Fact-sheet: Multiple sclerosis
Updated on 03/20/2025 at 4:14 PM
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Definition
Most common chronic inflammatory demyelinating disease.
Affects all myelinated areas of the central nervous system: optic nerves, periventricular areas, corpus callosum, cerebellum, spinal cord...
Epidemiology:
- Young adults (20-40 years), female predominance (70%)
- 70,000 to 80,000 patients in France
- North/South gradient
Clinical features
Varied clinical manifestations, initially monosymptomatic (60% of cases) and related to the demyelinated area, appearing subacutely:
- Motor signs: monoparesis, hemiparesis, paraparesis
- Optic neuritis: visual acuity loss, periorbital pain, scotoma, red-green dyschromatopsia
- Sensory disturbances: anesthesia, hypoesthesia, paresthesias, neuropathic pain
- Cerebellar syndrome
- Cranial nerve involvement
- Bladder and sphincter dysfunction
- Cognitive impairment...
Evolves according to several patterns:
- Relapsing-remitting: successive relapses separated by periods of clinical stability or recovery
- Progressive (primary or secondary): progressive disability without identifiable clinical relapse (typically: gait disturbances with progressive reduction of walking distance associated with cognitive and bladder/sphincter dysfunction)
Diagnosis: based on dissemination in space and time
- Dissemination in time:
- Several relapses separated by symptom-free intervals: clinical, paraclinical, or anamnestic
- Presence of oligoclonal bands in the CSF (revised 2017 McDonald criteria)
- Dissemination in space: involvement of at least 2 areas of the central nervous system among:
- Periventricular
- Cortical/Juxtacortical
- Infratentorial
- Spinal cord
Laboratory findings
Lumbar puncture: detection of oligoclonal bands in the CSF
MRI
- White matter hyperintensities on T2-weighted sequences. Sometimes associated with T1 hypointensity in the case of a "black hole", which is rarer (and reflects a highly inflammatory history of this lesion)
- Predominantly periventricular, with long axis perpendicular to the ventricles
- Most common cause of optic neuritis
- Recent lesions (less than 3 months old) enhance after contrast administration
- Main features of a tumefactive inflammatory lesion: lesion >2 cm that may raise suspicion of a brain tumor on MRI.
- Little or no mass effect or edema (contrasting with the size of the lesion++).
- Incomplete peripheral "open-ring" enhancement of the lesion.
- Center of the lesion: hyperintense on T2/hypointense on T1 with increased ADC
- Periphery of the lesion: hypointense on T2 with decreased ADC.
- Mildly to moderately increased CBV (typically no hyperperfusion).
- More often supratentorial location (frontal +++, parietal +).
- History of demyelinating disease, prior lesions++.
- However, the imaging appearance of these lesions remains highly variable: infiltrative pseudocystic lesion, infiltrative lesion, target-like (cockade) lesion.
Management
Treatment of relapse:
- High-dose corticosteroid therapy (IV or PO) at 1 gram per day for 3 days (or 5 days by consensus in the case of a spinal cord relapse)
- If ineffective, plasma exchange may be considered
Disease-modifying therapies:
- Immunomodulators: INTERFERON BETA 1A, GLATIRAMER ACETATE...
- Immunosuppressants: NATALIZUMAB, FINGOLIMOD...
Symptomatic treatments
Differential diagnosis
Any systemic inflammatory disease: sarcoidosis, Behçet's disease, SLE, Sjögren's syndrome, arteritis, ...
Diagnosis of tumefactive inflammatory lesions
- Infection: encephalitis (JC virus, herpes), abscess
- Tumor: primary or secondary CNS lesion, radionecrosis, lymphoma
- Vascular: subacute ischemic stroke