Department of Neurology, Mayo Clinic, 13400 E Shea Boulevard, Scottsdale, AZ 85259, USA.
J Clin Immunol. 2013 Jan;33 Suppl 1:S33-7. doi: 10.1007/s10875-012-9796-7. Epub 2012 Sep 14.
Neuromyelitis optica (NMO) is an idiopathic central nervous system inflammatory demyelinating disease that causes optic neuritis, transverse myelitis, and other CNS syndromes. It is distinct from multiple sclerosis and is associated with autoantibodies that target aquaporin-4 (AQP4), an astrocyte water channel. Evidence indicating antibody-mediated immune injury in NMO includes its association with other autoimmune diseases, lesional pathology that reveals prominent complement activation and immunoglobulin deposition, pathogenic potential of AQP4 autoantibodies based on in vitro studies, and reports of putative animal models of the disease. The rationale and potential role for intravenous immunoglobulin in NMO will be discussed in the context of both relapse treatment and relapse prevention.
视神经脊髓炎（NMO）是一种特发性中枢神经系统炎症性脱髓鞘疾病，可引起视神经炎、横贯性脊髓炎和其他中枢神经系统综合征。它有别于多发性硬化症，与靶向水通道蛋白-4（AQP4）的自身抗体有关，AQP4 是一种星形胶质细胞水通道。表明 NMO 中存在抗体介导的免疫损伤的证据包括其与其他自身免疫性疾病的关联、病变病理学显示出明显的补体激活和免疫球蛋白沉积、基于体外研究的 AQP4 自身抗体的致病潜力，以及关于该病假定动物模型的报告。将在复发治疗和预防复发的背景下讨论静脉注射免疫球蛋白在 NMO 中的合理依据和潜在作用。
