idiopathic hypersomnolence


Summary: A sleep disorder of central nervous system origin characterized by prolonged nocturnal sleep and periods of daytime drowsiness. Affected individuals experience difficulty with awakening in the morning and may have associated sleep drunkenness, automatic behaviors, and memory disturbances. This condition differs from narcolepsy in that daytime sleep periods are longer, there is no association with CATAPLEXY, and the multiple sleep latency onset test does not record sleep-onset rapid eye movement sleep. (From Chokroverty, Sleep Disorders Medicine, 1994, pp319-20; Psychiatry Clin Neurosci 1998 Apr:52(2):125-129)

Top Publications

  1. Lopez R, Arnulf I, Drouot X, Lecendreux M, Dauvilliers Y. French consensus. Management of patients with hypersomnia: Which strategy?. Rev Neurol (Paris). 2017;173:8-18 pubmed publisher
    ..The next few years will see innovative emerging therapies, based on a physiopathological approach, aiming to restore hypocretinergic transmission or to interrupt the autoimmune processes causing the loss of hypocretin neurons. ..
  2. Anniss A, Young A, O Driscoll D. Importance of Urinary Drug Screening in the Multiple Sleep Latency Test and Maintenance of Wakefulness Test. J Clin Sleep Med. 2016;12:1633-1640 pubmed publisher
    ..These data support routine urinary drug screening in all patients undergoing MSLT or MWT studies to ensure accurate interpretation in the context of illicit and prescription drug use. ..
  3. Lee J, Na G, Joo E, Lee M, Lee J. Clinical and polysomnographic characteristics of excessive daytime sleepiness in children. Sleep Breath. 2017;21:967-974 pubmed publisher
    ..For pediatric patients with EDS, a systematic diagnostic approach including questionnaires for sleep habits and emotion and PSG is essential for accurate diagnosis and treatment. ..
  4. Saini P, Rye D. Hypersomnia: Evaluation, Treatment, and Social and Economic Aspects. Sleep Med Clin. 2017;12:47-60 pubmed publisher
  5. Ferri R, Pizza F, Vandi S, Iloti M, Plazzi G. Decreased sleep stage transition pattern complexity in narcolepsy type 1. Clin Neurophysiol. 2016;127:2812-9 pubmed publisher
    ..The lower probability of R-to-N2 transition occurrence in NT1 appears to be a reliable polysomnographic feature with potential application at the individual level, for supportive diagnostic purposes. ..
  6. Vandi S, Pizza F, Antelmi E, Neccia G, Iloti M, Mazzoni A, et al. A standardized test to document cataplexy. Sleep Med. 2017;: pubmed publisher
    ..Further multi-center studies are warranted to extend the present findings and integrate a shared procedure for the laboratory work-up of narcolepsy. ..
  7. Evangelista E, Lopez R, Dauvilliers Y. Update on treatment for idiopathic hypersomnia. Expert Opin Investig Drugs. 2017;: pubmed publisher
    ..Specific clinical instruments and objective tests are required in IH to better evaluate the severity of EDS and responsiveness to medications, but also prolonged sleep and sleep inertia, to optimize the whole management of IH patients...
  8. Lopez R, Doukkali A, Barateau L, Evangelista E, Chenini S, Jaussent I, et al. Test-Retest Reliability of the Multiple Sleep Latency Test in central disorders of hypersomnolence. Sleep. 2017;: pubmed publisher
  9. van Holst R, van der Cruijsen L, van Mierlo P, Lammers G, Cools R, Overeem S, et al. Aberrant Food Choices after Satiation in Human Orexin-Deficient Narcolepsy Type 1. Sleep. 2016;39:1951-1959 pubmed
    ..These mechanisms may contribute to their obesity, and suggest an important functional role for orexin in human eating behavior. Study registered at Netherlands Trial Register. URL: Trial ID: NTR4508. ..

More Information


  1. Lerousseau L. [Sleepiness, continuous positive airway pressure and the obstructive sleep apnea hypopnea syndrome]. Rev Mal Respir. 2018;35:116-133 pubmed publisher
    ..However, they can be administered by way of derogation to other neurological hypersomnias on prescription by a reference centre or a centre with expertise in hypersomnia. ..
