中国医学科学院学报

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中国医学科学院学报

中国医学科学院学报 ›› 2019, Vol. 41 ›› Issue (3): 344-350.doi: 10.3881/j.issn.1000-503X.11177

• 论著 • 上一篇    下一篇

抗富含亮氨酸胶质瘤失活蛋白1和抗接触蛋白相关蛋白2双抗体阳性疾病的临床特点

董立羚,关鸿志,黄颜,郝红琳,牛婧雯,柳青,卢强,徐丹,张君怡,周立新,金丽日,任海涛,朱以诚,彭斌,崔丽英,周祥琴()   

  1. 中国医学科学院 北京协和医学院 北京协和医院神经内科,北京 100005
  • 收稿日期:2019-01-17 出版日期:2019-06-30 发布日期:2019-07-04
  • 通讯作者: 周祥琴 E-mail:zwypumc@126.com

Clinical Characteristics of Autoimmune Disease with Dual Seropositive Antibodies of Leucine-rich Glioma Inactivated 1 and Contactin-associated Protein 2

DONG Liling,GUAN Hongzhi,HUANG Yan,HAO Honglin,NIU Jingwen,LIU Qing,LU Qiang,XU Dan,ZHANG Junyi,ZHOU Lixin,JIN Liri,REN Haitao,ZHU Yicheng,PENG Bin,CUI Liying,ZHOU Xiangqin()   

  1. Department of Neurology,PUMC Hospital,CAMS and PUMC,Beijing 100005,China
  • Received:2019-01-17 Online:2019-06-30 Published:2019-07-04
  • Contact: Xiangqin ZHOU E-mail:zwypumc@126.com

摘要:

目的 探讨血清抗富含亮氨酸胶质瘤失活蛋白1(LGI1)和抗接触蛋白相关蛋白2(Capsr2)双抗体阳性的自身免疫性疾病的临床特点。方法 回顾性分析2014年7月至2017年12月在北京协和医院神经内科收治的7例血清抗LGI1和抗Caspr2双抗体阳性患者的临床资料,总结抗LGI1和抗Capsr2双抗体阳性疾病的临床特点。结果 7例患者均存在中枢、周围和自主神经受累。在临床症状中,出现率最高的是失眠、肌肉颤搐、疼痛和多汗(100%);71%(5/7)伴有记忆下降或精神行为异常,57%(4/7)伴有尿便障碍,43%(3/7)伴有癫痫发作。在实验室检查中,100%(6//6)肌电图显示M波后发放电位和/或安静状态下异常自发放电;71%(5/7)脑电图显示慢波增多或基本节律变慢;71%(5/7)心电图显示窦性心动过速、电轴偏转、QT间期延长。1例在免疫治疗前因心律失常死亡,1例在治疗后因肺部感染死亡;5例临床症状明显改善,随诊1~2年,均未复发。结论 抗LGI1和抗Caspr2双抗体阳性疾病的神经系统受累广泛,全面累及中枢、周围和自主神经。对于急性或亚急性起病的神经精神症状,尤其是伴有失眠、肌肉颤搐和多汗者,需要考虑本病可能。

关键词: 抗电压门控性钾离子通道抗体, 抗富含亮氨酸胶质瘤失活蛋白1抗体, 抗接触蛋白相关蛋白2抗体

Abstract:

Objective To explore the clinical characteristics of autoimmune disease with dual seropositive antibodies of leucine-rich glioma inactivated 1(LGI1)and contactin-associated protein 2(Caspr2).Methods The clinical data of seven patients with dual seropositive LGI1 and Caspr2 antibodies who were admitted to the Neurology Department of Peking Union Medical College Hospital from July 2014 to December 2017 were retrospectively analyzed.Results Central,peripheral and autonomic nervous systems were all involved in the seven cases;100%(7/7)presented with insomnia,myokymia,neuropahic pain and hyperhydrosis;71%(5/7)showed memory decline or psychiatric and behavioral symptoms;57%(4/7)had urinary hesitation or constipation;and 43%(3/7)had seizure.Electromyography showed 100%(6/6) of the patients had prolonged afterdischarges following normal M waves and/or abnormal spontaneous firing.Electroencephalography revealed slow waves or basic rhythm slowing in 71%(5/7)of patients.Electrocardiography showed sinus tachycardia,axis deviation,and prolonged QT intervals in 71%(5/7)of patients.One patient died from arrhythmia before immunotherapy.One died from pulmonary infection after immunotherapy.Improvement with immunotherapy was documented in the other five cases.No relapse was noted during the 1-2-year follow-up.Conclusions Autoimmune disease with dual seropositive antibodies of LGI1 and Caspr2 can diffusely affect the central,peripheral,and autonomic nervous systems.The possibility of this disease should be considered in patients with acute and subacute onset of neuropsychiatric symptoms,especially in patients with accompanying insomnia,myokymia,and hyperhydrosis.

Key words: antibody of voltage-gated potassium channel, antibody of leucine-rich glioma inactivated 1, antibody of contactin-associated protein 2

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