Last updated: March 23, 2022

Summarytoggle arrow icon

Narcolepsy is a neurological disorder of the sleep-wake cycle characterized by excessive daytime sleepiness and, in some cases, cataplexy, sleep paralysis, and hallucinations upon waking or falling asleep. It most commonly manifests in teenagers and young adults. Primary narcolepsy type 1 may manifest with cataplexy and/or orexin deficiency. Patients with type 2 primary narcolepsy have normal orexin levels. Secondary narcolepsy can occur as a result of brain damage or genetic syndromes. Diagnosis requires a history ≥ 3 months of excessive daytime sleepiness and either typical findings on polysomnography or an abnormal level of hypocretin-1 (orexin A) in the cerebrospinal fluid (CSF). There is no cure for narcolepsy but daytime sleepiness can be managed with optimized sleep hygiene and CNS stimulants or sodium oxybate.

Epidemiologytoggle arrow icon

Epidemiological data refers to the US, unless otherwise specified.

Etiologytoggle arrow icon

Primary narcolepsy

Secondary narcolepsy

Clinical featurestoggle arrow icon

  • Excessive daytime sleepiness (EDS): Affected individuals experience an irresistible urge to sleep and sudden, short sleep attacks (< 30 minutes), which may occur in inappropriate situations (e.g., while driving a car).
    • One of the earliest manifestations of narcolepsy
    • Can occur despite adequate sleep
  • Abnormal REM sleep
    • Cataplexy: sudden muscle weakness in a fully conscious person, triggered by strong emotions (e.g., laughing, crying)
      • Typically manifests months or even years after EDS
      • The loss of muscle tone is similar to that observed during REM sleep.
      • Typically manifests as partial cataplexy: isolated weakness of distinct muscle groups (e.g., neck muscles weaken and head tilts forward)
      • Usually resolves within a few seconds, at most two minutes
    • Sleep paralysis: Complete paralysis occurs for 1–2 minutes after waking or before falling asleep (either during a nocturnal or narcoleptic sleep episode, i.e., begins or ends with REM sleep)
  • Sleep hallucinations
  • Automatic behavior: During narcoleptic episodes, patients often perform routine repetitive tasks automatically without conscious awareness of their environment.
  • Other: : depression, obesity, impotence or low sex drive, headaches, decreased functional performance

Hypnagogic hallucinations occur while going to sleep.

Diagnosticstoggle arrow icon

Approach [8][9][10]

Diagnostic criteria [8][10][11]

Sleep studies [12]

  • Daytime multiple sleep latency test (MSLT)
    • Includes 5 opportunities for the patient to nap during the daytime and measures :
      • Sleep latency: time needed to fall asleep
      • Sleep-onset REM periods (SOREMPs): REM periods that occur within 15 minutes of falling asleep; also referred to as shortened REM sleep latency
    • Characteristic findings
  • Nocturnal polysomnography (PSG)
    • Measures sleep duration, efficiency, and stages
    • Used to exclude other sleep disorders and may also show supportive findings for narcolepsy (e.g., SOREMP)

If feasible, medications affecting sleep (e.g., antidepressants and stimulants) should be paused for at least two weeks prior to a sleep study. [12]

Additional tests

Treatmenttoggle arrow icon

General measures

  • Optimize sleep hygiene. [9]
    • Ensure regular sleep periods during the night.
    • Avoid substances that disturb the sleep-wake cycle (e.g., alcohol, antipsychotics, opiates).
  • Consider scheduled naps throughout the day to reduce the urge to sleep. [16]

As motor vehicle collisions are a concern for patients with narcolepsy, to be allowed to drive, they should be symptom-free and taking treatment. State regulations vary on the legally required period of time that patients should be symptom-free before driving.

Medical therapy [17]

Principles of medical therapy

Commonly used agents

Sodium oxybate (gamma-hydroxybutyrate) may cause life-threatening respiratory depression and should never be taken with alcohol or other CNS depressants. Since it is used recreationally to induce sedation and euphoria, sodium oxybate has a high potential for misuse. [17]

Stimulants recommended for treating narcolepsy may cause fetal harm and reduce the effectiveness of oral contraception. [17]

Prognosistoggle arrow icon

  • Currently no cure available [16]
  • Associated with higher rates of morbidity (e.g., cardiovascular disease) [21][22]
  • Increased risk of motor vehicle accidents (adequate treatment may mitigate risk) [23]

