- CASE REPORT
- Sleeping Beauty Syndrome Is a Real Thing
- What is Kleine-Levin Syndrome (aka Sleeping Beauty Syndrome)?
- What is KLS?
- Defining KLS
- Identifying KLS
- Do I have KLS?
- Kleine-Levin Syndrome
- What is Kleine-Levin Syndrome?
- What causes Kleine-Levin Syndrome?
- Additional Kleine-Levin Resources
Mrs. X, a 43-year-old married female, homemaker, educated up to 8th standard, presented in our outpatient psychiatric clinic in November 2014 with hypersomnolence, body aches, fatigue and off and on low mood. According to her husband, “she was feeling drawn toward her bed,” or “reluctant to get up in the morning.” Although patient remained arousable, patient was irritable and aggressive whenever prevented from sleep. The need for sleep was so intense that once she was found sleeping on the floor near the bathroom. She used to sleep 20–22 h with an irresistible urge for sleep. During awake state, she would appear apathetic, sad and having minimal interaction with others. Her speech would remain slurred with low in tone and volume or sometimes even not understandable. During the episode, her daily activities, household chores, food habits, personal care, and interpersonal relationship would disturb significantly, and she used to demand spicy food such as kachori, namkeen, and specific vegetables, unlike her premorbid self.
On detailed evaluation of illness characteristics and course, they reported that at the age of 27 years, after the birth of her daughter (2nd child) in 1998, for the first time, she started sleeping for 20–22 h with significant disturbance in her overall functioning, activities of daily living, and interpersonal relationship. The total duration of her illness was 16 years, with episodic course of similar presentation as mentioned above. Duration of each episode was from 2 to 15 days, with complete remission for 15 days to 4 months in between the episodes. Her general, systemic and neurological examinations were unremarkable. Her routine biochemical and endocrine parameters were within normal range. Electrocardiogram, electroencephalography (EEG), magnetic resonance imaging (MRI) brain and magnetic resonance angiography were found to be normal. The patient did not cooperate for polysomnography.
In November 2014, during her first consultation at outpatient psychiatric clinic, diagnosis of KLS was made as per diagnostic criteria of International Classification of Sleep Disorders after ruling out other possible causes of hypersomnias such as idiopathic hypersomnia (IH), menstrual-related hypersomnia, narcolepsy, Kluver–Bucey syndrome, hypersomnia due to medical condition, hypersomnia due to drug or substance.
As in differential diagnoses- IH is another condition characterized by onset during adolescence, with increased daytime sleepiness, sleep drunkenness. Episodic course and presence of other features along with hypersomnia ruled out IH in the index case. Menstrual-related hypersomnia was excluded as our patient did not have the episodes in relation to menstrual cycle and the episodes lasted longer than a week. Narcolepsy was excluded because other obligatory criteria-cataplexy, hypnagogic or hypnopompic hallucinations, and sleep paralysis were absent in index case. Kluver–Bucy syndrome was ruled out because of the absence of hyperorality and visual agnosia. Brain and hypothalamic structural abnormality was ruled out by normal MRI brain.
It is noteworthy to mention the diagnostic delay of 16 years, as earlier she was labeled as depression or conversion disorder at other centers, as patient presented with low mood, body aches, fatigue, hypersomnia. Atypical depression may also present with hypersomnia, hyperphagia. As disease mimics and shares psychiatric conditions in many ways, so there was a delay in the diagnosing KLS. Periodicity and poor response to medications (antidepressants, antipsychotics, and anxiolytics) raised suspicion on the previous diagnoses at our first consultation.
Initially, she had good response with tablet lithium carbonate 400 mg twice a day during her follow-up at our center, but due to recurrent hypersomnia, dose of lithium carbonate was gradually hiked to 900 mg and later 1200 mg (serum lithium level were 0.61 and 0.73 mmol/L, respectively). She had significant improvement but developed fine tremors and weakness hence lithium was reduced to 900 mg/day. However, due to reemergence of hypersomnia, we added tablet modafinil 100 mg. The patient reported improvement in duration and frequency of hypersomnolence episodes. For fatigue, reduced interaction, and off and on low mood, tablet sertraline 100 mg was added later. At present, she has been maintaining well for last 12 months, except 5–6 shorter periods of hypersomnia for 12–24 h.
Sleeping Beauty Syndrome Is a Real Thing
Taking a super-long nap Sleeping-Beauty-style might sound sort of appealing if you’re feeling overwhelmed by everything going on in the world right now. But for a 22-year-old woman named Beth Goodier (shown above), crazy-long bouts of sleep are anything but a fairy tale. Goodier fell asleep in November five years ago and didn’t fully wake up for six months, reports the Daily Mail. It was discovered that Goodier had something called Kleine–Levin Syndrome (aka Sleeping Beauty Syndrome), an incredibly rare sleep disorder that causes excessive sleepiness accompanied by altered behavior upon waking, according to the KLS Foundation. (Did you know that snoring is actually a big deal? It could mean you have a sleep disorder.)