  2. Aflaki S, Hu S, Kamel R, Chung F, Singh M. Case Report of a Patient With Idiopathic Hypersomnia and a Family History of Malignant Hyperthermia Undergoing General Anesthesia: An Overview of the Anesthetic Considerations. A A Case Rep. 2017;8:238-241 pubmed publisher
    ..Anesthesia was maintained with total intravenous anesthesia via the use of propofol and remifentanil. The depth of anesthesia was monitored with entropy. There were no perioperative complications...
  3. Vrana M, Siffnerova V, Pecherkova P, Ratajova E, Sonka K. Distribution of HLA-DQB1 in Czech Patients with Central Hypersomnias. Arch Immunol Ther Exp (Warsz). 2016;64:89-98 pubmed publisher
    ..These results indicate that DQB1 contributes to the genetic predisposition to NT1, NT2, IH and no-CH in different manners. ..
  4. Trotti L, Bliwise D. Brain MRI findings in patients with idiopathic hypersomnia. Clin Neurol Neurosurg. 2017;157:19-21 pubmed publisher
    ..In all three, the imaging finding was predated by clear clinical abnormalities. Neuroimaging may be a complementary part of an idiopathic hypersomnia evaluation, but the decision to pursue imaging should be made on a case-by-case basis. ..
  5. Omokawa M, Ayabe T, Nagai T, Imanishi A, Omokawa A, Nishino S, et al. Decline of CSF orexin (hypocretin) levels in Prader-Willi syndrome. Am J Med Genet A. 2016;170A:1181-6 pubmed publisher
    ..Decreased cerebrospinal fluid orexin levels in Prader-Willi syndrome may play an important role in severity of obesity and excessive daytime sleepiness. ..
  6. Berkowski J, Shelgikar A. Disorders of Excessive Daytime Sleepiness Including Narcolepsy and Idiopathic Hypersomnia. Sleep Med Clin. 2016;11:365-78 pubmed publisher
    ..Elucidation of pathophysiology of these disorders in the future will be essential to better categorization and management. ..
  7. Barateau L, Jaussent I, Lopez R, Boutrel B, Leu Semenescu S, Arnulf I, et al. Smoking, Alcohol, Drug Use, Abuse and Dependence in Narcolepsy and Idiopathic Hypersomnia: A Case-Control Study. Sleep. 2016;39:573-80 pubmed publisher
    ..Although disruption of hypocretin signaling in rodents reduces drug-seeking behaviors, our results do not support that hypocretin deficiency constitutes a protective factor against the development of drug addiction in humans. ..
  8. Kelty E, Martyn V, O Neil G, Hulse G. Use of subcutaneous flumazenil preparations for the treatment of idiopathic hypersomnia: A case report. J Psychopharmacol. 2014;28:703-6 pubmed publisher
    ..Current data supports the need for further research into the use of flumazenil for the treatment of IH and to develop long term flumazenil delivery systems. ..
  9. Trotti L. Waking up is the hardest thing I do all day: Sleep inertia and sleep drunkenness. Sleep Med Rev. 2017;35:76-84 pubmed publisher
    ..This may represent an important treatment target, but evidence-based treatment guidance is not yet available. ..
  10. Khan Z, Trotti L. Central Disorders of Hypersomnolence: Focus on the Narcolepsies and Idiopathic Hypersomnia. Chest. 2015;148:262-273 pubmed publisher
    ..Emerging treatments for sleepiness include histamine H3 antagonists (eg, pitolisant) and possibly negative allosteric modulators of the gamma-aminobutyric acid-A receptor (eg, clarithromycin and flumazenil). ..
  11. Jennum P, Østergaard Pedersen L, Czarna Bahl J, Modvig S, Fog K, Holm A, et al. Cerebrospinal Fluid Biomarkers of Neurodegeneration Are Decreased or Normal in Narcolepsy. Sleep. 2017;40: pubmed publisher
    ..Lower CSF levels of β-amyloid, T-tau protein, and P-tau181 in narcolepsy may indicate that hypocretin deficiency and an abnormal sleep-wake pattern alter the turnover of these proteins in the central nervous system. ..
