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title: "ADEM, Brain"
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docid: "ed94b660-cf20-4ebb-8d6f-2b93505f2928"
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authors:
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- key: "99e1aff7-f42c-43a0-95ae-d89c8551aa01"
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value: "Kevin R. Moore, MD"
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breadcrumbs:
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-
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name: "Pediatrics"
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slug: "pediatrics"
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treeNodeId: "a915965c-d436-44cf-ae65-2f22e7246ea4"
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-
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name: "Diagnosis"
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slug: "diagnosis"
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treeNodeId: "2b5cea64-a083-489e-ac0c-ec14ba059026"
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-
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name: "Pediatric Neuroradiology"
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slug: "pediatric-neuroradiology"
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treeNodeId: "d0eb8f4a-e769-43dd-896c-8c9c27ce8759"
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-
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name: "Brain"
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slug: "brain"
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treeNodeId: "feaaadba-649b-4f0a-9aad-9188a8f9926a"
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-
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name: "Pathology-Based Diagnoses"
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slug: "pathology-based-diagnoses"
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treeNodeId: "2d26053f-23a7-4062-bf35-a93775ae1209"
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-
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name: "Inflammatory and Demyelinating Disease"
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slug: "inflammatory-and-demyelinating-dis-"
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treeNodeId: "cb319228-da96-4d29-8276-c72388a57656"
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-
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name: "ADEM, Brain"
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slug: "adem-brain"
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treeNodeId: null
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category: "Pediatrics"
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documentVersionId: "d15d7c30-1933-4a0f-afc7-3e9b13ce1ff7"
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imageCount: 26
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lastUpdated: "02/05/24"
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pageDescription: "ADEM, Brain"
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pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Inflammatory and Demyelinating Disease, ADEM, Brain"
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pageTitle: "ADEM, Brain | STATdx"
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enhancedTitle: "ADEM, Brain"
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type: "DX"
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references: true
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breadcrumbs:
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- "Pediatrics"
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- "Diagnosis"
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- "Pediatric Neuroradiology"
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- "Brain"
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- "Pathology-Based Diagnoses"
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- "Inflammatory and Demyelinating Disease"
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- "ADEM, Brain"
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---
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# KEY FACTS
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- ## Terminology
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- Autoimmune-mediated white matter (WM) demyelination of brain &/or spinal cord, usually with remyelination
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- ## Imaging
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- Best diagnostic clue: Multifocal WM and deep gray lesions days to weeks following infection/vaccination
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- May involve both brain and spinal cord; WM > gray matter, but usually both affected
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- Both supratentorial and infratentorial lesions
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- Multifocal punctate to large, flocculent FLAIR hyperintensities
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- Deep/juxtacortical WM > periventricular WM
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- Do not usually involve callososeptal interface
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- Most lesions show increased signal on DWI (T2 shine-through)
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- Punctate, ring, incomplete ring, peripheral enhancement
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- Absence of enhancement does not exclude diagnosis
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- MRS: ↓ NAA within lesions; may see ↑ Cho, ↑ lactate
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- ## Top Differential Diagnoses
