What is Sleeping Beauty Syndrome?

What is Sleeping Beauty Syndrome or Kleine-Levin Syndrome?

Sleeping Beauty Syndrome or Kleine-Levin Syndrome- an uncommon disorder - is often categorized by a person's need to sleep excessively for up to 20 hours a day, eat food haphazardly, show some behavioral changes and have a candid sexual drive. It is also know by the name of Kleine-Levin Hibernation Syndrome. This disorder affects males who are in their adolescence. When such people are awake, they may become irritating, lethargic and can lack apathy as well. They may be in a state of confusion or hallucination at all times. Sleeping Beauty Syndrome or Kleine-Levin Syndrome has cyclic symptoms and a person may not show any significant symptoms for a long time after suffering from the disorder. When the symptoms are present, they may last for days or weeks as well. Whilst this is the case, some also show a significant disappearance in the symptoms with age advancements. The episodes may recur in the later stages.

What is Sleeping Beauty Syndrome?

A specific cause that leads to the Sleeping Beauty Syndrome or Kleine-Levin Syndrome is not yet speculated. Researchers are of a notion that this may be due to hereditary factors that are associated with genetic predisposition. It is also believed that the symptoms are related to poor functioning of the brain part which is responsible for appetite, sleep and regulating body temperatures. Some also suggest that this syndrome may be autoimmune.

What are the Causes of Sleeping Beauty Syndrome or Kleine-Levin Syndrome?

The actual cause of Sleeping Beauty Syndrome or Kleine-Levin Syndrome has not been known, but several mechanisms have been anticipated. Circadian or hypothalamic dysfunction may be considered as a possible cause for Sleeping Beauty Syndrome.

Dopamine and serotonin metabolism may also be another possibility for causing this disease. Apart from all this, viral infections and neurotransmitter imbalance can play a significant role in its occurrence.

Triggers of Sleeping Beauty Syndrome or Kleine-Levin Syndrome affect the barrier of blood and brain, which can lead to this disease. The evidences of hypocretin's role-play are limited, but it plays a major role in influencing hypersomnia.

Sleeping Beauty Syndrome or Kleine-Levin Syndrome is observed to affect Jewish families and it might be due to the genetic factors other than the environmental factors. When genetic studies were held, it showed inconsistent results as very few sufferers were available for diagnosis.

Risk Factors for Sleeping Beauty Syndrome or Kleine-Levin Syndrome

The origin of Kleine-Levin Syndrome is not known, however, studies suggest that it may be due to alcohol consumption, viral infections or head trauma. An HLA subtype DQB1*02 and a primary hypothalamic dysfunction may be linked with the disease.

Signs and Symptoms of Sleeping Beauty Syndrome or Kleine-Levin Syndrome

Sleeping Beauty Syndrome or Kleine-Levin Syndrome show its appearance in the adolescents and the symptoms are observed to occur in a range of 2 and 12 times annually. When a single episode occurs, it can last for days or months. At the end of the episode, the patients lack their ability to recollect the instances. The symptoms can recur without any warning in the future.

When the health of a patient is diagnosed between the episodes, it shows normal. There are no registered evidences of physical or behavioral dysfunction. The patients usually lead a normal life and follow a regular pattern. As he or she advances into age, the episodes are less frequently observed and may disappear as well. In some cases, the patients who are in their late fifties have also been affected by the syndrome. Following is a list of symptoms that are observed in patients with Sleeping Beauty Syndrome or Kleine-Levin Syndrome:

  • Hypersomnia or Extreme Sleep: This is a basic symptom of Kleine-Levin Syndrome and is an essential condition speculated for diagnosis. Patients usually sleep from 12 to 24 hours and find it difficult to arouse as well.
  • Uncontrollable Eating (hyperphagia): The patients who are sufferers of Sleeping Beauty Syndrome or Kleine-Levin Syndrome are often subjected to various episodes and their occurrence. They often have cravings that are powerful and show association with junk food or sweets.
  • Aggressive Sexuality: The sexual urge is profound in some patients leading to unsuitable sexual advances at times. This hypersexual behavior includes excessive masturbation, indulgence with multiple partners and bawdy behavior.
  • Emotional Impairment: Patients are often subjected to diminished comprehension, memory problems, poor concentration, confusion, and communication problems. There might also be associated speech impairments like slurred, childish or monosyllabic speech.
  • Altered Approach: Most patients often feel disconnected and are often distorted like living in a dream. Some also experience visual hallucinations or auditory disturbances as well.

Other Symptoms of Sleeping Beauty Syndrome or Kleine-Levin Syndrome may include irritability, migraine headaches, hypersensitivity to light and noise as well as anxiety. Many patients also show certain changes like increased heart rate or elevated body temperature with symptoms of flu.

