Kleine-Levin syndrome is an episodic recurrent hypersomnia characterized by increased sleepiness, perceptual disturbances, and paradoxical waking behaviors. Presumably, a central nervous cause is present. To date, there is no established treatment option because of its low prevalence.
What is Kleine-Levin syndrome?
The medical profession knows Kleine-Levin syndrome as periodic hypersomnia in childhood or adolescence. More than two-thirds of sufferers are male. Recurrent periods of sleepiness are among the leading symptoms of the syndrome. The periods of increased need for sleep last about two weeks each and are accompanied by perceptual or behavioral disturbances. With a prevalence of 1:1,000,000 to 2,000,000, Kleine-Levin syndrome is a rather rare disorder whose cause has not yet been fully elucidated. Because of its low prevalence, there have been few studies or solid research findings. The syndrome is classified as hypersomnia of central nervous origin. #
In the further classification of the disease, science is so far in disagreement. While some understand the syndrome as an infectious autoimmune disease, for others Kleine-Levin syndrome belongs to the genetic hereditary diseases.
Causes
The cause of Kleine-Levin syndrome is controversial. Autoimmunologic cause, for example, is speculated. The onset of the disease is often associated with infection. However, exactly which infection could cause the disease is unknown to date. Since a familial accumulation has been observed in the past, a genetic component is also conceivable. However, those affected with the corresponding genes do not necessarily have to contract the disease. However, the genetic disposition for the disease in combination with an infection could lead to the onset of autoimmunological and central nervous hypersomia. However, the hereditary nature of the disease has not yet been confirmed. Nor is a primary infectious cause conclusively established. Also a craniocerebral trauma or alcohol addiction could be observed in connection with the disease. To what extent and to what extent the increased illness of male adolescents is related to the cause also remains unclear to date.
Symptoms, complaints, and signs
Kleine-Levin syndrome is characterized by recurrent periods of significantly prolonged sleep. The periods vary in duration from days to entire month. On average, they are two weeks long. During these periods, the affected person sleeps most of the day and is found awake for only two hours per day during acute phases. During the waking phases, communicativeness and the ability to orient oneself suffers. Sufferers appear apathetic and lethargic and suffer from cravings. They can be awakened from sleep, but immediately fall asleep again. They are hypersensitive to sounds and light. Hypersexual behavioral disorders are often associated with the leading symptoms. During acute phases, patients say they have the feeling that they are actually dreaming. Often, during the waking phases, memory loss occurs or hallucinations appear. Therefore, if criminal acts occur during the acute phase, the diagnosis is of penalty-reducing relevance.
Diagnosis and course of the disease
The Multiple Sleep Latency Test (MSLT) is the main tool used to diagnose Kleine-Levin syndrome. In addition to a shortened latency to fall asleep, this test reveals the disorder as a prolonged total sleep time and specifically a prolongation of deep sleep phases and the first sleep stage. There is a deficiency in SWS phases. SPECT shows hypoperfusions of the thalamus, basal ganglia, and in frontotemporal brain regions. Despite neurologic cause, CT or MRI findings remain unremarkable. Differentially, the condition must be differentiated from other hypersomnias of central nervous origin. Psychiatric or internal hypersomnia of the secondary form must also be excluded. The prognosis is considered favorable. Hypersomnia usually resolves in adulthood and only increases symptomatically in rare cases during its course.
Complications
Unfortunately, Kleine-Levin syndrome cannot be treated causally. This results in significant limitations in the patient’s life and also a reduced quality of life.Most of those affected suffer from sleep problems, disturbances in perception and unbalanced behavior. The behavior can seem strange and bizarre, especially to outsiders, which often leads to exclusion and other social discomfort. Especially in children, this can lead to bullying or teasing. In most cases, the effects of Kleine-Levin syndrome are only felt for a few hours on a few days each month. Cravings may also occur and sufferers are extremely sensitive to light. Severe sexual dysfunction and urges may also occur. Most patients suffer from memory loss and hallucinations. Involuntary injuries or accidents may also occur. Medication can be used to limit some of the symptoms. However, taking the medication can be associated with side effects. It is not uncommon for the parents and relatives of those affected to be affected by psychological symptoms and require psychological treatment. However, a general prediction of the course of the disease and life expectancy is not possible.
When should one go to the doctor?
People who have recurrent episodes of increased fatigue or a significantly prolonged need for sleep should see a doctor. If the period of wakefulness is less than 3 hours daily, there is cause for concern. If the affected person appears lethargic, has poor communication skills, or appears disoriented, they need help. A visit to the doctor is recommended so that the cause of the behavioral abnormalities can be diagnosed. If daily tasks can no longer be performed as usual, a doctor should be consulted. In case of apathy, loss of appetite or reduced food intake, a doctor should be consulted. There is a risk of an undersupply of the organism, which can lead to further complications. Alternatively, during awake phases, attacks of ravenous appetite can be observed in the affected persons. If the affected persons can only be awakened with difficulty and then abruptly fall asleep again, medical clarification of the observations is advisable. Hypersensitivity to sounds or light are further indications of a health impairment. Patients are often unable to distinguish between dreams and reality. In most cases, a temporary spontaneous healing of the complaints occurs. Since the complaints recur at periodic intervals, a physician should be consulted even if the patient is already free of symptoms.