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- Multiple sclerosis (MS)
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- Collagen-vascular disorders
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- Neoplasms
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- Hemophagocytic lymphohistiocytosis (HLH)
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- Mitochondrial diseases
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- Leukodystrophies
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- Acute hypertensive encephalopathy, PRES
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- Autoimmune-mediated vasculitis
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- Fabry disease
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- ## Pathology
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- > 30 different infectious agents reported
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- Anti-MOG IgG antibodies found more commonly in younger patients
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- ## Clinical Issues
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- Mean age: 5-8 years but can occur at any age
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- Male predominance (M:F = 1:0.6-0.8), unlike MS
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- Usually monophasic, self-limited
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- Complete recovery within 1 month: 50-60%
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- Mortality: 10-30%
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- ## Diagnostic Checklist
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- Imaging findings often lag behind symptom onset, resolution
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# TERMINOLOGY
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- ## Abbreviations
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- Acute disseminated encephalomyelitis (ADEM)
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- ## Definitions
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- Autoimmune-mediated white matter (WM) demyelination of brain &/or spinal cord, usually with remyelination
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Multifocal WM/basal ganglia lesions days to weeks following preceding infection
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- 93% within 3 weeks of infection
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- ### Location
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- Widespread WM and gray matter brain abnormalities
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- Both supratentorial and infratentorial lesions
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- Most conspicuous on T2-weighted and fluid-attenuated inversion recovery (FLAIR) sequences
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- May involve both brain and spinal cord; WM > gray matter, but usually both affected
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- Deep/juxtacortical WM > periventricular WM
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- ### Size
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- Tumefactive lesions may be large but with less mass effect than expected from similar size tumor
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- ### Morphology
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- Punctate to flocculent
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- Tumefactive, mass-like lesions possible
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- ## CT Findings
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- ### NECT
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- Initial CT normal in 40% of patients
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- ### CECT
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- Normal or multifocal punctate or ring-enhancing lesions
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- ## MR Findings
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- ### T1WI
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- Hypointense "black holes" uncommon
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- ### T2WI
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- Hyperintensities may be better visualized in brainstem and posterior fossa than on T2 FLAIR
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- ### FLAIR
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- Widespread multifocal punctate to large, flocculent FLAIR hyperintensities
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- Bilateral, asymmetric, frequently poorly marginated
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- Involve peripheral WM-gray matter junction subcortical WM
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- Thalami and basal ganglia symmetrically involved, particularly in children
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- Can involve brainstem and posterior fossa
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- Usually do **not** involve callososeptal interface
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- ### DWI
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- Variably hyperintense lesions on DWI (trace) images
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- ADC may be increased or decreased
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- Most lesions show increased signal (T2 shine-through)
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- Diffusion restriction uncommon, suggests worse prognosis
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- Diffusivity normal within normal-appearing WM (NAWM), unlike MS
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- ### T1WI C+
