Criteria

Transverse Myelitis Consortium Working Group[1][23]

Inclusion criteria:

  • Development of sensory, motor, or autonomic dysfunction attributable to the spinal cord

  • Bilateral signs and/or symptoms (though not necessarily symmetric)

  • Clearly defined sensory level

  • Exclusion of extra-axial compressive etiology by neuroimaging (magnetic resonance imaging [MRI] or computed tomography [CT] myelography)

  • Inflammation of the spinal cord demonstrated by cerebrospinal pleocytosis, elevated IgG index, or MRI gadolinium enhancement

  • Progression to nadir between 4 hours and 21 days following the onset of symptoms.

Exclusion criteria:

  • History of previous radiation to the spine within the last 10 years

  • Clear arterial distribution consistent with thrombosis of the anterior spinal artery

  • Abnormal flow voids on the spinal cord surface consistent with dural-based arteriovenous fistula

  • Serologic or clinical evidence of connective tissue disease

  • Central nervous system manifestations of syphilis, Lyme disease, HIV, human T-lymphotropic virus-1, Mycoplasma, other viral infection

  • Brain MRI abnormalities suggestive of multiple sclerosis (MS)

  • History of clinically apparent optic neuritis.

Criteria for diagnosis of neuromyelitis optica spectrum disorder (NMOSD)[74]

Core clinical characteristics

  1. Optic neuritis

  2. Acute myelitis

  3. Area postrema syndrome: episode of otherwise unexplained hiccups or nausea and vomiting

  4. Acute brain stem syndrome

  5. Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions

  6. Symptomatic cerebral syndrome with NMOSD-typical brain lesions

Diagnostic criteria for NMOSD with anti-aquaporin-4 IgG (AQP4-IgG)

  1. At least 1 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 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 longitudinally extensive TM (LETM), or area postrema syndrome

    • Dissemination in space (2 or more different core clinical characteristics)

    • Fulfillment of additional MRI requirements, as applicable

  2. Negative test(s) for AQP4-IgG using best available detection method, or testing unavailable

  3. Exclusion of alternative diagnoses

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 >1/2 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 prior history compatible with acute myelitis

  3. Area postrema syndrome: requires associated dorsal medulla/area postrema lesions

  4. Acute brain stem syndrome: requires associated peri-ependymal brain stem lesions

The 2017 revised McDonald diagnostic criteria for MS[75]

[Figure caption and citation for the preceding image starts]: The McDonald criteria for the diagnosis of multiple sclerosisCreated using data from Thompson AJ, et al. Lancet Neurol 2018;17:162–73 [Citation ends].com.bmj.content.model.Caption@3f0531ca

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