Kleine-Levin Syndrome: Symptoms, Causes, and Treatment

Kleine-Levin Syndrome (KLS), known as ‘Sleeping Beauty’ syndrome, is a rare disorder that affects the nervous system. KLS patients experience recurring periods of excessive sleep, altered behavior, cognitive difficulties, or changes in perception. The episodes come on abruptly and, equally swiftly, end – leaving individuals in a normal state in between these periods.

Understanding KLS is essential, as it can significantly impact a person’s quality of life.

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What Is Kleine-Levin Syndrome?

Kleine-Levin Syndrome is a sleep disorder (technically a subset known as a parasomnia) specifically characterized by hypersomnia. The disorder primarily affects adolescent males, although it can occur in females and across various age groups. It’s not just a young person’s condition, as adults and the elderly can have it too.

“The first cases of KL syndrome were described by Kleine and Levin, independently, in 1925 and 1929, respectively,” says Dr. Nayantara Santhi. “Whilst the causes of this condition are not well understood, it is thought that pathology of the hypothalamus, a brain area involved in circadian rhythms and appetite, is involved in this condition.”

“One of the distinctive features of this condition is hypersomnia and the frequency of occurrence of hypersomnia is one of the criteria for KL syndrome in the International Classification of Sleep Disorders.”

As Dr. Santhi noted, KLS is often characterized by recurring episodes of excessive sleep and altered behavior. While anyone can potentially develop KLS, studies show Verified Source National Library of Medicine (NIH) World’s largest medical library, making biomedical data and information more accessible. View source a higher incidence in the male population. This disparity, however, doesn’t undermine the severity of the condition in affected females, as they, too, face significant challenges.

Those with KLS often experience episodes that are interspersed with weeks or months of normal behavior. The unpredictability of these episodes can create disruptions to daily life, often leading to difficulties in maintaining regular school, work, or social routines.

Despite its designation as a sleep disorder, KLS also encompasses various symptoms that align with mood disorders, making it a complex condition that requires comprehensive understanding and management.

Symptoms of Kleine-Levin Syndrome

KLS patients exhibit a variety of symptoms, Verified Source National Library of Medicine (NIH) World’s largest medical library, making biomedical data and information more accessible. View source differing in intensity and frequency greatly among individuals. Some of the most common symptoms include:

• Excessive Sleep: Individuals with KLS may sleep for up to 20 hours a day during an episode.
• Increased Appetite: Specifically a type of excessive eating and abnormal appetite known as hyperphagia, Verified Source National Library of Medicine (NIH) World’s largest medical library, making biomedical data and information more accessible. View source typically associated with injury to the hypothalamus.
• Altered Behavior: One may observe altered behavior in them, which may include Verified Source National Library of Medicine (NIH) World’s largest medical library, making biomedical data and information more accessible. View source confusion, lethargy, or uncharacteristic behavior such as compulsive eating, irritability, or childishness. All part of the neurological disorders linked to KLS.
• Cognitive Difficulties: People may face challenges in concentration and may seem confused or disoriented.
• Changes in Perception: KLS patients might also experience changes in perception, including hallucinations or feeling like they’re in a dream.

Kleine-Levin Syndrome episodes can happen once or several times a year, lasting from a few days to weeks. The severity and duration of symptoms can also vary from one episode to the next, even within the same individual.

Between these episodes, individuals typically return to their normal state with no memory of the event, making KLS a challenging and unpredictable condition to manage.

Causes and Risk Factors of Kleine-Levin Syndrome

The exact causes of Kleine-Levin Syndrome are not fully understood. Verified Source National Library of Medicine (NIH) World’s largest medical library, making biomedical data and information more accessible. View source But there are several factors that may contribute to its development.

KLS is considered a neurological disorder, Verified Source Johns Hopkins Medicine University focused on medical research that produces thoroughly reviewed health articles. View source and research suggests that it may be associated with abnormalities in the brain’s hypothalamus and thalamus, which are involved in sleep regulation and consciousness. However, more research is needed to establish the specific underlying mechanisms.

