NMOSD 和 MOGAD:不断演变的疾病谱

IF 28.2 1区 医学 Q1 CLINICAL NEUROLOGY Nature Reviews Neurology Pub Date : 2024-09-13 DOI:10.1038/s41582-024-01014-1
Akiyuki Uzawa, Frederike Cosima Oertel, Masahiro Mori, Friedemann Paul, Satoshi Kuwabara
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摘要

神经脊髓炎视网膜(NMO)谱系障碍(NMOSD)是一种中枢神经系统的复发性炎症性疾病,其特征是存在血清水通道蛋白 4(AQP4)自身抗体(AQP4-IgGs)以及视神经炎、脊髓炎、脑或脑干综合征等核心临床表现。有些人表现出 NMOSD 的临床特征,但 AQP4-IgG 检测呈阴性,这些人中有一部分现在被认为具有针对髓鞘少突胶质细胞糖蛋白(MOG)的血清自身抗体,这种情况被称为 MOG 抗体相关疾病(MOGAD)。因此,NMOSD 的概念正在发生变化,出现了一种疾病谱,包括 AQP4-IgG 血清阳性 NMOSD、MOGAD 和双酮体阴性 NMOSD。MOGAD 与 NMOSD 具有相同的特征,包括视神经炎和脊髓炎,但其病理生理学、临床特征、神经影像学结果(包括急性播散性脑脊髓炎和/或皮质脑炎)和生物标志物却截然不同。AQP4-IgG-酮阴性 NMOSD 似乎是一种异质性疾病,需要进一步研究。MOGAD 可表现为单相或复发性疾病,而 NMOSD 通常是复发性的。本综述总结了 NMOSD 和 MOGAD 的历史和当前概念,比较了流行病学、临床特征、神经影像学、病理学和免疫学。此外,我们还讨论了针对补体、B 细胞或 IL-6 受体的 AQP4-IgG 血清阳性 NMOSD 的新型单克隆抗体疗法,这些疗法可能在不久的将来应用于 MOGAD。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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NMOSD and MOGAD: an evolving disease spectrum
Neuromyelitis optica (NMO) spectrum disorder (NMOSD) is a relapsing inflammatory disease of the CNS, characterized by the presence of serum aquaporin 4 (AQP4) autoantibodies (AQP4-IgGs) and core clinical manifestations such as optic neuritis, myelitis, and brain or brainstem syndromes. Some people exhibit clinical characteristics of NMOSD but test negative for AQP4-IgG, and a subset of these individuals are now recognized to have serum autoantibodies against myelin oligodendrocyte glycoprotein (MOG) — a condition termed MOG antibody-associated disease (MOGAD). Therefore, the concept of NMOSD is changing, with a disease spectrum emerging that includes AQP4-IgG-seropositive NMOSD, MOGAD and double-seronegative NMOSD. MOGAD shares features with NMOSD, including optic neuritis and myelitis, but has distinct pathophysiology, clinical profiles, neuroimaging findings (including acute disseminated encephalomyelitis and/or cortical encephalitis) and biomarkers. AQP4-IgG-seronegative NMOSD seems to be a heterogeneous condition and requires further study. MOGAD can manifest as either a monophasic or a relapsing disease, whereas NMOSD is usually relapsing. This Review summarizes the history and current concepts of NMOSD and MOGAD, comparing epidemiology, clinical features, neuroimaging, pathology and immunology. In addition, we discuss new monoclonal antibody therapies for AQP4-IgG-seropositive NMOSD that target complement, B cells or IL-6 receptors, which might be applied to MOGAD in the near future. This Review summarizes the history and current concepts of neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), including epidemiology, clinical and neuroimaging features and pathophysiology. It also discusses new molecularly targeted therapies for NMOSD that might be also applied to MOGAD in the future.
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来源期刊
Nature Reviews Neurology
Nature Reviews Neurology 医学-临床神经学
CiteScore
29.90
自引率
0.80%
发文量
138
审稿时长
6-12 weeks
期刊介绍: Nature Reviews Neurology aims to be the premier source of reviews and commentaries for the scientific and clinical communities we serve. We want to provide an unparalleled service to authors, referees, and readers, and we work hard to maximize the usefulness and impact of each article. The journal publishes Research Highlights, Comments, News & Views, Reviews, Consensus Statements, and Perspectives relevant to researchers and clinicians working in the field of neurology. Our broad scope ensures that the work we publish reaches the widest possible audience. Our articles are authoritative, accessible, and enhanced with clearly understandable figures, tables, and other display items. This page gives more detail about the aims and scope of the journal.
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