline structures. Note thin interhemispheric subdural bleed (curved arrows). Nuclear brain perfusion study (#3) demonstrates total lack of cerebral or cerebellar perfusion. Nonaccidental trauma remains the most common cause of traumatic infant death.', 'history': 'Found in coma several days after minor fall.', 'imagePoolId': 'be0460c7-7fa9-46a3-bda9-e0736f3140eb', 'name': 'Severe', 'teachingPoint': None, 'demographics': '4 Months old female'}, {'authors': [{'key': 'c313fa7b-5bff-4b39-a8cd-dcf06aa6a69d', 'value': 'A. Carlson Merrow, Jr., MD, FAAP'}], 'caseVersionId': 'f3b8d15f-f11a-493f-9eba-f1e4effcbe6a', 'description': 'Axial NECT images of the brain (#1-2) show a mixed hyper- and hypodense subdural collection overlying the right cerebral convexity (straight black arrows). There is generalized sulcal effacement (open white arrow) as compared to normal left cerebral sulci (curved white arrow, #1). Note that the right lateral ventricle is completely effaced (curved black arrow, #1). There is moderate to marked leftward midline shift (straight white arrow, #2). Axial T2WI (#3) and FLAIR (#4) MR images obtained 3 days later (after craniotomy) show right cerebral edema with increased cortical/subcortical signal and gyral swelling/sulcal effacement (open white arrows). A tiny subdural hematoma (straight white arrow) remains. Note the largely normal left cerebral hemisphere (open black arrow). On axial DWI MR (#5), there is restricted diffusion of the entire right cerebral hemisphere (curved arrow), confirmed on the ADC map (curved arrow, #6). This patient suffered child abuse with an extensive right cerebral injury and subdural hematoma.', 'history': 'Patient not moving left arm.', 'imagePoolId': '0800593d-f847-40e5-b1f9-4aa3221e2027', 'name': 'Subdural, infarction', 'teachingPoint': None, 'demographics': '12 Months old female'}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': '838e1722-2479-4fbd-a5fe-d965980a1a2c', 'value': 'Blaise V. Jones, MD'}], 'caseVersionId': '3f0a013a-e4a5-4bca-be4b-6340362e688e', 'description': 'This case illustrates an unusual complication of child abuse; an aneurysm of the anterior cerebral artery (pericallosal artery).\n\nNote the small focus of high attenuation in the interhemispheric fissure anteriorly (curved arrow, #2) in this child who presented after abusive head injury causing multiple extra-axial hemorrhages (#1). Follow-up CT (#3, 4) shows the development of post-traumatic hydrocephalus and a calcified mass at the site of the prior density (arrow, #4). \n\nCTA (#5, 6) shows that this is a partially calcified aneurysm (arrows), confirmed on arteriography (curved arrow, # 7) to be at the junction of the pericallosal and callosal-marginal arteries. The aneurysm was coiled (curved arrow, #8) and sagittal MR before (#9) and after (#10) show elimination of the aneurysm (curved arrow, #10).', 'history': "Beaten by mother's boyfriend.", 'imagePoolId': 'a600d880-39df-4121-80a5-51d3925f66d2', 'name': 'Aneurysm', 'teachingPoint': None, 'demographics': '4 Months old male'}], 'caseType': 'variant', 'name': 'VARIANT'} + + +## Images + + +### Selected Images + +![Axial NECT in a 9-week-old with seizures shows bilateral mixed attenuation subdural fluid collections with predominantly low attenuation anteriorly & high attenuation posteriorly , consistent with a hematohygroma. This should not be described as "acute on chronic" subdural hemorrhage.](images/app.statdx.com_image_thumbnail_795c997a-dde8-471f-9325-bac10af10458_annotated_true_size_900_quality_90_6e9fdd986dcd76130b5178638f05266054a50b24.jpg) +*Axial NECT in a 9-week-old with seizures shows bilateral mixed attenuation subdural fluid collections with predominantly low attenuation anteriorly & high attenuation posteriorly , consistent with a hematohygroma. This should not be described as "acute on chronic" subdural hemorrhage.* + +![Axial NECT in a 9-week-old with seizures shows bilateral mixed attenuation subdural fluid collections with predominantly low attenuation anteriorly & high attenuation posteriorly , consistent with a hematohygroma. This should not be described as "acute on chronic" subdural hemorrhage.](images/app.statdx.com_image_thumbnail_795c997a-dde8-471f-9325-bac10af10458_size_174_quality_85_12c4ddf2bca30b38a992318ededf2f7ce65ef234.jpg) +*Axial NECT in a 9-week-old with seizures shows bilateral mixed attenuation subdural fluid collections with predominantly low attenuation anteriorly & high attenuation posteriorly , consistent with a hematohygroma. This should not be described as "acute on chronic" subdural hemorrhage.* + +![Coronal NECT in the same patient shows the bilateral subdural collections as well as globular areas of hemorrhage in the parasagittal regions, consistent with bridging vein injuries/avulsions.](images/app.statdx.com_image_thumbnail_4726f7e5-8935-4948-9502-c6da47cd4dd9_annotated_true_size_900_quality_90_a321048f6f5a6eeadf2f6e76a9a1d87f4456594a.jpg) +*Coronal NECT in the same patient shows the bilateral subdural collections as well as globular areas of hemorrhage in the parasagittal regions, consistent with bridging vein injuries/avulsions.* + +![Axial DWI MR in the same patient performed 3 days after presentation shows asymmetric (right > left) diffusion restriction , consistent with brain parenchymal injury. MR is the best exam for detection & characterization of brain injury in accidental head trauma.](images/app.statdx.com_image_thumbnail_a8bc82a9-f51e-449d-8f5f-1ef6b2ca11f9_annotated_true_size_900_quality_90_475d9593504d2444882cb09fc93069d428df82a7.jpg) +*Axial DWI MR in the same patient performed 3 days after presentation shows asymmetric (right > left) diffusion restriction , consistent with brain parenchymal injury. MR is the best exam for detection & characterization of brain injury in accidental head trauma.* + +![Coronal SSFSE in the same patient 6 weeks later shows a membrane separating loculated hyperintense & hypointense subdural components, a finding that suggests some component of chronicity.](images/app.statdx.com_image_thumbnail_4518bd21-5c12-4908-ab69-ba42fb99a0a1_annotated_true_size_900_quality_90_296466084e58ca3812b28f6d47134c6f6528193e.jpg) +*Coronal SSFSE in the same patient 6 weeks later shows a membrane separating loculated hyperintense & hypointense subdural components, a finding that suggests some component of chronicity.* + +![Axial DWI MR in a 2-month-old with unexplained altered mental status & seizure shows nearly symmetric diffusion restriction in the bilateral parietooccipital regions.](images/app.statdx.com_image_thumbnail_a0653586-fe20-4448-a80c-d09252e065fd_annotated_true_size_900_quality_90_db08a6926ad60f25acd85c5a200be26044cff064.jpg) +*Axial DWI MR in a 2-month-old with unexplained altered mental status & seizure shows nearly symmetric diffusion restriction in the bilateral parietooccipital regions.* + +![Axial T2 MR in the same patient shows bilateral clefts with hematocrit levels, consistent with lacerations . Also note the bilateral subdural hemorrhages . This constellation of parenchymal injury, brain lacerations, & bilateral subdural hemorrhages is highly suspicious for abusive head trauma (AHT).](images/app.statdx.com_image_thumbnail_8f199ca6-b3c7-47a5-9441-cb96830958a9_annotated_true_size_900_quality_90_7728033edd0cde9fd48380f36d8a506fc859da24.jpg) +*Axial T2 MR in the same patient shows bilateral clefts with hematocrit levels, consistent with lacerations . Also note the bilateral subdural hemorrhages . This constellation of parenchymal injury, brain lacerations, & bilateral subdural hemorrhages is highly suspicious for abusive head trauma (AHT).* + +![Axial NECT in a 6-week-old with AHT shows hypodense subdural collections & left frontal arachnoid hemorrhage . ↓ attenuation throughout the cerebrum with loss of gray matter-white matter differentiation is consistent with injury. Compare to the normal cerebellum .](images/app.statdx.com_image_thumbnail_4b23e773-9832-4640-b979-5ffe9fc73d9c_annotated_true_size_900_quality_90_0e379e337a1709897385680f447c04ca78b643af.jpg) +*Axial NECT in a 6-week-old with AHT shows hypodense subdural collections & left frontal arachnoid hemorrhage . ↓ attenuation throughout the cerebrum with loss of gray matter-white matter differentiation is consistent with injury. Compare to the normal cerebellum .* + +![Axial SWI in a 3-month-old with AHT shows thin areas of ↓ signal , consistent with bilateral retinal hemorrhages. MR is insensitive for detection of retinal hemorrhages, but they should be reported when present.](images/app.statdx.com_image_thumbnail_f75722d5-ae3c-49db-83df-6d1eee2ab651_annotated_true_size_900_quality_90_c0b941357807146dd25c18262cbcb96d0dcbb2c3.jpg) +*Axial SWI in a 3-month-old with AHT shows thin areas of ↓ signal , consistent with bilateral retinal hemorrhages. MR is insensitive for detection of retinal hemorrhages, but they should be reported when present.* + +![Axial NECT in a 2-month-old with AHT shows subdural hemorrhage over the left frontal lobe & along the falx. Note the extensive areas of ↓ attenuation & loss of gray matter-white matter differentiation with significant left-to-right midline shift.](images/app.statdx.com_image_thumbnail_4ae0f2cf-ac95-46ac-8e1b-de306322e10a_annotated_true_size_900_quality_90_25b1b0e216bd9f869a4dea707091c191d6743310.jpg) +*Axial NECT in a 2-month-old with AHT shows subdural hemorrhage over the left frontal lobe & along the falx. Note the extensive areas of ↓ attenuation & loss of gray matter-white matter differentiation with significant left-to-right midline shift.* + +![Coronal FLAIR MR in the same patient 7 years later shows development of extensive areas of cystic & noncystic encephalomalacia affecting the left > right cerebral hemispheres.](images/app.statdx.com_image_thumbnail_ab314a5c-786c-40cb-ae40-1d7a31d1d957_annotated_true_size_900_quality_90_0f728e14d82299bc56b2e2883339b8c4f888b18b.jpg) +*Coronal FLAIR MR in the same patient 7 years later shows development of extensive areas of cystic & noncystic encephalomalacia affecting the left > right cerebral hemispheres.* + + +### Additional Images + +![Posterior oblique view of a 3D NECT in a 9-week-old who "fell off the couch" shows multiple complex skull fractures , including a displaced right parietal fracture . 3D renderings are helpful in improving the detection & characterization of skull fractures.](images/app.statdx.com_image_thumbnail_9eda7872-948d-4d30-9a92-edfa8cbeb9f7_annotated_true_size_900_quality_90_8c3e482701819878cb8a263dc1793aa9b69c1665.jpg) +*Posterior oblique view of a 3D NECT in a 9-week-old who "fell off the couch" shows multiple complex skull fractures , including a displaced right parietal fracture . 3D renderings are helpful in improving the detection & characterization of skull fractures.* + +![Axial NECT in the same 9-week-old shows a right subdural hematoma (SDH) & right opercular parenchymal laceration with significant midline shift & sulcal effacement. Parenchymal lacerations are seen in 10-15% of AHT patients.](images/app.statdx.com_image_thumbnail_98197aa6-1a01-4552-9200-d31bcd3696b6_annotated_true_size_900_quality_90_0af48008d9d3b6f8f6d1ce4a317c2e2c10ba6036.jpg) +*Axial NECT in the same 9-week-old shows a right subdural hematoma (SDH) & right opercular parenchymal laceration with significant midline shift & sulcal effacement. Parenchymal lacerations are seen in 10-15% of AHT patients.* + +![Axial NECT in a 4-month-old boy with seizure activity shows multiple bilateral foci of low attenuation with loss of cortical differentiation as well as a left frontal SDH . There was no fracture, making these findings highly concerning for the shaking type of AHT.](images/app.statdx.com_image_thumbnail_988a7133-57fa-4561-8c47-537c8de4e17d_annotated_true_size_900_quality_90_e353b0ea7fe5d0a0a53d1f306bb07dd911e3a27b.jpg) +*Axial NECT in a 4-month-old boy with seizure activity shows multiple bilateral foci of low attenuation with loss of cortical differentiation as well as a left frontal SDH . There was no fracture, making these findings highly concerning for the shaking type of AHT.* + +![Axial DWI MR in a 2-month-old boy with AHT shows areas of diffusion restriction in the right frontal lobe & bilateral parietal lobes, consistent with parenchymal injury. MR is the most sensitive examination for parenchymal injury.](images/app.statdx.com_image_thumbnail_bc56f7ad-e532-4bd9-9535-69ccd5d72d4e_annotated_true_size_900_quality_90_dcb0aeb652ce540141a6e7604fe341d9037b1c22.jpg) +*Axial DWI MR in a 2-month-old boy with AHT shows areas of diffusion restriction in the right frontal lobe & bilateral parietal lobes, consistent with parenchymal injury. MR is the most sensitive examination for parenchymal injury.* + +![Coronal NECT in a 3-month-old with ABT shows focal areas of parasagittal hemorrhage . The lesion on the left has a lollipop or pollywog appearance, typical for cortical vein avulsion, a finding that is fairly specific for AHT.](images/app.statdx.com_image_thumbnail_9324470f-ac2e-47ee-94da-11247ee8cf2d_annotated_true_size_900_quality_90_23a7ee0afe75c1e7855c02a66a5177cda8e0bd81.jpg) +*Coronal NECT in a 3-month-old with ABT shows focal areas of parasagittal hemorrhage . The lesion on the left has a lollipop or pollywog appearance, typical for cortical vein avulsion, a finding that is fairly specific for AHT.* + +![Initial coronal NECT in a 1-month-old girl with AHT shows bilateral paramedian extraaxial hemorrhages , a characteristic finding in AHT attributed to bridging vein injury & thrombosis. In all, 40-50% of AHT cases show evidence of bridging vein injury.](images/app.statdx.com_image_thumbnail_9e544eaf-35ec-499b-ac37-a1f9cd6e5083_annotated_true_size_900_quality_90_5c07e0c91e136c6d761e76a657159cac713a3b42.jpg) +*Initial coronal NECT in a 1-month-old girl with AHT shows bilateral paramedian extraaxial hemorrhages , a characteristic finding in AHT attributed to bridging vein injury & thrombosis. In all, 40-50% of AHT cases show evidence of bridging vein injury.* + +![Axial SWI in a 6-week-old shows subdural & subarachnoid hemorrhage as well as an evolving left frontal lobe laceration with hemorrhage level, a constellation of findings very suspicious of AHT.](images/app.statdx.com_image_thumbnail_75222a16-5260-4237-8d33-0e25c51beda3_annotated_true_size_900_quality_90_df178eca11d567e0414b2ee7f6551a19355da679.jpg) +*Axial SWI in a 6-week-old shows subdural & subarachnoid hemorrhage as well as an evolving left frontal lobe laceration with hemorrhage level, a constellation of findings very suspicious of AHT.* + +![Axial T2 SWI MR in a 4-month-old with AHT shows bilateral retinal hemorrhages . Although SWI is the most sensitive imaging sequence for retinal hemorrhages, it is insensitive relative to a funduscopic exam. A normal MR appearance of the globes does not exclude retinal hemorrhages.](images/app.statdx.com_image_thumbnail_ce7e0b82-699c-4d84-a8e5-da8e56362460_annotated_true_size_900_quality_90_cc8a12f8eab7724620995b6ea34f478ca38084bb.jpg) +*Axial T2 SWI MR in a 4-month-old with AHT shows bilateral retinal hemorrhages . Although SWI is the most sensitive imaging sequence for retinal hemorrhages, it is insensitive relative to a funduscopic exam. A normal MR appearance of the globes does not exclude retinal hemorrhages.* + +![Initial axial NECT in the same patient shows SDH layering along the left tentorium with a slight ↑ in bifrontal hypodense extraaxial spaces .](images/app.statdx.com_image_thumbnail_df90fab6-3dfc-4fab-bfe6-90011e080f11_annotated_true_size_900_quality_90_66235ad5b6a9c862ec9539bfb2db8aab681e38e3.jpg) +*Initial axial NECT in the same patient shows SDH layering along the left tentorium with a slight ↑ in bifrontal hypodense extraaxial spaces .* + +![Axial NECT in the same patient 3 days later shows more conspicuous layering of a SDH in the posterior fossa. Also note the significant enlargement of the low-attenuation extraaxial collections , most of which are nonhemorrhagic & isodense to CSF, consistent with enlarging subdural hygromas (SDHy). As illustrated by this case, it is impossible to say that a predominantly nonhemorrhagic collection with a small amount of acute SDH is an "acute-on-chronic" SDH. Rather, when such an appearance is encountered on initial imaging, it is more likely an acute mixed SDH/SDHy.](images/app.statdx.com_image_thumbnail_f0756f8b-32c3-4750-82a6-712b3c9a722b_annotated_true_size_900_quality_90_6882d028632528fcde2a6c3795ea232bb79aea71.jpg) +*Axial NECT in the same patient 3 days later shows more conspicuous layering of a SDH in the posterior fossa. Also note the significant enlargement of the low-attenuation extraaxial collections , most of which are nonhemorrhagic & isodense to CSF, consistent with enlarging subdural hygromas (SDHy). As illustrated by this case, it is impossible to say that a predominantly nonhemorrhagic collection with a small amount of acute SDH is an "acute-on-chronic" SDH. Rather, when such an appearance is encountered on initial imaging, it is more likely an acute mixed SDH/SDHy.* + +![Axial T2WI in the same 1-month-old girl with AHT on day 2 shows significant enlargement of the extraaxial collections , most of which is nonhemorrhagic & isointense to CSF, consistent with a growing SDHy.](images/app.statdx.com_image_thumbnail_9ce2017f-5940-4f24-94b6-21ffe372bf13_annotated_true_size_900_quality_90_8c29910b0320f1583ddd8269f6b0b3db704a8622.jpg) +*Axial T2WI in the same 1-month-old girl with AHT on day 2 shows significant enlargement of the extraaxial collections , most of which is nonhemorrhagic & isointense to CSF, consistent with a growing SDHy.* + +![Axial PD MR shows bilateral SDHs after a shaking injury. Note how this sequence clearly differentiates between the subdural collections & the underlying subarachnoid space .](images/app.statdx.com_image_thumbnail_3486be2b-7ed0-44b0-b738-ead582a3b381_annotated_true_size_900_quality_90_5ed7c72e049fe6b6a5deb18c7001d01ce986ab77.jpg) +*Axial PD MR shows bilateral SDHs after a shaking injury. Note how this sequence clearly differentiates between the subdural collections & the underlying subarachnoid space .* + +![Axial DWI MR in the same patient shows a small focus of parenchymal diffusion restriction in the left anterior operculum, consistent with parenchymal injury, which was not evident on CT. MR is the most sensitive test for parenchymal injury in the setting of AHT.](images/app.statdx.com_image_thumbnail_9f990ddd-914f-48f7-8e50-dfa719f11a5b_annotated_true_size_900_quality_90_283244d13a2a943d70ce85634dddb4d4a7539c2a.jpg) +*Axial DWI MR in the same patient shows a small focus of parenchymal diffusion restriction in the left anterior operculum, consistent with parenchymal injury, which was not evident on CT. MR is the most sensitive test for parenchymal injury in the setting of AHT.* + +![Axial NECT in a 6-month-old girl with AHT shows high attenuation in the posterior right globe, consistent with retinal hemorrhage. Retinal hemorrhage is rarely seen on CT but strongly suggests nonaccidental trauma (NAT).](images/app.statdx.com_image_thumbnail_2b60892a-b509-4d20-850a-ea9c77962a81_annotated_true_size_900_quality_90_dbae4f5636412ca9488baffbc7b817d574492414.jpg) +*Axial NECT in a 6-month-old girl with AHT shows high attenuation in the posterior right globe, consistent with retinal hemorrhage. Retinal hemorrhage is rarely seen on CT but strongly suggests nonaccidental trauma (NAT).* + +![Lateral radiograph shows multiple fractures that cross sutures & are diastatic. There is also coronal suture diastasis .](images/app.statdx.com_image_thumbnail_ea630675-0029-43b3-97bf-d2c193310286_annotated_true_size_900_quality_90_1ea4447b02be77cf14cc02c461e389d593f61980.jpg) +*Lateral radiograph shows multiple fractures that cross sutures & are diastatic. There is also coronal suture diastasis .* + +![Axial NECT in a 3-month-old presenting with skull fractures shows subdural blood overlying most of the right cerebral hemisphere & exerting mild mass effect on the underlying brain. Blood on either side of the sagittal sinus gives rise to the empty delta sign , a mimic of sinus thrombosis.](images/app.statdx.com_image_thumbnail_7e2ada1a-3e42-4d6e-99a6-5651cf93ce08_annotated_true_size_900_quality_90_26e4cfafb9f7efbf79278fc3412771a2e9d0a73b.jpg) +*Axial NECT in a 3-month-old presenting with skull fractures shows subdural blood overlying most of the right cerebral hemisphere & exerting mild mass effect on the underlying brain. Blood on either side of the sagittal sinus gives rise to the empty delta sign , a mimic of sinus thrombosis.* + +![Coronal T2 MR in a 2-month-old presenting to the emergency department unresponsive shows regions of hyperintensity with loss of gray matter-white matter differentiation in the left temporal lobe & parasagittal regions . Hypointense signal in the right parasagittal cortex reflects hemorrhagic staining.](images/app.statdx.com_image_thumbnail_475c5961-427b-4554-bfef-5d2c2d074cb9_annotated_true_size_900_quality_90_76dea626b5897bff49a3a64fd57c5c44d1251062.jpg) +*Coronal T2 MR in a 2-month-old presenting to the emergency department unresponsive shows regions of hyperintensity with loss of gray matter-white matter differentiation in the left temporal lobe & parasagittal regions . Hypointense signal in the right parasagittal cortex reflects hemorrhagic staining.* + +![Coronal T2 MR in the same child 9 months later shows focal volume loss in both areas of injury , with associated enlargement of the ventricles & interhemispheric fissure.](images/app.statdx.com_image_thumbnail_828cad14-96d6-4587-8c69-fa2eef6718a6_annotated_true_size_900_quality_90_64c1719c0ad0ef45938effd3e5367c7231735e4d.jpg) +*Coronal T2 MR in the same child 9 months later shows focal volume loss in both areas of injury , with associated enlargement of the ventricles & interhemispheric fissure.* + +![Lateral catheter DSA in an infant with AHT shows a posttraumatic pseudoaneurysm at the junction of the pericallosal & callosal-marginal arteries .](images/app.statdx.com_image_thumbnail_ccb52667-4b9e-4859-81d2-14cc9656ec10_annotated_true_size_900_quality_90_63b87028166e53e9b92098231bed098eddb20772.jpg) +*Lateral catheter DSA in an infant with AHT shows a posttraumatic pseudoaneurysm at the junction of the pericallosal & callosal-marginal arteries .* + +![Axial PD MR shows a SDH over the left cerebral hemisphere. Although blood along the falx would be seen, the small volume laterally could be difficult to detect on CT.](images/app.statdx.com_image_thumbnail_c46acb0c-cdaf-42c1-a772-f544929f216e_annotated_true_size_900_quality_90_6bf3ef3956c7cef6de7db536180a03f118c14652.jpg) +*Axial PD MR shows a SDH over the left cerebral hemisphere. Although blood along the falx would be seen, the small volume laterally could be difficult to detect on CT.* + +![Axial NECT shows generally poor gray matter-white differentiation & subdural blood along the tentorium in a 5-month-old with a shaking-induced injury.](images/app.statdx.com_image_thumbnail_15aada4b-fd99-4288-b34e-ed7875df4642_annotated_true_size_900_quality_90_389f56f1069ddbf3069ee9dbf4f6725d72e62caf.jpg) +*Axial NECT shows generally poor gray matter-white differentiation & subdural blood along the tentorium in a 5-month-old with a shaking-induced injury.* + +![Axial NECT in the same child 12 hours later shows the rapid development of multiple regions of cytotoxic edema with effacement of the 3rd ventricle & cisterns.](images/app.statdx.com_image_thumbnail_6b763523-60f4-4863-bc58-8220cc6f8c7b_annotated_true_size_900_quality_90_9d08b99581dde9ba291bb516c6b78f9ef0a8bd5b.jpg) +*Axial NECT in the same child 12 hours later shows the rapid development of multiple regions of cytotoxic edema with effacement of the 3rd ventricle & cisterns.* + diff --git a/docs_md/articles/acute-cerebral-ischemia-infarction_a405285f-aaea-43ca-8dc4-6f8120eaabc1.md b/docs_md/articles/acute-cerebral-ischemia-infarction_a405285f-aaea-43ca-8dc4-6f8120eaabc1.md new file mode 100644 index 0000000..bc6a733 --- /dev/null +++ b/docs_md/articles/acute-cerebral-ischemia-infarction_a405285f-aaea-43ca-8dc4-6f8120eaabc1.md @@ -0,0 +1,531 @@ +--- +title: "Acute Cerebral Ischemia/Infarction" +docid: "a405285f-aaea-43ca-8dc4-6f8120eaabc1" +authors: + - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850" + value: "Anne G. Osborn, MD, FACR" + - key: "095c34cb-da44-4830-98c9-7e1a24bdda5b" + value: "Edward P. Quigley, III, MD, PhD" +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: "Stroke" + slug: "stroke" + treeNodeId: "12307683-f1ff-4823-a7d3-b10b40f9fd82" + - + name: "Cerebral Ischemia and Infarction" + slug: "cerebral-ischemia-and-infarction" + treeNodeId: "51051846-a223-42f7-b626-2a5a26cf6c44" + - + name: "Acute Cerebral Ischemia/Infarction" + slug: "acute-cerebral-ischemiainfarction" + treeNodeId: null +category: "Brain" +cmeTopicId: "5b2b9f3f-8472-4797-99d7-a20ba36317ba" +documentVersionId: "2480a23f-7616-42e8-aeeb-0ad3fc43e710" +imageCount: 42 +lastUpdated: "08/21/20" +pageDescription: "Acute Cerebral Ischemia/Infarction" +pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Cerebral Ischemia and Infarction, Acute Cerebral Ischemia/Infarction" +pageTitle: "Acute Cerebral Ischemia/Infarction | STATdx" +enhancedTitle: "Acute Cerebral Ischemia/Infarction" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Pathology-Based Diagnoses" + - "Stroke" + - "Cerebral Ischemia and Infarction" + - "Acute Cerebral Ischemia/Infarction" +--- +# KEY FACTS + +- ## Terminology + + + - Interrupted blood flow to brain resulting in cerebral ischemia/infarction + - Stroke, brain attack = descriptive terms, not diagnosis +- ## Imaging + + + - Major artery (territorial) infarct + - Generally wedge-shaped; both gray matter (GM) and white matter (WM) involved + - Embolic infarcts + - Often focal/small, at GM-WM interface + - NECT + - Hyperdense vessel = clot (dense MCA sign) + - Loss of GM-WM distinction in first 3 hours (50-70%) + - Insular ribbon sign: GM-WM interface lost + - "Disappearing" basal ganglia sign + - Calcified embolus + - Do not miss this (high risk of recurrent stroke) + - CTA: Excellent for large vessel occlusions (LVOs) + - pCT: CBF/CBV "mismatch" estimates penumbra + - Beware ghost infarct core! + - May exclude patients who would benefit from endovascular treatment + - MR + - Parenchymal ± intraarterial FLAIR hyperintensity + - ↑ intensity on DWI with corresponding ↓ on ADC + - ↓ CBF, variable ↓ CBV on MR perfusion +- ## Top Differential Diagnoses + + + - Hyperdense vessel mimics (normal; polycythemia) + - Parenchymal hypodensity (many nonvascular causes) +- ## Pathology + + + - Severely ischemic core + - CBF < (6-8 cm³)/(100 g/min) + - Peripheral penumbra + - CBF between (10-20 cm³)/(100 g/min) +- ## Clinical Issues + + + - 2nd most common cause of death worldwide + - Most common cause of morbidity in USA + - Thrombectomy = treatment of choice for LVOs + +# TERMINOLOGY + +- ## Synonyms + + + - Stroke and brain attack + - Not diagnosis; terms for sudden onset of neurologic deficit +- ## Definitions + + + - Interrupted blood flow to brain resulting in cerebral ischemia/infarction + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - High signal on DWI + low signal on ADC = reduced diffusivity + - ↓ cerebral blood flow (CBF), variable cerebral blood volume (CBV) on CT perfusion (pCT) or MR perfusion (pMR) + - ### Location + + + - Vascular territory or at border zones (watershed) + - ### Size + + + - Dependent on degree of compromise and collateral circulation + - ### Morphology + + + - Large vessel occlusion (LVO) + - Conforms to arterial territory [most common = middle cerebral artery (MCA)] + - Generally wedge-shaped, involves both gray matter (GM) and white matter (WM) + - Embolic infarcts (often focal, at GM-WM interface) + - Watershed infarcts (border zone between perforating, cortical arteries) +- ## CT Findings + + + - ### NECT + + + - Hyperdense vessel (high specificity, low sensitivity) + - Represents acute thrombus in cerebral vessel(s) + - Hyperdense M1 MCA in 35-50% + - Dot sign: Occluded MCA branches in sylvian fissure (16-17%) + - Loss of GM-WM distinction in first 3 hours (50-70%) + - Obscuration of deep gray nuclei + - "Disappearing" basal ganglia + - Loss of cortical "ribbon" + - Parenchymal hypodensity + - **A**lberta **S**troke **P**rogram **E**arly **C**omputed **T**omographic **S**core (ASPECTS) + - Numerical calculation (1 point subtracted for each affected area) + - Can be automatically generated with artificial intelligence (AI) + - Gyral swelling, sulcal effacement appears between 12-24 hours + - "Hemorrhagic transformation" in 15-45% + - Delayed onset (24-48 hours) most typical + - Can be gross (parenchymal) or petechial + - Calcified embolus (1-2%) + - Round/ovoid hyperdensity in vessel lumen or sulcus + - Calcific valvular disease > cervical atrioventricular septal defect (ASVD) as source + - High risk for recurrent strokes + - ### CECT + + + - Enhancing cortical vessels = slow flow or collateralization + - Absent vessels = occlusion + - Cortical/gyral enhancement after 48-72 hours + - CTA: Identify LVOs, dissections, stenoses, status of collaterals + - pCT + - Shows CBF, CBV, TTP, or MTT + - AI programs provide rapid, easy-to-read, real-time views of brain perfusion + - Select stroke patients with LVOs for thrombectomy + - Ischemic core = volume of area with > 70% ↓ in CBF (rCBF < 0.3) + - Often overestimates initial infarct core → ghost infarct core (GIC) + - GIC = initial core - final infarct > 10 mL + - Common in patients imaged in early time window with fast, complete reperfusion (TICI2b) + - pCT CBF may exclude patients who would benefit from endovascular treatment! +- ## MR Findings + + + - ### T1WI + + + - Early cortical swelling and hypointensity, loss of GM-WM borders + - ### T2WI + + + - Cortical swelling, hyperintensity develops by 12-24 hours + - May normalize 2-3 weeks post ictus (MR "fogging") + - ### FLAIR + + + - Parenchymal hyperintensity appears while other sequences normal + - Intraarterial hyperintensity = sign of major vessel occlusion or slow flow + - **Absence** of FLAIR intravascular hyperintensity associated with future lack of recanalization + - ### T2* GRE + + + - Arterial blooming (thrombosed vessel) ± parenchymal hemorrhage + - May see susceptibility from calcified embolus + - ### DWI + + + - Hyperintense (cytotoxic edema) + - Improves hyperacute stroke detection to 95% + - Usually correlates to "infarct core" (final infarct size); some diffusion abnormalities reversible (TIA, migraine) + - Restriction typically lasts 7-10 days + - Can persist up to 2 months post ictus + - Corresponding low signal on ADC maps + - May normalize after tissue reperfusion + - After 10 days hyper- or isointensity on ADC map (T2 shine-through) + - May mimic diffusion restriction on DWI + - DTI + - DTI with at least 6 directions can calculate DTI trace, ADC maps + - More sensitive for small ischemic foci, emboli, distal cortical strokes + - Distinguish cytotoxic from vasogenic edema in complicated cases + - ### PWI + + + - Dynamic contrast bolus or arterial spin-labeled techniques + - Maximum slope gives rCBF, rCBV + - Deconvolution gives absolute values + - Bolus-tracking T2* gadolinium perfusion imaging (PWI) with CBV map + - ↓ perfusion; 75% larger than DWI abnormality + - DWI/PWI "mismatch": Penumbra or "at-risk" tissue + - ### T1WI C+ + + + - Variable enhancement patterns evolve over time + - Hyperacute: Intravascular enhancement (stasis from slow antegrade or retrograde collateral flow) + - Acute: Meningeal enhancement (pial collateral flow appears in 24-48 hours, resolves over 3-4 days) + - Subacute: Parenchymal enhancement (appears after 24-48 hours, can persist for weeks/months) + - MRA: Major vessel occlusions, stenoses, status of collaterals + - MRS: ↑ lactate, ↓ NAA +- ## Angiographic Findings + + + - DSA + - Only used if thrombectomy is considered + - Vessel occlusion (cut-off, tapered "rat tail," clot with "tram-track") or stenosis + - ± slow antegrade flow, assess retrograde collateral flow + - "Bare area" of non- or underperfused brain in late arterial/capillary phases +- ## Imaging Recommendations + + + - ### Protocol advice + + + - Initial NECT (exclude hemorrhage/stroke mimic) + - CTA + pCT + - ± MR with fast DWI, FLAIR, T2* GRE + - ± MRA, PWI + - DSA if thrombectomy is option (selected patients up to 24 hours) + +# DIFFERENTIAL DIAGNOSIS + +- ## Hyperdense Vessel Mimics + + + - Intraarterial blood is always slightly hyperdense to normal brain! + - High hematocrit (polycythemia) + - Microcalcification in vessel wall + - Diffuse cerebral edema makes vessels appear relatively hyperdense +- ## Parenchymal Hypodensity (Nonvascular Causes) + + + - Infiltrating neoplasm (e.g., astrocytoma) + - Cerebral contusion + - Inflammation (cerebritis, encephalitis) + - Evolving encephalomalacia + - Dural venous thrombosis with parenchymal venous congestion and edema + - Seizure + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Many causes (thrombotic vs. embolic, dissection, vasculitis, hypoperfusion) + - Early: Critical disturbance in CBF + - Severely ischemic core has CBF < (6-8 cm³)/(100 g/min) [normal ~ (60 cm³)/(100 g/min)] + - Oxygen depletion, energy failure, terminal depolarization, ion homeostasis failure + - Bulk of final infarct → cytotoxic edema, cell death + - Later: Evolution from ischemia to infarction depends on many factors (e.g., hyperglycemia influences "destiny" of ischemic brain tissue) + - Ischemic penumbra CBF between (10-20 cm³)/(100 g/min) + - Theoretically salvageable tissue + - ### Associated abnormalities + + + - Cardiac disease, prothrombotic states + - Additional stroke risk factors: C-reactive protein, homocysteine +- ## Gross Pathologic & Surgical Features + + + - Acute thrombosis of major vessel + - Pale, swollen brain; GM-WM boundaries blurred +- ## Microscopic Features + + + - After 4 hours: Eosinophilic neurons with pyknotic nuclei + - 15-24 hours: Neutrophils invade, necrotic nuclei look like "eosinophilic ghosts" + - 2-3 days: Blood-derived phagocytes + - 1 week: Reactive astrocytosis, ↑ capillary density + - End result: Fluid-filled cavity lined by astrocytes + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Focal acute neurologic deficit + - Paresis, aphasia, ↓ mental status +- ## Demographics + + + - ### Age + + + - Any age; most common in older adults + - Consider underlying disease (sickle cell, moyamoya, neurofibromatosis type 1, cardiac, drugs) in children, young adults + - ### Sex + + + - No sex predilection + - ### Epidemiology + + + - 2nd most common cause of death worldwide + - Most common cause of morbidity in USA +- ## Natural History & Prognosis + + + - Clinical diagnosis inaccurate in 15-20% of strokes + - ~ 50% of patients with LVO, thrombectomy achieve functional independence + - Prognosis poor if ASPECTS ≤ 5 + - Malignant MCA infarct (coma, death) + - Up to 10% of all stroke patients (↑ risk with large infarct volumes) + - ↑ inflammasome activation → proinflammatory cytokines; fatal brain swelling with ↑ ICP +- ## Treatment + + + - "Time is brain" + - IV thrombolysis = ~ 10% successful/sufficient recanalization + - IV rTPA window < 3 hours if thrombectomy not available + - Thrombolysis in cerebral infarction (TICI scale) = reperfusion grade + - Grade ≥ 2B is "successful" reperfusion; 2C = near-perfect reperfusion + - TICI 3 = best functional outcome + - Now procedure of choice with LVO = mechanical thrombectomy + - Stent retriever better outcome than aspiration only + - ± new devices to ↓ distal embolization + - Patient selection most important factor in outcome + - AHA/ASA guidelines: Age ≥ 18 years, ASPECTS/NIHSS score ≥ 6 + - Symptom onset < 6 hours + - No parenchymal hematoma on CT + - DAWN, DEFUSE 3 trials have broadened window + - Some advocate treating "almost anyone" (ASPECTS = 0-5) + - Up to 24 hours in some cases + - Even failed/incomplete recanalization shows ↓ likelihood for very poor outcome, not generally harmful + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Rarely, ischemia or seizure may mimic tumor or encephalitis + + 0e086d6e-36ea-4341-abf7-2d480bc1842f + +## References + +# Selected References + +1. [Aoki J et al: Negative-FLAIR vascular hyperintensities serve as a marker of no recanalization during hospitalization in acute stroke. J Clin Neurosci. 72:233-7, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31836384%5Bpmid%5D) +1. [Atchaneeyasakul K et al: Impact of MRI selection on triage of endovascular therapy in acute ischemic stroke: the mri in acute management of ischemic stroke (MIAMIS) registry. Interv Neurol. 8(2-6):135-43, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32508895%5Bpmid%5D) +1. [Atchaneeyasakul K et al: Thrombectomy outcomes in acute ischemic stroke due to middle cerebral artery M2 occlusion with stent retriever versus aspiration: a multicenter experience. Interv Neurol. 8(2-6):180-6, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32508900%5Bpmid%5D) +1. [Broocks G et al: Incomplete or failed thrombectomy in acute stroke patients with ASPECTS 0-5 - how harmful is trying? Eur J Neurol. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32449311%5Bpmid%5D) +1. [Dhand S et al: Acute ischemic stroke: acute management and selection for endovascular therapy. Semin Intervent Radiol. 37(2):109-18, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32419723%5Bpmid%5D) +1. [Miao J et al: Predictors of malignant cerebral edema in cerebral artery infarction: a meta-analysis. J Neurol Sci. 409:116607, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31830611%5Bpmid%5D) +1. [Patel P et al: Hyperacute management of ischemic strokes: JACC Focus Seminar. J Am Coll Cardiol. 75(15):1844-56, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32299596%5Bpmid%5D) +1. [Sakamoto Y et al: Reducing door-to-reperfusion time in acute stroke endovascular therapy using magnetic resonance imaging as a screening modality. J Neurointerv Surg. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32051322%5Bpmid%5D) +1. [Heit JJ et al: Perfusion computed tomography in acute ischemic stroke. Radiol Clin North Am. 57(6):1109-16, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31582038%5Bpmid%5D) +1. [Zhang M et al: Characteristics of cerebral perfusion and diffusion associated with crossed cerebellar diaschisis after acute ischemic stroke. Jpn J Radiol. 38(2):126-34, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31720951%5Bpmid%5D) +1. [Martins N et al: Ghost infarct core and admission computed tomography perfusion: redefining the role of neuroimaging in acute ischemic stroke. Interv Neurol. 7(6):513-21, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30410531%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Coronal graphic illustrates left M1 occlusion. Proximal occlusion affects the entire middle cerebral artery (MCA) territory, including the basal ganglia (perfused by lenticulostriate arteries ). Acute ischemia is often identified by subtle loss of the gray matter-white matter interfaces with blurring of the basal ganglia and an insular ribbon sign on the initial CT.](images/app.statdx.com_image_thumbnail_84905917-7cc1-4d88-8ef5-4571a93e6d43_annotated_true_size_900_quality_90_2fb275876b5dedb1082c95a18b3baa814a1346c3.jpg) +*Coronal graphic illustrates left M1 occlusion. Proximal occlusion affects the entire middle cerebral artery (MCA) territory, including the basal ganglia (perfused by lenticulostriate arteries ). Acute ischemia is often identified by subtle loss of the gray matter-white matter interfaces with blurring of the basal ganglia and an insular ribbon sign on the initial CT.* + +![Coronal graphic illustrates left M1 occlusion. Proximal occlusion affects the entire middle cerebral artery (MCA) territory, including the basal ganglia (perfused by lenticulostriate arteries ). Acute ischemia is often identified by subtle loss of the gray matter-white matter interfaces with blurring of the basal ganglia and an insular ribbon sign on the initial CT.](images/app.statdx.com_image_thumbnail_84905917-7cc1-4d88-8ef5-4571a93e6d43_size_174_quality_85_6f707289df1b923454df5e77f0ff816b7e0ef678.jpg) +*Coronal graphic illustrates left M1 occlusion. Proximal occlusion affects the entire middle cerebral artery (MCA) territory, including the basal ganglia (perfused by lenticulostriate arteries ). Acute ischemia is often identified by subtle loss of the gray matter-white matter interfaces with blurring of the basal ganglia and an insular ribbon sign on the initial CT.* + +![Axial NECT in a 46-year-old man shows a very "dense" left MCA compared to the normal minimally hyperdense right MCA .](images/app.statdx.com_image_thumbnail_2e2efdc2-7058-493c-abcd-f260a1b51cc7_annotated_true_size_900_quality_90_6e93d92fafc2eb30a87ca067c854a2853605d723.jpg) +*Axial NECT in a 46-year-old man shows a very "dense" left MCA compared to the normal minimally hyperdense right MCA .* + +![Graphic shows anatomic regions for calculating the ASPECTS score. M1-M2 represent the MCA cortex with each area allotted 1 point. The insular cortex (I), lentiform nuclei (L), caudate head (C), and internal capsule (IC) are scored with 1 point each.](images/app.statdx.com_image_thumbnail_d7844a70-c220-4301-b8f5-4ba1ac458ab1_annotated_true_size_900_quality_90_39241c4be579e03a6a831cccf97036c9e52dd7a2.jpg) +*Graphic shows anatomic regions for calculating the ASPECTS score. M1-M2 represent the MCA cortex with each area allotted 1 point. The insular cortex (I), lentiform nuclei (L), caudate head (C), and internal capsule (IC) are scored with 1 point each.* + +![More cephalad graphic shows the superior 3 MCA territories. The ASPECTS score is calculated by subtracting 1 point for each affected area from 10 (normal total score).](images/app.statdx.com_image_thumbnail_9d33f784-7884-4b87-8c7d-3d6b5f397bef_annotated_true_size_900_quality_90_5d46ba43497732c9821a087596a1d13884b66cea.jpg) +*More cephalad graphic shows the superior 3 MCA territories. The ASPECTS score is calculated by subtracting 1 point for each affected area from 10 (normal total score).* + +![Axial NECT in a 47-year-old woman with sudden onset of right hemiparesis shows hypodensity in the left lateral basal ganglia, insula, and parietal lobe. The total ASPECTS score was 6.](images/app.statdx.com_image_thumbnail_475de780-5ea1-4c00-8fb9-eddd1ea94ec4_annotated_true_size_900_quality_90_ab14f3e11f5194a54deaf3d075c6cf549559e0e2.jpg) +*Axial NECT in a 47-year-old woman with sudden onset of right hemiparesis shows hypodensity in the left lateral basal ganglia, insula, and parietal lobe. The total ASPECTS score was 6.* + +![MIP of the CTA in the same patient shows an abrupt cut-off of the proximal M1 MCA segment.](images/app.statdx.com_image_thumbnail_c7c2e723-2194-4a7c-be9e-1ad7a3851c26_annotated_true_size_900_quality_90_50dc526ad2b322c0d8a77f37ad8b52c9793deecc.jpg) +*MIP of the CTA in the same patient shows an abrupt cut-off of the proximal M1 MCA segment.* + +![Axial CT perfusion with cerebral blood volume (CBV) shows reduced CBV in the left temporal and parietal lobes, as well as the lateral basal ganglia, external capsule, and insula.](images/app.statdx.com_image_thumbnail_c92c387f-c1c9-42ff-b391-95fb77acdd99_annotated_true_size_900_quality_90_fd3eb7d9ec2e08c0e33646c36363b4e4ce75653d.jpg) +*Axial CT perfusion with cerebral blood volume (CBV) shows reduced CBV in the left temporal and parietal lobes, as well as the lateral basal ganglia, external capsule, and insula.* + +![Axial cerebral blood flow (CBF) map shows a more extensive area of reduced perfusion, suggesting there is a significant penumbra of brain that is ischemic but not infarcted. CBF in the left basal ganglia and thalamus is also reduced, suggesting the proximal M1 occlusion has also compromised the deep gray nuclei.](images/app.statdx.com_image_thumbnail_ccd474be-6aed-445f-912a-8d4a4e7cab55_annotated_true_size_900_quality_90_a9c9f0d9f3dcb24cd21817f119d196743e197258.jpg) +*Axial cerebral blood flow (CBF) map shows a more extensive area of reduced perfusion, suggesting there is a significant penumbra of brain that is ischemic but not infarcted. CBF in the left basal ganglia and thalamus is also reduced, suggesting the proximal M1 occlusion has also compromised the deep gray nuclei.* + +![Automated CT perfusion in the same case shows CBF < 30% = 68 mL, volume of brain with Tmax > 6.0 seconds = 108 mL, and mismatch volume (penumbra) of 40 mL.](images/app.statdx.com_image_thumbnail_c15822d8-4665-47dd-acc2-dae366b69622_annotated_true_size_900_quality_90_3626ab0836b2ad78912230da9dc45c11e036c4fa.jpg) +*Automated CT perfusion in the same case shows CBF < 30% = 68 mL, volume of brain with Tmax > 6.0 seconds = 108 mL, and mismatch volume (penumbra) of 40 mL.* + +![(L) Pretreatment AP view of the left internal carotid artery (ICA) DSA in the same case shows the proximal MCA occlusion . (R) Following stent-retriever thrombectomy, the clot has been removed and the M1 MCA appears nearly normal. Blood flow to the distal MCA is mostly restored.](41dc2704-b2db-427e-adf7-14824612cda9) +*(L) Pretreatment AP view of the left internal carotid artery (ICA) DSA in the same case shows the proximal MCA occlusion . (R) Following stent-retriever thrombectomy, the clot has been removed and the M1 MCA appears nearly normal. Blood flow to the distal MCA is mostly restored.* + +![Axial T1WI MRs show the 2 vascular watershed zones (WSZs). Blue depicts the cortical (external) WSZs between the major territorial arteries [anterior cerebral artery (ACA), MCA, posterior cerebral artery (PCA)]. The yellow depicts the subcortical (internal or deep) WSZs between perforating arteries and major territorial arteries.](41e4888b-7a0d-4bc7-8435-a08b5c22a03c) +*Axial T1WI MRs show the 2 vascular watershed zones (WSZs). Blue depicts the cortical (external) WSZs between the major territorial arteries [anterior cerebral artery (ACA), MCA, posterior cerebral artery (PCA)]. The yellow depicts the subcortical (internal or deep) WSZs between perforating arteries and major territorial arteries.* + +![Axial FLAIR MR in a 48-year-old woman with TIAs shows white matter hyperintensities aligned front to back just above the level of the lateral ventricles.](5f7a4a0b-99ae-4a17-ae58-9fde305de939) +*Axial FLAIR MR in a 48-year-old woman with TIAs shows white matter hyperintensities aligned front to back just above the level of the lateral ventricles.* + +![Axial DWI MR in the same patient shows acute lacunar infarcts in almost a "string of pearls" configuration.](ae6577fa-47d6-4da3-85a7-4fd96b13e81e) +*Axial DWI MR in the same patient shows acute lacunar infarcts in almost a "string of pearls" configuration.* + +![Axial ADC confirms the deep white matter lesions exhibit acutely restricted diffusion.](05a6d4be-acf9-41f3-b6fd-cd385db4ef0d) +*Axial ADC confirms the deep white matter lesions exhibit acutely restricted diffusion.* + +![2D TOF MRA of the left ICA in the same patient shows a "flow gap" at the junction of the cavernous and supraclinoid segments, indicating a high-grade stenosis.](65bebd08-c969-48e2-b62c-748314ef73cc) +*2D TOF MRA of the left ICA in the same patient shows a "flow gap" at the junction of the cavernous and supraclinoid segments, indicating a high-grade stenosis.* + +![2D TOF MRA of the right ICA in the same patient shows a high-grade stenosis of the right supraclinoid ICA just before the origin of the PCA. The critical stenoses in both ICAs resulted in the deep WSZ infarcts.](d550e7ac-845c-461b-87be-c235c4ced447) +*2D TOF MRA of the right ICA in the same patient shows a high-grade stenosis of the right supraclinoid ICA just before the origin of the PCA. The critical stenoses in both ICAs resulted in the deep WSZ infarcts.* + +![Axial NECT in a 65-year-old man with TIAs and a history of mitral valve replacement was initially read as normal. However, this image shows a calcified cerebral embolus in the right sylvian fissure.](24615aed-aeab-48f6-ac3b-d568fb860a9a) +*Axial NECT in a 65-year-old man with TIAs and a history of mitral valve replacement was initially read as normal. However, this image shows a calcified cerebral embolus in the right sylvian fissure.* + +![More cephalad NECT in the same patient shows a 2nd calcified embolus . A 3rd embolus was present in the interhemispheric fissure (not shown). Calcified cerebral emboli carry ~ 50% risk of repeated strokes. Cardiac sources are most common followed by calcified ASVD plaques at carotid bifurcation.](c3832702-f2da-41ac-8a19-7f5ebfdfb332) +*More cephalad NECT in the same patient shows a 2nd calcified embolus . A 3rd embolus was present in the interhemispheric fissure (not shown). Calcified cerebral emboli carry ~ 50% risk of repeated strokes. Cardiac sources are most common followed by calcified ASVD plaques at carotid bifurcation.* + +![Axial NECT for a brain attack patient in the ER with sudden-onset aphasia is normal.](40d883bc-d763-43c4-a1b8-2dd3816d0f3a) +*Axial NECT for a brain attack patient in the ER with sudden-onset aphasia is normal.* + +![Axial CT perfusion in the same patient was obtained immediately following the NECT. The CBV appears grossly normal.](9a38203c-db10-4b64-9921-367f42b6af2c) +*Axial CT perfusion in the same patient was obtained immediately following the NECT. The CBV appears grossly normal.* + +![Axial CBF map in the same patient shows markedly reduced perfusion in the inferior division of the left MCA .](91b5b3aa-1896-4071-8019-e07be0af5f0a) +*Axial CBF map in the same patient shows markedly reduced perfusion in the inferior division of the left MCA .* + +![TTD in the same patient shows severely reduced TTD, consistent with acute ischemia without infarction. IV TPA was administered and the symptoms resolved.](2c876512-9c65-46f7-b3bb-c587dca5db7b) +*TTD in the same patient shows severely reduced TTD, consistent with acute ischemia without infarction. IV TPA was administered and the symptoms resolved.* + + +### Additional Images + +![Axial NECT in a 60-year-old woman admitted for rapid stroke evaluation shows hypodensity in the right posterior frontal lobe . The right MCA appears slightly hyperdense.](2aa9bae2-647a-46b1-9e5c-1023aab2d0cf) +*Axial NECT in a 60-year-old woman admitted for rapid stroke evaluation shows hypodensity in the right posterior frontal lobe . The right MCA appears slightly hyperdense.* + +![Axial CTA in the same patient shows an abrupt cut-off of contrast in the right MCA just distal to its origin from the ICA.](cdbf31e8-6d11-400d-9153-b433dbde076e) +*Axial CTA in the same patient shows an abrupt cut-off of contrast in the right MCA just distal to its origin from the ICA.* + +![Axial T2* GRE MR in the same patient shows striking blooming from a right M1/proximal M2 thrombus.](8e73e7ee-e376-43bf-8dd6-b03bb040ebd4) +*Axial T2* GRE MR in the same patient shows striking blooming from a right M1/proximal M2 thrombus.* + +![Axial FLAIR MR in the same patient shows edematous right posterior frontal gyri as well as hyperintensity in the ipsilateral insula , caudate head, and putamen .](8fc072ec-e921-446f-8c0e-d9b38adc74dc) +*Axial FLAIR MR in the same patient shows edematous right posterior frontal gyri as well as hyperintensity in the ipsilateral insula , caudate head, and putamen .* + +![Axial DWI MR confirms acute infarction in the territories of the lateral lenticulostriate arteries and superior division of the right MCA.](6879e537-6c6f-4842-8e58-9ea13742679f) +*Axial DWI MR confirms acute infarction in the territories of the lateral lenticulostriate arteries and superior division of the right MCA.* + +![Axial NECT in an 89-year-old man who had several visits to the ER for several falls (to "rule out subdural hematoma") shows a calcified cerebral embolus in a right hemisphere sulcus.](53306c01-08bf-4635-aa9e-cf0b61f7fcc1) +*Axial NECT in an 89-year-old man who had several visits to the ER for several falls (to "rule out subdural hematoma") shows a calcified cerebral embolus in a right hemisphere sulcus.* + +![Sagittal reformatted NECT in the same patient shows the location in the right superior temporal sulcus . The patient was subsequently shown to have calcific mitral valve disease. Calcified cerebral emboli carry a high risk of repeated stroke.](1452ba99-b5d2-4514-ab44-e8a466a757e4) +*Sagittal reformatted NECT in the same patient shows the location in the right superior temporal sulcus . The patient was subsequently shown to have calcific mitral valve disease. Calcified cerebral emboli carry a high risk of repeated stroke.* + +![Axial FLAIR MR shows intravascular signal beginning near the genu.](1ad58bf1-0482-459c-84ce-54578c4ee190) +*Axial FLAIR MR shows intravascular signal beginning near the genu.* + +![More cephalad FLAIR MR in the same patient shows gyral hyperintensity and intravascular signal in the M2 (insular) MCA segments .](2e130585-f0ac-4870-9b08-c806b0034890) +*More cephalad FLAIR MR in the same patient shows gyral hyperintensity and intravascular signal in the M2 (insular) MCA segments .* + +![Axial NECT in a 35-year-old man shows a dense left MCA , indicating acute thrombus involving the entire MCA from its origin to its bifurcation.](f9581fa7-0e5f-4632-8166-14d737bb2e4d) +*Axial NECT in a 35-year-old man shows a dense left MCA , indicating acute thrombus involving the entire MCA from its origin to its bifurcation.* + +![More cephalad NECT shows the basal ganglia are effaced and the gray matter-white matter interfaces in the insula, left posterior frontal lobe, and opercula are poorly defined.](4c243ab6-a419-4af4-9387-a64d0d0d12c5) +*More cephalad NECT shows the basal ganglia are effaced and the gray matter-white matter interfaces in the insula, left posterior frontal lobe, and opercula are poorly defined.* + +![The left basal ganglia are edematous and have "disappeared" as they are now nearly the same density as the surrounding white matter. The gray matter-white matter interfaces in the posterior temporal and anterior parietal lobes are effaced.](b5b8a431-e3c4-44cb-906e-494d268ed023) +*The left basal ganglia are edematous and have "disappeared" as they are now nearly the same density as the surrounding white matter. The gray matter-white matter interfaces in the posterior temporal and anterior parietal lobes are effaced.* + +![More cephalad NECT shows loss of sulci and gray matter-white matter differentiation in the left parietal lobe. Because of his poor ASPECTS score (2, being generous) he was not considered a good candidate for thrombectomy despite his relatively young age.](18d9efa8-81c8-4273-ac12-8290b72262c1) +*More cephalad NECT shows loss of sulci and gray matter-white matter differentiation in the left parietal lobe. Because of his poor ASPECTS score (2, being generous) he was not considered a good candidate for thrombectomy despite his relatively young age.* + +![Axial FLAIR MR scan obtained 3 hours later in the same patient shows hyperintensity in the left basal ganglia, insula, and the entirety of the left cerebral hemisphere supplied by the MCA.](bfe98667-dcc6-4c1f-9256-b19116eb2833) +*Axial FLAIR MR scan obtained 3 hours later in the same patient shows hyperintensity in the left basal ganglia, insula, and the entirety of the left cerebral hemisphere supplied by the MCA.* + +![Axial DWI MR in the same patient shows restricted diffusion in the complete left MCA territory.](c7332f4b-64be-42a4-87f8-db635a37f55a) +*Axial DWI MR in the same patient shows restricted diffusion in the complete left MCA territory.* + +![Axial NECT at 6 hours in the same patient shows thrombus in the horizontal MCA as well as hypodensity in the left frontal and anterior temporal lobes . The suprasellar cistern is normal and there is as yet no evidence for descending transtentorial herniation.](bf20f26c-2e6c-4cc6-8d9c-2b2acd7699a9) +*Axial NECT at 6 hours in the same patient shows thrombus in the horizontal MCA as well as hypodensity in the left frontal and anterior temporal lobes . The suprasellar cistern is normal and there is as yet no evidence for descending transtentorial herniation.* + +![Axial NECT through the basal ganglia and insula shows extensive hypodensity in the entire left MCA territory . Mass effect is developing with compression of the left frontal horn .](fc93cf03-5fe3-4cf7-8550-993db97d0804) +*Axial NECT through the basal ganglia and insula shows extensive hypodensity in the entire left MCA territory . Mass effect is developing with compression of the left frontal horn .* + +![More cephalad NECT in the same patient at 6 hours following ictus shows the wedge-shaped hypodensity involves the complete left frontal and parietal opercula. The massive edema developing in the entire left MCA territory makes this a so-called "malignant" MCA infarct.](d8a4750b-79a9-48b3-b4f9-0d142698ed84) +*More cephalad NECT in the same patient at 6 hours following ictus shows the wedge-shaped hypodensity involves the complete left frontal and parietal opercula. The massive edema developing in the entire left MCA territory makes this a so-called "malignant" MCA infarct.* + +![Twelve hours after admission, the same patient became unresponsive. His left pupil was dilated and he was rushed to the operating room where an emergent craniectomy was performed. Postoperative NECT at 20 hours shows hypodensity throughout the left MCA territory. Note the brain bulging out through the craniectomy defect.](8c7f4165-6f4f-4986-9847-d3c3c0ff7a32) +*Twelve hours after admission, the same patient became unresponsive. His left pupil was dilated and he was rushed to the operating room where an emergent craniectomy was performed. Postoperative NECT at 20 hours shows hypodensity throughout the left MCA territory. Note the brain bulging out through the craniectomy defect.* + +![More cephalad NECT in the same patient shows the completed total MCA territory infarct.](f6ba9cf2-efd2-48c8-ad32-85afe227def6) +*More cephalad NECT in the same patient shows the completed total MCA territory infarct.* + diff --git a/docs_md/articles/acute-hypertensive-encephalopathy-pres_890c1bd4-c108-49a1-8557-c8c701a7f278.md b/docs_md/articles/acute-hypertensive-encephalopathy-pres_890c1bd4-c108-49a1-8557-c8c701a7f278.md new file mode 100644 index 0000000..570b18f --- /dev/null +++ b/docs_md/articles/acute-hypertensive-encephalopathy-pres_890c1bd4-c108-49a1-8557-c8c701a7f278.md @@ -0,0 +1,512 @@ +--- +title: "Acute Hypertensive Encephalopathy, PRES" +docid: "890c1bd4-c108-49a1-8557-c8c701a7f278" +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: "Pathology-Based Diagnoses" + slug: "pathology-based-diagnoses" + treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" + - + name: "Acquired Toxic/Metabolic/Degenerative Disorders" + slug: "acquired-toxicmetabolicdegenerativ-" + treeNodeId: "ba3cfeaf-64d9-4117-91e8-d2ce58783fc5" + - + name: "Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations" + slug: "toxic-metabolic-nutritional-system-" + treeNodeId: "06bd883b-8269-4044-8411-70f7ab75bb7a" + - + name: "Acute Hypertensive Encephalopathy, PRES" + slug: "acute-hypertensive-encephalopathy--" + treeNodeId: null +category: "Brain" +cmeTopicId: "24478bee-4cae-4449-96aa-8a6942891c49" +documentVersionId: "4f172c49-3201-4058-9250-8a09dbb11c26" +imageCount: 30 +lastUpdated: "09/29/20" +pageDescription: "Acute Hypertensive Encephalopathy, PRES" +pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations, Acute Hypertensive Encephalopathy, PRES" +pageTitle: "Acute Hypertensive Encephalopathy, PRES | STATdx" +enhancedTitle: "Acute Hypertensive Encephalopathy, PRES" +type: "DX" +references: true +ddx: true +cases: 2 +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Pathology-Based Diagnoses" + - "Acquired Toxic/Metabolic/Degenerative Disorders" + - "Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations" + - "Acute Hypertensive Encephalopathy, PRES" +--- +# KEY FACTS + +- ## Terminology + + + - Cerebrovascular autoregulatory disorder + - Many etiologies with HTN as common component + - Preeclampsia, eclampsia + - Drug toxicity (e.g., chemotherapy) + - Uremic encephalopathies +- ## Imaging + + + - General + - Patchy parietooccipital cortical/subcortical edema in patient with severe acute/subacute HTN + - CT: May be normal or subtly abnormal + - If PRES suspected, perform MR to confirm + - MR: Parietooccipital T2/FLAIR hyperintensities in 90% of cases + - ± basal ganglia, pontine, cerebellar involvement + - 3 patterns of hemorrhage: Focal parenchymal hemorrhage, microhemorrhages, convexity SAH + - Generally no restriction on DWI + - Variable patchy enhancement; atypical imaging patterns common +- ## Top Differential Diagnoses + + + - Acute cerebral ischemia-infarction + - Hypoglycemia + - Reversible cerebral vasoconstriction syndrome + - Status epilepticus + - Thrombotic microangiopathies + - Cerebral hyperperfusion syndrome +- ## Pathology + + + - Failure of autoregulation + - Endothelial dysfunction + - Result = vasogenic (not cytotoxic) edema +- ## Clinical Issues + + + - Headache, seizure, ↓ mental status, visual symptoms + - Caution: Some patients may be normotensive or have only minimally elevated BP + +# TERMINOLOGY + +- ## Abbreviations + + + - Posterior reversible encephalopathy syndrome (PRES) +- ## Synonyms + + + - Acute hypertensive encephalopathy + - Reversible posterior leukoencephalopathy syndrome (RPLS) +- ## Definitions + + + - Variant of acute hypertensive encephalopathy characterized by headache, visual disturbances, altered mental function + - Cerebrovascular autoregulatory disorder + - Multiple etiologies; most caused by acute HTN + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Patchy parietooccipital cortical/subcortical edema in patient with severe acute/subacute HTN + - ### Location + + + - Most common: Cortex, subcortical white matter + - Parietooccipital lobes (≥ 90%) + - Superior frontal (≥ 70%), temporal lobes (≥ 60%), cerebellum (≥ 50%) + - Basal ganglia (≥ 30%), midbrain (< 20%), pons, medulla, splenium of corpus callosum (< 10%) + - At junctions of vascular watershed zones + - Usually bilateral, often asymmetric + - Rare: Predominant/exclusive brainstem involvement + - ### Size + + + - Extent of abnormalities highly variable + - ### Morphology + + + - Patchy > confluent; atypical patterns common +- ## CT Findings + + + - ### NECT + + + - May be normal or subtly abnormal + - If PRES suspected, perform MR to confirm + - Common: Bilateral nonconfluent hypodense foci + - Posterior parietooccipital lobes + - Cortical watershed zones + - Less common: Petechial cortical/subcortical or basal ganglionic hemorrhages + - Uncommon: Thalamic, basal ganglia, brainstem, cerebellar hypodensities + - ### CECT + + + - Usually no enhancement + - Occasionally mild patchy/punctate enhancement + - ### CTA + + + - Major vessels usually normal + - Distal vessels may show diffuse vasoconstriction, focal irregularity, & beaded appearance +- ## MR Findings + + + - ### T1WI + + + - Hypointense cortical/subcortical lesions + - ### T2WI + + + - **Typical**PRES **(p****arietooccipital pattern****)** + - Parietooccipital lobes, cortical watershed zones + - **Atypical**PRES: Almost as common as **typical**PRES + - **Superior frontal sulcus pattern** + - Hyperintensities in mid & posterior aspects of superior frontal sulcus + - **Holohemispheric watershed pattern** + - Involvement of frontal, parietal, & occipital internal watershed zones + - Hyperintensities in basal ganglia, brainstem, cerebellum + - ### FLAIR + + + - Same as T2 + - "Leaky" blood-brain barrier may cause gadolinium accumulation in CSF, FLAIR hyperintensity + - ### T2* GRE + + + - 3 patterns of hemorrhage: Focal parenchymal hemorrhage, microhemorrhages, convexity subarachnoid hemorrhage (SAH) + - ### DWI + + + - Usually negative + - 15-30% small foci of restricted diffusion within larger regions of vasogenic edema + - ### PWI + + + - CT & MR perfusion may show both ↑ & ↓ relative cerebral blood volume (rCBV) in occipital regions, cortical watershed zones + - ### T1WI C+ + + + - Variable patchy cortical/subcortical enhancement + - ### MRS + + + - May show widespread metabolic abnormalities + - ↑ Cho, Cr, mildly ↓ NAA + - Usually return to normal within 2 months + - DTI + - Shows foci of ↑ diffusivity & anisotropy loss +- ## Nuclear Medicine Findings + + + - SPECT + - Variable findings reported; more common hypoperfusion in affected areas +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Contrast-enhanced MR + DWI + - ### Protocol advice + + + - Repeat scan after BP normalized + +# DIFFERENTIAL DIAGNOSIS + +- [Acute Cerebral Ischemia-Infarction](/document/acute-cerebral-ischemiainfarction/a405285f-aaea-43ca-8dc4-6f8120eaabc1) + - Middle cerebral artery distribution > > posterior cerebral artery + - Infarcts restrict on DWI; PRES usually does not +- [Hypoglycemia](/document/adult-hypoglycemia/38e4de6e-07c4-485e-bac1-f4dd4815b3b8) + - Severe parietooccipital edema + - Can resemble PRES, so history important +- [Reversible Cerebral Vasoconstriction Syndrome](/document/reversible-cerebral-vasoconstricti-/e5d89e00-aaa7-4809-8d9b-82c0f89d5d01) + - Shares some features with PRES + - Typically limited to solitary sulcus or few adjacent sulci +- [Status Epilepticus](/document/status-epilepticus/a058b733-4b80-46a1-8097-d68685ecf921) + - May cause transient gyral edema, enhancement + - Can mimic PRES, stroke, infiltrating neoplasm + - Unilateral (PRES often bilateral) +- ## Thrombotic Microangiopathies + + + - Malignant HTN, DIC, hemolytic uremic syndrome (HUS), TTP + - Significant overlap as PRES common imaging manifestation +- [Cerebral Hyperperfusion Syndrome](/document/cerebral-hyperperfusion-syndrome/e66febb9-d79e-4f04-88b1-205ba8a0822f) + - Postcarotid endarterectomy, angioplasty, or stenting + - Hyperperfusion syndrome occurs in 5-9% of cases + - Perfusion MR imaging or CT scans show elevated relative cerebral blood flow (rCBF) + - Aggressive control of BP associated with clinical, radiologic improvement + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Not yet completely understood + - Diverse causes & clinical entities + - **2 leading theories** regarding pathophysiology of PRES + - **1st hypothesis** + - Rapid ↑ of arterial BP above upper autoregulatory limit → cerebral hyperperfusion → vascular leakage & vasogenic edema + - ↑ cerebral perfusion pressure → blood-brain barrier dysfunction → extravasation of plasma & macromolecules through tight-junction proteins + - **2nd hypothesis** + - PRES triggered by endothelial dysfunction caused by circulating endogenous or exogenous toxins + - Excessive release of proinflammatory cytokines → vascular leakage & edema formation + - Predilection for parietooccipital lobes + - Posterior circulation sparsely innervated by sympathetic nerves + - Frank infarction with cytotoxic edema rare in PRES + - ### Associated abnormalities + + + - Acute/subacute systemic HTN + - Preeclampsia, eclampsia + - Typically occurs after 20-weeks gestation + - Rare: Headache, seizures up to several weeks post partum + - Drug toxicity ± tumor lysis syndrome + - Chemotherapeutic agents (e.g., cyclosporine, cisplatin) + - Thrombotic microangiopathies (DIC, TTP, malignant HTN) + - Uremic encephalopathies + - Acute glomerulonephritis, lupus nephropathy, etc. + - Severe infection + - 25% of septic patients in shock develop PRES + - BP can be normal or elevated +- ## Gross Pathologic & Surgical Features + + + - Common + - Cortical/subcortical edema + - ± petechial hemorrhage in parietooccipital lobes + - Less common + - Anterior frontal lobes, basal ganglia, brainstem, cerebellum + - Uncommon + - Lobar hemorrhage + - Frank infarction +- ## Microscopic Features + + + - Autopsy in severe cases shows microvascular fibrinoid necrosis, ischemic microinfarcts, variable hemorrhage + - Chronic: Demyelination, laminar necrosis, older hemorrhage + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Headache, seizure, ↓ mental status, visual disturbances + - Caution: Some patients, especially children, may be normotensive or have only minimally elevated BP + - ### Clinical profile + + + - Pregnant female with acute systemic HTN, headache ± seizure + - Middle-aged, older adult on chemotherapy + - Child with kidney disease or transplant +- ## Demographics + + + - ### Age + + + - Any, but young > old + - ### Sex + + + - F > > M + - ### Epidemiology + + + - Preeclampsia in 5% of pregnancies + - Eclampsia has lower rate (< 1%) +- ## Natural History & Prognosis + + + - Usually no residual abnormalities after HTN corrected + - Reversibility related to BP normalization + - Brainstem, deep white matter lesions less reversible than cortical/subcortical + - Eclampsia more reversible than drug-related PRES + - Severe PRES may be life-threatening + - Permanent infarction rare +- ## Treatment + + + - Control BP, remove precipitating factors + - Delayed diagnosis/therapy can result in chronic neurologic sequelae + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Patchy bilateral parietooccipital hypodensities may be earliest NECT manifestation of PRES +- ## Image Interpretation Pearls + + + - Major DDx of PRES is cerebral ischemia; DWI is positive in latter, usually negative in former + + 700d7e3a-7e08-44be-8153-ef41cef115a6 + +## References + +# Selected References + +1. [Liman TG et al: Posterior reversible encephalopathy syndrome. Curr Opin Neurol. 32(1):25-35, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30531559%5Bpmid%5D) +1. [Racchiusa S et al: Posterior reversible encephalopathy syndrome (PRES) and infection: a systematic review of the literature. Neurol Sci. 40(5):915-22, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30604335%5Bpmid%5D) +1. [Tetsuka S et al: Posterior reversible encephalopathy syndrome: a review with emphasis on neuroimaging characteristics. J Neurol Sci. 404:72-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31349066%5Bpmid%5D) +1. [Brady E et al: The imaging spectrum of posterior reversible encephalopathy syndrome: a pictorial review. Clin Imaging. 47:80-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=28910681%5Bpmid%5D) +1. [Fischer M et al: Posterior reversible encephalopathy syndrome. J Neurol. 264(8):1608-16, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28054130%5Bpmid%5D) +1. [Pereira PR et al: Clinical, imagiological and etiological spectrum of posterior reversible encephalopathy syndrome. Arq Neuropsiquiatr. 73(1):36-40, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25608125%5Bpmid%5D) +1. [Thompson RJ et al: Posterior reversible encephalopathy syndrome in the emergency department: case series and literature review. West J Emerg Med. 16(1):5-10, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25671001%5Bpmid%5D) +1. [Gao B et al: Central-variant posterior reversible encephalopathy syndrome: more than meets the eye. AJR Am J Roentgenol. 203(4):W454, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25247980%5Bpmid%5D) +1. [Junewar V et al: Neuroimaging features and predictors of outcome in eclamptic encephalopathy: a prospective observational study. AJNR Am J Neuroradiol. 35(9):1728-34, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24722310%5Bpmid%5D) +1. [Lamy C et al: Posterior reversible encephalopathy syndrome. Handb Clin Neurol. 121:1687-701, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24365441%5Bpmid%5D) +1. [Rykken JB et al: Posterior reversible encephalopathy syndrome. Semin Ultrasound CT MR. 35(2):118-35, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24745888%5Bpmid%5D) +1. [Brewer J et al: Posterior reversible encephalopathy syndrome in 46 of 47 patients with eclampsia. Am J Obstet Gynecol. 208(6):468, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23395926%5Bpmid%5D) +1. [Khosravani H et al: Emergency noninvasive angiography for acute intracerebral hemorrhage. AJNR Am J Neuroradiol. 34(8):1481-7, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23124634%5Bpmid%5D) +1. [Li R et al: Is hypertension predictive of clinical recurrence in posterior reversible encephalopathy syndrome? J Clin Neurosci. 20(2):248-52, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23219827%5Bpmid%5D) +1. [McKinney AM et al: Detection of microhemorrhage in posterior reversible encephalopathy syndrome using susceptibility-weighted imaging. AJNR Am J Neuroradiol. 33(5):896-903, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22241378%5Bpmid%5D) +1. [Peter P et al: Posterior reversible encephalopathy syndrome and the pediatric population. J Pediatr Neurosci. 7(2):136-8, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23248696%5Bpmid%5D) +1. [McCoy B et al: Childhood posterior reversible encephalopathy syndrome. Eur J Paediatr Neurol. 15(2):91-4, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21074464%5Bpmid%5D) +1. [Hefzy HM et al: Hemorrhage in posterior reversible encephalopathy syndrome: imaging and clinical features. AJNR Am J Neuroradiol. 30(7):1371-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19386731%5Bpmid%5D) +1. [Bartynski WS: Posterior reversible encephalopathy syndrome, part 1: fundamental imaging and clinical features. AJNR Am J Neuroradiol. 29(6):1036-42, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18356474%5Bpmid%5D) +1. [Bartynski WS: Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema. AJNR Am J Neuroradiol. 29(6):1043-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18403560%5Bpmid%5D) +1. [Bartynski WS et al: Posterior reversible encephalopathy syndrome in infection, sepsis, and shock. AJNR Am J Neuroradiol. 27(10):2179-90, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17110690%5Bpmid%5D) +1. [Ishikura K et al: Posterior reversible encephalopathy syndrome in children: its high prevalence and more extensive imaging findings. Am J Kidney Dis. 48(2):231-8, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16860188%5Bpmid%5D) +1. [Mirza A: Posterior reversible encephalopathy syndrome: a variant of hypertensive encephalopathy. J Clin Neurosci. 13(5):590-5, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16769518%5Bpmid%5D) +1. [Narbone MC et al: PRES: posterior or potentially reversible encephalopathy syndrome? Neurol Sci. 27(3):187-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16897633%5Bpmid%5D) +1. [Pande AR et al: Clinicoradiological factors influencing the reversibility of posterior reversible encephalopathy syndrome: a multicenter study. Radiat Med. 24(10):659-68, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17186320%5Bpmid%5D) +1. [Kaito E et al: The role of tumor lysis in reversible posterior leukoencephalopathy syndrome. Pediatr Radiol. 35(7):722-7, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=15756541%5Bpmid%5D) +1. [Striano P et al: Clinical spectrum and critical care management of posterior reversible encephalopathy syndrome (PRES). Med Sci Monit. 11(11):CR549-53, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16258402%5Bpmid%5D) + +## Differential diagnosis + +### Intracranial Hemorrhage +DDX:3a9cbed6-aa2c-45a0-88bf-b39f1523ee85 + +## Cases + +- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'fde3bc1c-5b51-4c9f-b4a1-ac78603790e5', 'description': 'Axial FLAIR scan (#1) shows gyral edema in both occipital lobes and the posterior left frontal lobe. T1C+ scan (#2) shows multifocal punctate areas of enhancement indicating active blood-brain barrier disruption in this case of PRES.', 'history': 'Patient with bone marrow transplant on cyclosporin developed severe hypertension.', 'imagePoolId': '35fe9e0a-6629-447c-a764-160f8e332bbf', 'name': 'Enhances', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'aee88fd4-9fc2-4b54-8eda-c503d4959535', 'description': "Axial NECT scan was initially read as normal but shows subtle hypodensities in both occipital lobes (, Fig. 1). T2WI shows subtle hyperintensity in the right occipital lobe and definite hyperintensity on the left side (, Fig. 2). DWI (Fig. 3) was normal. ADC shows mild "T2 shine-through" in the left occipital lobe (, Fig. 4).", 'history': 'Pregnant patient developed eclampsia.', 'imagePoolId': '7095dae7-cac3-492b-9b35-3459f05bc035', 'name': 'Subtle', 'teachingPoint': None, 'demographics': '26 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'b33ecc59-2ab9-4d2c-abfa-19dd54d7265b', 'description': 'Axial NECT scans (#1-2) show relatively symmetric low density in the posterior parietal and occipital lobes (arrows). A suggestion of low density foci more anteriorly along the vascular watershed zone (#2, open arrows) can be identified. The basal ganglia appears normal. Axial T2 weighted (#3) and FLAIR scans (#5-7) show bioccipital foci of high signal intensity involving the cortex and subcortical white matter. Findings are classic for acute hypertensive encephalopathy, which has many possible etiologies.', 'history': 'Young patient with HUS/TTP and severe hypertension.', 'imagePoolId': '84d7acc7-1001-4c62-9fc5-fa881c44b9f9', 'name': 'Classic', 'teachingPoint': None, 'demographics': '20 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'b8ec88d1-96ba-46cf-beba-357ff73fefe7', 'description': 'Axial T2WI MR images (#1, 2) show bilateral cortical/subcortical occipital hyperintensities (arrows) with more subtle lesions in the anterior watershed zones (open arrows, #2). Axial T1 C+ MR images (#3, 4) show patchy enhancement in the posterior circulation lesions (arrows) as well as in the more anterior watershed zone (open arrows, #4). Images #5 and 6 are post-contrast T1WI obtained two days after delivery and normalization of blood pressure. The enhancement has disappeared.\n\nComment: Classic PRES represents transient opening of the blood-brain barrier and usually resolves without either clinical or imaging residua.', 'history': 'Patient presents with a history of eclampsia.', 'imagePoolId': '4137cb09-f09d-45bd-93ec-97888416d291', 'name': 'Classic', 'teachingPoint': None}, {'authors': [{'key': '815f3e98-b5da-43c7-8f99-d3db52947320', 'value': 'Hank Baskin, MD'}], 'caseVersionId': 'ad497cf6-cde6-4c6c-9f00-14a0b1018819', 'description': 'Axial FLAIR images (#1,2) show patchy areas of abnormal, increased signal in the bilateral occipital lobes (arrows), a typical location to see signal changes associated with acute hypertensive encephalopathy. As is typical, there is no diffusion restriction on diffusion-weighted imaging (#3) and no abnormal enhancement on post-contrast imaging (#4).', 'history': 'Young girl with renal artery stenosis and hypertension presented with metal status changes.', 'imagePoolId': 'd440e808-f5fc-4959-9f11-10fc37408c9a', 'name': 'No enhancement or diffusion abnormality', 'teachingPoint': None, 'demographics': '12 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '48dc907f-f3c2-450b-a232-8670a575b5d0', 'description': 'Axial T2 (#1-6) and FLAIR (#7-11) MR scans show symmetric increased signal intensity in the white matter of the cerebellum as well as the centrum semiovale of the cerebral hemispheres (arrows). Abnormal signal intensity is also present in the pons and basal ganglia (curved arrows) as well as the cortex and subcortical white matter in the territory supplied by the posterior cerebral arteries and along the vascular watershed zone (open arrows). DWI sequence (#12-15) shows no definite restriction, the usual pattern observed in patients with acute hypertensive encephalopathy (PRES).', 'history': 'Patient on Cyclosporin with acute onset of extreme systolic hypertension.', 'imagePoolId': 'f47d1fda-d2d2-4b02-b126-226330cfe495', 'name': 'Florid', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '62822f9f-426c-4331-b921-f9cedabf6bb0', 'description': 'Axial T1WI shows sulcal effacement, blurred gray-white interface in both parieto-occipital lobes (arrows, #1). T2WIs (#2,3) show loss of CSF hyperintensity in the adjacent sulci caused by gyral edema, well-seen compared to the normal frontal, temporal lobe sulci. Note subtle hyperintensity in the parieto-occipital cortex, underlying white matter, and watershed zone (arrows, #2-4). FLAIR hyperintensity in the same areas is easier to distinguish (arrows,#5-7). Coronal T1 C+ scan shows some blood-brain-barrier leakage with patchy contrast enhancement (arrows, #8). \n\nComment: Findings are those of PRES, posterior reversible encephalopathy syndrome. Findings resolved when cyclosporine was stopped and blood pressure normalized.', 'history': 'Patient with acute myelogenous leukemia, bone marrow transplant, on cyclosporine. Developed acute onset of hypertension, seizures.', 'imagePoolId': 'b45439b3-cbe9-430f-b2f1-760bb3713f26', 'name': 'Seizure', 'teachingPoint': None, 'demographics': '41 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'a41ccec1-f7c0-488e-bed9-d65bdab740ab', 'description': 'Axial NECT scans (#1-3) show profound hypodensity in the basal ganglia and thalami (open arrows). The brain is generally edematous as seen by the lack of surface sulci on the most superior scan (#3). \n\nComment: Involvement of the basal ganglia without affecting the occipital lobes, white matter or watershed zones is a variant pattern of PRES.', 'history': 'Child with known hemolytic-uremic syndrome (HUS) and renal failure presented with severe hypertension, GI bleeding and acute neurologic decline. The patient died 2 days after this scan was obtained.\n', 'imagePoolId': '9632dde5-4612-4a30-b4b8-8caf4912ec43', 'name': 'Atypical involvement', 'teachingPoint': None, 'demographics': '4 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '015f2e1f-e7d5-482b-9f2f-11dd5a6fee68', 'description': 'Axial T1WIs (#1,2) show swollen cortex in both occipital lobes with blurring of gray-white junctions (arrows). T2WIs (#3-5) and FLAIR (#6,7) show hyperintensity in these same areas. Also note anterior extension along the vascular watershed zones (arrows, #5,7). T2*GRE scans (#8-10) show several small hemorrhagic foci (open arrows). DWI and ADC (#11-14) show T2 "shine-through" but no definite restriction. T1C+ scans (#15-17) show no definite enhancement.\n\nComment: Except for the microhemorrhages, this is a typical case of PRES. The presence of microhemorrhages may indicate a component of thrombotic microangiopathy which can be caused by acute malignant hypertension. Most cases of typical PRES do not cause hemorrhage although "variant" PRES with unusual location, hemorrhage, and/or diffusion restriction has been reported to occur in up to 15% of cases.', 'history': 'Acute malignant hypertensive crisis with markedly elevated blood pressure.', 'imagePoolId': '39fe796f-beac-49cb-b603-f095f5e98fdf', 'name': 'PRES plus microhemorrhages', 'teachingPoint': None, 'demographics': '46 Years old female'}, {'authors': [{'key': '5d8b4b75-dddc-4514-9e16-4073e4b8b24a', 'value': 'Nivedita Agarwal, MD'}], 'caseVersionId': '03d6e392-e864-4d75-9211-bf11c80528fd', 'description': 'FLAIR and T2W images show a hyperintense lesion in the right cerebellar hemisphere (arrows, #1-2). Part of this lesion restricts in diffusion-weighted images (arrow, #3) and presents blooming artifact on GRE images, most likely representing blood products (arrows, #5). There is slight enhancement (open arrow, #4) with a prominent vessel seen after contrast administration, a sign of a leaky blood brain barrier (arrow, #4). \n\nThe differential diagnosis with this constellation of findings includes possible infarct with hemorrhagic transformation (either arterial or venous infarct) and/or variant posterior reversible encephalopathic syndrome (PRES). Repeat MR after 3 weeks shows complete resolution of MR findings (#6-8) except for persisting blooming artifact on GRE images (arrow, #9).\n\nComment: The patient has been symptom-free since her first MR. During the 3 weeks, she has been treated with antihypertensive drugs. Given the follow-up MR findings, the most likely diagnosis is PRES with hemorrhagic transformation likely due to a prolonged vasoconstriction and delayed hypertensive treatment.\n\nPRES is typically seen in the posterior part of the brain, though not exclusively. While its etiology remains largely unknown, it is important to consider this diagnosis in patients with hypertension and positive MR findings in the cerebellum, brain stem, and occipital regions.', 'history': 'Patient with sudden headache and seizure-like episode; history of breast cancer; on chemotherapeutic drugs; hypertension noted on admission.', 'imagePoolId': '3b507201-7070-43f5-a36d-26ffb3f1ee41', 'name': 'Complete resolution, GRE remains positive', 'teachingPoint': None, 'demographics': '52 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '2e6ea715-afb5-4306-af99-de03fb3fe716', 'description': 'Axial NECT scans (#1,2) show hypodensity in both occipital lobes and cortical/subcortical watershed zones (arrows). Axial T1WI MR obtained 5 days after emergency delivery (#3), shows large, asymmetric hemorrhagic occipital infarcts (arrows). Axial T2WI MR (#4) shows very large mixed signal lesions in both occipital lobes. \n\nComment: Frank ischemia/infarction are uncommon complications of PRES.', 'history': 'Pregnant female patient with headaches, severe HTN and cortical blindness. CT was obtained on admission. Emergency delivery was performed. Follow-up MR scan was obtained approximately 5 days after initial presentation.', 'imagePoolId': '1bb243df-cfd5-4224-afa5-8785c50b8bb6', 'name': 'Sequelae (gross hemorrhage)', 'teachingPoint': None, 'demographics': '15 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '64c52f30-61ab-4bfa-b3d4-a3badea8b047', 'description': "A series of CECT scans (Figs. 1-4) show hypodensity in the cerebellar white matter with severe cerebellar swelling (, Fig. 1). Upward herniation of the cerebellum into the tentorial incisura displaces the quadrigeminal plate cistern anteriorly (, Fig. 2). The aqueduct is occluded, and there is severe hydrocephalus with transependymal CSF flow. The cerebral white matter appears more hypodense than normal on image 4; this could be from the obstructive hydrocephalus or can also be seen with acute hypertensive encephalopathy, also known as PRES (posterior reversible encephalopathy syndrome). Comment: This case is deemed a variant because of the cerebellar (rather than occipital lobe) predominance.", 'history': 'Patient with acute renal failure presented with seizures. Patient arrested and died after the scan was obtained.', 'imagePoolId': '0e9d3349-ed98-46f1-93fc-ee1b6261cb6f', 'name': 'Cerebellar PRES', 'teachingPoint': None, 'demographics': '2 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '6ac9187c-383c-4516-83a9-6649068bc46c', 'description': 'Axial NECT scans (#1-3) show bilateral hypodensities in both occipital lobes (arrows) with a single focus of high density indicating hemorrhage (open arrow, #1). MR was obtained. Sagittal (#4) and axial (#5) T1WIs show hypodensity in the occipital cortex and subcortical white matter (arrows). T2WIs (#6-8) and FLAIR (#9-11) show hyperintensity in the same areas along with a very hyperintense focus (open arrows, #6, 10) of acute hemorrhage. No enhancement was seen on T1 C+ study (#12). DWI scans (#13-15) showed some hyperintensity in both occipital lobes that are also hyperintense on ADC (#16-18). The hemorrhage is hypointense.\n\nComment: Hemorrhage is uncommon (although not rare) in cases of PRES.', 'history': 'Renal transplant patient on cyclosporin developed severe headaches, vision problems. Markedly elevated blood pressure on admission.', 'imagePoolId': 'ba656ee6-6fba-454f-8d92-ff00565111f1', 'name': 'Hemorrhage', 'teachingPoint': None, 'demographics': '24 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '7ad1099c-4b7a-42c4-8c36-3f0a46cf3f6a', 'description': 'Axial FLAIR scans (#1-8) show multiple cortical/subcortical foci of increased signal intensity (arrows) mostly in the occipital lobes, cerebellum, thalami and watershed zones. Signal in the subarachnoid spaces indicates some subarachnoid hemorrhage as well (open arrows, #5,6). T2WIs (#9-16) show multifocal hyperintensities (arrows) in the same areas. Coronal T2* GRE scans (#17-22) show no evidence for petechial hemorrhage.\n\nComment: Findings of PRES-like changes in malignant hypertension with thrombocytopenia and hemolytic anemia are consistent with one of the causes of thrombotic microangiopathy, TTP.', 'history': 'History of COPD and poorly controlled hypertension presented with cutaneous hemorrhages, seizure, deteriorating mental status, markedly elevated blood pressure. Laboratory findings on admission indicated anemia with thrombocytopenia, acute renal failure.\n\n', 'imagePoolId': '0360329d-f9fa-4680-b158-b534991a139d', 'name': 'TTP', 'teachingPoint': None, 'demographics': '66 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '7cede56b-33c4-4c03-9d81-b750f79fab9d', 'description': 'Axial FLAIR scans (#1, 2) show patchy areas of increased signal intensity in the pons and both caudate nuclei. A small focus of hyperintensity is also present in the left putamen/external capsule. Axial DWI scans (#3, 4) show restriction in all areas that were abnormal on the FLAIR scans. The putamen lesion (#4, arrow) shows only mild restriction; the other lesions show striking hyperintensity on DWI. \n\nThe remainder of the brain, including the posterior cortex, was normal.\n\nThis case is unusual for two reasons: (1) The distribution of the lesions, with pontine and caudate involvement sparing the occipital lobes and (2) the presence of restricted diffusion on DWI. Most cases of so-called posterior reversible encephalopathy syndrome (PRES) involve areas supplied by the posterior cerebral artery and are diffusion-negative.', 'history': 'Pregnant patient with eclampsia.', 'imagePoolId': 'dc63ab19-6d53-4e80-8d21-9514cb642ac5', 'name': 'DWI positive', 'teachingPoint': None, 'demographics': '25 Years old female'}, {'authors': [{'key': '07a2c087-6202-49e7-870b-7aa162d18f06', 'value': 'Bronwyn E. Hamilton, MD'}], 'caseVersionId': 'bf1937b7-c97c-4f80-9c4e-88331be0dd8d', 'description': "Axial T2 (#1) and FLAIR (#2) on day 1 demonstrate bilateral confluent areas of hyperintensity (arrows) that were extensive but demonstrated a posterior hemispheric predilection. Cortex is spared, as is typical for uncomplicated PRES. T1-weighted images show mild mass effect with sulcal effacement, without significant signal abnormality (#3). No abnormal enhancement was seen on post contrast scan (#4). DWI (#5) shows no acute restriction but extensive vasogenic edema (curved arrows) on ADC (#6). \n\nPatient returned for limited perfusion imaging on the 2nd day, which showed low relative cerebral blood volume in the affected white matter (arrows, #7). DWI obtained concurrently (not shown) was stable, with no acute restriction. Day\n2 axial FLAIR (#8) shows extensive abnormal hyperintense signal diffusely throughout the subarachnoid fluid spaces (open arrows), compatible with gadolinium enhancement due to extravascular leakage of contrast. \n\nComment: Since the patient's renal function remained normal throughout her hospitalization, the contrast accumulation is consistent with altered blood-brain barrier permeability during the patient's acute hypertensive crisis. The patient also had no other known explanations for cerebrospinal fluid hyperintensity on FLAIR, such as scanner artifacts or administration of high fractional inspired oxygen concentration during the exam.", 'history': 'Patient admitted with acute hypertensive crisis and altered mental status. During hospital stay, renal function was normal. ', 'imagePoolId': '1232d9c8-7a70-4f6e-8ca7-cdfb887bd45a', 'name': 'Enhancing CSF', 'teachingPoint': None, 'demographics': '78 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'cf6e03ba-3b5c-482b-9b74-aad31df98e3d', 'description': 'Axial T2 (Figs. 1-5) and FLAIR (Figs. 6-9) scans show hyperintensity in the pons, midbrain, both medial thalami, and occipital lobes. No diffusion restriction is seen, although there is mild T2 shine-through on the DWI image (Fig. 10).', 'history': 'Eclamptic patient presents with seizures, severe hypertension.', 'imagePoolId': '91599018-c7e8-4190-9ab6-3550cab38f48', 'name': 'Bilateral thalami', 'teachingPoint': 'The brainstem and thalamic involvement in this patient with posterior reversible encephalopathy syndrome (PRES) is unusually striking. In a few cases, involvement of the brainstem and cerebellum may be the only manifestations of PRES. In this case, the occipital involvement and clinical history make the diagnosis all but certain.', 'demographics': '22 Years old female'}, {'authors': [{'key': '815f3e98-b5da-43c7-8f99-d3db52947320', 'value': 'Hank Baskin, MD'}, {'key': 'f7b0bbaf-fa5e-42e3-9d52-3d0c72fbf3ba', 'value': 'Jill Stein, MD'}], 'caseVersionId': 'e8767621-6380-4cb8-b716-f41f17656bed', 'description': "Axial T2 FLAIR MR images (Figs. 1-4) show multiple foci of abnormal hyperintense signal throughout the brainstem and deep nuclei, including the pons, left cerebral peduncle, left anterior thalamus, and left centrum semiovale .\n\nAxial T1 post-contrast (Fig. 5) and diffusion-weighted images (Fig. 6) demonstrate lack of enhancement and restricted diffusion, respectively. This pattern of brainstem and deep nuclei involvement can be seen in an atypical distribution of acute hypertensive encephalopathy [also known as Posterior reversible encephalopathy syndrome (PRES)].", 'history': 'Patient presents with altered mental status and hypoplastic left heart syndrome.', 'imagePoolId': '33566052-c477-4dd5-9a8b-fb544232d86b', 'name': 'Atypical distribution in the brainstem and deep nuclei', 'teachingPoint': None, 'demographics': '3 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'b0a7c4a5-53e1-48e5-ad08-9933940c239a', 'description': 'While acute hypertensive encephalopathy typically affects the posterior circulation, occasionally it can predominately or exclusively involve the brainstem, cerebellum, or thalami.\n\nAxial T2 weighted MR scans (#1-4) show increased signal intensity in the cerebellum, pons, midbrain and thalami. The posterior circulation is completely normal.', 'history': 'Pregnant patient with eclampsia, coma.', 'imagePoolId': '0efc4e48-53ab-4786-b98a-4032c5d56f49', 'name': 'Striking BS', 'teachingPoint': None, 'demographics': '40 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '82d712d4-cc39-4eb9-8778-4d821fc80bf1', 'description': 'The majority of cases with acute hypertensive encephalopathy resolve without residua. In exceptionally severe cases, gross hemorrhage with hydrocephalus, brain herniation and death may result.\n\nAxial NECT scans show massive intraventricular hemorrhage with severe obstructive hydrocephalus and blood-CSF levels (#1, open arrows). Presence of symmetric low density in both basal ganglia (#1, arrows) and at the posterior parieto-occipital cortex and subcortical white matter (#2, curved arrow) suggest this case represents a variant of posterior reversible encephalopathy syndrome (PRES).', 'history': 'Pregnant patient with seizure followed by coma and decerebrate posturing.', 'imagePoolId': '4e227daa-264d-4f24-a3ae-4354fcd6ed40', 'name': 'Atypical eclampsia', 'teachingPoint': None, 'demographics': '30 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '567e2296-a61c-47de-814f-865a137b7691', 'description': 'Axial NECT scans (#1-3) show profound hypodensity throughout the cerebral white matter and especially within both parietal lobes (open arrows, #2, 3). There is a focal hyperdense area, (arrow, #2) that probably represents frank parenchymal hemorrhage.\n\nThe findings are those of acute hypertensive encephalopathy or PRES (Posterior Reversible Encephalopathy Syndrome). The findings are identical to PRES caused by eclampsia, cyclosporine toxicity, etc. This case is deemed variant because cocaine is an unusual cause of PRES.', 'history': 'Patient presents with extreme, sudden hypertension after using cocaine.', 'imagePoolId': '636ad180-0c98-4293-8b68-f290a9678dbc', 'name': 'Florid', 'teachingPoint': None, 'demographics': '18 Years old female'}], 'caseType': 'variant', 'name': 'VARIANT'} + + +## Images + + +### Selected Images + +![Axial graphic shows the classic posterior circulation cortical/subcortical vasogenic edema characteristic of posterior reversible encephalopathy syndrome (PRES). Petechial hemorrhage occurs in some cases.](images/app.statdx.com_image_thumbnail_cf88eb40-d933-4e88-baf1-0fd98da9de6c_annotated_true_size_900_quality_90_9a4d32f9388f213cdaae74ff51893908cdd7eb2e.jpg) +*Axial graphic shows the classic posterior circulation cortical/subcortical vasogenic edema characteristic of posterior reversible encephalopathy syndrome (PRES). Petechial hemorrhage occurs in some cases.* + +![Axial graphic shows the classic posterior circulation cortical/subcortical vasogenic edema characteristic of posterior reversible encephalopathy syndrome (PRES). Petechial hemorrhage occurs in some cases.](images/app.statdx.com_image_thumbnail_cf88eb40-d933-4e88-baf1-0fd98da9de6c_size_174_quality_85_0dfa29bb35835786a008d38f332705e208db4ead.jpg) +*Axial graphic shows the classic posterior circulation cortical/subcortical vasogenic edema characteristic of posterior reversible encephalopathy syndrome (PRES). Petechial hemorrhage occurs in some cases.* + +![Gross pathology of a patient with complicated PRES demonstrates diffuse cerebral edema with swollen gyri. Multifocal petechial microhemorrhages are present in the occipital cortex with several areas of focal encephalomalacia secondary to infarction . (Courtesy R. Hewlett, MD.)](images/app.statdx.com_image_thumbnail_ed09a209-7899-41a6-bd0a-461a0c2e1b78_annotated_true_size_900_quality_90_d4822b32246c9675fca63308715b688d7eb3e3aa.jpg) +*Gross pathology of a patient with complicated PRES demonstrates diffuse cerebral edema with swollen gyri. Multifocal petechial microhemorrhages are present in the occipital cortex with several areas of focal encephalomalacia secondary to infarction . (Courtesy R. Hewlett, MD.)* + +![Axial NECT of a 54-year-old patient with liver transplant on Tacrolimus FK 506 who presented with seizures demonstrates asymmetric subcortical edema in the occipital lobes bilaterally . Findings are typical of PRES.](images/app.statdx.com_image_thumbnail_d3d649dc-6807-4936-9796-76b0ab07c078_annotated_true_size_900_quality_90_7f3fdeb067ea4f5ddd0c00025d3e4f084eebf2f4.jpg) +*Axial NECT of a 54-year-old patient with liver transplant on Tacrolimus FK 506 who presented with seizures demonstrates asymmetric subcortical edema in the occipital lobes bilaterally . Findings are typical of PRES.* + +![Axial FLAIR MR in a 20-year-old eclamptic woman who presented with severe HTN, seizures, & altered sensorium shows classic findings of PRES with occipital cortical/subcortical edema . Her BP at presentation was 210/140.](images/app.statdx.com_image_thumbnail_3425d950-8ee3-44bf-b2db-861811f8cb18_annotated_true_size_900_quality_90_95663eab8924715f4e91e20fd528abcb74faad7d.jpg) +*Axial FLAIR MR in a 20-year-old eclamptic woman who presented with severe HTN, seizures, & altered sensorium shows classic findings of PRES with occipital cortical/subcortical edema . Her BP at presentation was 210/140.* + +![Axial FLAIR MR in a patient with renal failure presenting with headache, seizures, & visual disturbances demonstrates bilateral parietooccipital cortical/subcortical hyperintensities .](images/app.statdx.com_image_thumbnail_b69ba14c-afa9-446e-93e0-2bdc27518cee_annotated_true_size_900_quality_90_84e6322adcb9e88c81a3f6d5a92c66c10592ea36.jpg) +*Axial FLAIR MR in a patient with renal failure presenting with headache, seizures, & visual disturbances demonstrates bilateral parietooccipital cortical/subcortical hyperintensities .* + +![Axial DWI MR in the same patient shows patchy areas of cortical restricted diffusion . Diffusion is usually negative in PRES. PRES with restricted diffusion occurs in 15-30% of cases & is usually seen as smaller cortical foci of restricted diffusion within larger regions of vasogenic edema, as in this case.](images/app.statdx.com_image_thumbnail_9f7efe50-65d8-4f6c-a2bb-b7ede9f71153_annotated_true_size_900_quality_90_67220013168fbe3dd1da1c721891daaaafb07cf8.jpg) +*Axial DWI MR in the same patient shows patchy areas of cortical restricted diffusion . Diffusion is usually negative in PRES. PRES with restricted diffusion occurs in 15-30% of cases & is usually seen as smaller cortical foci of restricted diffusion within larger regions of vasogenic edema, as in this case.* + +![Axial FLAIR MR in an 11-year-old patient with acute lymphoblastic leukemia (ALL) on chemotherapy shows cortical/subcortical edema in frontal (superior frontal sulcus pattern) & parietal lobes (typical parietooccipital pattern).](images/app.statdx.com_image_thumbnail_86798adb-110f-482b-95a2-a0ebb7c3aa54_annotated_true_size_900_quality_90_84eec1bf0c0c20ade1c3ee6b236fab1ef51a76f2.jpg) +*Axial FLAIR MR in an 11-year-old patient with acute lymphoblastic leukemia (ALL) on chemotherapy shows cortical/subcortical edema in frontal (superior frontal sulcus pattern) & parietal lobes (typical parietooccipital pattern).* + +![Axial T1 C+ MR in the same patient shows extensive patchy areas of juxtacortical enhancement . Although etiology of PRES is not completely understood, leading theories include loss of autoregulation & endothelial dysfunction. FLAIR changes & enhancement resolved on follow-up MR.](images/app.statdx.com_image_thumbnail_04e809b1-a78f-4050-945a-2abb78307360_annotated_true_size_900_quality_90_30618104d18095ab43ec81125745b053d769a174.jpg) +*Axial T1 C+ MR in the same patient shows extensive patchy areas of juxtacortical enhancement . Although etiology of PRES is not completely understood, leading theories include loss of autoregulation & endothelial dysfunction. FLAIR changes & enhancement resolved on follow-up MR.* + +![Axial SWI MR in a hypertensive patient who presented with visual disturbances & seizures demonstrates the 3 patterns of hemorrhages which can be seen in PRES. This case shows lobar hemorrhage , cortical SAH , and microhemorrhages .](images/app.statdx.com_image_thumbnail_c197f8ad-f0b9-4445-99b7-0dc7ab22960a_annotated_true_size_900_quality_90_95bf81cbcdbe69c1507b33da28bcca2df0dea33e.jpg) +*Axial SWI MR in a hypertensive patient who presented with visual disturbances & seizures demonstrates the 3 patterns of hemorrhages which can be seen in PRES. This case shows lobar hemorrhage , cortical SAH , and microhemorrhages .* + +![Axial FLAIR MR in a patient with preeclampsia shows atypical findings of PRES with vasogenic edema involving the pons & cerebellum . It is important to remember that edema in PRES is not just posterior and not always reversible.](12ffa839-3fee-43d3-9370-a65b5ab7ef3f) +*Axial FLAIR MR in a patient with preeclampsia shows atypical findings of PRES with vasogenic edema involving the pons & cerebellum . It is important to remember that edema in PRES is not just posterior and not always reversible.* + + +### Additional Images + +![Axial NECT shows variant findings of PRES, notably bilateral but asymmetric occipital lobe hypodensities & focal hemorrhage . Most cases of PRES do not hemorrhage. If they do, multifocal small cortical petechial-type bleeds are typical. Gross hemorrhage is uncommon.](473e8fb3-ecec-4151-bfb2-fbddc8de5fe5) +*Axial NECT shows variant findings of PRES, notably bilateral but asymmetric occipital lobe hypodensities & focal hemorrhage . Most cases of PRES do not hemorrhage. If they do, multifocal small cortical petechial-type bleeds are typical. Gross hemorrhage is uncommon.* + +![Axial NECT in an eclamptic patient shows some variant changes of PRES with bilateral basal ganglia lesions & intraventricular hemorrhage with fluid-fluid levels in the occipital horns .](8a3b705a-293e-4a5f-969a-8175e8a9af53) +*Axial NECT in an eclamptic patient shows some variant changes of PRES with bilateral basal ganglia lesions & intraventricular hemorrhage with fluid-fluid levels in the occipital horns .* + +![Axial NECT in an eclamptic woman shows bilateral hypodensities in the occipital lobes , characteristic of PRES.](946cb173-e600-466e-b299-76c27fd39b1b) +*Axial NECT in an eclamptic woman shows bilateral hypodensities in the occipital lobes , characteristic of PRES.* + +![Axial NECT shows bioccipital as well as watershed hypodensities in this patient with severe HTN.](3d9136b5-86f3-4325-aa4b-32638aea6521) +*Axial NECT shows bioccipital as well as watershed hypodensities in this patient with severe HTN.* + +![Axial FLAIR MR in the same patient shows hyperintensity in the white matter of both occipital lobes . Subtle cortical hypointensity may represent petechial hemorrhage.](33f4e29c-fc28-4eb3-8141-b0d5da39d59f) +*Axial FLAIR MR in the same patient shows hyperintensity in the white matter of both occipital lobes . Subtle cortical hypointensity may represent petechial hemorrhage.* + +![Axial FLAIR MR shows cortical & subcortical edema in both occipital lobes, as well as the posterior left frontal lobe .](d7152266-fa3e-4b06-8c5f-a7d9c60899fd) +*Axial FLAIR MR shows cortical & subcortical edema in both occipital lobes, as well as the posterior left frontal lobe .* + +![Axial T1 C+ MR in the same patient shows multifocal punctate areas of enhancement indicating active blood-brain barrier disruption in this case of PRES.](d32f4d04-f527-4bd1-8457-13fc8d6223c5) +*Axial T1 C+ MR in the same patient shows multifocal punctate areas of enhancement indicating active blood-brain barrier disruption in this case of PRES.* + +![Axial FLAIR MR shows bilateral occipital hyperintensity in the cortex & subcortical white matter . There is striking hyperintensity in the pons .](b8581875-f1dd-4d4b-9175-74284df20657) +*Axial FLAIR MR shows bilateral occipital hyperintensity in the cortex & subcortical white matter . There is striking hyperintensity in the pons .* + +![Axial NECT in a 4-year-old hypertensive child with hemolytic uremic syndrome & renal failure shows hypodensity in the basal ganglia & thalami . The occipital lobes are normal.](46b13dbb-7bb6-4041-bc4a-21b6cd7e5ae2) +*Axial NECT in a 4-year-old hypertensive child with hemolytic uremic syndrome & renal failure shows hypodensity in the basal ganglia & thalami . The occipital lobes are normal.* + +![Axial NECT of a 26-year-old pregnant patient with eclampsia was initially read as normal; however, it shows subtle but definite hypodensities in the cortex & subcortical white matter of both occipital lobes.](9ae3ce5f-6721-4b4e-bcdd-86635fd1ffd1) +*Axial NECT of a 26-year-old pregnant patient with eclampsia was initially read as normal; however, it shows subtle but definite hypodensities in the cortex & subcortical white matter of both occipital lobes.* + +![Axial T2 MR in the same patient shows hyperintensities in both occipital lobes corresponding to the hypodensities noted on NECT. DWI (not shown) was normal. If clinical suspicion of PRES is high & NECT is scan normal/subtly abnormal, MR with T2WI, FLAIR, & DWI is helpful.](fd3b899f-389b-4de2-b1c7-eafcf373ddd6) +*Axial T2 MR in the same patient shows hyperintensities in both occipital lobes corresponding to the hypodensities noted on NECT. DWI (not shown) was normal. If clinical suspicion of PRES is high & NECT is scan normal/subtly abnormal, MR with T2WI, FLAIR, & DWI is helpful.* + +![Axial T2 MR in a patient on cyclosporine who developed acute onset of extreme HTN shows symmetric hyperintensities in both cerebellar hemispheres .](ff7988ec-9821-4174-a45e-eb8e1ee83053) +*Axial T2 MR in a patient on cyclosporine who developed acute onset of extreme HTN shows symmetric hyperintensities in both cerebellar hemispheres .* + +![Axial T2 MR in the same patient shows florid changes in the cortical watershed zones . DWI showed no restriction, which is typical even in severe cases of PRES. All findings resolved when the patient was taken off chemotherapy & BP normalized.](fca3dc05-b8f8-492b-b67d-703f9d011c20) +*Axial T2 MR in the same patient shows florid changes in the cortical watershed zones . DWI showed no restriction, which is typical even in severe cases of PRES. All findings resolved when the patient was taken off chemotherapy & BP normalized.* + +![Axial T2 MR in the same patient shows striking hyperintensity in both basal ganglia with relatively subtle findings in the occipital poles .](776db345-8ee5-45a7-9fef-73805f334374) +*Axial T2 MR in the same patient shows striking hyperintensity in both basal ganglia with relatively subtle findings in the occipital poles .* + +![Axial T2 MR in a patient with PRES shows pontine-predominant pattern with only subtle change in the occipital lobe . Sometimes pontine or cerebellar abnormalities can be found without other imaging evidence of PRES.](ed1306a8-406e-428c-b58b-6eaf0390b793) +*Axial T2 MR in a patient with PRES shows pontine-predominant pattern with only subtle change in the occipital lobe . Sometimes pontine or cerebellar abnormalities can be found without other imaging evidence of PRES.* + +![Axial T1 C+ MR in a patient with eclampsia shows numerous patchy cortical & subcortical enhancing foci in both occipital lobes & along the watershed zones. T2WIs (not shown) demonstrated hyperintensities in the same areas.](8563178a-3925-451b-bdd3-05da3fc001fd) +*Axial T1 C+ MR in a patient with eclampsia shows numerous patchy cortical & subcortical enhancing foci in both occipital lobes & along the watershed zones. T2WIs (not shown) demonstrated hyperintensities in the same areas.* + +![Repeat scan was obtained 2 days after delivery & normalization of BP. MR is normal with the disappearance of the enhancing foci previously seen. Even florid MR changes of PRES usually resolve without clinical or imaging residua.](dbd77781-6aad-4318-b98b-2e4bd3d54c52) +*Repeat scan was obtained 2 days after delivery & normalization of BP. MR is normal with the disappearance of the enhancing foci previously seen. Even florid MR changes of PRES usually resolve without clinical or imaging residua.* + +![Axial FLAIR MR in a patient with systemic lupus erythematosus (SLE) & renal failure demonstrates cortical/subcortical edema in the frontal & parietal lobes .The frontal lobes are involved in > 70 % of PRES cases.](143af35b-0b9b-465c-8a20-bceb9ad1dc6f) +*Axial FLAIR MR in a patient with systemic lupus erythematosus (SLE) & renal failure demonstrates cortical/subcortical edema in the frontal & parietal lobes .The frontal lobes are involved in > 70 % of PRES cases.* + +![Axial DWI MR in the same patient shows some patchy areas of frontal cortical restricted diffusion . Because most cases of PRES are caused by vasogenic & not cytotoxic edema, DWI is usually negative. Diffusion restriction has been reported like in this case & may lead to residual abnormalities.](256fbdb2-67ab-47e4-8520-35501e3982e7) +*Axial DWI MR in the same patient shows some patchy areas of frontal cortical restricted diffusion . Because most cases of PRES are caused by vasogenic & not cytotoxic edema, DWI is usually negative. Diffusion restriction has been reported like in this case & may lead to residual abnormalities.* + +![Axial T2 MR in a patient with sepsis & slightly elevated BP shows atypical findings of PRES. There is extensive edema involving the pons & both the middle cerebellar peduncles .](92cf02e6-bcc3-4212-b672-0c60f8fbb192) +*Axial T2 MR in a patient with sepsis & slightly elevated BP shows atypical findings of PRES. There is extensive edema involving the pons & both the middle cerebellar peduncles .* + diff --git a/docs_md/articles/acute-ischemic-stroke_69a7a1f7-9c78-4ad1-82dd-9b13f2e717b3.md b/docs_md/articles/acute-ischemic-stroke_69a7a1f7-9c78-4ad1-82dd-9b13f2e717b3.md new file mode 100644 index 0000000..6ba7664 --- /dev/null +++ b/docs_md/articles/acute-ischemic-stroke_69a7a1f7-9c78-4ad1-82dd-9b13f2e717b3.md @@ -0,0 +1,482 @@ +--- +title: "Acute Ischemic Stroke" +docid: "69a7a1f7-9c78-4ad1-82dd-9b13f2e717b3" +authors: + - key: "07a2c087-6202-49e7-870b-7aa162d18f06" + value: "Bronwyn E. Hamilton, MD" +breadcrumbs: + - + name: "Vasculature" + slug: "vasculature" + treeNodeId: "9d3db335-364f-44ec-b2e2-30b03ce93228" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "4a210126-9f87-404e-b419-a73f44d0e94c" + - + name: "Extracranial Cerebral Arteries" + slug: "extracranial-cerebral-arteries" + treeNodeId: "593e17de-cd84-4587-8349-872ed33d28c4" + - + name: "Acute Ischemic Stroke" + slug: "acute-ischemic-stroke" + treeNodeId: null +category: "Vasculature" +cmeTopicId: "269a1301-f57a-4247-b62a-de2d927ea3bf" +documentVersionId: "90d363cc-bb77-48d7-ae31-85fefda3de0c" +imageCount: 32 +lastUpdated: "04/18/16" +pageDescription: "Acute Ischemic Stroke" +pageKeywords: "Vasculature, Diagnosis, Extracranial Cerebral Arteries, Acute Ischemic Stroke" +pageTitle: "Acute Ischemic Stroke | STATdx" +enhancedTitle: "Acute Ischemic Stroke" +type: "DX" +references: true +breadcrumbs: + - "Vasculature" + - "Diagnosis" + - "Extracranial Cerebral Arteries" + - "Acute Ischemic Stroke" +--- +# KEY FACTS + +- ## Terminology + + + - Interrupted blood flow to brain resulting in cerebral ischemia/infarction with variable neurologic deficit +- ## Imaging + + + - Major artery (territorial) infarct + - Generally wedge-shaped; both GM & WM involved + - Embolic infarcts + - Often focal/small, at GM-WM interface + - NECT + - Hyperdense vessel (high specificity, low sensitivity) + - "Dense MCA" sign: Acute thrombus in middle cerebral artery + - Loss of GM-WM distinction in 1st 3 hours (50-70%) + - "Insular ribbon" sign: Loss of GM-WM differentiation of insular cortex + - MR + - Best diagnostic clue is high signal on DWI with corresponding low signal on ADC + - ↓ CBF and ↓ CBV on perfusion MR (or CT) +- ## Top Differential Diagnoses + + + - Hyperdense vessel mimics + - Parenchymal hypodensity (nonvascular causes) +- ## Pathology + + + - Severely ischemic core + - CBF < (6-8 mL)/(100 g/min) + - Peripheral penumbra + - CBF = (10-20 mL)/(100 g/min) +- ## Clinical Issues + + + - 2nd most common cause of death worldwide + - Leading cause of morbidity in USA + - Treatment + - IV thrombolysis (< 3 hours of symptom onset) + - IA thrombolysis (selected acute strokes < 6 hours) + - Clinical diagnosis inaccurate in 15-20% of strokes + +# TERMINOLOGY + +- ## Synonyms + + + - Stroke, brain attack, cerebrovascular accident +- ## Definitions + + + - Interrupted blood flow to brain resulting in cerebral ischemia/infarction with variable neurologic deficit + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - High signal on DWI with corresponding low signal on ADC + - Decreased cerebral blood flow (CBF) and cerebral blood volume (CBV) on CT or MR perfusion + - ### Location + + + - 1 or more vascular territories or at border zones (watershed) + - ### Size + + + - Dependent on degree of compromise and collateral circulation + - ### Morphology + + + - Territorial infarct + - Conforms to arterial territory + - Generally wedge-shaped + - Both gray matter (GM) and white matter (WM) are involved + - Embolic infarcts (often focal, at GM-WM interface) +- ## CT Findings + + + - ### NECT + + + - Hyperdense vessel (high specificity, low sensitivity) + - Represents acute thrombus in cerebral vessel(s) + - Hyperdense M1 segment of middle cerebral artery (MCA) in 35-50%; most common vessel involved + - "Dot" sign: Occluded MCA branches in sylvian fissure (16-17%) + - Loss of gray-white matter (GM-WM) distinction in 1st 3 hours (50-70%) + - Obscuration of deep gray nuclei + - Loss of cortical "ribbon" + - Parenchymal hypodensity + - If > 1/3 MCA territory initially hypodense, then larger lesion usually develops later + - Temporary transition to isodensity (up to 54%) at 2-3 weeks post ictus (CT "fogging") + - Gyral swelling, sulcal effacement 12-24 hours + - "Hemorrhagic transformation" in 15-45% + - Delayed onset (24-48 hours) most typical + - Can be gross (parenchymal) or petechial + - ### CECT + + + - Enhancing cortical vessels: Slow flow or collateralization acutely + - Absent vessels: Occlusion + - Perfusion CT (pCT): Assess ischemic core vs. penumbra; identify patients who benefit most from revascularization + - pCT calculates CBF, CBV, time to peak (TTP) + - Deconvolution can give mean transit time (MTT) + - Cortical/gyral enhancement after 48-72 hours + - CTA: Identify occlusions, dissections, stenoses, collaterals +- ## MR Findings + + + - ### T1WI + + + - Early cortical swelling and hypointensity, loss of GM-WM borders + - ### T2WI + + + - Cortical swelling, hyperintensity after 12-24 hours + - May normalize 2-3 weeks post ictus (MR "fogging") + - ### FLAIR + + + - Parenchymal hyperintensity appears (6 hours post ictus) while other sequences normal + - Intraarterial FLAIR hyperintensity is early sign of major vessel occlusion or slow flow + - ### T2* GRE + + + - Detection of acute blood products + - Arterial "blooming" (thrombosed vessel) from clot susceptibility + - ### DWI + + + - Hyperintense restriction from cytotoxic edema + - Improves hyperacute stroke detection to 95% + - Best correlates with "ischemic core" (final infarct size); some diffusion abnormalities reverse + - May have reduced sensitivity in brainstem and medulla during 1st 24 hours + - Restriction typically lasts 7-10 days + - High signal can persist up to 2 months post ictus + - After 10 days, T2 effect may predominate over low ADC: T2 "shine-through" + - Corresponding low signal on ADC maps + - May normalize after tissue reperfusion + - Hyper- or isointensity on ADC map (T2 "shine-through") may mimic diffusion restriction + - Distinguish cytotoxic from vasogenic edema in complicated cases + - May be helpful to evaluate new deficits after tumor resection + - ### PWI + + + - Dynamic contrast bolus or arterial spin-labeling techniques + - Maximum slope gives relative CBF and CBV + - Deconvolution gives absolute values + - Bolus-tracking T2* gadolinium PWI with CBV map + - ↓ perfusion; 75% larger than DWI abnormality + - DWI/PWI mismatch may identify penumbra (potentially viable but at-risk tissue) + - ### T1WI C+ + + + - Variable enhancement patterns evolve over time + - Hyperacute: Intravascular enhancement (stasis from slow antegrade or retrograde collateral flow) + - Acute: Meningeal enhancement (pial collateral flow appears in 24-48 hours, resolves over 3-4 days) + - Subacute: Parenchymal enhancement (appears after 24-48 hours, can persist for weeks/months) + - MRA: Major vessel occlusions, stenoses, status of collaterals + - MRS: Elevated lactate, decreased NAA + - Conventional MR sequences positive in 70-80% + - Restricted diffusion improves accuracy to 95% + - Diffusion tensor imaging (DTI) + - Multidirectional diffusion-weighted images; at least 6 directions can be used to calculate DTI trace and generate ADC maps + - Higher spatial resolution + - May be more sensitive for small ischemic foci, emboli, cortical strokes +- ## Angiographic Findings + + + - Conventional: Vessel occlusion (cut off, tapered, "tram track") + - Slow antegrade flow and slow retrograde collateral flow + - Intraluminal thrombus = filling defect + - Neurointerventional: Intraarterial (IA) fibrinolytic therapy for treatment of selected acute nonhemorrhagic stroke within 6-hour window + - IA mechanical clot removal with retriever device +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR + DWI; T2* GRE + - ### Protocol advice + + + - NECT as initial study to exclude hemorrhage/mass + - CT perfusion and CTA if available + - MR using DWI/FLAIR/GRE ± MRA, PWI + - DSA with thrombolysis in selected patients + +# DIFFERENTIAL DIAGNOSIS + +- ## Hyperdense Vessel Mimics + + + - High hematocrit (polycythemia) + - Microcalcification in vessel wall + - Diffuse cerebral edema makes vessels appear relatively hyperdense + - Normal circulating blood always slightly hyperdense to normal brain +- ## Parenchymal Hypodensity (Nonvascular Causes) + + + - Infiltrating neoplasm (e.g., astrocytoma) + - Cerebral contusion + - Inflammation (cerebritis, encephalitis) + - Evolving encephalomalacia + - Dural venous thrombosis with parenchymal venous congestion and edema + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Common causes + - Thrombotic vs. embolic, dissection, vasculitis, hypoperfusion + - Unusual causes + - Complicated vasculopathy, including posterior reversible encephalopathy syndrome and reversible cerebral vasoconstriction syndrome; venous stroke + - Early: Critical disturbance in CBF + - Severely ischemic core: CBF < (6-8 mL)/(100 g/min) + - Normal CBF ~ (60 mL)/(100 g/min) + - Oxygen depletion, energy failure, terminal depolarization, ion homeostasis failure + - Bulk of final infarct → cytotoxic edema, cell death + - Later: Evolution from ischemia to infarction depends on many factors (e.g., hyperglycemia influences "destiny" of ischemic brain tissue) + - Ischemic penumbra: CBF = (10-20 mL)/(100 g/min) + - Theoretically salvageable tissue + - Target of thrombolysis, neuroprotective agents + - ### Associated abnormalities + + + - Cardiac disease, prothrombotic states + - Additional stroke risk factors: C-reactive protein, homocysteine +- ## Gross Pathologic & Surgical Features + + + - Acute thrombosis of major vessel + - Pale, swollen brain; GM-WM boundaries blurred +- ## Microscopic Features + + + - After 4 hours: Eosinophilic neurons with pyknotic nuclei + - 15-24 hours: Neutrophils invade, and necrotic nuclei look like "eosinophilic ghosts" + - 2-3 days: Blood-derived phagocytes + - 1 week: Reactive astrocytosis, ↑ capillary density + - End result: Fluid-filled cavity lined by astrocytes + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Focal acute neurologic deficit + - Paresis, aphasia, decreased mental status +- ## Demographics + + + - ### Age + + + - Usually older adults + - ### Gender + + + - No gender predilection + - ### Epidemiology + + + - 2nd most common cause of death worldwide + - Among leading causes of morbidity in USA +- ## Natural History & Prognosis + + + - Clinical diagnosis inaccurate in 15-20% of strokes + - Malignant MCA infarct (coma, death) + - Up to 10% of all stroke patients + - Fatal brain swelling with increased ICP +- ## Treatment + + + - "Time is brain": IV thrombolytic therapy window < 3 hours + - IA window < 6 hours except for vertebrobasilar thrombosis (up to 24 hours because of high morbidity and mortality) + - Patient selection most important factor in outcome + - Symptom onset < 6 hours + - No parenchymal hematoma on CT + - < 1/3 MCA territory hypodensity + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - DWI positive for acute stroke only if ADC correlates + - Rarely, ischemia may mimic tumor or encephalitis + + ba323bb3-7781-4789-81b2-774e4fcadb49 + +## References + +# Selected References + +1. [Parrilla G et al: Hemorrhage/contrast staining areas after mechanical intra-arterial thrombectomy in acute ischemic stroke: imaging findings and clinical significance. AJNR Am J Neuroradiol. 33(9):1791-6, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22538076%5Bpmid%5D) +1. [Wang DJ et al: The value of arterial spin-labeled perfusion imaging in acute ischemic stroke: comparison with dynamic susceptibility contrast-enhanced MRI. Stroke. 43(4):1018-24, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22328551%5Bpmid%5D) +1. [Harris AD et al: Diffusion and perfusion MR imaging of acute ischemic stroke. Magn Reson Imaging Clin N Am. 17(2):291-313, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19406360%5Bpmid%5D) +1. [Kranz PG et al: Does diffusion-weighted imaging represent the ischemic core? An evidence-based systematic review. AJNR Am J Neuroradiol. 30(6):1206-12, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19357385%5Bpmid%5D) +1. [Lee KY et al: Distal hyperintense vessels on FLAIR: an MRI marker for collateral circulation in acute stroke? Neurology. 72(13):1134-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19211928%5Bpmid%5D) +1. [Sanossian N et al: Angiography reveals that fluid-attenuated inversion recovery vascular hyperintensities are due to slow flow, not thrombus. AJNR Am J Neuroradiol. 30(3):564-8, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19022866%5Bpmid%5D) +1. [Soares BP, Chien JD, Wintermark M. MR and CT monitoring of recanalization, reperfusion, and penumbra salvage: everything that recanalizes does not necessarily reperfuse! Stroke. 40(3 Suppl):S24-7, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19064812%5Bpmid%5D) +1. [Chen Z et al: Evaluating ischemic stroke with diffusion tensor imaging. Neurol Res. 30(7):720-6, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18513464%5Bpmid%5D) +1. [Provenzale JM et al: Optimization of perfusion imaging for acute cerebral ischemia: review of recent clinical trials and recommendations for future studies. AJR Am J Roentgenol. 191(4):1263-70, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18806174%5Bpmid%5D) +1. [Lell MM et al: New techniques in CT angiography. Radiographics. 26 Suppl 1:S45-62, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=17050518%5Bpmid%5D) +1. [Bourekas EC et al: Intraarterial thrombolytic therapy within 3 hours of the onset of stroke. Neurosurgery. 54(1):39-44; discussion 44-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14683539%5Bpmid%5D) +1. [Diaz J et al: Cerebral ischemia: new risk factors. Cerebrovasc Dis. 17 Suppl 1:43-50, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14694279%5Bpmid%5D) +1. [Fiebach JB et al: Stroke magnetic resonance imaging is accurate in hyperacute intracerebral hemorrhage: a multicenter study on the validity of stroke imaging. Stroke. 35(2):502-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14739410%5Bpmid%5D) +1. [Fiehler J et al: Predictors of apparent diffusion coefficient normalization in stroke patients. Stroke. 35(2):514-9, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14739409%5Bpmid%5D) +1. [Gass A et al: Diffusion-weighted MRI for the "small stuff": the details of acute cerebral ischaemia. Lancet Neurol. 3(1):39-45, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14693110%5Bpmid%5D) +1. [Kelly PJ et al: Inflammation, homocysteine, and vitamin B6 status after ischemic stroke. Stroke. 35(1):12-5, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14657454%5Bpmid%5D) +1. [Kidwell CS et al: Comparison of MRI and CT for detection of acute intracerebral hemorrhage. JAMA. 292(15):1823-30, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15494579%5Bpmid%5D) +1. [Mahagne MH et al: Voxel-based mapping of cortical ischemic damage using Tc 99m L,L-ethyl cysteinate dimer SPECT in acute stroke. J Neuroimaging. 14(1):23-32, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14748205%5Bpmid%5D) +1. [Nakajima M et al: Relationships between angiographic findings and National Institutes of Health stroke scale score in cases of hyperacute carotid ischemic stroke. AJNR Am J Neuroradiol. 25(2):238-41, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14970023%5Bpmid%5D) +1. [Borisch I et al: Preoperative evaluation of carotid artery stenosis: comparison of contrast-enhanced MR angiography and duplex sonography with digital subtraction angiography. AJNR Am J Neuroradiol. 24(6):1117-22, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12812936%5Bpmid%5D) +1. [Eastwood JD et al: Quantitative assessment of the time course of infarct signal intensity on diffusion-weighted images. AJNR Am J Neuroradiol. 24(4):680-7, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12695203%5Bpmid%5D) +1. [Leary MC et al: Validation of computed tomographic middle cerebral artery "dot"sign: an angiographic correlation study. Stroke. 34(11):2636-40, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14593125%5Bpmid%5D) +1. [Tomandl BF et al: Comprehensive imaging of ischemic stroke with multisection CT. Radiographics. 23(3):565-92, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12740462%5Bpmid%5D) +1. [Toyoda K et al: Fluid-attenuated inversion recovery intraarterial signal: an early sign of hyperacute cerebral ischemia. AJNR Am J Neuroradiol. 