Risk factors for KLS are not well-defined, but certain factors may increase the likelihood of developing the syndrome. Some potential risk factors include:

• Age and Gender: KLS most commonly affects adolescents and young adults, with onset typically between the ages of 15 and 30. Additionally, it appears Verified Source ScienceDirect One of the largest hubs for research studies and has published over 12 million different trusted resources. View source to be more prevalent in males than females.
• Genetic Factors: There may be a genetic predisposition to KLS. In some cases, there is a family history Verified Source National Library of Medicine (NIH) World’s largest medical library, making biomedical data and information more accessible. View source of the disorder, suggesting a potential genetic link. Verified Source National Library of Medicine (NIH) World’s largest medical library, making biomedical data and information more accessible. View source
• Triggers: Some cases of KLS may be triggered Verified Source Oxford Academic Research journal published by Oxford University. View source by infections, illnesses, or head injuries. Emotional stress and physical exertion have also been associated with the onset of episodes in some individuals.
• Autoimmune Factors: There is ongoing research Verified Source National Library of Medicine (NIH) World’s largest medical library, making biomedical data and information more accessible. View source exploring the possibility of autoimmune mechanisms contributing to KLS, as some individuals have shown elevated levels of certain antibodies during episodes.

It’s important to note that while these factors may be associated with KLS, the condition remains rare and not all individuals with these risk factors will develop the syndrome. The exact interplay of genetics, environmental factors, and neurological processes that lead to KLS requires further investigation.

How is Kleine-Levin Syndrome Diagnosed?

Diagnosing Kleine-Levin Syndrome (KLS) can feel like solving a tough puzzle. It’s mostly about carefully listening to the person’s story and symptoms.

Doctors need to hear about recurring periods when someone just can’t stay awake and how these sleep marathons are paired with noticeable changes in behavior and thinking.

Now, you might wonder – don’t other sleep disorders also involve being extra sleepy?

Yes, they do. Sometimes, these symptoms can be confused with conditions like hypersomnia Verified Source National Library of Medicine (NIH) World’s largest medical library, making biomedical data and information more accessible. View source or sleep apnea, which also cause excessive sleepiness.

But KLS has a particular rhythm to it that hypersomnia doesn’t- the intense sleepiness and strange behaviors come in episodes, and then there are breaks when the person goes back to their usual self. This on-again, off-again pattern sets KLS apart. Still, hypersomnia is one of the diagnostic criteria for KL syndrome in ICSD-3.

Another thing is that when a KLS episode hits, it can turn behaviors and moods upside down in ways not seen in most other sleep disorders. People with KLS might eat way more than usual, behave unusually around others, or even feel like they’re living in a dream.

But how can doctors be sure it’s KLS and not something else?

Sometimes they have to play detective – they might conduct a thorough physical examination, order blood tests, brain scans, or even a sleep study to rule out other conditions.

(See also Does Lack of Sleep Affect My Blood Test Results?)

Treatments for Kleine-Levin Syndrome

Treating KLS doesn’t have a standard approach, but a range of options exist to help manage the symptoms and reduce their impact on daily life. Because KLS is so unique and less understood, we don’t have a specific cure yet.

But that doesn’t mean we can’t do anything about it! The goal of treatment is to help manage the symptoms and make the episodes less of a disruption to a person’s life.

Often, medications Verified Source National Library of Medicine (NIH) World’s largest medical library, making biomedical data and information more accessible. View source are a key part of the game plan. Mood stabilizers like lithium can be helpful, especially if mood changes are a big part of someone’s KLS episodes.

Clinical trials for drugs like antipsychotics or stimulants are sometimes used to manage symptoms like excessive food intake or other psychiatric symptoms.

Another star player in this game is the support system – the family, friends, and caretakers who help make sure the person with KLS is safe and comfortable during episodes.

How to Live with Kleine-Levin Syndrome?

Living with Kleine-Levin Syndrome is a challenge, but with the right strategies, it can be manageable. Here’s how you can manage to live with it.

• Understanding KLS: The first step to managing life with KLS is to understand the condition. Learning about this neuropsychiatric disorder, recognizing its symptoms, and understanding its effects is crucial. This knowledge can prepare you to face the episodes when they occur and also provide a foundation for explaining your condition to others.
• Creating a Safe Environment: Since KLS episodes can cause Verified Source ScienceDirect One of the largest hubs for research studies and has published over 12 million different trusted resources. View source individuals to sleep for extended periods, it’s essential to ensure that the environment is safe and comfortable. Remove any potential hazards and make sure the person can rest undisturbed.
• Adapting Your Lifestyle: Learn to adapt to the patterns of KLS. Keep a symptom diary to track episodes and look for any potential triggers or patterns. This can help you plan and prepare for episodes.
• Maintaining a Healthy Lifestyle: Regular exercise, a balanced diet, and good sleep hygiene contribute to overall health and well-being, which may help Verified Source National Library of Medicine (NIH) World’s largest medical library, making biomedical data and information more accessible. View source reduce the severity of KLS episodes.
• Seeking Support: Don’t hesitate to seek support. Connecting with others who understand the challenges of living with KLS can be a great source of comfort and practical advice.
• Keeping Open Communication: Openly communicate with your employer or school about your condition so they can provide necessary accommodations.

