New Treatment for ‘Sleeping Beauty’ Syndrome?

Primary hypersomnia—'Sleeping Beauty Syndrome' — causes excessive daytime sleepiness, disorientation and inattention

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Most of us have experienced it:  that dull, dragging semi-conscious state of deadened awareness and desperate urge to nap that comes from sleep deprivation.  For people with primary hypersomnia, however, this is the way they go through life, constantly feeling only half-awake but never able to get enough good sleep to arise truly refreshed.  Also known as “Sleeping Beauty Syndrome,” the condition leaves those with the worst cases languishing in bed in what seems like the opposite of a fairy tale, without a prince’s kiss to cure them.

But a new study, published in Science Translational Medicine, suggests both a possible cause and a potential treatment for the condition, which may ultimately lead to treatments for other sleep disorders.  The origin of primary hypersomnia, which has some genetic components is still unknown, as is the number of people who are affected by it.

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One particularly striking form of the disease, Kleine-Levin syndrome, produces such tiredness and sleep-drunkenness that people are unable to attend school or work.  In males, it can include hypersexual behavior, compulsive masturbation, a desire for promiscuous sex or making inappropriate sexual advances, all while in a sleepy, semi-conscious state.

In the latest study, researchers led by David Rye of Emory University in Atlanta studied 10 men and 22 women seeking treatment for primary hypersomnia.  In the patients’ spinal fluid, the scientists discovered a previously uncharacterized chemical that stimulates the GABA-A receptor.  This 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 finding suggested a possible treatment. A drug, known as flumanezil can treat Valium and Xanax overdoses or to reverse the effects of related compounds used in anesthesia. Could it block or reverse the effects of the unknown agent that was activating GABA-A receptors in primary hypersomnia?

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The authors conducted a brief placebo controlled trial with seven patients—including one with Kleine-Levin symptoms — to find out. Indeed, injections of flumanezil improved the participants’ ability to pay attention and remain alert.  One participant has now taken the drug daily for four years.  “Although her nightly sleep duration remained at 9 to 10 hours, she nearly always awakened refreshed without an alarm and daytime sleepiness was markedly reduced,” the researchers write.

Further research is needed to see whether flumanezeil or a similar drug can be an effective treatment for primary hypersomnia.  And more studies are needed to understand the chemical in these patients that is influencing the GABA-A receptors in the first place. Figuring out what it is and how it changes with normal sleep and wake cycles might also lead to the discovery of better drugs to treat not just “sleeping beauties” but abnormal sleepiness and insomnia as well.

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