摘要
目的总结莫旺综合征(MoS)临床表现、神经电生理特征及治疗转归情况,为尽早确诊疾病及进一步改善患者预后提供参考。方法回顾性收集河南省人民医院神经内科自2018年1月至2023年10月收治入院的13例MoS患者资料,包括患者临床症状、治疗方案及预后等临床数据,实验室检查结果及电生理检查结果。结果13例MoS患者临床主要表现为肌颤搐、疼痛、肢体麻木、周身瘙痒、多汗症、尿便障碍、心动过速、失眠、焦虑抑郁等。10例完成自身免疫脑炎抗体检测,3例仅抗接触蛋白相关蛋白2(CASPR2)抗体阳性,2例仅抗富含亮氨酸的胶质瘤失活蛋白1(LGI1)抗体阳性,2例双抗体阳性。11例完成肿瘤筛查,有4例肿瘤患者,2例患胸腺瘤、1例患肺鳞癌、1例患肾上腺非霍奇金淋巴瘤。10例完成心电图检查,其中3例静息性心动过速,2例ST段抬高样改变。所有患者均完成肌电图检查,12例出现异常运动单位电位,如肌颤搐电位、束颤电位和神经性肌强直电位;F波检查均可见F波和(或)M波后发放电位。随访1~12个月,9例非肿瘤患者中6例行免疫治疗,其中5例症状好转;余3例对症治疗患者中1例症状好转。4例肿瘤患者中3例接受免疫治疗,仅1例症状好转。结论肌颤搐、疼痛、多汗、尿便障碍、严重失眠为MoS典型症状。血清抗CASPR2/LGI1抗体及神经电生理检查可为诊断MoS提供依据。早期免疫治疗可改善MoS预后,合并肿瘤患者临床预后差。
Objective To summarize the clinical manifestations,neuroelectrophysiological characteristics and prognoses of Movan syndrome(MoS),and provide references for early diagnoses and prognoses.Methods A retrospective analysis was performed.The clinical data,such as clinical symptoms,treatments and prognoses,laboratory test results and electrophysiological test results,of 13 patients with confirmed MoS in Department of Neurology,He'nan Provincial People's Hospital from January 2018 to October 2023 were collected.Results Ten male MoS patients and 3 female ones were included.Main clinical manifestations of 13 patients with MoS included myokymia,pain,numbness of limbs,itching all over the body,hyperhidrosis,urinary and defecation disorder,tachycardia,insomnia,anxiety and depression.Ten patients completed the autoimmune encephalitis antibody detection:3 only had positive anti-contactin-associated protein-like 2(CASPR2)antibody,2 only had positive anti-leucine-rich glioma-inactivated protein1(LGI1)antibody,and 2 had both positive anti-CASPR2 antibody and anti-LGI1 antibody.Eleven patients completed tumor screening and 4 tumors(thymoma[n=2],lung squamous cell carcinoma[n=1]and adrenal non-Hodgkin's lymphoma[n=1])were noted.Ten patients completed electrocardiogram,including 3 patients with resting tachycardia and 2 patients with ST segment elevation.All patients completed the electromyographic examination;12 patients showed abnormal motor unit potential,including myokymia potential,fasciculation potential and neuromyotonic potential;F-wave and/or M-wave post-discharge potentials were found in all patients.Follow up was performed for 1-12 months;in 9 non-tumor patients,5 were improved in 6 patients accepted immunotherapy and one was improved in 3 patients received symptomatic treatment;in 4 tumor patients,only one was improved in 3 received immunotherapy.Conclusion Myokymia,pain,urinary and defecation disorder,and severe insomnia are typical symptoms for MoS patients;serum anti-CASPR2/LGI1 antibody and electromyography results
作者
董泽钦
梅文丽
左绍敏
徐佳佳
袁丽品
李玮
刘慧勤
Dong Zeqin;Mei Wenli;Zuo Shaomin;Xu Jiajia;Yuan Lipin;Li Wei;Liu Huiqin(Department of Neurology,People's Hospital of He'nan University(He'nan Provincial People's Hospital),Zhengzhou 450003,China;Department of Electromyography,People's Hospital of Zhengzhou University(He'nan Provincial People's Hospital),Zhengzhou 450003,China)
出处
《中华神经医学杂志》
CAS
CSCD
北大核心
2024年第5期471-477,共7页
Chinese Journal of Neuromedicine
基金
河南省医学科技攻关计划联合共建项目(LHGJ20200027)
河南省科技攻关计划项目(212102310833)。
