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Mechanisms of disease: aquaporin-4 antibodies in neuromyelitis optica

Item Type:Review
Title:Mechanisms of disease: aquaporin-4 antibodies in neuromyelitis optica
Creators Name:Jarius, S. and Paul, F. and Franciotta, D. and Waters, P. and Zipp, F. and Hohlfeld, R. and Vincent, A. and Wildemann, B.
Abstract:Neuromyelitis optica (NMO) is a rare CNS inflammatory disorder that predominantly affects the optic nerves and spinal cord. Recent serological findings strongly suggest that NMO is a distinct disease rather than a subtype of multiple sclerosis. In NMO, serum antibodies, collectively known as NMO-IgG, characteristically bind to cerebral microvessels, pia. mater and Virchow-Robin spaces. The main target antigen for this immunoreactivity has been identified as aquaporin-4 (AQP4). The antibodies are highly specific for NMO, and they are also found in patients with longitudinally extensive transverse myelitis without optic neuritis, which is thought to be a precursor to NMO in some cases. An antibody-mediated pathogenesis for NMO is supported by several observations, including the characteristics of the AQP4 antibodies, the distinct NMO pathology-which includes IgG and complement deposition and loss of AQP4 from spinal cord lesions-and emerging evidence of the beneficial effects of B-cell depletion and plasma exchange. Many aspects of the pathogenesis, however, remain unclear.
Keywords:Aquaporin-4 Antibodies, Devic Syndrome, Multiple Sclerosis, Neuromyelitis Optica, Pathogenesis
Source:Nature Clinical Practice Neurology
ISSN:1745-834X
Publisher:Nature Publishing Group (U.S.A.)
Volume:4
Number:4
Page Range:202-214
Date:April 2008
Official Publication:https://doi.org/10.1038/ncpneuro0764
PubMed:View item in PubMed

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