Lambert-Eaton综合征(LEMS)20例临床特点分析

 目的  探讨Lambert-Eaton综合征的临床和电生理特点。方法  收集1998至2012年我院收治的20例经临床和电生理证实的Lambert-Eaton综合征患者,分析并总结其临床和电生理特点。结果  20例Lambert-Eaton综合征患者3 Hz低频重复电刺激均阳性,30 Hz高频重复电刺激复合肌肉动作电位(compound muscle action potential,CMAP)波幅递增均超过100%。女性6例、男性14例,年龄43~83岁,平均(62.3±12.7)岁;起病年龄40~79岁,平均(55.0±13.3)岁。首发症状为双下肢无力者10例,四肢无力者9例,眼睑下垂伴四肢无力者1例;病情波动易疲劳者10例,轻度活动后肌无力改善者3例;伴植物神经系统症状者12例。经检查随访后有12例发现恶性肿瘤,10例为肺癌,2例为纵膈淋巴瘤,其中8例接受肿瘤相关治疗。16例患者接受吡啶斯的明治疗,有效者10例;15例患者使用强的松治疗,其中联合硫唑嘌呤治疗者6例,联用丙种球蛋白者6例;经治疗随访后20例患者中有10例症状呈一定程度改善,另10例症状改善不明显。 结论  本组Lambert-Eaton综合征好发于中老年人群,多伴有肺癌,肌无力以双下肢为主。患者对吡啶斯的明和免疫抑制治疗的反应不一。高频重复电刺激是确诊本病的关键,一旦确诊必须筛查潜在的恶性肿瘤。

Clinical analysis of 20 patients with Lambert-Eaton syndrome (LEMS)

Objective  To explore the clinical and electrophysiologic features of Lambert-Eaton syndrome (LEMS).Methods  Twenty patients with LEMS clinically and electrophysiologically defined by our hospital from 1998 to 2012 were analyzed.Results  Twenty patients with LEMS were positive in low rate stimulation （3 Hz） and marked incremental reponse (+100%) at high-rate stimulation (30 Hz).Among the 20 patients,there were 6 females and 14 males,aged from 43 to 83 years old,with an average age of (62.3±12.7) years,and the onset aged from 40 to 79 years old,with an average age of (55.0±13.3) years.The presenting signs were lower limb weakness (10 cases),upper and lower limbs weakness (9 cases),ophthalmoplegia with limb weakness (1 case);10 cases had exertional weakness,among which only 3 cases had transient improvement after brief exercise followed by weakness;12 cases had autonomic dysfunction.After follow-up,12 patients were associated with malignant tumors,i.e.,10 were lung cancer,and 2 were mediastinum lymphoma,among which 8 cases accepted chemotherapy.Pridostigmine had been used for symptomatic treatment in 16 patients,among which 10 cases proved to be effective.Prednisone had been used for immunosuppressive therapy in 15 patients,among which 6 cases were treated combining with azathioprine (AZA) and 6 cases with intravenous immunoglobulin (IVIg).After the above treatment,10 out of 20 patients had symptomatic improvement,other 10 cases had no improvement.Conclusions  The LEMS in our study usually occurred in elder people,usually combining with small cell lung cancer (SCLC).The major manifestation was lower limb weakness.LEMS in our study had different response to the pridostigmine and immunosuppressive therapy.High-RNS was critical for LEMS diagnosis.After diagnosis we should constructively find the potential tumor.