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