539 lines
44 KiB
Markdown
539 lines
44 KiB
Markdown
---
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title: "Multiple Sclerosis"
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docid: "89599954-599e-4410-a517-eb22125cedfb"
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authors:
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value: "Jeffrey S. Ross, MD"
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name: "Inflammatory and Autoimmune Disorders"
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name: "Multiple Sclerosis"
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lastUpdated: "02/12/25"
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pageDescription: "Multiple Sclerosis"
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pageKeywords: "Spine, Diagnosis, Infection and Inflammatory Disorders, Inflammatory and Autoimmune Disorders, Multiple Sclerosis"
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anatomy:
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- "Inflammatory and Autoimmune Disorders"
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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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- Primary demyelinating disease of CNS with multiple lesions disseminated over time & space
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- Concomitant intracranial lesions in periventricular, subcallosal, brainstem, or cerebellar white matter
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- ## Imaging
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- Isolated spinal cord disease (10-20%)
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- Cervical segment is most commonly affected
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- Dorsolateral aspect of cord
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- < 1/2 of cross-sectional area of spinal cord
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- < 2 vertebral segments in length
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- Sagittal & axial T1WI/T2WI sequences with gadolinium
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- Lesions typically oval, peripheral, & asymmetric
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- Discrete vs. vague hyperintense lesions
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- Enhancement lasts 1-2 months but does not reflect disease progression
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- ## Top Differential Diagnoses
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- Intramedullary neoplasm
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- Idiopathic transverse myelitis
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- Neuromyelitis optica
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- ## Pathology
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- Autoimmune, cell-mediated inflammatory process focused on CNS myelin
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- ## Clinical Issues
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- Peak onset: 20-40 years
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- Adult females more susceptible than males (1.7:1)
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- Relapsing remitting (RR)
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- Secondary progressive (SP)
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- Primary progressive (PP)
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- Progressive relapsing (PR)
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- ## Diagnostic Checklist
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- Imaging findings must be correlated with clinical & laboratory features to confirm diagnosis
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# TERMINOLOGY
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- ## Abbreviations
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- Spinal cord multiple sclerosis (MS)
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- ## Definitions
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- Primary demyelinating disease of CNS with multiple lesions disseminated over time & space
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Concomitant T2 lesions in ≥ 2 of 4 areas of CNS: Periventricular, cortical or juxtacortical, infratentorial, & spinal cord
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- ### Location
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- Isolated spinal cord disease in 10-20%
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- Cervical segment is most commonly affected
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- 2/3 of cord lesions
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- Dorsolateral aspect of cord
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- Does not respect gray-white boundary
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- ### Size
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- < 1/2 of cross-sectional area of spinal cord
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- < 2 vertebral segments in length
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- ### Morphology
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- Wedge-shaped on axial MR
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- Apex directed centrally
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- ## MR Findings
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- ### T1WI
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- Iso- to hypointense lesions
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- In cord (unlike brain), rarely visible as hypointense
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- 30% of brain lesions are dark, "black holes"
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- ### T2WI
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- Discrete or ill-defined hyperintense lesions
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- May be related to extent of demyelination
