Lambert-Eaton肌无力综合征45例临床及电生理回顾性分析

Clinical and electrophysiological characteristics of Lambert-Eaton myasthenic syndrome： a retrospective study in forty-five patients

目的 通过对我院Lambert-Eaton肌无力综合征（Lambert-Eaton myasthenia syndrome,LEMS）患者的回顾性分析,分析此病的临床表现和伴发的神经电生理异常.方法 总结1993-2008年我院诊断的45例LEMS患者的一般情况、神经系统临床表现和体征、伴发的内科和全身疾病的情况.所有患者均进行神经电生理检测,包括神经传导速度（NCV）和重复神经电刺激（RNS）.部分患者行针极肌电图和皮肤交感反射（SSR）检查.结果 （1）患者出现神经系统症状的平均年龄为（51.2±6.8）岁.最常见临床表现为双下肢无力（35例）,其后依次为双上肢无力（10例）、构音障碍（3例）和颈肌无力（2例）.神经系统体征中最常见为双下肢或上肢轻度力弱（40例）,双下肢腱反射或跟反射减低或消失（38例）以及口干或便秘等自主神经症状（30例）.（2）神经电生理检查：所有患者尺神经高频刺激递增达156% ～636%,其中29例同时出现低频递减.所有患者行NCV检查,感觉神经传导速度（SCV）异常或SCV合并运动神经传导速度异常者19例（42%）.30例患者行针极肌电图检查,有异常发现者20例.25例行SSR的患者中发现13例异常.结论 LEMS最常见的临床症状为双下肢无力,其次为自主神经症状.患者除了有重复神经电刺激的异常,还伴有周围神经和肌肉的电生理异常以及自主神经系统的异常,提示临床表现可能混杂有神经或肌肉病变的原因.

Objective To analyze the symptoms and signs in forty-five Lambert-Eaton myasthenia syndrome （LEMS） patients retrospectively. Characteristics of electrophysiological examinations were investgated. Methods Forty-five LEMS patients were reviewed and information gathered regarding clinical complains neurological symptoms, and other concomitant diseases. The records showed that repetitive nerve stimulation （RNS） and nerve conduction velocity （NCV） were performed in all patients. Needle electromyography （EMG） and skin sympathetic response （SSR） were performed in some patients. Results （1） The mean age of neurological clinical onset age was （51.2 ±6. 8） years old. The two most common symptoms were slight weakness of lower extremities （ n = 35 ） and upper extremities （n= 5）. Dysarthria was found in 3 patients and neck weakness in 2 patients. Tendon reflex decreased and disappeared in 38 patients. Autonomic nervous system manifestations were presented in 30 patients. （2） RNS increasing was observed in all patients from 156% to 636%. Low frequency RNS abnormalities were found in 29 patients.Sensory nerve conduction velocity abnormalities or sensory nerve conduction velocity combined motor nerve conduction velocity abnormalities were found in 19 patients （ 42% ）. Of the 30 patients who underwent a needle EMG examination, 20 had myogenic or neurological damage. Thirteen abnormal findings were observed in 25 patients who underwent SSR examination. Conclusion The most common manifestations were weakness in lower extremities and autonomic nervous system dysfunction. Many abnormal electrophysiological results were found in LEMS patients, including NCV and EMG abnormalities. These findings indicated that clinical manifestations exceed the neuromuscular junction and perhaps included the peripheral nerve and muscle.