Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1, Inohana, Chuo-ku, Chiba 260-8670, Japan.
Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1, Inohana, Chuo-ku, Chiba 260-8670, Japan.
J Clin Neurosci. 2014 Jan;21(1):12-21. doi: 10.1016/j.jocn.2012.12.022. Epub 2013 Aug 2.
Neuromyelitis optica (NMO) is an inflammatory disorder of the central nervous system (CNS) that predominantly affects the optic nerves and spinal cord. Previously, it has been considered to be a severe variant of multiple sclerosis (MS), especially common in Asia. However, the finding that most NMO patients have autoantibodies against aquaporin-4 (AQP4) has improved our knowledge of its pathogenesis and led to the concept that NMO is a disease distinct from MS. Although the 2006 NMO revised criteria are useful for diagnosing NMO, their usefulness in the diagnosis of early-stage NMO is limited. Hence, there is an urgent need for new and more precise diagnostic methods. Interleukin-6 may play important roles in NMO pathogenesis, as it is involved in the survival of plasmablasts that produce anti-AQP4 antibody in the peripheral circulation and in the enhancement of inflammation in the CNS. Severe blood-brain barrier disruption in NMO allows the anti-AQP4 antibody to access the astrocytic endfeet. The anti-AQP4 antibody causes astrocytic damage through complement activation. Thus, NMO is an astrocytopathic, rather than a demyelinating, disease. Some brain lesions specific to NMO have recently been reported. Significant advances in the understanding of NMO pathogenesis are beginning to improve existing treatment strategies and will help develop new treatments. This review focuses on the current advances in NMO research and its clinical characteristics, immunological findings, neuroimaging and pathophysiology.
视神经脊髓炎（NMO）是一种中枢神经系统（CNS）的炎症性疾病，主要影响视神经和脊髓。以前，它被认为是多发性硬化症（MS）的严重变异，尤其常见于亚洲。然而，大多数 NMO 患者存在针对水通道蛋白-4（AQP4）的自身抗体的发现，提高了我们对其发病机制的认识，并导致 NMO 是一种与 MS 不同的疾病的概念。尽管 2006 年的 NMO 修订标准有助于诊断 NMO，但它们在早期 NMO 的诊断中的有用性有限。因此，迫切需要新的更精确的诊断方法。白细胞介素-6 可能在 NMO 发病机制中发挥重要作用，因为它参与了在外周循环中产生抗 AQP4 抗体的浆母细胞的存活，并增强了 CNS 中的炎症。NMO 中严重的血脑屏障破坏允许抗 AQP4 抗体进入星形胶质细胞足突。抗 AQP4 抗体通过补体激活引起星形胶质细胞损伤。因此，NMO 是一种星形胶质细胞病，而不是脱髓鞘疾病。最近报道了一些特定于 NMO 的脑损伤。对 NMO 发病机制的理解的重大进展开始改善现有的治疗策略，并将有助于开发新的治疗方法。这篇综述重点介绍了 NMO 研究的最新进展及其临床特征、免疫学发现、神经影像学和病理生理学。
