摘要
目的探讨肿瘤相关和非肿瘤相关的Lambert-Eaton肌无力综合征(LEMS)患者在临床、电生理及生存预后的差异。方法回顾性收集1997年1月至2023年6月经电生理确诊的80例LEMS患者的临床资料、电生理及血清抗体,以及从肌无力发病至诊断后1年肿瘤筛查结果及生存状态。根据有无合并肿瘤可分为肿瘤相关LEMS(T-LEMS)组(44例)和非肿瘤相关LEMS(NT-LEMS)组(36例)。结果与NT-LEMS组比较,T-LEMS组中男性患者更常见(77.3%对比52.8%,P=0.021),发病年龄更晚(59岁对比54岁,P=0.012),病程更短(6个月对比12个月,P=0.014),且合并其他副肿瘤综合征的比例明显升高(27.3%对比0,P=0.001)。但NT-LEMS组合并自身免疫病比例高于T-LEMS组(19.4%对比4.5%,P=0.036)。静息状态下,NT-LEMS组拇外展肌及趾长伸肌复合肌肉运动电位(CMAP)波幅显著高于T-LEMS组。两组在肌无力分布、腱反射、自主神经受累、重复电刺激等方面均差异无显著性。T-LEMS组的中位生存期显著短于NT-LEMS组(29.5个月对比62.0个月,P=0.046)。结论依据不同的临床特点早期鉴别两组LEMS,对疾病监测具有重要临床价值。
Aim To explore the clinical,electrophysiological features and prognosis in tumor associated and non-tumor-associated Lambert-Eaton syndrome(LEMS).Methods A total of 80 patients with LEMS who were diagnosed by repeated nerve stimuli(RNS)in Huashan Hospital,Fudan University from January 1997 to June 2023 were enrolled.Clinical,electromyographical findings and serum voltage gated calcium channel antibody titers were retrospectively reviewed.The tumor screening results were retrospectively collected.The patients divided into a tumor-associated LEMS(T-LEMS)group(44 cases)and a non-tumor-associated(NT-LEMS)group(36 cases).Results Compared with the NT-LEMS group,the T-LEMS group has a high proportion of male patients(77.3%vs.52.8%,P=0.021),a later onset-age(59 vs.54 years,P=0.012),a shorter disease duration(6 vs.12 months,P=0.014),and a higher concurrence rate with paraneoplastic syndrome(27.3%vs.0%,P=0.001).In contrast,NT-LEMS was more frequently associated with other autoimmune diseases(19.4%vs.4.5%,P=0.036).The resting amplitude of complex muscle motor potential(CMAP)on abductor pollicis and extensor digital longis in the NT-LEMS group was significantly higher than that in the T-LEMS group.There are no significant differences in muscle involvement,tendon reflexes,autonomic neuropathy,or RNS.Survival analysis revealed a worse outcome in T-LEMS(29.5 vs.62 months,P=0.046).Conclusion According to the different clinical characteristics,early identification of the two groups of LEMS has important clinical value for disease surveillance.
作者
李明哲
罗叶婷
刘逸奇
闫翀
宋捷
奚剑英
赵重波
罗苏珊
LI Ming-zhe;LUO Ye-ting;LIU Yi-qi;YAN Chong;SONG Jie;XI Jie-ying;ZHAO Chong-bo;LUO Su-shan(Department of Neurology,District Center Hospital of Zhengzhou,Zhengzhou University,Zhengzhou 450000,China;Department of Neurology,Ganzhou Hospital,Nanchang University,Ganzhou 341000,China;Department of Neurology,Huashan Hospital,Fudan University,Shanghai 200040,China;National Center for Neurological Discorders,Shanghai 200040,China)
出处
《中国临床神经科学》
2025年第2期160-167,共8页
Chinese Journal of Clinical Neurosciences
基金
国家重点研发计划(编号:2022YFC3501303)
上海市医企融合创新成果转化专项课题(编号:SHDC2023CRD007)。