Frequently Asked Questions

Is Kleine-Levin syndrome a mental illness?

While KLS primarily affects sleep patterns and behavior, it is essential to differentiate it from mental illnesses such as depression or bipolar disorder. KLS is believed to have neurological origins, involving specific brain regions that regulate sleep and behavior. It is classified as a sleep disorder, not a mental illness.

The effects on mental health primarily stem from the disruptive nature of the episodes and associated behavioral changes. During KLS episodes, individuals may experience cognitive impairment, confusion, and changes in behavior, which can impact their emotional well-being. The altered perception of the world and prolonged excessive sleepiness during these episodes may lead to feelings of disconnection, frustration, and anxiety.

What separates Kleine-Levin syndrome from narcolepsy?

KLS and narcolepsy are distinct sleep disorders with different characteristics. KLS is marked by recurring episodes of excessive sleepiness lasting for days, weeks, or months, interspersed with periods of normal sleep. During KLS episodes, individuals may sleep for 15 to 20 hours a day or more and may experience cognitive changes, altered behavior, increased appetite, and a sense of derealization.

In contrast, narcolepsy is a chronic condition with ongoing excessive daytime sleepiness, without distinct episodes. People with narcolepsy experience constant sleepiness throughout the day, leading to sudden and uncontrollable sleep attacks. Narcolepsy is often accompanied by other symptoms, such as cataplexy (muscle weakness triggered by emotions), sleep paralysis, and vivid hallucinations during sleep-wake transitions.

The age of onset also differs. KLS usually begins during adolescence (between ages 15 and 25). Meanwhile, narcolepsy symptoms can develop during adolescence or young adulthood, but also later in life. The underlying causes of both disorders are not fully understood, and it is crucial to seek medical attention for a proper diagnosis.

Can Kleine-Levin Syndrome go away on its own?

Kleine-Levin Syndrome typically does not resolve on its own without treatment. However, over time, the frequency and intensity of KLS episodes often decrease, and in some cases, they may even stop altogether. While the syndrome may improve with age, it is crucial to seek proper medical attention and management to ensure the best possible outcome.

How long do episodes of Kleine-Levin Syndrome usually last?

A typical Kleine-Levin Syndrome episode lasts for about a week or two. But in some cases, episodes can extend up to a month. It’s important to note that the duration of episodes can vary significantly from one individual to another. The length and severity of each episode should be carefully monitored by healthcare professionals to provide appropriate treatment and support.

What triggers episodes of Kleine-Levin Syndrome?

The specific triggers for episodes of Kleine-Levin Syndrome remain largely unknown. While some theories suggest that factors such as infections or physical stress might be potential triggers, clear and definitive causes have not been established. Understanding the underlying triggers is crucial for effective management, and ongoing research is essential to gain more insight into the mechanisms of this rare sleep disorder.

Is Kleine-Levin Syndrome a disability?

Yes, KLS can be considered a disability, depending on its impact on an individual’s daily functioning and ability to perform essential tasks. The severity and frequency of KLS episodes can vary from person to person. In some cases, individuals may experience frequent and prolonged episodes that significantly interfere with their ability to work, attend school, or carry out daily activities. As a result, they may qualify for disability benefits or accommodations.

Conclusion

Kleine-Levin Syndrome, though rare, can significantly impact an individual’s life due to its intermittent and unpredictable nature. The key to managing KLS is a proper diagnosis, distinguishing it from other sleep disorders, and understanding the treatments available, including medication and lifestyle adaptations.

While triggers for episodes are unclear, the condition often improves over time. People with KLS can lead a relatively normal life between episodes, though they need to be prepared to manage sudden symptom onset. Understanding and support from family, friends, and healthcare providers is vital for individuals navigating life with KLS.