Claudio L A Bassetti1, Ulf Kallweit2, Luca Vignatelli3, Giuseppe Plazzi3,4, Michel Lecendreux5,6, Elisa Baldin3, Leja Dolenc-Groselj7,8, Poul Jennum9, Ramin Khatami1,10, Mauro Manconi1,11, Geert Mayer12,13, Markku Partinen14, Thomas Pollmächer15, Paul Reading16, Joan Santamaria17, Karel Sonka18, Yves Dauvilliers19, Gert J Lammers20,21. 1. Department of Neurology, Inselspital, Bern University Hospital and University of Bern, Bern, Switzerland. 2. Center for Narcolepsy/Hypersomnias, Clin. Sleep and Neuroimmunology, Institute of Immunology, University Witten/Herdecke, Witten, Germany. 3. IRCCS, Istituto delle Scienze Neurologiche di Bologna, UOC Clinica Neurologica, Bologna, Italy. 4. Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, Modena, Italy. 5. AP-HP, Pediatric Sleep Center, CHU Robert-Debré, Paris, France. 6. National Reference Centre for Orphan Diseases, Narcolepsy, Idiopathic Hypersomnia and Kleine-Levin Syndrome (CNR narcolepsie-hypersomnie), Paris, France. 7. Institute of Clinical Neurophysiology, University Medical Centre Ljubljana, Ljubljana, Slovenia. 8. Department of Neurology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia. 9. Danish Center for Sleep Medicine, Department of Clinical Neurophysiology, Faculty of Health Sciences, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. 10. Center of Sleep Medicine, Sleep Research and Epileptology, Clinic Barmelweid, Barmelweid, Switzerland. 11. Sleep Center, Faculty of Biomedical Sciences, Neurocenter of Southern Switzerland, Università della Svizzera Italiana, Lugano, Switzerland. 12. Neurology Department, Hephata Klinik, Schwalmstadt, Germany. 13. Department of Neurology, Philipps-Universität Marburg, Marburg, Germany. 14. Department of Clinial Neurosciences, Clinicum, Helsinki Sleep Clinic, Vitalmed Research Center, Terveystalo Biobank and Clinical Research, University of Helsinki, Helsinki, Finland. 15. Center for Mental Health, Klinikum Ingolstadt, Ingolstadt, Germany. 16. Department of Neurology, James Cook University Hospital, Middlesbrough, UK. 17. Neurology Service, Hospital Clínic of Barcelona, Barcelona, Spain. 18. Department of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic. 19. National Reference Centre for Orphan Diseases, Narcolepsy, Idiopathic Hypersomnia, Sleep Unit, Department of Neurology, Gui-de-Chauliac Hospital, CHU Montpellier, University of Montpellier, INM INSERM, Montpellier, France. 20. Sleep Wake Centre SEIN, Heemstede, The Netherlands. 21. Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands.
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.
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.
Authors: Yves Dauvilliers; Richard K Bogan; Karel Šonka; Markku Partinen; Nancy Foldvary-Schaefer; Michael J Thorpy Journal: Nat Sci Sleep Date: 2022-03-29