European guideline and expert statements on the management of narcolepsy in adults and children

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Narcolepsy is a disabling hypothalamic disorder that presents with a variety of sleep-wake and other symptoms. 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 [Bassetti et al., 2019; Yoss & Daly, 1957]). 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 a heterogeneous disorder (Bassetti et al., 2019).

Although the precise etiology of narcolepsy is unknown, most evidence suggests it is usually a sporadic acquired immune-mediated condition that develops in people who are genetically predisposed (Latorre et al., 2018; Liblau et al., 2015; Partinen et al., 2014; Yoss & Daly, 1957).

Worldwide prevalence estimates suggest that approximately 25–50 persons out of 100,000 are affected (Oyahon et al., 2002; Partinen & Kronholm, 2017). Some recent studies indicate, that narcolepsy may be less frequent (Tió et al., 2018). Narcolepsy potentially affects every aspect of daily life with considerable personal, social and economic consequences. As a result, quality of life measures of both patients and their families are significantly reduced (Dodel et al., 2007; Jennum et al., 2012). Current treatments include non-pharmacological and pharmacological approaches (Bassetti et al., 2019; Dauvilliers et al., 2017; Kallweit & Bassetti, 2017).

Treatment guidelines for narcolepsy were first published in Europe in 2006 (Billiard, Bassetti et al., 2006) and in the USA in 2007 (Morgenthaler et al., 2007). Criteria for diagnosing narcolepsy into types 1 and 2 (NT1 and NT2) were revised in 2014 by the American Academy of Sleep Medicine. A recent paper addressed the limitations of the current International Classification of Sleep Disorders 3 (ICSD-3) diagnostic criteria and made suggestions for future improvements (Lammers et al., 2020).

Since 2006, there has been considerable developments in our understanding of both the etiopathology and clinical characteristics in narcolepsy. Furthermore, several new drugs have become available. This has prompted the European Academy of Neurology (EAN), European Sleep Research Society (ESRS) and European Narcolepsy Network (EU-NN) to join forces to provide up-to-date, evidence-based recommendations for narcolepsy treatment

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