MCID: KLN009
MIFTS: 38

Kleine-Levin Hibernation Syndrome

Categories: Genetic diseases, Mental diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Kleine-Levin Hibernation Syndrome

MalaCards integrated aliases for Kleine-Levin Hibernation Syndrome:

Name: Kleine-Levin Hibernation Syndrome 57 19
Kleine-Levin Syndrome 57 11 52 58 75 43 14 16 71 33
Familial Kleine-Levin Syndrome 19 71
Familial Hibernation Syndrome 19
Hypersomnia-Bulimia Syndrome 33
Recurrent Hypersomnolence 33
Kleine Levin Syndrome 19

Characteristics:


Inheritance:

Autosomal dominant 57

Prevelance:

Kleine-Levin Syndrome: 1-9/1000000 (France) 58

Age Of Onset:

Kleine-Levin Syndrome: Adolescent,Adult,Childhood 58

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 11 DOID:0060165
OMIM® 57 148840
MeSH 43 D017593
MESH via Orphanet 44 D017593
ICD10 via Orphanet 32 G47.8
UMLS via Orphanet 72 C0206085
Orphanet 58 ORPHA33543
MedGen 40 C0206085
ICD11 33 1180849398
UMLS 71 C0206085 C2936703

Summaries for Kleine-Levin Hibernation Syndrome

GARD: 19 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 TRANK1 (Tetratricopeptide Repeat And Ankyrin Repeat Containing 1). 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

NINDS: 52 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.

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 24-Oct-2022)

Orphanet: 58 Kleine-Levin syndrome (KLS) is a rare neurological disorder of unknown origin characterised by relapsing-remitting episodes of hypersomnia in association with cognitive and behavioural disturbances.

Disease Ontology: 11 A recurrent hypersomnia that is characterized by recurring periods of excessive amounts of sleep and altered behavior.

Wikipedia: 75 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 121)
# Related Disease Score Top Affiliating Genes
1 hypersomnia 32.1 HLA-DQB1 HCRT
2 idiopathic hypersomnia 32.0 HLA-DQB1 HCRT
3 sleep disorder 30.9 IGLON5 HRH3 HLA-DQB1 HCRT
4 narcolepsy 30.7 P2RY11 HLA-DQB1 HCRT
5 apnea, obstructive sleep 30.5 IGLON5 HCRT
6 narcolepsy 2 30.4 HLA-DQB1 HCRT
7 narcolepsy 1 30.4 P2RY11 HLA-DQB1 HCRT
8 rem sleep behavior disorder 30.2 IGLON5 HCRT
9 viral encephalitis 29.9 PNMA2 LGI1
10 recurrent hypersomnia 28.5 TRANK1 TOR1B PNMA2 P2RY11 LMOD3 LGI1
11 cranioectodermal dysplasia 11.4
12 rare sleep disorder 10.7
13 hypothalamic disease 10.6
14 mood disorder 10.5
15 bulimia nervosa 10.5
16 encephalitis 10.5
17 amnestic disorder 10.5
18 eating disorder 10.5
19 psychotic disorder 10.4
20 encephalopathy 10.4
21 prader-willi syndrome 10.3
22 autism 10.3
23 autism spectrum disorder 10.3
24 bipolar i disorder 10.3
25 central nervous system origin vertigo 10.3
26 bipolar disorder 10.3
27 periodic limb movement disorder 10.1 HLA-DQB1 HCRT
28 spondyloarthropathy 1 10.1
29 photoparoxysmal response 1 10.1
30 holt-oram syndrome 10.1
31 attention deficit-hyperactivity disorder 10.1
32 migraine with or without aura 1 10.1
33 thrombocytopenic purpura, autoimmune 10.1
34 obesity-hypoventilation syndrome 10.1
35 hypothyroidism, congenital, nongoitrous, 1 10.1
36 allergic rhinitis 10.1
37 anxiety 10.1
38 major depressive disorder 10.1
39 immunodeficiency, common variable, 10 10.1
40 deficiency anemia 10.1
41 sleep apnea 10.1
42 cardiac arrest 10.1
43 covid-19 10.1
44 mild cognitive impairment 10.1
45 migraine with aura 10.1
46 tonsillitis 10.1
47 nasopharyngitis 10.1
48 hydrocephalus 10.1
49 depersonalization disorder 10.1
50 iron deficiency anemia 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:

30
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 confusion 30 HP:0001289
2 polyphagia 30 HP:0002591
3 vivid hallucinations 30 HP:0006803
4 episodic hypersomnia 30 HP:0007200

Symptoms via clinical synopsis from OMIM®:

57 (Updated 24-Oct-2022)
Neuro:
vivid hallucinations
episodic hypersomnia
disorientation
episodic attacks of aberrant behavior
compulsive hyperphagia
more
Misc:
predominantly males

Clinical features from OMIM®:

148840 (Updated 24-Oct-2022)

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

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased homologous recombination repair frequency GR00151-A-1 8.62 ADCY2 HCRT

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 Microglial Activation in Narcolepsy Type 1 and Kleine-Levin Syndrome: Positron Emission Tomography (PET) Study in [18F] DPA-714 Unknown status NCT03754348
2 Brain Scintigraphy in Normal Versus Kleine-Levin Syndrome Subjects Completed NCT02337023
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

Organs/tissues related to Kleine-Levin Hibernation Syndrome:

MalaCards : Thalamus, Hypothalamus, Brain, Pituitary, Eye, Heart

Publications for Kleine-Levin Hibernation Syndrome

Articles related to Kleine-Levin Hibernation Syndrome:

