507 lines
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Markdown
507 lines
42 KiB
Markdown
---
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title: "Multiple Sclerosis"
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docid: "7892b2a2-f52a-4d7f-9858-a326f2b7ab04"
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authors:
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- key: "a25c450b-3d34-4f64-bba3-cc0834813df6"
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value: "Miral D. Jhaveri, MD, MBA"
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name: "Diagnosis"
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slug: "diagnosis"
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treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8"
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name: "Pathology-Based Diagnoses"
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slug: "pathology-based-diagnoses"
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treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77"
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name: "Infectious, Inflammatory, and Demyelinating Disease"
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slug: "infectious-inflammatory-and-demyel-"
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treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607"
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name: "Inflammatory and Demyelinating Disease"
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slug: "inflammatory-and-demyelinating-dis-"
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treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc"
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name: "Multiple Sclerosis"
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slug: "multiple-sclerosis"
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treeNodeId: null
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category: "Brain"
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documentVersionId: "7ced4781-f9a4-4a48-a5ed-954fa6ea87cf"
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imageCount: 27
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lastUpdated: "10/08/20"
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pageDescription: "Multiple Sclerosis"
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pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, Multiple Sclerosis"
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pageTitle: "Multiple Sclerosis | STATdx"
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enhancedTitle: "Multiple Sclerosis"
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type: "DX"
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references: true
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tables: 1
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breadcrumbs:
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- "Brain"
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- "Diagnosis"
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- "Pathology-Based Diagnoses"
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- "Infectious, Inflammatory, and Demyelinating Disease"
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- "Inflammatory and Demyelinating Disease"
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- "Multiple Sclerosis"
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---
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# KEY FACTS
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- ## Terminology
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- Idiopathic chronic inflammatory demyelinating disease of CNS
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- ## Imaging
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- Multiple perpendicular callososeptal T2 hyperintensities characteristic of multiple sclerosis (MS)
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- Perivenular extension: "Dawson fingers"
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- Bilateral, asymmetric linear/ovoid FLAIR hyperintensities
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- Periventricular/perivenular, callososeptal interface
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- May also commonly involve brachium pontis, brainstem, spinal cord, cortex/juxtacortical
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- SWI central vein sign
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- Transient enhancement during active demyelination
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- Rare: Large tumefactive enhancing rings
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- Advanced imaging techniques show disease in normal-appearing white matter
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- ## Top Differential Diagnoses
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- **Multifocal T2/FLAIR hyperintensities**
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- Arteriolosclerosis, ADEM, NMOSD, vasculitis, Susac syndrome, Lyme disease
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- **Mass-like ("tumefactive") lesion(s)**
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- Neoplasms, PML/PML-IRIS
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- ## Pathology
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- Major clinical subtypes from least to most severe
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- Radiologically isolated syndrome, clinically isolated syndrome, relapsing-remitting, relapsing-progressive, a.k.a. secondary progressive & primary progressive MS
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- ## Clinical Issues
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- ~ 2.5 million people in world have MS
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- ## Diagnostic Checklist
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- Requires dissemination in time & space in CNS for diagnosis
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- McDonald criteria: Consensus statement for diagnostic criteria, last revised in 2017
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# TERMINOLOGY
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- ## Abbreviations
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- Multiple sclerosis (MS)
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- ## Definitions
