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Kleine-Levin Syndrome Kleine in 1925 and Levin in 1936 described an episodic disorder characterized by somnolence and overeating For days or weeks, the patients, mostly adolescent boys, sleep 18 h or more a day, awakening only long enough to eat and attend to toilet needs They appeared dull, often confused, and restless and were sometimes troubled by hallucinations In the series of 18 cases collected by Critchley, the age range was from puberty to 45 years There may be a brief prodromal period of inertia and drowsiness The duration of nocturnal sleep may be greatly prolonged, or, as in our patients referred to below, they may sleep for days on end Food intake during and around the period of hypersomnia may exceed three times the normal (bulimia) and occurs almost compulsively during brief periods of semiwakefulness; to a variable extent, there are other behavioral changes such as social withdrawal, negativism, slowness of thinking, incoherence, inattentiveness, and disturbances of memory The somnolence has been well studied by modern laboratory methods; except for the total duration of sleep, the individual components of the NREM and REM cycles are normal Between episodes these patients are behaviorally and cognitively normal The basis of this condition has never been elucidated A psychogenic mechanism has been proposed, but without foundation in our opinion The condition usually disappears during adulthood, and there is limited pathologic material (see page 488) We have cared for a sibling pair who had the illness into young adulthood (Katz and Ropper) The hyperphagia has suggested a hypothalamic disorder, but anatomic veri cation is lacking The case reported by Carpenter and coworkers, in which an acute and chronic in ammation in the medial thalamus but not the hypothalamus was found, must be questioned as representative of the idiopathic adolescent condition Their patient was a man 39 years of age who had episodes of diurnal drowsiness, hyperphagia (intermittently relieved by methylphenidate), and hypersexuality over a period of months In some patients with this disorder, schizophrenic and sociopathic symptoms have been recorded between attacks, raising doubt as to whether all the reported cases are of the same type In most instances, the disease is self-limited and disappears by early adult life, as mentioned We have seen variants of this syndrome manifesting themselves in drowsiness and extreme inactivity lasting for a few weeks, then with a complete return to normalcy In two of our patients, the use of serotonergic antidepressants lengthened the interval between episodes No consistent change in the level of hypocretin (orexin) has been found in the spinal uid, as occurs in narcolepsy (see further on), and the two disorders are distinct In one typical case there was pronounced hypoperfusion of the left medial temporal lobe both during and between attacks, but the intepretation of this nding is unclear (Portilla et al) No treatment has been consistently effective (eg, antidepressant drugs), but some of the stimulants that are used for the treatment of narcolepsy may be useful (see further on) Finally, it should be mentioned that sleep laboratories now recognize a form of idiopathic hypersomnia in which there are repeated episodes of drowsiness throughout the day This condition is discussed further on, in relation to the diagnosis of narcolepsy, with which it is most often confused. java qr code generator maven Tested: Java midlet QR code readers - James Royal-Lawson
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