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Neuromyelitis optica

Item Type:Review
Title:Neuromyelitis optica
Creators Name:Wildemann, B. and Jarius, S. and Paul, F.
Abstract:Neuromyelitis optica (NMO, Devic syndrome) is an autoimmune-mediated disorder of the central nervous system (CNS) with optic neuritis and longitudinally extensive transverse myelitis as key clinical features. The presence of highly specific serum autoantibodies, which target the water channel aquaporin-4 (AQP4), classifies NMO as a distinct entity and as the first inflammatory multiple sclerosis-like disorder of the CNS with a defined autoantigen. Recent observations strongly suggest that the AQP4 antibodies are pathophysiologically relevant and thus provide a rationale for the use of therapeutic strategies directed predominantly against the humoral effector arm of autoimmune responses such as plasma exchange or pharmacological B-cell depletion.
Keywords:Neuromyelitis Optica, Devic Syndrome, Antibodies to Aquaporin-4 (NMO-IgG), Longitudinally Extensive Transverse Myelitis, Optic Neuritis
Source:Aktuelle Neurologie
ISSN:0302-4350
Publisher:Thieme
Volume:39
Number:1
Page Range:33-41
Date:February 2012
Official Publication:https://doi.org/10.1055/s-0031-1297261

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