Diagnostic Criteria for Neuromyelitis Optica Spectrum Disorder |
| Diagnostic Criteria for NMOSD with AQP4-IgG |
- At least 1 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 2 core clinical characteristics occurring as a result of one or more clinical attacks and meeting all of the following requirements:
- At least 1 core clinical characteristic must be optic neuritis, acute myelitis with LETM, or area postrema syndrome
- Dissemination in space (2 or more different clinical characteristics)
- Fulfillment of additional MRI requirements, as applicable
- Negative test 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 or 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 of T1-weighted gadolinium-enhancing lesion extending over >1/2 optic nerve length or involving optic chiasm
- Acute myelitis: requires associated intramedullary MRI lesion extending ≥3 contiguous segments (LETM) OR ≥3 contiguous segments of focal spinal cord atrophy in pts with history compatible with acute myelitis
- Area postrema syndrome requires associated dorsal medulla/area postrema lesions
- Acute brainstem syndrome requires periependymal brainstem lesions
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