Kleine-Levin Syndrome: The Facts Behind Sleeping Beauty Disease
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Kleine-Levin Syndrome: The Facts Behind Sleeping Beauty Disease

Kleine-Levin Syndrome (KLS) is a rare neurological disorder that was first described in 1786. It is characterized by extreme hypersomnolence, cognitive impairment, photophobia, phonophobia and compulsive overeating and hypersexual behavior. Risk factors for developing the syndrome include infection, alcohol abuse and brain injury. Research studies suggest it may be an autoimmune disorder that attacks the brain. A deficiency in dopamine may be responsible for some of the observed symptoms.

Kleine-Levin Syndrome  (KLS) is also known by the deceptively innocuous name, 'Sleeping Beauty Syndrome'. This is because the main presenting symptom is hypersomnia, or excessive sleeping. Patients have also reported sensitivity to light and noise, compulsive overeating, promiscuous sexual behavior and depression. KLS was first described in 1786 by a French physician. It is named for Willi Klein and Max Levin who conducted independent reviews of the scientific literature up to 1936.

Sufferers have trouble holding down jobs, while others acquire criminal records as a result of their compulsive sexual behavior. Young patients are unable to attend school or care for themselves. Most patients are bedridden. 

In 2005, a consortium of scientists in California, Massachusetts and Paris published a systematic review of 95 research articles published between 1969 and 2004 (Arnulf et al 2005). The aim was to collate information about the symptoms, risk factors and treatments. They reported:

  • The median age of onset was 15 (range 4-82)
  • KLS lasted an average of 8 years
  • The disease lasted longer in female patients than in men
  • Most commonly reported risk factors were infection (38%), head trauma (9%) and alcohol consumption (5%).
  • Stimulants were successful in reducing somnolence in 40% of cases.
  • Neuroleptics and antidepressants were of little use
  • Lithium produced a 41% response rate in controlling relapse, while carbamazepine and other anti-epileptics did not help.
  • Patients with KLS secondary to other medical conditions tended to be older with more frequent and longer-lasting episodes than those with primary KLS.

Some researchers believe KLS to be an autoimmune disorder. As well as the A, B, O blood type antigens present on the outer surface of red blood cells, there are subtypes of Human Leukocyte Antigens (HLA) on the outer surface of white blood cells. A specific type of HLA antigen (HLA DQB1*0602) has been found in a higher frequency in KLS-affected individuals than in unaffected controls (Huang et al 2012)

Symptoms may be the result of a malfunction of that part of the brain that controls appetite, sleep and temperature regulation. Others have suggested a link between KLS and a deficiency of dopamine receptors in the lower striatum (Hoexter et al 2008).

The Kleine-Levin Syndrome Foundation was formed in 1999 to assist those with KLS and to support research into its causes and cures. Their first International Conference was held in San Francisco, California, in August 2011. More than 200 patients, families and researchers met to share experiences. Those who could not attend were able to participate remotely. 

References

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Comments (4)

Great information. Thank you dear Kimberly

great recap

Today exist a lot ofdisease which people can't treat and this is a huge problem for them, even if they know how to write a resume they could not find the way of treating. 

Excellent,informative article, thank you!

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