MCID: KLN009
MIFTS: 37

Kleine-Levin Hibernation Syndrome

Categories: Genetic diseases, Mental diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Kleine-Levin Hibernation Syndrome

MalaCards integrated aliases for Kleine-Levin Hibernation Syndrome:

Name: Kleine-Levin Hibernation Syndrome 57 20
Kleine-Levin Syndrome 57 12 73 53 58 44 15 17 70
Familial Kleine-Levin Syndrome 20 70
Familial Hibernation Syndrome 20
Kleine Levin Syndrome 20

Characteristics:

Orphanet epidemiological data:

58
kleine-levin syndrome
Prevalence: 1-9/1000000 (France); Age of onset: Adolescent,Adult,Childhood;

OMIM®:

57 (Updated 20-May-2021)
Inheritance:
autosomal dominant


HPO:

31
kleine-levin hibernation syndrome:
Inheritance autosomal dominant inheritance


Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:0060165
OMIM® 57 148840
MeSH 44 D017593
MESH via Orphanet 45 D017593
ICD10 via Orphanet 33 G47.8
UMLS via Orphanet 71 C0206085
Orphanet 58 ORPHA33543
MedGen 41 C0206085
UMLS 70 C0206085 C2936703

Summaries for Kleine-Levin Hibernation Syndrome

GARD : 20 Kleine Levin syndrome is a rare disorder characterized by recurrent episodes of excessive sleep (hypersomnia) along with cognitive and behavioral changes. Affected individuals may sleep for up to 20 hours per day during an episode. These episodes usually last for a few days to a few weeks. An episode may start abruptly and is sometimes preceded by an upper-respiratory-type infection. During an episode, people with Kleine Levin syndrome can also display abnormal behavior, such as excessive food intake (hyperphagia), irritability, childishness, disorientation, hallucinations, and an abnormally uninhibited sex drive. Affected individuals are symptom-free between episodes. The time between episodes varies. Kleine Levin syndrome primarily affects adolescent males, but it also affects females and individuals of other ages. The underlying cause of this condition is unknown. Episodes tend to decrease with advancing age. There are no consistently effective therapies, although stimulants (modafinil, methylphenidate, amphetamine) and mood stabilizers (lithium) may be prescribed with varying results.

MalaCards based summary : Kleine-Levin Hibernation Syndrome, also known as kleine-levin syndrome, is related to hypersomnia and idiopathic hypersomnia. An important gene associated with Kleine-Levin Hibernation Syndrome is HCRT (Hypocretin Neuropeptide Precursor), and among its related pathways/superpathways is Monoamine Transport. The drugs Radiopharmaceuticals and Technetium Tc 99m bicisate have been mentioned in the context of this disorder. Affiliated tissues include thalamus, hypothalamus and brain, and related phenotypes are confusion and polyphagia

Disease Ontology : 12 A recurrent hypersomnia that is characterized by recurring periods of excessive amounts of sleep and altered behavior.

OMIM® : 57 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). (148840) (Updated 20-May-2021)

NINDS : 53 Kleine-Levin syndrome is a rare disorder that primarily affects adolescent males (approximately 70 percent of those with Kleine-Levin syndrome are male). It is characterized by recurring but reversible periods of excessive sleep (up to 20 hours per day). Symptoms occur as "episodes," typically lasting a few days to a few weeks. Episode onset is often abrupt, and may be associated with flu-like symptoms. Excessive food intake, irritability, childishness, disorientation, hallucinations, and an abnormally uninhibited sex drive may be observed during episodes. Mood can be depressed as a consequence, but not a cause, of the disorder. Affected individuals are completely normal between episodes, although they may not be able to remember afterwards everything that happened during the episode. It may be weeks or more before symptoms reappear. Symptoms may be related to malfunction of the hypothalamus and thalamus, parts of the brain that govern appetite and sleep.

Wikipedia : 73 Kleine-Levin syndrome (KLS) is a rare disorder characterized by persistent episodic hypersomnia and... more...

Related Diseases for Kleine-Levin Hibernation Syndrome

Diseases related to Kleine-Levin Hibernation Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 96)
# Related Disease Score Top Affiliating Genes
1 hypersomnia 32.0 HLA-DQB1 HCRT
2 idiopathic hypersomnia 30.7 HLA-DQB1 HCRT
3 sleep disorder 30.4 SLC6A3 IGLON5 HRH3 HCRT
4 narcolepsy 30.4 SLC6A3 HLA-DQB1 HCRT
5 eating disorder 30.2 SLC6A3 HCRT
6 dysthymic disorder 30.0 SLC6A3 HCRT
7 rem sleep behavior disorder 29.7 SLC6A3 IGLON5 HCRT
8 migraine with or without aura 1 29.5 SLC6A3 HRH3 HCRT
9 recurrent hypersomnia 28.3 TRANK1 TOR1B SLC6A3 PNMA2 LMOD3 IGLON5
10 rare sleep disorder 10.7
11 mood disorder 10.5
12 encephalitis 10.5
13 amnestic disorder 10.5
14 prader-willi syndrome 10.3
15 autism 10.3
16 major affective disorder 8 10.3
17 major affective disorder 9 10.3
18 sleep apnea 10.3
19 bipolar i disorder 10.3
20 psychotic disorder 10.3
21 bipolar disorder 10.3
22 encephalopathy 10.3
23 head injury 10.3
24 narcolepsy 2 10.2 HLA-DQB1 HCRT
25 narcolepsy 1 10.1 HLA-DQB1 HCRT
26 spondyloarthropathy 1 10.1
27 autoimmune disease 10.1
28 holt-oram syndrome 10.1
29 allergic rhinitis 10.1
30 anxiety 10.1
31 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
32 ifap syndrome 2 10.1
33 deficiency anemia 10.1
34 autism spectrum disorder 10.1
35 mild cognitive impairment 10.1
36 migraine with aura 10.1
37 tonsillitis 10.1
38 nasopharyngitis 10.1
39 hydrocephalus 10.1
40 depersonalization disorder 10.1
41 iron deficiency anemia 10.1
42 inflammatory spondylopathy 10.1
43 pica disease 10.1
44 hypothyroidism 10.1
45 normal pressure hydrocephalus 10.1
46 endogenous depression 10.1
47 mental depression 10.1
48 conversion disorder 10.1
49 cannabis dependence 10.1
50 impotence 10.1

Graphical network of the top 20 diseases related to Kleine-Levin Hibernation Syndrome:



Diseases related to Kleine-Levin Hibernation Syndrome

Symptoms & Phenotypes for Kleine-Levin Hibernation Syndrome

Human phenotypes related to Kleine-Levin Hibernation Syndrome:

31
# Description HPO Frequency HPO Source Accession
1 confusion 31 HP:0001289
2 polyphagia 31 HP:0002591
3 vivid hallucinations 31 HP:0006803
4 episodic hypersomnia 31 HP:0007200

Symptoms via clinical synopsis from OMIM®:

57 (Updated 20-May-2021)
Neuro:
vivid hallucinations
episodic hypersomnia
disorientation
episodic attacks of aberrant behavior
compulsive hyperphagia
more
Misc:
predominantly males

Clinical features from OMIM®:

148840 (Updated 20-May-2021)

Drugs & Therapeutics for Kleine-Levin Hibernation Syndrome

Drugs for Kleine-Levin Hibernation Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Radiopharmaceuticals
2 Technetium Tc 99m bicisate

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Brain Scintigraphy in Normal Versus Kleine-Levin Syndrome Subjects Completed NCT02337023
2 Microglial Activation in Narcolepsy Type 1 and Kleine-Levin Syndrome: Positron Emission Tomography (PET) Study in [18F] DPA-714 Recruiting NCT03754348
3 Coordination of Rare Diseases at Sanford Recruiting NCT01793168

Search NIH Clinical Center for Kleine-Levin Hibernation Syndrome

Cochrane evidence based reviews: kleine-levin syndrome

Genetic Tests for Kleine-Levin Hibernation Syndrome

Anatomical Context for Kleine-Levin Hibernation Syndrome

MalaCards organs/tissues related to Kleine-Levin Hibernation Syndrome:

40
Thalamus, Hypothalamus, Brain, Pituitary, Eye, Heart

Publications for Kleine-Levin Hibernation Syndrome

Articles related to Kleine-Levin Hibernation Syndrome:

(show top 50) (show all 374)
# Title Authors PMID Year
1
Kleine-Levin syndrome: a systematic study of 108 patients. 57 61
18438947 2008
2
Polysomnography in Kleine-Levin syndrome. 57 61
18316691 2008
3
Kleine-Levin syndrome: history and brief review. 61 57
18667831 2008
4
Episodic diencephalic hypoperfusion in Kleine-Levin syndrome. 57 61
16944679 2006
5
Familial Kleine-Levin syndrome: two siblings with unusually long hypersomnic spells. 61 57
12470186 2002
6
Kleine-Levin syndrome: an autoimmune hypothesis based on clinical and genetic analyses. 61 57
12473762 2002
7
A pathologic basis for Kleine-Levin syndrome. 57 61
6948538 1982
8
Kleine-Levin Syndrome. 20 61
28216256 2018
9
Kleine-Levin Syndrome. 20 61
27137943 2016
10
Kleine-Levin Syndrome. 61 20
26055863 2015
11
Periodic hypersomnia and megaphagia in adolescent males. 57
14023898 1962
12
Speculating on Kleine-Levin Syndrome mechanisms. 61
33416047 2021
13
Kleine-Levin syndrome is associated with birth difficulties and genetic variants in the TRANK1 gene loci. 61
33737391 2021
14
Kleine-Levin syndrome, GABA, and glutamate. 61
33283755 2021
15
Sleep Disturbances Associated with Neurological Autoimmunity. 61
33786802 2021
16
A strange New Year's Eve: triggers in Kleine-Levin syndrome. 61
33025902 2021
17
Excellent response to amantadine in Kleine Levin syndrome. 61
32917543 2020
18
Sleep and Sex: A Review of the Interrelationship of Sleep and Sexuality Disorders in the Female Population, Through the Lens of Sleeping Beauty Syndrome. 61
33023862 2020
19
[Tularaemia in two patients referred on suspicion of cancer mammae and cancer occulta]. 61
33000721 2020
20
Possible description of Kleine-Levin syndrome in "The Daredevils of Sassoun" Armenian medieval epic poem. 61
32861982 2020
21
Idiopathic Hypersomnia and Other Hypersomnia Syndromes. 61
32901432 2020
22
Central Disorders of Hypersomnolence. 61
32756227 2020
23
Sleep-Wake Disorders in Childhood. 61
32756235 2020
24
Neuroimaging of Narcolepsy and Primary Hypersomnias. 61
32111133 2020
25
Excessive daytime sleepiness in narcolepsy and central nervous system hypersomnias. 61
31154621 2020
26
Kleine-Levin syndrome is associated with LMOD3 variants. 61
32449287 2020
27
The neurophysiological basis of excessive daytime sleepiness: suggestions of an altered state of consciousness. 61
31140116 2020
28
A case of Kleine-Levin syndrome with positive anti-NMDA-type glutamate receptor antibodies. 61
32246569 2020
29
Twin Data: The Lives That Drive the Findings/Twin Research Reviews: Twin Concordance for Kleine-Levin Syndrome; Twin Study of Political Discussion; Twin Relationship Quality and Urinary Cortisol Level; Twin Research Guide/In the News: Twins' Same Day Deliveries; Death of a Twin Holocaust Survivor; Identical Vindman Twins; Twin Festival in France; Tiniest Twins. 61
32228740 2020
30
Obstructive sleep apnoea with co existent Kleine-Levine syndrome in a middle-aged man. 61
31850697 2019
31
Usefulness of brain SPECT imaging in the study of recurrent hypersomnia: Kleine-Levin syndrome. 61
28869046 2019
32
The Rare Sides of Twin Research: Important to Remember/Twin Research Reviews: Representation of Self-Image; Twins With Kleine-Levin Syndrome; Heteropaternal Lemur Twins; Risk of Dental Caries/In the Media: High-Society Models; 'Winkelevii' Super Bowl Twins; Multiple Birth × Three; Twin Sister Surrogate; A Presidential Twin? 61
31608853 2019
33
Kleine-Levin syndrome is associated with LMOD3 variants. 61
29923248 2019
34
New-Onset Psychosis in a Child with Suspected Kleine-Levin Syndrome. 61
30843731 2019
35
An Atypical Case of Kleine-Levin Syndrome. 61
30664337 2019
36
[Sleep disorders in neurological department and Kleine-Levin syndrome]. 61
31464294 2019
37
Clinical neurophysiology of CNS hypersomnias. 61
31307613 2019
38
Clinical Sleep-Wake Disorders I: Focus on Hypersomnias and Movement Disorders During Sleep. 61
30341725 2019
39
Putative Mechanisms of Action and Clinical Use of Lithium in Children and Adolescents: A Critical Review. 61
29256353 2019
40
Late diagnosis of a rare urea cycle disorder mimicking Kleine-Levin syndrome. 61
30588389 2018
41
Sleeping beauty syndrome presenting with insomnia. 61
30317200 2018
42
Emerging psychiatric disorders in Kleine-Levin syndrome. 61
29655261 2018
43
Kleine-Levin syndrome: clues to aetiology. 61
29532411 2018
44
The clinical characteristics of Kleine-Levin syndrome according to ethnicity and geographic location. 61
29397778 2018
45
Fluctuating CSF hypocretin-1 levels in mild brain trauma-induced Kleine-Levin syndrome. 61
30103953 2018
46
Breathing abnormalities during sleep in Kleine-Levin Syndrome: fact or coincidence? 61
29553974 2018
47
Neuroimaging in the Kleine-Levin Syndrome. 61
30030664 2018
48
Kleine-Levin syndrome: the contribution of PET scan and hydrogen breath test for small intestinal bacterial overgrowth. 61
29773218 2018
49
[Kleine Levin syndrome: Review of diagnosed cases of Buenos Aires, Argentina]. 61
30778405 2018
50
Kleine-Levin syndrome: A neuropsychiatric disorder. 61
29606318 2018

Variations for Kleine-Levin Hibernation Syndrome

Expression for Kleine-Levin Hibernation Syndrome

Search GEO for disease gene expression data for Kleine-Levin Hibernation Syndrome.

Pathways for Kleine-Levin Hibernation Syndrome

Pathways related to Kleine-Levin Hibernation Syndrome according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
10.26 SLC6A3 HRH3

GO Terms for Kleine-Levin Hibernation Syndrome

Sources for Kleine-Levin Hibernation Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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