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- Idiopathic chronic inflammatory demyelinating disease of CNS
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Multiple perpendicular callososeptal T2 hyperintensities
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- ### Location
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- > 85% periventricular/perivenular, callososeptal interface
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- Brachium pontis, brainstem, spinal cord
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- Infratentorial (< 10% in adults, more common in children)
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- Gray matter [cortex & basal ganglia (BG) ~ 10%]
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- Juxtacortical: Lesion in cerebral white matter (WM) abutting cortex
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- Cortical: Lesion within cerebral cortex
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- Leukocortical (inner aspect ± involvement of juxtacortical WM)
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- Intracortical (purely within cortex)
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- Subpial (at its outer aspect)
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- ### Size
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- Small (5-10 mm); tumefactive lesions several cm
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- ### Morphology
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- Linear, round, or ovoid; beveled, target
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- ## CT Findings
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- NECT often normal early in disease course
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- Solitary/multiple ill-defined WM hypodensities
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- Mild/moderate punctate, patchy, ring enhancement in acute/subacute lesions
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- ## MR Findings
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- ### T1WI
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- Typically hypo- or isointense
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- Hypointensity correlates with axonal destruction ("black holes"), faint mildly hyperintense rim
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- Chronic/severe: Volume loss, thin corpus callosum
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- ### T2WI
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- Hyperintense, linear foci radiating from ventricles
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- ### FLAIR
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- Early: Alternating linear hyperintensity along ependyma on sagittal FLAIR: Ependymal dot-dash sign
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- Bilateral, asymmetric, linear/ovoid hyperintensities
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- Perivenular extension; "Dawson fingers"
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- Hyperintensities become confluent with severity
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- Cortical lesions may precede classic WM lesions
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- ### T2* GRE
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- SWI central vein sign (CVS) = vein located centrally within MS lesion
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- Magnetic resonance imaging in multiple sclerosis (MAGNIMS) study
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- CVS sensitivity of 68.1% & specificity of 82.9% in distinguishing MS from radiologic mimics using 35% CVS proportion threshold
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- Chronic lesions: Paramagnetic rims at lesion edge
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- Iron accumulation in deep gray matter structures
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- SWI C+ to better detect BBB dysfunction in MS plaques
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- Natalizumab-associated progressive multifocal leukoencephalopathy (PML): Juxtacortical low signal
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- ### DWI
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- Majority of acute plaques: Normal or ↑ diffusivity
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- May show restricted diffusion at plaque margins
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- Subacute/chronic plaques show ↑ diffusivity
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- DTI: Reduced longitudinal diffusivity due to axonal injury
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- ### T1WI C+
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- Transient enhancement during active demyelination
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- Punctate, nodular, linear, incomplete ring
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- Semilunar, "open" nonenhancing segment facing cortex
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- ± leptomeningeal enhancement on delayed FLAIR MR, marker for cortical demyelination
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- ### MRS
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- ↓ NAA , ↑ choline (Cho/Cr), ↑ myoinositol
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- Abnormalities found in normal-appearing WM (NAWM)
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- Secondary progressive MS may show ↓ NAA in normal-appearing gray matter (NAGM)
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- May allow early distinction between relapsing-remitting & secondary progressive
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- 3D double inversion recovery (DIR) sequence
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- ↑ intracortical lesion detection
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- Perfusion MR: Low rCBV, can help differentiate tumefactive MS from neoplasm
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- Magnetization transfer (MT)
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- ↓ MT ratio (MTR) in lesions/NAWM
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- Functional connectivity MR (fcMR)
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- ↓ functional connectivity between right/left hemisphere primary visual & motor cortices
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- 3T vs. 1.5T: ↑ number of contrast-enhancing lesions, enhancing lesion volume, total lesion volume
