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[Kleine-Levin syndrome].

The Kleine-Levin syndrome is a rare and probably underdiagnosed syndrome. It is characterized by periodic attacks of the triad: hypersomnia, vegative disturbances such as hyperphagia and hypersexuality, psychopathological changes in the level of consciousness and control of emotions. Boys and young men in the age group 10-20 years are most commonly affected. Spontaneous remission with a tendency to remission is observed and the disease "burns out" after a prolonged period of years. The etiology and pathogenesis are unknown. Theories have been propounded suggesting dysfunction of the hypothalamus. No pathognomonic findings have been observed in the early phase of sleep during the daylight hours. Central stimulating drugs have been reported to have some effect on the hypersomnia. The diagnosis is based on the clinical picture. Frequently, a long period can elapse before the diagnosis is established and some cases are never diagnosed. The literature is reviewed and is illustrated by two case reports.

Adolescent↗

Kleine-Levin syndrome associated with fire setting.

Kleine-Levin syndrome is characterized by periodic hypersomnia associated with megaphagia and striking behavioral and psychiatric symptoms; it occurs primarily in adolescent boys. We treated a 17-year-old boy who had typical recurring somnolent episodes. His bizarre behavior included fire setting and stealing, both of which may have represented compulsions. His EEG during a sleepy episode was diffusely abnormal with generalized slowing of background activity; as he awoke and improved clinically, his EEG returned to normal. Between episodes, his EEG was normal. The relationship between Kleine-Levin syndrome and other sleep disorders is discussed. The neurochemistry and neurocircuitry that may provide the requisite substrate for this complex and fascinating neuropsychiatric disorder are briefly reviewed.

Adolescent↗

[Kleine-Levin syndrome. Female cases and catamneses].

The Kleine-Levin syndrome (periodic hypersomnia and megaphagia in adolescence) was considered till 1962 to be a disorder limited to the male adolescents. Since 1924, however, a few female cases have been reported about. These, being approximately 20 patients, are registered by their essential symptoms together with 2 additional cases observed by the author. Moreover, prospective catamneses, covering periods of more than 12 years, were described for the first time. Probably, the Kleine-Levin-syndrome is the product of disturbed functions involving structures of hypothalamus and the reticular system. The syndrome seems to belong to the same groups of periodical diseases as migraine: in particular it exhibits similar relations to the genital cycle. Therapeutical success was attained by administration of amphetamines. In course of time, the episodes become spontaneously rarer and mostly cease.

Adolescent↗

The Kleine-Levine syndrome--a variant?

A case of Kleine-Levin syndrome in an older man is described, with a review of the literature. An important point is emphasized in our case in which the patient reacted unfavorably to Ritalin, becoming sexually aroused, although his hypersomnia improved. A possibly different mechanism in hypersomnia and hypersexuality is speculated.

Adult↗

Sleep patterns in Kleine-Levin syndrome.

Diurnal and nocturnal sleep records were obtained from a male and a female with Kleine-Levin syndrome, during excessive daytime sleep attacks and while they were asymptomatic. A common pattern of abnormal sleep was seen in both patients even during the asymptomatic period. The female, aflicted with a severe form of periodic hypersomnia, showed nocturnal and diurnal sleep onset REM periods. The different pattern of sleep abnormality in the female could be an expression of the severity of her symptoms or might indicate a variant of sleep abnormality present only in females with Kleine-Levin syndrome.

Adolescent↗

Kleine-Levin syndrome with periodic apnea during hypersomnic stages--E.E.G. study.

A 33 year old male, suffering from Kleine-Levine syndrome associated with periods of apnea during the hypersomnic attacks, is reported. Ventilatory studies negate the Pickwickian syndrome. The E.E.G.'s recorded during the hypersomnic attacks and the apneic periods showed a direct correlation between high-voltage delta waves paroxysmal E.E.G. activity, and apneic period. Medications known to improve Kleine-Levin syndrome, in our case, had no effect upon the clinical hypersomnic and apnea periods, nor on the correlatives E.E.G.'s pattern and spirometric studies. Theoretical considerations let us assume that these paroxysmal E.E.G. patterns associated with apnea are NRem-sleep serotonin dependent, and have an inhibitory influence on the respiratory centers, by alternating the equilibrium between the catecholamines and acetylcholine activities.

Adult↗

Behavioral manifestations of the Kleine-Levin syndrome.

The authors present a case of the Kleine-Levin syndrome, and review the literature to assess the various ways this illness can appear. Psychiatric manifestations not only obscure the diagnosis, but may lead to inappropriate treatment.

Adolescent↗

Electrophysiological changes during episodes of the Kleine-Levin syndrome.

Diurnal EEGs of a 17 year old male with the Kleine-Levin syndrome revealed moderate diffuse abnormalities and stage REM at sleep onset during attacks. Overnight, stages 3, 4, and REM of sleep were decreased, but sleep onset REM stage was not seen. These records returned to normal between attacks.

Adolescent↗

A menstruation-linked periodic hypersomnia. Kleine-Levin syndrome or new clinical entity?

A 13-year-old girl showed periodic episodes of somnolence without megaphagia recurring in association with each menstruation. During somnolent episodes total sleep time averaged 14 hours and 19 minutes per 24 hours. The level of performance evaluated by means of the Wilkinson Addition Test was significantly impaired. There was an abnormal increase of 5-hydroxyindolacetic acid in the cerebrospinal fluid after probenecid test, suggesting an increase of the turnover of 5-hydroxytryptamine during periodic hypersomnia. Investigation of the menstrual cycle failed to document any striking hormonal abnormality. Nevertheless, the close relationship between the episodes of hypersomnia and the end of the menstrual cycle led us to hypothesize a role of progesterone and to try a hormonal type of treatment that is thus far successful.

Adolescent↗

The more unusual sleep disturbances of childhood.

The sleep patterns of children often cause anxiety to their parents. Some disturbances are unusual, and therefore may cause diagnostic difficulties. Sleep walking and night terrors can be confused with epileptic seizures. The sudden sleep of narcolepsy can lead to false accusations, when in fact the episodes are beyond the child's control. The associated phenomena of cataplexy, hypnogogic hallucinations and sleep paralysis can be particularly alarming, especially if they occur in the absence of narcolepsy. The overlap between narcolepsy and the Kleine-Levin syndrome is confirmed. Although of a different nature the sleep apnoea syndrome is equally important from the point of view of diagnosis and treatment.

Child↗