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- Punctate, ring, incomplete ring, peripheral enhancement
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- Cranial nerve(s) may enhance
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- Absence of enhancement does not exclude ADEM
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- ### MRS
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- NAA low within lesions; lactate may be elevated
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- Choline often elevated in acute lesions
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- NAA normalizes with resolution of symptoms/MR abnormalities
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- Magnetization transfer ratio (MTR)
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- ADEM MTR normal within NAWM, unlike MS
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- ## Imaging Recommendations
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- ### Best imaging tool
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- Contrast-enhanced brain MR, including FLAIR T2 imaging
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- Initial imaging often normal but more sensitive than CT
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- May appear identical to MS; repeat MR necessary to distinguish with certainty
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- ## Nuclear Medicine Findings
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- Tc-99m-HMPAO SPECT shows more extensive hypoperfusion than T2 lesions
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# DIFFERENTIAL DIAGNOSIS
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- [Multiple Sclerosis](/document/pediatric-multiple-sclerosis-brain/f2592b04-f800-4235-9eea-a43f2bf4adfe)
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- Predilection for periventricular WM (callososeptal interface), subcortical U fibers, posterior fossa
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- Lesions often more symmetric than ADEM
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- Relapsing-remitting course common
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- ## Collagen-Vascular Disorders
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- e.g., systemic lupus erythematosus (SLE), Sjögren syndrome, and neuro-Behçet disease
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- Can present with primarily CNS involvement in children
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- Imaging findings mimic ADEM
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- Elevated markers of systemic inflammation help distinguish
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- Abnormal antinuclear antibody profile, positive double-stranded DNA antibodies, elevated angiotensin converting enzyme, antiphospholipid antibodies
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- Behcet: Classic triad of oral and genital ulcerations with uveitis
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- ## Neoplasms
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- Mimic features of pediatric demyelinating processes
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- Optic pathway neoplasms, primary brain tumors, intramedullary spinal cord tumors
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- Specific imaging features and clinical history can help distinguish from pediatric demyelinating disorders
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- ## Hemophagocytic Lymphohistiocytosis
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- With primary CNS involvement can (rarely) precede systemic disease manifestations
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- Mimics clinical presentation of ADEM
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- Imaging manifestations are protean
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- ## Mitochondrial Diseases
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- MELAS and MERRF may present in setting of intercurrent acute infection
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- Typical brain MR features include abnormal T2-hyperintense signal in basal ganglia and nonvascular distribution hemispheric lesions
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- DWI MR will show acute restriction reflecting "metabolic stroke"
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- Helps distinguish from nonrestricting demyelinating processes
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- Elevation of serum, CSF lactate levels
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- [Leukodystrophies](/document/leukodystrophies/f4ff3738-131c-46bf-be71-1811f2c1776c)
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- Genetic WM diseases are typically symmetrical, sometimes with characteristic location or appearance
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- e.g., metachromatic leukodystrophy, X-linked adrenoleukodystrophy, Alexander disease, globoid cell leukodystrophy (Krabbe disease)
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- [Autoimmune-Mediated Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0)
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- Multifocal gray matter-WM lesions
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- Bilateral, usually cortical/subcortical, basal ganglia/thalami
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- Ring-enhancing lesions may mimic infection
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- [Acute Hypertensive Encephalopathy, PRES](/document/acute-hypertensive-encephalopathy--/efc6f9c2-dad9-4eb8-bad2-421bfaf1ec57)
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- Typically posterior circulation in cortex/subcortical WM
