Annals of Indian Academy of Neurology
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REVIEW ARTICLE
Year : 2015  |  Volume : 18  |  Issue : 5  |  Page : 16-23

Therapy of NMO spectrum disorders


1 Department of Neurology, Bangur Institute of Neurosciences, Kolkata, India
2 Department of Neurology, Vivekananda Institute of Medical Sciences, Kolkata, West Bengal, India

Correspondence Address:
Arabinda Mukherjee
40A, Lake Temple Road, Kolkata - 700 029, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.164818

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Neuromyelitis optica (NMO) is an autoimmune demyelinating condition of the central nervous system often associated with aquaporin-4 (AQP4) autoantibodies manifesting as severe optic neuritis and long segment myelitis with tendency to relapse. Seronegative patients and who do not meet the NMO criteria are classified as having NMO Spectrum Disorder (NMOSD), but are treated identically to clinically definite NMO. Acute relapse is treated with intravenous methylprednisolone for 5 days with or without subsequent treatment with plasma exchange (PE). This must be followed by oral steroid to prevent rebound worsening and further relapse. For relapse prevention, immunosuppressive agents that have been found to be effective are azathioprine, rituximab, mycophenolate mofetil, methotrexate, and mitoxantrone; although none of which have been validated in randomized, controlled trial. Some patients do relapse with monotherapy, and switching to more effective agent or use of combination therapy is beneficial in such situation. There is no consensus about the duration of preventive therapy, but generally 2-3 years of relapse-free period is considered the minimum, taking into account the risks of long-term toxicity of these agents.


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