International panel for NMO diagnosis criteria

Last revised by Bahman Rasuli on 12 Apr 2022

The 2015 International Panel for NMO Diagnosis criteria is, as the name implies, a set of criteria used to diagnose neuromyelitis optica spectrum disorder (NMOSD) 1. They are based on a combination of clinical, laboratory, and neuroimaging findings.

Criteria

Diagnostic criteria for NMOSD with AQP4-IgG +
  • at least one core clinical characteristic
  • positive AQP4-IgG test
  • exclusion of alternative diagnoses
Diagnostic criteria for NMOSD without AQP4-IgG + or NMOSD with an unknown AQP4-IgG status
  • at least 2 core clinical characteristics following one or more clinical episodes and meeting all the following requirements:
    • at least one of the core clinical characteristic is: optic neuritis, acute myelitis with longitudinally extensive transverse myelitis (LETM) or area postrema syndrome
    • dissemination in space (two or more different core clinical characteristics)
    • fulfillment of additional MRI requirements, as applicable 
  • negative AQP4-IgG test or testing unavailable
  • exclusion of alternative diagnoses
Core clinical characteristics
  • optic neuritis
  • acute myelitis
  • area postrema syndrome
  • acute brainstem syndrome
  • symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic lesions on MRI
  • symptomatic cerebral syndrome with NMOSD-typical brain lesions
Additional imaging (MRI) requirements for NMOSD without AQP4-IgG + and NMOSD with an unknown AQP4-IgG status
  • acute optic neuritis
  • acute myelitis
    • requires associated intramedullary MRI lesion extending over more than 3 contiguous segments (LETM) or more than 3 contiguous segments of focal spinal cord atrophy in patients with a history compatible with acute myelitis
  • area postrema syndrome
    • requires associated dorsal medulla/area postrema lesions
  • acute brainstem syndrome
    • requires associated periependymal brain lesions

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