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- May affect deep gray nuclei
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- [Aging Brain With Hyperintense White Matter Lesions](/document/normal-aging-brain/2a315550-b2ea-4afe-a2ef-f93a2209f276)
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- Atherosclerotic brain changes in 50% patients > 50 years old
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- Found in normotensive patients; more common in hypertensives
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- Present in 10-30% of cognitively normal older adult patients
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- MR: Scattered, asymmetric WM lesions, without enhancement
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- Often periatrial; posterior fossa uncommon
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- Spares callososeptal interface, subcortical U fibers
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- [Fabry Disease](/document/fabry-disease/83fd222a-9b37-4087-afab-34ba74525887)
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- Synonym: Angiokeratoma corporis diffusum universalis
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- X-linked recessive; incidence 1/40,000
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- Deficiency α-galactosidase A; overaccumulation of glycosphingolipids within lysosomes
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- MR: Scattered, asymmetric WM lesions without enhancement
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- May involve brainstem and posterior fossa
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- Spares callososeptal interface and subcortical U fibers
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- Cranial MR sensitive to identify neurologic involvement in asymptomatic patients
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- Present with renal failure/heart disease
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Autoimmune-mediated severe acute demyelination
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- Pathogenesis incompletely understood, but thought to be triggered by environmental stimulus in genetically susceptible individuals
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- Myelin autoantigens, such as myelin basic protein (MBP), proteolipid protein (PLP), and myelin oligodendrocyte glycoprotein (MOG), share antigenic determinants with those of infecting pathogen
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- Often follows antecedent nonspecific upper respiratory tract infection
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- > 30 different infectious agents and immunizations reported
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- Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2 or COVID) infection has been reported with ADEM, but overall incidence appears to be low
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- Some cases are spontaneous (no known cause)
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- Neurologic symptoms typically appear 4-13 days after preceding infection
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- Early reports suggested that minority of ADEM cases followed immunization
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- Current studies show little or no association between ADEM and immunizations (postvaccinal ADEM)
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- Implicated viral pathogens associated with ADEM are myriad and include coronavirus, coxsackievirus, cytomegalovirus, Epstein-Barr, herpes simplex, hepatitis A, HIV, influenza, measles, rubella, varicella zoster, and West Nile viruses
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- Bacterial organisms associated with ADEM include *Borrelia burgdorferi*, *Chlamydia trachomatis*, *Leptospira*, *Mycoplasma pneumoniae*,*rickettsia*, and beta hemolytic *Streptococcus*
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- ### Genetics
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- ADEM associated with DRB1*01 and DRB1*017(03) in Russian population
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- Acute hemorrhagic leukoencephalopathy variant associated with ulcerative colitis and asthma
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- Anti-MOG IgG antibodies found more commonly in younger patients
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- Most cases probably reflect MOGAD
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- ## Gross Pathologic & Surgical Features
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- None, unless hemorrhage (rare) or tumefactive edema
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- ## Microscopic Features
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- Acute myelin breakdown
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- Perivenous inflammation; lymphocytic infiltrates
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- Relative axonal preservation; atypical astrogliosis
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- Virus particles generally not found, unlike viral encephalitides
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- Similar to experimental allergic encephalomyelitis, supporting autoimmune-related etiology
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Usually preceded by prodromal phase: Fever, malaise, myalgia
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- Multifocal neurologic symptoms, 2 days to 4 weeks after viral illness
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- Initial symptoms: Headache, fever, drowsiness
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- Cranial nerve palsies, hemiparesis
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- Decreased consciousness (from lethargy to coma)
