Jacob Anu, Matiello Marcelo, Wingerchuk Dean M, Lucchinetti Claudia F, Pittock Sean J, Weinshenker Brian G
Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
J Neuroimmunol. 2007 Jul;187(1-2):126-38. doi: 10.1016/j.jneuroim.2007.04.009. Epub 2007 May 21.
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.
视神经脊髓炎（NMO；德维克病）及视神经脊髓炎谱系障碍是特发性炎性脱髓鞘疾病，累及中枢神经系统，且好发于视神经和脊髓。NMO-IgG作为疾病特异性标志物以及水通道蛋白4作为靶抗原的发现，重新引发了人们对NMO的关注。基于目前的数据，我们怀疑外周B细胞产生的自身抗体与微血管无腔面星形胶质细胞足突上表达的水通道蛋白4结合，激活补体并引发炎性脱髓鞘和坏死。动物模型的建立以及对NMO-IgG与疾病严重程度及治疗反应之间关联的进一步分析，将阐明NMO的病理生物学机制。
