J Korean Neuropsychiatr Assoc.  2020 Feb;59(1):25-28. 10.4306/jknpa.2020.59.1.25.

Recent Updates on Narcolepsy and Hypersomnia

  • 1Department of Psychiatry, St. Vicent's Hospital, College of Medicine, The Catholic University of Korea, Suwon, Korea. hscjohn@hotmail.com


Narcolepsy is a chronic neurological sleep disorder caused by hypocretin neuron loss, resulting in excessive daytime sleepiness, disturbed nocturnal sleep, and intrusions of aspects of rapid eye movement sleep in wakefulness, such as cataplexy, sleep paralysis, and hypnopompic/hypnagogic hallucinations. Narcolepsy disrupts the maintenance and orderly occurrence of the wake and sleep stages. Cataplexy is a highly specific symptom of narcolepsy, but many other symptoms can be observed in a variety of sleep disorders. The diagnosis of narcolepsy type 1 requires a history of excessive daytime sleepiness and one of the following : 1) a low cerebrospinal fluid hypocretin-1 level or 2) cataplexy and a positive multiple sleep latency test result. The diagnosis of narcolepsy type 2 requires a history of excessive daytime sleepiness and a positive mean sleep-latency test result. The mean sleep-latency test must be preceded by nighttime polysomnography to exclude other sleep disorders and to document adequate sleep. The mean sleep-latency test result can be falsely positive in other sleep disorders, such as shift work, sleep apnea, or sleep deprivation, and it is influenced by age, sex, and puberty. Modafinil and armodafinil can reduce the excessive daytime sleepiness without many of the side effects associated with older stimulants. Although there is no cure for narcolepsy, the treatments are often effective and include both behavioral and pharmacologic approaches.


Narcolepsy; Narcolepsy cataplexy syndrome; Hypersomnia; Hypersomnolence disorders

MeSH Terms

Cerebrospinal Fluid
Disorders of Excessive Somnolence*
Sleep Apnea Syndromes
Sleep Deprivation
Sleep Paralysis
Sleep Stages
Sleep Wake Disorders
Sleep, REM