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- Lesions ↑ in size due to edema associated with inflammatory infiltrates → reach max size at 4 weeks
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- Slow ↓ in size over 6-8 weeks as edema resolves ± remyelination
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- Lesions typically oval, peripheral, & asymmetric
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- ### PD/intermediate
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- Hyperintense lesions
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- ### STIR
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- Improved lesion detection
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- ### FLAIR
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- Lower sensitivity compared to STIR in cord imaging
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- ### DWI
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- ↑ mean diffusivity, ↓ fractional anisotropy in plaques & areas without T2 abnormality
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- ### T1WI C+
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- Variable
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- Homogeneous, nodular, or ring enhancement during acute or subacute phase
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- Enhancement lasts 1-2 months
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- Does not reflect disease progression
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- No enhancement during chronic phase
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- ### MRS
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- ↓ N-acetylaspartate level
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- ↑ choline levels, even in normal-appearing white matter
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- Solitary or multifocal lesions
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- Larger lesions formed by coalescence of smaller plaques
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- Normal or mild focal cord expansion
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- Cord edema
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- Resolves after 6-8 weeks
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- Cord atrophy
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- Usually in late stage
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- May be seen in early disease course
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- Useful for monitoring disease progression & therapeutic efficacy
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- Correlates with clinical disability
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- fMRI
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- Tactile-associated cervical cord fMRI activity ↑ in relapse-onset MS patients
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- Overactivation more prominent in patients with more severe locomotor disability
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- Suggests abnormality of cord functional properties may be among factors associated with clinical status of MS patient
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- ## Nonvascular Interventions
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- ### Myelography
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- Nonspecific mild cord expansion
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- ## Other Modality Findings
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- Magnetization transfer (MT) imaging
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- ↓ MT ratio in spinal cord
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- Better correlation with disability & axonal loss
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- ↓ MT ratios in enhancement patterns in which myelin known to be ↓ histopathologically
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- ## Imaging Recommendations
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- ### Best imaging tool
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- T1WI/T2WI spinal cord MR in sagittal & axial planes with gadolinium
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# DIFFERENTIAL DIAGNOSIS
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- [Intramedullary Neoplasm](/document/spinal-cord-astrocytoma/43d5efa2-7a6d-4972-bfc6-c300cc31f9af)
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- Cord expansion with holocord involvement
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- Peritumoral edema
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- Diffuse or partial enhancement
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- Cystic ± hemorrhagic components
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- [Spinal Cord Infarction](/document/spinal-cord-infarction/5afcaea7-09a0-49b7-8f23-f93734d627fb)
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- Sudden onset of symptoms
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- Posterior columns typically spared in anterior spinal infarct
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- [Idiopathic Transverse Myelitis](/document/idiopathic-acute-transverse-myelit-/6e82fa47-19b7-45ac-b195-3c21687fa648)
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- Lesion centrally located
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- 3-4 segments in length
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- Involving > 2/3 of cord cross-sectional area
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- Variable enhancement
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- No associated intracranial lesions
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- Diagnosis of exclusion
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- [Syringohydromyelia](/document/syringomyelia/fb362df4-5033-4f7c-9f4d-01d701ebab84)
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- Central cystic lesion
