Department of Multiple Sclerosis Therapeutics, Fukushima Medical University School of Medicine and Southern TOHOKU Research Institute for Neuroscience, Koriyama, Japan.
University of Utah School of Medicine, Salt Lake City, Utah, USA.
Curr Opin Neurol. 2020 Jun;33(3):300-308. doi: 10.1097/WCO.0000000000000828.
We reviewed present topics on neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein (MOG)-antibody-associated disease (MOGAD).
The number of NMOSD-related publications have increased year by year after the discovery of aquaporin 4 (AQP4)-antibody, and those on MOGAD started to surge since 2012-2013. Recent clinic-epidemiological surveys in NMOSD suggest that some racial differences in the prevalence and the clinical course. At present, experts feel the 2015 diagnostic criteria of AQP4-antibody-seronegative NMOSD should be revised. Randomized controlled trials of monoclonal antibodies in NMOSD have demonstrated a significant risk reduction of relapse, especially in AQP4-antibody-positive cases. Meanwhile, the efficacy in seronegative NMOSD was unclear. MOGAD can show NMO and other clinical phenotypes, but the clinical manifestations and frequencies are different in children and adults. One pathological study has suggested that MOGAD is distinct from AQP4-antibody-positive NMOSD, but may share some features with multiple sclerosis and acute disseminated encephalomyelitis. Immunosuppressive therapy can reduce relapse in MOGAD, but, unlike AQP4-antibody-positive NMOSD, some MOGAD patients treated with rituximab experience relapses despite a complete B-cell depletion.
Our understanding and therapy of AQP4-antibody-positive NMOSD has made a significant progress, and recent research has identified challenges in seronegative NMOSD and MOGAD.
本文回顾了视神经脊髓炎谱系疾病（NMOSD）和髓鞘少突胶质细胞糖蛋白（MOG）抗体相关疾病（MOGAD）的现有研究热点。
水通道蛋白 4（AQP4）抗体发现后，NMOSD 相关出版物数量逐年增加，而 MOGAD 相关出版物自 2012-2013 年开始激增。最近的 NMOSD 临床流行病学调查表明，该疾病的患病率和临床病程存在一些种族差异。目前，专家认为应修订 2015 年 AQP4 抗体阴性 NMOSD 的诊断标准。NMOSD 的单克隆抗体随机对照试验表明，复发风险显著降低，尤其是在 AQP4 抗体阳性病例中。同时，阴性 NMOSD 的疗效尚不清楚。MOGAD 可表现为 NMO 和其他临床表型，但在儿童和成人中的临床表现和频率不同。一项病理学研究表明，MOGAD 与 AQP4 抗体阳性 NMOSD 不同，但可能与多发性硬化和急性播散性脑脊髓炎具有一些共同特征。免疫抑制治疗可降低 MOGAD 的复发率，但与 AQP4 抗体阳性 NMOSD 不同，一些接受利妥昔单抗治疗的 MOGAD 患者尽管 B 细胞完全耗竭仍会复发。
我们对 AQP4 抗体阳性 NMOSD 的认识和治疗取得了重大进展，最近的研究也提出了阴性 NMOSD 和 MOGAD 面临的挑战。