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- 7T: Improved detection of cortical lesions
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- ## PET With Translocator Protein Radioligands
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- In vivo quantification of microglial activation
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- Presence of widespread inflammation in NAWM
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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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- Contrast-enhanced MR with sagittal FLAIR
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- Fat saturation to assess for optic neuritis
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- **Consortium of Multiple Sclerosis Centers (CMSC) 2018 guidelines**
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- Brain MR C+ recommended for diagnosis of MS
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- Spinal cord MR if brain MR nondiagnostic or presenting symptoms referable to cord
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- Follow-up brain MR recommended
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- Demonstrate dissemination in time
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- Detect clinically silent disease activity on Rx
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- **PML**surveillance while on Rx
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- Evaluate unexpected clinical worsening
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- New baseline MR before starting/modifying Rx
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- Every 6 months to 2 years for relapsing MS
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- MR brain core sequences
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- 2D/3D sagittal & axial FLAIR, 2D/3D axial T2, 2D axial DWI, 3D IR-prep GE T1 ± 2D/3D axial T1 C+
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# DIFFERENTIAL DIAGNOSIS
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- ## Multifocal T2/FLAIR Hyperintensities
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- [Arter io lo sc lerosis](/document/arteriolosclerosis/ce5a75ed-3a88-42a4-a0e8-4f339375c062)
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- Patchy confluent & focal lesions; subcortical/deep WM & BG involved; ± cortical infarcts, vascular risk factors
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- [Acute disseminated encephalomyelitis](/document/adem/a3fafeb7-5861-4364-beb8-c0e30220564e)
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- Viral prodrome, monophasic, more common in children
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- Can mimic MS; gray matter often involved
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- Lesions tend to be larger & often symmetric
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- [Neuromyelitis optica spectrum disorders](/document/neuromyelitis-optica-spectrum-diso-/54d4a8bc-9267-4df6-98c1-f22aae051d01)
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- Optic neuritis & spinal cord lesions
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- Tend to border midline CSF spaces
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- [Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0)
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- Enhancing lesions spare callososeptal interface
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- Beaded angiogram appearance
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- [Susac syndrome](/document/susac-syndrome/af240d31-5918-4981-971f-c6780f7b405f)
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- Classic triad: Encephalopathy, branch retinal artery occlusions, hearing loss
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- [Lyme Disease](/document/lyme-disease/ca3da7a8-0f21-4e06-9961-a43e6af1b0cc)
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- Can be identical to MS (skin rash common)
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- ## Mass-Like ("Tumefactive") Lesion(s)
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- **Neoplasms**:****Glioblastoma, metastasis
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- [PML/PML-IRIS : HIV/AIDS, Natalizumab-treated MS](/document/progressive-multifocal-leukoenceph-/fc94bcd6-17a1-4966-8e27-51f5c7df6537)
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Multifactorial disease, precise pathogenesis unknown
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- Autoimmune, environmental, & genetic factors
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- ### Genetics
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- Multifactorial; ↑ incidence in 1st-order relatives
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- ## Staging, Grading, & Classification
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- **Major clinical subtypes from least to most severe**
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- **Radiologically isolated syndrome****(RIS)**
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- Asymptomatic with routine MR findings highly suggestive of MS
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- **Clinically isolated syndrome (CIS)**
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- Single episode of neurologic symptoms suggestive of MS, chance of developing MS → CIS(+) MR(-) (20%) & if CIS(+) MR(+) (60-80%)
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- **Relapsing-remitting** **(RR-MS)**85% initial presentation
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- Relapses alternating with remission phases
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- **Relapsing-progressive** **(RP-MS)**
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- Secondary progressive MS, progressive worsening of neurologic function over time
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- **Primary progressive (PP-MS)**
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- 5-10% of patients progressive from start
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- **MS variants/subtypes**
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- **Malignant/Marburg disease**: 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 WM
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- ## Gross Pathologic & Surgical Features