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- CSF intensity on all sequences
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- No abnormal enhancement
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- [Neuromyelitis Optica Spectrum Disorder (NMOSD)](/document/neuromyelitis-optica/11d42d8d-e7bb-4ecf-85ce-d96f0afeb076)
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- Autoimmune inflammatory disorder involving myelin of neurons of optic nerves & spinal cord
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- Limited brain involvement
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- Longitudinally extensive transverse myelitis (LETM) (> 3 vertebral segments), T2 hyperintensity within cord + enhancement of optic nerves
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- T2 abnormality involves entire cross section of cord
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- ## Myelin Oligodendrocyte Glycoprotein Autoantibody Myelitis (MOGAD)
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- LETM similar to AQP4 (+) NMOSD but more often involving conus
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- H pattern of cord involvement
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- Children often present with ADEM phenotype
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- ## Parainfectious myelitis (Viral)
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- Long segment of T2 signal abnormality
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- Usually prominent irregular enhancement
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Autoimmune, cell-mediated inflammatory process focused on CNS myelin
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- Infectious agents may play primary or secondary role
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- Humoral mechanism: Cross reactivity between infectious & self-epitopes
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- ### Genetics
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- MS may be inherited as complex multifactorial disorder resulting from interaction of genetic & environmental factors
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- Estimated risk to siblings of proband ~ 3.0-5.0%, ↑ to 29.5% if 1 or both parents have MS
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- Risk to offspring of person with MS is 2.0-3.0% & higher if both parents have MS
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- ### Associated abnormalities
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- 90% incidence of associated intracranial lesions
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- Different serum thyroid hormone & complement C3, C4, & CH50 levels in neuromyelitis optica vs. MS
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- Thyroid hormones may play different role in modulating complement activation in MS & neuromyelitis optica
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- Focal regions of demyelination of varying size & age scattered throughout CNS white matter
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- ## Staging, Grading, & Classification
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- Dissemination in space (DIS)
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- Demonstrated by ≥ 1 T2-hyperintense lesions that are characteristic of MS in ≥ 2 of 4 areas of CNS
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- Periventricular, cortical or juxtacortical, infratentorial brain regions, & spinal cord
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- Dissemination in time (DIT)
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- Simultaneous presence of gadolinium-enhancing & nonenhancing lesions at any time
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- New T2-hyperintense or gadolinium-enhancing lesion on follow-up MR, with reference to baseline scan, irrespective of timing of baseline MR
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- ## Microscopic Features
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- Discrete lesions of myelin destruction
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- Active lesions with macrophages & lymphocytes
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- Chronic lesions with gliosis & cavitation
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- Perivascular cuffs of lymphocytes & mononuclear cells
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- Involvement of dorsal horns common
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Cord lesions may be asymptomatic
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- Paresthesia
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- ### Other signs/symptoms
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- Muscle weakness, hyperreflexia, gait disturbance
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- Bladder/bowel dysfunction
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- Surveillance includes periodic neurologic examination to track disease progression & periodic brain & spinal cord MRs to monitor disease activity
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- Additional examination techniques → ambulation index, 25-foot timed walk, & 25-foot walk combined with 9-hole peg test & paced serial auditory addition test
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- ## Demographics
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- ### Age
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- Peak onset: 20-40 years
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- Onset: < 18 years in 3-5% of MS cases
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- ### Sex
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- Women more susceptible than men (1.7:1)
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- Men more likely to have progressive relapsing (PR) & secondary progressive (SP) MS
