Neuromyelitis optica: Changing concepts

Abstract Neuromyelitis optica (NMO; Devic's disease) and the NMO spectrum disorders are idiopathic inflammatory demyelinating disorders that affect the central nervous system and have a predilection for optic nerves and spinal cord. The identification of NMO-IgG as a disease-specific marker and...

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Bibliographic Details
Published in:Journal of neuroimmunology 2007-07, Vol.187 (1), p.126-138
Main Authors: Jacob, Anu, Matiello, Marcelo, Wingerchuk, Dean M, Lucchinetti, Claudia F, Pittock, Sean J, Weinshenker, Brian G
Format: Article
Language:English
Subjects:
Allergy and Immunology
Animals
Aquaporin
Aquaporin 4 - genetics
Aquaporin 4 - metabolism
Devic's
Disease Models, Animal
Humans
Multiple sclerosis
Neurology
Neuromyelitis optica
Neuromyelitis Optica - epidemiology
Neuromyelitis Optica - genetics
Neuromyelitis Optica - immunology
Neuromyelitis Optica - pathology
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Summary:Abstract Neuromyelitis optica (NMO; Devic's disease) and the NMO spectrum disorders are idiopathic inflammatory demyelinating disorders that affect the central nervous system and have a predilection for optic nerves and spinal cord. The identification of NMO-IgG as a disease-specific marker and aquaporin 4 as the target antigen has renewed interest in NMO. Based on current data, we suspect that autoantibodies arising from peripheral B cells bind to aquaporin 4 expressed on astrocyte foot processes on the abluminal surface of microvessels, activate complement and initiate inflammatory demyelination and necrosis. The development of animal models and further analysis of the association of NMO-IgG with disease severity and treatment response will elucidate the pathobiology of NMO.
ISSN:0165-5728
1872-8421
DOI:10.1016/j.jneuroim.2007.04.009