1). Dauvilliers Y, Arnulf I, Mignot E. Narcolepsy with cataplexy. Lancet. 2007; 369:499–511.
2). Aldrich MS. The clinical spectrum of narcolepsy and idiopathic hypersomnia. Neurology. 1996; 46:393–401.
3). American Academy of Sleep Medicine. International classification of sleep disorders. 3rd ed.Darien, IL: American Academy of Sleep Medicine;2014.
4). Dauvilliers Y, Montplaisir J, Molinari N, Carlander B, Ondze B, Besset A, et al. Age at onset of narcolepsy in two large populations of patients in France and Quebec. Neurology. 2001; 57:2029–2033.
5). Zeman A, Britton T, Douglas N, Hansen A, Hicks J, Howard R, et al. Narcolepsy and excessive daytime sleepiness. BMJ. 2004; 329:724–728.
6). Mattarozzi K, Bellucci C, Campi C, Cipolli C, Ferri R, Franceschini C, et al. Clinical, behavioural and polysomnographic correlates of cataplexy in patients with narcolepsy/cataplexy. Sleep Med. 2008; 9:425–433.
7). Overeem S, van Nues SJ, van der Zande WL, Donjacour CE, van Mierlo P, Lammers GJ. The clinical features of cataplexy: a questionnaire study in narcolepsy patients with and without hypocretin-1 deficiency. Sleep Med. 2011; 12:12–18.
8). Ohayon MM, Priest RG, Caulet M, Guilleminault C. Hypnagogic and hypnopompic hallucinations: pathological phenomenon? Br J Psychiatry. 1996; 169:459–467.
9). Guilleminault C, Eldridge F, Dement WC. Insomnia, narcolepsy, and sleep apneas. Bull Physiopathol Respir (Nancy). 1972; 8:1127–1138.
10). Morrish E, King MA, Smith IE, Shneerson JM. Factors associated with a delay in the diagnosis of narcolepsy. Sleep Med. 2004; 5:37–41.
11). Mignot E, Hayduk R, Black J, Grumet FC, Guilleminault C. HLA DQB1∗0602 is associated with cataplexy in 509 narcoleptic patients. Sleep. 1997; 20:1012–1020.
12). Pelin Z, Guilleminault C, Risch N, Grumet FC, Mignot E. HLA-DQB1∗0602 homozygosity increases relative risk for narcolepsy but not disease severity in two ethnic groups. US Modafinil in Narcolepsy Multicenter Study Group. Tissue Antigens. 1998; 51:96–100.
13). Haba-Rubio J, Rossetti AO, Tafti M, Heinzer R. [Narcolepsy with cataplexy associated with H1N1 vaccination]. Rev Neurol (Paris). 2011; 167:563–566.
14). Han F, Lin L, Warby SC, Faraco J, Li J, Dong SX, et al. Narcolepsy onset is seasonal and increased following the 2009 H1N1 pandemic in China. Ann Neurol. 2011; 70:410–417.
15). Poli F, Overeem S, Lammers GJ, Plazzi G, Lecendreux M, Bassetti CL, et al. Narcolepsy as an adverse event following immunization: case definition and guidelines for data collection, analysis and presentation. Vaccine. 2013; 31:994–1007.
16). Ohayon MM, Ferini-Strambi L, Plazzi G, Smirne S, Castronovo V. How age influences the expression of narcolepsy. J Psychosom Res. 2005; 59:399–405.
17). Mignot E, Lammers GJ, Ripley B, Okun M, Nevsimalova S, Overeem S, et al. The role of cerebrospinal fluid hypocretin measurement in the diagnosis of narcolepsy and other hypersomnias. Arch Neurol. 2002; 59:1553–1562.
18). de Lecea L, Kilduff TS, Peyron C, Gao X, Foye PE, Danielson PE, et al. The hypocretins: hypothalamus-specific peptides with neuroex-citatory activity. Proc Natl Acad Sci U S A. 1998; 95:322–327.
19). Lin L, Faraco J, Li R, Kadotani H, Rogers W, Lin X, et al. The sleep disorder canine narcolepsy is caused by a mutation in the hypocretin (orexin) receptor 2 gene. Cell. 1999; 98:365–376.
20). Nishino S, Ripley B, Overeem S, Lammers GJ, Mignot E. Hypocretin (orexin) deficiency in human narcolepsy. Lancet. 2000; 355:39–40.
21). Baumann CR, Mignot E, Lammers GJ, Overeem S, Arnulf I, Rye D, et al. Challenges in diagnosing narcolepsy without cataplexy: a consensus statement. Sleep. 2014; 37:1035–1042.
22). Moscovitch A, Partinen M, Guilleminault C. The positive diagnosis of narcolepsy and narcolepsy's borderland. Neurology. 1993; 43:55–60.
23). van den Hoed J, Kraemer H, Guilleminault C, Zarcone VP Jr, Miles LE, Dement WC, et al. Disorders of excessive daytime somnolence: polygraphic and clinical data for 100 patients. Sleep. 1981; 4:23–37.
24). Boutrel B, Koob GF. What keeps us awake: the neuropharmacology of stimulants and wakefulness-promoting medications. Sleep. 2004; 27:1181–1194.
25). Mignot E, Nishino S, Guilleminault C, Dement WC. Modafinil binds to the dopamine uptake carrier site with low affinity. Sleep. 1994; 17:436–437.
26). Lankford DA. Armodafinil: a new treatment for excessive sleepiness. Expert Opin Investig Drugs. 2008; 17:565–573.
27). Wozniak DR, Quinnell TG. Unmet needs of patients with narcolepsy: perspectives on emerging treatment options. Nat Sci Sleep. 2015; 7:51–61.
28). Kent JM. SNaRIs, NaSSAs, and NaRIs: new agents for the treatment of depression. Lancet. 2000; 355:911–918.
Full Text Links
  • JKNA
export Copy
  • Twitter
  • Facebook
Similar articles
Copyright © 2022 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr