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Idiopathic Hypersomnia – Kleine Levin Syndrome

Idiopathic and Recurrent Hypersomnia – Symptoms – Diagnosis – Treatment – Prognosis

Idiopathic Hypersomnia

The cardinal symptom of idiopathic hypersomnia is nonrefreshing sleep. No matter how long the person sleeps, there is no sense of refreshment. Naps do not bring any relief, unlike narcolepsy, in which patients feel recharged for a few hours after nap.

Similar to narcolepsy, sudden sleep attacks are common.

Patients usually sleep long hours at night. Change of sleep pattern at night makes no difference in the sense of well-being during the day.
Idiopathic hypersomnia does not produce REM sleep associated symptoms (cataplexy, sleep paralysis, hallucinations or fragmented sleep) because it is not a REM sleep disorder.

Multiple sleep latency test demonstrates a very short sleep latency (the amount of time required to fall asleep) but there is no REM sleep phase intrusion.

Unlike narcolepsy, hypocretin (orexin) level is normal in cases of idiopathic hypersomnia.

There is some familial predisposition but no HLA type association. Idiopathic hypersomnia typically manifests in teenagers and young adults.

The cause of idiopathic hypersomnia remains elusive. Idiopathic hypersomnia is about 10 times less common than narcolepsy. This is a diagnosis of exclusion, meaning that all other possible causes of hypersomnia have to be excluded before the diagnosis of idiopathic hypersomnia is made.

Stimulants and wake-promoting agents are the mainstay of treatment.

Naps and antidepressants provide no benefit.

There is some evidence that melatonin and thyroid hormones may offer some improvement of quality of life.

Recurrent Hypersomnia (Kleine-Levin Syndrome)

Kleine-Levin syndrome is characterized by periods of excessive sleepiness lasting from 2 days to 4 weeks and occurring at least once a year. At the time of excessive sleepiness, sleep need is increased to more than 12 hours per 24 hour period.

During the symptomatic period patients go on an eating binge and exhibit inappropriate sexual arousal upon awakening. The recall about events during hypersomnia is often limited.

Kleine-Levin syndrome sufferers typically have normal cognitive functioning and alertness level between the episodes.
The initial episode is often precipitated by infection with high fever, stress, sleep deprivation or excessive alcohol consumption.

Over time the recurrence of episodes becomes less frequent, less severe, and shorter in duration. In patients with disease onset prior to adulthood and with lack of hypersexuality persistent remissions were reported.

Recurrent hypersomnia is a rear condition with prevalence about 1 per million. It is twice as common in males. Disproportionally large numbers of Ashkenazi Jews are affected. The initial presentation is reported from 4 to 80 years old, but the onset is more typical in the second decade of life. Familial cases are rare.

There is no confirmatory diagnostic testing available. Sleep study and Brain MRI do not demonstrate any specific findings.

There are no proven treatment options for Kleine-Levin Syndrome.

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