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Neuromyelitis Optica: Immunopathogenesis, Clinical Manifestations, and Treatments

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Book cover Inflammatory Disorders of the Nervous System

Part of the book series: Current Clinical Neurology ((CCNEU))

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Abstract

Neuromyelitis optica (NMO) is an autoimmune inflammatory astrocytopathy characterized by severe optic neuritis, longitudinally extensive transverse myelitis, and the area postrema syndrome. Complement-fixing antibodies directed against aquaporin-4 (a water channel located on astrocytic foot processes) are generated from dysregulated B-cells in NMO. NMO typically affects young women and shows no racial predilection. It often coexists with other autoimmune diseases. The diagnosis of NMO can be made based on clinical manifestations, radiologic data, and presence of antibodies against aquaporin-4 antibody. Acute NMO attacks should be treated with high-dose intravenous corticosteroids and/or plasmapheresis. Long-term control of disease activity can be achieved with rituximab, as well as various immunosuppressive therapies.

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Authors and Affiliations

  1. Department of Neurology and Neurotherapeutics, Multiple Sclerosis & Neuroimmunology Program, University of Texas Southwestern School of Medicine, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA

    Shin C. Beh MD

  2. Department of Neurology and Neurotherapeutics, University of Texas Southwestern School of Medicine, Dallas, TX, USA

    Teresa C. Frohman PA-C & Elliot M. Frohman MD, PhD

  3. Department of Ophthalmology, University of Texas Southwestern School of Medicine, Dallas, TX, USA

    Elliot M. Frohman MD, PhD

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  1. Shin C. Beh MD
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  2. Teresa C. Frohman PA-C
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Correspondence to Shin C. Beh MD .

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Editors and Affiliations

  1. Department of Neurology, LSU Health Sciences Center, Shreveport, Louisiana, USA

    Alireza Minagar

  2. Department of Molecular and Cellular Physiology, LSU Health Sciences Center, Shreveport, Louisiana, USA

    J. Steven Alexander

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Beh, S.C., Frohman, T.C., Frohman, E.M. (2017). Neuromyelitis Optica: Immunopathogenesis, Clinical Manifestations, and Treatments. In: Minagar, A., Alexander, J. (eds) Inflammatory Disorders of the Nervous System. Current Clinical Neurology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-51220-4_9

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  • DOI: https://doi.org/10.1007/978-3-319-51220-4_9

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