To be clear, Beth didn’t sleep for six months straight without any breaks, but was unable to function normally as a result of her need to sleep and was forced to drop out of school because of the illness. “Patients with KLS are tough to awaken,” explains Josna Adusumilli, M.D., neurologist and sleep disorders physician at Massachusetts General Hospital. “The need for sleep is so strong that they can be found sleeping in unusual places, such as the hallway outside a classroom or on the sidewalk. They wake up spontaneously to eat and use the restroom. After waking up from an episode, they can be irritable, aggressive, and confused,” she says. For some, their behavior can take on child-like quality, while others experience things like hypersexuality and excessive food cravings. In other words, this is a disorder of extremes, which is particularly troubling because the onset is generally during the adolescent years, although it can affect children and adults too.
But don’t worry. “It’s incredibly rare,” says Thomas Roth, Ph.D., sleep research expert at Henry Ford Health System. “We don’t know the origins of KLS and the treatment is basically symptomatic,” he adds. Because so few people have this disorder, it’s incredibly hard to figure out what causes it or to do meaningful research on the best treatments. Essentially, the only way to treat KLS is to try to cope with the symptoms, which Roth says is sometimes done with the use of stimulants in order to keep patients awake during the day. (Here, we bust 12 common sleep myths.)
If all of this is freaking you out, don’t get too nervous. Roth says that he’s been in sleep medicine since 1975 and has only seen one case in his years of practice-and he sees thousands of patients per year. It’s also worth noting that for many people, the disorder goes away over time, so it’s not a permanent condition. In any case, we’ll never think of Sleeping Beauty the same way again.
What is Kleine-Levin Syndrome (aka Sleeping Beauty Syndrome)?
In some cases, stimulates, such as amphetamines, may provide temporary relief in reducing the need for excessive sleep in individuals with Kleine-Levin syndrome. In other cases, an anticonvulsant drug used to treat epilepsy, called phenytoin, may be an effective treatment. Still, another drug, lithium, which is used to treat mood disorders, has been an effective treatment. . Carbamazepine and lithium may also be given to help prevent further episodes since there are similarities in certain mood disorders and Kleine-Levin Syndrome. Other types of possible treatments might include modafinil, methylphenidate and other stimulant pills to treat sleepiness.
That said, more studies are needed to determine the long-term effectiveness of these drugs for the treatment of this sleeping beauty syndrome.
There is usually a decrease in episodes and their intensity and frequency over a period of eight to 12 years.
If you or a loved one wants to learn more about this sleep disorder, please call us at 425-279-7151 or click the botton below to request a free phone consultaion today.
What is KLS?
Kleine-Levin Syndrome (KLS) is a rare and complex neurological disorder characterized by recurring periods of excessive amounts of sleep, altered behavior, and a reduced understanding of the world. The disorder strikes adolescents primarily but can occur in younger children and adults. At the onset of an episode the patient becomes progressively drowsy and sleeps for most of the day and night (hypersomnolence), sometimes waking only to eat or go to the bathroom. Each episode lasts days, weeks or months during which time all normal daily activities stop. Individuals are not able to care for themselves or attend school and work. In between episodes, those with KLS appear to be in perfect health with no evidence of behavioral or physical dysfunction. KLS episodes may continue for 10 years or more. KLS is sometimes referred to in the media as “Sleeping Beauty” syndrome.
In addition to excessive sleep, a Kleine-Levin Syndrome (KLS) patient’s whole demeanor is changed, often appearing “spacey” or childlike. When awake the patient experiences confusion, disorientation, complete lack of energy (lethargy), and lack of emotions (apathy). Most patients report that everything seems out of focus, and that they are hypersensitive to noise and light. In some cases, food cravings (compulsive hyperphagia) are exhibited. Instances of uninhibited hyper-sexuality during an episode have also been reported.
Kleine-Levin Syndrome (KLS) episodes are cyclical. When present, KLS symptoms persist for days, weeks or even months, during which time all normal daily activities stop. Individuals are not able to attend school, work or care for themselves. Most are bedridden, tired and uncommunicative even when awake. Not everyone affected by KLS exhibits all of the symptoms described above.
Affected individuals may go for a period of weeks, months or even years without experiencing any symptoms, and then symptoms reappear with little warning. In between episodes, those diagnosed with KLS appear to be in perfect health with no evidence of behavioral or physical dysfunction. However they function daily with the frightful reality that they could become sick again at any moment. KLS episodes may continue to reoccur for a decade or longer with devastating effects on the adolescent’s life and family. KLS robs children and young adults of big pieces of their lives, one agonizing episode at a time.
