Guideline sources The following summarized guidelines for the management of narcolepsy are prepared by our editorial team based on guidelines from the British Thoracic Society (BTS 2023), the European Academy of Neurology (EAN 2021), and the American Academy of Sleep Medicine (AASM 2021). 1 2 3 Guidelines 1. Medical management Management of excessive daytime sleepiness: As per AASM 2021 guidelines: Offer the following medications for the treatment of excessive daytime sleepiness in adult patients with narcolepsy: modafinil pitolisant sodium oxybate solriamfetol. A https://web.pathway.md/diseases/rec8Chs8jAjkFuT4P 1/4 6/30/23, 1:14 AM Narcolepsy Pathway Consider offering the following medications for the treatment of excessive daytime sleepiness in adult patients with narcolepsy: armodafinil dextroamphetamine methylphenidate. C As per EAN 2021 guidelines, offer the following medications as first-line therapy for excessive daytime sleepiness in adult patients with narcolepsy: modafinil pitolisant sodium oxybate solriamfetol. B Show 3 more Management of cataplexy: As per AASM 2021 guidelines: Offer the following medications for the treatment of cataplexys in adult patients with narcolepsy: pitolisant sodium oxybate. A Consider offering dextroamphetamine for the treatment of cataplexy in adult patients with narcolepsy. C As per EAN 2021 guidelines, offer sodium oxybate for the treatment of cataplexy in adult patients with narcolepsy type 1. B Show 3 more Management of disturbed nighttime sleep: offer sodium oxybate for the treatment of moderate- to-severe disturbed nighttime sleep in patients with narcolepsy. B Show 3 more Management of sleep paralysis and sleep-related hallucinations: Consider offering the following medications for the treatment of moderate-to-severe sleep paralysis and/or sleep-related hallucinations in patients with narcolepsy: sodium oxybate pitolisant antidepressants. C Agents with no evidence for benefit: Do not use the following agents in patients with narcolepsy, at any age: corticosteroids monoclonal antibodies IVIG. D https://web.pathway.md/diseases/rec8Chs8jAjkFuT4P 2/4 6/30/23, 1:14 AM Narcolepsy Pathway 2. Nonpharmacologic interventions Scheduled naps: offer planned daytime naps to improve immediate subjective and objective sleepiness both in drug-na ve patients and in patients taking stimulant medication, at any age. B 3. Therapeutic procedures Plasmapheresis: do not perform plasmapheresis in patients with narcolepsy, at any age. D 4. Specific circumstances Pediatric patients (evaluation): suspect other diagnoses, including narcolepsy, with possible coexistent cataplexy, sleep paralysis, hypnagogic and hypnopompic hallucinations, and circadian rhythm disorders, in pediatric patients with persisting excessive daytime sleepiness despite exclusion or effective treatment of sleep-disordered breathing. B Show 3 more Pediatric patients, management: As per BTS 2023 guidelines, manage pediatric patients with narcolepsy by a clinician with special expertise in the management of narcolepsy, either by a pediatric neurologist or a sleep physician, depending on local service arrangements. E As per AASM 2021 guidelines: Consider offering the following medications for the treatment of excessive daytime sleepiness in pediatric patients with narcolepsy: modafinil sodium oxybate. C Consider offering sodium oxybate for the treatment of cataplexy in pediatric patients with narcolepsy. C As per EAN 2021 guidelines, offer sodium oxybate for the treatment of excessive daytime sleepiness, cataplexy, and disturbed nighttime sleep in > 6 years old pediatric patients with narcolepsy. Obtain a regular careful benefit-risk ratio assessment. B Show 5 more Patients with other sleep disorders: Consider offering positive airways pressure for the treatment of moderate-to-severe sleep- disordered breathing in patients with narcolepsy, in accordance to the general principles in non- narcoleptic patients with sleep apnea. C Treat restless leg syndrome/periodic leg movements and REM sleep behavior disorder in accordance to the general principles in non-narcoleptic patients. B Clinical findings https://web.pathway.md/diseases/rec8Chs8jAjkFuT4P 3/4 6/30/23, 1:14 AM Narcolepsy Pathway Symptoms Neurological exam Excessive daytime sleepiness Cataplexy Frequent nighttime awakenings Hypersomnia Sleepiness Parasomnia Sleeping disorder Sleep paralysis Sleep-related hallucinations References 1. Claudio L A Bassetti, Ulf Kallweit, Luca Vignatelli et al. European guideline and expert statements on the management of narcolepsy in adults and children. Eur J Neurol. 2021 Sep;28 9 2815 2830. Open 2. Hazel J Evans, Neil A Gibson, Joanna Bennett et al. British Thoracic Society guideline for diagnosing and monitoring paediatric sleep-disordered breathing. Thorax. 2023 Jun;78 Suppl 2):s1-s27. Open 3. Kiran Maski, Lynn Marie Trotti, Suresh Kotagal et al. Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine clinical practice guideline. J Clin Sleep Med. 2021 Sep 1;17 9 1881 1893. Open 4. M Billiard, C Bassetti, Y Dauvilliers et al. EFNS guidelines on management of narcolepsy. Eur J Neurol. 2006 Oct;13 10 1035 48. Open https://web.pathway.md/diseases/rec8Chs8jAjkFuT4P 4/4