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title: "Demyelinating Diseases"
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docid: "e3ba880e-d924-4594-a6f4-c21c5f1f0ae7"
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authors:
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- key: "838e1722-2479-4fbd-a5fe-d965980a1a2c"
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value: "Blaise V. Jones, 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: "Brain"
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slug: "brain"
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treeNodeId: "95caa0da-bc4f-4103-8551-f58d6e415781"
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-
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name: "Metabolic, Infectious, and Inflammatory Disorders"
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slug: "metabolic-infectious-and-inflammat-"
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treeNodeId: "ed178695-cb39-40bc-981d-d0031c835a98"
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-
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name: "Demyelinating Diseases"
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slug: "demyelinating-diseases"
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treeNodeId: null
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category: "Pediatrics"
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cmeTopicId: "273337e9-da4e-4caa-8a31-95fa5c11a1b9"
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documentVersionId: "5557de59-59d3-4508-b2dd-6913988c047d"
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imageCount: 23
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lastUpdated: "11/04/21"
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pageDescription: "Demyelinating Diseases"
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pageKeywords: "Pediatrics, Diagnosis, Brain, Metabolic, Infectious, and Inflammatory Disorders, Demyelinating Diseases"
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pageTitle: "Demyelinating Diseases | STATdx"
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enhancedTitle: "Demyelinating Diseases"
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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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- "Brain"
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- "Metabolic, Infectious, and Inflammatory Disorders"
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- "Demyelinating Diseases"
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---
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# KEY FACTS
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- ## Diagnostic Checklist
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- Multiple sclerosis (MS)
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- Demyelinating disease characterized by multiple lesions disseminated in time & space
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- Brain lesions: Multiple T2- & FLAIR MR hyperintense foci, typically small (5-10 mm), ovoid, discrete, periventricular, & perpendicular to ventricular margins
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- Optic neuritis (ON): Unilateral, short segment, intraorbital; myelitis: < 2 vertebrae in length, < 50% of cross-sectional area, typically peripheral
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- Acute disseminated encephalomyelitis (ADEM)
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- Acute demyelinating disease with encephalopathy, without NMOSD or anti-MOG associated antibodies
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- Characteristically arises subsequent to infection (viral respiratory) or vaccination
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- Brain: Ill-defined, larger T2-/FLAIR hyperintense lesions
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- Neuromyelitis optica spectrum disorders (NMOSD)
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- Inflammatory CNS disease caused by antibodies to aquaporin-4 (AQP-4) on astrocytic end feet
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- ON & transverse myelitis predominate clinically
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- Brain: Commonly periventricular but parallel to ependymal lining; area postrema involvement is classic
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- ON: Bilateral, posterior predominant (including chiasm)
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- Myelitis: Longitudinally extensive transverse myelitis (LETM) (> 3 vertebrae), > 50% of cord cross section, central
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- Anti-MOG syndromes
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- Acute demyelinating disease caused by antibodies to myelin oligodendrocyte glycoprotein (MOG)
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- Extensive clinical overlap with ADEM & NMOSD; encephalopathy in younger patients, ON in older
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- Brain: Similar to ADEM
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- ON: Bilateral anterior predominant (including optic disc) with perineural enhancement
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- Myelitis: LETM vs. short segment; conus involvement
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- Lyme disease
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- 11% develop neurologic manifestations
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- May be accompanied by ON or other CN inflammation
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# TERMINOLOGY
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- ## Definitions
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- Acquired demyelinating processes characterized by inflammation
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- Multiple sclerosis (MS)
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- Demyelinating disease characterized by multiple lesions disseminated in time & space
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- Acute disseminated encephalomyelitis (ADEM)
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- Acute demyelinating disease with encephalopathy, without NMOSD or anti-MOG-associated antibodies
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- Neuromyelitis optica spectrum disorders (NMOSD)
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- Inflammatory CNS disease caused by antibodies to aquaporin-4 (AQP-4) on astrocytic end feet
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- Clinically characterized by optic neuritis & transverse myelitis
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- Antimyelin oligodendrocyte glycoprotein (MOG) syndromes
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- Acute demyelinating disease caused by antibodies to MOG
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- Extensive clinical overlap with ADEM & NMOSD
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- Lyme disease
