Kleine-Levin syndrome

Kleine-Levin Syndrome General description

Kleine-Levin syndrome (Sleeping Beauty syndrome) – is a rare sleeping disorder that characterized by recurrent periods of excessive sleep (hypersomnia), cognitive and behavioral problems. Around 1 million people are affected by Kleine-Levin syndrome (KLS). Most of them are men. Kleine-Levin syndrome usually appears in adolescence, but in some cases, children may face this illness too.

KLS can appear few times per year. The episodes of KLS last days, weeks or even months and in this time all normal activities stop. During the period of hypersomnia which lasts up for several weeks, the time of daily sleep increases dramatically, reaching 15-16 hours or more. During sleep, the patients can be woken up and you can have some short conversation with them but then they fall asleep again. The quality of sleep is bad; it doesn’t give any pleasure or feeling of rest and doesn’t include dreams. The picture of EEG doesn’t differ from normal one asleep. Between the episodes, patients are having a perfect health condition and there is no evidence of physical or behavioral changes.

Usually, patients sleep for 12-24 hours and wake up only to eat and go to the bathroom. During wakefulness, they feel irritability, apathy, depression, disorientation, aggression, hypersexuality, hallucinations, confusion, and lethargy. Also, they complain that everything seems out of control, they become very sensitive to noise and light.  Moreover, they may not be able to remember what happened with them during episode. People who have KLS cannot care about themselves and have normal daily life, so they would need someone to help and support them.

Vulnerable populations

Most of all, KLS affects adolescent males like around 16 years old. However, there have been reported cases of females and older men. More than 500 cases have been reported in general. But we can suppose that the amount is bigger in reality because some cases are not easy to recognize and make a diagnosis of KLS.

Symptoms of KLS

The symptoms and signs appear during episodes and mostly related to the functionality of hypothalamus and thalamus, the parts of the brain that are responsible for sleep and appetite.  Affected individuals can face following symptoms:

  •        Hypersomnia or excessive sleep (lasts for 12-24 hours per day): this is the main symptom of KLS, so the diagnosis has to include it.
  •        Cognitive damage: affected people may have confusion, disorientation, diminished comprehension, memory problems, speech problems.
  •        Altered perception: patients are becoming disconnected from the real world. In addition, some of them may feel they live in dream world, have hallucinations and delusions.
  •        Eating disorders (hyperphagia): this may happen during episodes as well when food cravings change and affected individuals may have interest to food they never like or urge to eat that can lead to obesity.
  •        Hypersexuality: usually it more related to males and they might have increased sexual urges during episodes of KLS.

Sometimes symptoms may include migraine headaches, irritability, anxiety, changes in mood, autonomic nervous system malfunctions, and high body temperature, heart rate, and blood pressure.

If a person is facing KLS first time the symptoms may include flu-like signs.

In some cases, people who have Kleine-Levin syndrome may even not know about it because it doesn’t cause any symptoms per weeks, months or years.

Possible complications

In some cases, there were nervous fever and bulimia reported. Often it is accompanied by a change in the sugar curve. In rare cases, Kleine-Levin syndrome is complicated by narcolepsy – chronic neurological disorder characterized by brain’s dysfunction linked to regulation of sleep-wake cycles.

Causes

It is unknown what causes KLS till now, but researchers are working on this topic and several theorems were proposed already. They are based on the suggestion about a malfunction in the hypothalamus and thalamus. These brain parts are responsible for regulating functions such as sleep, appetite, body temperature and sex drive.kleine-levin syndrome

In rare cases, KLS develops after flu-like symptoms present, basing on infection as the main reason, so researchers suppose that autoimmune process is important for developing this illness.  The autoimmune process appears when organism is fighting with «foreign» antibodies that attack healthy tissue. These autoimmune factors have been suggested because a lot of patients had flu-like symptoms at the beginning of KLS. The infection may have bigger effect on people who have genetic disposition towards Kleine-Levin syndrome. Another possible cause is related to the metabolism of serotonin and dopamine. An imbalance in the neurotransmitter pathways of these chemicals might have an effect too.

Related disorders

Kleine-Levin syndrome is not easy to identify because it may have similar symptoms that belong to other diseases and disorders. For example narcolepsy (a sleep disorder described by irregular sleepiness, sudden muscle weakness, confusion, hallucinations, paralysis during nap), cataplexy, delayed sleep phase syndrome, sleep apnea, African sleeping illness and idiopathic hypersomnia.

Treatment

KLS may be suspected according to deep clinical evaluation and patient’s history. It will be confirmed if a patient has three main disorders: excessive sleep, the desire to eat all available food, hypersexuality. Some medical tests can be performed for finding additional symptoms as epilepsy, meningitis, encephalitis.

There is not ultimate treatment for the Kleine-Levin syndrome, but one can use medications for relieving the symptoms. Several drugs such as modafinil and methylphenidate can remove effects of hypersomnia, but don’t have any impact on cognitive problems.

Also, in some cases, doctors may prescribe mood alerting drugs like carbamazepine and lithium. Antipsychotics and benzodiazepines can help to deal with psychotic and anxiety related symptoms.

All healthcare centers recommend caring for the person who has KLS at home, don’t leave this individual alone and manage anxieties and other behavioral disorders during awake.

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