接触蛋白相关性蛋白2抗体相关疾病睡眠障碍临床研究  

A study of sleep manifestations in patients with caspr2 antibody-associated disease

作　　者：黄颜[1] 关鸿志[1] 柳青[1] 郝红琳[1] 孙鹤阳[1] 任海涛[1] 卢强[1] 金丽日[1] 周立新[1] 张君怡[1] 彭斌[1] 崔丽英[1,2] HUANG Yan;GUAN Hongzhi;LIU Qing;HAO Honglin;SUN Heyang;REN Haitao;LU Qiang;JIN Liri;ZHOU Lixin;ZHANG Junyi;PENG Bin;CUI Liying(Department of Neurology,Peking Union Medical College Hospital,Beijing 100730,China;Department of Neurology,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院神经内科,100730 [2]中国医学科学院神经科学中心,100730

出　　处：《中国神经免疫学和神经病学杂志》2018年第6期403-407,共5页Chinese Journal of Neuroimmunology and Neurology

摘　　要：目的对接触蛋白相关蛋白2(CASPR2)抗体相关神经系统疾病临床特点和伴发的睡眠障碍进行研究。方法回顾分析作者医院2014-12-2017-06期间确诊的CASPR2抗体阳性的患者9例,分析其临床、影像、多导睡眠监测、肌电图、脑脊液、血等检查的特点。结果 9例患者中7例临床表现具有典型的莫旺综合征,1例神经性肌强直,1例边缘性脑炎。所有患者均有失眠主诉,4例伴梦样异常行为,2例睡眠中肢体运动增多。9例患者中5例完成多导睡眠图(PSG)检查:4例莫旺综合征患者中2例完全没有睡眠;2例睡眠效率降低,睡眠结构紊乱,几乎没有典型的睡眠纺锤、顶部尖波,慢波睡眠消失,仅见片段REM睡眠插入到N1期,其中1例周期性肢体运动指数44.9次/h;1例神经性肌强直患者PSG可见睡眠效率42%。8例患者接受免疫治疗(人丙种球蛋白和/或糖皮质激素),其中7例患者神经系统症状包括睡眠障碍明显改善,1例治疗6个月后,病情难以控制,死于感染性休克;1例患者在入院第4天猝死,尚未进行免疫治疗。结论 CASPR2抗体相关神经系统疾病临床表现复杂多样,包括多种综合征如莫旺综合征,神经性肌强直和边缘性脑炎,严重失眠是其常见症状。早期诊断并及时进行免疫治疗,可明显改善预后。Objective A study was undertaken to describe the clinical spectrum,especially sleep disorder in Nine patients associated with CASPR2 antibody.Methods Nine consecutive patients were identified with CASPR2 antibody in the Department of Neurology,Peking Union Medical College Hospital between December 2014 to June 2017.The characteristics of the 9 patients were studied from several aspects such as clinical presentation,imaging,polysomnography(PSG),cerebrospinal fluid and serum test. These patients were identified with Morvan syndrome(n=7),peripheral nervous system hyperexcitability(n=1),limbic encephalitis(n=1).Insomnia present in all the patients,4 patients had dream enactment behavior,2 patients with excessive motor activity.5 patients completed PSG examination.PSG in 2 patients with Morvan syndrome revealed complete absence of recognizable sleep;PSG in the other 2 patients with Morvan syndrome documented severe insomnia,lack of cyclic sleep organization with a predominance of stage 1 NREM intermixed with brief REM sleep episodes,and a marked reduction of spindles and delta sleep,period limb movement index(PLMI)in 1 patient was 44.9/h.The sleep efficiency of the patient with peripheral nervous system hyperexcitability was 42%.8 patients received immunotherapy(human immunoglobulin and/or corticosteroids),Sleep disorders and other symptoms resolved completely or almost completely in 7 patients.1 patient died from septic shock after 6 months.1 patient died from sudden cardiac death before immunotherapy.Conclusions The resulting syndrome may vary among patients with Caspr2 antibodies,including Morvan syndrome,neuromyotonia,limbic encephalitis.Severe insomnia isa cardinal manifestations of the patients associated with Caspr2 antibodies.Recognition of this spectrum of symptoms and consideration of the protracted clinical course are important for early diagnosis of this disorder.Prompt immunotherapy often result in improvements.

关 键 词：莫旺综合征 失眠 多道睡眠描记术 CASPR2抗体 

分 类 号：R512.3[医药卫生—内科学]