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

Mauro Manconi, Gert J Lammers, Yves Dauvilliers, Karel Sonka, Joan Santamaria, Paul Reading, Thomas Pollmächer, Markku Partinen, Geert Mayer, Claudio L A Bassetti, Ramin Khatami, Poul Jennum, Leja Dolenc-Groselj, Elisa Baldin, Michel Lecendreux, Giuseppe Plazzi, Luca Vignatelli, Ulf Kallweit

Journal: Journal of sleep research 2021;30(6):e13387

PMID: 34173288

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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.

Address: Department of Neurology, Inselspital, Bern University Hospital and University of Bern, Bern, Switzerland.; Center for Narcolepsy/Hypersomnias, Clin. Sleep and Neuroimmunology, Institute of Immunology, University Witten/Herdecke, Witten, Germany.; IRCCS, Istituto delle Scienze Neurologiche di Bologna, UOC Clinica Neurologica, Bologna, Italy.; Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, Modena, Italy.; AP-HP, Pediatric Sleep Center, CHU Robert-Debré, Paris, France.; National Reference Centre for Orphan Diseases, Narcolepsy, Idiopathic Hypersomnia and Kleine-Levin Syndrome (CNR narcolepsie-hypersomnie), Paris, France.; Institute of Clinical Neurophysiology, University Medical Centre Ljubljana, Ljubljana, Slovenia.; Department of Neurology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.; Danish Center for Sleep Medicine, Department of Clinical Neurophysiology, Faculty of Health Sciences, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.; Center of Sleep Medicine, Sleep Research and Epileptology, Clinic Barmelweid, Barmelweid, Switzerland.; Sleep Center, Faculty of Biomedical Sciences, Neurocenter of Southern Switzerland, Università della Svizzera Italiana, Lugano, Switzerland.; Neurology Department, Hephata Klinik, Schwalmstadt, Germany.; Department of Neurology, Philipps-Universität Marburg, Marburg, Germany.; Department of Clinial Neurosciences, Clinicum, Helsinki Sleep Clinic, Vitalmed Research Center, Terveystalo Biobank and Clinical Research, University of Helsinki, Helsinki, Finland.; Center for Mental Health, Klinikum Ingolstadt, Ingolstadt, Germany.; Department of Neurology, James Cook University Hospital, Middlesbrough, UK.; Neurology Service, Hospital Clínic of Barcelona, Barcelona, Spain.; Department of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic.; 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.; Sleep Wake Centre SEIN, Heemstede, The Netherlands.; Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands.

DOI: 10.1111/jsr.13387

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MeSH Terms: Adult, Cataplexy, Child, Humans, Modafinil, Narcolepsy, Sleep, Sodium Oxybate