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- CNS inflammation associated with *Borrelia burgdorferi* infection
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# IMAGING
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- ## General Features
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- MS
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- Brain: Multiple T2- & FLAIR MR hyperintense lesions, typically small (5-10 mm), ovoid, discrete
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- > 85% are periventricular: Callosal involvement, hemispheric white matter; perpendicular to ventricle margin in perivenular distribution
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- Variable enhancement: Presumed to reflect active demyelination
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- Nodular, diffuse, or ring-like
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- Can be mass-like: Tumefactive MS
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- Diffusion restriction in acute lesions
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- Diffusely abnormal ADC values
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- "Black holes" (due to axonal destruction) on T1 are much more likely to be seen in MS than ADEM
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- Optic neuritis (ON): Unilateral, short length, intraorbital
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- Myelitis: < 2 vertebral lengths, < 50% of cord cross section, cervical > thoracic
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- ADEM
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- Brain: Ill-defined larger T2-/FLAIR hyperintense lesions
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- Lesions are more likely to be diffuse & bilateral
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- Frequent brainstem & thalamic involvement
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- ON: Less common; myelitis: Less common
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- NMOSD
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- Brain: May have extensive lesions
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- Commonly periventricular but parallel
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- Dorsal brainstem (especially area postrema)
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- ON: Bilateral long segment
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- Posterior predominant, including chiasm
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- Myelitis: Longitudinally extensive transverse myelitis (LETM) (> 3 segments), typically central & > 50% circumference
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- Anti-MOG syndromes
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- Brain: Similar in appearance to ADEM
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- Much less likely to involve corpus callosum
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- ON: Bilateral long length with perineural enhancement
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- Anterior predominant, including optic disc
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- Myelitis: LETM vs. short segment; conus often affected
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- Lyme disease
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- Presents as meningoencephalitis
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- May be accompanied by ON or other cranial nerve inflammation; Bell palsy is characteristic
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- ## Imaging Recommendations
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- ### Best imaging tool
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- MR
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- ### Protocol advice
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- FLAIR MR imaging for detection
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- Postcontrast FLAIR may ↑ detection of meningeal disease
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- Fat-saturated, high-resolution postcontrast orbital MR for assessment of ON
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- Spine imaging with contrast & axial T2-weighted sequences
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# DIFFERENTIAL DIAGNOSIS
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- [Posterior Reversible Encephalopathy Syndrome](/document/acute-hypertensive-encephalopathy--/efc6f9c2-dad9-4eb8-bad2-421bfaf1ec57)
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- Subcortical vasogenic edema associated with hypertension
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- [Viral Encephalitis](/document/acute-encephalitis/a45f63bb-c25b-481d-a001-9c520c58060b)
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- Widely variable, but often affects white matter & deep gray nuclei
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- [Autoimmune-Mediated Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0)
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- Enhancing lesions spare callososeptal interface
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- Reported in COVID-19 infection
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- Beaded angiogram appearance
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- [Leukodystrophies](/document/leukodystrophies/f4ff3738-131c-46bf-be71-1811f2c1776c)
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- Patterns vary by metabolic defect
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- Metachromatic leukodystrophy, Alexander disease, X-linked adrenoleukodystrophy
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- ## Toxin-Induced Brain Injury
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- Carbon monoxide or methanol poisoning
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- Bilateral symmetric basal ganglia lesions
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- Accompanying subcortical lesions in methanol poisoning
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- MS
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- Possibly viral-incited autoimmune reaction in genetically susceptible individuals
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- No "trigger" identified
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- Activated T cells attack myelinated axons
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- B cells, antibodies, macrophages, & microglia all contribute to lesions
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- Cox-2, iNOS may cause excitotoxic death of oligodendrocytes
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- ADEM
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- Autoimmune-mediated demyelination
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- Characteristically arises subsequent to infection (viral respiratory) or vaccination
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- NMOSD
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- Antibodies to aquaporin-4 (AQP-4) channels on astrocytic end feet
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- Technically not demyelinating
