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重症肌无力:诊断与管理综述

Myasthenia gravis: review of diagnosis and management.

作者信息

Patten B M

出版信息

Muscle Nerve. 1978 May-Jun;1(3):190-205. doi: 10.1002/mus.880010304.

Abstract

Although the cause of myasthenia gravis is still unknown, its pathogenesis appears clear: immunologic attack on synaptic receptors in muscle causes receptor deficiency, decreased miniature endplate potentials, and decrements in the compound action potentials evoked from muscles on repetitive stimulation of peripheral nerves. In addition to the involvement of skeletal muscle, some MG patients may manifest subtle alterations of the function of heart, lung, smooth muscle, and CNS, indicating that this is truly a systemic disorder. Modern therapy involves adjusting treatment to the needs of individual patients. Anticholinesterases, calcium, ephedrine, potassium, and germine partially correct the defect in neuromuscular transmission; prednisone, ACTH, cytotoxic drugs, antilymphocyte serums, gamma globulin, thoracic duct drainage, plasmapheresis, and thymectomy partially modify the abnormalities of the immune system.

摘要

尽管重症肌无力的病因仍不明,但发病机制似乎已明确:对肌肉突触受体的免疫攻击导致受体缺乏、微小终板电位降低以及在重复刺激外周神经时肌肉诱发的复合动作电位递减。除骨骼肌受累外,一些重症肌无力患者可能表现出心脏、肺、平滑肌和中枢神经系统功能的细微改变,表明这确实是一种全身性疾病。现代治疗方法是根据个体患者的需求调整治疗方案。抗胆碱酯酶、钙、麻黄碱、钾和新斯的明可部分纠正神经肌肉传递缺陷;泼尼松、促肾上腺皮质激素、细胞毒性药物、抗淋巴细胞血清、γ球蛋白、胸导管引流、血浆置换和胸腺切除术可部分改善免疫系统异常。

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