Sleeping Beauty Syndrome, a rare form of narcolepsy, is also called Kleine–Levin Syndrome (KLS). The sleep disorder, named after Willi Kleine and Max Levin, medical researchers that investigated the properties of the syndrome, has baffled modern researchers for decades. Until recently two theories dominated: some scientists believed the syndrome is primarily driven by genetics, while other scientists suspected a failure of the autoimmune system led to the syndrome.
Lending weight to the organic hypothesis was evidence that in about half the cases of KLS patients initially experienced a brief flu-like illness.
Doctors report KLS patients are chronically tired and the sleepiness often overwhelms a person with little advance notice. Between periods of lengthy sleep (that can last days, even weeks) patients partially wake to eat or go to the bathroom. Even then the sufferers of this remarkable illness are lethargic and relatively unresponsive. They appear to be walking in their sleep.
Research into the malady has revealed most sufferers are teenage boys, although KLS has been diagnosed in both sexes and gas been detected in children as young as six and adults as old ad 59.
According to Oxford’s “Brain: The Journal of Neurology,” the first recognized case of KLS was documented in 1862 by Brierre de Boismont.
Brain published a study in 2005 [“Kleine–Levin syndrome: a systematic review of 186 cases in the literature”] that led many investigators to suspect the root cause of KLS is organic, not genetic.
The authors of the study note “most notably the possibility of a viral or post-infectious autoimmune encephalitis with primary impact on the hypothalamus. In favour of autoimmunity, a European group recently identified the human leucocyte antigen (HLA) subtype DQB1*02 as possibly being associated with the disease.”
Another study pf 108 KLS patients [“Kleine–Levin Syndrome: A Systematic Study of 108 Patients”] was not able to definitively link a virus propr to the onset of the first symptoms of the disease. Using various medicines in an effort to reduce or eliminate the symptoms had limited success.
Taiwanese study suggests KLS organic origin
The 2012 study, “Relationship between Kleine-Levin Syndrome and Upper Respiratory Infection in Taiwan,” reveals a link between viral infection and the onset of KLS.
Using “a group of well-documented KLS patients” (26 males and 4 females), the researchers followed the progression of their symptoms from a little less than two years to a maximum of six. All subject experienced multiple episodes of hypersomnia per year with some having as many as seven episodes.
Those that had suffered early upper respiratory infections (URI) were also tracked.
After analysis of the gathered data, the researchers found “The results showed a strong positive correlation between higher URI reports in the general agematched Taiwanese population and the occurrence of symptomatic episodes in KLS patients.”
Significantly the study confirmed “that most first attacks of KLS occurred after URI symptoms or fever…”
Newest study offers hope
A medical team led by David Rye at Emory University in Atlanta worked with 32 sufferers of the sleep disorder hypersomnia. The 22 females and 10 males had a formerly unknown chemical present in samples of their spinal fluid. That chemical stimulates the GABA-A receptor.
TIME Magazine reports that the GABA-A receptor “is best known as the site where sleep-inducing drugs like Valium and Xanax have their effects, since activating GABA-A receptors can result in drowsiness.”
The discovery may lead to a pathway for treatment. Pharmaceuticals like flumanezil that is used to treat Xanax and Valium overdoses can be tested to combat or overcome the symptoms of hypersomnia and KLS.
