眼重症肌无力:当前综述。

IF 3.1 Q1 OPHTHALMOLOGY Eye and Brain Pub Date : 2023-01-01 DOI:10.2147/EB.S389629
Raed Behbehani
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引用次数: 0

摘要

眼重症肌无力(OMG)是一种神经肌肉疾病,其特征是在神经肌肉连接处产生针对突触后蛋白的自身抗体。肌无力的病理生理自身免疫机制是多种多样的,这主要是由自身抗体产生的类型决定的。OMG的诊断主要依赖于临床评估,使用血清抗体检测乙酰胆碱受体(AchR)、肌肉特异性酪氨酸激酶(MusK)和低密度脂蛋白4 (LPR4)。其他针对突触后蛋白的自身抗体,如皮质蛋白和agrin,已经被检测到;但其诊断价值和致病作用尚不明确。临床试验,如冰试验和电生理试验,特别是单纤维肌电图,在诊断中具有重要作用。OMG的治疗主要是通过胆碱酯酶抑制剂(吡哆斯的明),在眼麻痹的病例中经常需要类固醇。其他免疫抑制疗法包括抗代谢物(硫唑嘌呤、霉酚酸酯、甲氨蝶呤)和生物制剂,如b细胞消耗剂(利妥昔单抗)和补体抑制剂(eculizumab)。免疫抑制疗法对OMG自然病程的影响尚缺乏证据。临床医生必须警惕使用免疫检查抑制剂的患者出现肌无力综合征。需要可靠和一致的生物标志物来评估疾病严重程度和对治疗的反应,以优化OMG的管理。本综述的目的是总结OMG诊断和治疗的当前趋势和最新进展。
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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Ocular Myasthenia Gravis: A Current Overview.

Ocular myasthenia gravis (OMG) is a neuromuscular disease characterized by autoantibody production against post-synaptic proteins in the neuromuscular junction. The pathophysiological auto-immune mechanisms of myasthenia are diverse, and this is governed primarily by the type of autoantibody production. The diagnosis of OMG relies mainly on clinical assessment, the use of serological antibody assays for acetylcholine receptors (AchR), muscle-specific tyrosine kinase (MusK), and low-density lipoprotein 4 (LPR4). Other autoantibodies against post-synaptic proteins, such as cortactin and agrin, have been detected; however, their diagnostic value and pathogenic effect are not yet clearly defined. Clinical tests such as the ice test and electrophysiologic tests, particularly single-fiber electromyography, have a valuable role in diagnosis. The treatment of OMG is primarily through cholinesterase inhibitors (pyridostigmine), and steroids are frequently required in cases of ophthalmoplegia. Other immunosuppressive therapies include antimetabolites (azathioprine, mycophenolate mofetil, methotrexate) and biological agents such as B-cell depleting agents (Rituximab) and complement inhibitors (eculizumab). Evidence is scarce on the effect of immunosuppressive therapy on altering the natural course of OMG. Clinicians must be vigilant of a myasthenic syndrome in patients using immune-check inhibitors. Reliable and consistent biomarkers are required to assess disease severity and response to therapy to optimize the management of OMG. The purpose of this review is to summarize the current trends and the latest developments in diagnosing and treating OMG.

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来源期刊
Eye and Brain
Eye and Brain OPHTHALMOLOGY-
CiteScore
7.90
自引率
2.30%
发文量
12
审稿时长
16 weeks
期刊介绍: Eye and Brain is an international, peer-reviewed, open access journal focusing on basic research, clinical findings, and expert reviews in the field of visual science and neuro-ophthalmology. The journal’s unique focus is the link between two well-known visual centres, the eye and the brain, with an emphasis on the importance of such connections. All aspects of clinical and especially basic research on the visual system are addressed within the journal as well as significant future directions in vision research and therapeutic measures. This unique journal focuses on neurological aspects of vision – both physiological and pathological. The scope of the journal spans from the cornea to the associational visual cortex and all the visual centers in between. Topics range from basic biological mechanisms to therapeutic treatment, from simple organisms to humans, and utilizing techniques from molecular biology to behavior. The journal especially welcomes primary research articles or review papers that make the connection between the eye and the brain. Specific areas covered in the journal include: Physiology and pathophysiology of visual centers, Eye movement disorders and strabismus, Cellular, biochemical, and molecular features of the visual system, Structural and functional organization of the eye and of the visual cortex, Metabolic demands of the visual system, Diseases and disorders with neuro-ophthalmic manifestations, Clinical and experimental neuro-ophthalmology and visual system pathologies, Epidemiological studies.
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