22(6):1021-9, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11415892%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Coronal graphic illustrates a left M1 occlusion. A proximal occlusion affects the entire middle cerebral artery (MCA) territory, including the basal ganglia, which are perfused by lenticulostriate (perforating) arteries . Acute ischemia is often identified by subtle loss of the gray-white matter interfaces with blurring of the basal ganglia and an "insular ribbon" sign on the initial CT.](images/app.statdx.com_image_thumbnail_d7b90847-cdd7-4a27-af45-d16fbaf0825c_annotated_true_size_900_quality_90_ae4a6e1524c3805d3bfd0264856d430bf0f42ad8.jpg) +*Coronal graphic illustrates a left M1 occlusion. A proximal occlusion affects the entire middle cerebral artery (MCA) territory, including the basal ganglia, which are perfused by lenticulostriate (perforating) arteries . Acute ischemia is often identified by subtle loss of the gray-white matter interfaces with blurring of the basal ganglia and an "insular ribbon" sign on the initial CT.* + +![Coronal graphic illustrates a left M1 occlusion. A proximal occlusion affects the entire middle cerebral artery (MCA) territory, including the basal ganglia, which are perfused by lenticulostriate (perforating) arteries . Acute ischemia is often identified by subtle loss of the gray-white matter interfaces with blurring of the basal ganglia and an "insular ribbon" sign on the initial CT.](images/app.statdx.com_image_thumbnail_d7b90847-cdd7-4a27-af45-d16fbaf0825c_size_174_quality_85_10f7bafc073632a03ddc99e94f3c5076aed23eaa.jpg) +*Coronal graphic illustrates a left M1 occlusion. A proximal occlusion affects the entire middle cerebral artery (MCA) territory, including the basal ganglia, which are perfused by lenticulostriate (perforating) arteries . Acute ischemia is often identified by subtle loss of the gray-white matter interfaces with blurring of the basal ganglia and an "insular ribbon" sign on the initial CT.* + +![Axial NECT demonstrates a hyperdense MCA sign representing acute thrombus in a patient with acute stroke symptoms.](images/app.statdx.com_image_thumbnail_cd6ff05d-2068-480a-aca5-aaa34b8c816d_annotated_true_size_900_quality_90_770644d4466fb8eb403758d518b3098b5807d48d.jpg) +*Axial NECT demonstrates a hyperdense MCA sign representing acute thrombus in a patient with acute stroke symptoms.* + +![Axial NECT shows subtle loss of the right temporal gray-white matter interfaces representing an "insular ribbon" sign.](images/app.statdx.com_image_thumbnail_9e5a223f-b4a0-42b6-a5bb-15b9940f4fcd_annotated_true_size_900_quality_90_a27bb99af99a4d4b83537fd705774cfb468ff5d5.jpg) +*Axial NECT shows subtle loss of the right temporal gray-white matter interfaces representing an "insular ribbon" sign.* + +![Axial pCT (CBF) shows decreased blood flow in the right hemisphere related to hyperacute MCA ischemia. The CBF and CBV color maps cephalad to this slice showed a large MCA wedge-shaped defect. There was a similar perfusion abnormality on the TTP maps (not shown). Lack of a mismatch between CBV and TTP maps suggests that no ischemic penumbra is present.](images/app.statdx.com_image_thumbnail_5cc848a6-5f37-47ba-a21a-e67604191219_annotated_true_size_900_quality_90_f486338b48928fc19f790476769c9e0319a3b8ff.jpg) +*Axial pCT (CBF) shows decreased blood flow in the right hemisphere related to hyperacute MCA ischemia. The CBF and CBV color maps cephalad to this slice showed a large MCA wedge-shaped defect. There was a similar perfusion abnormality on the TTP maps (not shown). Lack of a mismatch between CBV and TTP maps suggests that no ischemic penumbra is present.* + +![Axial DWI MR shows a large wedge-shaped hyperintensity related to restricted diffusion representing acute ischemia in a left MCA distribution. There is sparing of the basal ganglia, consistent with distal M1 occlusion.](images/app.statdx.com_image_thumbnail_0f80c43f-b4da-4fef-ad8a-86bcbd6d4bd6_annotated_true_size_900_quality_90_684828ab35ac603104d554bb52944edd88ea6ff5.jpg) +*Axial DWI MR shows a large wedge-shaped hyperintensity related to restricted diffusion representing acute ischemia in a left MCA distribution. There is sparing of the basal ganglia, consistent with distal M1 occlusion.* + +![Axial NECT shows a hypodense wedge-shaped region of acute infarct with mild mass effect and sulcal effacement related to a right M1 embolic occlusion due to a calcified thrombus .](images/app.statdx.com_image_thumbnail_bb73fb8d-fcf8-4b77-bb6e-fb50954439b3_annotated_true_size_900_quality_90_d7bef81f894536c8a416f7099039b0cde8d46da5.jpg) +*Axial NECT shows a hypodense wedge-shaped region of acute infarct with mild mass effect and sulcal effacement related to a right M1 embolic occlusion due to a calcified thrombus .* + +![Axial NECT demonstrates bilateral posterior circulation hypodensities in a 20-month-old boy presenting with seizures after recent circumcision complicated by hematoma.](images/app.statdx.com_image_thumbnail_41e21631-0dbb-46e2-93f5-0621368dd75d_annotated_true_size_900_quality_90_37375e09a67910f0d91a167986ab69d77c588cc0.jpg) +*Axial NECT demonstrates bilateral posterior circulation hypodensities in a 20-month-old boy presenting with seizures after recent circumcision complicated by hematoma.* + +![Axial NECT shows hyperdense thrombus in the distal basilar artery of a 66-year-old woman with altered sensorium. Percutaneous thrombolysis is usually considered at later time points, up to 24 hours, because of the high morbidity and mortality associated with basilar thrombosis.](images/app.statdx.com_image_thumbnail_3061df18-1a49-4b69-b814-76d57d71e6eb_annotated_true_size_900_quality_90_c714b8a854530d96a507cee65dbdb27714847669.jpg) +*Axial NECT shows hyperdense thrombus in the distal basilar artery of a 66-year-old woman with altered sensorium. Percutaneous thrombolysis is usually considered at later time points, up to 24 hours, because of the high morbidity and mortality associated with basilar thrombosis.* + +![Axial DWI MR shows hyperintensity related to restricted diffusion in a patient with vertebrobasilar disease and a posterior inferior cerebellar artery acute infarct. MR is superior to CT in evaluation of a posterior fossa stroke.](images/app.statdx.com_image_thumbnail_5570a6b6-3141-429e-aefd-a45e7d346c52_annotated_true_size_900_quality_90_90a75c12d5a50adf4886f5806f231fc4e43f7446.jpg) +*Axial DWI MR shows hyperintensity related to restricted diffusion in a patient with vertebrobasilar disease and a posterior inferior cerebellar artery acute infarct. MR is superior to CT in evaluation of a posterior fossa stroke.* + +![Coronal CTA MIP reconstruction shows a focal filling defect within the proximal M1 segment in a patient with acute MCA ischemia. Intraarterial thrombolysis may be helpful if the patient presents to the emergency department within 6 hours of symptoms onset.](cd70d1c4-869e-483d-b534-7c49c3c1696f) +*Coronal CTA MIP reconstruction shows a focal filling defect within the proximal M1 segment in a patient with acute MCA ischemia. Intraarterial thrombolysis may be helpful if the patient presents to the emergency department within 6 hours of symptoms onset.* + +![Angiography in a 27-year-old man with a history of methamphetamine and tobacco use shows focal tight stenosis within the distal right M1 segment . He presented with stuttering symptoms of left-sided weakness and face droop.](03e8a370-d0ac-4d38-b8db-955a61ad371b) +*Angiography in a 27-year-old man with a history of methamphetamine and tobacco use shows focal tight stenosis within the distal right M1 segment . He presented with stuttering symptoms of left-sided weakness and face droop.* + +![Sagittal T2WI MR shows multiple watershed ischemic foci in the deep white matter in a "string of pearls" configuration.](a6fe6f87-7d1b-456b-96ec-1302e8c2485b) +*Sagittal T2WI MR shows multiple watershed ischemic foci in the deep white matter in a "string of pearls" configuration.* + +![Axial T2* GRE MR shows multifocal hemorrhages within an ischemic infarct in a 13-year-old boy with 3 weeks of fatigue, epistaxis, and acute loss of consciousness. He was found to have leukemia complicated by disseminated intravascular coagulation.](b7b5acbb-7b1a-4159-8883-940ef0177dc1) +*Axial T2* GRE MR shows multifocal hemorrhages within an ischemic infarct in a 13-year-old boy with 3 weeks of fatigue, epistaxis, and acute loss of consciousness. He was found to have leukemia complicated by disseminated intravascular coagulation.* + +![Axial NECT shows cerebellar infarcts in a 34-year-old woman with bilateral vertebral artery dissections. Note effacement of basal cisterns and temporal horn dilation indicating upward transtentorial herniation.](c449ce0d-6973-4cc5-ba76-0a95fe8c2a8b) +*Axial NECT shows cerebellar infarcts in a 34-year-old woman with bilateral vertebral artery dissections. Note effacement of basal cisterns and temporal horn dilation indicating upward transtentorial herniation.* + +![Axial T2WI MR shows bilateral wedge-shaped occipital areas of hyperintensity in a 77-year-old woman, which do not allow for a reliable distinction between chronic and acute ischemia.](601ac781-9129-48ee-97d2-e29d57ee20f4) +*Axial T2WI MR shows bilateral wedge-shaped occipital areas of hyperintensity in a 77-year-old woman, which do not allow for a reliable distinction between chronic and acute ischemia.* + +![Axial DWI MR in the same patient accurately reflects the acute area of left occipital ischemia , while encephalomalacia is apparent in the right occipital lobe .](f9ba77ec-9bc1-4758-a875-e9c6e0b65bf4) +*Axial DWI MR in the same patient accurately reflects the acute area of left occipital ischemia , while encephalomalacia is apparent in the right occipital lobe .* + +![Axial NECT shows multifocal hypodensities in the left cerebellum , consistent with embolic infarction within the left PICA distribution in this 40-year-old man with longstanding insulin-dependent diabetes and chronic renal failure. He presented with acute severe headache, nausea, and vomiting without localizing neurological finding.](edaacccd-09c7-4784-8614-54a1680ea399) +*Axial NECT shows multifocal hypodensities in the left cerebellum , consistent with embolic infarction within the left PICA distribution in this 40-year-old man with longstanding insulin-dependent diabetes and chronic renal failure. He presented with acute severe headache, nausea, and vomiting without localizing neurological finding.* + +![Axial CTA shows occlusion of the left vertebral artery . Compare with a normal dominant right vertebral artery .](90757d46-0ec3-42f8-9b54-e3f007b40b60) +*Axial CTA shows occlusion of the left vertebral artery . Compare with a normal dominant right vertebral artery .* + +![Axial CTA shows intimal flap in a 47-year-old woman with bilateral internal carotid artery dissections.](978258e7-b662-491d-863d-d2263ed87bcb) +*Axial CTA shows intimal flap in a 47-year-old woman with bilateral internal carotid artery dissections.* + +![Axial NECT shows hyperdense left deep nuclei in a patient post recent IV thrombolytic therapy followed by mechanical thrombectomy for left MCA occlusion. These may reflect contrast staining &/or hemorrhage. Contrast gradually fades over time and does not imply worse prognosis. Matching hypointensity on GRE suggests hemorrhage.](5caa5561-78f2-48e5-8430-56c1f5a21367) +*Axial NECT shows hyperdense left deep nuclei in a patient post recent IV thrombolytic therapy followed by mechanical thrombectomy for left MCA occlusion. These may reflect contrast staining &/or hemorrhage. Contrast gradually fades over time and does not imply worse prognosis. Matching hypointensity on GRE suggests hemorrhage.* + +![Axial T1 C+ MR shows heterogeneous gyriform enhancement in right MCA territory due to breakdown of BBB in subacute infarction. This appearance can mimic glioblastoma. Follow-up imaging may be important in patients without available imaging at the time of ictus to ensure appropriate evolution.](83fbdac8-5971-48dd-895a-ede27073f256) +*Axial T1 C+ MR shows heterogeneous gyriform enhancement in right MCA territory due to breakdown of BBB in subacute infarction. This appearance can mimic glioblastoma. Follow-up imaging may be important in patients without available imaging at the time of ictus to ensure appropriate evolution.* + +![Anteroposterior angiography shows left M1 occlusion and associated prominent lenticulostriate vessels .](65234520-550c-4ff3-8d2b-51022469fce6) +*Anteroposterior angiography shows left M1 occlusion and associated prominent lenticulostriate vessels .* + + +### Additional Images + +![Axial DWI MR in a patient 2 hours after stroke onset shows restricted diffusion. Correlative ADC hypointensity was also demonstrated within the same geographic area (not shown).](8faf1892-f90c-47fc-ad19-a3c6951115e1) +*Axial DWI MR in a patient 2 hours after stroke onset shows restricted diffusion. Correlative ADC hypointensity was also demonstrated within the same geographic area (not shown).* + +![Axial CECT shows abrupt right MCA cut-off in a patient with hyperacute stroke symptoms. (Courtesy J. Eastwood, MD.)](8e7a62ed-8fc4-44b2-9a96-c06e94ec61c6) +*Axial CECT shows abrupt right MCA cut-off in a patient with hyperacute stroke symptoms. (Courtesy J. Eastwood, MD.)* + +![Axial DWI MR shows small emboli infarcts in the left hemisphere.](770b1793-e8cd-4a1c-8b46-567bd2d0af23) +*Axial DWI MR shows small emboli infarcts in the left hemisphere.* + +![Axial CT perfusion map in the same patient reveals significantly prolonged mean transit time within the MCA distribution (red region).](47c3d108-a851-4f62-ad01-c248be33d58c) +*Axial CT perfusion map in the same patient reveals significantly prolonged mean transit time within the MCA distribution (red region).* + +![Axial DWI MR shows restricted diffusion within the right occipital lobe in a patient with sudden onset of visual symptoms.](5f51af3e-628f-4fc9-b784-a21e35867cfe) +*Axial DWI MR shows restricted diffusion within the right occipital lobe in a patient with sudden onset of visual symptoms.* + +![Axial FLAIR MR shows multiple foci of intraarterial high signal suggesting slow flow in this patient with left internal carotid artery dissection.](7fd9688b-3c8e-42fa-bee4-b5016535f2dc) +*Axial FLAIR MR shows multiple foci of intraarterial high signal suggesting slow flow in this patient with left internal carotid artery dissection.* + +![Coronal CTA shows slight irregularity within the reconstituted left vertebral artery segment , consistent with dissection in this symptomatic patient.](bd5f66dd-8018-4133-9ef8-0c482f93bc94) +*Coronal CTA shows slight irregularity within the reconstituted left vertebral artery segment , consistent with dissection in this symptomatic patient.* + +![Axial single-phase arterial spin-labeling (ASL) perfusion shows hemispheric asymmetry, decreased on the left , in a patient with acute left internal carotid artery dissection.](6804c6ab-e118-4325-8074-7a72b4130ee1) +*Axial single-phase arterial spin-labeling (ASL) perfusion shows hemispheric asymmetry, decreased on the left , in a patient with acute left internal carotid artery dissection.* + +![Axial FLAIR MR shows classic deep white matter watershed ischemic foci in a "string of pearls" appearance.](c6eae492-dc76-4e69-898d-b4a8ec31e02e) +*Axial FLAIR MR shows classic deep white matter watershed ischemic foci in a "string of pearls" appearance.* + +![Axial DWI MR in a 35-year-old woman post transsphenoidal surgery for Cushing disease shows multifocal infarctions as a complication of Enterobacter meningitis.](87fbd34b-6bf7-4f60-a79f-be23bacbadf4) +*Axial DWI MR in a 35-year-old woman post transsphenoidal surgery for Cushing disease shows multifocal infarctions as a complication of Enterobacter meningitis.* + diff --git a/docs_md/articles/carotid-stenosis-extracranial_1ebd8530-ebfc-4b36-9cd9-d9723c06f976.md b/docs_md/articles/carotid-stenosis-extracranial_1ebd8530-ebfc-4b36-9cd9-d9723c06f976.md new file mode 100644 index 0000000..c6d3598 --- /dev/null +++ b/docs_md/articles/carotid-stenosis-extracranial_1ebd8530-ebfc-4b36-9cd9-d9723c06f976.md @@ -0,0 +1,370 @@ +--- +title: "Carotid Stenosis, Extracranial" +docid: "1ebd8530-ebfc-4b36-9cd9-d9723c06f976" +authors: + - key: "07a2c087-6202-49e7-870b-7aa162d18f06" + value: "Bronwyn E. Hamilton, MD" +breadcrumbs: + - + name: "Vasculature" + slug: "vasculature" + treeNodeId: "9d3db335-364f-44ec-b2e2-30b03ce93228" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "4a210126-9f87-404e-b419-a73f44d0e94c" + - + name: "Extracranial Cerebral Arteries" + slug: "extracranial-cerebral-arteries" + treeNodeId: "593e17de-cd84-4587-8349-872ed33d28c4" + - + name: "Carotid Stenosis, Extracranial" + slug: "carotid-stenosis-extracranial" + treeNodeId: null +category: "Vasculature" +cmeTopicId: "a5bae72a-b8c6-4e22-bcbb-7ac49e488023" +documentVersionId: "e75ba73b-3257-4af2-b6dd-6aac9c6eae33" +imageCount: 18 +lastUpdated: "07/09/21" +pageDescription: "Carotid Stenosis, Extracranial" +pageKeywords: "Vasculature, Diagnosis, Extracranial Cerebral Arteries, Carotid Stenosis, Extracranial" +pageTitle: "Carotid Stenosis, Extracranial | STATdx" +enhancedTitle: "Carotid Stenosis, Extracranial" +type: "DX" +references: true +breadcrumbs: + - "Vasculature" + - "Diagnosis" + - "Extracranial Cerebral Arteries" + - "Carotid Stenosis, Extracranial" +--- +# KEY FACTS + +- ## Terminology + + + - Narrowing of cervical internal carotid artery or common carotid artery +- ## Imaging + + + - Extracranial carotid atherosclerotic vascular disease is most common at carotid bulb + - Carotid duplex US shows vessel narrowing with turbulent flow, increased peak systolic velocity, and spectral broadening + - CTA allows estimation of stenosis severity + - MRA flow gap can occur in stenoses > 95%, causing misdiagnosis of occlusion + - DSA is gold standard for evaluating severity of stenosis + - "String" sign = very high grade stenosis + - Slow antegrade "trickle" blood flow +- ## Top Differential Diagnoses + + + - Dissection + - Fibromuscular dysplasia + - Extrinsic compressive lesion (rare) +- ## Pathology + + + - Risk of stroke increases with stenosis severity, an indirect measure of plaque volume and potential for complicated plaque or embolization +- ## Clinical Issues + + + - NASCET showed that symptomatic patients with stenosis ≥ 70% (associated with stroke risk) benefit from carotid endarterectomy (CEA) + - ACAS showed that asymptomatic patients with 60% stenosis benefit from CEA + - SAPPHIRE compared CEA to carotid artery stenting (CAS) in high-risk patients with carotid stenosis + - Lower complication rate with CAS + - No difference in stroke after 3 years + +# TERMINOLOGY + +- ## Synonyms + + + - Carotid atherosclerotic vascular disease (ASVD) +- ## Definitions + + + - Narrowing of cervical segment of internal carotid artery (ICA) or common carotid artery (CCA) + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Carotid duplex US shows vessel narrowing with turbulent flow, increased peak systolic velocity, and spectral broadening + - ### Location + + + - Extracranial carotid ASVD is most common at carotid bulb + - ### Size + + + - Variable severity and length of stenosis; usually < 3 cm + - Smooth or irregular narrowing ± ulceration ± intraluminal thrombus +- ## CT Findings + + + - ### NECT + + + - Calcified ASVD plaque at CCA bifurcation ± ICA + - May show thromboembolic or hemodynamic cerebral infarction + - Typically ipsilateral anterior circulation + - Posterior cerebral artery (PCA) stroke possible via posterior communicating artery or fetal PCA + - ### CTA + + + - Useful as screening tool + - CTA allows estimation of stenosis severity + - Multiplanar reformatted images in sagittal and coronal planes are helpful + - Accuracy is reduced if extensive lesional calcification is present + - Maximal carotid wall thickness ≥ 4 mm is predictive of future carotid ischemic stroke + - Dental amalgam artifacts may hinder visualization + - May show intraluminal thrombus as filling defect within enhanced vessel + - Unreliable visualization of plaque ulceration + - Patchy/homogeneous low density in wall may be seen with large necrotic/lipid plaque +- ## MR Findings + + + - ### T1WI + + + - Reduced caliber of ICA flow void ± intraluminal signal due to thrombus or slow flow + - Fat-saturated sequence if dissection is suspected as alternate etiology + - Intramural crescentic high signal represents methemoglobin in vessel wall (dissection) + - ### DWI + + + - Most sensitive and specific for acute/subacute ischemia or infarction + - ### MRA + + + - Provides multidirectional imaging (vs. conventional DSA) + - Time-of-flight (TOF) MRA: Intravoxel dephasing causes signal loss with flow turbulence due to stenosis + - Affects 2D > 3D TOF images + - Accentuates severity of stenosis + - Gadolinium-enhanced MRA is superior to TOF sequences + - Flow gap can occur in stenoses > 95%, causing misdiagnosis of occlusion + - Brain T2WI, FLAIR, and DWI may show rosary-like lesions in centrum semiovale ipsilateral to stenosis, indicative of watershed ischemia or infarction +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Calcified plaque causes acoustic shadowing and may limit assessment of vessel lumen + - ### Pulsed Doppler + + + - Duplex US: Flow velocity within stenosis is proportional to severity of stenosis + - Flow turbulence within and beyond stenosis + - Spectral broadening: Increased range of velocities is seen in moderate to severe stenoses +- ## Angiographic Findings + + + - Conventional + - DSA is gold standard for evaluation of carotid stenosis severity + - Use of reverse-curve catheters (e.g., Simmons) can avoid inadvertent crossing of carotid bifurcation stenosis with guidewire and dislodgement of plaque + - Intraluminal thrombus is seen as filling defect in contrast column + - Can evaluate collateral flow to ischemic hemisphere from communicating arteries and leptomeningeal collaterals by studying contralateral ICA and dominant vertebral artery + - "String" sign = very high grade stenosis, slow antegrade "trickle" blood flow + - Typically seen during late phase of angiogram + - May require prolonged DSA acquisitions for visualization + - Preocclusive state with high risk of stroke + - Important as carotid endarterectomy (CEA) or carotid artery stenting (CAS) may be an option if ICA is still patent + - More sensitive and specific than CTA and MRA for subtotal occlusion with string sign +- ## Other Modality Findings + + + - CT/MR perfusion + - Can provide assessment of collateral flow to territory normally perfused by stenotic carotid artery + - Collateral circulation correlates with risk of hemodynamic ischemia or infarction + - Measurement of carotid stenosis severity + - North American Symptomatic Carotid Endarterectomy Trial (NASCET) method is most widely accepted + - NASCET: Denominator is normal poststenotic ICA diameter + - European Carotid Surgery Trial (ECST): Denominator is estimated normal diameter of carotid bulb +- ## Imaging Recommendations + + + - Ultrasound or CTA as screening tool + - CTA/MRA for comprehensive cerebrovascular evaluation + - DSA if US/CTA/MRA is equivocal or shows "occlusion" + +# DIFFERENTIAL DIAGNOSIS + +- ## Dissection + + + - Typically spares carotid bulb and ICA origin + - Usually no calcification (dystrophic Ca++ is rare) + - Intimal flap with differential filling of true and false lumens on DSA + - Crescentic intramural high signal (methemoglobin) on T1WI MR +- ## Fibromuscular Dysplasia + + + - Affects medium to large arteries + - M:F = 1:3 + - Age peak: 25-50 years + - Classically shows alternating segments of beading and stenoses involving extracranial ICA and external carotid, vertebral, and renal arteries +- ## Extrinsic Compressive Lesion (Rare) + + + - Carotid space neoplasm (e.g., carotid body paraganglioma, glomus jugulare tumor) + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Risk of stroke increases with stenosis severity, an indirect measure of plaque volume and potential for complicated plaque/embolization + - Larger plaques are complicated by hemorrhage, necrosis, and disruption of fibrous cap and intima, causing embolization + - Plaque composition and surface morphology are also stroke risk factors + - Irregular plaque surface: ↑ stroke risk on medical treatment for all degrees of stenosis + - Hypoperfusion may cause watershed infarcts &/or centrum semiovale lesions + - Significant ICA narrowing is identified in 20-30% of carotid territory stroke patients (vs. 5-10% of general population) +- ## Gross Pathologic & Surgical Features + + + - Fatty streak: Raised lesion due to fatty deposit in intima + - Fibrous (fibrolipid) plaque: Cholesterol + fibrous tissue with collagen cap + - Complicated plaque: Unstable; may rupture, thrombose, calcify, or hemorrhage +- ## Microscopic Features + + + - ASVD: Fatty streaks, lipid-laden macrophages and smooth muscle cells, fibrous cap, cholesterol deposits, foam cells, plaque rupture ± thrombus + +# CLINICAL ISSUES + +- ## Presentation + + + - Stroke is 3rd most common cause of death in Western countries + - Transient ischemic attack (TIA): Neurological deficit that spontaneously resolves in < 24 hours + - 80% resolve in < 1 hour + - Precedes 30% of strokes + - 50% of subsequent strokes occur < 1 year from TIA + - Reversible ischemic neurological deficit: Neurological deficit > 24 hours but < 3 weeks + - Amaurosis fugax (transient, monocular embolic blindness) + - Asymptomatic carotid bruit: 20% have > 60% ICA stenosis (3x normal population) +- ## Natural History & Prognosis + + + - Progressive +- ## Treatment + + + - Reduction of risk factors, which include hypertension, smoking, diabetic control, and hypercholesterolemia + - Medical: Aspirin, statins + - NASCET (1991) + - Symptomatic stenosis ≥ 70% (associated with significant stroke risk) benefits from CEA + - Symptomatic moderate stenosis (50-69%) also benefits from endarterectomy in selected cases + - Asymptomatic Carotid Atherosclerosis Study (ACAS, 1995) + - Asymptomatic patients with 60% stenosis benefit from CEA + - CAS is becoming increasingly utilized and substantiated as viable alternative to CEA + - CAS with distal protection device is associated with risk of periprocedural stroke ≤ CEA + - Stenting and Angioplasty With Protection in Patients at High Risk for Endarterectomy (SAPPHIRE) 2004 study + - Compared CEA with CAS in high-risk patients (comorbidities, age > 80 years, recent surgery, etc.) with symptomatic and asymptomatic carotid stenoses + - Lower complication rate with CAS; no difference in stroke incidence after 3 years (7.1% CAS vs. 6.7% CEA) + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Use of reverse-curve catheters for catheterization of CCA when carotid stenosis is suspected +- ## Image Interpretation Pearls + + + - MRA often exaggerates degree of stenosis + - Look for intraluminal filling defect (CAS is contraindicated if intraluminal thrombus is present) +- ## MIPS Considerations + + + - MIPS Measure 195: Radiology: Stenosis Measurement in Carotid Imaging Reports + - Last updated 2021 + - Percentage of final reports for carotid imaging studies (neck MRA, neck CTA, neck duplex ultrasound, carotid angiogram) performed that include **direct or indirect reference to** **measurements of distal internal carotid diameter as denominator for stenosis measurement** + + 60075056-b951-47fe-b646-4817b546b29d + +## References + +# Selected References + +1. [MIPS Measure 195: Radiology: Stenosis Measurement in Carotid Imaging Reports (to reference if using CQMS). Centers for Medicare and Medicaid Services (CMS). 2021.](https://qpp.cms.gov/docs/QPP_quality_measure_specifications/CQM-Measures/2021_measure_195_MIPSCQM.pdf) +1. [MIPS Measure 195: Radiology: Stenosis Measurement in Carotid Imaging Reports (to reference if using Medicare Part B claims). Centers for Medicare and Medicaid Services (CMS). 2021.](https://qpp.cms.gov/docs/QPP_quality_measure_specifications/Claims-Registry-Measures/2021_measure_195_MedicarePartBClaims.pdf) +1. [Magge R et al: Clinical risk factors and CT imaging features of carotid atherosclerotic plaques as predictors of new incident carotid ischemic stroke: a retrospective cohort study. AJNR Am J Neuroradiol. 34(2):402-9, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=22859283%5Bpmid%5D) +1. [Brott TG et al: 2011 ASA/ACCF/AHA/AANN/AANS/ACR/ASNR/CNS/SAIP/SCAI/SIR/SNIS/SVM/SVS guideline on the management of patients with extracranial carotid and vertebral artery disease: executive summary: Stroke. 42(8):e420-63, 2011. Erratum in: Stroke. 42(8):e541, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=-1%5Bpmid%5D) +1. [Halliday A et al: Prevention of disabling and fatal strokes by successful carotid endarterectomy in patients without recent neurological symptoms: randomised controlled trial. Lancet. 363(9420):1491-502, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15135594%5Bpmid%5D) +1. [Yadav JS: Carotid stenting in high-risk patients: design and rationale of the SAPPHIRE trial. Cleve Clin J Med. 71 Suppl 1:S45-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14964484%5Bpmid%5D) +1. [No authors listed: Carotid endarterectomy for patients with asymptomatic internal carotid artery stenosis. National Institute of Neurological Disorders and Stroke. J Neurol Sci. 129(1):76-7, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7751850%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal reformat CTA shows irregularity and focal high-grade stenosis of the proximal internal carotid artery (ICA) , typical of atherosclerotic disease. Note areas of calcified plaque , which indicate an atherosclerotic etiology.](images/app.statdx.com_image_thumbnail_a7c9d3ad-79c4-4398-83ea-e2a42b608fc1_annotated_true_size_900_quality_90_decfca96ff71555d2478b4e3e802d1412cbef678.jpg) +*Sagittal reformat CTA shows irregularity and focal high-grade stenosis of the proximal internal carotid artery (ICA) , typical of atherosclerotic disease. Note areas of calcified plaque , which indicate an atherosclerotic etiology.* + +![Sagittal reformat CTA shows irregularity and focal high-grade stenosis of the proximal internal carotid artery (ICA) , typical of atherosclerotic disease. Note areas of calcified plaque , which indicate an atherosclerotic etiology.](images/app.statdx.com_image_thumbnail_a7c9d3ad-79c4-4398-83ea-e2a42b608fc1_size_174_quality_85_e6365393d30085665766faa1924bb69c9e5dc521.jpg) +*Sagittal reformat CTA shows irregularity and focal high-grade stenosis of the proximal internal carotid artery (ICA) , typical of atherosclerotic disease. Note areas of calcified plaque , which indicate an atherosclerotic etiology.* + +![Lateral DSA confirms similar findings to the CTA (same patient) typical of atherosclerotic high-grade carotid stenosis: Irregular short-segment narrowing with more proximal ulceration .](images/app.statdx.com_image_thumbnail_554c56d7-afca-483e-85f4-ec0e33fe3fa1_annotated_true_size_900_quality_90_ed73622a46c53ad2f0fa361a4e3eeade9d07c924.jpg) +*Lateral DSA confirms similar findings to the CTA (same patient) typical of atherosclerotic high-grade carotid stenosis: Irregular short-segment narrowing with more proximal ulceration .* + +![Sagittal MRA shows a flow gap in the ICA . MRA overestimates stenosis and occlusions; therefore, this must be confirmed with another vascular imaging modality to avoid misinterpretation.](images/app.statdx.com_image_thumbnail_3de530da-0db2-455e-a05f-24e81812f6e3_annotated_true_size_900_quality_90_77166eded4ac432f2c3fe5bf4d85322bbedd162a.jpg) +*Sagittal MRA shows a flow gap in the ICA . MRA overestimates stenosis and occlusions; therefore, this must be confirmed with another vascular imaging modality to avoid misinterpretation.* + +![Color Doppler ultrasound (same patient) shows high flow velocities, anatomical narrowing, and spectral broadening, confirming that not an occlusion but a high-grade and hemodynamically significant stenosis (~ 80-99%) of the ICA bifurcation is present.](images/app.statdx.com_image_thumbnail_24ff922e-400a-44b8-ae57-7c2ba29dc35b_annotated_true_size_900_quality_90_7674b20b758d379f0c3f59554de057ba99bd42ac.jpg) +*Color Doppler ultrasound (same patient) shows high flow velocities, anatomical narrowing, and spectral broadening, confirming that not an occlusion but a high-grade and hemodynamically significant stenosis (~ 80-99%) of the ICA bifurcation is present.* + +![Sagittal reformat CTA demonstrates a high-grade stenosis of the internal carotid artery distal to its origin and irregular narrowing and ulceration more proximally at the carotid bifurcation , findings typical for atherosclerotic narrowing.](images/app.statdx.com_image_thumbnail_6c59948f-f41e-41bd-968c-b8ad23d9d2f0_annotated_true_size_900_quality_90_818734c0ad7a3ef81ace1df943a0eeaedd5ea98f.jpg) +*Sagittal reformat CTA demonstrates a high-grade stenosis of the internal carotid artery distal to its origin and irregular narrowing and ulceration more proximally at the carotid bifurcation , findings typical for atherosclerotic narrowing.* + +![Lateral DSA (same patient) demonstrates similar findings compared with CTA: Ulceration and narrowing at the internal carotid artery origin and more distal high-grade stenosis .](images/app.statdx.com_image_thumbnail_bda36bef-5460-4538-8b10-fc0ebbba1af6_annotated_true_size_900_quality_90_01a382b7cef39aa83940d3b82917ffdb40079c40.jpg) +*Lateral DSA (same patient) demonstrates similar findings compared with CTA: Ulceration and narrowing at the internal carotid artery origin and more distal high-grade stenosis .* + +![Sagittal CTA shows irregular ulcerated plaque at the internal carotid artery origin, typical of atherosclerotic disease. Although a hemodynamically significant stenosis may not be present, this plaque is morphology prone to embolic complications.](images/app.statdx.com_image_thumbnail_83af6955-319e-4ebe-857b-d4ae691959c1_annotated_true_size_900_quality_90_0e2b4954d4b441afd8ea3030fefaf82a3e01502f.jpg) +*Sagittal CTA shows irregular ulcerated plaque at the internal carotid artery origin, typical of atherosclerotic disease. Although a hemodynamically significant stenosis may not be present, this plaque is morphology prone to embolic complications.* + +![Oblique 3D reformation of a CTA shows diffuse beading of the distal cervical internal carotid artery , typical in appearance for fibromuscular dysplasia. Both internal carotid and renal arteries (not shown) were similarly affected.](images/app.statdx.com_image_thumbnail_28eb6460-dcf6-4cf5-b8b7-22dfacbee75d_annotated_true_size_900_quality_90_b2b53264088a8e3cece348341ed7fb05e5f738d0.jpg) +*Oblique 3D reformation of a CTA shows diffuse beading of the distal cervical internal carotid artery , typical in appearance for fibromuscular dysplasia. Both internal carotid and renal arteries (not shown) were similarly affected.* + +![Coronal MRA appears nearly normal in this patient with distal cervical left ICA dissection. Note the mild smoothly marginated caliber change that is easily missed until compared with the contralateral side. The ICAs, unlike the vertebral arteries, normally demonstrate a symmetric size in the neck.](images/app.statdx.com_image_thumbnail_9a440bd0-35f3-4865-8d96-f7a82ca04238_annotated_true_size_900_quality_90_e21b9ff01b0e73254a5c72d939dde43544871636.jpg) +*Coronal MRA appears nearly normal in this patient with distal cervical left ICA dissection. Note the mild smoothly marginated caliber change that is easily missed until compared with the contralateral side. The ICAs, unlike the vertebral arteries, normally demonstrate a symmetric size in the neck.* + +![Axial T1WI FS MR can be useful to confirm suspected dissection, as in this case (same patient) where crescentic mural hematoma is visible around the luminal flow void.](2a971700-4658-4aea-86ef-fcda0d233f3e) +*Axial T1WI FS MR can be useful to confirm suspected dissection, as in this case (same patient) where crescentic mural hematoma is visible around the luminal flow void.* + + +### Additional Images + +![Oblique CCA DSA shows a calcified plaque at the carotid bifurcation extending into the ICA with associated stenosis . An intraluminal filling defect is seen. It represented a thrombus for which the patient was anticoagulated. DSA 5 days later revealed resolution of the thrombus, and carotid artery stenting was undertaken at that time.](ad10e78e-f5fa-4aad-92ba-c167709b2a31) +*Oblique CCA DSA shows a calcified plaque at the carotid bifurcation extending into the ICA with associated stenosis . An intraluminal filling defect is seen. It represented a thrombus for which the patient was anticoagulated. DSA 5 days later revealed resolution of the thrombus, and carotid artery stenting was undertaken at that time.* + +![Carotid duplex spectral waveform in the same patient shows spectral broadening and a peak systolic velocity of 598 cm/s in keeping with a 70-99% stenosis.](661a24d3-c3bc-4e6f-8fdd-71b88b8367f0) +*Carotid duplex spectral waveform in the same patient shows spectral broadening and a peak systolic velocity of 598 cm/s in keeping with a 70-99% stenosis.* + +![Oblique CCA DSA shows an ulcerated ASVD plaque at the carotid bifurcation . There is an additional plaque distally but no significant carotid stenosis.](f28beccc-3ece-425a-ad6c-ad03d9542bb3) +*Oblique CCA DSA shows an ulcerated ASVD plaque at the carotid bifurcation . There is an additional plaque distally but no significant carotid stenosis.* + +![Carotid duplex ultrasound of the proximal ICA shows a moderate stenosis due to ASVD . Within the stenotic segment there is flow turbulence as depicted by variations in color and intensity .](d47bc88d-e488-4823-9a33-83084247a192) +*Carotid duplex ultrasound of the proximal ICA shows a moderate stenosis due to ASVD . Within the stenotic segment there is flow turbulence as depicted by variations in color and intensity .* + +![Sagittal gadolinium-enhanced MRA of the carotid bifurcation shows a flow gap at the ICA origin . MRA typically overestimates the degree of stenosis.](bc0f17a9-c169-481e-9303-190e4d278998) +*Sagittal gadolinium-enhanced MRA of the carotid bifurcation shows a flow gap at the ICA origin . MRA typically overestimates the degree of stenosis.* + +![Sagittal CTA in a different patient shows a pinhole stenosis at the ICA origin . Note adjacent calcifications within the ASVD plaque and artifact from dental amalgam .](bc7f4455-caf2-4e15-a1aa-9d09679f91db) +*Sagittal CTA in a different patient shows a pinhole stenosis at the ICA origin . Note adjacent calcifications within the ASVD plaque and artifact from dental amalgam .* + +![Lateral CCA DSA shows a high-grade stenosis of the ICA and indentation of the vessel lumen by plaque . Note gracile cervical ICA due to proximal flow restriction.](d5491583-f67c-487d-94bf-f35e786d2cc5) +*Lateral CCA DSA shows a high-grade stenosis of the ICA and indentation of the vessel lumen by plaque . Note gracile cervical ICA due to proximal flow restriction.* + +![Oblique CCA DSA shows a high-grade ASVD stenosis at the carotid bulb with associated calcifications .](6ccbe61c-042b-4e0d-991c-0b177924c13e) +*Oblique CCA DSA shows a high-grade ASVD stenosis at the carotid bulb with associated calcifications .* + diff --git a/docs_md/articles/cerebral-hyperperfusion-syndrome_e66febb9-d79e-4f04-88b1-205ba8a0822f.md b/docs_md/articles/cerebral-hyperperfusion-syndrome_e66febb9-d79e-4f04-88b1-205ba8a0822f.md new file mode 100644 index 0000000..cdafbc3 --- /dev/null +++ b/docs_md/articles/cerebral-hyperperfusion-syndrome_e66febb9-d79e-4f04-88b1-205ba8a0822f.md @@ -0,0 +1,439 @@ +--- +title: "Cerebral Hyperperfusion Syndrome" +docid: "e66febb9-d79e-4f04-88b1-205ba8a0822f" +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: "Stroke" + slug: "stroke" + treeNodeId: "12307683-f1ff-4823-a7d3-b10b40f9fd82" + - + name: "Cerebral Ischemia and Infarction" + slug: "cerebral-ischemia-and-infarction" + treeNodeId: "51051846-a223-42f7-b626-2a5a26cf6c44" + - + name: "Cerebral Hyperperfusion Syndrome" + slug: "cerebral-hyperperfusion-syndrome" + treeNodeId: null +category: "Brain" +cmeTopicId: "32753552-21d2-4b3a-bb83-721ad78e8c95" +documentVersionId: "26eadfe0-1ff5-4d74-8dfb-927c31c6e893" +imageCount: 15 +lastUpdated: "08/05/20" +pageDescription: "Cerebral Hyperperfusion Syndrome" +pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Cerebral Ischemia and Infarction, Cerebral Hyperperfusion Syndrome" +pageTitle: "Cerebral Hyperperfusion Syndrome | STATdx" +enhancedTitle: "Cerebral Hyperperfusion Syndrome" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Pathology-Based Diagnoses" + - "Stroke" + - "Cerebral Ischemia and Infarction" + - "Cerebral Hyperperfusion Syndrome" +--- +# KEY FACTS + +- ## Terminology + + + - Rare disorder most commonly occurring as complication of cerebral revascularization + - Other etiologies less common + - Status epilepticus + - MELAS + - Major increase in ipsilateral cerebral blood flow (CBF) well above normal metabolic demands +- ## Imaging + + + - Ipsilateral gyral swelling, sulcal effacement in post carotid endarterectomy (CEA) patient + - ↑ CBF, cerebral blood volume (CBV) on perfusion MR (pMR), perfusion CT (pCT) + - Early draining vein, capillary blush on DSA after revascularization +- ## Top Differential Diagnoses + + + - Acute cerebral ischemia-infarction + - Status epilepticus + - MELAS + - Acute hypertensive encephalopathy, PRES + - Hypercapnia +- ## Pathology + + + - Cerebral hyperperfusion syndrome (CHS) probably caused by maladaptive autoregulatory mechanisms, altered cerebral hemodynamics + - "Normal perfusion pressure breakthrough" + - Rapid restoration of normal perfusion following revascularization → hyperperfusion in previously underperfused brain +- ## Clinical Issues + + + - ~ 3% of post-CEA patients develop CHS + - Triad of unilateral headache, neurologic deficit, seizures + - Variable cognitive impairment + - Ipsilateral face, eye pain +- ## Diagnostic Checklist + + + - Need to distinguish stroke/TIA from CHS + +# TERMINOLOGY + +- ## Abbreviations + + + - Cerebral hyperperfusion syndrome (CHS) +- ## Synonyms + + + - Post-CEA hyperperfusion + - Luxury perfusion +- ## Definitions + + + - Rare (3.5%) disorder most commonly occurring as complication of cerebral revascularization + - Mildly ↑ cerebral blood flow (CBF) common after carotid endarterectomy (CEA), typically asymptomatic + - CHS defined as ≥ 100% increase in rCBF compared to preoperative values + - Major increase in ipsilateral CBF well above normal metabolic demands + - Usually following carotid revascularization procedure + - Carotid endarterectomy + - Angioplasty with stenting + - Thrombolysis + - May occur in other settings [e.g., status epilepticus, mitochondrial encephalopathy lactic acidosis and stroke-like episodes (MELAS)] + - After drainage of chronic subdural hematomas + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Ipsilateral gyral swelling, sulcal effacement in post-CEA patient + - ↑ CBF, cerebral blood volume (CBV) on perfusion MR (pMR), perfusion CT (pCT) + - ### Size + + + - Variable + - ### Morphology + + + - Follows vascular distribution +- ## Angiographic Findings + + + - Sentinel signs suggestive of maximal arteriolar dilation, disrupted cerebral autoregulation + - Early draining vein in treated ischemic territory + - Early contrast filling vein(s) in late arterial or capillary phase + - Prominent capillary blush (luxury perfusion) denser than rest of arterial territory + - Persists late into venous phase +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR with DWI, PWI + - SPECT + - ### Protocol advice + + + - Add T2* (GRE or SWI) to look for hemorrhage +- ## CT Findings + + + - ### NECT + + + - Gyral swelling + - Cortical effacement + - Patchy or diffuse white matter (WM) edema + - Posterior parietooccipital lobe most common + - ± hypodensity (may occur without attenuation alterations) + - Frank hemorrhage in < 1% + - ### CECT + + + - Prominent vessels with ↑ intravascular enhancement + - May demonstrate contrast extravasation in severe cases (rare) + - CT perfusion + - Elevated CBF, ↓ TTP +- ## MR Findings + + + - ### T1WI + + + - Cortical swelling + - ± mild hypointensity + - Sulci effaced + - ### T2WI + + + - Gyral swelling, hyperintensity + - ### FLAIR + + + - Hyperintense cortex + - Hyperintensity in subarachnoid spaces on postcontrast FLAIR reported 2° to blood-brain barrier (BBB) disruption + - ### T2* GRE + + + - Frank hemorrhage in < 1% + - Blooming on GRE or SWI + - ### DWI + + + - Usually normal, as edema is vasogenic, not cytotoxic + - ~ 25% show small foci of restricted diffusion compared to preoperative DWI + - ### PWI + + + - Elevated CBV, CBF + - Prolonged MTT + - Side-to-side difference of 3 seconds predictive of CHS + - ### T1WI C+ + + + - May be normal + - May show slightly increased prominence of cerebral vessels + - Parenchymal enhancement in severe cases + - ### MRA + + + - Preoperative ↓ signal intensity in middle cerebral artery (MCA) may identify patients at risk for CHS +- ## Other Modality Findings + + + - SPECT + - N-isopropyl-p-I-123-iodoamphetamine or I-123-iomazenil SPECT + - Shows hyperperfusion in ipsilateral cerebral hemisphere after surgery + - CBF ≥ 100% in revascularized territory from baseline + - Can be detected even in asymptomatic patients + - May be correlated with long-term neuronal damage that CT, MR do not detect + - May be associated with crossed cerebellar diaschisis +- ## Ultrasonographic Findings + + + - Transcranial color duplex (TCD) + - 1.5-2x increase in MCA flow velocity + +# DIFFERENTIAL DIAGNOSIS + +- [Acute Cerebral Ischemia-Infarction](/document/acute-cerebral-ischemiainfarction/a405285f-aaea-43ca-8dc4-6f8120eaabc1) + - TTP/MTT prolonged (not decreased) + - Typically shows restriction on DWI (CHS often negative) +- [Status Epilepticus](/document/status-epilepticus/a058b733-4b80-46a1-8097-d68685ecf921) + - Metabolic hyperperfusion in affected brain + - History of seizure helpful but may not be available +- [Acute Hypertensive Encephalopathy, PRES](/document/acute-hypertensive-encephalopathy--/890c1bd4-c108-49a1-8557-c8c701a7f278) + - Failed autoregulation → hyperperfusion → endothelial injury/vasogenic edema + - Predilection for posterior circulation + - Markedly elevated blood pressure (many etiologies) + - Eclampsia, preeclampsia + - Chemotherapy + - Renal failure + - Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura + - Drug abuse (especially cocaine) +- ## MELAS + + + - Acute oxidative phosphorylation defect + - Stroke-like episodes related to vasogenic edema, hyperperfusion, neuronal damage + - Cortical hyperintensity, enhancement + - Perform MRS in unaffected region, look for lactate +- ## Hypercapnia + + + - Carbon dioxide is potent stimulator of CBF + - Vasodilatory effect on cerebral vasculature + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Theories + - Impaired cerebral autoregulation + - Damage from free radicals + - Baroreceptor reflex breakdown + - Trigeminovascular reflex (vasoactive neuropeptide release) + - CHS probably caused by maladaptive autoregulatory mechanisms, altered cerebral hemodynamics + - "Normal perfusion pressure breakthrough" + - Chronic ischemia → impaired autoregulation + - Loss of normal vasoconstriction + - "Resistance" vessels become chronically dilated + - Rapid restoration of normal perfusion following revascularization → hyperperfusion in previously underperfused brain + - Cognitive impairment after CEA or angioplasty/stenting may result from + - Cerebral embolization during dissection, stenting + - Global cerebral hypoperfusion during carotid cross-clamping + - Cerebral hyperperfusion syndrome + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Symptoms range from mild to severe/life-threatening + - Ipsilateral headache, neurologic deficit and seizures + - Other signs/symptoms + - Variable cognitive impairment + - Face, eye pain + - Timing + - Peaks at 12 h after carotid angioplasty/stenting (CAS) + - 6 days after CEA + - Can be delayed by up to 1 month +- ## Demographics + + + - ### Age + + + - For postendarterectomy CHS, generally older patients + - For other etiologies (e.g., seizure, MELAS), any age + - ### Epidemiology + + + - ~ 3-4% of post-CEA patients develop mild CHS + - Highest risk = impaired cerebrovascular reserve, asymptomatic stenosis + - > 100% increase in CBF after treatment + - Contralateral stenosis, chronic hypertension do not influence risk of CHS after CEA + - Covariate clinical risk factors + - Age + - Hypertension (especially postoperative) + - Diabetes + - Bilateral lesions + - Extent of ICA stenosis + - High grade > low grade + - Presence of contralateral carotid occlusion or high-grade stenosis + - Duration of cross-clamping + - Diminished carotid reserve + - Poor collateral blood flow + - Decreased cerebrovascular reactivity to acetazolamide challenge +- ## Natural History & Prognosis + + + - Neurologic emergency + - If not treated promptly/adequately, can cause death or severe disability + - If no intracranial hemorrhage + - Usually reversible + - No major tissue destruction + - May result in persistent mild cognitive impairment + - 1% of CHS with intracranial hemorrhage + - Poor prognosis +- ## Treatment + + + - Prevention + - Minimize intraoperative cerebral ischemia + - Consider continuing postoperative anesthesia/continuous sedation + - Strict postoperative blood pressure control + - Staged angioplasty in at-risk patients can ↓ + - Efficacious for patients with severe impairment of hemodynamic reserve in I-123 IMP SPECT + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Post-CEA/carotid artery stenting patient with neurologic deficit + - Need to distinguish stroke/transient ischemic attack from CHS + + 8b8b5253-2a1e-4d24-9d1b-dc85e0dde35e + +## References + +# Selected References + +1. [Lin YH et al: Update on cerebral hyperperfusion syndrome. J Neurointerv Surg. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32414892%5Bpmid%5D) +1. [Murai S et al: Safety and efficacy of staged angioplasty for patients at risk of hyperperfusion syndrome: a single-center retrospective study. Neuroradiology. 62(4):503-10, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31915841%5Bpmid%5D) +1. [Pavlov O: Rapid evacuation of chronic subdural hematoma - A possible traumatic brain injury (TBI). Med Hypotheses. 137:109539, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31952019%5Bpmid%5D) +1. [Sakata H et al: Symptomatic cerebral hyperperfusion after cerebral vasospasm associated with aneurysmal subarachnoid hemorrhage. World Neurosurg. 137:379-83, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32105869%5Bpmid%5D) +1. [Fassaert LMM et al: Transcranial Doppler 24 hours after carotid endarterectomy accurately identifies patients not at risk of cerebral hyperperfusion syndrome. Eur J Vasc Endovasc Surg. 58(3):320-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31350134%5Bpmid%5D) +1. [Ghuman M et al: Sentinel angiographic signs of cerebral hyperperfusion after angioplasty and stenting of intracranial atherosclerotic stenosis: A technical note. AJNR Am J Neuroradiol. 40(9):1523-5, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31345945%5Bpmid%5D) +1. [Lin T et al: ASL perfusion features and type of circle of Willis as imaging markers for cerebral hyperperfusion after carotid revascularization: a preliminary study. Eur Radiol. 29(5):2651-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30443757%5Bpmid%5D) +1. [Omura T et al: Cerebral hyperperfusion syndrome after a burr hole drainage surgery for chronic subdural hematoma. World Neurosurg. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30610989%5Bpmid%5D) +1. [Sharma P et al: Cerebral hyperperfusion syndrome after chronic subdural hematoma drainage. World Neurosurg. 126:694, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31546329%5Bpmid%5D) +1. [Huibers AE et al: Editor's choice - Cerebral hyperperfusion syndrome after carotid artery stenting: A systematic review and meta-analysis. Eur J Vasc Endovasc Surg. 56(3):322-33, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30196814%5Bpmid%5D) +1. [Kirchoff-Torres KF et al: Cerebral hyperperfusion syndrome after carotid revascularization and acute ischemic stroke. Curr Pain Headache Rep. 22(4):24, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29556806%5Bpmid%5D) +1. [Galyfos G et al: Cerebral hyperperfusion syndrome and intracranial hemorrhage after carotid endarterectomy or carotid stenting: A meta-analysis. J Neurol Sci. 381:74-82, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28991720%5Bpmid%5D) +1. [Cano EJ et al: Asymmetric brain edema after cardiac transplantation: cerebroautoregulatory failure and relative hyperperfusion. Transplant Proc. 47(1):194-7, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25645802%5Bpmid%5D) +1. [Horie N et al: De novo ivy sign indicates postoperative hyperperfusion in moyamoya disease. Stroke. 45(5):1488-91, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24713526%5Bpmid%5D) + + +## Images + + +### Selected Images + +![A 56-year-old man with > 70% stenosis of his proximal left cervical internal carotid artery (ICA) underwent carotid endarterectomy. A few hours after surgery, he became acutely confused and developed right-sided weakness. Perfusion source image shows markedly increased vasculature in the left hemisphere .](images/app.statdx.com_image_thumbnail_ef11e61a-6a71-484c-bf37-ab7becb42d11_annotated_true_size_900_quality_90_2224a462838d90e085b09b31ce6fc9a5005f0762.jpg) +*A 56-year-old man with > 70% stenosis of his proximal left cervical internal carotid artery (ICA) underwent carotid endarterectomy. A few hours after surgery, he became acutely confused and developed right-sided weakness. Perfusion source image shows markedly increased vasculature in the left hemisphere .* + +![A 56-year-old man with > 70% stenosis of his proximal left cervical internal carotid artery (ICA) underwent carotid endarterectomy. A few hours after surgery, he became acutely confused and developed right-sided weakness. Perfusion source image shows markedly increased vasculature in the left hemisphere .](images/app.statdx.com_image_thumbnail_ef11e61a-6a71-484c-bf37-ab7becb42d11_size_174_quality_85_abe8205402311f16f095c349f5547aa65e6c028f.jpg) +*A 56-year-old man with > 70% stenosis of his proximal left cervical internal carotid artery (ICA) underwent carotid endarterectomy. A few hours after surgery, he became acutely confused and developed right-sided weakness. Perfusion source image shows markedly increased vasculature in the left hemisphere .* + +![CT perfusion obtained in the same patient appears relatively normal, but cerebral blood flow (CBF) on the left (2a, 2b ROIs) is increased compared to the right side.](images/app.statdx.com_image_thumbnail_730787c1-5f79-482b-b252-bf72c2f07e00_annotated_true_size_900_quality_90_5211e7a477dde3c61875d1f546e4e7d8d0de20c0.jpg) +*CT perfusion obtained in the same patient appears relatively normal, but cerebral blood flow (CBF) on the left (2a, 2b ROIs) is increased compared to the right side.* + +![TTP in the same patient is even more striking. The abnormal side is not the right middle cerebral artery (MCA) distribution (green) but is the left side (blue) where the TTP is markedly shortened.](images/app.statdx.com_image_thumbnail_4d243507-e1fc-45da-a8bc-25428634381b_annotated_true_size_900_quality_90_abae79d92c1d82b4be895eeb3932bd8852ca2d64.jpg) +*TTP in the same patient is even more striking. The abnormal side is not the right middle cerebral artery (MCA) distribution (green) but is the left side (blue) where the TTP is markedly shortened.* + +![Axial T2 MR in the same patient shows gyral swelling, sulcal effacement, and hyperintensity in the left temporal and parietooccipital cortex/subcortical white matter , basal ganglia . DWI (not shown) was normal. This is a classic example of postcarotid endarterectomy hyperperfusion syndrome.](images/app.statdx.com_image_thumbnail_3d6324ff-3891-40be-9cd0-fc74b988f2bb_annotated_true_size_900_quality_90_d376d60cf9436f3cabf8dccfcd7bb1da6aabd039.jpg) +*Axial T2 MR in the same patient shows gyral swelling, sulcal effacement, and hyperintensity in the left temporal and parietooccipital cortex/subcortical white matter , basal ganglia . DWI (not shown) was normal. This is a classic example of postcarotid endarterectomy hyperperfusion syndrome.* + +![Anteroposterior view of DSA shows abrupt occlusion of the left MCA just distal to its origin in a patient with a sudden onset of right-sided weakness and stroke-like symptoms. Little collateral filling of the distal MCA is seen.](images/app.statdx.com_image_thumbnail_6657c897-6a36-49c6-95dd-a8f22c3ab8c2_annotated_true_size_900_quality_90_96ab8a1a009fc5a2d813339458c86e0273154297.jpg) +*Anteroposterior view of DSA shows abrupt occlusion of the left MCA just distal to its origin in a patient with a sudden onset of right-sided weakness and stroke-like symptoms. Little collateral filling of the distal MCA is seen.* + +![After superselective catheterization of the left MCA and infusion of tissue plasminogen activator for 2 hours, normal circulation was restored, as shown on this AP DSA.](images/app.statdx.com_image_thumbnail_fd9d3ac3-d4ea-4db7-a374-2d6ea2c1167e_annotated_true_size_900_quality_90_e20f70631b5bdde041012919122834bb6ad0fe89.jpg) +*After superselective catheterization of the left MCA and infusion of tissue plasminogen activator for 2 hours, normal circulation was restored, as shown on this AP DSA.* + +![Following restoration of normal blood flow in the previously occluded left MCA, the patient experienced worsening right-sided weakness and throbbing headache. Axial MR perfusion study shows elevated (red area ), not decreased, CBF in the left temporal and parietal lobes.](images/app.statdx.com_image_thumbnail_ad81614e-6598-475d-b45e-9622e38cfccc_annotated_true_size_900_quality_90_b9d3557b6afbb54d318dc6f2604e31006112b2de.jpg) +*Following restoration of normal blood flow in the previously occluded left MCA, the patient experienced worsening right-sided weakness and throbbing headache. Axial MR perfusion study shows elevated (red area ), not decreased, CBF in the left temporal and parietal lobes.* + +![Axial MR perfusion in the same patient shows elevated cerebral blood volume .](images/app.statdx.com_image_thumbnail_71a52af7-e497-41b9-bd11-cab3f1d21c26_annotated_true_size_900_quality_90_8dd5454a2780eb71178e3a9e0908ffb359a4cf27.jpg) +*Axial MR perfusion in the same patient shows elevated cerebral blood volume .* + +![Axial T1 C+ FS MR shows cerebral hyperperfusion in status epilepticus in a 52-year-old woman with left-sided weakness following prolonged seizure. Note the increased intravascular, sulcal enhancement in the right temporal lobe compared to the left hemisphere .](images/app.statdx.com_image_thumbnail_192dcbcc-cf34-4b94-b09a-21eedcae0d9a_annotated_true_size_900_quality_90_94eb879977ad5fa26eb34e03b61bd47441750476.jpg) +*Axial T1 C+ FS MR shows cerebral hyperperfusion in status epilepticus in a 52-year-old woman with left-sided weakness following prolonged seizure. Note the increased intravascular, sulcal enhancement in the right temporal lobe compared to the left hemisphere .* + +![pMR in the same patient shows increased CBF in the right temporal lobe , corresponding to the increased intravascular enhancement noted on previous image.](8b40e507-6a3e-43db-a67d-9a572596a434) +*pMR in the same patient shows increased CBF in the right temporal lobe , corresponding to the increased intravascular enhancement noted on previous image.* + + +### Additional Images + +![Axial NECT in a patient with confusion, right-sided weakness following left CEA shows subtle increased hypodensity in the cortex and subcortical WM of the left parieto-occipital lobes.](e23fed07-a6d9-4469-989e-2c071118f6ef) +*Axial NECT in a patient with confusion, right-sided weakness following left CEA shows subtle increased hypodensity in the cortex and subcortical WM of the left parieto-occipital lobes.* + +![Axial FLAIR MR in the same patient shows hyperintensity in the cortex and basal ganglia .](e996b817-e5ce-4ddf-be8d-532fc9a11d7a) +*Axial FLAIR MR in the same patient shows hyperintensity in the cortex and basal ganglia .* + +![Axial DWI MR in the same patient shows no evidence of restricted diffusion.](2387598a-ee27-4a6d-853a-8edad7cafac3) +*Axial DWI MR in the same patient shows no evidence of restricted diffusion.* + +![Axial T1 C+ FS MR in the same patient shows increased vascularity in the left parieto-occipital region.](dae6993f-94dd-4a1d-b6d9-ab4b5539c09a) +*Axial T1 C+ FS MR in the same patient shows increased vascularity in the left parieto-occipital region.* + +![Coronal T1 C+ FS MR in the same patient shows a faint capillary blush in the same area. This was cerebral hyperperfusion syndrome.](96e0c025-9e48-470e-aab2-b9577e4beb93) +*Coronal T1 C+ FS MR in the same patient shows a faint capillary blush in the same area. This was cerebral hyperperfusion syndrome.* + diff --git a/docs_md/articles/chiari-1-malformation_97837e15-0d39-4c87-8af0-028652b399a6.md b/docs_md/articles/chiari-1-malformation_97837e15-0d39-4c87-8af0-028652b399a6.md new file mode 100644 index 0000000..d5f16dd --- /dev/null +++ b/docs_md/articles/chiari-1-malformation_97837e15-0d39-4c87-8af0-028652b399a6.md @@ -0,0 +1,388 @@ +--- +title: "Chiari 1 Malformation" +docid: "97837e15-0d39-4c87-8af0-028652b399a6" +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: "Pathology-Based Diagnoses" + slug: "pathology-based-diagnoses" + treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" + - + name: "Congenital Malformations" + slug: "congenital-malformations" + treeNodeId: "3595c1ab-3f1d-4896-b6bf-21d939d620b7" + - + name: "Chiari Malformations" + slug: "chiari-malformations" + treeNodeId: "62d04f46-bd30-4031-ac59-1b1f8ad544ed" + - + name: "Chiari 1 Malformation" + slug: "chiari-1-malformation" + treeNodeId: null +category: "Brain" +documentVersionId: "dd3b117c-bb35-4cb2-b42e-a22fc96536e7" +imageCount: 20 +lastUpdated: "07/31/20" +pageDescription: "Chiari 1 Malformation" +pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Congenital Malformations, Chiari Malformations, Chiari 1 Malformation" +pageTitle: "Chiari 1 Malformation | STATdx" +enhancedTitle: "Chiari 1 Malformation" +type: "DX" +references: true +ddx: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Pathology-Based Diagnoses" + - "Congenital Malformations" + - "Chiari Malformations" + - "Chiari 1 Malformation" +--- +# KEY FACTS + +- ## Terminology + + + - Chiari 1 malformation (CM1); synonyms: Chiari type 1, Chiari 1 deformity, cerebellar tonsillar ectopia +- ## Imaging + + + - Pointed cerebellar tonsils extending ≥ 5 mm below foramen magnum (basion-opisthion/McRae line) with effacement of CSF spaces + - ± retroflexed odontoid, horizontal shortened clivus, basilar invagination, atlanto-occipital assimilation + - ± caudal descent of brainstem, brainstem compression, medullary kink + - ± syringohydromyelia, scoliosis +- ## Top Differential Diagnoses + + + - Normal low-lying cerebellar tonsils + - Chiari 2 malformation + - Tonsillar herniation secondary to increased intracranial pressure + - Intracranial hypotension +- ## Pathology + + + - Most common cause believed to be small/underdeveloped posterior fossa; no association with open spinal dysraphism + - Can be result of premature closure of sutures + - Causes include shunted infantile hydrocephalus, bone dysplasias, genetic syndromes +- ## Clinical Issues + + + - Most common presenting symptom: Occipital headache + - Up to 30% of patients asymptomatic + - Goal of surgery in symptomatic patients: Restore normal CSF flow at foramen magnum + - Suboccipital decompression, resection of C1 posterior arch ± duraplasty, cerebellar tonsil cautery +- ## Diagnostic Checklist + + + - Degree of tonsillar descent does not always correlate with symptoms: CM1 frequently picked up incidentally + - Look for presence of syrinx → makes surgical intervention more likely + +# TERMINOLOGY + +- ## Synonyms + + + - Chiari type 1, Chiari 1 deformity +- ## Definitions + + + - Chiari 1 malformation (CM1): Compressed & pointed cerebellar tonsils extending below foramen magnum with effacement of CSF spaces + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Pointed cerebellar tonsils (unilateral or bilateral) extending ≥ 5 mm below foramen magnum (basion-opisthion line, a.k.a. McRae line) + - Mild variations in measurement reported in literature; measurement on its own may not be definitive of diagnosis + - No consensus statement on exact definition + - ### Location + + + - Craniocervical junction (CCJ) +- ## Radiographic Findings + + + - Shortened horizontal clivus, basilar invagination, CCJ segmentation anomalies, scoliosis +- ## CT Findings + + + - Crowding of foramen magnum on axial CT images + - Sagittal reconstructed images are very helpful + - Partially imaged superior aspect of spinal cord syrinx may be identified + - Associated osseous anomalies may include small posterior fossa, short horizontal clivus, retroverted dens, basilar invagination, platybasia, hypoplastic occipital condyles, segmentation anomalies (such as atlantooccipital assimilation), scoliosis +- ## MR Findings + + + - T1WI, T2WI, FLAIR + - Pointed (not rounded) cerebellar tonsils extending ≥ 5 mm below foramen magnum + - Crowded foramen magnum with small/effaced cisterns ± brainstem compression (kinking) + - ± small posterior fossa, elongated 4th ventricle + - ± syringohydromyelia/syrinx, scoliosis + - Syrinx reported in 30-70% of cases + - Patients with syrinx more likely to have scoliotic curve > 20⁰ (~ 70%) than those without syrinx (~ 45%) + - Other descriptions usually considered subtypes + - Chiari 1.5: Brainstem herniation + - Obex located below foramen magnum + - Complex Chiari: Medullary kink, retroflexed dens, abnormal clival-cervical angle, atlantooccipital assimilation, basilar invagination, platybasia + - MR cine + - Restricted CSF flow through foramen magnum ± ↑ brainstem/cerebellar tonsil motion (pistoning) + - Clinical utility of this sequence debatable +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Multiplanar MR + - Axial True FISP/FIESTA MR of CCJ helpful in assessing degree of foramen magnum crowding + - Spine imaging to look for spinal cord syrinx + - Syrinx makes surgical intervention more likely + +# DIFFERENTIAL DIAGNOSIS + +- ## Normal Variation of Cerebellar Tonsil Position + + + - Tonsils may normally lie below foramen magnum + - May be accentuated by certain head positions + - Tonsils retain normal rounded configuration +- [Chiari 2 Malformation](/document/chiari-2/008f0235-43ba-47e6-b51c-cfd8526afd68) + - Numerous intracranial findings centered around very small posterior fossa with hindbrain herniation in setting of open spinal dysraphism + - More severe clinical phenotype than CM1 +- ## Tonsillar Herniation Secondary to Increased Intracranial Pressure + + + - Neoplasm, hemorrhage, hydrocephalus, infarct +- [Intracranial Hypotension](/document/intracranial-hypotension/b7e1fbcf-a25c-4b70-b825-0d4c51afc99d) + - Look for "slumped" brainstem + - Sagging midbrain, sunken hindbrain with diffuse dural thickening/enhancement, distended veins/dural sinuses, ± subdural hygromas +- ## Chiari 0 + + + - Syringomyelia without cerebellar tonsillar ectopia; syrinx resolves after posterior fossa decompression + - Diagnosis of exclusion (many other causes of spinal cord syrinx) + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Primary congenital malformation vs. secondarily acquired morphologic changes + - Primary: Posterior fossa underdevelopment theory most common + - Underdevelopment of endochondral occipital bone → small posterior fossa vault + downward hindbrain herniation + - Not all Chiari 1 patients have small posterior fossa + - Secondary: Premature closure of cranial sutures &/or generalized abnormal bone formation + - Shunted infantile hydrocephalus + - Calvarial thickening of bone dysplasias or thalassemia + - Genetic syndromes + - Seen in 2-10% of patients with idiopathic intracranial hypertension (a.k.a. pseudotumor cerebri) + - ### Genetics + + + - Small posterior fossa in isolation is heritable + - Multiple potential causative genes on chromosome 1 & 22 identified + - Syndromic/familial associations (up to 60% of cases) + - Craniosynostosis syndromes (ERF-related, FGFR-related: Apert, Crouzon, Pfeiffer) + - Osteopathic syndromes (achondroplasia, rickets) + - Vertebral anomalies (Klippel-Feil, VACTERL) + - Craniofacial anomalies (Pierre-Robin, Goldenhar) + - Macrocerebellum (Costello syndrome, Sotos syndrome, macrocephaly-capillary malformation syndrome, Alexander disease) + - Increased brain volume (NF1/RAS/MAPK mutations/RASopathies, PTEN-PI3K/AKT mutations/PTENopathies) + - Many others: Ehlers-Danlos syndrome, Marfan syndrome, Williams syndrome, Kabuki syndrome +- ## Gross Pathologic & Surgical Features + + + - Herniated cerebellar tonsils become atrophic/gliotic/necrotic + - Arachnoid scarring & adhesions at foramen magnum + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Occipital headache + - Exacerbated by cough, Valsalva, neck extension, or physical exertion + - Less common: Cerebellar, brainstem, bulbar, cord motor/sensory symptoms + - Cerebellar symptoms: Ataxia, dysarthria, oscillopsia, nystagmus + - Brainstem and Bulbar symptoms: Vertigo, diplopia, dysphagia, aspiration, apnea, syncope, bradycardia, sudden death (rare) + - Spinal cord dysfunction: Motor and sensory losses, hyporeflexia, hyperreflexia, clonus, gait disturbance, neuropathic joint, urinary incontinence, positive Babinski sign, scoliosis + - 15-30% of adults with CM1 are asymptomatic, up to 35% of children with 5-10 mm of tonsillar herniation are asymptomatic + - Not much difference in clinical symptoms between complex Chiari & typical Chiari 1 +- ## Demographics + + + - True prevalence is unknown given how frequently it is picked up incidentally + - Epidemiology: 0.5-3.5% of general population + - Age: Evenly distributed in adult & pediatric patients + - 3% of children & 1% of adults have imaging findings of CM1, though age of clinical presentation unclear + - One series reports median age of presentation in children ≈ 8 years + - Sex: F > M (as high as 3:1) +- ## Natural History & Prognosis + + + - Natural history not clearly understood + - Many patients asymptomatic for prolonged periods + - Increasing ectopia + time → ↑ likelihood of symptoms + - Children respond better to treatment than adults + - Patients selected for nonsurgical management usually have benign course, though spontaneous improvement & worsening have been described +- ## Treatment + + + - Posterior fossa decompression: Suboccipital craniectomy with C1 laminectomy ± duraplasty, arachnoid opening/dissection, cerebellar tonsil cautery/resection + - ↓ of syrinx size in majority of patients after decompression + - Complex Chiari 1 may also require odontoid resection or craniocervical junction fusion + - Scoliosis may improve from decompression alone but often requires bracing or additional surgery + - Postoperative complications in approximately 20% of adults and 37% of children + - Most common: CSF leak, pseudomeningocele, infection + - Increased risk with duraplasty + - 1-11% postoperative mortality + - Conservative management for asymptomatic or minimally symptomatic children without syrinx + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Degree of tonsillar descent does not always correlate with symptoms: CM1 frequently picked up incidentally +- ## Image Interpretation Pearls + + + - Description of additional posterior fossa findings helpful for surgical planning + - Look for variant occipital venous sinus (at site of future decompression) + + 24070612-ef62-48e4-90d7-6bdaad64902a + +## References + +# Selected References + +1. [Taylor DG et al: Cerebrospinal fluid area and syringogenesis in Chiari malformation type I. J Neurosurg. 1-6, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32084641%5Bpmid%5D) +1. [Dangouloff-Ros V et al: Incidental brain MRI findings in children: a systematic review and meta-analysis. AJNR Am J Neuroradiol. 40(11):1818-23, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31624116%5Bpmid%5D) +1. [Saletti V et al: Chiari I malformation in defined genetic syndromes in children: are there common pathways? Childs Nerv Syst. 35(10):1727-39, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31363831%5Bpmid%5D) +1. [Poretti A et al: Chiari type 1 deformity in children: pathogenetic, clinical, neuroimaging, and management aspects. Neuropediatrics. 47(5):293-307, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27337547%5Bpmid%5D) +1. [Arnautovic A et al: Pediatric and adult Chiari malformation type I surgical series 1965-2013: a review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr. 15(2):161-77, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25479580%5Bpmid%5D) +1. [Brockmeyer DL et al: Complex Chiari malformations in children: diagnosis and management. Neurosurg Clin N Am. 26(4):555-60, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26408065%5Bpmid%5D) +1. [Leonard JR et al: Chiari I malformation: adult and pediatric considerations. Neurosurg Clin N Am. 26(4):xiii-xiv, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26408069%5Bpmid%5D) +1. [Rozenfeld M et al: MRI findings after surgery for Chiari malformation type I. AJR Am J Roentgenol. 205(5):1086-93, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26496557%5Bpmid%5D) +1. [Strahle J et al: The association between Chiari malformation Type I, spinal syrinx, and scoliosis. J Neurosurg Pediatr. 15(6):607-11, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26030330%5Bpmid%5D) +1. [Godzik J et al: Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation Type I with associated syringomyelia. J Neurosurg Pediatr. 13(4):368-74, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24527859%5Bpmid%5D) +1. [Lee S et al: Surgical outcome of Chiari I malformation in children: clinico-radiological factors and technical aspects. Childs Nerv Syst. 30(4):613-23, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24604349%5Bpmid%5D) +1. [Markunas CA et al: Genetic evaluation and application of posterior cranial fossa traits as endophenotypes for Chiari type I malformation. Ann Hum Genet. 78(1):1-12, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24359474%5Bpmid%5D) +1. [McVige JW et al: Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 32(1):95-126, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24287386%5Bpmid%5D) +1. [Moore HE et al: Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation. Pediatr Radiol. 44(11):1403-11, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24898393%5Bpmid%5D) +1. Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Philadelphia: Lippincott Williams & Wilkins. 491-96, 2012 +1. [Bollo RJ et al: Complex Chiari malformations in children: an analysis of preoperative risk factors for occipitocervical fusion. J Neurosurg Pediatr. 10(2):134-41, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22725652%5Bpmid%5D) +1. [Hwang SW et al: Outcomes of Chiari I-associated scoliosis after intervention: a meta-analysis of the pediatric literature. Childs Nerv Syst. 28(8):1213-9, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22526438%5Bpmid%5D) +1. [Brockmeyer DL: The complex Chiari: issues and management strategies. Neurol Sci. 32 Suppl 3:S345-7, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21822705%5Bpmid%5D) +1. [Strahle J et al: Natural history of Chiari malformation type I following decision for conservative treatment. J Neurosurg Pediatr. 8(2):214-21, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21806365%5Bpmid%5D) +1. [Strahle J et al: Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr. 8(2):205-13, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21806364%5Bpmid%5D) +1. [Tubbs RS et al: Institutional experience with 500 cases of surgically treated pediatric Chiari malformation type I. J Neurosurg Pediatr. 7(3):248-56, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21361762%5Bpmid%5D) +1. [Hofkes SK et al: Differentiation between symptomatic Chiari I malformation and asymptomatic tonsilar ectopia by using cerebrospinal fluid flow imaging: initial estimate of imaging accuracy. Radiology. 245(2):532-40, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17890352%5Bpmid%5D) +1. [Tubbs RS et al: A critical analysis of the Chiari 1.5 malformation. J Neurosurg. 101(2 Suppl):179-83, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15835105%5Bpmid%5D) +1. [Milhorat TH et al: Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 44(5):1005-17, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10232534%5Bpmid%5D) + +## Differential diagnosis + +### Cisterna Magna Mass +DDX:047add0c-7e4f-40a0-9933-8d6fa00a24f7 + +### Congenital Cerebellar Malformation +DDX:e0a671d3-a236-4ff9-a232-a9684218d010 + +### Foramen Magnum Mass +DDX:a79c61b3-b26a-48c8-bcfb-bf8afd3ef25e + + +## Images + + +### Selected Images + +![Sagittal graphic demonstrates pointed cerebellar tonsils extending below the foramen magnum to the inferior aspect of the C1 posterior arch. The obex is inferiorly displaced as well.](images/app.statdx.com_image_thumbnail_272c608b-434b-4ce8-9576-95fb7814b8e6_annotated_true_size_900_quality_90_9c6d247ad8c9846d6da7d01e369550c9cf3b5e56.jpg) +*Sagittal graphic demonstrates pointed cerebellar tonsils extending below the foramen magnum to the inferior aspect of the C1 posterior arch. The obex is inferiorly displaced as well.* + +![Sagittal graphic demonstrates pointed cerebellar tonsils extending below the foramen magnum to the inferior aspect of the C1 posterior arch. The obex is inferiorly displaced as well.](images/app.statdx.com_image_thumbnail_272c608b-434b-4ce8-9576-95fb7814b8e6_size_174_quality_85_a5f1f814eec3d1b3d4f5d9d8b5a57d8d8d1f21cc.jpg) +*Sagittal graphic demonstrates pointed cerebellar tonsils extending below the foramen magnum to the inferior aspect of the C1 posterior arch. The obex is inferiorly displaced as well.* + +![Sagittal T1 MR of an 8-year-old with boy incidentally noted Chiari 1 malformation (CM1) demonstrates pointed, low-lying cerebellar tonsils reaching the level of the posterior C1 arch as well as downward displacement of the brainstem with cervicomedullary junction just below C1 posterior arch .](images/app.statdx.com_image_thumbnail_66985e1d-37b2-4340-aaf6-60662dd6d9ce_annotated_true_size_900_quality_90_ec887e29d469463874a8fb8610109bc94e729379.jpg) +*Sagittal T1 MR of an 8-year-old with boy incidentally noted Chiari 1 malformation (CM1) demonstrates pointed, low-lying cerebellar tonsils reaching the level of the posterior C1 arch as well as downward displacement of the brainstem with cervicomedullary junction just below C1 posterior arch .* + +![Coronal T1 MR in the same patient demonstrates the left cerebellar tonsil is lower than the right. In CM1, either one or both cerebellar tonsils may be involved.](images/app.statdx.com_image_thumbnail_9b2e4ac6-ee05-4365-852e-d087deaed27e_annotated_true_size_900_quality_90_e192c7b61105f73640e7b9e09b7a30ebf947c466.jpg) +*Coronal T1 MR in the same patient demonstrates the left cerebellar tonsil is lower than the right. In CM1, either one or both cerebellar tonsils may be involved.* + +![Axial FIESTA MR through the foramen magnum in the same patient demonstrates posterior displacement of the dens with associated deformity of the ventral caudally displaced medulla. Partial effacement of the CSF spaces surrounding the cerebellar tonsils denotes moderate foramen magnum crowding.](images/app.statdx.com_image_thumbnail_37bb3fef-5332-4f60-97c3-95f631358ae5_annotated_true_size_900_quality_90_cb868d8b361f00cb9b154875041b6b37caa0685d.jpg) +*Axial FIESTA MR through the foramen magnum in the same patient demonstrates posterior displacement of the dens with associated deformity of the ventral caudally displaced medulla. Partial effacement of the CSF spaces surrounding the cerebellar tonsils denotes moderate foramen magnum crowding.* + +![Sagittal T2 MR in a 5 year old with occipital headaches demonstrates pointed, low-lying cerebellar tonsils with associated effacement of CSF spaces. Note the short horizontal clivus , retroverted dens , and segmentation anomaly at the C3-4 levels .](images/app.statdx.com_image_thumbnail_5b20647f-d230-418f-b551-e380b948416f_annotated_true_size_900_quality_90_ada47242920afff77757d2cf4f7639ad8429589c.jpg) +*Sagittal T2 MR in a 5 year old with occipital headaches demonstrates pointed, low-lying cerebellar tonsils with associated effacement of CSF spaces. Note the short horizontal clivus , retroverted dens , and segmentation anomaly at the C3-4 levels .* + +![Coronal T2 MR in the same patient demonstrates pointed, low-lying cerebellar tonsils extending below the C1 ring , typical of CM1.](images/app.statdx.com_image_thumbnail_9648454a-28e6-4add-8eb3-632c27eec0fd_annotated_true_size_900_quality_90_df00b76be12a0cac017bd9369d70734f3533600b.jpg) +*Coronal T2 MR in the same patient demonstrates pointed, low-lying cerebellar tonsils extending below the C1 ring , typical of CM1.* + +![Sagittal phase contrast cine MR (with hyperintense signal at sites of active CSF or venous flow) in the same patient shows absence of CSF flow at the craniocervical junction due to the CM1.](images/app.statdx.com_image_thumbnail_2302352a-9e5c-4da9-9478-2759ad0a4f33_annotated_true_size_900_quality_90_ddb138e5204616c88f7a8d8c92cef2d9c98136d0.jpg) +*Sagittal phase contrast cine MR (with hyperintense signal at sites of active CSF or venous flow) in the same patient shows absence of CSF flow at the craniocervical junction due to the CM1.* + +![Sagittal phase contrast cine MR in a 16-year-old girl with occipital headaches being worked up for CM1 demonstrates normal CSF flow-related signal ventral and dorsal to the brainstem. No CM1 was identified on conventional sequences.](images/app.statdx.com_image_thumbnail_b22a3389-0ea5-461e-9411-142067d25234_annotated_true_size_900_quality_90_ccf9ea8d9f187e15512655c2e2fda66f0367d059.jpg) +*Sagittal phase contrast cine MR in a 16-year-old girl with occipital headaches being worked up for CM1 demonstrates normal CSF flow-related signal ventral and dorsal to the brainstem. No CM1 was identified on conventional sequences.* + +![Sagittal bone CT in a patient with hyperreflexia and severe CM1 demonstrates odontoid retroflexion and enlargement of the anterior C1 ring (which abnormally articulates with the remodeled clivus).](images/app.statdx.com_image_thumbnail_f6dd429e-8c9e-45c6-b0a1-5677d26aa466_annotated_true_size_900_quality_90_5c1ce86b0038f73e474a55657dfd214ef1d9b386.jpg) +*Sagittal bone CT in a patient with hyperreflexia and severe CM1 demonstrates odontoid retroflexion and enlargement of the anterior C1 ring (which abnormally articulates with the remodeled clivus).* + +![Sagittal T2 MR of the cervical spine in the same patient shows the odontoid process retroflexion as well as the cerebellar tonsillar ectopia and associated syringohydromyelia .](images/app.statdx.com_image_thumbnail_0a43355e-8521-48f6-92d1-759d2b2da587_annotated_true_size_900_quality_90_88aa91355df8fe5ccf62bdcb6d6c96cd6128d6fc.jpg) +*Sagittal T2 MR of the cervical spine in the same patient shows the odontoid process retroflexion as well as the cerebellar tonsillar ectopia and associated syringohydromyelia .* + +![A 5-year-old girl with CM1 demonstrates downward displacement of the cerebellar tonsils below the plane of the foramen magnum caudal to the posterior arch of C1 with an abnormal pointed morphology.](10b13965-71dd-48e5-804c-ee2f199f6676) +*A 5-year-old girl with CM1 demonstrates downward displacement of the cerebellar tonsils below the plane of the foramen magnum caudal to the posterior arch of C1 with an abnormal pointed morphology.* + +![Sagittal T1 MR in the same patient status post suboccipital decompression with suboccipital craniectomy, C1 laminectomy, expansive duraplasty, and cerebellar tonsillar shrinkage is shown. There is no residual cerebellar ectopia, and the inferior cerebellum has a normal, rounded morphology.](eae36e81-cb9b-4bee-8947-e36c78860a60) +*Sagittal T1 MR in the same patient status post suboccipital decompression with suboccipital craniectomy, C1 laminectomy, expansive duraplasty, and cerebellar tonsillar shrinkage is shown. There is no residual cerebellar ectopia, and the inferior cerebellum has a normal, rounded morphology.* + +![Axial FIESTA MR in the same patient with CM1 status post posterior fossa decompression demonstrates absence of the posterior arch of C1 from C1 laminectomy and expansive duraplasty . There is patent CSF surrounding the cervical spinal cord with no evidence of crowding.](a5dcb5ec-b9ef-433f-953f-0e3322675764) +*Axial FIESTA MR in the same patient with CM1 status post posterior fossa decompression demonstrates absence of the posterior arch of C1 from C1 laminectomy and expansive duraplasty . There is patent CSF surrounding the cervical spinal cord with no evidence of crowding.* + +![Sagittal T2 MR depicts marked cerebellar tonsillar ectopia . Clivus is mildly foreshortened. There is central edema in the cervical spinal cord without frank syringohydromyelia, a finding that has been described as presyrinx edema.](5da56cfd-ed66-4521-9ec1-1746b2da9ce0) +*Sagittal T2 MR depicts marked cerebellar tonsillar ectopia . Clivus is mildly foreshortened. There is central edema in the cervical spinal cord without frank syringohydromyelia, a finding that has been described as presyrinx edema.* + +![Sagittal T1 MR in a patient with osteopetrosis shows cerebellar tonsillar ectopia with extension of the elongated cerebellar tonsils to the C2/C3 level. The hypointense marrow signal reflects diffuse sclerosis.](1748f3c0-ac65-4a8e-941d-8c0dee156728) +*Sagittal T1 MR in a patient with osteopetrosis shows cerebellar tonsillar ectopia with extension of the elongated cerebellar tonsils to the C2/C3 level. The hypointense marrow signal reflects diffuse sclerosis.* + +![Axial T2 MR in the same patient reveals characteristic crowding of the foramen magnum with extension of the ectopic cerebellar tonsils into the upper cervical spinal canal.](652fef45-7edf-43c6-9397-6e1b5df0ea95) +*Axial T2 MR in the same patient reveals characteristic crowding of the foramen magnum with extension of the ectopic cerebellar tonsils into the upper cervical spinal canal.* + + +### Additional Images + +![Sagittal T2 MR in an asymptomatic Chiari 1 patient demonstrates severe cerebellar tonsillar ectopia . The tonsils produce deformation of the upper cervical spinal cord. There is abnormal T2 prolongation in the upper spinal cord reflecting edema and potentially a presyrinx state.](585f7730-106c-4922-b84b-7bb3bce2d9c6) +*Sagittal T2 MR in an asymptomatic Chiari 1 patient demonstrates severe cerebellar tonsillar ectopia . The tonsils produce deformation of the upper cervical spinal cord. There is abnormal T2 prolongation in the upper spinal cord reflecting edema and potentially a presyrinx state.* + +![Sagittal T2 MR from a 10-year-old patient demonstrates pointed cerebellar tonsils extending below the foramen magnum to the lower C1 level , typical of CM1. The CSF is largely effaced at the craniocervical junction, and a syrinx is partially seen in the cervical spinal cord.](44374f59-441a-4b4a-a254-bcbc07e5f4b5) +*Sagittal T2 MR from a 10-year-old patient demonstrates pointed cerebellar tonsils extending below the foramen magnum to the lower C1 level , typical of CM1. The CSF is largely effaced at the craniocervical junction, and a syrinx is partially seen in the cervical spinal cord.* + +![Axial true FISP/FIESTA MR through the foramen magnum in the same patient shows crowding and effacement of the CSF spaces by the low cerebellar tonsils .](fc721de9-3d7e-486a-a6bc-2fdfa99a2b8b) +*Axial true FISP/FIESTA MR through the foramen magnum in the same patient shows crowding and effacement of the CSF spaces by the low cerebellar tonsils .* + +![Sagittal T1 MR of the cervical spine in the same patient further demonstrates the cerebellar tonsillar ectopia and large cervicothoracic spinal cord syrinx .](b7824d1b-b1b5-43b3-a406-52dbd795acb5) +*Sagittal T1 MR of the cervical spine in the same patient further demonstrates the cerebellar tonsillar ectopia and large cervicothoracic spinal cord syrinx .* + diff --git a/docs_md/articles/childhood-stroke_12f14b63-8dd0-4523-afe1-6fda2331e6bf.md b/docs_md/articles/childhood-stroke_12f14b63-8dd0-4523-afe1-6fda2331e6bf.md new file mode 100644 index 0000000..8208d87 --- /dev/null +++ b/docs_md/articles/childhood-stroke_12f14b63-8dd0-4523-afe1-6fda2331e6bf.md @@ -0,0 +1,461 @@ +--- +title: "Childhood Stroke" +docid: "12f14b63-8dd0-4523-afe1-6fda2331e6bf" +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: "Pathology-Based Diagnoses" + slug: "pathology-based-diagnoses" + treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" + - + name: "Stroke" + slug: "stroke" + treeNodeId: "12307683-f1ff-4823-a7d3-b10b40f9fd82" + - + name: "Cerebral Ischemia and Infarction" + slug: "cerebral-ischemia-and-infarction" + treeNodeId: "51051846-a223-42f7-b626-2a5a26cf6c44" + - + name: "Childhood Stroke" + slug: "childhood-stroke" + treeNodeId: null +category: "Brain" +cmeTopicId: "b9fb5260-1c19-4564-8317-85020cff8575" +documentVersionId: "b5f22640-3bb2-4c58-8b6f-4193ac9ef6db" +imageCount: 31 +lastUpdated: "08/06/20" +pageDescription: "Childhood Stroke" +pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Cerebral Ischemia and Infarction, Childhood Stroke" +pageTitle: "Childhood Stroke | STATdx" +enhancedTitle: "Childhood Stroke" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Pathology-Based Diagnoses" + - "Stroke" + - "Cerebral Ischemia and Infarction" + - "Childhood Stroke" +--- +# KEY FACTS + +- ## Terminology + + + - Acute neurologic dysfunction due to loss of vascular integrity +- ## Imaging + + + - NECT: ↓ attenuation of affected gray matter + - Insular ribbon sign → loss of distinct insular cortex + - Hyperdense MCA sign → thrombosed MCA + - MR: ↓ diffusion within ~ 30 minutes of arterial occlusion + - Cytotoxic edema evident in affected territory on FLAIR/T2 by 4-6 hours after arterial occlusion + - Enhancement of infarct typically occurs after 5-7 days + - CTA/MRA: Critical for early evaluation & identification of possible etiology (e.g., dissection, arteriopathy) + - MR perfusion imaging can provide valuable information regarding region at risk in setting of acute stroke + - Arterial spin labeling (ASL) can provide useful perfusion information without contrast administration +- ## Top Differential Diagnoses + + + - Seizure-related injury + - Acute encephalitis + - Mitochondrial encephalopathies + - Posterior reversible encephalopathy syndrome +- ## Pathology + + + - Major causes: Cardiac disease (~ 25%), moyamoya-type arteriopathy, dissection, vasculitis, hematologic/metabolic + - No underlying cause discovered in ~ 25% of cases +- ## Clinical Issues + + + - Incidence: 2-3/100,000 per year in USA + - Mortality: 0.6/100,000 + - Children typically present later than adults (> 24 hours) + - Focal deficit may be masked by lethargy, coma, irritability + - Treatment in pediatric acute stroke usually conservative + - Thrombolysis/thrombectomy not well studied in children + - Capacity for recovery in children much > adults +- ## Diagnostic Checklist + + + - When stroke is suspected clinically or by imaging, do not hesitate to perform vessel imaging + +# TERMINOLOGY + +- ## Synonyms + + + - Cerebrovascular accident, cerebral infarct, cerebral ischemia +- ## Definitions + + + - Acute alteration of neurologic function due to loss of vascular integrity + - This chapter specifically addresses arterial ischemia beyond perinatal period + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Cytotoxic edema & restricted diffusion (acutely) in affected vascular territory + - ### Location + + + - Proximal & distal middle cerebral artery (MCA) territory most commonly affected + - ### Morphology + + + - Stroke caused by arterial occlusion typically conforms to 1 arterial territory +- ## CT Findings + + + - ### NECT + + + - ↓ attenuation of affected gray matter with loss of normal gray matter-white matter differentiation + - ↓ in white matter attenuation less pronounced + - Often wedge-shaped & localized to 1 arterial territory + - Diffuse ischemic injury can lead to reversal sign with gray matter diffusely ↓ in attenuation relative to white matter + - Insular ribbon sign → loss of distinct of insular cortex + - Hyperdense MCA sign → ↑ density of acutely thrombosed MCA + - Hemorrhagic transformation (HT) + - Symptomatic HT in 3%; asymptomatic HT in 30% + - Asymptomatic HT usually parenchymal + - White matter or deep nuclear hemorrhage often mass-like → hematoma within infarcted tissue + - ### CECT + + + - Enhancement of infarcted territory typically occurs after 5-7 days + - ### CTA + + + - Invaluable for demonstrating focal vascular abnormalities in acute setting + - Intimal flap in acutely dissected vessel + - Major arterial occlusion may prompt thrombolysis or mechanical thrombectomy in appropriate setting +- ## MR Findings + + + - **T1WI**:**** Acute: ↓ signal with gyral swelling + - Chronic: ± ↑ signal in cortical laminar necrosis + - **T1WI FS**: Allows identification of mural hematoma (↑ signal) in dissected vessel + - **T2WI**: Loss of flow void in thrombosed vessel + - **FLAIR**: ↑ signal with gyral swelling (within 4-6 hours) + - Abnormal sulcal ↑ signal (climbing ivy sign) of chronic, slow-flow collaterals in setting of longstanding proximal vascular occlusion + - **DWI**: Most sensitive for early detection of ischemia + - Acute: Restricted diffusion (↑ DWI, ↓ ADC signal) ≤ 30 minutes after ischemic insult + - Subacute (7-14 days): Pseudonormalization of signal + - ↑ DWI, ADC ~ brain parenchyma + - Chronic: Facilitated diffusion in gliotic brain + - ↑/~ DWI, ↑ ADC + - **SWI/T2* GRE**: May see ↑ size & number of cortical vessels**** + - Suggests ↑ extraction fraction & possibly recoverable brain + - **T1WI C+**: Cortical & leptomeningeal enhancement seen after 5-7 days following acute infarct + - Enhancing climbing ivy sign + - **MRA**: Can detect arterial occlusion & stenosis in large- & medium-sized cerebral vessels + - Important to identify underlying dissection or arteriopathy + - **PWI**: Provides valuable information about affected brain + - Ischemic penumbra: ↓ perfusion, no DWI change (PWI-DWI mismatch) + - May define brain salvageable with acute stroke therapy + - Arterial spin labeling can provide useful perfusion information without contrast administration + - **MRS**: ↑ lactate hallmark of ischemia/infarct + - Not specific + - **Vessel wall imaging**: Vessel wall enhancement patterns improve discrimination of underlying stroke etiology +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Affected territory hyperechoic in acute/subacute stage + - ### Color Doppler + + + - Direct Doppler evaluation ideal for surveillance of vascular occlusion in neonate with open sutures + - Transcranial Doppler evaluation of circle of Willis through temporal squamosa + - ↑ velocities can predict stenoses detectable by MRA + - Used as screening tool in children with sickle cell anemia +- ## Angiographic Findings + + + - Catheter angiography rarely necessary in acute evaluation of childhood stroke + - Justified if contemplating endovascular therapy + - Best modality for detailed evaluation of primary arteriopathies +- ## Nuclear Medicine Findings + + + - PET & SPECT techniques can be used to + - Identify salvageable regions at risk (ischemic penumbra) + - Demonstrate effects of synangiosis surgery in moyamoya-type vasculopathies +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - CT initial imaging test for signs/symptoms of stroke; excellent for excluding hemorrhagic stroke (more common in children vs. adults) + - MR with DWI, MRA, PWI + - ### Protocol advice + + + - Contrast can help in assessing timing of injury & performing perfusion imaging + - Consider dedicated vessel wall imaging + +# DIFFERENTIAL DIAGNOSIS + +- ## Seizure-Related Injury + + + - Swelling & restricted diffusion secondary to persistent seizure activity + - Differentiation by clinical presentation & EEG +- [Acute Encephalitis](/document/acute-encephalitis/a45f63bb-c25b-481d-a001-9c520c58060b) + - Acute parenchymal inflammation secondary to infectious agents, typically viral + - Slower onset with encephalopathy +- [Mitochondrial Encephalopathies](/document/mitochondrial-encephalopathies/40004435-b768-4baf-a31e-651f8a174fe2) + - Symmetric basal ganglia involvement common + - Usually have manifestations beyond CNS +- [Posterior Reversible Encephalopathy Syndrome](/document/acute-hypertensive-encephalopathy--/efc6f9c2-dad9-4eb8-bad2-421bfaf1ec57) + - Patchy cortical/subcortical edema most common in parietal & occipital lobes, typically in setting of hypertension + - Diffusion restriction uncommon +- [Neonatal Herpes Encephalitis](/document/herpes-encephalitis-type-1/556f5f76-c20b-44ca-a913-c53b11c93341) + - Infant with seizures 2-5 weeks after birth + - DWI most sensitive for detection in early disease + - Often bilateral with temporal predominance, but can occur anywhere +- ## MELAS + + + - **M**itochondrial **e**ncephomyopathy, **l**actic acidosis, **s**troke-like episodes + - Areas of ischemia crossing arterial territories, often parietal + - MRS: ↑ lactate in normal-appearing brain +- [Group B Strep Meningitis](/document/group-b-streptococcal-meningitis/bafa10c7-e65b-4432-9959-b8e5e4af708c) + - Associated vasculitis causes ischemia in small perforating arteries + - Unilateral or bilateral deep gray nuclei ischemia + +# PATHOLOGY + +- ## General Features + + + - 6 major causes of arterial stroke in children + - Cardiac disease (~ 25%) + - Congenital heart disease, valvular heart disease, arrhythmias, & cardiomyopathies + - Moyamoya-type arteriopathy + - Sickle cell disease + - Neurofibromatosis type 1 + - Radiation therapy + - Trisomy 21 + - Alagille syndrome + - Arterial dissection (e.g., trauma) + - CNS vasculitis + - Hematologic/metabolic (e.g., coagulopathy) + - Idiopathic (~ 25%) + - No underlying cause discovered + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Depend on patient age, etiology, & involved artery + - < 1 year: Seizures, encephalopathy > focal neurologic + - > 1 year: Usually focal neurologic (e.g., hemiplegia, early hand preference) + - Speech difficulties, gait abnormality, seizure + - Seizure → deficit often attributed to postictal state (Jacksonian paralysis) + - Embolic cause: Sudden onset of symptoms + - Stenoocclusive cause: Gradual/intermittent (e.g., TIA) + - Focal deficit may be masked by lethargy, coma, irritability + - Children typically present later than adults (> 24 hours) + - Poor recognition/understanding of symptoms by child, caregiver, physician + - Uncommon diagnosis in children requires high degree of suspicion +- ## Demographics + + + - ### Age + + + - Incidence/mortality greatest < 1 year + - Large percentage occur in perinatal period + - Perinatal arterial ischemic stroke (PAIS) + - ### Epidemiology + + + - Incidence: 2-3/100,000 per year in USA + - Mortality: 0.6/100,000 + - Underrecognized as significant source of morbidity in pediatric population +- ## Natural History & Prognosis + + + - Capacity for recovery better than in adults, due to + - Better compensatory mechanisms, collateral recruitment, neuronal plasticity + - Fewer concomitant risk factors +- ## Treatment + + + - Clinical window of opportunity/benefit not as well understood in children as compared to adults + - Mainstay of chronic therapy for fixed vascular lesions & vasculopathies: Aspirin + - Transfusion therapy for at-risk children with sickle cell + - Mechanical thrombectomy may be considered in certain patient presentations + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - Use same imaging signs as adults + - Have low threshold for use of CTA + + 994b2dbd-754f-4f23-9516-6f515e4a2678 + +## References + +# Selected References + +1. [Felling RJ et al: Predicting recovery and outcome after pediatric stroke: results from the International Pediatric Stroke Study. Ann Neurol. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32215969%5Bpmid%5D) +1. [Ibrahim AY et al: Fractional flow on TOF-MRA as a measure of stroke risk in children with intracranial arterial stenosis. AJNR Am J Neuroradiol. 41(3):535-41, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32115418%5Bpmid%5D) +1. [Morotti A et al: Pediatric ischemic stroke. J Neurol. 267(4):1221-2, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32157384%5Bpmid%5D) +1. [Donahue MJ et al: Neuroimaging advances in pediatric stroke. Stroke. 50(2):240-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30661496%5Bpmid%5D) +1. [Dlamini N et al: Arterial wall imaging in pediatric stroke. Stroke. 49(4):891-8, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29581340%5Bpmid%5D) +1. [Khalaf A et al: Pediatric stroke imaging. Pediatr Neurol. 86:5-18, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30122281%5Bpmid%5D) +1. [Beslow LA: Stroke Diagnosis in the pediatric emergency department: an ongoing challenge. Stroke. 48(5):1132-3, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28336680%5Bpmid%5D) +1. [Satti S et al: Mechanical thrombectomy for pediatric acute ischemic stroke: review of the literature. J Neurointerv Surg. 9(8):732-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27448827%5Bpmid%5D) +1. [Wilson JL et al: Endovascular therapy in pediatric stroke: utilization, patient characteristics, and outcomes. Pediatr Neurol. 69:87-92.e2, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28233666%5Bpmid%5D) +1. [Madaelil TP et al: Mechanical thrombectomy in pediatric acute ischemic stroke: clinical outcomes and literature review. Interv Neuroradiol. 22(4):426-31, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26945589%5Bpmid%5D) +1. [Polan RM et al: Susceptibility-weighted imaging in pediatric arterial ischemic stroke: a valuable alternative for the noninvasive evaluation of altered cerebral hemodynamics. AJNR Am J Neuroradiol. 36(4):783-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25477354%5Bpmid%5D) +1. [Bernard TJ et al: Emergence of the primary pediatric stroke center: impact of the thrombolysis in pediatric stroke trial. Stroke. 45(7):2018-23, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24916908%5Bpmid%5D) +1. [Gemmete JJ et al: Arterial ischemic stroke in children. Neuroimaging Clin N Am. 23(4):781-98, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24156865%5Bpmid%5D) +1. [Freundlich CL et al: Pediatric stroke. Emerg Med Clin North Am. 30(3):805-28, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22974650%5Bpmid%5D) +1. [Kitchen L et al: The pediatric stroke outcome measure: a validation and reliability study. Stroke. 43(6):1602-8, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22474056%5Bpmid%5D) +1. [Beslow LA et al: Hemorrhagic transformation of childhood arterial ischemic stroke. Stroke. 42(4):941-6, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21350202%5Bpmid%5D) +1. [Cárdenas JF et al: Pediatric stroke. Childs Nerv Syst. 27(9):1375-90, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21336993%5Bpmid%5D) +1. [Dowling MM et al: Intracardiac shunting and stroke in children: a systematic review. J Child Neurol. 26(1):72-82, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21212453%5Bpmid%5D) +1. [Lanni G et al: Pediatric stroke: clinical findings and radiological approach. Stroke Res Treat. 2011:172168, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21603166%5Bpmid%5D) +1. [Larrue V et al: Etiologic investigation of ischemic stroke in young adults. Neurology. 76(23):1983-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21646623%5Bpmid%5D) +1. [Munot P et al: Characteristics of childhood arterial ischemic stroke with normal MR angiography. Stroke. 42(2):504-6, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21193747%5Bpmid%5D) +1. [Sedney CL et al: Cervical abnormalities causing vertebral artery dissection in children. J Neurosurg Pediatr. 7(3):272-5, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21361766%5Bpmid%5D) +1. [Shellhaas RA et al: Mimics of childhood stroke: characteristics of a prospective cohort. Pediatrics. 118(2):704-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16882826%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right middle cerebral artery (MCA) territory . Note the sulcal effacement & loss of the gray matter-white matter differentiation.](images/app.statdx.com_image_thumbnail_4aa0e379-57fe-407d-af6f-af58fde1c979_annotated_true_size_900_quality_90_3b3be24eb74b4794faea29ad8ec6f6c94ab0bb32.jpg) +*Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right middle cerebral artery (MCA) territory . Note the sulcal effacement & loss of the gray matter-white matter differentiation.* + +![Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right middle cerebral artery (MCA) territory . Note the sulcal effacement & loss of the gray matter-white matter differentiation.](images/app.statdx.com_image_thumbnail_4aa0e379-57fe-407d-af6f-af58fde1c979_size_174_quality_85_abbf2b5373f00df1c3a6f18b8eee80cfe04d1607.jpg) +*Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right middle cerebral artery (MCA) territory . Note the sulcal effacement & loss of the gray matter-white matter differentiation.* + +![Axial DWI MR in the same patient confirms restricted diffusion in the right MCA territory . Also note the focus of restricted diffusion in the left periventricular region . Multiple infarcts in multiple vascular territories should raise suspicion of a proximal embolic source.](images/app.statdx.com_image_thumbnail_c47fcfac-a966-4233-9ab7-d170e8ed5763_annotated_true_size_900_quality_90_8e1912bbc856b0097561b1b2df24336a84d8d027.jpg) +*Axial DWI MR in the same patient confirms restricted diffusion in the right MCA territory . Also note the focus of restricted diffusion in the left periventricular region . Multiple infarcts in multiple vascular territories should raise suspicion of a proximal embolic source.* + +![Axial DWI MR in a 6 year old with imbalance and acute infarct of the left basal ganglia shows diffusion restriction (↓ADC not shown). Acute infarct in a child should prompt further evaluation with MRA or CTA to detect an underlying vessel abnormality.](images/app.statdx.com_image_thumbnail_f8c3d7cd-ebe4-40e8-bebc-49de656e3e57_annotated_true_size_900_quality_90_03a1d848299e6e60cffde1b47e4ad9035e9496d8.jpg) +*Axial DWI MR in a 6 year old with imbalance and acute infarct of the left basal ganglia shows diffusion restriction (↓ADC not shown). Acute infarct in a child should prompt further evaluation with MRA or CTA to detect an underlying vessel abnormality.* + +![3D MRA of the circle of Willis in the same patient shows irregular narrowing of the left proximal and distal segments of the middle cerebral artery, consistent with vasculitis.](images/app.statdx.com_image_thumbnail_60361896-c4fb-4710-9770-98c052a5cb95_annotated_true_size_900_quality_90_fa3ba3cad53a57c9668cc8932c5ad255f0d361dc.jpg) +*3D MRA of the circle of Willis in the same patient shows irregular narrowing of the left proximal and distal segments of the middle cerebral artery, consistent with vasculitis.* + +![Axial TOF MRA in a 2 year old with multiple infarcts of various ages shows multiple small areas of flow-related signal in the bilateral thalami, consistent with lenticulostriate collaterals of moyamoya.](images/app.statdx.com_image_thumbnail_a44f7e28-cc05-4312-bc74-9dcf223ca9d4_annotated_true_size_900_quality_90_75a0aff8703f310d52843a62ba766f8a3e9e8ff8.jpg) +*Axial TOF MRA in a 2 year old with multiple infarcts of various ages shows multiple small areas of flow-related signal in the bilateral thalami, consistent with lenticulostriate collaterals of moyamoya.* + +![Axial DWI MR in the same 2-year-old girl with moyamoya-type vasculopathy shows diffusion restriction in the right frontoparietal foci of signal abnormality , suggesting an acute/subacute infarct. However, there is no diffusion restriction in the left parietal region , suggesting this infarct is of an older age.](images/app.statdx.com_image_thumbnail_5b17aa5b-17f0-4ac2-a66f-d851a1a101d6_annotated_true_size_900_quality_90_37023263c4fe22601539ed7f5d9549229f44657d.jpg) +*Axial DWI MR in the same 2-year-old girl with moyamoya-type vasculopathy shows diffusion restriction in the right frontoparietal foci of signal abnormality , suggesting an acute/subacute infarct. However, there is no diffusion restriction in the left parietal region , suggesting this infarct is of an older age.* + +![Axial T1 C+ MR in a 1 year old with arteriopathy & subacute infarction shows gyriform enhancement of the cortical ribbon. Enhancement is common in the subacute phase of infarction. Precontrast T1 is necessary to distinguish true enhancement from the intrinsic ↑ T1 seen in cortical laminar necrosis.](images/app.statdx.com_image_thumbnail_b0846fe4-f337-4ea4-83d1-26eec0c66971_annotated_true_size_900_quality_90_d70a5976f441aeb9a1f2ae95e2b20e26f9d5cd57.jpg) +*Axial T1 C+ MR in a 1 year old with arteriopathy & subacute infarction shows gyriform enhancement of the cortical ribbon. Enhancement is common in the subacute phase of infarction. Precontrast T1 is necessary to distinguish true enhancement from the intrinsic ↑ T1 seen in cortical laminar necrosis.* + +![Axial ADC map in the same patient shows modestly ↓ ADC within the affected cortex but resolution of acute gyral swelling, as evidenced by prominent sulci , suggesting the infarct is in the subacute phase.](images/app.statdx.com_image_thumbnail_0d5bf999-2754-4e3d-b1dc-9c28b7780388_annotated_true_size_900_quality_90_fd2a20e088d1531d0c96b828e7de14d34cde446d.jpg) +*Axial ADC map in the same patient shows modestly ↓ ADC within the affected cortex but resolution of acute gyral swelling, as evidenced by prominent sulci , suggesting the infarct is in the subacute phase.* + +![Axial DWI MR in a 16-year-old boy involved in a motor vehicle collision (MVC) shows multiple small foci of diffusion restriction , consistent with small infarcts. Multiple infarcts should raise concern for dissection, especially when confined to a single arterial territory.](images/app.statdx.com_image_thumbnail_55cc705f-7ee3-4248-9fa0-637d8e50be2c_annotated_true_size_900_quality_90_752c914826242fde18ffa25d88b41594420361e3.jpg) +*Axial DWI MR in a 16-year-old boy involved in a motor vehicle collision (MVC) shows multiple small foci of diffusion restriction , consistent with small infarcts. Multiple infarcts should raise concern for dissection, especially when confined to a single arterial territory.* + +![Axial CTA in the same patient shows vessel wall irregularity & an intimal flap in the left internal carotid artery (ICA) . The right ICA is small & showed areas of irregularity on other images (not shown). The findings are consistent with bilateral ICA dissections.](1962d738-4661-4faf-96cc-b9e443de25e3) +*Axial CTA in the same patient shows vessel wall irregularity & an intimal flap in the left internal carotid artery (ICA) . The right ICA is small & showed areas of irregularity on other images (not shown). The findings are consistent with bilateral ICA dissections.* + + +### Additional Images + +![Axial T1 C+ MR in a 2-year-old girl shows cortical enhancement in the region of a right frontoparietal infarct, suggesting that it is at least a week old.](d22c1eeb-7f8f-4dd9-aa03-6792c2a5e9b7) +*Axial T1 C+ MR in a 2-year-old girl shows cortical enhancement in the region of a right frontoparietal infarct, suggesting that it is at least a week old.* + +![Axial TOF MRA in a 2 year old with multiple infarcts of various ages shows multiple tiny foci of flow-related signal in the bilateral thalami . This appearance is consistent with lenticulostriate collaterals of moyamoya-type vasculopathy in the setting of bilateral carotid terminus occlusions.](6d9b09d2-974a-4376-a81b-d8193cbb10c3) +*Axial TOF MRA in a 2 year old with multiple infarcts of various ages shows multiple tiny foci of flow-related signal in the bilateral thalami . This appearance is consistent with lenticulostriate collaterals of moyamoya-type vasculopathy in the setting of bilateral carotid terminus occlusions.* + +![Axial T2 MR in a high school football player who developed vomiting, confusion, & vertigo during a game shows gyral swelling & hyperintense signal in the medial temporal lobe , which is in the vascular territory of the left posterior cerebral artery. Intracranial MRA acquired at the same time showed a small embolus in the left posterior cerebral artery (PCA).](0ddbae8c-f3b4-44cb-b5a2-a9b92abaccca) +*Axial T2 MR in a high school football player who developed vomiting, confusion, & vertigo during a game shows gyral swelling & hyperintense signal in the medial temporal lobe , which is in the vascular territory of the left posterior cerebral artery. Intracranial MRA acquired at the same time showed a small embolus in the left posterior cerebral artery (PCA).* + +![Axial CTA of the cervical arteries in the same patient shows a subtle linear filling defect consistent with an intimal flap in the left vertebral artery.](771449b0-686a-476f-984f-1b8dce30cdce) +*Axial CTA of the cervical arteries in the same patient shows a subtle linear filling defect consistent with an intimal flap in the left vertebral artery.* + +![Axial NECT in a 2 day old with congenital heart disease & seizures shows a well-defined, wedge-shaped region of decreased attenuation corresponding to the left MCA vascular territory, consistent with an acute/subacute arterial ischemic stroke.](07441420-2a03-4499-913b-34da9ec2b9e8) +*Axial NECT in a 2 day old with congenital heart disease & seizures shows a well-defined, wedge-shaped region of decreased attenuation corresponding to the left MCA vascular territory, consistent with an acute/subacute arterial ischemic stroke.* + +![Axial T1 C+ MR in an 8 year old with a history of neurofibromatosis type 1 & known bilateral carotid terminus occlusions (resulting in a moyamoya-type vasculopathy pattern) shows abnormal sulcal enhancement (climbing ivy sign) due to arterial collaterals distal to a proximal occlusion.](832f83d1-ae22-4b27-b7f8-b92c1f50f972) +*Axial T1 C+ MR in an 8 year old with a history of neurofibromatosis type 1 & known bilateral carotid terminus occlusions (resulting in a moyamoya-type vasculopathy pattern) shows abnormal sulcal enhancement (climbing ivy sign) due to arterial collaterals distal to a proximal occlusion.* + +![Note the segment of the insular cortical ribbon that is no longer visible on this axial NECT in a 9 year old with acute right hemiparesis. This subtle finding may be the 1st indicator of an acute stroke.](ff842701-0806-4e13-bb38-c3fc39682757) +*Note the segment of the insular cortical ribbon that is no longer visible on this axial NECT in a 9 year old with acute right hemiparesis. This subtle finding may be the 1st indicator of an acute stroke.* + +![Axial T1 C+ MR shows the typical climbing ivy pattern of arterial collateral enhancement in distal territories caused by proximal occlusion from a moyamoya-type vasculopathy. Note the white matter infarct on left .](d10575ab-01fe-4337-a256-6a35c20123cf) +*Axial T1 C+ MR shows the typical climbing ivy pattern of arterial collateral enhancement in distal territories caused by proximal occlusion from a moyamoya-type vasculopathy. Note the white matter infarct on left .* + +![Axial DWI MR in the same child shows an acute infarct on the right with T2 shine-through in an old left-sided stroke .](4396dd36-1b45-4d0c-beb3-44feaee6b2a2) +*Axial DWI MR in the same child shows an acute infarct on the right with T2 shine-through in an old left-sided stroke .* + +![Coronal T2 MR shows multiple areas of infarction resulting from left hemisphere herniation. Secondary infarction from herniation can cause more morbidity than the initial insult.](0245d650-3c22-439f-a116-3b74f3cfb550) +*Coronal T2 MR shows multiple areas of infarction resulting from left hemisphere herniation. Secondary infarction from herniation can cause more morbidity than the initial insult.* + +![Axial DWI MR shows a characteristic "watershed" distribution of infarction in the right cerebral hemisphere. This infarct was the result of a carotid terminus stenosis that developed from bacterial meningitis & vasculitis.](8352777c-1977-46a7-9134-16af53932c25) +*Axial DWI MR shows a characteristic "watershed" distribution of infarction in the right cerebral hemisphere. This infarct was the result of a carotid terminus stenosis that developed from bacterial meningitis & vasculitis.* + +![Axial NECT in a 14-year-old boy with acute right hemiparesis shows a hyperdense MCA sign , indicating acute thrombus in a proximal middle cerebral artery branch.](7da40604-c377-4162-9dd2-d3a552566f54) +*Axial NECT in a 14-year-old boy with acute right hemiparesis shows a hyperdense MCA sign , indicating acute thrombus in a proximal middle cerebral artery branch.* + +![Coronal FLAIR MR in the same patient shows edema in the insular cortex & frontal operculum supplied by the affected MCA branch . The patient had complete recovery without direct treatment, & no etiology was found.](f86dc40c-a8f4-4f0d-b6db-c8817a1b8449) +*Coronal FLAIR MR in the same patient shows edema in the insular cortex & frontal operculum supplied by the affected MCA branch . The patient had complete recovery without direct treatment, & no etiology was found.* + +![Axial FLAIR MR in a 13-year-old girl with seizures after using ephedra shows foci of increased cortical & subcortical white matter signal in the right PCA & left superior cerebellar artery distributions .](f3ca5ae5-9b94-4eb6-a038-c377a432b072) +*Axial FLAIR MR in a 13-year-old girl with seizures after using ephedra shows foci of increased cortical & subcortical white matter signal in the right PCA & left superior cerebellar artery distributions .* + +![Sagittal oblique volume-rendered MRA in the same child shows multiple foci of arterial narrowing & dilation due to a primary arteritis of the CNS.](166b04bc-2c8a-41b4-8d46-c2a3fe73446d) +*Sagittal oblique volume-rendered MRA in the same child shows multiple foci of arterial narrowing & dilation due to a primary arteritis of the CNS.* + +![Axial CECT shows a subtle linear filling defect in the left ICA of a child presenting with a left hemisphere infarct after mandibular surgery. The defect represents an arterial dissection.](27faf219-b885-4c52-9ed2-79a4471ff833) +*Axial CECT shows a subtle linear filling defect in the left ICA of a child presenting with a left hemisphere infarct after mandibular surgery. The defect represents an arterial dissection.* + +![Axial T2 MR shows predominately cortical/subcortical swelling & abnormal signal of the left parietal lobe, typical of a subacute left MCA territory infarct. ~ 1/3 of childhood strokes will not have an underlying etiology diagnosed.](28cc3c6b-3074-4f61-b6a6-d4e13cfc9598) +*Axial T2 MR shows predominately cortical/subcortical swelling & abnormal signal of the left parietal lobe, typical of a subacute left MCA territory infarct. ~ 1/3 of childhood strokes will not have an underlying etiology diagnosed.* + +![Axial DWI MR in a 17-year-old girl shows a geographic area of diffusion restriction in the right insular region, consistent with an infarct. Work-up revealed a hypercoagulable state (antiphospholipid antibody).](7495a5bb-db7b-451c-bcce-ba7e9f96d22b) +*Axial DWI MR in a 17-year-old girl shows a geographic area of diffusion restriction in the right insular region, consistent with an infarct. Work-up revealed a hypercoagulable state (antiphospholipid antibody).* + +![Axial ADC map in a 17-year-old girl shows a geographic area of diffusion restriction in the right insular region, consistent with an infarct. Work-up revealed a hypercoagulable state (antiphospholipid antibody).](bfb8c416-c61c-4e67-9877-c0c9477f6af8) +*Axial ADC map in a 17-year-old girl shows a geographic area of diffusion restriction in the right insular region, consistent with an infarct. Work-up revealed a hypercoagulable state (antiphospholipid antibody).* + +![Axial FLAIR MR in a 2-year-old girl shows multiple areas of cytotoxic edema in both cerebral hemispheres in this patient with moyamoya-type vasculopathy.](82e9effd-55c6-438b-8dfd-5f3df8400952) +*Axial FLAIR MR in a 2-year-old girl shows multiple areas of cytotoxic edema in both cerebral hemispheres in this patient with moyamoya-type vasculopathy.* + +![Axial T2WI MR shows a small, periventricular infarct in a 6 month old. MRA revealed left carotid aneurysm. Proximal arterial pathology should always be investigated at presentation.](3c5ed391-8986-45ff-838f-681163ba29d9) +*Axial T2WI MR shows a small, periventricular infarct in a 6 month old. MRA revealed left carotid aneurysm. Proximal arterial pathology should always be investigated at presentation.* + diff --git a/docs_md/articles/childhood-stroke_ac8a5544-dee5-4712-ad19-7c649e8af035.md b/docs_md/articles/childhood-stroke_ac8a5544-dee5-4712-ad19-7c649e8af035.md new file mode 100644 index 0000000..6d33eaf --- /dev/null +++ b/docs_md/articles/childhood-stroke_ac8a5544-dee5-4712-ad19-7c649e8af035.md @@ -0,0 +1,481 @@ +--- +title: "Childhood Stroke" +docid: "ac8a5544-dee5-4712-ad19-7c649e8af035" +authors: + - key: "47381de4-c9fd-4999-8dd0-1808cd72db6b" + value: "Luke L. Linscott, 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: "Stroke" + slug: "stroke" + treeNodeId: "83689efc-5f25-40a3-9ae7-06fb2a4a069f" + - + name: "Childhood Stroke" + slug: "childhood-stroke" + treeNodeId: null +category: "Pediatrics" +cmeTopicId: "6ffd1c9a-a481-4419-87b0-75324caf579a" +documentVersionId: "67f2456c-3756-4d1d-acbb-eb2e485fb755" +imageCount: 35 +lastUpdated: "02/14/24" +pageDescription: "Childhood Stroke" +pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Stroke, Childhood Stroke" +pageTitle: "Childhood Stroke | STATdx" +enhancedTitle: "Childhood Stroke" +type: "DX" +references: true +breadcrumbs: + - "Pediatrics" + - "Diagnosis" + - "Pediatric Neuroradiology" + - "Brain" + - "Pathology-Based Diagnoses" + - "Stroke" + - "Childhood Stroke" +--- +# KEY FACTS + +- ## Terminology + + + - Acute neurologic dysfunction due to loss of vascular integrity +- ## Imaging + + + - NECT: ↓ attenuation of affected gray matter + - Insular ribbon sign → loss of distinct insular cortex + - Hyperdense MCA sign → thrombosed MCA + - MR: ↓ diffusion within ~ 30 minutes of arterial occlusion + - Cytotoxic edema evident in affected territory on FLAIR/T2 by 4-6 hours after arterial occlusion + - Enhancement of infarct typically occurs after 5-7 days + - CTA/MRA: Critical for early evaluation & identification of possible etiology (e.g., dissection, arteriopathy) + - CTA 1st line for rapid identification of large vessel occlusion amenable to catheter-directed thrombectomy + - MR perfusion imaging can provide valuable information regarding region at risk in setting of acute stroke + - Arterial spin labeling (ASL) can provide useful perfusion information without contrast administration +- ## Top Differential Diagnoses + + + - Seizure-related injury + - Acute encephalitis + - Mitochondrial encephalopathies + - Posterior reversible encephalopathy syndrome (PRES) +- ## Pathology + + + - Major causes: Cardiac disease (~ 25%), moyamoya, dissection, vasculitis, RCVS, hematologic + - No underlying cause discovered in ~ 25% of cases +- ## Clinical Issues + + + - Incidence: 2-3/100,000 per year in USA + - Mortality: 0.6/100,000 + - Children typically present later than adults (> 24 hours) + - Focal deficit may be masked by lethargy, coma, irritability + - Catheter-based thrombectomy increasingly used in children + - Capacity for recovery in children much > adults +- ## Diagnostic Checklist + + + - When stroke is suspected clinically or by imaging, do not hesitate to perform vessel imaging + +# TERMINOLOGY + +- ## Synonyms + + + - Cerebrovascular accident, cerebral infarct, cerebral ischemia +- ## Definitions + + + - Acute alteration of neurologic function due to loss of vascular integrity + - This document specifically addresses arterial ischemia beyond perinatal period + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Cytotoxic edema & restricted diffusion (acutely) in affected vascular territory + - ### Location + + + - Proximal & distal middle cerebral artery (MCA) territory most commonly affected + - ### Morphology + + + - Stroke caused by arterial occlusion typically conforms to 1 arterial territory +- ## CT Findings + + + - ### NECT + + + - ↓ attenuation of affected gray matter with loss of normal gray matter-white matter differentiation + - ↓ in white matter attenuation less pronounced + - Often wedge-shaped & localized to 1 arterial territory + - Diffuse ischemic injury can lead to reversal sign with gray matter diffusely ↓ in attenuation relative to white matter + - Insular ribbon sign → loss of distinct of insular cortex + - Hyperdense MCA sign → ↑ density of acutely thrombosed MCA + - Hemorrhagic transformation (HT) + - Symptomatic HT in 3%; asymptomatic HT in 30% + - Asymptomatic HT usually parenchymal + - White matter or deep nuclear hemorrhage often mass-like → hematoma within infarcted tissue + - ### CECT + + + - Enhancement of infarcted territory typically occurs after 5-7 days + - ### CTA + + + - Invaluable for demonstrating focal vascular abnormalities in acute setting + - CTA 1st line for rapid identification of large vessel occlusion amenable to catheter-directed thrombectomy + - Intimal flap in acutely dissected vessel +- ## MR Findings + + + - **T1WI**:**** Acute: ↓ signal with gyral swelling + - Chronic: ± ↑ signal in cortical laminar necrosis + - **T1WI FS**: Allows identification of mural hematoma (↑ signal) in dissected vessel + - **T2WI**: Loss of flow void in thrombosed vessel + - **FLAIR**: ↑ signal with gyral swelling (within ~4-6 hours) + - Abnormal sulcal ↑ signal (climbing ivy sign) of chronic, slow-flow collaterals in setting of longstanding proximal vascular occlusion + - **DWI**: Most sensitive for early detection of ischemia + - Acute: Restricted diffusion (↑ DWI, ↓ ADC signal) ≤ 30 minutes after ischemic insult + - Subacute (7-14 days): Pseudonormalization of signal + - ↑ DWI, ADC ~ brain parenchyma + - Chronic: Facilitated diffusion in gliotic brain + - ↑/~ DWI, ↑ ADC + - **SWI/T2* GRE**: May see ↑ size & number of cortical vessels**** + - Suggests ↑ extraction fraction & possibly recoverable brain + - **T1WI C+**: Cortical & leptomeningeal enhancement seen after ~5-7 days following acute infarct + - Enhancing climbing ivy sign + - **MRA**: Can detect arterial occlusion & stenosis in large- & medium-sized cerebral vessels + - Important to identify underlying dissection or arteriopathy + - **PWI**: Provides valuable information about affected brain + - Ischemic penumbra: ↓ perfusion, no DWI change (PWI-DWI mismatch) + - May define brain salvageable with acute stroke therapy + - Arterial spin labeling can provide useful perfusion information without contrast administration + - **MRS**: ↑ lactate hallmark of ischemia/infarct + - Not specific + - **Vessel wall imaging**: Vessel wall enhancement patterns improve discrimination of underlying stroke etiology +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Affected territory hyperechoic in acute/subacute stage + - ### Color Doppler + + + - Direct Doppler evaluation ideal for surveillance of vascular occlusion in neonate with open sutures + - Transcranial Doppler evaluation of circle of Willis through temporal squamosa + - ↑ velocities can predict stenoses detectable by MRA + - Used as screening tool in children with sickle cell anemia +- ## Angiographic Findings + + + - Catheter angiography rarely necessary in acute evaluation of childhood stroke + - Justified if contemplating endovascular therapy + - Best modality for detailed evaluation of primary arteriopathies +- ## Nuclear Medicine Findings + + + - PET & SPECT techniques can be used to + - Identify salvageable regions at risk (ischemic penumbra) + - Demonstrate effects of synangiosis surgery in moyamoya-type vasculopathies +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - CT initial imaging test for signs/symptoms of stroke; excellent for excluding hemorrhagic stroke (more common in children vs. adults) + - MR with DWI, MRA, PWI + - ### Protocol advice + + + - Contrast can help in assessing timing of injury & performing perfusion imaging + - Dedicated vessel wall MR imaging to define underlying etiology [e.g. focal cerebral arteriopathy (FCA)] + +# DIFFERENTIAL DIAGNOSIS + +- ## Seizure-Related Injury + + + - Swelling & restricted diffusion secondary to persistent seizure activity + - Differentiation by clinical presentation & EEG +- [Acute Encephalitis](/document/acute-encephalitis/a45f63bb-c25b-481d-a001-9c520c58060b) + - Acute parenchymal inflammation secondary to infectious agents, typically viral + - Slower onset with encephalopathy +- [Mitochondrial Encephalopathies](/document/mitochondrial-encephalopathies/40004435-b768-4baf-a31e-651f8a174fe2) + - Symmetric basal ganglia involvement common + - Usually have manifestations beyond CNS +- [Posterior Reversible Encephalopathy Syndrome (PRES)](/document/acute-hypertensive-encephalopathy--/efc6f9c2-dad9-4eb8-bad2-421bfaf1ec57) + - Patchy cortical/subcortical edema most common in parietal & occipital lobes, typically in setting of hypertension + - Diffusion restriction uncommon +- [Neonatal Herpes Encephalitis](/document/herpes-encephalitis-type-1/556f5f76-c20b-44ca-a913-c53b11c93341) + - Infant with seizures 2-5 weeks after birth + - DWI most sensitive for detection in early disease + - Often bilateral with temporal predominance but can occur anywhere +- ## MELAS + + + - **M**itochondrial **e**ncephomyopathy, **l**actic acidosis, **s**troke-like episodes + - Areas of ischemia crossing arterial territories, often parietal + - MRS: ↑ lactate in normal-appearing brain +- [Group B Strep Meningitis](/document/group-b-streptococcal-meningitis/bafa10c7-e65b-4432-9959-b8e5e4af708c) + - Associated vasculitis causes ischemia in small perforating arteries + - Unilateral or bilateral deep gray nuclei ischemia + +# PATHOLOGY + +- ## General Features + + + - 6 major causes of arterial stroke in children + - Cardiac disease (~ 25%) + - Congenital heart disease, valvular heart disease, arrhythmias, & cardiomyopathies + - Moyamoya-type arteriopathy + - Sickle cell disease + - Neurofibromatosis type 1 + - Radiation therapy + - Trisomy 21 + - Alagille syndrome + - Arterial dissection (e.g., trauma) + - FCA of childhood + - Reversible cerebral vasoconstriction syndrome (RCVS) + - Hematologic/metabolic (e.g., coagulopathy) + - Idiopathic (~ 25%) + - No underlying cause discovered + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Depends on patient age, etiology, & involved artery + - < 1 year: Seizures, encephalopathy > focal neurologic + - > 1 year: Usually focal neurologic (e.g., hemiplegia, early hand preference) + - Speech difficulties, gait abnormality, seizure + - Seizure → deficit often attributed to postictal state (Jacksonian paralysis) + - Embolic cause: Sudden onset of symptoms + - Stenoocclusive cause: Gradual/intermittent (e.g., TIA) + - Focal deficit may be masked by lethargy, coma, irritability + - Children typically present later than adults (> 24 hours) + - Poor recognition/understanding of symptoms by child, caregiver, physician + - Uncommon diagnosis in children, requires high degree of suspicion +- ## Demographics + + + - ### Age + + + - Incidence/mortality greatest < 1 year + - Large percentage occur in perinatal period + - Perinatal arterial ischemic stroke (PAIS) + - ### Epidemiology + + + - Incidence: 2-3/100,000 per year in USA + - Mortality: 0.6/100,000 + - Underrecognized as significant source of morbidity in pediatric population +- ## Natural History & Prognosis + + + - Capacity for recovery better than in adults, due to + - Better compensatory mechanisms, collateral recruitment, neuronal plasticity + - Fewer concomitant risk factors +- ## Treatment + + + - Clinical window of opportunity/benefit not as well understood in children as compared to adults + - Mechanical thrombectomy frequently employed for acute large vessel occlusion + - Mainstay of chronic therapy for fixed vascular lesions & vasculopathies: Aspirin + - Transfusion therapy for at-risk children with sickle cell + - Dissection: Anticoagulation, vessel occlusion, or stenting + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - Use same imaging signs as adults + - Have low threshold for use of CTA + - Dedicated vessel wall MR imaging often helpful to identify underlying etiology + + 196ff412-dec6-4912-8197-f6e80f84bc65 + +## References + +# Selected References + +1. [Jiang B et al: Neuroimaging in pediatric stroke. Semin Pediatr Neurol. 