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

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  • Claudio L A Bassetti
  • Ulf Kallweit
  • Luca Vignatelli
  • Giuseppe Plazzi
  • Michel Lecendreux
  • Elisa Baldin
  • Leja Dolenc-Groselj
  • Poul Jennum
  • Ramin Khatami
  • Mauro Manconi
  • Geert Mayer
  • Markku Partinen
  • Thomas Pollmächer
  • Paul Reading
  • Joan Santamaria
  • Karel Sonka
  • Yves Dauvilliers
  • Gert J Lammers
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BACKGROUND AND AIM: Narcolepsy is an uncommon hypothalamic disorder of presumed autoimmune origin that usually requires lifelong treatment. This paper aims to provide evidence-based guidelines for the management of narcolepsy in both adults and children.

METHODS: 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. According to the EAN recommendations, 10 relevant clinical questions were formulated in PICO format. Following a systematic review of the literature (performed in Fall 2018 and updated in July 2020) recommendations were developed according to the GRADE approach.

RESULTS: A total of 10,247 references were evaluated, 308 studies were assessed and 155 finally included. 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). Treatment choices should be tailored to each patient's symptoms, comorbidities, tolerance and risk of potential drug interactions.

CONCLUSION: 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.

Original languageEnglish
JournalEuropean Journal of Neurology
Issue number9
Pages (from-to)2815-2830
Number of pages16
Publication statusPublished - Sep 2021

Bibliographical note

© 2021 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.

    Research areas

  • Adult, Cataplexy, Child, Humans, Modafinil/therapeutic use, Narcolepsy/diagnosis, Sleep, Sodium Oxybate/therapeutic use

ID: 72157769