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- Acute: Poorly delineated, yellowish-white, periventricular plaques
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- Chronic: Gray, granular, well-demarcated plaques ± generalized volume loss
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- ## Microscopic Features
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- Relatively sharp borders, macrophage infiltration (interstitial & perivascular), perivascular inflammation
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- Acute: Foamy macrophages, perivascular T-cell lymphocytic cuffing, microglial activation
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- Chronic: Marked loss of myelin, oligodendrocytes; dense astrogliosis; minimal/no perivascular inflammation
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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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- Weakness, numbness, tingling, gait disturbances, impaired vision, diplopia
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- ↓ sphincter control, paralysis, dementia
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- Cranial nerve palsy; usually multiple
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- Spinal cord symptoms
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- ## Demographics
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- ### Age
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- 20-40 years; median: 30 years
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- ### Sex
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- Adults: M:F = 1:1.77; adolescents: M:F = 1:3-5
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- ### Ethnicity
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- All groups but most common in white patients
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- ### Epidemiology
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- ~ 2.5 million people worldwide
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- Most often occurs in temperate zones
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- ## Natural History & Prognosis
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- 1/3 have initial episode followed by near-normal function
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- Majority: Protracted course with progression of deficits
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- Late: Severe disability, cognitive impairment
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- ## Treatment
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- Immunomodulators &/or immunosuppressants
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# DIAGNOSTIC CHECKLIST
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- ## Image Interpretation Pearls
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- 95% with clinically definite MS have positive MR
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b58b4061-1b92-4b32-abf6-1debb49d180c
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## References
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# Selected References
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1. [Gaitán MI et al: SWAN-Venule: an optimized MRI technique to detect the central vein sign in MS plaques. AJNR Am J Neuroradiol. 41(3):456-60, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32054616%5Bpmid%5D)
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1. [Sastre-Garriga J et al: MAGNIMS consensus recommendations on the use of brain and spinal cord atrophy measures in clinical practice. Nat Rev Neurol. 16(3):171-82, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32094485%5Bpmid%5D)
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1. [do Amaral LLF et al: Gadolinium-enhanced susceptibility-weighted imaging in multiple sclerosis: optimizing the recognition of active plaques for different mr imaging sequences. AJNR Am J Neuroradiol. 40(4):614-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30846435%5Bpmid%5D)
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1. [Filippi M et al: Association between pathological and MRI findings in multiple sclerosis. Lancet Neurol. 18(2):198-210, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30663609%5Bpmid%5D)
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1. [Hartung HP et al: Diagnosis of multiple sclerosis: revisions of the McDonald criteria 2017 - continuity and change. Curr Opin Neurol. 32(3):327-37, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30985371%5Bpmid%5D)
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1. [Maranzano J et al: Comparison of multiple sclerosis cortical lesion types detected by multicontrast 3T and 7T MRI. AJNR Am J Neuroradiol. 40(7):1162-9, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31221631%5Bpmid%5D)
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1. [Suthiphosuwan S et al: The central vein sign in radiologically isolated syndrome. AJNR Am J Neuroradiol. 40(5):776-83, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31000526%5Bpmid%5D)
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1. [Zipp F et al: Implementing the 2017 McDonald criteria for the diagnosis of multiple sclerosis. Nat Rev Neurol. 15(8):441-5, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31086264%5Bpmid%5D)
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1. [Filippi M et al: MRI in multiple sclerosis: what is changing? Curr Opin Neurol. 31(4):386-95, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29952834%5Bpmid%5D)
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1. [Inglese M et al: MRI in multiple sclerosis: clinical and research update. Curr Opin Neurol. 31(3):249-55, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29561520%5Bpmid%5D)
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1. [Thompson AJ et al: Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol. 17(2):162-73, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29275977%5Bpmid%5D)
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1. [Castellaro M et al: Heterogeneity of cortical lesion susceptibility mapping in multiple sclerosis. AJNR Am J Neuroradiol. 38(6):1087-95, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28408633%5Bpmid%5D)
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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. [Roosendaal SD et al: Imaging phenotypes in multiple sclerosis. Neuroimaging Clin N Am. 25(1):83-96, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25476514%5Bpmid%5D)
