TY - JOUR

T1 - European guideline and expert statements on the management of narcolepsy in adults and children

AU - Bassetti, Claudio L. A.

AU - Kallweit, Ulf

AU - Vignatelli, Luca

AU - Plazzi, Giuseppe

AU - Lecendreux, Michel

AU - Baldin, Elisa

AU - Dolenc-Groselj, Leja

AU - Jennum, Poul

AU - Khatami, Ramin

AU - Manconi, Mauro

AU - Mayer, Geert

AU - Partinen, Markku

AU - Pollmaecher, Thomas

AU - Reading, Paul

AU - Santamaria, Joan

AU - Sonka, Karel

AU - Dauvilliers, Yves

AU - Lammers, Gert J.

PY - 2021

Y1 - 2021

N2 - 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.

AB - 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.

KW - cataplexy

KW - European

KW - guideline

KW - management

KW - narcolepsy

KW - EXCESSIVE DAYTIME SLEEPINESS

KW - QUALITY-OF-LIFE

KW - ADMINISTERED SODIUM OXYBATE

KW - ONSET CHILDHOOD NARCOLEPSY

KW - GAMMA-HYDROXYBUTYRATE GHB

KW - STEVENS-JOHNSON SYNDROME

KW - PERIODIC LEG MOVEMENTS

KW - ORAL JZP-110 ADX-N05

KW - LONG-TERM USE

KW - DOUBLE-BLIND

U2 - 10.1111/ene.14888

DO - 10.1111/ene.14888

M3 - Journal article

C2 - 34173695

VL - 28

SP - 2815

EP - 2830

JO - European Journal of Neurology

JF - European Journal of Neurology

SN - 1351-5101

IS - 9

ER -