摘要目的 对Lambert-Eaton肌无力综合征(LEMS)的临床表现和自主神经系统表现进行回顾性分析,总结自主神经系统损害和皮肤交感反射(skin sympathic response,SSR)的异常.方法 对53例临床确诊的LEMS进行回顾性研究,包括患者主诉、体征(尤其是自主神经系统症状和体征)、SSR的异常发现.结果 (1)最早出现的临床表现为双下肢力弱(n=41),最常见的自主神经系统表现为便秘(n=25)和口干(n=23),并可以在出现肢体力弱前出现(n=7).(2)自主神经系统表现:心血管系统异常4例:1例心动过缓,1例体位性低血压,2例心动过速.分泌腺体异常34例:23例口干,6例眼干,8例泌汗异常.28例患者有程度不同的胃肠道症状包括便秘或腹泻.2例患者主诉膀胱尿激惹症状.7例男性患者有性功能障碍.17例患者有皮肤划痕征异常.(3)33例行SSR检查,18例异常.结论 LEMS的自主神经系统表现广泛而常见.SSR可以在部分患者中发现异常.

abstractsObjective To retrospectively analyze clinical manifestations of autonomic nervous system (ANS) and skin sympathetic response (SSR) in Lambert-Eaton myasthenic syndrome (LEMS).Methods Fifty-three LEMS patients' medical records were reviewed and information regarding clinical symptoms and signs of ANS and SSR testing results were collected.Results ( 1 ) The most common initial symptom of LEMS was weakness of lower extremities ( n =41 ) and the most common symptom of ANS dysfunction was constipation ( n =25 ) and dry-mouth ( n =23),which could be occurred before the onset of the legs (n =7).(2) In symptoms of ANS,cardiovascular system dysfunction was found in 4 patients include one of ingone of bradycardia,one of postural hypotension and 2 of tachycardia- Secretory glands dysfunction was found in 34 patients:23 dry-mouth,6 dry-eyes,and 8 patients sweating dysfunctions.Twenty-eight patients complained of alimentary dysfunction including constipation and diarrhea.Bladder dysfunction was found in 2 patients,who complained of urinary incontinence.Seven male patients complained of sexual dysfunction.Abnormal skin scratch test was found in 17 patients.(3) SSR was performed in 33 patients and 18 found abnormal.Conclusions ANS manifestations are common and prominent in LEMS patients.SSR abnormality is also common in LEMS.More electrophysiology tests are needed in LEMS patients.