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- Behavioral changes
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- ### Other signs/symptoms
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- Seizures in 10-35%
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- ### Clinical profile
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- CSF normal in 60%
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- If abnormal (lymphocyte pleocytosis, elevated protein) usually lacks CSF oligoclonal bands
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- ## Demographics
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- ### Age
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- Children > adults
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- Mean age: 5-8 years but can occur at any age
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- ### Sex
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- Male predominance (M:F = 1.0:0.6-0.8), unlike MS
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- ### Epidemiology
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- Pediatric ADEM is uncommon illness
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- Estimated annual incidence 0.2-0.5 per 100,000 children
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- Typically delay between symptom onset, imaging findings
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- Rare, yet most common para-/postinfectious disorder
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- Most common in winter and spring
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- Exact epidemiology unknown but increasingly reported
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- ## Natural History & Prognosis
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- Usually monophasic and self-limited
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- Variable prognosis
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- Complete recovery within 1 month (50-60%)
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- Neurologic sequelae (most commonly seizures) (20-30%)
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- Mortality (10-30%)
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- Relapses are rare
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- "Relapsing disseminated encephalomyelitis"
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- Many of these cases may actually be MOG antibody disease (MOGAD)
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- May be difficult to distinguish from relapsing-remitting MS
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- Varicella and rubella ADEM have preferential patterns
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- Varicella ADEM characterized by cerebellar ataxia and mild pyramidal dysfunction
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- Rubella ADEM characterized by acute explosive onset, seizures, coma, and moderate pyramidal signs
|
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- Rare manifestations of ADEM
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- Acute hemorrhagic leukoencephalopathy (2%)
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- Young patients with abrupt symptom onset and fulminant clinical course
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- Inflammatory hemorrhagic WM demyelination
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- These variants are more rapidly progressive and clinically severe than typical ADEM
|
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- Otherwise, their symptomatology is similar to typical ADEM with meningismus, headache, seizures, multifocal and asymmetric neurologic deficits, &/or coma
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- Prognosis for survival or recovery of neurologic function is worse than for classic ADEM
|
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- Bilateral striatal necrosis (usually in infants, may be reversible)
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- ## Treatment
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||||
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||||
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||||
- Immunosuppressive/immunomodulatory therapy
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- MR may show prompt improvement after therapy
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- Plasma exchange therapy
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- 40% of patients failing steroid treatment may show marked improvement
|
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# DIAGNOSTIC CHECKLIST
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||||
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||||
- ## Image Interpretation Pearls
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||||
|
||||
|
||||
- Imaging findings often lag behind both symptom onset and resolution
|
||||
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31576fd8-140b-43b2-9f58-b6f33ef20ca3
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## References
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# Selected References
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1. [Kalita IR et al: Acute abducens nerve palsy with acute disseminated encephalomyelitis-like presentation following COVID-19 vaccination. Indian J Ophthalmol. 71(5):2279-81, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37202971%5Bpmid%5D)
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1. [Chen LW et al: Prognostic factors for functional recovery in children with moderate to severe acute disseminated encephalomyelitis. Mult Scler Relat Disord. 66:104056, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=35878513%5Bpmid%5D)
