European guideline and expert statements on the managementof narcolepsy in adults and children

    Eur. j. neurol; 28 (9), 2021
    Año de publicación: 2021

    Narcolepsy is an uncommon hypothalamic disorder of presumedautoimmune origin that usually requires lifelong treatment. This paper aims to provideevidence-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 nar-colepsy specialists. According to the EAN recommendations, 10 relevant clinical ques-tions were formulated in PICO format. Following a systematic review of the literature(performed in Fall 2018 and updated in July 2020) recommendations were developedaccording to the GRADE approach.

    Results:

    A total of 10,247 references were evaluated, 308 studies were assessed and155 finally included.

    The main recommendations can be summarized as follows:

    (i) exces-sive 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 pa-tient’s symptoms, comorbidities, tolerance and risk of potential drug interactions.

    Conclusion:

    The management of narcolepsy involves non-pharmacological and pharma-cological approaches with an increasing number of symptomatic treatment options foradults and children that have been studied in some detail.