Referencestoggle arrow icon

  1. Longstreth WT et al.. The Epidemiology of Narcolepsy. Sleep. 2007; 30 (1): p.13-26.doi: 10.1093/sleep/30.1.13 . | Open in Read by QxMD
  2. Chieffi S, Carotenuto M, Monda V, et al. Orexin System: The Key for a Healthy Life. Frontiers in Physiology. 2017; 8.doi: 10.3389/fphys.2017.00357 . | Open in Read by QxMD
  3. Jennum P. Definition, Epidemiology and Socioeconomic Burden of Narcolepsy. US Neurology. 2008; 04 (01): p.57.doi: 10.17925/usn.2008.04.01.57 . | Open in Read by QxMD
  4. Sarkanen TO, Alakuijala APE, Dauvilliers YA, Partinen MM. Incidence of narcolepsy after H1N1 influenza and vaccinations: Systematic review and meta-analysis. Sleep Med Rev. 2017; 38: p.177-186.doi: 10.1016/j.smrv.2017.06.006 . | Open in Read by QxMD
  5. Edwards K, Hanquet G, Black S, et al. Meeting report narcolepsy and pandemic influenza vaccination: What we know and what we need to know before the next pandemic? A report from the 2nd IABS meeting. Biologicals. 2019; 60: p.1-7.doi: 10.1016/j.biologicals.2019.05.005 . | Open in Read by QxMD
  6. Narcolepsy Fact Sheet. Updated: January 1, 2017. Accessed: March 29, 2017.
  7. Kim LJ, Coelho FM, Hirotsu C, et al. Frequencies and associations of narcolepsy-related symptoms: a cross-sectional study. J Clin Sleep Med. 2015; 11 (12): p.1377-1384.doi: 10.5664/jcsm.5268 . | Open in Read by QxMD
  8. Kallweit U, Schmidt M, Bassetti CL. Patient-Reported Measures of Narcolepsy: The Need for Better Assessment. J Clin Sleep Med. 2017; 13 (05): p.737-744.doi: 10.5664/jcsm.6596 . | Open in Read by QxMD
  9. Ramar K, Olson EJ. Management of common sleep disorders. Am Fam Physician. 2013; 88 (4): p.231-238.
  10. Bassetti CLA, Adamantidis A, Burdakov D, et al. Narcolepsy — clinical spectrum, aetiopathophysiology, diagnosis and treatment. Nat Rev Neurol. 2019; 15 (9): p.519-539.doi: 10.1038/s41582-019-0226-9 . | Open in Read by QxMD
  11. American Academy of Sleep Medicine. International classification of sleep disorders, 3rd edition. American Academy of Sleep Medicine
  12. Markun LC, Sampat A. Clinician-Focused Overview and Developments in Polysomnography. Curr Sleep Med Rep. 2020; 6 (4): p.309-321.doi: 10.1007/s40675-020-00197-5 . | Open in Read by QxMD
  13. Gabryelska A, Szmyd B, Maschauer EL, et al. Utility of measuring CSF hypocretin-1 level in patients with suspected narcolepsy. Sleep Med. 2020; 71: p.48-51.doi: 10.1016/j.sleep.2020.03.009 . | Open in Read by QxMD
  14. Mignot E, Lammers GJ, Ripley B, et al. The Role of Cerebrospinal Fluid Hypocretin Measurement in the Diagnosis of Narcolepsy and Other Hypersomnias. Arch Neurol. 2002; 59 (10): p.1553.doi: 10.1001/archneur.59.10.1553 . | Open in Read by QxMD
  15. Baumann CR, Mignot E, Lammers GJ, et al. Challenges in Diagnosing Narcolepsy without Cataplexy: A Consensus Statement. Sleep. 2014; 37 (6): p.1035-1042.doi: 10.5665/sleep.3756 . | Open in Read by QxMD
  16. Bassetti CLA, Kallweit U, Vignatelli L, et al. European guideline and expert statements on the management of narcolepsy in adults and children. J Sleep Res. 2021.doi: 10.1111/jsr.13387 . | Open in Read by QxMD
  17. Maski K, Trotti LM, Kotagal S, et al. Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine clinical practice guideline. J Clin Sleep Med. 2021; 17 (9): p.1881-1893.doi: 10.5664/jcsm.9328 . | Open in Read by QxMD
  18. Morgenthaler TI, Kapur VK, Brown T, et al. Practice parameters for the treatment of narcolepsy and other hypersomnias of central origin.. Sleep. 2007; 30 (12): p.1705-11.doi: 10.1093/sleep/30.12.1705 . | Open in Read by QxMD
  19. Thorpy MJ, Shapiro C, Mayer G, et al. A randomized study of solriamfetol for excessive sleepiness in narcolepsy. Ann Neurol. 2019; 85 (3): p.359-370.doi: 10.1002/ana.25423 . | Open in Read by QxMD
  20. Dauvilliers Y et al.. Pitolisant versus placebo or modafinil in patients with narcolepsy: a double-blind, randomised trial. The Lancet Neurology. 2013; 12 (11): p.1068-1075.doi: 10.1016/s1474-4422(13)70225-4 . | Open in Read by QxMD
  21. Jennum P, Thorstensen EW, Pickering L, Ibsen R, Kjellberg J. Morbidity and mortality of middle-aged and elderly narcoleptics. Sleep Med. 2017; 36: p.23-28.doi: 10.1016/j.sleep.2017.03.029 . | Open in Read by QxMD
  22. Ohayon MM, Black J, Lai C, Eller M, Guinta D, Bhattacharyya A. Increased Mortality in Narcolepsy. Sleep. 2014; 37 (3): p.439-444.doi: 10.5665/sleep.3470 . | Open in Read by QxMD
  23. Pizza F, Jaussent I, Lopez R, et al. Car Crashes and Central Disorders of Hypersomnolence: A French Study. PLoS ONE. 2015; 10 (6): p.e0129386.doi: 10.1371/journal.pone.0129386 . | Open in Read by QxMD

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