Roth Thomas
Sleep Disorders and Research Center, Henry Ford Health System, 2799 W Grand Blvd, Detroit, MI, 48202, USA.
CNS Drugs. 2025 Mar;39(Suppl 1):37-51. doi: 10.1007/s40263-024-01150-8. Epub 2025 Mar 20.
Narcolepsy is a rare and chronic hypersomnolence disorder characterized by excessive daytime sleepiness, disrupted nighttime sleep, sleep paralysis, and hypnagogic hallucinations and occurs with or without cataplexy. Orexin neuron loss has been implicated in the underlying pathophysiology of narcolepsy type 1 through dysregulation of sleep/wake patterns and rapid eye movement sleep. γ-Aminobutyric acid (GABA) has been shown to play a role in modulation of orexin neuronal activity during transitions from wakefulness to sleep. γ-Hydroxybutyrate (GHB), an endogenous analog of GABA, has demonstrated therapeutic benefit in treatment of narcolepsy through early investigations, but use has historically been limited owing to existing stigma related to illicit use and abuse risk. Initial regulatory approval of its sodium salt derivative, sodium oxybate (SXB), for cataplexy in patients with narcolepsy occurred in 2002, and additional formulations have been developed. The efficacy and safety of SXB in narcolepsy have been supported by decades of clinical use and research. This review discusses the history and clinical application of GHB and its SXB derivatives in the treatment of individuals with narcolepsy, including clinical safety and effect on sleep.
发作性睡病是一种罕见的慢性过度嗜睡症,其特征为日间过度嗜睡、夜间睡眠紊乱、睡眠麻痹和入睡幻觉,伴或不伴猝倒。通过睡眠/觉醒模式和快速眼动睡眠的失调,食欲素神经元丧失与1型发作性睡病的潜在病理生理学有关。γ-氨基丁酸(GABA)已被证明在从觉醒到睡眠的转变过程中对食欲素神经元活动的调节起作用。γ-羟基丁酸(GHB)是GABA的内源性类似物,早期研究表明其在发作性睡病的治疗中具有治疗益处,但由于与非法使用和滥用风险相关的现有污名,其使用在历史上受到限制。其钠盐衍生物羟丁酸钠(SXB)于2002年首次获得监管批准,用于治疗发作性睡病患者的猝倒,并且已经开发了其他制剂。数十年的临床应用和研究支持了SXB在发作性睡病中的疗效和安全性。本综述讨论了GHB及其SXB衍生物在发作性睡病治疗中的历史和临床应用,包括临床安全性和对睡眠的影响。