43:100989, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36344022%5Bpmid%5D) +1. [Chabrier S et al: Hyperacute recanalization strategies and childhood stroke in the evidence age. Stroke. 52(1):381-4, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33349018%5Bpmid%5D) +1. [Oesch G et al: Focal cerebral arteriopathy of childhood: clinical and imaging correlates. Stroke. 52(7):2258-65, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34039030%5Bpmid%5D) +1. [Visser MJ et al: Automated perfusion-diffusion magnetic resonance imaging in childhood arterial ischemic stroke. Stroke. 52(10):3296-304, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34404238%5Bpmid%5D) +1. [Fearn ND et al: Focal cerebral arteriopathy and childhood stroke. Curr Opin Neurol. 33(1):37-46, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31815778%5Bpmid%5D) +1. [Donahue MJ et al: Neuroimaging advances in pediatric stroke. Stroke. 50(2):240-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30661496%5Bpmid%5D) +1. [Dlamini N et al: Arterial wall imaging in pediatric stroke. Stroke. 49(4):891-8, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29581340%5Bpmid%5D) +1. [Khalaf A et al: Pediatric stroke imaging. Pediatr Neurol. 86:5-18, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30122281%5Bpmid%5D) +1. [Beslow LA: Stroke Diagnosis in the pediatric emergency department: an ongoing challenge. Stroke. 48(5):1132-33, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28336680%5Bpmid%5D) +1. [Satti S et al: Mechanical thrombectomy for pediatric acute ischemic stroke: review of the literature. J Neurointerv Surg. 9(8):732-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27448827%5Bpmid%5D) +1. [Wilson JL et al: Endovascular therapy in pediatric stroke: utilization, patient characteristics, and outcomes. Pediatr Neurol. 69:87-92.e2, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28233666%5Bpmid%5D) +1. [Madaelil TP et al: Mechanical thrombectomy in pediatric acute ischemic stroke: clinical outcomes and literature review. Interv Neuroradiol. 22(4):426-31, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26945589%5Bpmid%5D) +1. [Polan RM et al: Susceptibility-weighted imaging in pediatric arterial ischemic stroke: a valuable alternative for the noninvasive evaluation of altered cerebral hemodynamics. AJNR Am J Neuroradiol. 36(4):783-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25477354%5Bpmid%5D) +1. [Bernard TJ et al: Emergence of the primary pediatric stroke center: impact of the thrombolysis in pediatric stroke trial. Stroke. 45(7):2018-23, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24916908%5Bpmid%5D) +1. [Gemmete JJ et al: Arterial ischemic stroke in children. Neuroimaging Clin N Am. 23(4):781-98, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24156865%5Bpmid%5D) +1. [Freundlich CL et al: Pediatric stroke. Emerg Med Clin North Am. 30(3):805-28, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22974650%5Bpmid%5D) +1. [Kitchen L et al: The pediatric stroke outcome measure: a validation and reliability study. Stroke. 43(6):1602-8, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22474056%5Bpmid%5D) +1. [Beslow LA et al: Hemorrhagic transformation of childhood arterial ischemic stroke. Stroke. 42(4):941-6, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21350202%5Bpmid%5D) +1. [Cárdenas JF et al: Pediatric stroke. Childs Nerv Syst. 27(9):1375-90, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21336993%5Bpmid%5D) +1. [Dowling MM et al: Intracardiac shunting and stroke in children: a systematic review. J Child Neurol. 26(1):72-82, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21212453%5Bpmid%5D) +1. [Lanni G et al: Pediatric stroke: clinical findings and radiological approach. Stroke Res Treat. 2011:172168, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21603166%5Bpmid%5D) +1. [Larrue V et al: Etiologic investigation of ischemic stroke in young adults. Neurology. 76(23):1983-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21646623%5Bpmid%5D) +1. [Munot P et al: Characteristics of childhood arterial ischemic stroke with normal MR angiography. Stroke. 42(2):504-6, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21193747%5Bpmid%5D) +1. [Sedney CL et al: Cervical abnormalities causing vertebral artery dissection in children. J Neurosurg Pediatr. 7(3):272-5, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21361766%5Bpmid%5D) +1. [Shellhaas RA et al: Mimics of childhood stroke: characteristics of a prospective cohort. Pediatrics. 118(2):704-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16882826%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial CTA MIP in a 14-year-old with right-sided weakness and history of congenital heart disease shows abrupt cutoff of the M1 segment of the left middle cerebral artery (MCA). MIP imaging is particularly helpful to identify vessel occlusion in stroke. This patient was treated with catheter-directed thrombectomy.](images/app.statdx.com_image_thumbnail_e9efd3af-1f86-490f-a63c-6cd6681f5e8a_annotated_true_size_900_quality_90_e8cb6bc5cda044278e00db0f2f498638f78fb9e9.jpg) +*Axial CTA MIP in a 14-year-old with right-sided weakness and history of congenital heart disease shows abrupt cutoff of the M1 segment of the left middle cerebral artery (MCA). MIP imaging is particularly helpful to identify vessel occlusion in stroke. This patient was treated with catheter-directed thrombectomy.* + +![Axial CTA MIP in a 14-year-old with right-sided weakness and history of congenital heart disease shows abrupt cutoff of the M1 segment of the left middle cerebral artery (MCA). MIP imaging is particularly helpful to identify vessel occlusion in stroke. This patient was treated with catheter-directed thrombectomy.](images/app.statdx.com_image_thumbnail_e9efd3af-1f86-490f-a63c-6cd6681f5e8a_size_174_quality_85_e7f6c71df145b80959f5dbb59a48980569188c20.jpg) +*Axial CTA MIP in a 14-year-old with right-sided weakness and history of congenital heart disease shows abrupt cutoff of the M1 segment of the left middle cerebral artery (MCA). MIP imaging is particularly helpful to identify vessel occlusion in stroke. This patient was treated with catheter-directed thrombectomy.* + +![Axial DWI in the same patient after thrombectomy shows restricted diffusion in the left basal ganglia, consistent with acute infarction. Note preservation of the remainder of the left MCA territory.](images/app.statdx.com_image_thumbnail_e0761bd2-c3ae-4b2d-99ce-e79a406d31a2_annotated_true_size_900_quality_90_298500ddc8141460ee5ff288842fbf14c07b03f9.jpg) +*Axial DWI in the same patient after thrombectomy shows restricted diffusion in the left basal ganglia, consistent with acute infarction. Note preservation of the remainder of the left MCA territory.* + +![Axial time-of-flight MRA in a 2-year-old with multiple infarcts of various ages shows multiple small areas of flow-related signal in the bilateral thalami, consistent with lenticulostriate collaterals of moyamoya.](images/app.statdx.com_image_thumbnail_56212f29-7784-416c-b877-cc53a522328c_annotated_true_size_900_quality_90_b414a8015c0d7b2d899d4374045a394beff70d21.jpg) +*Axial time-of-flight MRA in a 2-year-old with multiple infarcts of various ages shows multiple small areas of flow-related signal in the bilateral thalami, consistent with lenticulostriate collaterals of moyamoya.* + +![Axial DWI MR in the same patient with moyamoya-type vasculopathy shows diffusion restriction in the right frontoparietal foci of signal abnormality , suggesting an acute/subacute infarct. However, there is no diffusion restriction in the left parietal region , suggesting this infarct is of an older age.](images/app.statdx.com_image_thumbnail_17c090a1-c06e-4cec-9184-a9bfa5f382e4_annotated_true_size_900_quality_90_e5489f517d44643e909fbe6abbb4af0411ff37d0.jpg) +*Axial DWI MR in the same patient with moyamoya-type vasculopathy shows diffusion restriction in the right frontoparietal foci of signal abnormality , suggesting an acute/subacute infarct. However, there is no diffusion restriction in the left parietal region , suggesting this infarct is of an older age.* + +![Axial DWI MR in a 6-year-old with imbalance and acute infarct of the left basal ganglia shows diffusion restriction (↓ ADC not shown). Acute infarct in a child should prompt further evaluation with MRA or CTA to detect an underlying vessel abnormality.](1da69a2b-e771-44e7-b56f-c7f177ba9b63) +*Axial DWI MR in a 6-year-old with imbalance and acute infarct of the left basal ganglia shows diffusion restriction (↓ ADC not shown). Acute infarct in a child should prompt further evaluation with MRA or CTA to detect an underlying vessel abnormality.* + +![3D MRA of the circle of Willis in the same patient shows irregular narrowing of the left proximal and distal segments of the MCA, consistent with vasculitis.](images/app.statdx.com_image_thumbnail_052a36b9-d6de-499f-9fc2-9279bcaa289e_annotated_true_size_900_quality_90_3d26ce2477648b3364b1fbb25c281cb96b98965f.jpg) +*3D MRA of the circle of Willis in the same patient shows irregular narrowing of the left proximal and distal segments of the MCA, consistent with vasculitis.* + +![Axial DWI MR in a 12-year-old with reversible cerebral vasoconstriction syndrome (RCVS) who recently started mycophenolate and presented with acute onset of left-sided weakness shows multifocal cortical and subcortical infarcts.](images/app.statdx.com_image_thumbnail_ddfa195d-fa7f-49d6-aa17-0187321f5e46_annotated_true_size_900_quality_90_5b7e1cfd75ee7272a8460503df679782a6afb1e6.jpg) +*Axial DWI MR in a 12-year-old with reversible cerebral vasoconstriction syndrome (RCVS) who recently started mycophenolate and presented with acute onset of left-sided weakness shows multifocal cortical and subcortical infarcts.* + +![Lateral projection DSA from an internal carotid artery (ICA) injection in the same patient shows multifocal areas of medium vessel narrowing and irregularity , a common feature of RCVS. The spectrum of underlying etiologies for childhood stroke is diverse.](images/app.statdx.com_image_thumbnail_d3ca62ee-f3f8-4f6b-b235-c29499d8e3a7_annotated_true_size_900_quality_90_468fbedb411350574a2857bc02af77f9fea98bd7.jpg) +*Lateral projection DSA from an internal carotid artery (ICA) injection in the same patient shows multifocal areas of medium vessel narrowing and irregularity , a common feature of RCVS. The spectrum of underlying etiologies for childhood stroke is diverse.* + +![Axial DWI MR in a 16-year-old boy involved in a motor vehicle accident shows multiple small foci of diffusion restriction , consistent with small infarcts. Multiple infarcts should raise concern for dissection, especially when confined to a single arterial territory.](e26fb106-2d34-46cc-b517-cf194e39ff30) +*Axial DWI MR in a 16-year-old boy involved in a motor vehicle accident shows multiple small foci of diffusion restriction , consistent with small infarcts. Multiple infarcts should raise concern for dissection, especially when confined to a single arterial territory.* + +![Axial CTA in the same patient shows vessel wall irregularity and an intimal flap in the left ICA . The right ICA is small and revealed areas of irregularity on other images (not shown). These findings are consistent with bilateral ICA dissections.](49acc471-47dc-48cd-bf62-0a0a3acd45e3) +*Axial CTA in the same patient shows vessel wall irregularity and an intimal flap in the left ICA . The right ICA is small and revealed areas of irregularity on other images (not shown). These findings are consistent with bilateral ICA dissections.* + + +### Additional Images + +![Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right MCA territory . Note the sulcal effacement and loss of gray matter-white matter differentiation.](7157c69d-478c-4757-b778-1f9f9ddb82fd) +*Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right MCA territory . Note the sulcal effacement and loss of gray matter-white matter differentiation.* + +![Axial DWI MR in the same patient confirms restricted diffusion in the right MCA territory . Also note the focus of restricted diffusion in the left periventricular region . Multiple infarcts in multiple vascular territories should raise suspicion of a proximal embolic source.](954bb253-7f64-420c-8641-8e6f77ef4b05) +*Axial DWI MR in the same patient confirms restricted diffusion in the right MCA territory . Also note the focus of restricted diffusion in the left periventricular region . Multiple infarcts in multiple vascular territories should raise suspicion of a proximal embolic source.* + +![Axial T1 C+ MR in a 1-year-old with arteriopathy and subacute infarction shows gyriform enhancement of the cortical ribbon. Enhancement is common in the subacute phase of infarction. Precontrast T1 is necessary to distinguish true enhancement from the intrinsic ↑ T1 seen in cortical laminar necrosis.](a35cb5c1-02fe-4b3b-a6a0-90c7d2c394ff) +*Axial T1 C+ MR in a 1-year-old with arteriopathy and subacute infarction shows gyriform enhancement of the cortical ribbon. Enhancement is common in the subacute phase of infarction. Precontrast T1 is necessary to distinguish true enhancement from the intrinsic ↑ T1 seen in cortical laminar necrosis.* + +![Axial ADC map in the same patient shows modestly ↓ ADC within the affected cortex but resolution of acute gyral swelling, as evidenced by prominent sulci , suggesting the infarct is in the subacute phase.](2866704c-8405-4701-9e0b-01260a22076d) +*Axial ADC map in the same patient shows modestly ↓ ADC within the affected cortex but resolution of acute gyral swelling, as evidenced by prominent sulci , suggesting the infarct is in the subacute phase.* + +![Axial T1 C+ MR in a 2-year-old girl shows cortical enhancement in the region of a right frontoparietal infarct, suggesting that it is at least a week old.](8c7df14b-52a8-4902-b362-04537e9c7fda) +*Axial T1 C+ MR in a 2-year-old girl shows cortical enhancement in the region of a right frontoparietal infarct, suggesting that it is at least a week old.* + +![Axial time-of-flight MRA in a 2-year old with multiple infarcts of various ages shows multiple tiny foci of flow-related signal in the bilateral thalami . This appearance is consistent with lenticulostriate collaterals of moyamoya-type vasculopathy in the setting of bilateral carotid terminus occlusions.](cd3cbe16-7624-4875-94f7-fd767a99905f) +*Axial time-of-flight MRA in a 2-year old with multiple infarcts of various ages shows multiple tiny foci of flow-related signal in the bilateral thalami . This appearance is consistent with lenticulostriate collaterals of moyamoya-type vasculopathy in the setting of bilateral carotid terminus occlusions.* + +![Axial T2 MR in a high school football player who developed vomiting, confusion, and vertigo during a game shows gyral swelling and hyperintense signal in the medial temporal lobe , which is in the vascular territory of the left posterior cerebral artery (PCA). Intracranial MRA acquired at the same time showed a small embolus in the left PCA.](d5fe886d-4f20-43f5-ad53-2cebd11b44fd) +*Axial T2 MR in a high school football player who developed vomiting, confusion, and vertigo during a game shows gyral swelling and hyperintense signal in the medial temporal lobe , which is in the vascular territory of the left posterior cerebral artery (PCA). Intracranial MRA acquired at the same time showed a small embolus in the left PCA.* + +![Axial CTA of the cervical arteries in the same patient shows a subtle linear filling defect consistent with an intimal flap in the left vertebral artery.](dca2f196-8ca3-4528-87e5-76e1701d86c3) +*Axial CTA of the cervical arteries in the same patient shows a subtle linear filling defect consistent with an intimal flap in the left vertebral artery.* + +![Axial NECT in a 2-day-old with congenital heart disease and seizures shows a well-defined, wedge-shaped region of ↓ attenuation corresponding to the left MCA vascular territory, consistent with an acute/subacute arterial ischemic stroke.](d0acd7e9-03f9-434c-b1f1-3e050dd3ed77) +*Axial NECT in a 2-day-old with congenital heart disease and seizures shows a well-defined, wedge-shaped region of ↓ attenuation corresponding to the left MCA vascular territory, consistent with an acute/subacute arterial ischemic stroke.* + +![Axial T1 C+ MR in an 8-year-old with a history of neurofibromatosis type 1 and known bilateral carotid terminus occlusions (resulting in a moyamoya-type vasculopathy pattern) shows abnormal sulcal enhancement (climbing ivy sign) due to arterial collaterals distal to a proximal occlusion.](76d178ba-f2cc-4770-bb53-f354520cb34b) +*Axial T1 C+ MR in an 8-year-old with a history of neurofibromatosis type 1 and known bilateral carotid terminus occlusions (resulting in a moyamoya-type vasculopathy pattern) shows abnormal sulcal enhancement (climbing ivy sign) due to arterial collaterals distal to a proximal occlusion.* + +![Note the segment of the insular cortical ribbon that is no longer visible on this axial NECT in a 9-year-old with acute right hemiparesis. This subtle finding may be the 1st indicator of an acute stroke.](08df8d9b-1433-4b74-8445-fcf52ef44056) +*Note the segment of the insular cortical ribbon that is no longer visible on this axial NECT in a 9-year-old with acute right hemiparesis. This subtle finding may be the 1st indicator of an acute stroke.* + +![Axial T1 C+ MR shows the typical climbing ivy pattern of arterial collateral enhancement in distal territories caused by proximal occlusion from a moyamoya-type vasculopathy. Note the white matter infarct on the left .](b2492277-540b-4428-beb3-9f76a4c22484) +*Axial T1 C+ MR shows the typical climbing ivy pattern of arterial collateral enhancement in distal territories caused by proximal occlusion from a moyamoya-type vasculopathy. Note the white matter infarct on the left .* + +![Axial DWI MR shows an acute infarct on the right with T2 shine-through in an old left-sided stroke .](c2c0cb8e-21d0-41ea-84e9-5e3262ebc692) +*Axial DWI MR shows an acute infarct on the right with T2 shine-through in an old left-sided stroke .* + +![Coronal T2 MR shows multiple areas of infarction resulting from left hemisphere herniation. Secondary infarction from herniation can cause more morbidity than the initial insult.](072e6c90-829c-40dc-bc6c-8dee40c02b81) +*Coronal T2 MR shows multiple areas of infarction resulting from left hemisphere herniation. Secondary infarction from herniation can cause more morbidity than the initial insult.* + +![Axial DWI MR shows a characteristic "watershed" distribution of infarction in the right cerebral hemisphere. This infarct was the result of a carotid terminus stenosis that developed from bacterial meningitis and vasculitis.](2676919e-3056-4763-9ad8-a87533d59211) +*Axial DWI MR shows a characteristic "watershed" distribution of infarction in the right cerebral hemisphere. This infarct was the result of a carotid terminus stenosis that developed from bacterial meningitis and vasculitis.* + +![Axial NECT in a 14-year-old boy with acute right hemiparesis shows a hyperdense MCA sign , indicating acute thrombus in a proximal MCA branch.](01758363-1463-4918-aea9-09fc8de84df8) +*Axial NECT in a 14-year-old boy with acute right hemiparesis shows a hyperdense MCA sign , indicating acute thrombus in a proximal MCA branch.* + +![Coronal FLAIR MR in the same patient shows edema in the insular cortex and frontal operculum supplied by the affected MCA branch . The patient had complete recovery without direct treatment, and no etiology was found.](a48a8dae-8e44-4c6d-8d88-a1f49f95e700) +*Coronal FLAIR MR in the same patient shows edema in the insular cortex and frontal operculum supplied by the affected MCA branch . The patient had complete recovery without direct treatment, and no etiology was found.* + +![Axial FLAIR MR in a 13-year-old girl with seizures after using ephedra shows foci of increased cortical and subcortical white matter signal in the right PCA and left superior cerebellar artery distributions .](a32a42fd-ab2c-461a-a440-5103bff70f2b) +*Axial FLAIR MR in a 13-year-old girl with seizures after using ephedra shows foci of increased cortical and subcortical white matter signal in the right PCA and left superior cerebellar artery distributions .* + +![Sagittal oblique volume-rendered MRA in the same patient shows multiple foci of arterial narrowing and dilation due to a primary arteritis of the CNS.](d1870554-4f7a-42b8-967e-23ace6bcb6ec) +*Sagittal oblique volume-rendered MRA in the same patient shows multiple foci of arterial narrowing and dilation due to a primary arteritis of the CNS.* + +![Axial CECT shows a subtle linear filling defect in the left ICA of a child presenting with a left hemisphere infarct after mandibular surgery. The defect represents an arterial dissection.](cde5f596-f27a-453f-8357-92323b52c1ac) +*Axial CECT shows a subtle linear filling defect in the left ICA of a child presenting with a left hemisphere infarct after mandibular surgery. The defect represents an arterial dissection.* + +![Axial T2 MR shows predominately cortical/subcortical swelling and abnormal signal of the left parietal lobe, typical of a subacute left MCA territory infarct. Approximately 1/3 of childhood strokes will not have an underlying etiology diagnosed.](ff66ddee-3869-468b-ba7d-1e9551be531d) +*Axial T2 MR shows predominately cortical/subcortical swelling and abnormal signal of the left parietal lobe, typical of a subacute left MCA territory infarct. Approximately 1/3 of childhood strokes will not have an underlying etiology diagnosed.* + +![Axial DWI MR in a 17-year-old girl shows a geographic area of diffusion restriction in the right insular region, consistent with an infarct. Work-up revealed a hypercoagulable state (antiphospholipid antibody).](b2a5cd72-ce3e-4337-a72d-6097b5cc63b9) +*Axial DWI MR in a 17-year-old girl shows a geographic area of diffusion restriction in the right insular region, consistent with an infarct. Work-up revealed a hypercoagulable state (antiphospholipid antibody).* + +![Axial ADC map in a 17-year-old girl shows a geographic area of diffusion restriction in the right insular region, consistent with an infarct. Work-up revealed a hypercoagulable state (antiphospholipid antibody).](190a67a7-28d5-4916-86aa-388a178102b6) +*Axial ADC map in a 17-year-old girl shows a geographic area of diffusion restriction in the right insular region, consistent with an infarct. Work-up revealed a hypercoagulable state (antiphospholipid antibody).* + +![Axial FLAIR MR in a 2-year-old girl shows multiple areas of cytotoxic edema in both cerebral hemispheres in this patient with moyamoya-type vasculopathy.](58dd037b-48f2-40cb-bdb1-74657703db1e) +*Axial FLAIR MR in a 2-year-old girl shows multiple areas of cytotoxic edema in both cerebral hemispheres in this patient with moyamoya-type vasculopathy.* + +![Axial T2WI MR shows a small periventricular infarct in a 6-month-old. MRA revealed left carotid aneurysm. Proximal arterial pathology should always be investigated at presentation.](075d672c-777a-4d34-86d6-cf28e000dfa3) +*Axial T2WI MR shows a small periventricular infarct in a 6-month-old. MRA revealed left carotid aneurysm. Proximal arterial pathology should always be investigated at presentation.* + diff --git a/docs_md/articles/childhood-stroke_dc608435-4c6c-4b53-985a-4630cd24d5ce.md b/docs_md/articles/childhood-stroke_dc608435-4c6c-4b53-985a-4630cd24d5ce.md new file mode 100644 index 0000000..3b7d888 --- /dev/null +++ b/docs_md/articles/childhood-stroke_dc608435-4c6c-4b53-985a-4630cd24d5ce.md @@ -0,0 +1,415 @@ +--- +title: "Childhood Stroke" +docid: "dc608435-4c6c-4b53-985a-4630cd24d5ce" +authors: + - key: "47381de4-c9fd-4999-8dd0-1808cd72db6b" + value: "Luke L. Linscott, 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: "Traumatic and Vascular Lesions" + slug: "traumatic-and-vascular-lesions" + treeNodeId: "1b07bd39-2fac-4687-8460-9ea81fa3f9c9" + - + name: "Childhood Stroke" + slug: "childhood-stroke" + treeNodeId: null +category: "Pediatrics" +cmeTopicId: "8bec57b6-3f2c-4787-8f1b-04c07c1848c5" +documentVersionId: "3b5f228c-631c-4212-a6aa-4fdbe7fd5d76" +imageCount: 24 +lastUpdated: "11/01/21" +pageDescription: "Childhood Stroke" +pageKeywords: "Pediatrics, Diagnosis, Brain, Traumatic and Vascular Lesions, Childhood Stroke" +pageTitle: "Childhood Stroke | STATdx" +enhancedTitle: "Childhood Stroke" +type: "DX" +references: true +breadcrumbs: + - "Pediatrics" + - "Diagnosis" + - "Brain" + - "Traumatic and Vascular Lesions" + - "Childhood Stroke" +--- +# KEY FACTS + +- ## Terminology + + + - Acute alteration of neurologic function due to loss of vascular integrity +- ## Imaging + + + - NECT: ↓ attenuation of affected gray matter + - Insular ribbon sign → loss of distinct insular cortex + - Hyperdense middle cerebral artery (MCA) sign → thrombosed MCA + - MR: ↓ diffusion within ~ 30 minutes of arterial occlusion + - Cytotoxic edema is evident in affected territory on FLAIR/T2 by 4-6 hours after arterial occlusion + - Enhancement of infarct typically occurs after 5-7 days + - CTA/MRA: Critical for early evaluation & identification of possible etiology (e.g., dissection, arteriopathy) + - MR perfusion imaging can provide valuable information regarding region at risk in setting of acute stroke + - Arterial spin labeling can provide useful perfusion information without contrast administration + - MR vessel wall imaging is helpful to identify inflammatory arteriopathy +- ## Top Differential Diagnoses + + + - Complex migraine + - Seizure-related injury + - Acute encephalitis + - Mitochondrial encephalopathies + - Posterior reversible encephalopathy syndrome +- ## Pathology + + + - Major causes: Cardiac disease (~ 25%), moyamoya-type arteriopathy, dissection, vasculitis, hematologic/metabolic + - No underlying cause discovered in ~ 25% of cases +- ## Clinical Issues + + + - Incidence: 2-3/100,000 per year in USA + - Mortality: 0.6/100,000 + - Children typically present later than adults (> 24 hours) + - Focal deficit may be masked by lethargy, coma, irritability + - Treatment in pediatric acute stroke is often conservative + - Thrombolysis/thrombectomy not well studied in children + - Capacity for recovery in children much better than adults + +# TERMINOLOGY + +- ## Synonyms + + + - Cerebrovascular accident, cerebral infarct, cerebral ischemia +- ## Definitions + + + - Acute alteration of neurologic function due to loss of vascular integrity + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Cytotoxic edema & restricted diffusion (acutely) in affected vascular territory + - ### Location + + + - Proximal & distal middle cerebral artery (MCA) territories are most commonly affected + - ### Morphology + + + - Stroke caused by arterial occlusion typically conforms to 1 arterial territory +- ## CT Findings + + + - ### NECT + + + - ↓ attenuation of affected gray matter (GM) with loss of normal GM-white matter (WM) differentiation + - ↓ in WM attenuation is less pronounced + - Often wedge-shaped & localized to 1 arterial territory + - Diffuse ischemic injury can lead to reversal sign with GM diffusely ↓ in attenuation relative to WM + - Insular ribbon sign → loss of distinct insular cortex + - Hyperdense middle cerebral artery (MCA) sign → ↑ density of acutely thrombosed MCA + - Hemorrhagic transformation (HT) + - Symptomatic HT in 3%; asymptomatic HT in 30% + - Asymptomatic HT is usually parenchymal + - WM or deep nuclear hemorrhage is often mass-like → hematoma within infarcted tissue + - ### CECT + + + - Enhancement of infarcted territory typically occurs after 5-7 days + - ### CTA + + + - Invaluable for demonstrating focal vascular abnormalities in acute setting + - Intimal flap in acutely dissected vessel + - Major arterial occlusion may prompt thrombolysis or mechanical thrombectomy in appropriate setting +- ## MR Findings + + + - **T1**: Acute: ↓ signal with gyral swelling + - Chronic: ± ↑ signal in cortical laminar necrosis + - **T1 FS**: Allows identification of mural hematoma (↑ signal) in dissected vessel + - **T2**: Loss of flow void in thrombosed vessel + - **FLAIR**: ↑ signal with gyral swelling (within 4-6 hours) + - Abnormal sulcal ↑ signal (climbing ivy sign) of chronic slow flow collaterals in setting of longstanding proximal vascular occlusion + - **DWI**: Most sensitive for early detection of ischemia + - Acute: Restricted diffusion (↑ DWI, ↓ ADC signal) ≤ 30 minutes after ischemic insult + - Subacute (7-14 days): Pseudonormalization of signal + - ↑ DWI, ADC ≈ brain parenchyma + - Chronic: Facilitated diffusion in gliotic brain + - ↑/≈ DWI, ↑ ADC + - **SWI/T2* GRE**: May see ↑ size & number of cortical vessels**** + - Suggests ↑ extraction fraction & possibly recoverable brain + - **T1 C+**: Cortical & leptomeningeal enhancement is seen after 5-7 days following acute infarct + - Enhancing climbing ivy sign + - **MRA**: Can detect arterial occlusion & stenosis in large- & medium-sized cerebral vessels + - Important to identify underlying dissection or arteriopathy + - **PWI**: Provides valuable information about affected brain + - Ischemic penumbra: ↓ perfusion, no DWI change (PWI-DWI mismatch) + - May define brain that is salvageable with acute stroke therapy + - Arterial spin labeling can provide useful perfusion information without contrast administration + - **MRS**: ↑ lactate is hallmark of ischemia/infarct + - Not specific + - **Vessel wall imaging**: Vessel wall enhancement suggests inflammatory arteriopathy + - Vessel wall enhancement patterns improve discrimination of underlying stroke etiology +- ## Ultrasonographic Findings + + + - ### Grayscale ultrasound + + + - Affected territory is hyperechoic in acute/subacute stage + - ### Color Doppler + + + - Direct Doppler evaluation is ideal for surveillance of vascular occlusion in neonate with open sutures + - Transcranial Doppler evaluation of circle of Willis through temporal squamosa + - ↑ velocities can predict stenoses detectable by MRA + - Used as screening tool in children with sickle cell anemia +- ## Angiographic Findings + + + - Catheter angiography is rarely necessary in acute evaluation of childhood stroke + - Only justified if contemplating endovascular therapy + - Best modality for detailed evaluation of primary arteriopathies +- ## Nuclear Medicine Findings + + + - PET & SPECT techniques can be used to + - Identify salvageable regions at risk (ischemic penumbra) + - Demonstrate effects of synangiosis surgery in moyamoya-type vasculopathies +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - CT is initial imaging test for signs/symptoms of stroke; excellent for excluding hemorrhagic stroke (more common in children vs. adults) + - MR with DWI, MRA, PWI + - ### Protocol advice + + + - Contrast can help in assessing timing of injury & performing perfusion imaging + +# DIFFERENTIAL DIAGNOSIS + +- ## Complex Migraine + + + - ↓ (early) or ↑ (late) perfusion with normal DWI + - Engorgement of vessels on SWI +- ## Seizure-Related Injury + + + - Swelling & restricted diffusion secondary to persistent seizure activity + - Differentiation by clinical presentation & EEG +- [Acute Encephalitis](/document/acute-encephalitis/a45f63bb-c25b-481d-a001-9c520c58060b) + - Acute parenchymal inflammation secondary to infectious agents, typically viral + - Slower onset with encephalopathy +- [Mitochondrial Encephalopathies](/document/mitochondrial-encephalopathies/40004435-b768-4baf-a31e-651f8a174fe2) + - Symmetric basal ganglia involvement is common + - Usually have manifestations beyond CNS +- [Posterior Reversible Encephalopathy Syndrome](/document/acute-hypertensive-encephalopathy--/efc6f9c2-dad9-4eb8-bad2-421bfaf1ec57) + - Patchy cortical/subcortical edema is most common in parietal & occipital lobes, typically in setting of hypertension + - Diffusion restriction is uncommon + +# PATHOLOGY + +- ## General Features + + + - 6 major causes of arterial stroke in children + - Cardiac disease (~ 25%) + - Congenital heart disease, valvular heart disease, arrhythmias, & cardiomyopathies + - Moyamoya-type arteriopathy + - Sickle cell disease + - Neurofibromatosis type I + - Idiopathic + - Arterial dissection (e.g., trauma) + - CNS vasculitis + - Hematologic/metabolic (e.g., coagulopathy) + - Idiopathic (~ 25%) + - No underlying cause discovered + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Depends on patient age, etiology, & involved artery + - < 1 year: Seizures, encephalopathy > focal neurologic + - > 1 year: Usually focal neurologic (e.g., hemiplegia) + - Speech difficulties, gait abnormality, seizure + - Embolic cause: Sudden onset of symptoms + - Stenoocclusive cause: Gradual/intermittent (e.g., TIA) + - Focal deficit may be masked by lethargy, coma, irritability + - Preceding transient events occur in 25% + - Children typically present later than adults (> 24 hours) + - Poor recognition/understanding of symptoms by child, caregiver, physician +- ## Demographics + + + - ### Age + + + - Incidence/mortality greatest < 1 year + - ### Epidemiology + + + - Incidence: 2-3/100,000 per year in USA + - Mortality: 0.6/100,000 + - Underrecognized as significant source of morbidity in pediatric population +- ## Natural History & Prognosis + + + - Capacity for recovery is better than in adults, due to + - Better compensatory mechanisms, collateral recruitment, neuronal plasticity + - Fewer concomitant risk factors +- ## Treatment + + + - Clinical window of opportunity/benefit is not as well understood in children as compared to adults + - Mainstay of chronic therapy for fixed vascular lesions & vasculopathies: Aspirin + - Transfusion therapy for at-risk children with sickle cell disease + +# DIAGNOSTIC CHECKLIST + +- ## Image Interpretation Pearls + + + - Use same imaging signs as adults + - Have low threshold for use of CTA + + 4fee7a61-bc2c-4ce0-a2d5-84ed346ac7d5 + +## References + +# Selected References + +1. [van Es ACGM et al: Endovascular treatment for acute ischemic stroke in children: experience from the MR CLEAN Registry. Stroke. 52(3):781-8, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33617341%5Bpmid%5D) +1. [Visser MJ et al: Automated perfusion-diffusion magnetic resonance imaging in childhood arterial ischemic stroke. Stroke. 52(10):3296-304, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34404238%5Bpmid%5D) +1. [Donahue MJ et al: Neuroimaging advances in pediatric stroke. Stroke. 50(2):240-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30661496%5Bpmid%5D) +1. [Dlamini N et al: Arterial wall imaging in pediatric stroke. Stroke. 49(4):891-98, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29581340%5Bpmid%5D) +1. [Khalaf A et al: Pediatric stroke imaging. Pediatr Neurol. 86:5-18, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30122281%5Bpmid%5D) +1. [Beslow LA: Stroke diagnosis in the pediatric emergency department: an ongoing challenge. Stroke. 48(5):1132-3, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28336680%5Bpmid%5D) +1. [Satti S et al: Mechanical thrombectomy for pediatric acute ischemic stroke: review of the literature. J Neurointerv Surg. 9(8):732-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27448827%5Bpmid%5D) +1. [Wilson JL et al: Endovascular therapy in pediatric stroke: utilization, patient characteristics, and outcomes. Pediatr Neurol. 69:87-92.e2, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28233666%5Bpmid%5D) +1. [Madaelil TP et al: Mechanical thrombectomy in pediatric acute ischemic stroke: clinical outcomes and literature review. Interv Neuroradiol. 22(4):426-31, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26945589%5Bpmid%5D) +1. [Polan RM et al: Susceptibility-weighted imaging in pediatric arterial ischemic stroke: a valuable alternative for the noninvasive evaluation of altered cerebral hemodynamics. AJNR Am J Neuroradiol. 36(4):783-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25477354%5Bpmid%5D) +1. [Bernard TJ et al: Emergence of the primary pediatric stroke center: impact of the thrombolysis in pediatric stroke trial. Stroke. 45(7):2018-23, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24916908%5Bpmid%5D) +1. [Gemmete JJ et al: Arterial ischemic stroke in children. Neuroimaging Clin N Am. 23(4):781-98, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24156865%5Bpmid%5D) +1. [Kitchen L et al: The pediatric stroke outcome measure: a validation and reliability study. Stroke. 43(6):1602-8, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22474056%5Bpmid%5D) +1. [Beslow LA et al: Hemorrhagic transformation of childhood arterial ischemic stroke. Stroke. 42(4):941-6, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21350202%5Bpmid%5D) +1. [Cárdenas JF et al: Pediatric stroke. Childs Nerv Syst. 27(9):1375-90, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21336993%5Bpmid%5D) +1. [Dowling MM et al: Intracardiac shunting and stroke in children: a systematic review. J Child Neurol. 26(1):72-82, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21212453%5Bpmid%5D) +1. [Lanni G et al: Pediatric stroke: clinical findings and radiological approach. Stroke Res Treat. 2011:172168, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21603166%5Bpmid%5D) +1. [Larrue V et al: Etiologic investigation of ischemic stroke in young adults. Neurology. 76(23):1983-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21646623%5Bpmid%5D) +1. [Munot P et al: Characteristics of childhood arterial ischemic stroke with normal MR angiography. Stroke. 42(2):504-6, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21193747%5Bpmid%5D) +1. [Sedney CL et al: Cervical abnormalities causing vertebral artery dissection in children. J Neurosurg Pediatr. 7(3):272-5, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21361766%5Bpmid%5D) +1. [Lopez-Vicente M et al: Diagnosis and management of pediatric arterial ischemic stroke. J Stroke Cerebrovasc Dis. 19(3):175-83, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20434043%5Bpmid%5D) +1. [Shellhaas RA et al: Mimics of childhood stroke: characteristics of a prospective cohort. Pediatrics. 118(2):704-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16882826%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial DWI MR in a 4-day-old term neonate presenting with seizures shows diffusion restriction throughout the left middle cerebral artery (MCA) territory, consistent with a perinatal arterial ischemic stroke (PAIS).](images/app.statdx.com_image_thumbnail_a3c930b4-482e-4cf0-90e4-b39fe2956e04_annotated_true_size_900_quality_90_0ab7ec77182d251abcc32ec93dc514df91cd0baa.jpg) +*Axial DWI MR in a 4-day-old term neonate presenting with seizures shows diffusion restriction throughout the left middle cerebral artery (MCA) territory, consistent with a perinatal arterial ischemic stroke (PAIS).* + +![Axial DWI MR in a 4-day-old term neonate presenting with seizures shows diffusion restriction throughout the left middle cerebral artery (MCA) territory, consistent with a perinatal arterial ischemic stroke (PAIS).](images/app.statdx.com_image_thumbnail_a3c930b4-482e-4cf0-90e4-b39fe2956e04_size_174_quality_85_80f5ab910133900e39d9bb5b819a3b9d41218bbd.jpg) +*Axial DWI MR in a 4-day-old term neonate presenting with seizures shows diffusion restriction throughout the left middle cerebral artery (MCA) territory, consistent with a perinatal arterial ischemic stroke (PAIS).* + +![Axial T2 MR in the same patient 2 years later shows cystic encephalomalacia throughout left MCA territory & passive enlargement of the left lateral ventricle . Patients with PAIS who do not present near birth with seizures may later present with early hand preference or extremity weakness.](images/app.statdx.com_image_thumbnail_be6b168d-b342-49c4-9de1-5e6a0226ff5c_annotated_true_size_900_quality_90_685838e55d91cbe0c752474ca3a75ad7b5594d01.jpg) +*Axial T2 MR in the same patient 2 years later shows cystic encephalomalacia throughout left MCA territory & passive enlargement of the left lateral ventricle . Patients with PAIS who do not present near birth with seizures may later present with early hand preference or extremity weakness.* + +![Axial FLAIR MR in a 2-year-old girl shows multiple areas of cytotoxic edema in both cerebral hemispheres in this patient with moyamoya-type vasculopathy.](images/app.statdx.com_image_thumbnail_7199d868-43a2-4218-bcf0-550d4b57028a_annotated_true_size_900_quality_90_8b262bcefd2fc927baa9f8c52665245fd01c0231.jpg) +*Axial FLAIR MR in a 2-year-old girl shows multiple areas of cytotoxic edema in both cerebral hemispheres in this patient with moyamoya-type vasculopathy.* + +![Axial DWI MR in the same patient with moyamoya-type vasculopathy shows diffusion restriction in the right frontoparietal foci of signal abnormality , suggesting an acute/subacute infarct. However, there is no diffusion restriction in the left parietal region , suggesting this infarct is of an older age. Acute stroke should prompt careful arterial evaluation.](images/app.statdx.com_image_thumbnail_2ed900c4-b7c2-4d7c-98d7-ec2fdc3a765b_annotated_true_size_900_quality_90_36d86a963b4e78d21f25bde022e6ec6ecb27cd17.jpg) +*Axial DWI MR in the same patient with moyamoya-type vasculopathy shows diffusion restriction in the right frontoparietal foci of signal abnormality , suggesting an acute/subacute infarct. However, there is no diffusion restriction in the left parietal region , suggesting this infarct is of an older age. Acute stroke should prompt careful arterial evaluation.* + + +### Additional Images + +![Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right MCA territory . Note the sulcal effacement & loss of the gray matter-white matter differentiation.](images/app.statdx.com_image_thumbnail_20436327-00d8-4e86-83a0-411f6da8bddd_annotated_true_size_900_quality_90_fc343c2a802a63468922a8d5c17d35d42531777f.jpg) +*Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right MCA territory . Note the sulcal effacement & loss of the gray matter-white matter differentiation.* + +![Axial DWI MR in the same patient confirms restricted diffusion in the right MCA territory . Also note the focus of restricted diffusion in the left periventricular region . Multiple infarcts in multiple vascular territories should raise suspicion of a proximal embolic source.](images/app.statdx.com_image_thumbnail_0cf1ee69-2d45-47fc-abbf-a405593abc8e_annotated_true_size_900_quality_90_c3fbd5e76773f37c39afe7fa5e53e21920941d8c.jpg) +*Axial DWI MR in the same patient confirms restricted diffusion in the right MCA territory . Also note the focus of restricted diffusion in the left periventricular region . Multiple infarcts in multiple vascular territories should raise suspicion of a proximal embolic source.* + +![Axial DWI MR in a 16-year-old boy involved in a motor vehicle collision (MVC) shows multiple small foci of diffusion restriction , consistent with small infarcts. Multiple infarcts should raise concern for dissection, especially when confined to a single arterial territory.](images/app.statdx.com_image_thumbnail_08013541-3569-428b-a073-46a440578918_annotated_true_size_900_quality_90_e88d994a3efe08014119586abc3d1b283fd47527.jpg) +*Axial DWI MR in a 16-year-old boy involved in a motor vehicle collision (MVC) shows multiple small foci of diffusion restriction , consistent with small infarcts. Multiple infarcts should raise concern for dissection, especially when confined to a single arterial territory.* + +![Axial CTA in the same patient with multiple infarcts shows vessel wall irregularity & an intimal flap in the left internal carotid artery (ICA) , consistent with dissection. The right ICA is small & showed areas of irregularity on other images (not shown). The findings are consistent with bilateral ICA dissections.](images/app.statdx.com_image_thumbnail_4087c464-48ba-4d24-bfaa-8b3e28717e77_annotated_true_size_900_quality_90_2398208c6d0186ddb2a4b3ede41b920daa5f972f.jpg) +*Axial CTA in the same patient with multiple infarcts shows vessel wall irregularity & an intimal flap in the left internal carotid artery (ICA) , consistent with dissection. The right ICA is small & showed areas of irregularity on other images (not shown). The findings are consistent with bilateral ICA dissections.* + +![Axial T1 C+ MR in a 2-year-old girl shows cortical enhancement in the region of a right frontoparietal infarct, suggesting that it is at least a week old.](f529ad80-3bab-4832-9bfd-37ffd148e5d8) +*Axial T1 C+ MR in a 2-year-old girl shows cortical enhancement in the region of a right frontoparietal infarct, suggesting that it is at least a week old.* + +![Axial 3D TOF MRA in a 2-year-old with multiple infarcts of various ages shows multiple tiny foci of flow-related signal in the bilateral thalami . This appearance is consistent with lenticulostriate collaterals of moyamoya-type vasculopathy in the setting of bilateral carotid terminus occlusions.](e1364043-c1b5-4d16-94a3-fc68e9a0f9d1) +*Axial 3D TOF MRA in a 2-year-old with multiple infarcts of various ages shows multiple tiny foci of flow-related signal in the bilateral thalami . This appearance is consistent with lenticulostriate collaterals of moyamoya-type vasculopathy in the setting of bilateral carotid terminus occlusions.* + +![Axial T2 MR in a high school football player who developed vomiting, confusion, & vertigo during a game shows gyral swelling & hyperintense signal in the medial temporal lobe , which is in the vascular territory of the left posterior cerebral artery. Intracranial MRA acquired at the same time showed a small embolus in the left posterior cerebral artery (PCA).](dc16aa78-80c3-4b6b-97d0-2794e588c407) +*Axial T2 MR in a high school football player who developed vomiting, confusion, & vertigo during a game shows gyral swelling & hyperintense signal in the medial temporal lobe , which is in the vascular territory of the left posterior cerebral artery. Intracranial MRA acquired at the same time showed a small embolus in the left posterior cerebral artery (PCA).* + +![Axial CTA of the cervical arteries in the same patient shows a subtle linear filling defect , consistent with an intimal flap in the left vertebral artery.](f3b3551b-cb0b-4ac2-b489-4610cb12d28b) +*Axial CTA of the cervical arteries in the same patient shows a subtle linear filling defect , consistent with an intimal flap in the left vertebral artery.* + +![Axial NECT in a 2-da-old with congenital heart disease & seizures shows a well-defined, wedge-shaped region of ↓ attenuation corresponding to the left MCA vascular territory, consistent with an acute/subacute arterial ischemic stroke.](167963a3-a5d4-41ba-b048-101de8e48c69) +*Axial NECT in a 2-da-old with congenital heart disease & seizures shows a well-defined, wedge-shaped region of ↓ attenuation corresponding to the left MCA vascular territory, consistent with an acute/subacute arterial ischemic stroke.* + +![Axial T1 C+ MR in an 8-year-old with a history of neurofibromatosis type I & known bilateral carotid terminus occlusions (resulting in a moyamoya-type vasculopathy pattern) shows abnormal sulcal enhancement (the climbing ivy sign) due to arterial collaterals distal to a proximal occlusion.](6333898f-b0d4-48d5-8092-4d9044f4121d) +*Axial T1 C+ MR in an 8-year-old with a history of neurofibromatosis type I & known bilateral carotid terminus occlusions (resulting in a moyamoya-type vasculopathy pattern) shows abnormal sulcal enhancement (the climbing ivy sign) due to arterial collaterals distal to a proximal occlusion.* + +![Axial NECT shows a segment of the left insular cortical ribbon that is no longer visible on this axial NECT in a 9-year-old with acute right hemiparesis. This subtle finding may be the first indicator of an acute stroke.](6db3a366-dbe9-4643-80e6-e15c54002d04) +*Axial NECT shows a segment of the left insular cortical ribbon that is no longer visible on this axial NECT in a 9-year-old with acute right hemiparesis. This subtle finding may be the first indicator of an acute stroke.* + +![Axial T1 C+ MR shows the typical climbing ivy pattern of arterial collateral enhancement in distal territories caused by proximal occlusion from a moyamoya-type vasculopathy. Note the white matter infarct on the left .](63ff6aa5-cff0-4e42-b280-c61fc310a0d3) +*Axial T1 C+ MR shows the typical climbing ivy pattern of arterial collateral enhancement in distal territories caused by proximal occlusion from a moyamoya-type vasculopathy. Note the white matter infarct on the left .* + +![Coronal T2 MR shows multiple areas of infarction resulting from left hemisphere herniation. Secondary infarction from herniation can cause more morbidity than the initial insult.](80ae88ad-05f4-453e-9598-a44242f3594f) +*Coronal T2 MR shows multiple areas of infarction resulting from left hemisphere herniation. Secondary infarction from herniation can cause more morbidity than the initial insult.* + +![Axial DWI MR shows a characteristic watershed distribution of infarction in the right cerebral hemisphere. This infarct was the result of a carotid terminus stenosis that developed from bacterial meningitis & vasculitis.](2f63d07a-0f89-49ce-8810-2c9d1ee922df) +*Axial DWI MR shows a characteristic watershed distribution of infarction in the right cerebral hemisphere. This infarct was the result of a carotid terminus stenosis that developed from bacterial meningitis & vasculitis.* + +![Axial NECT in a 14-year-old boy with acute right hemiparesis shows a hyperdense MCA sign , indicating acute thrombus in a proximal middle cerebral artery branch.](9df8cd0b-6d68-467c-8b9c-af6dde1f6cfb) +*Axial NECT in a 14-year-old boy with acute right hemiparesis shows a hyperdense MCA sign , indicating acute thrombus in a proximal middle cerebral artery branch.* + +![Coronal FLAIR MR in the same patient shows edema in the insular cortex & frontal operculum supplied by the affected MCA branch . The patient had complete recovery without direct treatment, & no etiology was found.](fbbd6bf9-9fa8-4dc8-af4d-f4295840073e) +*Coronal FLAIR MR in the same patient shows edema in the insular cortex & frontal operculum supplied by the affected MCA branch . The patient had complete recovery without direct treatment, & no etiology was found.* + +![Axial FLAIR MR in a 13-year-old girl with seizures after using ephedra shows foci of ↑ cortical & subcortical white matter signal in the right PCA & left superior cerebellar artery distributions .](0e9f8a37-be57-4405-9495-a44649f1def6) +*Axial FLAIR MR in a 13-year-old girl with seizures after using ephedra shows foci of ↑ cortical & subcortical white matter signal in the right PCA & left superior cerebellar artery distributions .* + +![Sagittal oblique volume-rendered MRA in the same child shows multiple foci of arterial narrowing & dilation due to a primary arteritis of the CNS.](a09d3907-0d0a-4fbb-b7e9-a576765c3b7d) +*Sagittal oblique volume-rendered MRA in the same child shows multiple foci of arterial narrowing & dilation due to a primary arteritis of the CNS.* + +![Axial CECT shows a subtle linear filling defect in the left ICA of a child presenting with a left hemisphere infarct after mandibular surgery. The defect represents an arterial dissection.](935f0b6f-b82f-4816-827d-d655b80c3672) +*Axial CECT shows a subtle linear filling defect in the left ICA of a child presenting with a left hemisphere infarct after mandibular surgery. The defect represents an arterial dissection.* + +![Axial T2 MR shows predominately cortical/subcortical swelling & abnormal signal of the left parietal lobe, typical of a subacute left MCA territory infarct. Approximately 1/3 of childhood strokes will not have an underlying etiology diagnosed.](8e6dea72-c90f-4204-a73b-05e766c665d5) +*Axial T2 MR shows predominately cortical/subcortical swelling & abnormal signal of the left parietal lobe, typical of a subacute left MCA territory infarct. Approximately 1/3 of childhood strokes will not have an underlying etiology diagnosed.* + diff --git a/docs_md/articles/cisterna-magna-mass_047add0c-7e4f-40a0-9933-8d6fa00a24f7.md b/docs_md/articles/cisterna-magna-mass_047add0c-7e4f-40a0-9933-8d6fa00a24f7.md index bb7889c..d73ae2e 100644 --- a/docs_md/articles/cisterna-magna-mass_047add0c-7e4f-40a0-9933-8d6fa00a24f7.md +++ b/docs_md/articles/cisterna-magna-mass_047add0c-7e4f-40a0-9933-8d6fa00a24f7.md @@ -200,14 +200,26 @@ breadcrumbs: **Herniation Syndromes, Intracranial** *Sagittal T1 C+ MR shows a cystic mass with an enhancing nodule , consistent with hemangioblastoma, pushing the tonsils inferiorly through the foramen magnum . Note effaced 4th ventricle , enlarged foramen of Monro , and enlarged 3rd and lateral ventricles, consistent with obstructive hydrocephalus.* +![Sagittal T1 C+ MR shows a cystic mass with an enhancing nodule , consistent with hemangioblastoma, pushing the tonsils inferiorly through the foramen magnum . Note effaced 4th ventricle , enlarged foramen of Monro , and enlarged 3rd and lateral ventricles, consistent with obstructive hydrocephalus.](images/app.statdx.com_image_thumbnail_d93e03ab-ee6a-4de8-b332-8660df2448e1_annotated_true_size_900_quality_90_c96838274df7ff30ca2c110018853d2c1935108f.jpg) +**Herniation Syndromes, Intracranial** +*Sagittal T1 C+ MR shows a cystic mass with an enhancing nodule , consistent with hemangioblastoma, pushing the tonsils inferiorly through the foramen magnum . Note effaced 4th ventricle , enlarged foramen of Monro , and enlarged 3rd and lateral ventricles, consistent with obstructive hydrocephalus.* + ![Sagittal T1 C+ MR shows a cystic mass with an enhancing nodule , consistent with hemangioblastoma, pushing the tonsils inferiorly through the foramen magnum . Note effaced 4th ventricle , enlarged foramen of Monro , and enlarged 3rd and lateral ventricles, consistent with obstructive hydrocephalus.](images/app.statdx.com_image_thumbnail_d93e03ab-ee6a-4de8-b332-8660df2448e1_size_174_quality_85_c6e99252.jpg) **Herniation Syndromes, Intracranial** *Sagittal T1 C+ MR shows a cystic mass with an enhancing nodule , consistent with hemangioblastoma, pushing the tonsils inferiorly through the foramen magnum . Note effaced 4th ventricle , enlarged foramen of Monro , and enlarged 3rd and lateral ventricles, consistent with obstructive hydrocephalus.* +![Sagittal T1 C+ MR shows a cystic mass with an enhancing nodule , consistent with hemangioblastoma, pushing the tonsils inferiorly through the foramen magnum . Note effaced 4th ventricle , enlarged foramen of Monro , and enlarged 3rd and lateral ventricles, consistent with obstructive hydrocephalus.](images/app.statdx.com_image_thumbnail_d93e03ab-ee6a-4de8-b332-8660df2448e1_size_174_quality_85_cc0d5b70d1f575b33fbafa4089ff979be07c8005.jpg) +**Herniation Syndromes, Intracranial** +*Sagittal T1 C+ MR shows a cystic mass with an enhancing nodule , consistent with hemangioblastoma, pushing the tonsils inferiorly through the foramen magnum . Note effaced 4th ventricle , enlarged foramen of Monro , and enlarged 3rd and lateral ventricles, consistent with obstructive hydrocephalus.* + ![Sagittal T2 MR in a patient with occipital headaches shows a significantly pointed configuration of a low-lying cerebellar tonsil to the level of the midposterior ring of C2. Notice also the dorsally tilted dens , effacement of CSF at the foramen magnum, and associated cervicothoracic syrinx .](images/app.statdx.com_image_thumbnail_f32667fd-ebff-4c5c-bd14-80a9c76d4c17_annotated_true_size_900_quality_90_0d739782.jpg) **Chiari 1** *Sagittal T2 MR in a patient with occipital headaches shows a significantly pointed configuration of a low-lying cerebellar tonsil to the level of the midposterior ring of C2. Notice also the dorsally tilted dens , effacement of CSF at the foramen magnum, and associated cervicothoracic syrinx .* +![Sagittal T2 MR in a patient with occipital headaches shows a significantly pointed configuration of a low-lying cerebellar tonsil to the level of the midposterior ring of C2. Notice also the dorsally tilted dens , effacement of CSF at the foramen magnum, and associated cervicothoracic syrinx .](images/app.statdx.com_image_thumbnail_f32667fd-ebff-4c5c-bd14-80a9c76d4c17_annotated_true_size_900_quality_90_3856a5408550d23f6ce73201ca76207875600145.jpg) +**Chiari 1** +*Sagittal T2 MR in a patient with occipital headaches shows a significantly pointed configuration of a low-lying cerebellar tonsil to the level of the midposterior ring of C2. Notice also the dorsally tilted dens , effacement of CSF at the foramen magnum, and associated cervicothoracic syrinx .* + ![Sagittal T2 MR shows a small posterior fossa with extension of the cerebellar peg through the foramen magnum to the level of C6. The cervicomedullary junction and 4th ventricle are low-lying and there is mild tectal beaking and moderate prominence of the massa intermedia .](images/app.statdx.com_image_thumbnail_3c1182ce-603f-414b-9ecd-728488f8ea13_annotated_true_size_900_quality_90_73ce0e43.jpg) **Chiari 2** *Sagittal T2 MR shows a small posterior fossa with extension of the cerebellar peg through the foramen magnum to the level of C6. The cervicomedullary junction and 4th ventricle are low-lying and there is mild tectal beaking and moderate prominence of the massa intermedia .* @@ -267,10 +279,18 @@ breadcrumbs: **Dandy-Walker Continuum** *Sagittal T1WI MR demonstrates a markedly enlarged posterior fossa with cystic dilatation of the 4th ventricle , upwardly rotated hypoplastic vermis , and low-lying torcular Herophili .* +![Sagittal T2 MR in a patient with occipital headaches shows a significantly pointed configuration of a low-lying cerebellar tonsil to the level of the midposterior ring of C2. Notice also the dorsally tilted dens and effacement of CSF at the foramen magnum.](images/app.statdx.com_image_thumbnail_dac4c49b-c2b5-416f-a085-f7b7e6de8e51_annotated_true_size_900_quality_90_124996df6709de408f0f71443497b1d554b5bac4.jpg) +**Chiari 1** +*Sagittal T2 MR in a patient with occipital headaches shows a significantly pointed configuration of a low-lying cerebellar tonsil to the level of the midposterior ring of C2. Notice also the dorsally tilted dens and effacement of CSF at the foramen magnum.* + ![Sagittal T2 MR in a patient with occipital headaches shows a significantly pointed configuration of a low-lying cerebellar tonsil to the level of the midposterior ring of C2. Notice also the dorsally tilted dens and effacement of CSF at the foramen magnum.](images/app.statdx.com_image_thumbnail_dac4c49b-c2b5-416f-a085-f7b7e6de8e51_annotated_true_size_900_quality_90_60d55480.jpg) **Chiari 1** *Sagittal T2 MR in a patient with occipital headaches shows a significantly pointed configuration of a low-lying cerebellar tonsil to the level of the midposterior ring of C2. Notice also the dorsally tilted dens and effacement of CSF at the foramen magnum.* +![Sagittal T1WI MR shows inferior extension of the peg-like cerebellar tonsils well below the level of the foramen magnum , consistent with Chiari 1.](images/app.statdx.com_image_thumbnail_bf765af5-15a4-473e-8df5-a11a991e90bc_annotated_true_size_900_quality_90_0ceddc8d28a40a3fb37de01ff06a003db234bdd5.jpg) +**Chiari 1** +*Sagittal T1WI MR shows inferior extension of the peg-like cerebellar tonsils well below the level of the foramen magnum , consistent with Chiari 1.* + ![Sagittal T1WI MR shows inferior extension of the peg-like cerebellar tonsils well below the level of the foramen magnum , consistent with Chiari 1.](images/app.statdx.com_image_thumbnail_bf765af5-15a4-473e-8df5-a11a991e90bc_annotated_true_size_900_quality_90_1e09c50d.jpg) **Chiari 1** *Sagittal T1WI MR shows inferior extension of the peg-like cerebellar tonsils well below the level of the foramen magnum , consistent with Chiari 1.* @@ -279,14 +299,30 @@ breadcrumbs: **Herniation Syndromes, Intracranial** *Axial T2WI MR at the level of the foramen magnum demonstrates downward tonsillar herniation secondary to mass affect from brain death.* +![Axial T2WI MR at the level of the foramen magnum demonstrates downward tonsillar herniation secondary to mass affect from brain death.](images/app.statdx.com_image_thumbnail_4ec0f703-c702-49e8-a1ed-adb06f9da9c5_annotated_true_size_900_quality_90_9c73d7b8c98a0750e1e39da7ca30d691345c61f0.jpg) +**Herniation Syndromes, Intracranial** +*Axial T2WI MR at the level of the foramen magnum demonstrates downward tonsillar herniation secondary to mass affect from brain death.* + ![Sagittal T1WI MR shows cerebellar tonsillar herniation from a large left posterior fossa mass. Note compression of 4th ventricle . Supratentorial ventricles are enlarged .](images/app.statdx.com_image_thumbnail_8aaa21ec-1c19-4e6c-8181-592b3df7869d_annotated_true_size_900_quality_90_60eba5bd.jpg) **Herniation Syndromes, Intracranial** *Sagittal T1WI MR shows cerebellar tonsillar herniation from a large left posterior fossa mass. Note compression of 4th ventricle . Supratentorial ventricles are enlarged .* +![Sagittal T1WI MR shows cerebellar tonsillar herniation from a large left posterior fossa mass. Note compression of 4th ventricle . Supratentorial ventricles are enlarged .](images/app.statdx.com_image_thumbnail_8aaa21ec-1c19-4e6c-8181-592b3df7869d_annotated_true_size_900_quality_90_bff1399a0f1c25ad652a2e142b450c818359330b.jpg) +**Herniation Syndromes, Intracranial** +*Sagittal T1WI MR shows cerebellar tonsillar herniation from a large left posterior fossa mass. Note compression of 4th ventricle . Supratentorial ventricles are enlarged .* + +![Sagittal CINE phase-contrast CSF flow - diastolic shows diminished posterior CSF flow compared to anterior CSF flow at the site of tonsillar impaction.](images/app.statdx.com_image_thumbnail_bbdce702-8985-4ec6-a3e4-cad6dea34ada_annotated_true_size_900_quality_90_46dc142616e37b8a1603cb941dffb273010b2d9e.jpg) +**Chiari 1** +*Sagittal CINE phase-contrast CSF flow - diastolic shows diminished posterior CSF flow compared to anterior CSF flow at the site of tonsillar impaction.* + ![Sagittal CINE phase-contrast CSF flow - diastolic shows diminished posterior CSF flow compared to anterior CSF flow at the site of tonsillar impaction.](images/app.statdx.com_image_thumbnail_bbdce702-8985-4ec6-a3e4-cad6dea34ada_annotated_true_size_900_quality_90_9fd2b7f0.jpg) **Chiari 1** *Sagittal CINE phase-contrast CSF flow - diastolic shows diminished posterior CSF flow compared to anterior CSF flow at the site of tonsillar impaction.* +![Sagittal T2WI MR shows a classic case of Chiari 1 with pointed cerebellar tonsils protruding through the foramen magnum and effacing the cisterna magna.](images/app.statdx.com_image_thumbnail_6840be04-8ddf-44dd-90f5-533d79cc1e88_annotated_true_size_900_quality_90_0c4ea20fe9e90a19f0729cd70fccb1e840ba42fa.jpg) +**Chiari 1** +*Sagittal T2WI MR shows a classic case of Chiari 1 with pointed cerebellar tonsils protruding through the foramen magnum and effacing the cisterna magna.* + ![Sagittal T2WI MR shows a classic case of Chiari 1 with pointed cerebellar tonsils protruding through the foramen magnum and effacing the cisterna magna.](images/app.statdx.com_image_thumbnail_6840be04-8ddf-44dd-90f5-533d79cc1e88_annotated_true_size_900_quality_90_19b200f9.jpg) **Chiari 1** *Sagittal T2WI MR shows a classic case of Chiari 1 with pointed cerebellar tonsils protruding through the foramen magnum and effacing the cisterna magna.* diff --git a/docs_md/articles/cpa-mass-adult_f3cd22f6-53b9-4392-be23-512d221d2e02.md b/docs_md/articles/cpa-mass-adult_f3cd22f6-53b9-4392-be23-512d221d2e02.md new file mode 100644 index 0000000..7271b09 --- /dev/null +++ b/docs_md/articles/cpa-mass-adult_f3cd22f6-53b9-4392-be23-512d221d2e02.md @@ -0,0 +1,342 @@ +--- +title: "CPA Mass, Adult" +docid: "f3cd22f6-53b9-4392-be23-512d221d2e02" +authors: + - key: "07a2c087-6202-49e7-870b-7aa162d18f06" + value: "Bronwyn E. Hamilton, MD" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c" + - + name: "CPA-IAC and Posterior Fossa" + slug: "cpa-iac-and-posterior-fossa" + treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992" + - + name: "Anatomically Based Differentials" + slug: "anatomically-based-differentials" + treeNodeId: "debfb06c-8656-4f5d-92c1-eaa468185d78" + - + name: "CPA Mass, Adult" + slug: "cpa-mass-adult" + treeNodeId: null +category: "Head and Neck" +documentVersionId: "3389aa3f-4eea-4b0c-aab3-7b7265e22a43" +imageCount: 25 +lastUpdated: "01/18/24" +pageDescription: "CPA Mass, Adult" +pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Anatomically Based Differentials, CPA Mass, Adult" +pageTitle: "CPA Mass, Adult | STATdx" +enhancedTitle: "CPA Mass, Adult" +type: "DDX" +references: true +breadcrumbs: + - "Head and Neck" + - "Differential Diagnosis" + - "CPA-IAC and Posterior Fossa" + - "Anatomically Based Differentials" + - "CPA Mass, Adult" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Idealized imaging protocol in evaluating CPA mass lesions + - T1 C+ fat-saturated MR is gold standard + - Fat saturation differentiates lipoma from vestibular schwannoma + - Add DWI for possible epidermoid cyst + - Add GRE for aneurysm wall clot & calcification; also useful for tumor calcifications + - T2 thin-section, high-resolution MR gives more surgical data when vestibular schwannoma is diagnosed + - CISS or FIESTA most commonly used + - Helps define amount of CSF cap in lateral IAC + - Assesses relationship of cochlear nerve canal to lesion + - If small schwannoma, may define nerve of origin (superior vs. inferior vestibular schwannoma) + - Knowledge of relative incidence of lesions key in CPA-IAC lesion assessment + - Vestibular schwannoma: ~ **90%** of all CPA-IAC masses + - Meningioma, epidermoid cyst, aneurysm, & arachnoid cyst together represent ~ **8%** of all CPA-IAC masses + - All other diagnoses in differential list: ~ **2%** + - Other factors relevant to imaging CPA masses + - 3D facial nerve tractography for CPA masses may aid surgical planning to reduce risk of facial nerve injury + - CPA tumors in women independent risk factor for intracranial aneurysms +- ## Helpful Clues for Common Diagnoses + + + - **Vestibular Schwannoma** + - Morphology + - Ovoid intracanalicular mass (IAC) + - Ice cream on cone shape (CPA-IAC) + - T1 C+ MR + - Enhancing well-defined tumor ± intramural cysts + - High-resolution thin 0.6-mm 3D T2 MR alternative screening modality for schwannomas without contrast + - SWI + - Intratumoral microhemorrhages favor schwannoma, rare in meningioma +- ## Helpful Clues for Less Common Diagnoses + + + - **Meningioma in CPA-IAC** + - Morphology + - Mushroom-shaped dural-based mass capping IAC asymmetrically + - T1 C+ MR + - Enhancing mass, ± dural tails, ± CSF-vascular cleft if CPA component is larger + - 25% of CPA meningiomas have direct extension or dural tail projecting into IAC + - **Epidermoid Cyst in CPA-IAC** + - Morphology + - Insinuating ± scalloping brainstem margin + - MR imaging + - T1 C+ MR: Nonenhancing + - If known or suspected epidermoid develops enhancing margins, consider rare malignant transformation (squamous cell carcinoma); PET avidity can be helpful to confirm suspicion + - DWI: Restricted diffusion (high signal) makes diagnosis + - T2 MR: Follows fluid signal intensity + - FLAIR: Lack of complete fluid suppression + - **Aneurysm****in****CPA-IAC** + - Morphology + - Ovoid or fusiform; rarely IAC + - MR imaging + - T1 & T1 C+ MR: Complex signal mass from wall calcification, clot, & flow + - MRA (CTA, angiography) sorts out diagnosis + - **Arachnoid Cyst****in****CPA** + - Morphology + - Fills cistern with rounded margins + - MR imaging + - T1 C+ MR: No enhancement + - FLAIR: Lesion attenuates + - DWI: No restricted diffusion + - **Metastases in CPA-IAC** + - Morphology + - Irregular invasive margins + - MR imaging + - T1 C+ MR: Single or multiple enhancing masses in CPA + - 4 sites primarily involved: Flocculus, choroid plexus, arachnoid-dura, or pia +- ## Helpful Clues for Rare Diagnoses + + + - **Neurofibromatosis Type****2** + - Morphology + - Bilateral ovoid IAC or ice cream on cone-shaped CPA-IAC masses + - MR imaging + - T1 C+ MR: Bilateral enhancing CPA-IAC masses + - ± additional schwannomas & meningiomas + - **Sarcoidosis****in****CPA-IAC** + - Laboratory + - CSF lymphocytosis + - ↑ blood angiotensin converting enzyme + - Morphology + - En plaque or nodular dural lesion(s) + - MR imaging + - T1 C+ MR: Enhancing multifocal dural-based lesions + - **Choroid Plexus Papilloma****in****CPA** + - Morphology + - Dumbbell shape with 4th ventricle & CPA cistern components + - Pear-shaped if begins in foramen of Luschka + - MR imaging + - T1 C+ MR: Avidly enhancing mass in 4th ventricle projecting through foramen of Luschka into CPA + - **Lipoma****in****CPA-IAC** + - Morphology + - Ovoid if IAC only + - CPA lesion may be broad-based against lateral pons + - Nonenhanced CT + - Fat-density lesion of CPA ± IAC ± inner ear + - MR imaging + - T1 MR: High-signal lesion, suppresses with fat saturation + - Caveat: If T1 C+ without fat saturation, lipoma may be mistaken for vestibular schwannoma + - **Ependymoma in CPA** + - Morphology + - Irregular soft tumor squeezes out through 4th ventricle foramen of Luschka into CPA + - Tumor margins amorphous + - Bone CT + - Calcifications in 50% of cases + - MR imaging + - T1 C+ MR: Heterogeneous enhancement of solid tumor components + - Marginal enhancement of tumor cyst wall + - **Hypertrophic Pachymeningitis** + - Varied causes require tissue diagnosis + - IgG4-related disease (↑ plasma or tissue IgG4) + - Granulomatosis with polyangiitis: Antineutrophil cytoplasmic antibody (ANCA) (+) associated vasculitis + - Idiopathic (a.k.a. intracranial idiopathic inflammatory pseudotumor) + - Morphology + - En plaque + - MR imaging + - T1 C+ MR: Thickened enhancing dura + - Caveat: May mimic meningioma, sarcoidosis, or metastatic disease + - **F****acial Nerve****Schwannoma in CPA-IAC** + - Morphology + - CPA-IAC mass with labyrinthine tail + - Bone CT + - Labyrinthine segment of CNVII may be enlarged + - MR imaging + - T1 C+ MR: Enhancing tubular mass in CPA-IAC & labyrinthine segment of CNVII + - Caveat: If labyrinthine segment of CNVII not involved, cannot be differentiated from vestibular schwannoma + - **Jugular Foramen Schwannoma** + - Morphology + - Lobular mass projects superomedially from jugular foramen toward lateral brainstem + - MR imaging + - T1 C+ MR: Enhancing mass arising from jugular foramen + - **IAC Venous Malformation ("Hemangioma")** + - Morphology + - Poorly marginated IAC mass with punctate calcifications + - Bone CT + - Punctate calcifications in IAC mass + - MR imaging + - T1 C+ MR: Enhancing IAC mass with focal low-signal foci (calcifications) + - **Neurenteric Cyst** + - Morphology + - Rounded ovoid mass in prepontine cistern + - MR imaging + - T1: Intermediate to high signal T1 prepontine mass + - Nonenhancing + - Caveat: ↑ T1 signal differentiates from epidermoid cyst + +## References + +# Selected References + +1. [Shimanuki MN et al: Imaging of temporal bone mass lesions: a pictorial review. Diagnostics (Basel). 13(16), 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37627924%5Bpmid%5D) +1. [Shimojima Y et al: Hypertrophic pachymeningitis in ANCA-associated vasculitis: clinical and immunopathological features and insights. Autoimmun Rev. 22(6):103338, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37062439%5Bpmid%5D) +1. [Ota Y et al: Advanced MRI to differentiate schwannomas and metastases in the cerebellopontine angle/internal auditory canal. J Neuroimaging. 32(6):1177-84, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35879866%5Bpmid%5D) +1. [Ozaki K et al: Arachnoid cyst alone causes hemifacial spasm: illustrative case. J Neurosurg Case Lessons. 3(15), 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36303502%5Bpmid%5D) +1. [Sakamoto H et al: Radio-pathological characteristics of malignant transformation of an epidermoid cyst in the cerebellopontine angle: a case report. 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[Pamela Ferreira Neto B et al: Noncystic cerebellopontine angle hemangioblastoma: A case of an atypical location. Int J Surg Case Rep. 74:234-7, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32892127%5Bpmid%5D) +1. [Melenotte C et al: Clinical presentation, treatment and outcome of IgG4-related pachymeningitis: from a national case registry and literature review. Semin Arthritis Rheum. 49(3):430-7, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31155444%5Bpmid%5D) +1. [Zheng SF et al: Cerebellopontine angle tumors are associated with a greater incidence of unruptured intracranial aneurysms. World Neurosurg. 122:e561-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31108072%5Bpmid%5D) +1. [Dunn IF et al: Congress of neurological surgeons systematic review and evidence-based guidelines on the role of imaging in the diagnosis and management of patients with vestibular schwannomas. Neurosurgery. 82(2):E32-4, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29309686%5Bpmid%5D) +1. [Prabhu V et al: Preserved cochlear CISS signal is a predictor for hearing preservation in patients treated for vestibular schwannoma with stereotactic radiosurgery. Otol Neurotol. 39(5):628-31, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29561382%5Bpmid%5D) +1. [Mishra A et al: Susceptibility weighted imaging - a problem-solving tool in differentiation of cerebellopontine angle schwannomas and meningiomas. Neuroradiol J. 30(3):253-8, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28627983%5Bpmid%5D) +1. [Schulze M et al: Improvement in imaging common temporal bone pathologies at 3 T MRI: small structures benefit from a small field of view. Clin Radiol. 72(3):267.e1-12, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28034444%5Bpmid%5D) +1. [Rueckriegel SM et al: Probabilistic fiber-tracking reveals degeneration of the contralateral auditory pathway in patients with vestibular schwannoma. AJNR Am J Neuroradiol. 37(9):1610-6, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27256855%5Bpmid%5D) +1. [Watanabe N et al: Imaging alterations due to squamous metaplasia in intracranial neurenteric cysts: A report of two cases. Neuroradiol J. 29(3):187-92, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27009777%5Bpmid%5D) +1. [Mukherjee P et al: Intracranial lipomas affecting the cerebellopontine angle and internal auditory canal: a case series. Otol Neurotol. 32(4):670-5, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21358448%5Bpmid%5D) +1. [Warren FM et al: Imaging characteristics of metastatic lesions to the cerebellopontine angle. Otol Neurotol. 29(6):835-8, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18636029%5Bpmid%5D) +1. [Barrera JE et al: Cavernous hemangioma of the internal auditory canal: a case report and review of the literature. Am J Otolaryngol. 25(3):199-203, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15124171%5Bpmid%5D) +1. [Nakamura M et al: Meningiomas of the internal auditory canal. Neurosurgery. 55(1):119-27; discussion 127-8, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15214980%5Bpmid%5D) +1. [Swartz JD: Lesions of the cerebellopontine angle and internal auditory canal: diagnosis and differential diagnosis. Semin Ultrasound CT MR. 25(4):332-52, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15497614%5Bpmid%5D) +1. [Daniels RL et al: Causes of unilateral sensorineural hearing loss screened by high-resolution fast spin echo magnetic resonance imaging: review of 1,070 consecutive cases. Am J Otol. 21(2):173-80, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10733180%5Bpmid%5D) +1. [Kohan D et al: Uncommon lesions presenting as tumors of the internal auditory canal and cerebellopontine angle. Am J Otol. 18(3):386-92, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9149836%5Bpmid%5D) +1. [Smirniotopoulos JG et al: Cerebellopontine angle masses: radiologic-pathologic correlation. Radiographics. 13(5):1131-47, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8210595%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T1 C+ MR demonstrates a mixed solid and cystic enhancing mass in the right CPA cistern . A small amount of tumor extension is visible extending into the IAC .](images/app.statdx.com_image_thumbnail_8b05ff12-1b6e-4f10-b2ef-b957b56a3fb7_annotated_true_size_900_quality_90_4ed330ffaef11f383ae38df21c6c60eaec019564.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ MR demonstrates a mixed solid and cystic enhancing mass in the right CPA cistern . A small amount of tumor extension is visible extending into the IAC .* + +![Axial T1 C+ MR demonstrates a mixed solid and cystic enhancing mass in the right CPA cistern . A small amount of tumor extension is visible extending into the IAC .](images/app.statdx.com_image_thumbnail_8b05ff12-1b6e-4f10-b2ef-b957b56a3fb7_size_174_quality_85_664dbecbf307d3bb0eadb5f233cfae5aa9569b0e.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ MR demonstrates a mixed solid and cystic enhancing mass in the right CPA cistern . A small amount of tumor extension is visible extending into the IAC .* + +![Axial T1 C+ MR demonstrates a mixed solid and cystic enhancing mass in the right CPA cistern . A small amount of tumor extension is visible extending into the IAC .](images/app.statdx.com_image_thumbnail_8b05ff12-1b6e-4f10-b2ef-b957b56a3fb7_size_174_quality_85_fdcfccaf.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ MR demonstrates a mixed solid and cystic enhancing mass in the right CPA cistern . A small amount of tumor extension is visible extending into the IAC .* + +![Axial T1 C+ MR demonstrates a homogeneously enhancing meningioma in the right CPA . There was no substantial IAC component, and a dural tail was present on additional images (not shown).](images/app.statdx.com_image_thumbnail_a1f98781-dee8-489f-bfb5-977b02248044_annotated_true_size_900_quality_90_fab980560fc964151c80dc26110ca12bf52fc96d.jpg) +**Meningioma in CPA-IAC** +*Axial T1 C+ MR demonstrates a homogeneously enhancing meningioma in the right CPA . There was no substantial IAC component, and a dural tail was present on additional images (not shown).* + +![Axial T2 MR shows a heterogeneous right CPA mass with insinuating contours around the brainstem and cerebellum , typical of an epidermoid cyst. Corresponding DWI showed bright signal (restricted diffusion), and FLAIR showed lack of fluid suppression (not shown).](images/app.statdx.com_image_thumbnail_f48d02f8-3f86-4c47-abe2-003730c4ab76_annotated_true_size_900_quality_90_64878f550dce95897a6191e7bfd660f5872850d0.jpg) +**Epidermoid Cyst in CPA-IAC** +*Axial T2 MR shows a heterogeneous right CPA mass with insinuating contours around the brainstem and cerebellum , typical of an epidermoid cyst. Corresponding DWI showed bright signal (restricted diffusion), and FLAIR showed lack of fluid suppression (not shown).* + +![Axial T1 C+ MR demonstrates a large enhancing distal vertebral artery aneurysm projecting up into the CPA cistern and compressing the area where CNVII and CNVIII exit the brainstem .](677e1c8f-7355-4a17-b864-e21f51d64ae4) +**Aneurysm in CPA-IAC** +*Axial T1 C+ MR demonstrates a large enhancing distal vertebral artery aneurysm projecting up into the CPA cistern and compressing the area where CNVII and CNVIII exit the brainstem .* + +![Axial T2 FS MR shows a high-signal cystic mass in the low CPA cistern. Note the anterior displacement of the proximal vestibulocochlear nerve by the arachnoid cyst . The high signal results from the absence of CSF flow-related artifact.](6e53e3db-1cff-4152-b5e9-270facbcbdd9) +**Arachnoid Cyst in CPA** +*Axial T2 FS MR shows a high-signal cystic mass in the low CPA cistern. Note the anterior displacement of the proximal vestibulocochlear nerve by the arachnoid cyst . The high signal results from the absence of CSF flow-related artifact.* + +![Axial T1 C+ FS MR reveals an inhomogeneously enhancing metastatic focus arising from the dura along the prepontine cistern. This metastasis reaches the anterior margin of the porus acusticus .](77635050-835e-4b56-9e8c-e6b90ac5d712) +**Metastases in CPA-IAC** +*Axial T1 C+ FS MR reveals an inhomogeneously enhancing metastatic focus arising from the dura along the prepontine cistern. This metastasis reaches the anterior margin of the porus acusticus .* + +![Axial T1 C+ MR shows bilateral enhancing CPA-IAC schwannomas . The left-sided schwannoma involves the intratemporal anterior genu of facial nerve , indicating it is most likely a facial nerve schwannoma.](2ade87d6-7047-4b45-8ad5-289c23f24595) +**Neurofibromatosis Type 2** +*Axial T1 C+ MR shows bilateral enhancing CPA-IAC schwannomas . The left-sided schwannoma involves the intratemporal anterior genu of facial nerve , indicating it is most likely a facial nerve schwannoma.* + +![Axial T1 C+ MR shows a heaped-up, dural-based sarcoid deposit in the right CPA cistern that enters the IAC . The Meckel cave is also affected. This lesion mimics meningioma.](e6ad4713-980e-486c-bd32-0b1c59301c94) +**Sarcoidosis in CPA-IAC** +*Axial T1 C+ MR shows a heaped-up, dural-based sarcoid deposit in the right CPA cistern that enters the IAC . The Meckel cave is also affected. This lesion mimics meningioma.* + +![Axial T1 C+ MR reveals a pear-shaped, inhomogeneously enhancing papilloma projecting from the lateral recess of the 4th ventricle through the foramen of Luschka into the low CPA cistern .](0c1054cb-e25b-4d59-b91f-c5144b169de6) +**Choroid Plexus Papilloma in CPA** +*Axial T1 C+ MR reveals a pear-shaped, inhomogeneously enhancing papilloma projecting from the lateral recess of the 4th ventricle through the foramen of Luschka into the low CPA cistern .* + +![Axial NECT performed for trauma shows large, bilateral, fat-attenuation masses within both CPA cisterns , compatible with lipomas. The patient has a longstanding history of deafness. Surgery is not indicated, since hearing does not improve with resection.](e7b651ec-6476-4987-a3cb-9cbc3ff9cfe3) +**Lipoma in CPA-IAC** +*Axial NECT performed for trauma shows large, bilateral, fat-attenuation masses within both CPA cisterns , compatible with lipomas. The patient has a longstanding history of deafness. Surgery is not indicated, since hearing does not improve with resection.* + +![Axial T1 C+ MR demonstrates an aggressive mixed cystic-solid enhancing ependymoma of the right CPA cistern , 4th ventricle , and cerebellar hemisphere .](a2794882-d6ec-48fe-9886-9f4d1adbd0f7) +**Ependymoma in CPA** +*Axial T1 C+ MR demonstrates an aggressive mixed cystic-solid enhancing ependymoma of the right CPA cistern , 4th ventricle , and cerebellar hemisphere .* + +![Axial T1 C+ MR demonstrates an extensive area of enhancing dural thickening along the right low CPA cistern. Pachymeningitis also involves the subjacent jugular foramen .](951cf3ca-23e1-4083-acfa-a6428a5d9fb1) +**Hypertrophic Pachymeningitis** +*Axial T1 C+ MR demonstrates an extensive area of enhancing dural thickening along the right low CPA cistern. Pachymeningitis also involves the subjacent jugular foramen .* + +![Axial T1 C+ MR shows a variant facial nerve schwannoma with a solid enhancing CPA-IAC component extending into the geniculate ganglion . Note the associated arachnoid cyst .](b783b160-7a75-4e8d-b3e0-a4d1d74c7a1a) +**Facial Nerve Schwannoma in CPA-IAC** +*Axial T1 C+ MR shows a variant facial nerve schwannoma with a solid enhancing CPA-IAC component extending into the geniculate ganglion . Note the associated arachnoid cyst .* + +![Axial T1 C+ FS MR shows a bilobed intensely enhancing mass in the right CPA cistern distorting adjacent brain. A large enhancing component within the enlarged right jugular foramen indicated the site of origin. No extension into the adjacent IAC (not shown) was seen.](d939360e-b8ae-4036-8d74-e455d1f8456d) +**Jugular Foramen Schwannoma** +*Axial T1 C+ FS MR shows a bilobed intensely enhancing mass in the right CPA cistern distorting adjacent brain. A large enhancing component within the enlarged right jugular foramen indicated the site of origin. No extension into the adjacent IAC (not shown) was seen.* + + +### Additional Images + +![Axial T1 C+ MR shows a heterogeneous enhancing CPA mass filling and expanding the right IAC . Note characteristic internal cystic foci .](images/app.statdx.com_image_thumbnail_1bd5549c-c05e-4987-8d09-f549eba5d260_annotated_true_size_900_quality_90_d2d61777394cc0d263660345072cc7685997a31b.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ MR shows a heterogeneous enhancing CPA mass filling and expanding the right IAC . Note characteristic internal cystic foci .* + +![Axial T1 C+ MR shows an typical, heterogeneously enhancing right CPA schwannoma with a colocated arachnoid cyst .](images/app.statdx.com_image_thumbnail_2293ecf4-c26e-42b2-931d-bf3467dac8d9_annotated_true_size_900_quality_90_de18ebec90a7c909c109761ffe78a9ef287c55c4.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ MR shows an typical, heterogeneously enhancing right CPA schwannoma with a colocated arachnoid cyst .* + +![Axial T2 MR shows a hyperintense mass in the right CPA with "insinuating" margins , typical of epidermoid cysts. No enhancement was noted on postcontrast imaging and DWI (not shown) showed lesion restriction.](images/app.statdx.com_image_thumbnail_e661eeb6-a345-48d3-9b41-ece28d8fc46c_annotated_true_size_900_quality_90_b9f0463ce561777347b8d8217190e7c90af195e5.jpg) +**Epidermoid Cyst in CPA-IAC** +*Axial T2 MR shows a hyperintense mass in the right CPA with "insinuating" margins , typical of epidermoid cysts. No enhancement was noted on postcontrast imaging and DWI (not shown) showed lesion restriction.* + +![Axial T1 C+ MR shows an enhancing IAC mass with multiple punctate low-signal foci . CT showed that calcifications were present, supporting the diagnosis of IAC hemangioma.](e19eaf8f-ba72-4f69-96d6-c54fddb22e19) +**IAC Venous Malformation ("Hemangioma")** +*Axial T1 C+ MR shows an enhancing IAC mass with multiple punctate low-signal foci . CT showed that calcifications were present, supporting the diagnosis of IAC hemangioma.* + +![Axial FLAIR MR demonstrates an area of high signal in the low CPA cistern that was found to be a neurenteric cyst at surgery.](5af52315-fc7e-4c9e-ba2a-05450a38d3aa) +**Neurenteric Cyst** +*Axial FLAIR MR demonstrates an area of high signal in the low CPA cistern that was found to be a neurenteric cyst at surgery.* + +![Axial T1 C+ MR reveals an enhancing mass filling the CPA and IAC . Note that the cochlear nerve canal is involved , making resection with hearing preservation difficult.](images/app.statdx.com_image_thumbnail_13dc8b5e-a602-4b88-ae31-2980159ac065_annotated_true_size_900_quality_90_b37ad7e33cba9f1e1cc0f701c610a28adce94345.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ MR reveals an enhancing mass filling the CPA and IAC . Note that the cochlear nerve canal is involved , making resection with hearing preservation difficult.* + +![Axial T1 C+ FS MR reveals an enhancing dural-based mass centered over the IAC but with minimal IAC involvement . The shape and the associated dural tail make meningioma the diagnosis.](images/app.statdx.com_image_thumbnail_7d9c4459-880f-424e-aae8-6f8017189d94_annotated_true_size_900_quality_90_709b0ff5087714d7d31ff4bc5fd55db0d1efbc38.jpg) +**Meningioma in CPA-IAC** +*Axial T1 C+ FS MR reveals an enhancing dural-based mass centered over the IAC but with minimal IAC involvement . The shape and the associated dural tail make meningioma the diagnosis.* + +![Axial T1WI MR shows a low-signal mass in the right CPA cistern that insinuates and enlarges the foramen of Luschka and scallops the ventral cerebellar hemisphere .](10a3963e-c42e-42a7-bb00-73e0100aa8a9) +**Epidermoid Cyst in CPA-IAC** +*Axial T1WI MR shows a low-signal mass in the right CPA cistern that insinuates and enlarges the foramen of Luschka and scallops the ventral cerebellar hemisphere .* + +![Axial T1WI MR shows a variant 3-part lipoma affecting the CPA cistern , the high anterior jugular foramen , and the vestibule of the inner ear . Surgical resection is not performed for such lesions.](58f47c7f-849a-41a5-b8bc-a69c8c7e0c16) +**Lipoma in CPA-IAC** +*Axial T1WI MR shows a variant 3-part lipoma affecting the CPA cistern , the high anterior jugular foramen , and the vestibule of the inner ear . Surgical resection is not performed for such lesions.* + +![Coronal T1 C+ FS MR reveals a schwannoma projecting cephalad from the jugular foramen into the CPA cistern. Note that the normal IAC is at the level of the upper margin of the tumor.](34b9815b-a605-429a-9760-b4bc433e3ff5) +**Jugular Foramen Schwannoma** +*Coronal T1 C+ FS MR reveals a schwannoma projecting cephalad from the jugular foramen into the CPA cistern. Note that the normal IAC is at the level of the upper margin of the tumor.* + +![Axial T1 C+ MR shows a partially cystic prepontine and left CPA mass with marginal nodular areas of enhancement due to epidermoid cyst, which in this case was complicated by rare malignant transformation into squamous cell carcinoma. Areas of diffusion restriction were noted in the cystic component; however, soft tissue enhancement along the margins are unexpected for epidermoid, and raise concern for malignancy.](f7514127-58a7-46c8-9496-e4e7f925f257) +**Epidermoid Cyst in CPA-IAC** +*Axial T1 C+ MR shows a partially cystic prepontine and left CPA mass with marginal nodular areas of enhancement due to epidermoid cyst, which in this case was complicated by rare malignant transformation into squamous cell carcinoma. Areas of diffusion restriction were noted in the cystic component; however, soft tissue enhancement along the margins are unexpected for epidermoid, and raise concern for malignancy.* + diff --git a/docs_md/articles/cpa-mass-child_76d2535b-050d-4826-a344-877e5bae4230.md b/docs_md/articles/cpa-mass-child_76d2535b-050d-4826-a344-877e5bae4230.md new file mode 100644 index 0000000..523f693 --- /dev/null +++ b/docs_md/articles/cpa-mass-child_76d2535b-050d-4826-a344-877e5bae4230.md @@ -0,0 +1,174 @@ +--- +title: "CPA Mass, Child" +docid: "76d2535b-050d-4826-a344-877e5bae4230" +authors: + - key: "d19354f3-7ff2-495a-ad3f-064122e45602" + value: "Bernadette L. Koch, MD" + - key: "e8af6d26-3aad-47c9-9083-5128aab09af2" + value: "Susan I. Blaser, MD, FRCPC" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c" + - + name: "CPA-IAC and Posterior Fossa" + slug: "cpa-iac-and-posterior-fossa" + treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992" + - + name: "Anatomically Based Differentials" + slug: "anatomically-based-differentials" + treeNodeId: "debfb06c-8656-4f5d-92c1-eaa468185d78" + - + name: "CPA Mass, Child" + slug: "cpa-mass-child" + treeNodeId: null +category: "Head and Neck" +documentVersionId: "388e4ad1-934e-45b0-8fc0-80caff2df890" +imageCount: 11 +lastUpdated: "03/28/22" +pageDescription: "CPA Mass, Child" +pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Anatomically Based Differentials, CPA Mass, Child" +pageTitle: "CPA Mass, Child | STATdx" +enhancedTitle: "CPA Mass, Child" +type: "DDX" +references: true +breadcrumbs: + - "Head and Neck" + - "Differential Diagnosis" + - "CPA-IAC and Posterior Fossa" + - "Anatomically Based Differentials" + - "CPA Mass, Child" +--- +# ESSENTIAL INFORMATION + +- ## Helpful Clues for Common Diagnoses + + + - [Arachnoid Cyst in CPA-IAC](/document/cpa-iac-arachnoid-cyst/f1af2d0f-adfd-49c7-a3b9-8a1c66dce2be) + - Sharply demarcated extraaxial cyst with CSF density/signal intensity + - Isointense to CSF on all MR sequences, including complete fluid attenuation on FLAIR images + - Nonenhancing + - No diffusion restriction +- ## Helpful Clues for Less Common Diagnoses + + + - [Vestibular Schwannoma](/document/vestibular-schwannoma/48772166-59dc-4909-bc75-538de7dd9ddf) + - Ovoid when within internal auditory canal (IAC) + - Ice cream on cone shape when CPA-IAC + - Hypointense relative to hyperintense CSF on T2 or CISS images, ± intramural cysts + - Enhancing, well-circumscribed tumor + - Rare in children, unless neurofibromatosis type 2 + - Infratentorial Ependymoma + - Heterogeneously enhancing 4th ventricular mass with irregular margins + - Extends through foramen of Luschka → CPA cistern + - Calcification in up to 50% + - [Infantile Hemangioma in CPA-IAC](/document/infantile-hemangioma/4c91584c-4788-4d4d-b854-15d22fdff6b7) + - Smoothly marginated, intensely enhancing mass in CPA-IAC (proliferating phase) + - Proliferating, involuting, and involuted phases + - Usually, involutional timing similar to extracranial hemangiomas; therefore, no surgical intervention + - Glut-1 marker positive + - ± PHACE syndrome + - **P**osterior fossa malformations, **h**emangioma, **a**rterial cerebrovascular anomalies, **c**oarctation of aorta and cardiac defects, **e**ye abnormalities + - [Epidermoid Cyst in CPA-IAC](/document/cpa-iac-epidermoid-cyst/5e83f596-1ca4-41cb-95aa-469147ca5f8f) + - Insinuating mass with irregular or scalloped margins + - ± scalloping brainstem margin + - Iso- to slightly hyperintense to CSF + - Nonenhancing, with restricted diffusion +- ## Helpful Clues for Rare Diagnoses + + + - Atypical Teratoid/Rhabdoid Tumor + - CPA tumor with lytic bone destruction in infants + - Hypointense compared with CSF on T2, with restricted diffusion, enhancing + - [Meningioma in CPA-IAC](/document/cpa-iac-meningitis/c3269e91-15d4-4421-917a-216f4d08a038) + - Dural-based globular or en plaque mass + - Enhancing mass ± dural tail: 23% of CPA meningiomas have extension/dural tail into IAC + - Permeative-sclerotic or hyperostotic bone + - [Lipoma in CPA-IAC](/document/lipoma-in-cpa-iac/3dc1a638-a325-4390-844f-97e01961734d) + - Ovoid or broad-based against lateral pons + - Fat signal intensity/density on MR and CT + - May be mistaken for vestibular schwannoma if T1 C+ images are performed without fat saturation + - [Metastases in CPA-IAC](/document/cpa-iac-metastases/451451c8-7b49-4ce9-bf22-7c02b4652f23) + - Irregular, invasive mass; single or multiple + - Primarily involves leptomeninges, flocculus, choroid plexus, or dura + - Unilateral or bilateral + - [Choroid Plexus Papilloma in CPA](/document/choroid-plexus-papilloma/18e712f5-8553-487d-a939-044336cbf0ad) + - Dumbbell-shaped mass in 4th ventricle, extending through foramen of Luschka → CPA cistern + - Avid contrast enhancement + +## References + +# Selected References + +1. [Vernon V et al: Surgical management of cerebellopontine angle epidermoid cysts: an institutional experience of 10 years. Br J Neurosurg. 1-10, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33410366%5Bpmid%5D) +1. [Bartindale M et al: Facial schwannoma management outcomes: a systematic review of the literature. Otolaryngol Head Neck Surg. 163(2):293-301, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32228141%5Bpmid%5D) +1. [D'Arco F et al: The link between inner ear malformations and the rest of the body: what we know so far about genetic, imaging and histology. Neuroradiology. 62(5):539-44, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32125475%5Bpmid%5D) +1. [Giordano M et al: Surgical management of cerebellopontine angle arachnoid cysts associated with hearing deficit in pediatric patients. J Neurosurg Pediatr. 21(2):119-23, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29171799%5Bpmid%5D) +1. [Bonneville F et al: Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions. Eur Radiol. 17(11):2908-20, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17569053%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T2WI MR in a 3-year-old child demonstrates a moderate-sized right extraaxial CPA cyst , isointense to intraventricular fluid, moderately compressing the right cerebellar hemisphere and midbrain.](images/app.statdx.com_image_thumbnail_228bffa7-8d4e-4a26-95e4-720792522d1c_annotated_true_size_900_quality_90_e133e62eb1c98a46edd9ea21dbedcd80bca60293.jpg) +**Arachnoid Cyst in CPA-IAC** +*Axial T2WI MR in a 3-year-old child demonstrates a moderate-sized right extraaxial CPA cyst , isointense to intraventricular fluid, moderately compressing the right cerebellar hemisphere and midbrain.* + +![Axial T2WI MR in a 3-year-old child demonstrates a moderate-sized right extraaxial CPA cyst , isointense to intraventricular fluid, moderately compressing the right cerebellar hemisphere and midbrain.](images/app.statdx.com_image_thumbnail_228bffa7-8d4e-4a26-95e4-720792522d1c_size_174_quality_85_378fa03f.jpg) +**Arachnoid Cyst in CPA-IAC** +*Axial T2WI MR in a 3-year-old child demonstrates a moderate-sized right extraaxial CPA cyst , isointense to intraventricular fluid, moderately compressing the right cerebellar hemisphere and midbrain.* + +![Axial T2WI MR in a 3-year-old child demonstrates a moderate-sized right extraaxial CPA cyst , isointense to intraventricular fluid, moderately compressing the right cerebellar hemisphere and midbrain.](images/app.statdx.com_image_thumbnail_228bffa7-8d4e-4a26-95e4-720792522d1c_size_174_quality_85_3e5929d1f49a064e4ddc6828e276a2935dc24ae5.jpg) +**Arachnoid Cyst in CPA-IAC** +*Axial T2WI MR in a 3-year-old child demonstrates a moderate-sized right extraaxial CPA cyst , isointense to intraventricular fluid, moderately compressing the right cerebellar hemisphere and midbrain.* + +![Axial FLAIR MR in the same patient demonstrates complete attenuation of signal intensity within the cyst , typical of an arachnoid cyst. There was no diffusion restriction on DW images (not shown), unlike an epidermoid cyst, which typically demonstrates diffusion restriction.](images/app.statdx.com_image_thumbnail_07938734-d209-4ca2-a19f-661872a14feb_annotated_true_size_900_quality_90_d5c4596b8a7821b0cc8f6bff5b8ca26012d99974.jpg) +**Arachnoid Cyst in CPA-IAC** +*Axial FLAIR MR in the same patient demonstrates complete attenuation of signal intensity within the cyst , typical of an arachnoid cyst. There was no diffusion restriction on DW images (not shown), unlike an epidermoid cyst, which typically demonstrates diffusion restriction.* + +![Axial T1 C+ MR in a 12-year-old boy shows bilateral enhancing CPA-IAC masses and a well-defined, enhancing mass involving the left 6th cranial nerve , consistent with multiple schwannomas in a child with NF2.](images/app.statdx.com_image_thumbnail_4be628c0-3a57-47cf-9e11-2b20c4478a40_annotated_true_size_900_quality_90_43587fd68f998965a87decbbe1ac90be8e14abd0.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ MR in a 12-year-old boy shows bilateral enhancing CPA-IAC masses and a well-defined, enhancing mass involving the left 6th cranial nerve , consistent with multiple schwannomas in a child with NF2.* + +![Axial T1 C+ MR in a 2-year-old child demonstrates a large, heterogeneously enhancing posterior fossa mass filling the 4th ventricle, extending through the foramen of Luschka and into the right CPA cistern . Notice extension across the midline toward the left foramen of Luschka .](images/app.statdx.com_image_thumbnail_feb109de-024f-48c5-9306-908aef68e2d0_annotated_true_size_900_quality_90_f017a1f62e70b972143bc53b40f678052f1eea01.jpg) +**Infratentorial Ependymoma** +*Axial T1 C+ MR in a 2-year-old child demonstrates a large, heterogeneously enhancing posterior fossa mass filling the 4th ventricle, extending through the foramen of Luschka and into the right CPA cistern . Notice extension across the midline toward the left foramen of Luschka .* + +![Axial T1 C+ MR in a 1-year-old infant shows a massive facial hemangioma , a right CPA-IAC hemangioma , and ipsilateral cerebellar hemisphere hypoplasia . These findings are consistent with PHACE syndrome.](images/app.statdx.com_image_thumbnail_3ac997c7-8edb-4b2b-a21b-0c76bd769946_annotated_true_size_900_quality_90_393efc18456dbf9a6753ab580d897996d9d5a098.jpg) +**Infantile Hemangioma in CPA-IAC** +*Axial T1 C+ MR in a 1-year-old infant shows a massive facial hemangioma , a right CPA-IAC hemangioma , and ipsilateral cerebellar hemisphere hypoplasia . These findings are consistent with PHACE syndrome.* + +![Axial T1 C+ MR in a teenager with occipital headaches shows an extraaxial hypointense mass , similar in signal intensity to intraventricular CSF, compressing the pons. Differential diagnosis would include arachnoid cyst or dermoid/epidermoid cyst.](images/app.statdx.com_image_thumbnail_57932a31-3c96-4356-bf70-9a9c6685715e_annotated_true_size_900_quality_90_af1bd86cd54fc8d60b74a733faf772b9f64cd155.jpg) +**Epidermoid Cyst in CPA-IAC** +*Axial T1 C+ MR in a teenager with occipital headaches shows an extraaxial hypointense mass , similar in signal intensity to intraventricular CSF, compressing the pons. Differential diagnosis would include arachnoid cyst or dermoid/epidermoid cyst.* + +![Axial FLAIR MR in the same patient as shows heterogeneous, "dirty" signal intensity within the lesion adjacent to the pons, hyperintense relative to CSF. Incomplete suppression on FLAIR sequences is typical of epidermoid cysts.](images/app.statdx.com_image_thumbnail_28738bd9-ded6-4fd1-a445-dc87579a0919_annotated_true_size_900_quality_90_a3461bcd1ab7bbfc586e3bf44519ac7711adf69f.jpg) +**Epidermoid Cyst in CPA-IAC** +*Axial FLAIR MR in the same patient as shows heterogeneous, "dirty" signal intensity within the lesion adjacent to the pons, hyperintense relative to CSF. Incomplete suppression on FLAIR sequences is typical of epidermoid cysts.* + +![Axial DWI MR in the same patient shows hyperintense signal intensity relative to intraventricular CSF, which on ADC map proved to be hypointense (not shown), consistent with restricted diffusion typical of epidermoid cysts.](bb7f1ddc-099c-424b-be3b-57d9cf915cad) +**Epidermoid Cyst in CPA-IAC** +*Axial DWI MR in the same patient shows hyperintense signal intensity relative to intraventricular CSF, which on ADC map proved to be hypointense (not shown), consistent with restricted diffusion typical of epidermoid cysts.* + + +### Additional Images + +![Axial FIESTA in a teenager with epilepsy shows an incidental left CPA arachnoid cyst , isointense to intraventricular CSF, without mass effect on the cisternal segments of the 7th/8th cranial nerves and without extension into the IAC.](images/app.statdx.com_image_thumbnail_7664331b-5556-440b-a7e2-b7a2f1900040_annotated_true_size_900_quality_90_994b1022db28738757e67c72524cc3515311327d.jpg) +**Arachnoid Cyst in CPA-IAC** +*Axial FIESTA in a teenager with epilepsy shows an incidental left CPA arachnoid cyst , isointense to intraventricular CSF, without mass effect on the cisternal segments of the 7th/8th cranial nerves and without extension into the IAC.* + +![Axial T2WI MR in a teenager with occipital headaches demonstrates an oblong, T2-hyperintense epidermoid cyst , mildly compressing the left ventral pons.](8e56a9d7-8007-43f5-a46f-aa353cb3cee5) +**Epidermoid Cyst in CPA-IAC** +*Axial T2WI MR in a teenager with occipital headaches demonstrates an oblong, T2-hyperintense epidermoid cyst , mildly compressing the left ventral pons.* + +![Axial ADC map in the same patient shows hypointense signal on ADC map image consistent with diffusion restriction within the lesion, typical of epidermoid cyst.](ef2a1c6c-aa6d-440c-93ff-799e006f1f85) +**Epidermoid Cyst in CPA-IAC** +*Axial ADC map in the same patient shows hypointense signal on ADC map image consistent with diffusion restriction within the lesion, typical of epidermoid cyst.* + diff --git a/docs_md/articles/cystic-cpa-mass_6c60db6d-8093-4df5-8cbb-c6f6570ae167.md b/docs_md/articles/cystic-cpa-mass_6c60db6d-8093-4df5-8cbb-c6f6570ae167.md new file mode 100644 index 0000000..fccea19 --- /dev/null +++ b/docs_md/articles/cystic-cpa-mass_6c60db6d-8093-4df5-8cbb-c6f6570ae167.md @@ -0,0 +1,325 @@ +--- +title: "Cystic CPA Mass" +docid: "6c60db6d-8093-4df5-8cbb-c6f6570ae167" +authors: + - key: "07a2c087-6202-49e7-870b-7aa162d18f06" + value: "Bronwyn E. Hamilton, MD" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c" + - + name: "CPA-IAC and Posterior Fossa" + slug: "cpa-iac-and-posterior-fossa" + treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992" + - + name: "Generic Imaging Patterns" + slug: "generic-imaging-patterns" + treeNodeId: "ba996846-af9d-4714-b15b-84315b9ad282" + - + name: "Cystic CPA Mass" + slug: "cystic-cpa-mass" + treeNodeId: null +category: "Head and Neck" +cmeTopicId: "75974523-991b-4e4d-90c7-5a70d21f2598" +documentVersionId: "dd13cf85-d7fc-40b3-b753-b3231a135c6b" +imageCount: 23 +lastUpdated: "02/09/24" +pageDescription: "Cystic CPA Mass" +pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Generic Imaging Patterns, Cystic CPA Mass" +pageTitle: "Cystic CPA Mass | STATdx" +enhancedTitle: "Cystic CPA Mass" +type: "DDX" +references: true +breadcrumbs: + - "Head and Neck" + - "Differential Diagnosis" + - "CPA-IAC and Posterior Fossa" + - "Generic Imaging Patterns" + - "Cystic CPA Mass" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - This differential diagnosis is constructed around lesions of cerebellopontine angle (CPA) that may have "cystic" imaging features + - Typically cystic lesions: Epidermoid, arachnoid, & neurenteric cysts; neurocysticercosis & large endolymphatic sac anomaly + - Many solid CPA tumors may have either intramural cysts, necrosis, or extramural cysts as typical or variant MR imaging manifestation + - Schwannoma: Vestibular, facial nerve, trigeminal, or jugular foramen schwannoma with intramural or extramural cysts can all be found in CPA area + - Hemangioblastoma: Cerebellar cystic & solid tumor that may project into CPA cistern + - Endolymphatic sac tumor: CPA involved if large + - Cystic meningioma + - Idealized imaging protocol in evaluating cystic CPA masses + - T1 C+ fat-saturated MR is gold standard + - Contrast helps differentiate solid from cystic components of tumors, such as vestibular or facial nerve schwannoma & hemangioblastoma + - DWI for possible epidermoid cyst (restricted diffusion) + - T2 thin-section, high-resolution MR + - Also sorts out solid and cystic components of lesions + - Helps with cranial nerve & vascular anatomy +- ## Helpful Clues for Common Diagnoses + + + - **Epidermoid Cyst** + - Key facts + - Definition: Congenital rest of epithelial tissue in CPA + - Imaging findings + - Insinuating or scalloping brainstem margin + - T1 MR: Iso- to slightly hyperintense relative to CSF + - T1 C+ MR: Typically nonenhancing cyst; may be hard to see + - Minimal marginal cyst enhancement in 25% + - Soft tissue enhancement present in rare cases of malignant degeneration to squamous cell carcinoma; PET avidity can help confirm suspicion + - T2 MR: High-signal, well-circumscribed CPA mass + - FLAIR: Lack of complete fluid suppression + - DWI: Restricted diffusion (high signal) makes diagnosis + - **Arachnoid Cyst** + - Key facts + - Definition: Congenital lesion resulting from failure of embryonic meninges to merge results in cyst between split arachnoid membrane + - Imaging findings + - Fills cistern with rounded margins + - T1 C+ MR: No enhancement + - FLAIR: Lesion attenuates (black like CSF) + - DWI: Isointense to CSF (no restriction) +- ## Helpful Clues for Less Common Diagnoses + + + - **Vestibular Schwannoma W****ith Intramural Cyst(s)** + - Key facts + - Definition: Vestibular schwannoma may have either intramural or extramural cysts associated + - Imaging findings + - Solid CPA-IAC mass with intramural cysts + - Bone CT: Larger lesions flare medial IAC component + - T1 C+ MR: Enhancing solid tumor component ± intramural cysts (common) ± extramural cyst (rare) + - Microhemorrhages on T2* GRE or SWI favor schwannoma over meningioma + - **Hemangioblastoma** + - Key facts + - Definition: Benign tumor composed of stromal cells in small blood vessels of CNS + - Adult with intraaxial posterior fossa mass abutting pia + - Associated with von Hippel Lindau (VHL) in 25-40% + - Supratentorial hemangioblastomas more likely VHL related + - Imaging findings + - Cerebellar cystic & solid tumor + - T1 C+ MR: 60% of tumors with solid enhancing & cystic components (40% solid only) + - **Large Endolymphatic Sac Anomaly (IP-II)** + - Key facts + - Bilateral congenital sensorineural hearing loss that appears in child with cascading hearing loss pattern + - Most common congenital imaging abnormality + - Recommendation to avoid contact sports + - Imaging findings + - Bone CT: Enlarged bony vestibular aqueduct + - T2 high-resolution MR: Enlarged endolymphatic sac & duct + mild cochlear malformation (modiolar deficiency, deficient apical septation, & scalar chamber asymmetry) + - **Neurocysticercosis** + - Key facts + - Definition: Intracranial infection caused by pork tapeworm (*Taenia solium*) + - Imaging findings + - Cysts with "dots" inside + - Appearance varies with stage + - T1 C+ MR: Cysts with enhancing thin or thick wall + - May be associated with hydrocephalus +- ## Helpful Clues for Rare Diagnoses + + + - **Vestibular Schwannoma W****ith Extramural Cyst** + - Key facts + - Vestibular schwannoma with extramural cyst + - Cyst may be "trapped CSF" or actual arachnoid cyst + - Neurootologists call extramural cyst "herald cyst" + - Imaging findings + - CPA-IAC mass with extramural cyst + - Bone CT: Large lesions flare IAC medial component + - T1 C+ MR: Enhancing solid tumor component ± extramural cyst + - Microhemorrhages on T2* GRE or SWI favor schwannoma over meningioma + - **Facial Nerve Schwannoma in CPA-IAC W****ith Cyst** + - Key facts + - Rare CPA-IAC mass with labyrinthine tail involving labyrinthine segment of facial nerve canal + - Often presents with hearing loss before facial nerve symptoms + - Imaging findings + - Bone CT: Labyrinthine segment of facial nerve may be enlarged + - T1 C+ MR: Enhancing tubular mass in CPA-IAC & labyrinthine segment of facial nerve; intramural or extramural cyst visible + - **Schwannoma, Trigeminal, Skull Base** + - Key facts + - Most common presentation is ipsilateral facial pain + - Imaging findings + - CPA cystic enhancing mass with tail extending toward or into Meckel cave is characteristic + - Look for denervation of masticator space muscles + - **Neurenteric****C****yst** + - Key facts + - Arises at time of notochordal development during transitory existence of neurenteric canal + - Benign endodermal lesion of CNS + - Often presents as incidental rounded to ovoid mass in prepontine cistern + - Imaging findings + - MR shows intermediate- to high-signal T1 prepontine "cystic" mass + - **Jugular Foramen Schwannoma W****ith Intramural Cyst** + - Key facts + - Presents with mixture of 9-12 cranial neuropathy + - Imaging findings + - Bone CT: Enlarged, sharply marginated jugular foramen + - T1 MR: Iso-g to hyperintense compared to CSF + - T1 C+ MR: Enhancing mass with intramural cysts arising from jugular foramen + - Intramural cysts in 25% of jugular foramen schwannomas + - Other MR findings: Mass projects superomedially into CPA cistern, often with brainstem compression + - **Cystic Metastasis in CPA** + - Key facts + - Usually in patients with known primary cancer + - Imaging findings + - T1 C+ MR: Enhancing mass with cystic component may mimic schwannoma or cystic meningioma + - Likely to exhibit rapid growth pattern and more symptoms, such as facial nerve palsy, than benign tumors like schwannoma + - **Endolymphatic Sac Tumor** + - Key facts + - Sporadic or associated with VHL + - Bilateral tumors &/or concurrent hemangioblastoma indicates VHL + - Imaging findings + - Bone CT: Permeative destruction of posterior petrous temporal bone centered at vestibular aqueduct + - T1 MR: Hyperintense foci due to internal hemorrhage are characteristic + - T1 C+ MR: Partly cystic, heterogenous enhancing mass + - DOTATATE scan may show mild uptake due to somatostatin receptor type 2A expression in tumor vasculature not tumor cells + - **Meningioma, Cystic** + - T1 C+ MR: Rare, heterogeneous, partly cystic enhancing mass + - Rare meningioma variant that mimics schwannoma; most are clear cell subtype + +## References + +# Selected References + +1. [Ali NE et al: Natural history of cystic vestibular schwannomas. Ann Otol Rhinol Laryngol. 132(7):795-9, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=35993287%5Bpmid%5D) +1. [Adachi S et al: Unusual imaging characteristics of cystic meningioma in cerebellopontine angle. Neuroradiol J. 35(6):777-9, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35503008%5Bpmid%5D) +1. [Geng Y et al: Endolymphatic sac tumour: exploring the role of CT and MRI features in the diagnosis of 22 cases. Clin Radiol. 77(8):e592-8, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35643739%5Bpmid%5D) +1. [Talukdar R et al: Endolymphatic sac tumor: single-institution series of seven cases with updated review of literature. Eur Arch Otorhinolaryngol. 279(5):2591-8, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=34410472%5Bpmid%5D) +1. [Parlak S et al: 3 Tesla MR imaging of the large endolymphatic duct and sac anomaly with audiological correlation. Eur J Radiol. 145:110064, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34839211%5Bpmid%5D) +1. [Saigal G et al: Utility of microhemorrhage as a diagnostic tool in distinguishing vestibular schwannomas from other cerebellopontine angle (CPA) Tumors. Indian J Otolaryngol Head Neck Surg. 73(3):321-6, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34471620%5Bpmid%5D) +1. [Lou R et al: 68Ga-DOTATATE uptake in an endolymphatic sac tumor: radiologic-pathologic correlation. Clin Nucl Med. 45(7):563-5, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32433163%5Bpmid%5D) +1. [Connor SEJ et al: Is CT or MRI the optimal imaging investigation for the diagnosis of large vestibular aqueduct syndrome and large endolymphatic sac anomaly? Eur Arch Otorhinolaryngol. 276(3):693-702, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30635710%5Bpmid%5D) +1. [Eliezer M et al: Clinical and radiological characteristics of malignant tumors located to the cerebellopontine angle and/or internal acoustic meatus. Otol Neurotol. 40(9):1237-45, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31469787%5Bpmid%5D) +1. [Le H et al: Clinicoradiologic characteristics of endolymphatic sac tumors. Eur Arch Otorhinolaryngol. 276(10):2705-14, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31197530%5Bpmid%5D) +1. [Touska P et al: Temporal bone tumors: an imaging update. Neuroimaging Clin N Am. 29(1):145-72, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30466638%5Bpmid%5D) +1. [Schnack DT et al: Sporadic endolymphatic sac tumor-a very rare cause of hearing loss, tinnitus, and dizziness. J Int Adv Otol. 13(2):289-91, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28716765%5Bpmid%5D) +1. [Agarwal A: Intracranial trigeminal schwannoma. Neuroradiol J. 28(1):36-41, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25924170%5Bpmid%5D) +1. [Dispenza F et al: Imaging of vestibular schwannoma with prevalent cystic component: cystic vestibular schwannoma. Otol Neurotol. 30(5):681-2, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18716565%5Bpmid%5D) +1. [Piccirillo E et al: Cystic vestibular schwannoma: classification, management, and facial nerve outcomes. Otol Neurotol. 30(6):826-34, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19704364%5Bpmid%5D) +1. [Zhang L et al: Trigeminal schwannomas: a report of 42 cases and review of the relevant surgical approaches. Clin Neurol Neurosurg. 111(3):261-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19081670%5Bpmid%5D) +1. [Bonneville F et al: Imaging of cerebellopontine angle lesions: an update. Part 1: enhancing extra-axial lesions. Eur Radiol. 17(10):2472-82, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17562049%5Bpmid%5D) +1. [Bonneville F et al: Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions. Eur Radiol. 17(11):2908-20, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17569053%5Bpmid%5D) +1. [Kiliçkesmez O: Endolymphatic sac tumor in a patient with von Hippel-Lindau disease: MR imaging findings. Diagn Interv Radiol. 12(1):14-6, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16538578%5Bpmid%5D) +1. [Patel NP et al: The radiologic diagnosis of endolymphatic sac tumors. Laryngoscope. 116(1):40-6, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16481807%5Bpmid%5D) +1. [Preece MT et al: Intracranial neurenteric cysts: imaging and pathology spectrum. AJNR Am J Neuroradiol. 27(6):1211-6, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16775266%5Bpmid%5D) +1. [Nelson MD Jr et al: A different approach to cysts of the posterior fossa. Pediatr Radiol. 34(9):720-32, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=15316692%5Bpmid%5D) +1. [Bonneville F et al: Unusual lesions of the cerebellopontine angle: a segmental approach. Radiographics. 21(2):419-38, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11259705%5Bpmid%5D) +1. [Davidson HC et al: MR evaluation of vestibulocochlear anomalies associated with large endolymphatic duct and sac. AJNR Am J Neuroradiol. 20(8):1435-41, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10512225%5Bpmid%5D) +1. [Koeller KK et al: Congenital cystic masses of the neck: radiologic-pathologic correlation. Radiographics. 19(1):121-46; quiz 152-3, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=9925396%5Bpmid%5D) +1. [Lau KY et al: MRI demonstration of subarachnoid neurocysticercosis simulating metastatic disease. Neuroradiology. 40(11):724-6, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9860122%5Bpmid%5D) +1. [Mukherji SK et al: Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients. Radiology. 202(3):801-8, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9051037%5Bpmid%5D) +1. [Tong KA et al: Large vestibular aqueduct syndrome: a genetic disease? AJR Am J Roentgenol. 168(4):1097-101, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9124122%5Bpmid%5D) +1. [Friedman DP et al: Vascular neoplasms and malformations, ischemia, and hemorrhage affecting the spinal cord: MR imaging findings. AJR Am J Roentgenol. 162(3):685-92, 1994](http://www.ncbi.nlm.nih.gov/pubmed/?term=8109522%5Bpmid%5D) +1. [Kollias SS et al: Cystic malformations of the posterior fossa: differential diagnosis clarified through embryologic analysis. Radiographics. 13(6):1211-31, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8031352%5Bpmid%5D) +1. [Smirniotopoulos JG et al: Cerebellopontine angle masses: radiologic-pathologic correlation. Radiographics. 13(5):1131-47, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8210595%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T1 C+ MR reveals a low signal intensity, nonenhancing epidermoid cyst that insinuates into the foramen of Luschka and along the right cerebellar hemisphere . DWI MR sequence would show restricted diffusion. FLAIR would show lack of complete fluid suppression.](images/app.statdx.com_image_thumbnail_9c20ab07-d905-4fe9-825e-f669857ed535_annotated_true_size_900_quality_90_e351d8febf9476ec73cfce123b434851a06e0145.jpg) +**Epidermoid Cyst** +*Axial T1 C+ MR reveals a low signal intensity, nonenhancing epidermoid cyst that insinuates into the foramen of Luschka and along the right cerebellar hemisphere . DWI MR sequence would show restricted diffusion. FLAIR would show lack of complete fluid suppression.* + +![Axial T1 C+ MR reveals a low signal intensity, nonenhancing epidermoid cyst that insinuates into the foramen of Luschka and along the right cerebellar hemisphere . DWI MR sequence would show restricted diffusion. FLAIR would show lack of complete fluid suppression.](images/app.statdx.com_image_thumbnail_9c20ab07-d905-4fe9-825e-f669857ed535_size_174_quality_85_6e8cf09baa5ca522a92db8265f55ecb0c30a7537.jpg) +**Epidermoid Cyst** +*Axial T1 C+ MR reveals a low signal intensity, nonenhancing epidermoid cyst that insinuates into the foramen of Luschka and along the right cerebellar hemisphere . DWI MR sequence would show restricted diffusion. FLAIR would show lack of complete fluid suppression.* + +![Axial T1 C+ FS MR demonstrates a right cerebellopontine angle (CPA) cistern arachnoid cyst displacing the proximal facial and vestibulocochlear nerves anteriorly .](images/app.statdx.com_image_thumbnail_3c4ccfba-a118-420e-b3fe-9c8060624960_annotated_true_size_900_quality_90_196626e9d948a819f4d28a8d5c2cce6415f1af6a.jpg) +**Arachnoid Cyst** +*Axial T1 C+ FS MR demonstrates a right cerebellopontine angle (CPA) cistern arachnoid cyst displacing the proximal facial and vestibulocochlear nerves anteriorly .* + +![Axial T1 C+ MR shows a large enhancing vestibular schwannoma projecting from the internal auditory canal (IAC) into the CPA cistern. The tumor has a large intramural cyst that compresses the brainstem and cerebellum.](images/app.statdx.com_image_thumbnail_7bf8f980-b96a-4a73-8723-f4065bec6928_annotated_true_size_900_quality_90_4865caae16e5a0b266a793cefc08bfe19e56e74c.jpg) +**Vestibular Schwannoma With Intramural Cyst(s)** +*Axial T1 C+ MR shows a large enhancing vestibular schwannoma projecting from the internal auditory canal (IAC) into the CPA cistern. The tumor has a large intramural cyst that compresses the brainstem and cerebellum.* + +![Axial T2 MR shows a heterogeneous CPA mass extending into the left internal auditory canal. Note numerous large intramural tumor cysts .](images/app.statdx.com_image_thumbnail_c0eea3bd-14f3-436b-bd9e-2818f22f0815_annotated_true_size_900_quality_90_a453d30064cbb01de279d0559dfd07ad5bee5732.jpg) +**Vestibular Schwannoma With Intramural Cyst(s)** +*Axial T2 MR shows a heterogeneous CPA mass extending into the left internal auditory canal. Note numerous large intramural tumor cysts .* + +![Axial T1 C+ FS MR shows a superficial, intracerebellar mixed cystic-solid hemangioblastoma projecting into the left CPA cistern area. The solid nodule is avidly enhancing.](images/app.statdx.com_image_thumbnail_d1e63db5-1084-4898-b8fa-f79e0f3ca30a_annotated_true_size_900_quality_90_2bab8529cee1a7b2563c33080b40f35460f8ca1b.jpg) +**Hemangioblastoma** +*Axial T1 C+ FS MR shows a superficial, intracerebellar mixed cystic-solid hemangioblastoma projecting into the left CPA cistern area. The solid nodule is avidly enhancing.* + +![Axial T2WI MR shows a large endolymphatic sac within the posterior wall of the temporal bone. CT (not shown) would reveal a large bony vestibular aqueduct in this patient with large endolymphatic sac anomaly.](images/app.statdx.com_image_thumbnail_b37a1a80-0202-4d7e-ae55-3e9bda0fc7ff_annotated_true_size_900_quality_90_939d6d673c5ef8efc2b3757675d3afc0389b853e.jpg) +**Large Endolymphatic Sac Anomaly (IP-II)** +*Axial T2WI MR shows a large endolymphatic sac within the posterior wall of the temporal bone. CT (not shown) would reveal a large bony vestibular aqueduct in this patient with large endolymphatic sac anomaly.