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- Women more likely to have relapsing remitting (RR) MS
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- Both sexes equally affected in primary progressive (PP) MS
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- ### Ethnicity
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- Western Europeans have higher risk
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- ### Epidemiology
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- Increasing prevalence further north from equator
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- 30-80 per 100,000 in northern USA & Europe
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- 6-14 per 100,000 in southern USA & Europe
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- 1 per 100,000 in equatorial regions
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- ## Natural History & Prognosis
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- Benign (20%)
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- Complete recovery after 1-2 attacks
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- Some may experience progressive MS after 10-15 years
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- RR (25%)
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- Distinct periods of new or worsening symptoms alternating with complete or partial recovery
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- 90% will evolve into progressive MS after 25 years
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- SP (40%)
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- From RR MS
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- Worsening deficits & disabilities
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- Incomplete & infrequent remission
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- PP (12%)
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- Steady progression of symptoms
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- Motor dysfunction common
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- Primary cord involvement; no distinct attacks
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- PR (3%)
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- Similar to PP MS
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- Distinct periods of exacerbation but without recovery
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- High mortality rate
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- ## Treatment
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- Traditional disease-modifying therapies (DMTs)
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- Interferon β (several types, such as interferon β-1b, interferon β-1a)
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- Enhancing suppressor T-cell activity & reducing proinflammatory cytokine production
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- Glatiramer acetate
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- Synthetic protein similar to myelin protein
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- Shifts immune response from proinflammatory to antiinflammatory state
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- S1P receptor modulators
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- Fingolimod, siponimod, ozanimod
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- Small-molecule oral agents, which prevent lymphocytes from leaving lymph nodes & decreasing number in peripheral blood that may cross to CNS
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- Fumarates
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- Dimethyl fumarate, diroximel fumarate, monomethyl fumarate
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- Increases antioxidant activity
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- Teriflunomide
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- Inhibits proliferation of rapidly dividing T & B cells & leaving resting lymphocytes unaltered
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- Early aggressive treatment strategy
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- Natalizumab
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- Blocks α-4 integrin & ability of leukocytes to cross blood-brain barrier
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- Increased risk of progressive multifocal leukoencephalopathy (PML)
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- Anti-CD20 monoclonal antibodies
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- Rituximab, ocrelizumab, ublituximab, ofatumumab
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- Selectively deplete CD20-expressing B cells
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- Alemtuzumab
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- Binds to surface antigen CD52, which is expressed on T & B lymphocytes resulting in antibody-dependent cellular cytolysis
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- Cladribine
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- Interferes with DNA synthesis & repair of T & B lymphocytes resulting in lymphocyte depletion
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- Physical therapy
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# DIAGNOSTIC CHECKLIST
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- ## Consider
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- Brain MR, including high-resolution fast spin-echo T2 through corpus callosum
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- Periventricular, subcallosal, brainstem, or cerebellar white matter lesions suggest MS
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- Gray matter atrophy correlates with disability
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- ## Image Interpretation Pearls
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- Imaging findings must be correlated with clinical & laboratory features to confirm diagnosis
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- Acute MS can mimic cord neoplasm
