发作性睡病中的自身免疫
Autoimmunity in narcolepsy.
作者信息
Bonvalet Melodie, Ollila Hanna M, Ambati Aditya, Mignot Emmanuel
机构信息
aDepartment of Psychiatry and Behavioral Sciences, Center for Sleep Sciences, Stanford University School of Medicine, Palo Alto, California, USA bNational Institute for Health and Welfare, Public Genomics Unit cInstitute for Molecular Medicine FIMM, University of Helsinki, Helsinki, Finland.
出版信息
Curr Opin Pulm Med. 2017 Nov;23(6):522-529. doi: 10.1097/MCP.0000000000000426.
PURPOSE OF REVIEW
Summarize the recent findings in narcolepsy focusing on the environmental and genetic risk factors in disease development.
RECENT FINDINGS
Both genetic and epidemiological evidence point towards an autoimmune mechanism in the destruction of orexin/hypocretin neurons. Recent studies suggest both humoral and cellular immune responses in the disease development.
SUMMARY
Narcolepsy is a severe sleep disorder, in which neurons producing orexin/hypocretin in the hypothalamus are destroyed. The core symptoms of narcolepsy are debilitating, extreme sleepiness, cataplexy, and abnormalities in the structure of sleep. Both genetic and epidemiological evidence point towards an autoimmune mechanism in the destruction of orexin/hypocretin neurons. Importantly, the highest environmental risk is seen with influenza-A infection and immunization. However, how the cells are destroyed is currently unknown. In this review we summarize the disease symptoms, and focus on the immunological findings in narcolepsy. We also discuss the environmental and genetic risk factors as well as propose a model for disease development.
综述目的
总结发作性睡病的最新研究成果，重点关注疾病发展中的环境和遗传风险因素。
最新发现
遗传和流行病学证据均指向自身免疫机制在食欲素/下丘脑泌素神经元破坏过程中的作用。近期研究表明，体液免疫和细胞免疫反应均参与了疾病的发展。
总结
发作性睡病是一种严重的睡眠障碍，下丘脑产生食欲素/下丘脑泌素的神经元被破坏。发作性睡病的核心症状包括使人衰弱的极度嗜睡、猝倒以及睡眠结构异常。遗传和流行病学证据均指向自身免疫机制在食欲素/下丘脑泌素神经元破坏过程中的作用。重要的是，甲型流感感染和免疫接种带来的环境风险最高。然而，目前尚不清楚这些细胞是如何被破坏的。在本综述中，我们总结了疾病症状，并重点关注发作性睡病的免疫学研究结果。我们还讨论了环境和遗传风险因素，并提出了一个疾病发展模型。
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