Idiopathic hypersomnia
| Classification according to ICD-10 | |
|---|---|
| F51.1 | Inorganic hypersomnia |
| G47.1 | Pathologically increased need for sleep, hypersomnia |
| ICD-10 online (WHO version 2019) | |
The Idiopathic hypersomnia is a rare sleep disorder from the group of hypersomnia . Despite prolonged sleep at night , those affected experience unwanted, unavoidable sleep during the day with extended, unrefreshing or usually exhausting sleep episodes. The disease has significant negative effects on the patient in the social environment and at work.
The characteristic of hypersomnia is excessive daytime sleepiness , which cannot be explained by the prolonged sleep duration. The transition times after waking up to being awake are typically characterized by drowsiness .
According to the ICSD-2 classification system for sleep disorders, a distinction is made between the two forms idiopathic hypersomnia with long sleep times and idiopathic hypersomnia without long sleep times .
distribution
According to Orphanet , a rare disease database, information on prevalence is based on uncertain estimates. For the form with a long sleep time 1: 10,000 to 1: 25,000 are specified, for the form without a long sleep time 1: 11,000 to 1: 100,000. Men and women are affected roughly equally. The disease often begins in young adulthood.
Compared to narcolepsy , which is also one of the hypersomnias of central nervous origin , the disease occurs much less frequently.
Cause and development of the disease
The cause of idiopathic hypersomnia is unknown. The orexin / hypocretin levels is normal and there is no associated HLA characteristics.
Clinical Appearances and Symptoms
Idiopathic hypersomnia with long sleep times
In the anamnesis , the night sleep extended to more than 10 hours is noticeable. During the day, constant excessive daytime sleepiness leads to unavoidable and unrefreshing sleep episodes. These additional sleep episodes can last for several hours. Difficulties in waking up with drowsiness and morning stiffness after sleeping at night and also after the extra sleep episodes.
Idiopathic hypersomnia without long sleep
In the anamnesis, the excessive daytime sleepiness with unavoidable falling asleep during the day is noticeable. Those affected do not consider these sleep episodes to be very restful, although daytime sleepiness remains. The night sleep is not or only extended to about 10 hours.
Sleep paralysis and hypnagogic hallucinations can occur in both forms of the disease, and they are often associated with autonomic dysfunctions such as orthostatic hypotension.
Investigation methods
During the diagnosis, the increased sleep duration must first be recognized as such. Then numerous other possible causes of drowsiness must be ruled out.
The evaluation of the severity of hypersomnia using the levels light, medium and severe takes into account the frequency of involuntary sleep episodes, the conditions under which they occur and the social and occupational impairment as criteria.
Diagnosis
When the detected history of state lasts for at least three months, is for further diagnosis in a sleep laboratory , the polysomnography and the multiple sleep latency test used. There is no evidence-based literature on diagnosis using these methods. The application is used for differential diagnostics.
Idiopathic hypersomnia is a rare sleep disorder that, in the absence of a diagnostic gold standard, is diagnosed by exclusion. Similar is z. B. the subvigilance syndrome .
Differential diagnostics
By evaluating a sleep diary and using actigraphy , chronically insufficient sleep can be excluded as a cause of daytime sleepiness. By evaluating the polysomnography, narcolepsy , circadian sleep-wake arrhythmias and fragmented night sleep as a result of sleep-related breathing disorders (such as the forms of sleep apnea syndrome ) and sleep-related movements (such as restless legs syndrome , periodic limb movement disorder ) are excluded. Psychological examinations and neuro-radiological examinations serve to rule out further causes.
HLA and hypocretin determinations are not useful.
Idiopathic hypersomnia is never associated with cataplexy .
treatment
With regard to daytime sleepiness, about 75% of patients benefit from stimulants, but not with regard to intoxication.
forecast
According to Orphanet, the symptoms remain stable over time.
The disease has a significant, negative impact on both working life and other aspects of participation in community life. The guideline expressly names professional and social decline as frequent consequences. Idiopathic hypersomnia has the same handicap as narcolepsy.
Research needs
There is a lack of epidemiological surveys, data on triggers, genetics and course, and therapy studies based on the quality criteria of evidence-based medicine. The overlap of individual clinical features between idiopathic hypersomnia and narcolepsy has been repeatedly noted, but has not yet been explained.
Web links
- Idiopathic hypersomnia. In: Orphanet (Rare Disease Database).
Individual evidence
- ↑ a b c d S3 guideline for non-restful sleep / sleep disorders of the German Society for Sleep Research and Sleep Medicine (DGSM). In: AWMF online (as of 2009)
- ↑ www.hypersomniafoundation.org . Retrieved October 29, 2017.
- ^ A b c Claudio Bassetti, Michael S. Aldrich: Idiopathic hypersomnia. A series of 42 patients . In: Brain . Volume 120, No. 8 , 1997, pp. 1423-1435 , PMID 9278632 .
- ↑ International classification of sleep disorders, revised: Diagnostic and coding manual . American Academy of Sleep Medicine. 2001. Archived from the original on July 26, 2011. Retrieved January 25, 2013.