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23e58f52-8b8e-4c96-9a03-de07a43975cd
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## References
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# Selected References
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1. [Hernandez J: Multiple sclerosis treatment review for primary care providers. Nurse Pract. 49(7):38-47, 2024](http://www.ncbi.nlm.nih.gov/pubmed/?term=38915149%5Bpmid%5D)
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1. [Solomon AJ et al: Differential diagnosis of suspected multiple sclerosis: an updated consensus approach. Lancet Neurol. 22(8):750-68, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37479377%5Bpmid%5D)
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1. [Ciccarelli O et al: Spinal cord involvement in multiple sclerosis and neuromyelitis optica spectrum disorders. Lancet Neurol. 18(2):185-97, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30663608%5Bpmid%5D)
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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. [Bigi S et al: Outcomes after early administration of plasma exchange in pediatric central nervous system inflammatory demyelination. J Child Neurol. 30(7):874-80, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25246301%5Bpmid%5D)
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1. [Kearney H et al: Spinal cord grey matter abnormalities are associated with secondary progression and physical disability in multiple sclerosis. J Neurol Neurosurg Psychiatry. 86(6):608-14, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25097217%5Bpmid%5D)
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1. [Riederer I et al: Double inversion recovery sequence of the cervical spinal cord in multiple sclerosis and related inflammatory diseases. AJNR Am J Neuroradiol. 36(1):219-25, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25169924%5Bpmid%5D)
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1. [Russi AE et al: The meninges: new therapeutic targets for multiple sclerosis. Transl Res. 165(2):255-69, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25241937%5Bpmid%5D)
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1. [De Stefano N et al: Spinal cord imaging in multiple sclerosis: filling the gap with the brain. Neurology. 83(15):1306-7, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25186859%5Bpmid%5D)
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1. [Makary MS et al: Tumefactive demyelinating disease with isolated spinal cord involvement. Acta Radiol Short Rep. 3(5):2047981614539324, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25298871%5Bpmid%5D)
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1. [Schlaeger R et al: Spinal cord gray matter atrophy correlates with multiple sclerosis disability. Ann Neurol. 76(4):568-80, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25087920%5Bpmid%5D)
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1. [Toosy AT et al: Voxel-based cervical spinal cord mapping of diffusion abnormalities in MS-related myelitis. Neurology. 83(15):1321-5, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=25186861%5Bpmid%5D)
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1. [Simka M et al: Reinterpreting the magnetic resonance signs of hemodynamic impairment in the brains of multiple sclerosis patients from the perspective of a recent discovery of outflow block in the extracranial veins. J Neurosci Res. 88(9):1841-5, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20127806%5Bpmid%5D)
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1. [Tallantyre EC et al: Clinico-pathological evidence that axonal loss underlies disability in progressive multiple sclerosis. Mult Scler. 16(4):406-11, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20215480%5Bpmid%5D)
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1. [Valsasina P et al: Cervical cord functional MRI changes in relapse-onset MS patients. J Neurol Neurosurg Psychiatry. 81(4):405-8, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=19965858%5Bpmid%5D)
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1. [Zamboni P et al: Chronic cerebrospinal venous insufficiency in patients with multiple sclerosis. J Neurol Neurosurg Psychiatry. 80(4):392-9, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19060024%5Bpmid%5D)
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1. [Zhang B et al: Correlation between serum thyroxine and complements in patients with multiple sclerosis and neuromyelitis optica. Neuro Endocrinol Lett. 29(2):256-60, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18404143%5Bpmid%5D)
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1. [Yukawa Y et al: MR T2 image classification in cervical compression myelopathy: predictor of surgical outcomes. Spine (Phila Pa 1976). 32(15):1675-8; discussion 1679, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17621217%5Bpmid%5D)
|
||
1. [Stüve O, Oksenberg J. Multiple sclerosis overview. 1993-, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=20301492%5Bpmid%5D)
|
||
1. [International Working Group for Treatment Optimization in MS: Treatment optimization in multiple sclerosis: report of an international consensus meeting. Eur J Neurol. 11(1):43-7, 2004](http://www.ncbi.nlm.nih.gov/pubmed/?term=14692887%5Bpmid%5D)
|
||
1. [Pretorius PM et al: The role of MRI in the diagnosis of MS. Clin Radiol. 58(6):434-48, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=12788312%5Bpmid%5D)
|
||
1. [Filippi M et al: Overview of diffusion-weighted magnetic resonance studies in multiple sclerosis. J Neurol Sci. 186 Suppl 1:S37-43, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11334988%5Bpmid%5D)
|
||
1. [Institute of Medicine (US) Committee on Multiple Sclerosis: current status and strategies for the future et al: 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=25057543%5Bpmid%5D)
|
||