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1. [Wang Y et al: SARS-CoV-2-associated acute disseminated encephalomyelitis: a systematic review of the literature. J Neurol. 269(3):1071-92, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=34459986%5Bpmid%5D)
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1. [Benallegue N et al: Neurological involvement in secondary hemophagocytic lymphohistiocytosis in children. Eur J Paediatr Neurol. 34:110-7, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34482125%5Bpmid%5D)
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1. [Pujari SS et al: Acute haemorrhagic leukoencephalitis (AHLE) - our experience and a short review. J Neuroimmunol. 361:577751, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34739912%5Bpmid%5D)
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1. [Baumann M et al: MRI of the first event in pediatric acquired demyelinating syndromes with antibodies to myelin oligodendrocyte glycoprotein. J Neurol. 265(4):845-55, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29423614%5Bpmid%5D)
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1. [Aubert-Broche B et al: Monophasic demyelination reduces brain growth in children. Neurology. 88(18):1744-50, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28381515%5Bpmid%5D)
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1. [Bester M et al: Neuroimaging of multiple sclerosis, acute disseminated encephalomyelitis, and other demyelinating diseases. Semin Roentgenol. 49(1):76-85, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24342677%5Bpmid%5D)
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1. [Longoni G et al: White matter changes in paediatric multiple sclerosis and monophasic demyelinating disorders. Brain. 140(5):1300-15, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28334875%5Bpmid%5D)
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1. [Koelman DLH et al: Acute disseminated encephalomyelitis: prognostic value of early follow-up brain MRI. J Neurol. 264(8):1754-62, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28695361%5Bpmid%5D)
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1. [Marziali S et al: Acute disseminated encephalomyelitis following Campylobacter jejuni gastroenteritis: Case report and review of the literature. Neuroradiol J. 30(1):65-70, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27888275%5Bpmid%5D)
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1. [Kanekar S et al: A pattern approach to focal white matter hyperintensities on magnetic resonance imaging. Radiol Clin North Am. 52(2):241-61, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24582339%5Bpmid%5D)
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1. [Daida K et al: Cytomegalovirus-associated encephalomyelitis in an immunocompetent adult: a two-stage attack of direct viral and delayed immune-mediated invasions. case report. BMC Neurol. 16(1):223, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27855658%5Bpmid%5D)
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1. [Pohl D et al: Acute disseminated encephalomyelitis: updates on an inflammatory CNS syndrome. Neurology. 87(9 Suppl 2):S38-45, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27572859%5Bpmid%5D)
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1. [Yuan JL et al: Acute disseminated encephalomyelitis following vaccination against hepatitis B in a child: a case report and literature review. Case Rep Neurol Med. 2016:2401809, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27478662%5Bpmid%5D)
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1. [Baumann M et al: Clinical and neuroradiological differences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein. J Neurol Neurosurg Psychiatry. 86(3):265-72, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25121570%5Bpmid%5D)
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1. [Karussis D: The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review. J Autoimmun. 48-49:134-42, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24524923%5Bpmid%5D)
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1. [Mariotto S et al: Clinical spectrum and IgG subclass analysis of anti-myelin oligodendrocyte glycoprotein antibody-associated syndromes: a multicenter study. J Neurol. 264(12):2420-30, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=29063242%5Bpmid%5D)
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1. [Nakamura Y et al: Anti-MOG antibody-positive ADEM following infectious mononucleosis due to a primary EBV infection: a case report. BMC Neurol. 17(1):76, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28420330%5Bpmid%5D)
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1. [Tenembaum SN: Acute disseminated encephalomyelitis. Handb Clin Neurol. 112:1253-62, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23622336%5Bpmid%5D)
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1. [Wingerchuk DM et al: Acute disseminated encephalomyelitis, transverse myelitis, and neuromyelitis optica. Continuum (Minneap Minn). 19(4 Multiple Sclerosis):944-67, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23917095%5Bpmid%5D)
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1. [Callen DJ et al: Role of MRI in the differentiation of ADEM from MS in children. Neurology. 72(11):968-73, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19038851%5Bpmid%5D)
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1. [Noorbakhsh F et al: Acute disseminated encephalomyelitis: clinical and pathogenesis features. Neurol Clin. 26(3):759-80, ix, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18657725%5Bpmid%5D)
