title: The spectrum of neuromyelitis optica.
abstract: 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.
authors: Department of Neurology, Mayo Clinic College of Medicine, Scottsdale, AZ 85259, USA. wingerchuk.dean@mayo.edu
authors: Dean M
authors: DM
authors: Wingerchuk
authors: Vanda A
authors: VA
authors: Lennon
authors: Claudia F
authors: CF
authors: Lucchinetti
authors: Sean J
authors: SJ
authors: Pittock
authors: Brian G
authors: BG
authors: Weinshenker
mesh_terms: D000818:Animals; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D001921:Brain / Q000473:pathology; D018450:Disease Progression; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D018448:Models, Immunological; D009471:Neuromyelitis Optica / Q000097:blood / Q000276:immunology* / Q000473:pathology* / Q000628:therapy*
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies