title: Immunopathogenesis of neuromyelitis optica.
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.
authors.affiliation: Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA. Electronic address: mlevy@jhmi.edu.
authors.forename: Michael
authors.initials: M
authors.lastname: Levy
authors.affiliation: Division of Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Wildemann
authors.affiliation: Division of Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany.
authors.forename: Sven
authors.initials: S
authors.lastname: Jarius
authors.affiliation: Department of Neurology & Neurotherapeutics, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA.
authors.forename: Benjamine
authors.initials: B
authors.lastname: Orellano
authors.affiliation: Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
authors.forename: Saranya
authors.initials: S
authors.lastname: Sasidharan
authors.affiliation: Department of Neuropathology, University Medical Center, Georg August University, Göttingen, Germany; Department of Neurology, University Medical Center, Georg August University, Göttingen, Germany.
authors.forename: Martin S
authors.initials: MS
authors.lastname: Weber
authors.affiliation: Department 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.
authors.forename: Olaf
authors.initials: O
authors.lastname: Stuve
mesh_terms: D000818:Animals; D000920:Antibody-Dependent Cell Cytotoxicity / Q000276:immunology*; D051401:Aquaporin 4 / Q000009:adverse effects / Q000276:immunology*; D001323:Autoantibodies / Q000096:biosynthesis* / Q000502:physiology; D016175:B-Lymphocyte Subsets / Q000276:immunology / Q000473:pathology; D006801:Humans; D007113:Immunity, Innate; D009471:Neuromyelitis Optica / Q000209:etiology / Q000276:immunology* / Q000473:pathology*; D016176:T-Lymphocyte Subsets / Q000276:immunology / Q000473:pathology
chemical_list: D051401:Aquaporin 4; D001323:Autoantibodies
keywords: Aquaporin-4; Immunopathogenesis; NMO-IgG; Neuromyelitis optica