| Diagnostic criteria for NMOSD with AQP4-IgG |
- At least one core clinical characteristic
- Positive test for AQP4-IgG using best available detection method (cell-based assay strongly recommended)
- Exclusion of alternative diagnoses
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| Diagnostic criteria for NMOSD without AQP4-IgG or NMOSD with unknown AQP4-IgG status |
- At least two core clinical characteristics occurring as a result of one or more clinical attacks and meeting all of the following requirements:
- At least one core clinical characteristic must be optic neuritis, acute myelitis with LETM, or area postrema syndrome
- Dissemination in space (two or more different core clinical characteristics)
- Fulfillment of additional MRI requirements, as applicable
- Negative tests for AQP4-IgG using best available detection method, or testing unavailable
- Exclusion of alternative diagnoses
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| Core clinical characteristics |
- Optic neuritis
- Acute myelitis
- Area postrema syndrome: Episode of otherwise unexplained hiccups or nausea and vomiting
- Acute brainstem syndrome
- Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions
- Symptomatic cerebral syndrome with NMOSD-typical brain lesions
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| Additional MRI requirements for NMOSD without AQP4-IgG and NMOSD with unknown AQP4-IgG status |
- Acute optic neuritis: Requires brain MRI showing (a) normal findings or only nonspecific white matter lesions, or (b) optic nerve MRI with T2-hyperintense lesion or T1-weighted gadolinium enhancing lesion extending over more than one-half the optic nerve length or involving optic chiasm
- Acute myelitis: Requires associated intramedullary MRI lesion extending over ≥3 contiguous segments (LETM) or ≥3 contiguous segments of focal spinal cord atrophy in patients with history compatible with acute myelitis
- Area postrema syndrome: Requires associated dorsal medulla/area postrema lesions
- Acute brainstem syndrome: Requires associated periependymal brainstem lesions
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