Department of Medicine and Surgery, University of Parma, Parma, Italy.
Department of Biomedical and Neuromotor Sciences, Alma Mater Studiorum, University of Bologna, Bologna, Italy.
Neurotherapeutics. 2021 Jan;18(1):6-19. doi: 10.1007/s13311-021-01051-4. Epub 2021 Apr 22.
Narcolepsy is a rare, chronic, and disabling central nervous system hypersomnia; two forms can be recognized: narcolepsy type 1 (NT1) and narcolepsy type 2 (NT2). Its etiology is still largely unknown, but studies have reported a strong association between NT1 and HLA, as well as a pathogenic association with the deficiency of cerebrospinal hypocretin-1. Thus, the most reliable pathogenic hypothesis is an autoimmune process destroying hypothalamic hypocretin-producing cells. A definitive cure for narcolepsy is not available to date, and although the research in the field is highly promising, up to now, current treatments have aimed to reduce the symptoms by means of different pharmacological approaches. Moreover, overall narcolepsy symptoms management can also benefit from non-pharmacological approaches such as cognitive behavioral therapies (CBTs) and psychosocial interventions to improve the patients' quality of life in both adult and pediatric-affected individuals as well as the well-being of their families. In this review, we summarize the available therapeutic options for narcolepsy, including the pharmacological, behavioral, and psychosocial interventions.
发作性睡病是一种罕见的、慢性的、使人致残的中枢神经系统嗜睡症;可识别两种类型:发作性睡病 1 型(NT1)和发作性睡病 2 型(NT2)。其病因仍很大程度上未知,但研究报告 NT1 与 HLA 之间存在强烈关联,并且与脑脊液下丘脑泌素-1 缺乏存在发病关联。因此,最可靠的发病假说为破坏下丘脑泌素产生细胞的自身免疫过程。到目前为止,还没有根治发作性睡病的方法,尽管该领域的研究极具前景,但迄今为止,目前的治疗方法旨在通过不同的药物方法来减轻症状。此外,发作性睡病的整体症状管理还可以受益于非药物方法,如认知行为疗法(CBT)和心理社会干预,以提高成年和儿童发作性睡病患者以及其家人的生活质量和幸福感。在这篇综述中,我们总结了发作性睡病的现有治疗选择,包括药物、行为和心理社会干预。