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1. [Rossi A: Imaging of acute disseminated encephalomyelitis. Neuroimaging Clin N Am. 18(1):149-61; ix, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18319160%5Bpmid%5D)
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1. [Tenembaum S et al: Acute disseminated encephalomyelitis. Neurology. 68(16 Suppl 2):S23-36, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17438235%5Bpmid%5D)
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1. [Menge T et al: Acute disseminated encephalomyelitis: an update. Arch Neurol. 62(11):1673-80, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16286539%5Bpmid%5D)
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1. [Yeh EA et al: Detection of coronavirus in the central nervous system of a child with acute disseminated encephalomyelitis. Pediatrics. 113(1 Pt 1):e73-6, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14702500%5Bpmid%5D)
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1. [Dale RC: Acute disseminated encephalomyelitis. Semin Pediatr Infect Dis. 14(2):90-5, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12881796%5Bpmid%5D)
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1. [Garg RK: Acute disseminated encephalomyelitis. Postgrad Med J. 79(927):11-17, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12566545%5Bpmid%5D)
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1. [Idrissova ZhR et al: Acute disseminated encephalomyelitis in children: clinical features and HLA-DR linkage. Eur J Neurol. 10(5):537-46, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12940836%5Bpmid%5D)
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1. [Okamoto K et al: MR features of diseases involving bilateral middle cerebellar peduncles. AJNR Am J Neuroradiol. 24(10):1946-54, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14625215%5Bpmid%5D)
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1. [Sener RN: Neuro-Behcet's disease: diffusion MR imaging and proton MR spectroscopy. AJNR Am J Neuroradiol. 24(8):1612-4, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=13679280%5Bpmid%5D)
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1. [Stonehouse M et al: Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians. Arch Dis Child. 88(2):122-4, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12538312%5Bpmid%5D)
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1. [Inglese M et al: Magnetization transfer and diffusion tensor MR imaging of acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 23(2):267-72, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=11847052%5Bpmid%5D)
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1. [Murthy JM: Acute disseminated encephalomyelitis. Neurol India. 50(3):238-43, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12391446%5Bpmid%5D)
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1. [Tenembaum S et al: Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology. 59(8):1224-31, 2002](http://www.ncbi.nlm.nih.gov/pubmed/?term=12391351%5Bpmid%5D)
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1. [Bizzi A et al: Quantitative proton MR spectroscopic imaging in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 22(6):1125-30, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11415908%5Bpmid%5D)
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1. [Honkaniemi J et al: Delayed MR imaging changes in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 22(6):1117-24, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11415907%5Bpmid%5D)
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1. [Straussberg R et al: Improvement of atypical acute disseminated encephalomyelitis with steroids and intravenous immunoglobulins. Pediatr Neurol. 24(2):139-43, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11275464%5Bpmid%5D)
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1. [Dale RC et al: Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain. 123 Pt 12:2407-22, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11099444%5Bpmid%5D)
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||||
1. [Rust RS: Multiple sclerosis, acute disseminated encephalomyelitis, and related conditions. Semin Pediatr Neurol. 7(2):66-90, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10914409%5Bpmid%5D)
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1. [Schaefer PW et al: Diffusion-weighted MR imaging of the brain. Radiology. 217(2):331-45, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11058626%5Bpmid%5D)
|
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1. [Kocer N et al: CNS involvement in neuro-Behcet syndrome: an MR study. AJNR Am J Neuroradiol. 20(6):1015-24, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10445437%5Bpmid%5D)
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## Images
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|
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### Selected Images
|
||||
|
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|
||||
*Axial FLAIR MR in a child with ADEM shows peripheral, confluent areas of hyperintensity predominantly involving subcortical white matter. Bilateral but asymmetric pattern is typical of ADEM.*
|
||||
|
||||
|
||||
*Axial T1 C+ MR in the same patient shows marked, irregular enhancement of nearly all lesions. As ADEM is a monophasic illness, enhancement of most lesions is typical; all lesions have a similar time course. Enhancement of multiple sclerosis lesions is more variable.*
|
||||
|
||||
|
||||
*Axial FLAIR MR in a 5-year-old with ADEM following an EBV infection demonstrates more subtle findings with abnormal hyperintensity in the left basal ganglia <img src='/img/arrows/WS.png'/> and right parietal cortex/subcortical white matter <img src='/img/arrows/WO.png'/>.*
|
||||
|
||||
|
