In 1925, Frankfurt Neurologist Willi Kleine published a paper he titled “Periodische Schlafsucht” (periodic sleepiness). In his paper, Kleine described 5 patients who mysteriously slept for abnormally long periods. Neither Kleine nor his colleagues could explain the cause of the sleepiness. Four years later, New York psychiatrist Max Levin, studying narcolepsy, came across a young man who both slept and ate too much. He collected five more such cases, one of whom happened to be one of Kleine’s patients. Dr. Macdonald Critchley described the strange condition in even more detail, and named it Kleine-Levin Syndrome.
Understanding Kleine-Levin Syndrome
In the days before the internet, doctors spent a lot of time in libraries. They scanned thousands of pages of old medical journals, often written in languages they did not speak. One such search turned up a case of a teenage girl who appeared to have Kleine-Levin Syndrome. This occurred in France in the late 18th century:
‘In her fourteenth year, she was overcome with a lethargic sleep which lasted several days; and it was so profound that she was believed dead. 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 … During the first four years of her disease, this poor girl had appetites as bizarre as they were dangerous, causing her to eat lime, plaster, soil, and vinegar. Thereafter, these appetites subsided, and she nourished herself indiscriminately with all sorts of aliment, excepting bread, for which she maintained an insuperable loathing till she was perfectly cured. This food always occasioned vomiting.’
Over time, investigators gathered more cases. They were then able to sketch the strange disease in more detail.
- Over 70% of the victims were teenage boys.
- Patients would sleep up to 22 hours per day.
- Episodes of sleepiness would last about 10 days, and would recur every 3-4 months.
- Odd compulsive eating behaviors are common, as is bizarre sexual behavior.
- The illness would last about 8 years and then vanish as mysteriously as it appeared.
- Over half the cases follow a viral infection
In the 20th century, though most of the sufferers of Kleine-Levin Syndrome were adolescent boys, the disease began to be called “Sleeping Beauty Syndrome”. The real illness has nothing of a fairy tale quality to it, however.
The Kleine-Levin Syndrome Foundation gives an unusual description of the effect the disease has on the minds of its victims. In addition to the sleepiness and excessive hunger, KLSF describes sufferers as having “a reduced understanding of the world.”
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A Reduced Understanding of the World
It’s a description that’s both frightening and arresting. What does KLSF mean by “a reduced understanding of the world”?
During episodes, these young people display confusion and apathy. They appear withdrawn (in the few hours per day they were awake). Then there is the bizarre hunger and hyper-sexuality.
All we can say about the cause of Kleine-Levin Syndrome is that it appears to be a brain disease. It may have a viral trigger. KLS may represent an autoimmune disease. Because it is so rare (1 case per 1 million population), KLS is difficult to study.
There is no specific treatment for KLS. The best doctors and the victim’s family can do is support them. There is some suggestion that the drug Lithium can reduce the number of episodes. With time, the symptoms disappear and the young person resumes his life. He’s lost a huge chunk of his young life, but he emerges without apparent long-term consequences.
As frightening and as devastating as KLS is for the sufferer and his family, the disease resolves and never comes back. This is an important difference between KLS and Narcolepsy, which can last a lifetime.