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- AQP-4 is dispersed throughout CNS
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- Highly expressed in optic nerves & spinal cord
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- AQP-4 antibodies are more highly expressed in peripheral blood than CSF
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- Anti-MOG syndromes: Antibodies to MOG
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- MOG is CNS specific protein expressed on outer surface of myelin sheath
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- Lyme disease
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- Caused by spirochete *Borrelia burgdorferi*
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- Tick-borne disease; deer tick (Ixodes scapularis) or Western black-legged tick (Ixodes pacificus)
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- ## Staging, Grading, & Classification
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- Major clinical subtypes of MS
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- Relapsing-remitting**** (85% initial presentation)
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- Primary-progressive****, a.k.a. chronic progressive (5-10%)
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- Progressive from start
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- Secondary-progressive****, a.k.a. relapsing progressive
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- By 10 years 50% & by 25 years 90% of relapsing-remitting patients enter secondary-progressive phase
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- Progressive-relapsing****
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- Rare; defined as progressive disease with clear acute relapses ± full recovery
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- Periods between relapses are characterized by continuing disease progression
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- Clinically isolated syndrome (CIS): Single episode > 24 hours; vast majority progress to MS after number of years
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- MS variants/subtypes
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- Malignant****: Younger patients, febrile prodrome, clinically fulminant, death in months
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- Schilder ****type ("diffuse sclerosis"): Extensive, confluent, asymmetric demyelination in bilateral supra-/infratentorial parenchyma
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- Baló ****type ("concentric sclerosis"): Large lesions with alternating zones of demyelinated/myelinated white matter
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- ## Gross Pathologic & Surgical Features
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- Acute MS: Poorly delineated, yellowish-white, periventricular plaques
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- Chronic MS: Gray, granular, well-demarcated plaques ± generalized volume loss
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- ## Microscopic Features
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- MS
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- Perivenous demyelination & oligodendrocyte loss
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- Active: Foamy macrophages with myelin fragments, lipids; reactive astrocytes + perivascular inflammation; some are hypercellular with atypical reactive astrocytes & mitoses (mimics tumor)
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- Chronic: Marked loss of myelin & oligodendrocytes; dense astrogliosis; minimal/no perivascular inflammation
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- Axonal transection
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- CSF positive for oligoclonal bands
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- MS
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- Variable
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- Initially impaired/double vision of acute ON (50% with positive MR develop MS)
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- Weakness, numbness, tingling, gait disturbances
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- ↓ sphincter control, blindness, paralysis, dementia
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- Cranial nerve palsies; usually multiple, 1-5% isolated (CNV & VI are most common)
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- Spinal cord symptoms in 80%
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- ADEM
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- Cranial nerve palsies, encephalopathy, headache 2 days to 4 weeks after prodrome
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- Seizures in 10-35%
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- Monophasic illness
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- Can recur in small percentage of cases (controversial)
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- NMO
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- Rapid onset of vision loss
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- Subsequent spinal cord symptoms, paralysis
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- Lyme disease
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- Stereotypical expanding rash around tick bite: Erythema chronicum migrans
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- Infrequently recognized
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- Bell palsy, meningitis, arthralgias
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- ## Demographics
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- Estimated 2,500,000 have MS worldwide
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- MS most often occurs in temperate climates
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- Most common disabling CNS disease of young adults: 1 in 1,000 in Western world
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- 3-5% of MS is diagnosed before age 15 years
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- 20% of childhood MS is initially diagnosed as ADEM
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- 1/3 of acute demyelinating disease in children is anti-MOG
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- ## Natural History & Prognosis
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- MS: 45% of MS patients are not severely affected & are nearly normal
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- > 80% with "probable" MS & positive MR progress to clinically definite MS
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- ADEM: Characteristically monophasic
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- Recurrence suggests anti-MOG
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- Anti-MOG syndromes are more frequently seen in young
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- Encephalopathy is more common in younger patients, ON in older
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- > 90% of NMOSD in pediatrics have relapsing disease
