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

Claudio L A Bassetti¹, Ulf Kallweit², Luca Vignatelli³, Giuseppe Plazzi^{3

4}, Michel Lecendreux^{5

6}, Elisa Baldin³, Leja Dolenc-Groselj^{7

8}, Poul Jennum⁹, Ramin Khatami^{1

10}, Mauro Manconi^{1

11}, Geert Mayer^{12

13}, Markku Partinen¹⁴, Thomas Pollmächer¹⁵, Paul Reading¹⁶, Joan Santamaria¹⁷, Karel Sonka¹⁸, Yves Dauvilliers¹⁹, Gert J Lammers^{20

21}

Affiliations

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.

PMID: 34173288
DOI: 10.1111/jsr.13387

Free article

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

Claudio L A Bassetti et al. J Sleep Res. 2021 Dec.

Free article

. 2021 Dec;30(6):e13387.

doi: 10.1111/jsr.13387. Epub 2021 Jun 25.

Authors

Affiliations

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.

PMID: 34173288
DOI: 10.1111/jsr.13387

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.

Keywords: European; cataplexy; guideline; management; narcolepsy.

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References

REFERENCES

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

Affiliations

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

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