Kleine-Levin Syndrome is a rare sleep disorder, with the main feature being that people spend exceptionally long periods of time asleep.
They often only wake to eat or use the bathroom, and even when awake aren’t really alert. It’s sometimes called sleeping beauty syndrome, but it’s unlikely that those who have it would see it so romantically.
If you, or someone you know, are one of the very few people who have Kleine-Levin Syndrome, then you may well be frustrated by the lack of information about it.
This article may not answer all of your questions, but hopefully it will give you a solid overview, and some useful information you may not have heard before.
You’ll find out exactly what Kleine-Levin Syndrome is, how rare it is and what the symptoms are. I’ll also cover the limited theories of possible causes, as well as treatment options.
How long do people with Kleine-Levin sleep for?
During an episode, people with Kleine-Levin Syndrome might sleep for up to 20 hours every day. These sleep episodes can last for a few days, weeks or even months.
There may be days, weeks, months or even years between each episode. And people may suffer from the disorder for many years, perhaps as many as 10-12 years.
In medical terms it may also be seen as a case of recurrent hypersomnia. Although in simple terms, hypersomnia is more about feeling tired, sleepy and falling asleep during the day.
People who have hypersomnia may sleep for many hours at night, and possibly have long naps during the day. They may also find themselves falling asleep at various points during the day. Those with Kleine-Levin Syndrome, however, will stay asleep virtually all day.
How many people have it?
It’s estimated that about 1 person in a million suffers from Kleine-Levin Syndrome. This could mean just a few hundred people in the USA, for example.
It’s more common in men (70%-90% of cases). It more commonly starts in adolescence, although it can occur at any time in your life. There may also be a genetic aspect, as there appears to be a higher rate of Jewish boys who suffer from the condition.
It’s so rare that it’s unlikely your doctor will have come across it before. Judging by some individual stories, this can result in either misdiagnosis or a frustrating process of establishing the correct diagnosis.
As it often starts during teenage years, an initial impression may be formed that it might just be normal teenager behavior. But if you look closely at teenager sleep patterns, you’ll find that this syndrome isn’t at all normal for teenagers.
The other challenge is the lack of large-scale research into the disorder. More common sleep disorders can have medical trials based on hundreds or even thousands of people with the problem.
For Kleine-Levin Syndrome, however, most of the limited research is based on either individual case studies or small scale programs. And unsurprisingly, nearly every research paper ends by saying further research or larger scale trials are needed before definitive conclusions can be reached.
The primary symptom is the need to sleep for 16 to 18 hours every day, over extended periods lasting for days or weeks.
According to the international classification of sleep disorders published in 2014, a typical episode lasts for 10 days on average. Furthermore, these sleep episodes aren’t isolated events, but usually reoccur at least once a year.
They generally start without any warning, although in some cases you might experience flu like symptoms at the onset.
Other common symptoms include:
- Excessive appetite and eating. You may feel an overwhelming urge to eat anything available, often resulting in weight gain.
- Disorientation. People report that they feel vague, dream-like, confused or disorientated when awake. You may also suffer from audio or visual hallucinations. Parents, friends, colleagues and caregivers may find your behavior childish, immature or confusing. They may also think you show signs of irritability, lethargy and apathy.
- Abnormal speech. Your speech may be affected, resulting in slurred or child-like speech.
- Hyper-sexuality. You may find that you have an abnormally high and uninhibited sexual drive during an episode.
- Mood changes. You may feel depressed, irritable or moody during and between episodes. It’s believed that this is a result of the disorder and not a cause.
- Amnesia. You may not be able to remember everything that you do during one of the sleep episodes.
Early thinking was that most of these symptoms were experienced by people with Kleine-Levin Syndrome and this may have influenced medical diagnosis.
However, more recent studies suggest that these other symptoms may not be present in all cases. For example, one small survey found that 75% of people may experience excessive eating and only about 50% the hyper-sexuality symptom.
There are very few theories about the likely causes of Kleine-Levin Syndrome. One theory, which has some expert support, is that it might be caused by a malfunction of the hypothalamus and/or thalamus parts of the brain.
These areas govern appetite and sleep, which matches the sleep problem and the related symptom of excessive eating. But as mentioned earlier, the rarity of the disorder means there’s limited research being carried out to try and truly identify the causes.
