Table 2.
Epidemiological and clinical comparison between AQP4-antibody-seropositive NMOSD, MOG-antibody disease, and MS.
| AQP4-antibody disease | MOG-antibody disease | MS | |
|---|---|---|---|
| Mean age at onset | 40 years | More common in children than in adults | 30 years |
| Female:male ratio | 9:1 | Around 1:1 | 2–4:1 |
| North–South gradient | No increased prevalence with increasing latitude | No data | Increased prevalence with increasing latitude from the equator (either toward North or South) |
| Prevalence | East Asians: 3.5/100,000 Whites: 1/100,000 Blacks: range from 1.8 to 10/100,000 |
More common in children than in adults | Up to 100–200/100,000 in White populations, but <5–50/100,000 in many Asian and African countries Rising in most parts of the world |
| Annual incidence | Around 0.5–0.8/million in Whites Higher annual incidence in non-White populations |
Dutch nationwide study: 1.6/million; adults: 1.3/million; children: 3.1/million More data are needed |
Up to 100/million in White populations, but was low in many equatorial countries |
| Disease course | Relapsing | Monophasic or relapsing | Relapsing, with the majority eventually converting to a secondary progressive disease Up to 15% are primary progressive in Whites |
| Clinical manifestations | Optic neuritis Myelitis Area postrema syndrome Other brain syndromes |
Optic neuritis Myelitis ADEM/MDEM Brainstem/cerebral cortical encephalitis Cranial nerve involvement |
Optic neuritis Myelitis Brain syndromes |
| Optic neuritis | Unilateral/chiasmal, long (>1/2 of optic nerve) | Unilateral/simultaneous bilateral, long; frequent optic disc swelling (papillitis) | Unilateral, short |
| Myelitis | Long (>3 vertebral segments) in 85%; centrally located; affects cervical or thoracic cord | Often long, but may be <3 vertebral segments; gadolinium enhancement less common than AQP4-antibody disease; relatively more common in the lumbosacral region | Non-transverse, short; peripheral/dorsolateral |
| Attack severity | Moderate to severe | Mild to moderate | Mild to moderate |
| Recovery | Variable, but commonly poor | Fair to good | Fair to good |
| Disability | Attack-related | Attack-related | Mainly due to progression |
| Pathology | Astrocytopathy | Demyelination | Demyelination |
| Treatment | Immunosuppressants; some MS drugs may be harmful | Consider immunosuppressants if recurrent; some MS drugs may be ineffective | MS disease-modifying drugs |
ADEM/MDEM, acute disseminated encephalomyelitis/multiphasic disseminated encephalomyelitis; AQP4, aquaporin 4; MOG, myelin oligodendrocyte glycoprotein; MS, multiple sclerosis; NMOSD, neuromyelitis optica spectrum disorder.