  12. Galbiati A, Abutalebi J, Iannaccone S, Borsa V, Musteata S, Zucconi M, et al. The effects of Transcranial Direct Current Stimulation (tDCS) on Idiopathic Hypersomnia: a pilot study. Arch Ital Biol. 2016;154:1-5 pubmed publisher
    ..Our results indicate that tDCS may foster the management of EDS in IH, improving also the attentional domain. ..
  13. Hulse G, Kelty E, Hood S, Norman A, Basso M, Reece A. Novel Indications for Benzodiazepine Antagonist Flumazenil in GABA Mediated Pathological Conditions of the Central Nervous System. Curr Pharm Des. 2015;21:3325-42 pubmed
  14. Sforza E, Roche F, Barthélémy J, Pichot V. Diurnal and nocturnal cardiovascular variability and heart rate arousal response in idiopathic hypersomnia. Sleep Med. 2016;24:131-136 pubmed publisher
    ..These findings suggest an impaired parasympathetic function that may explain some vegetative symptoms present in this type of central hypersomnia. ..
  15. Thakrar C, Patel K, D ancona G, Kent B, Nesbitt A, Selsick H, et al. Effectiveness and side-effect profile of stimulant therapy as monotherapy and in combination in the central hypersomnias in clinical practice. J Sleep Res. 2017;: pubmed publisher
    ..Combination therapy appears to be a useful and safe option in patients with refractory symptoms...
  16. Ferini Strambi L, Sforza M, Poletti M, Giarrusso F, Galbiati A. Daytime sleepiness: more than just Obstructive Sleep Apnea (OSA). Med Lav. 2017;108:260-266 pubmed publisher
  17. Sowa N. Idiopathic Hypersomnia and Hypersomnolence Disorder: A Systematic Review of the Literature. Psychosomatics. 2016;57:152-64 pubmed publisher
    ..IH and HD are relatively uncommon disorders and little is known about them. However, they are distinct from psychiatric disorders and respond well to treatment once properly identified. ..
  18. Barateau L, Lopez R, Arnulf I, Lecendreux M, Franco P, Drouot X, et al. Comorbidity between central disorders of hypersomnolence and immune-based disorders. Neurology. 2017;88:93-100 pubmed publisher
  19. Kotagal S. Treatment of narcolepsy and other organic hypersomnias in children. Paediatr Respir Rev. 2018;25:19-24 pubmed publisher
    ..Dual therapy, with both anti-cataplectic and stimulant medications may be required, as is close monitoring for treatment-emergent side effects. ..
  20. Trotti L. Idiopathic Hypersomnia. Sleep Med Clin. 2017;12:331-344 pubmed publisher
    ..Even with current treatment options, quality of life and safety may remain impaired. ..
  21. 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;17:38-44 pubmed publisher
    ..The benefit/risk ratio of sodium oxybate in IH- was similar to NT1-associated sleepiness, with additional benefits on severe morning inertia, despite using smaller doses in more refractory patients. ..
  22. Leu Semenescu S, Quera Salva M, Dauvilliers Y. French consensus. Idiopathic hypersomnia: Investigations and follow-up. Rev Neurol (Paris). 2017;173:32-37 pubmed publisher
    ..In this article, we propose recommendations for the work-up to be carried out during diagnosis and follow-up for patients suffering from idiopathic hypersomnia. ..
  23. Dauvilliers Y, Evangelista E, Lopez R, Barateau L, Jaussent I, Cens T, et al. Absence of ?-aminobutyric acid-a receptor potentiation in central hypersomnolence disorders. Ann Neurol. 2016;80:259-68 pubmed publisher
    ..Ann Neurol 2016;80:259-268. ..
  24. Billiard M, Sonka K. Idiopathic hypersomnia. Sleep Med Rev. 2016;29:23-33 pubmed publisher
  25. Barateau L, Lopez R, Franchi J, Dauvilliers Y. Hypersomnolence, Hypersomnia, and Mood Disorders. Curr Psychiatry Rep. 2017;19:13 pubmed publisher
    ..The current diagnostic tools seem to be limited in that condition, and further research in that field is warranted. ..
