Narcolepsy Features in Young Patients

A. Govi, E. Antelmi, F. Pizza, F. Ingravallo, G. Plazzi
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Abstract

Abstract Narcolepsy is a lifelong central hypersomnia characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, hypnagogic hallucinations, and disrupted nocturnal sleep. Behavioral and psychiatric comorbidities are often associated clinical features. It is divided into two subtypes, narcolepsy type-1, and narcolepsy type-2, depending on the presence of cataplexy and the cerebrospinal fluid hypocretin-1 levels. An autoimmune process, along with environmental factors, has been hypothesized to cause the disease. Among children and adolescents, incidence in Europe falls between 0.14 and 0.3 in 100,000, with a reported increase in the incidence after the 2009 H1N1 pandemic influence and vaccination. Currently, specific pediatric diagnostic criteria and cut-off instrumental values are lacking. Clinical presentation of the disease may differ between children and adults, and misdiagnoses or diagnostic delays are still an issue. The treatment is based on behavioral and pharmacological therapy, but drugs in children are prescribed off-label. Overall, pathogenic mechanism of narcolepsy and data on drug efficacy in children are still limited: more research is needed to develop new drugs and to reach approval of current treatments in the pediatric population.
年轻患者发作性睡病的特点
嗜睡症是一种终生的中枢性嗜睡症,其特征是白天嗜睡过度、猝厥、睡眠瘫痪、嗜睡幻觉和夜间睡眠中断。行为和精神合并症通常是相关的临床特征。根据发作性猝睡和脑脊液下丘脑泌素-1水平的不同,可分为两种亚型:发作性猝睡1型和发作性猝睡2型。据推测,自身免疫过程和环境因素共同导致了这种疾病。在儿童和青少年中,欧洲的发病率在10万分之0.14至0.3之间,据报告,在2009年H1N1大流行影响和接种疫苗后,发病率有所增加。目前,缺乏具体的儿科诊断标准和截止仪器值。该疾病的临床表现在儿童和成人之间可能有所不同,误诊或诊断延迟仍然是一个问题。治疗是基于行为和药物治疗,但儿童的药物是在标签外开的。总的来说,儿童发作性睡病的发病机制和药物疗效数据仍然有限:需要更多的研究来开发新的药物,并使目前的治疗方法在儿科人群中获得批准。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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