The spectrum of neuromyelitis optica

Summary Neuromyelitis optica (also known as Devic's disease) is an idiopathic, severe, demyelinating disease of the central nervous system that preferentially affects the optic nerve and spinal cord. Neuromyelitis optica has a worldwide distribution, poor prognosis, and has long been thought of...

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Bibliographic Details
Published in:Lancet neurology 2007-09, Vol.6 (9), p.805-815
Main Authors: Wingerchuk, Dean M, MD, Lennon, Vanda A, PhD, Lucchinetti, Claudia F, MD, Pittock, Sean J, MD, Weinshenker, Brian G, MD
Format: Article
Language:English
Subjects:
Animals
Aquaporin 4 - immunology
Aquaporins
Autoantibodies - blood
Autoimmune diseases
Brain - pathology
Disease
Disease Progression
Female
Humans
Laboratories
Magnetic resonance imaging
Magnetic Resonance Imaging - methods
Male
Medical imaging
Models, Immunological
Multiple sclerosis
Nervous system
Neuroimaging
Neurology
Neuromyelitis Optica - blood
Neuromyelitis Optica - immunology
Neuromyelitis Optica - pathology
Neuromyelitis Optica - therapy
Neutrophils
Spinal cord
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Summary:Summary Neuromyelitis optica (also known as Devic's disease) is an idiopathic, severe, demyelinating disease of the central nervous system that preferentially affects the optic nerve and spinal cord. Neuromyelitis optica has a worldwide distribution, poor prognosis, and has long been thought of as a variant of multiple sclerosis; however, clinical, laboratory, immunological, and pathological characteristics that distinguish it from multiple sclerosis are now recognised. The presence of a highly specific serum autoantibody marker (NMO-IgG) further differentiates neuromyelitis optica from multiple sclerosis and has helped to define a neuromyelitis optica spectrum of disorders. NMO-IgG reacts with the water channel aquaporin 4. Data suggest that autoantibodies to aquaporin 4 derived from peripheral B cells cause the activation of complement, inflammatory demyelination, and necrosis that is seen in neuromyelitis optica. The knowledge gained from further assessment of the exact role of NMO-IgG in the pathogenesis of neuromyelitis optica will provide a foundation for rational therapeutic trials for this rapidly disabling disease.
ISSN:1474-4422
1474-4465
DOI:10.1016/S1474-4422(07)70216-8