  26. Trimmel K, Żebrowska M, Böck M, Stefanic A, Mayer D, Klosch G, et al. Wanted: a better cut-off value for the Epworth Sleepiness Scale. Wien Klin Wochenschr. 2018;130:349-355 pubmed publisher
    ..other diagnoses) and that the commonly used cut-off of 11 points may be insufficient for clinical practice. ..
  27. Takenoshita S, Nishino S. Pharmacologic Management of Excessive Daytime Sleepiness. Sleep Med Clin. 2017;12:461-478 pubmed publisher
    ..The side effects of some stimulants are potent and careful selection and management are required. ..
  28. Heier M, Evsiukova T, Vilming S, Gjerstad M, Schrader H, Gautvik K. CSF hypocretin-1 levels and clinical profiles in narcolepsy and idiopathic CNS hypersomnia in Norway. Sleep. 2007;30:969-73 pubmed
    ..To evaluate the relationship between CSF hypocretin-1 levels and clinical profiles in narcolepsy and CNS hypersomnia in Norwegian patients...
  29. Ali M, Auger R, Slocumb N, Morgenthaler T. Idiopathic hypersomnia: clinical features and response to treatment. J Clin Sleep Med. 2009;5:562-8 pubmed
    ..We describe various clinical and polysomnographic features of patients with idiopathic hypersomnia, with an emphasis on response to pharmacotherapy...
  30. Vernet C, Arnulf I. Idiopathic hypersomnia with and without long sleep time: a controlled series of 75 patients. Sleep. 2009;32:753-9 pubmed
    ..To characterize the clinical, psychological, and sleep pattern of idiopathic hypersomnia with and without long sleep time, and provide normative values for 24-hour polysomnography...
  31. Kotagal S. Hypersomnia in children: interface with psychiatric disorders. Child Adolesc Psychiatr Clin N Am. 2009;18:967-77 pubmed publisher
    ..Treatment of a sleep disorder can have a favorable impact on alertness and quality of life. A high index of suspicion for sleep problems should be maintained in children and adolescents with psychiatric disorders...
  32. Sasai T, Inoue Y, Komada Y, Sugiura T, Matsushima E. Comparison of clinical characteristics among narcolepsy with and without cataplexy and idiopathic hypersomnia without long sleep time, focusing on HLA-DRB1( *)1501/DQB1( *)0602 finding. Sleep Med. 2009;10:961-6 pubmed publisher
    ..We investigated clinical features of NA w/o CA, particularly addressing HLA-DRB1( *)1501/DQB1( *)0602...
  33. Anderson K, Pilsworth S, Sharples L, Smith I, Shneerson J. Idiopathic hypersomnia: a study of 77 cases. Sleep. 2007;30:1274-81 pubmed
    ..To review the clinical and polysomnographic characteristics of idiopathic hypersomnia as well as the long-term response to treatment...
  34. Poli F, Plazzi G, Di Dalmazi G, Ribichini D, Vicennati V, Pizza F, et al. Body mass index-independent metabolic alterations in narcolepsy with cataplexy. Sleep. 2009;32:1491-7 pubmed
    ..To contribute to the anthropometric and metabolic phenotyping of orexin-A-deficient narcoleptic patients, and to explore a possible risk of their developing a metabolic syndrome...
  35. Oosterloo M, Lammers G, Overeem S, de Noord I, Kooij J. Possible confusion between primary hypersomnia and adult attention-deficit/hyperactivity disorder. Psychiatry Res. 2006;143:293-7 pubmed
    ..It remains unclear whether our findings indicate pathophysiological overlap...
  36. Martinez Rodriguez J, Iranzo A, Casamitjana R, Graus F, Santamaria J. [Comparative analysis of patients with narcolepsy-cataplexy, narcolepsy without cataplexy and idiopathic hypersomnia]. Med Clin (Barc). 2007;128:361-4 pubmed
  37. Maestri M, Monzani F, Bonanni E, Di Coscio E, Cignoni F, Dardano A, et al. Insulinoma presenting as idiopathic hypersomnia. Neurol Sci. 2010;31:349-52 pubmed publisher
    ..Clinicians should keep in mind that neuroglycopenia should be considered in the differential diagnosis of patients with hypersomnia, particularly if the clinical scenario does not conform to standard criteria...
