Table 1.
Main features in demyelinating myelopathies of different etiology.
| MS | ADEM | NMOSD | MOG-IgG Disease | GFAP-IgG Disease | |
|---|---|---|---|---|---|
| Estimated F:M ratio | 3:1 | 1:1 | 9:1 | 1.3:1 | 1:1 |
| Age * (yrs) | 30 | 6 | 37 | 33 | 40 |
| Myelitis clinical features | Sensory loss, gait impairment, weakness, sphincter involvement | Transverse myelitis | Transverse myelitis | Paraparesis, sensory symptoms and sphincter involvement | Sensory symptoms, sphincter disfunction |
| Clinical course | Relapsing (85%) or progressive (15%) | Typically monophasic (69–90%) | Relapsing (90%) | Monophasic (58%) or relapsing (42%) | Relapsing (50%), monophasic (27%) or progressive (23%) |
| Serology findings | Not relevant | Not relevant | Serum AQP4-IgG. coexistence with other systemic disease antibodies (ANA, SSA or SSB). |
Serum MOG-IgG | Anti-GFAP ab + in serum or CSF (Serum Anti-AQP4-IgG and/or anti-NMDAr ab coexistence, |
| Presence of OCB | 80–95% | 0% to 29% (usually transient) | Up to 30% (usually transient) |
Up to 12% | Up to 50% |
| CSF | Generally normal or mild inflammatory changes | Mild pleocytosis and increased proteins up to 62% | Pleocytosis (neutrophils and eosinophils can be found) and mild elevated proteins | Normal or slightly inflammatory changes | Marked elevation of white blood cells and elevated protein levels |
| Brain MRI | Dawson fingers, lesions perpendicular to ventricles Cortical/yuxtacortical lesions Perivenular Nodular or ring/open-ring enhancing lesions Unilateral short optic nerve enhancement |
Subcortical or deep gray matter bilateral, sometimes poorly-defined Simultaneous enhancement with gadolinium | Periependimal lesions Tumefactive lesions Involvement of corticospinal tract Marked enhancement, ‘cloud like’ Bilateral, long optic nerve enhancement |
Non—specific supratentorial subcortical or small deep white matter foci. Occasionally T2 lesions in brainstem, and infratentorial regions Anterior bilateral ON with perineural optic nerved enhancement |
Linear radial periventricular contrast enhancement pattern |
| Spinal cord MRI | Small, peripheral, posterolateral lesions Less than 3 segments Gadolinium enhancement during acute phase |
LETM or multiple short segment myelitis Edematous lesions and gadolinium enhancement in acute phase |
Central LETM Edematous Necrosis or cavitation Gadolinium enhancement in acute phase |
LETM or short myelitis, frequent conus medullaris involvement Linear gadolinium enhancement of the ependymal canal |
LETM Central lesions |
Ab: antibodies, ADEM: acute disseminated encephalomyelitis, AQP4: Aquaporin 4, F: female, GFAP: glial fibrillary acid protein, LETM: longitudinally extensive transverse myelitis, M: male, MOG: myelin oligodendrocyte glycoprotein, MRI: magnetic resonance imaging, MS: multiple sclerosis, NMDAr: N-Methyl-d-aspartate receptor, NMOSD: neuromyelitis optica spectrum disorder, OCB: oligoclonal bands. * estimated media.