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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

Idiopathic Hypersomnia: Neurobiology, Diagnosis, and Management.
Morse AM, Naik S
CNS Drugs 2023 Apr;37(4):305-322. Epub 2023 Apr 18 doi: 10.1007/s40263-023-00998-6. PMID: 37069414
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 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
Drugs Used in Narcolepsy and Other Hypersomnias.
Lammers GJ
Sleep Med Clin 2018 Jun;13(2):183-189. doi: 10.1016/j.jsmc.2018.02.009. PMID: 29759269
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

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
Evaluation of hypersomnolence: From symptoms to diagnosis, a multidimensional approach.
Peter-Derex L, Micoulaud-Franchi JA, Lopez R, Barateau L
Rev Neurol (Paris) 2023 Oct;179(7):715-726. Epub 2023 Aug 8 doi: 10.1016/j.neurol.2023.07.004. PMID: 37563022
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
Nocturnal sleep architecture in idiopathic hypersomnia: a systematic review and meta-analysis.
Plante DT
Sleep Med 2018 May;45:17-24. Epub 2017 Nov 2 doi: 10.1016/j.sleep.2017.10.005. PMID: 29680423Free 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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