Table 4.
Diagnostic criteria of AQP4+NMOSD and MOGAD.
| AQP4+NMOSD | MOGAD | ||
|---|---|---|---|
| Antibody test (CBA) positive (AQP4+NMOSD) or clear positive (MOGAD) | Core clinical features | Optic neuritis | Optic neuritis |
| Myelitis | Myelitis | ||
| Area postrema syndrome Acute brainstem syndrome | Brainstem or cerebellar deficits | ||
| Symptomatic cerebral syndrome with AQP4+NMOSD-typical brain MRI lesions | ADEM | ||
| Symptomatic narcolepsy or acute diencephalic clinical syndrome with AQP4+NMOSD-typical diencephalic MRI lesions | Cerebral monofocal or polyfocal deficits | ||
| Cerebral cortical encephalitis often with seizures | |||
| Antibody test (CBA) negative/unknown (AQP4+NMOSD) or low positive/positive without titre in serum or negative in serum but CSF positive (MOGAD) | Supporting features | ||
| Optic neuritis | Normal findings or only nonspecific white matter lesions in the brain | Bilateral simultaneous clinical involvement | |
| Longitudinal optic nerve involvement (> 50% length of the optic nerve on T2 or postgadolinium T1) | Longitudinal optic nerve involvement (> 50% length of the optic nerve) | ||
| Optic chiasm | Perineural optic sheath enhancement | ||
| Optic disc oedema | |||
| Myelitis | Longitudinally extensive myelitis | Longitudinally extensive myelitis | |
| Longitudinally extensive focal spinal cord atrophy in patients with history compatible with acute myelitis | Central cord lesion or H-sign | ||
| Conus lesion | |||
| Area postrema syndrome | Dorsal medulla/area postrema lesions | - | |
| Brain, brainstem, or cerebral syndromes | Periependymal brainstem lesions | Multiple ill-defined T2 hyperintense lesions in supratentorial and often infratentorial white matter | |
| Deep grey matter involvement | |||
| Ill-defined T2-hyperintensity involving pons, middle cerebellar peduncle, or medulla | |||
| Cortical lesion with or without lesional and overlying meningeal enhancement | |||
| Exclusion of better diagnoses including multiple sclerosis | Yes | Yes |
Abbreviations: CBA=cell-based assay; CSF=cerebrospinal fluid; MOGAD= myelin oligodendrocyte glycoprotein antibody-associated disease; AQP4+NMOSD= aquaporin-4-IgG positive neuromyelitis optica spectrum disorder.