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视神经脊髓炎谱系疾病和髓鞘少突胶质细胞糖蛋白相关疾病-视神经炎:诊断和治疗的综合综述。

Neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein associated disorder-optic neuritis: a comprehensive review of diagnosis and treatment.

机构信息

Department of Ophthalmology, Duke University School of Medicine, Durham, NC, USA.

Departments of Ophthalmology and Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.

出版信息

Eye (Lond). 2021 Mar;35(3):753-768. doi: 10.1038/s41433-020-01334-8. Epub 2020 Dec 15.

Abstract

Optic neuritis (ON) is the most common cause of acute optic neuropathy in patients younger than 50 years of age and is most frequently idiopathic or associated with multiple sclerosis. However, the discovery of aquaporin-4 immunoglobulin G (IgG) and myelin oligodendrocyte glycoprotein (MOG)-IgG as biomarkers for two separate central nervous system inflammatory demyelinating diseases has revealed that neuromyelitis optica spectrum disorder (NMSOD) and MOG-IgG-associated disease (MOGAD) are responsible for clinically distinct subsets of ON. NMOSD-ON and MOGAD-ON both demonstrate tendencies for bilateral optic nerve involvement and often exhibit a relapsing course with the potential for devastating long-term visual outcomes. Early and accurate diagnosis is therefore essential. This review will summarize the current understanding of the clinical spectra of NMOSD and MOGAD, the radiographic and serological findings which support their diagnoses, and the current evidence behind various acute and long-term therapeutic strategies for ON related to these conditions. A particular emphasis is placed on a number of recent multi-centre randomized placebo-controlled trials, which provide the first level I evidence for long-term treatment of NMOSD.

摘要

视神经炎(ON)是 50 岁以下患者急性视神经病变的最常见原因,最常为特发性或与多发性硬化症相关。然而,水通道蛋白-4 免疫球蛋白 G(IgG)和髓鞘少突胶质细胞糖蛋白(MOG)-IgG 作为两种独立的中枢神经系统炎症性脱髓鞘疾病的生物标志物的发现表明,视神经脊髓炎谱系障碍(NMOSD)和 MOG-IgG 相关疾病(MOGAD)是导致 ON 临床不同亚群的原因。NMOSD-ON 和 MOGAD-ON 均表现出双侧视神经受累的倾向,且常呈复发病程,具有潜在的破坏性长期视力结局。因此,早期和准确的诊断至关重要。这篇综述将总结 NMOSD 和 MOGAD 的临床谱、支持其诊断的影像学和血清学发现,以及针对这些疾病的 ON 的各种急性和长期治疗策略的现有证据。特别强调了一些最近的多中心随机安慰剂对照试验,这些试验为 NMOSD 的长期治疗提供了首个一级证据。

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