* + +![Axial T1 C+ FS MR demonstrates a cystic mass in the right CPA cistern with a peripherally enhancing wall . Adjacent enhancing, thickened meninges are also seen.](images/app.statdx.com_image_thumbnail_b44f5a1f-4fb3-4472-b46b-f696341e792a_annotated_true_size_900_quality_90_8b1339b20b8eeec3c1fb2431e3a1207e8b4467c2.jpg) +**Neurocysticercosis** +*Axial T1 C+ FS MR demonstrates a cystic mass in the right CPA cistern with a peripherally enhancing wall . Adjacent enhancing, thickened meninges are also seen.* + +![Axial T2WI MR shows a vestibular schwannoma projecting from the IAC into the CPA cistern. An associated (extramural) arachnoid cyst is visible compressing the brainstem and 4th ventricle .](images/app.statdx.com_image_thumbnail_6ce94b8c-5228-4e64-ba8c-73a07f250d9e_annotated_true_size_900_quality_90_054cf049744f0a94edf30b6a0b7dec78fd873573.jpg) +**Vestibular Schwannoma With Extramural Cyst** +*Axial T2WI MR shows a vestibular schwannoma projecting from the IAC into the CPA cistern. An associated (extramural) arachnoid cyst is visible compressing the brainstem and 4th ventricle .* + +![Axial T1 C+ MR reveals an enhancing CPA mass , with intramural cysts, which projected into the IAC but did not involve the labyrinthine segment. Note atrophy of the right platysma muscle from CNVII denervation.](images/app.statdx.com_image_thumbnail_fd005ccd-437a-4a14-9656-afa375f84039_annotated_true_size_900_quality_90_ed15f34e2656f0d3eb999951c956965f618988d6.jpg) +**Facial Nerve Schwannoma in CPA-IAC With Cyst** +*Axial T1 C+ MR reveals an enhancing CPA mass , with intramural cysts, which projected into the IAC but did not involve the labyrinthine segment. Note atrophy of the right platysma muscle from CNVII denervation.* + +![Axial T1 C+ MR demonstrates a large CPA cystic mass "pointing" toward the Meckel cave , providing a clue to the origin of this mass. Note chronic denervation changes of volume loss and fatty infiltration in the left suprazygomatic masticator space compared to the right , partly seen here.](images/app.statdx.com_image_thumbnail_1ab17495-4701-4a87-9d4a-875ce4753c55_annotated_true_size_900_quality_90_e86c6a1ba3b74a7241f2fc52e98f0ef87625e4fd.jpg) +**Schwannoma, Trigeminal, Skull Base** +*Axial T1 C+ MR demonstrates a large CPA cystic mass "pointing" toward the Meckel cave , providing a clue to the origin of this mass. Note chronic denervation changes of volume loss and fatty infiltration in the left suprazygomatic masticator space compared to the right , partly seen here.* + +![Axial T1WI MR shows a small mass anterior to the pontomedullary junction . The neurenteric cyst is well delineated, demonstrating nearly isointense signal to brain, and does not enhance significantly.](images/app.statdx.com_image_thumbnail_a91eb077-bbb8-4541-aadd-8d6b97771d71_annotated_true_size_900_quality_90_e3d2f496364c8a8dd9faf7da8b733ec9a48edf5d.jpg) +**Neurenteric Cyst** +*Axial T1WI MR shows a small mass anterior to the pontomedullary junction . The neurenteric cyst is well delineated, demonstrating nearly isointense signal to brain, and does not enhance significantly.* + +![Axial T2 FS MR reveals a large, sharply marginated lesion expanding the left jugular foramen . The high-signal mass projects medially into the low CPA cistern where it compresses the brainstem .](images/app.statdx.com_image_thumbnail_e02d8050-4c8c-47ab-9dc6-72fb657fc755_annotated_true_size_900_quality_90_84e1aec7a076f0c6e074fbaae5e14193c96e08d3.jpg) +**Jugular Foramen Schwannoma With Intramural Cyst** +*Axial T2 FS MR reveals a large, sharply marginated lesion expanding the left jugular foramen . The high-signal mass projects medially into the low CPA cistern where it compresses the brainstem .* + +![Parasagittal T1 C+ FS MR shows a heterogeneous, cystic enhancing mass in the right CPA . Adjacent brain edema is noted . Although the mass might mimic a cystic schwannoma, the presence of a 2nd enhancing mass and a clinical history of lung cancer helps establish diagnosis of metastasis.](images/app.statdx.com_image_thumbnail_5b3cba8d-233b-4c26-bf69-1bf1724ec332_annotated_true_size_900_quality_90_5e5c0f26dd9bc5d45d0b3e88a6b40564905cdbd5.jpg) +**Cystic Metastasis in CPA** +*Parasagittal T1 C+ FS MR shows a heterogeneous, cystic enhancing mass in the right CPA . Adjacent brain edema is noted . Although the mass might mimic a cystic schwannoma, the presence of a 2nd enhancing mass and a clinical history of lung cancer helps establish diagnosis of metastasis.* + +![Axial high-resolution T2 MR shows a heterogeneous cystic CPA mass . The key to diagnosis is identifying the mass origin from the posterior temporal bone in the expected location of the endolymphatic sac.](images/app.statdx.com_image_thumbnail_c4086a2f-86ac-45ec-8b77-0450dd7a3253_annotated_true_size_900_quality_90_84105a86bfffb5992b08be1debd3af8f6940dc25.jpg) +**Endolymphatic Sac Tumor** +*Axial high-resolution T2 MR shows a heterogeneous cystic CPA mass . The key to diagnosis is identifying the mass origin from the posterior temporal bone in the expected location of the endolymphatic sac.* + + +### Additional Images + +![Axial T1 C+ MR shows an atypical heterogeneously enhancing left vestibular schwannoma with an associated arachnoid cyst .](images/app.statdx.com_image_thumbnail_9f150021-4d87-49a7-925e-4ea3b64654b4_annotated_true_size_900_quality_90_8a9e7d3d2ed808245de9fd1ef4ad27165926f6da.jpg) +**Vestibular Schwannoma With Extramural Cyst** +*Axial T1 C+ MR shows an atypical heterogeneously enhancing left vestibular schwannoma with an associated arachnoid cyst .* + +![Coronal T1 C+ MR shows variant MR case of a jugular foramen schwannoma with intramural cyst and very large cisternal component. The jugular foramen connection is seen on the next image.](images/app.statdx.com_image_thumbnail_24ab3e04-2e79-4dae-8c22-8c416a4660a1_annotated_true_size_900_quality_90_df237998eb576c906901bdfb971e6c95f4d91046.jpg) +**Jugular Foramen Schwannoma With Intramural Cyst** +*Coronal T1 C+ MR shows variant MR case of a jugular foramen schwannoma with intramural cyst and very large cisternal component. The jugular foramen connection is seen on the next image.* + +![Coronal T1 C+ MR reveals an enhancing schwannoma in the jugular foramen . The tumor projects superomedially to fill the CPA cistern and compress the brainstem.](images/app.statdx.com_image_thumbnail_10f067d8-6bbf-4dfc-8fb6-a6519378d65e_annotated_true_size_900_quality_90_9dbda4d9250e0a170600d7abb1287a092d1ebdc2.jpg) +**Jugular Foramen Schwannoma With Intramural Cyst** +*Coronal T1 C+ MR reveals an enhancing schwannoma in the jugular foramen . The tumor projects superomedially to fill the CPA cistern and compress the brainstem.* + +![Axial T1 C+ MR in a patient presenting with left facial pain demonstrates a peripherally enhancing cystic mass in the left CPA with a vector of spread extending towards Meckel cave , that was the clue to its origin.](images/app.statdx.com_image_thumbnail_8e1b3910-0e46-4a50-b25b-a246b6075585_annotated_true_size_900_quality_90_aac5b46dd4eb5e66d40e9bfdf3867afadd041967.jpg) +**Schwannoma, Trigeminal, Skull Base** +*Axial T1 C+ MR in a patient presenting with left facial pain demonstrates a peripherally enhancing cystic mass in the left CPA with a vector of spread extending towards Meckel cave , that was the clue to its origin.* + +![Coronal T1 C+ FS MR shows multiple cysts in the right CPA cistern causing mass effect on the brainstem. Secondary hydrocephalus is present.](images/app.statdx.com_image_thumbnail_325f707f-6cd9-4ff6-b3f4-1afb2531d896_annotated_true_size_900_quality_90_5df80fbfd269508d2e9dbf873fb1f7014a21a8ab.jpg) +**Neurocysticercosis** +*Coronal T1 C+ FS MR shows multiple cysts in the right CPA cistern causing mass effect on the brainstem. Secondary hydrocephalus is present.* + +![Axial T2WI MR reveals an intracerebellar high signal hemangioblastoma projecting into the CPA cistern area. Contrast is required to define enhancing nodule if present.](images/app.statdx.com_image_thumbnail_ab3c32c2-0d27-4ff0-9624-ee4d210b4639_annotated_true_size_900_quality_90_bd21cbeceb2e3e67412b922072d0f0ce9e48cefe.jpg) +**Hemangioblastoma** +*Axial T2WI MR reveals an intracerebellar high signal hemangioblastoma projecting into the CPA cistern area. Contrast is required to define enhancing nodule if present.* + +![Axial T1 C+ MR demonstrates an ovoid enhancing mass in the low CPA cistern . Multiple intramural cysts suggest the diagnosis of schwannoma. Extension into the jugular foramen is evident.](images/app.statdx.com_image_thumbnail_d7deb934-06d4-420e-94e5-31ffeeb65acf_annotated_true_size_900_quality_90_33f2136141742fa30e085373936a82852b2ed232.jpg) +**Jugular Foramen Schwannoma With Intramural Cyst** +*Axial T1 C+ MR demonstrates an ovoid enhancing mass in the low CPA cistern . Multiple intramural cysts suggest the diagnosis of schwannoma. Extension into the jugular foramen is evident.* + +![Axial T1 C+ MR shows nodular soft tissue enhancement along the margins of a cystic prepontine and left CPA epidermoid cyst , due to a rare complication: Malignant degeneration into squamous cell carcinoma.](images/app.statdx.com_image_thumbnail_35f1691e-62c9-498c-b7cf-91f688ef64bd_annotated_true_size_900_quality_90_76f27969c2043b645695c0c222f5b2eda0358039.jpg) +**Epidermoid Cyst** +*Axial T1 C+ MR shows nodular soft tissue enhancement along the margins of a cystic prepontine and left CPA epidermoid cyst , due to a rare complication: Malignant degeneration into squamous cell carcinoma.* + +![Axial T1 C+ MR shows a partly cystic, partly solid mass in the right CPA and posterior Meckel cave . Biopsy supported meningioma, which rarely may have cystic morphologies that mimic schwannoma.](images/app.statdx.com_image_thumbnail_3a0efbd0-7cbc-459a-9138-2b8124db1709_annotated_true_size_900_quality_90_8ebabac71d4ead80a517b96323940e2adbef86ea.jpg) +**Meningioma, Cystic** +*Axial T1 C+ MR shows a partly cystic, partly solid mass in the right CPA and posterior Meckel cave . Biopsy supported meningioma, which rarely may have cystic morphologies that mimic schwannoma.* + diff --git a/docs_md/articles/epilepsy-adult_c936f9e1-b6c6-4c4a-afc6-f2e1a968a7b0.md b/docs_md/articles/epilepsy-adult_c936f9e1-b6c6-4c4a-afc6-f2e1a968a7b0.md index 22e3470..3d67e79 100644 --- a/docs_md/articles/epilepsy-adult_c936f9e1-b6c6-4c4a-afc6-f2e1a968a7b0.md +++ b/docs_md/articles/epilepsy-adult_c936f9e1-b6c6-4c4a-afc6-f2e1a968a7b0.md @@ -123,94 +123,94 @@ breadcrumbs: ### Selected Images -![Coronal NECT shows hyperdense acute hemorrhage in the inferior frontal lobes in a patient with a history of motor vehicle collision. The inferior frontal and anterior temporal lobes are the most common locations for traumatic contusions.](images/app.statdx.com_image_thumbnail_d8928373-1988-431b-ad93-b4649cc33628_size_168_quality_85_ca966ee0_20251014T204349Z.jpg) +![Coronal NECT shows hyperdense acute hemorrhage in the inferior frontal lobes in a patient with a history of motor vehicle collision. The inferior frontal and anterior temporal lobes are the most common locations for traumatic contusions.](images/app.statdx.com_image_thumbnail_d8928373-1988-431b-ad93-b4649cc33628_annotated_true_size_900_quality_90_9d3f64a2b42f10e5a051cd0c4031f8cc77603940.jpg) **Trauma** *Coronal NECT shows hyperdense acute hemorrhage in the inferior frontal lobes in a patient with a history of motor vehicle collision. The inferior frontal and anterior temporal lobes are the most common locations for traumatic contusions.* -![Coronal NECT shows hyperdense acute hemorrhage in the inferior frontal lobes in a patient with a history of motor vehicle collision. The inferior frontal and anterior temporal lobes are the most common locations for traumatic contusions.](images/app.statdx.com_image_thumbnail_d8928373-1988-431b-ad93-b4649cc33628_size_174_quality_85_dbd40ce8.jpg) +![Coronal NECT shows hyperdense acute hemorrhage in the inferior frontal lobes in a patient with a history of motor vehicle collision. The inferior frontal and anterior temporal lobes are the most common locations for traumatic contusions.](images/app.statdx.com_image_thumbnail_d8928373-1988-431b-ad93-b4649cc33628_size_174_quality_85_93e1cbf218133ebeab6f5fa8497eab8967ad5e07.jpg) **Trauma** *Coronal NECT shows hyperdense acute hemorrhage in the inferior frontal lobes in a patient with a history of motor vehicle collision. The inferior frontal and anterior temporal lobes are the most common locations for traumatic contusions.* -![Axial NECT shows loss of gray-white differentiation of the frontal and temporal operculum as well as the insular cortex consistent with infarct . Note the hyperdensity in the sylvian fissure from thrombosed middle cerebral artery (MCA) branches .](images/app.statdx.com_image_thumbnail_bfbb1626-c8fd-4e2a-8ec6-fe3428a3719f_size_168_quality_85_fd8b83c0_20251014T204349Z.jpg) +![Axial NECT shows loss of gray-white differentiation of the frontal and temporal operculum as well as the insular cortex consistent with infarct . Note the hyperdensity in the sylvian fissure from thrombosed middle cerebral artery (MCA) branches .](images/app.statdx.com_image_thumbnail_bfbb1626-c8fd-4e2a-8ec6-fe3428a3719f_annotated_true_size_900_quality_90_a3150f4840bb3e0b1535c33b3a8d2f5ae966a8e6.jpg) **Stroke** *Axial NECT shows loss of gray-white differentiation of the frontal and temporal operculum as well as the insular cortex consistent with infarct . Note the hyperdensity in the sylvian fissure from thrombosed middle cerebral artery (MCA) branches .* -![Sagittal T1 C+ MR shows abnormal enhancement in the suprasellar and prepontine cistern , inferior frontal lobe , and quadrigeminal plate cistern , consistent with tuberculomas. CNS tuberculosis is the most common cause of seizures from infection worldwide.](images/app.statdx.com_image_thumbnail_46b5932b-7e89-4fe6-bb70-3e1fe7f07d42_size_168_quality_85_57bde864_20251014T204349Z.jpg) +![Sagittal T1 C+ MR shows abnormal enhancement in the suprasellar and prepontine cistern , inferior frontal lobe , and quadrigeminal plate cistern , consistent with tuberculomas. CNS tuberculosis is the most common cause of seizures from infection worldwide.](images/app.statdx.com_image_thumbnail_46b5932b-7e89-4fe6-bb70-3e1fe7f07d42_annotated_true_size_900_quality_90_3939bbebe3f2155839b2189dfde6ac4109cac147.jpg) **Infection** *Sagittal T1 C+ MR shows abnormal enhancement in the suprasellar and prepontine cistern , inferior frontal lobe , and quadrigeminal plate cistern , consistent with tuberculomas. CNS tuberculosis is the most common cause of seizures from infection worldwide.* -![Axial b=1000 DWI MR shows increased signal in the tail of the hippocampi , medial thalami , insular cortex , and cingulate cortex in a patient with hyperammonemia from hepatic encephalopathy.](images/app.statdx.com_image_thumbnail_af3507db-e71b-45e1-b538-155678f68c36_size_168_quality_85_3b861d49_20251014T204349Z.jpg) +![Axial b=1000 DWI MR shows increased signal in the tail of the hippocampi , medial thalami , insular cortex , and cingulate cortex in a patient with hyperammonemia from hepatic encephalopathy.](images/app.statdx.com_image_thumbnail_af3507db-e71b-45e1-b538-155678f68c36_annotated_true_size_900_quality_90_04ec6f99c3faa513312ce572f6bcebe2ee96de3d.jpg) **Metabolic** *Axial b=1000 DWI MR shows increased signal in the tail of the hippocampi , medial thalami , insular cortex , and cingulate cortex in a patient with hyperammonemia from hepatic encephalopathy.* -![Axial T1 C+ MR shows an irregular ring-enhancing mass in the left medial temporal lobe and occipital lobe. This was a glioblastoma, IDH-wildtype at biopsy.](images/app.statdx.com_image_thumbnail_9b80b063-2a68-44e5-9cc4-784e3b34fd0f_size_168_quality_85_e073a8af_20251014T204349Z.jpg) +![Axial T1 C+ MR shows an irregular ring-enhancing mass in the left medial temporal lobe and occipital lobe. This was a glioblastoma, IDH-wildtype at biopsy.](images/app.statdx.com_image_thumbnail_9b80b063-2a68-44e5-9cc4-784e3b34fd0f_annotated_true_size_900_quality_90_54012bfe16e5ba1d0d4bc209652c9f91c9e8dab0.jpg) **Neoplasms** *Axial T1 C+ MR shows an irregular ring-enhancing mass in the left medial temporal lobe and occipital lobe. This was a glioblastoma, IDH-wildtype at biopsy.* -![Axial FLAIR MR shows a large, T2-hyperintense mass in the left frontal lobe extending across the corpus callosum to the right frontal lobe and centrally to involve the basal ganglia. This was a 1p/19q co-deleted oligodendroglioma at biopsy. These tumors are often calcified and located in the frontal lobe.](9d79274b-c751-481d-8593-de92a88444fd) +![Axial FLAIR MR shows a large, T2-hyperintense mass in the left frontal lobe extending across the corpus callosum to the right frontal lobe and centrally to involve the basal ganglia. This was a 1p/19q co-deleted oligodendroglioma at biopsy. These tumors are often calcified and located in the frontal lobe.](images/app.statdx.com_image_thumbnail_9d79274b-c751-481d-8593-de92a88444fd_annotated_true_size_900_quality_90_35c3c5763268747a6630f3aea59d66b84c616533.jpg) **Neoplasms** *Axial FLAIR MR shows a large, T2-hyperintense mass in the left frontal lobe extending across the corpus callosum to the right frontal lobe and centrally to involve the basal ganglia. This was a 1p/19q co-deleted oligodendroglioma at biopsy. These tumors are often calcified and located in the frontal lobe.* -![Coronal FLAIR MR shows increased T2 signal and relative volume loss of the right hippocampal formation , consistent with mesial temporal sclerosis in this patient with temporal lobe seizures.](images/app.statdx.com_image_thumbnail_9644fd19-2c08-493a-b6c0-86829d88616e_size_168_quality_85_351aab5c_20251014T204349Z.jpg) +![Coronal FLAIR MR shows increased T2 signal and relative volume loss of the right hippocampal formation , consistent with mesial temporal sclerosis in this patient with temporal lobe seizures.](images/app.statdx.com_image_thumbnail_9644fd19-2c08-493a-b6c0-86829d88616e_annotated_true_size_900_quality_90_5979947690f04584025a85cf3ad75ed5d115b4b5.jpg) **Mesial Temporal Sclerosis** *Coronal FLAIR MR shows increased T2 signal and relative volume loss of the right hippocampal formation , consistent with mesial temporal sclerosis in this patient with temporal lobe seizures.* -![Axial FLAIR MR shows bilateral T2 hyperintensity in the occipital lobe cortex and subcortical white matter. In this patient with hypertension and renal failure, PRES was diagnosed. The DWI images were negative. Imaging of PRES often completely resolves when hypertension is controlled.](4017a3e8-d8cf-4b15-bee3-e7fe81e93fc0) +![Axial FLAIR MR shows bilateral T2 hyperintensity in the occipital lobe cortex and subcortical white matter. In this patient with hypertension and renal failure, PRES was diagnosed. The DWI images were negative. Imaging of PRES often completely resolves when hypertension is controlled.](images/app.statdx.com_image_thumbnail_4017a3e8-d8cf-4b15-bee3-e7fe81e93fc0_annotated_true_size_900_quality_90_a084bc401580225361a21f44b7f9bda686739dbe.jpg) **Posterior Reversible Encephalopathy Syndrome** *Axial FLAIR MR shows bilateral T2 hyperintensity in the occipital lobe cortex and subcortical white matter. In this patient with hypertension and renal failure, PRES was diagnosed. The DWI images were negative. Imaging of PRES often completely resolves when hypertension is controlled.* ### Additional Images -![Coronal T1 C+ MR shows central heterogeneous enhancement of a low- intensity tumor involving the cortex of the posterior frontal lobe, consistent with oligodendroglioma.](images/app.statdx.com_image_thumbnail_ac3fd4f1-6d7c-4979-8c8e-22ec6d3c7e27_size_168_quality_85_0be67a4a_20251014T204349Z.jpg) +![Coronal T1 C+ MR shows central heterogeneous enhancement of a low- intensity tumor involving the cortex of the posterior frontal lobe, consistent with oligodendroglioma.](images/app.statdx.com_image_thumbnail_ac3fd4f1-6d7c-4979-8c8e-22ec6d3c7e27_annotated_true_size_900_quality_90_51a6ca9cf6d271b58519be9cb9b83d22212905ce.jpg) **Oligodendroglioma, IDH-Mutant and 1p/19q-Co-Deleted** *Coronal T1 C+ MR shows central heterogeneous enhancement of a low- intensity tumor involving the cortex of the posterior frontal lobe, consistent with oligodendroglioma.* -![Axial FLAIR MR shows bilateral hyperintensity of the hippocampi and medial temporal lobes . In this patient with a history of lung cancer, this is consistent with autoimmune encephalitis.](images/app.statdx.com_image_thumbnail_e08a1aef-9ebb-4a33-8ea7-4d7c44b75c73_size_168_quality_85_8b30e647_20251014T204349Z.jpg) +![Axial FLAIR MR shows bilateral hyperintensity of the hippocampi and medial temporal lobes . In this patient with a history of lung cancer, this is consistent with autoimmune encephalitis.](images/app.statdx.com_image_thumbnail_e08a1aef-9ebb-4a33-8ea7-4d7c44b75c73_annotated_true_size_900_quality_90_6bb839f0acae4d68f9844dbdcbdad055f2c8cb65.jpg) **Paraneoplastic and Autoimmune Encephalitis** *Axial FLAIR MR shows bilateral hyperintensity of the hippocampi and medial temporal lobes . In this patient with a history of lung cancer, this is consistent with autoimmune encephalitis.* -![Axial FLAIR MR shows bilateral hyperintensity of the insular cortex in this patient with ovarian cancer, consistent with autoimmune paraneoplastic encephalitis.](ec2a7de9-065a-4e3e-bd42-0b1af21c97a5) +![Axial FLAIR MR shows bilateral hyperintensity of the insular cortex in this patient with ovarian cancer, consistent with autoimmune paraneoplastic encephalitis.](images/app.statdx.com_image_thumbnail_ec2a7de9-065a-4e3e-bd42-0b1af21c97a5_annotated_true_size_900_quality_90_05fe64b7c0c68744842ce9f469ef6b39845e15cf.jpg) **Paraneoplastic and Autoimmune Encephalitis** *Axial FLAIR MR shows bilateral hyperintensity of the insular cortex in this patient with ovarian cancer, consistent with autoimmune paraneoplastic encephalitis.* -![Coronal FLAIR MR shows a cortical hyperintense mass in the posterior frontal lobe with a focal cyst . This was a pleomorphic xanthoastrocytoma at surgery.](1345d1aa-7c33-4b8b-954c-cfe17b967da3) +![Coronal FLAIR MR shows a cortical hyperintense mass in the posterior frontal lobe with a focal cyst . This was a pleomorphic xanthoastrocytoma at surgery.](images/app.statdx.com_image_thumbnail_1345d1aa-7c33-4b8b-954c-cfe17b967da3_annotated_true_size_900_quality_90_6330c8776862aac3fb298cb20cbd7501b6be2edd.jpg) **Pleomorphic Xanthoastrocytoma** *Coronal FLAIR MR shows a cortical hyperintense mass in the posterior frontal lobe with a focal cyst . This was a pleomorphic xanthoastrocytoma at surgery.* -![Sagittal T2 MR shows a well-circumscribed T2-hyperintense mass involving the posterior frontal cortex . This is a typical location and appearance for oligodendroglioma. 70-90% of patients with this tumor present with seizures due to its cortical nature.](d55a6a70-1a01-44e0-8ca2-38abc0981c88) +![Sagittal T2 MR shows a well-circumscribed T2-hyperintense mass involving the posterior frontal cortex . This is a typical location and appearance for oligodendroglioma. 70-90% of patients with this tumor present with seizures due to its cortical nature.](images/app.statdx.com_image_thumbnail_d55a6a70-1a01-44e0-8ca2-38abc0981c88_annotated_true_size_900_quality_90_7b2315e25dcb5fedff27ea984ff304dde28c2ced.jpg) **Oligodendroglioma, IDH-Mutant and 1p/19q-Co-Deleted** *Sagittal T2 MR shows a well-circumscribed T2-hyperintense mass involving the posterior frontal cortex . This is a typical location and appearance for oligodendroglioma. 70-90% of patients with this tumor present with seizures due to its cortical nature.* -![Coronal FLAIR MR shows atrophy and hyperintensity of the left hippocampus , consistent with left mesial temporal sclerosis. There is also loss of the normal internal architecture of the left hippocampus and ex vacuo dilatation of the left temporal horn .](aa0b092b-064f-459a-9efd-8f878004a054) +![Coronal FLAIR MR shows atrophy and hyperintensity of the left hippocampus , consistent with left mesial temporal sclerosis. There is also loss of the normal internal architecture of the left hippocampus and ex vacuo dilatation of the left temporal horn .](images/app.statdx.com_image_thumbnail_aa0b092b-064f-459a-9efd-8f878004a054_annotated_true_size_900_quality_90_47ae1e04718e72c5c666d8ebc20881bc454c737f.jpg) **Mesial Temporal Sclerosis** *Coronal FLAIR MR shows atrophy and hyperintensity of the left hippocampus , consistent with left mesial temporal sclerosis. There is also loss of the normal internal architecture of the left hippocampus and ex vacuo dilatation of the left temporal horn .* -![Axial CBF map from arterial spin labeling shows relative hypoperfusion of the left temporal lobe compared to the right in this patient with left mesial temporal sclerosis. This is consistent with interictal seizure focus.](6de5dc2d-fa1c-49db-98ed-a3fda780dba6) +![Axial CBF map from arterial spin labeling shows relative hypoperfusion of the left temporal lobe compared to the right in this patient with left mesial temporal sclerosis. This is consistent with interictal seizure focus.](images/app.statdx.com_image_thumbnail_6de5dc2d-fa1c-49db-98ed-a3fda780dba6_annotated_true_size_900_quality_90_21cf153d7f2db6772912ee0ddf405fac575924b3.jpg) **Mesial Temporal Sclerosis** *Axial CBF map from arterial spin labeling shows relative hypoperfusion of the left temporal lobe compared to the right in this patient with left mesial temporal sclerosis. This is consistent with interictal seizure focus.* -![Axial T1 C+ MR shows a cyst and heterogeneously enhancing nodule involving the cortex. There is an incidental developmental venous anomaly .](7e9f029e-0fb4-48da-afd3-cf5b752062b0) +![Axial T1 C+ MR shows a cyst and heterogeneously enhancing nodule involving the cortex. There is an incidental developmental venous anomaly .](images/app.statdx.com_image_thumbnail_7e9f029e-0fb4-48da-afd3-cf5b752062b0_annotated_true_size_900_quality_90_f78c09c7cde5dc95c258b129326708f514dff2dd.jpg) **Pleomorphic Xanthoastrocytoma** *Axial T1 C+ MR shows a cyst and heterogeneously enhancing nodule involving the cortex. There is an incidental developmental venous anomaly .* -![Coronal FLAIR MR shows hyperintensity and swelling of the right hippocampus and bilateral parahippocampal gyri . In a patient with acute encephalopathy, seizures, and fever, herpes encephalitis must be excluded.](acb5e817-1a3c-4979-bdcd-be26118da2ec) +![Coronal FLAIR MR shows hyperintensity and swelling of the right hippocampus and bilateral parahippocampal gyri . In a patient with acute encephalopathy, seizures, and fever, herpes encephalitis must be excluded.](images/app.statdx.com_image_thumbnail_acb5e817-1a3c-4979-bdcd-be26118da2ec_annotated_true_size_900_quality_90_66a015a2520620df0f8bc53973c3da166de666fb.jpg) **Infection** *Coronal FLAIR MR shows hyperintensity and swelling of the right hippocampus and bilateral parahippocampal gyri . In a patient with acute encephalopathy, seizures, and fever, herpes encephalitis must be excluded.* -![Axial T2 FS MR shows bilateral hippocampal hyperintensity and edema . Herpes encephalitis typically involves the medial temporal lobes asymmetrically and the insular cortex. The basal ganglia is usually spared, and there is deceased diffusion of the cortex early in the disease.](faeb3244-f4ae-42cd-b75f-6d90c365ee6a) +![Axial T2 FS MR shows bilateral hippocampal hyperintensity and edema . Herpes encephalitis typically involves the medial temporal lobes asymmetrically and the insular cortex. The basal ganglia is usually spared, and there is deceased diffusion of the cortex early in the disease.](images/app.statdx.com_image_thumbnail_faeb3244-f4ae-42cd-b75f-6d90c365ee6a_annotated_true_size_900_quality_90_f15dddcdd73372dcb6845c71729ea126c260f469.jpg) **Infection** *Axial T2 FS MR shows bilateral hippocampal hyperintensity and edema . Herpes encephalitis typically involves the medial temporal lobes asymmetrically and the insular cortex. The basal ganglia is usually spared, and there is deceased diffusion of the cortex early in the disease.* -![Axial NECT shows a heterogeneous mass causing a seizure in the right frontal lobe extending to the basal ganglia with midline shift. There are areas of hyperdensity suggesting a high-grade neoplasm. This was a glioblastoma at biopsy.](efaf4f17-91c0-47d6-b338-13ddefefb91c) +![Axial NECT shows a heterogeneous mass causing a seizure in the right frontal lobe extending to the basal ganglia with midline shift. There are areas of hyperdensity suggesting a high-grade neoplasm. This was a glioblastoma at biopsy.](images/app.statdx.com_image_thumbnail_efaf4f17-91c0-47d6-b338-13ddefefb91c_annotated_true_size_900_quality_90_81bfdb8370c68579700ac3813fe20474034a3d5f.jpg) **Neoplasms** *Axial NECT shows a heterogeneous mass causing a seizure in the right frontal lobe extending to the basal ganglia with midline shift. There are areas of hyperdensity suggesting a high-grade neoplasm. This was a glioblastoma at biopsy.* -![Axial FLAIR MR in this patient with ovarian cancer shows T2 hyperintensity of the temporal lobes , consistent with autoimmune, paraneoplastic, limbic encephalitis. Compared to herpes encephalitis, autoimmune encephalitis is more likely to be bilateral, symmetric without decreased diffusion. The basal ganglia are more commonly involved.](01f65532-afcb-4ef7-b05b-25b67af8c6da) +![Axial FLAIR MR in this patient with ovarian cancer shows T2 hyperintensity of the temporal lobes , consistent with autoimmune, paraneoplastic, limbic encephalitis. Compared to herpes encephalitis, autoimmune encephalitis is more likely to be bilateral, symmetric without decreased diffusion. The basal ganglia are more commonly involved.](images/app.statdx.com_image_thumbnail_01f65532-afcb-4ef7-b05b-25b67af8c6da_annotated_true_size_900_quality_90_4120485c3ffbf36824354f7289bdc9b57f132926.jpg) **Paraneoplastic and Autoimmune Encephalitis** *Axial FLAIR MR in this patient with ovarian cancer shows T2 hyperintensity of the temporal lobes , consistent with autoimmune, paraneoplastic, limbic encephalitis. Compared to herpes encephalitis, autoimmune encephalitis is more likely to be bilateral, symmetric without decreased diffusion. The basal ganglia are more commonly involved.* -![Axial FLAIR MR shows bilateral T2 hyperintensity in the parietal lobe cortex and subcortical white matter. In this patient with malignant hypertension, PRES was suspected.](0a69bb1c-f819-4ebe-a267-afb05719b3ca) +![Axial FLAIR MR shows bilateral T2 hyperintensity in the parietal lobe cortex and subcortical white matter. In this patient with malignant hypertension, PRES was suspected.](images/app.statdx.com_image_thumbnail_0a69bb1c-f819-4ebe-a267-afb05719b3ca_annotated_true_size_900_quality_90_fea1af25c06b7b365a624ac7aa4d437cbd615e2e.jpg) **Posterior Reversible Encephalopathy Syndrome** *Axial FLAIR MR shows bilateral T2 hyperintensity in the parietal lobe cortex and subcortical white matter. In this patient with malignant hypertension, PRES was suspected.* diff --git a/docs_md/articles/fusiform-arterial-enlargement_31d50b93-b057-4da3-86b5-4cc8fb0bc806.md b/docs_md/articles/fusiform-arterial-enlargement_31d50b93-b057-4da3-86b5-4cc8fb0bc806.md index d6583dc..3e3302d 100644 --- a/docs_md/articles/fusiform-arterial-enlargement_31d50b93-b057-4da3-86b5-4cc8fb0bc806.md +++ b/docs_md/articles/fusiform-arterial-enlargement_31d50b93-b057-4da3-86b5-4cc8fb0bc806.md @@ -119,19 +119,23 @@ breadcrumbs: ### Selected Images -![Axial CT shows fusiform dilatation and tortuosity of the basilar artery in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.](images/app.statdx.com_image_thumbnail_38e1497f-31e3-49db-8b46-4ab561bf1eac_annotated_true_size_900_quality_90_736abe5f4de2b9d4c525081213fb245efd7b5c64.jpg) +![Axial CT shows fusiform dilatation and tortuosity of the basilar artery in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.](images/app.statdx.com_image_38e1497f-31e3-49db-8b46-4ab561bf1eac_fb437f8b_20251018T080401Z.jpg) **Dolichoectasia** *Axial CT shows fusiform dilatation and tortuosity of the basilar artery in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.* -![Axial CT shows fusiform dilatation and tortuosity of the basilar artery in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.](images/app.statdx.com_image_thumbnail_38e1497f-31e3-49db-8b46-4ab561bf1eac_size_174_quality_85_24eac592d5fc1ee7f252fa6ad07576792d231936.jpg) +![Axial CT shows fusiform dilatation and tortuosity of the basilar artery in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.](images/app.statdx.com_image_thumbnail_38e1497f-31e3-49db-8b46-4ab561bf1eac_size_168_quality_85_e3c2ebff_20251018T080328Z.jpg) **Dolichoectasia** *Axial CT shows fusiform dilatation and tortuosity of the basilar artery in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.* -![Coronal CTA shows fusiform dilatation of the right supraclinoid internal carotid artery (ICA). Irregularity from atherosclerotic disease can be seen of the M1 segment of the middle cerebral artery . No significant mural thrombus was noted in this fusiform aneurysm.](images/app.statdx.com_image_thumbnail_f63a9221-71c6-4320-af66-1ed581202eb9_annotated_true_size_900_quality_90_2dc4da92e68e6a19e1643376a347ab007f8ccfd5.jpg) +![Axial CT shows fusiform dilatation and tortuosity of the basilar artery in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.](images/app.statdx.com_image_thumbnail_38e1497f-31e3-49db-8b46-4ab561bf1eac_size_174_quality_85_9fba439d_20251018T080313Z.jpg) +**Dolichoectasia** +*Axial CT shows fusiform dilatation and tortuosity of the basilar artery in an octogenarian related to dolichoectasia. Fusiform dolichoectasia is a common finding in the vertebrobasilar arteries in older patients.* + +![Coronal CTA shows fusiform dilatation of the right supraclinoid internal carotid artery (ICA). Irregularity from atherosclerotic disease can be seen of the M1 segment of the middle cerebral artery . No significant mural thrombus was noted in this fusiform aneurysm.](images/app.statdx.com_image_thumbnail_f63a9221-71c6-4320-af66-1ed581202eb9_size_168_quality_85_8a3ae196_20251018T080328Z.jpg) **Atherosclerotic Fusiform Aneurysm** *Coronal CTA shows fusiform dilatation of the right supraclinoid internal carotid artery (ICA). Irregularity from atherosclerotic disease can be seen of the M1 segment of the middle cerebral artery . No significant mural thrombus was noted in this fusiform aneurysm.* -![Dissecting pseudoaneurysm in the V4 segment of the right vertebral artery seen on 3D TOF MRA , T2 , and T1 pre- and post DANTE VWI sequences shows peripheral enhancement, suggestive of instability.](images/app.statdx.com_image_thumbnail_68b41ae0-9f3a-4484-8b02-69d6772626db_annotated_true_size_900_quality_90_e912e6a241bbefcc9c65c9e56f112aec17a1b602.jpg) +![Dissecting pseudoaneurysm in the V4 segment of the right vertebral artery seen on 3D TOF MRA , T2 , and T1 pre- and post DANTE VWI sequences shows peripheral enhancement, suggestive of instability.](images/app.statdx.com_image_thumbnail_68b41ae0-9f3a-4484-8b02-69d6772626db_size_168_quality_85_8d79e53e_20251018T080328Z.jpg) **Dissecting Aneurysm/Pseudoaneurysm** *Dissecting pseudoaneurysm in the V4 segment of the right vertebral artery seen on 3D TOF MRA , T2 , and T1 pre- and post DANTE VWI sequences shows peripheral enhancement, suggestive of instability.* @@ -139,7 +143,7 @@ breadcrumbs: **Ehlers-Danlos** *3D MIP MRA of the vertebrobasilar arteries in a teenage female with a history of type 4 Ehlers-Danlos shows fusiform dilatation of the vertebral artery . The affected gene is COL3A1, and this specific type of Ehlers-Danlos has a higher risk of aneurysm and vascular rupture.* -![Axial MIP from CT arteriography shows fusiform dilatation of the left middle cerebral artery bifurcation in this child with a history of Marfan syndrome.](9c20720f-93e4-49ad-b302-93e2d3e03a65) +![Axial MIP from CT arteriography shows fusiform dilatation of the left middle cerebral artery bifurcation in this child with a history of Marfan syndrome.](images/app.statdx.com_image_thumbnail_9c20720f-93e4-49ad-b302-93e2d3e03a65_size_168_quality_85_2e4b9fef_20251018T080329Z.jpg) **Marfan Syndrome** *Axial MIP from CT arteriography shows fusiform dilatation of the left middle cerebral artery bifurcation in this child with a history of Marfan syndrome.* @@ -147,34 +151,34 @@ breadcrumbs: **Familial Thoracic Aneurysm &/or Dissection** *Coronal MIP reformat from CT arteriography shows bilateral fusiform aneurysms of supraclinoid ICAs . This patient also had thoracic aortic aneurysm, which is consistent with familial thoracic aortic aneurysm and dissection and is associated with a mutation of ACTA2. This gene is responsible for a component of vascular smooth muscle.* -![Axial T2WI MR shows strikingly enlarged middle cerebral arteries in this child with congenital HIV/AIDS (an uncommon but well-recognized cause of pediatric fusiform arteriopathy). The stroke-like presentations of HIV infection may relate to vasculopathies, including large-vessel aneurysmal vasculopathy.](b62dcdb8-45c4-477b-a466-98935b57a9f5) +![Axial T2WI MR shows strikingly enlarged middle cerebral arteries in this child with congenital HIV/AIDS (an uncommon but well-recognized cause of pediatric fusiform arteriopathy). The stroke-like presentations of HIV infection may relate to vasculopathies, including large-vessel aneurysmal vasculopathy.](images/app.statdx.com_image_thumbnail_b62dcdb8-45c4-477b-a466-98935b57a9f5_size_168_quality_85_562a643f_20251018T080329Z.jpg) **HIV Infection** *Axial T2WI MR shows strikingly enlarged middle cerebral arteries in this child with congenital HIV/AIDS (an uncommon but well-recognized cause of pediatric fusiform arteriopathy). The stroke-like presentations of HIV infection may relate to vasculopathies, including large-vessel aneurysmal vasculopathy.* -![Axial NECT demonstrates a giant serpentine aneurysm in the basilar artery with associated mural thrombus seen on sagittal CTA.](8fb2594c-2abc-43d9-84bc-b8b67664090e) +![Axial NECT demonstrates a giant serpentine aneurysm in the basilar artery with associated mural thrombus seen on sagittal CTA.](images/app.statdx.com_image_thumbnail_8fb2594c-2abc-43d9-84bc-b8b67664090e_size_168_quality_85_e273dd7e_20251018T080329Z.jpg) **Giant Serpentine Aneurysm** *Axial NECT demonstrates a giant serpentine aneurysm in the basilar artery with associated mural thrombus seen on sagittal CTA.* ### Additional Images -![Sagittal T1WI MR shows an elongated basilar artery with a slow-flow, thickened wall . The apex of the tortuous basilar artery indents the hypothalamus, 3rd ventricle .](images/app.statdx.com_image_thumbnail_5a50f257-ef7f-4680-94e0-670c586154aa_annotated_true_size_900_quality_90_8128efea5aa0f5f7258343bba032fc12f4dda32c.jpg) +![Sagittal T1WI MR shows an elongated basilar artery with a slow-flow, thickened wall . The apex of the tortuous basilar artery indents the hypothalamus, 3rd ventricle .](images/app.statdx.com_image_thumbnail_5a50f257-ef7f-4680-94e0-670c586154aa_size_168_quality_85_75bc027c_20251018T080401Z.jpg) **Dolichoectasia** *Sagittal T1WI MR shows an elongated basilar artery with a slow-flow, thickened wall . The apex of the tortuous basilar artery indents the hypothalamus, 3rd ventricle .* -![Axial T2WI MR shows an elongated, tortuous basilar artery with a thickened arterial wall , typical for atherosclerosis-associated fusiform ectasia.](images/app.statdx.com_image_thumbnail_b0b88e99-082c-43e8-88d1-21dbf84262c4_annotated_true_size_900_quality_90_21b6c5512739d034e843a08379f41083777fa91e.jpg) +![Axial T2WI MR shows an elongated, tortuous basilar artery with a thickened arterial wall , typical for atherosclerosis-associated fusiform ectasia.](images/app.statdx.com_image_thumbnail_b0b88e99-082c-43e8-88d1-21dbf84262c4_size_168_quality_85_7b35a214_20251018T080401Z.jpg) **Dolichoectasia** *Axial T2WI MR shows an elongated, tortuous basilar artery with a thickened arterial wall , typical for atherosclerosis-associated fusiform ectasia.* -![Lateral angiography shows a large fusiform middle cerebral artery aneurysm that extends into smaller, more distal branches . This is an unusual example because of the location (ICA, middle cerebral artery).](images/app.statdx.com_image_thumbnail_5b2eae83-b875-4f2c-9903-07a167a394a0_annotated_true_size_900_quality_90_f3c9b05228b58159489c082f0f8dea94c83b602b.jpg) +![Lateral angiography shows a large fusiform middle cerebral artery aneurysm that extends into smaller, more distal branches . This is an unusual example because of the location (ICA, middle cerebral artery).](5b2eae83-b875-4f2c-9903-07a167a394a0) **Atherosclerotic Fusiform Aneurysm** *Lateral angiography shows a large fusiform middle cerebral artery aneurysm that extends into smaller, more distal branches . This is an unusual example because of the location (ICA, middle cerebral artery).* -![Axial T1WI MR shows an enlarged right vertebral artery with high signal intensity as well as an absent flow void of the left vertebral artery .](images/app.statdx.com_image_thumbnail_c349506a-b897-4a04-9191-85e5090f0d6e_annotated_true_size_900_quality_90_9a3e27aaefea8cccd9e59bda31b9339862cc3bed.jpg) +![Axial T1WI MR shows an enlarged right vertebral artery with high signal intensity as well as an absent flow void of the left vertebral artery .](images/app.statdx.com_image_thumbnail_c349506a-b897-4a04-9191-85e5090f0d6e_size_168_quality_85_37c748bd_20251018T080328Z.jpg) **Nonaneurysmal Dissection** *Axial T1WI MR shows an enlarged right vertebral artery with high signal intensity as well as an absent flow void of the left vertebral artery .* -![Anteroposterior oblique view of the left vertebral angiogram shows focal elongations and widening of the basilar artery in a 6-year-old child with Ehlers-Danlos type 4.](184ad0e5-e723-4b46-babc-4d119e6e3cab) +![Anteroposterior oblique view of the left vertebral angiogram shows focal elongations and widening of the basilar artery in a 6-year-old child with Ehlers-Danlos type 4.](images/app.statdx.com_image_thumbnail_184ad0e5-e723-4b46-babc-4d119e6e3cab_size_168_quality_85_0cd5001e_20251018T080329Z.jpg) **Ehlers-Danlos Syndrome** *Anteroposterior oblique view of the left vertebral angiogram shows focal elongations and widening of the basilar artery in a 6-year-old child with Ehlers-Danlos type 4.* @@ -182,7 +186,7 @@ breadcrumbs: **Giant Serpentine Aneurysm** *Axial MRA submentovertex view shows an unusual nonatherosclerotic giant serpentine fusiform aneurysm. The patent channel lies within the clot in the partially thrombosed lumen.* -![Lateral angiography in 30-year-old man with a subarachnoid hemorrhage shows an elongated, bizarre-appearing, multilobulated aneurysm with long aspect ratio, tit-like projections.](9ca263bc-486c-4775-b315-df4cbc180a32) +![Lateral angiography in 30-year-old man with a subarachnoid hemorrhage shows an elongated, bizarre-appearing, multilobulated aneurysm with long aspect ratio, tit-like projections.](images/app.statdx.com_image_thumbnail_9ca263bc-486c-4775-b315-df4cbc180a32_size_168_quality_85_2d099724_20251018T080355Z.jpg) **Atypical Saccular Aneurysm** *Lateral angiography in 30-year-old man with a subarachnoid hemorrhage shows an elongated, bizarre-appearing, multilobulated aneurysm with long aspect ratio, tit-like projections.* diff --git a/docs_md/articles/hemifacial-spasm_1b390143-1212-4447-beb3-ed9e85ef34e4.md b/docs_md/articles/hemifacial-spasm_1b390143-1212-4447-beb3-ed9e85ef34e4.md new file mode 100644 index 0000000..21dc559 --- /dev/null +++ b/docs_md/articles/hemifacial-spasm_1b390143-1212-4447-beb3-ed9e85ef34e4.md @@ -0,0 +1,304 @@ +--- +title: "Hemifacial Spasm" +docid: "1b390143-1212-4447-beb3-ed9e85ef34e4" +authors: + - key: "eef2f839-5706-47b9-89c3-60d8315b2b3a" + value: "Nicholas A. Koontz, MD" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c" + - + name: "CPA-IAC and Posterior Fossa" + slug: "cpa-iac-and-posterior-fossa" + treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992" + - + name: "Clinically Based Differentials" + slug: "clinically-based-differentials" + treeNodeId: "55dd15ac-e67d-48dd-8134-f52884dab28b" + - + name: "Hemifacial Spasm" + slug: "hemifacial-spasm" + treeNodeId: null +category: "Head and Neck" +documentVersionId: "959076b8-9c94-4244-89b0-0721f3a2387b" +imageCount: 23 +lastUpdated: "08/15/18" +pageDescription: "Hemifacial Spasm" +pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Clinically Based Differentials, Hemifacial Spasm" +pageTitle: "Hemifacial Spasm | STATdx" +enhancedTitle: "Hemifacial Spasm" +type: "DDX" +references: true +breadcrumbs: + - "Head and Neck" + - "Differential Diagnosis" + - "CPA-IAC and Posterior Fossa" + - "Clinically Based Differentials" + - "Hemifacial Spasm" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Overall statistics + - In > 95% of cases, **arterial vascular loop** is cause of hemifacial spasm (HFS) + - All other causes listed account for < 5% of cases + - HFS + - Definition: Segmental myoclonus of muscles of face innervated by facial nerve + - Presentation: Patients 50-80 years old, unilateral + - Begins around eye, spreads gradually to other facial muscles + - Principal symptom: Rhythmic, involuntary, myoclonic facial muscle contractions + - Pathophysiology: Irritation of facial nerve or facial nucleus + - Vascular loop syndrome affecting CNVII (aka pimary HFS) + - By far most common cause of HFS + - Aberrant or ectatic vessels in cistern + - Anterior inferior cerebellar artery (AICA) most common offending artery (40-50%) + - Other less common causal vessels include posterior inferior cerebellar artery (PICA) (~ 30%), vertebral artery (VA) (~ 20%), or large vein (< 5%) + - Multivessel impingement is frequent (~ 40%) + - High-resolution MR-MRA routinely identifies compressive aberrant or ectatic arteries + - T2 SPACE, CISS, or FIESTA sequences most commonly employed + - Critical to recognize that vascular contact of facial nerve is very common (~ 50% of population) with only tiny minority (< 0.01 %) manifesting HFS + - **In absence of HFS symptoms, this is incidental finding that should be ignored** +- ## Helpful Clues for Common Diagnoses + + + - **Vascular Loop Syndrome Affecting CNVII** + - Negative high-resolution MR exam does not preclude surgery for smaller vascular loop causing HFS + - High-resolution MR makes this situation far less common + - Imaging findings + - MR-MRA: Asymmetric looping artery impinges on CNVII in CPA + - Root exit zone and attached segment (where CNVII is adherent to pons) are most sensitive to neurovascular compression + - AICA > PICA > VA > venous, though all are possible culprits +- ## Helpful Clues for Less Common Diagnoses + + + - **Epidermoid Cyst in CPA** + - Morphology: Assumes shape of cistern it occupies + - Insinuating margins, encasing cranial nerves and vessels + - Imaging findings + - Near CSF signal intensity of epidermoid cyst makes it difficult to see on T1, T2, and FLAIR sequences + - DWI shows reduced diffusivity + - **Meningioma in CPA** + - Morphology: Dural-based sessile mass + - Imaging findings + - Bone CT: Bony hyperostosis possible + - MR: Enhancing mass with dural tail(s) + - **Aneurysm in CPA****-IAC** + - Morphology: Ovoid or fusiform shape + - Imaging findings + - MR: Complex lesion signal from wall calcification, clot, and flow + - **Facial Nerve Schwannoma in CPA-IAC** + - Morphology: CPA-IAC "ice cream on cone" mass and labyrinthine segment tail + - Imaging findings + - Bone CT: Labyrinthine segment CNVII enlarged + - MR: Enhancing tubular mass with tail; may have intramural cysts + - **Facial Nerve Schwannoma in T-Bone** + - Morphology: Tubular mass within enlarged facial nerve canal may pedunculate into middle ear cavity (tympanic segment CNVII) or mastoid air cells (mastoid segment CNVII) + - Imaging findings + - Bone CT: Obvious enlargement of CNVII canal; geniculate ganglion most commonly affected + - MR: Enhancing mass enlarges bony facial nerve canal + - **Facial Nerve Perineural Tumor** + - Morphology: Enlargement of intratemporal CNVII connected through stylomastoid foramen (usually from invasive parotid malignancy) + - Imaging findings + - Mastoid segment most common + - CT: Soft tissue replacement of fat at stylomastoid foramen ± enlargement of bony CNVII canal + - MR: Enhancing minimally enlarged intratemporal CNVII + - **Facial Nerve Venous Malformation ("Hemangioma") in T-Bone** + - Lesion of abnormal vascular morphogenesis; thus "hemangioma" is misnomer + - Morphology: Amorphous geniculate ganglion area mass + - Imaging findings + - Bone CT: "Honeycomb" bone matrix (50%) + - MR: Avidly enhancing mass with foci of low signal intensity and gradient susceptibility (calcifications) +- ## Helpful Clues for Rare Diagnoses + + + - **A****cute****Cerebral Ischemia-Infarction** + - Acute onset of brainstem-related symptoms + - Pontine CVA secondary to basilar artery perforator injury + - Imaging findings + - MR: DWI shows reduced diffusivity in pons + - **Multiple Sclerosis** + - HFS is rare presentation of multiple sclerosis + - Imaging findings + - Plaques in vicinity of facial nerve nucleus in floor of 4th ventricle may or may not be seen + - MR: T2/FLAIR show ↑ signal intensity of supratentorial white matter plaques + - **Arteriovenous Malformation** + - More commonly supratentorial + - Imaging findings + - MR: Large ectatic arterial flow voids + - Enhancing nidus on T1 C+ fat-saturated sequence + - Large draining veins + - **Arachnoid Cyst in CPA** + - More common than epidermoid cyst in CPA, but epidermoid cyst in CPA more often associated with HFS + - Morphology + - Fills cistern with rounded or flat margins + - Imaging findings + - T1 C+: No enhancement + - FLAIR: Follows dark CSF signal intensity + - DWI: No reduced diffusivity + - **Venous Malformation ("Hemangioma") in IAC** + - Lesion of abnormal vascular morphogenesis; thus "hemangioma" is misnomer + - Morphology: Distal intracanalicular (IAC) ovoid to round cystic mass + - Imaging findings + - Bone CT: Lesion with punctate calcifications + - MR: Avidly enhancing IAC lesion with foci of low signal intensity and gradient susceptibility (calcifications) +- ## Alternative Differential Approaches + + + - Radiologist generally searches for cause of cranial neuropathy by following cranial nerve from origin to functional endplate + - Such anatomic approach permits segmentation of potential causes into anatomic groups + - Anatomic delineation of HFS causes + - 3 general anatomic sites where facial nerve may be injured causing HFS + - Intraaxial (nuclear) + - Cisternal (CPA or IAC cistern) + - Intratemporal (intratemporal facial nerve canal) + - Intraaxial (nuclear) + - [Acute cerebral ischemia-infarction](/document/acute-cerebral-ischemiainfarction/a405285f-aaea-43ca-8dc4-6f8120eaabc1) + - [Multiple sclerosis](/document/multiple-sclerosis/7892b2a2-f52a-4d7f-9858-a326f2b7ab04) + - [Arteriovenous malformation](/document/arteriovenous-malformation/55b35b26-df2e-4baf-8860-8073297cb738) + - Cisternal (CPA or IAC cistern) + - [Vascular loop syndrome affecting CNVII](/document/hemifacial-spasm/00871c72-c6c8-4913-b993-ebdb3da21947) + - [Epidermoid cyst in CPA](/document/cpa-iac-epidermoid-cyst/5e83f596-1ca4-41cb-95aa-469147ca5f8f) + - [Meningioma in CPA](/document/cpa-iac-meningioma/88301b77-f1c8-4efc-acf7-405999b42c3d) + - [Aneurysm in CPA-IAC](/document/cpa-iac-aneurysm/548f4994-c72a-40b8-a94c-d96fd6c39a21) + - [Facial nerve schwannoma in CPA-IAC](/document/cpa-iac-facial-nerve-schwannoma/9db01630-23a4-4f42-ad83-0ec399503495) + - [Arachnoid cyst in CPA](/document/cpa-iac-arachnoid-cyst/f1af2d0f-adfd-49c7-a3b9-8a1c66dce2be) + - [Venous malformation ("hemangioma") in IAC](/document/iac-venous-malformation/3a3d68e3-a087-4749-b13d-758c9ae8b9eb) + - Intratemporal (intratemporal CNVII canal) + - [Facial nerve schwannoma in T-bone](/document/temporal-bone-facial-nerve-schwann-/cf2bcc82-4a1b-4989-adeb-f4e82116111b) + - Facial nerve perineural tumor + - [Facial nerve venous malformation ("hemangioma") in T-bone](/document/temporal-bone-facial-nerve-venous--/dcd6a44e-cbe6-457c-9b03-598a2b874ece) + +## References + +# Selected References + +1. [Donahue JH et al: Imaging of vascular compression syndromes. Radiol Clin North Am. 55(1):123-138, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27890181%5Bpmid%5D) +1. [Deep NL et al: Magnetic resonance imaging assessment of vascular contact of the facial nerve in the asymptomatic patient. J Neurol Surg B Skull Base. 77(6):503-509, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27857878%5Bpmid%5D) +1. [Haller S et al: Imaging of neurovascular compression syndromes: trigeminal neuralgia, hemifacial spasm, vestibular paroxysmia, and glossopharyngeal neuralgia. AJNR Am J Neuroradiol. 37(8):1384-92, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26892985%5Bpmid%5D) +1. [Öcal R et al: Comparison of brain MRI angiography and brain MRI cisternography in patients with hemifacial spasm. Acta Neurol Belg. 116(4):593-598, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26908032%5Bpmid%5D) +1. [Ray DK et al: Surgical outcome and improvement in quality of life after microvascular decompression for hemifacial spasms: a case series assessment using a validated disease-specific scale. Stereotact Funct Neurosurg. 88(6):383-9, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20948243%5Bpmid%5D) +1. [Pyen JS et al: Tic convulsif caused by cerebellopontine angle schwannoma. Yonsei Med J. 2001 Apr;42(2):255-7. Retraction in: Yonsei Med J. 49(6):1060, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19108035%5Bpmid%5D) +1. [Desai K et al: Cerebellopontine angle epidermoid tumor presenting with hemifacial spasms. Neurol India. 51(2):288-9, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14571040%5Bpmid%5D) +1. [Iwai Y et al: Hemifacial spasm due to cerebellopontine angle meningiomas--two case reports. Neurol Med Chir (Tokyo). 41(2):87-9, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11255633%5Bpmid%5D) +1. [Takano S et al: Facial spasm and paroxysmal tinnitus associated with an arachnoid cyst of the cerebellopontine angle--case report. Neurol Med Chir (Tokyo). 38(2):100-3, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9557537%5Bpmid%5D) +1. [Illingworth RD et al: Hemifacial spasm: a prospective long-term follow up of 83 cases treated by microvascular decompression at two neurosurgical centres in the United Kingdom. J Neurol Neurosurg Psychiatry. 60(1):72-7, 1996](http://www.ncbi.nlm.nih.gov/pubmed/?term=8558156%5Bpmid%5D) +1. [Moriuchi S et al: Hemifacial spasm due to compression of the facial nerve by vertebral artery-posterior inferior cerebellar artery aneurysm and elongated vertebral artery--case report. Neurol Med Chir (Tokyo). 36(12):884-7, 1996](http://www.ncbi.nlm.nih.gov/pubmed/?term=9002718%5Bpmid%5D) +1. [Nagata S et al: Hemifacial spasm caused by CP angle AVM associated with ruptured aneurysm in the feeding artery--case report. Neurol Med Chir (Tokyo). 31(7):406-9, 1991](http://www.ncbi.nlm.nih.gov/pubmed/?term=1720219%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T2 FS MR in a patient with left hemifacial spasm (HFS) shows neurovascular compression of the left facial nerve by an ectatic vertebral artery . Note the point of neurovascular impingement against the adjacent cerebellar flocculus .](images/app.statdx.com_image_thumbnail_868473ec-1fb3-4586-bb14-f0b30dc104c9_annotated_true_size_900_quality_90_9c7b7945f82ade9954f6dbf50bbc9e94723276bb.jpg) +**Vascular Loop Syndrome Affecting CNVII** +*Axial T2 FS MR in a patient with left hemifacial spasm (HFS) shows neurovascular compression of the left facial nerve by an ectatic vertebral artery . Note the point of neurovascular impingement against the adjacent cerebellar flocculus .* + +![Axial T2 FS MR in a patient with left hemifacial spasm (HFS) shows neurovascular compression of the left facial nerve by an ectatic vertebral artery . Note the point of neurovascular impingement against the adjacent cerebellar flocculus .](images/app.statdx.com_image_thumbnail_868473ec-1fb3-4586-bb14-f0b30dc104c9_size_174_quality_85_c6b0711a_20251018T125015Z.jpg) +**Vascular Loop Syndrome Affecting CNVII** +*Axial T2 FS MR in a patient with left hemifacial spasm (HFS) shows neurovascular compression of the left facial nerve by an ectatic vertebral artery . Note the point of neurovascular impingement against the adjacent cerebellar flocculus .* + +![Axial T2 FS MR in a patient with left hemifacial spasm (HFS) shows neurovascular compression of the left facial nerve by an ectatic vertebral artery . Note the point of neurovascular impingement against the adjacent cerebellar flocculus .](images/app.statdx.com_image_thumbnail_868473ec-1fb3-4586-bb14-f0b30dc104c9_size_174_quality_85_f8783f5627c6edba338446e1098e97f27f9d8b6e.jpg) +**Vascular Loop Syndrome Affecting CNVII** +*Axial T2 FS MR in a patient with left hemifacial spasm (HFS) shows neurovascular compression of the left facial nerve by an ectatic vertebral artery . Note the point of neurovascular impingement against the adjacent cerebellar flocculus .* + +![Coronal T2 MR shows an ectatic vertebral artery "lifting" the posterior inferior cerebellar artery into the root exit zone of the facial nerve . Note compression of the lateral pons.](images/app.statdx.com_image_thumbnail_1c2cf033-b676-4750-bdfe-01396e480e87_annotated_true_size_900_quality_90_608cb24c670cef68c39e749c0562b67ef9d0d6ee.jpg) +**Vascular Loop Syndrome Affecting CNVII** +*Coronal T2 MR shows an ectatic vertebral artery "lifting" the posterior inferior cerebellar artery into the root exit zone of the facial nerve . Note compression of the lateral pons.* + +![Axial DWI MR in a patient with chronic left HFS shows a left CPA mass with reduced diffusivity and scalloped, insinuating margins, typical of an epidermoid cyst.](images/app.statdx.com_image_thumbnail_d802fc1c-e67c-4b88-9a47-eadef2e0c4dc_annotated_true_size_900_quality_90_d4d3e924c7eb85081e8714a628a5cf451d93ea00.jpg) +**Epidermoid Cyst in CPA** +*Axial DWI MR in a patient with chronic left HFS shows a left CPA mass with reduced diffusivity and scalloped, insinuating margins, typical of an epidermoid cyst.* + +![Axial T1 C+ FS MR in a patient with right HFS shows an avidly enhancing mass with the configuration of "ice cream" (CPA component) "on cone" (IAC component) . Note enhancing dural tails , which help differentiate this histologically-proven CPA-IAC meningioma from a schwannoma.](images/app.statdx.com_image_thumbnail_f0566ee5-cd29-4577-914c-1e0721b91b61_annotated_true_size_900_quality_90_311ee152e4639402ce14505ffbd422cc22674e2b.jpg) +**Meningioma in CPA** +*Axial T1 C+ FS MR in a patient with right HFS shows an avidly enhancing mass with the configuration of "ice cream" (CPA component) "on cone" (IAC component) . Note enhancing dural tails , which help differentiate this histologically-proven CPA-IAC meningioma from a schwannoma.* + +![Axial T2 FS MR shows a giant right vetebral artery aneurysm with complex signal intensity. The aneurysm obliterates the pontomedullary junction at the region of the right CNVII root exit zone . The same tortuous right vertebral artery also effaces the left CNVII root exit zone region.](images/app.statdx.com_image_thumbnail_1faeea37-724d-42f2-8739-916131f4a80d_annotated_true_size_900_quality_90_795a5473574bc32bcf3e1360209f55ab6a00e02c.jpg) +**Aneurysm in CPA-IAC** +*Axial T2 FS MR shows a giant right vetebral artery aneurysm with complex signal intensity. The aneurysm obliterates the pontomedullary junction at the region of the right CNVII root exit zone . The same tortuous right vertebral artery also effaces the left CNVII root exit zone region.* + +![Axial SPGR C+ MR of left facial nerve schwannoma shows an avidly enhancing CPA-IAC mass with a labyrinthine tail of enhancement extending to the geniculate ganglion , which differentiates it from a vestibular schwannoma.](images/app.statdx.com_image_thumbnail_0e3de358-b718-4444-bdbf-015499126776_annotated_true_size_900_quality_90_81faf4adee3642418bbd014aedd337f6061e3f46.jpg) +**Facial Nerve Schwannoma in CPA-IAC** +*Axial SPGR C+ MR of left facial nerve schwannoma shows an avidly enhancing CPA-IAC mass with a labyrinthine tail of enhancement extending to the geniculate ganglion , which differentiates it from a vestibular schwannoma.* + +![Axial T1 C+ MR shows an aggressive schwannoma involving the tympanic segment , posterior genu , and descending mastoid segment of the right facial nerve. Note several nonenhancing intramural cysts .](images/app.statdx.com_image_thumbnail_e0cf1289-1313-472f-8d34-680dc6e0bdb4_annotated_true_size_900_quality_90_4c54e0a2fc44c57382de7dafb969db01b706d8e4.jpg) +**Facial Nerve Schwannoma in T-Bone** +*Axial T1 C+ MR shows an aggressive schwannoma involving the tympanic segment , posterior genu , and descending mastoid segment of the right facial nerve. Note several nonenhancing intramural cysts .* + +![Coronal T1 MR shows invasive parotid space malignancy with perineural tumor spread cephalad through the stylomastoid foramen to involve the mastoid segment of the facial nerve .](images/app.statdx.com_image_thumbnail_a1bbe9c0-77f1-4978-a4c6-cbfa971feade_annotated_true_size_900_quality_90_98e6a310f6fb40793726d81da5f32f9f6f3d318b.jpg) +**Facial Nerve Perineural Tumor** +*Coronal T1 MR shows invasive parotid space malignancy with perineural tumor spread cephalad through the stylomastoid foramen to involve the mastoid segment of the facial nerve .* + +![Axial T1 C+ FS MR shows an enhancing lesion within enlarged geniculate fossa. Note the black central dot of low signal intensity corresponding with punctate calcification and suggesting the diagnosis of facial nerve venous malformation.](9140bb17-c1d5-4cc8-bc4c-06ed94b83744) +**Facial Nerve Venous Malformation ("Hemangioma") in T-Bone** +*Axial T1 C+ FS MR shows an enhancing lesion within enlarged geniculate fossa. Note the black central dot of low signal intensity corresponding with punctate calcification and suggesting the diagnosis of facial nerve venous malformation.* + +![Axial DWI MR shows an acute left pontine infarction with reduced diffusivity. Corresponding hypointensity was present on the ADC map (not shown). Patients with brainstem infarctions may develop hemifacial spasm in the subacute to chronic phase.](3264930f-dc90-4f01-be39-a7fa97f1cbcb) +**Acute Cerebral Ischemia-Infarction** +*Axial DWI MR shows an acute left pontine infarction with reduced diffusivity. Corresponding hypointensity was present on the ADC map (not shown). Patients with brainstem infarctions may develop hemifacial spasm in the subacute to chronic phase.* + +![Axial T2 FS MR in patient with multiple sclerosis shows a subtle demyelinating plaque in the left lateral pons near the root exit zone of the facial nerve . Demyelinating lesions accounting for HFS are often subtle and may not always be seen.](797b34fb-36a9-4794-baf7-de7698b72de4) +**Multiple Sclerosis** +*Axial T2 FS MR in patient with multiple sclerosis shows a subtle demyelinating plaque in the left lateral pons near the root exit zone of the facial nerve . Demyelinating lesions accounting for HFS are often subtle and may not always be seen.* + +![Axial T2 MR shows a left cerebellopontine cistern arteriovenous malformation nidus with a large posterior draining vein .](0b86ce5a-18a2-45f3-a4fb-4fd60798423c) +**Arteriovenous Malformation** +*Axial T2 MR shows a left cerebellopontine cistern arteriovenous malformation nidus with a large posterior draining vein .* + +![Axial T2 FS MR shows a right CPA arachnoid cyst , which exerts mild mass effect upon the cisternal segments of the vestibulocochlear and facial nerves . The cyst also flattens the lateral cerebellum. This cyst follows CSF signal intensity on all sequences.](bdadf53f-361b-46e6-9674-4d0a2e7f3301) +**Arachnoid Cyst in CPA** +*Axial T2 FS MR shows a right CPA arachnoid cyst , which exerts mild mass effect upon the cisternal segments of the vestibulocochlear and facial nerves . The cyst also flattens the lateral cerebellum. This cyst follows CSF signal intensity on all sequences.* + +![Coronal T1 C+ MR of an lAC venous malformation shows a lateral IAC enhancing mass with focus of internal low signal intensity from punctate intralesional calcification .](cb51c460-6823-4c04-9273-0a8f6442f929) +**Venous Malformation ("Hemangioma") in IAC** +*Coronal T1 C+ MR of an lAC venous malformation shows a lateral IAC enhancing mass with focus of internal low signal intensity from punctate intralesional calcification .* + + +### Additional Images + +![Axial T2 MR shows a markedly asymmetric left vertebral artery lifting the posterior inferior cerebellar artery into the medial aspect of the CPA cistern in the CNVII root exit zone vicinity.](images/app.statdx.com_image_thumbnail_0914bc05-9ac9-48c0-8d51-ab81b49d2ba4_annotated_true_size_900_quality_90_1835dad65bfbfeb0c6e4a66ca1f7fbdc3f1ce34c.jpg) +**Vascular Loop Syndrome Affecting CNVII** +*Axial T2 MR shows a markedly asymmetric left vertebral artery lifting the posterior inferior cerebellar artery into the medial aspect of the CPA cistern in the CNVII root exit zone vicinity.* + +![Axial T2 MR demonstrates a right CPA cistern epidermoid cyst with penetration of the porus acusticus and lobulated mass effect on the lateral margin of the brachium pontis .](images/app.statdx.com_image_thumbnail_0a3bdb08-3c5f-4088-aecf-a552778b35d4_annotated_true_size_900_quality_90_9926985e828133e0fd468bcd36ce80bf031f113f.jpg) +**Epidermoid Cyst in CPA** +*Axial T2 MR demonstrates a right CPA cistern epidermoid cyst with penetration of the porus acusticus and lobulated mass effect on the lateral margin of the brachium pontis .* + +![Axial T2 MR through the CPA cistern reveals a CSF intensity arachnoid cyst on the left . The arachnoid cyst flattens the cerebellar hemisphere and bows the facial and vestibulocochlear nerves anteromedially.](7a956883-fe0e-49d6-9beb-356661a83be0) +**Arachnoid Cyst in CPA** +*Axial T2 MR through the CPA cistern reveals a CSF intensity arachnoid cyst on the left . The arachnoid cyst flattens the cerebellar hemisphere and bows the facial and vestibulocochlear nerves anteromedially.* + +![Axial T2 MR shows a right CPA cistern epidermoid cyst scalloping the cerebellar contour and bowing the cisternal facial nerve anteriorly . The root exit zone is also affected .](images/app.statdx.com_image_thumbnail_e4e9106b-28bc-45dc-8fb9-a5a8d2f929b2_annotated_true_size_900_quality_90_217770251af4d21a133ab6a25e96a16ebb878373.jpg) +**Epidermoid Cyst in CPA** +*Axial T2 MR shows a right CPA cistern epidermoid cyst scalloping the cerebellar contour and bowing the cisternal facial nerve anteriorly . The root exit zone is also affected .* + +![Axial T2 MR reveals a dural-based CPA mass with IAC penetration with a "CSF-vascular cleft" between it and the adjacent brachium pontis-pons. Note the normal root exit zone of the contralateral left CNVII .](images/app.statdx.com_image_thumbnail_56873bf0-5409-4185-bb5d-600b4ddee58f_annotated_true_size_900_quality_90_763f5ae4dd85cb1746a13ce1a6f8893d11a7fed7.jpg) +**Meningioma in CPA** +*Axial T2 MR reveals a dural-based CPA mass with IAC penetration with a "CSF-vascular cleft" between it and the adjacent brachium pontis-pons. Note the normal root exit zone of the contralateral left CNVII .* + +![Axial T1 MR shows complex signal associated with a vertebral artery aneurysm . The aneurysm is wedged into the medial CPA cistern in the immediate vicinity of the CNVII root exit zone.](images/app.statdx.com_image_thumbnail_a52a1d60-eb64-465b-a566-47ba97872d6f_annotated_true_size_900_quality_90_eb28ea7b752a5007d8c0c552afac526470f92c2d.jpg) +**Aneurysm in CPA-IAC** +*Axial T1 MR shows complex signal associated with a vertebral artery aneurysm . The aneurysm is wedged into the medial CPA cistern in the immediate vicinity of the CNVII root exit zone.* + +![Axial T1 C+ MR shows a small facial nerve schwannoma of the lateral IAC , labyrinthine segment , and geniculate ganglion portions of the facial nerve.](images/app.statdx.com_image_thumbnail_268b6807-986d-4118-91a3-dcc6e4fb3130_annotated_true_size_900_quality_90_0dc24de0931049fbcbc2ed6d91ea2e0167a2968d.jpg) +**Facial Nerve Schwannoma in CPA-IAC** +*Axial T1 C+ MR shows a small facial nerve schwannoma of the lateral IAC , labyrinthine segment , and geniculate ganglion portions of the facial nerve.* + +![Axial T1 C+ MR demonstrates a mastoid segment facial nerve schwannoma . Notice that the enhancing tumor has dehisced the posterior wall of the external auditory canal .](images/app.statdx.com_image_thumbnail_cb641770-bf4b-403f-a267-cfe82586918b_annotated_true_size_900_quality_90_ee87ac357cb62aa4c927f4390cc0a018f9ab7c41.jpg) +**Facial Nerve Schwannoma in T-Bone** +*Axial T1 C+ MR demonstrates a mastoid segment facial nerve schwannoma . Notice that the enhancing tumor has dehisced the posterior wall of the external auditory canal .* + +![Axial T2 MR shows a multiple sclerosis plaque situated in the lateral right pons near the root exit zone of the facial nerve. Note a 2nd more subtle plaque in the left cerebellum .](386b3f44-4649-4810-8363-e80491f7a99a) +**Multiple Sclerosis** +*Axial T2 MR shows a multiple sclerosis plaque situated in the lateral right pons near the root exit zone of the facial nerve. Note a 2nd more subtle plaque in the left cerebellum .* + diff --git a/docs_md/articles/hypertrophic-olivary-degeneration_78257543-6d52-4879-84b1-445f3611d996.md b/docs_md/articles/hypertrophic-olivary-degeneration_78257543-6d52-4879-84b1-445f3611d996.md index 9397f67..6617bc2 100644 --- a/docs_md/articles/hypertrophic-olivary-degeneration_78257543-6d52-4879-84b1-445f3611d996.md +++ b/docs_md/articles/hypertrophic-olivary-degeneration_78257543-6d52-4879-84b1-445f3611d996.md @@ -32,6 +32,7 @@ breadcrumbs: slug: "hypertrophic-olivary-degeneration" treeNodeId: null category: "Brain" +cmeTopicId: "b70885e6-d7ea-4f0f-8b2c-c871245fd05c" documentVersionId: "0c307ba9-ac00-479c-9a0f-4201c66bc1f1" imageCount: 26 lastUpdated: "09/30/20" @@ -332,7 +333,7 @@ breadcrumbs: - Avoid misdiagnosis of tumor or multiple sclerosis - Bilateral & symmetrical lesions in ION argue against subacute infarct & vertebral artery dissection - 88b7142c-a73e-4046-a5d4-0c12bddb8133 + 9342df55-d4e7-4743-8a78-b03338f00dc0 ## References @@ -368,195 +369,87 @@ breadcrumbs: ### Selected Images -![Axial graphic of the upper medulla shows the medullary pyramids on each side of the ventral median fissure. The olives lie just posterior to the preolivary sulci .](images/app.statdx.com_image_e2ab6980-4968-4bcb-9864-1fa85eceedca_6ebcdb95_20251014T204755Z.jpg) +![Axial graphic of the upper medulla shows the medullary pyramids on each side of the ventral median fissure. The olives lie just posterior to the preolivary sulci .](images/app.statdx.com_image_thumbnail_e2ab6980-4968-4bcb-9864-1fa85eceedca_annotated_true_size_900_quality_90_eafe6900_20251018T122607Z.jpg) *Axial graphic of the upper medulla shows the medullary pyramids on each side of the ventral median fissure. The olives lie just posterior to the preolivary sulci .* -![Axial graphic of the upper medulla shows the medullary pyramids on each side of the ventral median fissure. The olives lie just posterior to the preolivary sulci .](images/app.statdx.com_image_thumbnail_e2ab6980-4968-4bcb-9864-1fa85eceedca_size_168_quality_85_20601fcc_20251014T204754Z.jpg) +![Axial graphic of the upper medulla shows the medullary pyramids on each side of the ventral median fissure. The olives lie just posterior to the preolivary sulci .](images/app.statdx.com_image_thumbnail_e2ab6980-4968-4bcb-9864-1fa85eceedca_size_174_quality_85_b06dd025_20251018T122603Z.jpg) *Axial graphic of the upper medulla shows the medullary pyramids on each side of the ventral median fissure. The olives lie just posterior to the preolivary sulci .* -![Axial graphic of the upper medulla shows the medullary pyramids on each side of the ventral median fissure. The olives lie just posterior to the preolivary sulci .](images/app.statdx.com_image_thumbnail_e2ab6980-4968-4bcb-9864-1fa85eceedca_size_168_quality_85_41df60ec_20251014T204719Z.jpg) -*Axial graphic of the upper medulla shows the medullary pyramids on each side of the ventral median fissure. The olives lie just posterior to the preolivary sulci .* - -![Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).](images/app.statdx.com_image_87b7bf23-7410-4d44-a5f1-8dac3d81f82f_023985c7_20251014T204757Z.jpg) +![Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).](images/app.statdx.com_image_thumbnail_87b7bf23-7410-4d44-a5f1-8dac3d81f82f_annotated_true_size_900_quality_90_5e71e83b_20251018T122607Z.jpg) *Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).* -![Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).](images/app.statdx.com_image_thumbnail_87b7bf23-7410-4d44-a5f1-8dac3d81f82f_size_168_quality_85_7d2edc18_20251014T204719Z.jpg) -*Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).* - -![Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).](images/app.statdx.com_image_thumbnail_87b7bf23-7410-4d44-a5f1-8dac3d81f82f_size_168_quality_85_87d93765_20251014T204754Z.jpg) -*Coronal graphic of the midbrain, pons, and medulla is sectioned to depict the Guillain-Mollaret triangle (GMT). The GMT is composed of the ipsilateral inferior olivary nucleus (green), dentate nucleus (blue) of the contralateral cerebellum, and the ipsilateral red nucleus (RN, red).* - -![Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain invading the RN (R > L). RN is a component of GMT.](images/app.statdx.com_image_6489d4cd-9c84-4d0a-8fc5-8e987f5d8077_7638176e_20251014T204759Z.jpg) +![Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain invading the RN (R > L). RN is a component of GMT.](images/app.statdx.com_image_thumbnail_6489d4cd-9c84-4d0a-8fc5-8e987f5d8077_annotated_true_size_900_quality_90_3eee82f5_20251018T122607Z.jpg) *Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain invading the RN (R > L). RN is a component of GMT.* -![Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain invading the RN (R > L). RN is a component of GMT.](images/app.statdx.com_image_thumbnail_6489d4cd-9c84-4d0a-8fc5-8e987f5d8077_size_168_quality_85_128fac13_20251014T204719Z.jpg) -*Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain invading the RN (R > L). RN is a component of GMT.* - -![Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain invading the RN (R > L). RN is a component of GMT.](images/app.statdx.com_image_thumbnail_6489d4cd-9c84-4d0a-8fc5-8e987f5d8077_size_168_quality_85_2f16fdc1_20251014T204754Z.jpg) -*Axial T2 MR of a 40-year-old woman with brainstem glioma and secondary hypertrophic olivary degeneration (HOD) shows a heterogeneous mass lesion involving midbrain invading the RN (R > L). RN is a component of GMT.* - -![Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal indicating HOD. Also note normal-appearing left olivary nucleus and preolivary sulcus .](images/app.statdx.com_image_9b10efdb-4747-4a85-adcb-e23c73ffe4cc_8b39bbda_20251014T204801Z.jpg) +![Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal indicating HOD. Also note normal-appearing left olivary nucleus and preolivary sulcus .](images/app.statdx.com_image_thumbnail_9b10efdb-4747-4a85-adcb-e23c73ffe4cc_annotated_true_size_900_quality_90_de6979c0_20251018T122607Z.jpg) *Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal indicating HOD. Also note normal-appearing left olivary nucleus and preolivary sulcus .* -![Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal indicating HOD. Also note normal-appearing left olivary nucleus and preolivary sulcus .](images/app.statdx.com_image_thumbnail_9b10efdb-4747-4a85-adcb-e23c73ffe4cc_size_168_quality_85_1ba77311_20251014T204754Z.jpg) -*Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal indicating HOD. Also note normal-appearing left olivary nucleus and preolivary sulcus .* - -![Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal indicating HOD. Also note normal-appearing left olivary nucleus and preolivary sulcus .](images/app.statdx.com_image_thumbnail_9b10efdb-4747-4a85-adcb-e23c73ffe4cc_size_168_quality_85_6aecad07_20251014T204719Z.jpg) -*Axial T2 MR at the level of medulla in the same patient shows enlarged right inferior olivary nucleus with hyperintense signal indicating HOD. Also note normal-appearing left olivary nucleus and preolivary sulcus .* - -![Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) due to encephalomalacia (DN is a component of GMT).](images/app.statdx.com_image_45d7e872-98da-486f-ba07-c5778f1207a7_2e22a519_20251014T204802Z.jpg) +![Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) due to encephalomalacia (DN is a component of GMT).](images/app.statdx.com_image_thumbnail_45d7e872-98da-486f-ba07-c5778f1207a7_annotated_true_size_900_quality_90_14f511a6_20251018T122607Z.jpg) *Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) due to encephalomalacia (DN is a component of GMT).* -![Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) due to encephalomalacia (DN is a component of GMT).](images/app.statdx.com_image_thumbnail_45d7e872-98da-486f-ba07-c5778f1207a7_size_168_quality_85_0a07159d_20251014T204719Z.jpg) -*Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) due to encephalomalacia (DN is a component of GMT).* - -![Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) due to encephalomalacia (DN is a component of GMT).](images/app.statdx.com_image_thumbnail_45d7e872-98da-486f-ba07-c5778f1207a7_size_168_quality_85_e224aaf6_20251014T204754Z.jpg) -*Axial FLAIR MR of a 58-year-old woman presenting with palatal myoclonus and a history of treated CNS lymphoma shows volume loss and hyperintense signal in left dentate nucleus (DN) due to encephalomalacia (DN is a component of GMT).* - -![Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei indicating HOD.](images/app.statdx.com_image_949d9854-f619-4e10-87e8-08b1674cd7b0_90f421ff_20251014T204803Z.jpg) +![Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei indicating HOD.](images/app.statdx.com_image_thumbnail_949d9854-f619-4e10-87e8-08b1674cd7b0_annotated_true_size_900_quality_90_732f280d_20251018T122607Z.jpg) *Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei indicating HOD.* -![Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei indicating HOD.](images/app.statdx.com_image_thumbnail_949d9854-f619-4e10-87e8-08b1674cd7b0_size_168_quality_85_976e7715_20251014T204719Z.jpg) -*Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei indicating HOD.* - -![Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei indicating HOD.](images/app.statdx.com_image_thumbnail_949d9854-f619-4e10-87e8-08b1674cd7b0_size_168_quality_85_d519f596_20251014T204754Z.jpg) -*Axial T2 MR in the same patient at the level of medulla shows mild hypertrophy and increased signal involving bilateral inferior olivary nuclei indicating HOD.* - -![Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons due to CM.](images/app.statdx.com_image_7c52868b-ffa4-46aa-bf88-27a6ca7a5281_17405b87_20251014T204804Z.jpg) +![Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons due to CM.](images/app.statdx.com_image_thumbnail_7c52868b-ffa4-46aa-bf88-27a6ca7a5281_annotated_true_size_900_quality_90_28eaaafe_20251018T122607Z.jpg) *Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons due to CM.* -![Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons due to CM.](images/app.statdx.com_image_thumbnail_7c52868b-ffa4-46aa-bf88-27a6ca7a5281_size_168_quality_85_5c3b0041_20251014T204754Z.jpg) -*Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons due to CM.* - -![Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons due to CM.](images/app.statdx.com_image_thumbnail_7c52868b-ffa4-46aa-bf88-27a6ca7a5281_size_168_quality_85_ab85dadf_20251014T204719Z.jpg) -*Axial T2 of a 67-year-old man with left para median pontine cavernous malformation (CM) involving central tegmental tract resulting in ipsilateral HOD shows hyperintense popcorn lesion with rim of hemosiderin in left para median pons due to CM.* - -![Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal due to HOD.](images/app.statdx.com_image_5f1f5911-5f58-460f-b712-eff1a657b51e_bc24d3df_20251014T204806Z.jpg) +![Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal due to HOD.](images/app.statdx.com_image_thumbnail_5f1f5911-5f58-460f-b712-eff1a657b51e_annotated_true_size_900_quality_90_351a3f56_20251018T122607Z.jpg) *Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal due to HOD.* -![Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal due to HOD.](images/app.statdx.com_image_thumbnail_5f1f5911-5f58-460f-b712-eff1a657b51e_size_168_quality_85_2eaa4324_20251014T204754Z.jpg) -*Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal due to HOD.* - -![Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal due to HOD.](images/app.statdx.com_image_thumbnail_5f1f5911-5f58-460f-b712-eff1a657b51e_size_168_quality_85_576c80ec_20251014T204719Z.jpg) -*Axial FLAIR MR in the same patient shows enlarged left inferior olivary nucleus with hyperintense signal due to HOD.* - -![Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN and normal right olive . Note light enlargement and ↑ signal in right olive , progressive enlargement and ↑ signal in olive , and lack of enhancement in olive on postcontrast T1WI (bottom right).](images/app.statdx.com_image_bfd80fc4-8257-4305-be57-f6f17e5d2725_068d6772_20251014T204807Z.jpg) +![Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN and normal right olive . Note light enlargement and ↑ signal in right olive , progressive enlargement and ↑ signal in olive , and lack of enhancement in olive on postcontrast T1WI (bottom right).](images/app.statdx.com_image_thumbnail_bfd80fc4-8257-4305-be57-f6f17e5d2725_annotated_true_size_900_quality_90_0ec94d8f_20251018T122607Z.jpg) *Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN and normal right olive . Note light enlargement and ↑ signal in right olive , progressive enlargement and ↑ signal in olive , and lack of enhancement in olive on postcontrast T1WI (bottom right).* -![Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN and normal right olive . Note light enlargement and ↑ signal in right olive , progressive enlargement and ↑ signal in olive , and lack of enhancement in olive on postcontrast T1WI (bottom right).](images/app.statdx.com_image_thumbnail_bfd80fc4-8257-4305-be57-f6f17e5d2725_size_168_quality_85_2d84ce83_20251014T204719Z.jpg) -*Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN and normal right olive . Note light enlargement and ↑ signal in right olive , progressive enlargement and ↑ signal in olive , and lack of enhancement in olive on postcontrast T1WI (bottom right).* - -![Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN and normal right olive . Note light enlargement and ↑ signal in right olive , progressive enlargement and ↑ signal in olive , and lack of enhancement in olive on postcontrast T1WI (bottom right).](images/app.statdx.com_image_thumbnail_bfd80fc4-8257-4305-be57-f6f17e5d2725_size_168_quality_85_5ac0e6ac_20251014T204754Z.jpg) -*Axial T2 MR at 1 day (top left), 4 months (top right), and 7 months (bottom left) postoperative follow-up show edema in left DN and normal right olive . Note light enlargement and ↑ signal in right olive , progressive enlargement and ↑ signal in olive , and lack of enhancement in olive on postcontrast T1WI (bottom right).* - -![Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives corresponding to the FLAIR hyperintensity in the previous image.](images/app.statdx.com_image_af8d64b8-e6d3-4f2c-bae7-0c7e3b0fba51_00a8db06_20251014T204808Z.jpg) -*Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives corresponding to the FLAIR hyperintensity in the previous image.* - -![Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives corresponding to the FLAIR hyperintensity in the previous image.](images/app.statdx.com_image_thumbnail_af8d64b8-e6d3-4f2c-bae7-0c7e3b0fba51_size_168_quality_85_c50c0f98_20251014T204719Z.jpg) -*Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives corresponding to the FLAIR hyperintensity in the previous image.* - -![Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives corresponding to the FLAIR hyperintensity in the previous image.](images/app.statdx.com_image_thumbnail_af8d64b8-e6d3-4f2c-bae7-0c7e3b0fba51_size_168_quality_85_e0e343e4_20251014T204754Z.jpg) +![Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives corresponding to the FLAIR hyperintensity in the previous image.](af8d64b8-e6d3-4f2c-bae7-0c7e3b0fba51) *Axial graphic of the midbrain at the level of the hypoglossal nuclei shows the distinct wavy pattern of the olives corresponding to the FLAIR hyperintensity in the previous image.* ### Additional Images -![Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense , secondary to HOD.](images/app.statdx.com_image_3e5effcb-b025-48ed-ba4b-2b20975d18d0_80366b9a_20251014T204809Z.jpg) +![Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense , secondary to HOD.](3e5effcb-b025-48ed-ba4b-2b20975d18d0) *Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense , secondary to HOD.* -![Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense , secondary to HOD.](images/app.statdx.com_image_thumbnail_3e5effcb-b025-48ed-ba4b-2b20975d18d0_size_168_quality_85_e47b3356_20251014T204719Z.jpg) -*Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense , secondary to HOD.* - -![Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense , secondary to HOD.](images/app.statdx.com_image_thumbnail_3e5effcb-b025-48ed-ba4b-2b20975d18d0_size_168_quality_85_f2db0be1_20251014T204754Z.jpg) -*Axial T2WI MR demonstrates hypertrophy of both inferior olivary nuclei, which are also hyperintense , secondary to HOD.* - -![Sagittal FLAIR MR shows abnormally ↑ signal intensity in an anterior medullary area that corresponds to the inferior olivary nucleus.](images/app.statdx.com_image_thumbnail_2698fac6-694f-43bc-904f-c368445620a9_size_168_quality_85_39b0c1ce_20251014T204754Z.jpg) +![Sagittal FLAIR MR shows abnormally ↑ signal intensity in an anterior medullary area that corresponds to the inferior olivary nucleus.](2698fac6-694f-43bc-904f-c368445620a9) *Sagittal FLAIR MR shows abnormally ↑ signal intensity in an anterior medullary area that corresponds to the inferior olivary nucleus.* -![Sagittal FLAIR MR shows abnormally ↑ signal intensity in an anterior medullary area that corresponds to the inferior olivary nucleus.](images/app.statdx.com_image_thumbnail_2698fac6-694f-43bc-904f-c368445620a9_size_168_quality_85_9037044c_20251014T204719Z.jpg) -*Sagittal FLAIR MR shows abnormally ↑ signal intensity in an anterior medullary area that corresponds to the inferior olivary nucleus.* - -![Axial FLAIR MR in the same patient who suffered midbrain hemorrhage (not shown) depicts bilateral hyperintense and hypertrophied inferior olivary nuclei .](images/app.statdx.com_image_thumbnail_3b581539-62f7-42f9-8909-56a190ba83e1_size_168_quality_85_36b746e4_20251014T204754Z.jpg) +![Axial FLAIR MR in the same patient who suffered midbrain hemorrhage (not shown) depicts bilateral hyperintense and hypertrophied inferior olivary nuclei .](3b581539-62f7-42f9-8909-56a190ba83e1) *Axial FLAIR MR in the same patient who suffered midbrain hemorrhage (not shown) depicts bilateral hyperintense and hypertrophied inferior olivary nuclei .* -![Axial FLAIR MR in the same patient who suffered midbrain hemorrhage (not shown) depicts bilateral hyperintense and hypertrophied inferior olivary nuclei .](images/app.statdx.com_image_thumbnail_3b581539-62f7-42f9-8909-56a190ba83e1_size_168_quality_85_a4513686_20251014T204719Z.jpg) -*Axial FLAIR MR in the same patient who suffered midbrain hemorrhage (not shown) depicts bilateral hyperintense and hypertrophied inferior olivary nuclei .* - -![Axial FLAIR MR shows high signal intensity and asymmetric enlargement of right anterior medulla corresponding to the region of hypertrophic degeneration of the right inferior olivary nucleus .](images/app.statdx.com_image_thumbnail_9dcb9590-4f66-4d9a-b58b-fd84da758053_size_168_quality_85_4bf0f302_20251014T204754Z.jpg) +![Axial FLAIR MR shows high signal intensity and asymmetric enlargement of right anterior medulla corresponding to the region of hypertrophic degeneration of the right inferior olivary nucleus .](9dcb9590-4f66-4d9a-b58b-fd84da758053) *Axial FLAIR MR shows high signal intensity and asymmetric enlargement of right anterior medulla corresponding to the region of hypertrophic degeneration of the right inferior olivary nucleus .* -![Axial FLAIR MR shows high signal intensity and asymmetric enlargement of right anterior medulla corresponding to the region of hypertrophic degeneration of the right inferior olivary nucleus .](images/app.statdx.com_image_thumbnail_9dcb9590-4f66-4d9a-b58b-fd84da758053_size_168_quality_85_9540ce82_20251014T204719Z.jpg) -*Axial FLAIR MR shows high signal intensity and asymmetric enlargement of right anterior medulla corresponding to the region of hypertrophic degeneration of the right inferior olivary nucleus .* - -![Axial T2WI MR in the same patient shows a right pontine infarct, the primary lesion that led to right HOD.](images/app.statdx.com_image_thumbnail_c6ad71d6-76b6-407d-8d3f-5520dbb1bbca_size_168_quality_85_25516031_20251014T204719Z.jpg) +![Axial T2WI MR in the same patient shows a right pontine infarct, the primary lesion that led to right HOD.](c6ad71d6-76b6-407d-8d3f-5520dbb1bbca) *Axial T2WI MR in the same patient shows a right pontine infarct, the primary lesion that led to right HOD.* -![Axial T2WI MR in the same patient shows a right pontine infarct, the primary lesion that led to right HOD.](images/app.statdx.com_image_thumbnail_c6ad71d6-76b6-407d-8d3f-5520dbb1bbca_size_168_quality_85_dfe86ce5_20251014T204754Z.jpg) -*Axial T2WI MR in the same patient shows a right pontine infarct, the primary lesion that led to right HOD.* - -![Axial T2WI MR shows bilateral symmetric hypertrophy with ↑ signal intensity confined to inferior olivary nuclei, with loss of pre- and postolivary sulci .](images/app.statdx.com_image_thumbnail_d3122192-cad3-4f63-ae3b-d0bfe6f8b428_size_168_quality_85_ab9a5054_20251014T204754Z.jpg) +![Axial T2WI MR shows bilateral symmetric hypertrophy with ↑ signal intensity confined to inferior olivary nuclei, with loss of pre- and postolivary sulci .](d3122192-cad3-4f63-ae3b-d0bfe6f8b428) *Axial T2WI MR shows bilateral symmetric hypertrophy with ↑ signal intensity confined to inferior olivary nuclei, with loss of pre- and postolivary sulci .* -![Axial T2WI MR shows bilateral symmetric hypertrophy with ↑ signal intensity confined to inferior olivary nuclei, with loss of pre- and postolivary sulci .](images/app.statdx.com_image_thumbnail_d3122192-cad3-4f63-ae3b-d0bfe6f8b428_size_168_quality_85_bbf068ad_20251014T204719Z.jpg) -*Axial T2WI MR shows bilateral symmetric hypertrophy with ↑ signal intensity confined to inferior olivary nuclei, with loss of pre- and postolivary sulci .* - -![Axial T2WI MR in the same patient shows the primary midbrain lesion that caused the occurrence of bilateral HOD.](images/app.statdx.com_image_thumbnail_7f3adfa6-8b44-448b-acbc-7bec45f4c5f8_size_168_quality_85_18b8546d_20251014T204754Z.jpg) +![Axial T2WI MR in the same patient shows the primary midbrain lesion that caused the occurrence of bilateral HOD.](7f3adfa6-8b44-448b-acbc-7bec45f4c5f8) *Axial T2WI MR in the same patient shows the primary midbrain lesion that caused the occurrence of bilateral HOD.* -![Axial T2WI MR in the same patient shows the primary midbrain lesion that caused the occurrence of bilateral HOD.](images/app.statdx.com_image_thumbnail_7f3adfa6-8b44-448b-acbc-7bec45f4c5f8_size_168_quality_85_c64bfc5e_20251014T204719Z.jpg) -*Axial T2WI MR in the same patient shows the primary midbrain lesion that caused the occurrence of bilateral HOD.* - -![Axial T2WI MR in a patient who developed onset of dysarthria and upper extremity dysmetria 15 months following stereotaxic XRT for midbrain arteriovenous malformation shows mixed hyper-/hypointensity in the residual vascular malformation .](images/app.statdx.com_image_thumbnail_717f7010-1fdc-42fa-adfe-f828d24b650a_size_168_quality_85_8310efb4_20251014T204719Z.jpg) +![Axial T2WI MR in a patient who developed onset of dysarthria and upper extremity dysmetria 15 months following stereotaxic XRT for midbrain arteriovenous malformation shows mixed hyper-/hypointensity in the residual vascular malformation .](717f7010-1fdc-42fa-adfe-f828d24b650a) *Axial T2WI MR in a patient who developed onset of dysarthria and upper extremity dysmetria 15 months following stereotaxic XRT for midbrain arteriovenous malformation shows mixed hyper-/hypointensity in the residual vascular malformation .* -![Axial T2WI MR in a patient who developed onset of dysarthria and upper extremity dysmetria 15 months following stereotaxic XRT for midbrain arteriovenous malformation shows mixed hyper-/hypointensity in the residual vascular malformation .](images/app.statdx.com_image_thumbnail_717f7010-1fdc-42fa-adfe-f828d24b650a_size_168_quality_85_9517d921_20251014T204754Z.jpg) -*Axial T2WI MR in a patient who developed onset of dysarthria and upper extremity dysmetria 15 months following stereotaxic XRT for midbrain arteriovenous malformation shows mixed hyper-/hypointensity in the residual vascular malformation .* - -![Axial T2WI MR in the same patient shows bilateral inferior olivary hyperintensity and hypertrophy .](images/app.statdx.com_image_thumbnail_81bec60a-3dee-42d5-bb77-cf3045ec6b06_size_168_quality_85_00d7b27e_20251014T204754Z.jpg) +![Axial T2WI MR in the same patient shows bilateral inferior olivary hyperintensity and hypertrophy .](81bec60a-3dee-42d5-bb77-cf3045ec6b06) *Axial T2WI MR in the same patient shows bilateral inferior olivary hyperintensity and hypertrophy .* -![Axial T2WI MR in the same patient shows bilateral inferior olivary hyperintensity and hypertrophy .](images/app.statdx.com_image_thumbnail_81bec60a-3dee-42d5-bb77-cf3045ec6b06_size_168_quality_85_5f3ff6c1_20251014T204719Z.jpg) -*Axial T2WI MR in the same patient shows bilateral inferior olivary hyperintensity and hypertrophy .* - -![Axial T2WI MR (CISS) shows the normal shape of the medullary olives .](images/app.statdx.com_image_thumbnail_7cd64580-3d32-4887-900a-30be8b06e436_size_168_quality_85_01ed2b3f_20251014T204719Z.jpg) +![Axial T2WI MR (CISS) shows the normal shape of the medullary olives .](7cd64580-3d32-4887-900a-30be8b06e436) *Axial T2WI MR (CISS) shows the normal shape of the medullary olives .* -![Axial T2WI MR (CISS) shows the normal shape of the medullary olives .](images/app.statdx.com_image_thumbnail_7cd64580-3d32-4887-900a-30be8b06e436_size_168_quality_85_e8047204_20251014T204754Z.jpg) -*Axial T2WI MR (CISS) shows the normal shape of the medullary olives .* - -![Axial T2WI MR in a patient who developed palatal myoclonus ~ 6 months after resection of a midbrain CM shows hyperintensity and enlargement of both olives . This pattern is typical in the subacute stage of HOD, which typically appears between 6 months and 3-4 years after injury to the dentato-rubro-olivary pathway.](images/app.statdx.com_image_thumbnail_842f79ca-da5e-4b88-a632-22de84d9f8df_size_168_quality_85_a834833e_20251014T204719Z.jpg) +![Axial T2WI MR in a patient who developed palatal myoclonus ~ 6 months after resection of a midbrain CM shows hyperintensity and enlargement of both olives . This pattern is typical in the subacute stage of HOD, which typically appears between 6 months and 3-4 years after injury to the dentato-rubro-olivary pathway.](842f79ca-da5e-4b88-a632-22de84d9f8df) *Axial T2WI MR in a patient who developed palatal myoclonus ~ 6 months after resection of a midbrain CM shows hyperintensity and enlargement of both olives . This pattern is typical in the subacute stage of HOD, which typically appears between 6 months and 3-4 years after injury to the dentato-rubro-olivary pathway.* -![Axial T2WI MR in a patient who developed palatal myoclonus ~ 6 months after resection of a midbrain CM shows hyperintensity and enlargement of both olives . This pattern is typical in the subacute stage of HOD, which typically appears between 6 months and 3-4 years after injury to the dentato-rubro-olivary pathway.](images/app.statdx.com_image_thumbnail_842f79ca-da5e-4b88-a632-22de84d9f8df_size_168_quality_85_c66c4ac0_20251014T204754Z.jpg) -*Axial T2WI MR in a patient who developed palatal myoclonus ~ 6 months after resection of a midbrain CM shows hyperintensity and enlargement of both olives . This pattern is typical in the subacute stage of HOD, which typically appears between 6 months and 3-4 years after injury to the dentato-rubro-olivary pathway.* - -![Axial SWI MR demonstrates hemosiderin staining in the dorsal aspect of the brainstem in the midline and to the right due to an old hemorrhage.](images/app.statdx.com_image_thumbnail_2c2646c5-4793-4f2e-9d9e-6600ed9bfc6f_size_168_quality_85_6d72ebd6_20251014T204754Z.jpg) +![Axial SWI MR demonstrates hemosiderin staining in the dorsal aspect of the brainstem in the midline and to the right due to an old hemorrhage.](2c2646c5-4793-4f2e-9d9e-6600ed9bfc6f) *Axial SWI MR demonstrates hemosiderin staining in the dorsal aspect of the brainstem in the midline and to the right due to an old hemorrhage.* -![Axial SWI MR demonstrates hemosiderin staining in the dorsal aspect of the brainstem in the midline and to the right due to an old hemorrhage.](images/app.statdx.com_image_thumbnail_2c2646c5-4793-4f2e-9d9e-6600ed9bfc6f_size_168_quality_85_f6de8b6b_20251014T204719Z.jpg) -*Axial SWI MR demonstrates hemosiderin staining in the dorsal aspect of the brainstem in the midline and to the right due to an old hemorrhage.* - -![Axial FLAIR MR in the same patient at the level of the medulla shows mild hypertrophy with hyperintensity in the region of the right inferior olivary nucleus . Findings are typical for HOD caused by primary lesions in dentato-rubro-olivary pathway (anatomical GMT).](images/app.statdx.com_image_thumbnail_f5a3dae8-1682-4b14-a65e-db91929e2ca2_size_168_quality_85_8c890454_20251014T204719Z.jpg) +![Axial FLAIR MR in the same patient at the level of the medulla shows mild hypertrophy with hyperintensity in the region of the right inferior olivary nucleus . Findings are typical for HOD caused by primary lesions in dentato-rubro-olivary pathway (anatomical GMT).](f5a3dae8-1682-4b14-a65e-db91929e2ca2) *Axial FLAIR MR in the same patient at the level of the medulla shows mild hypertrophy with hyperintensity in the region of the right inferior olivary nucleus . Findings are typical for HOD caused by primary lesions in dentato-rubro-olivary pathway (anatomical GMT).* -![Axial FLAIR MR in the same patient at the level of the medulla shows mild hypertrophy with hyperintensity in the region of the right inferior olivary nucleus . Findings are typical for HOD caused by primary lesions in dentato-rubro-olivary pathway (anatomical GMT).](images/app.statdx.com_image_thumbnail_f5a3dae8-1682-4b14-a65e-db91929e2ca2_size_168_quality_85_d9562458_20251014T204754Z.jpg) -*Axial FLAIR MR in the same patient at the level of the medulla shows mild hypertrophy with hyperintensity in the region of the right inferior olivary nucleus . Findings are typical for HOD caused by primary lesions in dentato-rubro-olivary pathway (anatomical GMT).* - -![Axial T2WI MR through the medulla shows that the ipsilateral olive is atrophic and hyperintense . This patient also has crossed cerebellar atrophy due to interruption of the ponto-cerebellar pathway.](images/app.statdx.com_image_thumbnail_a47e6de5-44e1-4f0f-9855-8278718b020f_size_168_quality_85_9d9e41c9_20251014T204719Z.jpg) +![Axial T2WI MR through the medulla shows that the ipsilateral olive is atrophic and hyperintense . This patient also has crossed cerebellar atrophy due to interruption of the ponto-cerebellar pathway.](a47e6de5-44e1-4f0f-9855-8278718b020f) *Axial T2WI MR through the medulla shows that the ipsilateral olive is atrophic and hyperintense . This patient also has crossed cerebellar atrophy due to interruption of the ponto-cerebellar pathway.* -![Axial T2WI MR through the medulla shows that the ipsilateral olive is atrophic and hyperintense . This patient also has crossed cerebellar atrophy due to interruption of the ponto-cerebellar pathway.](images/app.statdx.com_image_thumbnail_a47e6de5-44e1-4f0f-9855-8278718b020f_size_168_quality_85_f4dc3265_20251014T204754Z.jpg) -*Axial T2WI MR through the medulla shows that the ipsilateral olive is atrophic and hyperintense . This patient also has crossed cerebellar atrophy due to interruption of the ponto-cerebellar pathway.* - -![Axial T2WI MR in a patient who developed palatal myoclonus several months following midbrain surgery for CM. Imaging obtained 1 year later shows residual CM .](images/app.statdx.com_image_thumbnail_47d78ac6-02eb-4674-bfda-316586ec456d_size_168_quality_85_0c26fca7_20251014T204754Z.jpg) +![Axial T2WI MR in a patient who developed palatal myoclonus several months following midbrain surgery for CM. Imaging obtained 1 year later shows residual CM .](47d78ac6-02eb-4674-bfda-316586ec456d) *Axial T2WI MR in a patient who developed palatal myoclonus several months following midbrain surgery for CM. Imaging obtained 1 year later shows residual CM .* -![Axial T2WI MR in a patient who developed palatal myoclonus several months following midbrain surgery for CM. Imaging obtained 1 year later shows residual CM .](images/app.statdx.com_image_thumbnail_47d78ac6-02eb-4674-bfda-316586ec456d_size_168_quality_85_490d2382_20251014T204719Z.jpg) -*Axial T2WI MR in a patient who developed palatal myoclonus several months following midbrain surgery for CM. Imaging obtained 1 year later shows residual CM .* - -![Axial FLAIR MR in the same patient delineates the somewhat wavy appearance of the hyperintensity conforming to the configuration of the olives . The pyramids are spared, helping differentiate HOD from perforating artery infarction.](images/app.statdx.com_image_thumbnail_24cc4357-0f7a-464d-be25-3f85a555f29e_size_168_quality_85_24074433_20251014T204719Z.jpg) -*Axial FLAIR MR in the same patient delineates the somewhat wavy appearance of the hyperintensity conforming to the configuration of the olives . The pyramids are spared, helping differentiate HOD from perforating artery infarction.* - -![Axial FLAIR MR in the same patient delineates the somewhat wavy appearance of the hyperintensity conforming to the configuration of the olives . The pyramids are spared, helping differentiate HOD from perforating artery infarction.](images/app.statdx.com_image_thumbnail_24cc4357-0f7a-464d-be25-3f85a555f29e_size_168_quality_85_c8eb04b6_20251014T204754Z.jpg) +![Axial FLAIR MR in the same patient delineates the somewhat wavy appearance of the hyperintensity conforming to the configuration of the olives . The pyramids are spared, helping differentiate HOD from perforating artery infarction.](24cc4357-0f7a-464d-be25-3f85a555f29e) *Axial FLAIR MR in the same patient delineates the somewhat wavy appearance of the hyperintensity conforming to the configuration of the olives . 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Koontz, MD" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c" + - + name: "CPA-IAC and Posterior Fossa" + slug: "cpa-iac-and-posterior-fossa" + treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992" + - + name: "Anatomically Based Differentials" + slug: "anatomically-based-differentials" + treeNodeId: "debfb06c-8656-4f5d-92c1-eaa468185d78" + - + name: "Large IAC" + slug: "large-iac" + treeNodeId: null +category: "Head and Neck" +documentVersionId: "fe254fbf-9caf-4b75-a18f-dd4c006eafc1" +imageCount: 16 +lastUpdated: "09/04/18" +pageDescription: "Large IAC" +pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Anatomically Based Differentials, Large IAC" +pageTitle: "Large IAC | STATdx" +enhancedTitle: "Large IAC" +type: "DDX" +references: true +breadcrumbs: + - "Head and Neck" + - "Differential Diagnosis" + - "CPA-IAC and Posterior Fossa" + - "Anatomically Based Differentials" + - "Large IAC" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Bilateral large internal auditory canal (IAC) + - Can be normal variant if imaging otherwise normal + - Inner ear malformation + - Cystic cochleovestibular malformation (CCVM) + - X-linked stapes gusher + - Risk of CSF gusher at surgery + - Bilateral large IAC and tumors: Consider NF2 + - Unilateral large IAC + - Ipsilateral cerebellar hypoplasia suggests PHACES + - Inner ear malformation, e.g., CCVM + - Cerebellopontine angle (CPA)-IAC tumor +- ## Helpful Clues for Common Diagnoses + + + - [Vestibular Schwannoma](/document/vestibular-schwannoma/48772166-59dc-4909-bc75-538de7dd9ddf) + - Large IAC + enhancing CPA-IAC tumor with extension along vestibular nerve(s) ± cochlear nerve + - Variable signal on T2, depends on cellularity + - Presence of microhemorrhages on T2* GRE or SWI may help differentiate schwannoma from meningioma in this location +- ## Helpful Clues for Less Common Diagnoses + + + - [Neurofibromatosis Type 2, CPA-IAC](/document/neurofibromatosis-type-2/cdb266b4-1e86-4dbf-a6b8-e009585e25d6) + - Bilateral vestibular schwannomas is hallmark lesion; effectively pathognomonic + - Bilateral vestibular > > facial or cochlear schwannomas ± schwannomas &/or meningiomas of other cranial nerves/dura + - Unilateral or bilateral large IAC with sharply marginated tumor in CPA-IAC + - Variable signal on T2WI, depends on cellularity + - Intense enhancement of solid component + - [Metastases, CPA-IAC](/document/cpa-iac-metastases/451451c8-7b49-4ce9-bf22-7c02b4652f23) + - Uni- or bilateral large IAC due to IAC-CPA mass(es) + - Hematogenous mets: Lytic/permeative bony destruction, hypointensity on T2WI, decreased diffusivity, and variable enhancement + - CSF tumor dissemination of 1° intracranial tumor ± large IAC, variable MR appearance depending on tumor type + - **PHACES Association** + - **P**osterior fossa malformation, infantile craniofacial **h**emangioma, aortic and cerebral **a**rterial anomalies, **c**ardiac, **e**ye and **s**ternal/midline anomalies + - Unilateral flared, large IAC + - Ipsilateral cerebellar hypoplasia +- ## Helpful Clues for Rare Diagnoses + + + - [Schwannoma, Facial Nerve, CPA-IAC](/document/cpa-iac-facial-nerve-schwannoma/9db01630-23a4-4f42-ad83-0ec399503495) + - Unilateral large IAC and facial nerve canal + - **Atypical Teratoid/Rhabdoid Tumor** + - Unilateral large IAC and IAC-CPA tumor with lytic bone destruction in infant + - T2WI hypointensity, decreased diffusivity on DWI, and variable enhancement + - **Cystic Cochleovestibular Malformation (IP-I)** + - IAC most commonly enlarged; may be small or normal + - Plump cochlea lacks internal septation/modiolus + - Globular vestibule and lateral semicircular canal (SCC) or SCC anlage anomaly + - [X-Linked Stapes Gusher (DFNX2)](/document/x-linked-stapes-gusher-dfnx2/498c58d9-181b-4ba7-beb8-e98cfd0bcaf6) + - Lateral aspect IAC wide (bulbous) bilaterally + - Corkscrew-shaped cochlea lacks normal interscalar septum and modiolus + - **Neurofibromatosis Type 1** + - Common disorder, wide IACs uncommon + - Symmetric large IACs from dural ectasia **not**tumor + +## References + +# Selected References + +1. [Plotkin SR et al: Neurofibromatosis and schwannomatosis. Semin Neurol. 38(1):73-85, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29548054%5Bpmid%5D) +1. [Dağkıran M et al: Radiological imaging findings of patients with congenital totally hearing loss. J Int Adv Otol. 12(1):43-8, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27340982%5Bpmid%5D) +1. [Meltzer DE et al: Enlargement of the internal auditory canal and associated posterior fossa anomalies in PHACES association. AJNR Am J Neuroradiol. 36(11):2159-62, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26159514%5Bpmid%5D) +1. [Wang X et al: Atypical teratoid/rhabdoid tumor (AT/RT) arising from the acoustic nerve in a young adult: a case report and a review of literature. Medicine (Baltimore). 94(4):e439, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25634176%5Bpmid%5D) +1. [Saylisoy S et al: Computed tomographic findings of X-linked deafness: a spectrum from child to mother, from young to old, from boy to girl, from mixed to sudden hearing loss. J Comput Assist Tomogr. 38(1):20-4, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24424552%5Bpmid%5D) +1. [Thamburaj K et al: Intratumoral microhemorrhages on T2*-weighted gradient-echo imaging helps differentiate vestibular schwannoma from meningioma. AJNR Am J Neuroradiol. 29(3):552-7, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18079187%5Bpmid%5D) +1. [Kumar G et al: X-linked stapes gusher: CT findings in one patient. AJNR Am J Neuroradiol. 24(6):1130-2, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12812938%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T1 C+ FS MR shows a large, avidly enhancing left CPA-IAC vestibular schwannoma that markedly enlarges the IAC as well as exerts mass effect upon the adjacent pons, brachium pontis, and cerebellum.](images/app.statdx.com_image_thumbnail_88ac0486-9a5c-4aad-a128-03d6be231847_annotated_true_size_900_quality_90_1d87d6515091edad943ed5f4ee70a011438a4ba0.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ FS MR shows a large, avidly enhancing left CPA-IAC vestibular schwannoma that markedly enlarges the IAC as well as exerts mass effect upon the adjacent pons, brachium pontis, and cerebellum.* + +![Axial T1 C+ FS MR shows a large, avidly enhancing left CPA-IAC vestibular schwannoma that markedly enlarges the IAC as well as exerts mass effect upon the adjacent pons, brachium pontis, and cerebellum.](images/app.statdx.com_image_thumbnail_88ac0486-9a5c-4aad-a128-03d6be231847_size_174_quality_85_0ce7e751.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ FS MR shows a large, avidly enhancing left CPA-IAC vestibular schwannoma that markedly enlarges the IAC as well as exerts mass effect upon the adjacent pons, brachium pontis, and cerebellum.* + +![Axial T1 C+ FS MR shows large CPA-IAC masses with heterogeneous, but avid enhancement that enlarge the IACs bilaterally . When present, the finding of bilateral vestibular schwannomas is effectively pathognomonic for the diagnosis of NF2.](images/app.statdx.com_image_thumbnail_1a309ca6-ca3d-4944-aa62-39b46d718561_annotated_true_size_900_quality_90_6aba34f5a714a83b2613bdd5fd61167eaa928764.jpg) +**Neurofibromatosis Type 2, CPA-IAC** +*Axial T1 C+ FS MR shows large CPA-IAC masses with heterogeneous, but avid enhancement that enlarge the IACs bilaterally . When present, the finding of bilateral vestibular schwannomas is effectively pathognomonic for the diagnosis of NF2.* + +![Axial bone NECT shows a large, destructive left petrous apex metastasis that has eroded into and widened the left IAC . Note the caliber of the normal right IAC for comparison.](images/app.statdx.com_image_thumbnail_2b9ac7d3-bb46-4f9a-a1d7-3dddb0d8c434_annotated_true_size_900_quality_90_317c3805d7f0cc53f4e3ac9d205902e19c619abd.jpg) +**Metastases, CPA-IAC** +*Axial bone NECT shows a large, destructive left petrous apex metastasis that has eroded into and widened the left IAC . Note the caliber of the normal right IAC for comparison.* + +![Axial T2 FS MR in an infant with a facial hemangioma (not shown) demonstrates left cerebellar hypoplasia with large retrocerebellar CSF space and enlarged ipsilateral IAC , a constellation of findings indicative of PHACES.](d145c1f8-395d-4830-a6db-3262dba780fc) +**PHACES Association** +*Axial T2 FS MR in an infant with a facial hemangioma (not shown) demonstrates left cerebellar hypoplasia with large retrocerebellar CSF space and enlarged ipsilateral IAC , a constellation of findings indicative of PHACES.* + +![Axial 3D T2-SPACE MR shows a CPA-IAC mass extending along the posterior wall of the IAC to the fundus, then along the labyrinthine segment of CNVII. Although CNVII sits anteriorly in the IAC, this schwannoma was displaced by a large cystic component that is slightly hypointense to CSF .](b5fa047e-2c0e-4599-9a80-cacfbb37ec37) +**Schwannoma, Facial Nerve, CPA-IAC** +*Axial 3D T2-SPACE MR shows a CPA-IAC mass extending along the posterior wall of the IAC to the fundus, then along the labyrinthine segment of CNVII. Although CNVII sits anteriorly in the IAC, this schwannoma was displaced by a large cystic component that is slightly hypointense to CSF .* + +![Axial bone CT in an infant with a CPA-IAC mass shows a large irregular IAC and facial nerve canal due to atypical teratoid/rhabdoid tumor, diagnosed following resection.](2f24d86f-f737-4802-9a78-1b404dbb8481) +**Atypical Teratoid/Rhabdoid Tumor** +*Axial bone CT in an infant with a CPA-IAC mass shows a large irregular IAC and facial nerve canal due to atypical teratoid/rhabdoid tumor, diagnosed following resection.* + +![Axial T2 MR shows a malformed, featureless vestibule and cochlea . The wide IAC houses CNVIII and CNVII , which are splayed apart. Note a hypoplastic pons and a malformed cerebellum. The mastoid and middle ear fluid could be serous (common) or conceivably result from a perilymph fistula (rare).](6856a487-5052-432d-8525-5ea56406b712) +**Cystic Cochleovestibular Malformation (IP-I)** +*Axial T2 MR shows a malformed, featureless vestibule and cochlea . The wide IAC houses CNVIII and CNVII , which are splayed apart. Note a hypoplastic pons and a malformed cerebellum. The mastoid and middle ear fluid could be serous (common) or conceivably result from a perilymph fistula (rare).* + +![Axial bone CT in a boy with X-linked mixed hearing loss shows a wide lateral IAC . The corkscrew-shaped cochlea lacks internal septation or a modiolus. The wide lateral SCC is partially ossified .](b1dc45b9-866f-44fd-8658-b8b225fe40d7) +**X-Linked Stapes Gusher (DFNX2)** +*Axial bone CT in a boy with X-linked mixed hearing loss shows a wide lateral IAC . The corkscrew-shaped cochlea lacks internal septation or a modiolus. The wide lateral SCC is partially ossified .* + + +### Additional Images + +![Axial T1 C+ MR in an ataxic teenager shows an avidly enhancing mass widening the CPA cistern , distorting the pons, and expanding the IAC , consistent with schwannoma, which was confirmed after resection.](images/app.statdx.com_image_thumbnail_9a0cb6f0-784b-42f2-b822-a25650cff8f7_annotated_true_size_900_quality_90_c0bfe289f983427be1db0f4df650371386d998db.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ MR in an ataxic teenager shows an avidly enhancing mass widening the CPA cistern , distorting the pons, and expanding the IAC , consistent with schwannoma, which was confirmed after resection.* + +![Axial T1 C+ FS MR in a patient with NF2 shows bilateral, avidly enhancing IAC tumors extending into the left cochlear canal and along the vestibular nerves . A V3 schwannoma is also seen .](images/app.statdx.com_image_thumbnail_f6f61471-bd7d-493f-ad33-6dbc46ac6771_annotated_true_size_900_quality_90_05ebde3146babae5f1b1948c45cd13f8f3fe4cc8.jpg) +**Neurofibromatosis Type 2, CPA-IAC** +*Axial T1 C+ FS MR in a patient with NF2 shows bilateral, avidly enhancing IAC tumors extending into the left cochlear canal and along the vestibular nerves . A V3 schwannoma is also seen .* + +![Axial 3D T2 SPACE MR in a teenager with NF2 shows bilateral IAC-CPA schwannomas in widened IACs . There is also a V3 schwannoma in the foramen ovale .](images/app.statdx.com_image_thumbnail_13ea0702-80c6-40dd-8d56-44b50ca0d461_annotated_true_size_900_quality_90_d6b4faa7984b80273de11ed20df6c9d83e2c8b69.jpg) +**Neurofibromatosis Type 2, CPA-IAC** +*Axial 3D T2 SPACE MR in a teenager with NF2 shows bilateral IAC-CPA schwannomas in widened IACs . There is also a V3 schwannoma in the foramen ovale .* + +![Axial T1WI C+ FS MR in an infant with otalgia and CNVII palsy shows an enhancing tumor expanding the IAC and CPA cistern, invading the petrous apex , CNVII , and the middle ear space. Bony destruction was seen on CT. The differential diagnosis included rhabdomyosarcoma, ATRT, or metastases. A final diagnosis of metastatic neuroblastoma was made after middle ear biopsy.](images/app.statdx.com_image_thumbnail_47ee0c39-5fcf-4e15-b9bf-eeffa4878021_annotated_true_size_900_quality_90_5f2e1eeaaa4264684afa91b70d2cfd6f152fb66c.jpg) +**Metastases, CPA-IAC** +*Axial T1WI C+ FS MR in an infant with otalgia and CNVII palsy shows an enhancing tumor expanding the IAC and CPA cistern, invading the petrous apex , CNVII , and the middle ear space. Bony destruction was seen on CT. The differential diagnosis included rhabdomyosarcoma, ATRT, or metastases. A final diagnosis of metastatic neuroblastoma was made after middle ear biopsy.* + +![Coronal T2 FSE MR shows a patient with CSF dissemination of glioneuronal neoplasm. The hyperintense tumor filling the widened IACs is hard to distinguish from CSF on this image but was more evident on FLAIR images. The patient had large IACs at the time of presentation. As CSF tumor dissemination/metastatic disease occurred, the IACs showed progressive massive enlargement over time on successive MR studies.](images/app.statdx.com_image_thumbnail_91a2ffeb-107e-43d1-8340-755256605248_annotated_true_size_900_quality_90_05f8cf99981253a201661009dc8d24527516acf9.jpg) +**Metastases, CPA-IAC** +*Coronal T2 FSE MR shows a patient with CSF dissemination of glioneuronal neoplasm. The hyperintense tumor filling the widened IACs is hard to distinguish from CSF on this image but was more evident on FLAIR images. The patient had large IACs at the time of presentation. As CSF tumor dissemination/metastatic disease occurred, the IACs showed progressive massive enlargement over time on successive MR studies.* + +![Axial T2 MR in an infant with a facial hemangioma shows left cerebellar hypoplasia with a prominent adjacent CSF space, large flared left IAC , and enlarged Meckel cave .](f7ef04fb-9994-491f-ae3e-c96182cd6127) +**PHACES Association** +*Axial T2 MR in an infant with a facial hemangioma shows left cerebellar hypoplasia with a prominent adjacent CSF space, large flared left IAC , and enlarged Meckel cave .* + +![Axial bone CT in a patient with profound SNHL shows an enlarged vestibule and lateral SCC with an enlarged IAC . Extensive opacification of the mastoid air cells and middle ear space is also seen.](9381e43a-a30f-40f5-9961-26e5f346aef8) +**Cystic Cochleovestibular Malformation (IP-I)** +*Axial bone CT in a patient with profound SNHL shows an enlarged vestibule and lateral SCC with an enlarged IAC . Extensive opacification of the mastoid air cells and middle ear space is also seen.* + +![Axial T2 MR in a teenager with NF1 shows large IACs bilaterally with no evidence of inner ear anomaly or IAC mass lesion. This is a feature of mild dural ectasia in NF1.](4a78e8d6-6747-442e-aab8-235f2c5f4b7b) +**Neurofibromatosis Type 1** +*Axial T2 MR in a teenager with NF1 shows large IACs bilaterally with no evidence of inner ear anomaly or IAC mass lesion. This is a feature of mild dural ectasia in NF1.* + diff --git a/docs_md/articles/mesial-temporal-sclerosis_3861ee73-c82c-49f2-a60f-8fd08f7e6165.md b/docs_md/articles/mesial-temporal-sclerosis_3861ee73-c82c-49f2-a60f-8fd08f7e6165.md new file mode 100644 index 0000000..8c75013 --- /dev/null +++ b/docs_md/articles/mesial-temporal-sclerosis_3861ee73-c82c-49f2-a60f-8fd08f7e6165.md @@ -0,0 +1,463 @@ +--- +title: "Mesial Temporal Sclerosis" +docid: "3861ee73-c82c-49f2-a60f-8fd08f7e6165" +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: "Pathology-Based Diagnoses" + slug: "pathology-based-diagnoses" + treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77" + - + name: "Acquired Toxic/Metabolic/Degenerative Disorders" + slug: "acquired-toxicmetabolicdegenerativ-" + treeNodeId: "ba3cfeaf-64d9-4117-91e8-d2ce58783fc5" + - + name: "Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations" + slug: "toxic-metabolic-nutritional-system-" + treeNodeId: "06bd883b-8269-4044-8411-70f7ab75bb7a" + - + name: "Mesial Temporal Sclerosis" + slug: "mesial-temporal-sclerosis" + treeNodeId: null +category: "Brain" +cmeTopicId: "3cf4636d-0dca-4ef5-9436-e9548e51ffaa" +documentVersionId: "50d12781-ca5e-4e04-95ef-7b0e6babf338" +imageCount: 22 +lastUpdated: "07/31/20" +pageDescription: "Mesial Temporal Sclerosis" +pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations, Mesial Temporal Sclerosis" +pageTitle: "Mesial Temporal Sclerosis | STATdx" +enhancedTitle: "Mesial Temporal Sclerosis" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Pathology-Based Diagnoses" + - "Acquired Toxic/Metabolic/Degenerative Disorders" + - "Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations" + - "Mesial Temporal Sclerosis" +--- +# KEY FACTS + +- ## Terminology + + + - Seizure-associated neuronal loss & gliosis in hippocampus & adjacent structures +- ## Imaging + + + - Primary features: Abnormal T2 hyperintensity, hippocampal volume loss/atrophy, obscuration of internal architecture + - Secondary signs: Ipsilateral fornix & mammillary body atrophy, enlarged ipsilateral temporal horn, & choroidal fissure + - ↑ hyperintensity on DWI (T2 shine-through) + - ↓ NAA in hippocampus, temporal lobe +- ## Top Differential Diagnoses + + + - Status epilepticus + - Low-grade astrocytoma + - Choroidal fissure cyst + - Hippocampal sulcus remnant + - Focal cortical dysplasia +- ## Pathology + + + - Prolonged febrile seizures may produce acute hippocampal injury → subsequent atrophy + - Coexistent 2nd developmental lesion in 15% of patients with mesial temporal sclerosis (MTS) +- ## Clinical Issues + + + - Partial complex seizures + - Often history of childhood febrile or medically intractable seizures + - Surgical temporal lobectomy reserved for medically intractable seizures, intolerable drug side effects +- ## Diagnostic Checklist + + + - Most common cause of partial complex epilepsy in adult age group + - Low-grade neoplasms & focal cortical dysplasia more common causes of partial complex epilepsy than MTS in pediatric age group + +# TERMINOLOGY + +- ## Abbreviations + + + - Mesial temporal sclerosis (MTS) +- ## Synonyms + + + - Ammon horn sclerosis, hippocampal sclerosis (HS) +- ## Definitions + + + - Seizure-associated neuronal loss & gliosis in hippocampus & adjacent structures + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Primary features: Abnormal T2 hyperintensity, hippocampal volume loss/atrophy, obscuration of internal architecture + - Secondary signs: Ipsilateral fornix & mammillary body atrophy, enlarged ipsilateral temporal horn, & choroidal fissure + - Additional findings: Loss of ipsilateral hippocampal head (pes) digitations, parahippocampal gyrus white matter (WM) atrophy, ↑ T2 signal in anterior temporal WM + - ### Location + + + - Mesial temporal lobe(s), 15-20% bilateral but usually asymmetric + - Hippocampus > amygdala > fornix > mammillary bodies + - ### Size + + + - Slight to marked ↓ in hippocampal volume + - ### Morphology + + + - Abnormal shape, size of affected hippocampus +- ## CT Findings + + + - ### NECT + + + - Usually normal; CT insensitive to MTS +- ## MR Findings + + + - ### T1WI + + + - ↓ hippocampal size + - Loss of normal hippocampal gray-white differentiation + - ± ipsilateral fornix, mammillary body atrophy + - Quantitative hippocampal volumetry: ↑ sensitivity of MTS detection (particularly bilateral MTS) + - ### T2WI + + + - Hippocampal atrophy + - Obscuration of normal internal architecture + - ↑ hippocampal signal intensity + - ± ipsilateral fornix, mammillary body atrophy, dilatation of ipsilateral temporal horn + - ± abnormal hyperintensity, volume loss in ipsilateral anterior temporal lobe + - ### FLAIR + + + - Hyperintense signal in abnormal hippocampus + - ### DWI + + + - ↑ hyperintensity on DWI (T2 shine-through) + - ↑ diffusivity on ADC + - DTI: ↓ fractional anisotropy affecting widespread WM tracts, extensive areas with ↑ radial diffusivities independent of disease side + - ### T1WI C+ + + + - No enhancement + - ### MRS + + + - ↓ NAA in hippocampus, temporal lobe + - ↓ NAA/Cho & ↓ NAA/Cho+Cr suggests MTS + - ± lactate/lipid peaks after 24 hours of continual seizure +- ## Angiographic Findings + + + - Presurgical Wada testing: Neuropsychologic testing after intracarotid amobarbital (Amytal) injection + - Lateralize memory & language functions + - Predict postoperative memory loss, feasibility of surgery + - May help lateralize seizure onset + - fMRI mapping replacing Wada testing +- ## Nuclear Medicine Findings + + + - FDG PET: Hypometabolism in abnormal mesial temporal lobe + - SPECT: Hypoperfusion (interictal) or hyperperfusion (ictal) in epileptogenic zone (EZ) + - Sensitivity of ictal > interictal +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - High-resolution MR imaging + - MRS, quantitative volumetry may help lateralize MTS in difficult cases + - ### Protocol advice + + + - Thin-section coronal T2WI & FLAIR (3 mm), coronal 3D SPGR (1-2 mm), angled perpendicular to long axis of hippocampus + +# DIFFERENTIAL DIAGNOSIS + +- [Status Epilepticus](/document/status-epilepticus/a058b733-4b80-46a1-8097-d68685ecf921) + - Clinical history of multiple seizures or status epilepticus + - Temporary T2 hyperintensity ± gyriform enhancement in affected cortex, hippocampus +- ## Low-Grade Astrocytoma + + + - Hyperintense temporal lobe WM mass (usually nonenhancing) + - ± seizures, young adults typical +- [Choroidal Fissure Cyst](/document/choroid-fissure-cyst/f60887bb-f021-401f-80b2-1d79e0a758e5) + - Asymptomatic CSF signal cyst in choroidal fissure distorts normal hippocampus + - Round on axial, coronal images + - Oval, parallels temporal lobe long axis on sagittal imaging + - No abnormal T2 hyperintensity in mesial temporal lobe +- [Hippocampal Sulcus Remnant](/document/hippocampal-sulcus-remnant-cysts/3b54dc78-2c77-4cbb-9ab4-e5c6f5d8b228) + - Failure of normal hippocampal sulcus involution → asymptomatic cyst between dentate gyrus, cornu ammonis (CA) + - Common normal variant (10-15%) +- [Cavernous Malformation](/document/cavernous-malformation/d6c0dfc6-25d3-4713-941f-373c68ca8f0d) + - Heterogeneous hyperintense "popcorn" lesion with dark complete hemosiderin rim + - ± seizures +- ## Dysembryoplastic Neuroepithelial Tumor + + + - [Demarcated "bubbly," variably enhancing cortical mass ± regional cortical dysplasia](/document/dnet/30baaad9-4835-4cf0-8b95-974d6517511e) + - Partial complex seizures +- [Focal Cortical Dysplasia](/document/focal-cortical-dysplasia/046564e0-5bb7-4f23-8a3e-010a68cfbafe) + - Most common dual pathology associated with MTS + - T2 hyperintensity in anterior temporal WM + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Controversial whether acquired or developmental + - Acquired: Follows complicated febrile seizures (FS), status epilepticus, encephalitis + - Developmental: 2nd developmental lesion identified in 15% + - 2-hit hypothesis: (1) Initial precipitating injury (like complicated seizures), (2) ↑ vulnerability (such as genetic predisposition or developmental anomaly) + - Most likely MTS represents common outcome of both acquired & developmental processes + - FS most common childhood seizure disorder (2-5%) + - Prolonged FS may produce acute hippocampal injury → subsequent atrophy + - ### Genetics + + + - Familial cases of mesial temporal lobe epilepsy (TLE), FS reported + - Recent studies suggest relationship between FS & later epilepsy development may be genetic + - Syndrome-specific genes for FS (channelopathies) account for small proportion of FS cases + - ### Associated abnormalities + + + - Coexistent 2nd developmental lesion (15%) +- ## Gross Pathologic & Surgical Features + + + - Normal hippocampus divided anatomically into head (pes), body, tail + - Subdivision into Ammon horn, dentate gyrus, hippocampal sulcus, fimbria, alveus, subiculum, parahippocampal gyrus, collateral sulcus + - Mesial temporal lobe atrophy: Hippocampal body (85-90%), tail (60%), head (50%), amygdala (10%) + - Absence of hemorrhage or necrosis + - HS described by gross pathologists as shrunken & hardened hippocampus with characteristic histologic neuronal loss, glial proliferation +- ## Microscopic Features + + + - Chronic astrogliosis with fine fibrillary background of bland astrocytic nuclei & ↓ residual neurons + - Selective loss of inhibitory interneurons, abnormal axonal sprouting, reorganization of neural transmitter receptors, alterations in 2nd messenger systems, & hyperexcitability of granule cells + - Ammon horn, CA, contains 4 zones of granular cells: CA1, CA2, CA3, CA4 + - CA1, CA4 pyramidal cell layers most susceptible to ischemia + - All hippocampal regions may show varying neuronal cell loss + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Partial complex seizures, automatisms + - Simple at younger ages, increasingly complex & discrete with age + - Motor manifestations ↓ with ↑ age; less abundant in adults + - ### Other signs/symptoms + + + - May progress to generalized tonic-clonic seizures + - ### Clinical profile + + + - Often history of childhood febrile or medically intractable seizures + - History of complex or prolonged FS ↑ risk for development of hippocampal injury, MTS + - Surface electro- (EEG) or magneto- (MEG) encephalogram helpful for localization (60-90%) + - Intracranial EEG (subdural or depth electrodes) may be indicated if noninvasive studies discordant +- ## Demographics + + + - ### Age + + + - Disease of older children, young adults + - ### Sex + + + - No sex predominance + - ### Epidemiology + + + - MTS accounts for majority of epilepsy patients undergoing temporal lobe seizure surgery +- ## Natural History & Prognosis + + + - Anterior temporal lobectomy 70-90% successful in MTS, 40-55% if MR normal + - ↓ surgical success when amygdala involved +- ## Treatment + + + - Clinical management based on phenotypic features of initial febrile & subsequent seizures + - Medical treatment initial approach + - Surgical temporal lobectomy reserved for medically intractable seizures, intolerable drug side effects + - Resection includes anterior temporal lobe, majority of hippocampus, variable portions of amygdala + - Surgical resection of EZ + - MR-guided laser interstitial thermal therapy (MRgLITT) less invasive option for stereotactic ablation of EZ + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Most common cause of partial complex epilepsy in adults + - Bilateral in 15-20%; difficult to detect without quantitative volumetry unless severe + - MTS imaging findings not found in normal seizure-free patients (controversial) +- ## Image Interpretation Pearls + + + - Coronal high-resolution T2WI, FLAIR MR most sensitive for MTS; dual pathology in 15% + - In pediatric age group, low-grade neoplasms & cortical dysplasia more common causes of partial complex epilepsy than MTS + + f1594712-2ab3-4d9e-9a3d-a75b51f1c879 + +## References + +# Selected References + +1. 