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Kleine–Levin syndrome

Kleine-Levin Syndrome Overview: – Patients experience recurring episodes of prolonged sleep (hypersomnia). – During episodes, patients sleep 15 to 21 hours a day. – Common […]

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Kleine-Levin Syndrome Overview:
– Patients experience recurring episodes of prolonged sleep (hypersomnia).
– During episodes, patients sleep 15 to 21 hours a day.
– Common symptoms include hyperphagia, hypersexuality, mood changes, and cognitive difficulties.
– The thalamus and temporal lobe are believed to play a role in the condition.
– Imbalance in serotonin and dopamine pathways and viral infections are possible causes.

Diagnosis and Epidemiology:
– KLS is diagnosed after ruling out similar conditions.
– Diagnostic tests include MRI, CT scans, lumbar puncture, and toxicology tests.
– SPECT shows thalamic hypoperfusion in patients during episodes.
– The condition primarily affects teenagers, especially males.
– Prevalence is approximately 1 in 500,000, with a bias towards males.

Treatment and Prevention:
– There is no known cure for KLS.
– Lithium and stimulants may have limited effects in some patients.
– Drug treatments have uncertain effectiveness.
– Lithium shows a preventive effect in 20-40% of cases.
– Modafinil and certain medications can alleviate symptoms.

Prognosis and Management:
– KLS episodes vary in duration and frequency.
– Median episode duration is approximately ten days.
– Symptoms typically cease by age 30, with a potential cure after six years symptom-free.
– Management includes the use of antipsychotics, benzodiazepines, and mood stabilizers.

Historical and Research Aspects:
– Early descriptions date back to the 19th century, with formal classification in 1935.
– Diagnostic criteria were established in 1990.
– Genetic studies show promise but yield inconsistent results.
– Research continues to explore the etiology, diagnosis, and treatment of KLS.
– Studies have investigated the association of genetic variants with KLS, shedding light on potential underlying mechanisms.

Kleine–Levin syndrome (Wikipedia)

Kleine–Levin syndrome (KLS) is a rare neurological disorder characterized by persistent episodic hypersomnia accompanied by cognitive and behavioral changes. These changes may include disinhibition, sometimes manifested through hypersexuality, hyperphagia or emotional lability, and other symptoms, such as derealization. Patients generally experience recurrent episodes of the condition for more than a decade, which may return at a later age. Individual episodes generally last more than a week, sometimes lasting for months. The condition greatly affects the personal, professional, and social lives of those with KLS. The severity of symptoms and the course of the syndrome vary between those with KLS. Patients commonly have about 20 episodes over about a decade. Several months may elapse between episodes.

Kleine–Levin syndrome
Other names
  • Familial hibernation syndrome
  • Sleeping Beauty Syndrome
SpecialtyNeurology Edit this on Wikidata

The onset of the condition usually follows a viral infection (72% of patients); several different viruses have been observed to trigger KLS. It is generally only diagnosed after similar conditions have been excluded; MRI, CT scans, lumbar puncture, and toxicology tests are used to rule out other possibilities. The syndrome's mechanism is not known, but the thalamus is thought to possibly play a role. SPECT has shown thalamic hypoperfusion in patients during episodes.

KLS is very rare, occurring at a rate of 1 in 500,000, which limits research into genetic factors. The condition primarily affects teenagers (81% of reported patients), with a bias towards males (68-72% of cases), though females can also be affected, and the age of onset varies. There is no known cure, and there is little evidence supporting drug treatment. Lithium has been reported to have limited effects in case reports, decreasing the length of episodes and duration between them in some patients. Stimulants have been shown to promote wakefulness during episodes, but they do not counteract cognitive symptoms or decrease the duration of episodes. The condition is named after Willi Kleine and Max Levin, who described cases of the disease in the early 20th century. It was added to the International Classification of Sleep Disorders in 1990.

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