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- Lyme disease: 11% develop neurologic manifestations
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- ## Treatment
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- MS & NMOSD are both treated with immune-modulating therapy
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- ADEM is typically treated with high-dose steroids
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- Alternative treatments include IVIg & plasma exchange
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- Anti-MOG syndromes respond quickly to steroid & IVIg treatment
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- Lyme is treated with antibiotics
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- No evidence that antibiotic therapy alters natural history
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# DIAGNOSTIC CHECKLIST
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- ## Image Interpretation Pearls
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- 95% with definite MS clinically have positive MR
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12c5cb99-14a6-4793-9a7d-a6f1c3511f06
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## References
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# Selected References
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1. [Chhabda S et al: Relapsing demyelinating syndromes in children: a practical review of neuroradiological mimics. Front Neurol. 11:627, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32849169%5Bpmid%5D)
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1. [Padilha IG et al: Pediatric multiple sclerosis: from clinical basis to imaging spectrum and differential diagnosis. Pediatr Radiol. 50(6):776-92, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=31925460%5Bpmid%5D)
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1. [Bulut E et al: Brain MRI findings in pediatric-onset neuromyelitis optica spectrum disorder: challenges in differentiation from acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 40(4):726-31, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30846436%5Bpmid%5D)
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1. [Galardi MM et al: Differential diagnosis of pediatric multiple sclerosis. Children (Basel). 6(6), 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31163654%5Bpmid%5D)
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1. [Lana-Peixoto MA et al: Neuromyelitis optica spectrum disorder and anti-MOG syndromes. Biomedicines. 7(2), 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31212763%5Bpmid%5D)
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1. [Troxell RM et al: Atypical pediatric demyelinating diseases of the central nervous system. Curr Neurol Neurosci Rep. 19(12):95, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31773416%5Bpmid%5D)
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1. [Reich DS et al: Multiple sclerosis. N Engl J Med. 378(2):169-80, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29320652%5Bpmid%5D)
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1. [Berzero G et al: Diagnosis and therapy of acute disseminated encephalomyelitis and its variants. Expert Rev Neurother. 16(1):83-101, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26620160%5Bpmid%5D)
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1. [Faguy K: Multiple sclerosis: an update. Radiol Technol. 87(5):529-50, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27146176%5Bpmid%5D)
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1. [Filippi M et al: MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines. Lancet Neurol. 15(3):292-303, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26822746%5Bpmid%5D)
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1. [Borchers AT et al: Lyme disease: a rigorous review of diagnostic criteria and treatment. J Autoimmun. 57:82-115, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25451629%5Bpmid%5D)
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1. [Koelman DL et al: Acute disseminated encephalomyelitis: current controversies in diagnosis and outcome. J Neurol. 262(9):2013-24, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25761377%5Bpmid%5D)
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1. [Wingerchuk DM et al: International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 85(2):177-89, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=26092914%5Bpmid%5D)
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1. [Flanagan EP et al: Neuromyelitis optica spectrum disorders. Curr Neurol Neurosci Rep. 14(9):483, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25027264%5Bpmid%5D)
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1. [Ketelslegers IA et al: A comparison of MRI criteria for diagnosing pediatric ADEM and MS. Neurology. 74(18):1412-5, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20335562%5Bpmid%5D)
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1. [VanLandingham M et al: An uncommon illness with a rare presentation: neurosurgical management of ADEM with tumefactive demyelination in children. Childs Nerv Syst. 26(5):655-61, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19949803%5Bpmid%5D)
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1. [Calabrese M et al: Cortical lesions in primary progressive multiple sclerosis: a 2-year longitudinal MR study. Neurology. 72(15):1330-6, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19365054%5Bpmid%5D)
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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. [Filippi M et al: Conventional MRI in multiple sclerosis. J Neuroimaging. 17 Suppl 1:3S-9S, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17425730%5Bpmid%5D)
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1. [Janardhan V et al: Multiple sclerosis: hyperintense lesions in the brain on nonenhanced T1-weighted MR images evidenced as areas of T1 shortening. Radiology. 244(3):823-31, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17690319%5Bpmid%5D)
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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)
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1. [Polman CH et al: Diagnostic criteria for multiple sclerosis: 2005 revisions to the "McDonald Criteria". Ann Neurol. 58(6):840-6, 2005](http://www.ncbi.nlm.nih.gov/pubmed/?term=16283615%5Bpmid%5D)
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## Images
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### Selected Images
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*Sagittal T2 MR in a 9-year-old with optic neuritis shows multiple ill-defined hyperintensities in the medulla & cervical cord. Subsequent serum testing revealed antibodies to aquaporin 4, confirming a diagnosis of neuromyelitis optica spectrum disorders (NMOSD).*
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*Sagittal T2 MR in a 9-year-old with optic neuritis shows multiple ill-defined hyperintensities in the medulla & cervical cord. Subsequent serum testing revealed antibodies to aquaporin 4, confirming a diagnosis of neuromyelitis optica spectrum disorders (NMOSD).*
