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重症肌无力病史再探。

History of Myasthenia Gravis Revisited.

作者信息

Deymeer Feza

机构信息

İstanbul University Faculty of Medicine Retired Faculty Member, İstanbul, Turkey.

出版信息

Noro Psikiyatr Ars. 2020 Nov 7;58(2):154-162. doi: 10.29399/npa.27315. eCollection 2021 Jun.

Abstract

The first description of myasthenia gravis (MG) was given by Thomas Willis in 1672. MG was the focus of attention after mid-nineteenth century and a great amount of information has been accumulated in a span of 150 years. The aim of this review is to convey this information according to a particular systematic and to briefly relate the experience of Istanbul University. MG history was examined in four periods: 1868-1930, 1930-1960, 1960-1990, and 1990-2020. In , all the clinical characteristics of MG were defined. Physiological/pharmacological studies on the transmission at the neuromuscular junction were initiated, and the concept of repetitive nerve stimulation emerged. A toxic agent was believed to be the cause of MG which appeared to resemble curare intoxication. Association of MG with thymus was noticed. No noteworthy progress was made in its treatment. In , acetylcholine was discovered to be the transmitter at the neuromuscular junction. Repetitive nerve stimulation was used as a diagnostic test. The autoimmune nature of MG was suspected and experiments to this end started to give results. The hallmark of this period was the use of anticholinesterases and thymectomy in the treatment of MG. can probably be considered a revolutionary era for MG. Important immunological mechanisms (acetylcholine receptor isolation, discovery of anti-acetylcholine receptor antibodies) were clarified and the autoimmune nature of MG was demonstrated. Treatment modalities which completely changed the prognosis of MG, including positive pressure mechanic ventilation and corticosteroids as well as plasma exchange/IVIg and azathioprine, were put to use. In , more immunological progress, including the discovery of anti-MuSK antibodies, was achieved. Videothoracoscopic thymectomy reduced the morbidity and mortality rate associated with surgery. New drugs emerged and clinical trials were performed. Valuable guidelines were published. In the last part of the review, the experience in MG of Istanbul University, a pioneer in Turkey, is related.

摘要

重症肌无力(MG)的首次描述由托马斯·威利斯于1672年作出。19世纪中叶之后,MG成为关注焦点,在150年的时间里积累了大量信息。本综述的目的是按照特定体系传达这些信息,并简要介绍伊斯坦布尔大学的经验。MG的历史分为四个时期进行考察:1868年至1930年、1930年至1960年、1960年至1990年以及1990年至2020年。在第一个时期,MG的所有临床特征得以明确。针对神经肌肉接头处传递的生理/药理研究启动,重复神经刺激的概念应运而生。当时认为一种毒性物质是MG的病因,其表现似乎类似于箭毒中毒。人们注意到MG与胸腺有关联。在治疗方面没有取得显著进展。在第二个时期,乙酰胆碱被发现是神经肌肉接头处的递质。重复神经刺激被用作诊断测试。人们怀疑MG的自身免疫性质,为此开展的实验开始得出结果。这个时期的标志是使用抗胆碱酯酶药物和胸腺切除术治疗MG。第三个时期可能被视为MG的一个变革时代。重要的免疫机制(乙酰胆碱受体分离、抗乙酰胆碱受体抗体的发现)得以阐明,MG的自身免疫性质得到证实。彻底改变MG预后的治疗方式开始应用,包括正压机械通气、皮质类固醇以及血浆置换/静脉注射免疫球蛋白和硫唑嘌呤。在第四个时期,取得了更多免疫方面的进展,包括抗肌肉特异性激酶(MuSK)抗体的发现。电视胸腔镜胸腺切除术降低了与手术相关的发病率和死亡率。新型药物出现并开展了临床试验。有价值的指南得以发布。在综述的最后部分,介绍了土耳其的先驱伊斯坦布尔大学在MG方面的经验。

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