What is Kleine-Levin Syndrome?

Kleine-Levin Syndrome (KLS) is a central disorder of hypersomnolence where patients have periods of severe sleepiness alternating with periods of normal sleep need and wakefulness. You may sleep for as long as sixteen to eighteen hours per day and this can last for a few days or for several weeks. This explains why KLS is often referred to as “Sleeping Beauty Syndrome”. Alternate names include recurrent hypersomnia or periodic hypersomnolence. Despite the prolonged bouts of sleep, you will wake up only to eat and use the bathroom. These episodes can appear weeks or months apart and occur one to ten times a year.

In addition to the severe sleepiness patients may also experience other atypical behaviors such as overeating, aggressive behavior, and other psychiatric features during these episodes. The combination of features can be very distressing and have a negative impact on quality of life. Your sleep and general behavior, however, are normal before and after the episodes.

Severe sleepiness is also a sign of other sleep disorders. It can be a sign of sleep apnea or narcolepsy. In these cases, the complaint of sleepiness is daily. It does not come and go for extended periods of time.

KLS is not caused by something else that disturbs your normal pattern of sleep. It is not a sign of a sleep disorder to sleep all day after having surgery. Maybe you slept all day after staying up all night with a sick child. This is also not an example of a sleep disorder.

What are symptoms of Kleine-Levin Syndrome?

Signs of illness often show up just before the first attack. You can have headaches and feel very tired just before a sleep attack. These headaches might last for a few hours. Patients often have a red face and sweat a lot during an episode. They can also gain a few pounds of weight. KLS has the most negative impact upon your work and social life.

Patients show odd behavior during the sleep attacks. This includes:

Unrestrained “binge” eating
Hypersexuality – excessive sexual desire and activity
Irritability
Aggression
Feelings of unreality
Confusion
Hallucinations

Menstrual-related hypersomnia is a subtype of KLS. Sleep attacks occur at the same time as the menstrual cycle. It shows up just months after the first menstrual period. Episodes tend to last one week and end very quickly. It is likely caused by a hormonal imbalance.

Who gets Kleine-Levin Syndrome?

It is a very rare sleep disorder. There are only about 200 reports of people who have had it. You might be more likely to have it if a family member has a mood disorder. It tends to first appear in the early teenage years. It has been reported in children as young as 10 years old. Males are four times as likely to have KLS as females.

How to diagnose Kleine-Levin Syndrome?

It is important to first know if there is something else that is causing your sleep problems. They may be a result of one of the following:

Another sleep disorder
A medical condition
Medication use
A mental disorder
Substance abuse

If your bouts of extreme sleepiness cannot be explained by any of the above, take the self-test below to see if you may have KLS.

Self test

Do you have sleepiness that lasts at least two days and up to three weeks at a time?

Do you sleep for up to sixteen or eighteen hours a day during these episodes of sleepiness?

Do these episodes happen at least once or twice a year?

Do you act and sleep normally in between these attacks?

If your answers to all these questions are yes, then you might have KLS, but only a sleep doctor can help make the proper diagnosis.

Diagnosis

Do I need to see a sleep doctor?

Yes. This is a very rare sleep disorder. You need someone with the proper training and experience to help treat it.

What will the doctor need to know?

You may need to complete a sleep diary for two weeks. This will give the doctor clues as to what might be causing you problems. You can also rate your sleep with a simple questionnaire. This will help show how your sleepiness is affecting your daily life. The doctor will need to know your complete medical history. Be sure to inform her of any past or present drug and medication use.

Will I need to take any tests?

Yes, two tests are typically needed. These tests will determine if you have another sleep disorder that may better explain your symptoms.

Sleep study

A sleep study is an overnight test that records a full night of sleep. It is also called polysomnography. The polysomnography charts your brain waves, heartbeat, and breathing as you sleep. It also records how your arms and legs move. This shows if there are other problems that are causing your sleepiness. Two examples of these problems are sleep apnea and periodic limb movement disorder.

Multiple Sleep Latency Test (MSLT)

This is a daytime test that measures how you tend to fall asleep in quiet situations. It shows what kind of sleep you have during the day. Your doctor may ask that you take a test to screen for drugs before you have the MSLT. There are several drugs that can affect the results of the sleep study. The drug screen will help the doctor to know what the MSLT really says about your sleep problem.

You may also take a Maintenance of Wakefulness Test. This measures your ability to stay awake in quiet situations.

How to treat Kleine-Levin Syndrome?

Case reports show that the symptoms tend to decrease on their own over several years. There has been a lack of long-term follow-up with patients. This makes it hard to know for sure when the disorder is gone. In rare cases, it may continue to show up for a period of 10 to 20 years. Medications have been used to help people stay awake and be able to work or go to school during attacks. Talk to your doctor to find out more about these medications.

Those who have menstrual-related hypersomnia have found that taking birth control pills can help keep the sleep attacks from returning.