Lambert-Eaton肌无力综合征及其药物治疗的研究进展  

Research advance of Lambert-Eaton myasthenic syndrome and its corresponding therapies

作　　者：刘世鹏[1] 冯文化[1] 

机构地区：[1]中国医学科学院北京协和医学院药物研究所,北京100050

出　　处：《中国新药杂志》2017年第11期1279-1283,共5页Chinese Journal of New Drugs

基　　金：中国医学科学院医学与健康科技创新工程经费资助(2016-I2M-1-002)

摘　　要：Lambert-Eaton肌无力综合征(LEMS)是一种自身免疫介导的神经肌肉接头疾病。不同于重症肌无力由乙酰胆碱受体抗体引起,LEMS是由于血清中存在P/Q型电压门控式钙离子通道抗体,导致突触前膜释放乙酰胆碱障碍,从而产生一系列临床症状。本文系统地介绍了LEMS的流行病学、发病机制及临床诊断,同时说明了治疗方案和相关药物的基本信息,为今后的临床治疗和药物研究提供帮助。磷酸二氨吡啶是一种钾离子通道阻断剂,通过阻滞神经细胞前膜的钾离子通道使钙离子通道开放时间增多,从而使更多的乙酰胆碱得以释放,是目前用于治疗此疾病的优选药物。Lambert-Eaton myasthenic syndrome （LEMS） is a rare human autoimmune disorder of the neuromuscular junction. With the difference from myasthenia gravis caused by the interaction between acetylcholine receptor and its relevant antibodies, LEMS is closely related with P/Q voltage-gated calcium channel antibodies existing in the serum. These antibodies can impair the release of acetylcholine in the presynaptic membrane and consequently give rise to a series of clinical symptoms. This article systematically describes the epidemiology, pathogenesis, and clinical diagnosis of LEMS, and explains the treatment and the basic information of drugs, with a view to help clinical therapy and drug research in the future. Amifampridine phosphate, an important drug for LEMS, is a potassium channel blocker. It blocks the potassium channel on the neuronal membrane, and then increases the opening time of the calcium channel, finally results in releasing more acetylcholine, which makes it the top preference for the treatment of LEMS.

关 键 词：LAMBERT-EATON肌无力综合征 电压门控式钙离子通道 神经肌肉接头 磷酸二氨吡啶 

分 类 号：R979.5[医药卫生—药品]