目的:提高对 Lambert-Eaton 肌无力综合征(Lambert-Eaton myasthenic syndrome,LEMS)的临床表现、肌电图( electromyographic,EMG)特征及其治疗的认识。

方法:报告1例 LEMS 患者的临床诊治经过,结合文献复习,重点讨论 LEMS 的临床表现、分型、EMG 表现、诊治及预后。

结果:1 例老年女性患者,以“反复波动性全身无力6年余,加重半年”为主诉,症状呈波动性。先后检测到抗 Ma1 及抗 lgLON5-lgG 抗体阳性，多次肿瘤筛查阴性，EMG 提示神经肌肉接头疾病,突触前膜疾病可能,诊断考虑为 LEMS。

结论:LEMS 是一种罕见病,既往报道合并肿瘤尤其小细胞肺癌最为多见。本例患者反复多次肿瘤筛查阴性,虽 LEMS 特异性抗体阴性,但先后査到抗 Ma1 及抗 lgLON5-lgG 抗体阳性，提示该患者为免疫因素所致可能大。重复电刺激幅度改变是诊断本病的电生理金标准,早期完善及作出正确诊断,积极免疫调节治疗是本病的关键。

Objective: To improve the understanding of cinical manifestations, electromyographic (EMG)characteristics and treatments of Lambert-Eaton myasthenic syndrome(LEMS).

Methods: The clinical diagnosis and treatment process of one LEMS was reported. The clinical manifestations, subtypes, EMG characteristics, diagnosis, treatment and prognosis of LEMS were focused with literature review.

Results: One elderly female patient complained of recurrent and fluctuating systemic weakness for more than 6 years and worsening for half a year. She successively tested positive for anti Mal and anti lgLON5-lgG antibodies, and multiple negative screenings for tumors. EMG suggested neuromuscular junction disease, highlighting the possibility of anterior membrane disease, and LEMS was considered.

Conclusion: LEMS is a rare disease, which has often been reported to be associated with tumors, especially small cell lung cancer. The patient was negative for multiple tumor screenings, but positive for anti Ma1 and anti lgLON5-lgG antibodies, it suggested that the immune factors may contribute for the pathogeny. The amplitude change of repetitive nerve stimulation is the electrophysiological gold standard for diagnosing LEMS, early test and correct diagnosis, as well as active immunomodulatory therapy are the key to this disease.