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Kleine-Levin Syndrome

 Kleine-Levin Syndrome



Overview

Kleine-Levin syndrome (KLS), also known as “sleeping beauty syndrome” or “familial hibernation syndrome,” is an extremely rare condition that causes intermittent episodes where you sleep for long periods of time, which prevents you from staying awake during the day (hypersomnia). KLS also affects your behavior. People diagnosed with KLS often sleep for 16 to 20 hours per day during a KLS episode.

Kleine-Levin syndrome can affect anyone. Men and people assigned male at birth are more likely to have the condition. Symptoms of the condition usually start in early adolescence, and episodes of sleepiness decrease in frequency and severity over a median course of 14 years.

Symptoms

Symptoms usually begin in early adolescence and recur usually more than once per year. The average duration of KLS is 14 years. Symptoms include:

Extreme sleepiness and inability to stay awake.

Increased appetite (hyperphagia).

Increased sex drive (hypersexuality).

Hallucinations.

Irritability or behavioral changes.

Anxiousness or depression.

Confusion or amnesia.

An episode occurs when you experience these symptoms for at least two days. KLS episodes can last for a few days, average around 10 days, or could last for a couple of weeks. One study suggests that people diagnosed with KLS have an average of 20 episodes during their lifetime.

Most people have trouble remembering what happens during an episode. They can wake up during an episode to eat or use the restroom but have limited physical function due to excessive sleepiness.

After you experience an episode, you’ll go back to your normal patterns of behavior and not have any symptoms of the condition except for possibly mild cases of memory loss.

Certain events trigger symptoms of KLS, including:

Flu-like illness or infection.

Drug and alcohol use.

Head trauma.

Physical exertion.

Stress.

Causes

The exact cause of KLS is unknown, but some doctors believe certain factors can increase your risk for this condition.

For example, KLS may arise from an injury in the hypothalamus, the part of the brain that controls sleep, appetite, and body temperature. A possible injury could be falling and hitting your head, although more research is needed to confirm this link.

Some people develop KLS after an infection like the flu. This has led some researchers to believe KLS may be a type of autoimmune disorder. An autoimmune disease is when the body’s immune system attacks its own healthy tissue.

Some incidents of KLS may also be genetic. There are cases where the disorder affects more than one person in a family.

Diagnosis

Diagnosis of Kleine-Levin syndrome is challenging. There isn’t a specific test that can diagnose KLS. Healthcare providers diagnose KLS by eliminating other conditions with similar symptoms. They’ll begin your evaluation and work up after asking you about your symptoms, including how long you’ve had them and the severity.

Providers may offer tests including:

Sleep test to measure electrical activity in your brain.

Memory tests.

Blood tests.

Imaging tests like an MRI.

Treatment

There isn’t a definitive treatment for KLS. In most cases, people with KLS don’t even take medication. During episodes, your healthcare provider can help you make accommodations — such as time off work or school — so you can sleep as much as your body needs. Other possible treatment options target symptoms of KLS. While some treatment options help keep you awake, one treatment doesn’t target all symptoms, especially symptoms that affect behavior and cognitive function. Treatment includes taking medicines like:

Lithium: May reduce the frequency of episodes.

IV steroids: May shorten prolonged episodes greater than 30 days.

Stimulants or wake-promoting agents: Helps prevent sleepiness.

Your provider might suggest supportive therapy to work with a professional who can address changes to your behavior, hypersexuality, anxiety and depression.

There’s no single medicine to treat Kleine-Levin syndrome. Your provider will work closely with you to monitor your symptoms and how your body reacts to certain treatments, especially amphetamines, as there’s a high risk of dependency associated with the drug.

Type of Doctor Department :Neurologist


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