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1. [Aliaga ES et al: MRI mimics of multiple sclerosis. Handb Clin Neurol. 122:291-316, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24507523%5Bpmid%5D)
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1. [Ciccarelli O et al: Pathogenesis of multiple sclerosis: insights from molecular and metabolic imaging. Lancet Neurol. 13(8):807-22, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25008549%5Bpmid%5D)
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1. [Fernandez O et al: Biomarkers in multiple sclerosis: an update for 2014. Rev Neurol. 58(12):553-70, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24915032%5Bpmid%5D)
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1. [Hardy TA et al: Baló's concentric sclerosis. Lancet Neurol. 13(7):740-6, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24943346%5Bpmid%5D)
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1. [Miller TR et al: Advances in multiple sclerosis and its variants: conventional and newer imaging techniques. Radiol Clin North Am. 52(2):321-36, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24582342%5Bpmid%5D)
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1. [Steenwijk MD et al: What explains gray matter atrophy in long-standing multiple sclerosis? Radiology. 272(3):832-42, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24761837%5Bpmid%5D)
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1. [Klawiter EC: Current and new directions in MRI in multiple sclerosis. Continuum (Minneap Minn). 19(4 Multiple Sclerosis):1058-73, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23917101%5Bpmid%5D)
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1. [Polman CH et al: Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol. 69(2):292-302, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21387374%5Bpmid%5D)
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## Tables
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# 2017 McDonald Criteria for Diagnosis of Multiple Sclerosis
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| Clinical Presentation | Additional Criteria to Make Multiple Sclerosis Diagnosis |
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| --- | --- |
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| ≥ 2 attacks & clinical evidence of ≥ 2 lesions; or ≥ 2 attacks & clinical evidence of 1 lesion with historical evidence of prior attack involving lesion in different location | None ; DIS and DIT have been met |
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| ≥ 2 attacks and clinical evidence of 1 lesion | DIS shown by 1 of these criteria - Additional clinical attack implicating different CNS site - ≥ 1 MS-typical T2 lesions in ≥ 2 areas of CNS: Periventricular, cortical, juxtacortical, infratentorial, or spinal cord |
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| 1 attack and clinical evidence of ≥ 2 lesions | DIT shown by 1 of these criteria - Additional clinical attack - Simultaneous presence of enhancing & nonenhancing MS-typical MR lesions or new T2/enhancing MR lesion compared to baseline - CSF oligoclonal bands |
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| 1 attack and clinical evidence of 1 lesion | DIS + DIT criteria - CSF oligoclonal bands |
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## Images
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### Selected Images
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*Sagittal graphic illustrates multiple sclerosis (MS) plaques involving the corpus callosum, pons, and 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 demonstrates numerous well-defined and ill-defined callososeptal hyperintensities <img src='img/arrows/CC.png'/> radiating from the lateral ventricular margin with a typical perpendicular orientation, characteristic of MS.*
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*Sagittal FLAIR demonstrates numerous well-defined and ill-defined callososeptal hyperintensities <img src='img/arrows/CC.png'/> radiating from the lateral ventricular margin with a typical perpendicular orientation, characteristic of MS.*
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![Axial FLAIR MR shows subcortical <img src='img/arrows/CC.png'/> and cortical <img src='img/arrows/CS.png'/> 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)
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*Axial FLAIR MR shows subcortical <img src='img/arrows/CC.png'/> and cortical <img src='img/arrows/CS.png'/> 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).*
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![Axial FLAIR MR shows subcortical <img src='img/arrows/CC.png'/> and cortical <img src='img/arrows/CS.png'/> 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)
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*Axial FLAIR MR shows subcortical <img src='img/arrows/CC.png'/> and cortical <img src='img/arrows/CS.png'/> 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).*
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*Axial T1 C+ MR in a patient with MS demonstrate multiple enhancing plaques due to active demyelination. Punctate <img src='img/arrows/CS.png'/>, nodular <img src='img/arrows/CC.png'/>, and rim patterns <img src='img/arrows/CO.png'/> are seen.*
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*Axial T1 C+ MR in a patient with MS demonstrate multiple enhancing plaques due to active demyelination. Punctate <img src='img/arrows/CS.png'/>, nodular <img src='img/arrows/CC.png'/>, and rim patterns <img src='img/arrows/CO.png'/> are seen.*
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*Sagittal T1 MR in a patient with longstanding MS shows ovoid lesions in the periventricular WM with ill-defined hyperintense rims <img src='img/arrows/CC.png'/> surrounding the plaques, giving the distinct lesion within a lesion appearance.*
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*Sagittal T1 MR in a patient with longstanding MS shows ovoid lesions in the periventricular WM with ill-defined hyperintense rims <img src='img/arrows/CC.png'/> surrounding the plaques, giving the distinct lesion within a lesion appearance.*
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*Axial SWI (R) demonstrates characteristic perivenular location of a demyelinating plaque <img src='img/arrows/CC.png'/> with the medullary vein <img src='img/arrows/CS.png'/> coursing through it. Axial SWI (L) in the same patient shows findings related to Natalizumab-associated PML <img src='img/arrows/WC.png'/> with juxtacortical hypointense rim <img src='img/arrows/CO.png'/>.*
