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This disorder is characterized by optic neuritis and acute myelitis with MRI changes that extend over at least three segments of the spinal cord. An isolated myelitis or optic neuritis may also occur. Previously known as Devic disease and once regarded as a variant of multiple sclerosis, neuromyelitis optica is associated with a specific antibody marker (NMO-IgG) targeting the water channel aquaporin-4 in 80% of cases, and with antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) in approximately 33% of NMO-IgG seronegative patients. MRI of the brain typically does not show widespread white matter involvement, but such changes do not exclude the diagnosis. Treatment is by long-term immunosuppression. First-line therapy is with rituximab (two 1 g intravenous infusions spaced by 2 weeks, or four weekly infusions of 375 mg/m2; re-dosing may occur every 6 months or when CD19/20-positive or CD27-positive lymphocytes become detectable), mycophenolate mofetil (500–1500 mg orally twice daily, titrated until the absolute lymphocyte count falls below 1500/mcL), or azathioprine (2.5–3 mg/kg orally). Acute relapses are treated with corticosteroids at doses similar to those outlined for multiple sclerosis and with plasma exchange for severe relapses unresponsive to corticosteroids.

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Nikoo  Z  et al. Comparison of the efficacy of azathioprine and rituximab in neuromyelitis optica spectrum disorder: a randomized clinical trial. J Neurol. 2017 Sep;264(9):2003–9.
[PubMed: 28831548]  
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Wingerchuk  DM  et al; International Panel for NMO Diagnosis. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015 Jul 14;85(2):177–89.
[PubMed: 26092914]  

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