  38. Grossman A, Barenboim E, Azaria B, Sherer Y, Goldstein L. The maintenance of wakefulness test as a predictor of alertness in aircrew members with idiopathic hypersomnia. Aviat Space Environ Med. 2004;75:281-3 pubmed
    ..Herein we report 2 cases of aviators who were returned to flying duty despite a pathologic MSLT. These aviators were waivered based on a normal MWT and safety history obtained from their commanders...
  39. Dauvilliers Y, Buguet A. Hypersomnia. Dialogues Clin Neurosci. 2005;7:347-56 pubmed
  40. Kanbayashi T, Inoue Y, Chiba S, Aizawa R, Saito Y, Tsukamoto H, et al. CSF hypocretin-1 (orexin-A) concentrations in narcolepsy with and without cataplexy and idiopathic hypersomnia. J Sleep Res. 2002;11:91-3 pubmed
    ..Low CSF hypocretin-1 is therefore very specific for HLA DR2 positive narcolepsy-cataplexy, and the deficiency is likely to be established at the early stage of the disease...
  41. Smart T, Singh B. Excessive daytime sleepiness in a trainee military pilot. Aviat Space Environ Med. 2006;77:753-7 pubmed
    ..This case illustrates the difficulties in determining aeromedical disposition in borderline cases of EDS where a clear diagnosis cannot be made...
  42. Billiard M. Diagnosis of narcolepsy and idiopathic hypersomnia. An update based on the International classification of sleep disorders, 2nd edition. Sleep Med Rev. 2007;11:377-88 pubmed
    ..hypersomnia without long sleep time, two forms of the same condition or two different conditions? Is there a pathophysiological relationship between narcolepsy without cataplexy and idiopathic hypersomnia without long sleep time?..
  43. Dauvilliers Y, Paquereau J, Bastuji H, Drouot X, Weil J, Viot Blanc V. Psychological health in central hypersomnias: the French Harmony study. J Neurol Neurosurg Psychiatry. 2009;80:636-41 pubmed publisher
  44. Coelho F, Pradella Hallinan M, Predazzoli Neto M, Bittencourt L, Tufik S. Prevalence of the HLA-DQB1*0602 allele in narcolepsy and idiopathic hypersomnia patients seen at a sleep disorders outpatient unit in São Paulo. Braz J Psychiatr. 2009;31:10-4 pubmed
    ..The presence of the HLA-DQB1* 0602 allele may be important for the differential diagnosis of situations that resemble those sleep disorders such as secondary changes in sleep structure due to drugs' consumption. ..
  45. Takegami M, Suzukamo Y, Wakita T, Noguchi H, Chin K, Kadotani H, et al. Development of a Japanese version of the Epworth Sleepiness Scale (JESS) based on item response theory. Sleep Med. 2009;10:556-65 pubmed publisher
    ..Various Japanese versions of the Epworth Sleepiness Scale (ESS) have been used, but none was developed via standard procedures. Here we report on the construction and testing of the developer-authorized Japanese version of the ESS (JESS)...
  46. Haba Rubio J. Psychiatric aspects of organic sleep disorders. Dialogues Clin Neurosci. 2005;7:335-46 pubmed
    ..After treatment, depression scores may or may not improve to the level of population norms, suggesting that this relationship is more complex than one of mere cause and effect...
  47. Kok S, Overeem S, Visscher T, Lammers G, Seidell J, Pijl H, et al. Hypocretin deficiency in narcoleptic humans is associated with abdominal obesity. Obes Res. 2003;11:1147-54 pubmed
    ..e., reduced physical activity) on these anthropometric measures...
  48. Vgontzas A, Bixler E, Kales A, Criley C, Vela Bueno A. Differences in nocturnal and daytime sleep between primary and psychiatric hypersomnia: diagnostic and treatment implications. Psychosom Med. 2000;62:220-6 pubmed
    ..The aim of this study was to assess whether polysomnography aids in the differential diagnosis of these two disorders...