NMOSD diagnostic criteria
AQP4-IgG status
Positive
Negative
Unknown
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 on MRI
Additional MRI requirements for NMOSD without AQP4-IgG or 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 >1/2 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 oblongata/area postrema lesions
Acute brainstem syndrome: requires associated periependymal brainstem lesions
Alternative diagnoses excluded
Yes, no better explanation of the clinical syndrome exists
No, there are diagnoses that can better explain the clinical syndrome
References
International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology 2015 Jul 14; 85(2): 177–189
doi:
10.1212/WNL.0000000000001729
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