老年男性,波动性肌无力并口干、眼干3个月——重症肌无力Lambert-Eaton叠加综合征  

Old male with fluctuating weakness and dry mouth and eye for 3 months-myasthenia gravis Lambert-Eaton overlap syndrome(MLOS)

作　　者：陈艺聪[1] 曾慧鈃 刘刚[1] 曾进胜[1] 余剑[1] CHEN Yicong;ZENG Huixing;LIU Gang;ZENG Jinsheng;YU Jian(Department of Neurology,The First Affiliated Hospital,Sun Yat-sen University/Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases/National Key Clinical Department and Key Discipline of Neurology,No.58 Zhongshan Road 2,Guangzhou,510080,China)

机构地区：[1]中山大学附属第一医院神经科,广东省重大神经疾病诊治研究重点实验室,国家临床重点专科和国家重点学科,广州510080

出　　处：《中国神经精神疾病杂志》2022年第8期508-512,共5页Chinese Journal of Nervous and Mental Diseases

基　　金：国家自然科学基金青年科学基金项目(编号:81901077);广东省基础与应用基础研究基金自然科学基金面上项目(编号:2021A1515012216);中山大学附属第一医院“柯麟新星人才支持计划”(编号:R08014);广东省重大神经疾病诊治研究重点实验室(编号:2020B1212060017);广东省神经系统疾病临床医学研究中心(编号:2020B1111170002);华南神经疾病早期干预及功能修复研究国际联合研究中心(编号:2015B050501003、2020A0505020004);广东省神经系统重大疾病诊治工程技术研究中心;广东省神经系统重大疾病诊治转化医学创新平台和广州市神经系统重大疾病临床医学研究与转化中心项目(编号:201604020010)

摘　　要：本文报告1例重症肌无力Lambert-Eaton叠加综合征。患者男,64岁,波动性四肢近端无力、吞咽费力和活动后呼吸困难3个月,呈晨轻暮重和病态疲劳,伴口干和眼干的自主神经受损表现,神经系统查体示四肢近端肌力约4级,四肢腱反射减退,新斯的明试验阳性,电生理显示多条神经静止时运动传导波幅减低(左正中、面和尺神经均≤5.0 mV),大力收缩15 s后波幅上升超过2倍,左面神经、双尺神经重复电刺激(repetitive nerve stimulation,RNS)低频(3 Hz、5 Hz)递减(17.4%~35.6%)、高频(15 Hz、20 Hz)递增(153%~328%),血清AChR-Ab和Titin-Ab阳性,VGCC-Ab阴性,合并胸腺瘤,最终诊断为重症肌无力Lambert-Eaton叠加综合征(myasthenia gravis Lambert-Eaton overlap syndrome,MLOS)。经小剂量糖皮质激素(甲泼尼龙8~24 mg/d)抑制免疫联合胆碱酯酶抑制剂(溴比斯的明60 mg,2~4次/d)控制症状,病情好转后行胸腺瘤切除,肌无力症状明显改善、眼干消失。MLOS临床罕见,以波动性肌无力为主要表现时,容易忽略自主神经受累表现和遗漏检查,影响准确而全面的诊断。通过报告1例MLOS病例,以提高对MLOS诊断和治疗要点的认识。We report a case of myasthenia gravis Lambert-Eaton overlap syndrome(MLOS).A 64-year-old male,manifested as fluctuating proximal muscle and oculo-bulbar weakness,and dyspnea after activity for 3 months,accompanied by autonomic symptoms with dry mouth and eye.Weakness was light in the morning and heavy in the evening.Neurological examination showed reduction in bilateral proximal muscle strength(grade 4)and tendon reflexes.Electrophysiology showed low compound muscle action potential at rest(left median,facial,and ulnar nerves were≤5.0 mV);amplitude increment more than twice after 15 seconds of maximum voluntary contraction;amplitude decrement of 17.4%~35.6%at low frequency(3 Hz,5 Hz)and increment of 153%~328%at high frequency(15 Hz,20 Hz)with repetitive nerve stimulation(RNS).Serum AChR-Ab and Titin-Ab were positive,while VGCC-Ab was negative.Thymoma was detected by histopathological examination.The final diagnosis was myasthenia gravis Lambert-Eaton overlap syndrome(MLOS).Thymectomy was performed after the condition was stabilized by treatment with cholinesterase inhibitor(Pyridostigmine,60 mg,2-4 times/day)and low dose glucocorticoid(Methylprednisolone,8-24 mg/day).Myasthenia symptoms were significantly improved and dry eyes was resolved following thymectomy.MLOS is rare in clinical practice.The typical nature of manifestation of fluctuating myasthenia make it easy to ignore autonomic symptoms,thereby leading to insufficient examinations.As a result,it affects the accurate and comprehensive diagnosis.This case report may improve our understanding of the diagnosis and treatment of MLOS.

关 键 词：波动性肌无力 自主神经受损 重症肌无力 LAMBERT-EATON肌无力综合征 Lambert-Eaton叠加综合征 

分 类 号：R746.1[医药卫生—神经病学与精神病学]