MCID: IDP031
MIFTS: 32

Idiopathic Hypersomnia

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Idiopathic Hypersomnia

MalaCards integrated aliases for Idiopathic Hypersomnia:

Name: Idiopathic Hypersomnia 53 59 55
Primary Hypersomnia 53 59 73
Hypersomnolence, Idiopathic 73
Idiopathic Hypersomnolence 53

Characteristics:

Orphanet epidemiological data:

59
idiopathic hypersomnia
Prevalence: 1-5/10000 (Europe); Age of onset: All ages; Age of death: normal life expectancy;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Orphanet 59 ORPHA33208
ICD10 via Orphanet 34 F51.1
UMLS via Orphanet 74 C0751757
SNOMED-CT via HPO 69 271782001 79519003 77692006

Summaries for Idiopathic Hypersomnia

NIH Rare Diseases : 53 Idiopathic hypersomnia (IH) is a neurological sleep disorder that can affect many aspects of a person's life. Symptoms often begin between adolescence and young adulthood and develop over weeks to months. People with IH have a hard time staying awake and alert during the day (chronic excessive daytime sleepiness, or EDS). They may fall asleep unintentionally or at inappropriate times, interfering with daily functioning. They may also have difficulty waking up from nighttime sleep or daytime naps. Sleeping longer at night does not appear to improve daytime sleepiness. IH is a chronic disorder. Symptoms may remain generally stable over time, or severity may fluctuate over time. The cause of IH is not known. Some people with IH have other family members with a sleep disorder such as IH or narcolepsy. Currently there is no treatment approved by the FDA specifically for IH, but some people may be helped by medications used to treat other disorders ("off-label") such as narcolepsy. Unlike in narcolepsy, in which scheduled naps may help, daytime naps in people with IH are often long yet unrefreshing. People with IH may experience improvement with medication; however, medications do not work well for all people or may stop working over time.

MalaCards based summary : Idiopathic Hypersomnia, also known as primary hypersomnia, is related to hypersomnia and narcolepsy. An important gene associated with Idiopathic Hypersomnia is HCRT (Hypocretin Neuropeptide Precursor). The drugs Clarithromycin and Flumazenil have been mentioned in the context of this disorder. Affiliated tissues include heart, testes and skin, and related phenotypes are excessive daytime somnolence and hypersomnia

Wikipedia : 76 Idiopathic hypersomnia is a neurological disorder which is characterized primarily by excessive daytime... more...

Related Diseases for Idiopathic Hypersomnia

Diseases related to Idiopathic Hypersomnia via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 16)
# Related Disease Score Top Affiliating Genes
1 hypersomnia 30.3 HCRT HLA-DQB1
2 narcolepsy 29.2 HCRT HLA-DQB1
3 kleine-levin hibernation syndrome 28.6 HCRT HLA-DQB1
4 idiopathic hypersomnia without long sleep time 12.2
5 idiopathic hypersomnia with long sleep time 12.0
6 sleep apnea 9.9
7 cerebritis 9.9
8 sleep disorder 9.9
9 apnea, obstructive sleep 9.7
10 insulinoma 9.7
11 hypothyroidism 9.7
12 malignant hyperthermia 9.7
13 headache 9.7
14 recurrent hypersomnia 9.5 HCRT HLA-DQB1
15 acute disseminated encephalomyelitis 9.4 HCRT HLA-DQB1
16 guillain-barre syndrome 9.2 HCRT HLA-DQB1

Graphical network of the top 20 diseases related to Idiopathic Hypersomnia:



Diseases related to Idiopathic Hypersomnia

Symptoms & Phenotypes for Idiopathic Hypersomnia

Human phenotypes related to Idiopathic Hypersomnia:

59 32
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 excessive daytime somnolence 59 32 hallmark (90%) Very frequent (99-80%) HP:0001262
2 hypersomnia 59 32 hallmark (90%) Very frequent (99-80%) HP:0100786
3 sleep disturbance 59 Very frequent (99-80%)

Drugs & Therapeutics for Idiopathic Hypersomnia

Drugs for Idiopathic Hypersomnia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Clarithromycin Approved Phase 2 81103-11-9 84029
2
Flumazenil Approved Phase 1, Phase 2 78755-81-4 3373
3 GABA Agents Phase 1, Phase 2
4 GABA Modulators Phase 1, Phase 2
5 Neurotransmitter Agents Phase 1, Phase 2
6 Cytochrome P-450 CYP3A Inhibitors Phase 2
7 Cytochrome P-450 Enzyme Inhibitors Phase 2
8 Anti-Bacterial Agents Phase 2
9 Antidotes Phase 1, Phase 2
10 Anti-Infective Agents Phase 2
11 Protective Agents Phase 1, Phase 2

Interventional clinical trials:

