Department of Multiple Sclerosis Therapeutics, Fukushima Medical University School of Medicine and Southern TOHOKU Research Institute for Neuroscience, Koriyama, Japan.
Curr Opin Neurol. 2019 Jun;32(3):385-394. doi: 10.1097/WCO.0000000000000694.
The diagnostic criteria of neuromyelitis optica spectrum disorders (NMOSD) has been revised in the past 20 years and pathological and therapeutic data have been accumulated. This review provides an overview of evolution and broadening of the concept of NMOSD.
NMOSD encompassing brain syndrome as well as optic neuritis and acute myelitis is now classified into aquaporine-4 (AQP)-antibody-seropositive and aquaporine-4 (AQP)-antibody-seronegative diseases, detecting more patients earlier than before. Seronegative NMOSD includes cases of myelin oligodendrocyte glycoprotein (MOG)-antibody-seropositive disease with its unique clinical spectrum somewhat different from AQP4-antibody-seropositive NMOSD. Pathologically, NMOSD includes AQP4-antibody-seropositive autoimmune astrocytopathic disease and MOG-antibody-seropositive inflammatory demyelinating disease. Double seronegative group needs further research. Therapeutic options of NMOSD has taken shape and first-ever clinical trials of monoclonal antibodies have been done. In retrospect, relapsing NMO in the studies preceding the discovery of AQP4-antibody had features of AQP4-antibody-seropositive NMO whereas monophasic NMO was similar to AQP4-antibody-seronegative/MOG-antibody-seropositive NMO.
The clinical, pathological and therapeutic concepts of NMOSD have evolved and broadened over the last two decades following the detection of AQP4 antibodies and MOG antibodies in the patients. Double seronegative NMOSD is a current research focus, but now we may need to reconsider how NMOSD should be defined.
视神经脊髓炎谱系疾病（NMOSD）的诊断标准在过去 20 年中经历了修订，同时也积累了病理学和治疗数据。本文综述了 NMOSD 概念的演变和扩展。
NMOSD 涵盖了脑病综合征以及视神经炎和急性脊髓炎，现在分为水通道蛋白 4（AQP4）-抗体阳性和水通道蛋白 4（AQP4）-抗体阴性疾病，可以比以前更早地发现更多的患者。血清阴性 NMOSD 包括髓鞘少突胶质细胞糖蛋白（MOG）-抗体阳性疾病，其独特的临床谱与 AQP4 抗体阳性 NMOSD 略有不同。从病理学角度来看，NMOSD 包括 AQP4 抗体阳性自身免疫性星形胶质细胞病和 MOG 抗体阳性炎症性脱髓鞘病。双阴性组需要进一步研究。NMOSD 的治疗选择已经形成，并且已经进行了首个单克隆抗体的临床试验。回顾过去，在发现 AQP4 抗体之前的研究中，复发性 NMO 具有 AQP4 抗体阳性 NMO 的特征，而单相 NMO 与 AQP4 抗体阴性/MOG 抗体阳性 NMO 相似。
在发现 AQP4 抗体和 MOG 抗体后，NMOSD 的临床、病理和治疗概念在过去 20 年中得到了发展和扩展。双阴性 NMOSD 是当前的研究重点，但现在我们可能需要重新考虑如何定义 NMOSD。
