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Expert Opin Pharmacother. 2017 Jun;18(8):809-817. doi: 10.1080/14656566.2017.1323877. Epub 2017 May 17.

Pharmacological management of narcolepsy with and without cataplexy.

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a Department of Neurology , Bern University Hospital and University of Bern , Bern , Switzerland.
b Department of Neurology, Narcolepsy-Center , HELIOS Klinik Hagen Ambrock , Hagen , Germany.
c Department of Rehabilitation , University of Witten/Herdecke , Witten , Germany.


Narcolepsy is an orphan neurological disease and presents with sleep-wake, motoric, neuropsychiatric and metabolic symptoms. Narcolepsy with cataplexy is most commonly caused by an immune-mediated process including genetic and environmental factors, resulting in the selective loss of hypocretin-producing neurons. Narcolepsy has a major impact on workableness and quality of life. Areas covered: This review provides an overview of the temporal available treatment options for narcolepsy (type 1 and 2) in adults, including authorization status by regulatory agencies. First- and second-line options are discussed as well as combination therapies. In addition, treatment options for frequent coexisting co-morbidities and different phenotypes of narcolepsy are presented. Finally, this review considers potential future management strategies. Non-pharmacological approaches are important in the management of narcolepsy but will not be covered in this review. Expert opinion: Concise evaluation of symptoms and type of narcolepsy, coexisting co-morbidities and patients┬┤ distinct needs is mandatory in order to identify a suitable, individual pharmacological treatment. First-line options include Modafinil/Armodafinil (for excessive daytime sleepiness, EDS), Sodium Oxybate (for EDS and/with cataplexy), Pitolisant (for EDS and cataplexy) and Venlafaxine (for cataplexy (off-label) and co-morbid depression). New symptomatic and causal treatment most probably will be completed by hypocretin-replacement and immune-modifying strategies.


Narcolepsy; cataplexy; co-morbidities; excessive daytime sleepiness; hypocretin; management; pharmacological treatment

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