Kleine-Levin syndrome: Wikis

  

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Kleine-Levin syndrome
Classification and external resources
ICD-10 G47.8
ICD-9 327.13
OMIM 148840
DiseasesDB 29520
MeSH D017593

Kleine-Levin Syndrome or KLS (also known as Sleeping Beauty Syndrome) is a neurological disorder characterized by recurring periods of excessive amounts of sleep and altered behavior. At the onset of an episode the patient becomes drowsy and sleeps for most of the day and night (hypersomnolence), waking only to eat or go to the bathroom. When awake, the patient’s whole demeanor is changed, often appearing “spacey” or childlike. They also experience confusion, disorientation, complete lack of energy (lethargy), and lack of emotions (apathy). Individuals are not able to attend school or work or care for themselves. Most are bedridden, tired, and uncommunicative even when awake.

Most patients report that everything seems out of focus, and that they are hypersensitive to noise and light. In some cases, food cravings (compulsive hyperphagia) are exhibited. Instances of uninhibited hypersexuality during episodes (mostly males) and instances of depression (mostly females) have also been reported.

Affected individuals may go for a period of weeks, months or even years without experiencing any symptoms, and then they reappear with little warning. In between episodes those diagnosed with KLS appear to be in perfect health with no evidence of behavioral or physical dysfunction.

The cause of Kleine-Levin syndrome is not known. Thus, family support and education are the best management currently available.[1] [2]

Contents

Etymology

It is named for Willi Kleine and Max Levin. [3][4]

Presentation

Individual sufferers may often become irritable, lethargic, and/or apathetic. KLS patients may appear disoriented and report hallucinations. Symptoms are cyclical; with days to weeks (even up to months) of suffering interspersed by weeks or months (even up to years) symptom-free. Although resolution of the disorder may occur for some in later life, this is not universal.

Causes

While some researchers speculate that there may be a hereditary predisposition, others believe the condition may be the result of an autoimmune disorder.[5] Both proposals need not be mutually exclusive with the result being a malfunction of the portion of the brain that helps to regulate functions such as sleep, appetite, and body temperature (hypothalamus).[6] Recent studies also suggest that there may be a link to a deficiency of dopamine transporter density in the lower striatum. [7]

Demographics

A 2008 study of 108 KLS patients found that the majority of cases were present in adolescent males, with a mean sex ratio of 3:1. The average age of onset was 15.7 years, with 81.7% experiencing their first episode between 10 and 20 years of age. Age ranged from 6 to 59 years when patients experienced their first episode. Females tended to be slightly older than males at first onset, even though they tended to experience puberty earlier. In the US population, KLS presents in Caucasians with three times the expected frequency, and with six times the expected frequency in those of Jewish heritage.[8]

First onset

About 90% of KLS patients associate an event with their first KLS episode. This event is most often a type of infection or cold, but may also be associated with stress, sleep deprivation, alcohol or marijuana use, physical exertion, traveling, or head trauma. This causes some to think that there may be some immunological link, but so far there is no clear proof of this. Only a quarter of KLS patients report events triggering subsequent episodes.[9][10]

Symptoms

Hypersomnia is a primary symptom of KLS, and is present in all subjects. Subjects are often treated initially for a sleeping disorder. During a KLS episode, subjects often spend 18 hours a day asleep. Another defining symptom of KLS is an altered mental state during the episode. Subjects are hard to arouse from this sleep, and are irritable or aggressive when prevented from sleeping. Subjects also frequently show cognitive impairment[11], and can show confusion, amnesia for the event, hallucinations, delusions, or experience a dream-like state. About 75% of KLS patients experience changes in eating behavior during episodes, with the majority of these exhibiting megaphagia. Reports describe patients who will eat anything placed in front of them with very little discrimination. Reports also describe consumption of excessive amounts of food, but KLS is distinct from bulimia since no purging occurs after binging. Almost half of KLS patients also experience some sort of hypersexual behavior during the event, including promiscuity, excessive masturbation, inappropriate sexual advances, and other risqué, atypical behavior. Hypersexual behavior is more common in males than females, and is associated with a much longer disease course.[12] Other unusual compulsions reported during the event are also not uncommon.[13]

Genetics

There are no known genetic markers for KLS. There is no protein or antibody markers available for a positive diagnosis of KLS. Some researchers have explored hereditable immunity traits that may predispose individuals for KLS. One such trial focused on a family of twelve in which the father and five children were all affected by KLS. Human leukocyte antigen (HLA) typing revealed that the father was identical to two of his children that were both affected by KLS. Furthermore, all of his affected children shared one-half of his HLA antigens.[14]

Diagnosis

Diagnosis of KLS is very difficult since there are no symptoms that allow for a positive diagnosis. KLS is instead a diagnosis of exclusion, where a doctor must first eliminate a long list of other conditions that could mimic the symptoms. Because hypersomnia is the primary symptom, many patients are initially treated for a sleeping disorder. Potential KLS patients are often referred to an endocrinologist early on to check for metabolic problems including diabetes and hypothyroidism. Several other disorders can also mimic KLS symptoms, but many can be positively diagnosed by MRI—including ones caused by a lesion, tumor, or inflammation. Multiple sclerosis also has neurological components that can mimic the symptom profile for KLS.

