Multiple Sclerosis¶
Background¶
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Progressive inflammatory disorder primarily manifesting with demyelination of the central white matter
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Optic neuritis and transverse myelitis (spinal cord lesion) are common presentations
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Generally develop over a few days; very uncommon to happen suddenly (e.g., patients will complain about a dark spot appearing in their vision that expands over several days)
Evaluation¶
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MRI w/ and w/o contrast can identify plaques and determine if they are more acute
- “Active” MS plaque will enhance, and continues to for weeks (even after treatment)
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Modified MacDonald Criteria: ≥3 characteristic demyelinating lesions (>1 cm, periventricular, infratentorial (brainstem/cerebellum/cord) or juxtacortical in location) with evidence of separation in time (active and chronic)
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LP with studies for oligoclonal bands, IgG index, cell count and protein, anti-mog, anti-aqp4
Management¶
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Treat flares and optic neuritis with steroids
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Speeds up recovery, but does not improve the degree of recovery
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Often dosing starts with methylpred 1g
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If a patient with known MS has worsening symptoms that are not new, then recrudescence is the likely cause infectious/toxic/metabolic workup and imaging is needed
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There are several long-term medications; common side effects listed below:
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Interferon (SQ injections) – flu-like symptoms, injection site reactions
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Glatiramer acetate (SQ) – injection site reactions
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Fingolimod (PO) – macular edema, liver injury, increased risk of skin cancer
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Teriflunomide (PO) – liver injury, hair loss, immunosuppression, teratogenic
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Dimethyl fumarate (PO) – GI side effects, lymphocytopenia, liver injury
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Natalizumab (IV) – PML concern, immunosuppression
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Ocrelizumab (IV) – contraindicated in active HBV infection, cannot give live vaccines
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Alemtuzumab (IV) – autoimmune disease, rash, headache
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Neuromyelitis Optica and Spectrum Disorder¶
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Demyelinating disease due to Ab against aquaporin-4 (on oligodendrocytes)
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Classically causes optic neuritis and longitudinally extensive transverse myelitis
- Can be more aggressive than MS
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Diagnose with NMO antibodies and MS workup as above
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Treated with steroids, but in severe or refractory cases may require PLEX