摘要视神经脊髓炎(NMO)是一种主要累及视神经和脊髓的中枢神经系统(CNS)自身免疫性炎症性疾病,其复发率高、预后差;其中NMO相关性视神经炎是引起患者视力严重下降的常见神经眼科疾病.水通道蛋白4(AQP4)抗体是NMO的一种特异性和致病性自身抗体.尽管AQP4可以在多个组织中表达,但NMO病变主要局限于CNS.目前NMO的治疗以皮质类固醇联合其他免疫抑制剂为主,以达到减少复发频率及急性发作严重程度的目的.多途径多视角的基础研究提高了我们对NMO发病机制的认识,有助于临床深入了解免疫机制和非免疫机制.临床治疗NMO的方法应该包括针对发病过程中各种途径的药物,而如何把这些药物从实验室转化到临床,并在临床研究的基础上确定更为安全有效的治疗方法,减轻患者CNS损害和维持视觉功能是今后研究值得探讨的重要课题.

abstractsNeuromyelitis optica (NMO) is an autoimmune inflammatory diseases of the central nervous systems (CNS) mainly affecting the optic nerves and spinal cord.It has the characteristics of high recurrence rate and poor prognosis.NMO related optic neuritis is a common neuro-ophthalmic disease which often results in permanent visual loss or even blindness.Aquaporin 4 (AQP4) antibody is a specific and pathogenic autoantibody in NMO patients.Although AQP4 is expressed in multiple tissues,NMO pathology is remarkably limited to the CNS.Corticosteroids and other immunosuppressive drugs are the standard managements for NMO patients,in order to reduce the relapses and the severity of the acute attack.Multiple avenues of investigation in the laboratory have significantly advanced our understanding of NMO pathophysiology,which is helpful for our understanding of immunologic and nonimmunologic mechanisms.Many offer significant means for NMO therapy by selectively targeting pathways.In the future,moving these agents from the bench to the bedside offers the opportunity to identify safe and effective therapies that limit CNS injury and preserve visual function.