Tests to Diagnose Sleeping Beauty Syndrome or Kleine-Levin Syndrome

The diagnosis of Sleeping Beauty Syndrome or Kleine-Levin Syndrome can be done when there is apathy, derealization or confusion along with frequent attacks of tiredness and protracted sleep. MRI can be used to determine the exact symptoms which could be caused by Multiple Sclerosis, stroke and other brain disorders. Encephalitis can be determined using lumbar puncture. Kleine-Levin Syndrome must be distinguished from other abuses with the use of toxicology tests. Electroencephalography (EEG) can help in ruling out temporal status epilepticus from the associated symptoms. The result is normal in most patients while a blend of high amplitude and low frequency waves may be observed while waking.

Sleeping Beauty Syndrome or Kleine-Levin Syndrome often show similarity to bipolar depression. Individuals who are suffering from the Klüver - Bucy syndrome and frontal-lobe syndromes also show similar symptoms. The conditions can be distinguished by the appearance of brain lesions. The hypersomnia that is caused due to menstruation should also be very well distinguished using the right means.

The vital criteria for clinical recognition of Sleeping Beauty Syndrome or Kleine-Levin Syndrome are continuous episodes of hypersomnia. Additionally, the patients also have to show at least one amongst the following symptoms during the occurrence of the episodes:

  1. Mood disturbances or cognitive behavior like derealization, irritability, confusion, mutism, delusion, aggressiveness, hallucinations. They are normally present in all the patients.
  2. Compulsive eating with megaphagia.
  3. Inappropriate or bawdy behavior with hyper sexuality
  4. Odd behavior with aggressiveness and irritability at all times

When all such symptoms are associated, it becomes easier to distinguish the disorder without any ambiguities. The treatment should be done only after speculating the right symptoms.

Treatment for Sleeping Beauty Syndrome or Kleine-Levin Syndrome

The treatment for Sleeping Beauty Syndrome or Kleine-Levin Syndrome is indefinite. Waiting-at-home watchfully is advisable than relying on pharmacotherapy. Sleepiness can be treated using pills like modafinil, amphetamines and methylphenidate. This may, however, give rise to other cognitive symptoms and might develop irritability. The closeness in symptoms between Kleine-Levin syndrome and other mood disorders have made it compulsory for the doctors to prescribe carbamazepine and lithium in some cases for its prevention.

The disorder should be distinguished from the periodic reappearance of sleepiness that is associated with the pre-menstrual cycle in teenagers and can be controlled using birth-control tablets. The syndrome should also be set aside from recurrent depression, encephalopathy and psychosis.

Prevention of Sleeping Beauty Syndrome or Kleine-Levin Syndrome

Lithium is the only element or drug that has a preventive effect on Sleeping Beauty Syndrome or Kleine-Levin Syndrome. Studies have shown that the drugs, which consisted of Lithium helped in preventing the symptom recurrence in most cases. The curative properties of other mood stabilizers have not yet known and their effect on the condition has not been determined. Recurrence of Sleeping Beauty Syndrome or Kleine-Levin Syndrome cannot be prevented with anti-depressants.

Prognosis of Sleeping Beauty Syndrome or Kleine-Levin Syndrome

The frequency of episodes decrease with time and their intensity also lowers down within a period of 8 to 12 years.

5 Tips for Managing Sleeping Beauty Syndrome or Kleine-Levin Syndrome

5 Tips for Managing Sleeping Beauty Syndrome or Kleine-Levin Syndrome

  1. Get A Professional Evaluation:

    Sleeping Beauty Syndrome or Kleine-Levin Syndrome is an uncommon disorder. The medical literature does not mention much about this syndrome and you may not witness major cases of sufferers as well. Considering its one-off appearance, a sleep specialist may only provide a correct assessment for the right diagnosis.

  2. Look after Yourself And Others:

    The patients must ensure that the people who are in his or her network should be aware of the behavioral abnormalities and the associated threats. They should be well prepared to tackle all such episodes when the sufferer tries to hurt himself in the event of aggressiveness.

  3. Do Not Agitate The Patient:

    Doctors suggest that the person who is suffering from Sleeping Beauty Syndrome or Kleine-Levin Syndrome should not be awakened when he or she is struck with certain disturbances. You must also prevent them from hurting themselves or others. Therefore, it is better to avoid waking them up from their sleep.

  4. Join a Supportive Group:

    Sleeping Beauty Syndrome or Kleine-Levin Syndrome does not have a particular treatment. An individual suffering from such disorder should be in a support group, where they are aware of the syndrome and its effects on the patient's life. It is quite obvious that the person cannot visit a school or office when such episodes take place.

  5. Talk to Your Physician:

    Patients should indulge in an informative conversation with the physician about the prescribed medications which are related to the Sleeping Beauty Syndrome or Kleine-Levin Syndrome. This will help them in getting a clear understanding about the medications.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: March 25, 2016

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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