MS | NMOSD | |
---|---|---|
Frequency of disease | Common | Rare |
Latitude gradient | Present | Not proven |
Female sex, % | 70 | 90 |
Ethnic variation | Common in Caucasians | Common in Africans and Asians |
Age at onset | 20–40 | 40–60 |
Progressive course | Common | Rare |
Coexistent autoimmune disease | Rare | Common: MG, SLE, Sjogren’s, thyroid, APL |
Tissue involvement | White matter | White and gray matter |
Necrosis/cavitation | Rare | Common |
Leukocyte infiltrate | T and B lymphocytes | Neutrophils and eosinophils |
Perivascular IgG and complement | Common | Common |
Attack severity | Often mild | Often severe |
Spinal cord | Short-segment peripheral cord lesions | Usually LETM, central cord involvement |
May be asymptomatic | Extension into medulla | |
Usually symptomatic | ||
Acute T1 hypointensity | ||
Optic nerve | Short segment inflammation, anterior | Long segment inflammation posterior |
Unilateral | Unilateral/bilateral | |
Good recovery | Poor recovery | |
Brainstem | Any location | Area postrema/dorsal medulla MRI lesion |
Ventral or dorsal pontine lesion | May be contiguous with spinal lesion | |
Clearly defined borders | ||
Diencephalon | Uncommon | Hypothalamic, thalamic, periependymal 3rd ventricle region |
Corpus callosum | Very common | Uncommon |
Small lesions | Long lesions | |
Anterior/posterior CC | Callosal-septal interface in middle and posterior thirds of CC | |
Cerebral hemispheres | Ovoid lesions perpendicular to lateral ventricle (Dawson’s fingers) | Large, confluent subcortical or deep white matter lesions |
Lesion adjacent to body of lateral ventricle and in inferior temporal lobe | Long corticospinal tract lesions | |
Juxtacortical U-fibre lesions | ||
CSF | Mild pleocytosis; mononuclearOCBs 85% | Occasional prominent pleocytosis, lymphocytes, PMN and mononuclear cells |
OCBs uncommon | ||
Permanent disability | Usually in later progressive disease phase | Usually attack-related |
Treatment | ||
Relapse | Consider steroids (5 days) for severe acute relapses | High dose steroids |
Earlier treatment is essential. Escalation to PLEX if needed | ||
Long-term | In the UK disease modifying therapies are considered in active MS (≥2 relapses in 2 years) | AZA/MMF/RTX for all AQP4-Ab positive NMOSD or relapsing seronegative NMOSD |
APL = antiphospholipid syndrome; AQP4-Ab = aquaporin-4 antibodies; AZA = azathioprine; CC = corpus callosum; CSF = cerebrospinal fluid; Ig = immunoglobulin; LETM = longitudinally extensive transverse myelitis; MAC = membrane attack complex; MG = myasthenia gravis; MMF = mycophenolate mofetil; MRI = magnetic resonance imaging; OCBs = oligoclonal bands; PLEX = plasma exchange; PMN = polymorphonuclear cells; RRMS = relapsing remitting multiple sclerosis; RTX = rituximab; SLE = systemic lupus erythematosus.