非肿瘤相关Lambert-Eaton肌无力综合征1例报告并文献复习  

Non tumor related Lambert-Eaton myasthenic a syndrome:one case report and literature review

作　　者：高枚春[1] 张瑛[1] 管阳太[1] GAO Meichun;ZHANG Ying;GUAN Yangtai(Department of Neurology,Renji Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200127,China)

机构地区：[1]上海交通大学医学院附属仁济医院神经内科,上海200127

出　　处：《神经病学与神经康复学杂志》2023年第2期66-72,共7页Journal of Neurology and Neurorehabilitation

摘　　要：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IgLON5-IgG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,但先后查到抗Ma1及抗IgLON5-IgG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 clinical 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,EMGcharacteristics,diagnosis,treatment and prognosis of LEMSwere 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 Ma1 and anti IgLON5-IgGantibodies,and multiple negative screenings for tumors.EMG suggested neuromuscular junction disease,highlighting the possibility of anterior membrane disease,and LEMSwas 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 IgLON5-IgG 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.

关 键 词：LAMBERT-EATON肌无力综合征 肌电图 免疫调节治疗 

分 类 号：R746[医药卫生—神经病学与精神病学]