- Ronald D Chervin, MD, MS
Ronald D Chervin, MD, MS
- Editor-in-Chief — Sleep Medicine
- Section Editor — Pediatric Sleep Medicine
- Professor of Neurology
- University of Michigan
Idiopathic hypersomnia is a sleep disorder that is characterized by chronic excessive daytime sleepiness (daily periods of irrepressible need to sleep or daytime lapses into sleep) and often difficulty waking up from nocturnal sleep or daytime naps. The condition is categorized as a central disorder of hypersomnolence; other sleep disorders in this category include narcolepsy type 1 and type 2 and Kleine-Levin syndrome (recurrent hypersomnia) .
The pathophysiology of idiopathic hypersomnia is not well understood, and diagnosis requires exclusion of other more common causes of excessive sleepiness. When a diagnosis of idiopathic hypersomnia is established, treatment is largely pharmacologic and aimed at control of symptoms, with generally good, but somewhat variable results.
This topic will discuss the epidemiology, clinical features, diagnosis, and treatment of idiopathic hypersomnia. An approach to the patient with excessive daytime sleepiness is presented separately. (See "Approach to the patient with excessive daytime sleepiness".)
The prevalence of idiopathic hypersomnia in the general population is unknown , in part because laboratory-based polysomnography and other assessments that would be necessary to rule out other causes of excessive daytime sleepiness have not been feasible in large numbers of unselected, representative subjects from the community. Based largely on sleep center referrals, idiopathic hypersomnia appears to be one-tenth to one-half as common as narcolepsy, suggesting a prevalence of approximately 20 to 50 cases per million [3-6]. There is no clear gender association.
The onset of idiopathic hypersomnia typically occurs between 10 and 30 years of age. In a series of 77 patients, the mean age of symptom onset was 17 and the mean age of diagnosis was 30 .
- American Academy of Sleep Medicine. International Classification of Sleep Disorders, 3rd ed, American Academy of Sleep Medicine, Darien, IL 2014.
- Ohayon MM. From wakefulness to excessive sleepiness: what we know and still need to know. Sleep Med Rev 2008; 12:129.
- Anderson KN, Pilsworth S, Sharples LD, et al. Idiopathic hypersomnia: a study of 77 cases. Sleep 2007; 30:1274.
- Bassetti C, Aldrich MS. Idiopathic hypersomnia. A series of 42 patients. Brain 1997; 120 ( Pt 8):1423.
- Coleman RM, Roffwarg HP, Kennedy SJ, et al. Sleep-wake disorders based on a polysomnographic diagnosis. A national cooperative study. JAMA 1982; 247:997.
- Billiard M. Idiopathic hypersomnia. Neurol Clin 1996; 14:573.
- Bassetti C, Dauvilliers Y. Idiopathic hypersomnia. In: Principles and Practice of Sleep Medicine, Kryger MH, Roth T, Dement WC (Eds), Elsevier Saunders, Philadelphia 2011. p.969.
- Montplaisir J, de Champlain J, Young SN, et al. Narcolepsy and idiopthic hypersomnia: biogenic amines and related compounds in CSF. Neurology 1982; 32:1299.
- Faull KF, Thiemann S, King RJ, Guilleminault C. Monoamine interactions in narcolepsy and hypersomnia: reanalysis. Sleep 1989; 12:185.
- Rye DB, Bliwise DL, Parker K, et al. Modulation of vigilance in the primary hypersomnias by endogenous enhancement of GABAA receptors. Sci Transl Med 2012; 4:161ra151.
- Trotti LM, Saini P, Bliwise DL, et al. Clarithromycin in γ-aminobutyric acid-Related hypersomnolence: A randomized, crossover trial. Ann Neurol 2015; 78:454.
- Dauvilliers Y, Evangelista E, Lopez R, et al. Absence of γ-aminobutyric acid-a receptor potentiation in central hypersomnolence disorders. Ann Neurol 2016; 80:259.
- Dauvilliers Y, Baumann CR, Carlander B, et al. CSF hypocretin-1 levels in narcolepsy, Kleine-Levin syndrome, and other hypersomnias and neurological conditions. J Neurol Neurosurg Psychiatry 2003; 74:1667.
- Bassetti C, Gugger M, Bischof M, et al. The narcoleptic borderland: a multimodal diagnostic approach including cerebrospinal fluid levels of hypocretin-1 (orexin A). Sleep Med 2003; 4:7.
- Aldrich MS. The clinical spectrum of narcolepsy and idiopathic hypersomnia. Neurology 1996; 46:393.
- Vernet C, Arnulf I. Idiopathic hypersomnia with and without long sleep time: a controlled series of 75 patients. Sleep 2009; 32:753.
- Guilleminault C, Stoohs R, Clerk A, et al. A cause of excessive daytime sleepiness. The upper airway resistance syndrome. Chest 1993; 104:781.
- Guilleminault C, Stoohs R, Clerk A, et al. Excessive daytime somnolence in women with abnormal respiratory efforts during sleep. Sleep 1993; 16:S137.
- Vanková J, Nevsímalová S, Sonka K, et al. Increased REM density in narcolepsy-cataplexy and the polysymptomatic form of idiopathic hypersomnia. Sleep 2001; 24:707.
- Littner MR, Kushida C, Wise M, et al. Practice parameters for clinical use of the multiple sleep latency test and the maintenance of wakefulness test. Sleep 2005; 28:113.
- Morgenthaler TI, Kapur VK, Brown T, et al. Practice parameters for the treatment of narcolepsy and other hypersomnias of central origin. Sleep 2007; 30:1705.
- Bastuji H, Jouvet M. Successful treatment of idiopathic hypersomnia and narcolepsy with modafinil. Prog Neuropsychopharmacol Biol Psychiatry 1988; 12:695.
- Lavault S, Dauvilliers Y, Drouot X, et al. Benefit and risk of modafinil in idiopathic hypersomnia vs. narcolepsy with cataplexy. Sleep Med 2011; 12:550.
- Mayer G, Benes H, Young P, et al. Modafinil in the treatment of idiopathic hypersomnia without long sleep time--a randomized, double-blind, placebo-controlled study. J Sleep Res 2015; 24:74.
- Leu-Semenescu S, Louis P, Arnulf I. Benefits and risk of sodium oxybate in idiopathic hypersomnia versus narcolepsy type 1: a chart review. Sleep Med 2016; 17:38.