(show all 11)
# Name Status NCT ID Phase Drugs
1 A Multicenter Study of the Efficacy and Safety of JZP-258 in the Treatment of Idiopathic Hypersomnia (IH) With an Open-label Safety Extension Not yet recruiting NCT03533114 Phase 3 JZP-258;Placebo Oral Tablet
2 Flumazenil for the Treatment of Primary Hypersomnia Completed NCT01183312 Phase 1, Phase 2 Flumazenil
3 Clarithromycin for the Treatment of Hypersomnia Completed NCT01146600 Phase 2 Clarithromycin followed by placebo;Placebo then Clarithromycin
4 A Study of Oral BTD-001 in Adults With Idiopathic Hypersomnia Recruiting NCT03542851 Phase 2 Pentetrazol (PTZ);Placebo oral capsule
5 A Study of Safety and Efficacy of BTD-001 in Treatment of Patients With Idiopathic Hypersomnia (IH) or Narcolepsy Type 2 Active, not recruiting NCT02512588 Phase 2 BTD-001;Placebo
6 Neurophysiologic Correlates of Hypersomnia Unknown status NCT01719315
7 Cardiovascular Variability and Heart Rate Arousal Response in Idiopathic Hypersomnia Completed NCT02913651
8 Amylase and Hypersomnia Completed NCT01926405 Not Applicable
9 The Role of the Circadian System in Neurological Sleep-wake Disorders Recruiting NCT03356938 Not Applicable
10 Importance of Sleep Deprivation in Differential Diagnosis of Primary Hypersomnia (Actisom dépistage) Recruiting NCT03459300
11 Rare Disease Patient Registry & Natural History Study - Coordination of Rare Diseases at Sanford Recruiting NCT01793168

Search NIH Clinical Center for Idiopathic Hypersomnia

Genetic Tests for Idiopathic Hypersomnia

Anatomical Context for Idiopathic Hypersomnia

MalaCards organs/tissues related to Idiopathic Hypersomnia:

41
Heart, Testes, Skin, Brain

Publications for Idiopathic Hypersomnia

Articles related to Idiopathic Hypersomnia:

(show top 50) (show all 72)
# Title Authors Year
1
Update on treatment for idiopathic hypersomnia. ( 29250981 )
2018
2
Alternative diagnostic criteria for idiopathic hypersomnia: A 32-hour protocol. ( 29323727 )
2018
3
Idiopathic Hypersomnia Patients Revealed Longer Circadian Period Length in Peripheral Skin Fibroblasts. ( 29930532 )
2018
4
Associations Between Neuropsychological, Neurobehavioral and Emotional Functioning and Either Narcolepsy or Idiopathic Hypersomnia in Children and Adolescents. ( 29609710 )
2018
5
Nocturnal sleep architecture in idiopathic hypersomnia: a systematic review and meta-analysis. ( 29680423 )
2018
6
[18F]Fludeoxyglucose-Positron Emission Tomography Evidence for Cerebral Hypermetabolism in the Awake State in Narcolepsy and Idiopathic Hypersomnia. ( 28775709 )
2017
7
Brain MRI findings in patients with idiopathic hypersomnia. ( 28376374 )
2017
8
Idiopathic Hypersomnia. ( 28778232 )
2017
9
Case Report of a Patient With Idiopathic Hypersomnia and a Family History of Malignant Hyperthermia Undergoing General Anesthesia: An Overview of the Anesthetic Considerations. ( 28328583 )
2017
10
Altered Regional Cerebral Blood Flow in Idiopathic Hypersomnia. ( 28958044 )
2017
11
Benefits and risk of sodium oxybate in idiopathic hypersomnia versus narcolepsy type 1: a chart review. ( 26847972 )
2016
12
Diurnal and nocturnal cardiovascular variability and heart rate arousal response in idiopathic hypersomnia. ( 27810179 )
2016
13
French consensus. Idiopathic hypersomnia: Investigations and follow-up. ( 27838089 )
2016
14
Idiopathic Hypersomnia and Hypersomnolence Disorder: A Systematic Review of the Literature. ( 26895727 )
2016
15
Disorders of Excessive Daytime Sleepiness Including Narcolepsy and Idiopathic Hypersomnia. ( 27542882 )
2016
16
The effects of Transcranial Direct Current Stimulation (tDCS) on Idiopathic Hypersomnia: a pilot study. ( 27548094 )
2016
17
Narcolepsy with and without cataplexy, idiopathic hypersomnia with and without long sleep time: a cluster analysis. ( 25576137 )
2015
18
The Prevalence and Characteristics of Primary Headache and Dream-Enacting Behaviour in Japanese Patients with Narcolepsy or Idiopathic Hypersomnia: A Multi-Centre Cross-Sectional Study. ( 26418536 )
2015
19
Smoking, Alcohol, Drug Use, Abuse and Dependence in Narcolepsy and Idiopathic Hypersomnia: A Case-Control Study. ( 26564129 )
2015
20
Central Disorders of Hypersomnolence: Focus on the Narcolepsies and Idiopathic Hypersomnia. ( 26149554 )
2015
21
An association analysis of HLA-DQB1 with narcolepsy without cataplexy and idiopathic hypersomnia with/without long sleep time in a Japanese population. ( 27081540 )
2015
22
Idiopathic hypersomnia. ( 26599679 )
2015
23
Evaluation of the Effect of Modafinil on Cognitive Functions in Patients with Idiopathic Hypersomnia with P300. ( 26116438 )
2015
24
Use of subcutaneous flumazenil preparations for the treatment of idiopathic hypersomnia: A case report. ( 24554692 )
2014
25
Altered dynamics in the circadian oscillation of clock genes in dermal fibroblasts of patients suffering from idiopathic hypersomnia. ( 24454829 )
2014
26
Manual characterization of sleep spindle index in patients with narcolepsy and idiopathic hypersomnia. ( 24800086 )
2014
27
Modafinil in the treatment of idiopathic hypersomnia without long sleep time-a randomized, double-blind, placebo-controlled study. ( 25196321 )
2014
28
Polysomnographic study of nocturnal sleep in idiopathic hypersomnia without long sleep time. ( 23061443 )
2013
29
Characterization of REM sleep without atonia in patients with narcolepsy and idiopathic hypersomnia using AASM scoring manual criteria. ( 23853561 )
2013
30
Test-retest reliability of the multiple sleep latency test in narcolepsy without cataplexy and idiopathic hypersomnia. ( 23946709 )
2013
31
Differences in findings of nocturnal polysomnography and multiple sleep latency test between narcolepsy and idiopathic hypersomnia. ( 21723190 )
2012
32
Quality of life in patients with narcolepsy with cataplexy, narcolepsy without cataplexy, and idiopathic hypersomnia without long sleep time: comparison between patients on psychostimulants, drug-naA^ve patients and the general Japanese population. ( 22137109 )
2012
33
Effect of levothyroxine on prolonged nocturnal sleep time and excessive daytime somnolence in patients with idiopathic hypersomnia. ( 21570346 )
2011
34
Kleine-levin syndrome and idiopathic hypersomnia: spectrum disorders. ( 22345850 )
2011
35
Benefit and risk of modafinil in idiopathic hypersomnia vs. narcolepsy with cataplexy. ( 21576035 )
2011
36
Idiopathic hypersomnia: a report of three adolescent-onset cases in a two-generation family. ( 21270467 )
2011
37
Insulinoma presenting as idiopathic hypersomnia. ( 20119740 )
2010
38
IgG abnormality in narcolepsy and idiopathic hypersomnia. ( 20221267 )
2010
39
Subjective symptoms in idiopathic hypersomnia: beyond excessive sleepiness. ( 20408941 )
2010
40
CSF histamine contents in narcolepsy, idiopathic hypersomnia and obstructive sleep apnea syndrome. ( 19238805 )
2009
41
Comparison of clinical characteristics among narcolepsy with and without cataplexy and idiopathic hypersomnia without long sleep time, focusing on HLA-DRB1( *)1501/DQB1( *)0602 finding. ( 19410508 )
2009
42
Is low histamine a fundamental cause of sleepiness in narcolepsy and idiopathic hypersomnia? ( 19238797 )
2009
43
Prevalence of the HLA-DQB1*0602 allele in narcolepsy and idiopathic hypersomnia patients seen at a sleep disorders outpatient unit in SALo Paulo. ( 19506770 )
2009
44
From narcolepsy with cataplexy to idiopathic hypersomnia without long sleep time. ( 19342293 )
2009
45
Idiopathic hypersomnia: clinical features and response to treatment. ( 20465024 )
2009
46
Idiopathic hypersomnia with and without long sleep time: a controlled series of 75 patients. ( 19544751 )
2009
47
Successful treatment with levothyroxine for idiopathic hypersomnia patients with subclinical hypothyroidism. ( 19269544 )
2009
48
What is idiopathic hypersomnia? ( 18460283 )
2008
49
Health-related quality of life among drug-naA^ve patients with narcolepsy with cataplexy, narcolepsy without cataplexy, and idiopathic hypersomnia without long sleep time. ( 19110887 )
2008
50
Idiopathic hypersomnia: a study of 77 cases. ( 17969461 )
2007

Variations for Idiopathic Hypersomnia

Expression for Idiopathic Hypersomnia

Search GEO for disease gene expression data for Idiopathic Hypersomnia.

Pathways for Idiopathic Hypersomnia

GO Terms for Idiopathic Hypersomnia

Sources for Idiopathic Hypersomnia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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