Version June 2024
| Diagnostic criteria for NMOSD with AQP4-IgG |
| 1. At least one core clinical characteristic |
| 2. Positive test for AQP4-IgG using best available detection method (cell-based assay strongly recommended) |
| 3. Exclusion of alternative diagnoses |
| Diagnostic criteria for NMOSD without AQP4-IgG or NMOSD with unknown AQP4-IgG status |
| 1. At least two core clinical characteristics occurring as a result of one or more clinical attacks and meeting all of the following requirements: |
| a. At least one core clinical characteristic must be optic neuritis, acute myelitis with LETM, or area postrema syndrome |
| b. Dissemination in space (two or more different core clinical characteristics) |
| c. Fulfillment of additional MRI requirements, as applicable |
| 2. Negative tests for AQP4-IgG using best available detection method, or testing unavailable |
| 3. Exclusion of alternative diagnoses |
| Core clinical characteristics |
| 1. Optic neuritis |
| 2. Acute myelitis |
| 3. Area postrema syndrome: Episode of otherwise unexplained hiccups or nausea and vomiting |
| 4. Acute brainstem syndrome |
| 5. Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions |
| 6. Symptomatic cerebral syndrome with NMOSD-typical brain lesions |
| Additional MRI requirements for NMOSD without AQP4-IgG and NMOSD with unknown AQP4-IgG status |
| 1. 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 |
| 2. 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 |
| 3. Area postrema syndrome: Requires associated dorsal medulla/area postrema lesions |
| 4. Acute brainstem syndrome: Requires associated periependymal brainstem lesions |
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