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Idiopathic hypersomnia

MedGen UID:
155626
•Concept ID:
C0751757
•
Disease or Syndrome
Synonyms: CNS Hypersomnolence, Idiopathic; CNS Hypersomnolences, Idiopathic; Hypersomnia, Idiopathic; Hypersomnias, Idiopathic; Hypersomnolence, CNS, Idiopathic; Hypersomnolence, Idiopathic; Hypersomnolence, Idiopathic CNS; Hypersomnolences, Idiopathic; Hypersomnolences, Idiopathic CNS; Idiopathic Central Nervous System Hypersomnolence; Idiopathic CNS Hypersomnolence; Idiopathic CNS Hypersomnolences; Idiopathic Hypersomnia; Idiopathic Hypersomnias; Idiopathic Hypersomnolence; Idiopathic Hypersomnolences
SNOMED CT: Idiopathic hypersomnia (3731000119107)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
Monarch Initiative: MONDO:0018044
Orphanet: ORPHA33208

Definition

A rare neurologic disease characterized by an excessive daytime sleepiness with long and unrefreshing naps, and/or prolonged and undisturbed nocturnal sleep, impaired daytime alertness, and/or sleep inertia (ie, great difficulty in waking up after sleep) and where other causes have been excluded. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIdiopathic hypersomnia

Professional guidelines

PubMed

Update on the treatment of idiopathic hypersomnia: Progress, challenges, and expert opinion.
Arnulf I, Thomas R, Roy A, Dauvilliers Y
Sleep Med Rev 2023 Jun;69:101766. Epub 2023 Feb 25 doi: 10.1016/j.smrv.2023.101766. PMID: 36921459
Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine systematic review, meta-analysis, and GRADE assessment.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Swick TJ, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1895-1945. doi: 10.5664/jcsm.9326. PMID: 34743790Free PMC Article
Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine clinical practice guideline.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1881-1893. doi: 10.5664/jcsm.9328. PMID: 34743789Free PMC Article

Recent clinical studies

Etiology

Understanding and approaching excessive daytime sleepiness.
Pérez-Carbonell L, Mignot E, Leschziner G, Dauvilliers Y
Lancet 2022 Sep 24;400(10357):1033-1046. Epub 2022 Sep 14 doi: 10.1016/S0140-6736(22)01018-2. PMID: 36115367
European guideline and expert statements on the management of narcolepsy in adults and children.
Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
J Sleep Res 2021 Dec;30(6):e13387. Epub 2021 Jun 25 doi: 10.1111/jsr.13387. PMID: 34173288
Excessive Daytime Sleepiness: A Clinical Review.
Gandhi KD, Mansukhani MP, Silber MH, Kolla BP
Mayo Clin Proc 2021 May;96(5):1288-1301. Epub 2021 Apr 9 doi: 10.1016/j.mayocp.2020.08.033. PMID: 33840518
Central Disorders of Hypersomnolence.
Trotti LM
Continuum (Minneap Minn) 2020 Aug;26(4):890-907. doi: 10.1212/CON.0000000000000883. PMID: 32756227Free PMC Article
A new method for measuring daytime sleepiness: the Epworth sleepiness scale.
Johns MW
Sleep 1991 Dec;14(6):540-5. doi: 10.1093/sleep/14.6.540. PMID: 1798888

Diagnosis

Narcolepsy and Idiopathic Hypersomnia.
Blattner M, Maski K
Sleep Med Clin 2023 Jun;18(2):183-199. Epub 2023 Mar 8 doi: 10.1016/j.jsmc.2023.01.003. PMID: 37120161
Understanding and approaching excessive daytime sleepiness.
Pérez-Carbonell L, Mignot E, Leschziner G, Dauvilliers Y
Lancet 2022 Sep 24;400(10357):1033-1046. Epub 2022 Sep 14 doi: 10.1016/S0140-6736(22)01018-2. PMID: 36115367
Narcolepsy.
Barateau L, Pizza F, Plazzi G, Dauvilliers Y
J Sleep Res 2022 Aug;31(4):e13631. Epub 2022 May 27 doi: 10.1111/jsr.13631. PMID: 35624073
Idiopathic Hypersomnia and Other Hypersomnia Syndromes.
Trotti LM, Arnulf I
Neurotherapeutics 2021 Jan;18(1):20-31. doi: 10.1007/s13311-020-00919-1. PMID: 32901432Free PMC Article
A new method for measuring daytime sleepiness: the Epworth sleepiness scale.
Johns MW
Sleep 1991 Dec;14(6):540-5. doi: 10.1093/sleep/14.6.540. PMID: 1798888

