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Table 2 Neuromyelitis optica spectrum disorders diagnostic criteria for adult patients

From: Demyelinating syndrome in systemic sclerosis and neuromyelitis optica

Diagnostic criteria for NMOSD with AQP4IgG
 1. At least one core clinical characteristic
 2. Positive test for AQP4IgG using best available detection method (cell-based assay strongly recommended)
 3. Exclusion of alternative diagnoses
Diagnostic criteria for NMOSD without AQP4IgG or NMOSD with unknown AQP4IgG 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 AQP4IgG 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 AQP4IgG and NMOSD with unknown AQP4IgG 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 T1weighted 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
  1. AQP4 aquaporin4, IgG immunoglobulin G, LETM longitudinally extensive transverse myelitis lesions, NMOSD neuromyelitis optica spectrum disorders