富亮氨酸胶质瘤失活1蛋白抗体相关边缘性脑炎睡眠障碍研究
Study of sleep disorders in patients with limbic encephalitis associated with anti leucine rich glioma inactivated protein 1 antibody
目的 探讨富亮氨酸胶质瘤失活1蛋白抗体相关边缘性脑炎睡眠障碍的特征及多导睡眠图表现.方法 回顾性收集我院2012年12月至2017年8月确诊的13例富亮氨酸胶质瘤失活1蛋白抗体相关边缘性脑炎患者的临床资料,分析其临床、影像、多导睡眠监测、脑脊液、自身免疫抗体等检查的特点.结果 13例患者血清和(或)脑脊液富亮氨酸胶质瘤失活1蛋白抗体呈阳性,确诊为富亮氨酸胶质瘤失活1蛋白抗体相关边缘性脑炎.其临床表现均符合边缘性脑炎特点,包括记忆下降、精神行为异常、癫痫发作等.13例患者均有睡眠障碍,症状包括睡眠中不自主运动及梦境相关异常行为、失眠和嗜睡.全夜多导睡眠监测显示其中10例患者睡眠效率低于80%,10例N3期慢波睡眠减少,13例快速眼球运动期睡眠减少;7例患者的周期性肢体运动指数增高(15.6~224.4次/h),其中4例可见肢体非周期性活动明显增多;6例患者的视频多导睡眠图可见夜间频繁面臂肌张力障碍样发作(全夜发作20~ 83次);3例患者确诊为快速眼球运动期睡眠行为障碍.13例患者接受免疫治疗后,神经系统症状缓解,其中10例睡眠障碍得到明显改善.结论 睡眠障碍是富亮氨酸胶质瘤失活1蛋白抗体相关边缘性脑炎的常见表现,可见多种类型睡眠障碍包括失眠、嗜睡、快速眼球运动期睡眠行为障碍和不自主运动.免疫治疗可明显改善睡眠障碍及其他神经系统症状.
更多Objective To identify the spectrum of sleep disorders in the patients diagnosed with limbic encephalitis associated with anti leucine rich glioma inactivated protein 1 (LGI1) antibody.Methods Thirteen patients were identified with limbic encephalitis associated with LGI1 antibody in the Department of Neurology,Peking Union Medical College Hospital between December 2012 and August 2017.The characters of the 13 cases were studied from several aspects such as clinical presentation,imaging,polysomnography,cerebrospinal fluid and serum.Results Serum test and/or cerebrospinal fluid test showed LGI1 antibody positive in all the 13 patients.Clinical presentation included:cognitive impairment,seizures,neuropsychiatric features.Sleep disorders presented in all patients,including insomnia,dream enactment behaviors,hypersomnia,involuntary movement.Polysomnogram in the 13 patients revealed that sleep efficiency decreased in 10 patients (< 80%),N3 sleep decreased in 10 patients,rapid eye movement sleep decreased in 13 patients;period limb movement disorder was identified in seven patients,period limb movement index was 15.6-224.4/h,four of the seven patients also had frequent irregular motor activity;six patients had frequent faciobrachial dystonic seizures (20-83 times throughout the night);rapid eye movement sleep behavior disorder (RBD) was diagnosed in three patients.Sleep disorder resolved almost completely in 10 of 13 patients who received immunotherapy.Conclusions Sleep disorders are cardinal manifestations of limbic encephalitis associated with LGI1 antibody,including insomnia,RBD,hypersomnia,involuntary movement.They may respond favorably to immunotherapy.
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