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[Kumlien E et al: Treatment outcome in patients with mesial temporal sclerosis. Seizure. 11(7):413-7, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12237065%5Bpmid%5D) +1. [Kuzniecky RI et al: Neuroimaging of epilepsy. Semin Neurol. 22(3):279-88, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12528053%5Bpmid%5D) +1. [Lewis DV et al: Do prolonged febrile seizures produce medial temporal sclerosis? Hypotheses, MRI evidence and unanswered questions. Prog Brain Res. 135:263-78, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12143347%5Bpmid%5D) +1. [Scott RC et al: Magnetic resonance imaging findings within 5 days of status epilepticus in childhood. Brain. 125(Pt 9):1951-9, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12183341%5Bpmid%5D) +1. [Spencer SS: When should temporal-lobe epilepsy be treated surgically? Lancet Neurol. 1(6):375-82, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12849399%5Bpmid%5D) +1. [Castillo M et al: Proton MR spectroscopy in patients with acute temporal lobe seizures. AJNR Am J Neuroradiol. 22(1):152-7, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11158901%5Bpmid%5D) +1. [Moore KR et al: Incidental detection of hippocampal sclerosis on MR images: is it significant? AJNR Am J Neuroradiol. 20(9):1609-12, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10543629%5Bpmid%5D) +1. [Ho SS et al: Temporal lobe developmental malformations and epilepsy: dual pathology and bilateral hippocampal abnormalities. Neurology. 50(3):748-54, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9521268%5Bpmid%5D) +1. [Lee DH et al: MR in temporal lobe epilepsy: analysis with pathologic confirmation. AJNR Am J Neuroradiol. 19(1):19-27, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9432153%5Bpmid%5D) +1. [Bronen RA et al: Regional distribution of MR findings in hippocampal sclerosis. AJNR Am J Neuroradiol. 16(6):1193-200, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7677010%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Coronal graphic depicts the characteristic appearance of mesial temporal sclerosis (MTS). The right hippocampus is small (atrophic) with loss of normal internal architecture, reflecting neuronal loss and gliosis. Note concordant atrophy of the ipsilateral fornix and widening of the ipsilateral temporal horn and choroidal fissure.](images/app.statdx.com_image_thumbnail_7d1af415-dfe9-43d5-b1fc-066a3bf8596b_annotated_true_size_900_quality_90_036a6961a4b8720aa4a9dff2b22d51cabee4f91e.jpg) +*Coronal graphic depicts the characteristic appearance of mesial temporal sclerosis (MTS). The right hippocampus is small (atrophic) with loss of normal internal architecture, reflecting neuronal loss and gliosis. Note concordant atrophy of the ipsilateral fornix and widening of the ipsilateral temporal horn and choroidal fissure.* + +![Coronal T2 MR at 3.0T demonstrates normal bilateral hippocampal anatomy , size, and signal intensity. Note distinct layers of gray and white matter in the hippocampus.](images/app.statdx.com_image_thumbnail_5a791ed0-a397-49d3-9136-42f932e8575f_annotated_true_size_900_quality_90_94cab71fb76799e82e9e64fe2c4dfa6abb3d50ff.jpg) +*Coronal T2 MR at 3.0T demonstrates normal bilateral hippocampal anatomy , size, and signal intensity. Note distinct layers of gray and white matter in the hippocampus.* + +![Coronal T1 true inversion recovery MR at 3.0T shows asymmetric right hippocampal volume loss and obscuration of normal internal gray-white differentiation. The ipsilateral fornix is smaller than the normal left fornix.](images/app.statdx.com_image_thumbnail_71823a62-6d76-4742-b5ff-1d85bf0f8431_annotated_true_size_900_quality_90_a9eea9db237ba0a5f39aeced5764882a73552503.jpg) +*Coronal T1 true inversion recovery MR at 3.0T shows asymmetric right hippocampal volume loss and obscuration of normal internal gray-white differentiation. The ipsilateral fornix is smaller than the normal left fornix.* + +![Coronal T2 MR at 3.0T in the same patient with right hippocampal sclerosis (HS) shows hippocampal volume loss and obscuration of normal internal architecture but normal T2 signal intensity. FLAIR better shows the increase in signal intensity.](images/app.statdx.com_image_thumbnail_07c5b79f-0792-4872-b20a-8d5090303401_annotated_true_size_900_quality_90_9e3035fdd5e6039063552a0e48822ba21a2a0f6e.jpg) +*Coronal T2 MR at 3.0T in the same patient with right hippocampal sclerosis (HS) shows hippocampal volume loss and obscuration of normal internal architecture but normal T2 signal intensity. FLAIR better shows the increase in signal intensity.* + +![Coronal T2 MR in a patient with epilepsy shows a vertical left collateral sulcus and a globular hippocampus . This common variation is due to failure of hippocampal inversion.](images/app.statdx.com_image_thumbnail_94bc080d-63d6-48ab-9abb-da369015f505_annotated_true_size_900_quality_90_d9f3bda3d2fbd5b11890a9c80badccd7341abeba.jpg) +*Coronal T2 MR in a patient with epilepsy shows a vertical left collateral sulcus and a globular hippocampus . This common variation is due to failure of hippocampal inversion.* + +![Coronal T2 MR in a patient with temporal lobe epilepsy demonstrates primary and secondary imaging features of MTS. There is severe left hippocampal atrophy and hyperintensity . Secondary features are atrophy of the left fornix and mammillary body as well as dilation of temporal horn .](images/app.statdx.com_image_thumbnail_02ff763e-4461-4fa8-8e1e-777ae26bb1a6_annotated_true_size_900_quality_90_69c45cf047e85d16f79d487a42d986972570f60d.jpg) +*Coronal T2 MR in a patient with temporal lobe epilepsy demonstrates primary and secondary imaging features of MTS. There is severe left hippocampal atrophy and hyperintensity . Secondary features are atrophy of the left fornix and mammillary body as well as dilation of temporal horn .* + +![Coronal T2-weighted MR at 3.0T in a patient with prolonged febrile seizure shows abnormal enlargement and T2 hyperintensity in the right hippocampus . DWI (not shown) revealed reduced diffusion. The patient later developed HS.](images/app.statdx.com_image_thumbnail_74b0b992-2cdf-45eb-8017-85fc1e87611e_annotated_true_size_900_quality_90_3f5feaac83668e19a878ee41c2e943eecc95b7f7.jpg) +*Coronal T2-weighted MR at 3.0T in a patient with prolonged febrile seizure shows abnormal enlargement and T2 hyperintensity in the right hippocampus . DWI (not shown) revealed reduced diffusion. The patient later developed HS.* + +![Coronal FLAIR MR in a patient with longstanding partial complex epilepsy shows bilateral hyperintense hippocampi with loss of normal architecture, volume loss, R > L, consistent with bilateral mesial temporal sclerosis.](images/app.statdx.com_image_thumbnail_4baba424-f042-4233-9ee7-54e4780a61cf_annotated_true_size_900_quality_90_597f982c61c3d3471423b53cd1b35cf280483532.jpg) +*Coronal FLAIR MR in a patient with longstanding partial complex epilepsy shows bilateral hyperintense hippocampi with loss of normal architecture, volume loss, R > L, consistent with bilateral mesial temporal sclerosis.* + +![Coronal T2WI MR in a patient with chronic seizures with large right temporal lobe cavernous malformation demonstrates all 3 primary determinants of right HS (volume loss, T2 hyperintensity, and loss of internal architecture).](images/app.statdx.com_image_thumbnail_7e17968d-3747-4a67-9267-e700cbe7d354_annotated_true_size_900_quality_90_06145e7a230f709c09d1602bd00fa9b5b2d34167.jpg) +*Coronal T2WI MR in a patient with chronic seizures with large right temporal lobe cavernous malformation demonstrates all 3 primary determinants of right HS (volume loss, T2 hyperintensity, and loss of internal architecture).* + +![Coronal FLAIR MR in the same patient with right temporal lobe cavernous malformation better shows HS . Hyperintensity is usually more conspicuous on FLAIR, while T2 is better for depicting internal structure.](images/app.statdx.com_image_thumbnail_f365f7c5-ef03-453c-ab72-5e5a013fd7ed_annotated_true_size_900_quality_90_7b1eab3ade8a4f5ce6f2d370699340d2a6ad6f24.jpg) +*Coronal FLAIR MR in the same patient with right temporal lobe cavernous malformation better shows HS . Hyperintensity is usually more conspicuous on FLAIR, while T2 is better for depicting internal structure.* + + +### Additional Images + +![Coronal T2WI MR shows classic left hippocampal sclerosis with abnormal T2 hyperintensity, ipsilateral atrophy, and loss of internal architecture.](images/app.statdx.com_image_thumbnail_20da7e0c-b7da-4c58-948a-a05d30898252_annotated_true_size_900_quality_90_dbd8b754c54d0c28891b2802a0db7fa7f22cc3fc.jpg) +*Coronal T2WI MR shows classic left hippocampal sclerosis with abnormal T2 hyperintensity, ipsilateral atrophy, and loss of internal architecture.* + +![Coronal T1WI 3D SPGR demonstrates classic left HS with ipsilateral atrophy and loss of normal internal gray-white differentiation.](images/app.statdx.com_image_thumbnail_e4dae355-94eb-46ee-a004-6593e2f19865_annotated_true_size_900_quality_90_ff9dfb5ce3a2b13cdfba9e269d93851462afd250.jpg) +*Coronal T1WI 3D SPGR demonstrates classic left HS with ipsilateral atrophy and loss of normal internal gray-white differentiation.* + +![Coronal FLAIR MR in a patient with complex partial epilepsy with left hippocampal sclerosis shows ipsilateral volume loss and conspicuous hyperintensity resulting from gliosis.](images/app.statdx.com_image_thumbnail_6b2c6161-10e8-48cc-b72f-64be747eecb2_annotated_true_size_900_quality_90_0c6c5bbe354dfdc0d5c6dafef56c39418727d646.jpg) +*Coronal FLAIR MR in a patient with complex partial epilepsy with left hippocampal sclerosis shows ipsilateral volume loss and conspicuous hyperintensity resulting from gliosis.* + +![Coronal T2WI MR in a 23 year old with complex partial seizures reveals abnormal hyperintensity and atrophy of the right hippocampus . Loss of internal architecture is also seen, typical of MTS.](images/app.statdx.com_image_thumbnail_82bd91b0-0ea8-4335-9c91-07c8748ff073_annotated_true_size_900_quality_90_e12442430b18bfd85385dcd45f2708b57159efce.jpg) +*Coronal T2WI MR in a 23 year old with complex partial seizures reveals abnormal hyperintensity and atrophy of the right hippocampus . Loss of internal architecture is also seen, typical of MTS.* + +![Coronal T2WI MR shows abnormal hyperintense signal and atrophy of the right anterior temporal lobe related to prior injury.](aa315f34-25ff-4833-a882-aa73bbeece45) +*Coronal T2WI MR shows abnormal hyperintense signal and atrophy of the right anterior temporal lobe related to prior injury.* + +![Coronal T2WI MR in a patient with acute complex partial seizures shows abnormal enlargement and hyperintensity of the right hippocampus . Follow-up imaging 9 months later (not shown) confirmed subsequent development of right MTS.](75e76424-620b-4878-b335-00ca48858d58) +*Coronal T2WI MR in a patient with acute complex partial seizures shows abnormal enlargement and hyperintensity of the right hippocampus . Follow-up imaging 9 months later (not shown) confirmed subsequent development of right MTS.* + +![Coronal STIR MR at 3.0T in a normal nonepileptic patient shows a prominent left collateral sulcus that changes the morphology of the adjacent normal hippocampus. This common anatomical variant can be mistaken for HS.](c59e6abd-cef6-40e5-8956-b6e0d31a06ec) +*Coronal STIR MR at 3.0T in a normal nonepileptic patient shows a prominent left collateral sulcus that changes the morphology of the adjacent normal hippocampus. This common anatomical variant can be mistaken for HS.* + +![Coronal STIR MR at 3.0T in an individual who had been born prematurely with developmental delay shows diffuse white matter volume loss (L > R) and concordant left hippocampal volume loss with the abnormal T2 hyperintensity of HS.](1b9cc8f7-349a-44c9-b7b6-be26f0f6072a) +*Coronal STIR MR at 3.0T in an individual who had been born prematurely with developmental delay shows diffuse white matter volume loss (L > R) and concordant left hippocampal volume loss with the abnormal T2 hyperintensity of HS.* + +![Coronal T2WI FS MR in a patient with longstanding partial complex epilepsy shows bilateral shrunken, hyperintense hippocampi associated with diminished white matter volume in temporal lobes, consistent with bilateral HS.](ca10ec92-72c0-4b27-87ef-3fca0659c0a0) +*Coronal T2WI FS MR in a patient with longstanding partial complex epilepsy shows bilateral shrunken, hyperintense hippocampi associated with diminished white matter volume in temporal lobes, consistent with bilateral HS.* + +![Coronal STIR MR at 3.0 T in a normal nonepileptic patient imaged for headaches demonstrates normal bilateral hippocampal anatomy , size, and signal intensity.](56e7abb7-09b7-4b92-990b-b4889c385a93) +*Coronal STIR MR at 3.0 T in a normal nonepileptic patient imaged for headaches demonstrates normal bilateral hippocampal anatomy , size, and signal intensity.* + +![Coronal T2 MR in a patient with epilepsy shows a vertical left collateral sulcus and a globular hippocampus . This common variation can be mistaken for HS.](49fcab4e-9e3a-4310-a2ef-697a78d2f99d) +*Coronal T2 MR in a patient with epilepsy shows a vertical left collateral sulcus and a globular hippocampus . This common variation can be mistaken for HS.* + +![Coronal T2 MR in a patient with longstanding partial complex epilepsy shows bilateral hyperintense hippocampi with volume loss, R > L, consistent with bilateral MTS.](ce5f4cd7-f97e-4b62-baff-1684a6631f09) +*Coronal T2 MR in a patient with longstanding partial complex epilepsy shows bilateral hyperintense hippocampi with volume loss, R > L, consistent with bilateral MTS.* + diff --git a/docs_md/articles/moyamoya_c820f6bf-ddb6-4e75-b0ca-61263ed63b21.md b/docs_md/articles/moyamoya_c820f6bf-ddb6-4e75-b0ca-61263ed63b21.md new file mode 100644 index 0000000..b092fe3 --- /dev/null +++ b/docs_md/articles/moyamoya_c820f6bf-ddb6-4e75-b0ca-61263ed63b21.md @@ -0,0 +1,422 @@ +--- +title: "Moyamoya" +docid: "c820f6bf-ddb6-4e75-b0ca-61263ed63b21" +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: "Stroke" + slug: "stroke" + treeNodeId: "12307683-f1ff-4823-a7d3-b10b40f9fd82" + - + name: "Nonatheromatous Vasculopathy" + slug: "nonatheromatous-vasculopathy" + treeNodeId: "ed2d2a03-ebd6-4a72-8608-effc92deb342" + - + name: "Moyamoya" + slug: "moyamoya" + treeNodeId: null +category: "Brain" +cmeTopicId: "6d619830-666a-4fbd-8e1c-9768a7d22b02" +documentVersionId: "61b569aa-0c21-432e-9ddc-9990f9d19651" +imageCount: 18 +lastUpdated: "10/08/20" +pageDescription: "Moyamoya" +pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Nonatheromatous Vasculopathy, Moyamoya" +pageTitle: "Moyamoya | STATdx" +enhancedTitle: "Moyamoya" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Pathology-Based Diagnoses" + - "Stroke" + - "Nonatheromatous Vasculopathy" + - "Moyamoya" +--- +# KEY FACTS + +- ## Terminology + + + - Progressive narrowing of distal ICA/proximal circle of Willis (COW) vessels with secondary collateralization + - Collateral vessels → cloud-like "puff of smoke" (moyamoya) at angiography + - Moyamoya disease (MMD) = primary (idiopathic) moyamoya + - Marked East-West gradient (more common in Japan, Korea) + - Moyamoya pattern of collaterals = secondary to many etiologies +- ## Imaging + + + - Best diagnostic clue: Attenuated COW with multiple tiny basal ganglia flow voids on MR + - Best imaging tool + - T1 MR C+/MRA + - DSA for delineating vascular details +- ## Pathology + + + - MMD + - RNF213 polymorphism in 95% of familial East Asian population, 79% of sporadic + - Correlated with early onset, severe form of MMD + - Secondary moyamoya (many causes) + - Syndromic (e.g., NF1), inflammatory states, prothrombotic states, premature aging, congenital mesenchymal defects, suprasellar irradiation in childhood +- ## Clinical Issues + + + - Bimodal age peaks + - 5-10 years and 2nd peak during 4th decade + - Most frequent cause of stroke in Asian children + - Presentation + - Children: Recurrent transient ischemic attacks (TIAs); hemorrhage rare + - Adults: TIAs, infarcts, hemorrhage (20%), migraines + +# TERMINOLOGY + +- ## Abbreviations + + + - Moyamoya disease (MMD) +- ## Synonyms + + + - Idiopathic progressive arteriopathy of childhood, spontaneous occlusion of circle of Willis (COW) +- ## Definitions + + + - Progressive bilateral stenosis or occlusion of terminal internal carotid artery (ICA)/proximal COW with abnormal vascular network at base of brain + - Compensatory development of collateral vascular network = "puff of smoke" (hazy network of basal collaterals) + - Primary (idiopathic) moyamoya = MMD + - More common in Japan, Korea + - Secondary (acquired) moyamoya + - Cranial radiation + - Atherosclerosis + - Neurofibromatosis (NF) + +# IMAGING + +- ## General Features + + + - Best diagnostic clue: Attenuated COW with multiple tiny basal ganglia (BG) flow voids on MR + - Location: Supraclinoid ICA/COW; anterior > > posterior circulation + - Size: Large vessel occlusion + - Morphology: "Puff of smoke" (moyamoya in Japanese) + - Cloud-like lenticulostriate and thalamostriate collaterals on angiography +- ## CT Findings + + + - ### NECT + + + - Children: 50-60% show anterior > posterior atrophy + - Can present with stroke (children) or intracranial (IC) hemorrhage (more common in adults) + - CECT + - Enhancing dots (enlarged lenticulostriate arteries) in BG + - Abnormal net-like vessels at base of brain + - CTA: Abnormal COW and net-like collaterals + - CT perfusion + - Depicts penumbra, infarct core in ischemic MMD + - Can quantify effect of revascularization therapies +- ## MR Findings + + + - T1WI: Multiple dot-like flow voids in BG + - T2WI + - ↑ signal in small vessel cortical and white matter infarcts + - Collateral vessels = net-like filling defects in basal cisterns + - FLAIR + - Bright sulci = leptomeningeal ivy sign + - Slow-flowing engorged pial vessels, thickened arachnoid membranes + - Correlates with ↓ cerebral vascular reserve + - T2* GRE + - Hemosiderin if prior hemorrhage + - Asymptomatic microbleeds occasionally seen in adults + - DWI: Very useful for "acute on chronic" disease + - T1WI C+ + - Lenticulostriate collaterals → enhancing "dots" in BG and net-like thin vessels in cisterns + - Leptomeningeal enhancement (contrast-enhanced ivy sign) ↓ after effective bypass surgery + - MRA: Narrowed distal ICA and proximal COW vessels, ± synangiosis + - MRV: Some vasculopathies may also involve veins + - MRS: Lactate in acutely infarcted tissue + - NAA:Cr and Cho:Cr ratios frontal white matter improve/↑ after revascularization + - pMR: ↓ perfusion in deep hemispheric white matter, relative ↑ perfusion in posterior circulation + - Can also be helpful in evaluation of MMD following revascularization +- ## Ultrasonographic Findings + + + - Grayscale: Reduction of ICA lumen size + - Pulsed Doppler + - Doppler spectral waveforms in ICA show no flow (occluded) or high resistance (stenotic) flow pattern + - ↑ end-diastolic flow velocity, ↓ vascular resistance in external carotid artery (ECA) collaterals + - Color Doppler: Aliasing suggests stenoses + - Power Doppler: Contrast injection improves visualization of slow-flow stenotic vessels and collaterals +- ## Angiographic Findings + + + - Conventional + - Predominantly (not exclusively) anterior circulation + - Narrow proximal COW and ICA (earliest) + - Lenticulostriate and thalamoperforator collaterals (intermediate) + - Transdural and transosseous extracranial (EC)-IC collaterals (late) + - Dilatation of anterior choroidal artery + branches predicts adult hemorrhagic events +- ## Nuclear Medicine Findings + + + - PET: ↓ hemodynamic reserve capacity + - SPECT I-123-iomazenil: Neuronal density preserved if asymptomatic, ↓ if symptomatic +- ## Imaging Recommendations + + + - Best imaging tool: T1 C+ MR/MRA + - DSA for mapping collaterals, grading + - Protocol advice + - Contrast improves detection: Synangiosis, collaterals + - Catheter angiography defines anatomy of occlusions prior to bypass + - Diagnostic criteria: MR/MRA or catheter angiography + - Stenosis/occlusion of terminal ICA or proximal anterior cerebral artery (ACA) and middle cerebral artery (MCA) + - Abnormal vascular network/flow voids in BG + - Bilateral + - Unilateral findings in MCA = rete MCA anomaly + +# DIFFERENTIAL DIAGNOSIS + +- ## Ivy Sign + + + - Leptomeningeal metastases, subarachnoid hemorrhage, meningitis, ↑ inspired oxygen +- ## Punctate Foci in Basal Ganglia + + + - Cribriform lacunar state: No enhancement +- ## Severely Attenuated Circle of Willis + + + - Subarachnoid hemorrhage, meningitis, tumor encasement +- ## Rete Middle Cerebral Artery Anomaly + + + - Unilateral, not bilateral + - Weblike rete anomaly of MCA + - Twig-like horizontal MCA + branches + - May occur with aneurysm (50%) + - Occurs predominantly in Asians + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - MMD + - Ring finger protein 213 polymorphism in 95% of familial East Asian population, 79% of sporadic + - Correlated with early-onset, severe form of MMD + - Also recently reported in non-MMD disorders + - RNF213 vasculopathy spectrum (IC atherosclerosis, peripheral pulmonary artery stenosis, and rental artery stenosis) + - Secondary moyamoya + - Down syndrome, tuberous sclerosis, sickle cell disease, connective tissue disease, progeria, NF1 + - NF1 with suprasellar tumor and radiation is disastrous + - Morning glory syndrome; syndromes with aneurysms, cardiac and ocular defects + - Inflammatory: CNS angiitis (of childhood), basal meningitis, atherosclerosis, head and neck infections + - Vasculopathies and prothrombotic states: XRT, Kawasaki, anticardiolipin antibody, factor V Leiden, polyarteritis nodosa, Behçet, SLE + - Epidemiology: MMD + - Incidence in Japan: 1:100,000 + - Incidence in North America, Europe: 0.1:100,000 + - 10-15% familial +- ## Staging, Grading, & Classification + + + - Staging criteria (after Suzuki) + - Stage 1: Narrowing of ICA bifurcation + - Stage 2: ACA, MCA, posterior cerebral artery (PCA) dilated + - Stage 3: Maximal basal collaterals; small ACA/MCA + - Stage 4: Fewer collaterals (vessels); small PCA + - Stage 5: Further ↓ collaterals; absent ACA/MCA/PCA + - Stage 6: Extensive ECA-pial collaterals +- ## Gross Pathologic & Surgical Features + + + - ↑ perforating (early) and ECA-ICA (late) collaterals in atrophic brain + - Hemorrhage (subarachnoid, intraventricular > parenchymal) adults + - ↑ saccular aneurysms (especially basilar in adults) +- ## Microscopic Features + + + - Intimal hyperplasia, medial layer thinness + - Excessive infolding, "waving" of internal elastic lamina + - Progressive narrowing, eventual obliteration of vessel lumen + - Periventricular pseudoaneurysms (cause of hemorrhage) + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Children: Transient ischemic attacks (TIAs), alternating hemiplegia (exacerbated by crying), headache + - Adults: TIAs, cerebral infarct, or hemorrhage + - Hemorrhagic presentation more common in Asian adults + - ### Other signs/symptoms + + + - Children: Developmental delay, poor feeding, chorea + - ### Clinical profile + + + - Children more likely to have TIAs and to progress; adults more likely to infarct (but slower progression) + - Children more likely to have ipsilateral anterior plus posterior circulation involvement +- ## Demographics + + + - ### Age + + + - Bimodal age peaks (5-10 years and 2nd peak during 4th decade) + - Japan, Korea: 6 years > 35 years + - North America, Europe: 35 years > 6 years + - ### Sex + + + - M:F = 1:1.8; in familial cases, M:F = 1:5 + - Most frequent cause of stroke in Asian children +- ## Natural History & Prognosis + + + - Progressive narrowing, collateralization, and ischemia + - Prognosis depends on etiology, ability to form collaterals, age/stage at diagnosis + - Pediatric cases usually advance to stage 5 within 10 years of onset + - Infantile moyamoya progresses faster + - Pediatric cases present with stroke + - Hemorrhagic moyamoya more common in older patients with large collateral vessels + - Has poorer outcome +- ## Treatment + + + - MMD + - Indirect bypass: Encephaloduroarteriomyosynangiosis (EDAMS) more effective in children + - 5-year risk of ipsilateral stroke post encephaloduroarteriosynangiosis (EDAS) = 15% + - Direct bypass: Superficial temporal artery-MCA (STA-MCA) more common in adults + - Combined EDAMS, STA-MCA + - Anticoagulation; correct/control prothrombotic states and inflammatory etiologies + - Hypertransfusion for sickle cell-related moyamoya + - Perivascular sympathectomy or superior cervical ganglionectomy (adults) + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Seek secondary causes of moyamoya +- ## Image Interpretation Pearls + + + - Enhanced asymmetric atrophy found on childhood CT, look for abnormal vascular pattern + - Adult moyamoya can present with IC hemorrhage +- ## Reporting Tips + + + - Successful revascularization = ↓ basal collaterals, ↑ flow in MCA branches, ↑ caliber of STA (direct bypass) + + 16877c2f-bb02-4c68-afaf-2bfadf0fb33f + +## References + +# Selected References + +1. [Han Q et al: Quantitative analysis of revascularization in ischemic moyamoya disease via whole-brain computed tomography perfusion: a retrospective single-center study. Medicine (Baltimore). 99(7):e19168, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32049846%5Bpmid%5D) +1. [Ravindra VM et al: Preoperative computed tomography perfusion in pediatric moyamoya disease: a single-institution experience. J Neurosurg Pediatr. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31978885%5Bpmid%5D) +1. [Terrell D et al: Cerebral revascularization for moyamoya syndrome associated with sickle cell disease: a systematic review of the literature on the role of extracranial-intracranial bypass in treating neurologic manifestations of pediatric patients with sickle cell disease. World Neurosurg. 137:62-70, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32014541%5Bpmid%5D) +1. [Bang OY et al: Moyamoya disease and spectrums of RNF213 vasculopathy. Transl Stroke Res. 11(4):580-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31650369%5Bpmid%5D) +1. [Boulouis G et al: Nontraumatic pediatric intracerebral hemorrhage. Stroke. 50(12):3654-61, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31637968%5Bpmid%5D) +1. [Goyal P et al: Neuroimaging of pediatric arteriopathies. J Neuroimaging. 29(3):287-308, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30920080%5Bpmid%5D) +1. [Li J et al: Imaging of moyamoya disease and moyamoya syndrome: current status. J Comput Assist Tomogr. 43(2):257-63, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30589721%5Bpmid%5D) +1. [Liu ZW et al: Collateral circulation in moyamoya disease: a new grading system. Stroke. 50(10):2708-15, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31409266%5Bpmid%5D) +1. [Ravindran K et al: Surgical outcomes for pediatric moyamoya: a systematic review and meta-analysis. J Neurosurg Pediatr. 1-10, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31518973%5Bpmid%5D) +1. [Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31582039%5Bpmid%5D) +1. [Acker G et al: Surgical management of moyamoya disease. Stroke. 49(2):476-82, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29343587%5Bpmid%5D) +1. [Cho KC et al: Rete middle cerebral artery anomalies: a unifying name, case series, and literature review. J Neurosurg. 131(2):453-61, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30074465%5Bpmid%5D) +1. [Huang S et al: Etiology and pathogenesis of moyamoya disease: an update on disease prevalence. Int J Stroke. 12(3):246-53, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28381201%5Bpmid%5D) +1. [Fujimura M et al: Moyamoya disease. Front Neurol Neurosci. 40:204-20, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27960175%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Coronal graphic shows severe tapering of both distal internal carotid arteries (ICAs) and strikingly enlarged lenticulostriate arteries coursing through basal ganglia. This is the puff of smoke (moyamoya) pattern.](images/app.statdx.com_image_thumbnail_f8a053e9-37d0-4b49-b29b-df7a88f86a26_annotated_true_size_900_quality_90_27837fe3723fbc371110af5310b071c54042de4d.jpg) +*Coronal graphic shows severe tapering of both distal internal carotid arteries (ICAs) and strikingly enlarged lenticulostriate arteries coursing through basal ganglia. This is the puff of smoke (moyamoya) pattern.* + +![Coronal graphic shows severe tapering of both distal internal carotid arteries (ICAs) and strikingly enlarged lenticulostriate arteries coursing through basal ganglia. This is the puff of smoke (moyamoya) pattern.](images/app.statdx.com_image_thumbnail_f8a053e9-37d0-4b49-b29b-df7a88f86a26_size_174_quality_85_7f1ba71ed09110b13eb1476cf3ca27075f5c792e.jpg) +*Coronal graphic shows severe tapering of both distal internal carotid arteries (ICAs) and strikingly enlarged lenticulostriate arteries coursing through basal ganglia. This is the puff of smoke (moyamoya) pattern.* + +![Axial T2WI MR shows curvilinear net-like filling defects within the ambient (circummesencephalic) cistern corresponding to collateral moyamoya vessels. Note the asymmetric atrophy.](images/app.statdx.com_image_thumbnail_79737443-73b4-4c68-ba89-ee52dc8575c4_annotated_true_size_900_quality_90_4a28b01279ce7df2974b4d48ebc50f18f4bf8a7d.jpg) +*Axial T2WI MR shows curvilinear net-like filling defects within the ambient (circummesencephalic) cistern corresponding to collateral moyamoya vessels. Note the asymmetric atrophy.* + +![Lateral internal carotid artery DSA in a 3-year-old child with moyamoya disease (MMD) shows near-total supraclinoid ICA stenosis . Note innumerable tortuous enlarged collaterals forming the puff of smoke appearance typical of MMD.](images/app.statdx.com_image_thumbnail_a1e6e2a8-db6b-43f9-af11-e51f0718819c_annotated_true_size_900_quality_90_a22aca2b208fe4bf19b0faa1b12dfd0986f6d174.jpg) +*Lateral internal carotid artery DSA in a 3-year-old child with moyamoya disease (MMD) shows near-total supraclinoid ICA stenosis . Note innumerable tortuous enlarged collaterals forming the puff of smoke appearance typical of MMD.* + +![Lateral DSA in the same patient shows the collaterals (moyamoya vessels) are supplied primarily by thalamoperforating and medial choroidal branches.](images/app.statdx.com_image_thumbnail_a12a7439-0328-475f-a2d4-22a49f4379d3_annotated_true_size_900_quality_90_4c857fd9ac60a7ce1791c1b5f6f5a87224487ca3.jpg) +*Lateral DSA in the same patient shows the collaterals (moyamoya vessels) are supplied primarily by thalamoperforating and medial choroidal branches.* + +![Axial T2WI MR in adult moyamoya shows attenuated, almost thread-like supraclinoid ICAs and middle cerebral arteries (MCAs) with numerous tiny collateral vessels in the suprasellar cistern and around the midbrain . There is marked cortical atrophy along with enlarged temporal horns.](images/app.statdx.com_image_thumbnail_83f92ae8-be84-4a5d-9958-bbc9ab3afa23_annotated_true_size_900_quality_90_242953f7fd591c47bee93e3370a3e0095dc0b933.jpg) +*Axial T2WI MR in adult moyamoya shows attenuated, almost thread-like supraclinoid ICAs and middle cerebral arteries (MCAs) with numerous tiny collateral vessels in the suprasellar cistern and around the midbrain . There is marked cortical atrophy along with enlarged temporal horns.* + +![Axial T1 C+ FS MR in the same patient shows contrast in innumerable small arterial collateral vessels in the basal ganglia and deep white matter , creating a puff of smoke appearance. (Courtesy H. Els, MD.)](images/app.statdx.com_image_thumbnail_80a0a30f-d363-43c7-86b6-2082ea56a43e_annotated_true_size_900_quality_90_32ed61b2109d63a2a9a3221c62d7083ea2751639.jpg) +*Axial T1 C+ FS MR in the same patient shows contrast in innumerable small arterial collateral vessels in the basal ganglia and deep white matter , creating a puff of smoke appearance. (Courtesy H. Els, MD.)* + +![Axial T2WI MR in a 23-year-old man with left hemisphere transient ischemic attacks (TIAs) shows the flow void of a normal right M1 MCA . The left MCA appears very hypoplastic and twig-like . There is an unusual web-like tangle of vessels seen within the sylvian fissure .](images/app.statdx.com_image_thumbnail_c727aaee-ab4d-4747-a19e-c4bd174cffc3_annotated_true_size_900_quality_90_63dda29a2e0b8f27d75fd2b2d9f05fa38fc6af7a.jpg) +*Axial T2WI MR in a 23-year-old man with left hemisphere transient ischemic attacks (TIAs) shows the flow void of a normal right M1 MCA . The left MCA appears very hypoplastic and twig-like . There is an unusual web-like tangle of vessels seen within the sylvian fissure .* + +![Submentovertex MR angiogram in the same patient shows a normal right MCA . The left MCA is thread-like , and its distal M3 and M4 branches are attenuated compared to the normal right side.](images/app.statdx.com_image_thumbnail_d64bbe9e-6d58-4ecc-b594-956484cfb390_annotated_true_size_900_quality_90_41f624e890e2a8617ecf004840048bef034ef988.jpg) +*Submentovertex MR angiogram in the same patient shows a normal right MCA . The left MCA is thread-like , and its distal M3 and M4 branches are attenuated compared to the normal right side.* + +![Oblique view of the left internal carotid MR angiogram shows a normal anterior cerebral artery (ACA) , but the left MCA is severely attenuated with reduced distal branches. A web of numerous small, tangled vessels surrounds its M1 segment.](images/app.statdx.com_image_thumbnail_36fe0255-e979-4a6e-85aa-d11123e22998_annotated_true_size_900_quality_90_b973532faab52b7acc878b6112fc8b8609840c11.jpg) +*Oblique view of the left internal carotid MR angiogram shows a normal anterior cerebral artery (ACA) , but the left MCA is severely attenuated with reduced distal branches. A web of numerous small, tangled vessels surrounds its M1 segment.* + +![Oblique view of the left internal carotid DSA in the same patient shows the tangled web of vessels that surrounds the attenuated M1 MCA segment. This is thought to represent a rete MCA anomaly rather than segmental moyamoya.](97e3be9e-6205-41b9-919c-f86fd4cbcf92) +*Oblique view of the left internal carotid DSA in the same patient shows the tangled web of vessels that surrounds the attenuated M1 MCA segment. This is thought to represent a rete MCA anomaly rather than segmental moyamoya.* + + +### Additional Images + +![Axial FLAIR MR shows the ivy sign due to engorged vessels within sulci in another patient with moyamoya. The sulcal signal is so striking that this FLAIR scan resembles a T2WI.](99b5feea-5c17-4db5-9e92-b64e325950ab) +*Axial FLAIR MR shows the ivy sign due to engorged vessels within sulci in another patient with moyamoya. The sulcal signal is so striking that this FLAIR scan resembles a T2WI.* + +![Lateral view, selective internal carotid angiogram, shows severe stenosis of the supraclinoid ICA with a puff of smoke appearance from collateral lenticulostriate vessels.](96491607-0164-4f15-95a4-68d2c36cb0f7) +*Lateral view, selective internal carotid angiogram, shows severe stenosis of the supraclinoid ICA with a puff of smoke appearance from collateral lenticulostriate vessels.* + +![Lateral angiography, right ICA injection in the same patient, shows narrowing of the distal ICA and occlusion of the ACA and MCA with the puff of smoke appearance of enlarged lenticulostriate collaterals. Note the additional network of collaterals more posteriorly , likely thalamoperforators and posterior choroidal branches.](18200f99-680e-4a6c-8e58-737ca6303aa3) +*Lateral angiography, right ICA injection in the same patient, shows narrowing of the distal ICA and occlusion of the ACA and MCA with the puff of smoke appearance of enlarged lenticulostriate collaterals. Note the additional network of collaterals more posteriorly , likely thalamoperforators and posterior choroidal branches.* + +![Lateral DSA of the vertebrobasilar circulation in the same patient shows a striking puff of smoke appearance caused by innumerable dilated collateral branches arising from the thalamostriate arteries.](8bae1f67-f80b-4de1-a194-f56f6234b6ca) +*Lateral DSA of the vertebrobasilar circulation in the same patient shows a striking puff of smoke appearance caused by innumerable dilated collateral branches arising from the thalamostriate arteries.* + +![Axial MRA shows occlusion of both distal ICAs , nonvisualization of MCAs and ACAs, and stenosis of posterior cerebral arteries (PCAs) in an 8 year old with hemiparetic migraines in this case of idiopathic arteriopathy of childhood.](d0145a83-7a3f-4493-8ad2-eb404aa3fae6) +*Axial MRA shows occlusion of both distal ICAs , nonvisualization of MCAs and ACAs, and stenosis of posterior cerebral arteries (PCAs) in an 8 year old with hemiparetic migraines in this case of idiopathic arteriopathy of childhood.* + +![Axial MRA in another patient with idiopathic progressive arteriopathy of childhood shows occluded supraclinoid ICAs . Note the bilateral synangiosis .](bf9477e9-3f3d-4e85-b942-ea9dbdb0319d) +*Axial MRA in another patient with idiopathic progressive arteriopathy of childhood shows occluded supraclinoid ICAs . Note the bilateral synangiosis .* + +![Axial T1WI MR shows right frontal and left temporaoccipital atrophy from remote ischemia. There are multiple small basal ganglia flow voids from lenticulostriate collaterals.](e72d3c19-7267-4f54-8126-78d3f29a0a43) +*Axial T1WI MR shows right frontal and left temporaoccipital atrophy from remote ischemia. There are multiple small basal ganglia flow voids from lenticulostriate collaterals.* + +![Axial T1WI C+ MR in the same patient shows multifocal white "dots" due to slow flow in multiple, enlarged lenticulostriate collaterals with intravascular enhancement.](2515c9f8-54ac-44e6-b86e-9556b24a7ec9) +*Axial T1WI C+ MR in the same patient shows multifocal white "dots" due to slow flow in multiple, enlarged lenticulostriate collaterals with intravascular enhancement.* + diff --git a/docs_md/articles/moyamoya_e15385dc-824d-431a-8df0-2b28bf909a2d.md b/docs_md/articles/moyamoya_e15385dc-824d-431a-8df0-2b28bf909a2d.md new file mode 100644 index 0000000..2236a20 --- /dev/null +++ b/docs_md/articles/moyamoya_e15385dc-824d-431a-8df0-2b28bf909a2d.md @@ -0,0 +1,459 @@ +--- +title: "Moyamoya" +docid: "e15385dc-824d-431a-8df0-2b28bf909a2d" +authors: + - key: "47381de4-c9fd-4999-8dd0-1808cd72db6b" + value: "Luke L. Linscott, 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: "Stroke" + slug: "stroke" + treeNodeId: "83689efc-5f25-40a3-9ae7-06fb2a4a069f" + - + name: "Moyamoya" + slug: "moyamoya" + treeNodeId: null +category: "Pediatrics" +cmeTopicId: "8ba35550-ab01-4c3c-a79f-25aaa06da86c" +documentVersionId: "635703ac-cce9-46c4-a51b-3a94faa9236a" +imageCount: 25 +lastUpdated: "02/07/24" +pageDescription: "Moyamoya" +pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Stroke, Moyamoya" +pageTitle: "Moyamoya | STATdx" +enhancedTitle: "Moyamoya" +type: "DX" +references: true +breadcrumbs: + - "Pediatrics" + - "Diagnosis" + - "Pediatric Neuroradiology" + - "Brain" + - "Pathology-Based Diagnoses" + - "Stroke" + - "Moyamoya" +--- +# KEY FACTS + +- ## Terminology + + + - Progressive narrowing of distal internal carotid artery (ICA) & proximal circle of Willis (COW) vessels → characteristic adjacent clusters of collateral flow appearing as "puff of smoke" on real-time angiography + - Moyamoya disease = primary (idiopathic) moyamoya + - More common in Japan, Korea + - Moyamoya arteriopathy (a.k.a. moyamoya syndrome or secondary moyamoya) due to other disorders +- ## Imaging + + + - Absent or narrowed distal ICA & abnormal COW + - Excessive tiny collaterals in basal ganglia & cisterns + - "Puff of smoke" (moyamoya in Japanese) of lenticulostriate & thalamoperforator collaterals + - Prominent collaterals in sulci + - Ivy sign on FLAIR & T1 C+ MR + - Acute & chronic infarcts + - CT/CTA: Acute use for ischemia or hemorrhage + - MR C+/MRA: Vascular protocol with DWI & perfusion + - DWI: Helpful to identify "acute on chronic" injury +- ## Pathology + + + - Moyamoya disease: Inherited idiopathic disorder + - Moyamoya arteriopathy: Secondary process + - Sickle cell disease, trisomy 21, neurofibromatosis type 1, radiation therapy, Alagille syndrome, morning glory syndrome, TB meningitis, among others +- ## Clinical Issues + + + - Bimodal age peaks: 6 & 35 years + - Most frequent cause of stroke in Asian children + - Presentation (children): Transient ischemic attacks (TIAs), alternating hemiplegia (exacerbated by crying), headache + - Presentation (adults): TIAs, hemorrhage (~ 30%), & cerebral infarct + - Prognosis depends on etiology, ability to form collaterals, age/stage at diagnosis + - Treatment: Indirect (more common in children) or direct (more common in adults) vascular bypass +- ## Diagnostic Checklist + + + - Seek underlying causes of secondary moyamoya + +# TERMINOLOGY + +- ## Synonyms + + + - Progressive stenoocclusive arteriopathy; spontaneous occlusion of circle of Willis (COW) +- ## Definitions + + + - Progressive narrowing of distal internal carotid artery (ICA) & proximal COW vessels → characteristic adjacent clusters of collateral flow appearing as "puff of smoke" on real-time angiography + - Moyamoya disease: Primary (idiopathic) moyamoya + - More common in Japan, Korea + - Moyamoya arteriopathy (a.k.a. moyamoya syndrome or secondary moyamoya) occurs in association with other disorders or after radiation treatment + +# IMAGING + +- ## General Features + + + - Best diagnostic clue: Multiple enhancing punctate dots (CECT) & flow voids (MR) in basal ganglia & cisterns + - Arterial occlusions: Distal ICA, COW, branches + - Anterior > posterior circulation + - Posterior circulation affected in ~ 25% + - Leads to prominent clusters of nearby collaterals + - "Cloud-like" lenticulostriate & thalamoperforator collaterals on angiography: "Puff of smoke" (moyamoya in Japanese) + - Also leads to prominent sulcal collaterals distally +- ## CT Findings + + + - ### NECT + + + - Children: Acute ischemia ± old infarcts + - Older children/adults: Usually ischemia but may present with intracranial hemorrhage + - CTA: Abnormal COW + basilar net-like collaterals + - Xe-133 CT: ↓ cerebral reserve with acetazolamide challenge +- ## MR Findings + + + - ### T2WI + + + - ↑ signal in gliotic areas from prior infarcts + - Collateral vessels: Net-like cisternal flow voids + - ### FLAIR + + + - Bright sulci = leptomeningeal ivy sign + - Slow-flowing engorged pial collateral vessels, thickened arachnoid membranes + - Correlates with ↓ cerebral vascular reserve + - ### T2* GRE + + + - Hemosiderin if prior hemorrhage + - ### DWI + + + - Very useful for "acute on chronic" infarcts + - ### PWI + + + - ↓ cerebral blood flow (CBF) (ASL) in affected territories + - ↑ MTT in affected territories + - Variable rCBV depending on degree of collateral formation + - May be used to measure response to revascularization + - ### T1WI C+ + + + - Lenticulostriate collaterals → enhancing "dots" in basal ganglia & net-like thin vessels in cisterns + - Leptomeningeal enhancement (ivy sign) + - Vessel wall imaging + - Most consistent finding is negative remodeling (local shrinkage of vessel size) of affected vessels + - Variable enhancement of affected vessel segments + - ↑ wall thickening and ↑ stenosis correlates with ↑ wall enhancement + - May help distinguish from other vasculopathies + - ### MRA + + + - Narrowed/occluded distal ICA & COW vessels + - ### MRS + + + - Lactate in acutely infarcted tissue + - NAA:Cr & Cho:Cr ratios in frontal white matter improve ↑ after revascularization +- ## Ultrasonographic Findings + + + - Grayscale: Reduction of ICA lumen size + - Pulsed Doppler + - Spectral waveforms in ICA show no flow (occluded) or proximal high-resistance flow pattern + - ↑ end-diastolic flow velocity, ↓ vascular resistance in external carotid artery (ECA) collaterals + - Color Doppler: Aliasing suggests stenoses + - Power Doppler: Improves visualization of slow-flow stenotic vessels & collaterals + - Can be used for vessel mapping prior to revascularization surgery +- ## Angiographic Findings + + + - Predominantly (not exclusively) anterior circulation + - Narrow proximal COW & ICA (early phase) + - Lenticulostriate & thalamoperforator collaterals (intermediate phase) + - Transdural/transosseous ECA-ICA collaterals (late phase) + - Dilation & branch extension of anterior choroidal artery predict adult hemorrhagic events +- ## Nuclear Medicine Findings + + + - PET: ↓ hemodynamic reserve capacity + - SPECT I-123-iomazenil: Neuronal density preserved if asymptomatic, ↓ if symptomatic +- ## Imaging Recommendations + + + - Best imaging tool: MR C+/MRA + - Contrast improves detection: Collaterals, synangiosis + - Catheter angiography defines anatomy prior to bypass + - Protocol advice + - Acetazolamide challenge with ASL has been performed to measure cerebrovascular reserve, but Xe-133 CT is gold standard + +# DIFFERENTIAL DIAGNOSIS + +- ## Ivy Sign + + + - Leptomeningeal metastases, subarachnoid hemorrhage, meningitis, ↑ inspired oxygen, collateral veins of Sturge-Weber or other chronic venous occlusion +- [Large Vessel Inflammatory Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0) + - Postvaricella vasculitis, lupus, & other CNS vasculitides + - May be reversible with treatment +- [Cerebral Arterial Atherosclerosis](/document/intracranial-atherosclerosis/8d21d962-9c43-47bd-b995-c73f20e46b47) + - Very rare in children & young adults +- ## Severely Attenuated Circle of Willis + + + - Subarachnoid hemorrhage (spasm), meningitis, tumor encasement + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Moyamoya disease + - Inherited polygenic or autosomal dominant + - Low penetrance + - Gene loci: 3p26-p24.2, 17q25, 8q23 + - ↑ in growth factors, cytokines, adhesion molecules in CSF implicates inflammation + - Moyamoya arteriopathy (a.k.a. moyamoya syndrome or secondary moyamoya) + - Sickle cell disease, neurofibromatosis type 1 (NF1), radiation therapy, trisomy 21, Alagille syndrome, morning glory syndrome, tuberculous meningitis, many others + - NF1 + suprasellar tumor + radiation can be disastrous + - Epidemiology: Moyamoya disease + - Incidence in Japan: 1:100,000 + - Incidence in North America, Europe: 0.1:100,000 + - 10-15% familial +- ## Staging, Grading, & Classification + + + - Staging criteria (Suzuki) + - Stage 1: Narrowing of ICA bifurcation + - Stage 2: Anterior, middle, and posterior cerebral arteries (ACA, MCA, PCA) dilated + - Stage 3: Maximal basal collaterals; small ACA/MCA + - Stage 4: Fewer collaterals (vessels); small PCA + - Stage 5: Further ↓ in collaterals; absent ACA/MCA/PCA + - Stage 6: Extensive ECA-pial collaterals +- ## Gross Pathologic & Surgical Features + + + - ↑ perforating (early) & ECA-ICA (late) collaterals in atrophic brain + - Hemorrhage (subarachnoid, intraventricular > parenchymal) in adults + - ↑ saccular aneurysms in adults (especially basilar) +- ## Microscopic Features + + + - Intimal thickening & hyperplasia + - Excessive infolding & thickening of internal elastic lamina + - Periventricular pseudoaneurysms (cause of hemorrhage) + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Children: Transient ischemic attacks (TIAs), alternating hemiplegia (exacerbated by crying), headache + - Adults: TIAs, hemorrhage (~ 30%), cerebral infarct + - Hemorrhagic presentation more common in Asian adults + - ### Other signs/symptoms + + + - Children: Developmental delay, poor feeding, chorea +- ## Demographics + + + - ### Age + + + - Bimodal age peaks + - Japan, Korea: 6 years > 35 years + - North America, Europe: 35 years > 6 years + - ### Sex + + + - M:F = 1:1.8; in familial cases, M:F = 1:5 + - ### Epidemiology + + + - Most frequent cause of stroke in Asian children +- ## Natural History & Prognosis + + + - Progressive narrowing, collateralization, & ischemia + - Prognosis depends on etiology, ability to form collaterals, age/stage at diagnosis + - Pediatric cases usually advance to stage 5 in < 10 years + - Infantile moyamoya progresses faster + - Hemorrhagic moyamoya more common in older patients + - May be due to large collateral vessels + - Has poorer outcome +- ## Treatment + + + - Aspirin therapy + - Direct bypass: Superficial temporal artery (STA)-MCA more common in adults + - Indirect bypass + - Pial synangiosis & encephaloduroarteriosynangiosis with STA more common in children + - 5-year risk of ipsilateral stroke post encephaloduroarteriosynangiosis = 15% + - Dural inversion with middle meningeal artery + - Correct/control prothrombotic states & inflammatory etiologies + - Transfusion therapy for sickle cell-related moyamoya + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Seek underlying causes of secondary moyamoya +- ## Image Interpretation Pearls + + + - Enhanced asymmetric atrophy found on childhood CT; look for abnormal vascular pattern + - Adult moyamoya can present with intracranial hemorrhage +- ## Reporting Tips + + + - Successful revascularization = ↓ basal collaterals, ↑ flow in MCA branches, ↑ caliber of STA (direct bypass) + + 4447a660-ce0e-4510-a9b1-a3c441465e85 + +## References + +# Selected References + +1. [Larson AS et al: Vessel wall imaging features of moyamoya disease in a North American population: patterns of negative remodelling, contrast enhancement, wall thickening, and stenosis. BMC Med Imaging. 22(1):198, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36397005%5Bpmid%5D) +1. [Larson AS et al: Implementation and rationale for a unified clinical and imaging protocol for evaluation and treatment of moyamoya angiopathy: a single institutional experience. Front Neurol. 12:662393, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34079514%5Bpmid%5D) +1. [Wang LX et al: Ivy sign in moyamoya disease: a comparative study of the FLAIR vascular hyperintensity sign against contrast-enhanced MRI. AJNR Am J Neuroradiol. 42(4):694-700, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33664105%5Bpmid%5D) +1. [Fan AP et al: Identifying hypoperfusion in moyamoya disease with arterial spin labeling and an [15O]-water positron emission tomography/magnetic resonance imaging normative database. Stroke. 50(2):373-80, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30636572%5Bpmid%5D) +1. [Lehman VT et al: Contemporary and emerging magnetic resonance imaging methods for evaluation of moyamoya disease. Neurosurg Focus. 47(6):E6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31786551%5Bpmid%5D) +1. [Lee S et al: Monitoring cerebral perfusion changes after revascularization in patients with moyamoya disease by using arterial spin-labeling MR imaging. Radiology. 288(2):565-72, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29714677%5Bpmid%5D) +1. [Kim DY et al: Infarct pattern and collateral status in adult moyamoya disease: a multimodal magnetic resonance imaging study. Stroke. 48(1):111-6, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27909201%5Bpmid%5D) +1. [Qiao PG et al: Clinical assessment of cerebral hemodynamics in moyamoya disease via multiple inversion time arterial spin labeling and dynamic susceptibility contrast-magnetic resonance imaging: a comparative study. J Neuroradiol. 44(4):273-80, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28168990%5Bpmid%5D) +1. [Blauwblomme T et al: Cerebral blood flow improvement after indirect revascularization for pediatric moyamoya disease: a statistical analysis of arterial spin-labeling MRI. AJNR Am J Neuroradiol. 37(4):706-12, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26585258%5Bpmid%5D) +1. [Kim JS: Moyamoya disease: epidemiology, clinical features, and diagnosis. J Stroke. 18(1):2-11, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26846755%5Bpmid%5D) +1. [Mossa-Basha M et al: Added value of vessel wall magnetic resonance imaging in the differentiation of moyamoya vasculopathies in a non-Asian cohort. Stroke. 47(7):1782-8, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27272486%5Bpmid%5D) +1. [Takagi Y et al: Histopathological characteristics of distal middle cerebral artery in adult and pediatric patients with moyamoya disease. Neurol Med Chir (Tokyo). 56(6):345-9, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27087193%5Bpmid%5D) +1. [Titsworth WL et al: National analysis of 2454 pediatric moyamoya admissions and the effect of hospital volume on outcomes. Stroke. 47(5):1303-11, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27048697%5Bpmid%5D) +1. [Griessenauer CJ et al: Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease. J Neurosurg Pediatr. 16(1):64-73, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25837886%5Bpmid%5D) +1. [Derdeyn CP: Direct bypass reduces the risk of recurrent hemorrhage in moyamoya syndrome, but effect on functional outcome is less certain. Stroke. 45(5):1245-6, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24668205%5Bpmid%5D) +1. [Ryoo S et al: High-resolution magnetic resonance wall imaging findings of moyamoya disease. Stroke. 45(8):2457-60, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24947295%5Bpmid%5D) +1. [Hishikawa T et al: Assessment of the difference in posterior circulation involvement between pediatric and adult patients with moyamoya disease. J Neurosurg. 119(4):961-5, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23909250%5Bpmid%5D) +1. [Noguchi T et al: Arterial spin-labeling MR imaging in moyamoya disease compared with clinical assessments and other MR imaging findings. Eur J Radiol. 82(12):e840-7, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24055185%5Bpmid%5D) +1. [Currie S et al: Childhood moyamoya disease and moyamoya syndrome: a pictorial review. Pediatr Neurol. 44(6):401-13, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21555050%5Bpmid%5D) +1. [Mugikura S et al: Posterior circulation and high prevalence of ischemic stroke among young pediatric patients with Moyamoya disease: evidence of angiography-based differences by age at diagnosis. AJNR Am J Neuroradiol. 32(1):192-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=20801761%5Bpmid%5D) +1. [Ibrahimi DM et al: Moyamoya disease in children. Childs Nerv Syst. 26(10):1297-308, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20607248%5Bpmid%5D) +1. [Kim SK et al: Pediatric moyamoya disease: an analysis of 410 consecutive cases. Ann Neurol. 68(1):92-101, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20582955%5Bpmid%5D) +1. [Mori N et al: The leptomeningeal "ivy sign" on fluid-attenuated inversion recovery MR imaging in moyamoya disease: a sign of decreased cerebral vascular reserve? AJNR Am J Neuroradiol. 30(5):930-5, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19246527%5Bpmid%5D) +1. [Park TS: Moyamoya disease in children. Neurosurg Focus. 24(2):E16a; discussion E16a, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18275293%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial DWI MR in a 13-month-old with increasing seizures shows left MCA distribution ischemia as well as a remote infarct in the right MCA territory. This is a typical acute on chronic ischemic pattern of moyamoya.](images/app.statdx.com_image_thumbnail_6bbf5cd4-5420-4348-a31f-6d8f6a2bc67a_annotated_true_size_900_quality_90_d0b00ad65e0943749fc5f13367c878e6be1e5432.jpg) +*Axial DWI MR in a 13-month-old with increasing seizures shows left MCA distribution ischemia as well as a remote infarct in the right MCA territory. This is a typical acute on chronic ischemic pattern of moyamoya.* + +![Axial DWI MR in a 13-month-old with increasing seizures shows left MCA distribution ischemia as well as a remote infarct in the right MCA territory. This is a typical acute on chronic ischemic pattern of moyamoya.](images/app.statdx.com_image_thumbnail_6bbf5cd4-5420-4348-a31f-6d8f6a2bc67a_size_174_quality_85_0d268f97f015fd144cd0e7230363297498e59701.jpg) +*Axial DWI MR in a 13-month-old with increasing seizures shows left MCA distribution ischemia as well as a remote infarct in the right MCA territory. This is a typical acute on chronic ischemic pattern of moyamoya.* + +![Anterior 3D TOF MRA in the same patient at 8 years of age shows occlusions of the terminal ICAs , absence of the MCAs, & numerous lenticulostriate collaterals forming a "puff of smoke." The PCAs are also occluded. Note the enlarged ECA collaterals status post synangiosis & dural inversion.](images/app.statdx.com_image_thumbnail_3c6e5eec-bea7-4ef0-a828-0ea47ff5986e_annotated_true_size_900_quality_90_a4ea0607e984c6ad81ea8f4c54c5a49231c9d9de.jpg) +*Anterior 3D TOF MRA in the same patient at 8 years of age shows occlusions of the terminal ICAs , absence of the MCAs, & numerous lenticulostriate collaterals forming a "puff of smoke." The PCAs are also occluded. Note the enlarged ECA collaterals status post synangiosis & dural inversion.* + +![Axial 3D TOF MRA in a 5-year-old with idiopathic moyamoya arteriopathy shows multiple small lenticulostriate and thalamostriate vessels within the deep nuclear structures.](images/app.statdx.com_image_thumbnail_0e64957a-0ae8-4733-bd51-8ff8a4db4bb4_annotated_true_size_900_quality_90_c73b1afba6d065ed282f7875aeb942ea7679574b.jpg) +*Axial 3D TOF MRA in a 5-year-old with idiopathic moyamoya arteriopathy shows multiple small lenticulostriate and thalamostriate vessels within the deep nuclear structures.* + +![Axial FLAIR MR in the same patient shows areas of gliosis from vascular insufficiency, linear hyperintensities representing deep medullary collateral vessels, and hyperintensities conforming to the surface of the sulci representing pial collaterals (the leptomeningeal ivy sign).](images/app.statdx.com_image_thumbnail_81ef969b-96ab-4284-b2c6-7411aa98abd5_annotated_true_size_900_quality_90_6cd2099a5ea65d23b0a0236fb0aec89b9ab2fd87.jpg) +*Axial FLAIR MR in the same patient shows areas of gliosis from vascular insufficiency, linear hyperintensities representing deep medullary collateral vessels, and hyperintensities conforming to the surface of the sulci representing pial collaterals (the leptomeningeal ivy sign).* + +![Axial TOF MRA in a 14-year-old with neurofibromatosis type 1 shows absence of the right internal carotid terminus & MCA. In the expected location of the carotid terminus & MCA, there are multiple small leptomeningeal collaterals .](images/app.statdx.com_image_thumbnail_1f915fd4-118e-4287-9d28-fada490e37b0_annotated_true_size_900_quality_90_e939df8c3bb6332371e0f526b2f84f47ebc7679d.jpg) +*Axial TOF MRA in a 14-year-old with neurofibromatosis type 1 shows absence of the right internal carotid terminus & MCA. In the expected location of the carotid terminus & MCA, there are multiple small leptomeningeal collaterals .* + +![Axial FLAIR MR in a 21-year-old with sickle cell disease shows high signal within the right MCA distribution sulci, the so-called ivy sign. This abnormal signal corresponds to engorged pial collateral vessels & is often seen in moyamoya.](images/app.statdx.com_image_thumbnail_c781bef2-264c-4f63-b396-25c3b4a4e0f4_annotated_true_size_900_quality_90_e7cfea5e2eb8021771fd441e488a064397a40262.jpg) +*Axial FLAIR MR in a 21-year-old with sickle cell disease shows high signal within the right MCA distribution sulci, the so-called ivy sign. This abnormal signal corresponds to engorged pial collateral vessels & is often seen in moyamoya.* + +![Frontal 3D TOF MRA in a 10-year-old with history of nasal rhabdomyosarcoma at age 3 treated with XRT shows absence of flow in the left distal ICA , severe narrowing of the right MCA , & absent right ACA. Terminal ICA & proximal MCA are the most common locations for moyamoya arteriopathy.](images/app.statdx.com_image_thumbnail_2e640916-e8a5-4db0-bdf4-6ac7d2855e85_annotated_true_size_900_quality_90_c9b2f3372979c3bb6b8fe189733339965397e6b8.jpg) +*Frontal 3D TOF MRA in a 10-year-old with history of nasal rhabdomyosarcoma at age 3 treated with XRT shows absence of flow in the left distal ICA , severe narrowing of the right MCA , & absent right ACA. Terminal ICA & proximal MCA are the most common locations for moyamoya arteriopathy.* + +![Axial T1 C+ MR in the same patient shows extensive leptomeningeal enhancement in the bilateral ICA territories with prominent vessel enhancement in the basal ganglia.](images/app.statdx.com_image_thumbnail_c4538a98-287e-4c78-bc22-ec358887363b_annotated_true_size_900_quality_90_7c1e35ca917197cabfd20d10d18ace1315a796c9.jpg) +*Axial T1 C+ MR in the same patient shows extensive leptomeningeal enhancement in the bilateral ICA territories with prominent vessel enhancement in the basal ganglia.* + +![3D TOF MRA in a 6-year-old with trisomy 21 and severe moyamoya arteriopathy shows marked narrowing of the left ICA terminus & complete occlusion of the right MCA origin . Deep moyamoya collaterals have formed in the left basal ganglia.](images/app.statdx.com_image_thumbnail_9e783848-259f-496f-9a99-6047448c3304_annotated_true_size_900_quality_90_612fd268c9b8e6eca163d170ca57283b11a52594.jpg) +*3D TOF MRA in a 6-year-old with trisomy 21 and severe moyamoya arteriopathy shows marked narrowing of the left ICA terminus & complete occlusion of the right MCA origin . Deep moyamoya collaterals have formed in the left basal ganglia.* + +![Axial ASL MR perfusion in the same patient shows near absence of signal in the right MCA territory, consistent with dramatically reduced blood flow, corroborating the findings on MRA.](d7626f86-b8e6-4169-a0ad-c8b2f3497275) +*Axial ASL MR perfusion in the same patient shows near absence of signal in the right MCA territory, consistent with dramatically reduced blood flow, corroborating the findings on MRA.* + + +### Additional Images + +![Coronal graphic shows severe tapering of both distal ICAs & strikingly enlarged lenticulostriate arteries coursing through the basal ganglia. This is the characteristic puff of smoke (moyamoya) pattern.](2bfd6af4-572e-4cf4-a75b-9de6f00847e8) +*Coronal graphic shows severe tapering of both distal ICAs & strikingly enlarged lenticulostriate arteries coursing through the basal ganglia. This is the characteristic puff of smoke (moyamoya) pattern.* + +![Axial FLAIR MR in an 8-year-old boy with Alagille syndrome shows confluent gliosis in the right frontal region & more patchy gliosis in the left deep white matter. Also note the serpentine collateral vessels near the midline. Moyamoya arteriopathy can be seen with Alagille syndrome.](e37f4728-91e0-4658-98cd-93c5c649628b) +*Axial FLAIR MR in an 8-year-old boy with Alagille syndrome shows confluent gliosis in the right frontal region & more patchy gliosis in the left deep white matter. Also note the serpentine collateral vessels near the midline. Moyamoya arteriopathy can be seen with Alagille syndrome.* + +![Axial TOF MRA in an 8-year-old boy with Alagille syndrome shows absent signal in the region of the carotid termini & major ICA branch vessels. There are, however, small leptomeningeal collateral vessels in the midline inferior frontal & right anterior temporal regions & basilar cisterns . The findings are compatible with moyamoya arteriopathy.](7e546434-2a13-4238-9030-012dac21384c) +*Axial TOF MRA in an 8-year-old boy with Alagille syndrome shows absent signal in the region of the carotid termini & major ICA branch vessels. There are, however, small leptomeningeal collateral vessels in the midline inferior frontal & right anterior temporal regions & basilar cisterns . The findings are compatible with moyamoya arteriopathy.* + +![Anterior projection from a left ICA DSA injection shows no opacification of the expected ACA branches with multiple leptomeningeal collaterals seen in the midline subfrontal region . Also note the enlarged left middle meningeal artery , which supplies portions of the right ACA territory. This is an example of an ICA to ECA collateral pathway.](6623f703-9fe3-4b99-9a1b-53b639b8d1bc) +*Anterior projection from a left ICA DSA injection shows no opacification of the expected ACA branches with multiple leptomeningeal collaterals seen in the midline subfrontal region . Also note the enlarged left middle meningeal artery , which supplies portions of the right ACA territory. This is an example of an ICA to ECA collateral pathway.* + +![Axial T2 MR in a 21-year-old man with sickle cell disease shows absence of the carotid termini & major branches. Note the numerous small leptomeningeal collateral vessels within the basilar cisterns, typical of moyamoya arteriopathy.](886e4d77-a2b5-4521-a8f5-43abf483960e) +*Axial T2 MR in a 21-year-old man with sickle cell disease shows absence of the carotid termini & major branches. Note the numerous small leptomeningeal collateral vessels within the basilar cisterns, typical of moyamoya arteriopathy.* + +![Axial MRA in a 21-year-old man with sickle cell disease shows multiple punctate foci of flow-related signal within the basal ganglia & thalami, consistent with typical lenticulostriate & thalamoperforator collateral vessels of moyamoya arteriopathy.](da8e876d-922f-4b5c-be91-bdde873d3356) +*Axial MRA in a 21-year-old man with sickle cell disease shows multiple punctate foci of flow-related signal within the basal ganglia & thalami, consistent with typical lenticulostriate & thalamoperforator collateral vessels of moyamoya arteriopathy.* + +![Axial T1 C+ MR in a 21-year-old man with sickle cell disease shows abnormal leptomeningeal enhancement in the right MCA distribution. This is the equivalent of the ivy sign on FLAIR & represents slow-flowing leptomeningeal collaterals that develop in areas of decreased cerebrovascular reserve (secondary to moyamoya arteriopathy).](55f135b0-5abb-4275-ab4a-430d291c815c) +*Axial T1 C+ MR in a 21-year-old man with sickle cell disease shows abnormal leptomeningeal enhancement in the right MCA distribution. This is the equivalent of the ivy sign on FLAIR & represents slow-flowing leptomeningeal collaterals that develop in areas of decreased cerebrovascular reserve (secondary to moyamoya arteriopathy).* + +![3D MRA in a 16-year-old boy with moyamoya arteriopathy demonstrates severe stenosis/occlusion of the right carotid terminus . The patient is status post revascularization using a direct bypass. Note the prominent right superficial temporal artery , which is anastomosed to a distal right MCA branch .](ef490820-7608-48e5-b26c-5dc98e832327) +*3D MRA in a 16-year-old boy with moyamoya arteriopathy demonstrates severe stenosis/occlusion of the right carotid terminus . The patient is status post revascularization using a direct bypass. Note the prominent right superficial temporal artery , which is anastomosed to a distal right MCA branch .* + +![Lateral view of a selective internal carotid DSA shows severe stenosis of the supraclinoid ICA with a "puff of smoke" from the collateral lenticulostriate vessels.](4b237b46-0087-43cd-929c-7984216d51db) +*Lateral view of a selective internal carotid DSA shows severe stenosis of the supraclinoid ICA with a "puff of smoke" from the collateral lenticulostriate vessels.* + +![Axial TOF MRA shows occlusion of both distal ICAs , nonvisualization of the MCAs & ACAs, & stenosis of the PCAs in an 8-year-old with hemiparetic migraines. This patient had an idiopathic arteriopathy of childhood.](53dd2d81-d924-4dd5-afcf-2bceccd51252) +*Axial TOF MRA shows occlusion of both distal ICAs , nonvisualization of the MCAs & ACAs, & stenosis of the PCAs in an 8-year-old with hemiparetic migraines. This patient had an idiopathic arteriopathy of childhood.* + +![Axial TOF MRA in another patient with idiopathic progressive arteriopathy of childhood shows occluded supraclinoid ICAs . Note the bilateral synangiosis .](435e113e-0be3-44f6-8a0b-5ebd0c46ab77) +*Axial TOF MRA in another patient with idiopathic progressive arteriopathy of childhood shows occluded supraclinoid ICAs . Note the bilateral synangiosis .* + +![Axial T1 MR shows right frontal & left temporooccipital atrophy from remote ischemia. There are multiple small basal ganglia flow voids from lenticulostriate collaterals.](42e0b380-ae61-4ff6-8768-df296aaa930a) +*Axial T1 MR shows right frontal & left temporooccipital atrophy from remote ischemia. There are multiple small basal ganglia flow voids from lenticulostriate collaterals.* + +![Axial T1 C+ MR in the same patient shows multifocal white "dots" due to slow flow in multiple enlarged lenticulostriate collaterals.](374eec96-8734-408c-b33a-05aa263564a3) +*Axial T1 C+ MR in the same patient shows multifocal white "dots" due to slow flow in multiple enlarged lenticulostriate collaterals.* + +![Axial TOF MRA in a 2-year-old boy with moyamoya and multiple infarcts shows proliferation of small vessels in the thalamus.](3af0e4dd-763a-436f-823e-2013879bb923) +*Axial TOF MRA in a 2-year-old boy with moyamoya and multiple infarcts shows proliferation of small vessels in the thalamus.* + +![Axial FLAIR MR in the same patient shows multiple wedge-shaped areas of cortical signal abnormality , consistent with multifocal infarcts.](3b365f75-6f5f-4969-9672-9e10ebda423f) +*Axial FLAIR MR in the same patient shows multiple wedge-shaped areas of cortical signal abnormality , consistent with multifocal infarcts.* + diff --git a/docs_md/articles/multiinfarct-dementia_3823c4d4-5e98-46da-a717-892fef54b382.md b/docs_md/articles/multiinfarct-dementia_3823c4d4-5e98-46da-a717-892fef54b382.md index 02199c9..ed5c874 100644 --- a/docs_md/articles/multiinfarct-dementia_3823c4d4-5e98-46da-a717-892fef54b382.md +++ b/docs_md/articles/multiinfarct-dementia_3823c4d4-5e98-46da-a717-892fef54b382.md @@ -26,7 +26,7 @@ breadcrumbs: slug: "multiinfarct-dementia" treeNodeId: null category: "Nuclear Medicine" -cmeTopicId: "b2cd9aea-3e6d-4c30-9fd7-e49cc48a4549" +cmeTopicId: "29e9ca9b-f22e-44aa-94a4-19393851339d" documentVersionId: "2906e1eb-a93a-4cdb-8e7f-261c406e626c" imageCount: 24 lastUpdated: "07/21/25" @@ -232,7 +232,7 @@ breadcrumbs: - Lesions can include basal ganglia and other areas typically spared in other diseases - MR correlation helpful - 7855ad33-290d-4bb0-81a6-63126fea7e4f + 18eb7ef1-b90d-42fa-ae9c-fe185e01420c ## References @@ -252,90 +252,84 @@ breadcrumbs: ### Selected Images -![Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.](images/app.statdx.com_image_thumbnail_9790b351-4019-4c6d-aa10-5f455976b073_size_168_quality_85_6c88d907_20251014T185335Z.jpg) +![Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.](images/app.statdx.com_image_thumbnail_9790b351-4019-4c6d-aa10-5f455976b073_annotated_true_size_900_quality_90_e612492c0e4b5b90392f19e2a031291d7d77a3c4.jpg) *Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.* -![Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.](images/app.statdx.com_image_thumbnail_9790b351-4019-4c6d-aa10-5f455976b073_size_174_quality_85_0a048a3c_20251014T193347Z.jpg) +![Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.](images/app.statdx.com_image_thumbnail_9790b351-4019-4c6d-aa10-5f455976b073_size_174_quality_85_4cd6459c_20251018T155131Z.jpg) *Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.* -![Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.](images/app.statdx.com_image_thumbnail_9790b351-4019-4c6d-aa10-5f455976b073_size_174_quality_85_34cad0d2_20251014T185333Z.jpg) +![Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.](images/app.statdx.com_image_thumbnail_9790b351-4019-4c6d-aa10-5f455976b073_size_174_quality_85_a77e98a4d69e6053f6faa6bb10086de75ace83d3.jpg) *Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.* -![Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.](images/app.statdx.com_image_thumbnail_9790b351-4019-4c6d-aa10-5f455976b073_size_174_quality_85_63b05924_20251014T190917Z.jpg) -*Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.* - -![Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.](images/app.statdx.com_image_thumbnail_9790b351-4019-4c6d-aa10-5f455976b073_size_174_quality_85_85ee028f_20251014T204456Z.jpg) -*Axial graphic shows multifocal infarcts involving the cortical gray matter and subcortical white matter bilaterally.* - -![Coronal FLAIR MR of a 72-year-old woman demonstrates FLAIR signal abnormality in the periventricular and subcortical white matter (leukoaraiosis). This finding is consistent with a small vessel ischemic etiology.](images/app.statdx.com_image_thumbnail_e246e10c-9118-4293-96d2-18d1dc8192be_size_168_quality_85_00df6db0_20251014T185335Z.jpg) +![Coronal FLAIR MR of a 72-year-old woman demonstrates FLAIR signal abnormality in the periventricular and subcortical white matter (leukoaraiosis). This finding is consistent with a small vessel ischemic etiology.](images/app.statdx.com_image_thumbnail_e246e10c-9118-4293-96d2-18d1dc8192be_annotated_true_size_900_quality_90_38b46fb1ab1a335e57eed6ee88f2205029e9d5dd.jpg) *Coronal FLAIR MR of a 72-year-old woman demonstrates FLAIR signal abnormality in the periventricular and subcortical white matter (leukoaraiosis). This finding is consistent with a small vessel ischemic etiology.* -![Surface mapping of an FDG PET scan performed in a 96-year-old man demonstrates multiple areas of significant cortical abnormality in glucose metabolism, including the left frontal lobe , the right occipital lobe and the cingulate gyrus , consistent with multiinfarct dementia.](images/app.statdx.com_image_thumbnail_5f66168d-7754-460f-9e67-f9e5338828e7_size_168_quality_85_a11fae3d_20251014T185335Z.jpg) +![Surface mapping of an FDG PET scan performed in a 96-year-old man demonstrates multiple areas of significant cortical abnormality in glucose metabolism, including the left frontal lobe , the right occipital lobe and the cingulate gyrus , consistent with multiinfarct dementia.](images/app.statdx.com_image_thumbnail_5f66168d-7754-460f-9e67-f9e5338828e7_annotated_true_size_900_quality_90_e93c4d26b79a5d5f933d169bd4b8835bc499bff1.jpg) *Surface mapping of an FDG PET scan performed in a 96-year-old man demonstrates multiple areas of significant cortical abnormality in glucose metabolism, including the left frontal lobe , the right occipital lobe and the cingulate gyrus , consistent with multiinfarct dementia.* -![Axial F-18 FDG PET in a patient presenting with dementia shows more unilateral areas of hypometabolism and globally decreased F-18 FDG uptake, also consistent with vascular dementia (VaD).](images/app.statdx.com_image_thumbnail_d2ae652b-f028-4e9e-9cac-3b360345030e_size_168_quality_85_cbe960cd_20251014T185335Z.jpg) +![Axial F-18 FDG PET in a patient presenting with dementia shows more unilateral areas of hypometabolism and globally decreased F-18 FDG uptake, also consistent with vascular dementia (VaD).](images/app.statdx.com_image_thumbnail_d2ae652b-f028-4e9e-9cac-3b360345030e_annotated_true_size_900_quality_90_492fa70c1930f37903e3700341b443818a337817.jpg) *Axial F-18 FDG PET in a patient presenting with dementia shows more unilateral areas of hypometabolism and globally decreased F-18 FDG uptake, also consistent with vascular dementia (VaD).* ### Additional Images -![Alzheimer dementia is shown. Note the parietal and posterior temporal reductions and sparing of occipital and frontal lobes.](images/app.statdx.com_image_thumbnail_dc0a1eed-2c05-4a79-8b17-8b509ec07cfd_size_168_quality_85_07b57a2c_20251014T185335Z.jpg) +![Alzheimer dementia is shown. Note the parietal and posterior temporal reductions and sparing of occipital and frontal lobes.](images/app.statdx.com_image_thumbnail_dc0a1eed-2c05-4a79-8b17-8b509ec07cfd_annotated_true_size_900_quality_90_7d6c06e379c64647140a1a8f315addd9005be8ea.jpg) *Alzheimer dementia is shown. Note the parietal and posterior temporal reductions and sparing of occipital and frontal lobes.* -![Frontotemporal dementia (FTD) is shown. Note the frontal and anterior temporal reductions and sparing of parietal, posterior temporal, and occipital regions.](images/app.statdx.com_image_thumbnail_0038934c-33bc-4eb2-b4a8-3b0f64fe0315_size_168_quality_85_f569ead3_20251014T185335Z.jpg) +![Frontotemporal dementia (FTD) is shown. Note the frontal and anterior temporal reductions and sparing of parietal, posterior temporal, and occipital regions.](images/app.statdx.com_image_thumbnail_0038934c-33bc-4eb2-b4a8-3b0f64fe0315_annotated_true_size_900_quality_90_f625ae4d56bfd3c8d5c81dd9425e29795f356aae.jpg) *Frontotemporal dementia (FTD) is shown. Note the frontal and anterior temporal reductions and sparing of parietal, posterior temporal, and occipital regions.* -![Axial FDG PET in a patient with Lewy body disease shows parietal and posterior temporal reduction similar to Alzheimer disease (AD) , but occipital cortex is also involved.](images/app.statdx.com_image_thumbnail_cae51cd3-ac69-4862-9d82-17078b4400c9_size_168_quality_85_0d46f630_20251014T185335Z.jpg) +![Axial FDG PET in a patient with Lewy body disease shows parietal and posterior temporal reduction similar to Alzheimer disease (AD) , but occipital cortex is also involved.](images/app.statdx.com_image_thumbnail_cae51cd3-ac69-4862-9d82-17078b4400c9_annotated_true_size_900_quality_90_631ae1b35164ac73c19d8d7005109487a4e6639f.jpg) *Axial FDG PET in a patient with Lewy body disease shows parietal and posterior temporal reduction similar to Alzheimer disease (AD) , but occipital cortex is also involved.* -![Surface-rendered Tc-99m ECD SPECT in the same patient demonstrates severe reductions of parietal, temporal , and occipital cortex .](images/app.statdx.com_image_thumbnail_a10afb36-9b79-4677-b169-810b6303285b_size_168_quality_85_7735c389_20251014T185335Z.jpg) +![Surface-rendered Tc-99m ECD SPECT in the same patient demonstrates severe reductions of parietal, temporal , and occipital cortex .](images/app.statdx.com_image_thumbnail_a10afb36-9b79-4677-b169-810b6303285b_annotated_true_size_900_quality_90_6750937d4f73250ac4388139f46f8c71b5844319.jpg) *Surface-rendered Tc-99m ECD SPECT in the same patient demonstrates severe reductions of parietal, temporal , and occipital cortex .* -![Axial FDG PET in a case of autopsy-proven Creutzfeldt-Jakob disease (CJD) shows multiple cortical defects .](images/app.statdx.com_image_thumbnail_8d3cc9ee-c700-4302-b0ff-c256f3e6f9cc_size_168_quality_85_82b6b0bf_20251014T185335Z.jpg) +![Axial FDG PET in a case of autopsy-proven Creutzfeldt-Jakob disease (CJD) shows multiple cortical defects .](8d3cc9ee-c700-4302-b0ff-c256f3e6f9cc) *Axial FDG PET in a case of autopsy-proven Creutzfeldt-Jakob disease (CJD) shows multiple cortical defects .* -![Surface-rendered Tc-99m ECD SPECT shows a pattern similar to AD ; however, clinical course was that of a rapid progressive dementia ending in death within 12 months of onset.](images/app.statdx.com_image_thumbnail_9ca30abf-17a2-4fc4-8ce2-d132b969f4c3_size_168_quality_85_cd6dbb00_20251014T185335Z.jpg) +![Surface-rendered Tc-99m ECD SPECT shows a pattern similar to AD ; however, clinical course was that of a rapid progressive dementia ending in death within 12 months of onset.](9ca30abf-17a2-4fc4-8ce2-d132b969f4c3) *Surface-rendered Tc-99m ECD SPECT shows a pattern similar to AD ; however, clinical course was that of a rapid progressive dementia ending in death within 12 months of onset.* -![Axial Tc-99m ECD SPECT in a patient with clinical progressive supranuclear palsy (PSP) presentation shows severe frontal lobe decrease and mild reduction in caudate heads .](images/app.statdx.com_image_thumbnail_5dbf31bd-7f7f-4ce3-88da-c58bf3af58cf_size_168_quality_85_3d5a2562_20251014T185335Z.jpg) +![Axial Tc-99m ECD SPECT in a patient with clinical progressive supranuclear palsy (PSP) presentation shows severe frontal lobe decrease and mild reduction in caudate heads .](5dbf31bd-7f7f-4ce3-88da-c58bf3af58cf) *Axial Tc-99m ECD SPECT in a patient with clinical progressive supranuclear palsy (PSP) presentation shows severe frontal lobe decrease and mild reduction in caudate heads .* -![Surface-rendered Tc-99m ECD SPECT demonstrates severe frontal decrease relative to parietal and occipital cortex .](images/app.statdx.com_image_thumbnail_f145bf5c-b0f4-4973-983c-a9b658e92b51_size_168_quality_85_56feecd7_20251014T185335Z.jpg) +![Surface-rendered Tc-99m ECD SPECT demonstrates severe frontal decrease relative to parietal and occipital cortex .](f145bf5c-b0f4-4973-983c-a9b658e92b51) *Surface-rendered Tc-99m ECD SPECT demonstrates severe frontal decrease relative to parietal and occipital cortex .* -![Axial Tc-99m ECD SPECT (baseline) in a patient with early FTD shows frontal atrophy and mild reduction in perfusion .](images/app.statdx.com_image_thumbnail_17db9962-38b6-4fbc-9b7b-195575bfe8ab_size_168_quality_85_a6fe0add_20251014T185335Z.jpg) +![Axial Tc-99m ECD SPECT (baseline) in a patient with early FTD shows frontal atrophy and mild reduction in perfusion .](17db9962-38b6-4fbc-9b7b-195575bfe8ab) *Axial Tc-99m ECD SPECT (baseline) in a patient with early FTD shows frontal atrophy and mild reduction in perfusion .* -![Axial Tc-99m ECD SPECT in the same patient (18 months post baseline) demonstrates significant decrease of frontal lobe activity from baseline study , consistent with worsening dementia.](images/app.statdx.com_image_thumbnail_37c87d61-a119-4f80-81ce-8e922b6b0135_size_168_quality_85_f8a0896c_20251014T185335Z.jpg) +![Axial Tc-99m ECD SPECT in the same patient (18 months post baseline) demonstrates significant decrease of frontal lobe activity from baseline study , consistent with worsening dementia.](37c87d61-a119-4f80-81ce-8e922b6b0135) *Axial Tc-99m ECD SPECT in the same patient (18 months post baseline) demonstrates significant decrease of frontal lobe activity from baseline study , consistent with worsening dementia.* -![Surface-rendered Tc-99m ECD SPECT of the same patient (baseline) demonstrates mild frontal lobe findings .](images/app.statdx.com_image_thumbnail_01786172-6162-42ed-8a05-a359c16f3759_size_168_quality_85_e5c22c4a_20251014T185335Z.jpg) +![Surface-rendered Tc-99m ECD SPECT of the same patient (baseline) demonstrates mild frontal lobe findings .](01786172-6162-42ed-8a05-a359c16f3759) *Surface-rendered Tc-99m ECD SPECT of the same patient (baseline) demonstrates mild frontal lobe findings .* -![Surface-rendered Tc-99m ECD SPECT 18 months post baseline demonstrates worsening .](images/app.statdx.com_image_thumbnail_7956ee69-e5c6-425d-acc1-3d7a5f0add80_size_168_quality_85_080249b3_20251014T185335Z.jpg) +![Surface-rendered Tc-99m ECD SPECT 18 months post baseline demonstrates worsening .](7956ee69-e5c6-425d-acc1-3d7a5f0add80) *Surface-rendered Tc-99m ECD SPECT 18 months post baseline demonstrates worsening .* -![Axial FDG PET in a patient with Huntington disease and mild dementia shows severe reduction in basal ganglia .](images/app.statdx.com_image_thumbnail_e4888ead-8358-4e9f-bd18-4ec6c29e084b_size_168_quality_85_f5cdab9e_20251014T185335Z.jpg) +![Axial FDG PET in a patient with Huntington disease and mild dementia shows severe reduction in basal ganglia .](e4888ead-8358-4e9f-bd18-4ec6c29e084b) *Axial FDG PET in a patient with Huntington disease and mild dementia shows severe reduction in basal ganglia .* -![Surface-rendered Tc-99m ECD SPECT in the same patient shows mild frontal and parietal reductions .](images/app.statdx.com_image_thumbnail_e12e1edb-5d84-4862-8739-3d32d0ad0b88_size_168_quality_85_5cef7127_20251014T185335Z.jpg) +![Surface-rendered Tc-99m ECD SPECT in the same patient shows mild frontal and parietal reductions .](e12e1edb-5d84-4862-8739-3d32d0ad0b88) *Surface-rendered Tc-99m ECD SPECT in the same patient shows mild frontal and parietal reductions .* -![Surface-rendered Tc-99m ECD SPECT in a patient with normal pressure hydrocephalus (NPH) shows severe frontal and parietal defects with preservation of vertex .](images/app.statdx.com_image_thumbnail_07edad0d-74ce-4a31-9dff-da1fc76be8ba_size_168_quality_85_688dbc4f_20251014T185335Z.jpg) +![Surface-rendered Tc-99m ECD SPECT in a patient with normal pressure hydrocephalus (NPH) shows severe frontal and parietal defects with preservation of vertex .](07edad0d-74ce-4a31-9dff-da1fc76be8ba) *Surface-rendered Tc-99m ECD SPECT in a patient with normal pressure hydrocephalus (NPH) shows severe frontal and parietal defects with preservation of vertex .* -![In-111 DTPA cisternogram (24 hours) in the same patient shows abnormal ventricular activity .](images/app.statdx.com_image_thumbnail_b2483042-f995-4199-ab60-52d0e7c6144d_size_168_quality_85_f157c45a_20251014T185335Z.jpg) +![In-111 DTPA cisternogram (24 hours) in the same patient shows abnormal ventricular activity .](b2483042-f995-4199-ab60-52d0e7c6144d) *In-111 DTPA cisternogram (24 hours) in the same patient shows abnormal ventricular activity .* -![Axial Tc-99m ECD SPECT in a patient with multiinfarct dementia shows multiple infarcts of the frontal and parietal cortex .](images/app.statdx.com_image_thumbnail_8434672d-6281-4521-ab2c-d09f5e17b548_size_168_quality_85_86c243fe_20251014T185335Z.jpg) +![Axial Tc-99m ECD SPECT in a patient with multiinfarct dementia shows multiple infarcts of the frontal and parietal cortex .](8434672d-6281-4521-ab2c-d09f5e17b548) *Axial Tc-99m ECD SPECT in a patient with multiinfarct dementia shows multiple infarcts of the frontal and parietal cortex .* -![Surface-rendered Tc-99m ECD SPECT in the same patient shows asymmetric cortical findings .](images/app.statdx.com_image_thumbnail_cb09e173-1ef7-440a-8c92-0dfe31c76630_size_168_quality_85_e2d1c8bf_20251014T185335Z.jpg) +![Surface-rendered Tc-99m ECD SPECT in the same patient shows asymmetric cortical findings .](cb09e173-1ef7-440a-8c92-0dfe31c76630) *Surface-rendered Tc-99m ECD SPECT in the same patient shows asymmetric cortical findings .* -![Surface-rendered Tc-99m ECD SPECT in a patient with history of cocaine abuse and early dementia shows diffuse cortical findings.](images/app.statdx.com_image_thumbnail_344c72d7-e6ba-4274-8a15-d9b9f7cace6d_size_168_quality_85_7371e27d_20251014T185335Z.jpg) +![Surface-rendered Tc-99m ECD SPECT in a patient with history of cocaine abuse and early dementia shows diffuse cortical findings.](344c72d7-e6ba-4274-8a15-d9b9f7cace6d) *Surface-rendered Tc-99m ECD SPECT in a patient with history of cocaine abuse and early dementia shows diffuse cortical findings.* -![Surface-rendered Tc-99m ECD SPECT in a patient with history of methamphetamine abuse and early dementia is shown.](images/app.statdx.com_image_thumbnail_74ffbc46-1f77-45b4-a13a-22a16305c4ef_size_168_quality_85_cd398f87_20251014T185335Z.jpg) +![Surface-rendered Tc-99m ECD SPECT in a patient with history of methamphetamine abuse and early dementia is shown.](74ffbc46-1f77-45b4-a13a-22a16305c4ef) *Surface-rendered Tc-99m ECD SPECT in a patient with history of methamphetamine abuse and early dementia is shown.* 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 3a0db51..fce1b3f 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 @@ -30,7 +30,6 @@ breadcrumbs: slug: "multiple-sclerosis" treeNodeId: null category: "Brain" -cmeTopicId: "97aa8ddd-431a-415a-bb95-ee9dd7e2f7b8" documentVersionId: "7ced4781-f9a4-4a48-a5ed-954fa6ea87cf" imageCount: 27 lastUpdated: "10/08/20" @@ -348,7 +347,7 @@ breadcrumbs: - 95% with clinically definite MS have positive MR - 6559b86c-1d8c-4fba-8298-b9f6b25bee2e + b58b4061-1b92-4b32-abf6-1debb49d180c ## References @@ -394,87 +393,114 @@ breadcrumbs: ### 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_3151b2a8.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_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_9ced0f97.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.](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.* -![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_ba42cd98.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.](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 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_d82d6f23.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).](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.* -![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_d3924df1.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.](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.* -![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_dc979aa5.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.](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 .* -![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_2a272c0c.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 .](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.* -![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_a51567ed.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.](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.* -![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_8ae270d3.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.](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 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_24ea7299.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.](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_annotated_true_size_900_quality_90_0f607b49.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.](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_annotated_true_size_900_quality_90_63dd89fd.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 .](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_annotated_true_size_900_quality_90_9a29183f.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.](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_annotated_true_size_900_quality_90_67b5a9ed.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.](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_annotated_true_size_900_quality_90_b9e0b0c2.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.)](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_annotated_true_size_900_quality_90_03c00fdf.jpg) +![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_annotated_true_size_900_quality_90_dde3dcaa.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.](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_annotated_true_size_900_quality_90_c5ec2b8c.jpg) +![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_annotated_true_size_900_quality_90_8c39896e.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.](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_annotated_true_size_900_quality_90_748fde8c.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.](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_annotated_true_size_900_quality_90_60e0b345.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.](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_annotated_true_size_900_quality_90_f83b9f8c.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.](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_annotated_true_size_900_quality_90_90ce03b8.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.](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_annotated_true_size_900_quality_90_1a6a9f01.jpg) +![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_annotated_true_size_900_quality_90_4869582d.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.](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_annotated_true_size_900_quality_90_2a3054e6.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.](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_annotated_true_size_900_quality_90_36896162.jpg) +![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/docs_md/articles/multiple-sclerosis_89599954-599e-4410-a517-eb22125cedfb.md b/docs_md/articles/multiple-sclerosis_89599954-599e-4410-a517-eb22125cedfb.md index eaec77a..fafbffb 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 @@ -26,7 +26,7 @@ breadcrumbs: slug: "multiple-sclerosis" treeNodeId: null category: "Spine" -cmeTopicId: "22f4d8b2-7235-490f-a6d2-281c5a511765" +cmeTopicId: "692beaa3-dede-4124-9c3c-4c4548645bb3" documentVersionId: "63c33284-44a3-44de-86c1-af9e0cf914e2" imageCount: 22 lastUpdated: "02/12/25" @@ -36,6 +36,11 @@ pageTitle: "Multiple Sclerosis | STATdx" enhancedTitle: "Multiple Sclerosis" type: "DX" references: true +ddx: true +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'}" +cases: 2 breadcrumbs: - "Spine" - "Diagnosis" @@ -394,7 +399,7 @@ breadcrumbs: - Imaging findings must be correlated with clinical & laboratory features to confirm diagnosis - Acute MS can mimic cord neoplasm - c27dbea4-44c7-464e-aed3-e961e498f066 + 23e58f52-8b8e-4c96-9a03-de07a43975cd ## References @@ -438,6 +443,24 @@ breadcrumbs: 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) +## Differential diagnosis + +### Intramedullary Spinal Cord Lesion +DDX:56579195-7385-4918-872c-c25d965e8486 + +## Anatomy + +### 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 + +- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '84757fd8-c814-48a5-adad-10bddd50d9eb', 'description': 'Variability of plaque conspicuity with different sequences.\n\nSagittal T1 image (#1) is normal. Sagittal T2 image (#2) (TR 3100/TE 112) shows no definite abnormality within cord. Sagittal intermediate image (#3) (TR 4000/TE 12) show focal hyperintensity within cord at C3 and C5 levels (arrows). Sagittal STIR image (#4) (TR 3500/TE 13/TI 110) show best conspicuity of the plaques, with slightly less signal to noise.\n\nComment: Heavily T2 weighted images are not the optimum for evaluation of intrinsic cord lesions, since they tend to give an image with only two signals (CSF and soft tissue) and do not have good sensitivity to subtle changes in cord signal.', 'history': None, 'imagePoolId': '25f459bd-eb87-4b6a-bd90-4f6399b5cfd6', 'name': 'Comparison of sequences', 'teachingPoint': None}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '99626c28-2b97-4a0a-8140-b4aa51c1ad5d', 'description': 'Typical appearance of MS lesions in the cervical cord.\n\nMR study (#1-4) shows 2 focal lesions of increased signal on the STIR image (arrows, #1) that are less than 1 vertebral body in length. There is no definite cord expansion and no edema beyond the focal lesions. Axial GE image shows 1 of the lesions at C4 within the posterior aspect of the cord (arrow, #2). Following contrast, there is mild ill-defined enhancement of the C4 intramedullary plaque (arrows, #3,4). Incidental note is made of a small disc protrusion at C5-6.', 'history': 'Chronic myelopathy.', 'imagePoolId': 'fd0bbd9f-8683-4aec-8d0a-5a842598a071', 'name': 'Enhancing plaque', 'teachingPoint': None, 'demographics': '34 Years old female'}, {'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': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '27b5fe08-9933-4472-bdbd-5ae4bfa5f107', 'description': 'Typical pattern of involvement in Devic disease.\n\nBrain study (#1) is normal. Evaluation of the optic nerves (#2-4) show abnormal T2 hyperintensity in the right optic nerve that shows pronounced enhancement (arrow, #3,4). The cervical cord shows a long segment (4 vertebral bodies in length) of T2 hyperintensity and mild diffuse cord enlargement (#5-7). Axial T2 image (#8) shows central cord hyperintensity. Following contrast administration (#9) there is minimal patchy enhancement of the cord. The extensive nature of the cord involvement is given the title "longitudinally extensive" cord signal abnormality. This is distinct from the much more focal abnormalities typically seen with cord involvement with MS.', 'history': None, 'imagePoolId': '90c40690-dacf-4d4d-9b7c-04e39a144bc0', 'name': 'Long segment cord demyelination', 'teachingPoint': None}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '4513b381-b5d0-4fbf-a315-5a265090e55f', 'description': 'Typical pattern of cord involvement.\n\nMR study (#1-4) shows focal T2 hyperintensity within the cord at C5 level, which shows both central (arrow, #2,3) and peripheral involvement (curved arrow, #2-4). No cord expansion.', 'history': None, 'imagePoolId': 'df27b385-6074-49f9-bbcd-a963856b974f', 'name': '3T', 'teachingPoint': None}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '27734ddd-b837-4047-85bb-1eaecf654e82', 'description': 'Multiple foci of cord enhancement in multiple sclerosis, some well-defined, and other ill-defined.\n\nImages through the cervical and thoracic cord (#1-7) show multiple foci of T2 hyperintensity (arrows, #1, 4, 5) consistent with demyelinating disease. There are foci of enhancement which vary from focal (curved arrow, #2, 3) to ill-defined (open arrow, #2, 6, 7). \n\nComment: The multiplicity of lesions along with the lack of edema or significant cord expansion is typical for demyelinating disease.', 'history': None, 'imagePoolId': 'ff2932a9-1190-4f36-bcaa-37b354ccf75d', 'name': 'Enhancing plaques', 'teachingPoint': None}, {'authors': [{'key': '99e1aff7-f42c-43a0-95ae-d89c8551aa01', 'value': 'Kevin R. Moore, MD'}], 'caseVersionId': '12839b07-fb73-472c-9539-48a0b136dd84', 'description': 'Typical case of acute spinal cord demyelination.\n\nSagittal T1WI MR (#1) is normal. Sagittal T2WI MR (#2) and STIR MR (#3) demonstrate multiple hyperintense intramedullary lesions with focal cord enlargement, typical of demyelination. Although MS is often considered a white matter disease, in fact spinal cord and brain gray matter involvement is very common. Sagittal T1 C+ MR (#4) shows faint enhancement of several lesions (arrows). Axial T2WI MR (#5, 6) are useful to localize lesion in relation to cord somatotopy. Axial T1 C+ MR (#7, 8) confirm faint ring enhancement (arrows). Sagittal (#9) and axial (#10) FLAIR MR images of the brain demonstrate concurrent severe corpus callosum and white matter brain lesions typical of MS.', 'history': 'Patient presents with relapsing/remitting Multiple Sclerosis (MS) exacerbation, complaining of left arm weakness, facial numbness, and dysmetria.', 'imagePoolId': 'b3a41192-9565-42f9-9ffd-2b494611e94a', 'name': 'Classic', 'teachingPoint': None, 'demographics': '17 Years old female'}, {'authors': [{'key': '61869900-31b1-4db7-b4f5-d4da24fa86f3', 'value': 'Mark Z. Chen, MD'}], 'caseVersionId': '16a742ad-313d-439a-af55-981ce0aede3a', 'description': 'Typical case of thoracic intramedullary demyelinating plaque.\n\nSagittal T2WI FS MR (#1) demonstrates a focal intramedullary hyperintense lesion (arrow) expanding the thoracic spinal cord. Axial T2WI (#2) and T1WI MR (#3) confirm the eccentrically located intramedullary lesion (arrow). Axial T1 C+ MR (#4) reveals peripheral ring enhancement implying more acute demyelination (arrow). Sagittal T2WI MR through the brain (#5) shows a hyperintense lesion (arrow) involving the corpus callosum, typical of MS.', 'history': 'Multiple Sclerosis patient presents with myelopathic symptoms referable to the thoracic level. ', 'imagePoolId': '204e85f8-1235-414c-8d9e-227cca403070', 'name': 'Classic, acute intramedullary lesion', 'teachingPoint': None, 'demographics': '30 Years old female'}, {'authors': [{'key': '99e1aff7-f42c-43a0-95ae-d89c8551aa01', 'value': 'Kevin R. Moore, MD'}], 'caseVersionId': '4e0e98c9-4b87-4e35-be86-b63c45123852', 'description': 'Typical case of multiple intramedullary spinal cord demyelinating lesions.\n\nSagittal T1WI MR (#1, 2) show mild degenerative disc disease but are otherwise normal. Sagittal T2WI MR (#3, 4) reveal multiple T2 hyperintense intramedullary demyelinating lesions (arrows). Sagittal STIR MR (#5, 6) show the same lesions, as well as an additional dorsal spinal cord lesion not visualized on the T2WI MR images (open arrow). In general, STIR MR is the most sensitive imaging sequence for detecting spinal demyelinating lesions. Axial T2WI MR (#7, 8) are most useful to localize lesion center (arrows) in relationship to spinal cord long tracts.', 'history': 'Patient with known Multiple Sclerosis (MS) presents for imaging during acute MS exacerbation.', 'imagePoolId': '305fd70f-34c8-421d-bebf-71ef42f45d7c', 'name': 'Multiple intramedullary lesions', 'teachingPoint': None, 'demographics': '53 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': 'b975cd06-048d-477e-b75e-3d34569e5131', 'description': "Multiple sclerosis, neuromyelitis optica variant (Devic's disease).\n\nT2 weighted image (#1) shows mild fusiform thoracic cord enlargement with hyperintensity (arrows). Sagittal and axial images following contrast (#2-4) show a large area of ring enhancement within the cord, with small punctate cord peripheral enhancement caudal to the major lesion (open arrow). Axial images show near total transverse cord involvement with enhancement.", 'history': 'Patient developed waist and bilateral lower extremity numbness, progressed to weakness, and inability to walk with hospital\nadmission and treatment with IV steroids and plasma exchange therapy.\n', 'imagePoolId': 'b49b8ab0-c8e1-4046-9a41-0a45f103d8d7', 'name': "Devic's variant", 'teachingPoint': None, 'demographics': '46 Years old female'}, {'authors': [{'key': '99e1aff7-f42c-43a0-95ae-d89c8551aa01', 'value': 'Kevin R. Moore, MD'}], 'caseVersionId': 'c9a7e1ce-0904-4882-a79a-08eca9797985', 'description': 'Variant case depicts ring enhancement and intrinsic lesion T1 hyperintensity.\n\nSagittal T1WI MR (#1, 2) demonstrate mild peripheral "ring" T1 hyperintensity (arrow) within an intramedullary demyelinating lesion, which has been attributed to paramagnetic metal deposition. Sagittal T2WI (#3, 4) depict peripheral T2 hyperintensity (arrow) and mild cord swelling. Sagittal T1 C+ MR (#5, 6) reveal peripheral rim-enhancement (arrows) of the lesion margins, indicating the advancing "wave" of demyelination. Axial T2WI MR (#7) and T1 C+ MR (#8) confirm same findings within the right lateral spinal cord (arrow). This pattern of signal intensity on the previous sequences is commonly noted in brain lesions but unusual in the spinal cord.', 'history': 'Adult patient with known Multiple Sclerosis (MS) presents with acute myelopathy for imaging work-up.', 'imagePoolId': '926f079e-67f4-4591-96bc-030a70f10ea2', 'name': 'Ring lesion enhancement', 'teachingPoint': None, 'demographics': '43 Years old male'}], 'caseType': 'variant', 'name': 'VARIANT'} + ## Images @@ -447,46 +470,46 @@ breadcrumbs: ![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_6f4ec63b.jpg) +![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) *Sagittal T2 (left) & T1 C+ MR (right) show an active plaque at the C6-C7 level with ring enhancement & focal T2 hyperintensity .* -![Sagittal T2 (left), PD (middle), & STIR (right) MR show multiple short-segment multiple sclerosis (MS) plaques within the thoracic cord . Note the relatively improved conspicuity of the plaques on the PD & STIR relative to the routine T2 sequence.](images/app.statdx.com_image_thumbnail_6bfb1493-e878-43b1-a089-407b3f0efbba_annotated_true_size_900_quality_90_792ac687.jpg) +![Sagittal T2 (left), PD (middle), & STIR (right) MR show multiple short-segment multiple sclerosis (MS) plaques within the thoracic cord . Note the relatively improved conspicuity of the plaques on the PD & STIR relative to the routine T2 sequence.](images/app.statdx.com_image_thumbnail_6bfb1493-e878-43b1-a089-407b3f0efbba_annotated_true_size_900_quality_90_28a87781_20251018T142350Z.jpg) *Sagittal T2 (left), PD (middle), & STIR (right) MR show multiple short-segment multiple sclerosis (MS) plaques within the thoracic cord . Note the relatively improved conspicuity of the plaques on the PD & STIR relative to the routine T2 sequence.* -![Sagittal T2 (left), T2 (middle), & T1 C+ FS (right) MR of the thoracic spine show multiple short-segment foci of T2 hyperintensity in this patient with MS. Multiple lesions show solid enhancement .](images/app.statdx.com_image_thumbnail_b72c256f-7f30-4ff0-9b03-2848dbebf832_annotated_true_size_900_quality_90_1ba5536a.jpg) +![Sagittal T2 (left), T2 (middle), & T1 C+ FS (right) MR of the thoracic spine show multiple short-segment foci of T2 hyperintensity in this patient with MS. Multiple lesions show solid enhancement .](images/app.statdx.com_image_thumbnail_b72c256f-7f30-4ff0-9b03-2848dbebf832_annotated_true_size_900_quality_90_8f2ca444_20251018T142350Z.jpg) *Sagittal T2 (left), T2 (middle), & T1 C+ FS (right) MR of the thoracic spine show multiple short-segment foci of T2 hyperintensity in this patient with MS. Multiple lesions show solid enhancement .* -![Axial T2 MR shows focal MS plaques as T2 hyperintensity within both the right & left lateral aspects of the cervical cord .](images/app.statdx.com_image_thumbnail_5d6a0b48-ad9b-482b-99e5-bb3acf61fd6d_annotated_true_size_900_quality_90_fa543fc7.jpg) +![Axial T2 MR shows focal MS plaques as T2 hyperintensity within both the right & left lateral aspects of the cervical cord .](images/app.statdx.com_image_thumbnail_5d6a0b48-ad9b-482b-99e5-bb3acf61fd6d_annotated_true_size_900_quality_90_d2dc5fbb_20251018T142350Z.jpg) *Axial T2 MR shows focal MS plaques as T2 hyperintensity within both the right & left lateral aspects of the cervical cord .* -![Axial T1 C+ MR shows focal enhancement within the right & left lateral aspect of the cervical cord in this patient with active MS plaques.](images/app.statdx.com_image_thumbnail_65ddf85c-133c-416b-987b-f64f8360baea_annotated_true_size_900_quality_90_4f7690be.jpg) +![Axial T1 C+ MR shows focal enhancement within the right & left lateral aspect of the cervical cord in this patient with active MS plaques.](images/app.statdx.com_image_thumbnail_65ddf85c-133c-416b-987b-f64f8360baea_annotated_true_size_900_quality_90_9dd80371_20251018T142350Z.jpg) *Axial T1 C+ MR shows focal enhancement within the right & left lateral aspect of the cervical cord in this patient with active MS plaques.* -![Sagittal T2 (left) & T1 C+ MR (right) show active enhancing plaque at the C2 level with both focal well-defined (enhancing) T2 focus & a small amount of surrounding nonenhancing edema .](images/app.statdx.com_image_thumbnail_927e7fcc-08ba-4599-a057-8c54ea03bfad_annotated_true_size_900_quality_90_e623a162.jpg) +![Sagittal T2 (left) & T1 C+ MR (right) show active enhancing plaque at the C2 level with both focal well-defined (enhancing) T2 focus & a small amount of surrounding nonenhancing edema .](images/app.statdx.com_image_thumbnail_927e7fcc-08ba-4599-a057-8c54ea03bfad_annotated_true_size_900_quality_90_c2a635e9_20251018T142350Z.jpg) *Sagittal T2 (left) & T1 C+ MR (right) show active enhancing plaque at the C2 level with both focal well-defined (enhancing) T2 focus & a small amount of surrounding nonenhancing edema .* -![Sagittal T2 (left) & T1 C+ FS (right) MR show several T2-hyperintense foci in the cervical cord in this patient with MS. 2 of the lesions enhance reflecting active demyelination .](images/app.statdx.com_image_thumbnail_ddda2a05-d65f-416c-9100-45a824c86f76_annotated_true_size_900_quality_90_1c99d02e.jpg) +![Sagittal T2 (left) & T1 C+ FS (right) MR show several T2-hyperintense foci in the cervical cord in this patient with MS. 2 of the lesions enhance reflecting active demyelination .](images/app.statdx.com_image_thumbnail_ddda2a05-d65f-416c-9100-45a824c86f76_annotated_true_size_900_quality_90_a8ef1ebd_20251018T142350Z.jpg) *Sagittal T2 (left) & T1 C+ FS (right) MR show several T2-hyperintense foci in the cervical cord in this patient with MS. 2 of the lesions enhance reflecting active demyelination .* -![Axial T2WI MR shows focal T2-hyperintense demyelinating lesions with both central & peripheral involvement . T2-hyperintense lesions are not specific for plaque age, degree of myelin & axon loss, or amount of edema & inflammation.](images/app.statdx.com_image_thumbnail_e26b711f-9edd-4e0b-bf62-14e7fdc5e6e5_annotated_true_size_900_quality_90_78d8e2e7.jpg) +![Axial T2WI MR shows focal T2-hyperintense demyelinating lesions with both central & peripheral involvement . T2-hyperintense lesions are not specific for plaque age, degree of myelin & axon loss, or amount of edema & inflammation.](images/app.statdx.com_image_thumbnail_e26b711f-9edd-4e0b-bf62-14e7fdc5e6e5_annotated_true_size_900_quality_90_c6c3c128_20251018T142350Z.jpg) *Axial T2WI MR shows focal T2-hyperintense demyelinating lesions with both central & peripheral involvement . T2-hyperintense lesions are not specific for plaque age, degree of myelin & axon loss, or amount of edema & inflammation.* -![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.](images/app.statdx.com_image_thumbnail_c5b7cee6-36e2-4513-8201-dd8945569641_annotated_true_size_900_quality_90_08b23ace.jpg) +![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.](c5b7cee6-36e2-4513-8201-dd8945569641) *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.* ### Additional Images -![Sagittal T2WI MR shows diffuse cord enlargement & hyperintensity throughout cervical segment into upper thoracic cord in this case of acute MS exacerbation.](images/app.statdx.com_image_thumbnail_c54e8dc1-7734-4ed7-9b33-79ce219f52c6_annotated_true_size_900_quality_90_6eddbf8b.jpg) +![Sagittal T2WI MR shows diffuse cord enlargement & hyperintensity throughout cervical segment into upper thoracic cord in this case of acute MS exacerbation.](c54e8dc1-7734-4ed7-9b33-79ce219f52c6) *Sagittal T2WI MR shows diffuse cord enlargement & hyperintensity throughout cervical segment into upper thoracic cord in this case of acute MS exacerbation.* -![Sagittal T1WI C+ MR shows diffuse cord expansion & extensive multilevel cord enhancement in acute exacerbation of MS.](images/app.statdx.com_image_thumbnail_226aa411-0834-4f44-8e71-d7bd384946f2_annotated_true_size_900_quality_90_4698f831.jpg) +![Sagittal T1WI C+ MR shows diffuse cord expansion & extensive multilevel cord enhancement in acute exacerbation of MS.](226aa411-0834-4f44-8e71-d7bd384946f2) *Sagittal T1WI C+ MR shows diffuse cord expansion & extensive multilevel cord enhancement in acute exacerbation of MS.* -![Axial T1WI C+ MR with fat suppression of cervical cord in a different patient shows right peripheral nodular enhancement.](images/app.statdx.com_image_thumbnail_cc419118-3518-48c0-8d90-80cb7dbd02b0_annotated_true_size_900_quality_90_95d9db01.jpg) +![Axial T1WI C+ MR with fat suppression of cervical cord in a different patient shows right peripheral nodular enhancement.](cc419118-3518-48c0-8d90-80cb7dbd02b0) *Axial T1WI C+ MR with fat suppression of cervical cord in a different patient shows right peripheral nodular enhancement.* -![Sagittal T2WI MR of cervical cord shows a more discrete demyelinating focus at C3-C4.](images/app.statdx.com_image_thumbnail_3105c954-4abb-4532-aff9-e78e3f6d136d_annotated_true_size_900_quality_90_f737c42d.jpg) +![Sagittal T2WI MR of cervical cord shows a more discrete demyelinating focus at C3-C4.](3105c954-4abb-4532-aff9-e78e3f6d136d) *Sagittal T2WI MR of cervical cord shows a more discrete demyelinating focus at C3-C4.* ![Axial T2WI MR of cervical cord in another patient shows a poorly defined, wedge-shaped, mildly hyperintense plaque within right lateral aspect of the cord.](804853d2-0057-48d2-b52f-39b3793c192b) diff --git a/docs_md/articles/otosclerosis_ddc7b884-3c17-4834-9e96-d985c6b618a9.md b/docs_md/articles/otosclerosis_ddc7b884-3c17-4834-9e96-d985c6b618a9.md new file mode 100644 index 0000000..bbe5b6c --- /dev/null +++ b/docs_md/articles/otosclerosis_ddc7b884-3c17-4834-9e96-d985c6b618a9.md @@ -0,0 +1,456 @@ +--- +title: "Otosclerosis" +docid: "ddc7b884-3c17-4834-9e96-d985c6b618a9" +authors: + - key: "07a2c087-6202-49e7-870b-7aa162d18f06" + value: "Bronwyn E. Hamilton, MD" + - key: "4b6589b0-9b8d-4467-8a90-01a0a59742fc" + value: "Troy A. Hutchins, MD" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Diagnosis" + slug: "diagnosis" + treeNodeId: "19b6b986-97d0-40e7-b317-00f0c5cd8fa2" + - + name: "Temporal Bone" + slug: "temporal-bone" + treeNodeId: "9ad7d7b2-b2e4-4de2-be04-55ce607560c9" + - + name: "Inner Ear" + slug: "inner-ear" + treeNodeId: "1092a44e-f762-4fab-8991-36dff52535cb" + - + name: "Infectious and Inflammatory Lesions" + slug: "infectious-and-inflammatory-lesions" + treeNodeId: "c1b8dac8-34f8-4c89-8664-b9b153d878eb" + - + name: "Otosclerosis" + slug: "otosclerosis" + treeNodeId: null +category: "Head and Neck" +cmeTopicId: "ece2b7f0-702e-40e6-908c-dd1ffa3be6e3" +documentVersionId: "ca5797ec-4f56-46bd-b9d3-a583c34fa6fe" +imageCount: 18 +lastUpdated: "08/10/21" +pageDescription: "Otosclerosis" +pageKeywords: "Head and Neck, Diagnosis, Temporal Bone, Inner Ear, Infectious and Inflammatory Lesions, Otosclerosis" +pageTitle: "Otosclerosis | STATdx" +enhancedTitle: "Otosclerosis" +type: "DX" +references: true +breadcrumbs: + - "Head and Neck" + - "Diagnosis" + - "Temporal Bone" + - "Inner Ear" + - "Infectious and Inflammatory Lesions" + - "Otosclerosis" +--- +# KEY FACTS + +- ## Terminology + + + - Synonym:**Otospongiosis** + - Types: Fenestral otosclerosis (FOto), cochlear otosclerosis (COto) + - Pathologic appearance of lytic, spongy bone foci in bony labyrinth of unknown cause + - Starts perifenestral (FOto), progresses to surround cochlea (FOto + COto) + - **Fissula ante fenestram**: Cleft of fibrocartilaginous tissue between inner & middle ears just anterior to oval window +- ## Imaging + + + - Best diagnostic clue: Temporal bone CT shows **lytic (otospongiotic) foci** involving bony labyrinth + - FOto: Starts at anterior margin of oval window (fissula ante fenestram) + - COto: Affects pericochlear bony labyrinth +- ## Top Differential Diagnoses + + + - Chronic otitis media with tympanosclerosis + - Temporal bone Paget disease + - Temporal bone fibrous dysplasia + - Temporal bone osteoradionecrosis + - Temporal bone osteogenesis imperfecta +- ## Pathology + + + - Enchondral layer of bony labyrinth displays spongy, vascular, decalcified, irregular bone formation +- ## Clinical Issues + + + - Bilateral progressive conductive (FOto) or mixed (FOto + COto) hearing loss in young adult +- ## Diagnostic Checklist + + + - Typical otospongiotic plaques of otosclerosis are **lytic** & affect **bony** labyrinth + +# TERMINOLOGY + +- ## Abbreviations + + + - Fenestral otosclerosis (FOto) + - Cochlear otosclerosis (COto) +- ## Synonyms + + + - Otospongiosis, fenestral otospongiosis, cochlear otospongiosis +- ## Definitions + + + - Pathologic appearance of **lytic, spongy bone foci** in bony labyrinth of unknown cause + - Starts perifenestral (FOto), progresses to surround cochlea (FOto + COto) + - **Fissula ante fenestram**: Cleft of fibrocartilaginous tissue between inner & middle ears just anterior to oval window + - Cochlear cleft is fatty marrow due to incomplete ossification that parallels cochlea rather than localizing to fissula antefenestram + - May be present in children & adults, should be differentiated from otosclerosis + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Temporal bone CT: Lucent (otospongiotic) foci involving bony labyrinth + - Usually in context of normally aerated middle ear + - ### Location + + + - FOto: Starts at anterior margin of oval window (fissula ante fenestram) + - May involve any bony area along medial wall middle ear + - COto: Affects pericochlear bony labyrinth + - May involve any portion of bony labyrinth + - ### Size + + + - Millimeter punctate or linear foci; may become confluent + - ### Morphology + + + - FOto: Ovoid plaques most common + - COto: Ovoid to linear (confluent foci) +- ## CT Findings + + + - ### CECT + + + - No role for CECT in diagnosis of otosclerosis + - ### Bone CT + + + - **Early** temporal bone CT findings + - Begins as radiolucent focus at oval window anterior margin (FOto) + - Spreads to involve all margins of oval & round windows + - Abnormal thickening of otic capsule bone near oval window (> 2.3 mm) with bulging contour + - May spread to inner ear otic capsule (COto) + - Double ring sign or "halo" of radiolucency surrounds cochlea in severe COto + - Progressive disease may involve any portion of bony labyrinth, including internal auditory canal lateral walls + - **Late**, chronic (healing phase) temporal bone CT findings + - FOto: "Heaped up" new bone along oval & round window margins + - Healed plaque may occlude oval ± round window + - COto: Mixed radiolucent-radiodense foci present in bony labyrinth +- ## MR Findings + + + - ### T1WI + + + - Faint intermediate T1 signal of plaques + - ### T2WI + + + - Thin-section high-resolution T2 may not visualize otosclerosis, even when extensive + - Large plaques can show increased signal + - ### T1WI C+ + + + - Enhancing punctate foci in medial wall of middle ear (FOto) ± pericochlear bony labyrinth (COto) + - Most obvious when FOto & COto combined + - Enhancing lesions may be seen anywhere in bony labyrinth in severe cases +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Temporal bone CT + - ### Protocol advice + + + - T1 C+ MR shows enhancing foci in active phase + - High-resolution T2 MR may miss otosclerosis + +# DIFFERENTIAL DIAGNOSIS + +- [Chronic Otitis Media With Tympanosclerosis](/document/chronic-otomastoiditis-with-tympan-/e8b91656-ad18-405d-bf67-b0b902f04265) + - Clinical: Obvious chronic middle ear-mastoid inflammatory disease + - Imaging: Postinflammatory new bone deposition is not limited to oval & round windows as with most FOto + - Seen in tympanic membrane (TM), middle ear, ossicles, & mastoids + - New bone deposition is irregular, not smooth, in oval window area +- [Temporal Bone Paget Disease](/document/temporal-bone-paget-disease/d0c7cbca-1489-488e-ae7d-1b9aee88467d) + - Clinical: Bone disease of old age (> 50 years) + - Imaging: Diffuse skull base involvement is rule + - Diffuse involvement of bony labyrinth, not confined to lateral wall + - Usually seen as diffuse temporal bone cotton wool appearance +- [Temporal Bone Fibrous Dysplasia](/document/temporal-bone-fibrous-dysplasia/e5b44f77-f666-4f32-8eb0-6ed2da7d9898) + - Clinical: Bone disease of young (age < 30 years) + - Imaging: Involves all parts of temporal bone + - Relative sparing of inner ear is rule + - Usually sclerotic, ground-glass in appearance +- [Temporal Bone Osteoradionecrosis](/document/temporal-bone-osteoradionecrosis/95475621-6d44-4bbd-a779-476154a5a2cc) + - Clinical: History of skull base or nasopharyngeal radiation therapy + - Imaging: CT shows diffuse, permeative lucencies of otic capsule +- [Temporal Bone Osteogenesis Imperfecta](/document/temporal-bone-osteogenesis-imperfe-/d819723f-660b-4067-9b47-f8aeec3ba0b8) + - Clinical: Blue sclera; patients with mild form develop deafness by 40 years of age + - Imaging: Looks like severe COto with more generalized demineralization of bony labyrinth + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Unknown + - ### Genetics + + + - Sporadic or autosomal dominant gene transmission + - Bony otic capsule development: 3 layers + - Thin inner endosteal layer + - **Middle layer** of combined endochondral & intrachondral bone (**otosclerosis occurs here**) + - Outer periosteal layer + - Normal otosclerosis progression + - Begins at fissula ante fenestram (FOto) + - Disease spreads from fissula ante fenestram posteriorly along oval window margins to round window + - Continued active disease spreads to otic capsule (both FOto & COto present) + - Active **FOto fixes stapes footplate** in oval window niche + - This "donut" FOto ankyloses stapes footplate + - Pathophysiology of **conductive hearing loss** + - COto leads to sensorineural hearing loss + - Best hypothesis: Spiral ligament becomes compromised + - Secondary hypothesis: Toxic proteases affect cochlear nerve cells +- ## Staging, Grading, & Classification + + + - Symons/Fanning CT grading system of otosclerosis (2005) has high intra- & interobserver agreement + - Grade 1: Solely fenestral + - Grade 2: Patchy localized cochlear disease (± FOto) + - To basal cochlear turn (grade 2A) + - To middle/apical turns (grade 2B) + - Grade 3: Diffuse confluent cochlear involvement (± FOto) +- ## Gross Pathologic & Surgical Features + + + - Otoscopic vascular hue behind TM = **Schwartze sign** + - Active otosclerotic areas along margins of oval & round windows or beneath cochlear promontory + - Bony ankylosis of stapes footplate is reflected as stapes immobilization when pulled on by surgeon +- ## Microscopic Features + + + - Enchondral layer of bony labyrinth displays spongy, vascular, decalcified, irregular bone formation + - 3 pathologic phases of otosclerosis + - Acute phase: Deposition of islets of osteoid tissue + - Subacute phase: Spongiotic remodeling with osteoclasts causing focal bone resorption + - Chronic-sclerotic phase: Osteoblasts create new bone with irregular features resembling mosaic + - **Otospongiosis** better describes active disease process + - Chronic, healing phase appears truly **sclerotic** + - May be histologically indistinguishable from Paget disease + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Bilateral progressive conductive (FOto) or mixed (FOto + COto) hearing loss + - ### Other signs/symptoms + + + - Tinnitus (ringing in ears) + - Otoscopy: Vascular hue behind TM = Schwartze sign + - ### Clinical profile + + + - Young adult presenting with unexplained **bilateral progressive** conductive or **mixed** **hearing loss** +- ## Demographics + + + - ### Age + + + - Appears in 2nd to 3rd decades of life + - ### Sex + + + - M:F = 1:2 + - ### Epidemiology + + + - Occurs in 1% of population + - Most common type is **FOto alone (85%)**; COto in 15% + - **FOto** causes ~ **90%****conductive hearing loss in a****dults** +- ## Natural History & Prognosis + + + - FOto: Conductive hearing loss is progressive + - COto: Untreated, will evolve to profound hearing loss +- ## Treatment + + + - FOto: **Stapedectomy** with stapes prosthesis + - Results negatively impacted by concurrent COto + - If round window is obliterated, stapes prosthesis will fail + - If narrow oval window niche height (< 1.4 mm on coronal CT reformat), stapes surgery more challenging + - Cochlear implantation + - Used when severe FOto & COto present bilaterally, resulting in profound mixed hearing loss + - If round window obliteration present bilaterally, cochlear implantation may be more challenging + - **Fluoride** treatment if COto present + - Early treatment can arrest progression + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Always check oval window anterior margin for FOto in CT evaluation of conductive hearing loss + - Common blind spot; CT findings can be subtle + - If COto present, FOto also is present, so look for it + - MDCT sometimes shows normal fissula ante fenestram on pediatric temporal bone exams as focal radiolucency +- ## Image Interpretation Pearls + + + - Typical otospongiotic plaques of otosclerosis are lucent & affect bony labyrinth + - If bony fills in membranous labyrinth, diagnosis is **labyrinthine ossificans**, not COto +- ## Reporting Tips + + + - Assess oval & round window patency; narrowing or obliteration have important surgical implications + + 383a23f2-eea4-4589-bce6-050a80b7aac1 + +## References + +# Selected References + +1. 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[Rondini-Gilli E et al: [Otosclerosis surgical techniques and results in 150 patients] Ann Otolaryngol Chir Cervicofac. 119(4):227-33, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12410119%5Bpmid%5D) +1. [Stimmer H et al: Magnetic resonance imaging and high-resolution computed tomography in the otospongiotic phase of otosclerosis. ORL J Otorhinolaryngol Relat Spec. 64(6):451-3, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12499773%5Bpmid%5D) +1. [Chole RA et al: Pathophysiology of otosclerosis. Otol Neurotol. 22(2):249-57, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11300278%5Bpmid%5D) +1. [Ruckenstein MJ et al: Management of far advanced otosclerosis in the era of cochlear implantation. Otol Neurotol. 22(4):471-4, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11449102%5Bpmid%5D) +1. [Shin YJ et al: Correlations between computed tomography findings and family history in otosclerotic patients. 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Neuroradiology. 39(6):453-7, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9225330%5Bpmid%5D) +1. [Miura M et al: Computed tomographic image analysis of ears with otosclerosis. ORL J Otorhinolaryngol Relat Spec. 58(4):200-3, 1996](http://www.ncbi.nlm.nih.gov/pubmed/?term=8883105%5Bpmid%5D) +1. [Valvassori GE: Imaging of otosclerosis. Otolaryngol Clin North Am. 26(3):359-71, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8341568%5Bpmid%5D) +1. [Wilbrand HF: Radioanatomy of cochlear and stapedial otosclerosis. Scand Audiol Suppl. 30:181-3, 1988](http://www.ncbi.nlm.nih.gov/pubmed/?term=3227265%5Bpmid%5D) +1. [Mafee MF et al: Use of CT in stapedial otosclerosis. Radiology. 