Giannoccaro Maria Pia, Liguori Rocco, Plazzi Giuseppe, Pizza Fabio
IRCCS Istituto delle Scienze Neurologiche di Bologna, Ospedale Bellaria, Bologna, Italy.
Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
Nat Sci Sleep. 2021 May 11;13:557-577. doi: 10.2147/NSS.S275931. eCollection 2021.
Narcolepsy type 1 (NT1) is a lifelong sleep disorder, primarily characterized clinically by excessive daytime sleepiness and cataplexy and pathologically by the loss of hypocretinergic neurons in the lateral hypothalamus. Despite being a rare disorder, the NT1-related burden for patients and society is relevant due to the early onset and chronic nature of this condition. Although the etiology of narcolepsy is still unknown, mounting evidence supports a central role of autoimmunity. To date, no cure is available for this disorder and current treatment is symptomatic. Based on the hypothesis of the autoimmune etiology of this disease, immunotherapy could possibly represent a valid therapeutic option. However, contrasting and limited results have been provided so far. This review discusses the evidence supporting the use of immunotherapy in narcolepsy, the outcomes obtained so far, current issues and future directions.
1型发作性睡病（NT1）是一种终身性睡眠障碍，临床主要特征为日间过度嗜睡和猝倒，病理特征为下丘脑外侧促食欲素能神经元丧失。尽管这是一种罕见疾病，但由于其发病早且病程慢性，NT1给患者和社会带来的负担不容小觑。虽然发作性睡病的病因尚不清楚，但越来越多的证据支持自身免疫起核心作用。迄今为止，尚无治愈该疾病的方法，目前的治疗只是对症治疗。基于该疾病自身免疫病因的假说，免疫疗法可能是一种有效的治疗选择。然而，目前已有的结果相互矛盾且有限。本文综述了支持在发作性睡病中使用免疫疗法的证据、目前已取得的成果、当前存在的问题及未来的研究方向。
