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For further information, see CMDT Part 24-20: Neuromyelitis Optica
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Previously known as Devic disease and once regarded as a variant of multiple sclerosis
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 one third of NMO-IgG seronegative patients
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Long-term immunosuppression; three medications are approved by the FDA and demonstrate a reduced annual relapse rate or time to first relapse
Eculizumab, a complement inhibitor; its use requires prior immunization against meningococcus
Inebilizumab, a humanized anti-CD19 antibody that depletes B cells
Satralizumab, an interleukin-6 receptor antagonist
Rituximab is first-line therapy
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
Alternatives include
Azathioprine, 2.5–3 mg/kg orally
Mycophenolate mofetil, 500–1500 mg orally twice daily, titrated until the absolute lymphocyte count falls below 1500/mcL)
Corticosteroids used for acute relapses
Plasma exchange given when severe relapses do not respond to corticosteroids