156(3):709-14, 1985](http://www.ncbi.nlm.nih.gov/pubmed/?term=4023230%5Bpmid%5D) +1. [Swartz JD et al: Fenestral and cochlear otosclerosis: computed tomographic evaluation. Am J Otol. 6(6):476-81, 1985](http://www.ncbi.nlm.nih.gov/pubmed/?term=4073255%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Coronal graphic illustrates findings of fenestral otosclerosis with a "donut" otospongiotic plaque surrounding the stapes footplate in the oval window. The crisp margins of the oval window are obscured by plaque.](images/app.statdx.com_image_thumbnail_edf1b230-ca36-4909-9a2a-20b76cd87b30_annotated_true_size_900_quality_90_c1c8225a7c580f8738035e33ba9f30b58cd3c704.jpg) +*Coronal graphic illustrates findings of fenestral otosclerosis with a "donut" otospongiotic plaque surrounding the stapes footplate in the oval window. The crisp margins of the oval window are obscured by plaque.* + +![Coronal graphic illustrates findings of fenestral otosclerosis with a "donut" otospongiotic plaque surrounding the stapes footplate in the oval window. The crisp margins of the oval window are obscured by plaque.](images/app.statdx.com_image_thumbnail_edf1b230-ca36-4909-9a2a-20b76cd87b30_size_174_quality_85_3544281237a14ad55265220ad49af5019da70cec.jpg) +*Coronal graphic illustrates findings of fenestral otosclerosis with a "donut" otospongiotic plaque surrounding the stapes footplate in the oval window. The crisp margins of the oval window are obscured by plaque.* + +![Coronal right temporal bone CT shows a lytic focus anterior to the oval window , the typical appearance and location of an otospongiotic plaque of fenestral otosclerosis.](images/app.statdx.com_image_thumbnail_ebbe8d39-b2ce-4561-b322-aa06dbac9bdb_annotated_true_size_900_quality_90_2dd54fdaee91c1cc85708d804c7967bcf499649c.jpg) +*Coronal right temporal bone CT shows a lytic focus anterior to the oval window , the typical appearance and location of an otospongiotic plaque of fenestral otosclerosis.* + +![Axial graphic demonstrates a classic example of cochlear otosclerosis. Note otospongiotic plaques in a "halo" around the cochlea with concurrent fenestral otosclerosis .](images/app.statdx.com_image_thumbnail_4b2dd1e6-40ef-4071-8547-ad0ced2a33ed_annotated_true_size_900_quality_90_8217ff25c5054d3a65590e5f2e60edd6a3627047.jpg) +*Axial graphic demonstrates a classic example of cochlear otosclerosis. Note otospongiotic plaques in a "halo" around the cochlea with concurrent fenestral otosclerosis .* + +![Axial left temporal bone CT shows cochlear otosclerosis as osteolytic foci surrounding the cochlea . Concurrent fenestral otosclerosis is noted as bony lucency along cochlear promontory extending from the fissula ante fenestram .](images/app.statdx.com_image_thumbnail_1f23931f-1264-4ee9-9a09-aeeca44df50f_annotated_true_size_900_quality_90_b611357d0335923c661eac1e7e64bea2aaa0fa7e.jpg) +*Axial left temporal bone CT shows cochlear otosclerosis as osteolytic foci surrounding the cochlea . Concurrent fenestral otosclerosis is noted as bony lucency along cochlear promontory extending from the fissula ante fenestram .* + +![Axial bone CT shows a thick, lucent otosclerotic plaque anterior to the oval window in the expected location of the fissula ante fenestram. An abnormal bulging convex contour is also noted due to the thickened bone. Stapes prosthesis is noted in the oval window .](images/app.statdx.com_image_thumbnail_345b431a-5485-4d04-8d15-35a5084443e6_annotated_true_size_900_quality_90_f93b16581f39eb64b4e0ce65a626ed4488475b53.jpg) +*Axial bone CT shows a thick, lucent otosclerotic plaque anterior to the oval window in the expected location of the fissula ante fenestram. An abnormal bulging convex contour is also noted due to the thickened bone. Stapes prosthesis is noted in the oval window .* + +![Coronal right temporal bone CT in a patient with mixed hearing loss shows a "halo" of radiolucency surrounding the cochlea , representing cochlear otosclerosis. Also note the associated fenestral otosclerosis .](images/app.statdx.com_image_thumbnail_5b695628-d6da-434c-8068-f8c29006e516_annotated_true_size_900_quality_90_9d04d2017b15bb7775c5460da7af92a0c333b564.jpg) +*Coronal right temporal bone CT in a patient with mixed hearing loss shows a "halo" of radiolucency surrounding the cochlea , representing cochlear otosclerosis. Also note the associated fenestral otosclerosis .* + +![Axial bone CT shows a small lucent plaque of fenestral otosclerosis thickening the otic capsule bone immediately anterior to the oval window. Note the stapes visualized within the oval window .](images/app.statdx.com_image_thumbnail_0deddff0-28f6-4bb1-a157-339122e7497f_annotated_true_size_900_quality_90_286c485dc3367f02e0b5fa32afbf6815dffd354c.jpg) +*Axial bone CT shows a small lucent plaque of fenestral otosclerosis thickening the otic capsule bone immediately anterior to the oval window. Note the stapes visualized within the oval window .* + +![Axial left temporal bone CT demonstrates mixed lucent and sclerotic otospongiotic plaque obstructing the round window . This predisposes to stapes prosthesis failure and makes cochlear implantation more challenging.](images/app.statdx.com_image_thumbnail_3fd79262-9e87-4e80-bcf2-3e4472d01276_annotated_true_size_900_quality_90_2036a6d59cb24997b65bc3d21ffdd2705b8624d3.jpg) +*Axial left temporal bone CT demonstrates mixed lucent and sclerotic otospongiotic plaque obstructing the round window . This predisposes to stapes prosthesis failure and makes cochlear implantation more challenging.* + +![Axial left temporal bone CT shows typical lytic plaques of combined fenestral and cochlear otosclerosis . The patient has undergone stapedectomy with insertion of a stapes prosthesis. Note the metallic density stapes prosthesis at the oval window.](images/app.statdx.com_image_thumbnail_2aa90aa0-7e1b-478e-a95c-75e4be032029_annotated_true_size_900_quality_90_7c98fe22d21fd2c02bd3f6d42d5cd27bbb0471a3.jpg) +*Axial left temporal bone CT shows typical lytic plaques of combined fenestral and cochlear otosclerosis . The patient has undergone stapedectomy with insertion of a stapes prosthesis. Note the metallic density stapes prosthesis at the oval window.* + +![Axial T1WI C+ FS MR in the same patient reveals enhancement anterior to the oval window (fissula ante fenestram) and surrounding the cochlea , representing active fenestral and cochlear otosclerosis, respectively.](d233ee57-32fd-42a9-8c1a-1dd7895eaf65) +*Axial T1WI C+ FS MR in the same patient reveals enhancement anterior to the oval window (fissula ante fenestram) and surrounding the cochlea , representing active fenestral and cochlear otosclerosis, respectively.* + + +### Additional Images + +![Axial bone CT shows an extensive pericochlear lucent "halo" of cochlear otosclerosis.](69398b59-d88b-4d2a-b993-9dc1e3d83288) +*Axial bone CT shows an extensive pericochlear lucent "halo" of cochlear otosclerosis.* + +![Axial thin T2 MR in the same patient shows multifocal otic capsule hyperintensities that corresponded to plaques on CT.](b77b2e89-f579-4b0d-b9a8-420125c30337) +*Axial thin T2 MR in the same patient shows multifocal otic capsule hyperintensities that corresponded to plaques on CT.* + +![Coronal right temporal bone CT shows fenestral otosclerosis involving all the margins of the oval window . The net effect is to create a blurring and disappearance of the oval window niche.](ef834334-0bb6-4abc-a6c9-87fc765210b2) +*Coronal right temporal bone CT shows fenestral otosclerosis involving all the margins of the oval window . The net effect is to create a blurring and disappearance of the oval window niche.* + +![Axial temporal bone CT demonstrates a classic otospongiotic plaque as extra lucent foci on the anterior margin of the oval window (fissula ante fenestram location). The otic capsule is otherwise spared.](edb8cf6c-80bd-4e2d-b216-e9ae77986446) +*Axial temporal bone CT demonstrates a classic otospongiotic plaque as extra lucent foci on the anterior margin of the oval window (fissula ante fenestram location). The otic capsule is otherwise spared.* + +![Coronal bone CT in a patient who has undergone stapedectomy for fenestral otosclerosis shows metallic stapes prosthesis. Also note otospongiotic plaque just anterior to the oval window .](dbfe0708-d68d-49a6-a792-08a826b3afec) +*Coronal bone CT in a patient who has undergone stapedectomy for fenestral otosclerosis shows metallic stapes prosthesis. Also note otospongiotic plaque just anterior to the oval window .* + +![Axial bone CT demonstrates a severe case of combined fenestral and cochlear otosclerosis. The Schwartze sign was clearly seen on otoscopic examination.](9e6c9a4d-03c3-40c9-b3fe-009302402be2) +*Axial bone CT demonstrates a severe case of combined fenestral and cochlear otosclerosis. The Schwartze sign was clearly seen on otoscopic examination.* + +![Axial T1WI C+ FS MR of the right temporal bone in a severe case of combined fenestral and cochlear otosclerosis with enhancement signifying active disease is shown. Less avid enhancement in the vestibule and cochlea represents endolymphatic hydrops, which is sometimes associated with otosclerosis.](a8ed8660-79fd-4178-a3ad-9c64ff31b838) +*Axial T1WI C+ FS MR of the right temporal bone in a severe case of combined fenestral and cochlear otosclerosis with enhancement signifying active disease is shown. Less avid enhancement in the vestibule and cochlea represents endolymphatic hydrops, which is sometimes associated with otosclerosis.* + +![Coronal left temporal bone CT in the same patient again demonstrates the lytic otospongiotic plaque of fenestral otosclerosis in the expected location anterior to the oval window.](921cb3c2-e839-4f75-8729-4709f9f3498e) +*Coronal left temporal bone CT in the same patient again demonstrates the lytic otospongiotic plaque of fenestral otosclerosis in the expected location anterior to the oval window.* + 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 3c4079b..c796830 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 @@ -34,7 +34,7 @@ breadcrumbs: slug: "pediatric-multiple-sclerosis-brain" treeNodeId: null category: "Pediatrics" -cmeTopicId: "5c944d9c-ee53-49a3-9a90-e1f707594205" +cmeTopicId: "03b623c3-7d10-4a0f-88ec-e7830afde051" documentVersionId: "caa247d6-e650-4954-8d11-33d96c2d0244" imageCount: 27 lastUpdated: "02/14/24" @@ -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. 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[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 ### 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_2107f1fe.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_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_16009693.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_835d2990.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.](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_5ada3e09.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.](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_833f7c4a.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.](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_42250ed1.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).](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_feca178f.jpg) +![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_111bca6d.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 .](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_0952f4d2.jpg) +![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_87d62012.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.](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_50b115e6.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.](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_f0962fe8.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.](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_d15493a5.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.](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_8c03ce80.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).](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_4271c4b1.jpg) +![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_9e61bd7e.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.](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_c522346a.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.](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_2374bb1c.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.](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.](c74fc30d-c463-45b9-b324-2a8cffd7d113) +![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 .](4eb0a6d0-9539-4204-a570-926f106081ed) +![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.](7ddbe865-5df6-44ca-8781-de1475539664) +![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_8397af53.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.)](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_11308c3e.jpg) +![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_b9bfa142.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.](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_de803e77.jpg) +![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_5ca2d7aa.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.](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_7e795547.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.](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_10746ede.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.](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/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 index 1315a2a..9a6e062 100644 --- 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 @@ -34,7 +34,7 @@ breadcrumbs: slug: "pediatric-multiple-sclerosis-spine" treeNodeId: null category: "Pediatrics" -cmeTopicId: "8d4798d2-6c54-45d9-b0d5-a34cffcc7379" +cmeTopicId: "54a46ea3-7b26-4525-964c-6aa99ac50564" documentVersionId: "28aeb597-125a-4f17-91b9-738a90cdd336" imageCount: 19 lastUpdated: "02/09/24" @@ -409,7 +409,7 @@ breadcrumbs: - Imaging findings must be correlated with clinical & laboratory features to confirm diagnosis - Acute MS can mimic cord neoplasm - 9c9e280b-3eef-4a05-8a69-231f86f0ff65 + ec82b351-fbdc-43cf-8899-7d0ffed659d4 ## References @@ -460,34 +460,34 @@ breadcrumbs: ### 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_3f5cabad.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.](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_6a89ecb0.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.](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_9ed6c04f.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.](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_01d3524e.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.](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_a0b2475e.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.](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_5aeef48c.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.](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_50880e84.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.](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_94784ca9.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 .](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_18b8a871.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 .](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) diff --git a/docs_md/articles/pediatric-seizure_3e2ea1fa-0651-45eb-bc1f-b072af8dd434.md b/docs_md/articles/pediatric-seizure_3e2ea1fa-0651-45eb-bc1f-b072af8dd434.md new file mode 100644 index 0000000..2c27093 --- /dev/null +++ b/docs_md/articles/pediatric-seizure_3e2ea1fa-0651-45eb-bc1f-b072af8dd434.md @@ -0,0 +1,354 @@ +--- +title: "Pediatric Seizure" +docid: "3e2ea1fa-0651-45eb-bc1f-b072af8dd434" +authors: + - key: "b94c42b1-c572-4e72-ac48-bfe85989e2f3" + value: "Andrew T. Trout, MD" + - key: "0a00bb19-ed17-4500-8bae-c9463720a4fb" + value: "Nadeen K. Abu Ata, MD" + - key: "d2ed5cde-67ab-491a-963b-3c0f245d1fd8" + value: "Karol Cardenas, MD" +breadcrumbs: + - + name: "Nuclear Medicine" + slug: "nuclear-medicine" + treeNodeId: "2406533f-6523-4211-841e-b92d6f8cf34e" + - + name: "Pediatrics" + slug: "pediatrics" + treeNodeId: "8e60feb1-8a8a-42e6-9f90-5270fdc48da2" + - + name: "Central Nervous System" + slug: "central-nervous-system" + treeNodeId: "435fce7c-f625-479e-9355-82bab423f838" + - + name: "Pediatric Seizure" + slug: "pediatric-seizure" + treeNodeId: null +category: "Nuclear Medicine" +cmeTopicId: "57ed2edc-8f94-4608-8ae8-a421b16d7851" +documentVersionId: "0d72d140-13c0-4727-843d-665ecaa6a0eb" +imageCount: 23 +lastUpdated: "06/20/25" +pageDescription: "Pediatric Seizure" +pageKeywords: "Nuclear Medicine, Pediatrics, Central Nervous System, Pediatric Seizure" +pageTitle: "Pediatric Seizure | STATdx" +enhancedTitle: "Pediatric Seizure" +type: "DX" +references: true +breadcrumbs: + - "Nuclear Medicine" + - "Pediatrics" + - "Central Nervous System" + - "Pediatric Seizure" +--- +# KEY FACTS + +- ## Imaging + + + - Nuclear imaging is not part of routine evaluation of isolated seizures + - Plays important role in multidisciplinary/multimodality work-up of intractable epilepsy + - Nuclear imaging may identify structurally inconspicuous epileptogenic foci + - SPECT: Radiotracer deposition reflects regional cerebral blood flow + - Interictal: Epileptogenic focus appears as area of decreased radiotracer deposition (hypoperfusion) + - Ictal: Epileptogenic focus appears as area of increased radiotracer deposition (hyperperfusion) + - SISCOM may identify epileptogenic foci that are inconspicuous on visual assessment of ictal and interictal SPECT images + - F-18 FDG PET: Epileptogenic focus appears as area of hypometabolism (larger than epileptogenic focus) + - Statistical parametric mapping can help detect and confirm foci of hypometabolism + - MR is ideal modality to identify structural causes of seizure/epilepsy + - Mesial temporal sclerosis: Decreased volume, increased T2 signal in hippocampus + - Focal cortical dysplasia (FCD): Focal cortical thickening and increased T2 signal, blurring of gray-white junction +- ## Clinical Issues + + + - Most common cause of epilepsy + - Young: FCD + - Adolescent/young adult: Mesial temporal sclerosis + - Antiepileptic drugs are 1st line of therapy + - Surgical resection: Reserved for patients with intractable, focal epilepsy + +# TERMINOLOGY + +- ## Definitions + + + - Seizure: Clinical manifestation of aberrant neuronal electrical discharge(s) in brain + - Epilepsy: ≥ 2 unprovoked, afebrile seizures + +# IMAGING + +- ## General Features + + + - Imaging of isolated/acute seizures is generally structural (CT/MR) and reserved for patients with focal or complex seizures or focal neurologic signs + - Imaging is important component in management of epilepsy + - Structural imaging (CT/MR) often performed at diagnosis to exclude structural cause + - Multimodality imaging (including nuclear) plays substantial role in work-up of intractable epilepsy + - Work-up of intractable epilepsy is multidisciplinary/multimodality process + - Clinical assessment: History, seizure semiology + - Electroencephalography (EEG): Scalp and intracranial + - MR: Structural, ± functional + - Perfusion (SPECT) imaging + - Metabolic (F-18 FDG PET) imaging + - Magnetoencephalography +- ## Nuclear Medicine Findings + + + - Nuclear imaging not part of routine evaluation of isolated seizures + - May identify structurally inconspicuous epileptogenic foci + - **SPECT perfusion** + - Radiotracer deposition reflects regional cerebral blood flow + - Tc-99m hexamethylpropyleneamine oxime (HMPAO) and Tc-99m ethyl cysteinate dimer (ECD) most commonly used tracers + - Lipophilic, small molecules diffuse across blood-brain barrier + - ECD clears more rapidly from blood pool, has more linear extraction at high blood flow rates; less nonspecific scalp and soft tissue uptake than HMPAO + - High 1st-pass extraction, peak accumulation in ~ 2 min, no substantial redistribution + - Can image for at least 2 hours after injection without substantial loss of fidelity + - Interictal SPECT + - Epileptogenic focus appears as area of decreased radiotracer deposition (hypoperfusion) + - Ictal SPECT + - Epileptogenic focus appears as area of increased radiotracer deposition (hyperperfusion) + - Correspondence to epileptogenic focus depends on interval between seizure onset and injection; longer intervals allow more propagation to surrounding tissue + - Beware of pseudonormalization where hyperperfusion during ictus makes baseline hypoperfused focus appear symmetric to normal side + - Higher sensitivity than interictal SPECT for epileptogenic focus + - Subtraction ictal SPECT coregistered to MR (SISCOM) + - Means to compare ictal and interictal SPECT imaging and localize abnormalities to MR + - Foci of ictal hyperperfusion that correspond with interictal hypoperfusion appear as foci of activity on SISCOM + - May identify epileptogenic foci that are inconspicuous on visual assessment of ictal and interictal SPECT images + - **F-18 FDG PET** + - Interictal exam due to prolonged (~ 30 min) uptake of F-18 FDG + - If seizure occurs during uptake phase, may see hypermetabolism at epileptogenic focus and in surrounding brain + - Indirect marker of neuronal activity + - Epileptogenic focus appears as area of hypometabolism + - Area of hypometabolism is generally larger than epileptogenic focus + - Highest accuracy in temporal lobe epilepsy (TLE), less likely to identify epileptogenic focus in extratemporal epilepsy + - Contributes most in cases of structurally inconspicuous TLE or cases of suspected cortical dysplasia in children with apparent negative MR + - Higher sensitivity than interictal SPECT, especially in TLE (84% vs. 66% in one metaanalysis) + - Postprocessing with statistical parametric mapping (SPM) can help detect and confirm foci of hypometabolism + - SPM compares, on pixel by pixel basis, F-18 FDG uptake in patient to normal database to identify foci of abnormally decreased uptake + - In addition to identifying epileptogenic focus, it may provide prognostic information + - For example, in patients with unilateral temporal lobe epilepsy, presence of bitemporal glucose hypometabolism is associated with poor memory performance + - With both SPECT and PET imaging, may see corresponding downstream abnormality in cerebellar hemisphere opposite involved cerebral hemisphere + - Decreased radiotracer uptake on interictal SPECT and F-18 FDG PET + - Increased radiotracer uptake on ictal SPECT + - Hypometabolism of ipsilateral thalamus can be seen in cases of focal epilepsy (mostly in frontal and temporal cortex, medial temporal lobe epilepsy) + - **Other tracers** + - C-11 flumazenil: Binds CNS gamma-aminobutyric acid (GABA) receptors; GABA receptors are decreased in epileptogenic foci + - C-11 has short half-life (20 minutes), requiring onsite cyclotron (limiting its use) +- ## MR Findings + + + - Mesial temporal sclerosis: Decreased volume and increased T2 signal in mesial temporal structures, including hippocampal formation + - Resultant asymmetry in size of temporal horn of lateral ventricle + - Focal cortical dysplasia (FCD): Cortical thickening and increased T2 signal, extension of T2 signal to ventricle, blurring of gray-white junction + +# DIFFERENTIAL DIAGNOSIS + +- ## CNS Tumor + + + - May or may not be cause of seizures + - May appear as focus of hypo- or hyperperfusion on SPECT + - May appear as focus of hypometabolism on F-18 FDG PET if low grade +- ## Congenital Anomalies + + + - [Structural abnormalities: Heterotopic gray matter, abnormal sulcation, schizencephaly](/document/heterotopic-gray-matter/c88b27b7-d352-4231-b296-bd9d93b8c68b) + - May appear as focus of hypometabolism on FDG PET, even if not epileptogenic +- ## Tuberous Sclerosis + + + - Can be difficult to identify epileptogenic focus as majority of tubers are hypoperfused and hypometabolic + - Epileptogenic tubers generally show perfusion/metabolic abnormalities larger than area of structural abnormality +- ## Rasmussen Encephalitis + + + - Progressive inflammatory process involving unilateral cerebral hemisphere, generally with progressive cerebral atrophy + - Appears as large areas (lobar or hemispheric) of perfusion or metabolic abnormality + +# PATHOLOGY + +- ## Gross Pathologic & Surgical Features + + + - Mesial temporal sclerosis: Hard, shrunken hippocampus + - FCD: Firm, rubbery cortical focus +- ## Microscopic Features + + + - Mesial temporal sclerosis + - Variable distribution of pyramidal neuronal loss + - Gliosis + - FCD: Abnormalities in neuronal migration resulting in cortical dyslamination + - Type I: Abnormal cortical layering + - Ia: Abnormal radial migration of neurons with abundant microcolumns (> 8 neurons vertically arranged), most conspicuous in layer 3 + - May see immature small neurons or hypertrophic pyramidal neurons outside of layer 5 + - Ib: Abnormal tangential cortical layering + - May see no layering at all or abnormal layering of layers 2 &/or 4 + - May see immature small neurons, hypertrophic pyramidal neurons outside of layer 5, or normal neurons with disordered dendrites + - Ic: Abnormal radial and tangential lamination + - Type II: Abnormal cortical layering **and**cytologic abnormalities + - IIa: No identifiable cortical layering except layer 1 + - Dysmorphic neurons (enlarged cell body and nucleus, neurofilament accumulation in cytoplasm) + - IIb: No identifiable cortical layering except layer 1 + - Dysmorphic neurons **and** balloon cells (large cell body, multiple nuclei, eosinophilic cytoplasm) + - Type III: Abnormal cortical layering associated with primary brain lesion (adjacent or in same lobe) + - IIIa: FCD associated with hippocampal sclerosis + - IIIb: FCD associated with tumors + - IIIc: FCD associated with vascular malformation + - IIId: FCD associated with any other lesion acquired in early life + +# CLINICAL ISSUES + +- ## Demographics + + + - ### Epidemiology + + + - Seizure + - Most common causes of acute seizures: Fever, infection, head injury + - Febrile seizures + - Usually occur between 6 months and 5 years of age + - Occur in 3-8% of children < 5 years + - 60% risk of recurrence + - Epilepsy + - Highest incidence in 1st year of life (~ 90-200 per 100,000) + - Prevalence higher in rural areas + - Risk factors + - Family history + - Prior febrile seizure: 2-7% develop epilepsy + - Most common causes + - Young: FCD + - Adolescent/young adult: Mesial temporal sclerosis + - Intractable epilepsy occurs in 20-30% +- ## Natural History & Prognosis + + + - Epilepsy subtypes: Current terminology + - Genetic abnormality without discrete structural abnormality + - Structural or metabolic + - Discrete structural epileptogenic lesion + - Many of these are genetic in etiology + - Metabolic condition leading to propensity for seizures + - Unknown cause + - FCD: Malformation of cortical development + - Secondary to insult (genetic, infectious, ischemic) during development + - Mesial temporal sclerosis + - Often secondary to insult (infection, trauma, febrile seizures) early in life +- ## Treatment + + + - Antiepileptic drugs + - 1st line of therapy + - Managing clinician will often try multiple agents and combinations of agents to achieve seizure reduction (or freedom) with minimum of side effects + - Ketogenic diet + - Vagal nerve stimulator + - Used in patients with drug-resistant epilepsy who are not candidates for resection + - Deep brain stimulator + - Used in patients with drug-resistant epilepsy who are not candidates for resection + - Surgical resection: Reserved for patients with drug-resistant, focal epilepsy + - Outcomes better if resection includes sites identified on SISCOM + - Outcome of surgery for FCD is better if lesion visible by MR + + d21db106-d94e-4b66-b849-bb707a0aabd1 + +## References + +# Selected References + +1. [Ponisio MR et al: FDG-PET/MRI in the presurgical evaluation of pediatric epilepsy. Pediatr Radiol. 54(10):1589-602, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=39123082%5Bpmid%5D) +1. [Guo J et al: Seizure outcome after surgery for refractory epilepsy diagnosed by (18)F-fluorodeoxyglucose positron emission tomography ((18)F-FDG PET/MRI): a systematic review and meta-analysis. World Neurosurg. 173:34-43, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36746239%5Bpmid%5D) +1. [Juhász C et al: Utility of MRI, PET, and ictal SPECT in presurgical evaluation of non-lesional pediatric epilepsy. Seizure. 77:15-28, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31122814%5Bpmid%5D) +1. [Wong-Kisiel LC et al: Challenges in managing epilepsy associated with focal cortical dysplasia in children. Epilepsy Res. 145:1-17, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29803953%5Bpmid%5D) +1. [Manford M: Recent advances in epilepsy. J Neurol. 264(8):1811-24, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28120042%5Bpmid%5D) +1. [Mountz JM et al: Pediatric epilepsy: neurology, functional imaging, and neurosurgery. Semin Nucl Med. 47(2):170-87, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28237005%5Bpmid%5D) +1. [Duncan JS et al: Brain imaging in the assessment for epilepsy surgery. Lancet Neurol. 15(4):420-33, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26925532%5Bpmid%5D) +1. [Miyata H et al: Surgical pathology of epilepsy-associated non-neoplastic cerebral lesions: a brief introduction with special reference to hippocampal sclerosis and focal cortical dysplasia. Neuropathology. 33(4):442-58, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23530853%5Bpmid%5D) +1. [Sidhu R et al: Pediatric seizures. Pediatr Rev. 34(8):333-41; 342, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23908360%5Bpmid%5D) +1. [Kim S et al: SPECT Imaging of epilepsy: an overview and comparison with F-18 FDG PET. Int J Mol Imaging. 2011:813028, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21785722%5Bpmid%5D) +1. [O'Brien TJ et al: Subtraction peri-ictal SPECT is predictive of extratemporal epilepsy surgery outcome. Neurology. 55(11):1668-77, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11113221%5Bpmid%5D) +1. [Won HJ et al: Comparison of MR imaging with PET and ictal SPECT in 118 patients with intractable epilepsy. AJNR Am J Neuroradiol. 20(4):593-9, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10319968%5Bpmid%5D) +1. [Devous MD Sr et al: SPECT brain imaging in epilepsy: a meta-analysis. J Nucl Med. 39(2):285-93, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9476937%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Coronal interictal F-18 FDG PET in a 15-year-old with focal left temporal seizures on EEG and findings of left hippocampal sclerosis on MR shows diffuse hypometabolism of the left temporal lobe , indicative of a seizure focus. Interpretation of functional brain imaging is aided by the fact that perfusion (SPECT) and metabolism (F-18 FDG) should be symmetric between the cerebral hemispheres and between the cerebellar hemispheres.](images/app.statdx.com_image_thumbnail_a299a4be-cf98-4fae-9b94-9d63f01d1923_annotated_true_size_900_quality_90_ec7f85c342f846b721ec871004ce8d2b4dd18fd3.jpg) +*Coronal interictal F-18 FDG PET in a 15-year-old with focal left temporal seizures on EEG and findings of left hippocampal sclerosis on MR shows diffuse hypometabolism of the left temporal lobe , indicative of a seizure focus. Interpretation of functional brain imaging is aided by the fact that perfusion (SPECT) and metabolism (F-18 FDG) should be symmetric between the cerebral hemispheres and between the cerebellar hemispheres.* + +![Coronal interictal F-18 FDG PET in a 15-year-old with focal left temporal seizures on EEG and findings of left hippocampal sclerosis on MR shows diffuse hypometabolism of the left temporal lobe , indicative of a seizure focus. Interpretation of functional brain imaging is aided by the fact that perfusion (SPECT) and metabolism (F-18 FDG) should be symmetric between the cerebral hemispheres and between the cerebellar hemispheres.](images/app.statdx.com_image_thumbnail_a299a4be-cf98-4fae-9b94-9d63f01d1923_size_174_quality_85_bd69a85a183112b275f8c1a5bc35c3a44323bbe5.jpg) +*Coronal interictal F-18 FDG PET in a 15-year-old with focal left temporal seizures on EEG and findings of left hippocampal sclerosis on MR shows diffuse hypometabolism of the left temporal lobe , indicative of a seizure focus. Interpretation of functional brain imaging is aided by the fact that perfusion (SPECT) and metabolism (F-18 FDG) should be symmetric between the cerebral hemispheres and between the cerebellar hemispheres.* + +![Coronal FLAIR MR in a 16-year-old boy with intractable epilepsy shows an area of gliosis in the left parietal lobe related to remote trauma .](images/app.statdx.com_image_thumbnail_6a3b4a91-335c-4bd8-a946-b53291c24e5c_annotated_true_size_900_quality_90_8bb2173efa6473e1f78004c31d4ed6c3a907d838.jpg) +*Coronal FLAIR MR in a 16-year-old boy with intractable epilepsy shows an area of gliosis in the left parietal lobe related to remote trauma .* + +![Coronal F-18 FDG PET in the same patient shows hypometabolism corresponding to the left parietal gliosis , as well as hypometabolism (relative to the right) of adjacent parietal lobe . This corroborates SPECT findings and suggests a seizure focus.](images/app.statdx.com_image_thumbnail_cd215a5b-338e-4515-b87a-dcb2d92cca02_annotated_true_size_900_quality_90_e6fb8940fcfdce6f4c5436e4c612864b09081752.jpg) +*Coronal F-18 FDG PET in the same patient shows hypometabolism corresponding to the left parietal gliosis , as well as hypometabolism (relative to the right) of adjacent parietal lobe . This corroborates SPECT findings and suggests a seizure focus.* + +![Interictal Tc-99m ECD SPECT in the same patient shows hypoperfusion corresponding to the left parietal gliosis and involving the adjacent parietal lobe .](images/app.statdx.com_image_thumbnail_09fc0ae0-526b-46da-aa9a-aacfbea5c6e8_annotated_true_size_900_quality_90_7c3a6926a7cafde200b33ad804d7b5117319f1a4.jpg) +*Interictal Tc-99m ECD SPECT in the same patient shows hypoperfusion corresponding to the left parietal gliosis and involving the adjacent parietal lobe .* + +![Ictal Tc-99m ECD SPECT in the same patient shows hypoperfusion corresponding to the left parietal gliosis . The adjacent parietal lobe , however, is hyperperfused (relative to the right), reflecting seizure activity.](images/app.statdx.com_image_thumbnail_74f6d773-b5c4-4a50-9f78-05c00282edb2_annotated_true_size_900_quality_90_76a344ecdd6ccd7080289cc6b7b3a58f669953ae.jpg) +*Ictal Tc-99m ECD SPECT in the same patient shows hypoperfusion corresponding to the left parietal gliosis . The adjacent parietal lobe , however, is hyperperfused (relative to the right), reflecting seizure activity.* + +![Sagittal T1 C+ MR in a 17-year-old girl shows a frontal dysembryoplastic neuroepithelial tumor (DNET) as a nonenhancing, low-signal lesion .](images/app.statdx.com_image_thumbnail_2e122b37-46f0-472e-a108-6a07a37a69d2_annotated_true_size_900_quality_90_b18c5b7f062527fedc0348c045855d5b423465e9.jpg) +*Sagittal T1 C+ MR in a 17-year-old girl shows a frontal dysembryoplastic neuroepithelial tumor (DNET) as a nonenhancing, low-signal lesion .* + +![Sagittal F-18 FDG PET in the same patient shows focal hypometabolism associated with the DNET . In this case, the hypometabolism is reflective of both the low grade of this tumor and its epileptogenicity.](images/app.statdx.com_image_thumbnail_fa2a6270-345c-4cde-be09-f4e04b440fab_annotated_true_size_900_quality_90_639fdb63b2f62f548a4ce82582da4d057eae538e.jpg) +*Sagittal F-18 FDG PET in the same patient shows focal hypometabolism associated with the DNET . In this case, the hypometabolism is reflective of both the low grade of this tumor and its epileptogenicity.* + +![Axial T2 MR in a 12-year-old girl with epilepsy shows a right temporal focal cortical dysplasia (FCD), apparent as cortical and subcortical increased signal .](images/app.statdx.com_image_thumbnail_65677312-02de-4c9c-8b0f-a409a07f0b94_annotated_true_size_900_quality_90_ae262a44e7d83202dcd34e370f6f571489ecbe6d.jpg) +*Axial T2 MR in a 12-year-old girl with epilepsy shows a right temporal focal cortical dysplasia (FCD), apparent as cortical and subcortical increased signal .* + +![Axial F-18 FDG PET in the same patient shows hypometabolism associated with the FCD , supporting the epileptogenic nature of this lesion.](images/app.statdx.com_image_thumbnail_1621787d-7212-4cda-a23e-33a623675ef2_annotated_true_size_900_quality_90_9f7782dbb96148790de8d6ffadd589cdb230064a.jpg) +*Axial F-18 FDG PET in the same patient shows hypometabolism associated with the FCD , supporting the epileptogenic nature of this lesion.* + +![Coronal F-18 FDG PET in the same patient shows global hypometabolism of the right temporal lobe with more conspicuous hypometabolism of the hippocampus . These findings support a right temporal localization of the seizure focus.](201da07a-12c7-4b99-9259-3fc7741fed9a) +*Coronal F-18 FDG PET in the same patient shows global hypometabolism of the right temporal lobe with more conspicuous hypometabolism of the hippocampus . These findings support a right temporal localization of the seizure focus.* + +![Interictal Tc-99m ECD SPECT in a 2-year-old with intractable epilepsy shows enlargement of the left lateral ventricle and hypoperfusion of the left temporal lobe .](98fe500b-cfc6-4a9c-8906-6434df4d3c19) +*Interictal Tc-99m ECD SPECT in a 2-year-old with intractable epilepsy shows enlargement of the left lateral ventricle and hypoperfusion of the left temporal lobe .* + +![Ictal Tc-99m ECD SPECT in the same patient shows an area of relative hyperperfusion in the overall hypoperfused left temporal lobe, suggesting a seizure focus.](6673c88d-d29f-470b-ac79-11c87d1a8101) +*Ictal Tc-99m ECD SPECT in the same patient shows an area of relative hyperperfusion in the overall hypoperfused left temporal lobe, suggesting a seizure focus.* + +![Coronal SISCOM derived from the interictal and ictal SPECT and T1 MR in the same patient confirms an area of abnormal perfusion in the left temporal lobe , suggestive of a seizure focus. The left lateral ventricle is dilated due to extensive white matter loss.](287d206c-497b-4640-b03c-0c7c2fbf3dc2) +*Coronal SISCOM derived from the interictal and ictal SPECT and T1 MR in the same patient confirms an area of abnormal perfusion in the left temporal lobe , suggestive of a seizure focus. The left lateral ventricle is dilated due to extensive white matter loss.* + +![Axial FLAIR MR in a 12-year-old with tuberous sclerosis and increasing seizure frequency shows multiple cortical tubers .](75b14cfd-4c19-49c2-b9d5-1f3ed089636f) +*Axial FLAIR MR in a 12-year-old with tuberous sclerosis and increasing seizure frequency shows multiple cortical tubers .* + +![Axial interictal F-18 FDG PET in the same patient at the same level shows hypometabolism associated with the cortical tubers .](ee18dcf5-9b6d-4072-abfd-4a4ceaedab73) +*Axial interictal F-18 FDG PET in the same patient at the same level shows hypometabolism associated with the cortical tubers .* + +![Axial interictal Tc-99m ECD SPECT in the same patient shows hypoperfusion corresponding to the cortical tubers .](e7b23250-8786-449a-b649-874dc5c5c64a) +*Axial interictal Tc-99m ECD SPECT in the same patient shows hypoperfusion corresponding to the cortical tubers .* + +![Fused axial F-18 FDG PET and FLAIR MR in the same patient shows correspondence between areas of hypometabolism and the cortical tubers . Hypometabolism is similar in extent to the tubers. Areas of hypometabolism larger than underlying lesions can suggest an epileptogenic focus.](e27efde4-c24d-4f2a-97fb-d7b9708fb5f3) +*Fused axial F-18 FDG PET and FLAIR MR in the same patient shows correspondence between areas of hypometabolism and the cortical tubers . Hypometabolism is similar in extent to the tubers. Areas of hypometabolism larger than underlying lesions can suggest an epileptogenic focus.* + +![Coronal interictal F-18 FDG PET in a 7-year-old with intractable seizures shows hypometabolism in the right temporal lobe , particularly mesially in the hippocampal formation .](d4c77d8f-18b9-408b-ac3f-1224dc3873d0) +*Coronal interictal F-18 FDG PET in a 7-year-old with intractable seizures shows hypometabolism in the right temporal lobe , particularly mesially in the hippocampal formation .* + +![Coronal T2 FLAIR MR at the same level in the same patient shows abnormally increased signal in the right hippocampal formation in this child with mesial temporal sclerosis.](ca966ce9-e4ef-4a8c-9b2e-aa78be48ce80) +*Coronal T2 FLAIR MR at the same level in the same patient shows abnormally increased signal in the right hippocampal formation in this child with mesial temporal sclerosis.* + + +### Additional Images + +![Fused FDG PET MR in a 9-year-old boy with left lower extremity clonic movements shows a focal region of hypometabolism within the right paracentral lobule (left-foot motor control).](837f2b5c-2192-4552-8870-9e34f866a47b) +*Fused FDG PET MR in a 9-year-old boy with left lower extremity clonic movements shows a focal region of hypometabolism within the right paracentral lobule (left-foot motor control).* + +![FDG PET MR postprocessing demonstrates same area of hypometabolism localized within the right paracentral lobule.](35c63796-56f8-44ee-a99f-925c9f46b830) +*FDG PET MR postprocessing demonstrates same area of hypometabolism localized within the right paracentral lobule.* + +![Coronal T2 MR in the same patient shows focal area of blurring of the gray matter-white matter junction and abnormal gyration in the right paracentral lobule, suggesting FCD (initially missed on MR).](8ff53b4c-7616-4888-a02b-7a896fdb346d) +*Coronal T2 MR in the same patient shows focal area of blurring of the gray matter-white matter junction and abnormal gyration in the right paracentral lobule, suggesting FCD (initially missed on MR).* + +![Left ankle flexion fMRI activated the right superior frontal gyrus anterior to the expected location of the left paracentral lobule, suggesting motor remapping distant to FCD . Normal right foot motor activation centered about the left paracentral lobule .](8d7816c2-2143-4ab2-bf18-dc0e6634feaf) +*Left ankle flexion fMRI activated the right superior frontal gyrus anterior to the expected location of the left paracentral lobule, suggesting motor remapping distant to FCD . Normal right foot motor activation centered about the left paracentral lobule .* + diff --git a/docs_md/articles/posterior-fossa-neoplasm-adult_9d6bcceb-36a2-4f49-a5b2-1dd076541be8.md b/docs_md/articles/posterior-fossa-neoplasm-adult_9d6bcceb-36a2-4f49-a5b2-1dd076541be8.md new file mode 100644 index 0000000..aee2d1b --- /dev/null +++ b/docs_md/articles/posterior-fossa-neoplasm-adult_9d6bcceb-36a2-4f49-a5b2-1dd076541be8.md @@ -0,0 +1,314 @@ +--- +title: "Posterior Fossa Neoplasm, Adult" +docid: "9d6bcceb-36a2-4f49-a5b2-1dd076541be8" +authors: + - key: "26ebc2e8-e4f7-40ee-8f5c-d23fe383e15c" + value: "Yoshimi Anzai, MD, MPH" + - key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850" + value: "Anne G. Osborn, MD, FACR" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c" + - + name: "CPA-IAC and Posterior Fossa" + slug: "cpa-iac-and-posterior-fossa" + treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992" + - + name: "Anatomically Based Differentials" + slug: "anatomically-based-differentials" + treeNodeId: "debfb06c-8656-4f5d-92c1-eaa468185d78" + - + name: "Posterior Fossa Neoplasm, Adult" + slug: "posterior-fossa-neoplasm-adult" + treeNodeId: null +category: "Head and Neck" +documentVersionId: "8071b0df-9c2d-4d93-8775-a2b6a68bf9c8" +imageCount: 22 +lastUpdated: "08/06/18" +pageDescription: "Posterior Fossa Neoplasm, Adult" +pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Anatomically Based Differentials, Posterior Fossa Neoplasm, Adult" +pageTitle: "Posterior Fossa Neoplasm, Adult | STATdx" +enhancedTitle: "Posterior Fossa Neoplasm, Adult" +type: "DDX" +references: true +breadcrumbs: + - "Head and Neck" + - "Differential Diagnosis" + - "CPA-IAC and Posterior Fossa" + - "Anatomically Based Differentials" + - "Posterior Fossa Neoplasm, Adult" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - With exception of vestibular schwannoma, posterior fossa (PF) neoplasms rare in adults + - Most important question: Is lesion intra- or extraaxial + - **Extraaxial PF lesions** + - Most adult PF neoplasms are extraaxial + - By far, most common is **vestibular schwannoma** + - Meningioma > metastasis > other schwannomas > glomus jugulare paraganglioma + - **Intraaxial: Parenchymal or intraventricular** + - Adult parenchymal neoplasms all uncommon/rare + - Overall, most common by far is metastasis + - Hemangioblastoma most common primary + - Glioblastoma, astrocytomas, most common adult supratentorial tumors are rare in PF + - 4th ventricle + - Subependymoma > ependymoma > choroid plexus papilloma + - Number 1 site for ependymoma & subependymoma + - Ependymoma (usually in children) often extends into foramen of Luschka &/or Magendie + - Choroid plexus papilloma in body/lateral recess, cerebellopontine angle (CPA) +- ## Helpful Clues for Common Diagnoses + + + - **Vestibular Schwannoma** + - By far most common adult PF neoplasm + - All others less common or rare + - 90% of all CPA-IAC masses + - Imaging findings + - Looks like "ice cream on cone" (CPA-IAC) + - Enhances strongly + - ± intra- or extratumoral cysts + - Preserved fluid at IAC fundus is predictive of improved hearing preservation after resection +- ## Helpful Clues for Less Common Diagnoses + + + - **Meningioma****in****Cerebellopontine Angle** + - Imaging findings + - Mushroom-shaped mass caps IAC + - Flat base towards dural surface + - ± hyperostosis, dural tail sign + - 25% show IAC involvement + - **Metastases****in****Cerebellopontine Angle** + - CPA metastases can arise in 4 locations + - Dura-arachnoid + - Cranial nerves (VII, VIII most common) + - Flocculusmimi + - Choroid plexus (foramen of Luschka) + - Imaging findings + - Irregular, invasive margins + - Solid, enhancing tumor on dura, cranial nerve surfaces, in flocculus or choroid plexus + - **Metastasi****s****in Brainstem or Cerebellum** + - 2nd most common adult PF neoplasm + - Most common adult parenchymal PF tumor + - Rarely may be only brain metastasis + - Enhancing solid or cystic mass with adjacent brain edema + - **Hemangioblastoma** + - 95% are located in PF + - Hemispheres > > vermis > brainstem, 4th ventricle + - < 50% of patients have von Hippel-Lindau syndrome + - Look for multiple lesions: Spinal mass, visceral cysts + - Imaging findings + - 60% nonenhancing cyst with avidly enhancing mural nodule + - Large drainage vein, ± blood products + - **Other Schwannomas** + - **Trigeminal (CNV)****S****chwannoma** + - Upper CPA mass + - Look for dumbbell shape (CPA + Meckel cave components) + - **Facial****N****erve (CNVII) Schwannoma** + - CPA-IAC mass with labyrinthine tail + - Look for CNVII labyrinthine segment tumor + - If labyrinthine tail absent, mimics vestibular schwannoma + - **Jugular Foramen****Schwannoma** + - Enhancing mass arising from jugular foramen + - Smooth remodeling of bony margins + - Projects cephalad into CPA cistern + - **Hypoglossal (CNXII) Schwannoma** + - Enhancing mass + - Smooth remodeling or widening of hypoglossal canal + - Look for ipsilateral tongue atrophy + - **Subependymoma** + - Middle-aged/elderly adult; asymptomatic most often + - Imaging: T2 hyperintense lobulated intraventricular mass + - 4th ventricle (60%) > other locations + - ± cysts, calcifications, rare intramural hemorrhage + - **Choroid Plexus Papilloma** + - 40% occur in 4th ventricle or CPA cistern in adults + - Imaging findings + - Cauliflower- or frond-like excrescences + - Intense, relatively uniform enhancement +- ## Helpful Clues for Rare Diagnoses + + + - **Astrocytomas** + - **Glioblastoma** + - Infratentorial glioblastomas (GBM) rare + - Typically necrotic, ring enhancing + - **Anaplastic Astrocytoma** + - Rare in infratentorial area + - Infiltrative, variable enhancement + - **Low-****Grade Diffuse Astrocytoma** + - Young adults + - Focal or diffuse T2-hyperintense white matter mass, usually nonenhancing + - Enhancement suggests conversion to higher grade + - **Pilocytic Astrocytoma** + - Rare in adults + - Cystic mass with enhancing nodule typical + - **G****lomus Jugulare****Paraganglioma** + - Spreads superolaterally into middle ear much more frequently than superomedially into CPA + - Imaging findings + - Intense heterogeneous enhancement + - Look for salt and pepper flow voids + - Permeative; destructive, infiltrative + - **Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos)** + - May be familial or sporadic + - Molecular studies suggest high frequency of alterations in PTEN-AKT-mTOR pathway + - Association with Cowden syndrome + - Autosomal dominant; germline mutation in *PTEN*tumor suppressor gene + - ↑ risk of breast, thyroid, genitourinary malignancy, & mucocutaneous lesions + - Imaging findings + - Widened, irregular cerebellar folia with ↑ T2 + - Layered/laminated or tiger stripes appearance + - May cause significant mass effect + - Typically does not enhance (rarely may) + - **Medulloblastoma (Desmoplastic Variant)** + - Desmoplastic variant more common in 2nd-3rd decades + - Imaging findings + - Off-midline (lateral cerebellar hemisphere) location + - Enhances; cerebrospinal fluid spread uncommon + - **Hemangiopericytoma** + - Highly cellular & vascularized mesenchymal tumor mimics meningioma + - Imaging findings + - Lobular, avidly enhancing, extraaxial mass with dural attachment; ± skull erosion + - Mimics meningioma, but no calcifications or hyperostosis; often heterogeneous + - Commonly involve falx, tentorium, or dural sinuses; occipital most common + - Dural tail seen in ~ 50% + - **Ecchordosis Physaliphora** + - Small, gelatinous mass considered ectopic notochordal remnant + - Midline of craniospinal axis from dorsum sellae to sacrococcygeal region + - Erosion dorsal clivus to PF + - Found in 2% of autopsies, typically asymptomatic + - Imaging findings + - Hypointense on T1, hyperintense on T2 + - No enhancement + - ± stalk-like connection to mass + +## References + +# Selected References + +1. [Moinuddin FM et al: Bilateral lateral ventricular subependymoma with extensive multiplicity presenting with hemorrhage. Neuroradiol J. 31(1):27-31, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=28696174%5Bpmid%5D) +1. [Shrot S et al: Dysplasia and overgrowth: magnetic resonance imaging of pediatric brain abnormalities secondary to alterations in the mechanistic target of rapamycin pathway. Neuroradiology. 60(2):137-150, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29279945%5Bpmid%5D) +1. [Jiang T et al: Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) and Cowden syndrome: clinical experience from a single institution with long-term follow-up. World Neurosurg. 104:398-406, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28479525%5Bpmid%5D) +1. [Ciccarino P et al: Multifocal presentation of medulloblastoma in adulthood. J Neurooncol. 107(2):233-7, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22071791%5Bpmid%5D) +1. [Solis OE et al: Rosette-forming glioneuronal tumor: a pineal region case with IDH1 and IDH2 mutation analyses and literature review of 43 cases. J Neurooncol. 102(3):477-84, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=20872044%5Bpmid%5D) +1. [Goddard JC et al: Fundal fluid as a predictor of hearing preservation in the middle cranial fossa approach for vestibular schwannoma. Otol Neurotol. 31(7):1128-34, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20657334%5Bpmid%5D) +1. [Koyfman SA et al: Stereotactic radiosurgery for single brainstem metastases: the cleveland clinic experience. Int J Radiat Oncol Biol Phys. 78(2):409-14, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20133072%5Bpmid%5D) +1. [Andres RH et al: Lhermitte-Duclos disease with atypical vascularization--case report and review of the literature. Clin Neuropathol. 28(2):83-90, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19353838%5Bpmid%5D) +1. [Bonneville F et al: Imaging of cerebellopontine angle lesions: an update. Part 1: enhancing extra-axial lesions. Eur Radiol. 17(10):2472-82, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17562049%5Bpmid%5D) +1. [Bonneville F et al: Imaging of cerebellopontine angle lesions: an update. Part 2: intra-axial lesions, skull base lesions that may invade the CPA region, and non-enhancing extra-axial lesions. Eur Radiol. 17(11):2908-20, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17569053%5Bpmid%5D) +1. [Recinos PF et al: Brainstem tumors: where are we today? Pediatr Neurosurg. 43(3):192-201, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17409788%5Bpmid%5D) +1. [Koeller KK et al: From the archives of the AFIP: medulloblastoma: a comprehensive review with radiologic-pathologic correlation. Radiographics. 23(6):1613-37, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14615567%5Bpmid%5D) +1. [Zabek M: Primary posterior fossa tumours in adult patients. Folia Neuropathol. 41(4):231-6, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14977253%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T1 C+ MR shows a partly cystic and solid, heterogeneously enhancing, vestibular schwannoma involving the right cerebellopontine angle cistern and extending into the internal auditory canal .](images/app.statdx.com_image_thumbnail_df62b1b1-e2f2-4f73-8cb3-009ad02b8459_annotated_true_size_900_quality_90_72e555ee76ab95be5f3dc1dafc3b9f3573533a66.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ MR shows a partly cystic and solid, heterogeneously enhancing, vestibular schwannoma involving the right cerebellopontine angle cistern and extending into the internal auditory canal .* + +![Axial T1 C+ MR shows a partly cystic and solid, heterogeneously enhancing, vestibular schwannoma involving the right cerebellopontine angle cistern and extending into the internal auditory canal .](images/app.statdx.com_image_thumbnail_df62b1b1-e2f2-4f73-8cb3-009ad02b8459_size_174_quality_85_5b57047077ec48b97860b50e99ea134709bca401.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ MR shows a partly cystic and solid, heterogeneously enhancing, vestibular schwannoma involving the right cerebellopontine angle cistern and extending into the internal auditory canal .* + +![Axial T1 C+ MR shows a partly cystic and solid, heterogeneously enhancing, vestibular schwannoma involving the right cerebellopontine angle cistern and extending into the internal auditory canal .](images/app.statdx.com_image_thumbnail_df62b1b1-e2f2-4f73-8cb3-009ad02b8459_size_174_quality_85_5f47f585.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ MR shows a partly cystic and solid, heterogeneously enhancing, vestibular schwannoma involving the right cerebellopontine angle cistern and extending into the internal auditory canal .* + +![Axial T1 C+ MR shows a large, mushroom-shaped, enhancing mass in the right cerebellopontine angle cistern. The mass has a broad base toward the dural surface. Note the dural tail sign of reactive meningeal thickening in the internal auditory canal.](images/app.statdx.com_image_thumbnail_b79aca2e-1ac7-4379-a472-983bd7113c88_annotated_true_size_900_quality_90_0e416a56e6e47f3ef2a115826374038901501872.jpg) +**Meningioma in Cerebellopontine Angle** +*Axial T1 C+ MR shows a large, mushroom-shaped, enhancing mass in the right cerebellopontine angle cistern. The mass has a broad base toward the dural surface. Note the dural tail sign of reactive meningeal thickening in the internal auditory canal.* + +![Axial T2 MR in a female patient with breast carcinoma shows a lobulated extraaxial mass in the right flocculus with associated parenchymal edema . Note the normal flocculus on the left .](images/app.statdx.com_image_thumbnail_cb8dde9b-4465-4985-8e7e-e0f154940b9c_annotated_true_size_900_quality_90_33890117e9033b5c0ad5c9407505f0bc7062f169.jpg) +**Metastases in Cerebellopontine Angle** +*Axial T2 MR in a female patient with breast carcinoma shows a lobulated extraaxial mass in the right flocculus with associated parenchymal edema . Note the normal flocculus on the left .* + +![Coronal T1 C+ MR demonstrates a partly cystic, partly solid enhancing mass in the left cerebellar hemisphere with surrounding vasogenic edema. A 2nd enhancing mass in the left frontal lobe increases suspicion for metastatic disease in this patient with lung cancer (confirmed histologically).](images/app.statdx.com_image_thumbnail_39795a57-664a-4ae1-84d3-0cd121ce30d7_annotated_true_size_900_quality_90_1fcdda480dd65ebc0f301b81b48719e567db4010.jpg) +**Metastasis in Brainstem or Cerebellum** +*Coronal T1 C+ MR demonstrates a partly cystic, partly solid enhancing mass in the left cerebellar hemisphere with surrounding vasogenic edema. A 2nd enhancing mass in the left frontal lobe increases suspicion for metastatic disease in this patient with lung cancer (confirmed histologically).* + +![Axial T1 C+ MR in a patient with von Hippel-Lindau syndrome shows a cystic cerebellar mass with a nodule of enhancement , typical of hemangioblastoma. Retinal angiomas and an additional posterior fossa hemangioblastoma were also present (not shown).](images/app.statdx.com_image_thumbnail_a4aefad2-167d-4d84-92a2-b53f330d1eef_annotated_true_size_900_quality_90_202995e4dd1accadb9574577e42669c9b2d48f37.jpg) +**Hemangioblastoma** +*Axial T1 C+ MR in a patient with von Hippel-Lindau syndrome shows a cystic cerebellar mass with a nodule of enhancement , typical of hemangioblastoma. Retinal angiomas and an additional posterior fossa hemangioblastoma were also present (not shown).* + +![Axial T1 C+ MR shows a large, solid, intensely enhancing, extraaxial mass extending into the enlarged, smoothly remodeled jugular foramen . Intratumoral cysts, not present here, are common in posterior fossa schwannomas.](5fc85b39-363e-4eb2-91bc-495a0431341f) +**Jugular Foramen Schwannoma** +*Axial T1 C+ MR shows a large, solid, intensely enhancing, extraaxial mass extending into the enlarged, smoothly remodeled jugular foramen . Intratumoral cysts, not present here, are common in posterior fossa schwannomas.* + +![Axial T2 MR shows an unusually large lateral ventricle subependymoma causing obstructive hydrocephalus. Note the heterogeneous internal signal intensity. No enhancement was seen (not shown), typical of subependymomas. Subependymomas are more often small, incidentally discovered masses.](e6e5c2fe-fb1f-4fa6-a060-13e5c16a0e9c) +**Subependymoma** +*Axial T2 MR shows an unusually large lateral ventricle subependymoma causing obstructive hydrocephalus. Note the heterogeneous internal signal intensity. No enhancement was seen (not shown), typical of subependymomas. Subependymomas are more often small, incidentally discovered masses.* + +![Coronal T1 C+ MR in a 43-year-old woman with headaches shows a "speckled" or "bubbly," heterogeneously enhancing mass in the 4th ventricle with extension into the lateral recess .](de8fbb57-970f-4b74-8a01-79ccadd3ae77) +**Choroid Plexus Papilloma** +*Coronal T1 C+ MR in a 43-year-old woman with headaches shows a "speckled" or "bubbly," heterogeneously enhancing mass in the 4th ventricle with extension into the lateral recess .* + +![Axial T1 C+ MR in an older teenager with nausea and vomiting shows an inhomogeneously enhancing vermian mass with cystic and solid components. Preoperative diagnosis was malignant astrocytoma. However, a WHO grade II tumor was found at biopsy.](6ac3d887-59b2-47cf-9b67-c85d194d5a62) +**Astrocytomas** +*Axial T1 C+ MR in an older teenager with nausea and vomiting shows an inhomogeneously enhancing vermian mass with cystic and solid components. Preoperative diagnosis was malignant astrocytoma. However, a WHO grade II tumor was found at biopsy.* + +![Sagittal T2 MR in a 25-year-old woman with lower cranial nerve palsies shows a dorsally exophytic pontomedullary mass . Biopsy proved WHO grade II astrocytoma.](f43c0ab0-2ace-43f9-b54a-2b76a3ffb10d) +**Low-Grade Diffuse Astrocytoma** +*Sagittal T2 MR in a 25-year-old woman with lower cranial nerve palsies shows a dorsally exophytic pontomedullary mass . Biopsy proved WHO grade II astrocytoma.* + +![Axial T2 MR shows enlargement and hyperintensity involving the left cerebellar hemisphere . Note the classic tiger stripes appearance with overall preserved cerebellar foliar architecture.](c3651341-a464-4fb8-8beb-10f5a306514d) +**Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos)** +*Axial T2 MR shows enlargement and hyperintensity involving the left cerebellar hemisphere . Note the classic tiger stripes appearance with overall preserved cerebellar foliar architecture.* + +![Axial T2 MR in a 26-year-old man shows an inhomogeneously hyperintense mass in the lateral cerebellum . The mass enhanced heterogeneously (not shown). This is typical of desmoplastic medulloblastoma, although an atypical teratoid rhabdoid tumor could be considered in a child.](161da058-48b3-4cf9-a112-d19b44dc8f98) +**Medulloblastoma (Desmoplastic Variant)** +*Axial T2 MR in a 26-year-old man shows an inhomogeneously hyperintense mass in the lateral cerebellum . The mass enhanced heterogeneously (not shown). This is typical of desmoplastic medulloblastoma, although an atypical teratoid rhabdoid tumor could be considered in a child.* + +![Axial T1 C+ MR shows a large, inhomogeneously enhancing, destructive, transcalvarial mass with both intracranial and extracranial components.](d5c83b0f-e7b6-4a26-a580-379ccbbce549) +**Hemangiopericytoma** +*Axial T1 C+ MR shows a large, inhomogeneously enhancing, destructive, transcalvarial mass with both intracranial and extracranial components.* + +![Sagittal T1 MR shows a midline mass in front of and indenting the pons . Note the slightly increased signal of the mass relative to CSF. There is loss of a normal hypointense cortical margin in the clivus from which the mass originates. The mass was extremely hyperintense on T2 MR, consistent with its notochordal remnant origin.](4876753d-7d12-445e-8d99-e500a14e6bbc) +**Ecchordosis Physaliphora** +*Sagittal T1 MR shows a midline mass in front of and indenting the pons . Note the slightly increased signal of the mass relative to CSF. There is loss of a normal hypointense cortical margin in the clivus from which the mass originates. The mass was extremely hyperintense on T2 MR, consistent with its notochordal remnant origin.* + + +### Additional Images + +![Axial T1 C+ MR in a patient with adenocarcinoma shows enhancing metastatic lesions in both IAC cisterns . Note the transmodiolar extension on left .](images/app.statdx.com_image_thumbnail_8704e442-24fe-4cce-a794-8b1f21744c93_annotated_true_size_900_quality_90_97e7ce3f1227e45a08998e34fa1f993eea69ef28.jpg) +**Metastases in Cerebellopontine Angle** +*Axial T1 C+ MR in a patient with adenocarcinoma shows enhancing metastatic lesions in both IAC cisterns . Note the transmodiolar extension on left .* + +![Axial T1 C+ FS MR shows a large, enhancing, cerebellar mass . A 2nd mass in the left temporal lobe makes a diagnosis of metastases in a patient with known renal cell carcinoma easy.](images/app.statdx.com_image_thumbnail_d4e30b61-58dd-40e1-8d3d-497bd5f41286_annotated_true_size_900_quality_90_154db9b9399651243d8146d3c23564895bd3c729.jpg) +**Metastasis in Brainstem or Cerebellum** +*Axial T1 C+ FS MR shows a large, enhancing, cerebellar mass . A 2nd mass in the left temporal lobe makes a diagnosis of metastases in a patient with known renal cell carcinoma easy.* + +![Axial T1 C+ FS MR shows a mixed, solid, cystic enhancing mass in the cerebellum . Note the enlarged vessels , suggesting hypervascularity. A solid/mixed solid lesion with intratumoral cysts is less common than classic hemangioblastoma (a nodule and nonenhancing cyst).](images/app.statdx.com_image_thumbnail_05d7dc7b-60d7-4ddb-a400-284319997022_annotated_true_size_900_quality_90_b9012a4622bba3af21c707321d6a3c13cba641e8.jpg) +**Hemangioblastoma** +*Axial T1 C+ FS MR shows a mixed, solid, cystic enhancing mass in the cerebellum . Note the enlarged vessels , suggesting hypervascularity. A solid/mixed solid lesion with intratumoral cysts is less common than classic hemangioblastoma (a nodule and nonenhancing cyst).* + +![Sagittal T2 MR shows a small, mildly hyperintense mass in the inferior 4th ventricle, found incidentally in this 43-year-old man with headache and trigeminal neuralgia. No hydrocephalus was identified. Diagnosis was presumed subependymoma.](aa6dbef4-b6c3-40d3-b9e8-c4340df7a575) +**Subependymoma** +*Sagittal T2 MR shows a small, mildly hyperintense mass in the inferior 4th ventricle, found incidentally in this 43-year-old man with headache and trigeminal neuralgia. No hydrocephalus was identified. Diagnosis was presumed subependymoma.* + +![Axial T1 C+ FS MR shows a large, strongly enhancing, glomus jugulare paraganglioma centered in the jugular foramen. The destructive, erosive nature of the tumor is readily apparent with extension into the EAC .](e393ec84-9471-47dc-894f-ea1fff84ae1d) +**Glomus Jugulare Paraganglioma** +*Axial T1 C+ FS MR shows a large, strongly enhancing, glomus jugulare paraganglioma centered in the jugular foramen. The destructive, erosive nature of the tumor is readily apparent with extension into the EAC .* + +![Coronal T2 MR shows enlarged, dysplastic-appearing cerebellar folia with a striated, mixed hyper-/isointense mass in the right cerebellum .](3f19e559-5acb-4cbd-9967-e33fc96d4eb2) +**Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos)** +*Coronal T2 MR shows enlarged, dysplastic-appearing cerebellar folia with a striated, mixed hyper-/isointense mass in the right cerebellum .* + +![Axial T1 C+ MR in a 50-year-old man with supratentorial primary CNS lymphoma shows a subependymal/choroid plexus as well as dural-based spread of the tumor.](2f36edbc-0657-469a-b965-570d7920bffd) +*Axial T1 C+ MR in a 50-year-old man with supratentorial primary CNS lymphoma shows a subependymal/choroid plexus as well as dural-based spread of the tumor.* + +![Axial T2 FS MR shows a lobulated, hyperintense, extraaxial mass indenting the pons. Note the subtle clival involvement with focal destruction of a bony cortex typical of ecchordosis physaliphora.](f7d0a1ad-8adc-4d87-91f3-e8fc20e8f62b) +**Ecchordosis Physaliphora** +*Axial T2 FS MR shows a lobulated, hyperintense, extraaxial mass indenting the pons. Note the subtle clival involvement with focal destruction of a bony cortex typical of ecchordosis physaliphora.* + diff --git a/docs_md/articles/posterior-fossa-neoplasm-pediatric_22c55144-5db6-4235-9292-de3dd1315dd2.md b/docs_md/articles/posterior-fossa-neoplasm-pediatric_22c55144-5db6-4235-9292-de3dd1315dd2.md new file mode 100644 index 0000000..1a7a798 --- /dev/null +++ b/docs_md/articles/posterior-fossa-neoplasm-pediatric_22c55144-5db6-4235-9292-de3dd1315dd2.md @@ -0,0 +1,438 @@ +--- +title: "Posterior Fossa Neoplasm, Pediatric" +docid: "22c55144-5db6-4235-9292-de3dd1315dd2" +authors: + - key: "f184750a-90b4-47a7-907b-23b05d70357a" + value: "Chang Yueh Ho, MD" + - key: "e8af6d26-3aad-47c9-9083-5128aab09af2" + value: "Susan I. Blaser, MD, FRCPC" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c" + - + name: "CPA-IAC and Posterior Fossa" + slug: "cpa-iac-and-posterior-fossa" + treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992" + - + name: "Anatomically Based Differentials" + slug: "anatomically-based-differentials" + treeNodeId: "debfb06c-8656-4f5d-92c1-eaa468185d78" + - + name: "Posterior Fossa Neoplasm, Pediatric" + slug: "posterior-fossa-neoplasm-pediatric" + treeNodeId: null +category: "Head and Neck" +documentVersionId: "f9182a7b-2287-4827-8cc9-fd986d37f943" +imageCount: 57 +lastUpdated: "08/20/18" +pageDescription: "Posterior Fossa Neoplasm, Pediatric" +pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Anatomically Based Differentials, Posterior Fossa Neoplasm, Pediatric" +pageTitle: "Posterior Fossa Neoplasm, Pediatric | STATdx" +enhancedTitle: "Posterior Fossa Neoplasm, Pediatric" +type: "DDX" +references: true +breadcrumbs: + - "Head and Neck" + - "Differential Diagnosis" + - "CPA-IAC and Posterior Fossa" + - "Anatomically Based Differentials" + - "Posterior Fossa Neoplasm, Pediatric" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Unlike in adults, primary neoplasm is more common than metastases in children + - Most common pediatric posterior fossa neoplasms + - Astrocytomas: Pilocytic astrocytoma (PA), infiltrating glioma (astrocytoma, WHO grade II) + - Medulloblastoma + - Ependymoma + - Imaging + - Location helpful in differential diagnosis + - Tectum, cerebellum: PA + - Pons: Diffusely infiltrating astrocytomas + - Midline (vermis, 4th ventricle): Medulloblastoma, PA + - 4th ventricle + lateral recess/CPA mass: Ependymoma + - Diffusion-weighted imaging + - Can discriminate between high-grade & low-grade pediatric posterior fossa tumors + - Medulloblastoma & atypical teratoid-rhabdoid tumor (ATRT) show DWI restriction + - Examine entire neuraxis in child with posterior fossa tumor prior to surgery + - T1 C+ essential (look for CSF spread) +- ## Helpful Clues for Common Diagnoses + + + - **Pilocytic Astrocytoma** + - Child with cystic cerebellar hemispheric mass + enhancing mural nodule + - Solid component low-density NECT, high-signal T2 + - **Medulloblastoma** + - Most common malignant pediatric brain tumor + - Most common 4th ventricular neoplasm of childhood + - Arises from vermis & fills 4th ventricle + - Hydrocephalus common + - Hemispheric involvement is less common + - Hypercellular: ↑ density on NECT, ↓ T2 + - DWI: Decreased diffusion + - Previous classification based on histopathology: Desmoplastic/nodular; medulloblastoma with extensive nodularity; classic, large cell, & anaplastic variants + - Current classification with molecular subgroups predicts outcomes better + - Wingless (WNT): CPA predilection; uncommon in infants; good prognosis + - Associated with Turcot syndrome + - Sonic hedgehog (SHH): Cerebellar hemispheric location; good prognosis in infants; intermediate in older children & adults + - Associated with Gorlin syndrome + - Medulloblastoma with extensive nodularity & desmoplastic variants + - Group 3: 4th ventricle; ill-defined tumor margin; seen in infants & children; poor prognosis + - Group 4: 4th ventricle; minimal to no enhancement; intermediate prognosis seen in infants to adults + - **Ependymoma** + - Extrudes through 4th ventricle outlet foramina into cisterns + - Coarse calcifications + - Diffusion restriction uncommon, may predict anaplastic behavior + - **Brainstem Glioma, Pediatric** + - Exophytic brainstem tumors are typically low grade, PA, or diffuse astrocytoma + - Good outcome, may require chemotherapy or ventricular shunting for sequela of mass effect + - Tectal plate glioma + - NECT: Increased density progresses to Ca⁺⁺ + - CECT/MR: Faint or no enhancement + - Dorsal exophytic glioma + - Tumor protrudes into 4th ventricle + - If large, may be difficult to differentiate from PA + - Look for FLAIR signal change in dorsal brainstem or peduncles + - **Diffuse Intrinsic Pontine Glioma** + - Poor prognosis; near 100% mortality + - Expansile T2-hyperintense mass affecting at least 50% of central pons + - Enlarged pons engulfs basilar artery + - Enhances late in course, rarely at diagnosis + - Associated with histone mutations (H3-K27M) + - H3-K27M diffuse midline gliomas occur in pons, thalamus, & spinal cord + - H3-K27M midline gliomas have variable radiologic presentation, from nonenhancing, expansile T2 hyperintensity to central necrosis with peripheral enhancement +- ## Helpful Clues for Less Common Diagnoses + + + - **Ganglioglioma** + - Brainstem most common posterior fossa site + - Look for expansion of nucleus cuneatus/gracilis + - **Schwannoma** + - Contrast-enhancing masses along cranial nerves: Vestibular schwannoma (ICA/CPA) looks like ice cream on cone + - T2 hyperintensity helps differentiate from meningioma + - Multiple in neurofibromatosis type 2 (NF2) + - **Meningioma, CPA-IAC** + - Broad dural base, covers IAC + - Variable signal, but T2 hypointensity common + - Hyperostosis, tumoral calcifications + - May have intra- or juxtatumoral cyst(s) + - Uncommon in children + - Consider NF2 + - **Hemangioblastoma** + - Late teen or adult + - Intraaxial (cerebellum > medulla, cord) + - Cyst + nodule > solid + - Solid component shows flow voids, enhances avidly + - Multiple lesions diagnostic of von Hippel-Lindau (VHL) + - Avidly enhancing mural nodule abuts pia + - Look for visceral markers of VHL in any child/young adult with hemangioblastoma + - **Choroid Plexus Papilloma** + - Frond-like 4th ventricle or CPA tumor + - Avidly enhancing + - Hydrocephalus common +- ## Helpful Clues for Rare Diagnoses + + + - **Anaplastic Astrocytoma** + - Infiltrating mass involves predominantly white matter + - Enhancement none to sparse or patchy enhancement + - Ring enhancement suggests progression to glioblastoma + - **Atypical Teratoid-Rhabdoid Tumor** + - Imaging similar to medulloblastoma + - ATRT patients generally younger + - Cysts, hemorrhages more common + - CPA involvement more common + - Frequent metastases at diagnosis + - Both ATRT, medulloblastoma show diffusion restriction + - **Choroid Plexus Carcinoma** + - Similar to choroid plexus papilloma plus + - Cysts, necrosis, bleeds + - CSF/ependymal/parenchymal spread + - **Medulloblastoma Variants** + - Desmoplastic medulloblastoma + - 5-25% of all medulloblastomas + - 55-60% of medulloblastomas in children < 3 years old + - Associated with SHH subgroup + - Desmoplastic subtype of medulloblastoma in children < 2 is major diagnostic criterion for basal cell nevus syndrome (Gorlin syndrome) + - Nodular collections of neurocytic cells bounded by desmoplastic zones + - Lateral (cerebellar) location + - Medulloblastoma with extensive nodularity + - Formerly called cerebellar neuroblastoma + - Usually occurs in infants + - Gyriform or grape-like appearance + - May mature → better prognosis + - Associated with SHH subgroup + - **Medulloepithelioma** + - Rare embryonal brain &/or ocular tumor + - Heterogeneous signal, enhancement + - **Embryonal Tumor With Multilayered Rosettes** + - Aggressive primitive neuroectodermal tumor, subtype of CNS embryonal tumor + - Ependymoblastic rosettes + - Previously termed embryonal tumor with abundant neuropil & true rosettes + - Occurs < 4 years of age + - Large mass with heterogeneous contrast enhancement + - Can occur both supra- & infratentorially + - **Dysplastic Cerebellar Gangliocytoma** + - Diffuse or focal hemispheric mass + - Thick cerebellar folia with striated appearance + - Evaluate for Cowden syndrome + +## References + +# Selected References + +1. [Johnson DR et al: 2016 updates to the WHO brain tumor classification system: what the radiologist needs to know. Radiographics. 37(7):2164-2180, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29028423%5Bpmid%5D) +1. [Vijapura C et al: Genetic syndromes associated with central nervous system tumors. Radiographics. 37(1):258-280, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27911673%5Bpmid%5D) +1. [Castel D et al: Histone H3F3A and HIST1H3B K27M mutations define two subgroups of diffuse intrinsic pontine gliomas with different prognosis and phenotypes. Acta Neuropathol. 130(6):815-27, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26399631%5Bpmid%5D) +1. [Taylor MD et al: Molecular subgroups of medulloblastoma: the current consensus. Acta Neuropathol. 123(4):465-72, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22134537%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Sagittal T1 C+ FS MR shows a pilocytic astrocytoma involving the cerebellum with heterogeneous enhancement and a large cyst . Note the effacement of the 4th ventricle rather than expansion, which would be typical for medulloblastoma and ependymomas.](images/app.statdx.com_image_thumbnail_c50cb67e-93c0-4b5d-89c2-2c6bced6e023_annotated_true_size_900_quality_90_85e40bf8703443bd140eeb79008269b6a5ab6ecf.jpg) +**Pilocytic Astrocytoma** +*Sagittal T1 C+ FS MR shows a pilocytic astrocytoma involving the cerebellum with heterogeneous enhancement and a large cyst . Note the effacement of the 4th ventricle rather than expansion, which would be typical for medulloblastoma and ependymomas.* + +![Sagittal T1 C+ FS MR shows a pilocytic astrocytoma involving the cerebellum with heterogeneous enhancement and a large cyst . Note the effacement of the 4th ventricle rather than expansion, which would be typical for medulloblastoma and ependymomas.](images/app.statdx.com_image_thumbnail_c50cb67e-93c0-4b5d-89c2-2c6bced6e023_size_174_quality_85_6466dd57.jpg) +**Pilocytic Astrocytoma** +*Sagittal T1 C+ FS MR shows a pilocytic astrocytoma involving the cerebellum with heterogeneous enhancement and a large cyst . Note the effacement of the 4th ventricle rather than expansion, which would be typical for medulloblastoma and ependymomas.* + +![Axial T2 FS MR shows a T2-hyperintense solid mass filling the 4th ventricle. Postcontrast enhancement was seen (not shown), consistent with pilocytic astrocytoma. Low-grade tumors typically have a T2-hyperintense signal of the tumor parenchyma.](images/app.statdx.com_image_thumbnail_04cb6eec-25bf-4339-a5f4-bcbb2df3e1f2_annotated_true_size_900_quality_90_ca7612d37d8e8b825e75fadc0be26f576d508433.jpg) +**Pilocytic Astrocytoma** +*Axial T2 FS MR shows a T2-hyperintense solid mass filling the 4th ventricle. Postcontrast enhancement was seen (not shown), consistent with pilocytic astrocytoma. Low-grade tumors typically have a T2-hyperintense signal of the tumor parenchyma.* + +![Axial T1 C+ FS MR shows a heterogeneously enhancing mass filling the 4th ventricle. This was a group 1 medulloblastoma with Wingless (WNT) activation. These are rare medulloblastomas, but they have a very good prognosis. They are typically seen near the cerebellopontine angle in older children.](images/app.statdx.com_image_thumbnail_2b7d24df-8bb6-42ca-a9b2-acd0b42ec35a_annotated_true_size_900_quality_90_8136cff084803b0fa2890ddaae7c2f68c1fea3c3.jpg) +**Medulloblastoma** +*Axial T1 C+ FS MR shows a heterogeneously enhancing mass filling the 4th ventricle. This was a group 1 medulloblastoma with Wingless (WNT) activation. These are rare medulloblastomas, but they have a very good prognosis. They are typically seen near the cerebellopontine angle in older children.* + +![Axial DWI MR shows areas of decreased diffusion in this WNT pathway medulloblastoma. Decreased diffusion is typical for all medulloblastomas and high- grade neoplasms.](images/app.statdx.com_image_thumbnail_65f29b48-d43c-42c5-8014-a09777c1f43d_annotated_true_size_900_quality_90_e56281ea7b07e703f00f147d64a286cfedfa0238.jpg) +**Medulloblastoma** +*Axial DWI MR shows areas of decreased diffusion in this WNT pathway medulloblastoma. Decreased diffusion is typical for all medulloblastomas and high- grade neoplasms.* + +![Axial T2 MR in an infant shows an isointense nodular mass within the 4th ventricle with associated cerebellar edema . This neoplasm demonstrated intense enhancement and decreased diffusion (not shown). This neoplasm was SHH-activated, group 2 medulloblastoma classified histologically as a medulloblastoma with extensive nodularity.](images/app.statdx.com_image_thumbnail_4d3ef702-b3c7-49f3-a0f3-ab89582a8703_annotated_true_size_900_quality_90_b14442165feebdbc9417ebd5b101260ca26e5ecb.jpg) +**Medulloblastoma** +*Axial T2 MR in an infant shows an isointense nodular mass within the 4th ventricle with associated cerebellar edema . This neoplasm demonstrated intense enhancement and decreased diffusion (not shown). This neoplasm was SHH-activated, group 2 medulloblastoma classified histologically as a medulloblastoma with extensive nodularity.* + +![Sagittal T1 C+ MR shows nodular enhancement in this group 3 medulloblastoma, which has a poor outcome compared to other subgroups.](images/app.statdx.com_image_thumbnail_7b08b79d-df69-4f00-9d7c-78280abac731_annotated_true_size_900_quality_90_8bd31d1888966609dfa87def3f1d348267c788ac.jpg) +**Medulloblastoma** +*Sagittal T1 C+ MR shows nodular enhancement in this group 3 medulloblastoma, which has a poor outcome compared to other subgroups.* + +![Sagittal T1 C+ FS MR shows a minimally enhancing mass within the 4th ventricle . This is consistent with a group 4 medulloblastoma, which arises in the classic location but has minimal enhancement.](2fd29a95-3ae1-4c0a-aafe-6e4dc3adf48f) +**Medulloblastoma** +*Sagittal T1 C+ FS MR shows a minimally enhancing mass within the 4th ventricle . This is consistent with a group 4 medulloblastoma, which arises in the classic location but has minimal enhancement.* + +![Axial DWI MR shows that the intraventricular tumor has nearly uniform decreased diffusion compatible with a high-grade neoplasm. Despite minimal contrast enhancement, group 4 medulloblastomas show decreased diffusion. This most common subgroup has an intermediate prognosis.](3bec2ee5-3d05-4df2-af32-a5eff501d5c0) +**Medulloblastoma** +*Axial DWI MR shows that the intraventricular tumor has nearly uniform decreased diffusion compatible with a high-grade neoplasm. Despite minimal contrast enhancement, group 4 medulloblastomas show decreased diffusion. This most common subgroup has an intermediate prognosis.* + +![Axial T2 FS MR shows a T2 iso- to hyperintense mass filling the 4th ventricle but extending through the foramina inferiorly, particularly the right foramen of Luschka . This is a typical appearance for a posterior fossa ependymoma.](e1c0259e-b9ea-4239-92e2-8d00a1baee39) +**Ependymoma** +*Axial T2 FS MR shows a T2 iso- to hyperintense mass filling the 4th ventricle but extending through the foramina inferiorly, particularly the right foramen of Luschka . This is a typical appearance for a posterior fossa ependymoma.* + +![Sagittal T1 MR shows a mass involving the tectal plate, which is consistent with a tectal plate glioma . Pedunculated brainstem gliomas typically are low grade with good prognosis.](204beb84-6f62-4091-b254-bd6afd1e9483) +**Brainstem Glioma, Pediatric** +*Sagittal T1 MR shows a mass involving the tectal plate, which is consistent with a tectal plate glioma . Pedunculated brainstem gliomas typically are low grade with good prognosis.* + +![Axial FLAIR MR shows a hyperintense mass diffusely expanding the pons and encasing the basilar artery . Unlike pedunculated brain stem tumors, diffuse lesions have a poor prognosis.](583d6733-0dd1-40e8-af2e-b1b62e590588) +**Diffuse Intrinsic Pontine Glioma** +*Axial FLAIR MR shows a hyperintense mass diffusely expanding the pons and encasing the basilar artery . Unlike pedunculated brain stem tumors, diffuse lesions have a poor prognosis.* + +![Axial T1 C+ FS MR shows minimal asymmetric enhancement of the diffuse pontine mass and effacement of the 4th ventricle . With near 100% mortality, these tumors have been associated with H3-K27M mutations, which also contribute to diffuse midline gliomas in the thalamus and spinal cord.](f0cac644-3b8b-4937-9b5a-7e61b2a8d64c) +**Diffuse Intrinsic Pontine Glioma** +*Axial T1 C+ FS MR shows minimal asymmetric enhancement of the diffuse pontine mass and effacement of the 4th ventricle . With near 100% mortality, these tumors have been associated with H3-K27M mutations, which also contribute to diffuse midline gliomas in the thalamus and spinal cord.* + +![Axial T2 FS MR shows a heterogeneous, irregular mass in the left brachium pontis with cystic change and areas of hemorrhage . This was a ganglioglioma at resection.](bd971474-19c9-489a-9b9c-6f7cd4385fe9) +**Ganglioglioma** +*Axial T2 FS MR shows a heterogeneous, irregular mass in the left brachium pontis with cystic change and areas of hemorrhage . This was a ganglioglioma at resection.* + +![Axial T1 C+ FS MR shows that the left middle cerebellar peduncle mass has irregular amorphous contrast enhancement. Note the involvement at the origin of the VII and VIII cranial nerves . This child presented with left sensorineural hearing loss and facial weakness.](59576ed8-801e-4ed5-b293-9a87e234a577) +**Ganglioglioma** +*Axial T1 C+ FS MR shows that the left middle cerebellar peduncle mass has irregular amorphous contrast enhancement. Note the involvement at the origin of the VII and VIII cranial nerves . This child presented with left sensorineural hearing loss and facial weakness.* + +![Axial T1 C+ MR shows an intensely enhancing extraaxial mass displacing the cerebellum & medulla arising below the cerebellopontine angle with extension through the left jugular foramen . There is central cystic nonenhancement , typical for large schwannomas.](3d7554a2-597c-48a0-8e3e-5884cd743243) +**Schwannoma** +*Axial T1 C+ MR shows an intensely enhancing extraaxial mass displacing the cerebellum & medulla arising below the cerebellopontine angle with extension through the left jugular foramen . There is central cystic nonenhancement , typical for large schwannomas.* + +![Axial T1 C+ MR shows a homogeneous enhancing mass with a broad dural attachment along posterior dura, consistent with meningioma. Schwannomas & meningiomas are rare in children, and neurofibromatosis type 2 should be considered.](d54aadef-e3f1-4411-964e-897faafffed1) +**Meningioma, CPA-IAC** +*Axial T1 C+ MR shows a homogeneous enhancing mass with a broad dural attachment along posterior dura, consistent with meningioma. Schwannomas & meningiomas are rare in children, and neurofibromatosis type 2 should be considered.* + +![Sagittal T1 C+ MR shows a cystic mass involving the medulla and upper cervical cord. There are small nodules of enhancement along the rim of the cyst , consistent with hemangioblastoma in a child with von Hippel-Lindau disease.](220effb3-8b23-4265-adcf-d2d616a90860) +**Hemangioblastoma** +*Sagittal T1 C+ MR shows a cystic mass involving the medulla and upper cervical cord. There are small nodules of enhancement along the rim of the cyst , consistent with hemangioblastoma in a child with von Hippel-Lindau disease.* + +![Axial NECT shows a heavily calcified mass arising from the inferior 4th ventricle and extending into the cisterna magna with dilated temporal horns from obstructive hydrocephalus.](928fcd7b-0211-44fe-a9b0-d8f15bd44201) +**Choroid Plexus Papilloma** +*Axial NECT shows a heavily calcified mass arising from the inferior 4th ventricle and extending into the cisterna magna with dilated temporal horns from obstructive hydrocephalus.* + +![Axial T1 C+ MR shows a lobular enhancing mass at the left cerebellopontine angle without extension into the internal auditory canal. This is a rare location for a choroid plexus papilloma.](b3ccd8e5-2126-4df5-b81f-9493f0e39275) +**Choroid Plexus Papilloma** +*Axial T1 C+ MR shows a lobular enhancing mass at the left cerebellopontine angle without extension into the internal auditory canal. This is a rare location for a choroid plexus papilloma.* + +![Axial T2 FS MR shows a poorly circumscribed infiltrative mass in the left pons, cerebellar peduncle, and deep white matter of the right cerebellum. No significant enhancement with areas of decreased diffusion was seen (not shown). This was a biopsy-proven anaplastic astrocytoma.](8b8ebbe6-597d-43fc-86c6-830aac2c9358) +**Anaplastic Astrocytoma** +*Axial T2 FS MR shows a poorly circumscribed infiltrative mass in the left pons, cerebellar peduncle, and deep white matter of the right cerebellum. No significant enhancement with areas of decreased diffusion was seen (not shown). This was a biopsy-proven anaplastic astrocytoma.* + +![Axial T2 MR shows a heterogeneous mass with prominent cysts in the 4th ventricle. The solid component of tumor is iso- to hypointense on T2 & is consistent with high-grade neoplasm such as atypical teratoid-rhabdoid tumor (ATRT).](4b88027c-d283-4413-beda-de9ac57c354a) +**Atypical Teratoid-Rhabdoid Tumor** +*Axial T2 MR shows a heterogeneous mass with prominent cysts in the 4th ventricle. The solid component of tumor is iso- to hypointense on T2 & is consistent with high-grade neoplasm such as atypical teratoid-rhabdoid tumor (ATRT).* + +![Sagittal T1 C+ MR shows heterogeneous enhancement of the 4th ventricle with a prominent cyst . Although not exclusive, ATRT tends to have more cystic change than medulloblastoma and other high-grade neoplasms. This tumor also had decreased diffusion (not shown).](be9e7948-4218-466e-a8b6-7feb6e4ac700) +**Atypical Teratoid-Rhabdoid Tumor** +*Sagittal T1 C+ MR shows heterogeneous enhancement of the 4th ventricle with a prominent cyst . Although not exclusive, ATRT tends to have more cystic change than medulloblastoma and other high-grade neoplasms. This tumor also had decreased diffusion (not shown).* + +![Axial T2 MR shows an isointense mass within the 4th ventricle as well as a nodule in the right cerebellar hemisphere . Involvement of the cerebellar hemisphere in a medulloblastoma is suggestive of group 2 SHH-activation, which is histologically described as desmoplastic or extensive nodularity.](fefbcad1-5886-44eb-9e82-7fe3600a0a16) +**Medulloblastoma Variants** +*Axial T2 MR shows an isointense mass within the 4th ventricle as well as a nodule in the right cerebellar hemisphere . Involvement of the cerebellar hemisphere in a medulloblastoma is suggestive of group 2 SHH-activation, which is histologically described as desmoplastic or extensive nodularity.* + +![Axial DWI MR shows decreased diffusion in this medulloepithelioma involving the pons and right brachium pontis. These tumors are highly malignant and may have metastases to extracranial locations.](7ebdc288-ea87-4687-a868-8a3a6b3aa4d9) +**Medulloepithelioma** +*Axial DWI MR shows decreased diffusion in this medulloepithelioma involving the pons and right brachium pontis. These tumors are highly malignant and may have metastases to extracranial locations.* + +![Axial DWI MR shows a tumor with decreased diffusion within the prepontine cistern encasing the basilar artery . ETMR is an aggressive primitive neuroectodermal tumor with ependymoblastic rosettes occurring in young children.](df041cc0-5483-47fe-81cf-72b0bc178b5e) +**Embryonal Tumor With Multilayered Rosettes** +*Axial DWI MR shows a tumor with decreased diffusion within the prepontine cistern encasing the basilar artery . ETMR is an aggressive primitive neuroectodermal tumor with ependymoblastic rosettes occurring in young children.* + +![Axial T2 MR shows a large, infiltrative left cerebellar hemispheric mass with characteristic tigroid pattern from retained cerebellar folia architecture . This is classic for dysplastic cerebellar gangliocytoma (Lhermitte-Duclos), which is a benign hamartoma.](10c67893-b1c0-45c9-9b88-7a54c2500d83) +**Dysplastic Cerebellar Gangliocytoma** +*Axial T2 MR shows a large, infiltrative left cerebellar hemispheric mass with characteristic tigroid pattern from retained cerebellar folia architecture . This is classic for dysplastic cerebellar gangliocytoma (Lhermitte-Duclos), which is a benign hamartoma.* + + +### Additional Images + +![Axial T2 MR in a 10 month old shows a large mass with a lobular, expanded gyriform configuration .](9e9bf0be-b52d-4c57-a76c-f9055da091e5) +**Medulloblastoma Variants** +*Axial T2 MR in a 10 month old shows a large mass with a lobular, expanded gyriform configuration .* + +![Coronal T1 C+ MR in a 10 month old shows distinct grape-like lobular enhancement , characteristic of medulloblastoma but with extensive nodularity, a medulloblastoma variant with neuronal differentiation and a somewhat better prognosis.](3d8af554-085b-4241-96e0-f12cfcbfb0f7) +**Medulloblastoma Variants** +*Coronal T1 C+ MR in a 10 month old shows distinct grape-like lobular enhancement , characteristic of medulloblastoma but with extensive nodularity, a medulloblastoma variant with neuronal differentiation and a somewhat better prognosis.* + +![Axial T2 MR shows a predominately solid lateral cerebellar mass with scattered hyperintense foci .](0c4bd6ed-4012-4dbd-b218-fab701f0df88) +**Medulloblastoma Variants** +*Axial T2 MR shows a predominately solid lateral cerebellar mass with scattered hyperintense foci .* + +![Coronal T1 C+ MR in the same patient shows that the mass enhances quite heterogeneously. A desmoplastic variant of the medulloblastoma was found at surgery.](29247795-dbcd-47a1-b35c-60eef00bda7b) +**Medulloblastoma Variants** +*Coronal T1 C+ MR in the same patient shows that the mass enhances quite heterogeneously. A desmoplastic variant of the medulloblastoma was found at surgery.* + +![Axial T2 MR in a 2 1/2 year old with 1 month of vomiting and headache shows a large heterogeneous midline mass dorsal to, and displacing the 4th ventricle anteriorly. The mass is minimally hyperintense compared to gray matter.](e5b470e2-2c40-43f9-921c-1ac00fb7c61f) +**Medulloblastoma Variants** +*Axial T2 MR in a 2 1/2 year old with 1 month of vomiting and headache shows a large heterogeneous midline mass dorsal to, and displacing the 4th ventricle anteriorly. The mass is minimally hyperintense compared to gray matter.* + +![Axial T1 C+ FS MR in the same patient shows inhomogeneous enhancement. Histopathology was a desmoplastic variant of medulloblastoma, WHO grade IV.](17506f4a-ec2a-41d6-8213-e170c7bcf920) +**Medulloblastoma Variants** +*Axial T1 C+ FS MR in the same patient shows inhomogeneous enhancement. Histopathology was a desmoplastic variant of medulloblastoma, WHO grade IV.* + +![Sagittal T1 C+ MR shows a typical tumor cyst with an enhancing mural nodule . There is hydrocephalus and protrusion of the cerebellar tonsils through the foramen magnum (acquired Chiari type 1).](images/app.statdx.com_image_thumbnail_c3a7e47d-f531-4083-9e10-3713c56e2aa5_annotated_true_size_900_quality_90_6bd079f95c2afc82f8584e38a9a055750300c854.jpg) +**Pilocytic Astrocytoma** +*Sagittal T1 C+ MR shows a typical tumor cyst with an enhancing mural nodule . There is hydrocephalus and protrusion of the cerebellar tonsils through the foramen magnum (acquired Chiari type 1).* + +![Axial T2 MR shows an increased signal of the solid component of the mass. Interstitial edema is present in the temporal lobes.](images/app.statdx.com_image_thumbnail_ce523fd6-1443-492f-a5be-569cd9e4e82a_annotated_true_size_900_quality_90_a613ac8acba9dbe22497df790f69a1892db32157.jpg) +**Pilocytic Astrocytoma** +*Axial T2 MR shows an increased signal of the solid component of the mass. Interstitial edema is present in the temporal lobes.* + +![Sagittal T2 MR shows a hyperintense mass filling and expanding the 4th ventricle. The tumor does not extend through the 4th ventricular outlet foramina. There is hydrocephalus with acquired tonsillar herniation .](7faac2fd-d93d-4419-89d7-8b744c0cb6a6) +**Medulloblastoma** +*Sagittal T2 MR shows a hyperintense mass filling and expanding the 4th ventricle. The tumor does not extend through the 4th ventricular outlet foramina. There is hydrocephalus with acquired tonsillar herniation .* + +![Coronal T1 C+ MR shows heterogeneous enhancement of the 4th ventricular primitive neuroectodermal tumor/medulloblastoma](56b1b3ec-9dba-4bad-b5fe-064d51758274) +**Medulloblastoma** +*Coronal T1 C+ MR shows heterogeneous enhancement of the 4th ventricular primitive neuroectodermal tumor/medulloblastoma* + +![Sagittal T1 MR shows a large tumor filling the 4th ventricle and extruding through the obex into the upper spinal canal.](71a486c6-1ff6-4840-a7bc-2fae023b6dc6) +**Ependymoma** +*Sagittal T1 MR shows a large tumor filling the 4th ventricle and extruding through the obex into the upper spinal canal.* + +![Axial T2 MR shows a heterogeneous tumor expanding and extruding through the right foramen of Luschka . There are a few coarse calcific foci within the tumor.](22435260-8309-4d13-8e4c-53762c58bc3a) +**Ependymoma** +*Axial T2 MR shows a heterogeneous tumor expanding and extruding through the right foramen of Luschka . There are a few coarse calcific foci within the tumor.* + +![Sagittal T2 MR in an infant with a tectal plate glioma shows marked hydrocephalus involving the 3rd and lateral ventricles. The corpus callosum is stretched thin . The tectal plate is bulbous and slightly increased in signal intensity. The aqueduct of Sylvius is obstructed .](a1b37154-b363-4f92-b3d9-2ea2193ddd51) +**Brainstem Glioma, Pediatric** +*Sagittal T2 MR in an infant with a tectal plate glioma shows marked hydrocephalus involving the 3rd and lateral ventricles. The corpus callosum is stretched thin . The tectal plate is bulbous and slightly increased in signal intensity. The aqueduct of Sylvius is obstructed .* + +![Sagittal T2 MR in this child with a diffusely infiltrating pontine glioma shows homogeneous signal intensity of the expanded pons .](36e0383b-8bb9-4766-9983-cd6963eccd07) +**Brainstem Glioma, Pediatric** +*Sagittal T2 MR in this child with a diffusely infiltrating pontine glioma shows homogeneous signal intensity of the expanded pons .* + +![Sagittal T1 C+ MR shows marked expansion of the medulla by a complex mass with intralesional cystic areas and avid, but heterogeneous, enhancement in this child with dorsal exophytic brainstem glioma. The inferior 4th ventricle is deformed by the protruding mass.](11aec147-5738-41a7-aab0-1166558ecf73) +**Brainstem Glioma, Pediatric** +*Sagittal T1 C+ MR shows marked expansion of the medulla by a complex mass with intralesional cystic areas and avid, but heterogeneous, enhancement in this child with dorsal exophytic brainstem glioma. The inferior 4th ventricle is deformed by the protruding mass.* + +![Sagittal T2 MR shows marked expansion of the medulla and upper cervical spinal cord . The inferior 4th ventricle is deformed by the dorsally protruding mass.](e81e7ae0-b043-452f-ae4f-df09a3c5d348) +**Ganglioglioma** +*Sagittal T2 MR shows marked expansion of the medulla and upper cervical spinal cord . The inferior 4th ventricle is deformed by the dorsally protruding mass.* + +![Axial T2 MR shows a bulky heterogeneous right cerebellopontine angle mass , which crosses the midline. There is also extensive remodeling of the right internal auditory canal by this schwannoma.](671c3e68-4c13-45c7-9d73-06cf45c41ffa) +**Schwannoma** +*Axial T2 MR shows a bulky heterogeneous right cerebellopontine angle mass , which crosses the midline. There is also extensive remodeling of the right internal auditory canal by this schwannoma.* + +![Axial T1 C+ MR in another child shows small bilateral vestibular schwannomas. The right lesion assumes the appearance of an ice cream on a cone. Both demonstrate intralabyrinthine extension .](6c561c95-3a4d-42f0-b119-1e1743741270) +**Schwannoma** +*Axial T1 C+ MR in another child shows small bilateral vestibular schwannomas. The right lesion assumes the appearance of an ice cream on a cone. Both demonstrate intralabyrinthine extension .* + +![Axial T2 MR shows a low-signal, lobular cerebellopontine angle mass with hyperostosis of the adjacent petrous apex. There is mild rotation of the medulla due to mass effect.](3f5a667b-35be-413a-80eb-fd2cbd29092b) +**Meningioma, CPA-IAC** +*Axial T2 MR shows a low-signal, lobular cerebellopontine angle mass with hyperostosis of the adjacent petrous apex. There is mild rotation of the medulla due to mass effect.* + +![Coronal NECT shows diffuse hyperostosis adjacent to the meningioma .](a112f059-7c31-4d09-905c-eca2bf511409) +**Meningioma, CPA-IAC** +*Coronal NECT shows diffuse hyperostosis adjacent to the meningioma .* + +![Sagittal T2 MR shows a solid component with multiple flow voids , a cyst , and edema of the medulla and upper cervical cord .](762f2205-a4a4-46af-877c-106afcd7123c) +**Hemangioblastoma** +*Sagittal T2 MR shows a solid component with multiple flow voids , a cyst , and edema of the medulla and upper cervical cord .* + +![Sagittal T1 C+ MR of the same patient shows the cyst to better advantage. Here, the cyst's contents have slightly increased the signal.](1db0dba5-a11b-4fbe-b761-e3173123d3ec) +**Hemangioblastoma** +*Sagittal T1 C+ MR of the same patient shows the cyst to better advantage. Here, the cyst's contents have slightly increased the signal.* + +![Axial T2 MR shows multiple foci of abnormal signal intensity in the peripheral right cerebellar hemisphere and in the cerebellar white matter adjacent to the lateral recess of the 4th ventricle.](a0a28edb-7a1d-4c5d-a4cb-f9b0e1019146) +**Anaplastic Astrocytoma** +*Axial T2 MR shows multiple foci of abnormal signal intensity in the peripheral right cerebellar hemisphere and in the cerebellar white matter adjacent to the lateral recess of the 4th ventricle.* + +![Axial T1 C+ MR shows enhancement following gadolinium administration. The lesion adjacent to the 4th ventricle lateral recess has ill-defined margins.](e221a57d-ea42-4db3-883c-67c4d0c75ebc) +**Anaplastic Astrocytoma** +*Axial T1 C+ MR shows enhancement following gadolinium administration. The lesion adjacent to the 4th ventricle lateral recess has ill-defined margins.* + +![Sagittal T2 MR shows extensive posterior fossa , pineal region , and intraventricular low-signal intensity masses. Multifocal deposits of tumor at diagnosis are strongly suggestive of an atypical teratoid-rhabdoid tumor.](926b2752-e64e-4db3-80c3-0a7c32d2246d) +**Atypical Teratoid-Rhabdoid Tumor** +*Sagittal T2 MR shows extensive posterior fossa , pineal region , and intraventricular low-signal intensity masses. Multifocal deposits of tumor at diagnosis are strongly suggestive of an atypical teratoid-rhabdoid tumor.* + +![Sagittal T1 C+ MR shows quite variable enhancement of the posterior fossa , pineal region , and intraventricular tumor deposits. There is marked hydrocephalus.](36b3f310-0075-4ad5-8963-39a87e96402d) +**Atypical Teratoid-Rhabdoid Tumor** +*Sagittal T1 C+ MR shows quite variable enhancement of the posterior fossa , pineal region , and intraventricular tumor deposits. There is marked hydrocephalus.* + +![Axial T1 C+ MR shows a slightly heterogeneous but avidly enhancing mass within the right foramen of Luschka . There is an associated cyst .](c4ead918-9b19-4a46-9e82-306a3332a542) +**Choroid Plexus Carcinoma** +*Axial T1 C+ MR shows a slightly heterogeneous but avidly enhancing mass within the right foramen of Luschka . There is an associated cyst .* + +![Axial T2 MR in a different child undergoing treatment for choroid plexus carcinoma shows a large skull base metastatic deposit .](a1124d34-10b5-4516-986f-80002b67d512) +**Choroid Plexus Carcinoma** +*Axial T2 MR in a different child undergoing treatment for choroid plexus carcinoma shows a large skull base metastatic deposit .* + +![Axial NECT in a 1-day-old infant shows a dense, lobular mass filling the posterior fossa. Foci of increased density superimposed in the mass are due to hemorrhage. Note the blood-CSF level in the dilated infundibular recess .](6e743383-9e9d-4ac1-a009-205e92f271ef) +**Medulloepithelioma** +*Axial NECT in a 1-day-old infant shows a dense, lobular mass filling the posterior fossa. Foci of increased density superimposed in the mass are due to hemorrhage. Note the blood-CSF level in the dilated infundibular recess .* + +![Coronal T1 C+ MR in the same infant following biopsy shows extension into the spinal canal . Gas in the ventricular system follows neurosurgical intervention. There is extensive ependymal seeding .](c3ebb831-fe54-4143-8dd5-2b0e43292802) +**Medulloepithelioma** +*Coronal T1 C+ MR in the same infant following biopsy shows extension into the spinal canal . Gas in the ventricular system follows neurosurgical intervention. There is extensive ependymal seeding .* + +![Axial T1 C+ MR shows a large nonenhancing mass involving the left cerebellar hemisphere. Preservation of the cerebellar folia pattern, or striated cerebellum , is characteristic for dysplastic cerebellar gangliocytoma (Lhermitte-Duclos). This disease has a strong association with Cowden syndrome.](700ca6a3-e41d-4cdb-bd66-25af1ae64c73) +**Dysplastic Cerebellar Gangliocytoma** +*Axial T1 C+ MR shows a large nonenhancing mass involving the left cerebellar hemisphere. Preservation of the cerebellar folia pattern, or striated cerebellum , is characteristic for dysplastic cerebellar gangliocytoma (Lhermitte-Duclos). This disease has a strong association with Cowden syndrome.* + diff --git a/docs_md/articles/prepontine-cistern-mass_e0f71196-85c7-411c-9c71-e2606b2ee52f.md b/docs_md/articles/prepontine-cistern-mass_e0f71196-85c7-411c-9c71-e2606b2ee52f.md new file mode 100644 index 0000000..08323f1 --- /dev/null +++ b/docs_md/articles/prepontine-cistern-mass_e0f71196-85c7-411c-9c71-e2606b2ee52f.md @@ -0,0 +1,363 @@ +--- +title: "Prepontine Cistern Mass" +docid: "e0f71196-85c7-411c-9c71-e2606b2ee52f" +authors: + - key: "26ebc2e8-e4f7-40ee-8f5c-d23fe383e15c" + value: "Yoshimi Anzai, MD, MPH" + - key: "83f867a5-a183-4396-82ea-384015da4d2f" + value: "Gregory L. Katzman, MD, MBA" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c" + - + name: "CPA-IAC and Posterior Fossa" + slug: "cpa-iac-and-posterior-fossa" + treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992" + - + name: "Anatomically Based Differentials" + slug: "anatomically-based-differentials" + treeNodeId: "debfb06c-8656-4f5d-92c1-eaa468185d78" + - + name: "Prepontine Cistern Mass" + slug: "prepontine-cistern-mass" + treeNodeId: null +category: "Head and Neck" +documentVersionId: "8526aadd-9d1f-4f3f-8b6a-cab3c86d8196" +imageCount: 39 +lastUpdated: "08/02/18" +pageDescription: "Prepontine Cistern Mass" +pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Anatomically Based Differentials, Prepontine Cistern Mass" +pageTitle: "Prepontine Cistern Mass | STATdx" +enhancedTitle: "Prepontine Cistern Mass" +type: "DDX" +references: true +breadcrumbs: + - "Head and Neck" + - "Differential Diagnosis" + - "CPA-IAC and Posterior Fossa" + - "Anatomically Based Differentials" + - "Prepontine Cistern Mass" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Anatomy + - Extensive CSF space along ventral & lateral pons, dorsal to clivus (a.k.a. pontine cistern) + - Bounded superiorly by interpeduncular cistern, inferiorly by subarachnoid space of spinal cord, & continuous about medulla with cerebellomedullary cistern + - Many abnormalities, often from transspatial processes +- ## Helpful Clues for Common Diagnoses + + + - **CSF Flow Artifact** + - MR artifacts divided into 2 categories: TOF effects & turbulent flow + - Worsens with thinner slices, longer TE, & imaging perpendicular to flow + - Assess real vs. artifact in other planes & sequences + - Minimize TOF losses: Use short TE, image parallel to flow, acquire thicker slices + - Use 3D sequence in place of 2D + - Typically most pronounced on axial FLAIR + - Confirm artifact on T1 & T2 or other planes + - **Dolichoectasia (Vertebrobasilar)** + - Older patients + - Look for ASVD in other vessels + - Ectasia often extends into branches + - May have significant mass effect on brainstem & cisternal cranial nerves + - **Fusiform Aneurysm, ASVD** + - Long segment fusiform arterial dilatation + - Involves long nonbranching segments + - Calcifications common + - Lumen enhances strongly, clot does not + - **Meningioma** + - Clival region dural-based enhancing mass + - Infratentorial (8-10%): CPA most common + - Retroclival meningioma + - Best seen on sagittal T1 C+ FS MR + - Causes cranial neuropathies or ataxia + - **Metastases, Skull & Meningeal** + - Enhancing lesion(s) with skull/meningeal destruction/infiltration + - Skull involvement causes low marrow signal on T1 MR & enhancement on T1 C+ MR + - Skull involvement can spread to adjacent dura + - Metastases may also be dural/meningeal only + - DWI (mildly ↑ signal) may ↑ conspicuity of calvarial metastases + - Manifestations: May cause smooth linear thickening or nodular, fungating masses + - Image entire neuraxis +- ## Helpful Clues for Less Common Diagnoses + + + - **Epidermoid Cyst** + - Usually extends medially from CPA cistern + - Lobulated, irregular, insinuating CSF-like mass + - Doesn't completely suppress on FLAIR; restricts on DWI + - Cranial nerve involvement not infrequent + - **Chiari 2 ("Creeping Cerebellum****"****)** + - Small posterior fossa with low torcular Herophili + - Cerebellar hemispheres/tonsils herniate anteriorly → "creeping" cerebellum + - Pons, cranial nerve roots often elongated + - Fusion & inferior displacement of superior & inferior colliculi: "Tectal beaking" + - **Exophytic Brainstem Glioma, Pediatric** + - Nonenhancing mass markedly expanding pons + - May engulf basilar artery + - Infiltrative have poor survival + - Diffuse intrinsic brain stem glioma is now called diffuse midline glioma under 2016 WHO classification; H3, K23M mutant + - Focal glioma uncommon, better prognosis + - **Pituitary Macroadenoma (Giant)** + - No distinct pituitary gland is visible + - Bone CT variable; may be benign appearing or erosive + - Heterogeneous contrast enhancement + - Dural tail may mimic meningioma + - Typically extends to suprasellar cistern, though occasionally extends posteriorly to prepontine cistern + - **Neurocysticercosis** + - Cisterns > parenchyma > ventricles + - MR best modality; most isointense to CSF + - Appearance varies depending on stage + - Basal cistern cysts may be racemose + - Lobulated cisternal cysts, usually no scolex + - Cysts variable, typically 5-20 mm with 1-4 mm scolex + - Scolex is small enhancing soft tissue "dot" + - Colloidal vesicular stage + - Result from implantation of cestode *Taenia solium*(pork tapeworm) in human host + - 3D MR sequences optimize detection + - **Intracranial Hypotension** + - Sagittal shows brain descent in 40-50% + - Pons may be compressed against clivus + - Diffusely, intensely enhancing dura in 85% + - Bilateral subdural fluid collections in 15% +- ## Helpful Clues for Rare Diagnoses + + + - **Inflammatory Mass** + - **Tuberculosis** + - Basilar meningitis, pulmonary TB + - Thick basilar exudate ± tuberculomas/abscesses + - Basilar meningitis may cause infarction due to vascular wall invasion + - **Fungal diseases** + - Blastomycosis, coccidiomycosis, histoplasmosis, candidiasis + - Meningeal enhancement, multiple enhancing brain lesions + - Frequently ring-enhancing + - **Neurosarcoid** + - Classically infiltrates dura, leptomeninges, basal cisterns + - Solitary or multifocal CNS mass(es) ± abnormal CXR + - **Clival Neoplasms** + - **Chordoma, clivus** + - Destructive midline mass centered in clivus with high T2 signal intensity + - Sagittal images show tumor "thumb" indenting anterior pons + - **Chondrosarcoma, skull base** + - Arises from petrooccipital fissure + - May extend posteriorly into prepontine cistern + - Hyperintense on T2, enhances strongly but heterogeneously + - Chondroid matrix mineralization on CT in 50% + - **Plasmacytoma, skull base** + - Solitary intraosseous osteolytic soft tissue mass with nonsclerotic margins + - Peripherally displaced osseous expansion/fragmentation may be seen + - **Nasopharyngeal tumor (invading clivus)** + - Often squamous cell carcinoma arising from nasopharyngeal mucosal space + - MPR images best show invasion of clivus & prepontine cistern extension + - **Schwannoma** + - T2-hyperintense, enhance + - Larger lesion may be associated with cystic degeneration + - **Arachnoid Cyst** + - Extraaxial cyst follows CSF attenuation/signal + - Suppresses completely with FLAIR; no DWI restriction + - **Craniopharyngioma** + - 90% Ca⁺⁺, 90% cystic, 90% enhance + - May extend behind sella into posterior fossa + - Location: Anywhere along infundibulum, sellar, suprasellar, & floor of 3rd ventricle + - Adamantinomatous is most common type (> 90%) + - May contain T1 hyperintense cyst due to high protein content + - **Neurenteric Cyst** + - Round/lobulated nonenhancing mass, slightly hyperintense to CSF on T1, FLAIR + - Benign malformative endodermal CNS cyst + - **Ecchordosis Physaliphora** + - Congenital benign hamartomatous lesion from notochord remnant + - Extends from clivus into prepontine cistern + - Hyperintense on T2, no contrast enhancement + - Bone CT: Well-corticated defect in dorsal clivus + - Usually asymptomatic, found in 2% of autopsies + +## References + +# Selected References + +1. [Bazan R et al: Clinical symptoms, imaging features and cyst distribution in the cerebrospinal fluid compartments in patients with extraparenchymal neurocysticercosis. PLoS Negl Trop Dis. 10(11):e0005115, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27828966%5Bpmid%5D) +1. [Louis DN et al: The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 131(6):803-20, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27157931%5Bpmid%5D) +1. [Park HH et al: Ecchordosis physaliphora: typical and atypical radiologic features. Neurosurg Rev. 40(1):87-94, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27206421%5Bpmid%5D) +1. [Carrillo Mezo R et al: Relevance of 3D magnetic resonance imaging sequences in diagnosing basal subarachnoid neurocysticercosis. Acta Trop. 152:60-65, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26327445%5Bpmid%5D) +1. [Garcia HH et al: Clinical symptoms, diagnosis, and treatment of neurocysticercosis. Lancet Neurol. 13(12):1202-15, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25453460%5Bpmid%5D) +1. [Lerner A et al: Imaging of neurocysticercosis. Neuroimaging Clin N Am. 22(4):659-76, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=23122261%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial FLAIR MR reveals a hyperintense artifact due to turbulent flow within the prepontine cistern. This is a common location for flow artifacts. Also note sulcal hyperintensity (not artifact) from subarachnoid hemorrhage .](images/app.statdx.com_image_thumbnail_36c90ad4-3950-40b3-aefa-62fb1b3701a7_annotated_true_size_900_quality_90_8d94b1178e2f011450729e41e545e85df305aa8d.jpg) +**CSF Flow Artifact** +*Axial FLAIR MR reveals a hyperintense artifact due to turbulent flow within the prepontine cistern. This is a common location for flow artifacts. Also note sulcal hyperintensity (not artifact) from subarachnoid hemorrhage .* + +![Axial FLAIR MR reveals a hyperintense artifact due to turbulent flow within the prepontine cistern. This is a common location for flow artifacts. Also note sulcal hyperintensity (not artifact) from subarachnoid hemorrhage .](images/app.statdx.com_image_thumbnail_36c90ad4-3950-40b3-aefa-62fb1b3701a7_size_174_quality_85_f44b5250.jpg) +**CSF Flow Artifact** +*Axial FLAIR MR reveals a hyperintense artifact due to turbulent flow within the prepontine cistern. This is a common location for flow artifacts. Also note sulcal hyperintensity (not artifact) from subarachnoid hemorrhage .* + +![Axial T1 C+ MR demonstrates luminal enhancement of a dolichoectatic basilar artery with associated deformation of the pons .](images/app.statdx.com_image_thumbnail_75cee221-2bbe-4e36-aa2e-61e38ef5d5dd_annotated_true_size_900_quality_90_d78649737dc5dc4e52ca3664c894c769a539ae00.jpg) +**Dolichoectasia (Vertebrobasilar)** +*Axial T1 C+ MR demonstrates luminal enhancement of a dolichoectatic basilar artery with associated deformation of the pons .* + +![Sagittal T1 MR shows a large mass anterior to the pons and medulla . Note mixed hyper-, isointense signal caused by slow flow and laminated clot in this classic ASVD fusiform aneurysm.](images/app.statdx.com_image_thumbnail_6b077e2f-53ae-4cee-97ce-a3fca6e5f74b_annotated_true_size_900_quality_90_13507e6c335994df9995be2ed368ea8d2ef64dc8.jpg) +**Fusiform Aneurysm, ASVD** +*Sagittal T1 MR shows a large mass anterior to the pons and medulla . Note mixed hyper-, isointense signal caused by slow flow and laminated clot in this classic ASVD fusiform aneurysm.* + +![Sagittal T1 C+ MR demonstrates avid meningioma enhancement with prepontine cistern extension and mass effect on the brainstem. Note the presence of enhancing dural tails .](images/app.statdx.com_image_thumbnail_72e0e7a9-3297-489a-968a-12452669fcaa_annotated_true_size_900_quality_90_dca1e68c34c5cd9104ee739f36125065ab047304.jpg) +**Meningioma** +*Sagittal T1 C+ MR demonstrates avid meningioma enhancement with prepontine cistern extension and mass effect on the brainstem. Note the presence of enhancing dural tails .* + +![Axial T1 C+ MR demonstrates extensive renal cell metastatic disease involving the clivus and overlying dura , effacing the prepontine cistern .](56d0e1a1-55f1-49ce-8c38-b6508212f452) +**Metastases, Skull & Meningeal** +*Axial T1 C+ MR demonstrates extensive renal cell metastatic disease involving the clivus and overlying dura , effacing the prepontine cistern .* + +![Axial T1 C+ MR shows a typical MR case of leptomeningeal seeding of carcinoma along the folia of the cerebellum and the brainstem as well as within bilateral Meckel caves .](2249e8d7-5b0a-4101-b57b-d7cade35a7e3) +**Metastases, Skull & Meningeal** +*Axial T1 C+ MR shows a typical MR case of leptomeningeal seeding of carcinoma along the folia of the cerebellum and the brainstem as well as within bilateral Meckel caves .* + +![Sagittal T1 MR depicts a nearly CSF isointense, nonenhancing, multilobulated epidermoid within prepontine, interpeduncular, & quadrigeminal cisterns . Note associated flattening of the ventral pons .](images/app.statdx.com_image_thumbnail_186a1a97-4911-4efc-9bd1-d992ae43d805_annotated_true_size_900_quality_90_e4ddd0f2293cc5a20907f651310221ed9c22c5c5.jpg) +**Epidermoid Cyst** +*Sagittal T1 MR depicts a nearly CSF isointense, nonenhancing, multilobulated epidermoid within prepontine, interpeduncular, & quadrigeminal cisterns . Note associated flattening of the ventral pons .* + +![Axial T2 MR shows cerebellar hemispheres herniating or "creeping" anteriorly due to a congenitally small posterior fossa of Chiari 2 malformation.](images/app.statdx.com_image_thumbnail_33699400-7724-48f4-99d6-ce09d9668a3e_annotated_true_size_900_quality_90_36064b4d030a5e26fef4bef6a67f7ffb1f1d24f1.jpg) +**Chiari 2 (“Creeping Cerebellum”)** +*Axial T2 MR shows cerebellar hemispheres herniating or "creeping" anteriorly due to a congenitally small posterior fossa of Chiari 2 malformation.* + +![Axial FLAIR MR shows a diffuse brainstem glioma asymmetrically involving the pons with a small anterior exophytic component extending into the right prepontine cistern , next to the basilar artery .](55f40d88-9997-4d58-a143-d8aba3ed86bb) +**Exophytic Brainstem Glioma, Pediatric** +*Axial FLAIR MR shows a diffuse brainstem glioma asymmetrically involving the pons with a small anterior exophytic component extending into the right prepontine cistern , next to the basilar artery .* + +![Sagittal T2 MR shows a giant macroadenoma with suprasellar extension , invading anteriorly into basisphenoid , and posteriorly into the basiocciput . The pons and basilar artery are displaced posteriorly and flattened .](f75fea6d-397c-4378-af93-f6b3c71a4076) +**Pituitary Macroadenoma (Giant)** +*Sagittal T2 MR shows a giant macroadenoma with suprasellar extension , invading anteriorly into basisphenoid , and posteriorly into the basiocciput . The pons and basilar artery are displaced posteriorly and flattened .* + +![Axial T2 MR shows multiple racemose cysts in the subarachnoid spaces including the CPA and quadrigeminal and prepontine cisterns . Also note cysts within suprasellar cistern .](7d409cd3-fc78-47fd-ba0e-728bf74a94d7) +**Neurocysticercosis** +*Axial T2 MR shows multiple racemose cysts in the subarachnoid spaces including the CPA and quadrigeminal and prepontine cisterns . Also note cysts within suprasellar cistern .* + +![Sagittal T1 C+ MR demonstrates typical findings of intracranial hypotension, with obliteration of the suprasellar and prepontine cisterns with a sagging midbrain. Note pontine flattening against the clivus, dural enhancement, and cerebellar tonsillar descent.](e1ecaa8b-7a55-4c19-b7e7-126b4c9f92bc) +**Intracranial Hypotension** +*Sagittal T1 C+ MR demonstrates typical findings of intracranial hypotension, with obliteration of the suprasellar and prepontine cisterns with a sagging midbrain. Note pontine flattening against the clivus, dural enhancement, and cerebellar tonsillar descent.* + +![Axial T1 C+ MR demonstrates typical enhancement and thickened meninges filling the basilar cisterns in exudative tuberculous meningitis.](690c8b1f-d5d5-4ab3-a9b4-554e46b59f77) +**Tuberculosis** +*Axial T1 C+ MR demonstrates typical enhancement and thickened meninges filling the basilar cisterns in exudative tuberculous meningitis.* + +![Sagittal T1 C+ MR shows tuberculous abscesses within the basal and prepontine cisterns as well as within the anterior 3rd ventricle .](facccff6-f9e0-4ac3-9003-b17702ca612f) +**Tuberculosis** +*Sagittal T1 C+ MR shows tuberculous abscesses within the basal and prepontine cisterns as well as within the anterior 3rd ventricle .* + +![Axial T1 C+ MR shows thick enhancement in the subarachnoid space and along the pia filling the prepontine cistern and extending into the left internal auditory canal due to coccidioidal meningitis. Note also thin linear ependymal enhancement lining the 4th ventricle .](7f70d4c6-9acb-4758-896c-13442a628ebb) +**Fungal Diseases** +*Axial T1 C+ MR shows thick enhancement in the subarachnoid space and along the pia filling the prepontine cistern and extending into the left internal auditory canal due to coccidioidal meningitis. Note also thin linear ependymal enhancement lining the 4th ventricle .* + +![Axial T1 C+ MR demonstrates fine linear enhancement along the pia from candida meningitis.](53e7d43c-9851-405d-bc06-a3c252227e28) +**Fungal Diseases** +*Axial T1 C+ MR demonstrates fine linear enhancement along the pia from candida meningitis.* + +![Sagittal T1 C+ MR demonstrates a typical neurosarcoid appearance and location, with marked multifocal nodular appearing dural-based enhancement . The sella, parasellar, and basal cisternal locations are classic.](1f475c6f-0565-4eca-8149-be39cda8fb20) +**Neurosarcoid** +*Sagittal T1 C+ MR demonstrates a typical neurosarcoid appearance and location, with marked multifocal nodular appearing dural-based enhancement . The sella, parasellar, and basal cisternal locations are classic.* + +![Sagittal T1 C+ MR shows a honeycomb pattern of enhancement with replacement of the clivus , "thumbing" of the pons posteriorly , and anterior extension of tumor into the sphenoid sinus .](2881e16c-9caa-45eb-bd00-71a3f5a55abf) +**Chordoma, Clivus** +*Sagittal T1 C+ MR shows a honeycomb pattern of enhancement with replacement of the clivus , "thumbing" of the pons posteriorly , and anterior extension of tumor into the sphenoid sinus .* + +![Axial T2 MR reveals hyperintense chondrosarcoma originating from the right petrooccipital fissure, extending posterosuperiorly into Meckel cave, petrous apex, and the prepontine and cerebellopontine angle cisterns .](c96ec5ba-c5b3-4810-9712-67e131e150cd) +**Chondrosarcoma, Skull Base** +*Axial T2 MR reveals hyperintense chondrosarcoma originating from the right petrooccipital fissure, extending posterosuperiorly into Meckel cave, petrous apex, and the prepontine and cerebellopontine angle cisterns .* + +![Sagittal T1 MR demonstrates plasmacytoma expanding the clivus, invading sphenoid sinus and posterior nasal cavity, and elevating the pituitary gland .](51fec020-c66e-4ee7-b007-6fcfc246ab71) +**Plasmacytoma, Skull Base** +*Sagittal T1 MR demonstrates plasmacytoma expanding the clivus, invading sphenoid sinus and posterior nasal cavity, and elevating the pituitary gland .* + +![Sagittal T1 C+ FS MR demonstrates nasopharyngeal squamous cell carcinoma infiltration of the mucosal space as well as abnormal marrow signal and cortical destruction of the expanded clivus. Prepontine soft tissue cistern extension is noted posterior to the clivus .](fcb9db35-456f-46fe-8601-2a2fabcdf654) +**Nasopharyngeal Tumor (Invading Clivus)** +*Sagittal T1 C+ FS MR demonstrates nasopharyngeal squamous cell carcinoma infiltration of the mucosal space as well as abnormal marrow signal and cortical destruction of the expanded clivus. Prepontine soft tissue cistern extension is noted posterior to the clivus .* + +![Axial T2 MR shows a partly cystic mass in the left prepontine and cerebellopontine angle cistern . The mass displaces and compresses the brainstem. This was a large cystic trigeminal schwannoma.](3d5bff4b-9f79-4f29-8697-24d8fd16ea4c) +**Schwannoma** +*Axial T2 MR shows a partly cystic mass in the left prepontine and cerebellopontine angle cistern . The mass displaces and compresses the brainstem. This was a large cystic trigeminal schwannoma.* + +![Sagittal T1 MR demonstrates a primarily suprasellar cistern arachnoid cyst extending into the interpeduncular and prepontine cisterns . Note flattening of the ventral pons .](12a0c554-3e4e-4b60-afa8-965076e9d6e0) +**Arachnoid Cyst** +*Sagittal T1 MR demonstrates a primarily suprasellar cistern arachnoid cyst extending into the interpeduncular and prepontine cisterns . Note flattening of the ventral pons .* + +![Sagittal T1 C+ MR demonstrates a partially cystic and solid enhancing craniopharyngioma in the suprasellar cistern with extension inferior into the prepontine cistern .](3874d01b-d03a-44bb-a431-c98f9aed3608) +**Craniopharyngioma** +*Sagittal T1 C+ MR demonstrates a partially cystic and solid enhancing craniopharyngioma in the suprasellar cistern with extension inferior into the prepontine cistern .* + +![Sagittal T1 MR reveals a well-delineated, slightly ovoid, lobulated mass that was hyperintense to cerebrospinal fluid on all sequences.](e1bbe72b-27c0-44d5-9574-91f16e3d0fe1) +**Neurenteric Cyst** +*Sagittal T1 MR reveals a well-delineated, slightly ovoid, lobulated mass that was hyperintense to cerebrospinal fluid on all sequences.* + +![Axial T2 MR shows a lobulated cystic appearing mass in the prepontine cistern that indents the ventral pons . The mass is hyperintense relative to CSF. Note subtle dehiscence of clivus from where the lesion arose.](140daa05-4ead-48c4-b400-099fce0b4bc1) +**Ecchordosis Physaliphora** +*Axial T2 MR shows a lobulated cystic appearing mass in the prepontine cistern that indents the ventral pons . The mass is hyperintense relative to CSF. Note subtle dehiscence of clivus from where the lesion arose.* + + +### Additional Images + +![Axial T2WI MR shows a predominately hypointense fusiform aneurysm , representing a combination of slow intraluminal flow and subacute mural thrombus.](images/app.statdx.com_image_thumbnail_a52d5423-401f-49a2-b270-88db1f11b4d7_annotated_true_size_900_quality_90_a594b9e50f3ff29b1c0d6c935e5ba1531388f2aa.jpg) +**Fusiform Aneurysm, ASVD** +*Axial T2WI MR shows a predominately hypointense fusiform aneurysm , representing a combination of slow intraluminal flow and subacute mural thrombus.* + +![Axial DWI MR shows typical restricted diffusion with an epidermoid cyst involving the basal cisterns. Irregular "insinuating" margins as seen here are typical of larger epidermoid cysts, unlike the smooth lobulated borders of arachnoid cysts.](images/app.statdx.com_image_thumbnail_14a8c1c5-0e64-4ead-9c57-b22ce7c55a54_annotated_true_size_900_quality_90_32fc3d94f36165aa48a7563a168b25d527cedb08.jpg) +**Epidermoid Cyst** +*Axial DWI MR shows typical restricted diffusion with an epidermoid cyst involving the basal cisterns. Irregular "insinuating" margins as seen here are typical of larger epidermoid cysts, unlike the smooth lobulated borders of arachnoid cysts.* + +![Axial CECT shows a large, lobulated, enhancing, and destructive, skull base mass that extends into the anterior, middle, and posterior cranial fossae.](d2856338-50ca-40fd-9e5c-1ba44b992a75) +**Pituitary Macroadenoma (Giant)** +*Axial CECT shows a large, lobulated, enhancing, and destructive, skull base mass that extends into the anterior, middle, and posterior cranial fossae.* + +![Sagittal T1WI MR demonstrates a typical case of racemose (grape-like) neurocysticercosis in the basal cisterns and 3rd ventricle.](6280e918-1af6-4329-a4fd-707337aec6bb) +**Neurocysticercosis** +*Sagittal T1WI MR demonstrates a typical case of racemose (grape-like) neurocysticercosis in the basal cisterns and 3rd ventricle.