When fairy tales go wrong: Sleeping Beauty syndrome

Crystal Wang (Waterloo)
2 May 2017

Above: Image © iStock, YuliaBuchatskaya

The princess in the classic fairy tale Sleeping Beauty was cursed to sleep for 100 years. But what happens when a fairy tale becomes reality? People with Kleine-Levin syndrome (KLS) fall into a deep sleep that can last a very long time. And while Sleeping Beauty has a happy ending, KLS is a little more complicated.

What is KLS?

KLS is a rare neurological disorder that affects about one in a million people. A KLS episode can cause someone to sleep for up to 20 hours a day. These episodes can last for a whole week, or even a month or two. That’s why the media often calls KLS “Sleeping Beauty syndrome”.

You might think that a nice long sleep sounds great. But KLS can be scary. The episodes don’t necessarily happen often, and a person with KLS can go months or years without one. But when episodes do happen, they can arrive with very little warning. A person could be fine one day, then asleep the next. And there’s no telling when they might wake up again!

For the few hours that a person is awake during a KLS episode, life isn’t exactly normal. They might be disoriented, confused or irritable. They may act childishly, or even have hallucinations. Seems like Princess Aurora had it much better in the fairy tale!

Who gets KLS?

Scientists don’t know much about this disease. However, researchers have found that KLS episodes typically start during the teenage years. They’ve also found that 70% of KLS patients are male.

You may have noticed some reports of youth suffering from KLS in the media. For example, a British teen named Louisa Ball developed KLS while recovering from the flu. She had an episode that lasted for two weeks. She woke up once a day to eat, and then fell back asleep. Because of KLS, Louisa missed out on a big chunk of her life. She slept right through holidays, school and the extracurricular activities she loved.

Did You Know? The first case of Kleine-Levin Syndrome, also called Sleeping beauty syndrome, was reported in 1862.

How do doctors treat KLS?

Sadly, because scientists haven’t found the cause of KLS, they also haven’t found a cure or a standard treatment. One thing they know is that KLS happens more often in the fall and winter. It also usually follows another sickness, like how Louisa started having episodes after she had the flu.

But medical researchers don’t know much else about KLS. And because the symptoms are so mysterious, it usually takes doctors a while to properly diagnose someone with KLS.

Often, the best way to help someone with KLS is to stay home and take care of them during an episode. But doctors sometimes treat KLS in the same way as mood disorders, like bipolar disorder and depression. People with mood disorders have imbalances in serotonin and dopamine levels, two compounds that help regulate mood and memory. And people with KLS have many of the same symptoms as people with mood disorders. This could mean that KLS sufferers have serotonin and dopamine imbalances, too. That’s why doctors sometimes treat KLS with lithium, which is also used to treat bipolar disorder.

KLS may also involve problems in the hypothalamus, a peanut-sized part of the brain. Even though it’s small, the hypothalamus plays a part in regulating a lot of daily functions like body temperature, hormones, sleep and appetite. But the hypothalamus doesn’t seem to work like it should in people with KLS.

Is there hope for people with KLS?

Yes, there is. People with KLS usually have fewer episodes as they get older. Luckily for Louisa, she was diagnosed and put on medication that helped her sleep patterns go back to normal.

There are also online support groups where people with KLS can help each other. Their families can also share their experiences and support each other as their loved ones sleep.

But unlike Sleeping Beauty, KLS isn’t something a prince or princess can cure with a kiss. Scientists continue to run studies and learn more about the disorder. Hopefully, they will find better ways of helping people with KLS stay awake.

Kleine-Levin Syndrome: Why the rare 'Sleeping Beauty' illness is no fairytale (2014)
B. Jones, BBC News

Kleine–Levin syndrome: Etiology, diagnosis, and treatment (2010)
S. Ramdurg, Annals of Indian Academy of Neurology 13

Kleine-Levin Syndrome Information Page (n.d.)
National Institute of Neurological Disorders and Stroke

Crystal Wang (Waterloo)

My name is Crystal and I am currently studying biochemistry and the University of Waterloo. I hope to also be able to specialize in biotechnology during my undergraduate studies, and then move on to studying pharmacy. My goal for the future is to be able to work at a hospital to do research on cures for different diseases and to be able to help people in general. Outside of academics, I also like to read, travel, and spend time with friends and family.