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
  • Jennum, Poul
  • Ramin Khatami
  • Mauro Manconi
  • Geert Mayer
  • Markku Partinen
  • Thomas Pollmaecher
  • Paul Reading
  • Joan Santamaria
  • Karel Sonka
  • Yves Dauvilliers
  • Gert J. Lammers

Background and purpose 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 (EDS) in adults-scheduled naps, modafinil, pitolisant, sodium oxybate (SXB), solriamfetol (all strong); methylphenidate, amphetamine derivatives (both weak); (ii) cataplexy in adults-SXB, venlafaxine, clomipramine (all strong) and pitolisant (weak); (iii) EDS in children-scheduled naps, SXB (both strong), modafinil, methylphenidate, pitolisant, amphetamine derivatives (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.

OriginalsprogEngelsk
TidsskriftEuropean Journal of Neurology
Vol/bind28
Udgave nummer9
Sider (fra-til)2815-2830
ISSN1351-5101
DOI
StatusUdgivet - 2021

ID: 286856573