* + +![Axial T2WI MR shows typical marked T2 hyperintensity . Notice how the chordoma displaces the basilar artery towards the right and causes "thumbing," or indentation of the ventral pons .](4a2aa82a-9c61-4280-85b5-99968b2d66c4) +**Chordoma, Clivus** +*Axial T2WI MR shows typical marked T2 hyperintensity . Notice how the chordoma displaces the basilar artery towards the right and causes "thumbing," or indentation of the ventral pons .* + +![Axial bone CT demonstrates a typical case of a large chondrosarcoma involving the right petrooccipital fissure and the greater wing of the sphenoid. Internal matrix calcifications are present.](2d295562-55cb-4eca-a608-4635185bd077) +**Chondrosarcoma, Skull Base** +*Axial bone CT demonstrates a typical case of a large chondrosarcoma involving the right petrooccipital fissure and the greater wing of the sphenoid. Internal matrix calcifications are present.* + +![Axial T2WI MR reveals a plasmacytoma nearly isointense to brain . These T2 signal characteristics of this lesion would be unusual for a chordoma or chondrosarcoma.](a95589ce-fb65-437c-a2af-5b38db49f55a) +**Plasmacytoma, Skull Base** +*Axial T2WI MR reveals a plasmacytoma nearly isointense to brain . These T2 signal characteristics of this lesion would be unusual for a chordoma or chondrosarcoma.* + +![Axial T1 C+ MR shows extensive clival invasion, perineural extension into the left petrous apex and Meckel cave , as well as invasion and widening of the left pterygopalatine fossa .](9cc8a5d7-231e-4039-a0c6-fda11333a737) +**Nasopharyngeal Tumor (Invading Clivus)** +*Axial T1 C+ MR shows extensive clival invasion, perineural extension into the left petrous apex and Meckel cave , as well as invasion and widening of the left pterygopalatine fossa .* + +![Axial T1 C+ FS MR shows a typical, isolated, intracranial, trigeminal schwannoma bridging the prepontine cistern and Meckel cave affecting the preganglionic segment of the trigeminal nerve .](ceeb77de-562a-49bd-ab69-73779f12d0a4) +**Schwannoma** +*Axial T1 C+ FS MR shows a typical, isolated, intracranial, trigeminal schwannoma bridging the prepontine cistern and Meckel cave affecting the preganglionic segment of the trigeminal nerve .* + +![Axial T2WI MR demonstrates a primarily suprasellar cistern arachnoid cyst extending into the interpeduncular and prepontine cisterns. Note flattening of the pons .](efc21d8c-2ae7-4cb9-828a-1b814c2cddb7) +**Arachnoid Cyst** +*Axial T2WI MR demonstrates a primarily suprasellar cistern arachnoid cyst extending into the interpeduncular and prepontine cisterns. Note flattening of the pons .* + +![Axial NECT shows the classic findings of craniopharyngioma including a cystic suprasellar mass with rim and globular calcifications and fluid-fluid levels .](dcd9f1a3-a5bb-48a3-8620-5326a4c4cf6d) +**Craniopharyngioma** +*Axial NECT shows the classic findings of craniopharyngioma including a cystic suprasellar mass with rim and globular calcifications and fluid-fluid levels .* + +![Sagittal T1WI MR demonstrates hyperintense mass in prepontine cistern that is connected to suprasellar mass by a thin stalk . Craniopharyngioma was found at surgery. Predominance of tumor mass in posterior fossa is unusual.](96a55178-12f8-4961-a99a-9d3235bd27f2) +**Craniopharyngioma** +*Sagittal T1WI MR demonstrates hyperintense mass in prepontine cistern that is connected to suprasellar mass by a thin stalk . Craniopharyngioma was found at surgery. Predominance of tumor mass in posterior fossa is unusual.* + +![Sagittal T1WI MR shows a large, well-delineated, extraaxial mass elevating and displacing the pons and medulla. Note that the cyst internal signal intensity is slightly brighter than cerebrospinal fluid.](2a832593-9977-4c21-8cfe-56d013b432eb) +**Neurenteric Cyst** +*Sagittal T1WI MR shows a large, well-delineated, extraaxial mass elevating and displacing the pons and medulla. Note that the cyst internal signal intensity is slightly brighter than cerebrospinal fluid.* + diff --git a/docs_md/articles/seizure_a09dca6c-f7f3-4a33-8749-a362668690b4.md b/docs_md/articles/seizure_a09dca6c-f7f3-4a33-8749-a362668690b4.md new file mode 100644 index 0000000..552330b --- /dev/null +++ b/docs_md/articles/seizure_a09dca6c-f7f3-4a33-8749-a362668690b4.md @@ -0,0 +1,323 @@ +--- +title: "Seizure" +docid: "a09dca6c-f7f3-4a33-8749-a362668690b4" +authors: + - key: "d2ed5cde-67ab-491a-963b-3c0f245d1fd8" + value: "Karol Cardenas, MD" + - key: "1f262abe-db83-4f18-99af-00bd3045cd4d" + value: "Marc Benayoun, MD, PhD" +breadcrumbs: + - + name: "Nuclear Medicine" + slug: "nuclear-medicine" + treeNodeId: "2406533f-6523-4211-841e-b92d6f8cf34e" + - + name: "Central Nervous System" + slug: "central-nervous-system" + treeNodeId: "bd6b5c36-69df-4f18-af9c-96cc24b52d8f" + - + name: "Brain Perfusion" + slug: "brain-perfusion" + treeNodeId: "3ced37fe-6250-4954-966b-a68e73896d1c" + - + name: "Seizure" + slug: "seizure" + treeNodeId: null +category: "Nuclear Medicine" +cmeTopicId: "9e14995d-a641-404d-af0a-70dbcadadd1a" +documentVersionId: "cb45a88c-2980-43f9-83a8-422b98b5ccba" +imageCount: 10 +lastUpdated: "06/02/25" +pageDescription: "Seizure" +pageKeywords: "Nuclear Medicine, Central Nervous System, Brain Perfusion, Seizure" +pageTitle: "Seizure | STATdx" +enhancedTitle: "Seizure" +type: "DX" +references: true +breadcrumbs: + - "Nuclear Medicine" + - "Central Nervous System" + - "Brain Perfusion" + - "Seizure" +--- +# KEY FACTS + +- ## Imaging + + + - CT/MR done in acute seizures to rule out structural lesion + - Nuclear medicine not used in primary diagnosis but to evaluate patients with intractable epilepsy being considered for neurosurgical intervention + - Interictal brain perfusion SPECT/CT: Demonstrates alteration in cerebral perfusion + - Region of decreased radiotracer uptake (hypoperfusion) in epileptogenic zone + - Ictal brain perfusion SPECT/CT: Demonstrates alteration in cerebral perfusion + - Region of increased radiotracer uptake (hyperperfusion) in epileptogenic zone + - Interictal F-18 FDG PET/CT: Reflects cerebral glucose metabolism + - Region of decreased radiotracer uptake (hypometabolism) in epileptogenic zone + - Signs of lateralization: Ipsilateral thalamus and contralateral cerebellum hypometabolism +- ## Pathology + + + - Acute/provoked seizure: Manifestation of acute CNS injury, including metabolic, toxic, infectious, inflammatory, and structural provoking factors + - Unprovoked seizures: Related to progressive or remote CNS injury, including stroke, traumatic brain injury, and brain tumors + - Overall, most common cause of epilepsy in adults is MTS +- ## Clinical Issues + + + - Lifetime prevalence of experiencing seizure: ~ 10% + - Treatment based on seizure location, etiology, age, and comorbidities + - Antiseizure medication (ASM): 1st-line treatment; however, ineffective in 1/3 of patients + - Epilepsy surgery: Reserved for medically intractable epilepsy and selected cases + - Nuclear medicine critical in evaluation of surgical candidates and prediction of surgical outcome + +# TERMINOLOGY + +- ## Definitions + + + - Seizure: Transient occurrence of signs &/or symptoms due to abnormal excessive or simultaneous neuronal activity in brain + - Epileptogenic zone/focus: Part of brain that is having abnormal electric activities + - Acute/provoked seizure: Manifestation of acute CNS insult, which may not recur after underlying cause is removed + - Unprovoked seizure: Epileptic seizure in absence of acute precipitating factors + - Epilepsy: Pathologic and enduring disposition toward recurrent unprovoked seizures: (1) ≥ 2 unprovoked seizures > 24 hours apart, (2) 1 unprovoked seizure and ≥ 60% likelihood of recurrence within 10 years (by clinical picture, EEG, or imaging), or (3) epilepsy syndrome + - Epilepsy syndrome: Characteristic cluster of clinical and EEG features with specific etiologic findings that occur in recognizable pattern + - Medically intractable epilepsy: Failure of 2 appropriately chosen and dosed antiseizure medications (ASMs) + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Brain perfusion SPECT-CT + - Alteration of cerebral perfusion depends on electric status of brain + - Ictal phase: Blood flow in epileptogenic region can increase up to 300%; seen as area of hyperperfusion + - Interictal phase: Hypoperfusion or normal perfusion in epileptogenic region + - F-18 FDG PET/CT + - Cerebral glucose metabolism is closely linked to neuronal and synaptic function + - Ictal phase: Glucose metabolism increases during seizure; seen as area of hypermetabolism + - Interictal phase: Hypometabolism, very sensitive for lateralization and lobar regionalization of epileptogenic focus +- ## Radiographic Findings + + + - CT/MR usually performed in work-up of acute provoked and unprovoked seizures to rule out structural lesion + - Nuclear medicine plays critical role in work-up of intractable epilepsy as part of multimodality process +- ## MR Findings + + + - MR epilepsy-specific protocol is indicated in 1st seizure without evidence of provoking factors in initial work-up + - Periictal seizure-induced signal changes: DWI and FLAIR abnormalities in cerebral cortex, hippocampi, and thalami, not restricted to vascular territories + - Focal cortical dysplasia (FCD): Blurring of gray-white junction with focal cortical thickening and T2 hyperintensity extending toward ventricle + - MST: Asymmetric decreased volume of mesial temporal lobe with associated T2-hyperintense signal +- ## Nuclear Medicine Findings + + + - Not used in primary diagnosis or evaluation of recent-onset epilepsy but to evaluate patients with intractable epilepsy being considered for neurosurgical intervention + - Most beneficial in patients without visible lesion on MR, MR findings discordant with semiology/EEG, and multiple/bilateral structural lesions + - Brain perfusion SPECT/CT + - Alteration of cerebral perfusion depends on electrical status of brain + - Most commonly used radiotracers: Tc-99m hexamethylpropyleneamine oxime (HMPAO) and Tc-99m ethyl cysteinate dimer (ECD): Cross blood-brain barrier and reflect cerebral blood flow + - Ictal: Region of increased radiotracer uptake (hyperperfusion) in epileptogenic zone + - Interictal: Region of decreased radiotracer uptake (hypoperfusion) in epileptogenic zone + - Sensitivity in temporal lobe epilepsy (TLE): 44% (interictal), 97% (ictal); sensitivity in extra-TLE: 40% (interictal), 66% (ictal) + - Ictal SPECT is more sensitive than interictal SPECT; however, it is difficult to perform + - F-18 FDG PET/CT + - Radiolabeled glucose analogue, reflecting cerebral glucose metabolism, which is closely linked to neuronal and synaptic function + - Interictal: Region of decreased radiotracer uptake (hypometabolism) in epileptogenic zone + - Sensitivity in TLE: 87-90% (interictal); sensitivity in extra-TLE: 38-55% (interictal) + - Sensitivity and specificity in pediatric frontal lobe epilepsy: 92% and 62%, respectively + - Better imaging quality and more sensitive than interictal SPECT for epileptogenic focus + - Ictal PET/CT is not feasible due to uptake time of FDG (30 min); if seizure occurs during uptake, may demonstrate region of hypermetabolism in epileptogenic zone +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Nuclear medicine plays critical role in evaluation of intractable focal seizures + - Helps with prediction of surgical outcome by defining "functional deficit zone" + - Hypometabolism confined to epileptogenic zone is associated with better postoperative seizure control + - Discordant findings, severe extratemporal &/or bilateral hypometabolism is associated with higher incidence of postoperative seizures + - Provides important information on functional status of rest of brain + - ### Protocol advice + + + - No clinical or EEG seizure evidence for 24 hours + - Fasting at least 4-6 hours + - "Resting state": Awake in quiet, dark environment for at least 30 minutes prior to and after injection + - Sedation: At least 20 minutes after radiotracer injection + - Will cause global and regional (occipitocerebellar) reduction in metabolism and perfusion + - Blood glucose before FDG injection (150-200 mg/dL) + - Hyperglycemia: Competitive inhibition of F-18 FDG uptake appearing as globally reduced metabolism + - Begin imaging 30-60 minutes after injection + - Continuous EEG recording during image acquisition + +# DIFFERENTIAL DIAGNOSIS + +- ## Psychogenic Nonepileptic Seizures + + + - Typically normal study, as seizures are psychogenic in etiology, though patients with epilepsy can also experience psychogenic nonepileptic seizures in addition to true electrographic seizures + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Imbalance between neuronal electrical excitation and inhibition in different regions of CNS + - Not hierarchical categories according to ILAE task force: (1) Structural, (2) genetic, (3) infectious, (4) metabolic, (5) immune, (6) unknown + - Acute/provoked seizure: Metabolic, toxic, infectious, inflammatory, and structural provoking factors + - Stroke and CNS infection are most common causes + - Unprovoked seizures: CNS insult not at same time frame of seizures + - Related to progressive or remote CNS injury + - Stroke: ~ 6% of stroke patients will eventually develop epilepsy + - Traumatic brain injury (TBI): 2x increased risk of epilepsy after TBI + - Brain tumor: Highest seizure prevalence in low-grade gliomas (60-85%) + - No known etiology +- ## Staging, Grading, & Classification + + + - 3-level stepwise approach for classification + - Seizure type + - Origin of seizure: Focal, generalized, unknown + - Degree of awareness: Intact or impaired + - Level of body movement: Motor or nonmotor + - Epilepsy type: Focal, generalized, and combined + - Epilepsy syndrome + - (1) Neonatal and infantile, (2) childhood, (3) variable age, (4) idiopathic generalized epilepsies + - Further subdivided within each age group into generalized, focal, or generalized and focal + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Ictal semiology: Clinical characteristics of seizure, including both subjective symptoms and objective signs + - Can be broadly divided into (1) auras, (2) alteration of consciousness, (3) motor, (4) autonomic, and (5) cognitive manifestations + - ### Other signs/symptoms + + + - Postictal state: Changes in behavior, motor function, and neuropsychologic performance from end of seizure until return to normal neurologic function + - Cognitive and neuropsychologic disorders + - TLE: Memory impairment, anxiety, depression + - Frontal lobe epilepsy: Impairment in motor function, emotional control, inhibition +- ## Demographics + + + - Lifetime prevalence of experiencing seizure: ~ 10% + - Acute/provoked seizure incidence: 29-39/100,000/year + - Age predominance: < 1 year and older adult population + - Most common causes: Fever, TBI, cerebrovascular disease, drug withdrawal, infection, and metabolic insults + - Epilepsy lifetime prevalence: 7.6/1,000 + - Most common cause of epilepsy in adults is mesial temporal sclerosis (MTS) +- ## Treatment + + + - Choosing treatment is based on seizure location, etiology, age, and comorbidities + - ASM: 1st-line treatment; reduces chance of recurrence by > 50% + - Medically intractable epilepsy: Occurs in 1/3 of epilepsy patients (> 30%) + - Increased risk: Structural etiology (MTS, glioneuronal tumors, and FCD) + - Epilepsy surgery + - Offered to patients with medically intractable epilepsy + - "Early" surgery considered in patients with non-drug-resistant focal epilepsy with well-established clinical, imaging, and EEG correlations + - Favorable prognostic factor for epilepsy surgery: Presence of structural lesion + - Unfavorable prognostic factor for epilepsy surgery: Long disease duration + - Seizure remission rate > 80% in TLE and 55% in extra-TLE + - Neurostimulation + - Vagal nerve stimulation: 30% achieve seizure reduction and < 10% seizure freedom at long-term follow-up + - Deep brain stimulation: Significant and sustained reduction in seizures in patients with refractory focal epilepsies not eligible for surgery + - Responsive neurostimulation: > 60% reduction in seizure frequency, retained over long-term follow-up + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Interictal F-18 FDG PET more sensitive than interictal SPECT + - Highly sensitive for lateralization of focal seizures (95% of MR positive-, 69% of MR equivocal-, and 84% of MR-negative patients) + - Highly sensitive for localization of TLE, however, less sensitive for extra-TLE foci + - Overestimates size of epileptogenic focus + - If there are multiple potential candidates, does not clarify which one(s) may be offending lesion +- ## Image Interpretation Pearls + + + - Before imaging review, do not review specific clinical data, as it may increase reader bias and overcall + - Ensure absence of major imaging artifact and ensure axis is level and symmetric + - Fuse with high-resolution MR if available + - Window image according to deep gray nuclei and choose color scale with most step-offs + - Review images side-by-side and lobe-by-lobe, ideally starting with temporal lobes + - Start review in axial and confirm asymmetry in coronal + - Do not stop looking, even after finding candidate; there may be multiple + - Look for signs of lateralization: Ipsilateral thalamus, contralateral cerebellum + - After initial review, check areas of concern previously identified clinically on EEG &/or MR and reexamine + - Consider subtraction imaging when comparing ictal to interictal SPECT + - Compare to normal database (PET) when available + - Statistic parametric mapping can help identify focal areas of abnormality that are difficult to identify by visual analysis alone + + 98c6ea2a-7f72-4cf3-9ae4-cd85e8320f68 + +## References + +# Selected References + +1. [Huff JS et al: Seizure. StatPearls, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=28613516%5Bpmid%5D) +1. [Linka L et al: Effect of the revised definition of epilepsy on treatment decisions and seizure recurrence after a first epileptic seizure. Eur J Neurol. 30(6):1557-64, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=36883241%5Bpmid%5D) +1. [Audrey C et al: Prevalence of seizures in brain tumor: a meta-analysis. Epilepsy Res. 187:107033, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36274423%5Bpmid%5D) +1. [Pelliccia V et al: Early epilepsy surgery for non drug-resistant patients. Epilepsy Behav Rep. 19:100542, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35573058%5Bpmid%5D) +1. Rathore C: Classical and novel signs in seizure semiology. In Sylaja PN: IAN Reviews in Neurology 2022: Epilepsy - Innovations and Advances. Jaypee Brothers Medical Publishers Pvt Ltd, 2022 +1. Vera-González A: Pathophysiological mechanisms underlying the etiologies of seizures and epilepsy. In: Czuczwar SJ: Epilepsy. Exon Publications, 2022 +1. [Wirrell EC et al: Methodology for classification and definition of epilepsy syndromes with list of syndromes: report of the ILAE Task Force on Nosology and Definitions. Epilepsia. 63(6):1333-48, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35503715%5Bpmid%5D) +1. [Riva A et al: New trends and most promising therapeutic strategies for epilepsy treatment. Front Neurol. 12:753753, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34950099%5Bpmid%5D) +1. [Beghi E: The Epidemiology of epilepsy. Neuroepidemiology. 54(2):185-91, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31852003%5Bpmid%5D) +1. [Sarmast ST et al: Current classification of seizures and epilepsies: scope, limitations and recommendations for future action. Cureus. 12(9):e10549, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=33101797%5Bpmid%5D) +1. [Falco-Walter JJ et al: The new definition and classification of seizures and epilepsy. Epilepsy Res. 139:73-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29197668%5Bpmid%5D) +1. [Liu JT et al: Surgical versus medical treatment of drug-resistant epilepsy: a systematic review and meta-analysis. Epilepsy Behav. 82:179-88, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29576434%5Bpmid%5D) +1. [Fisher RS et al: ILAE official report: a practical clinical definition of epilepsy. Epilepsia. 55(4):475-82, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24730690%5Bpmid%5D) +1. [Gok B et al: The evaluation of FDG-PET imaging for epileptogenic focus localization in patients with MRI positive and MRI negative temporal lobe epilepsy. Neuroradiology. 55(5):541-50, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23223825%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Coronal T2* MR in a 45-year-old woman with focal seizures shows asymmetric decreased volume and increased T2 signal involving the right mesial temporal lobe, consistent with mesial temporal sclerosis.](images/app.statdx.com_image_thumbnail_eedbc7fa-2826-4aa4-a86b-db97213cfa9f_annotated_true_size_900_quality_90_1df7be88c8db9572ef73dc34fa3322d3a6ae1199.jpg) +*Coronal T2* MR in a 45-year-old woman with focal seizures shows asymmetric decreased volume and increased T2 signal involving the right mesial temporal lobe, consistent with mesial temporal sclerosis.* + +![Coronal T2* MR in a 45-year-old woman with focal seizures shows asymmetric decreased volume and increased T2 signal involving the right mesial temporal lobe, consistent with mesial temporal sclerosis.](images/app.statdx.com_image_thumbnail_eedbc7fa-2826-4aa4-a86b-db97213cfa9f_size_174_quality_85_d7bdd8afa8961d410e25438386a74f3670dc35f7.jpg) +*Coronal T2* MR in a 45-year-old woman with focal seizures shows asymmetric decreased volume and increased T2 signal involving the right mesial temporal lobe, consistent with mesial temporal sclerosis.* + +![Coronal interictal fused F-18 FDG PET/MR demonstrates corresponding hypometabolism involving the right mesial temporal lobe, consistent with a concordant epileptogenic zone.](images/app.statdx.com_image_thumbnail_61a192cb-428a-4aef-92e9-31aaeff96e5e_annotated_true_size_900_quality_90_b5fbe52cc1039ad4e3edbf95da73582e36dabf16.jpg) +*Coronal interictal fused F-18 FDG PET/MR demonstrates corresponding hypometabolism involving the right mesial temporal lobe, consistent with a concordant epileptogenic zone.* + +![Coronal T2* MR in a 20-year-old woman with focal seizures shows a cortically based T2-hyperintense lesion centered in the left parahippocampal gyrus, biopsy-proven DNET.](images/app.statdx.com_image_thumbnail_62f19679-01ea-4766-9171-68a272fe1e23_annotated_true_size_900_quality_90_039e698608d047838dd0db89c559c42d42787d31.jpg) +*Coronal T2* MR in a 20-year-old woman with focal seizures shows a cortically based T2-hyperintense lesion centered in the left parahippocampal gyrus, biopsy-proven DNET.* + +![Coronal fused F-18 FDG PET/MR shows a region of interictal hypometabolism in the left parahippocampal gyrus , extending beyond the lesion . It is critical for F-18 FDG PET to identify the extent of seizure focus and adjacent functional deficit zone for planning of seizure-free surgical outcomes.](images/app.statdx.com_image_thumbnail_78388d04-415a-4365-8e04-b9a170be9533_annotated_true_size_900_quality_90_eb921cbaf0509e139684895f17ba7289e835fef0.jpg) +*Coronal fused F-18 FDG PET/MR shows a region of interictal hypometabolism in the left parahippocampal gyrus , extending beyond the lesion . It is critical for F-18 FDG PET to identify the extent of seizure focus and adjacent functional deficit zone for planning of seizure-free surgical outcomes.* + +![Axial fused F-18 FDG PET/CT in a patient with global encephalopathy with EEG reporting nonlateralizing and nonlocalizing seizures shows subtle bilateral regions of hypometabolism and asymmetric decreased FDG uptake in the left thalamus , suggesting left lateralization.](images/app.statdx.com_image_thumbnail_9ebcb9e2-5c2f-4b5f-a765-e3473bc6006f_annotated_true_size_900_quality_90_acc60496ae92b4343ed137be6bf7df2112020bbd.jpg) +*Axial fused F-18 FDG PET/CT in a patient with global encephalopathy with EEG reporting nonlateralizing and nonlocalizing seizures shows subtle bilateral regions of hypometabolism and asymmetric decreased FDG uptake in the left thalamus , suggesting left lateralization.* + +![Axial fused PET/CT in the same patient shows asymmetric decreased FDG uptake in the left cerebellum suggests contralateral right lateralization (i.e., nonlateralizing).](images/app.statdx.com_image_thumbnail_0acbb434-ef3d-4a76-9558-4d517bbcdfa5_annotated_true_size_900_quality_90_ebf484eb8bed3d1b8d5bdee085f23b1073957483.jpg) +*Axial fused PET/CT in the same patient shows asymmetric decreased FDG uptake in the left cerebellum suggests contralateral right lateralization (i.e., nonlateralizing).* + +![Axial T1 MR in a 61-year-old woman with intractable epilepsy and diagnosis of tuberous sclerosis at age 19 shows a right parietal lesion with features suggesting DNET.](images/app.statdx.com_image_thumbnail_027ade9b-7d6e-4155-a84c-39c6e43c1c6c_annotated_true_size_900_quality_90_213be4304d2ef5fb7eaf2f43de3655635ce5cc66.jpg) +*Axial T1 MR in a 61-year-old woman with intractable epilepsy and diagnosis of tuberous sclerosis at age 19 shows a right parietal lesion with features suggesting DNET.* + +![Sagittal FLAIR MR also shows a focal area of gray-white interface obscuration with mild signal hyperintensity in the left postcentral gyrus and a subjacent linear hyperintensity extending to the ventricular margin, consistent with small focal cortical dysplasia (FCD).](images/app.statdx.com_image_thumbnail_7ebafd60-8ffa-40b5-a4cd-18c058a5c6be_annotated_true_size_900_quality_90_5896400c8d8edd9676fb7ead7ff32de99d95143c.jpg) +*Sagittal FLAIR MR also shows a focal area of gray-white interface obscuration with mild signal hyperintensity in the left postcentral gyrus and a subjacent linear hyperintensity extending to the ventricular margin, consistent with small focal cortical dysplasia (FCD).* + +![Axial F-18 FDG PET demonstrates photopenia in the region of the known right parietal mass.](images/app.statdx.com_image_thumbnail_6f471d37-7cea-4ef7-a4d5-a8a72cfddbd3_annotated_true_size_900_quality_90_888eb5d7d1edf4200b3e14fd06722f937fad6750.jpg) +*Axial F-18 FDG PET demonstrates photopenia in the region of the known right parietal mass.* + +![Sagittal F-18 FDG PET images show focal interictal hypometabolism in the left postcentral gyrus , correlating with FCD seen on MR. EEG reported all seizure onsets were in the right lateral posterior temporal lobe, corresponding to the region of the tumor. Patient underwent total resection of right parietal brain mass with no recurrent seizure activity after surgery.](cd14f174-1b04-46bd-8927-04b463b4c020) +*Sagittal F-18 FDG PET images show focal interictal hypometabolism in the left postcentral gyrus , correlating with FCD seen on MR. EEG reported all seizure onsets were in the right lateral posterior temporal lobe, corresponding to the region of the tumor. Patient underwent total resection of right parietal brain mass with no recurrent seizure activity after surgery.* + diff --git a/docs_md/articles/sensorineural-hearing-loss-in-adult_08d468da-fbc3-44f8-8212-6480e0a152c4.md b/docs_md/articles/sensorineural-hearing-loss-in-adult_08d468da-fbc3-44f8-8212-6480e0a152c4.md new file mode 100644 index 0000000..78d5533 --- /dev/null +++ b/docs_md/articles/sensorineural-hearing-loss-in-adult_08d468da-fbc3-44f8-8212-6480e0a152c4.md @@ -0,0 +1,326 @@ +--- +title: "Sensorineural Hearing Loss in Adult" +docid: "08d468da-fbc3-44f8-8212-6480e0a152c4" +authors: + - key: "eef2f839-5706-47b9-89c3-60d8315b2b3a" + value: "Nicholas A. Koontz, MD" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c" + - + name: "CPA-IAC and Posterior Fossa" + slug: "cpa-iac-and-posterior-fossa" + treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992" + - + name: "Clinically Based Differentials" + slug: "clinically-based-differentials" + treeNodeId: "55dd15ac-e67d-48dd-8134-f52884dab28b" + - + name: "Sensorineural Hearing Loss in Adult" + slug: "sensorineural-hearing-loss-in-adult" + treeNodeId: null +category: "Head and Neck" +documentVersionId: "2268573f-6f13-4d60-b402-841d07de264c" +imageCount: 32 +lastUpdated: "07/24/18" +pageDescription: "Sensorineural Hearing Loss in Adult" +pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Clinically Based Differentials, Sensorineural Hearing Loss in Adult" +pageTitle: "Sensorineural Hearing Loss in Adult | STATdx" +enhancedTitle: "Sensorineural Hearing Loss in Adult" +type: "DDX" +references: true +breadcrumbs: + - "Head and Neck" + - "Differential Diagnosis" + - "CPA-IAC and Posterior Fossa" + - "Clinically Based Differentials" + - "Sensorineural Hearing Loss in Adult" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Many diagnoses cause sensorineural hearing loss (SNHL) + - Relative statistical incidence of major differential diagnoses + - **Vestibular schwannoma**: **90%** of lesions causing SNHL + - CPA meningioma, epidermoid cyst, aneurysm: 5% of all lesions causing SNHL + - All other diagnoses in SNHL differential diagnosis list: 5% + - Best imaging tool + - MR best for SNHL patients + - High-resolution/volumetric T2: Best for surgical anatomy, nerve of origin, & fundal CSF cap in setting of vestibular schwannoma + - T1 C+ T1 fat-saturated: Helps make labyrinthitis, vestibular neuritis, Ramsay Hunt syndrome diagnoses + - Increasing role of pre- & postcontrast 3D FLAIR in assessing sudden (onset < 72 hours) SNHL + - Differentiates vascular (methemoglobin → bright inner ear signal on T1 C- & 3D FLAIR C-) from inflammatory (proteinaceous exudate → bright inner ear signal only on 3D FLAIR C-) etiologies + - Inner ear enhancement on 3D FLAIR C+ identifies blood-labyrinth barrier breakdown +- ## Helpful Clues for Common Diagnoses + + + - **Vestibular Schwannoma** + - Morphology + - "Ice cream on cone" mass aligned with CPA-IAC + - Imaging findings + - T1 C+ MR: Enhancing lesion ± intramural cysts + - High-resolution/volumetric T2: Fundal cap size, relationship of tumor to cochlear nerve canal, & (if small) nerve of origin + - GRE/SWI: ± microhemorrhages with blooming artifact + - **Meningioma in CPA** + - Morphology + - Dural-based mass often asymmetric to porus acusticus + - Imaging findings + - T1 C+ MR: Enhancing mass ± dural tails ± CSF-vascular cleft between mass & brainstem + - **Epidermoid Cyst in CPA** + - Morphology + - Insinuating with brainstem margin + - Imaging findings + - T1 C+ MR: Nonenhancing mass may be difficult to see + - DWI: Reduced diffusivity makes diagnosis + - **Aneurysm in CPA** + - Morphology + - Ovoid or fusiform CPA mass; rarely in IAC + - Imaging findings + - T1 & T1 C+ MR: Complex signal mass from wall calcification, clot, & flow + - MRA, CTA, or catheter angiography confirmatory + - **T-Bone****Fracture** + - T-bone CT essential; imaging findings + - Transverse, longitudinal, or complex fracture crosses inner ear structures ± pneumolabyrinth +- ## Helpful Clues for Less Common Diagnoses + + + - **Cochlear Otosclerosis** + - Pathophysiology: Etiology unknown; osteodystrophy of otic capsule + - Imaging findings + - Bone CT: Radiolucent foci in bony labyrinth + - T1 C+ MR: Multiple enhancing foci in bony labyrinth + - **Metastases in CPA-IAC** + - Imaging findings + - T1 C+ MR: Multiple enhancing lesions involving flocculus, choroid plexus, pia-arachnoid, or dura + - **Facial Nerve Schwannoma in CPA-IAC** + - Imaging findings + - Bone CT: Labyrinthine segment facial nerve canal enlarged = labyrinthine tail + - T1 C+ MR: Enhancing tubular mass affects CPA-IAC & labyrinthine segment of facial nerve + - **Lipoma in CPA-IAC** + - Imaging findings + - NECT: Fatty lesion of CPA, IAC ± inner ear + - T1 MR: High-signal lesion as above; fat saturation suppresses fat signal + - **Large Endolymphatic Sac Anomaly (IP-2)** + - Most common lesion found in children with bilateral congenital SNHL + - Imaging findings + - Bone CT: Large bony vestibular aqueduct ± mild cochlear malformation + - Axial CT: ≥ 1 mm at midpoint, ≥ 2 mm at operculum + - MR: Large endolymphatic sac & duct ± incomplete cochlear partitioning &/or deficient modiolus + - **Intralabyrinthine Schwannoma** + - Name based on anatomic location: Intracochlear, intralabyrinthine, vestibulocochlear, transmodiolar, transmacular, & transotic types + - Imaging findings + - T1 C+ MR: Intralabyrinthine enhancing lesion + - High-resolution T2 MR: Focal filling defect within high-signal intralabyrinthine fluid + - **Labyrinthitis** + - Imaging findings + - T1 C+ MR: Diffuse (less commonly focal) enhancement of labyrinth + - Facial or vestibulocochlear nerves may also enhance + - **Vestibulocochlear****Neuritis** + - Imaging findings: T1 C+ MR: Linear enhancement in CPA-IAC cisterns + - **Paget Disease in T-Bone** + - Clinical: Patient > 50 years of age + - Imaging findings + - Bone CT: Expansile bony lesion with cotton-wool appearance; may involve otic capsule + - T1 C+ MR: Heterogeneous enhancement within expanded T-bone, skull base, & calvarium + - **Fibrous Dysplasia****in****T-Bone** + - Clinical: Patient < 30 years of age + - Imaging findings + - Bone CT: Expansile lesion with ground-glass/sclerotic & cystic components; spares otic capsule + - MR: Expansile lesion with heterogeneous signal + - T1 C+ MR: Heterogeneous avid enhancement mixed with areas of minimal to no enhancement +- ## Helpful Clues for Rare Diagnoses + + + - **Endolymphatic Sac Tumor** + - Tumor centered in endolymphatic duct or sac area of posterior T-bone + - Imaging findings + - Bone CT: Spiculated or coarse calcifications within tumor matrix with thin posterior marginal calcification + - T1 MR: Multifocal high-signal tumor foci (blood products in tumor matrix) + - **Sarcoidosis in CPA** + - Laboratory: CSF lymphocystosis; increased blood angiotensin-converting enzyme (ACE) + - Morphology: En plaque, nodular, or linear masses + - Imaging findings + - T1 C+ MR: Multifocal dural-based enhancing lesions + - **Superficial Siderosis****in****CPA-IAC** + - Clinical: Bilateral SNHL with ataxia + - Imaging findings + - GRE/SWI MR: Blooming dark signal (hemosiderin) along surface of cerebellum & cranial nerves + - MR may also be used to identify site of chronic bleeding: Surgical site, aneurysm, tumor, or arteriovenous malformation, including spin + - **IAC/Temporal Bone Facial Nerve Venous Malformation ("Hemangioma")** + - Imaging findings + - CT: Punctate calcification in IAC lesion + - T1 C+ MR: Enhancing lesion in IAC with focal low-signal areas (calcifications) + - **Ramsay Hunt Syndrome** + - Clinical: External ear vesicles ± CNVII or CNVIII neuropathy + - Imaging findings: T1 C+ MR: Linear enhancing foci in IAC + - **Susac Syndrome (Retinocochleocerebral Vasculopathy)** + - Clinical: Classic triad of branch retinal artery occlusions, SNHL (often with vestibular symptoms), & encephalopathy (including headaches) + - Imaging findings + - T2/FLAIR MR: Multifocal white matter (WM) lesions, often round & involving mid callosal region + - T1 C+ MR: Variable enhancement of WM lesions; ± leptomeningeal enhancement + +## References + +# Selected References + +1. [Coffey N et al: Imaging findings in sensorineural hearing loss: a pictorial essay. Can Assoc Radiol J. 68(2):106-115, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27209216%5Bpmid%5D) +1. [Conte G et al: MR imaging in sudden sensorineural hearing loss. Time to Talk. AJNR Am J Neuroradiol. ePub, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28546251%5Bpmid%5D) +1. [Lee JI et al: Prognostic Value of Labyrinthine 3D-FLAIR Abnormalities in Idiopathic Sudden Sensorineural Hearing Loss. AJNR Am J Neuroradiol. 37(12):2317-2322, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27516239%5Bpmid%5D) +1. [Naganawa S et al: Heavily T₂-Weighted 3D-FLAIR Improves the Detection of Cochlear Lymph Fluid Signal Abnormalities in Patients with Sudden Sensorineural Hearing Loss. Magn Reson Med Sci. 15(2):203-11, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26597430%5Bpmid%5D) +1. [Pakdaman MN et al: Blood-Labyrinth Barrier Permeability in Menière Disease and Idiopathic Sudden Sensorineural Hearing Loss: Findings on Delayed Postcontrast 3D-FLAIR MRI. AJNR Am J Neuroradiol. ePub, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27256854%5Bpmid%5D) +1. [Cerqueira AC et al: Superficial siderosis of the central nervous system: an unusual cause of sensorineural hearing loss. Arq Neuropsiquiatr. 68(3):469-71, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20602058%5Bpmid%5D) +1. [Goyault G et al: Leptomeningeal carcinomatosis and sensorineural hearing loss: correlation of labyrinthine enhancement patterns with symptoms. J Neuroradiol. 36(2):98-101, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19144408%5Bpmid%5D) +1. [Thamburaj K et al: Intratumoral microhemorrhages on T2*-weighted gradient-echo imaging helps differentiate vestibular schwannoma from meningioma. AJNR Am J Neuroradiol. 29(3):552-7, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18079187%5Bpmid%5D) +1. [Daniels RL et al: Causes of unilateral sensorineural hearing loss screened by high-resolution fast spin echo magnetic resonance imaging: review of 1,070 consecutive cases. Am J Otol. 21(2):173-80, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10733180%5Bpmid%5D) +1. [Davidson HC et al: MR evaluation of vestibulocochlear anomalies associated with large endolymphatic duct and sac. AJNR Am J Neuroradiol. 20(8):1435-41, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10512225%5Bpmid%5D) +1. [Swartz JD: Sensorineural hearing deficit: a systematic approach based on imaging findings. Radiographics. 16(3):561-74, 1996](http://www.ncbi.nlm.nih.gov/pubmed/?term=8897624%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial T1 C+ FS MR shows the typical appearance of a CPA-IAC vestibular schwannoma with avid, heterogeneous enhancement. Note tumor growth along the location of the vestibular nerve within the posterior IAC .](images/app.statdx.com_image_thumbnail_d3735149-87ba-4946-a6be-f0b1f08d305f_annotated_true_size_900_quality_90_530bc584132808c88933cbc23f234521e5322f13.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ FS MR shows the typical appearance of a CPA-IAC vestibular schwannoma with avid, heterogeneous enhancement. Note tumor growth along the location of the vestibular nerve within the posterior IAC .* + +![Axial T1 C+ FS MR shows the typical appearance of a CPA-IAC vestibular schwannoma with avid, heterogeneous enhancement. Note tumor growth along the location of the vestibular nerve within the posterior IAC .](images/app.statdx.com_image_thumbnail_d3735149-87ba-4946-a6be-f0b1f08d305f_size_174_quality_85_30b429a2c8e4193029a090b3e5ed7c18acf8e4ab.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ FS MR shows the typical appearance of a CPA-IAC vestibular schwannoma with avid, heterogeneous enhancement. Note tumor growth along the location of the vestibular nerve within the posterior IAC .* + +![Axial SWI MR in a CPA-IAC vestibular schwannoma shows punctate foci of gradient susceptibility from microhemorrhage, a feature that can help distinguish CPA-IAC schwannoma from meningioma.](images/app.statdx.com_image_thumbnail_0bd14ef9-fa2b-49f4-a274-290f0a016831_annotated_true_size_900_quality_90_8295787439dc0641cc294b36bcc813f2b7179fbb.jpg) +**Vestibular Schwannoma** +*Axial SWI MR in a CPA-IAC vestibular schwannoma shows punctate foci of gradient susceptibility from microhemorrhage, a feature that can help distinguish CPA-IAC schwannoma from meningioma.* + +![Axial T1 C+ FS MR shows the typical appearance of a CPA meningioma , which is avidly enhancing and extends into the IAC . Note the characteristic dural tails , which help differentiate meningioma from schwannoma.](images/app.statdx.com_image_thumbnail_70e6386b-5773-4029-affe-fef0f880c417_annotated_true_size_900_quality_90_54baf818a25ca5b2af6b437a1b21754f1792a14b.jpg) +**Meningioma in CPA** +*Axial T1 C+ FS MR shows the typical appearance of a CPA meningioma , which is avidly enhancing and extends into the IAC . Note the characteristic dural tails , which help differentiate meningioma from schwannoma.* + +![Axial DTI trace image through the posterior fossa shows a typical epidermoid cyst with reduced diffusivity. Note the scalloped, insinuating margins , which frequently encase cranial nerves and vessels and can make surgical resection fraught with peril.](images/app.statdx.com_image_thumbnail_51a8ca03-d555-4a16-99d3-de9bccdefbfd_annotated_true_size_900_quality_90_65794016e65a6f10a04217089ade2948c54a0148.jpg) +**Epidermoid Cyst in CPA** +*Axial DTI trace image through the posterior fossa shows a typical epidermoid cyst with reduced diffusivity. Note the scalloped, insinuating margins , which frequently encase cranial nerves and vessels and can make surgical resection fraught with peril.* + +![Axial T2WI MR shows a CPA vertebral artery aneurysm as an ovoid mass with complex wall signal, which bows the vestibulocochlear nerve posterolaterally.](images/app.statdx.com_image_thumbnail_0a837932-9add-4268-8d4b-971743940ea0_annotated_true_size_900_quality_90_95859beecf4cdc34a5d2e7593427c4e9d7713a8b.jpg) +**Aneurysm in CPA** +*Axial T2WI MR shows a CPA vertebral artery aneurysm as an ovoid mass with complex wall signal, which bows the vestibulocochlear nerve posterolaterally.* + +![Axial bone CT shows a complex, transversely oriented T-bone fracture that involves the otic capsule and disrupts the cochlea . The mastoid air cells and middle ear are opacified with blood, corresponding with hemotympanum on otoscopy.](images/app.statdx.com_image_thumbnail_9431d1b5-a003-4659-99d1-5b5ef004bcc4_annotated_true_size_900_quality_90_6645df77c4bec2466807341ea1fde3f48f769f98.jpg) +**T-Bone Fracture** +*Axial bone CT shows a complex, transversely oriented T-bone fracture that involves the otic capsule and disrupts the cochlea . The mastoid air cells and middle ear are opacified with blood, corresponding with hemotympanum on otoscopy.* + +![Axial bone CT shows cochlear otosclerosis yielding SNHL. Note confluent otic capsule lucency adjacent to the cochlea. Patients commonly have fenestral involvement with lucency near the fissula ante fenestram , contributing a conductive component to hearing loss. Note the prior partial ossicular replacement prosthesis .](images/app.statdx.com_image_thumbnail_2f5b9b56-95df-4317-a7c4-3261238b0298_annotated_true_size_900_quality_90_49d67826aa09f54e625b4011cbdd996e7c73e446.jpg) +**Cochlear Otosclerosis** +*Axial bone CT shows cochlear otosclerosis yielding SNHL. Note confluent otic capsule lucency adjacent to the cochlea. Patients commonly have fenestral involvement with lucency near the fissula ante fenestram , contributing a conductive component to hearing loss. Note the prior partial ossicular replacement prosthesis .* + +![Axial FLAIR C+ MR shows leptomeningeal metastases from melanoma with enhancement at the fundus of bilateral IACs extending into the basal turn of the cochlea bilaterally.](images/app.statdx.com_image_thumbnail_cfa29a94-43b0-46fa-af87-716ba93681a3_annotated_true_size_900_quality_90_094d741ec6175b6947d8eafbbeacee6cfc359c46.jpg) +**Metastases in CPA-IAC** +*Axial FLAIR C+ MR shows leptomeningeal metastases from melanoma with enhancement at the fundus of bilateral IACs extending into the basal turn of the cochlea bilaterally.* + +![Axial T1 C+ SPGR MR shows a facial nerve schwannoma involving the left CPA-IAC. In this case, the labyrinthine tail of enhancement extends to the asymmetrically enhancing geniculate ganglion region , following the expected course of the facial nerve.](images/app.statdx.com_image_thumbnail_969ba618-203a-4678-947a-838f2f82db8e_annotated_true_size_900_quality_90_00460a6660590d9a3efe8e5b2908766829a7f2db.jpg) +**Facial Nerve Schwannoma in CPA-IAC** +*Axial T1 C+ SPGR MR shows a facial nerve schwannoma involving the left CPA-IAC. In this case, the labyrinthine tail of enhancement extends to the asymmetrically enhancing geniculate ganglion region , following the expected course of the facial nerve.* + +![Coronal T1 MR of a left CPA-IAC lipoma shows a T1-bright lobular mass extending into the IAC . The presence of chemical shift artifact at the tumor-CSF (i.e., water) margin confirms the presence of fat within this mass.](images/app.statdx.com_image_thumbnail_d1411935-09f1-44a5-bca4-52ed8b3169c2_annotated_true_size_900_quality_90_6bd538609d6cfeee36ae04b7ec6dbe300a810037.jpg) +**Lipoma in CPA-IAC** +*Coronal T1 MR of a left CPA-IAC lipoma shows a T1-bright lobular mass extending into the IAC . The presence of chemical shift artifact at the tumor-CSF (i.e., water) margin confirms the presence of fat within this mass.* + +![Axial T2WI FS MR shows a large endolymphatic sac along the posterior wall of the T-bone associated with a malformed cochlea (modiolar deficiency, incomplete apical septation, and bulbous apical turn) .](images/app.statdx.com_image_thumbnail_2dc85c37-ba3a-48ae-8599-2d27b5b9e24b_annotated_true_size_900_quality_90_172b78c489e25062b02d4948f01686f9ce8c2d41.jpg) +**Large Endolymphatic Sac Anomaly (IP-2)** +*Axial T2WI FS MR shows a large endolymphatic sac along the posterior wall of the T-bone associated with a malformed cochlea (modiolar deficiency, incomplete apical septation, and bulbous apical turn) .* + +![Coronal T1 C+ FS MR of an intracochlear schwannoma shows abnormal enhancement in the middle turn of the right cochlea . Note the normal postcontrast appearance of the contralateral cochlea for comparison .](images/app.statdx.com_image_thumbnail_de832bd1-399c-4dc1-bff0-35389fe7abac_annotated_true_size_900_quality_90_4b8cbcc566709a760574b794561cc6cda3864fe9.jpg) +**Intralabyrinthine Schwannoma** +*Coronal T1 C+ FS MR of an intracochlear schwannoma shows abnormal enhancement in the middle turn of the right cochlea . Note the normal postcontrast appearance of the contralateral cochlea for comparison .* + +![Axial T1 C+ FS MR in a patient with acute otomastoiditis complicated by labyrinthitis shows enhancement in the cochlea . There is additional vestibulocochlear neuritis with linear enhancement at the IAC fundus . Note enhancing mastoid/middle ear disease and dural enhancement due to marked inflammation.](images/app.statdx.com_image_thumbnail_339d6bcb-3739-4e06-8be4-a945b3e4d948_annotated_true_size_900_quality_90_2dcc16a8e0232f44a5755936e65bd2492e198879.jpg) +**Labyrinthitis** +*Axial T1 C+ FS MR in a patient with acute otomastoiditis complicated by labyrinthitis shows enhancement in the cochlea . There is additional vestibulocochlear neuritis with linear enhancement at the IAC fundus . Note enhancing mastoid/middle ear disease and dural enhancement due to marked inflammation.* + +![Axial T1 C+ FS MR shows vestibulocochlear neuritis in a patient with rapid onset of sensorineural hearing loss. Note the area of linear enhancement in the proximal IAC .](images/app.statdx.com_image_thumbnail_c3b665a2-294f-4422-947c-7f50d4af02b6_annotated_true_size_900_quality_90_426b5fe141e20ad6f61bb5aef845d8dc3c0aad49.jpg) +**Vestibulocochlear Neuritis** +*Axial T1 C+ FS MR shows vestibulocochlear neuritis in a patient with rapid onset of sensorineural hearing loss. Note the area of linear enhancement in the proximal IAC .* + + +### Additional Images + +![Axial bone CT shows expansile ground-glass density focus of fibrous dysplasia affecting the squamous, tympanic, & mastoid portions of the T-bone. Note relative sparing of the otic capsule .](images/app.statdx.com_image_thumbnail_b890e09a-c5bf-4dab-a3a1-1b88aff7ef3c_annotated_true_size_900_quality_90_bff9d3b6b07fb7e78b874d77f647fb63baa7dc7a.jpg) +**Fibrous Dysplasia in T-Bone** +*Axial bone CT shows expansile ground-glass density focus of fibrous dysplasia affecting the squamous, tympanic, & mastoid portions of the T-bone. Note relative sparing of the otic capsule .* + +![Axial T1WI MR reveals a tumor along the posterior wall of the T-bone with high-signal foci that is highly suggestive of an endolymphatic sac tumor.](images/app.statdx.com_image_thumbnail_c831d839-e038-4fc7-9526-4368043b8af4_annotated_true_size_900_quality_90_6ed4adc56ea49d102b206a3127d7da669fad0226.jpg) +**Endolymphatic Sac Tumor** +*Axial T1WI MR reveals a tumor along the posterior wall of the T-bone with high-signal foci that is highly suggestive of an endolymphatic sac tumor.* + +![Axial T1 C+ FS MR demonstrates thick enhancing dural-based sarcoidosis in the left CPA and IAC mimicking en plaque meningioma.](images/app.statdx.com_image_thumbnail_214e880c-ad77-4a3c-a4b8-9b63604e2af6_annotated_true_size_900_quality_90_0d468171485b91720da12a07a5450b0e5b3ebb6c.jpg) +**Sarcoidosis in CPA** +*Axial T1 C+ FS MR demonstrates thick enhancing dural-based sarcoidosis in the left CPA and IAC mimicking en plaque meningioma.* + +![Axial T2* GRE MR shows superficial siderosis on the surface of the posterior fossa structures (linear low signal), including cerebellar folia , flocculi , and vestibulocochlear nerves .](images/app.statdx.com_image_thumbnail_58bdb917-5a74-4472-97cc-2e063855266c_annotated_true_size_900_quality_90_a1870ad66097bcad83056a8deefed525ca1b9b8f.jpg) +**Superficial Siderosis in CPA-IAC** +*Axial T2* GRE MR shows superficial siderosis on the surface of the posterior fossa structures (linear low signal), including cerebellar folia , flocculi , and vestibulocochlear nerves .* + +![Axial bone CT reveals punctate calcifications in the IAC suggesting the diagnosis of IAC hemangioma. Enhanced MR showed an enhancing mass in this location.](682ad916-140b-4f22-91cc-d5b14bce6a63) +**IAC/Temporal Bone Facial Nerve Venous Malformation ("Hemangioma")** +*Axial bone CT reveals punctate calcifications in the IAC suggesting the diagnosis of IAC hemangioma. Enhanced MR showed an enhancing mass in this location.* + +![Axial T1 C+ MR shows area of crescentic enhancement in the IAC fundus associated with tympanic segment of the facial nerve asymmetric enhancement . EAC vesicles were present, characteristic of Ramsey Hunt syndrome.](images/app.statdx.com_image_thumbnail_948ebf8d-6a05-4d9a-96b8-bb5065b417ec_annotated_true_size_900_quality_90_a5110776d39e3188ddf8a8a43edb1563ee8104eb.jpg) +**Ramsay Hunt Syndrome** +*Axial T1 C+ MR shows area of crescentic enhancement in the IAC fundus associated with tympanic segment of the facial nerve asymmetric enhancement . EAC vesicles were present, characteristic of Ramsey Hunt syndrome.* + +![Coronal T2 FS MR in a patient with retinocochleocerebral vasculopathy (Susac syndrome) shows a characteristic round, hyperintense lesion in the splenium of the corpus callosum .](images/app.statdx.com_image_thumbnail_2c25695f-f944-4b52-b897-dc78ab64ba7b_annotated_true_size_900_quality_90_2db0ec3ee26194654aa6533c55943aa522111326.jpg) +**Susac Syndrome (Retinocochleocerebral Vasculopathy)** +*Coronal T2 FS MR in a patient with retinocochleocerebral vasculopathy (Susac syndrome) shows a characteristic round, hyperintense lesion in the splenium of the corpus callosum .* + +![Axial T1 C+ FS MR shows typical mid sized CPA-IAC vestibular schwannoma. "Ice cream" (CPA component) "on cone" (IAC component) morphology is highly suggestive of this diagnosis.](images/app.statdx.com_image_thumbnail_efacd024-ee59-4fe5-ab29-e8be4e5f7528_annotated_true_size_900_quality_90_7b0f26c4787a1f3f5563a4cd48e8b8c85a25ae5c.jpg) +**Vestibular Schwannoma** +*Axial T1 C+ FS MR shows typical mid sized CPA-IAC vestibular schwannoma. "Ice cream" (CPA component) "on cone" (IAC component) morphology is highly suggestive of this diagnosis.* + +![Axial T1 C+ FS MR shows the sessile morphology of meningioma as it "sits" on the posterior T-bone wall. Note the characteristic dural tail and dural artery feeding the tumor center.](images/app.statdx.com_image_thumbnail_e4b012d4-7edd-4ad8-ab9e-8965e2812505_annotated_true_size_900_quality_90_a10d60245c8f87d817be68823dd4e15d306d1333.jpg) +**Meningioma in CPA** +*Axial T1 C+ FS MR shows the sessile morphology of meningioma as it "sits" on the posterior T-bone wall. Note the characteristic dural tail and dural artery feeding the tumor center.* + +![Axial DWI MR shows a CPA epidermoid identified by its reduced diffusivity . CPA epidermoids often are not directly over the porus acusticus, as in this case.](images/app.statdx.com_image_thumbnail_fbe77c22-33bb-49c1-a1ff-fab6210cdff1_annotated_true_size_900_quality_90_21b23bf119d739735c3ddc5d274862a13b92c981.jpg) +**Epidermoid Cyst in CPA** +*Axial DWI MR shows a CPA epidermoid identified by its reduced diffusivity . CPA epidermoids often are not directly over the porus acusticus, as in this case.* + +![Axial bone CT shows an oblique T-bone fracture traversing the oval window and extending to the IAC area . Notice the air bubble (pneumolabyrinth) along the anterior margin of the vestibule .](images/app.statdx.com_image_thumbnail_d895c83e-9b53-42db-a384-fe6ff8e2e66b_annotated_true_size_900_quality_90_1b2d0f3d5c8bc9d34ccfcde26c8e720d9b71db42.jpg) +**T-Bone Fracture** +*Axial bone CT shows an oblique T-bone fracture traversing the oval window and extending to the IAC area . Notice the air bubble (pneumolabyrinth) along the anterior margin of the vestibule .* + +![Axial bone CT shows severe otosclerosis as radiolucent foci along the medial middle ear wall (fenestral otosclerosis) and within the bony labyrinth (cochlear otosclerosis).](images/app.statdx.com_image_thumbnail_774aa646-bf33-4be9-8ea9-fdf0302936e3_annotated_true_size_900_quality_90_aa35f7fc9309bbaadd6b8bf720edaa3483fc3c5b.jpg) +**Cochlear Otosclerosis** +*Axial bone CT shows severe otosclerosis as radiolucent foci along the medial middle ear wall (fenestral otosclerosis) and within the bony labyrinth (cochlear otosclerosis).* + +![Axial T2WI MR demonstrates a right floccular metastasis with associated high-signal cerebellar and brachium pontis edema. A normal left flocculus is also seen.](images/app.statdx.com_image_thumbnail_f44ac490-2a9f-403e-a381-a22c3cb6bca5_annotated_true_size_900_quality_90_2d45c68c58907891f264824fc0d3f2fd0c52869e.jpg) +**Metastases in CPA-IAC** +*Axial T2WI MR demonstrates a right floccular metastasis with associated high-signal cerebellar and brachium pontis edema. A normal left flocculus is also seen.* + +![Axial T1 C+ FS MR reveals an enhancing facial nerve schwannoma traversing the CPA and IAC into the facial nerve labyrinthine segment . This labyrinthine tail is characteristic.](images/app.statdx.com_image_thumbnail_b8497005-8071-4cb6-b5bc-cfb107cf7208_annotated_true_size_900_quality_90_edf91089289276b94becf49922e9c0851205bb37.jpg) +**Facial Nerve Schwannoma in CPA-IAC** +*Axial T1 C+ FS MR reveals an enhancing facial nerve schwannoma traversing the CPA and IAC into the facial nerve labyrinthine segment . This labyrinthine tail is characteristic.* + +![Axial T1 MR demonstrates a CPA and intravestibular lipoma. Notice that the CPA lipoma effaces the most proximal vestibulocochlear nerve bundle . These lesions are left alone.](images/app.statdx.com_image_thumbnail_2c109ca4-0f02-41c1-aeea-19557fe24fca_annotated_true_size_900_quality_90_c93f09e5c60022746a52182010222017928233ee.jpg) +**Lipoma in CPA-IAC** +*Axial T1 MR demonstrates a CPA and intravestibular lipoma. Notice that the CPA lipoma effaces the most proximal vestibulocochlear nerve bundle . These lesions are left alone.* + +![Axial T1 C+ FS MR demonstrates a nodule of enhancing tissue in the vestibule of the inner ear secondary to intralabyrinthine schwannoma. CT of the T-bone was normal.](images/app.statdx.com_image_thumbnail_d24b69e3-ba59-435d-8ff3-d121f4267cbd_annotated_true_size_900_quality_90_3492fa32f8e4d18387ea40d3b5cd1686b80431e7.jpg) +**Intralabyrinthine Schwannoma** +*Axial T1 C+ FS MR demonstrates a nodule of enhancing tissue in the vestibule of the inner ear secondary to intralabyrinthine schwannoma. CT of the T-bone was normal.* + +![Axial bone CT shows late-phase Paget disease with diffuse bony expansion with areas of demineralization . Notice that the bony labyrinth along the anterior cochlear surface is involved .](images/app.statdx.com_image_thumbnail_b22f9a49-efdd-4256-95a3-70cd3e9ef7e9_annotated_true_size_900_quality_90_0999b638e160de5bf8fb3d2cc5e13425b48da2be.jpg) +**Paget Disease in T-Bone** +*Axial bone CT shows late-phase Paget disease with diffuse bony expansion with areas of demineralization . Notice that the bony labyrinth along the anterior cochlear surface is involved .* + +![Axial T1 C+ FS MR shows enhancement of the middle ear , inner ear membranous labyrinth , and IAC in this pediatric patient with acute actinomycosis.](images/app.statdx.com_image_thumbnail_bc87da43-c7de-4d9b-a323-ffdfca7d9467_annotated_true_size_900_quality_90_81fce77b316959b2cd2c0c8b793e3bcaa204bade.jpg) +**Labyrinthitis** +*Axial T1 C+ FS MR shows enhancement of the middle ear , inner ear membranous labyrinth , and IAC in this pediatric patient with acute actinomycosis.* + diff --git a/docs_md/articles/sensorineural-hearing-loss-in-child_08c895da-f2aa-4076-abf0-af9aca1677cd.md b/docs_md/articles/sensorineural-hearing-loss-in-child_08c895da-f2aa-4076-abf0-af9aca1677cd.md new file mode 100644 index 0000000..70dde9b --- /dev/null +++ b/docs_md/articles/sensorineural-hearing-loss-in-child_08c895da-f2aa-4076-abf0-af9aca1677cd.md @@ -0,0 +1,377 @@ +--- +title: "Sensorineural Hearing Loss in Child" +docid: "08c895da-f2aa-4076-abf0-af9aca1677cd" +authors: + - key: "d19354f3-7ff2-495a-ad3f-064122e45602" + value: "Bernadette L. Koch, MD" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c" + - + name: "CPA-IAC and Posterior Fossa" + slug: "cpa-iac-and-posterior-fossa" + treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992" + - + name: "Clinically Based Differentials" + slug: "clinically-based-differentials" + treeNodeId: "55dd15ac-e67d-48dd-8134-f52884dab28b" + - + name: "Sensorineural Hearing Loss in Child" + slug: "sensorineural-hearing-loss-in-child" + treeNodeId: null +category: "Head and Neck" +documentVersionId: "89f04762-2cc9-4c62-aea2-256ed544510c" +imageCount: 44 +lastUpdated: "07/24/18" +pageDescription: "Sensorineural Hearing Loss in Child" +pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Clinically Based Differentials, Sensorineural Hearing Loss in Child" +pageTitle: "Sensorineural Hearing Loss in Child | STATdx" +enhancedTitle: "Sensorineural Hearing Loss in Child" +type: "DDX" +references: true +breadcrumbs: + - "Head and Neck" + - "Differential Diagnosis" + - "CPA-IAC and Posterior Fossa" + - "Clinically Based Differentials" + - "Sensorineural Hearing Loss in Child" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - History is important + - **Congenital sensorineural hearing loss (SNHL)** + - Failed newborn screening hearing test + - Look for inner ear anomalies with CT &/or MR + - **Fluctuating or "cascading" SNHL**in child (without history of meningitis) + - Look for large vestibular aqueduct (LVA) ± cochlear malformation & modiolar deficiency on CT + - Look for large endolymphatic sac/duct ± cochlear malformation & modiolar deficiency on MR + - **Congenital unilateral SNHL** + - Look for cochlear nerve & cochlear nerve canal (CNC) hypoplasia/aplasia + - **Trauma** + - Look for fracture involving inner ear structures ± pneumolabyrinth on CT + - **Genetic****disorders with common imaging findings** + - CHARGE, trisomy 21, Waardenburg, or Apert syndrome: Look for semicircular canal (SCC) malformation + - Pendred syndrome: Look for LVA + - Biallelic SLC26A4 mutations + - **Prior****meningitis** + - CT: Look for labyrinthine ossification + - T1 C+ MR: Labyrinthine enhancement during meningitis (acute phase) + - T2 MR: Normal high intensity replaced with low-intensity (fibrosis or ossification) membranous labyrinth + - Best imaging tool + - Thin-section T-bone CT for congenital inner ear anomalies & labyrinthine ossificans + - High-resolution T2 MR for large endolymphatic sac, cochlear malformation; cochlear nerve aplasia/hypoplasia + - C+ MR best for schwannoma, other tumors, acute labyrinthitis, & autoimmune labyrinthitis (delayed enhancement) +- ## Helpful Clues for Common Diagnoses + + + - **Large Endolymphatic Sac Anomaly (IP-****II****)** + - Most common congenital anomaly of inner ear found by imaging + - Axial CT: Vestibular aqueduct (VA) ≥ 2 mm at operculum or ≥ 1 mm at midpoint + - Associated with incomplete cochlear partition type II (IP-II), modiolar deficiency, vestibule, &/or SCC malformation + - Additional prognostic information + - **Avoid contact sports** or other activities that may lead to head trauma + - Genetic testing for**SLC26A4 mutation** recommended + - Up to 40% of all patients with LVA & IP-II will have pendrin gene mutation: Pendred syndrome (with thyroid organification defect ± goiter) or LVA + - **T-Bone Fractures** + - Thin-section T-bone CT (0.625-1 mm) + - Transverse, longitudinal, or complex fractures may cross inner ear structures, ± pneumolabyrinth + - Otic capsule violating vs. otic capsule sparing + - **Semicircular Canal Malformation** + - Spectrum of abnormalities: **≥ 1**SCC is malformed, hypoplastic, or aplastic + - Unilateral or bilateral (e.g., syndromic cases) + - Most common is short, dilated lateral SCC & vestibule forming single cavity or with small bone island + - ± cochlear malformation, oval window atresia, &/or ossicular anomalies + - **CHARGE** syndrome + - **Bilatera****l****hypoplasia or****absence of all SCCs** + - Associated anomalies: Small vestibule, absent cochlear nerve aperture ("isolated cochlea"), oval window atresia (± overlying tympanic segment of CNVII), ± choanal atresia, ± coloboma + - Lateral SCC last to form embryologically; anomalies most frequently affect lateral SCC + - **Except** if obliterated by labyrinthine ossificans or malformed in Waardenburg, branchiootorenal, & Alagille syndromes + - **Labyrinthine Ossificans** + - Synonyms: Labyrinthitis ossificans, labyrinthine ossification, chronic labyrinthitis, ossifying labyrinthitis + - Acute inflammatory response → fibrous & then osseous replacement of membranous labyrinth; weeks or years + - May involve cochlea ± vestibule ± SCCs + - **Bilateral in meningogenic** form (meningitis) & in hematogenic form (blood-borne infections) + - **Unilateral in tympanogenic** form (middle ear infection) + - T-bone CT: High-attenuation bone deposition in formerly fluid-filled membranous labyrinth + - Areas of ossification crucial to identify when planning cochlear implantation + - T2 MR: Focal or diffuse low intensity replaces high-intensity fluid, with apparent "enlargement" of modiolus if cochlea is involved + - T1 C+ MR: Enhancement of involved membranous labyrinth structures in early stage, may persist into ossifying stages +- ## Helpful Clues for Less Common Diagnoses + + + - **Labyrinthitis** + - Sudden onset of SNHL, vertigo, &/or tinnitus + - Viral disease: Imaging usually not indicated + - Bacterial, posttraumatic, or autoimmune causes + - Subacute inflammation of fluid-filled inner ear structures + - T-bone CT: Normal in early phases, may progress to labyrinthine ossificans + - T2 MR: Low intensity replaces normal high-intensity fluid signal within membranous labyrinth structures + - T1 C+ MR: Mild to moderate enhancement + - Enhancement may persist after symptoms resolve + - **Cochlear Nerve & Cochlear Nerve Canal Aplasia-Hypoplasia** + - CT: Small IAC & CNC + - MR: Fluid in CNC completely or partially replaced by low-signal bone + - Cochlear nerve diminutive or absent (if CNC aplasia) + - **Cystic****C****ochleovestibular Malformation (IP-I)** + - Cystic, featureless cochlea + dilated vestibule & horizontal SCC + - Cochlea: Absent internal septation & absent modiolus (IP-1), cochlea & vestibule form bilobed cyst + - Vestibule: Dilated, large, communicates with cochlea + - SCC: Dilated horizontal + vestibule → common cavity + - IAC: Small or dilated, defective fundus + - VA: Usually normal + - **C****PA-IAC****Lipoma** + - Congenital fatty lesion of CPA ± IAC ± inner ear + - Caveat: If T1 C+ MR without fat saturation, may be mistaken for vestibular schwannoma +- ## Helpful Clues for Rare Diagnoses + + + - **Vestibular Schwannoma** + - Enhancing lesion in IAC or ice cream on cone-shaped mass aligned with CPA-IAC + - Hypointense T2WI MR + - **I****ntralabyrinthine****Schwannoma** + - Rare in children + - **Intracochlear**: Schwannoma within cochlea + - **Intravestibular**: Schwannoma within vestibule + - **Vestibulocochlear**: Involves both vestibule & cochlea + - **Transmodiolar**: Crosses modiolus; cochlea to IAC fundus + - **Transmacular**: Crosses from vestibule into IAC fundus + - **Transotic**: Crosses entire inner ear from IAC fundus to middle ear + - **F****acial Nerve****Schwannoma in CPA-IAC** + - Rare in children + - SNHL with associated facial neuropathy + - Enhancing, well-circumscribed mass in CPA-IAC; extends into labyrinthine segment of CNVII + - Involvement of inner ear is secondary finding + - **Common Cavity Malformation** + - Featureless common cavity represents rudimentary cochlea, vestibule, & lateral SCC + - Variably sized common cavity + - Posterior & superior SCC: Absent, normal, or malformed + - IAC: Often small with defective fundus ± anomalous course & small or absent CNVIII components + - Middle ear space & ossicles: Normal or anomalous stapes & stenotic oval window + - VA: Normal or absent + - **Cochlear Aplasia** + - Absent cochlea with variable deformity of vestibule & SCCs + - Absent/flattened cochlear promontory helps differentiate from labyrinthine ossificans + - Absent CNC & nerve + - Hypoplastic IAC + - Normal VA & normal-sized middle ear cavity + - Normal or malformed stapes + - **Labyrinthine Aplasia** + - **Absent cochlea, vestibule**, &**SCCs** + - Absent/flattened cochlear promontory + - IAC aplasia/hypoplasia + - Absent vestibular & cochlear nerves + - Normal or malformed ossicles/middle ear + - Normal or absent carotid canal + - Old synonym: Michel anomaly + +## References + +# Selected References + +1. [Conte G et al: MR Imaging in Sudden Sensorineural Hearing Loss. Time to Talk. AJNR Am J Neuroradiol. ePub, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28546251%5Bpmid%5D) +1. [Johnson K et al: High-frequency sensorineural hearing loss in children. Laryngoscope. 126(5):1236-40, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26266337%5Bpmid%5D) +1. [Shupak A et al: Primary solitary intralabyrinthine schwannoma: A report of 7 cases and a review of the literature. Ear Nose Throat J. 95(12):481-491, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27929596%5Bpmid%5D) +1. [Prosser JD et al: Diagnostic evaluation of children with sensorineural hearing loss. Otolaryngol Clin North Am. 48(6):975-82, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26429334%5Bpmid%5D) +1. [Kenna MA et al: Temporal bone abnormalities in children with GJB2 mutations. Laryngoscope. 121(3):630-5, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21298644%5Bpmid%5D) +1. [Ozgen B et al: Comparison of 45 degrees oblique reformats with axial reformats in CT evaluation of the vestibular aqueduct. AJNR Am J Neuroradiol. 29(1):30-4, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=17947373%5Bpmid%5D) +1. [Vijayasekaran S et al: When is the vestibular aqueduct enlarged? A statistical analysis of the normative distribution of vestibular aqueduct size. AJNR Am J Neuroradiol. 28(6):1133-8, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17569973%5Bpmid%5D) +1. [Sennaroglu L et al: A new classification for cochleovestibular malformations. Laryngoscope. 112:2230-41, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12461346%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial bone CT in an 8-year-old child with severe bilateral sensorineural hearing loss (SNHL) shows incomplete partitioning between the middle and apical turns of the left cochlea , typical of IP-II morphology.](images/app.statdx.com_image_thumbnail_d09145d9-8022-4f26-8ead-38abff08e159_annotated_true_size_900_quality_90_1da4c6d97cf0ae1b56e24ab96964102b2ea3e50b.jpg) +**Large Endolymphatic Sac Anomaly (IP-II)** +*Axial bone CT in an 8-year-old child with severe bilateral sensorineural hearing loss (SNHL) shows incomplete partitioning between the middle and apical turns of the left cochlea , typical of IP-II morphology.* + +![Axial bone CT in an 8-year-old child with severe bilateral sensorineural hearing loss (SNHL) shows incomplete partitioning between the middle and apical turns of the left cochlea , typical of IP-II morphology.](images/app.statdx.com_image_thumbnail_d09145d9-8022-4f26-8ead-38abff08e159_size_174_quality_85_d8fed1c3682e721e49ac404f7f75130f9a53644f.jpg) +**Large Endolymphatic Sac Anomaly (IP-II)** +*Axial bone CT in an 8-year-old child with severe bilateral sensorineural hearing loss (SNHL) shows incomplete partitioning between the middle and apical turns of the left cochlea , typical of IP-II morphology.* + +![Axial bone CT in the same patient shows a markedly enlarged left vestibular aqueduct , much larger than 2 mm at the operculum.](images/app.statdx.com_image_thumbnail_f47ec3d8-3dbb-4b2c-94e3-6b9e0125ee8c_annotated_true_size_900_quality_90_3e5fab4c21ddce05207bd8048f3557451eeaf1d9.jpg) +**Large Endolymphatic Sac Anomaly (IP-II)** +*Axial bone CT in the same patient shows a markedly enlarged left vestibular aqueduct , much larger than 2 mm at the operculum.* + +![Axial FIESTA image in a 3 year old with SNHL shows bilateral enlargement of the endolymphatic sacs , incomplete cochlear partitioning , and mildly dysmorphic vestibules . SCL26A4 mutation/Pendred syndrome was found negative.](images/app.statdx.com_image_thumbnail_633cf7ba-3589-4e2f-98c7-8a8a2cb72c9d_annotated_true_size_900_quality_90_e96d0a174a2ebc338815f1b25e7db9d07533bcc7.jpg) +**Large Endolymphatic Sac Anomaly (IP-II)** +*Axial FIESTA image in a 3 year old with SNHL shows bilateral enlargement of the endolymphatic sacs , incomplete cochlear partitioning , and mildly dysmorphic vestibules . SCL26A4 mutation/Pendred syndrome was found negative.* + +![Axial bone CT in a 14 year old involved in a dirt bike accident reveals a horizontal, otic capsule violating right temporal bone fracture crossing the vestibule . Notice air in the vestibule and cochlea as well as multiple skull base and orbital fractures .](images/app.statdx.com_image_thumbnail_40512a19-efa9-46da-a4d1-50cb33ee82f6_annotated_true_size_900_quality_90_1fa83c749c0638d583b1fc3279548e94386abc40.jpg) +**T-Bone Fractures** +*Axial bone CT in a 14 year old involved in a dirt bike accident reveals a horizontal, otic capsule violating right temporal bone fracture crossing the vestibule . Notice air in the vestibule and cochlea as well as multiple skull base and orbital fractures .* + +![Axial bone CT in a 6-year-old girl demonstrates a short, dilated lateral semicircular canal (SCC) forming a single cavity with the vestibule , a common type of SCC anlage malformation.](images/app.statdx.com_image_thumbnail_28666f0e-2cbd-467b-8a82-4e0b0bfc1838_annotated_true_size_900_quality_90_4ba132bb4e68fc35ccf253b02c23ef46a94e92c3.jpg) +**Semicircular Canal Malformation** +*Axial bone CT in a 6-year-old girl demonstrates a short, dilated lateral semicircular canal (SCC) forming a single cavity with the vestibule , a common type of SCC anlage malformation.* + +![Axial bone CT in a 3-year-old child with a history of bilateral choanal atresia and hearing loss demonstrates diminutive vestibules and absence of the SCCs bilaterally, which is the characteristic appearance of the labyrinth in children with CHARGE syndrome.](ec141323-4ceb-4a97-aa8b-b721b8f57ebc) +**Semicircular Canal Malformation** +*Axial bone CT in a 3-year-old child with a history of bilateral choanal atresia and hearing loss demonstrates diminutive vestibules and absence of the SCCs bilaterally, which is the characteristic appearance of the labyrinth in children with CHARGE syndrome.* + +![Axial bone CT in a child with Alagille syndrome shows a relatively normal caliber posterior limb of the left superior SCC , aplasia of the anterior limb of the superior SCC , and aplasia of the posterior SCC . In the presence of a normal lateral SCC, these findings are typical of Alagille syndrome.](e310a518-b0e2-4c59-a8aa-d68f51b4da61) +**Semicircular Canal Malformation** +*Axial bone CT in a child with Alagille syndrome shows a relatively normal caliber posterior limb of the left superior SCC , aplasia of the anterior limb of the superior SCC , and aplasia of the posterior SCC . In the presence of a normal lateral SCC, these findings are typical of Alagille syndrome.* + +![Axial bone CT in a patient with prior meningitis and subsequent rapid onset hearing loss shows near complete osseous replacement of the posterior and lateral aspects of the lateral SSCs and the left cochlea .](27c1c7c2-ff33-4d28-9519-327c65e7e18e) +**Labyrinthine Ossificans** +*Axial bone CT in a patient with prior meningitis and subsequent rapid onset hearing loss shows near complete osseous replacement of the posterior and lateral aspects of the lateral SSCs and the left cochlea .* + +![Axial high-resolution CISS MR in a patient with prior meningitis and known partial labyrinthine ossification shows lack of normal T2 hyperintensity in the left membranous labyrinth and decreased T2 hyperintensity in the right. The right cochlea and vestibule are barely visible.](6d586659-4d2d-4aac-8bc3-a67ae62e802d) +**Labyrinthine Ossificans** +*Axial high-resolution CISS MR in a patient with prior meningitis and known partial labyrinthine ossification shows lack of normal T2 hyperintensity in the left membranous labyrinth and decreased T2 hyperintensity in the right. The right cochlea and vestibule are barely visible.* + +![Axial T1 C+ MR in the same patient shows mild, patchy abnormal enhancement of the bilateral cochlea and vestibule .](623d7e79-7254-4327-a31d-ae8646e3b00d) +**Labyrinthine Ossificans** +*Axial T1 C+ MR in the same patient shows mild, patchy abnormal enhancement of the bilateral cochlea and vestibule .* + +![Axial T2 FS MR shows corresponding loss of hyperintense T2 signal in the cochlea , vestibule , and IAC .](236ac50a-e76f-4e61-b4ed-f19123eac04d) +**Labyrinthitis** +*Axial T2 FS MR shows corresponding loss of hyperintense T2 signal in the cochlea , vestibule , and IAC .* + +![Axial T1 C+ FS MR shows abnormal contrast enhancement in the middle ear, mastoid, cochlea , vestibule , and IAC , secondary to actinomycosis labyrinthitis.](5f870778-af57-4d52-a3d3-c0cd086c58da) +**Labyrinthitis** +*Axial T1 C+ FS MR shows abnormal contrast enhancement in the middle ear, mastoid, cochlea , vestibule , and IAC , secondary to actinomycosis labyrinthitis.* + +![Axial bone CT in a 3-year-old girl with unilateral SNHL who failed her newborn hearing test shows an absent cochlear nerve canal , sometimes referred to as an "isolated" or "detached" cochlea.](c220a5e3-c793-45a8-9451-d595e9675ad0) +**Cochlear Nerve & Cochlear Nerve Canal Aplasia-Hypoplasia** +*Axial bone CT in a 3-year-old girl with unilateral SNHL who failed her newborn hearing test shows an absent cochlear nerve canal , sometimes referred to as an "isolated" or "detached" cochlea.* + +![Sagittal oblique T2 MR at the level of the left IAC clearly shows a normal-appearing facial nerve , superior/inferior vestibular nerve complex , and diminutive left cochlear nerve .](bf96d296-12cb-4290-b5ab-263a8174141d) +**Cochlear Nerve & Cochlear Nerve Canal Aplasia-Hypoplasia** +*Sagittal oblique T2 MR at the level of the left IAC clearly shows a normal-appearing facial nerve , superior/inferior vestibular nerve complex , and diminutive left cochlear nerve .* + +![Axial high-resolution T2 MR shows absence of the definable right cochlear nerve within the right IAC, and lack of a cochlear nerve canal and hypoplasia of the right modiolus .](1aaf9d02-8294-45f3-a5f7-db63c6b9f795) +**Cochlear Nerve & Cochlear Nerve Canal Aplasia-Hypoplasia** +*Axial high-resolution T2 MR shows absence of the definable right cochlear nerve within the right IAC, and lack of a cochlear nerve canal and hypoplasia of the right modiolus .* + +![Sagittal oblique T2 MR in the same child shows nonvisualization of the right cochlear nerve , which should normally be at least as large as the facial nerve on sagittal oblique images through the IAC.](50e74dd0-9ac4-4384-bf75-7ba41341ac25) +**Cochlear Nerve & Cochlear Nerve Canal Aplasia-Hypoplasia** +*Sagittal oblique T2 MR in the same child shows nonvisualization of the right cochlear nerve , which should normally be at least as large as the facial nerve on sagittal oblique images through the IAC.* + +![Axial bone CT shows the typical CT appearance of cystic cochleovestibular anomaly. The vestibule is globular .](7b110cc6-1cfb-45c5-bf33-23b0c614a31e) +**Cystic Cochleovestibular Malformation (IP-I)** +*Axial bone CT shows the typical CT appearance of cystic cochleovestibular anomaly. The vestibule is globular .* + +![Axial bone CT in the same patient reveals a featureless cochlea without a definable modiolus, also termed an IP-I anomaly.](621ad64f-8c3f-432a-b68b-3b616c2ca165) +**Cystic Cochleovestibular Malformation (IP-I)** +*Axial bone CT in the same patient reveals a featureless cochlea without a definable modiolus, also termed an IP-I anomaly.* + +![Axial NECT shows the typical CT appearance of a small lipoma in the left CPA cistern . There is low-attenuation focus of CPA lipoma.](4e17d42e-e164-40a9-9621-94d94bed6262) +**CPA-IAC Lipoma** +*Axial NECT shows the typical CT appearance of a small lipoma in the left CPA cistern . There is low-attenuation focus of CPA lipoma.* + +![Coronal T1 MR shows a hyperintense lipoma in the left CPA cistern .](fd279548-74ae-4c8f-99a3-d9f3f53d6f4a) +**CPA-IAC Lipoma** +*Coronal T1 MR shows a hyperintense lipoma in the left CPA cistern .* + +![Axial T1 C+ FS MR in a 2-year-old boy with neurofibromatosis type 2 clearly shows enhancing masses within the bilateral IAC, inseparable from the CNVII/CNVIII complexes.](e94a293d-2231-4ccd-974b-f6378b218274) +**Vestibular Schwannoma** +*Axial T1 C+ FS MR in a 2-year-old boy with neurofibromatosis type 2 clearly shows enhancing masses within the bilateral IAC, inseparable from the CNVII/CNVIII complexes.* + +![Axial T2WI MR in the same patient demonstrates corresponding hypointense signal within the IAC masses , replacing the normal hyperintense T2-signal fluid, typical of small schwannomas.](0510cbc3-44c3-4c34-878b-a5c33676f54c) +**Vestibular Schwannoma** +*Axial T2WI MR in the same patient demonstrates corresponding hypointense signal within the IAC masses , replacing the normal hyperintense T2-signal fluid, typical of small schwannomas.* + +![Axial T1 C+ FS MR in a 15-year-old girl with unilateral SNHL shows an enhancing intracochlear mass in the middle turn of the cochlea. The tumor enlarged over time and was subsequently resected, confirming intracochlear schwannoma.](9d978771-1ee7-4fb9-a6be-d2b1cd79eca6) +**Intralabyrinthine Schwannoma** +*Axial T1 C+ FS MR in a 15-year-old girl with unilateral SNHL shows an enhancing intracochlear mass in the middle turn of the cochlea. The tumor enlarged over time and was subsequently resected, confirming intracochlear schwannoma.* + +![Axial bone CT demonstrates a common cavity anomaly with a cystic structure representing the vestibule, rudimentary cochlear bud, and horizontal semicircular canal , with a relatively normal-appearing posterior semicircular canal .](4a2ed0bd-8558-4983-a136-761da307e9eb) +**Common Cavity Malformation** +*Axial bone CT demonstrates a common cavity anomaly with a cystic structure representing the vestibule, rudimentary cochlear bud, and horizontal semicircular canal , with a relatively normal-appearing posterior semicircular canal .* + +![Axial bone CT in a 1 year old with SNHL demonstrates a small left IAC and absence of the left cochlea . Notice also the mild associated hypoplasia of the left petrous apex .](fbf3d418-bbb6-4c1a-aed1-1e053844e2e3) +**Cochlear Aplasia** +*Axial bone CT in a 1 year old with SNHL demonstrates a small left IAC and absence of the left cochlea . Notice also the mild associated hypoplasia of the left petrous apex .* + +![Axial bone CT shows that the otic capsule is featureless and without definable labyrinthine structures. The lateral wall is flat , indicating congenital absence rather than acquired ossificans.](256ad236-e2d5-4b8c-b61b-029a911b591e) +**Labyrinthine Aplasia** +*Axial bone CT shows that the otic capsule is featureless and without definable labyrinthine structures. The lateral wall is flat , indicating congenital absence rather than acquired ossificans.* + + +### Additional Images + +![Axial bone CT shows complete osseous replacement of the right cochlea . Notice the presence of a normal right cochlear promontory , convex laterally, indicating acquired ossification rather than cochlear aplasia.](834c45da-92b9-4624-9694-5a5c336bc9e2) +**Labyrinthine Ossificans** +*Axial bone CT shows complete osseous replacement of the right cochlea . Notice the presence of a normal right cochlear promontory , convex laterally, indicating acquired ossification rather than cochlear aplasia.* + +![Axial high-resolution T2 MR in a 14 year old with a recent dirt bike accident (resulting in bilateral otic capsule fractures) shows decreased hyperintense T2 signal in the right vestibule and lateral semicircular canal (SCC) , consistent with early fibrous replacement of normal labyrinthine fluid.](e58a5cce-4574-41d1-bc89-f89c51c405b6) +**Labyrinthine Ossificans** +*Axial high-resolution T2 MR in a 14 year old with a recent dirt bike accident (resulting in bilateral otic capsule fractures) shows decreased hyperintense T2 signal in the right vestibule and lateral semicircular canal (SCC) , consistent with early fibrous replacement of normal labyrinthine fluid.* + +![Axial T1 C + FS MR in the same patient depicts abnormal contrast enhancement involving the right cochlea , vestibule , and lateral SCC , which may persist for months in labyrinthine ossificans.](5d112991-354c-44b8-b022-bacd8235a87d) +**Labyrinthine Ossificans** +*Axial T1 C + FS MR in the same patient depicts abnormal contrast enhancement involving the right cochlea , vestibule , and lateral SCC , which may persist for months in labyrinthine ossificans.* + +![Axial T1 C+ FS MR shows abnormal enhancement of the right cochlea , indicative of labyrinthitis in this patient with Cogan syndrome.](c0bb5390-0a42-4934-b41a-69f80e50175f) +**Labyrinthitis** +*Axial T1 C+ FS MR shows abnormal enhancement of the right cochlea , indicative of labyrinthitis in this patient with Cogan syndrome.* + +![Axial T1 C+ FS MR shows abnormal enhancement of the left cochlea , indicative of labyrinthitis in this patient with Cogan syndrome.](e885232f-2a5b-4cf2-bf32-75e133b461d1) +**Labyrinthitis** +*Axial T1 C+ FS MR shows abnormal enhancement of the left cochlea , indicative of labyrinthitis in this patient with Cogan syndrome.* + +![Axial T1 C+ MR shows a variant appearance of a heterogeneously enhancing extraaxial mass in the right CPA with extension into the porous acusticus of the right IAC .](11ab1c0a-67ce-4ea8-8394-7b852717c0b3) +**Facial Nerve Schwannoma in CPA-IAC** +*Axial T1 C+ MR shows a variant appearance of a heterogeneously enhancing extraaxial mass in the right CPA with extension into the porous acusticus of the right IAC .* + +![Coronal T1 C+ MR shows a large heterogeneously enhancing mass in the CPA cistern and proximal IAC .](902b80b0-dbb9-4724-839a-b59e5e95edbd) +**Facial Nerve Schwannoma in CPA-IAC** +*Coronal T1 C+ MR shows a large heterogeneously enhancing mass in the CPA cistern and proximal IAC .* + +![Axial bone CT shows a transverse otic capsule violating T-bone fracture with associated pneumolabyrinth and gas in the vestibule and lateral SCC.](images/app.statdx.com_image_thumbnail_89ab8da0-0c3c-4c67-9643-a00f01249885_annotated_true_size_900_quality_90_f925e894856fd2122dcad8a45797d6c48a4eb1eb.jpg) +**T-Bone Fractures** +*Axial bone CT shows a transverse otic capsule violating T-bone fracture with associated pneumolabyrinth and gas in the vestibule and lateral SCC.* + +![Axial bone CT demonstrates enlargement of the left bony vestibular aqueduct .](images/app.statdx.com_image_thumbnail_da713cb9-8a08-403f-b3d3-e4134e9b1567_annotated_true_size_900_quality_90_6f4998a52c90852a1329f9a73e98ac4057a72aad.jpg) +**Large Endolymphatic Sac Anomaly (IP-II)** +*Axial bone CT demonstrates enlargement of the left bony vestibular aqueduct .* + +![Axial bone CT shows incomplete partitioning of the left cochlea in a patient with large vestibular aqueduct.](images/app.statdx.com_image_thumbnail_a456f703-ed2f-43d9-9d09-1bc1313f71e2_annotated_true_size_900_quality_90_1a8a8c841af150befdc619f6f46ce5dba631392e.jpg) +**Large Endolymphatic Sac Anomaly (IP-II)** +*Axial bone CT shows incomplete partitioning of the left cochlea in a patient with large vestibular aqueduct.* + +![Axial bone CT shows longitudinal temporal bone fracture with associated pneumolabyrinth .](images/app.statdx.com_image_thumbnail_fe197af5-2391-4b41-8d8d-42e3df5f30da_annotated_true_size_900_quality_90_e01e96bdc119864c0398f861e0c1f79f7467b816.jpg) +**T-Bone Fractures** +*Axial bone CT shows longitudinal temporal bone fracture with associated pneumolabyrinth .* + +![Axial bone CT shows lack of the normal right cochlear aperture and severe hypoplasia of the vestibule and SCCs in a patient with CHARGE syndrome.](543563c7-a1cf-4894-8985-dae6bbb20523) +**Semicircular Canal Malformation** +*Axial bone CT shows lack of the normal right cochlear aperture and severe hypoplasia of the vestibule and SCCs in a patient with CHARGE syndrome.* + +![Axial T1WI C+ FS MR shows abnormal contrast enhancement in the middle ear, mastoid, cochlea , vestibule , and IAC secondary to actinomycosis labyrinthitis.](7254b176-8698-4013-8277-b63f0ff87296) +**Labyrinthitis** +*Axial T1WI C+ FS MR shows abnormal contrast enhancement in the middle ear, mastoid, cochlea , vestibule , and IAC secondary to actinomycosis labyrinthitis.* + +![Axial bone CT reveals hypoplastic right IAC related to right cochlear nerve deficiency.](ecd1f9df-b73b-4d9e-b18e-5d9c4caed184) +**Cochlear Nerve & Cochlear Nerve Canal Aplasia-Hypoplasia** +*Axial bone CT reveals hypoplastic right IAC related to right cochlear nerve deficiency.* + +![Axial bone CT demonstrates a malformed vestibule that communicates with a small IAC through a broad gap at the IAC fundus . There is no cochlea anterior to the vestibule.](25bb712b-4648-4f94-a941-4b6af6e26b80) +**Cochlear Aplasia** +*Axial bone CT demonstrates a malformed vestibule that communicates with a small IAC through a broad gap at the IAC fundus . There is no cochlea anterior to the vestibule.* + +![Axial T2WI MR shows a tiny left IAC and nonvisualization of the cochlear nerve.](9e1fb4f6-57ce-40fb-94c0-a1c8842b55f7) +**Cochlear Nerve & Cochlear Nerve Canal Aplasia-Hypoplasia** +*Axial T2WI MR shows a tiny left IAC and nonvisualization of the cochlear nerve.* + +![Sagittal oblique T2 MR shows nonvisualization of the cochlear nerve in association with a small IAC. CNVIII and CNVII formation in the IAC area provides the stimulus for IAC formation.](68058bcd-ca5b-402b-b42c-1d47d843c50b) +**Cochlear Nerve & Cochlear Nerve Canal Aplasia-Hypoplasia** +*Sagittal oblique T2 MR shows nonvisualization of the cochlear nerve in association with a small IAC. CNVIII and CNVII formation in the IAC area provides the stimulus for IAC formation.* + +![Axial high-resolution FSE MR in a child with bilateral hearing loss shows bilateral enlargement of the extraosseous endolymphatic sacs and cochlear modiolar deficiency .](images/app.statdx.com_image_thumbnail_0fe73f57-7ec4-42e1-a565-23d8bb0542b3_annotated_true_size_900_quality_90_00bc0095b56af73074b7f3f6c15d34f6c681681d.jpg) +**Large Endolymphatic Sac Anomaly (IP-II)** +*Axial high-resolution FSE MR in a child with bilateral hearing loss shows bilateral enlargement of the extraosseous endolymphatic sacs and cochlear modiolar deficiency .* + diff --git a/docs_md/articles/small-iac_9323a206-e7c6-4213-8493-7870b51c6adf.md b/docs_md/articles/small-iac_9323a206-e7c6-4213-8493-7870b51c6adf.md new file mode 100644 index 0000000..f7f33df --- /dev/null +++ b/docs_md/articles/small-iac_9323a206-e7c6-4213-8493-7870b51c6adf.md @@ -0,0 +1,166 @@ +--- +title: "Small IAC" +docid: "9323a206-e7c6-4213-8493-7870b51c6adf" +authors: + - key: "d19354f3-7ff2-495a-ad3f-064122e45602" + value: "Bernadette L. Koch, MD" +breadcrumbs: + - + name: "Head and Neck" + slug: "head-and-neck" + treeNodeId: "ed24ed8c-5d57-4629-879b-447b82d2973d" + - + name: "Differential Diagnosis" + slug: "differential-diagnosis" + treeNodeId: "40d68862-8975-4dde-ac2b-ebc43ab0fb5c" + - + name: "CPA-IAC and Posterior Fossa" + slug: "cpa-iac-and-posterior-fossa" + treeNodeId: "c590eedb-4a3b-4158-a04f-ad880564c992" + - + name: "Anatomically Based Differentials" + slug: "anatomically-based-differentials" + treeNodeId: "debfb06c-8656-4f5d-92c1-eaa468185d78" + - + name: "Small IAC" + slug: "small-iac" + treeNodeId: null +category: "Head and Neck" +documentVersionId: "590b726c-a68d-4f79-b2e1-e7bb638eaaa9" +imageCount: 11 +lastUpdated: "07/17/24" +pageDescription: "Small IAC" +pageKeywords: "Head and Neck, Differential Diagnosis, CPA-IAC and Posterior Fossa, Anatomically Based Differentials, Small IAC" +pageTitle: "Small IAC | STATdx" +enhancedTitle: "Small IAC" +type: "DDX" +references: true +breadcrumbs: + - "Head and Neck" + - "Differential Diagnosis" + - "CPA-IAC and Posterior Fossa" + - "Anatomically Based Differentials" + - "Small IAC" +--- +# ESSENTIAL INFORMATION + +- ## Key Differential Diagnosis Issues + + + - Small internal auditory canal (IAC) **≤ 2****mm diameter** + - CT for bony anatomy; MR to evaluate CNVII & CNVIII components & brainstem anatomy + - Occasional duplicated IAC: Partial or complete separation of IAC into 2 stenotic canals + - Superior canal transmits CNVII ± superior vestibular nerve (VN); inferior canal transmits inferior ± superior VN ± hypoplastic cochlear nerve + - Small IAC with cochlear nerve canal (CNC) stenosis/aplasia & hypoplastic/absent cochlear nerve + - Unilateral finding in otherwise normal T-bone suggests nonsyndromic unilateral congenital sensorineural hearing loss (SNHL) + - Bilateral finding with small horizontal semicircular canal (SCC) bone islands suggests trisomy 21 + - Small vestibule & small/absent SCC suggests CHARGE syndrome + - Severe inner ear anomaly: Cochlear aplasia, common cavity malformation, or cystic cochleovestibular anomaly + - Look for coexistent pontine/brainstem anomaly + - Occasional unusual origin & course of CNVII ± CNVIII hypoplasia/aplasia +- ## Helpful Clues for Common Diagnoses + + + - [Trisomy 21](/document/trisomy-21-down-syndrome/dea8cfda-4526-4373-bd25-2ca5c119d243) + - Small bone island horizontal SCC or globular vestibule & horizontal SCC + - Stenotic CNC, thickened modiolus ± small IAC, hypoplastic/absent cochlear nerve + - [Aplasia-Hypoplasia of Cochlear Nerve & Cochlear Nerve Canal](/document/cochlear-nerve-and-cochlear-nerve--/a669349b-22f5-4b5a-9d6d-6b70a5717ecc) + - Common finding in unilateral congenital SNHL + - Narrowed/absent CNC & thickened modiolus ± small IAC, hypoplastic/absent cochlear nerve +- ## Helpful Clues for Less Common Diagnoses + + + - [CHARGE Syndrome](/document/semicircular-canal-hypoplasia-apla-/2112211e-f2fd-448d-9fe8-5352b2900cee) + - Small vestibule & hypoplastic/absent SCC + - Variable cochlear segmentation deficiency + - Narrowed/absent CNC, thickened modiolus, & small IAC + - Hypoplasia/aplasia of some/all CNVIII components +- ## Helpful Clues for Rare Diagnoses + + + - **Cystic Cochleovestibular Malformation (IP-I)** + - Cochlea lacks internal septation/modiolus + - Globular vestibule & horizontal SCC ± stenotic/absent CNC ± small IAC + - [Common Cavity Malformation](/document/common-cavity-malformation/008305e1-2809-41b0-9e4e-730e30b05a66) + - Single primitive sac ± small IAC + - [Cochlear Hypoplasia](/document/cochlear-hypoplasia/58a34b9d-fbae-44a5-99cc-ee2319e0d13d) + - Small cochlea < 2 turns, CNC stenosis/atresia ± variable malformation SCC & vestibule + - ± small IAC, obtuse angle anterior genu of CNVII canal + - [Cochlear Aplasia](/document/cochlear-aplasia/82e9634a-f9ed-407d-b611-110b7328f9fb) + - Absent cochlea ± malformation of SCC & vestibule + - Malformation of SCC & vestibule variable, mild to severe + - ± small IAC, obtuse angle anterior genu of CNVII canal + - [T-Bone Fibrous Dysplasia](/document/temporal-bone-fibrous-dysplasia/e5b44f77-f666-4f32-8eb0-6ed2da7d9898) + - Progressive ground-glass fibroosseous thickening → IAC narrowing + - **Craniometaphyseal Dysplasia** + - Progressive osseous IAC narrowing + +## References + +# Selected References + +1. [da Costa Monsanto R et al: Otopathologic abnormalities in CHARGE syndrome. Otolaryngol Head Neck Surg. 166(2):363-72, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33874787%5Bpmid%5D) +1. [Dewyer NA et al: Pediatric single-sided deafness: a review of prevalence, radiologic findings, and cochlear implant candidacy. Ann Otol Rhinol Laryngol. 131(3):233-8, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34036833%5Bpmid%5D) +1. [O'Brien WT , Sr et al: Nonsyndromic congenital causes of sensorineural hearing loss in children: an illustrative review. AJR Am J Roentgenol. 1-8, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33502224%5Bpmid%5D) +1. [Ginat DT: Imaging findings in syndromes with temporal bone abnormalities. Neuroimaging Clin N Am. 29(1):117-28, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30466636%5Bpmid%5D) +1. [Tahir E et al: Bony cochlear nerve canal and internal auditory canal measures predict cochlear nerve status. J Laryngol Otol. 131(8):676-83, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28566097%5Bpmid%5D) +1. [Kenna MA et al: Temporal bone abnormalities in children with GJB2 mutations. Laryngoscope. 121(3):630-5, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21298644%5Bpmid%5D) +1. [Morimoto AK et al: Absent semicircular canals in CHARGE syndrome: radiologic spectrum of findings. AJNR Am J Neuroradiol. 27(8):1663-71, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16971610%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial bone CT in an infant with trisomy 21 and sensorineural hearing loss (SNHL) shows a small internal auditory canal (IAC) . The cochlear nerve canal (CNC) is absent. There is a mildly small horizontal semicircular canal (SCC) bone island .](images/app.statdx.com_image_thumbnail_69c10678-064c-4a14-b9bc-fc104bad6011_annotated_true_size_900_quality_90_022530d12bb1df8515bb78c38493c84592b1d804.jpg) +**Trisomy 21** +*Axial bone CT in an infant with trisomy 21 and sensorineural hearing loss (SNHL) shows a small internal auditory canal (IAC) . The cochlear nerve canal (CNC) is absent. There is a mildly small horizontal semicircular canal (SCC) bone island .* + +![Axial bone CT in an infant with trisomy 21 and sensorineural hearing loss (SNHL) shows a small internal auditory canal (IAC) . The cochlear nerve canal (CNC) is absent. There is a mildly small horizontal semicircular canal (SCC) bone island .](images/app.statdx.com_image_thumbnail_69c10678-064c-4a14-b9bc-fc104bad6011_size_174_quality_85_8c339968.jpg) +**Trisomy 21** +*Axial bone CT in an infant with trisomy 21 and sensorineural hearing loss (SNHL) shows a small internal auditory canal (IAC) . The cochlear nerve canal (CNC) is absent. There is a mildly small horizontal semicircular canal (SCC) bone island .* + +![Sagittal oblique T2 MR images of the bilateral IACs (right on the left and left on the right) shows a significantly smaller right IAC compared to the left, and nonvisualization of the cochlear nerve . Notice the normal left cochlear nerve .](fd6e92f8-4c55-4db1-b686-e31ac3f16b65) +**Aplasia-Hypoplasia of Cochlear Nerve & Cochlear Nerve Canal** +*Sagittal oblique T2 MR images of the bilateral IACs (right on the left and left on the right) shows a significantly smaller right IAC compared to the left, and nonvisualization of the cochlear nerve . Notice the normal left cochlear nerve .* + +![Coronal bone CT reformat in a 7-year-old girl with CHD7 mutation shows a small IAC , diminutive vestibule , and absent SCC. Facial nerve canal overlies the atretic oval window with fusion to malformed stapes. There is an emissary vein indenting the tegmen tympani .](images/app.statdx.com_image_thumbnail_cf3d36b2-cc2e-461a-8a7b-3ce9c7d8bf3d_annotated_true_size_900_quality_90_836f5c50a7f11ba292db1a36494295b2a13fa8e9.jpg) +**CHARGE Syndrome** +*Coronal bone CT reformat in a 7-year-old girl with CHD7 mutation shows a small IAC , diminutive vestibule , and absent SCC. Facial nerve canal overlies the atretic oval window with fusion to malformed stapes. There is an emissary vein indenting the tegmen tympani .* + +![Axial bone CT in a 10-year-old girl with SNHL shows small IACs and a globular right vestibule and horizontal SCC . The right cochlea (not shown) lacked internal septation (IP-I). A hypoplastic, isolated left cochlea is also seen.](images/app.statdx.com_image_thumbnail_cd4d3d47-6080-4b07-9e17-af75691687db_annotated_true_size_900_quality_90_3637bb2fa0293dabb75788331fc7d81113251cc9.jpg) +**Cystic Cochleovestibular Malformation (IP-I)** +*Axial bone CT in a 10-year-old girl with SNHL shows small IACs and a globular right vestibule and horizontal SCC . The right cochlea (not shown) lacked internal septation (IP-I). A hypoplastic, isolated left cochlea is also seen.* + +![Axial 3D T2 SPACE MR in a child with SNHL shows cochlear aplasia, a globular vestibule, and a horizontal SCC . There is a narrow, malformed IAC with a vestibular nerve noted posteriorly.](images/app.statdx.com_image_thumbnail_3f1345b3-b83f-4a8e-875c-0ed7b0a536c0_annotated_true_size_900_quality_90_71a061daa65df0a6a73c582aa536c83b0bd648d9.jpg) +**Cochlear Aplasia** +*Axial 3D T2 SPACE MR in a child with SNHL shows cochlear aplasia, a globular vestibule, and a horizontal SCC . There is a narrow, malformed IAC with a vestibular nerve noted posteriorly.* + +![Oblique sagittal T2 SPACE MR in the same child shows a narrow IAC containing only a single normal-sized cranial nerve (CNVIII vestibular branch) and a possible hypoplastic CNVII anteriorly.](images/app.statdx.com_image_thumbnail_48395920-1458-4f3f-a03b-06a26b1d091d_annotated_true_size_900_quality_90_55f1bab5280371efdb7a3798df854ea4ebf9f8a4.jpg) +**Cochlear Aplasia** +*Oblique sagittal T2 SPACE MR in the same child shows a narrow IAC containing only a single normal-sized cranial nerve (CNVIII vestibular branch) and a possible hypoplastic CNVII anteriorly.* + +![Axial bone CT in a teenage boy with polyostotic fibrous dysplasia and precocious puberty (McCune-Albright syndrome) shows severe involvement of the skull base with ground-glass opacification. The middle ear spaces , IACs , and other foramina are small. Note relative otic capsule sparing.](images/app.statdx.com_image_thumbnail_508c5499-4769-4a9b-bcfa-8339b4718289_annotated_true_size_900_quality_90_404847f7f6e9c105072852f2620d70be0149f05d.jpg) +**T-Bone Fibrous Dysplasia** +*Axial bone CT in a teenage boy with polyostotic fibrous dysplasia and precocious puberty (McCune-Albright syndrome) shows severe involvement of the skull base with ground-glass opacification. The middle ear spaces , IACs , and other foramina are small. Note relative otic capsule sparing.* + +![Axial bone CT in a young man with craniometaphyseal dysplasia shows bony overgrowth of the skull base with small middle ear spaces, ossicular fusion , and small inner ear structures and IACs .](03aefa37-9bb2-4212-8199-c8f8c485669b) +**Craniometaphyseal Dysplasia** +*Axial bone CT in a young man with craniometaphyseal dysplasia shows bony overgrowth of the skull base with small middle ear spaces, ossicular fusion , and small inner ear structures and IACs .* + + +### Additional Images + +![Axial 3D T2 SPACE MR in a teenager with SNHL shows small IACs . The right CNC is stenotic ; the left is absent. The modioli are thickened . The vestibular nerves and pons are hypoplastic.](3049cbef-1768-457f-9cee-3bb3957ae3f5) +**Aplasia-Hypoplasia of Cochlear Nerve & Cochlear Nerve Canal** +*Axial 3D T2 SPACE MR in a teenager with SNHL shows small IACs . The right CNC is stenotic ; the left is absent. The modioli are thickened . The vestibular nerves and pons are hypoplastic.* + +![Axial bone CT in a child with profound SNHL shows a small IAC and hypoplasia of the CNC . There is also a mildly large vestibular aqueduct . MR should be obtained in order to assess for aplasia or hypoplasia of the cranial nerve.](ba6e5c92-e87c-4c7a-b830-79c1c2a00d4a) +**Aplasia-Hypoplasia of Cochlear Nerve & Cochlear Nerve Canal** +*Axial bone CT in a child with profound SNHL shows a small IAC and hypoplasia of the CNC . There is also a mildly large vestibular aqueduct . MR should be obtained in order to assess for aplasia or hypoplasia of the cranial nerve.* + +![Axial T2WI MR in a teenage boy with polyostotic fibrous dysplasia and precocious puberty (McCune-Albright syndrome) shows severe involvement of the skull with fibrous dysplasia that appears hypointense on T2WI. The IACs are small due to progressive involvement of surrounding bone by fibrous dysplasia.](images/app.statdx.com_image_thumbnail_7c51af12-70f6-4050-b56b-8dd6ecd8347c_annotated_true_size_900_quality_90_5ea958b39d3b9fbf4641c8a6d59302b6f740e4f1.jpg) +**T-Bone Fibrous Dysplasia** +*Axial T2WI MR in a teenage boy with polyostotic fibrous dysplasia and precocious puberty (McCune-Albright syndrome) shows severe involvement of the skull with fibrous dysplasia that appears hypointense on T2WI. The IACs are small due to progressive involvement of surrounding bone by fibrous dysplasia.* + diff --git a/docs_md/articles/status-epilepticus_a058b733-4b80-46a1-8097-d68685ecf921.md b/docs_md/articles/status-epilepticus_a058b733-4b80-46a1-8097-d68685ecf921.md new file mode 100644 index 0000000..dad2638 --- /dev/null +++ b/docs_md/articles/status-epilepticus_a058b733-4b80-46a1-8097-d68685ecf921.md @@ -0,0 +1,554 @@ +--- +title: "Status Epilepticus" +docid: "a058b733-4b80-46a1-8097-d68685ecf921" +authors: + - key: "a25c450b-3d34-4f64-bba3-cc0834813df6" + value: "Miral D. Jhaveri, MD, MBA" + - 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: "Acquired Toxic/Metabolic/Degenerative Disorders" + slug: "acquired-toxicmetabolicdegenerativ-" + treeNodeId: "ba3cfeaf-64d9-4117-91e8-d2ce58783fc5" + - + name: "Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations" + slug: "toxic-metabolic-nutritional-system-" + treeNodeId: "06bd883b-8269-4044-8411-70f7ab75bb7a" + - + name: "Status Epilepticus" + slug: "status-epilepticus" + treeNodeId: null +category: "Brain" +cmeTopicId: "278eaa3d-09a7-46df-ade2-ce0997aab6eb" +documentVersionId: "02f328e2-8c74-4253-8ec4-2e6384af5444" +imageCount: 30 +lastUpdated: "06/06/20" +pageDescription: "Status Epilepticus" +pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Acquired Toxic/Metabolic/Degenerative Disorders, Toxic, Metabolic, Nutritional, Systemic Diseases With CNS Manifestations, Status Epilepticus" +pageTitle: "Status Epilepticus | STATdx" +enhancedTitle: "Status Epilepticus" +type: "DX" +references: true +anatomy: + - "{'authors': 'Jeffrey S. 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Synonyms: Transient seizure-related MR changes, reversible postictal cerebral edema +- ## Imaging + + + - T2/FLAIR hyperintensity in gray matter (GM) &/or subcortical white matter (WM) with mild mass effect + - May involve hippocampus, corpus callosum, thalamus (particularly pulvinar nucleus) + - Transient subcortical WM T2 hypointensity + - DWI: Restricted diffusion acutely + - T1WI C+: Variable gyriform or leptomeningeal enhancement + - PWI: Marked hyperemia, ↑ rCBF and rCBV in ictal state + - Interictal: Epileptogenic zone hypoperfusion/-metabolic +- ## Top Differential Diagnoses + + + - Cerebritis + - Cerebral ischemia-infarction + - Herpes encephalitis + - Astrocytoma +- ## Clinical Issues + + + - Active seizures &/or status epilepticus + - Other signs/symptoms: Location dependent +- ## Diagnostic Checklist + + + - Acute seizures or status epilepticus may mimic other pathology, such as tumor progression or cerebritis + - Clinical information and follow-up imaging often differentiate from other etiologies + - Look for underlying mass that may have caused seizures/status epilepticus + - Seizure-related changes usually resolve within days to weeks + +# TERMINOLOGY + +- ## Abbreviations + + + - Status epilepticus (SE) +- ## Synonyms + + + - Transient seizure-related MR changes, reversible postictal cerebral edema +- ## Definitions + + + - Seizure with ≥ 5 minutes continuous clinical &/or electrographic seizure activity, or recurrent seizure activity without recovery between seizures + - MR changes associated with seizures likely related to transient cerebral edema + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - T2 hyperintensity in gray matter (GM) &/or subcortical white matter (WM) with mild mass effect + - May focally involve hippocampus, corpus callosum, thalamus (particularly pulvinar nucleus) + - ### Location + + + - Supratentorial, related to epileptogenic focus + - Typically cortex &/or subcortical WM + - May involve focal structures + - Hippocampus (febrile or partial complex seizures) + - Splenium of corpus callosum + - Pulvinar of thalamus + - Occasionally cerebellar involvement +- ## CT Findings + + + - ### NECT + + + - Initial scans may be normal + - Hypodensity in cortex &/or subcortical WM + - Hippocampus, splenium of corpus callosum, thalamus (particularly pulvinar nucleus) may be involved + - No hemorrhage + - ### CECT + + + - Variable enhancement: None to marked +- ## MR Findings + + + - ### T1WI + + + - Hypointensity in cortex &/or subcortical WM + - Swelling and ↑ volume of involved cortical gyri + - Blurring of corticomedullary junction + - Hippocampus, splenium of corpus callosum, thalamus (particularly pulvinar nucleus) may be involved + - Rarely cerebellar involvement due to crossed cerebellar diaschisis + - Delayed: Cortical laminar necrosis with T1 hyperintensity + - ### T2WI + + + - Hyperintensity in cortex &/or subcortical WM + - Swelling and ↑ volume of involved cortical gyri + - Transient subcortical WM hypointensity + - Hippocampus, corpus callosum splenium, thalamus (particularly pulvinar nucleus) may be involved + - Delayed: Focal brain atrophy, mesial temporal sclerosis + - ### FLAIR + + + - Hyperintensity in cortex &/or subcortical WM + - Transient subcortical WM hypointensity + - Mild edema and mass effect + - Hippocampus, splenium of corpus callosum, pulvinar may be involved + - ### T2* GRE + + + - No hemorrhage + - SWI: Focally diminished cortical veins in hyperperfused ictal regions + - ### DWI + + + - Restricted diffusion with decrease in ADC map acutely + - ADC maps normal interictally, elevated in chronic seizures + - ### PWI + + + - Marked hyperemia in region of epileptic focus, elevated rCBF and rCBV maps + - ASL: Elevated CBF ictal state + - ### T1WI C+ + + + - Variable enhancement: None to marked + - May see gyriform or leptomeningeal enhancement + - ### MRS + + + - Lipids &/or lactate shown in hippocampi of temporal lobe epilepsy (TLE) patients within 24 hours of last seizure + - Follow-up MRS after seizures under control show no lipids/lactate +- ## Nuclear Medicine Findings + + + - Seizures: ↑ metabolism and perfusion + - PET: ↑ glucose metabolism and metabolic rate + - HMPAO SPECT: High uptake in affected cerebral lobe during or immediately after seizure + - Interictal: Epileptogenic zone hypoperfusion/-metabolic +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR is most sensitive + - ### Protocol advice + + + - Contrast-enhanced MR with DWI and perfusion + +# DIFFERENTIAL DIAGNOSIS + +- ## Cerebritis + + + - T2-hyperintense "mass" with mass effect + - Typically DWI positive, patchy enhancement +- [Cerebral Ischemia-Infarction](/document/acute-cerebral-ischemiainfarction/a405285f-aaea-43ca-8dc4-6f8120eaabc1) + - Typical vascular distribution (anterior cerebral artery, middle cerebral artery, posterior cerebral artery) + - Acute/subacute DWI positive + - Wedge-shaped, involves GM and WM + - Gyriform enhancement in subacute ischemia +- [Herpes Encephalitis](/document/herpes-encephalitis/6bbe5645-2178-411e-871e-8878d244f482) + - Confined to limbic system, temporal lobes + - Blood products, enhancement typical + - Acute onset, often with fever and seizures +- ## Astrocytoma + + + - Infiltrating WM mass, may extend to cortex, variable enhancement + - May cause epilepsy +- ## MELAS + + + - Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes + - Multifocal bilateral T2 hyperintensities + - Predominantly GM involvement, may involve subcortical WM + - Ischemia in > 1 vascular territory + - MRS shows lactate peak +- [Mesial Temporal Sclerosis](/document/mesial-temporal-sclerosis/3861ee73-c82c-49f2-a60f-8fd08f7e6165) + - Abnormal T2 hyperintensity in mesial temporal lobe + - Hippocampal volume loss and architectural distortion +- [Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0) + - Multiple small areas of T2 hyperintensity in deep and subcortical WM, often bilateral, ± enhancement + - GM involvement may be seen +- [Demyelination](/document/multiple-sclerosis/7892b2a2-f52a-4d7f-9858-a326f2b7ab04) + - Multifocal WM lesions, deep gray nuclei + - Incomplete rim or horseshoe-shaped enhancement + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - SE evolves through initiation phase to maintenance phase + - Initiation phase: Triggering stimuli evoke discrete seizures, tend to abate as soon as stimulus is removed + - Maintenance phase: Discrete seizures coalesce, triggering stimuli no longer required to sustain seizures + - Intensity and duration of stimulation has direct influence on transition from initiation to maintenance phase + - Persistent ictal activity → increase glucose utilization and oxygen extraction  → uncoupling of blood flow-metabolism ratio → ↓ ADP and tissue hypoxia → anaerobic glycolysis + - ↑ Glutamate and ↓ GABA , Na+/K+ pump failure, intracellular cytotoxic edema + - Blood-brain barrier breakdown → vasogenic edema + - MR signal abnormalities related to transient vasogenic &/or cytotoxic edema + - Redistribution of intracellular and extracellular water, related to alteration in cell membrane permeability or cytotoxic edema + - Hippocampus involvement by SE may result in mesial temporal sclerosis + - Involvement of corpus callosum splenium, 2 theories + - Transient focal edema related to transhemispheric connection of seizure activity + - Reversible demyelination related to antiepileptic drugs + - Anatomic considerations + - Portions of brain most vulnerable to damage from SE + - CA1, CA3 of hippocampus, amygdala, piriform cortex, cerebellar cortex, thalamus, cerebral cortex +- ## Staging, Grading, & Classification + + + - SE classified broadly as convulsive and nonconvulsive + - Convulsive SE further classified + - Tonic-clonic, tonic, clonic, myoclonic + - Nonconvulsive SE +- ## Gross Pathologic & Surgical Features + + + - Acutely: Swelling of cortex &/or subcortical WM or hippocampus + - Chronic: Atrophy of involved cortex &/or subcortical WM +- ## Microscopic Features + + + - Acutely + - Reactive astrocytes with swollen cytoplasm and neuropil, consistent with cytotoxic edema + - Chronic + - Marked neuronal loss with intense astrocytic reaction; reactive astrocytes replacing absent neurons + - Gliosis and neuronal loss affecting GM-WM junction with extension to cortex + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Active seizures &/or SE + - Other signs/symptoms: Location dependent + - Nonconvulsive SE: Abnormal mental status, unresponsiveness, ocular motor abnormalities + - ### Clinical profile + + + - EEG shows seizure activity +- ## Demographics + + + - ### Age + + + - Bimodal age distribution, peaks during infancy and in elderly + - ### Sex + + + - No sex predominance + - ### Epidemiology + + + - 7-40 cases/100,000 person-years +- ## Natural History & Prognosis + + + - Typically complete resolution with treatment of seizures + - May be complicated by infarction related to hypoxemia +- ## Treatment + + + - Treatment of underlying seizure disorder + - Antiepileptic medicines primary therapy + - Surgical resection in patients with intractable epilepsy + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Acute seizures or SE may mimic other pathology, such as tumor or cerebritis + - Clinical information and follow-up imaging often differentiate seizure-related MR changes from other etiologies +- ## Image Interpretation Pearls + + + - Look for underlying lesion that may have caused seizures/SE + - Seizure-related changes will usually resolve within days to weeks on follow-up imaging + + cc4f860e-ad34-4521-b6b5-a9e19faa6080 + +## References + +# Selected References + +1. [Husari KS et al: New-onset refractory status epilepticus in children: etiologies, treatments, and outcomes. Pediatr Crit Care Med. 21(1):59-66, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31568262%5Bpmid%5D) +1. [Guerriero RM et al: Imaging modalities to diagnose and localize status epilepticus. Seizure. 68:46-51, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30385179%5Bpmid%5D) +1. [Sculier C et al: New onset refractory status epilepticus (NORSE). Seizure. 68:72-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30482654%5Bpmid%5D) +1. [Strohm T et al: FDG-PET and MRI in the evolution of new-onset refractory status epilepticus. AJNR Am J Neuroradiol. 40(2):238-44, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30679215%5Bpmid%5D) +1. [Meletti S et al: Neuroimaging of status epilepticus. Epilepsia. 59 Suppl 2:113-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30160066%5Bpmid%5D) +1. [Betjemann JP et al: Status epilepticus in adults. Lancet Neurol. 14(6):615-24, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25908090%5Bpmid%5D) +1. [Cartagena AM et al: Reversible and irreversible cranial MRI findings associated with status epilepticus. Epilepsy Behav. 33:24-30, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24614522%5Bpmid%5D) +1. [Ohe Y et al: MRI abnormality of the pulvinar in patients with status epilepticus. J Neuroradiol. 41(4):220-6, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24485898%5Bpmid%5D) +1. [Di Bonaventura C et al: Diffusion-weighted magnetic resonance imaging in patients with partial status epilepticus. Epilepsia. 50 Suppl 1:45-52, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19125848%5Bpmid%5D) +1. [Goyal MK et al: Peri-ictal signal changes in seven patients with status epilepticus: interesting MRI observations. Neuroradiology. 51(3):151-61, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19057899%5Bpmid%5D) +1. [Katramados AM et al: Periictal diffusion abnormalities of the thalamus in partial status epilepticus. Epilepsia. 50(2):265-75, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18717714%5Bpmid%5D) +1. [Masterson K et al: Postictal deficit mimicking stroke: role of perfusion CT. J Neuroradiol. 36(1):48-51, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18835645%5Bpmid%5D) +1. [Milligan TA et al: Frequency and patterns of MRI abnormalities due to status epilepticus. Seizure. 18(2):104-8, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18723376%5Bpmid%5D) +1. [Nair PP et al: Role of cranial imaging in epileptic status. Eur J Radiol. 70(3):475-80, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=18359597%5Bpmid%5D) +1. [Buracchio T et al: Restricted diffusion on magnetic resonance imaging in partial status epilepticus. Arch Neurol. 65(2):278-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18268202%5Bpmid%5D) +1. [Goyal MK et al: Role of MR imaging in the evaluation of etiology of status epilepticus. J Neurol Sci. 272(1-2):143-50, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18602120%5Bpmid%5D) +1. [Provenzale JM et al: Hippocampal MRI signal hyperintensity after febrile status epilepticus is predictive of subsequent mesial temporal sclerosis. AJR Am J Roentgenol. 190(4):976-83, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18356445%5Bpmid%5D) +1. [Yu JT et al: Diffusion-weighted magnetic resonance imaging demonstrates parenchymal pathophysiological changes in epilepsy. Brain Res Rev. 59(1):34-41, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18514917%5Bpmid%5D) +1. [Kuster GW et al: Hippocampal sclerosis and status epilepticus: cause or consequence? A MRI study. Arq Neuropsiquiatr. 65(4B):1101-4, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=18345411%5Bpmid%5D) +1. [Parmar H et al: Acute symptomatic seizures and hippocampus damage: DWI and MRS findings. Neurology. 66(11):1732-5, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16769950%5Bpmid%5D) +1. [Calistri V et al: Visualization of evolving status epilepticus with diffusion and perfusion MR imaging. AJNR Am J Neuroradiol. 24(4):671-3, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12695201%5Bpmid%5D) +1. [Hicdonmez T et al: Reversible postictal MRI change mimicking structural lesion. Clin Neurol Neurosurg. 105(4):288-90, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12954549%5Bpmid%5D) +1. [Oster J et al: Diffusion-weighted imaging abnormalities in the splenium after seizures. Epilepsia. 44(6):852-4, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12790901%5Bpmid%5D) +1. [Cohen-Gadol AA et al: Transient postictal magnetic resonance imaging abnormality of the corpus callosum in a patient with epilepsy. Case report and review of the literature. J Neurosurg. 97(3):714-7, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12296661%5Bpmid%5D) +1. [Amato C et al: Transient MRI abnormalities associated with partial status epilepticus: a case report. Eur J Radiol. 38(1):50-4, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11287165%5Bpmid%5D) +1. [Castillo M et al: Proton MR spectroscopy in patients with acute temporal lobe seizures. AJNR Am J Neuroradiol. 22(1):152-7, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11158901%5Bpmid%5D) +1. [Kim JA et al: Transient MR signal changes in patients with generalized tonicoclonic seizure or status epilepticus: periictal diffusion-weighted imaging. AJNR Am J Neuroradiol. 22(6):1149-60, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11415912%5Bpmid%5D) +1. [Polster T et al: Transient lesion in the splenium of the corpus callosum: three further cases in epileptic patients and a pathophysiological hypothesis. J Neurol Neurosurg Psychiatry. 70(4):459-63, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11254767%5Bpmid%5D) +1. [Sagiuchi T et al: Transient seizure activity demonstrated by Tc-99m HMPAO SPECT and diffusion-weighted MR imaging. Ann Nucl Med. 15(3):267-70, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11545200%5Bpmid%5D) +1. [Men S et al: Selective neuronal necrosis associated with status epilepticus: MR findings. AJNR Am J Neuroradiol. 21(10):1837-40, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11110535%5Bpmid%5D) +1. [Kim SS et al: Focal lesion in the splenium of the corpus callosum in epileptic patients: antiepileptic drug toxicity? AJNR Am J Neuroradiol. 20(1):125-9, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=9974067%5Bpmid%5D) +1. [Aykut-Bingol C et al: Reversible MRI lesions after seizures. Seizure. 6(3):237-9, 1997](http://www.ncbi.nlm.nih.gov/pubmed/?term=9203254%5Bpmid%5D) +1. [Chan S et al: Reversible signal abnormalities in the hippocampus and neocortex after prolonged seizures. AJNR Am J Neuroradiol. 17(9):1725-31, 1996](http://www.ncbi.nlm.nih.gov/pubmed/?term=8896629%5Bpmid%5D) +1. [Cox JE et al: Seizure-induced transient hippocampal abnormalities on MR: correlation with positron emission tomography and electroencephalography. AJNR Am J Neuroradiol. 16(8):1736-8, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7502985%5Bpmid%5D) +1. [Wasterlain CG et al: Pathophysiological mechanisms of brain damage from status epilepticus. Epilepsia. 34 Suppl 1:S37-53, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8385002%5Bpmid%5D) + +## Anatomy + +### Default Mode Network +Brain/ANATOMY:a29f7551-d39d-4deb-933e-b8d2816168c3 + +### Gyral/Sulcal Anatomy +Brain/ANATOMY:849da2a0-4a32-4a07-8f00-c69291e59434 + +### Language Overview +Brain/ANATOMY:40f2ed79-0d31-4943-aaa2-7c3244a7e87b + +### Functional Network Overview +Brain/ANATOMY:ef0be4c8-3d36-4ca9-b4c5-f22f66d2b367 + +### Attention Control Network +Brain/ANATOMY:a1bedda5-6478-40b2-98e7-6c5f5363b06f + +### Limbic Network +Brain/ANATOMY:e1a20b61-b2c1-44c5-ba04-59843855bfef + +### Memory Overview +Brain/ANATOMY:40e2e25f-421b-4653-94e3-641b09b47e4d + +### Social Brain Anatomy +Brain/ANATOMY:0352d34a-5966-494e-b9c3-c26bde257bca + +### Cerebral Hemispheres Overview +Brain/ANATOMY:7006e397-5012-4027-aff8-e8d7158166ee + +### Gyral/Sulcal Anatomy +Brain/ANATOMY:299a5990-1805-4018-85b5-191d8416385b + +### Functional Network Overview +Brain/ANATOMY:7b97f239-0f6f-4809-ac44-594cdf4842d5 + +### Brain +Ultrasound/ANATOMY:080771c2-02f3-408d-ad70-04a80d849500 + +### Limbic System +Brain/ANATOMY:f2a117ed-9429-441d-baa0-5e99e05722ac + +## Cases + +- {'cases': [{'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}, {'key': '2f45db48-acf1-4676-90d6-fe76257ce931', 'value': 'Michael Brant-Zawadzki, MD'}], 'caseVersionId': '29a86215-0920-4c14-863a-8e640ee14244', 'description': "NECT (#1) and CECT scans (#2) showed no abnormality so MR was obtained. T2WI (#3), FLAIR (#4) and DWI (#5) showed no abnormality. MRA (#6) showed no occluded vessels. MR perfusion was obtained and shows markedly increased cerebral blood flow and cerebral blood volume in the right parietal lobe (arrows, #7-9) compared to the normal left side.\n\nThe patient's left-sided weakness resolved over the next several hours. It was concluded he had had an unobserved seizure with Todd's paralysis. The markedly increased cerebral blood flow is from hyperperfusion induced by the seizure.", 'history': 'Elderly patient found down with left hemiparesis and was taken to ER.', 'imagePoolId': '07b96b17-d107-4e84-9fc0-e4d70f9d2e46', 'name': 'After seizure', 'teachingPoint': None}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '64b22fed-89da-4dff-91dd-2a19d04743f7', 'description': 'Sagittal (#1) and axial (#2) T2WIs show hyperintensity in corpus callosum splenium (arrows) caused by transient status epilepticus.', 'history': 'Imaged following status epilepticus.', 'imagePoolId': 'a73a8738-c191-434d-8d07-6dfef3fdcb7e', 'name': 'Seizure hyperperfusion', 'teachingPoint': None}, {'authors': [{'key': '07a2c087-6202-49e7-870b-7aa162d18f06', 'value': 'Bronwyn E. Hamilton, MD'}, {'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'cae90331-1f21-443b-80dd-e30e2ce0e096', 'description': 'Typical case of MR hyperintensities related to status epilepticus. \n\nAxial diffusion image (#1) demonstrates a solitary round focus of increased signal (open arrow). Corresponding ADC map (#2) shows corresponding hypointensity, compatible with true restricted diffusion (arrow). Standard T2 weighted imaging (#3) shows a subtle focus of hyperintensity (curved arrow) in the area of diffusion abnormality. \n\nComments: Transient T2 hyperintense foci may be observed in patients during, or soon after, status epilepticus. Typical areas of involvement include gray matter and/or subcortical white matter, although isolated foci can affect the hippocampus or corpus callosum. Mild mass effect is common. When the corpus callosum is involved, there are two theories regarding pathophysiology: 1.) transient edema due to transhemispheric connectivity of seizure activity, or 2.) reversible demyelination from antiepileptic medications. \n\nDiffusion imaging of such foci usually shows restricted diffusion acutely. If perfusion imaging is performed, there is hyperemia ipsilateral to the epileptic focus, with rCBF elevation. Enhancement is variable; when present, it is typically gyriform or leptomeningeal. Many cases resolve completely in days to weeks. Imaging findings can overlap with infectious and inflammatory pathology such as cerebritis/encephalitis and demyelination (ADEM, MS), and cases of progressing tumor, some of which may also present with seizures, so the diagnosis is aided by clinical information and follow-up imaging.', 'history': 'Patient imaged after episode of status epilepticus. ', 'imagePoolId': 'a0aba9a0-aaf3-43c6-8061-76e61b2de68c', 'name': 'Splenium', 'teachingPoint': None, 'demographics': '58 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}, {'key': 'c0f4b502-f859-48ff-87af-9a4545e51a72', 'value': 'Majda M. Thurnher, MD'}], 'caseVersionId': 'e849ee5f-0bbf-4fac-aaf1-0af9864210ff', 'description': 'Axial DWIs (#1-4) show no definite abnormality. The perfusion images (#5-8) tell another story. They show markedly elevated perfusion in the right temporal and parietal lobes (arrows) compared to the normal left side. PWI MR obtained a few days later on a different scanner (#9) shows almost complete normalization of cerebral perfusion. \n\nComment: Cerebral hyperperfusion may occur after seizure (especially status epilepticus) or--less commonly--following carotid endarterectomy or stenting.', 'history': "Elderly female with high blood pressure, elevated cholesterol, brought to emergency unit after having seizure. Left hemiparesis (a Todd's paralysis) resolved slowly over the next week.", 'imagePoolId': '40bf73ac-3c14-44dd-a790-465b37f51572', 'name': 'Marked hyperperfusion on MR resolves', 'teachingPoint': None, 'demographics': '73 Years old female'}, {'authors': [{'key': '8d5254e9-8dda-478b-8f08-bdee97a32c79', 'value': 'Karen L. Salzman, MD, FACR'}], 'caseVersionId': 'da5cebc9-2f15-48c1-8b0c-1360d8d962fb', 'description': "Typical MR case of reversible seizure induced enhancement related to status epilepticus.\n\nAxial and coronal post-contrast images (#1-2) show gyriform and meningeal enhancement in the right parietal and occipital lobes. Ten days later, once the patient's seizures were well controlled, there is complete resolution of the previously seen enhancement on axial and coronal post-contrast images (#3-4).\n\nComment: MR changes associated with seizures are likely related to transient cerebral edema and/or hyperemia. Acute seizures or status epilepticus may mimic other pathology such as tumor progression or cerebritis.", 'history': 'Patient with a history of status epilepticus immediately prior to imaging. Imaging returned to normal after seizures became controlled.', 'imagePoolId': '9c5c0d2f-bd22-4543-b7c1-a48eba4702cf', 'name': 'Seizure induced enhancement, reversible', 'teachingPoint': None, 'demographics': '30 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'e285f0f1-3428-4aaa-ba3b-392a3a6091e3', 'description': 'Sagittal T1WI (#1) shows absolutely classic "popcorn" lesion at the right temporal tip (arrow). Lesion has locules of mixed iso- and hyperintense fluid and is surrounded by complete hemosiderin rim, best appreciated on the coronal T2WI (#2) where the rim "blooms" (arrow). Findings are characteristic of Zabramski type 2 cavernous malformation. On T1C+ scans an associated enhancing developmental venous anomaly is present (arrows, #3,4). \n\nCavernous-venous is the most common type of histologically mixed vascular malformation.', 'history': 'Temporal lobe epilepsy.', 'imagePoolId': 'd311884f-c3a4-489f-b121-b25eb4b1d025', 'name': 'DVA', 'teachingPoint': None, 'demographics': '18 Years old male'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '9bb600bd-fc5d-4421-b099-a36ff08c69f4', 'description': 'Axial NECT obtained in emergency department showed hyperdense lesion in the left temporal lobe (arrow, #1). An MR was obtained. Sagittal, axial T1WIs (#2-4) show multiple areas of T1 shortening (arrows). FLAIR scan shows a hyperintense ring around the left temporal lobe lesion (arrow, #5) which blooms on GRE (arrow, #6), indicating hemorrhage. T1C+ scans (#7-9) show dura-arachnoid lesions as well as other parenchymal lesions (arrows). \n\nComment: Biopsy of the left parietal dura-arachnoid lesion disclosed metastatic melanoma.', 'history': 'Patient with remote history of melanoma presented with first-time seizure.', 'imagePoolId': '0bc46271-cc7e-49e3-b728-7a60c2479408', 'name': 'Melanoma', 'teachingPoint': None}], 'caseType': 'typical', 'name': 'TYPICAL'} +- {'cases': [{'authors': [{'key': '07a2c087-6202-49e7-870b-7aa162d18f06', 'value': 'Bronwyn E. Hamilton, MD'}], 'caseVersionId': 'b6445eba-f87e-44e8-a6a5-2bfcf1e256af', 'description': "Typical MR imaging case of ictal hyperintensities with follow-up imaging showing resolution. \n\nInitial MR images (Figs. 1-4) performed shortly following a long episode of status epilepticus show increased T2 signal (Figs. 1-3) involving the left temporal lobe cortex and associated subcortical white matter . Coronal FLAIR image (Fig. 3) shows high signal in the left thalamus also . Enhanced image (Fig. 4) shows edema and mild vascular congestion in the left parietal and temporal lobes .