Kleine-Levin Syndrome | Drauzio Varella

In Kleine-Levin Syndrome, also known as Sleeping Beauty Syndrome, hypersomnia can last for hours, days, or weeks.

Kleine-Levin Syndrome (SKL), also known as “Ederna Sleep Syndrome”, was named after the main character in a German fairy tale, by the imposition of an evil witch, who was sentenced to sleep peacefully until a prince woke her up. up with a kiss of love.

In medicine, the name refers to a rare neurological disorder that is difficult to diagnose, known as Kleine-Levin Syndrome, in honor of two scientists who tried to describe the disease, which was classified as one of several types. hypersomnia.

See also: Sleep disorders

Among the main features of this syndrome of unknown origin are the following: 1) The appearance of repetitive and relieving episodes of excessive daytime or nighttime sleepiness, which tend to disappear over the years; 2) extreme resistance to waking up and staying awake during the day; 3) who knows little about the disorder that makes it difficult to define a diagnosis of the disease; 4) the intensity of the crisis decreases over time; 5) a favorable prognosis, even if the definitive cure is not yet known; 6) Appearances generally last for about ten days and may reappear every three or four months of the year.

The hypersomnia crisis can last for hours, days, or weeks, and along with uncontrolled cognitive changes and behaviors, endanger the quality of life of the person and those living with them, as well as impairing patients’ social, emotional, and professional performance. of the situation.

Kleine-Levin syndrome can occur at any age, but it mainly affects adolescent men and young adults. Occasionally, women and the elderly suffer from disorders associated with the classic trio of symptoms: hypersomnia (deep sleep), hyperphagia (compulsive eating), and overt and aggressive sexual hyperactivity.

As no effective treatment is known to promote the complete cure of the syndrome, clinical follow-up of patients with the disorder has shown that the disorder can go away on its own, without medication or special care, around the age of 35/40.

Causes of Kleine-Levin Syndrome

Kleine-Levin syndrome is a disorder whose cause has not yet been identified (which is why it is called idiopathic or primary). Several hypotheses have been raised to try to shed light on the origin of the disorder, starting with the infection of a type of virus that reappears from time to time.

Genetic, autoimmune and hereditary factors, head injuries and high stress load are other possible causes of the syndrome that are still being studied.

Some research suggests that there may be functional changes in certain areas of the brain (hypothalamuslimbic system, thalamus and frontodenporal cortex, for example) may be related to the regulation of sleep and emotion in patients with the syndrome.

Today, there is a tendency to accept that the condition may have a genetic component and may be related to immune and environmental factors.

Symptoms Kleine-Levin syndrome

Excessive sleepiness, food cravings, and repetitive sexual disinhibition are the main signs of Kleine-Levin syndrome.

When the attack begins, the hypersomnia section can be prolonged for hours and hours of uninterrupted sleep. The person wakes up after a lot of effort to use the toilet and eat out of control

Called “sleep inertia,” this phase of sleep-to-wake transition can be slow and quite confusing.

Even in crises, in short periods of truly awake, there can be drastic changes in behavior, extreme fatigue, apathy, mental disorder, attention and concentration impairment, memory impairment, irritability, lack of control over food, loss of inhibition. sex as well as hallucinations.

Another frequent complaint, in these cases, is the difficulty in distinguishing the dream from reality when the person tries to remember what happened during sleep (mild amnesia). It is also common, during waking hours, for the appearance of compulsive behavior, emotional distress, and the patient to be very sensitive to noise and light.

It should be noted that between one crisis and the next, the person returns to normal behavior and returns to normal activities, as if this sleep disorder had never been affected. In contrast, they appear to be in good health, in perfect physical and mental health, and only a minority show more persistent psychiatric disorders.

See also: Lack of sleep

Diagnosis Kleine-Levin syndrome

From a clinical point of view, the diagnosis of Kleine-Levin syndrome is made by an unidentified cause, preferably by a multidisciplinary team, led by a neurologist.

Therefore, laboratory blood tests and imaging tests (MRIultrasound, computed tomography) have been shown to be useful in establishing a differential diagnosis with other pathologies with similar symptoms.

However, the most reliable test for diagnosing hypersomnia in general and Kleine-Levin syndrome in particular remains polysomnography, a non-invasive procedure that allows the assessment of sleep quality and the diagnosis of related disorders.

To complete the diagnosis, it is necessary for the symptoms to last at least a month and have a negative impact on the patient’s quality of life.

Treatment of Kleine-Levin syndrome

To date, there is no known effective treatment for Kleine-Levin syndrome, a rare disorder with a low prevalence and unknown cause. In the face of this evidence, the attention of scholars has been to control and alleviate the symptoms. It is already well known that it is possible to improve the quality of life of patients with the disease by using certain stimulant drugs, such as amphetamines and antidepressants, which have shown some efficacy in similar clinical conditions and implicit psychological symptoms.

In terms of pharmacological resources, mood stabilizers and convulsions may be part of the therapeutic regimen for behavioral disorders associated with the syndrome. The prescription of lithium-based medications during the crisis has already been tested and shown to have a beneficial effect.

In crises, it is part of the treatment to let the person sleep without interference as long as he or she deems it necessary.

However, despite the different approaches, the goal of treatment remains the same: to reduce periods of excessive sleep during the day and to prolong the number of people who are able to stay fully awake as much as possible.

Unfortunately, effective treatment for the syndrome remains elusive. However, current research suggests that sleep deprivation syndrome is not as benign as previously thought. The current trend is to accept that this condition has a genetic origin related to immune and environmental factors.

Frequently Asked Questions about Kleine-Levin Syndrome

What are the symptoms of Kleine-Levin syndrome?

Excessive sleepiness, food cravings, and repetitive sexual disinhibition are the main signs of Kleine-Levin syndrome. In crises, periods of wakefulness, severe behavioral changes, extreme fatigue, apathy, mental confusion, attention and impaired concentration, memory deficits, irritability, lack of control over eating, loss of sexual inhibition, and even hallucinations.

In addition, there are also difficulties in distinguishing between dreams and reality and expressions of compulsive behavior, emotional distress, and sensitivity to noise and light.

What are the causes of Kleine-Levin syndrome?

Kleine-Levin syndrome is an unidentified cause of the disorder. Today, there is a tendency to accept that this condition may have a genetic component and may be related to immune and environmental factors.

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