A 2004 study reviewing previous research reported that 89% of patients remembered an illness or activity which happened just prior to suffering the first episode. This included the events listed below.
The figures in brackets are the percentage of those surveyed who reported each of these events, with some people recalling more than one event.
- Infections (72%, with 25% reporting having flu or cold-like symptoms, including a fever).
- Alcohol use (23%)
- Sleep deprivation (22%)
- Unusual stress (20%)
- Physical exertion (19%)
- Traveling (10%)
- Head injury (9%)
- Marijuana use (6%)
There appears to be wider support to the theory that some form of infection before the first occurrence of a sleep episode is one of the most likely causes.
The difficulty of getting a diagnosis
Kleine-Levin Syndrome is a difficult sleep disorder to diagnose. It’s usually the case that other possible explanations for your symptoms are first excluded, and then the diagnosis may be reached.
Your doctor needs to carefully consider your case history and symptoms to eliminate other medical conditions and sleep disorders. For example, narcolepsy, general hypersomnia, epilepsy, mental disorders such as bipolar disorder and depression, and drug or alcohol use.
For this reason most diagnoses of Kleine-Levin Syndrome will only really be confirmed after a second episode of prolonged sleep. And frustratingly, it can take a long time to receive a diagnosis.
Some research has been undertaken to see if other modern techniques, such as MRI scans, can help diagnoses. But even these haven’t proven conclusive.
For example, a Swedish study used single photon emission computed tomography to look at the brains of 24 people with Kleine-Levin Syndrome.
Although 48% showed anomalies, the others didn’t. So the researchers couldn’t recommend this as a definite method for diagnosing the condition, though it might still provide another clue for doctors.
Furthermore, people with this disorder can appear completely normal between episodes, making a diagnosis even harder to come to.
Unfortunately, there don’t really appear to be any effective treatments for Kleine-Levin Syndrome. Moreover, any suggested treatments are only supported by very limited trials. Two possibilities are:
A trial involving just three people suggested that taking Lithium as a medication may reduce the length and frequency of the sleep episodes. It can also reduce significantly the other behavioral symptoms.
However, it doesn’t seem to prevent the occurrence of the sleep episodes, so it’s not a cure. The researchers concluded:
…in KLS with a high frequency of episodes and severe behavioral changes lithium may become a treatment option.”
Stimulant pills, including amphetamines, methylphenidate and modafinil, are sometimes prescribed to counteract the sleepiness. However, they may increase irritability and won’t improve the disorientation people might feel. Furthermore, they don’t seem to have any effect on the underlying sleep problem.
In general, your doctor may advise that you don’t need to be cared for in hospital and home care is the best option. Parents, friends and caregivers may need to support and encourage you during the prolonged sleep episodes.
It’s recommended that your support network should also encourage you to maintain good sleep hygiene. In particular, encouraging you to try and maintain stable sleep patterns – as much as is possible considering the nature of the sleep disorder.
They may also need to ensure you’re not at risk by undertaking dangerous activities such as driving when awake.
Waking someone up
It may be possible to wake people up during a sleep episode. However, they are still likely to suffer the confused, irritable, childlike and vague symptoms mentioned above.
Even when awake, they may not be able to do much except the essentials of eating and using the bathroom. Certainly working or going to college wouldn’t seem possible.
Kleine-Levin Syndrome is an exceptionally rare sleep disorder. For example, it probably only affects a few hundred people in the whole of the United States.
The main symptom is sleeping for long periods of up to 20 hours a day, with repeat episodes of the sleep periods. Your life is effectively put on hold during the prolonged sleep episodes, as you’re unlikely to be able to work, socialize or study.
There are a range of other symptoms that you may experience, including overeating, childlike behavior and disorientation. It may also have a significant impact on your mood and general well-being.
The causes haven’t really been established, though some form of infection before suffering the first sleep episode does appear to be fairly common.
There are no known cures. In very limited trials the use of Lithium as a medication does seem to help reduce the frequency and severity of the symptoms, but it’s not a cure.
Being cared for at home is normally the recommended approach.
Your view and experience
Do you, or anyone you know, suffer from Kleine-Levin? How does it affect your life, and is there anything which helps you manage the sleep disorder and its impact on your life?
Feel free to leave a comment below to share your story and views.