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Adv Immunol. 2014;121:213-42. doi: 10.1016/B978-0-12-800100-4.00006-4.

Immunopathogenesis of neuromyelitis optica.

Author information

  • 1Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA. Electronic address: mlevy@jhmi.edu.
  • 2Division of Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany.
  • 3Department of Neurology & Neurotherapeutics, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA.
  • 4Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
  • 5Department of Neuropathology, University Medical Center, Georg August University, Göttingen, Germany; Department of Neurology, University Medical Center, Georg August University, Göttingen, Germany.
  • 6Department of Neurology & Neurotherapeutics, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA; Neurology Section, VA North Texas Health Care System, Medical Service, Dallas, Texas, USA; Department of Neurology, Klinikum rechts der Isar, Technische Universität München, München, Germany; Department of Neurology, Heinrich Heine University Düsseldorf, Düsseldorf, Germany. Electronic address: olaf.stuve@utsouthwestern.edu.

Abstract

Neuromyelitis optica (NMO, Devic's syndrome) is a clinical syndrome characterized by optic neuritis and (mostly longitudinally extensive) myelitis. If untreated, NMO usually takes a relapsing course and often results in blindness and tetra- or paraparesis. The discovery of autoantibodies to aquaporin-4, the most abundant water channel in the CNS, in 70-80% of patients with NMO (termed NMO-IgG or AQP4-Ab) and subsequent investigations into the pathogenic impact of this new reactivity have led to the recognition of NMO as an autoimmune condition and as a disease entity in its own right, distinct from classic multiple sclerosis. Here, we comprehensively review the current knowledge on the role of NMO-IgG/AQP4-Ab, B cells, T cells, and the innate immune system in the pathogenesis of NMO.

© 2014 Elsevier Inc. All rights reserved.

KEYWORDS:

Aquaporin-4; Immunopathogenesis; NMO-IgG; Neuromyelitis optica

PMID:
24388217
[PubMed - indexed for MEDLINE]
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