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

Bassetti, Claudio L. A.; Kallweit, Ulf; Vignatelli, Luca; Plazzi, Giuseppe; Lecendreux, Michel; Baldin, Elisa; Dolenc-Groselj, Leja; Jennum, Poul; Khatami, Ramin; Manconi, Mauro; Mayer, Geert; Partinen, Markku; Pollmächer, Thomas; Reading, Paul; Santamaria, Joan; Sonka, Karel; Dauvilliers, Yves; Lammers, Gert J (2021). European guideline and expert statements on the management of narcolepsy in adults and children. European journal of neurology, 28(9), pp. 2815-2830. Wiley 10.1111/ene.14888

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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.

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