Parr J R, Jayawant S
University of Oxford Department of Paediatrics, Children's Hospital, Oxford, UK.
Dev Med Child Neurol. 2007 Aug;49(8):629-35. doi: 10.1111/j.1469-8749.2007.00629.x.
In recent years, understanding of the pathogenesis and clinical presentation of distinct myasthenia subtypes has increased significantly. This article reviews the clinical manifestations of autoimmune myasthenia gravis (including myasthenia associated with anti-muscle-specific kinase antibodies), ocular myasthenia, and antibody negative myasthenia. The following treatments are examined: cholinesterase inhibitors, immunosuppressants, and thymectomy. Inherited congenital myasthenic syndromes (CMS) are now increasingly recognized, and most commonly present during childhood. This article outlines the presynaptic, synaptic basal lamina-associated, and postsynaptic classification of CMS and the clinical presentation and aetiology of individual syndromes. Relevant investigations and treatment options (including the role of pyridostigmine, 3,4-diaminopyridine, fluoxetine, and ephedrine) are discussed.
近年来,对不同类型重症肌无力发病机制和临床表现的认识有了显著提高。本文综述了自身免疫性重症肌无力(包括与抗肌肉特异性激酶抗体相关的重症肌无力)、眼肌型重症肌无力和抗体阴性重症肌无力的临床表现。文中探讨了以下治疗方法:胆碱酯酶抑制剂、免疫抑制剂和胸腺切除术。遗传性先天性肌无力综合征(CMS)现在越来越受到认可,最常见于儿童期。本文概述了CMS的突触前、突触基底膜相关和突触后分类以及各综合征的临床表现和病因。讨论了相关检查和治疗选择(包括溴吡斯的明、3,4-二氨基吡啶、氟西汀和麻黄碱的作用)。