视神经脊髓炎
多发性硬化
医学
水通道蛋白4
自身抗体
脱髓鞘病
视神经炎
免疫学
光谱紊乱
视神经
病理
抗体
眼科
精神科
作者
Dean M. Wingerchuk,Vanda A. Lennon,Claudia F. Lucchinetti,Sean J. Pittock,Brian G. Weinshenker
标识
DOI:10.1016/s1474-4422(07)70216-8
摘要
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.
科研通智能强力驱动
Strongly Powered by AbleSci AI