1. [Poser CM et al: Diagnostic criteria for multiple sclerosis. Clin Neurol Neurosurg. 103(1):1-11, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11311469%5Bpmid%5D)
|
||
1. [Steiner I et al: Infection and the etiology and pathogenesis of multiple sclerosis. Curr Neurol Neurosci Rep. 1(3):271-6, 2001](http://www.ncbi.nlm.nih.gov/pubmed/?term=11898529%5Bpmid%5D)
|
||
1. [Bastianello S et al: MRI of spinal cord in MS. J Neurovirol. 6 Suppl 2:S130-3, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10871800%5Bpmid%5D)
|
||
1. [Hickman SJ et al: Imaging of the spine in multiple sclerosis. Neuroimaging Clin N Am. 10(4):689-704 ,viii, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11359719%5Bpmid%5D)
|
||
1. [Simon JH: Brain and spinal cord atrophy in multiple sclerosis. Neuroimaging Clin N Am. 10(4):753-70 ,ix, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=11359723%5Bpmid%5D)
|
||
1. [Simon JH: The contribution of spinal cord MRI to the diagnosis and differential diagnosis of multiple sclerosis. J Neurol Sci. 172 Suppl 1:S32-5, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10606803%5Bpmid%5D)
|
||
1. [van Waesberghe JH et al: Magnetization transfer imaging of the spinal cord and the optic nerve in patients with multiple sclerosis. Neurology. 53(5 Suppl 3):S46-8, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10496211%5Bpmid%5D)
|
||
1. [McFarland HF: The lesion in multiple sclerosis: clinical, pathological, and magnetic resonance imaging considerations. J Neurol Neurosurg Psychiatry. 64 Suppl 1:S26-30, 1998](http://www.ncbi.nlm.nih.gov/pubmed/?term=9647281%5Bpmid%5D)
|
||
1. [Campi A et al: Acute transverse myelopathy: spinal and cranial MR study with clinical follow-up. AJNR Am J Neuroradiol. 16(1):115-23, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7900579%5Bpmid%5D)
|
||
1. [Miller DH: Magnetic resonance imaging and spectroscopy in multiple sclerosis. Curr Opin Neurol. 8(3):210-5, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7551120%5Bpmid%5D)
|
||
1. [Tartaglino LM et al: Multiple sclerosis in the spinal cord: MR appearance and correlation with clinical parameters. Radiology. 195(3):725-32, 1995](http://www.ncbi.nlm.nih.gov/pubmed/?term=7754002%5Bpmid%5D)
|
||
1. [Jeffery DR et al: Transverse myelitis. Retrospective analysis of 33 cases, with differentiation of cases associated with multiple sclerosis and parainfectious events. Arch Neurol. 50(5):532-5, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8489410%5Bpmid%5D)
|
||
1. [Thomas DJ et al: Magnetic resonance imaging of spinal cord in multiple sclerosis by fluid-attenuated inversion recovery. Lancet. 341(8845):593-4, 1993](http://www.ncbi.nlm.nih.gov/pubmed/?term=8094830%5Bpmid%5D)
|
||
1. [Maravilla KR et al: Magnetic resonance demonstration of multiple sclerosis plaques in the cervical cord. AJR Am J Roentgenol. 144(2):381-5, 1985](http://www.ncbi.nlm.nih.gov/pubmed/?term=3871287%5Bpmid%5D)
|
||
|
||
## Differential diagnosis
|
||
|
||
### Intramedullary Spinal Cord Lesion
|
||
DDX:56579195-7385-4918-872c-c25d965e8486
|
||
|
||
## Anatomy
|
||
|
||
### Spinal Cord and Cauda Equina
|
||
Spine/ANATOMY:7bc73286-326e-4017-a9d8-eabc87b88ac9
|
||
|
||
### Vertebral Column and Spinal Cord
|
||
Ultrasound/ANATOMY:daf8e6c7-c462-456a-ae66-ba4c913c42d3
|
||
|
||
## Cases
|
||
|
||
- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '84757fd8-c814-48a5-adad-10bddd50d9eb', 'description': 'Variability of plaque conspicuity with different sequences.\n\nSagittal T1 image (#1) is normal. Sagittal T2 image (#2) (TR 3100/TE 112) shows no definite abnormality within cord. Sagittal intermediate image (#3) (TR 4000/TE 12) show focal hyperintensity within cord at C3 and C5 levels (arrows). Sagittal STIR image (#4) (TR 3500/TE 13/TI 110) show best conspicuity of the plaques, with slightly less signal to noise.\n\nComment: Heavily T2 weighted images are not the optimum for evaluation of intrinsic cord lesions, since they tend to give an image with only two signals (CSF and soft tissue) and do not have good sensitivity to subtle changes in cord signal.', 'history': None, 'imagePoolId': '25f459bd-eb87-4b6a-bd90-4f6399b5cfd6', 'name': 'Comparison of sequences', 'teachingPoint': None}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '99626c28-2b97-4a0a-8140-b4aa51c1ad5d', 'description': 'Typical appearance of MS lesions in the cervical cord.\n\nMR study (#1-4) shows 2 focal lesions of increased signal on the STIR image (arrows, #1) that are less than 1 vertebral body in length. There is no definite cord expansion and no edema beyond the focal lesions. Axial GE image shows 1 of the lesions at C4 within the posterior aspect of the cord (arrow, #2). Following contrast, there is mild ill-defined enhancement of the C4 intramedullary plaque (arrows, #3,4). Incidental note is made of a small disc protrusion at C5-6.', 'history': 'Chronic myelopathy.', 'imagePoolId': 'fd0bbd9f-8683-4aec-8d0a-5a842598a071', 'name': 'Enhancing plaque', 'teachingPoint': None, 'demographics': '34 Years old female'}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': 'a9ec521a-8d2e-4d27-9e87-79eaf424d678', 'description': 'Classic appearance of Devic disease, involving the optic nerves and spinal cord, with no brain parenchymal abnormalities.\n\nBrain examination (#1-4) shows normal FLAIR (#1), and markedly enhancing right optic nerve and chiasm (arrows, #3, 4). There is also abnormal T2 hyperintensity in the left optic nerve on the STIR image (open arrow, #2).\n\nEvaluation of the spinal cord (#5-10) shows a long segment of cord enlargement with T2 hyperintensity, and ill-defined enhancement (arrows).', 'history': 'Myelopathic and blind in both eyes.', 'imagePoolId': '98716ff1-9b49-4826-be9e-8d2b4073814e', 'name': 'Devic Disease (neuromyelitis optica)', 'teachingPoint': None}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '27b5fe08-9933-4472-bdbd-5ae4bfa5f107', 'description': 'Typical pattern of involvement in Devic disease.\n\nBrain study (#1) is normal. Evaluation of the optic nerves (#2-4) show abnormal T2 hyperintensity in the right optic nerve that shows pronounced enhancement (arrow, #3,4). The cervical cord shows a long segment (4 vertebral bodies in length) of T2 hyperintensity and mild diffuse cord enlargement (#5-7). Axial T2 image (#8) shows central cord hyperintensity. Following contrast administration (#9) there is minimal patchy enhancement of the cord. The extensive nature of the cord involvement is given the title "longitudinally extensive" cord signal abnormality. This is distinct from the much more focal abnormalities typically seen with cord involvement with MS.', 'history': None, 'imagePoolId': '90c40690-dacf-4d4d-9b7c-04e39a144bc0', 'name': 'Long segment cord demyelination', 'teachingPoint': None}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '4513b381-b5d0-4fbf-a315-5a265090e55f', 'description': 'Typical pattern of cord involvement.\n\nMR study (#1-4) shows focal T2 hyperintensity within the cord at C5 level, which shows both central (arrow, #2,3) and peripheral involvement (curved arrow, #2-4). No cord expansion.', 'history': None, 'imagePoolId': 'df27b385-6074-49f9-bbcd-a963856b974f', 'name': '3T', 'teachingPoint': None}, {'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': '27734ddd-b837-4047-85bb-1eaecf654e82', 'description': 'Multiple foci of cord enhancement in multiple sclerosis, some well-defined, and other ill-defined.\n\nImages through the cervical and thoracic cord (#1-7) show multiple foci of T2 hyperintensity (arrows, #1, 4, 5) consistent with demyelinating disease. There are foci of enhancement which vary from focal (curved arrow, #2, 3) to ill-defined (open arrow, #2, 6, 7). \n\nComment: The multiplicity of lesions along with the lack of edema or significant cord expansion is typical for demyelinating disease.', 'history': None, 'imagePoolId': 'ff2932a9-1190-4f36-bcaa-37b354ccf75d', 'name': 'Enhancing plaques', 'teachingPoint': None}, {'authors': [{'key': '99e1aff7-f42c-43a0-95ae-d89c8551aa01', 'value': 'Kevin R. Moore, MD'}], 'caseVersionId': '12839b07-fb73-472c-9539-48a0b136dd84', 'description': 'Typical case of acute spinal cord demyelination.\n\nSagittal T1WI MR (#1) is normal. Sagittal T2WI MR (#2) and STIR MR (#3) demonstrate multiple hyperintense intramedullary lesions with focal cord enlargement, typical of demyelination. Although MS is often considered a white matter disease, in fact spinal cord and brain gray matter involvement is very common. Sagittal T1 C+ MR (#4) shows faint enhancement of several lesions (arrows). Axial T2WI MR (#5, 6) are useful to localize lesion in relation to cord somatotopy. Axial T1 C+ MR (#7, 8) confirm faint ring enhancement (arrows). Sagittal (#9) and axial (#10) FLAIR MR images of the brain demonstrate concurrent severe corpus callosum and white matter brain lesions typical of MS.', 'history': 'Patient presents with relapsing/remitting Multiple Sclerosis (MS) exacerbation, complaining of left arm weakness, facial numbness, and dysmetria.', 'imagePoolId': 'b3a41192-9565-42f9-9ffd-2b494611e94a', 'name': 'Classic', 'teachingPoint': None, 'demographics': '17 Years old female'}, {'authors': [{'key': '61869900-31b1-4db7-b4f5-d4da24fa86f3', 'value': 'Mark Z. Chen, MD'}], 'caseVersionId': '16a742ad-313d-439a-af55-981ce0aede3a', 'description': 'Typical case of thoracic intramedullary demyelinating plaque.\n\nSagittal T2WI FS MR (#1) demonstrates a focal intramedullary hyperintense lesion (arrow) expanding the thoracic spinal cord. Axial T2WI (#2) and T1WI MR (#3) confirm the eccentrically located intramedullary lesion (arrow). Axial T1 C+ MR (#4) reveals peripheral ring enhancement implying more acute demyelination (arrow). Sagittal T2WI MR through the brain (#5) shows a hyperintense lesion (arrow) involving the corpus callosum, typical of MS.', 'history': 'Multiple Sclerosis patient presents with myelopathic symptoms referable to the thoracic level. ', 'imagePoolId': '204e85f8-1235-414c-8d9e-227cca403070', 'name': 'Classic, acute intramedullary lesion', 'teachingPoint': None, 'demographics': '30 Years old female'}, {'authors': [{'key': '99e1aff7-f42c-43a0-95ae-d89c8551aa01', 'value': 'Kevin R. Moore, MD'}], 'caseVersionId': '4e0e98c9-4b87-4e35-be86-b63c45123852', 'description': 'Typical case of multiple intramedullary spinal cord demyelinating lesions.\n\nSagittal T1WI MR (#1, 2) show mild degenerative disc disease but are otherwise normal. Sagittal T2WI MR (#3, 4) reveal multiple T2 hyperintense intramedullary demyelinating lesions (arrows). Sagittal STIR MR (#5, 6) show the same lesions, as well as an additional dorsal spinal cord lesion not visualized on the T2WI MR images (open arrow). In general, STIR MR is the most sensitive imaging sequence for detecting spinal demyelinating lesions. Axial T2WI MR (#7, 8) are most useful to localize lesion center (arrows) in relationship to spinal cord long tracts.', 'history': 'Patient with known Multiple Sclerosis (MS) presents for imaging during acute MS exacerbation.', 'imagePoolId': '305fd70f-34c8-421d-bebf-71ef42f45d7c', 'name': 'Multiple intramedullary lesions', 'teachingPoint': None, 'demographics': '53 Years old female'}], 'caseType': 'typical', 'name': 'TYPICAL'}
|
||
- {'cases': [{'authors': [{'key': 'bee1f359-33fb-4cba-9e6b-ed1ca1842439', 'value': 'Jeffrey S. Ross, MD'}], 'caseVersionId': 'b975cd06-048d-477e-b75e-3d34569e5131', 'description': "Multiple sclerosis, neuromyelitis optica variant (Devic's disease).\n\nT2 weighted image (#1) shows mild fusiform thoracic cord enlargement with hyperintensity (arrows). Sagittal and axial images following contrast (#2-4) show a large area of ring enhancement within the cord, with small punctate cord peripheral enhancement caudal to the major lesion (open arrow). Axial images show near total transverse cord involvement with enhancement.", 'history': 'Patient developed waist and bilateral lower extremity numbness, progressed to weakness, and inability to walk with hospital\nadmission and treatment with IV steroids and plasma exchange therapy.\n', 'imagePoolId': 'b49b8ab0-c8e1-4046-9a41-0a45f103d8d7', 'name': "Devic's variant", 'teachingPoint': None, 'demographics': '46 Years old female'}, {'authors': [{'key': '99e1aff7-f42c-43a0-95ae-d89c8551aa01', 'value': 'Kevin R. Moore, MD'}], 'caseVersionId': 'c9a7e1ce-0904-4882-a79a-08eca9797985', 'description': 'Variant case depicts ring enhancement and intrinsic lesion T1 hyperintensity.\n\nSagittal T1WI MR (#1, 2) demonstrate mild peripheral "ring" T1 hyperintensity (arrow) within an intramedullary demyelinating lesion, which has been attributed to paramagnetic metal deposition. Sagittal T2WI (#3, 4) depict peripheral T2 hyperintensity (arrow) and mild cord swelling. Sagittal T1 C+ MR (#5, 6) reveal peripheral rim-enhancement (arrows) of the lesion margins, indicating the advancing "wave" of demyelination. Axial T2WI MR (#7) and T1 C+ MR (#8) confirm same findings within the right lateral spinal cord (arrow). This pattern of signal intensity on the previous sequences is commonly noted in brain lesions but unusual in the spinal cord.', 'history': 'Adult patient with known Multiple Sclerosis (MS) presents with acute myelopathy for imaging work-up.', 'imagePoolId': '926f079e-67f4-4591-96bc-030a70f10ea2', 'name': 'Ring lesion enhancement', 'teachingPoint': None, 'demographics': '43 Years old male'}], 'caseType': 'variant', 'name': 'VARIANT'}
|
||
|
||
|
||
## Images
|
||
|
||
|
||
### Selected Images
|
||
|
||
|
||
*Sagittal graphic depicts multiple demyelinating plaques within the cervical spinal cord, which are < 2 vertebral bodies in length.*
|
||
|
||
|
||
*Sagittal T2 (left) & T1 C+ MR (right) show an active plaque at the C6-C7 level with ring enhancement & focal T2 hyperintensity <img src='img/arrows/WS.png'/>.*
|
||
|
||
|
||
*Sagittal T2 (left), PD (middle), & STIR (right) MR show multiple short-segment multiple sclerosis (MS) plaques within the thoracic cord <img src='img/arrows/CS.png'/>. Note the relatively improved conspicuity of the plaques on the PD & STIR relative to the routine T2 sequence.*
|
||
|
||
|
||
*Sagittal T2 (left), T2 (middle), & T1 C+ FS (right) MR of the thoracic spine show multiple short-segment foci of T2 hyperintensity <img src='img/arrows/WS.png'/> in this patient with MS. Multiple lesions show solid enhancement <img src='img/arrows/CS.png'/>.*
|
||
|
||
|
||
*Axial T2 MR shows focal MS plaques as T2 hyperintensity within both the right & left lateral aspects of the cervical cord <img src='img/arrows/WS.png'/>.*
|
||
|
||
|
||
*Axial T1 C+ MR shows focal enhancement within the right & left lateral aspect of the cervical cord in this patient with active MS plaques.*
|
||
|
||
|
||
*Sagittal T2 (left) & T1 C+ MR (right) show active enhancing plaque at the C2 level with both focal well-defined (enhancing) T2 focus <img src='img/arrows/WS.png'/> & a small amount of surrounding nonenhancing edema <img src='img/arrows/WO.png'/>.*
|
||
|
||
|
||
*Sagittal T2 (left) & T1 C+ FS (right) MR show several T2-hyperintense foci in the cervical cord in this patient with MS. 2 of the lesions enhance reflecting active demyelination <img src='img/arrows/WS.png'/>.*
|
||
|
||
|
||
*Axial T2WI MR shows focal T2-hyperintense demyelinating lesions with both central <img src='img/arrows/WS.png'/> & peripheral involvement <img src='img/arrows/WC.png'/>. T2-hyperintense lesions are not specific for plaque age, degree of myelin & axon loss, or amount of edema & inflammation.*
|
||
|
||
|
||
*Sagittal high-resolution GRE MR of the thoracic cord shows multiple areas of ↑ signal <img src='img/arrows/WS.png'/> in this patient with MS. All lesions are ≤ 2 vertebral bodies in length, typical for MS.*
|
||
|
||
|
||
### Additional Images
|
||
|
||
|
||
*Sagittal T2WI MR shows diffuse cord enlargement & hyperintensity throughout cervical segment into upper thoracic cord in this case of acute MS exacerbation.*
|
||
|
||
|
||
*Sagittal T1WI C+ MR shows diffuse cord expansion & extensive multilevel cord enhancement in acute exacerbation of MS.*
|
||
|
||
|
||
*Axial T1WI C+ MR with fat suppression of cervical cord in a different patient shows right peripheral nodular enhancement.*
|
||
|
||
|
||
*Sagittal T2WI MR of cervical cord shows a more discrete demyelinating focus at C3-C4.*
|
||
|
||
|
||
*Axial T2WI MR of cervical cord in another patient shows a poorly defined, wedge-shaped, mildly hyperintense plaque within right lateral aspect of the cord.*
|
||
|
||
|
||
*Sagittal T2WI MR of cervical cord shows an ill-defined hyperintense intramedullary lesion at C5-C6.*
|
||
|
||
|
||
*Sagittal STIR MR shows a focal hyperintense demyelinating plaque <img src='img/arrows/WS.png'/> within the thoracic cord without significant cord expansion. STIR MR is more sensitive for lesion depiction than T2WI MR at the price of more artifacts.*
|
||
|
||
|
||
*Sagittal T2WI MR of the cervical spinal cord demonstrates multiple T2-hyperintense foci <img src='img/arrows/WS.png'/>, some well defined & others ill defined. The multiplicity of lesions & lack of edema or significant cord expansion is typical for demyelinating disease.*
|
||
|
||
|
||
*Sagittal T1WI C+ MR shows multiple enhancing demyelinating lesions within the cervical spinal cord. Enhancement varies from focal <img src='img/arrows/WC.png'/> to ill defined <img src='img/arrows/WO.png'/>. The enhancement pattern changes with evolution of inflammation.*
|
||
|
||
|
||
*T1WI C+ MR (sagittal on top, axial on bottom) illustrates an incomplete rim-enhancing lesion <img src='img/arrows/WS.png'/> in the dorsal cervical cord at the C3-C4 level. A 2nd small enhancing focus is noted in the ventral cord at the C6 level <img src='img/arrows/WC.png'/>.*
|
||
|
||
|
||
*Sagittal PD FSE MR of the cervical spinal cord demonstrates characteristic ovoid hyperintense intramedullary demyelinating lesions <img src='img/arrows/WO.png'/> without significant cord expansion.*
|
||
|
||
|
||
*Axial T1 C+ MR of the cervical spinal cord depicts focal ring enhancement <img src='img/arrows/WS.png'/> within an active MS demyelinating lesion.*
|
||
|