(show top 50) (show all 396)
# Title Authors PMID Year
1
Kleine-Levin syndrome: a systematic study of 108 patients. 62 57
18438947 2008
2
Polysomnography in Kleine-Levin syndrome. 62 57
18316691 2008
3
Kleine-Levin syndrome: history and brief review. 62 57
18667831 2008
4
Episodic diencephalic hypoperfusion in Kleine-Levin syndrome. 62 57
16944679 2006
5
Kleine-Levin syndrome: an autoimmune hypothesis based on clinical and genetic analyses. 62 57
12473762 2002
6
Familial Kleine-Levin syndrome: two siblings with unusually long hypersomnic spells. 62 57
12470186 2002
7
A pathologic basis for Kleine-Levin syndrome. 62 57
6948538 1982
8
Periodic hypersomnia and megaphagia in adolescent males. 57
14023898 1962
9
Kleine-Levin syndrome: report of a case with marked dysautonomic features. 62
35393940 2022
10
Proteomic biomarkers of Kleine-Levin syndrome. 62
35859339 2022
11
Neuroimaging in the Rare Sleep Disorder of Kleine-Levin Syndrome: A Systematic Review. 62
35735605 2022
12
General Anesthetic Management of a Patient With Kleine-Levin Syndrome. 62
35377928 2022
13
Kleine-Levin syndrome: The great mimicker. 62
35231775 2022
14
LMOD3 gene variant in familial periodic hypersomnolence. 62
35299092 2022
15
Two cases of Kleine-Levin syndrome with good responses to lithium treatment at low serum levels. 62
35312205 2022
16
A case of Kleine-Levin syndrome successfully treated with Escitalopram. 62
35847426 2022
17
An Overview on Sleep Medicine. 62
36217075 2022
18
COVID-19 and Central Nervous System Hypersomnias. 62
35911079 2022
19
Kleine-Levin syndrome and general anesthesia: A case report. 62
34416569 2021
20
"Sleeping Beauty Syndrome" and Psychosis as Precursory Symptoms of Multiple Sclerosis: A Rare Case and Literature Review. 62
34846357 2021
21
Kleine-Levin syndrome related to pregnancy: a case report. 62
34170245 2021
22
Self-representation in Kleine-Levin syndrome: a single case fMRI study. 62
34541988 2021
23
Treatment of Kleine-Levin Syndrome With Intranasal Photobiomodulation and Methylene Blue. 62
34659921 2021
24
Cognitive dysfunction in central disorders of hypersomnolence: A systematic review. 62
34166991 2021
25
Different circadian rest-active rhythms in Kleine-Levin syndrome: a prospective and case-control study. 62
33851710 2021
26
Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine clinical practice guideline. 62
34743789 2021
27
SARS-CoV-2 Infection Causes Relapse of Kleine-Levin Syndrome: Case Report and Review of Literature. 62
34294673 2021
28
Excessive Daytime Sleepiness: A Clinical Review. 62
33840518 2021
29
Sleep and Sex: A Review of the Interrelationship of Sleep and Sexuality Disorders in the Female Population, Through the Lens of Sleeping Beauty Syndrome. 62
33023862 2021
30
Kleine-Levin syndrome, GABA, and glutamate. 62
33283755 2021
31
Kleine-Levin syndrome is associated with birth difficulties and genetic variants in the TRANK1 gene loci. 62
33737391 2021
32
Speculating on Kleine-Levin Syndrome mechanisms. 62
33416047 2021
33
A strange New Year's Eve: triggers in Kleine-Levin syndrome. 62
33025902 2021
34
Functional brain imaging using 18F-fluorodeoxyglucose positron emission tomography/computerized tomography in 138 patients with Kleine-Levin syndrome: an early marker? 62
34189461 2021
35
Sleep disorders and the hypothalamus. 62
34266606 2021
36
Idiopathic Hypersomnia and Other Hypersomnia Syndromes. 62
32901432 2021
37
Sleep Disturbances Associated with Neurological Autoimmunity. 62
33786802 2021
38
Excellent response to amantadine in Kleine Levin syndrome. 62
32917543 2020
39
[Tularaemia in two patients referred on suspicion of cancer mammae and cancer occulta]. 62
33000721 2020
40
Possible description of Kleine-Levin syndrome in "The Daredevils of Sassoun" Armenian medieval epic poem. 62
32861982 2020
41
Sleep-Wake Disorders in Childhood. 62
32756235 2020
42
Central Disorders of Hypersomnolence. 62
32756227 2020
43
Neuroimaging of Narcolepsy and Primary Hypersomnias. 62
32111133 2020
44
Kleine-Levin syndrome is associated with LMOD3 variants. 62
32449287 2020
45
Excessive daytime sleepiness in narcolepsy and central nervous system hypersomnias. 62
31154621 2020
46
The neurophysiological basis of excessive daytime sleepiness: suggestions of an altered state of consciousness. 62
31140116 2020
47
A case of Kleine-Levin syndrome with positive anti-NMDA-type glutamate receptor antibodies. 62
32246569 2020
48
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. 62
32228740 2020
49
Obstructive sleep apnoea with co existent Kleine-Levine syndrome in a middle-aged man. 62
31850697 2019
50
Usefulness of brain SPECT imaging in the study of recurrent hypersomnia: Kleine-Levin syndrome. 62
28869046 2019

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

GO Terms for Kleine-Levin Hibernation Syndrome

Sources for Kleine-Levin Hibernation Syndrome

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 24-Oct-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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