以嗜睡为突出表现的抗Ma-2抗体相关脑炎  

Hypersomnia as the prominent symptom of anti-Ma2 associated encephalitis

作　　者：郝红琳[1] 黄颜[1] 关鸿志[1] 任海涛[1] 高晶[1] 孙鹤阳[1] 彭斌[1] 朱以诚[1] 崔丽英 

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

出　　处：《中华神经科杂志》2015年第12期1084-1087,共4页Chinese Journal of Neurology

摘　　要：目的 分析抗Ma-2抗体相关脑炎患者睡眠特点,探索睡眠障碍的机制.方法 回顾我院确诊的以过度嗜睡为首发症状的抗Ma-2抗体相关脑炎1例,分析其临床、影像、多导睡眠监测、脑脊液、病理检查等特点,结合文献进行讨论.结果 本例以嗜睡为突出症状,伴记忆力减退、精神行为异常、行走不稳等,磁共振成像提示边缘系统、下丘脑、上位脑干损害,多导睡眠监测表现为发作性睡病样特点及快速眼动期睡眠行为障碍,脑脊液下丘脑分泌素水平正常（186 pg/ml）,血及脑脊液抗Ma-2抗体阳性,结肠组织病理检查为淋巴瘤,确诊为淋巴瘤合并抗Ma-2抗体相关脑炎.结论 抗Ma-2抗体相关脑炎的睡眠障碍可以表现为过度嗜睡、发作性睡病样症状及快速眼动期睡眠行为障碍等多种形式,与下丘脑、脑干、边缘系统等睡眠相关结构损害有关,下丘脑分泌素之外的递质系统可能参与发作性睡病症状的发生.Objective To investigate the characteristics and mechanisms of sleep disorders in anti-Ma2 associated encephalitis.Methods Report a case of anti-Ma2 associated encephalitis with excessive sleepiness as the initial and prominent symptom who was diagnosed in our hospital.The characteristics of this case were studied from several aspects such as clinical, imaging, polysomnography and cerebrospinal fluid, etc.Results This case was characterized mainly by the symptom of lethargy, together with memory loss, mental and behavior disorder and walking instability.MRI examination indicated the damage of limbic system, hypothalamus and upper brainstem.The polysomnography showed narcolepsy-like symptoms and rapid eye movement sleep behavior disorder.In addition, the level of hypocretin from cerebrospinal fluid remained normal （186 pg/ml） , the anti-Ma2 antibodies in blood and cerebrospinal fluid showed positive, and the lymphoma was identified by colon histopathology.As a result, this case was diagnosed as lymphoma with anti-Ma2 associated encephalitis.Conclusions The sleep disorders in anti-Ma2 associated encephalitis manifest a variety of forms including excessive sleepiness, narcolepsy-like symptoms and rapid eye movement sleep behavior disorder, which are associated with the structural damage in hypothalamus, brainstem and limbic system.Further, the incidence of such narcolepsy symptoms might involve other neurotransmitter systems other than hypocretin.

关 键 词：边缘叶脑炎 自身抗体 过度嗜睡性障碍 睡眠障碍 抗原 肿瘤 神经组织蛋白质类 

分 类 号：R742.9[医药卫生—神经病学与精神病学]