Kleine Levin Syndrome
| Classification according to ICD-10 | |
|---|---|
| G47.8 | Other sleep disorders including Kleine Levin syndrome |
| ICD-10 online (WHO version 2019) | |
The Kleine Levin Syndrome ( KLS , also called Sleeping Beauty Syndrome ) is a disease with a periodically increased need for sleep ( hypersomnia ), perception and behavior disorders. It belongs to the group of hypersomnias of central nervous origin unrelated to sleep-related breathing disorders. The disease occurs preferentially in male adolescents. A genetic cause is assumed, but is not certain.
Symptoms
The main symptom are recurring periods with a considerably longer need for sleep, the duration of the periods varies from a few days to a month. Affected people oversleep most of the day and are only awake about 1 to 2 hours a day during the intensive phase of the disease. During these short periods of wakefulness, they are usually non-communicative, disoriented, lethargic, and apathetic. During the sleep phase the patients can be woken up, but they go back to sleep immediately. Hypersensitivity to light and noise often occurs. The periodic sleep disturbances recur at irregular intervals of several months, but they can also occur several times within a month. Food cravings can occur when you are awake. Hypersexual behavior often occurs . The addiction to sleep can last for several days to weeks and then subsides again. Patients report that they feel like they are living in a dream during the illness phase. There is no differentiation between dream and reality. Those affected are inconspicuous in the symptom-free intervals. The diagnosis is also of criminal legal importance if prohibited acts occur in phases of illness.
distribution
According to Orphanet , a rare disease database, the prevalence of this rare neurological disease of unknown cause is 1–2 / 1,000,000
Investigation methods
Diagnosis
In the anamnesis , the recurring occurrence of severe hypersomnia in connection with additional symptoms for a few days to several weeks and inconspicuous findings in the time in between are typical. In the hypersomnic phases, the possible additional symptoms include aggressiveness , eating disorders , sexual disinhibition, cognitive disorders and also hallucinations or confusion. To confirm the diagnosis and forensic reasons come in the sleep laboratory , the polysomnography (with video monitoring and possibly extended to 24 hours) and the multiple sleep latency test used.
However, no controlled studies are available for diagnosis.
Differential diagnostics
The delimitation is made to
- other hypersomnias of central nervous origin,
- secondary hypersomnias in psychiatric or neurological diseases and
- Hypersomnia in the context of sleep-related breathing disorders, circadian sleep-wake rhythm disorders and sleep- related movement disorders .
etymology
The disease is named after the German psychiatrist Willi Kleine and the American neurologist Max Levin , who described it in 1925 in the monthly magazine for psychiatry and neurology, in 1929 in the Archives of Neurology and Psychiatry and in 1936 in Brain . The neurologist Macdonald Critchley took up these descriptions in 1942 and used the term commonly used today for the syndrome. He also gave a more detailed description of the symptoms and the course of the disease.
Review
A review published in 2005 based on 186 cases reported from 1962 to 2004 showed a sporadic distribution worldwide with a slight preference for the male sex (68%). The age at the onset of symptoms fluctuated between the ages of four and 82, with a median of 15 years. 81% of the patients showed the first symptoms in the second decade of life. The median 10-day episodes occurred every three and a half months with a total duration of eight years.
Frequently (38.2%) the syndrome was preceded by an infection , less often a traumatic brain injury (9%) or alcohol consumption (5.4%). The symptoms were u. a. in hypersomnia (100%), cognitive disorders (96%), eating disorders (80%), sexual hyperactivity (43%) and depressive episodes (48%). In the 213 therapy studies, neuroleptics and antidepressants showed no therapeutic effect, stimulants (mainly amphetamines ) led to a decrease in the need for sleep. Episodes were suppressed more frequently (41%) with lithium than without therapy (19%).
literature
- Klaus Reichert : A casuistic contribution to the so-called Kleine-Levin-Syndrome. In: Christian Hoffstadt , Franz Peschke, Andreas Schulz-Buchta (eds.): We, the mechanics of body and soul. Collected writings of Klaus Reichert . (= Aspects of Medical Philosophy. Volume 4). Projektverlag, Bochum / Freiburg 2006, ISBN 3-89733-156-X , p. 313 ff.
Web links
- KLS Network eV
- Kleine Levin Syndrome. In: Orphanet (Rare Disease Database).
- Guideline S3-Non-restful sleep - sleep disorders . In: AWMF online
- The KLS Foundation (English)
Individual evidence
- ↑ HG Hess: Kleine Levin Syndrome. In: sleep. 2013; 2, pp. 89–93.
- ↑ a b 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)
- ^ Kleine W .: Periodic sleep addiction. In: Mtschr Psychiatrie Neurol . tape 57 , 1925, pp. 285-320 .
- ↑ Levin M .: Narcolepsy (Gélineau's syndrome) and other varieties of morbid somnolence. In: Arch Neurol Psychiatry . tape 22 , 1929, pp. 1172-1200 .
- ^ Levin M .: Periodic somnolence and morbid hunger: A new syndrome. In: Brain . tape 59 , 1936, pp. 494-504 .
- ^ M. Critchley, HL Hoffman: The syndrome of periodic somnolence and morbid hunger (Kleine-Levin syndrome). In: British Medical Journal . London, (1942); 1, pp. 137-139.
- ^ M. Critchley: Periodic hypersomnia and megaphagia in adolescent males. In: Brain. (1962); 85, pp. 627-656.
- ↑ I. Arnulf et al .: Kleine-Levin syndrome: a systematic review of 186 cases in the literature. In: Brain. (2005); 128, pp. 2763-2776. PMID 16230322 .