Treatment and therapy
There is no single therapy for Kleine-Levin syndrome to date because of the small number of cases and research possibilities. Above all, because the cause is unknown, there can be no talk of causal therapy. Under certain circumstances, the symptoms can be treated and alleviated with medication. Various drugs are available for this purpose. Mood-stabilizing substances include, for example, lithium and carbamazepine or phenytoin. Psychostimulants can also be considered, as their stimulating effect on the nervous system can reduce the patient’s general drowsiness under certain circumstances. Substances such as methylphenidate may be considered with this goal in mind. The drugs are used off-label and thus on a trial basis. The patient and parents must be made aware of the trial basis. As studies have shown, neuroleptics and antidepressants are not promising therapeutic options. They have shown little therapeutic effect in Kleine-Levin syndrome in the past. In contrast, the effect of stimulants such as amphetamines was able to reduce the general need for sleep in studies. The administration of lithium also often resulted in a suppression of sleep episodes. If no drug therapy is desired by the patients or their parents, then supportive therapy can take place. Especially the relatives, but also the patients themselves, are provided with a psychotherapist as part of this therapy. However, psychotherapeutic support for patients usually cannot take place during acute phases.
Outlook and prognosis
The prognosis of Kleine-Levin syndrome is unfavorable. Despite medical advances and different therapeutic approaches, no optimal treatment option for the disease has yet been found. Therefore, a cure does not occur under the current circumstances. Patients receive individualized care after diagnosis.The focus of a treatment lies on the one hand in the clarification of the possible symptoms and on the other hand in a need-based approach to the onset of symptoms. The difficulty for an optimized prognosis lies in the fact that the cause of the health disorder has not been clarified to date. Various observations could be documented as the reason for the disease. Since the disease only occurs to a small extent, open questions, the clarification of which contributes to progress in finding a solution, can only be answered hesitantly. This circumstance makes it difficult for scientists and researchers to find more concrete approaches to a comprehensive cure. So far it is certain that a healthy way of life contributes to an improvement in development. The mental and psychological well-being should be stable, so that a decrease of the complaints can be mounted. A recovery is nevertheless not given. It is helpful and supportive for the further course of the disease, if the consultation of a physician takes place already early and thus with the first complaints. This allows a quick intervention to alleviate existing complaints.
Prevention
The etiology of Kleine-Levin syndrome is unknown. Because medical science is unclear about the causes, the syndrome cannot be prevented to date.
Follow-up
In most cases, there are no direct aftercare measures available to the person affected by Kleine-Levin syndrome, so early diagnosis by a physician is primarily necessary for this disease. As a rule, self-healing is not possible, so that the affected person should consult a doctor as soon as the first symptoms appear. A complete cure is not possible in many cases, as the disease is still largely unexplored. Those affected are usually dependent on taking various medications. Regular and correct intake should always be ensured, and the appropriate dosage should also be observed. If there is any uncertainty or if questions arise, as well as in the case of undesirable side effects, a doctor should be contacted first so that the symptoms do not continue to worsen. Contact with other people affected by Kleine-Levin syndrome can also be very useful, as it is not uncommon for information to be exchanged, which can further alleviate the symptoms. Likewise, loving support and help from one’s family and friends has a positive effect on the course of the syndrome. It cannot be universally predicted whether Kleine-Levin syndrome will reduce the life expectancy of the affected person.
Here’s what you can do yourself
Patients with Kleine-Levin syndrome can go about their daily lives largely normally in the absence of symptoms associated with the condition. Without acute symptoms, sufferers can live symptom-free and are completely unremarkable in their feeling, thinking, and social behavior. During the excessive sleep phases, however, patients can sleep for up to 20 hours, which considerably restricts their everyday life. They then only get up to eat and drink. As a result, sufferers are usually dependent on help. To alleviate the phases of symptoms, patients can avoid specific triggers. These include heavy alcohol consumption and lack of sleep. Stress and physical exertion can also trigger the symptoms. Febrile infections should also be avoided, so it is important to strengthen the immune system. A healthy lifestyle with sufficient sleep and a balanced diet help. The drug lithium can help many patients to minimize the frequency and severity of symptoms during the sleep phase. It is also important for relatives and friends not to leave the affected person alone during the sleep phases. This is also important to avoid depression, frustration and isolation of the patients. Exchanges with other sufferers can be of great benefit.
