Abstract | 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.
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Authors | 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 |
Journal | Journal of sleep research
(J Sleep Res)
Vol. 30
Issue 6
Pg. e13387
(12 2021)
ISSN: 1365-2869 [Electronic] England |
PMID | 34173288
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review, Systematic Review)
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Copyright | © 2021 The Authors. Journal of Sleep Research published by John Wiley & Sons Ltd on behalf of European Academy of Neurology and European Sleep Research Society. |
Chemical References |
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Topics |
- Adult
- Cataplexy
- Child
- Humans
- Modafinil
(therapeutic use)
- Narcolepsy
(diagnosis, drug therapy)
- Sleep
- Sodium Oxybate
(therapeutic use)
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