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*Axial T1 C+ FS MR through the orbits shows diffuse bilateral optic nerve enhancement <img src='/img/arrows/WS.png'/> in this 9-year-old with vision loss. Clinical features were suggestive of NMOSD, but CSF analysis confirmed anti-myelin oligodendrocyte glycoprotein (MOG) disease.*
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*Axial NECT in a 16-year-old with progressive left-sided weakness after minor trauma shows a large, low-attenuation white matter lesion in the anterior right frontal lobe <img src='/img/arrows/WS.png'/> & a smaller one near the right motor strip <img src='/img/arrows/WO.png'/>.*
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*Sagittal T1 C+ MR in the same patient shows the borders of the large lesion nearest to the cortex to be nonenhancing <img src='/img/arrows/WS.png'/> as compared to the other margins <img src='/img/arrows/CS.png'/>. This open ring appearance can help distinguish tumefactive MS from abscess or neoplasm (which more typically have complete ring enhancement).*
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### Additional Images
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*Sagittal graphic illustrates MS plaques involving the corpus callosum, pons, & spinal cord. Note the characteristic perpendicular orientation of the lesions <img src='/img/arrows/BS.png'/> at the callososeptal interface along penetrating venules.*
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*Sagittal FLAIR MR shows numerous MS plaques with typical perpendicular orientation at the callososeptal interface along penetrating venules ("Dawson fingers") as well as in the subcortical white matter.*
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*Sagittal FLAIR MR shows perpendicular callosal/pericallosal MS plaques with hyperintense rims & hypointense centers (with corresponding hypointensities also demonstrated on T1 as "black holes," not shown). Note an additional posterior fossa lesion <img src='/img/arrows/WS.png'/>.*
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*Axial T1 C+ MR demonstrates multiple nodular, enhancing multiple sclerosis plaques <img src='/img/arrows/CS.png'/>. Note the common periventricular location with perpendicular orientation as well as the involvement of subcortical white matter.*
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*Axial FLAIR MR shows confluent multiple sclerosis plaques in commonly seen periventricular locations.*
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*Axial FLAIR MR in a 9-year-old patient with altered mental status & hyperreflexia shows ill-defined, hyperintense lesions in the thalami <img src='/img/arrows/WC.png'/>, basal ganglia <img src='/img/arrows/WS.png'/>, & insula <img src='/img/arrows/CS.png'/>. Involvement of the deep nuclei is a relatively common feature of acute disseminated encephalomyelitis.*
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*Axial FLAIR MR shows large lesions in the thalamus & basal ganglia <img src='/img/arrows/WS.png'/> in this 16-year-old with a headache & weakness 2 weeks after a viral illness. Acute disseminated encephalomyelitis will frequently affect deep gray matter structures.*
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*Coronal FLAIR MR in a 12-year-old patient with neuromyelitis optica & bladder dysfunction shows large lesions extending across the corpus callosum <img src='/img/arrows/CS.png'/> & left cerebral peduncle <img src='/img/arrows/WS.png'/>.*
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*Axial NECT in a 14-year-old patient with vomiting shows a nonspecific, low-attenuation lesion <img src='/img/arrows/WO.png'/> in the left posterior frontal subcortical white matter.*
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*Axial FLAIR MR in the same patient acquired the next day shows several ovoid MS plaques <img src='/img/arrows/WS.png'/>. Active lesions will also show contrast enhancement & restricted diffusion.*
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*Axial FLAIR MR in a 14-year-old with MS shows multiple ovoid lesions oriented perpendicular to the long axis of the lateral ventricles <img src='/img/arrows/WS.png'/> with hazy ↑ signal intensity in the white matter between them.*
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*Axial T2 MR in a 17-year-old with Baló concentric sclerosis <img src='/img/arrows/CS.png'/>.*
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*Sagittal T1 C+ FS MR shows an enhancing MS lesion in the dorsal aspect of the cervical cord <img src='/img/arrows/WC.png'/>. Approximately 2/3 of spinal cord MS lesions are found in the cervical cord. Typical features include a dorsal intramedullary lesion spanning < 2 vertebral segments in length.*
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*Axial FLAIR MR shows numerous peripheral white matter & cortical lesions that exhibited robust contrast enhancement (not shown) in an 18-year-old woman with malignant (Marburg) MS. The patient presented with a 2-week history of behavioral changes & leg pain & died 3 weeks after presentation. The autopsy showed typical demyelinating pathology.*
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*Axial T1 C+ FS MR in a patient with MS shows ring-enhancing masses of active demyelination. The rings of enhancement are incomplete with each ring defect pointing towards an adjacent cortex.*
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*Coronal T1 C+ MR shows a superficial hypointense mass in the left parasagittal posterior frontal region with a peripheral crescent of incomplete or "horseshoe" enhancement <img src='/img/arrows/WS.png'/>. This enhancement pattern is classic for tumefactive demyelinating disease, most commonly MS.*
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*Axial FLAIR MR shows a case of proven tumefactive MS <img src='/img/arrows/BC.png'/> with extensive surrounding white matter edema <img src='/img/arrows/WS.png'/>. Note that the imaging features present in this case could also be seen with neoplasm.*
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*Long TE MRS in a case of tumefactive MS reveals elevated choline <img src='/img/arrows/WS.png'/>, ↓ NAA <img src='/img/arrows/WO.png'/>, & a lactate doublet <img src='/img/arrows/WC.png'/>. These MRS findings could be consistent with acute demyelination & probably reflect a combination of membrane disruption, neuronal loss or dysfunction, & inflammation. Note that the MRS findings in MS are not specific. The spectral pattern of demyelination & low-grade neoplasms can be similar & should therefore be interpreted cautiously.*
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*Axial T1 C+ MR shows numerous enhancing MS plaques that were present throughout the infratentorial & supratentorial brain. MS lesions may show homogeneous enhancement but may also exhibit ring or incomplete ring patterns of enhancement.*
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Reference in New Issue
Block a user