Immunopathogenesis of neuromyelitis optica

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


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.

Keywords: Aquaporin-4; Immunopathogenesis; NMO-IgG; Neuromyelitis optica.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Antibody-Dependent Cell Cytotoxicity / immunology*
  • Aquaporin 4 / adverse effects
  • Aquaporin 4 / immunology*
  • Autoantibodies / biosynthesis*
  • Autoantibodies / physiology
  • B-Lymphocyte Subsets / immunology
  • B-Lymphocyte Subsets / pathology
  • Humans
  • Immunity, Innate
  • Neuromyelitis Optica / etiology
  • Neuromyelitis Optica / immunology*
  • Neuromyelitis Optica / pathology*
  • T-Lymphocyte Subsets / immunology
  • T-Lymphocyte Subsets / pathology


  • Aquaporin 4
  • Autoantibodies