Immunopathogenesis of neuromyelitis optica

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

doi:10.1016/B978-0-12-800100-4.00006-4

Immunopathogenesis of neuromyelitis optica

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

Authors

Michael Levy¹, Brigitte Wildemann², Sven Jarius², Benjamine Orellano³, Saranya Sasidharan⁴, Martin S Weber⁵, Olaf Stuve⁶

Affiliations

¹ Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA. Electronic address: mlevy@jhmi.edu.
² Division of Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany.
³ Department of Neurology & Neurotherapeutics, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA.
⁴ Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
⁵ Department of Neuropathology, University Medical Center, Georg August University, Göttingen, Germany; Department of Neurology, University Medical Center, Georg August University, Göttingen, Germany.
⁶ 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.

PMID: 24388217
DOI: 10.1016/B978-0-12-800100-4.00006-4

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.

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

Substances

Aquaporin 4
Autoantibodies