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Kleine-Levin Syndrome - Symptoms, Causes, Diagnosis, and Treatment

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Kleine-Levin syndrome is a rare sleeping disorder primarily affecting adolescent males. Learn more about this syndrome in detail in the article below.

Medically reviewed by

Dr. Vishal Anilkumar Gandhi

Published At October 30, 2023
Reviewed AtOctober 30, 2023

What Is Kleine Levin Syndrome?

Kleine-Levin syndrome (KLS) is a rare disorder that results in repetitive periods of excessive sleepiness. This could mean sleeping up to 20 hours a day for some individuals. Because of this reason, the condition is commonly referred to as “sleeping beauty syndrome.” KLS can also cause confusion and changes in behavior. This condition can affect anyone, but adolescent boys develop the disorder more than any other group. About 70 percent of individuals affected with this disorder are male. Episodes can have a recurring tendency over an extended period. Sometimes they come and go for as long as ten years. During each episode, attending school, working, or participating in other activities can be difficult.

What Are the Symptoms of Kleine Levin Syndrome?

The onset of KLS is sudden and may present like the flu at first. The first and foremost symptom is excessive sleepiness, which makes one sleep for extremely long periods, often leading to more than 18 hours a day. Several additional changes in behavior and cognition accompany the need to sleep excessively:

  • Increased Appetite: Hyperphagia is a common symptom of KLS, which means a heightened focus on food, and growing food intake. Typically, people with KLS accept to eat any food offered but especially crave sweet foods. As a result, their food intake may increase to as much as six to eight meals daily.

  • Increased Sex Drive: Another common symptom that people with KLS usually experience is hypersexuality, which may involve frequent masturbation, sexual comments, and undesired sexual advances.

  • Hallucinations: People may also experience the feeling of derealization during the episodes of KLS, which is the dissociative feeling of being detached from the world around them.

  • Irritability and Childishness: People with KLS may have uncharacteristic irritability or aggression, especially if their food is denied. They may also have verbal outbursts that are rude, offensive, or sexual.

  • Anxious or Depressed Mood: During episodes of KLS, such people may withdraw because they cannot socialize as they usually do. About 15 percent of people with KLS report suicidal ideation.

  • Confusion and Amnesia: Some people with KLS may not be able to recall their entire experiences during the KLS episodes.

These symptoms subside with the cessation of KLS episodes, and a person resumes their usual mood and demeanor. However, some people may experience a continuation of memory loss.

What Causes Kleine Levin Syndrome?

The exact cause of KLS is currently unknown, but some researchers have hypothesized that certain factors can increase the risk for this syndrome, including

  • Infection.

  • Psychological disturbance.

  • Physical trauma.

  • Toxins.

  • Autoimmunity, in which the body attacks its organs and tissues.

For example, KLS may occur due to an injury to the hypothalamus which is the part of the brain responsible for controlling sleep, appetite, and body temperature. Possible damage could be falling and hitting the head. However, more research is necessary to confirm the association between head injury and KLS. Some people develop KLS post an infection such as the flu. This made some researchers believe KLS may be an autoimmune disorder. An autoimmune disease is one in which the body’s immune system attacks its healthy tissues.

How Is Kleine Levin Syndrome Diagnosed?

Healthcare providers usually initially rule out other conditions with the possibility of similar symptoms. Healthcare providers also check whether the drugs or medications are causing the symptoms. Apart from the symptoms, for the diagnosis of KLS, a person must also meet the criteria for recurrent hypersomnia:

  • Episodes of excessive sleepiness that last between two days and five weeks.

  • Every 18 months, the episodes can occur once and are often more frequent.

  • Expected behavior and cognition between episodes.

  • Other causes for hypersomnia have been ruled out.

Sleep studies can check for the amount of time spent sleeping. Sleep tests can also measure electrical activity in the brain. In cases of KLS, the activity of the brain is slowed down as much as 70 percent during sleep. Brain scans are typical, as expected. The performance of patients with KLS is usually poor for any memory test the physicians may conduct.

How Is Kleine Levin Syndrome Treated?

The treatment for KLS is not consistently effective. However, supportive care that can be given at home, especially through parents or guardians, can help in ensuring a safe environment during KLS episodes. Medications are prescribed only as a secondary treatment. Some medicines can help reduce symptoms rather than eliminate KLS.

1. Supportive Care

Patient support is important to overall well-being. Caregivers can:

  • Supervise the patient with KLS to ensure a comfortable and safe environment.

  • Delay school or postpone other activities until the cessation of KLS episodes.

  • Prevent the operation of a vehicle or heavy machinery by the patient.

  • Watch for symptoms of anxiety and depression and suicidal tendency.

  • Maintain consistency of sleep routines between episodes of KLS.

  • Encourage the patient to avoid alcohol and contact with ill people.

2. Medications

Medications do not entirely prevent future episodes of KLS. There is limited research that suggests that lithium can lessen the number of episodes but cannot eliminate them.

Stimulant pills are also an option to treat KLS. Although they can lead to irritability, these medicines promote wakefulness and reduce sleepiness. Options include Modafinil and Methylphenidate. Some medications may improve a few KLS symptoms but have a negative impact on others. For example, temporarily using antidepressants or mood-stabilizing drugs may aggravate psychiatric symptoms.

Conclusion:

Because episodes of KLS can occur for ten years or even more, living with this condition can tremendously impact ones life. For example, it can intervene with the ability to work, attend school, and cultivate relationships with family and friends. But unfortunately, it can also lead to anxiety or depression, mainly because it is not possible to predict when an episode will occur or how long it will last.

Talking to a healthcare provider will be best to identify an upcoming episode. Tiredness and sleepiness caused by KLS can have a sudden onset. In addition, one could injure oneself or even others if an episode occurs while operating a machinery or driving a motor vehicle. Therefore, learning to identify an impending episode makes it possible to get oneself out of a potentially dangerous situation.

Dr. Vishal Anilkumar Gandhi
Dr. Vishal Anilkumar Gandhi

Psychiatry

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