Kleine–Levin Syndrome



Figs. 28.1
a. Kumbhakarna asleep. b. The awakening of Kumbhakarna : the demons try to rouse the giant by hitting him with weapons and clubs and shouting in his ear. A miniature painting by the artist Sahib Din, from an illustrated manuscript of the Sanskrit epic Ramayana, prepared between 1649 and 1653 for Maharana Jagat Singh of Udaipur (Western India), AD 1652. 1977, The British Library Board



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Fig. 28.2
A relation of an extraordinary sleepy person, at Tinsburg, near Bath (UK) by Dr William Oliver, F.R.S. Philosophical Transactions, 1704–1705, vol 24.




May the 13th, Anno 1694, one Samuel Chilton, of Tinsburg near Bath, a Labourer, about 25 years of age, of a robust habit of Body, not fat, but fleshy, and a dark brown Hair, happen’d, without any visible cause, or evident sign, to fall into a very profound Sleep, out of which no Art used by those that were near him, cou’d rouze him, till after a month time; then rose of himself, put on his Cloaths, and went about his business of Husbandry as usual; slept, cou’d eat and drink as before, but spoke not one word till about a month after. All the time he slept Victuals stood by him; his Mother fearing he would be starv’d, in that sullen humour, as she thought it, put Bread and Cheese and Small Beer before him, which was spent every day, and supposed by him, tho no one ever saw him eat or drink all that time.

From this time he remained free of any drowsiness or sleepiness till about the 9th of April 1696, and then fell into his Sleeping sit again just as he did before. After some days they were prevail’d with to try what effect Medicines might have on him and accordingly one Mr Gibs, a very able Apothecary of Bath, went to him, Bled, Blister’d, Capp’d and Scarrified him, and used all the external irritating Medicines he could think on, but all to no purpose, nothing of all these making any manner of impression on him; and after the first fortnight he was never observed to open his Eyes. Victuals stood by him.

The second one is by a French physician who studied and graduated in Montpellier, France, Edmé Pierre Chauvot de Beauchêne (1786) [10].

„A girl, in her fourteenth year, was overcome with a lethargic sleep which lasted several days. From that point forward, the affection of sleep recurred at irregular intervals; it usually lasted eight to ten days, continuing at times for fifteen; and upon one sole occasion, it persisted into the seventeenth day.“

There was no typical overeating but “during the first four years of her disease this poor girl had appetite as bizarre as they were dangerous, causing her to eat lime, plaster, soil and vinegar. Thereafter, these appetites subsided, and she nourished herself indiscriminate with all sorts of aliment. This food always occasioned vomiting” (Fig. 28.3).



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Fig. 28.3
Flyleaf of the article by Edmé Pierre Chauvot de Beauchêne

During the nineteenth century more cases were published. In a report read at the Royal College of Physicians of London in April 1815, and published later the same year in Medical Transitions,



“Richard Patrick Satterly described the case of a 16 year old boy who on his return from school was observed to be pale and unwell. He felt cold and complained of a frontal headache. During the following three days, his symptoms appeared to be abating; then the headache worsened, he became flushed, restless and agitated, and his pulse rate was raised. About the seventh day, the patient developed a voracious appetite…he would eat a pound-and-a-half of beef steaks, a large fowl, or a couple of rabbits, at one meal without apparently satisfying his appetite”….“The craving for food came on regularly with the paroxysms of fever, and continued unabated until that subsided, when he usually fell into a sound sleep. The period of the recurrence of the paroxysm was very uncertain, but it was marked by a distinct circumscribed redness of one or both cheeks; the moment this spot became visible, the boy would rouse himself (for he was at other times either sleeping, or dull and torpid)” [11].

Later on, in 1862, Brière de Boismont reported the case of



a child who, in a recurrent manner, slept a lot, was difficult to arouse, and as soon as he was awakened extemporaneously sang, recited, and acted with great ardor and aplomb. When he was not asleep, he ate ravenously. As soon as he got out of his bed, he would go close to another patient’s bed, and overtly seize without any scruple all the food he could find. Apart from this intriguing disease, he was intelligent and skilful [12].

From 1862 onwards, reports became more frequent. Mendel described the case of a soldier, aged 25, who had attacks of 3–5 days’ duration at a frequency of 1–2 years. There was no mention whether the patient showed excessive appetite [13]. The same applies to an adolescent girl who had her first attack while sitting in church, went home, and did not wake up for 3 days [14]. Anfimoff, a Russian physician, reported a youth of 19 years who slept deeply during the first 2 or 3 days of his attacks and then woke up frequently and went to sleep again [15]. His appetite was excellent. In dream, he saw a horse and was scared and afraid of everything. These attacks reappeared every few months.