||||
*Axial T1 C+ MR in the same patient reveals no abnormal lesional contrast enhancement.*
|
||||
|
||||
|
||||
*Axial T2 MR in a 21-month-old who presented with seizures and encephalopathy demonstrates numerous lesions involving both subcortical white matter and gray matter (caudate nuclei, left basal ganglia, both thalami).*
|
||||
|
||||
|
||||
*Coronal FLAIR MR in the same patient confirms characteristic lesion distribution. Contrast-enhanced imaging (not shown) demonstrated no lesional enhancement.*
|
||||
|
||||
|
||||
*Axial T2WI MR shows hyperintense lesions in the brachium pontis bilaterally, typical for demyelination. The right-sided lesion shows a targetoid <img src='/img/arrows/WS.png'/> appearance. Enhancement of several lesions was present on postcontrast T1 images (not shown).*
|
||||
|
||||
|
||||
*Axial FLAIR MR shows large, confluent regions of hyperintense signal <img src='/img/arrows/CC.png'/> in the periventricular and subcortical white matter in a 14-year-old who presented with neck stiffness, fatigue, and seizures.*
|
||||
|
||||
|
||||
*Axial SWI MR in the same patient shows petechial hemorrhages <img src='/img/arrows/CS.png'/> in regions of FLAIR signal abnormality.*
|
||||
|
||||
|
||||
*Sagittal T1 C+ MR in same patient shows extensive irregular ring enhancement <img src='/img/arrows/CC.png'/> involving multiple subcortical white matter lesions. Acute hemorrhagic leukoencephalopathy (AHL) is a rare manifestation of ADEM, occurring in 2% of cases. AHL is associated with a very poor prognosis. Aggressive therapeutic management is a prerequisite to avoid usual disease course with fatal outcome.*
|
||||
|
||||
|
||||
### Additional Images
|
||||
|
||||
|
||||
*Axial T1WI C+ MR shows an incomplete ring of peripheral enhancement, typical of a demyelinating process. Other contrast enhancement patterns include ovoid or punctate homogeneous enhancement.*
|
||||
|
||||
|
||||
*Axial DWI MR shows increased signal in areas of FLAIR hyperintensity (not shown). The foci were hypointense on ADC images, indicating diffusion restriction. Both white matter and gray matter involvement is present. Diffusion restriction is an uncommon imaging finding and is associated with a worse prognosis.*
|
||||
|
||||
|
||||
*Coronal T2WI MR shows large, confluent regions of hyperintense signal in the white matter <img src='/img/arrows/WO.png'/> and deep gray nuclei <img src='/img/arrows/WS.png'/> of a child with ADEM. Although ADEM predominantly involves white matter, gray matter is often affected.*
|
||||
|
||||
|
||||
*MRS at long echo time (TE) in a patient with acute lesions in ADEM demonstrates an ↑ choline <img src='/img/arrows/CC.png'/>, ↓ NAA <img src='/img/arrows/CS.png'/>, and the presence of a lactate doublet <img src='/img/arrows/WS.png'/>. Increase in choline with corresponding reductions in NAA normalize as the clinical and conventional neuroimaging abnormalities resolve.*
|
||||
|
||||
|
||||
*Axial FLAIR MR shows bilateral, multiple asymmetric, flocculent, hyperintense lesions of ADEM.*
|
||||
|
||||
|
||||
*Coronal T1WI C+ MR demonstrates partial peripheral enhancement around multiple asymmetric flocculent lesions of acute disseminated encephalomyelitis. Note the supra- and infratentorial lesions.*
|
||||
|
||||
|
||||
*Axial FLAIR MR shows asymmetric, flocculent, nearly confluent, hyperintense lesions of ADEM within posterior fossa structures.*
|
||||
|
||||
|
||||
*Axial FLAIR MR reveals multiple asymmetric, primarily punctate, hyperintense lesions of ADEM.*
|
||||
|
||||
|
||||
*Axial FLAIR MR demonstrates a large, tumefactive, hyperintense lesion. Less mass effect is present than expected for lesion size. Smaller lesions were also present at other locations.*
|
||||
|
||||
|
||||
*Axial T1WI C+ MR in the same patient demonstrates a large, tumefactive, hypointense lesion with minimal partial peripheral enhancement. Less mass effect is present than expected for lesion size. More lesions were seen elsewhere.*
|
||||
|
||||
|
||||
*Axial FLAIR MR demonstrates a rare manifestation of ADEM: Bilateral striatal necrosis, evidenced by asymmetric confluent hyperintensity involving the gray matter and white matter of bilateral corpus striatum.*
|
||||
|
||||
|
||||
*Axial DWI MR confirms the rare manifestation of ADEM, displaying bilateral striatal necrosis, as evidenced by asymmetric confluent restricted diffusion involving gray matter and white matter of bilateral corpus striatum.*
|
||||
|
||||
|
||||
*Axial T2WI MR shows multiple bilateral but asymmetric, T2-hyperintense foci <img src='/img/arrows/WS.png'/>. None of the lesions demonstrates significant mass effect in this adult patient with ADEM. Imaging mimics multiple sclerosis, vasculitis, and microvascular ischemia.*
|
||||
|
||||
|
||||
*Axial FLAIR MR shows a large, tumefactive, hyperintense ADEM lesion <img src='/img/arrows/WS.png'/> with mass effect less than expected for the size of the lesion. Another clue to its nonneoplastic nature is the right-sided lesion <img src='/img/arrows/WC.png'/>.*
|
||||
|
||||
|
||||
*MRS at a long TE in the same patient shows the tumefactive lesion has a depressed choline <img src='/img/arrows/WC.png'/> and NAA <img src='/img/arrows/WO.png'/> metabolites in the presence of a large lactate doublet <img src='/img/arrows/WS.png'/>. This MRS helps distinguish this lesion from a neoplasm. MRS of ADEM may show elevated choline acutely.*
|
||||
|
||||
|
||||
*Axial FLAIR MR shows typical findings of ADEM with peripheral, subcortical hyperintense foci <img src='/img/arrows/WC.png'/>. Bilateral insular involvement is seen <img src='/img/arrows/WS.png'/>. Periventricular and callososeptal lesions, which are typical of multiple sclerosis, are not commonly seen in ADEM.*
|
||||
|
||||
Reference in New Issue
Block a user