Attribute | MOGAD | AQP4-NMOSD | Multiple sclerosis |
Antecedent infection/immunization | Common | Rare | Rare |
Ages affected | Any (children and young adults more predisposed; median age of onset in third decade) | Any (median age at onset in fourth decade) | Any (median age at onset in third decade) |
Sex (female:male) | 1:1 | 9:1 | 2:1 |
Epidemiology | Prevalence: rare Race/ethnicity: no predilection yet identified Geographic: variable | Prevalence: rare Race/ethnicity: African American and Afro-Caribbean individuals more predisposed Geographic: higher proportion of total demyelinating disease is AQP4-IgG positive NMOSD in regions where multiple sclerosis prevalence is low | Prevalence: common Race/ethnicity: White individuals are more predisposed Geographic: higher prevalence in regions farthest from equator |
Most common manifestations | Initial episode: optic neuritis (with optic disc edema in >85%), ADEM, NMOSD, myelitis, unilateral cortical encephalitis Relapse: optic neuritis | NMOSD (any combination of myelitis, optic neuritis, area postrema syndrome) | Myelitis, optic neuritis, brainstem, cerebral episodes; myelopathy for progressive multiple sclerosis |
Course | Monophasic or relapsing; no reports of secondary progression | Typically relapsing; usually no secondary progression | Relapsing-remitting from onset in 85% (most later develop secondary progression); 10 to 15% progressive from onset |
Attack severity | Usually moderate to severe | Usually moderate to severe | Usually mild to moderate |
Recovery from attacks | Good | Often incomplete | Good |
CSF | White blood cell count variable (lymphocytic); oligoclonal bands in 5 to 20% | White blood cell count variable (usually lymphocytic but can be neutrophilic or eosinophilic); oligoclonal bands in 30% | White blood cell count <50/mm3 × 106/L (lymphocytic) or can be normal; oligoclonal bands in 85% |
Blood biomarker | MOG-IgG (false positives may occur) | AQP4-IgG (false positives very rare) | NA |
Brain MRI | ADEM-like fluffy white matter, deep gray matter, diffuse/confluent brainstem including cerebellar peduncles, unilateral cortical swelling, leptomeningeal enhancement | Often normal/nonspecific; if present, area postrema, peri- third/fourth ventricle, splenium, diffuse corpus callosum, pencil-thin ependymal or cloud enhancement | Ovoid periventricular (Dawson fingers), juxtacortical, cortical, infratentorial peripheral, ring-/open ring-enhancing |
Optic nerve MRI | Unilateral or bilateral; enhancement of >50% of optic nerve; anterior optic pathway (hence optic disc edema common) | Unilateral or bilateral; enhancement of >50% of optic nerve; posterior optic pathway involving chiasm | Usually unilateral; enhancement of <50% of nerve affected; middle of optic nerve |
Spine MRI | Multiple lesions (75% longitudinally extensive transverse myelitis; 25% short); conus involved; central on axial, often with H-sign; enhancement <50% | Single lesion (longitudinally extensive transverse myelitis 85%; short 15%); central on axial; ring or variable enhancement | Multiple lesions; short lesions; periphery of cord (dorsal/lateral column); ring or variable enhancement |
Acute treatment | IV glucocorticoids; plasma exchange (sometimes required); IVIG (used in children) | IV glucocorticoids; plasma exchange (often required) | IV glucocorticoids; plasma exchange (rarely required) |
Maintenance treatment | None approved; none needed if monophasic; glucocorticoids-sparing for relapsing disease (azathioprine, IVIG mycophenolate, methotrexate, rituximab) | FDA-approved medications in the United States: eculizumab, inebilizumab, and satralizumab Off label: azathioprine, mycophenolate, rituximab, tocilizumab | Variety of approved immunomodulatory medications |
Prognosis | Most disability with first attack; transient seropositivity predicts monophasic course; persistent seropositivity and high titer predict relapsing disease | Attack-related accumulation of disability; secondary progression rarely if ever occurs | Majority ambulatory after 20 years; most disability occurs in progressive phase |
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