Later on, case reports concentrated in Germany. Stöcker reported on a 21-year-old man who had experienced headache and frequent epistaxis since childhood, and who, from the ages 19 to 22 years, had recurrent attacks of sleepiness and indifference [16]. Schröder commented on a 17-year-old adolescent who periodically, at intervals of 3 months’ duration, had prolonged sleep episodes associated with transient psychological changes and indecent behavior [17]. Such episodes stopped after 3 years. Krüger reported two cases [18]. The first one was in a 44-year-old single woman with recurrent episodes of abnormal sleep at the ages 16, 19, 34, 43, and 44. She did not take any food during the second one and ate normally during the following ones. The second case was a 20-year-old man who had his first attack of sleep during his military service after a grueling day followed by three more attacks lasting for 2–5 days each time at 6–18-month intervals.

Finally, a last case report was by a Russian physician, J.W. Kanabich [19]. The patient was a 19-year-old man who had two attacks of sleepiness accompanied by restlessness, nervousness, and apathy at the age of 14 and 14.5 years.

Thus, from 1786 to 1924, 12 cases of recurrent hypersomnia were published, with an acceleration of the publications in the second half of the nineteenth century. There were nine male and three female cases. The median age of onset was 17 with a range of 12–25. Circumstances at onset included overwork in three cases [16, 18], struggle with a comrade [13], strong emotion [15], sitting in church [14], cold and fever [11], and seasickness [16] in one case each. The duration of the episodes was from 2 days to 12 weeks and the interval between episodes varied from 1 to 2 months to 15 years in one case [18]. Hypersomnia was present in all cases, overeating in four [1012,15], disinhibited sexuality in one [17], odd behavior, childish, singing, and incoherent utterance in one [10], singing, recitation, and acting with great ardor and aplomb in one [12], agitation in one [11], psychological changes (dull, indifferent, inertia of thought, visual and auditory hallucinations, apathy) in five [1519], mental symptom (anxiety) in one [15], dysautonomic signs in two [11, 18], and poor sleep for several nights on recovery in three [1618]. In total, almost all the symptoms and signs later described in KLS were present in a dissociated way.



Kleine, Lewis, and Levin’s Period (1925–1939)


In 1925, Willi Kleine, then a young psychiatrist working at Kleist’s clinic in Frankfurt, reported on five examples of episodic somnolence, two of which being remarkable for a wealth of symptoms: Hypersomnia, overeating, disinhibited sexuality, odd behavior (in one case), irritability, cognitive disorder, and mental symptom (in one case) [3].

In 1926, Nolan Lewis, a psychiatrist in Washington, introduced a psychoanalytic approach to the problem of four children under 12 years of age, one of whom, aged 10, presenting with attacks of sleepiness, gluttony, odd behavior, irritability, and cognitive symptoms in the form of visual hallucinations [4].

Next, in 1929, Max Levin, a psychiatrist in Baltimore, described a young man of 19 years with recurrent episodes of hypersomnia, polyphagia, odd behavior, restlessness, irritability, and cognitive symptoms (dull, taciturn) since the age of 16 [5].

Thus, within 4 years, three different authors, all psychiatrists, one from Germany and two from the USA, described patients, all boys or young male adults, presenting with recurrent episodes of severe sleepiness lasting some days associated with hyperphagia, odd behavior, cognitive symptoms, and in one patient disinhibited sexuality.

After a further report by Daniels of a young man aged 18, with four episodes [20] and two reports by Kaplinsky and Schulmann, one in a 14-year-old boy who had attacks lasting for 14–20 days and one in a youth aged 20 years who had two attacks [21], Levin, in 1936, called attention to “a syndrome characterized by recurring periods of somnolence and morbid hunger” and starting from seven “good cases” of this syndrome previously reported in literature [35,19, 20], gave the very first comprehensive description of the syndrome:



There are attacks of sleepiness lasting from several days to several weeks with the longest recorded being three months. During the attack the patient sleeps excessively day and night, in extreme instances waking only to eat and go to the toilet. He can always be roused. When roused he usually is irritable and wants to be alone so that he can go back to sleep. He is abnormally hungry and eats excessively. These attacks are separated by intervals of normal health. Besides the two main symptoms, somnolence and hunger, there are incidental symptoms….(excitement, irritability, difficulty in thinking, forgetfulness, incoherent speech and hallucinations), insomnia at the close of an attack, male sex with a single exception, age of onset in the second decade, onset soon after an acute illness and spontaneous cure in some cases. [6]


Critchley’s Period (1940–2004)


Hylkema reported a boy aged 15 who, 2 months after a severe bout of influenza, began to develop recurrent episodes of sleepiness three to twelve times yearly, associated with excessive hunger and drinking, profuse sweating, whistling, and singing to himself [22]. This was followed by the reports of periodic somnolence and morbid hunger in two men, ages 20 and 25, by Critchley and Hoffman (1942) who coined the term KLS, ignoring Lewis’s name [1].

In December 1962, Critchley published his milestone article “Periodic hypersomnia and megaphagia in adolescent males” in which he collected 15 “genuine” instances from the literature and 11 cases of his own, gave a comprehensive description of each and defined—



“a syndrome composed of recurrent episodes of undue sleepiness, lasting some days, associated with an inordinate intake of food, and often with abnormal behaviour” [7].

In addition he emphasized four hallmark features:

Mar 18, 2017 | Posted by in PSYCHIATRY | Comments Off on Kleine–Levin Syndrome

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