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Kleine-Levin syndrome

MedGen UID:
61511
•Concept ID:
C0206085
•
Disease or Syndrome
Synonyms: Familial hibernation syndrome; Familial Kleine-Levin syndrome; Kleine-Levin hibernation syndrome
SNOMED CT: Kleine-Levin syndrome (111488004); Kleine Levin syndrome (111488004)
 
Monarch Initiative: MONDO:0007863
OMIM®: 148840
Orphanet: ORPHA33543

Definition

The Kleine-Levin hibernation syndrome, a rare disorder that occurs predominantly in males, is characterized by episodic attacks of aberrant behavior, hypersomnia, and increased feeding (megaphagia) and sex drives (Kleine, 1925; Levin, 1929). [from OMIM]

Clinical features

From HPO
Confusion
MedGen UID:
3587
•Concept ID:
C0009676
•
Mental or Behavioral Dysfunction
Lack of clarity and coherence of thought, perception, understanding, or action.
See: Feature record | Search on this feature
Polyphagia
MedGen UID:
9369
•Concept ID:
C0020505
•
Finding
A neurological anomaly with gross overeating associated with an abnormally strong desire or need to eat.
See: Feature record | Search on this feature
Episodic hypersomnia
MedGen UID:
870479
•Concept ID:
C4024925
•
Disease or Syndrome
See: Feature record | Search on this feature
Vivid hallucinations
MedGen UID:
870530
•Concept ID:
C4024978
•
Mental or Behavioral Dysfunction
See: Feature record | Search on this feature

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKleine-Levin syndrome
Follow this link to review classifications for Kleine-Levin syndrome in Orphanet.

Professional guidelines

PubMed

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
Pharmacological treatment for Kleine-Levin syndrome.
de Oliveira MM, Conti C, Prado GF
Cochrane Database Syst Rev 2016 May 6;2016(5):CD006685. doi: 10.1002/14651858.CD006685.pub4. PMID: 27153153Free PMC Article

Recent clinical studies

Etiology

The orexin story, sleep and sleep disturbances.
Pizza F, Barateau L, Dauvilliers Y, Plazzi G
J Sleep Res 2022 Aug;31(4):e13665. Epub 2022 Jun 13 doi: 10.1111/jsr.13665. PMID: 35698789
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
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
Incidence of narcolepsy after H1N1 influenza and vaccinations: Systematic review and meta-analysis.
Sarkanen TO, Alakuijala APE, Dauvilliers YA, Partinen MM
Sleep Med Rev 2018 Apr;38:177-186. Epub 2017 Jun 20 doi: 10.1016/j.smrv.2017.06.006. PMID: 28847694

Diagnosis

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
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
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
Kleine-Levin Syndrome.
Afolabi-Brown O, Mason TBA 2nd
Paediatr Respir Rev 2018 Jan;25:9-13. Epub 2016 Dec 23 doi: 10.1016/j.prrv.2016.12.004. PMID: 28216256

Therapy

The orexin story, sleep and sleep disturbances.
Pizza F, Barateau L, Dauvilliers Y, Plazzi G
J Sleep Res 2022 Aug;31(4):e13665. Epub 2022 Jun 13 doi: 10.1111/jsr.13665. PMID: 35698789
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
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 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

Prognosis

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
Incidence of narcolepsy after H1N1 influenza and vaccinations: Systematic review and meta-analysis.
Sarkanen TO, Alakuijala APE, Dauvilliers YA, Partinen MM
Sleep Med Rev 2018 Apr;38:177-186. Epub 2017 Jun 20 doi: 10.1016/j.smrv.2017.06.006. PMID: 28847694
Kleine-Levin Syndrome.
Afolabi-Brown O, Mason TBA 2nd
Paediatr Respir Rev 2018 Jan;25:9-13. Epub 2016 Dec 23 doi: 10.1016/j.prrv.2016.12.004. PMID: 28216256
Kleine-Levin syndrome; An update and mini-review.
Gadoth N, Oksenberg A
Brain Dev 2017 Sep;39(8):665-671. Epub 2017 Apr 20 doi: 10.1016/j.braindev.2017.04.003. PMID: 28434769
Kleine-Levin Syndrome.
Miglis MG, Guilleminault C
Curr Neurol Neurosci Rep 2016 Jun;16(6):60. doi: 10.1007/s11910-016-0653-6. PMID: 27137943

Clinical prediction guides

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
Sleep-Wake Disorders in Childhood.
Licis A
Continuum (Minneap Minn) 2020 Aug;26(4):1034-1069. doi: 10.1212/CON.0000000000000897. PMID: 32756235
Incidence of narcolepsy after H1N1 influenza and vaccinations: Systematic review and meta-analysis.
Sarkanen TO, Alakuijala APE, Dauvilliers YA, Partinen MM
Sleep Med Rev 2018 Apr;38:177-186. Epub 2017 Jun 20 doi: 10.1016/j.smrv.2017.06.006. PMID: 28847694
Kleine-Levin syndrome; An update and mini-review.
Gadoth N, Oksenberg A
Brain Dev 2017 Sep;39(8):665-671. Epub 2017 Apr 20 doi: 10.1016/j.braindev.2017.04.003. PMID: 28434769
Kleine-Levin syndrome: a case report and review of literature.
Das S, Gupta R, Dhyani M, Raghuvanshi S
Pediatr Neurol 2014 Apr;50(4):411-6. Epub 2014 Jan 6 doi: 10.1016/j.pediatrneurol.2014.01.003. PMID: 24630285

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
Incidence of narcolepsy after H1N1 influenza and vaccinations: Systematic review and meta-analysis.
Sarkanen TO, Alakuijala APE, Dauvilliers YA, Partinen MM
Sleep Med Rev 2018 Apr;38:177-186. Epub 2017 Jun 20 doi: 10.1016/j.smrv.2017.06.006. PMID: 28847694

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