Table 1.
Comparison of demographics, clinical, laboratory and MRI characteristics in MOGAD compared to AQP4-IgG+NMOSD, and MS.
| MOGAD | AQP4-IgG-NMOSD | MS | |
|---|---|---|---|
| Demographics | |||
| Most commonly affected age | 0–40 | 30–50 | 20–40 |
| Sex (F:M) | 1:1 | 9:1 | 2–3:1 |
| Ethnicity | No clear racial predominance | Any, African-American and Asian at higher risk | Any, mostly Caucasian |
| Clinical features | |||
| Antecedent infection/immunization | Common | Rare | Rare |
| Disease course | Relapsing (50%) or monophasic (50%); a progressive course is rare | Generally relapsing; a progressive course is extremely rare | Relapsing (85%) or progressive from onset (15%) |
| Optic neuritis | +++ | +++ | ++ |
| Myelitis | ++ | +++ | +++ |
| Area postrema syndrome | Rare | ++ | Rare |
| Encephalopathy | ++ | Rare | Rare |
| Seizures | + | Rare | Rare |
| CSF | |||
| Oligoclonal bands | <20% (transient) | <20% (transient) | >85% (persistent) |
| White cell count >50/μl | 35% | 13–35% | Rare |
| MRI | |||
| Acute attacks | |||
| Optic nerve | Uni-/bilateral, long lesions (>50%), mainly anterior segments, perineural enhancement | Uni-/bilateral, mainly posterior segments including chiasm | Generally unilateral, short lesions, mainly along the intraorbital tract |
| Spinal cord | Longitudinally extensive (60%), generally >1 lesion, conus involved, H-sign axially, enhancement in 50% | Longitudinally extensive (85%), single lesion. Central/diffuse on axial images. Elongated ring or patchy enhancement | Multiple short lesions; periphery of cord. Ring or nodular enhancement |
| Brain | ADEM-like, “fluffy” lesions in both white and deep gray matter; extensive involvement of cerebellar peduncles. Cortical hyperintensity may occur | Often non-specific; area postrema, peri- 3rd/4th ventricle, corticospinal tracts, sometimes extensive white matter lesions | Ovoid periventricular, infratentorial, juxtacortical. Central vein sign. Ring enhancement |
| Initially normal MRI | Up to 10% | Rare | Rare |
| Post-attack MRI | |||
| T2-lesion resolution | 50–80% | Rare | Rare |
| New asymptomatic T2-lesions occurrence | Rare | Rare | Common |
| Residual T1-hypointensity | Rare | Common | Common |
| Persistent acute gadolinium enhancement >6 months | Rare | Rare | Rare |
| Treatment | |||
| Acute | IVMP; PLEX if severe episode; IVIg possible alternative, especially for children | IVMP; low threshold to follow with PLEX | IVMP; PLEX reserved for the rare very severe attacks |
| Recovery | Generally good despite severe attacks | Often incomplete | Generally good |
| Maintenance | No proven treatments with class 1 evidence. Common empiric options include mycophenolate, rituximab, periodic IVIg, tocilizumab, oral steroids | Class 1 evidence for: eculizumab, inebilizumab, rituximab and satralizumab; other: azathioprine, mycophenolate, oral steroids, tocilizumab | Large variety of MS medications proven to be effective in class 1 studies |
ADEM, acute disseminated encephalomyelitis; AQP4-IgG+NMOSD, aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder; CSF, cerebrospinal fluid; IVIg, intravenous immunoglobulins; IVMP, intravenous methyl-prednisone; MOGAD, myelin oligodendrocyte glycoprotein antibody-associated disease MS, multiple sclerosis; PLEX, plasma exchange.