\n \nImaging performed 1 month later (Figs. 5-7), after treatment and clinical improvement, show near-complete resolution of the previous imaging abnormalities. Subtle increased signal persists (Figs. 5-6) along the left insular cortex and left medial temporal cortex (, Fig. 7).", 'history': 'New onset atypical seizures.', 'imagePoolId': '7b4df45b-9cc7-41a6-a899-bf9f9648d087', 'name': 'Transient', 'teachingPoint': 'Status epilepticus can result in MR changes that are believed related to transient cerebral edema. T2 hyperintensity in affected areas is most typical with associated mass effect. Although the hippocampus and splenium of corpus callosum are commonly involved, the abnormalities can be quite extensive, as in this case. Cortical and subcortical white matter involvement is characteristic. Restricted diffusion can be present acutely, and enhancement varies from none to extensive gyriform or leptomeningeal enhancement. Perfusion MR shows marked hyperemia on the side of the epileptic focus acutely with elevated rCBF maps. Follow-up imaging can demonstrate complete resolution in days to weeks. If the clinical course was complicated by hypoxemia, secondary ischemia can occur however, with resultant atrophy &/or gliosis of involved areas.', 'demographics': '48 Years old female'}, {'authors': [{'key': '07a2c087-6202-49e7-870b-7aa162d18f06', 'value': 'Bronwyn E. Hamilton, MD'}, {'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': '6175bd6a-35ab-49d0-8c7f-f1327aa34208', 'description': "MR imaging case of acute status epilepticus signal hyperintensities with lobar involvement mimicking encephalitis. \n\nAxial T1 images (Figs. 1-2) suggest mild thickening of the right temporal lobe cortex and right hippocampus . T2 imaging (Figs. 3-4) show to better advantage the cortical thickening, and demonstrates hyperintense T2 signal changes in the right temporal cortex and hippocampus .", 'history': 'Status epilepticus.', 'imagePoolId': 'a16619fc-9f09-4e05-97ea-ed2c5e2a9ff0', 'name': 'Temporal lobe', 'teachingPoint': 'Mild edema and mass effect with corresponding T2 hyperintensity are typical imaging findings, which may be seen in acute status epilepticus. FLAIR and T2 images are most sensitive to detect signal abnormalities. Enhancement is variably present, and is typically gyriform or leptomeningeal. MR spectroscopy has shown lipids &/or lactate acutely in the hippocampi of temporal lobe epilepsy patients within 24 hours of their last seizure; however, follow-up imaging has not shown persistence of the metabolic abnormalities. While imaging hyperintensities may be focal, commonly localized to the hippocampi &/or corpus callosum (particularly the splenium), more diffuse involvement can occur, as in this case. Cortical and subcortical white matter involvement is characteristic, and can mimic infectious/inflammatory and neoplastic conditions. Follow-up imaging in such cases usually shows resolution of the acute imaging abnormalities in treated patients, with development of atrophy in some cases. If the clinical course is complicated by hypoxemia, infarction can occur however.', 'demographics': '20 Years old female'}, {'authors': [{'key': '5cff4116-3654-4b3a-bb75-5ebe0b8c9850', 'value': 'Anne G. Osborn, MD, FACR'}], 'caseVersionId': 'f0ff580f-9f85-433f-8c03-4cf1ea7775aa', 'description': 'NECT scan (#1) shows faint hyperdensity in the left posterior temporal lobe (arrow). CECT scan (#2) shows mild enhancement (arrow) when compared to the normal right side. Follow-up scan two weeks later was normal. This case is unusual because edema is low density. Why the hyperdensity? Perhaps mild increase in cerebral blood volume? The enhancement would indicate some degree of blood-brain-barrier disruption.', 'history': 'Prolonged left temporal lobe seizure imaged 36 hours after ictus.', 'imagePoolId': '63956ca6-353e-4161-8da4-0b6c1c700630', 'name': 'Hyperemia seizure induced', 'teachingPoint': None, 'demographics': '30 Years old male'}], 'caseType': 'variant', 'name': 'VARIANT'} + + +## Images + + +### Selected Images + +![Axial DWI on day 1 in a patient presenting with status epilepticus (SE) demonstrates gyriform restricted diffusion .](images/app.statdx.com_image_thumbnail_7d6ed913-44f2-4be5-965e-afdd3c921b6c_annotated_true_size_900_quality_90_c48275e4ea082a6bf228f3ce3e5ea2723748e84b.jpg) +*Axial DWI on day 1 in a patient presenting with status epilepticus (SE) demonstrates gyriform restricted diffusion .* + +![Axial DWI on day 1 in a patient presenting with status epilepticus (SE) demonstrates gyriform restricted diffusion .](images/app.statdx.com_image_thumbnail_7d6ed913-44f2-4be5-965e-afdd3c921b6c_size_174_quality_85_f19f60ae041ce976150bfacb9eb392a23800810e.jpg) +*Axial DWI on day 1 in a patient presenting with status epilepticus (SE) demonstrates gyriform restricted diffusion .* + +![Axial FLAIR MR in the same patient on day 1 shows cortical and subcortical edema with blurring of the gray matter-white matter interface. Postcontrast images (not shown) demonstrated gyriform enhancement. Prolonged ictal activity induces hypermetabolism with increased glucose utilization. MR signal abnormalities in SE are related to transient vasogenic &/or cytotoxic edema.](images/app.statdx.com_image_thumbnail_a589f756-e538-4fc5-a2fb-58898117aa19_annotated_true_size_900_quality_90_fb1e9954e62faad1ddc1fbc5373a763f26ce91ba.jpg) +*Axial FLAIR MR in the same patient on day 1 shows cortical and subcortical edema with blurring of the gray matter-white matter interface. Postcontrast images (not shown) demonstrated gyriform enhancement. Prolonged ictal activity induces hypermetabolism with increased glucose utilization. MR signal abnormalities in SE are related to transient vasogenic &/or cytotoxic edema.* + +![Axial FLAIR MR in the same patient on day 25 shows prominence of the sulci due to cortical volume loss. Note subcortical white matter hyperintensity due to gliosis.](images/app.statdx.com_image_thumbnail_c061b205-bd0b-4d1e-b5f1-41bad9ab9e97_annotated_true_size_900_quality_90_17e035d5c48688d97e62d704c4f203765c470b4a.jpg) +*Axial FLAIR MR in the same patient on day 25 shows prominence of the sulci due to cortical volume loss. Note subcortical white matter hyperintensity due to gliosis.* + +![Axial T1 MR in the same patient on day 25 shows cortical T1 hyperintensity due to laminar necrosis. Although SE-related imaging abnormalities may completely reverse following cessation of seizures, prolonged seizure activity can result in permanent abnormalities, as seen in this case.](images/app.statdx.com_image_thumbnail_56366665-0dbd-4ac3-b014-e3d1955e5034_annotated_true_size_900_quality_90_7bca9aacbed9e10ecedacdefaa6e3632f6f48ab6.jpg) +*Axial T1 MR in the same patient on day 25 shows cortical T1 hyperintensity due to laminar necrosis. Although SE-related imaging abnormalities may completely reverse following cessation of seizures, prolonged seizure activity can result in permanent abnormalities, as seen in this case.* + +![Axial FLAIR MR in a patient with SE demonstrates extensive cortical and subcortical edema in the right posterior cerebral hemisphere with mass effect. DWI (not shown) demonstrated cortical restricted diffusion.](images/app.statdx.com_image_thumbnail_bcee17a7-f589-41fa-9e2f-7f4b432df51f_annotated_true_size_900_quality_90_419a4615e8be4e11514f1f56ed0e48f3e0b1d546.jpg) +*Axial FLAIR MR in a patient with SE demonstrates extensive cortical and subcortical edema in the right posterior cerebral hemisphere with mass effect. DWI (not shown) demonstrated cortical restricted diffusion.* + +![Axial rCBV map in the same patient shows increased CBV in the right posterior cerebral hemisphere compared with the left. Cerebral hyperperfusion may occur after seizures, particularly with SE.](images/app.statdx.com_image_thumbnail_8e678832-c710-4d6d-b254-cbd0b73ff342_annotated_true_size_900_quality_90_7798282422cb625c38a7abc7e78226d85666166b.jpg) +*Axial rCBV map in the same patient shows increased CBV in the right posterior cerebral hemisphere compared with the left. Cerebral hyperperfusion may occur after seizures, particularly with SE.* + +![Sagittal T1 C+ MR in the same patient demonstrates extensive gyriform enhancement . SE leads to increased glucose utilization and oxygen extraction as well as blood-brain barrier breakdown.](images/app.statdx.com_image_thumbnail_dcd2c8c8-1ec9-4508-bdbc-9a9ffc437b24_annotated_true_size_900_quality_90_b8a64aa01b0380d5d6d5ce04875c8ab5766806c7.jpg) +*Sagittal T1 C+ MR in the same patient demonstrates extensive gyriform enhancement . SE leads to increased glucose utilization and oxygen extraction as well as blood-brain barrier breakdown.* + +![Axial DWI MR shows restricted diffusion in the splenium of the corpus callosum . Follow-up MR showed complete resolution. A transient splenial lesion can be seen with seizures, antiepileptic medications, PRES, viral infections, and hypoglycemia.](images/app.statdx.com_image_thumbnail_ecf60cb3-4121-4453-ad96-96d3f0ae5551_annotated_true_size_900_quality_90_f9773a5abaf7c643d69526cc982330834352910c.jpg) +*Axial DWI MR shows restricted diffusion in the splenium of the corpus callosum . Follow-up MR showed complete resolution. A transient splenial lesion can be seen with seizures, antiepileptic medications, PRES, viral infections, and hypoglycemia.* + +![Axial FLAIR MR in a patient with SE shows right temporal and parietal cortex/subcortical hyperintensity . Note high signal in the pulvinar nucleus of right thalamus .](images/app.statdx.com_image_thumbnail_2c69ee00-7b4c-4f78-87fe-f8f905da7ce9_annotated_true_size_900_quality_90_a68c3bc071655776ade365f87d41bb79229e608a.jpg) +*Axial FLAIR MR in a patient with SE shows right temporal and parietal cortex/subcortical hyperintensity . Note high signal in the pulvinar nucleus of right thalamus .* + +![Axial FLAIR MR in the same patient after 1 year shows cystic encephalomalacia , gliosis , and volume loss in the same regions. Although in most cases imaging findings of SE are reversible, persistent imaging abnormalities, such as focal atrophy due to permanent neuronal damage, can occur, as in this case.](images/app.statdx.com_image_thumbnail_112ab8c0-b30e-4845-8431-3ea272fe4a05_annotated_true_size_900_quality_90_62661b944bcd88ac202a15a303a877f4d837ee03.jpg) +*Axial FLAIR MR in the same patient after 1 year shows cystic encephalomalacia , gliosis , and volume loss in the same regions. Although in most cases imaging findings of SE are reversible, persistent imaging abnormalities, such as focal atrophy due to permanent neuronal damage, can occur, as in this case.* + + +### Additional Images + +![Axial T2WI MR shows hyperintensity in the right temporal cortex with mild gyral expansion and sulcal effacement in this 20 year old with SE and temporal lobe epilepsy. Repeat imaging was normal.](images/app.statdx.com_image_thumbnail_2d7e6ea9-7952-4c60-a73d-8bbbd9f4a724_annotated_true_size_900_quality_90_099b2b6a78eb44ba461ec6a7e7a68afd90412e30.jpg) +*Axial T2WI MR shows hyperintensity in the right temporal cortex with mild gyral expansion and sulcal effacement in this 20 year old with SE and temporal lobe epilepsy. Repeat imaging was normal.* + +![Axial T2WI MR shows focal hyperintensity in the splenium of the corpus callosum . The lesion completely resolved after the patient's seizures were treated. (Courtesy D. Mendelson, MD.)](images/app.statdx.com_image_thumbnail_1cc57e48-c122-4a7d-a206-e6f2208e3206_annotated_true_size_900_quality_90_055b96ec2534d1b87b41d4799b11e3e16aaede57.jpg) +*Axial T2WI MR shows focal hyperintensity in the splenium of the corpus callosum . The lesion completely resolved after the patient's seizures were treated. (Courtesy D. Mendelson, MD.)* + +![Axial T2WI MR shows hyperintensity in the mesial temporal lobe that completely resolved on repeat MR in this 49-year-old man with temporal lobe epilepsy, poorly controlled by medication.](5659dca6-ebfe-4d22-ae87-03626556bbac) +*Axial T2WI MR shows hyperintensity in the mesial temporal lobe that completely resolved on repeat MR in this 49-year-old man with temporal lobe epilepsy, poorly controlled by medication.* + +![Axial T1WI MR in a patient with temporal lobe epilepsy and acute seizures shows abnormal hypointensity in the left mesial temporal lobe cortex and subcortical white matter with mild mass effect.](fb6ca82f-1555-460d-82ce-4d782edbcaba) +*Axial T1WI MR in a patient with temporal lobe epilepsy and acute seizures shows abnormal hypointensity in the left mesial temporal lobe cortex and subcortical white matter with mild mass effect.* + +![Axial T1WI C+ MR shows meningeal and gyriform enhancement in a patient with a history of brain tumor resection and new seizures. Although the imaging prompted concern for tumor recurrence, a repeat MR was normal after the seizures had been treated.](7775f755-8264-4abc-ac76-b4b54397c108) +*Axial T1WI C+ MR shows meningeal and gyriform enhancement in a patient with a history of brain tumor resection and new seizures. Although the imaging prompted concern for tumor recurrence, a repeat MR was normal after the seizures had been treated.* + +![Axial FLAIR MR shows hyperintensity in the subcortical white matter with sulcal effacement in this patient with a history of brain tumor resection and new seizures. Complete resolution of these findings was seen on repeat MR.](748efed0-cbe3-4cfa-88ef-9d6531591a45) +*Axial FLAIR MR shows hyperintensity in the subcortical white matter with sulcal effacement in this patient with a history of brain tumor resection and new seizures. Complete resolution of these findings was seen on repeat MR.* + +![Coronal T1WI C+ MR shows gyriform and meningeal enhancement in a patient with a history of SE immediately prior to imaging. The patient's seizures were treated, and repeat imaging was normal.](8e1137b8-ab55-45d1-b606-c5d5da4402d7) +*Coronal T1WI C+ MR shows gyriform and meningeal enhancement in a patient with a history of SE immediately prior to imaging. The patient's seizures were treated, and repeat imaging was normal.* + +![Axial T1WI MR shows mild thickening of the temporal lobe cortex in an SE patient. Cortical and subcortical white matter involvement is characteristic. Follow-up imaging in such cases usually shows resolution of the acute imaging abnormalities in treated patients. Atrophy may be seen chronically.](f5af2fe0-08c6-4d7b-9b1f-98dee7be0b0e) +*Axial T1WI MR shows mild thickening of the temporal lobe cortex in an SE patient. Cortical and subcortical white matter involvement is characteristic. Follow-up imaging in such cases usually shows resolution of the acute imaging abnormalities in treated patients. Atrophy may be seen chronically.* + +![Sagittal T2WI MR shows hyperintensity in the corpus callosum splenium caused by transient SE. Hyperintensity within the splenium may be caused by the seizures or by the antiepileptic medications.](7554ed24-e64d-4300-af84-1ac009a3ec5e) +*Sagittal T2WI MR shows hyperintensity in the corpus callosum splenium caused by transient SE. Hyperintensity within the splenium may be caused by the seizures or by the antiepileptic medications.* + +![Axial DWI MR shows acute restriction in the splenium of the corpus callosum in a patient with SE. Restricted diffusion is often present acutely in these patients. Focal involvement of the corpus callosum splenium or hippocampus may be seen.](4aa810d9-a50b-4781-8500-b9a526bdc724) +*Axial DWI MR shows acute restriction in the splenium of the corpus callosum in a patient with SE. Restricted diffusion is often present acutely in these patients. Focal involvement of the corpus callosum splenium or hippocampus may be seen.* + +![Axial PWI MR in an elderly woman presenting with seizures shows markedly elevated perfusion in the right hemisphere compared to the normal left side. Cerebral hyperperfusion may occur after seizures, particularly with SE.](55b35945-267d-41e6-9e2b-0f524f2ad72c) +*Axial PWI MR in an elderly woman presenting with seizures shows markedly elevated perfusion in the right hemisphere compared to the normal left side. Cerebral hyperperfusion may occur after seizures, particularly with SE.* + +![Coronal T2WI MR shows abnormal hyperintensity in the hippocampi bilaterally , related to temporal lobe SE. Imaging 1 year later showed mesial temporal sclerosis.](c2ba21eb-c44c-4bda-8cb3-e126d3608cd8) +*Coronal T2WI MR shows abnormal hyperintensity in the hippocampi bilaterally , related to temporal lobe SE. Imaging 1 year later showed mesial temporal sclerosis.* + +![Axial T1WI C+ MR in an elderly patient with seizures and a history of stroke shows extensive gyriform enhancement. This enhancement pattern is often seen in subacute stroke, encephalitis, and SE.](ab1f0f10-c057-42bc-88ed-a7ff08e2d1a6) +*Axial T1WI C+ MR in an elderly patient with seizures and a history of stroke shows extensive gyriform enhancement. This enhancement pattern is often seen in subacute stroke, encephalitis, and SE.* + +![Coronal FLAIR MR performed shortly after a long episode of SE shows increased signal involving the left temporal lobe cortex and associated subcortical white matter.](8b68326b-5493-4419-8324-6ad9b71900dd) +*Coronal FLAIR MR performed shortly after a long episode of SE shows increased signal involving the left temporal lobe cortex and associated subcortical white matter.* + +![Axial T1WI C+ FS MR in the same patient shows mild edema and vascular congestion in the left temporal lobe . Imaging 1 month later showed near-complete resolution of the signal abnormalities. SE may result in MR changes that are likely related to transient cerebral edema.](6286c867-986d-4f62-a7ac-f9f0f723cb29) +*Axial T1WI C+ FS MR in the same patient shows mild edema and vascular congestion in the left temporal lobe . Imaging 1 month later showed near-complete resolution of the signal abnormalities. SE may result in MR changes that are likely related to transient cerebral edema.* + +![Axial T1WI MR in a patient with SE shows mild thickening of the left temporal cortex .](06b38e23-730e-4c26-a8e2-abd7b525d956) +*Axial T1WI MR in a patient with SE shows mild thickening of the left temporal cortex .* + +![Axial FLAIR MR in the same patient shows cortical and subcortical white matter edema . MR signal abnormalities are related to transient vasogenic &/or cytotoxic edema. Follow-up imaging in such cases usually shows resolution of the acute imaging abnormalities in treated patients. Atrophy may be seen chronically.](045343b1-1f85-4b43-b95d-407d633cab4c) +*Axial FLAIR MR in the same patient shows cortical and subcortical white matter edema . MR signal abnormalities are related to transient vasogenic &/or cytotoxic edema. Follow-up imaging in such cases usually shows resolution of the acute imaging abnormalities in treated patients. Atrophy may be seen chronically.* + +![Axial DWI MR shows cortical restricted diffusion in a patient with SE. Transient diffusion changes related to seizures can involve the cortex/subcortical white matter, corpus callosum, hippocampus, and pulvinar nucleus of thalamus.](d81b3f39-a3a8-4861-881a-443bd9fce3a5) +*Axial DWI MR shows cortical restricted diffusion in a patient with SE. Transient diffusion changes related to seizures can involve the cortex/subcortical white matter, corpus callosum, hippocampus, and pulvinar nucleus of thalamus.* + +![Axial rCBV map in the same patient shows increased CBV in the same region as the diffusion abnormality. Cerebral hyperperfusion may occur after seizures, particularly with SE.](6e7802ea-8471-438e-ae2e-66cd76728ae9) +*Axial rCBV map in the same patient shows increased CBV in the same region as the diffusion abnormality. Cerebral hyperperfusion may occur after seizures, particularly with SE.* + +![Coronal T1 C+ MR shows gyriform and meningeal enhancement related to SE. After treatment, the MR changes are completely resolved. This enhancement pattern is often seen in subacute stroke and encephalitis.](f664a45e-5f51-4ee1-8a5e-0edc3c31c3b5) +*Coronal T1 C+ MR shows gyriform and meningeal enhancement related to SE. After treatment, the MR changes are completely resolved. This enhancement pattern is often seen in subacute stroke and encephalitis.* + diff --git a/docs_md/articles/stroke-therapy_eecb2d0f-ef14-44df-be8e-137567226412.md b/docs_md/articles/stroke-therapy_eecb2d0f-ef14-44df-be8e-137567226412.md new file mode 100644 index 0000000..020f0be --- /dev/null +++ b/docs_md/articles/stroke-therapy_eecb2d0f-ef14-44df-be8e-137567226412.md @@ -0,0 +1,612 @@ +--- +title: "Stroke Therapy" +docid: "eecb2d0f-ef14-44df-be8e-137567226412" +authors: + - key: "bccfc1a3-a6b8-4707-b2bf-589d9650f769" + value: "Jared Halpin, MD" + - key: "8fe1db5b-d23b-459d-9ab0-0cba2c16a0ac" + value: "Coleman O. Martin, MD" +breadcrumbs: + - + name: "Interventional Radiology" + slug: "interventional-radiology" + treeNodeId: "20225e4f-e0f0-447f-8bfc-151c06ebe73f" + - + name: "Procedures" + slug: "procedures" + treeNodeId: "4f9c2b36-60f0-40e8-8cfc-c1482c9f1ed0" + - + name: "Arterial Procedures" + slug: "arterial-procedures" + treeNodeId: "b554f24b-18b5-4e89-8f22-d22849ead8e7" + - + name: "Cranial Revascularization" + slug: "cranial-revascularization" + treeNodeId: "9c47d8e4-b6c5-4251-93f0-1f0554c79fd4" + - + name: "Stroke Therapy" + slug: "stroke-therapy" + treeNodeId: null +category: "Interventional Radiology" +cmeTopicId: "f63224f3-1455-4b82-a813-021294fdd3ad" +documentVersionId: "44037350-bdbf-46ea-91e6-1a6faa35f508" +imageCount: 36 +lastUpdated: "05/13/22" +pageDescription: "Stroke Therapy" +pageKeywords: "Interventional Radiology, Procedures, Arterial Procedures, Cranial Revascularization, Stroke Therapy" +pageTitle: "Stroke Therapy | STATdx" +enhancedTitle: "Stroke Therapy" +type: "PROCEDURE" +references: true +breadcrumbs: + - "Interventional Radiology" + - "Procedures" + - "Arterial Procedures" + - "Cranial Revascularization" + - "Stroke Therapy" +--- +# KEY FACTS + +- ## Terminology + + + - **Stroke**: Rapidly developing loss of brain function(s) due to disturbance in blood supply to brain + - **Core infarct**: Central area of severe ischemia/evolving infarction; unlikely to respond to treatment + - **Ischemic penumbra**: Surrounding ischemic tissue rim between normally perfused brain and evolving infarction +- ## Preprocedure + + + - Noncontrast head CT: Exclude hemorrhage, evaluate extent of core infarct [Alberta Stroke Program Early CT Score (ASPECTS)] + - CTA head/neck ± CT head perfusion: Localize arterial occlusion, detection of penumbra + - Thrombectomy inclusion criteria + - Large vessel occlusion; symptoms ≤ 6-8 hours + - Significant neurologic symptoms [National Institute of Health Stroke Scale (NIHSS) > 4] + - Minimal ischemic burden (ASPECTS ≥ 6) + - Salvageable tissue by perfusion imaging + - Absolute contraindication: Hemorrhage/well-established acute infarct involving > 1/3 of affected vascular territory +- ## Procedure + + + - Thrombectomy with stent retriever + - Placement of balloon guide catheter in internal carotid artery (ICA) or subclavian artery + - Deploy stent retriever device across thrombus, wait 5 min + - Balloon occlusion of ICA/subclavian while retracting device and thrombus into guide catheter + - Aspiration thrombectomy, angioplasty ± stenting, intraarterial thrombolysis as backup in select cases +- ## Post Procedure + + + - Successful recanalization: 72-93% + - Good clinical outcome: 51-60% + - Intracerebral hemorrhage: 3.6% + +# TERMINOLOGY + +- ## Definitions + + + - **Stroke**: Acute loss of brain function(s) due to disturbance in blood supply to brain + - Ischemic stroke: Embolic or thrombotic occlusion of arterial supply to brain (80% of strokes) + - 3 major clinical ischemic stroke subtypes + - Large artery/atherosclerotic strokes (40-50%) + - Cardioembolic disease (20-25%) + - Lacunar infarction (20-30%) + - Hemorrhagic stroke: Rupture/bleeding from brain blood vessel (15% of strokes) + - Nontraumatic subarachnoid hemorrhage (5%) + - Vasculitis + - Venous infarction (< 1%) + - **Core infarct**: Central area of severe ischemia/evolving infarction; unlikely to respond to treatment + - Severely reduced to almost nonexistent blood flow + - Inadequate supply of oxygen and glucose + - Neuronal and glial necrosis + - Irreversible damage; no response to reperfusion + - **Ischemic penumbra**: Surrounding rim of ischemic tissue between normally perfused tissue and area of evolving infarction + - Parenchymal tissue at risk of eventual infarction + - Dependent upon collateral perfusion + - Collateral circulation inadequate to maintain neuronal oxygen/glucose demand indefinitely + - May remain viable for several hours + - Target of acute stroke intervention + - **Endovascular therapy (ET)**: Standard of care for acute ischemic stroke with emergent large vessel occlusion (ELVO) + - Potential reperfusion techniques + - Intravenous (IV) thrombolysis [IV tissue plasminogen activator (tPA)] + - Mechanical thrombectomy (stent retriever, aspiration catheter) + - Balloon angioplasty/stenting + - Intraarterial (IA) thrombolysis + - **Stroke-related scales/grades** + - **National Institute of Health Stroke Scale (NIHSS)** + - Clinical assessment tool used to quantify neurologic impairment caused by acute stroke + - Predictor of short- and long-term outcome + +# PREPROCEDURE + +- ## Indications + + + - **IV tPA** + - Patients with acute neurologic deficit expected to result in long-term disability + - NECT shows no hemorrhage and no large infarct + - Clearly defined onset time (last known well time) and presentation within 3-4.5 hours of symptom onset + - No contraindication to tPA + - **ET** + - Patients with large vessel occlusions documented by CTA, DSA, or MRA + - Anterior circulation: When revascularization is feasible within 6-8 hours of symptom onset + - Posterior circulation: Time window undefined but within ≤ 12-24 hours of symptom onset + - NIHSS > 4, exceptions for cases of isolated aphasia or hemianopsia + - No large infarct on NECT [Alberta Stroke Program Early CT Score (ASPECTS) ≥ 6] + - Significant area of salvageable tissue seen on perfusion imaging +- ## Contraindications + + + - **Absolute contraindications to ET** + - Intracranial hemorrhage (ICH) + - Established acute infarct involving > 1/3 of affected vascular territory + - ASPECTS ≤ 5 has greater risk of hemorrhage + - CNS lesion with elevated likelihood of hemorrhage during revascularization + - Abscess + - Aneurysm + - Brain tumor + - Established bacterial endocarditis + - **Relative contraindications (general)** + - Mild or rapidly improving deficits + - Stroke or ICH in same territory within 3 months + - Suspected bacterial endocarditis + - Life expectancy < 1 year from other causes +- ## Preprocedure Imaging + + + - **Noncontrast head CT** + - Exclude ICH + - Look for established infarct (frank hypodensity) + - Calculate ASPECT score + - 10-point grading scale for severity of middle cerebral artery (MCA) stroke on NECT + - 1 point deducted from initial score of 10 for every region involved + - Caudate, putamen, internal capsule, insular cortex, and 6 regions of MCA cortex (3 superior, 3 inferior) + - **CTA head and neck** + - Localize site of arterial occlusion(s) or stenosis + - Evaluate for vascular tortuosity or variant vascular anatomy + - **CT head perfusion** + - Mean transit time + - Average blood transit time through brain region + - Highly sensitive parameter for detection of brain tissue oligemia + - Time to peak/TMax + - Time to peak density of contrast through brain region + - Highly sensitive parameter for detection of brain tissue oligemia + - Cerebral blood flow (CBF) + - Blood volume moving through brain + - Reasonable marker for extent of impending infarction + - Cerebral blood volume (CBV) + - Total blood volume in given unit volume of brain + - Reasonable marker for core infarct + - Larger core infarct is marker for poor outcome + - CBF/CBV mismatch defines extent of penumbra + - **MR diffusion (DWI) and MR perfusion (PWI) imaging** + - Added screening time and scan time limits usefulness in emergent stroke evaluation + - DWI most reliable estimate of core infarct + - DWI/PWI mismatch defines extent of penumbra + - Helpful in basilar distribution stroke where CT perfusion is less accurate +- ## Getting Started + + + - Things to check + - Focused clinical history/physical examination + - Pinpoint time of onset of stroke symptoms + - If symptoms present upon awakening, consider onset as when patient was last symptom free + - Obtain NIHSS + - Assess thrombolysis inclusion vs. exclusion + - Exclude conditions that mimic stroke (e.g., seizure, toxic/metabolic syndromes, hypoglycemia, tumor) + - Allergies to sedatives, contrast + - Current medications, including any anticoagulants + - Laboratory parameters + - Electrolytes, estimated glomerular filtration rate (eGFR) + - CBC + - Platelet count > 50,000/μL + - Coagulation profile + - Consent for procedure + - Discuss risk of hemorrhagic stroke, vascular injury, allergic reaction, renal failure, access site hematoma + - ### Medications + + + - IV tPA**** + - Infuse 0.9 mg/kg (maximum 90 mg) over 60 minutes; bolus first 10% of dose over 1 minute + - Maintain systolic blood pressure (SBP) < 180 mm Hg; diastolic blood pressure (DBP) < 105 mm Hg + - If new severe headache, acute hypertension, or nausea, obtain head CT + - Acceptable to stop infusion to perform thrombectomy or continue infusion during thrombectomy + - Patient management after thrombolysis + - No anticoagulation/antiplatelet therapy for 24 hours + - Procedural sedation + - Usually performed without sedation or with light sedation using fentanyl and midazolam if needed + - Use of general anesthesia is associated with worse neurologic outcomes + - Posterior circulation stroke may require airway support + - ### Equipment list + + + - Sheaths/guide catheters + - Various proprietary designs + - Femoral arterial sheath + - Balloon guide catheter for flow arrest during withdrawal of thrombus + - Guidewires + - Hydrophilic 0.035" or 0.038" guidewire + - Microwire for use with appropriate microcatheter + - Equipment for mechanical thrombectomy + - Stent retrievers for ensnaring and withdrawing thrombus + - Reperfusion catheters for local aspiration of thrombus + - May do combined stent retriever, local aspiration, and balloon guide technique + - Angioplasty balloons for concomitant angioplasty and carotid stenting + - Rapid-exchange or monorail systems preferred + +# PROCEDURE + +- ## Patient Position/Location + + + - Supine position, biplane angiography suite +- ## Procedure Steps + + + - **Diagnostic angiogram** + - Sterilely prepare/drape skin of access site + - Transfemoral access site preferred; transbrachial or transcarotid access if needed + - Conscious sedation if necessary + - Obtain femoral arterial access + - Place femoral artery access sheath + - 4-6 Fr (for diagnostic angiogram) + - 8 Fr (for thrombectomy with balloon guide) + - Select desired carotid/vertebral artery with Glidewire/diagnostic catheter + - Use diagnostic catheter to obtain DSA images + - Carotid/vertebral DSA: Evaluates collateral circulation/filling of vessels distal to occlusion + - Include cervical and cranial views + - Obtain magnified images of target vessel(s), document vascular occlusion + - **Thrombectomy with stent retriever device** + - Useful for occlusions out to M3 segment in MCA or P1 segment in posterior cerebral artery + - Advance balloon guide catheter over wire into internal carotid artery (ICA) + - Navigate microwire/microcatheter into occluded segment + - Pass occlusion site with microwire/microcatheter + - Obtain DSA; verify position of microcatheter tip within vessel, distal to thrombus + - Advance stent retriever device and deploy across thrombus + - Wait 5 minutes for device to interact with thrombus + - Inflate balloon at guide catheter tip within ICA for temporary occlusion + - Aspirate lumen of balloon guide catheter + - Check for thrombus within device or aspirate + - Check Touhy valve for thrombus + - Deflate balloon + - Verify backflow of blood out from balloon guide to ensure thrombus not trapped within guide catheter + - Obtain DSA run through guide catheter to evaluate for residual thrombus or distal emboli + - Assess thrombolysis in cerebral ischemia (TICI) score + - If needed, reload stent retriever device for additional passes + - Average 1.6 passes required + - Atherosclerotic stenosis or dissection can be treated with angioplasty/stenting in select cases + - Cervical run of ICA to ensure no spasm or dissection from guide catheter + - Pull guide catheter to iliac artery; pelvic run to ensure proper placement of sheath for hemostatic closure device + - **Thrombectomy with aspiration catheter** + - Most useful for occlusion within large straight segments, such as paraclinoid ICA, M1, or basilar + - Position long 6-Fr (or larger) sheath in carotid or subclavian artery + - Choose reperfusion catheter size based on occluded vessel diameter + - Advance reperfusion catheter into target vessel over microcatheter/microwire + - Confirm catheter position with DSA imaging + - Engage proximal face of thrombus with reperfusion catheter and aspirate by syringe or aspiration pump + - Follow results with DSA angiography + - **Thrombectomy combining stent retriever and local aspiration** + - Useful when balloon guide catheter placement in ICA not possible or contraindicated + - Position long 6-Fr sheath or balloon guide catheter in carotid or subclavian artery + - Choose reperfusion catheter based on occluded artery diameter + - Navigate reperfusion catheter over microcatheter and microwire to target artery + - Pass occlusion with microcatheter and deploy stent retriever device across thrombus + - While reperfusion catheter is being aspirated, withdraw stent retrieval device into catheter + - Search for thrombus ensnared in device or aspirate + - Repeat if necessary + - **Intracranial angioplasty ± stenting** (not FDA approved for acute stroke) + - Use if recanalization attempts unsuccessful due to underlying stenosis + - Angioplasty balloon size based on occluded vessel + - Use balloon slightly smaller than vessel caliber + - Recommend noncompliant angioplasty balloon + - Advance balloon into occluded segment, inflate + - Perform follow-up DSA + - **Associated cervical carotid stenosis/occlusion** + - Acute stenting of high-grade stenosis/occlusion may be required to achieve access for thrombectomy + - Probing stump of occluded ICA often reveals residual lumen in acute stroke + - Advance microcatheter to distal ICA + - Angiogram through microcatheter will demonstrate thrombus burden within ICA + - Place exchange wire in distal ICA to facilitate placement of distal embolic protection filter device, if possible + - Occluded ICA may have to initially be predilated with very small balloon + - Vagus nerve induced bradycardia may be profound + - Premedicate with atropine or glycopyrrolate + - Stent retriever use after stenting + - Requires placement of balloon guide catheter distal to stent or use of reperfusion catheter + - Avoid retracting stent retriever through ICA stent + - In setting of significant ischemic burden, consider ASA monotherapy vs. clopidogrel without load + - **Associated vertebral ostial stenosis with basilar occlusion** + - If possible, perform thrombectomy through contralateral (nondiseased) vertebral artery + - If thrombectomy requires intervention through stenotic/occluded vertebral ostium + - Angioplasty vertebral ostium without distal embolic protection + - Use reperfusion catheter in basilar ± stent retriever + - Stent vertebral ostium after basilar intervention complete + - **Extracranial carotid/vertebral dissection/stroke** + - Similar approach to cervical carotid stenosis/occlusion + - Distal embolic protection often not possible due to dissection extending to skull base + - Cross dissection with microcatheter + - Verify microcatheter placement in true lumen with DSA run + - Over exchange wire, place self-expanding stent(s) + - Stent often traps flap and thrombus, restoring flow +- ## Findings and Reporting + + + - Location/characteristics of lesion + - Devices/medications used intraprocedurally + - Procedure times + - Door to groin time + - Time of reperfusion + - TICI grade of perfusion after stroke intervention + - Angiographic degree of reperfusion + - 0: No perfusion beyond occlusion + - 1: Penetration past occlusion but no perfusion + - 2a: Partial perfusion; incomplete distal branch filling of < 50% of territory of occluded vessel + - 2b: Partial perfusion; incomplete distal branch filling of ≥ 50% of territory of occluded vessel + - 2c: Near-complete perfusion; no clearly visible thrombus but delayed contrast runoff + - 3: Full perfusion; filling of all distal branches of expected territory in normal fashion + - Complications +- ## Alternative Procedures/Therapies + + + - ### Radiologic + + + - Direct carotid access if unable to select common carotid artery/ICA due to tortuosity + - IA tPA + - Once considered primary treatment, now only in rare select cases + - Combined strategies: IV followed by thrombectomy + - ### Surgical + + + - Hemicraniectomy for large MCA strokes to avoid herniation from edema + +# POST PROCEDURE + +- ## Things to Do + + + - Patient management after IA therapy + - Admission to ICU + - Neurologic exam/vital signs q 15 minutes 2 hours, q 30 minutes for 6 hours, q 1 hour for 24 hours + - Maintain SBP of 100-160 mm Hg + - Head CT for any neurologic status changes + - Postprocedure medications (procedure dependent) + - If carotid stent required + - ASA 325 mg, clopidogrel 150- to 300-mg loading dose (if ischemic burden low) + - Maintain on daily clopidogrel 75 mg for 4-6 weeks, daily ASA 325 mg indefinitely + - Atropine or glycopyrrolate for vagus nerve-mediated bradycardia/hypotension; pressors if needed + +# OUTCOMES + +- ## Complications + + + - Brain hemorrhage + - May categorize postthrombolysis hemorrhagic transformations (HT) by CT-based classification of European Cooperative Acute Stroke Study (ECASS) + - No HT + - Hemorrhagic infarct type 1 (HI1): Small petechiae + - HI2: Medium petechiae + - Parenchymal hematoma type 1 (PH1): HI large (≤ 30% of infarcted area; mild mass effect) + - PH2: Hematoma (≥ 30% of infarcted area; significant mass effect) + - Mechanical thrombectomy complications + - Device-related complications + - Vascular perforation + - Arterial dissection + - Embolization to previously uninvolved territory + - Worsening brain infarction + - Malignant brain edema of infarcted territory + - Bleeding/hematoma at arterial access site + - Angioplasty/stenting complications + - Similar device-related complications + - Vascular perforation + - Arterial dissection + - Vagus nerve-mediated bradycardia/hypotension +- ## Management of Complications + + + - Asymptomatic/small hemorrhage (< 30 mL) + - Tight blood pressure control (SBP < 160 mm Hg) + - Frequent neurologic exams + - Reverse anticoagulation + - Consider giving clotting factors/platelets + - Symptomatic, significant hemorrhage (> 30 mL) + - Reverse anticoagulation + - Consider: Ventriculostomy (symptomatic hydrocephalus); craniotomy (clot evacuation) +- ## Expected Outcomes + + + - Mechanical thrombectomy + - Successful recanalization (72-93%) + - Good clinical outcome; modified Rankin scale ≤ 2 at 90 days (51-60%) + - Slight disability; able to look after own affairs without assistance, but unable to carry out all previous activities + - Significant procedural complications (1-3%) + - Symptomatic intracerebral hemorrhage (3.6%) + - Mortality (10-12%) + - Lower success rate and higher morbidity/mortality with basilar artery occlusions + + 330162ac-9d2d-4184-8687-392681ef35a5 + +## References + +# Selected References + +1. [Roaldsen MB et al: Intravenous thrombolytic treatment and endovascular thrombectomy for ischaemic wake-up stroke. Cochrane Database Syst Rev. 12:CD010995, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34850380%5Bpmid%5D) +1. [Weller JM et al: Endovascular treatment for acute stroke in cerebral amyloid angiopathy. Stroke. 52(10):e581-5, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34412512%5Bpmid%5D) +1. [Campbell BCV et al: Stroke. Lancet. 396(10244):129-42, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32653056%5Bpmid%5D) +1. [Herpich F et al: Management of acute ischemic stroke. Crit Care Med. 48(11):1654-63, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32947473%5Bpmid%5D) +1. [Sairanen T et al: Should we thrombolyse prior to endovascular treatment in acute stroke? Clin Neurol Neurosurg. 177:117-22, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30446414%5Bpmid%5D) +1. [Berkhemer OA et al: A randomized trial of intraarterial treatment for acute ischemic stroke. N Engl J Med. 372(1):11-20, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25517348%5Bpmid%5D) +1. [Goyal M et al: Randomized assessment of rapid endovascular treatment of ischemic stroke. N Engl J Med. 372(11):1019-30, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25671798%5Bpmid%5D) +1. [Saver JL et al: Stent-retriever thrombectomy after intravenous t-PA vs. t-PA alone in stroke. N Engl J Med. 372(24):2285-95, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25882376%5Bpmid%5D) +1. [Sheth SA et al: M2 occlusions as targets for endovascular therapy: comprehensive analysis of diffusion/perfusion MRI, angiography, and clinical outcomes. J Neurointerv Surg. 7(7):478-83, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=24821842%5Bpmid%5D) +1. [Campbell BC et al: A multicenter, randomized, controlled study to investigate EXtending the time for Thrombolysis in Emergency Neurological Deficits with Intra-Arterial therapy (EXTEND-IA). Int J Stroke. 9(1):126-32, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24207098%5Bpmid%5D) +1. [Nguyen TN et al: Balloon guide catheter improves revascularization and clinical outcomes with the Solitaire device: analysis of the North American Solitaire Acute Stroke Registry. Stroke. 45(1):141-5, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24302483%5Bpmid%5D) +1. [Davis MJ et al: Anesthetic management and outcome in patients during endovascular therapy for acute stroke. Anesthesiology. 116(2):396-405, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22222475%5Bpmid%5D) +1. [Saver JL et al: Solitaire flow restoration device versus the Merci Retriever in patients with acute ischaemic stroke (SWIFT): a randomised, parallel-group, non-inferiority trial. Lancet. 380(9849):1241-9, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22932715%5Bpmid%5D) +1. [Konstas AA et al: CT perfusion imaging of acute stroke: the need for arrival time, delay insensitive, and standardized postprocessing algorithms? Radiology. 254(1):22-5, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20032139%5Bpmid%5D) +1. [Konstas AA et al: Theoretic basis and technical implementations of CT perfusion in acute ischemic stroke, part 1: Theoretic basis. AJNR Am J Neuroradiol. 30(4):662-8, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19270105%5Bpmid%5D) +1. [Konstas AA et al: Theoretic basis and technical implementations of CT perfusion in acute ischemic stroke, part 2: technical implementations. AJNR Am J Neuroradiol. 30(5):885-92, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19299489%5Bpmid%5D) +1. Hurst RW et al: Interventional Neuroradiology. New York: Informa Healthcare, 2008 +1. [Smith WS et al: Mechanical thrombectomy for acute ischemic stroke: final results of the Multi MERCI trial. Stroke. 39(4):1205-12, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18309168%5Bpmid%5D) +1. Gonzalez RG et al: Acute Ischemic Stroke: Imaging and Intervention. Berlin Heidelberg: Springer-Verlag, 2006 +1. [Hacke W et al: Intravenous thrombolysis with recombinant tissue plasminogen activator for acute hemispheric stroke. The European Cooperative Acute Stroke Study (ECASS). JAMA. 274(13):1017-25, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7563451%5Bpmid%5D) +1. [Bonita R et al: Recovery of motor function after stroke. Stroke. 19(12):1497-500, 1988](http://www.ncbi.nlm.nih.gov/pubmed/?term=3201508%5Bpmid%5D) +1. [van Swieten JC et al: Interobserver agreement for the assessment of handicap in stroke patients. Stroke. 19(5):604-7, 1988](http://www.ncbi.nlm.nih.gov/pubmed/?term=3363593%5Bpmid%5D) +1. [Mahoney FI et al: Functional evaluation: the Barthel Index. Md State Med J. 14:61-5, 1965](http://www.ncbi.nlm.nih.gov/pubmed/?term=14258950%5Bpmid%5D) +1. [Rankin J: Cerebral vascular accidents in patients over the age of 60. II. Prognosis. Scott Med J. 2(5):200-15, 1957](http://www.ncbi.nlm.nih.gov/pubmed/?term=13432835%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Reformatted CTA shows occlusion of the right middle cerebral artery (MCA), M1 segment . The anterior temporal branch of the right MCA remains patent . The appearance is typical of an embolic occlusion.](images/app.statdx.com_image_thumbnail_77c1e3c7-7635-4c44-930c-be164ebede7c_annotated_true_size_900_quality_90_9dd3d252f7518896dc8cdcffc66749076fe41865.jpg) +**Preprocedural Planning: Acute Stroke Intervention (Initial CTA)** +*Reformatted CTA shows occlusion of the right middle cerebral artery (MCA), M1 segment . The anterior temporal branch of the right MCA remains patent . The appearance is typical of an embolic occlusion.* + +![Reformatted CTA shows occlusion of the right middle cerebral artery (MCA), M1 segment . The anterior temporal branch of the right MCA remains patent . The appearance is typical of an embolic occlusion.](images/app.statdx.com_image_thumbnail_77c1e3c7-7635-4c44-930c-be164ebede7c_size_174_quality_85_e020f7bc49a560f624995c88cf0897d3f8073935.jpg) +**Preprocedural Planning: Acute Stroke Intervention (Initial CTA)** +*Reformatted CTA shows occlusion of the right middle cerebral artery (MCA), M1 segment . The anterior temporal branch of the right MCA remains patent . The appearance is typical of an embolic occlusion.* + +![CT brain perfusion shows a prolonged mean transit time (MTT) in right MCA territory compared to the left side due to the MCA occlusion. MTT, which measures average blood transit time through a given region of the brain, is a sensitive parameter for restricted perfusion, as seen with embolic occlusion.](images/app.statdx.com_image_thumbnail_d66bd34e-1bf8-4b49-91ed-e3964f321c4e_annotated_true_size_900_quality_90_69b6656b449e0b6cd18757a05d9e6ab73826e1cf.jpg) +**Preprocedural Planning: Acute Stroke Intervention (CT Perfusion)** +*CT brain perfusion shows a prolonged mean transit time (MTT) in right MCA territory compared to the left side due to the MCA occlusion. MTT, which measures average blood transit time through a given region of the brain, is a sensitive parameter for restricted perfusion, as seen with embolic occlusion.* + +![Right internal carotid DSA shows occlusion of the right MCA, M1 segment , corresponding to the CTA. Acute stroke therapy with mechanical thrombectomy was then performed.](images/app.statdx.com_image_thumbnail_8b759bfe-0bc5-45bd-9ac2-6a7a345e9349_annotated_true_size_900_quality_90_7427aefce266fe14bf4a91eb2689f8e232717a6b.jpg) +**Intraprocedural: Acute Stroke Intervention (Initial Cerebral Angiography)** +*Right internal carotid DSA shows occlusion of the right MCA, M1 segment , corresponding to the CTA. Acute stroke therapy with mechanical thrombectomy was then performed.* + +![Right internal carotid DSA following mechanical thrombectomy shows a patent MCA and distal branches , which were previously nonperfused. The anterior temporal branch remains patent and is again seen arising from the mid-M1 segment.](images/app.statdx.com_image_thumbnail_578395b5-a256-411d-aea0-e2a1f061e480_annotated_true_size_900_quality_90_01f4ba0f6dccc92ed13115254251c71d94b4ae3e.jpg) +**Intraprocedural: Acute Stroke Intervention (Completion Arteriogram)** +*Right internal carotid DSA following mechanical thrombectomy shows a patent MCA and distal branches , which were previously nonperfused. The anterior temporal branch remains patent and is again seen arising from the mid-M1 segment.* + +![NECT in a 52-year-old man presenting with acute-onset right hemiplegia, aphasia, and neglect demonstrates dense left internal carotid artery (ICA) terminus .](images/app.statdx.com_image_thumbnail_d017316c-f1bc-4a1e-b2f4-4c8c1c8f6327_annotated_true_size_900_quality_90_b14dc64bcc69c9c13690c6eb198e3dd764a4d446.jpg) +**Preprocedural Planning: Left ICA Occlusion (Dense ICA Terminus)** +*NECT in a 52-year-old man presenting with acute-onset right hemiplegia, aphasia, and neglect demonstrates dense left internal carotid artery (ICA) terminus .* + +![CT perfusion in the same patient demonstrates a large ischemic penumbra in the left MCA and anterior carotid artery (ACA) territory secondary to the left ICA occlusion.](images/app.statdx.com_image_thumbnail_9aac286c-13ed-43b0-8699-863aae05963f_annotated_true_size_900_quality_90_e363d132a2d82f86f1912b73773aebd3181dac89.jpg) +**Preprocedural Planning: Left ICA Occlusion (Large Ischemic Penumbra)** +*CT perfusion in the same patient demonstrates a large ischemic penumbra in the left MCA and anterior carotid artery (ACA) territory secondary to the left ICA occlusion.* + +![Lateral DSA demonstrates a filling defect within the distal left ICA , consistent with acute thromboembolus. There is no filling of the left ACA or MCA. The left posterior communicating artery is patent.](images/app.statdx.com_image_thumbnail_dc6a1643-4531-45bc-adcc-1cdd2d630b20_annotated_true_size_900_quality_90_eb9c5ed4100cf9d3a072f07e7d3b0b6a1804681c.jpg) +**Intraprocedural: Left ICA Occlusion (DSA Confirmation)** +*Lateral DSA demonstrates a filling defect within the distal left ICA , consistent with acute thromboembolus. There is no filling of the left ACA or MCA. The left posterior communicating artery is patent.* + +![Lateral unsubtracted angiogram shows the Solitaire device deployed across the left ICA terminus occlusion.](images/app.statdx.com_image_thumbnail_6fa4758a-8c24-4b8f-9add-fea490ce0d74_annotated_true_size_900_quality_90_7a5658fde2672ffc399aa7ae69264e890c842997.jpg) +**Intraprocedural: Left ICA Occlusion (Solitaire Deployed)** +*Lateral unsubtracted angiogram shows the Solitaire device deployed across the left ICA terminus occlusion.* + +![Lateral DSA from the left ICA demonstrates complete restoration of flow to the left ICA territory [thrombolysis in cerebral ischemia (TICI) 3]. This was obtained after 1 pass with the Solitaire 4 x 40-mm device.](images/app.statdx.com_image_thumbnail_3b91212e-959a-490e-98c2-3aa5193b3e5f_annotated_true_size_900_quality_90_8f488f2c63fe93a459f56194614a9081fc7227c0.jpg) +**Intraprocedural: Left ICA Occlusion (Postthrombectomy Angiogram)** +*Lateral DSA from the left ICA demonstrates complete restoration of flow to the left ICA territory [thrombolysis in cerebral ischemia (TICI) 3]. This was obtained after 1 pass with the Solitaire 4 x 40-mm device.* + +![Axial DWI MR demonstrates small areas of acute infarction within the left caudate body and left parietal cortex . These are significantly smaller than the ischemic penumbra on CT perfusion. There was no hemorrhage on GRE sequence (not pictured).](9fd17794-500c-4cc4-92ad-b5bb13ac6ef9) +**Follow-Up: Left ICA Occlusion (Small Ischemic Infarct)** +*Axial DWI MR demonstrates small areas of acute infarction within the left caudate body and left parietal cortex . These are significantly smaller than the ischemic penumbra on CT perfusion. There was no hemorrhage on GRE sequence (not pictured).* + +![This 65-year-old man presented with acute onset of right hemiplegia, facial droop, aphasia, and neglect 3.5 hours prior to arrival to ED. National Institute of Health Stroke Scale (NIHSS) was 21. NECT demonstrates no acute hemorrhage or gray-white loss. There is chronic small vessel ischemic disease.](7a6106b3-1f28-4b5e-8ef0-25b3643a39ca) +**Preprocedural Planning: Acute Left MCA Stroke (Initial CT)** +*This 65-year-old man presented with acute onset of right hemiplegia, facial droop, aphasia, and neglect 3.5 hours prior to arrival to ED. National Institute of Health Stroke Scale (NIHSS) was 21. NECT demonstrates no acute hemorrhage or gray-white loss. There is chronic small vessel ischemic disease.* + +![However, 3D reformatted image from the same patient's CT angiogram shows occlusion of the proximal left MCA, M1 segment . The left ACA and fetal left posterior cerebral artery (PCA) remain patent.](4b34b9b6-8e9f-480e-81a4-c378ee79b12f) +**Preprocedural Planning: Acute Left MCA Stroke (CT Angiogram)** +*However, 3D reformatted image from the same patient's CT angiogram shows occlusion of the proximal left MCA, M1 segment . The left ACA and fetal left posterior cerebral artery (PCA) remain patent.* + +![DSA of the left ICA demonstrates abrupt occlusion of the left MCA, M1 segment . The left ACA and fetal left PCA remain patent.](f364ff4c-ba44-45d5-a5cc-7660a1e72642) +**Intraprocedural: Acute Left MCA Stroke (Initial Angiogram)** +*DSA of the left ICA demonstrates abrupt occlusion of the left MCA, M1 segment . The left ACA and fetal left PCA remain patent.* + +![Unsubtracted fluoroscopic image (A) and DSA (B) show deployment of the stent retriever device across the left MCA, M1 segment thrombus . Note that only the distal markers of the Solitaire device are radiopaque.](d404b045-531d-4162-b494-a7165561f4fc) +**Intraprocedural: Acute Left MCA Stroke (Solitaire Device Deployment)** +*Unsubtracted fluoroscopic image (A) and DSA (B) show deployment of the stent retriever device across the left MCA, M1 segment thrombus . Note that only the distal markers of the Solitaire device are radiopaque.* + +![DSA following mechanical thrombectomy shows restoration of flow to the left MCA with TICI 3 flow in the distal branches . Two passes with the Solitaire device were required in this patient. The left ACA and fetal left PCA remain patent.](f31e07cb-4bd1-4edb-86fd-93e12545ad5b) +**Intraprocedural: Acute Left MCA Stroke (Postthrombectomy DSA)** +*DSA following mechanical thrombectomy shows restoration of flow to the left MCA with TICI 3 flow in the distal branches . Two passes with the Solitaire device were required in this patient. The left ACA and fetal left PCA remain patent.* + +![Axial DWI MR demonstrates only a punctate area of acute infarction in the left MCA territory . There was no hemorrhage on gradient-echo sequence (not pictured).](9b8426ca-d274-41f8-a5b0-07ec4021a81b) +**Follow-Up: Acute Left MCA Stroke (DWI MR)** +*Axial DWI MR demonstrates only a punctate area of acute infarction in the left MCA territory . There was no hemorrhage on gradient-echo sequence (not pictured).* + +![Axial NECT in a patient with an acute stroke shows a hyperdense basilar artery believed to represent intraluminal clot. NECT is initially obtained in acute stroke to exclude intracranial hemorrhage, which is a thrombolysis contraindication.](0f1420fb-200f-4831-964b-3319d7d93021) +**Preprocedural Planning: Basilar Stroke Intervention (Initial NECT)** +*Axial NECT in a patient with an acute stroke shows a hyperdense basilar artery believed to represent intraluminal clot. NECT is initially obtained in acute stroke to exclude intracranial hemorrhage, which is a thrombolysis contraindication.* + +![Selective AP left vertebral DSA obtained in preparation for transcatheter treatment of acute thrombotic occlusion of the basilar artery shows the level of occlusion is just distal to the anterior inferior cerebellar arteries (AICAs) .](bf4dc1c4-5b3c-4afb-8856-3db87c83eb06) +**Intraprocedural: Basilar Stroke Intervention (AP Basilar DSA)** +*Selective AP left vertebral DSA obtained in preparation for transcatheter treatment of acute thrombotic occlusion of the basilar artery shows the level of occlusion is just distal to the anterior inferior cerebellar arteries (AICAs) .* + +![The basilar artery occlusion is better demonstrated on the lateral DSA arteriogram, with a meniscus seen at the inferior margin of the occluding thrombus in the basilar artery. In addition to the AICAs , the left posterior inferior cerebellar artery (PICA) remains patent.](de6540b0-729e-4462-a28f-0a67e2bb2f0d) +**Intraprocedural: Basilar Stroke Intervention (Lateral Basilar DSA)** +*The basilar artery occlusion is better demonstrated on the lateral DSA arteriogram, with a meniscus seen at the inferior margin of the occluding thrombus in the basilar artery. In addition to the AICAs , the left posterior inferior cerebellar artery (PICA) remains patent.* + +![DSA following thrombectomy shows improved basilar artery patency and flow in the left PCA . There is perfusion of the proximal right PCA , which terminates abruptly .](ff9e749a-3276-4a73-9d0c-17c0f90dd72a) +**Intraprocedural: Basilar Stroke Intervention (Postthrombectomy DSA)** +*DSA following thrombectomy shows improved basilar artery patency and flow in the left PCA . There is perfusion of the proximal right PCA , which terminates abruptly .* + +![Lateral roadmap fluoroscopy shows that a reperfusion catheter has been advanced distally in the right PCA in an attempt to restore perfusion using thromboaspiration. The patent contralateral left PCA can be seen for comparison.](3725ae59-2f9a-4cb1-a3da-3dfd587dd03f) +**Intraprocedural: Basilar Stroke Intervention (Advancement of Penumbra)** +*Lateral roadmap fluoroscopy shows that a reperfusion catheter has been advanced distally in the right PCA in an attempt to restore perfusion using thromboaspiration. The patent contralateral left PCA can be seen for comparison.* + +![Final DSA after aspiration use shows that the right PCA is perfused throughout the entire length. The basilar artery has a normal appearance, as do all branch vessels. This is an example of TICI grade 3 reperfusion.](8c93e4f0-2b47-4b4e-bc4f-69ec0ae3493c) +**Intraprocedural: Basilar Stroke Intervention (Completion Basilar DSA)** +*Final DSA after aspiration use shows that the right PCA is perfused throughout the entire length. The basilar artery has a normal appearance, as do all branch vessels. This is an example of TICI grade 3 reperfusion.* + + +### Additional Images + +![Axial 3D CTA reformation in a patient who presented within 3 hours of onset of right-sided weakness and right facial droop shows abrupt termination of the left MCA , consistent with occlusion.](fb87b2a2-6cdc-4843-bbf3-56d61846fd57) +**Acute Left MCA Stroke** +*Axial 3D CTA reformation in a patient who presented within 3 hours of onset of right-sided weakness and right facial droop shows abrupt termination of the left MCA , consistent with occlusion.* + +![Axial DWI MR in a patient who presented within 3 hours of onset of right-sided weakness and right facial droop shows an area of restricted diffusion in the left corona radiata , reflecting the ischemic core zone.](33bfc542-eb6d-4a15-a5dc-5a637856466c) +**Acute Left MCA Stroke** +*Axial DWI MR in a patient who presented within 3 hours of onset of right-sided weakness and right facial droop shows an area of restricted diffusion in the left corona radiata , reflecting the ischemic core zone.* + +![Axial PWI MR at the same anatomic level in the same patient shows a much more extensive area of decreased perfusion in the left cerebral hemisphere, reflecting ischemic tissue at risk of infarction (penumbra).](6945d076-c3d0-47d6-ab31-3febf2e4b355) +**Acute Left MCA Stroke** +*Axial PWI MR at the same anatomic level in the same patient shows a much more extensive area of decreased perfusion in the left cerebral hemisphere, reflecting ischemic tissue at risk of infarction (penumbra).* + +![(A) DWI MR shows restricted diffusion in the left corona radiata , while the corresponding (B) PWI MR shows a more extensive area of decreased perfusion , reflecting cerebral tissue at risk for infarction (penumbra).](cd64cba0-fb8a-4475-b904-48de0c8ca871) +**Acute Left MCA Stroke** +*(A) DWI MR shows restricted diffusion in the left corona radiata , while the corresponding (B) PWI MR shows a more extensive area of decreased perfusion , reflecting cerebral tissue at risk for infarction (penumbra).* + +![CT evaluation for stroke shows a hyperdense basilar artery due to a clot.](ac03cd50-f8f7-4314-8c8c-e49300bc4b7d) +**Basilar Artery Recanalization** +*CT evaluation for stroke shows a hyperdense basilar artery due to a clot.* + +![Basilar artery clot/occlusion extending into the left PCA is shown. Note contrast opacification of the right PCA through the posterior communicating artery . There is also some flow in the left PCA through the collateral circulation.](0371f583-8e62-42c9-8924-7aeb90bac6fa) +**Basilar Artery Recanalization** +*Basilar artery clot/occlusion extending into the left PCA is shown. Note contrast opacification of the right PCA through the posterior communicating artery . There is also some flow in the left PCA through the collateral circulation.* + +![Axial CT perfusion shows prolongation of the MTT in the left cerebellar hemisphere in comparison to the contralateral side . Note that there is also MTT prolongation in the left aspect of the pons .](7ef4e36a-6f00-4dfd-b6ea-429da3a41e27) +**Basilar Artery Recanalization** +*Axial CT perfusion shows prolongation of the MTT in the left cerebellar hemisphere in comparison to the contralateral side . Note that there is also MTT prolongation in the left aspect of the pons .* + +![Selective (A) anteroposterior and (B) lateral right vertebral DSA shows extensive thrombus filling the basilar artery. There are high-grade stenoses of the distal right vertebral artery felt to be the etiology for the acute basilar artery thrombosis. Thrombus also extends into the distal left vertebral artery.](10edeca0-9b86-4506-9b0e-fa5b54520f73) +**Unsuccessful Stroke Intervention: Right Vertebral DSA** +*Selective (A) anteroposterior and (B) lateral right vertebral DSA shows extensive thrombus filling the basilar artery. There are high-grade stenoses of the distal right vertebral artery felt to be the etiology for the acute basilar artery thrombosis. Thrombus also extends into the distal left vertebral artery.* + +![Lateral fluoroscopic roadmap image shows (A) that a Merci retrieval device has been advanced into the basilar thrombus for attempted thrombectomy. (B) Subsequently, microwire clot maceration was performed through a microcatheter .](c9f5c073-c9f1-481e-a4f5-0414e4397f47) +**Unsuccessful Stroke Intervention: Merci Clot Retrieval** +*Lateral fluoroscopic roadmap image shows (A) that a Merci retrieval device has been advanced into the basilar thrombus for attempted thrombectomy. (B) Subsequently, microwire clot maceration was performed through a microcatheter .* + +![Lateral fluoroscopic roadmap image shows a Merci retrieval device has been advanced into the basilar thrombus.](f40d0b8f-30e3-416b-94c9-7edff4d2ba2f) +**Unsuccessful Stroke Intervention: Merci Clot Retrieval** +*Lateral fluoroscopic roadmap image shows a Merci retrieval device has been advanced into the basilar thrombus.* + +![Lateral DSA shows persistent occlusive thrombus in the distal basilar artery and a dissection at the base of the artery. The intervention was unsuccessful despite using multiple techniques for reestablishing perfusion. This represents TICI grade 0 perfusion after stroke intervention.](34cedcb4-05e5-4ebc-95bb-dddf423bdd80) +**Unsuccessful Stroke Intervention: Completion Arteriogram** +*Lateral DSA shows persistent occlusive thrombus in the distal basilar artery and a dissection at the base of the artery. The intervention was unsuccessful despite using multiple techniques for reestablishing perfusion. This represents TICI grade 0 perfusion after stroke intervention.* + +![Graphic of Merci device thrombectomy is shown. (A) The device is advanced via a guide catheter across the clot where (B) it is rotated counterclockwise, engaging the clot. (C) The microcatheter, retriever, and clot are withdrawn into the guide catheter while aspirating the clot.](a8286614-2d90-47e8-997f-0f14f439da33) +**Acute Stroke Intervention: Merci Retrieval System** +*Graphic of Merci device thrombectomy is shown. (A) The device is advanced via a guide catheter across the clot where (B) it is rotated counterclockwise, engaging the clot. (C) The microcatheter, retriever, and clot are withdrawn into the guide catheter while aspirating the clot.* + +![Lateral fluoroscopic spot radiograph shows that an angioplasty balloon has been inflated in the basilar artery in an attempt to further fragment and macerate the clot. Angioplasty of the vertebrobasilar stenosis was also performed in an attempt to improve arterial inflow.](f9ec249d-de49-410e-8cd4-bbd3436a00d5) +**Unsuccessful Stroke Intervention: Balloon Angioplasty** +*Lateral fluoroscopic spot radiograph shows that an angioplasty balloon has been inflated in the basilar artery in an attempt to further fragment and macerate the clot. Angioplasty of the vertebrobasilar stenosis was also performed in an attempt to improve arterial inflow.* + +![Coronal DWI MR shows increased central and paracentral signal in the pons due to restricted diffusion. This represents the ischemic core and correlates with irreversible injury, which will not respond to reperfusion. PWI evaluates for the presence of penumbra, while DWI/PWI mismatch defines its extent.](46afcf92-0fc7-4ce2-927a-1fd014556058) +**Unsuccessful Stroke Intervention: Initial DWI MR** +*Coronal DWI MR shows increased central and paracentral signal in the pons due to restricted diffusion. This represents the ischemic core and correlates with irreversible injury, which will not respond to reperfusion. PWI evaluates for the presence of penumbra, while DWI/PWI mismatch defines its extent.* + diff --git a/docs_md/articles/subacute-cerebral-infarction_0109f4c0-c84a-4d85-97cb-afe437b9cc43.md b/docs_md/articles/subacute-cerebral-infarction_0109f4c0-c84a-4d85-97cb-afe437b9cc43.md new file mode 100644 index 0000000..c5b1d5b --- /dev/null +++ b/docs_md/articles/subacute-cerebral-infarction_0109f4c0-c84a-4d85-97cb-afe437b9cc43.md @@ -0,0 +1,467 @@ +--- +title: "Subacute Cerebral Infarction" +docid: "0109f4c0-c84a-4d85-97cb-afe437b9cc43" +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: "Stroke" + slug: "stroke" + treeNodeId: "12307683-f1ff-4823-a7d3-b10b40f9fd82" + - + name: "Cerebral Ischemia and Infarction" + slug: "cerebral-ischemia-and-infarction" + treeNodeId: "51051846-a223-42f7-b626-2a5a26cf6c44" + - + name: "Subacute Cerebral Infarction" + slug: "subacute-cerebral-infarction" + treeNodeId: null +category: "Brain" +cmeTopicId: "56146806-ca6e-440e-a0cf-9c7a446b1906" +documentVersionId: "3dccb47d-0565-41ae-855b-a166b848e50f" +imageCount: 28 +lastUpdated: "08/10/20" +pageDescription: "Subacute Cerebral Infarction" +pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Cerebral Ischemia and Infarction, Subacute Cerebral Infarction" +pageTitle: "Subacute Cerebral Infarction | STATdx" +enhancedTitle: "Subacute Cerebral Infarction" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Pathology-Based Diagnoses" + - "Stroke" + - "Cerebral Ischemia and Infarction" + - "Subacute Cerebral Infarction" +--- +# KEY FACTS + +- ## Terminology + + + - Subacute infarction ~ 2-14 days following initial ischemic event +- ## Imaging + + + - Best diagnostic clue: Gyral edema and enhancement within basal ganglia and cortex + - Typically wedge-shaped abnormality involving gray and white matter within vascular distribution + - Hemorrhagic transformation of initial ischemic infarction occurs in 20-25% of middle cerebral artery (MCA) occlusions, usually by 48-72 hours + - "2-2-2" rule = enhancement begins at 2 days, peaks at 2 weeks, disappears by 2 months + - MRS: ↑ lactate, ↓ NAA within infarcted tissue + - DWI: ↑ diffusion restriction, ↓ ADC initially, reversing as it proceeds into/through subacute stage + - "Fogging" effect = normal T2WI with striking enhancement on T1WI C+ 1-2 weeks following ictus +- ## Top Differential Diagnoses + + + - Neoplasm + - Venous infarction + - Encephalitis/cerebritis +- ## Clinical Issues + + + - Acute-onset focal neurologic deficit + - Elderly patient with typical risk factors: Hypertension, diabetes, smoking history, obesity, hypercholesterolemia + - 1st month after infarction, mortality predominantly from neurologic complications; 1:4 die of recurrent stroke event + - Acute anticoagulation after 1st infarction reduces mortality +- ## Diagnostic Checklist + + + - Enhancement is key to defining subacute stage of cerebral infarction + - Subacute ischemia often mimics neoplasm + - Recommend short-term follow-up to ensure expected course of evolution + +# TERMINOLOGY + +- ## Abbreviations + + + - Subacute stroke, subacute cerebrovascular accident (CVA) +- ## Definitions + + + - Focal brain necrosis following obstruction of blood flow to localized area of brain + - Subacute infarct ~ 2-14 days following initial ischemic event + - May occur ± hemorrhagic transformation (HT) + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Gyral edema, enhancement in basal ganglia/cortex + - Look for HT + - Typically occurs in 20-25% of cases 2-7 days after acute event + - Related to reperfusion, spontaneous or following therapy + - ### Location + + + - Cerebral hemispheres, brainstem, cerebellum in territorial vascular distribution + - ### Size + + + - Extremely variable + - Ranges from focal ("lacunes") to global (hemispheric) + - ### Morphology + + + - Variable depending on location, size, etiology + - Typically wedge-shaped; involves both gray and white matter + - Recognizable vascular distribution +- ## CT Findings + + + - ### NECT + + + - Wedge-shaped area of ↓ attenuation involving gray and white matter + - Mass effect initially ↑, then ↓ by 7-10 days; often less than expected given lesion size as acuity resolves + - HT of ischemic infarct occurs in 20-25% of middle cerebral artery (MCA) occlusions, usually by 48-72 hours + - Common locations are basal ganglia and cortex + - Hemorrhagic foci detected in majority of medium/large subacute infarcts + - ### CECT + + + - Enhancement typically patchy or gyral + - May appear as early as 2-3 days after ictus; persists up to 8-10 weeks + - "2-2-2" rule = enhancement begins at 2 days, peaks at 2 weeks, disappears by 2 months + - ### CTA + + + - Evidence of subacute occlusion correlates strongly, independently with poor clinical outcome + - Significantly worse discharge National Institutes of Health Stroke Scale (NIHSS) score + - CT perfusion + - More useful in acute > subacute stroke + - Helpful in predicting tissue outcome + - Significant difference between infarct and periinfarct tissue for both relative cerebral blood flow (rCBF), relative cerebral blood volume (rCBV) +- ## MR Findings + + + - ### T1WI + + + - Hypointense edema with mass effect + - HT: Signal changes of hemorrhage + - May see gyriform ↑ signal (pseudolaminar necrosis) + - ### T2WI + + + - Hyperintense edema with mass effect + - "Fogging" effect = normal T2WI with striking enhancement on T1WI C+ 1-2 weeks following ictus + - HT: Signal changes of evolving hemorrhage + - Early wallerian degeneration can occur + - Look for well-defined hyperintense band in corticospinal tract + - ### FLAIR + + + - Hyperintense edema with mass effect + - Hyperintensity (dot sign) in slow-flowing/occluded vessels + - By 1 week, final infarct volume corresponds to FLAIR-defined abnormality + - May see "fogging" effect, similar to T2WI + - ### T2* GRE + + + - May see blooming if HT has occurred + - ### DWI + + + - ↑ diffusion restriction, ↓ ADC initially, reversing as it proceeds into/through subacute stage + - DWI, T1WI C+ complement each other in detecting subacute infarcts + - Early subacute can be ↑ DWI and ↓ T1WI C+ + - ### T1WI C+ + + + - Intravascular enhancement in initial 48 hours; disappears at 3-4 days as vessels recanalize + - Parenchymal enhancement (typically patchy or gyral) + - May appear as early as 2-3 days after ictus + - Can persist up to 8-10 weeks + - ### MRA + + + - Vessel occlusion (large vessel) + - ### MRS + + + - ↑ lactate, ↓ NAA within infarcted tissue + - In subacute and chronic infarction, lactate/choline and NAA/choline ratios correlate with outcome + - Positive correlation between NAA and Scandinavian Stroke Scale (SSS) scores + - Positive correlation between NAA reduction and Barthel index scores + - Lactate presence correlates with lower SSS scores + - MR T2* perfusion + - ↓ rCBV of acute infarct ↑ in subacute stage, reflecting reperfusion hyperemia + - ↓ again in chronic stage + - SWI: May see hypointensity related to microhemorrhage +- ## Angiographic Findings + + + - Conventional + - May see intraluminal thrombus &/or vessel occlusion + - Slow antegrade flow with delayed arterial emptying + - Slow retrograde filling through collateral vessels + - "Bare" areas = regions of nonperfused or slowly perfused brain tissue +- ## Nuclear Medicine Findings + + + - Diminished/absence of perfusion with SPECT or PET + - HMPAO SPECT may show reflow hyperemia after reperfusion in acute and subacute stages +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - MR with DWI, T2*, T1WI C+ + - Consider CT or MR perfusion (more helpful in acute stroke) + - ### Protocol advice + + + - CT and MR: C+ for assessing subacute age + +# DIFFERENTIAL DIAGNOSIS + +- [Neoplasm](/document/glioblastoma/45c3147e-3a1b-4fbf-a626-ed6e99a02ac2) + - DWI: Vasogenic ("tumoral") edema instead of cytotoxic edema + - Enhancing mass instead of patchy, gyral enhancement + - Will not regress on follow-up imaging +- [Venous Infarction](/document/cortical-venous-thrombosis/d314f5f7-21b6-46d1-a51c-e796cacc012c) + - Nonarterial distribution + - Venous instead of arterial occlusion, typically major dural sinus + - More commonly hemorrhagic, primarily affecting white matter instead of cortex + - Different clinical presentation/setting (trauma, hypercoagulable states, pregnancy, dehydration) +- [Encephalitis/Cerebritis](/document/abscess/552b58e6-aa5e-49b4-b9aa-e0413c07bf3c) + - DWI: Strong restriction + - Nonvascular distribution + - Gyriform, ring-enhancing patterns (late cerebritis) + - Different clinical presentation + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - Prolonged cerebral ischemia + - Duration and severity of ischemic insult determines cellular viability + - Less commonly, may be result of infectious etiologies + - Sequelae of meningitis (bacterial, mycobacterial, etc.) + - May also be result of inflammatory diseases, such as vasculopathy, angiitis, etc. + - Uncontrolled, unilateral, supratentorial expanding lesions can cause descending tentorial herniation → ischemic infarction of occipital lobe + - Ischemia/infarction involves typical vascular territories or watershed (border zone) distributions depending on etiology + - Sequelae of infarction vary with sensitivity of individual cell types to ischemia + - Other factors: Adequacy of collateral blood supply, degree, duration, and distribution of flow reduction + - ### Genetics + + + - Hypercholesterolemia, diabetes, hypertension, and homocysteine ↑ stroke risk +- ## Gross Pathologic & Surgical Features + + + - Blurring of gray-white demarcation + - Mass effect with narrowing of sulci, displacement of adjacent structures + - Softening of ischemic tissues from water retention +- ## Microscopic Features + + + - Fragmentation of axons and early disintegration of myelin sheaths; loss of oligodendrocytes, astrocytes + - 48 hours: Neutrophils begin to pass through vessel walls into brain tissue + - 72-96 hours: Macrophages aggregate around vessels + - 2 weeks: Macrophages are predominate reactive cells + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - Acute-onset focal neurologic deficit + - ~ 50% of patients with infarction → permanent neurologic deficits have preceding TIAs + - ### Clinical profile + + + - Elderly patient with typical risk factors: Hypertension, diabetes, smoking history, obesity, hypercholesterolemia, etc. +- ## Demographics + + + - ### Age + + + - Usually > 55 years + - Women often slightly older than men at presentation + - ### Sex + + + - Females often more disabled after age adjustment + - Fatality rates similar + - ### Epidemiology + + + - Highest cause of USA adult morbidity + - 3rd cause of USA adult mortality +- ## Natural History & Prognosis + + + - 1st month after infarction, mortality predominantly from neurologic complications + - 1:4 die of recurrent stroke event + - Later mortality from respiratory, cardiovascular causes + - Survival after 1st infarction: 1 week (92%), 30 days (83%), 6 months (77%), 1 year (71%), 5 years (46%), 10 years (28%) +- ## Treatment + + + - To improve long-term survival, aggressive management of pulmonary and cardiac disease is critical + - Acute anticoagulation after 1st infarction reduces mortality + - Current research: Therapeutic hypothermia and gene therapy (antiapoptotic protein BCL-2) during acute stroke event + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Is affected area another space-occupying pathology (i.e., tumor)? + - Recommend short-term follow-up to ensure expected course of evolution +- ## Image Interpretation Pearls + + + - Enhancement is key to defining subacute stage of cerebral infarction + - Appearance on DWI/ADC often helpful + + 62b91377-75c2-40c0-8e3d-3e9ff4f24cbd + +## References + +# Selected References + +1. [Mistry EA et al: White matter disease and outcomes of mechanical thrombectomy for acute ischemic stroke. AJNR Am J Neuroradiol. 41(4):639-44, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32165366%5Bpmid%5D) +1. [Wessell AP et al: A critical assessment of the golden hour and the impact of procedural timing in stroke thrombectomy. AJNR Am J Neuroradiol. 41(5):822-7, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32414902%5Bpmid%5D) +1. [Bill O et al: Focal hypoperfusion in acute ischemic stroke perfusion CT: clinical and radiologic predictors and accuracy for infarct prediction. AJNR Am J Neuroradiol. 40(3):483-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30792249%5Bpmid%5D) +1. [Majidi S et al: MRI-based thrombolytic therapy in patients with acute ischemic stroke presenting with a low NIHSS. Neurology. 93(16):e1507-13, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31519779%5Bpmid%5D) +1. [Sotoudeh H et al: Misleading CT perfusion in subacute ischemic stroke. Emerg Radiol. 26(5):581-6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31432350%5Bpmid%5D) +1. [Arnold Fiebelkorn C et al: Frequency of acute and subacute infarcts in a population-based study. Mayo Clin Proc. 93(3):300-6, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29426582%5Bpmid%5D) +1. [Yang YM et al: Normalization of T2 relaxation time and apparent diffusion coefficient in relation to the inflammatory changes in the substantia nigra of rats with focal cerebral ischemia. Acta Radiol. 56(7):837-43, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25260416%5Bpmid%5D) +1. [Zöllner JP et al: Changes of pH and energy state in subacute human ischemia assessed by multinuclear magnetic resonance spectroscopy. Stroke. 46(2):441-6, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25503553%5Bpmid%5D) +1. [Brunner IC et al: Plasticity and response to action observation: a longitudinal FMRI study of potential mirror neurons in patients with subacute stroke. Neurorehabil Neural Repair. 28(9):874-84, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24642381%5Bpmid%5D) +1. [Ntaios G et al: Acute imaging does not improve ASTRAL score's accuracy despite having a prognostic value. Int J Stroke. 9(7):926-31, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24894405%5Bpmid%5D) +1. [Qiao Y et al: Intracranial plaque enhancement in patients with cerebrovascular events on high-spatial-resolution MR images. Radiology. 271(2):534-42, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24475850%5Bpmid%5D) +1. [Drier A et al: Prediction of subacute infarct size in acute middle cerebral artery stroke: comparison of perfusion-weighted imaging and apparent diffusion coefficient maps. Radiology. 265(2):511-7, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22923715%5Bpmid%5D) +1. [Kamalian S et al: CT perfusion mean transit time maps optimally distinguish benign oligemia from true "at-risk" ischemic penumbra, but thresholds vary by postprocessing technique. AJNR Am J Neuroradiol. 33(3):545-9, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22194372%5Bpmid%5D) +1. [Donnan GA et al: Penumbral selection of patients for trials of acute stroke therapy. Lancet Neurol. 8(3):261-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19233036%5Bpmid%5D) +1. [Elkind MS: Outcomes after stroke: risk of recurrent ischemic stroke and other events. Am J Med. 122(4 Suppl 2):S7-13, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19332241%5Bpmid%5D) +1. [Olivot JM et al: Perfusion MRI (Tmax and MTT) correlation with xenon CT cerebral blood flow in stroke patients. Neurology. 72(13):1140-5, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19332690%5Bpmid%5D) +1. [Muñoz Maniega S et al: Changes in NAA and lactate following ischemic stroke: a serial MR spectroscopic imaging study. Neurology. 71(24):1993-9, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=19064881%5Bpmid%5D) +1. [Vernino S et al: Cause-specific mortality after first cerebral infarction: a population-based study. Stroke. 34(8):1828-32, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12855836%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Axial CT at 3 days after initial onset of weakness and speech difficulties shows the classic appearance of an early subacute cerebral infarct. Note the wedge-shaped, low-density area involving both the gray and white matter in the MCA distribution with blood products in the right basal ganglia.](images/app.statdx.com_image_thumbnail_d27f250c-3367-425e-9138-93a6082c618d_annotated_true_size_900_quality_90_38d5a718da65cae350d5c76f66e43e8cf888c2f7.jpg) +*Axial CT at 3 days after initial onset of weakness and speech difficulties shows the classic appearance of an early subacute cerebral infarct. Note the wedge-shaped, low-density area involving both the gray and white matter in the MCA distribution with blood products in the right basal ganglia.* + +![Axial CT at 3 days after initial onset of weakness and speech difficulties shows the classic appearance of an early subacute cerebral infarct. Note the wedge-shaped, low-density area involving both the gray and white matter in the MCA distribution with blood products in the right basal ganglia.](images/app.statdx.com_image_thumbnail_d27f250c-3367-425e-9138-93a6082c618d_size_174_quality_85_46e3053651c3552028ca377bd9853960b5d13510.jpg) +*Axial CT at 3 days after initial onset of weakness and speech difficulties shows the classic appearance of an early subacute cerebral infarct. Note the wedge-shaped, low-density area involving both the gray and white matter in the MCA distribution with blood products in the right basal ganglia.* + +![Axial FLAIR MR in a 58-year-old man 1 week after onset of visual changes shows classic imaging of a subacute infarct with hyperintensity in the cortex and subcortical white matter of the occipital lobe in a PCA distribution.](images/app.statdx.com_image_thumbnail_e3dea8a5-436f-4b1a-a1be-3d4d434c90e7_annotated_true_size_900_quality_90_b7d1b33ad35de7ea0c06cdf2c9c179b8315084f4.jpg) +*Axial FLAIR MR in a 58-year-old man 1 week after onset of visual changes shows classic imaging of a subacute infarct with hyperintensity in the cortex and subcortical white matter of the occipital lobe in a PCA distribution.* + +![Axial DWI MR in the same patient shows hyperintensity within the left PCA distribution. The hyperintensity represents a combination of true diffusion restriction and T2 shine-through.](images/app.statdx.com_image_thumbnail_4c657eaa-bb5a-44b9-9724-daa645bbe36f_annotated_true_size_900_quality_90_b67d6a866f944cf8ddbebd537937d3e559b0442b.jpg) +*Axial DWI MR in the same patient shows hyperintensity within the left PCA distribution. The hyperintensity represents a combination of true diffusion restriction and T2 shine-through.* + +![Axial T1 C+ MR in the same patient shows gyriform enhancement along the cortex of the occipital lobe . This enhancement may be seen as early as 2 days and may last up to 2 months after the patient's initial ischemic event. Without clinical history, imaging may mimic a tumor, venous infarct, or cerebritis.](images/app.statdx.com_image_thumbnail_cadb394c-ce1e-4dee-be0d-bc66f9562db6_annotated_true_size_900_quality_90_18b6e5d398443bb02aeb9998423eb73a8a9c06ba.jpg) +*Axial T1 C+ MR in the same patient shows gyriform enhancement along the cortex of the occipital lobe . This enhancement may be seen as early as 2 days and may last up to 2 months after the patient's initial ischemic event. Without clinical history, imaging may mimic a tumor, venous infarct, or cerebritis.* + +![Axial T1 C+ MR shows well-defined gyriform enhancement in the PCA distribution in a subacute infarct patient who was sent to a neurosurgeon for concerns of a cortical "tumor."](images/app.statdx.com_image_thumbnail_3042776d-2141-413a-9339-789270323c61_annotated_true_size_900_quality_90_058151c0cf98012f420cb3179f5bbdd77df6d584.jpg) +*Axial T1 C+ MR shows well-defined gyriform enhancement in the PCA distribution in a subacute infarct patient who was sent to a neurosurgeon for concerns of a cortical "tumor."* + +![Axial FLAIR MR in the same patient shows the "fogging" effect with near-normal signal on T2/FLAIR MR with striking enhancement on T1WI C+ MR. This "fogging" effect typically occurs 1-2 weeks following the acute stroke. Initially, the infarct shows hyperintensity, which decreases over time, with isointensity at 1-2 weeks.](images/app.statdx.com_image_thumbnail_fa7da41f-b797-4af4-a40b-117ee0996c71_annotated_true_size_900_quality_90_62813ff031ce1efbb64351faac20fcc2399d91fc.jpg) +*Axial FLAIR MR in the same patient shows the "fogging" effect with near-normal signal on T2/FLAIR MR with striking enhancement on T1WI C+ MR. This "fogging" effect typically occurs 1-2 weeks following the acute stroke. Initially, the infarct shows hyperintensity, which decreases over time, with isointensity at 1-2 weeks.* + +![Axial DWI MR in a 45-year-old woman with an abnormal cardiac valve resulting in embolic disease in multiple vascular distributions 5 days after a posterior circulation infarct shows high signal due to restricted diffusion in bilateral cerebellar hemispheres. DWI scans can be hyperintense up to 7-10 days following acute stroke onset.](images/app.statdx.com_image_thumbnail_df984c3a-3df5-4f3f-9cee-0287daec0a62_annotated_true_size_900_quality_90_c37c9a99fd90d23633135c2333879400f6f8ddf3.jpg) +*Axial DWI MR in a 45-year-old woman with an abnormal cardiac valve resulting in embolic disease in multiple vascular distributions 5 days after a posterior circulation infarct shows high signal due to restricted diffusion in bilateral cerebellar hemispheres. DWI scans can be hyperintense up to 7-10 days following acute stroke onset.* + +![Axial T1 C+ MR in the same patient shows patchy enhancement in the right cerebellar hemisphere . Cardioembolic disease represents 15-25% of major strokes.](images/app.statdx.com_image_thumbnail_ce44f21d-20a5-4cd9-bb2a-de2dc2a819ba_annotated_true_size_900_quality_90_59b6f233bdb09f333cae4f875915001ff233a24e.jpg) +*Axial T1 C+ MR in the same patient shows patchy enhancement in the right cerebellar hemisphere . Cardioembolic disease represents 15-25% of major strokes.* + +![Axial DWI trace MR in a 61-year-old patient 3 days after symptoms shows DWI restriction. True restricted diffusion persists several days after stroke onset and gradually reverses with DWI hypointensity and ADC hyperintensity.](images/app.statdx.com_image_thumbnail_afbb0589-4346-44a8-89e9-72626522ab87_annotated_true_size_900_quality_90_d1e8ae3b89bdaafb171c8e451d72ba44a80d553f.jpg) +*Axial DWI trace MR in a 61-year-old patient 3 days after symptoms shows DWI restriction. True restricted diffusion persists several days after stroke onset and gradually reverses with DWI hypointensity and ADC hyperintensity.* + +![Axial T1 C+ MR shows gyriform enhancement in the MCA distribution. Note lack of mass effect in this late subacute infarct. The major differential considerations for a subacute infarct include neoplasm and cerebritis. Repeat imaging may be necessary to exclude neoplasm.](4bb8e0f7-8d82-48c6-858a-3b7447e6d58d) +*Axial T1 C+ MR shows gyriform enhancement in the MCA distribution. Note lack of mass effect in this late subacute infarct. The major differential considerations for a subacute infarct include neoplasm and cerebritis. Repeat imaging may be necessary to exclude neoplasm.* + + +### Additional Images + +![Axial T1 C+ MR in the same patient shows striking curvilinear enhancement within the sulci and over the gyri of the affected area . This represents collateral flow in small vessels over and within the pia ("leptomeningeal collaterals"), adjacent to the infarcted brain.](61a1c016-ae51-4f47-9abe-05c720e9d89f) +*Axial T1 C+ MR in the same patient shows striking curvilinear enhancement within the sulci and over the gyri of the affected area . This represents collateral flow in small vessels over and within the pia ("leptomeningeal collaterals"), adjacent to the infarcted brain.* + +![Axial DWI MR demonstrates diffusion restriction in the right PCA distribution.](b06e0aac-fc19-4933-b29d-271764cfcca9) +*Axial DWI MR demonstrates diffusion restriction in the right PCA distribution.* + +![Axial T1 MR shows swollen gyri in the left PCA distribution , findings typical for subacute cerebral infarction.](557c8d6f-9e14-4629-8ef8-03099384bc66) +*Axial T1 MR shows swollen gyri in the left PCA distribution , findings typical for subacute cerebral infarction.* + +![Axial T2 MR shows gyral swelling and extensive hyperintensity involving both the gray and white matter of the right temporal lobe.](08445226-c91c-4a4c-b3d3-f48505956654) +*Axial T2 MR shows gyral swelling and extensive hyperintensity involving both the gray and white matter of the right temporal lobe.* + +![Axial T1 C+ MR demonstrates classic gyriform enhancement of a subacute cerebral infarction. Some underlying T1-hyperintense hemorrhage is masked by extensive enhancement.](b4212522-d8d1-4ca5-9503-0266383b40e0) +*Axial T1 C+ MR demonstrates classic gyriform enhancement of a subacute cerebral infarction. Some underlying T1-hyperintense hemorrhage is masked by extensive enhancement.* + +![Axial NECT shows a wedge-shaped, nonhemorrhagic infarct. Lack of mass effect and CSF-like hypodensity aid in diagnosing subacute age.](a299e18a-b669-49b7-991a-9b1610bbcb00) +*Axial NECT shows a wedge-shaped, nonhemorrhagic infarct. Lack of mass effect and CSF-like hypodensity aid in diagnosing subacute age.* + +![Axial DWI MR demonstrates hyperintense restricted diffusion of cytotoxic edema within both the right ACA and MCA vascular territories.](80f527bd-011c-45bd-931e-6861074e44e0) +*Axial DWI MR demonstrates hyperintense restricted diffusion of cytotoxic edema within both the right ACA and MCA vascular territories.* + +![Axial NECT shows a classic nonhemorrhagic left MCA territory infarction involving the basal ganglia. Note the relatively mild ventricular and sulcal mass effect given the size of the lesion during subacute stage.](dd780082-a38f-4acf-aae5-52c68faadeab) +*Axial NECT shows a classic nonhemorrhagic left MCA territory infarction involving the basal ganglia. Note the relatively mild ventricular and sulcal mass effect given the size of the lesion during subacute stage.* + +![Axial T1 MR shows a hemorrhagic subacute infarction involving gray matter as well as a small portion of subcortical white matter. Gyriform enhancement was also present.](541824ec-7f44-41b5-aa32-cd247ba331db) +*Axial T1 MR shows a hemorrhagic subacute infarction involving gray matter as well as a small portion of subcortical white matter. Gyriform enhancement was also present.* + +![Axial CECT demonstrates extensive gyral subacute infarct enhancement 6 weeks after ictus. Note the absence of mass effect given the lesion size as acuity diminishes.](23b38fb8-c09a-40d0-b510-c4d66c6a6866) +*Axial CECT demonstrates extensive gyral subacute infarct enhancement 6 weeks after ictus. Note the absence of mass effect given the lesion size as acuity diminishes.* + +![Axial NECT demonstrates cortical hemorrhage of a subacute left MCA distribution infarction. Note the lack of mass effect given the lesion size.](9c22a44e-487f-4c4f-94b7-035b1a85ad5c) +*Axial NECT demonstrates cortical hemorrhage of a subacute left MCA distribution infarction. Note the lack of mass effect given the lesion size.* + +![Axial collapsed view MRA reveals the lack of flow in the right PCA .](960aead8-4f98-41b5-ba43-aeec725a0fe4) +*Axial collapsed view MRA reveals the lack of flow in the right PCA .* + +![Axial CECT demonstrates gyriform enhancement in the left MCA territory, a finding seen in subacute infarcts.](11e6f7f0-4e19-41b2-97a0-b78f09135b52) +*Axial CECT demonstrates gyriform enhancement in the left MCA territory, a finding seen in subacute infarcts.* + +![Axial T2 MR shows almost no abnormality except for minimal hyperintensity on the T2 . Occasionally, subacute cerebral infarcts may be difficult to visualize on standard MR scans because of the so-called "fogging" effect.](9951ec52-4b42-44a7-a7cf-cc044b4c08d0) +*Axial T2 MR shows almost no abnormality except for minimal hyperintensity on the T2 . Occasionally, subacute cerebral infarcts may be difficult to visualize on standard MR scans because of the so-called "fogging" effect.* + +![Axial T1 C+ MR in the same patient demonstrates striking gyriform enhancement .](9faae1a6-7b5e-41af-ba28-90ca05f3d0dd) +*Axial T1 C+ MR in the same patient demonstrates striking gyriform enhancement .* + +![Sagittal T1 C+ MR shows well-defined gyriform enhancement in the right MCA distribution. Note the lack of mass effect in this late subacute infarct. The major differential considerations for a subacute infarct include neoplasm and cerebritis. Repeat imaging may be necessary to exclude neoplasm.](78673dc2-45f4-48d6-9fdd-23be6e49a8f2) +*Sagittal T1 C+ MR shows well-defined gyriform enhancement in the right MCA distribution. Note the lack of mass effect in this late subacute infarct. The major differential considerations for a subacute infarct include neoplasm and cerebritis. Repeat imaging may be necessary to exclude neoplasm.* + +![Axial NECT demonstrates gyriform hyperdensity related to cortical hemorrhagic transformation in a right hemispheric watershed infarct. Note the surrounding low-density edema.](eb26a7a1-2305-47b8-b66d-68f43d22beec) +*Axial NECT demonstrates gyriform hyperdensity related to cortical hemorrhagic transformation in a right hemispheric watershed infarct. Note the surrounding low-density edema.* + +![Axial CT obtained 48 hours after initial onset of weakness and speech difficulties shows the classic appearance of a late acute/early subacute cerebral infarct. Note the wedge-shaped, low-density area involving both the gray and white matter in the left MCA distribution.](fea4db24-35fe-4363-8e59-6068575888f3) +*Axial CT obtained 48 hours after initial onset of weakness and speech difficulties shows the classic appearance of a late acute/early subacute cerebral infarct. Note the wedge-shaped, low-density area involving both the gray and white matter in the left MCA distribution.* + diff --git a/docs_md/articles/vasospasm_341f9578-93fe-465b-93b8-71b878e06433.md b/docs_md/articles/vasospasm_341f9578-93fe-465b-93b8-71b878e06433.md new file mode 100644 index 0000000..9bc0d37 --- /dev/null +++ b/docs_md/articles/vasospasm_341f9578-93fe-465b-93b8-71b878e06433.md @@ -0,0 +1,431 @@ +--- +title: "Vasospasm" +docid: "341f9578-93fe-465b-93b8-71b878e06433" +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: "Stroke" + slug: "stroke" + treeNodeId: "12307683-f1ff-4823-a7d3-b10b40f9fd82" + - + name: "Nonatheromatous Vasculopathy" + slug: "nonatheromatous-vasculopathy" + treeNodeId: "ed2d2a03-ebd6-4a72-8608-effc92deb342" + - + name: "Vasospasm" + slug: "vasospasm" + treeNodeId: null +category: "Brain" +cmeTopicId: "77ecc93f-65f7-46cf-9974-72bfbcc4eb26" +documentVersionId: "cc53e4c7-f966-4a2b-acce-a001d859ee78" +imageCount: 17 +lastUpdated: "03/05/25" +pageDescription: "Vasospasm" +pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Nonatheromatous Vasculopathy, Vasospasm" +pageTitle: "Vasospasm | STATdx" +enhancedTitle: "Vasospasm" +type: "DX" +references: true +breadcrumbs: + - "Brain" + - "Diagnosis" + - "Pathology-Based Diagnoses" + - "Stroke" + - "Nonatheromatous Vasculopathy" + - "Vasospasm" +--- +# KEY FACTS + +- ## Terminology + + + - Reversible stenosis of intracranial arteries + - Caused by exposure to blood breakdown products +- ## Imaging + + + - General features (CTA/MRA/DSA) + - Typically occurs 4-14 days after subarachnoid hemorrhage (SAH) + - Smooth, relatively long segmental stenoses + - Seen as arterial luminal irregularity/undulations + - Multiple arteries, usually > 1 vascular territory + - Transcranial Doppler (TCD) + - > 80% accuracy for vasospasm detection if mean velocity in MCA > 120 cm/s, basilar artery > 70 cm/s + - Lindegaard ratio 3-6 mild to moderate vasospasm; ratio > 6 severe vasospasm + - ↑ flow velocities with Lindegaard ratio < 3 suggest hyperemia or other physiologic/induced state + - CT perfusion + - ↑ TTD, ↑ TTP, ↑ MTT, ↓ cerebral blood flow (CBF) + - ↓ cerebral blood volume (CBV) in areas of infarct + - TTD best parameter for delayed cerebral ischemia + - Vessel wall MR (VW-MR) + - Vessel wall enhancement → vasospasm later +- ## Top Differential Diagnoses + + + - Reversible cerebral vasoconstriction syndrome (RCVS), vasculitis, atherosclerosis + - VW-MR helps in differentiation + - Flow diverter-induced subacute segmental vasospasm + - 3-5 weeks after flow diversion, obscure pathophysiology + - Meningitis, acute hypertensive encephalopathy (PRES), migraine headache +- ## Pathology + + + - Blood breakdown products (e.g., oxyHgb) coat vessel walls → release of free radicals from vessel wall + - Multifactorial-like release of factors like serotonin, ↓ nitric oxide activity from endothelium, ↑ endothelin-1 activity + - Prolonged exposure of vessel wall to blood components +- ## Clinical Issues + + + - Delayed ischemic neurological deficit (DIND) + - ~ 1 week after SAH is typical + - "Triple H" therapy + - Endovascular (chemical or balloon angioplasty) +- ## Diagnostic Checklist + + + - TCD for proximal arteries, DSA for more distal vasospasm + +# TERMINOLOGY + +- ## Synonyms + + + - Subarachnoid hemorrhage (SAH) vasospasm +- ## Definitions + + + - Exposure to blood breakdown products → reversible stenosis of intracranial arteries + - Mild vasospasm: < 25% reduction in vessel patency + - Severe vasospasm: > 50% reduction in vessel patency + +# IMAGING + +- ## General Features + + + - ### Best diagnostic clue + + + - Segmental stenoses (CTA/MRA/DSA) + - DSA is gold standard; typically 4-14 days after SAH + - ### Location + + + - Affects any intradural (subarachnoid) artery + - Worst vasospasm typically adjacent to site of ruptured aneurysm (highest concentration of SAH) +- ## Imaging Recommendations + + + - ### Best imaging tool + + + - Gold standard is DSA (100% sensitive but nonspecific) + - May follow with intraarterial (IA) therapy + - Transcranial Doppler (TCD) useful as bedside monitoring/screening tool + - ### Protocol advice + + + - Multiterritorial vascular involvement is typical + - Visualize both carotids, dominant vertebral artery +- ## CT Findings + + + - ### NECT + + + - May see residual SAH + - Otherwise normal (unless ischemia/stroke) + - Hypodensity in involved vascular territory may herald ischemia/infarction + - Differentiate from retraction edema + - Adjacent to surgical clip, not confined to vascular territory + - ### CTA + + + - Screening tool for involvement of large vessels + - Circle of Willis, M1 segment, basilar artery + - Attenuation/stenosis of arteries + - Typically multiterritorial but asymmetric + - Insensitive for smaller vessels (e.g., M2, distal segments) + - CT perfusion (pCT) + - Hypoperfusion + - Ischemic penumbra: ↑ TTD, ↑ TTP, ↑ MTT, ↓ cerebral blood flow (CBF) + - Preserved or ↑ cerebral blood volume (CBV) indicates adequacy of collateral flow + - Irreversible ischemia with infarct: Further worse above parameters, & CBV is critically low + - TTD best parameter for delayed cerebral ischemia + - TTD defined as time to start + MTT; measures time for contrast agent to pass away from analyzed voxel + - Diagnostic accuracy for TTD & MTT significantly higher than other perfusion parameters + - TTD higher sensitivity than MTT; severity of vasospasm on TTD maps significantly higher correlation with DSA than other perfusion maps + - pCT maps generated using deconvolution algorithm (CBV, CBF, MTT, TTD) better than maps using maximum slope method (CBV, CBF, TTP) + - Deconvolution method provides significantly higher diagnostic accuracy for detecting arterial vasospasm, & higher correlation with angiographic findings + - May miss distal vasospasm if pCT limited to 2 slices + - Whole brain volume pCT recommended +- ## MR Findings + + + - ### DWI + + + - Most sensitive for vasospasm sequelae + - If ischemia → infarction + - ### MRA + + + - Not usually utilized for screening + - CT more easily performed in patients in ICU S/P SAH + - Vessel stenosis → signal void on TOF imaging + - Depends on vasospasm severity + - Vessel wall MR (VW-MR) + - Vessel wall enhancement (concentric in majority, eccentric in few) after endovascular treatment of ruptured aneurysms → angiographic vasospasm later + - Consider early preventive treatment to reduce potential severe morbidity & mortality + - Arterial wall enhancement in aneurysmal SAH likely due to inflammation & ultrastructural vessel wall changes + - Like tight junction disruption & endothelial & smooth muscle cell injury + - Endovascular procedure-related arterial vessel wall enhancement in segments which underwent balloon or stent-assisted coiling or flow diverter stenting + - May not → subsequent vasospasm + - Be aware of flow diverter-induced subacute segmental vasospasm at ~ 1 month + - Number of enhancing vessel wall segments much lower with treated unruptured aneurysms + - Could be even preexisting nonstenotic atherosclerotic plaques not detected on luminal imaging +- ## Ultrasonographic Findings + + + - TCD + - Bernoulli principle: ↑ mean flow velocity occurs due to ↓ arterial cross-sectional area from vasospasm/stenosis + - Low-frequency transducer used to evaluate larger arteries at base of brain + - Transtemporal window absent in 10% of patients + - > 80% accuracy for vasospasm detection if mean velocity in middle cerebral artery (MCA) > 120 cm/s, basilar artery > 70 cm/s + - Mean MCA velocity: Normal 55 cm/s; vasospasm: Mild > 120, moderate > 160, & severe > 200 cm/s + - Lindegaard ratio: Mean MCA velocity:ipsilateral extracranial internal carotid artery (ICA) velocity ratio + - Ratio 3-6 mild to moderate vasospasm; ratio > 6 severe vasospasm + - ↑ flow velocities with Lindegaard ratio < 3 suggest hyperemia or other physiologic/induced state + - Sensitivity ~ 60% (operator dependent) + - Specificity > 95% (in patients with known SAH) + - Upward trends in mean velocities may be more indicative of vasospasm than absolute values +- ## Angiographic Findings + + + - Arterial luminal irregularity/undulations + - Smooth, relatively long-segment stenoses + - Multiple arteries, > 1 vascular territory typical + +# DIFFERENTIAL DIAGNOSIS + +- [Atherosclerosis](/document/intracranial-atherosclerosis/8d21d962-9c43-47bd-b995-c73f20e46b47) + - Usually older patients + - Short- > long-segment stenoses + - Cavernous/extracranial ICA, vertebral artery often affected + - VW-MR shows eccentric arterial wall thickening + - With contrast-enhancing inner layer of plaque near lumen, outer nonenhancing layer, & occasionally another enhancing thin layer at periphery +- [Reversible Cerebral Vasoconstriction Syndrome, Migraine Headaches](/document/reversible-cerebral-vasoconstricti-/e5d89e00-aaa7-4809-8d9b-82c0f89d5d01) + - Transient, may look identical + - VW-MR shows no enhancement or only minimal concentric arterial wall enhancement + - Sometimes more than minimal wall enhancement, cannot be differentiated from vasculitis then + - Arterial smooth muscle cells shorten & overlap → 5x ↑ in wall thickness for 60% luminal narrowing +- [Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0) + - Can look identical to vasospasm, reversible cerebral vasoconstriction syndrome; typically shorter segmental stenoses/"beading" in vasculitis + - Absence of SAH on NECT is typical (SAH secondary to vasculitis is rare) + - Subacute SAH may be iso-/hypodense on NECT; CSF analysis may help to detect blood breakdown products + - Inflammatory markers in serum, CSF often elevated + - VW-MR typically shows smooth, homogeneous, concentric arterial wall thickening & enhancement + - Vasculitis sometimes shows eccentric wall findings like atherosclerosis (but lacks layered plaque appearance) +- ## Flow Diverter-Induced Subacute Segmental Vasospasm + + + - Frequent pathophysiologically obscure vascular reaction after aneurysm treatment with flow diverters in small cerebral arteries + - Potentially cause symptomatic ischemia or stroke, 3-5 weeks post procedure +- [Acute Hypertensive Encephalopathy](/document/acute-hypertensive-encephalopathy--/890c1bd4-c108-49a1-8557-c8c701a7f278) + - Posterior > anterior circulation +- [Meningitis](/document/meningitis/7fdf69fa-c171-4e6b-b6d7-4e8fc94fdc53) + - Sulcal/cisternal enhancement + +# PATHOLOGY + +- ## General Features + + + - ### Etiology + + + - SAH vasospasm most commonly seen after aneurysm rupture + - Other causes of SAH (e.g., trauma, arteriovenous malformation rupture) may also cause vasospasm + - Diffuse nonaneurysmal SAH has potential for vasospasm similar to aneurysmal SAH (aSAH) + - Exact pathophysiology unknown, likely multifactorial + - Blood breakdown products (e.g., oxyHgb) coat vessel walls → release of free radicals from vessel wall + - Release of factors, including serotonin, angiotensin, prostaglandins, thromboxane, protein kinase C, phospholipase C & A2 + - Possible role of ↓ nitric oxide activity from endothelium, ↑ endothelin-1 activity +- ## Staging, Grading, & Classification + + + - Fisher CT score corresponds to risk of vasospasm development + - 1: No SAH + - 2: Small SAH, < 1-mm vertical layers + - 3: Extensive SAH, > 1-mm vertical layers + - 4: Intraventricular hemorrhage +- ## Microscopic Features + + + - Prolonged exposure of vessel wall to blood components → thickening of tunica media, intimal edema, subintimal cellular proliferation with muscle cells & fibroblasts + +# CLINICAL ISSUES + +- ## Presentation + + + - ### Most common signs/symptoms + + + - 70% of patients after aSAH develop vasospasm seen on DSA; only 30% symptomatic + - Vasospasm is significant source of morbidity & mortality in patients with SAH + - Delayed ischemic neurologic deficit ~ 1 week after SAH is typical + - Focal neurologic deficit(s): Motor, language, vision + - ### Other signs/symptoms + + + - Altered mental status, ↓ level of consciousness +- ## Demographics + + + - ### Age + + + - Any age; more common in younger patients + - ### Epidemiology + + + - 30,000 people per year have SAH in USA +- ## Natural History & Prognosis + + + - ~ time course of vasospasm following SAH: Day 3-4: Vasospasm begins; day 7-10: Vasospasm peaks; day 14-21: Vasospasm subsides + - Hyperacute vasospasm occurs in 10% of patients (onset < 48 hours from SAH) + - Flow diverter-induced subacute segmental vasospasm occurs at 3-5 weeks + - Aggressive treatment/prophylaxis in SAH patients can prevent stroke, death from ischemic sequela +- ## Treatment + + + - Medical management + - "Triple H" therapy: Hypertension, hemodilution, hypervolemia + - Oral or IV Ca⁺⁺ antagonists (e.g., nimodipine) + - Magnesium + - Endovascular + - Chemical angioplasty: IA infusion of Ca⁺⁺ antagonist has superseded papaverine + - Less technically demanding than balloon angioplasty, can treat smaller distal vessels + - Duration of effect may be up to 24 hours, additional IA treatments may be needed + - Relatively low risk: Side effect = hypotension, which could exacerbate hypoperfusion + - Balloon angioplasty + - Progressive dilatation of larger basal arteries: Intradural ICA & vertebral arteries, basilar artery, MCA (M1 ± M2 segments), anterior cerebral artery (ACA) (A1 segment), posterior cerebral artery (PCA) (P1 segment) + - 1% risk of fatal vessel rupture, thromboembolic stroke, vessel dissection + - Intracisternal thrombolytic therapy + - Several clinical trials have shown moderate success; not widely accepted Rx + - Recombinant tPA infused via ventriculostomy to lyse blood in subarachnoid spaces → ↓ breakdown to oxyHgb → prevent vasospasm + +# DIAGNOSTIC CHECKLIST + +- ## Consider + + + - Vasospasm as etiology of clinical deterioration, ischemic changes on NECT 4-14 days after aSAH +- ## Image Interpretation Pearls + + + - TCD is insensitive to changes in vessels beyond intradural ICA, M1, A1, & basilar arteries + - Do DSA if more distal vessel involvement suspected (e.g., pericallosal aneurysm rupture, nonaneurysmal SAH) + + 45be7813-c057-4ff8-b70a-520e014e7ab9 + +## References + +# Selected References + +1. [Schob S et al: Delayed stroke after aneurysm treatment with flow diverters in small cerebral vessels: a potentially critical complication caused by subacute vasospasm. J Clin Med. 8(10), 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31658743%5Bpmid%5D) +1. [Vulcu S et al: Repetitive computed tomography perfusion for detection of cerebral vasospasm-related hypoperfusion in aneurysmal subarachnoid hemorrhage. World Neurosurg. 121:e739-46, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30308346%5Bpmid%5D) +1. [Afat S et al: Diagnostic performance of different perfusion algorithms for the detection of angiographical spasm. J Neuroradiol. 45(5):290-4, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29412162%5Bpmid%5D) +1. [Mossa-Basha M et al: Vessel wall MRI characteristics of endovascularly treated aneurysms: association with angiographic vasospasm. J Neurosurg. 1-9, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30239313%5Bpmid%5D) +1. [Mandell DM et al: Intracranial vessel wall MRI: principles and expert consensus recommendations of the American Society of Neuroradiology. AJNR Am J Neuroradiol. 38(2):218-29, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27469212%5Bpmid%5D) +1. [Othman AE et al: Volume perfusion CT imaging of cerebral vasospasm: diagnostic performance of different perfusion maps. Neuroradiology. 58(8):787-92, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27194077%5Bpmid%5D) +1. [Bacigaluppi S et al: Diagnosis of cerebral vasospasm and risk of delayed cerebral ischemia related to aneurysmal subarachnoid haemorrhage: an overview of available tools. Neurosurg Rev. 38(4):603-18, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25732522%5Bpmid%5D) +1. [Fontana J et al: Dynamic autoregulatory response after aneurysmal subarachnoid hemorrhage and its relation to angiographic vasospasm and clinical outcome. Neurocrit Care. 23(3):355-63, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25782447%5Bpmid%5D) +1. [Hollingworth M et al: Results of an international survey on the investigation and endovascular management of cerebral vasospasm and delayed cerebral ischemia. World Neurosurg. 83(6):1120-6, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25681601%5Bpmid%5D) +1. [Jones J et al: Cerebral vasospasm patterns following aneurysmal subarachnoid hemorrhage: an angiographic study comparing coils with clips. J Neurointerv Surg. 7(11):803-7, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25230840%5Bpmid%5D) +1. [Walcott BP et al: Diffuse patterns of nonaneurysmal subarachnoid hemorrhage originating from the Basal cisterns have predictable vasospasm rates similar to aneurysmal subarachnoid hemorrhage. J Stroke Cerebrovasc Dis. 24(4):795-801, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25680661%5Bpmid%5D) +1. [Eddleman CS et al: Endovascular options in the treatment of delayed ischemic neurological deficits due to cerebral vasospasm. Neurosurg Focus. 26(3):E6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19249962%5Bpmid%5D) +1. [Hänggi D et al: Feasibility and safety of intrathecal nimodipine on posthaemorrhagic cerebral vasospasm refractory to medical and endovascular therapy. Clin Neurol Neurosurg. 110(8):784-90, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18554777%5Bpmid%5D) +1. [Ionita CC et al: The value of CT angiography and transcranial doppler sonography in triaging suspected cerebral vasospasm in SAH prior to endovascular therapy. Neurocrit Care. 9(1):8-12, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18080806%5Bpmid%5D) +1. [Keuskamp J et al: High-dose intraarterial verapamil in the treatment of cerebral vasospasm after aneurysmal subarachnoid hemorrhage. J Neurosurg. 108(3):458-63, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18312091%5Bpmid%5D) +1. [Majoie CB et al: Perfusion CT to evaluate the effect of transluminal angioplasty on cerebral perfusion in the treatment of vasospasm after subarachnoid hemorrhage. Neurocrit Care. 6(1):40-4, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17356190%5Bpmid%5D) + + +## Images + + +### Selected Images + +![Autopsied case of ruptured MCA aneurysm with subarachnoid hemorrhage (SAH) in a patient who survived several days shows severe vasospasm of the parent MCA .](images/app.statdx.com_image_thumbnail_cbb44663-5319-4614-81a9-5e4c26f402f9_annotated_true_size_900_quality_90_2315c21f5ad360fb579ecb0827bd81896cdb1c00.jpg) +*Autopsied case of ruptured MCA aneurysm with subarachnoid hemorrhage (SAH) in a patient who survived several days shows severe vasospasm of the parent MCA .* + +![Autopsied case of ruptured MCA aneurysm with subarachnoid hemorrhage (SAH) in a patient who survived several days shows severe vasospasm of the parent MCA .](images/app.statdx.com_image_thumbnail_cbb44663-5319-4614-81a9-5e4c26f402f9_size_174_quality_85_0ac20225adbd81479b2cf0af7cb85e209b71f748.jpg) +*Autopsied case of ruptured MCA aneurysm with subarachnoid hemorrhage (SAH) in a patient who survived several days shows severe vasospasm of the parent MCA .* + +![T1 SPACE C+ vessel wall MR (VW-MR) in a patient with SAH shows concentric enhancement in multiple MCA branches . DSA after a few days shows mutifocal MCA branch stenoses . Consider early preventive measures initiation to reduce vasospasm later, if VW-MR done during presentation with SAH shows arterial wall enhancement.](images/app.statdx.com_image_thumbnail_b134b8ae-01f2-47f4-b742-0b06aa7ae176_annotated_true_size_900_quality_90_1cff9ad1973e45a0a67aa3f9225bf72931450240.jpg) +*T1 SPACE C+ vessel wall MR (VW-MR) in a patient with SAH shows concentric enhancement in multiple MCA branches . DSA after a few days shows mutifocal MCA branch stenoses . Consider early preventive measures initiation to reduce vasospasm later, if VW-MR done during presentation with SAH shows arterial wall enhancement.* + +![AP right ICA DSA in a patient with SAH in right sylvian & anterior interhemispheric fissures shows severe vasospasm in the right MCA M1 & M2 segments & in the right ACA A2 segment & carotid terminus .](images/app.statdx.com_image_thumbnail_69184192-4077-44a7-8c39-15c8c48c20dc_annotated_true_size_900_quality_90_2dbcd909db1c806dbfb518739d1ae88e48a88cf7.jpg) +*AP right ICA DSA in a patient with SAH in right sylvian & anterior interhemispheric fissures shows severe vasospasm in the right MCA M1 & M2 segments & in the right ACA A2 segment & carotid terminus .* + +![DSA after 25-mg verapamil infusion into right ICA shows increase in caliber of all vasospastic segments. The patient received additional IA verapamil in the following days. Recurrent vasospasm after chemical angioplasty is to be expected, but balloon angioplasty is more durable.](images/app.statdx.com_image_thumbnail_8587f1f8-559a-479b-ab0c-a139bacbf094_annotated_true_size_900_quality_90_f10f9c3081cd35291ff45f55cf0ba9df50bfe806.jpg) +*DSA after 25-mg verapamil infusion into right ICA shows increase in caliber of all vasospastic segments. The patient received additional IA verapamil in the following days. Recurrent vasospasm after chemical angioplasty is to be expected, but balloon angioplasty is more durable.* + +![Coronal reformatted NECT in a patient with severe headache shows extensive hyperdense acute SAH in the bilateral MCA cisterns, sylvian fissures, suprasellar cistern, & anterior interhemispheric fissure. Note a slight hyperdense space occupying lesion (SOL) in the left inferior frontal parasagittal region .](images/app.statdx.com_image_thumbnail_e29d4b8f-dbb9-49c9-be10-11fba82312b6_annotated_true_size_900_quality_90_9e5bba3c90762940e4ea3ea342e66ef3a509d761.jpg) +*Coronal reformatted NECT in a patient with severe headache shows extensive hyperdense acute SAH in the bilateral MCA cisterns, sylvian fissures, suprasellar cistern, & anterior interhemispheric fissure. Note a slight hyperdense space occupying lesion (SOL) in the left inferior frontal parasagittal region .* + +![Coronal reformatted MIP image of a CT angiogram in the same setting shows that the SOL is actually a ruptured left terminal ICA aneurysm . Patient underwent emergent aneurysm coiling.](images/app.statdx.com_image_thumbnail_d32caf7c-d965-4c34-88ac-c1a22a12790a_annotated_true_size_900_quality_90_367989ae9c2145586714a5c789c97f709db17e00.jpg) +*Coronal reformatted MIP image of a CT angiogram in the same setting shows that the SOL is actually a ruptured left terminal ICA aneurysm . Patient underwent emergent aneurysm coiling.* + +![CT perfusion done after 4 days, axial time to drain (TTD) CT perfusion map generated using deconvolution algorithm (DC) shows abnormal increased TTD in bilateral parasagittal frontoparietal ACA territories .](images/app.statdx.com_image_thumbnail_3448c858-fd29-4aaa-8414-82473060fac8_annotated_true_size_900_quality_90_266300c8f0b2b56403f29d132ce430ad060a81b6.jpg) +*CT perfusion done after 4 days, axial time to drain (TTD) CT perfusion map generated using deconvolution algorithm (DC) shows abnormal increased TTD in bilateral parasagittal frontoparietal ACA territories .* + +![Axial mean transit time (MTT) CT perfusion map generated using DC shows abnormal increased MTT in the same regions . Cerebral blood flow (CBF) was also reduced in these regions (not shown). As seen here, TTD has higher sensitivity to demonstrate ischemia than other color parametric maps.](images/app.statdx.com_image_thumbnail_ce49868f-c591-4b61-8696-cd222db90e51_annotated_true_size_900_quality_90_6eb82a62e6464db85a47f23cec0f5a701d0bd8d5.jpg) +*Axial mean transit time (MTT) CT perfusion map generated using DC shows abnormal increased MTT in the same regions . Cerebral blood flow (CBF) was also reduced in these regions (not shown). As seen here, TTD has higher sensitivity to demonstrate ischemia than other color parametric maps.* + +![AP DSA of the right ICA in the same patient 3 hours after the CT perfusion study shows severe right A1 ACA and mild right M1 MCA vasospasm.](images/app.statdx.com_image_thumbnail_8acd54d8-5e01-4e00-9317-12f3e69ae36a_annotated_true_size_900_quality_90_a771fdc5412e4b8e9794f041134f48b5ec794e20.jpg) +*AP DSA of the right ICA in the same patient 3 hours after the CT perfusion study shows severe right A1 ACA and mild right M1 MCA vasospasm.* + +![AP DSA of the left ICA shows severe left M1 MCA vasospasm. The left A1 ACA also had vasospasm seen in oblique projections (not shown). Vasospasm responded well to IA verapamil (images not shown). Note the aneurysm coil mass , which completely obliterates the flow in the left ICA aneurysm. SAH-associated vasospasm usually begins after 3-4 days.](4a221f63-f9b8-48f6-bd3b-371655e559f7) +*AP DSA of the left ICA shows severe left M1 MCA vasospasm. The left A1 ACA also had vasospasm seen in oblique projections (not shown). Vasospasm responded well to IA verapamil (images not shown). Note the aneurysm coil mass , which completely obliterates the flow in the left ICA aneurysm. SAH-associated vasospasm usually begins after 3-4 days.* + + +### Additional Images + +![Axial NECT in a 43-year-old male in the ER with "worst headache of life" shows diffuse SAH in the basal cisterns, especially in the left sylvian fissure.](100272ee-7073-46bb-a5a0-a59787ba7179) +*Axial NECT in a 43-year-old male in the ER with "worst headache of life" shows diffuse SAH in the basal cisterns, especially in the left sylvian fissure.* + +![CT angiogram in the same patient shows a lobulated aneurysm at the left distal ICA bifurcation.](f330fe10-5266-494c-b07c-557918d9778a) +*CT angiogram in the same patient shows a lobulated aneurysm at the left distal ICA bifurcation.* + +![Four days after emergency clipping of the aneurysm, the patient became drowsy and developed right-sided weakness. AP DSA of the left ICA shows mass effect, vasospasm , seen here as narrowing of the proximal ACA and MCA. The patient responded well to IA infusion of verapamil.](0d3fbe4b-50f1-4df4-a41e-8042968ab53d) +*Four days after emergency clipping of the aneurysm, the patient became drowsy and developed right-sided weakness. AP DSA of the left ICA shows mass effect, vasospasm , seen here as narrowing of the proximal ACA and MCA. The patient responded well to IA infusion of verapamil.* + +![Axial NECT in a patient S/P resection of a sphenoidal ridge meningioma complicated by significant intraoperative hemorrhage shows SAH in the right sylvian fissure and anterior interhemispheric fissure .](6b637430-25e3-454d-990e-08131c72f8a5) +*Axial NECT in a patient S/P resection of a sphenoidal ridge meningioma complicated by significant intraoperative hemorrhage shows SAH in the right sylvian fissure and anterior interhemispheric fissure .* + +![Axial NECT obtained 10 days later when the patient developed hemiparesis shows a focal area of low attenuation in the right frontal lobe , consistent with infarction. There is blurring of both the superficial and deep gray-white interface adjacent to the infarct.](a11fae5c-34bf-47bf-9e0f-06a6d19a8ff7) +*Axial NECT obtained 10 days later when the patient developed hemiparesis shows a focal area of low attenuation in the right frontal lobe , consistent with infarction. There is blurring of both the superficial and deep gray-white interface adjacent to the infarct.* + +![Anteroposterior 3D TOF MRA MIP image in the same patient shows no signal from flow-related enhancement along the right M1 segment . The M2 vessels are attenuated compared with the left side, suggestive of reduced flow.](690d430d-fedd-41f8-8ea6-fbc1f88abfcc) +*Anteroposterior 3D TOF MRA MIP image in the same patient shows no signal from flow-related enhancement along the right M1 segment . The M2 vessels are attenuated compared with the left side, suggestive of reduced flow.* + +![Axial DTI MR shows a large area of ischemia/infarction in the right frontal lobe . The constellation of imaging findings along with the delayed clinical deterioration 10 days after documented SAH made vasospasm the most likely etiology.](3f09584b-04c5-4e00-868b-1dbaa5d864cf) +*Axial DTI MR shows a large area of ischemia/infarction in the right frontal lobe . The constellation of imaging findings along with the delayed clinical deterioration 10 days after documented SAH made vasospasm the most likely etiology.* + diff --git a/results.json b/results.json index f1251d7..e49f4f6 100644 --- a/results.json +++ b/results.json @@ -1,112 +1,116 @@ [ { - "path": "docs_md/articles/fusiform-arterial-enlargement_31d50b93-b057-4da3-86b5-4cc8fb0bc806.md", - "title": "Fusiform Arterial Enlargement", - "docid": "31d50b93-b057-4da3-86b5-4cc8fb0bc806", + "path": "docs_md/articles/multiinfarct-dementia_3823c4d4-5e98-46da-a717-892fef54b382.md", + "title": "Multiinfarct Dementia", + "docid": "3823c4d4-5e98-46da-a717-892fef54b382", "breadcrumbs": [ - "Brain", - "Differential Diagnosis", - "Arteries", - "Anatomically Based Differentials", - "Fusiform Arterial Enlargement" + "Nuclear Medicine", + "Central Nervous System", + "Neurodegeneration", + "Multiinfarct Dementia" ], "authors": [ { - "key": "2bca6b86-1eca-4e93-b997-4e18913686a7", - "value": "Hediyeh Baradaran, MD, MS" + "key": "9d40c5b1-57d2-442c-9daf-8d8d9d53e24b", + "value": "Akiva Mintz, MD, PhD, MHA, CFA" }, { - "key": "f184750a-90b4-47a7-907b-23b05d70357a", - "value": "Chang Yueh Ho, MD" + "key": "bbc899b6-2885-44bb-a5b0-24eec7314d33", + "value": "Bryan J. 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Quigley, III, MD, PhD" + } + ], + "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Cerebral Ischemia and Infarction, Acute Cerebral Ischemia/Infarction" + }, + { + "path": "docs_md/articles/cerebral-hyperperfusion-syndrome_e66febb9-d79e-4f04-88b1-205ba8a0822f.md", + "title": "Cerebral Hyperperfusion Syndrome", + "docid": "e66febb9-d79e-4f04-88b1-205ba8a0822f", + "breadcrumbs": [ + "Brain", + "Diagnosis", + "Pathology-Based Diagnoses", + "Stroke", + "Cerebral Ischemia and Infarction", + "Cerebral Hyperperfusion Syndrome" + ], + "authors": [ + { + "key": "5cff4116-3654-4b3a-bb75-5ebe0b8c9850", + "value": "Anne G. Osborn, MD, FACR" + } + ], + "pageKeywords": "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Cerebral Ischemia and Infarction, Cerebral Hyperperfusion Syndrome" } ] \ No newline at end of file diff --git a/scrapers/document_to_markdown.py b/scrapers/document_to_markdown.py index 6046c34..97faf6f 100644 --- a/scrapers/document_to_markdown.py +++ b/scrapers/document_to_markdown.py @@ -511,12 +511,13 @@ def find_title_in_capture_index(docid: str, base_dir: str) -> str | None: return None -def process_file(path: str, out_dir: str, overwrite: bool = False) -> tuple[bool, str]: +def process_file(path: str, out_dir: str, overwrite: bool = False, verbose: bool = False) -> tuple[bool, str]: """Process one JSON file. Returns (success, output_path_or_error).""" base = os.path.basename(path) name, _ = os.path.splitext(base) - logger.debug(f"Processing file: {path}") + if verbose: + logger.debug(f"Processing file: {path}") # attempt to extract article name for nicer filenames try: @@ -698,8 +699,9 @@ def process_file(path: str, out_dir: str, overwrite: bool = False) -> tuple[bool # could be list or simple string ddx_list = ddx_entry else: - logger.debug(f"No cached DDX entry for docid {docid}") - logger.debug(f"DDX keys available: {list(DDX.keys())}") + if verbose: + logger.debug(f"No cached DDX entry for docid {docid}") + logger.debug(f"DDX keys available: {list(DDX.keys())}") if ddx_list: # render list representation