Worldwide prevalence estimates suggest that approximately 25–50 persons out of 100,000 are affected. Although the precise aetiology of narcolepsy is unknown, most evidence suggests it is usually a sporadic acquired immune-mediated condition that develops in people who are genetically predisposed. Whereas narcolepsy with typical cataplexy (type 1 narcolepsy, NT1) is considered a distinct entity, associated with hypocretin deficiency, narcolepsy without cataplexy (type 2 narcolepsy, NT2) is less clearly defined and when diagnosed following the current diagnostic criteria is a heterogeneous disorder. Excessive daytime sleepiness (EDS) is usually the most troublesome feature although significantly fragmented and disturbed nighttime sleep (DNS) is common with phenomena including sleep paralysis and hallucinatory experiences around sleep–wake transitions (hypnagogic and hypnopompic hallucinations). Narcolepsy is a disabling hypothalamic disorder that presents with a variety of sleep–wake and other symptoms. INTRODUCTION: BACKGROUND AND SCOPE Clinical need for a guideline The management of narcolepsy involves non-pharmacological and pharmacological approaches with an increasing number of symptomatic treatment options for adults and children that have been studied in some detail. Treatment choices should be tailored to each patient’s symptoms, comorbidities, tolerance and risk of potential drug interactions. The main recommendations can be summarized as follows: (i) excessive daytime sleepiness in adults-scheduled naps, modafinil, pitolisant, sodium oxybate (SXB), solriamfetol (all strong), methylphenidate, amphetamine derivates (both weak) (ii) cataplexy in adults-SXB, venlafaxine, clomipramine (all strong) and pitolisant (weak) (iii) excessive daytime sleepiness in children-scheduled naps, SXB (both strong), modafinil, methylphenidate, pitolisant, amphetamine derivates (all weak) (iv) cataplexy in children-SXB (strong), antidepressants (weak). ResultsĪ total of 10,247 references were evaluated, 308 studies were assessed and 155 finally included. Following a systematic review of the literature (performed in Fall 2018 and updated in July 2020) recommendations were developed according to the GRADE approach. According to the EAN recommendations, 10 relevant clinical questions were formulated in PICO format. The European Academy of Neurology (EAN), European Sleep Research Society (ESRS) and European Narcolepsy Network (EU-NN) nominated a task force of 18 narcolepsy specialists. This paper aims to provide evidence-based guidelines for the management of narcolepsy in both adults and children. All rights reserved.Narcolepsy is an uncommon hypothalamic disorder of presumed autoimmune origin that usually requires lifelong treatment. We performed a review of literature (MEDLINE and EMBASE database), without limited date or publication restrictions.Īngelman Syndrome Cataplexy Narcolepsy Niemann-Pick type C Disease Norrie Disease Prader-Willi Syndrome.Ĭopyright © 2019 Elsevier B.V. In this review, we described few conditions of cataplexy not related to narcolepsy. Therefore, it is essential disconnecting cataplexy from narcolepsy especially in pediatric population and after use of a few medications. Yet, the most prevalent causes of cataplexy without narcolepsy are rare genetic diseases which explains why cataplexy is classically linked to narcolepsy. In addition, cataplexy can be a side effect of several drugs (eg, lamotrigine, clozapine, and gamma-hydroxybutyrate). Other causes of cataplexy include Niemann-Pick type C Disease, Angelman Syndrome, Norrie Disease, Prader-Willi Syndrome. Cataplexy is a transient loss of muscle tone that can be triggered by emotions such as laughter, excitement or fear.
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