Kleine-Levin syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at January 10, 2017
StartDiseasesKleine-Levin syndrome

Kleine-Levin syndrome is a sleep disorder. It is exceptionally rare and is considered a neurological disorder. This condition manifests as hypersomnia, compulsive eating or hyperphagia, and hypersexuality. It is sometimes referred to as sleeping beauty syndrome.

Contents

Definition & Facts

Kleine-Levin syndrome causes a person to experience episodes in which they need up to 20 hours of sleep per day. It is most common among teenage males. It is possible that this disorder results from abnormalities of the hypothalamus, though its cause is unknown or idiopathic.

This condition was described in 1925 and 1936 by Willi Kleine and Max Levin, respectively. It was named in 1942. Approximately 200 cases have been documented in the medical literature, and it is estimated to affect 1 in a million people. Its average age of onset is 15 years of age. There is much that remains to be studied in regards to this disorder's etiology, diagnosis, and treatment.

Symptoms & Complaints

Kleine-Levin syndrome causes a number of severe symptoms that are episodic in nature. A person with this condition will experience symptoms for at least two days to a month at a time, but will otherwise function normally.

Hypersomnia is a major symptom. A person with this condition may sleep up to 20 hours a day and still report excessive daytime sleepiness. He or she will feel persistent lethargy and drowsiness. Feeing unrefreshed from sleep, they will experience a number of problems pertaining to cognition and mood.

Symptoms include cognitive deficits such as impaired memory. Amnesia may also occur. A person with this condition will find it difficult to recall words or images. He or she will find it difficult to concentrate or focus.

Social interaction and communication will likely be impaired. Other symptoms include confusion, hallucinations, and derealization. Derealization is experienced as a perception of one's external surroundings as being unreal or distorted. One may feel like he or she is in a dream or trance.

A person may also experience depression, anxiety, and suicidal ideation. Irritability and inability to perform daily functions and acts of self-care also manifest as symptoms.

Hypersexuality is a symptom of this condition and manifests as frequent masturbation, an increased libido, and inappropriate sexual advances and comments. Compulsive eating is another symptom. A person with this condition will have an insatiable appetite and will engage in binge eating.

Following symptomatic episodes, patients experience relief and euphoria. Episodes typically last between one to three weeks. Months elapse between episodes. Insomnia can sometimes follow episodes.

Causes

It is unclear what causes this condition. Alcohol abuse, traumatic head injury, sleep deprivation, infection, postpartum psychosis, and fever have been posited as possible triggers for episodes if not underlying causes. It may be possible that this condition is an autoimmune disorder. There may be genetic factors that contribute to the onset of this condition.

Diagnosis & Tests

Other sleep disorders and psychiatric disorders with similar symptoms will need to be ruled out for a health care professional to render a diagnosis of Kleine-Levin syndrome. Other hypersomnias that will need to be ruled out include idiopathic hypersomnia, narcolepsy, and posttraumatic hypersomnia. Bipolar disorder, epilepsy, multiple sclerosis, and substance abuse will also need to be ruled out for a diagnosis to occur.

Neuropsychological evaluations will need to be undertaken to assess the patient's symptoms pertaining to cognition and memory. Wechsler Memory Scale can help assess memory impairment. Psychological evaluations can help establish patterns of behavior, personality changes, and mood disturbances.

The Epworth Sleepiness Scale is a questionnaire that can help identify a person's drowsiness levels in different settings. Sleep studies or polysomnography may be conducted. These exams study a person's brain waves, heart rate, breathing, and other body functions while a person is asleep. Electroencephalography can be useful in ruling out epilepsy. Blood tests may help rule out Lyme disease. They do not reveal inflammation nor hormonal imbalances among those with this condition.

Cerebrospinal fluid analyses have shown that levels of hypocretin tend to be lower or deficient during symptomatic episodes than during healthy periods. Hypocretin is a neuropeptide that regulates wakefulness and appetite, and its deficiency is implicated in narcolepsy. However, these variations are not significant enough for them to be useful in diagnosis. Lumbar punctures or spinal taps may be useful, however, in ruling out meningitis which is inflammation of the meninges usually as a result of infection. The meninges are the membranes that cover the brain.

Imaging studies can reveal hypoperfusion in parts of the brain during episodes. Single-photon emission computed tomography (SPECT) is a type of imaging study that has shown hypoperfusion. Hypoperfusion describes a state in which oxygen and nutrients are inadequately supplied to tissue. Hypoperfusion can affect the hypothalamus, thalamus, and basal ganglia. Additional examples of imaging studies that may be taken are computed tomography (CT) scans and magnetic resonance imaging (MRI) scans.

Treatment & Therapy

Kleine-Levin syndrome is difficult to treat because its causes are unknown. Stimulants have been used to treat or mitigate sleepiness. Modafinil is a stimulant used to treat excessive daytime sleepiness; it promotes wakefulness. Amantadine is an antiparkinson medication and antiviral drug that is sometimes used to treat this condition. Amantadine has been known to cause blurred vision and dizziness.

Amphetamine and ephedrine may also be prescribed to promote wakefulness. These treatments, while mitigating sleepiness, do not improve cognition. Lithium has been used to treat the mood disturbances associated with this condition though its efficacy is unconfirmed.

Treatment focuses on supportive care, such as facilitating the patient to have adequate rest. The outlook is generally good, and for those whose symptoms spontaneously resolve, its total duration is four years.

Prevention & Prophylaxis

Kleine-Levin syndrome cannot be prevented because its etiology remains unknown. Though various medications such as selective serotonin reuptake inhibitors have been posited as possible prophylactic medications, there is no evidence that pharmacological approaches can prevent this condition.

A person who is experiencing symptoms should be closely monitored and should refrain from driving or operating heavy machinery due to the increased risk of injury or accident. Their schedule will need to be tailored to allow adequate time for rest.

When a person is not symptomatic, they ought to avoid possible triggers such as alcohol. They should practice proper hygiene to reduce the risk of infection.