Therapy

Update on the treatment of idiopathic hypersomnia: Progress, challenges, and expert opinion.
Arnulf I, Thomas R, Roy A, Dauvilliers Y
Sleep Med Rev 2023 Jun;69:101766. Epub 2023 Feb 25 doi: 10.1016/j.smrv.2023.101766. PMID: 36921459
European guideline and expert statements on the management of narcolepsy in adults and children.
Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
J Sleep Res 2021 Dec;30(6):e13387. Epub 2021 Jun 25 doi: 10.1111/jsr.13387. PMID: 34173288
Idiopathic Hypersomnia and Other Hypersomnia Syndromes.
Trotti LM, Arnulf I
Neurotherapeutics 2021 Jan;18(1):20-31. doi: 10.1007/s13311-020-00919-1. PMID: 32901432Free PMC Article
Idiopathic Hypersomnia.
Trotti LM
Sleep Med Clin 2017 Sep;12(3):331-344. Epub 2017 Jun 16 doi: 10.1016/j.jsmc.2017.03.009. PMID: 28778232Free PMC Article
Idiopathic hypersomnia.
Billiard M, Sonka K
Sleep Med Rev 2016 Oct;29:23-33. Epub 2015 Sep 3 doi: 10.1016/j.smrv.2015.08.007. PMID: 26599679

Prognosis

Role of Daytime Continuous Polysomnography in the Diagnosis of Pediatric Narcolepsy Type 1.
Pizza F, Vignatelli L, Vandi S, Zenesini C, Biscarini F, Franceschini C, Antelmi E, Ingravallo F, Mignot E, Bruni O, Nobili L, Veggiotti P, Ferri R, Plazzi G
Neurology 2024 Jan 9;102(1):e207815. Epub 2023 Dec 14 doi: 10.1212/WNL.0000000000207815. PMID: 38165365Free PMC Article
Prevalence and Course of Idiopathic Hypersomnia in the Wisconsin Sleep Cohort Study.
Plante DT, Hagen EW, Barnet JH, Mignot E, Peppard PE
Neurology 2024 Jan 23;102(2):e207994. Epub 2023 Dec 13 doi: 10.1212/WNL.0000000000207994. PMID: 38165322
Narcolepsies, update in 2023.
Barateau L, Pizza F, Chenini S, Peter-Derex L, Dauvilliers Y
Rev Neurol (Paris) 2023 Oct;179(7):727-740. Epub 2023 Aug 25 doi: 10.1016/j.neurol.2023.08.001. PMID: 37634997
Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine clinical practice guideline.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1881-1893. doi: 10.5664/jcsm.9328. PMID: 34743789Free PMC Article
French consensus. Idiopathic hypersomnia: Investigations and follow-up.
Leu-Semenescu S, Quera-Salva MA, Dauvilliers Y
Rev Neurol (Paris) 2017 Jan-Feb;173(1-2):32-37. Epub 2016 Nov 10 doi: 10.1016/j.neurol.2016.09.015. PMID: 27838089

Clinical prediction guides

The immunopathogenesis of narcolepsy type 1.
Liblau RS, Latorre D, Kornum BR, Dauvilliers Y, Mignot EJ
Nat Rev Immunol 2024 Jan;24(1):33-48. Epub 2023 Jul 3 doi: 10.1038/s41577-023-00902-9. PMID: 37400646
Safety and efficacy of pitolisant in children aged 6 years or older with narcolepsy with or without cataplexy: a double-blind, randomised, placebo-controlled trial.
Dauvilliers Y, Lecendreux M, Lammers GJ, Franco P, Poluektov M, Caussé C, Lecomte I, Lecomte JM, Lehert P, Schwartz JC, Plazzi G
Lancet Neurol 2023 Apr;22(4):303-311. doi: 10.1016/S1474-4422(23)00036-4. PMID: 36931805
Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine systematic review, meta-analysis, and GRADE assessment.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Swick TJ, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1895-1945. doi: 10.5664/jcsm.9326. PMID: 34743790Free PMC Article
Update on treatment for idiopathic hypersomnia.
Evangelista E, Lopez R, Dauvilliers Y
Expert Opin Investig Drugs 2018 Feb;27(2):187-192. Epub 2018 Jan 3 doi: 10.1080/13543784.2018.1417385. PMID: 29250981
A new method for measuring daytime sleepiness: the Epworth sleepiness scale.
Johns MW
Sleep 1991 Dec;14(6):540-5. doi: 10.1093/sleep/14.6.540. PMID: 1798888

Recent systematic reviews

Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine systematic review, meta-analysis, and GRADE assessment.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Swick TJ, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1895-1945. doi: 10.5664/jcsm.9326. PMID: 34743790Free PMC Article
Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine clinical practice guideline.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1881-1893. doi: 10.5664/jcsm.9328. PMID: 34743789Free PMC Article
European guideline and expert statements on the management of narcolepsy in adults and children.
Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
Eur J Neurol 2021 Sep;28(9):2815-2830. Epub 2021 Jun 25 doi: 10.1111/ene.14888. PMID: 34173695
European guideline and expert statements on the management of narcolepsy in adults and children.
Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
J Sleep Res 2021 Dec;30(6):e13387. Epub 2021 Jun 25 doi: 10.1111/jsr.13387. PMID: 34173288
Idiopathic Hypersomnia and Hypersomnolence Disorder: A Systematic Review of the Literature.
Sowa NA
Psychosomatics 2016 Mar-Apr;57(2):152-64. Epub 2015 Dec 17 doi: 10.1016/j.psym.2015.12.006. PMID: 26895727

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