Lambert-Eaton肌无力综合征临床分析

目的 增进人们对Lambert- Eaton肌无力综合征(LEMS)的认识,减少误诊.方法 对26例LEMS患者的临床资料做回顾性分析.结果 全组26例,超过40岁者占84.6%,在性别上男性居多,男∶女=2.25∶1,伴癌者21例(80%),其中小细胞肺癌(SCLC) 16例.首发症状以双下肢无力最常见(54%),随访期间有颅神经受累(70%)、上肢无力(78%)、下肢无力(100%)、自主神经症状(30%)、腱反射减低或消失(92%)等表现.肌无力症状均逐渐加剧,均以近端为主,且下肢较上肢明显,晨起、休息后肌无力加重,活动后症状改善.10例患者行脑脊液检查, 9例脑脊液异常,蛋白明显升高.20例行重复电刺激检查高频(20～50 Hz)重复神经电刺激波幅递增100%～720%,平均增加280%.有19例肌无力症状早于癌症症状平均5.1个月.抗肿瘤治疗12例,有8例LEMS症状显著改善或消失.结论 对具上述特征的患者,应警惕LEMS,LEMS与癌有显著相关倾向尤其与SCLC相关更明显.对40岁以上男性LEMS患者应积极寻找潜在的恶性肿瘤. Abstract： Objective To increase the cognition on Lambert-Eaton Myasthenic Syndrome(LEMS) and to avoid the misdiagnosis.Methods The clinical material of 26 cases LEMS were reviewed and analysed.Results 84.6% of the 26 cases were over 40 years. The male-to-female sex ration was 2.25∶1. Twenty-one cases were associated with carcinoma(80%), 16 of which had small cell lung cancer(SCLC). The lower limbs weakness(54%) as initial symptom was the most common, and was followed by cranial nerve symptoms (70%),upperlimb weakness(78%),lowerlimbs weakness(100%),autonomicsymptoms(30%) and tendon hyporeflexia or areflexia(92%) successively. All the patients experienced the ingravescence of muscle weakness,mainly in proximal limbs,and it's more obviously in pelvic limb than in pectoral limb,especially in the morning,with the rest can make it worsen,but fitting exercise can improve it. Ten cases performed the Examination of Cerebrospinal Fluid,which revealed the Cerebrospinal fluid were abviously high in 9 cases. When the repetitive nerve stimulation at high rate(20 Hz to 50 Hz) were applied in 20 cases, the average incrementin the compound motor action potential amplitude was 280% (range:100%～720%). Muscle weakness occurs early in 19 cases, 5.1 months before the cancer symptoms on average. Anti-tumour treatment for cancer in 12 cases, the symptoms of LEMS were improved significantly or disapeared in 8 cases. Conclusions Above mentioned characters can be alerts for us to coordinate the diagnosis of LEMS, Our observation has made us inclined to the association of LEMS with carcinoma, especially SCLC. The malignance should actively be searched for in LEMS of male patient above 40 years old.

Clinical analysis of the Lambert-Eaton Myasthenic Syndrome

Lambert-Eatonmyasthenic syndrome Lung cancer Autoimmunization