The mean diagnostic delay for proper Kleine-Levin Syndrome (KLS) diagnosis is four years. This means that it takes the average KLS patient four years before receiving an accurate diagnosis causing undue suffering to patients and families. The cause of Kleine-Levin Syndrome is not known.
Do I have KLS?
The following KLS definition was developed by the International Classification of Sleep Disorders (2005).
While the KLS Symptom Checker below captures the symptoms experienced by a vast majority of KLS cases, there is no medical test to to confirm Kleine-Levin Syndrome (KLS). Like with any medical condition, there are atypical cases that do not fall within the classic definition. The length of episodes and period of time between episodes in some cases are known to be outside of the periods noted in this checklist. If you think you might have KLS, please consult your doctor. A list of doctors familiar with KLS can be found here.
KLS Symptom Checker
A KLS patient would have symptom A, one or more of the B symptoms, and the pattern described in C.
A. Recurrent episodes of severe hypersomnia (2-31 days) B. Plus one or more of the associated features :
- Cognitive abnormalities such as feeling of unreality, confusion, hallucinations.
- Abnormal behavior such as irritability, aggression, odd behavior
- Binge eating
C. Interspersed with long periods of normal sleep, cognition, behavior, mood
Disclaimer: This is for informational purposes and is not intended to replace a diagnosis by a trained medical professional.
Lucia Brusetti Photography / Getty Images
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.
MORE: Losing Sleep Leads To Gains in Weight
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?
MORE: Why Lack of Sleep May Affect Vaccine Effectiveness
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.
The Big Idea: Sleeping beauty syndrome
Kleine Levin syndrome is often called sleeping beauty syndrome. But it is anything but beautiful.
NEW YORK – Dani Farber had a completely normal childhood.
“Loved to play sports, did very well in school, hung with my friends,” he said.
But he remembers when things suddenly changed.
“One day when I was 15, I woke up suddenly and I was sick.” Farber, now 40, said. “I was bedridden for weeks and, on some occasions, for months on end. When I was in these episodes, my life completely stopped.”
Farber said he would sleep around the clock, 22 to 23 hours a day. When he was awake, he had no energy to get up for food or water. He said he didn’t even look like himself.
That first episode lasted a week and a half but was soon followed by a second one and then another.
“It was terrifying,” Farber said.
Most of all because no one could figure out what was causing the sleeping spells. He underwent blood tests, spinal taps, MRIs and sleep studies, but everything came back normal. Frustration built.
“It was very obvious to me and my family that something very scary and something very serious was going on,” he said.
Finally, a doctor in the Boston area, where Farber lived, came up with a diagnosis: Kleine Levin syndrome. In the media, it’s often called “sleeping beauty syndrome.”
“It’s a true misnomer. There’s nothing beautiful about KLS,” Jennifer Grossman said. “It’s a beast, it’s a beast of a disease.”
A beast that Grossman’s family is currently facing down. Her 17-year-old son Cooper has a story much like Farber’s.
“He’s an amazing young, kind, polite, happy, healthy, energetic kid,” she said of Cooper.
But at 15, during his freshman year, his first episode struck.
“Lethargy turned into almost like a coma, his pupils were extremely dilated, his skin color became very pale, it was impossible for him to stay awake,” Grossman recalled.
According to the KLS Foundation, on which both Grossman and Farber are board members, it takes an average of four years for someone to get a diagnosis of Kleine-Levin Syndrome.
“It was incredibly frustrating,” Grossman said of the road to diagnosis.
“Every person that we met with basically said, there’s nothing wrong, and one doctor told me to pray, that was basically what she said, to pray for my child.” After countless doctors and tests, and still no answers, a family member’s Google search turned up articles on KLS.
The Grossmans, who live in Livingston, New Jersey, booked the first appointment they could get with Dr. Orrin Devinsky.
“This is very much a medical mystery,” Devinsky said at his office at NYU, where he is a professor of neurology and epilepsy.
Devnisky estimates he has treated as many as 75 KLS patients over the course of his career.
“To date, we really don’t know why it afflicts one person and not another,” he said.
“During an episode, essentially the sleep system is turned on and the wakefulness is turned off, and those are regulated and very deep, primitive ancient areas of our brain,” Devinsky added. “We think it’s some abnormality in that ancient part of the brain, possibly an auto-immune, possibly virus, possibly neurochemical, the exact cause and mechanisms are still something we are working on.”
Many patients have a flu-like virus, or infection before the onset of KLS, though Devinsky said that’s not necessarily a cause.