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*Axial SWI (R) demonstrates characteristic perivenular location of a demyelinating plaque <img src='img/arrows/CC.png'/> with the medullary vein <img src='img/arrows/CS.png'/> coursing through it. Axial SWI (L) in the same patient shows findings related to Natalizumab-associated PML <img src='img/arrows/WC.png'/> with juxtacortical hypointense rim <img src='img/arrows/CO.png'/>.*
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*Sagittal T1WI C+ MR shows a large hypointense mass <img src='img/arrows/CC.png'/> with a peripheral crescent of incomplete or "open ring" enhancement <img src='img/arrows/CS.png'/>. This enhancement pattern is classic for a tumefactive demyelinating disease, most commonly MS.*
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*Sagittal T1WI C+ MR shows a large hypointense mass <img src='img/arrows/CC.png'/> with a peripheral crescent of incomplete or "open ring" enhancement <img src='img/arrows/CS.png'/>. This enhancement pattern is classic for a tumefactive demyelinating disease, most commonly MS.*
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*MRS at 144 TE in the same patient demonstrates a large choline peak <img src='img/arrows/CC.png'/> with ↓ in NAA <img src='img/arrows/CS.png'/>. MRS in a tumefactive demyelinating lesion is not specific and can mimic a tumor profile. MR DSC perfusion (insert) shows marked ↓ rCBV <img src='img/arrows/WO.png'/>, which goes more in favor of a demyelinating lesion.*
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*MRS at 144 TE in the same patient demonstrates a large choline peak <img src='img/arrows/CC.png'/> with ↓ in NAA <img src='img/arrows/CS.png'/>. MRS in a tumefactive demyelinating lesion is not specific and can mimic a tumor profile. MR DSC perfusion (insert) shows marked ↓ rCBV <img src='img/arrows/WO.png'/>, which goes more in favor of a demyelinating lesion.*
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*Axial T1 C+ MR demonstrates concentric laminated "onion bulb" enhancement <img src='img/arrows/CC.png'/>, characteristic of acute Baló concentric sclerosis. Baló concentric sclerosis is a rare aggressive MS variant characterized by acute onset and rapid deterioration.*
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*Axial T1 C+ MR demonstrates concentric laminated "onion bulb" enhancement <img src='img/arrows/CC.png'/>, characteristic of acute Baló concentric sclerosis. Baló concentric sclerosis is a rare aggressive MS variant characterized by acute onset and rapid deterioration.*
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*Axial T1 C+ MR in a young male with rapid onset of visual disturbance demonstrates large enhancing demyelinating lesions <img src='img/arrows/CS.png'/> in the deep and periventricular WM. Marburg disease is an acute fulminant MS variant.*
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*Axial T1 C+ MR in a young male with rapid onset of visual disturbance demonstrates large enhancing demyelinating lesions <img src='img/arrows/CS.png'/> in the deep and periventricular WM. Marburg disease is an acute fulminant MS variant.*
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### Additional Images
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*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.*
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*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 <img src='img/arrows/WS.png'/>.*
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*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.*
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*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.*
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*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.)*
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*Sagittal FLAIR shows callososeptal hyperintensities radiating from the lateral ventricles with a typical perpendicular orientation, characteristic of MS.*
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*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.*
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*Axial FLAIR MR shows confluent periventricular WM hyperintensity typical of advanced, longstanding MS with loss of discrete, linear, periventricular lesions.*
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*Sagittal T1WI MR shows multiple hypointense lesions ("black holes") in the deep WM <img src='img/arrows/BS.png'/> related to axonal destruction. Note the associated moderate ventricular and sulcal enlargement.*
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*Coronal T1WI C+ MR shows a hypointense mass in the left 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 T1WI C+ FS shows bright enhancement of the optic nerves <img src='img/arrows/WS.png'/>, similar to the extraocular muscles, in a patient with MS and acute bilateral optic neuritis.*
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*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.*
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*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.*
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*Sagittal FLAIR shows callososeptal hyperintensities <img src='img/arrows/CC.png'/> radiating from the lateral ventricles with a typical perpendicular orientation, characteristic of MS.*
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*Axial FLAIR in a 35-year-old woman with MS shows extensive confluent periventricular hyperintense lesions <img src='img/arrows/CS.png'/>, typical of advanced, longstanding MS with loss of discrete, linear, periventricular lesions. Note prominence of the ventricles and cortical sulci due to diffuse atrophy.*
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*Sagittal T1WI MR shows multiple hypointense lesions ("black holes") in the periventricular WM <img src='img/arrows/CS.png'/> 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.*
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*Axial SWI demonstrates characteristic perivenular location of a demyelinating plaque <img src='img/arrows/CC.png'/> with the medullary vein <img src='img/arrows/CS.png'/> coursing through it.*
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