Arias-Carrión Oscar, Murillo-Rodríguez Eric
Experimental Neurology, Phillips University, Marburg, Germany.
CNS Neurol Disord Drug Targets. 2009 Aug;8(4):309-14. doi: 10.2174/187152709788921681.
Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness, cataplexy, hypnagogic hallucinations, and sleep-onset rapid eye movement (REM) sleep periods. Narcolepsy is now identified to be a neurodegenerative disease, as there is a massive loss of neurons containing the neuropeptide, hypocretin/orexin. Orexin neurons are solely located in the hypothalamus, particularly in its perifornical, dorsomedial and lateral portions. Orexin fibers widely project throughout the brain and generally have excitatory effects on their postsynaptic cells. Patients with narcolepsy have a severe reduction in the levels of orexins in the cerebrospinal fluid, a finding consistent with orexin neuronal loss. Experimental models have been generated in order to study the physiology of the orexin system and narcolepsy. The discovery of orexin deficiency in narcolepsy is redefining the clinical entity of narcolepsy and offering novel diagnostic procedures. This article reviews the current understanding of narcolepsy and discusses the opportunity to explore the potential use of transplants as a therapeutical tool in order to treat narcolepsy.
发作性睡病是一种睡眠障碍,其特征为日间过度嗜睡、猝倒、入睡前幻觉以及睡眠开始时的快速眼动(REM)睡眠期。现在已确定发作性睡病是一种神经退行性疾病,因为含有神经肽下丘脑分泌素/食欲素的神经元大量丧失。食欲素神经元仅位于下丘脑,特别是其穹窿周、背内侧和外侧部分。食欲素纤维广泛投射至整个大脑,通常对其突触后细胞具有兴奋作用。发作性睡病患者脑脊液中的食欲素水平严重降低,这一发现与食欲素神经元丧失一致。为了研究食欲素系统的生理学和发作性睡病,已经建立了实验模型。发作性睡病中食欲素缺乏的发现正在重新定义发作性睡病的临床实体,并提供新的诊断方法。本文综述了目前对发作性睡病的认识,并讨论了探索将移植作为治疗发作性睡病的治疗工具的可能性。