The spectrum of neuromyelitis optica.
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.
Wingerchuk
Dean M
DM
Department of Neurology, Mayo Clinic College of Medicine, Scottsdale, AZ 85259, USA. wingerchuk.dean@mayo.edu
Lennon
Vanda A
VA
Lucchinetti
Claudia F
CF
Pittock
Sean J
SJ
Weinshenker
Brian G
BG
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*
C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies