Immunopathogenesis of Neuromyelitis Optica

Michael Levy, Brigitte Wildemann, Sven Jarius, Benjamine Orellano, Saranya Sasidharan, Martin S. Weber, Olaf Stuve

Research output: Contribution to journalArticlepeer-review

52 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)213-242
Number of pages30
JournalAdvances in Immunology
StatePublished - 2014


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

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy


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