Doctors at Stanford are looking at whether KLS may have genetic tendencies. One reason for that is Dani Farber, because just two years after his diagnosis, his younger sister Ariel began having episodes too.
“Collectively, my sister and I had 40 episodes over a 10 year period,” he said. “It robbed both of us of large chunks of our teenage years which are some pretty transformative years, and it took a big toll on our families.”
The Farbers were the first documented siblings to have KLS, but the list has since grown.
“I do think genetic factors contribute,” Devinsky said. “But they’re not the major ones that seem to be driving it.”
KLS affects just 1 to 2 in 1 million people.
Because of its rarity, research and funding have lagged, though that’s something Grossman and the KLS foundation are fighting hard to change.
“Right now, there’s no known cause or cure,” Grossman said. “However there will be one, and I know there’s one out there.”
Cooper has now had six episodes over three years, missing about 70 days of high school, at a time when he should be focused on academics and applying to college.
Grossman said her family lives in fear of when the next episode will strike.
“Every day is like Russian roulette,” she said.
There is one silver lining of KLS. and that’s that it’s finite. Most patients stop having episodes by age 30.
“Knowing he can outgrow it is fantastic, and that’s what keeps me going, but when will that be? It’s a magic question, it’s a mystery, and I just don’t know when it’s going to be,” she said.
Grossman is planning the third annual Coop-A-Thon on Dec. 8. Named for her son Cooper, the cycling event raises money for the KLS Foundation.
What is Kleine-Levin Syndrome?
Hunger, fixation on sex, and sleeping in are normal among teenage boys. But when these behaviors are extreme, it may be the Kleine-Levin Syndrome (KLS), or what some have dubbed “the Sleeping Beauty syndrome.”
The KLS is a rare neurological and sleep disorder in the hypersomnia family that mainly affects teenage boys. Like other hypersomnias including narcolepsy, Kleine-Levin syndrome involves excessive daytime sleepiness.
How common is Kleine-Levin syndrome?
TThis condition is rare, affecting fewer than 200,000 patients in the U.S. The median age of onset is 15. About 70 percent of those with the disorder are adolescent males.
Symptoms of Kleine-Levin syndrome include excessive daytime sleepiness and sleeping for long stretches (patients sometimes wake only to eat and use the bathroom), sometimes accompanied by increased appetite and hypersexuality, for a period of 2 to 31 days. These periods are called “episodes”. During an episode, patients may also experience confusion, disorientation, and hallucinations.
On average, patients experience 7 to 19 episodes lasting 10 to 13 days each, and relapse every 3.5 months. In between episodes, symptoms disappear. Relapses can occur for up to a decade before the illness spontaneously resolves. The sudden onset of each episode can be accompanied or triggered by flu-like symptoms or infections (38.2%), head trauma (9%), alcohol consumption (5.4%), or sleep deprivation.
What causes Kleine-Levin Syndrome?
The cause of Kleine-Levin syndrome is unknown, although the disorder has a strong genetic component. This uncommon sleep disorder tends to run in families, especially of Jewish ancestry. About 15 percent of the Kleine-Levin syndrome patients are of Jewish origin and “the incidence reported in Israel is unproportionately high,” according to a paper in a Jewish medical journal.
“The most promising findings are the familial clustering and a potential Jewish founder effect, supporting a role for genetic susceptibility factors,” according to French researchers.
Practitioners use a comprehensive medical history to make a diagnosis of KLS. Because brain imaging does not show any abnormalities in Kleine-Levin syndrome patients, the criteria for diagnosis are clinical, relying on patients’ and families’ descriptions of abnormal behavior.
This disorder generally appears in adolescence and subsides on its own. Over time, episodes diminish in frequency and severity, becoming less disruptive to the individual and family. On average, it takes 8-14 years for patients to “outgrow” Kleine-Levin syndrome, with longer duration in men, in patients with hypersexuality, and when onset is after age 20.
Some medications that promote wakefulness can help correct sleep abnormalities and excessive sleepiness present in Kleine-Levin syndrome. Amphetamines and lithium have been used successfully in some patients. Amphetamines helped decrease the oversleeping in 40% of patients. According to the same review in the journal Brain, “lithium had a higher reported response rate (41%) for stopping relapses when compared to medical abstention (19%).”
Outside of these attempts to mitigate the symptoms, patients and their families are usually told to inform themselves, connect with others experiencing the same syndrome, and wait the disease out.
Additional Kleine-Levin Resources
- The Kleine-Levin Foundation offers up-to-date research database, a blog including patient diaries, inspirational and practically helpful videos from patients and researchers, and a patient registry.
- Stanford University’s Center for Narcolepsy has a page with information on KLS clinical trials.
- The National Center for Biotechnology Information contains 387 links to peer-reviewed research papers regarding Kleine-Levin.