兰伯特-伊顿肌无力综合征
Lambert-Eaton Myasthenic Syndrome.
作者信息
Kesner Vita G, Oh Shin J, Dimachkie Mazen M, Barohn Richard J
机构信息
Neurology Department, 12 Executive Park Drive NE, Atlanta, GA 30329, USA.
University of Alabama at Birmingham, Department of Neurology, SC 350, 1720 2nd Ave South, Birmingham, AL 35294, USA.
出版信息
Neurol Clin. 2018 May;36(2):379-394. doi: 10.1016/j.ncl.2018.01.008.
Abstract
Lambert-Eaton myasthenic syndrome is a paraneoplastic or primary autoimmune neuromuscular junction disorder characterized by proximal weakness, autonomic dysfunction and ariflexia. The characteristic symptoms are thought to be caused by antibodies generated against the P/Q-type voltage-gated calcium channels present on presynaptic nerve terminals and by diminished release of acetylcholine. More than half of Lambert-Eaton myasthenic syndrome cases are associated with small cell lung carcinoma. Diagnosis is confirmed by serologic testing and electrophysiologic studies. 3,4-diaminopyridine is effective symptomatic treatment of LEMS.
摘要
兰伯特-伊顿肌无力综合征是一种副肿瘤性或原发性自身免疫性神经肌肉接头疾病，其特征为近端肌无力、自主神经功能障碍和腱反射减弱。特征性症状被认为是由针对突触前神经末梢存在的P/Q型电压门控钙通道产生的抗体以及乙酰胆碱释放减少所致。超过一半的兰伯特-伊顿肌无力综合征病例与小细胞肺癌有关。通过血清学检测和电生理研究确诊。3,4-二氨基吡啶是兰伯特-伊顿肌无力综合征有效的对症治疗药物。
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