People with KLS are often mistakenly diagnosed with a psychiatric disorder. The periods of somnolence, hyperphagia, and withdrawal can mimic severe depression, and some people experience a brief period of high energy following these episodes which looks like a manic episode, so that some patients are incorrectly diagnosed with bipolar disorder. There can also be a number of other mood symptoms or perceptual disturbances which mimic primary psychiatric disorders. Narcolepsy and Klüver-Bucy syndrome can also produce similar symptom profiles. Before a final diagnosis can be made, all other possibilities must be carefully excluded, and the cluster of symptoms must fit with those commonly observed in KLS patients.[15]

Treatment

There is no definitive treatment for Kleine-Levin syndrome. Stimulants, including amphetamines, methylphenidate, and modafinil, administered orally, can be used to treat sleepiness, but unfortunately do not improve sluggish cognition or other elements of the altered mental state.[16] There are some similarities between Kleine-Levin syndrome and bipolar disorder, and lithium[17] and carbamazepine are reported to be beneficial in some cases in warding off or shortening episodes. Responses to treatment have often been limited. This disorder needs to be differentiated from cyclic re-occurrence of sleepiness during the premenstrual period in teenage girls that may be controlled with hormonal contraception.

Frequency and duration of episodes

A 2005 study of 168 KLS patients reported that in subjects where the disease terminates, the average age is 23 and the median duration is 4 years. They reported no correlation between age at onset and disease duration. Patients experienced an average of 12 episodes lasting an average of 12 days, although the range of symptoms reported varied from 2-130 episodes and lasted between 2.5 and 80 days. Subjects experienced an average duration of 6 months between episodes, but this ranged from .5 to 72 months. Subjects typically experienced less frequent and less intense attacks towards the end of the disease course, and the subject is considered cured if they do not experience an episode for 6 or more years. The median disease duration is 10 years in patients without hypersexuality, but 21 years in patients with hypersexuality. The duration also appears to be longer for patients initially struck as adults.[18]

Prognosis

Living with KLS can be a very serious affair. Many patients report depression during one or more episodes. While the major symptoms may seem innocent enough to some (copious amounts of sleeping, eating, and sexual behavior) the effects can be very debilitating. At very least, KLS makes it difficult to maintain a normal job—and in some cases criminal charges have resulted from unrestrained sexual behavior. Periodic binges associated with the episode can lead to weight gain, and KLS patients often exhibit an above-average BMI, but no mention of obesity is made in the literature. KLS patients also end up suffering from other’s psychosocial stigmas because they don’t understand some of the unusual activities resulting during an episode. Many patients report embarrassing episodes from early in their adolescence before KLS was considered a viable diagnosis.[19] Although the disorder is not fatal, so people can live with it. It can mess up their lives sometimes if the excessive sleepiness interferes with (school, jobs etc).

In many cases, the disorder disappears as mysteriously as it appears; often when patients reach their twenties. Of the patients that have been studied, more than 90% will outgrow KLS symptoms and even those who have some degree of it left after ten years, it is usually much, much milder.

References

  1. ^ Kleine-Levin Syndrome Foundation
  2. ^ I. Arnulf, L. Lin, N. Gadoth, J. File, M. Lecendreux, P. Franco, J. Zeitzer, B. Lo, J. H. Faraco, E. Mignot. Kleine-Levin syndrome: A systematic study of 108 patients. Ann Neurol, 2008 Apr; 63(4): 482-493.
  3. ^ W. Kleine. Periodische Schlafsucht. Monatsschrift für Psychiatrie und Neurologie, 1925, 57:285-320.
  4. ^ M. Levin. Periodic somnolence and morbid hunger: A new syndrome. Brain, Oxford, 1936, 59:494-504.
  5. ^ Kleine-Levin syndrome - Center for Narcolepsy - Stanford University School of Medicine
  6. ^ R. Poryazova, B. Schnepf, P. Boesiger, C. L. Bassetti. Magnetic resonance spectroscopy in a patient with Kleine-Levin syndrome. J Neurol, 2007, Oct, 254(10): 1445-1446.
  7. ^ M. Q. Hoexter, M. C. Shih, D. D. Mendes, C. Godeiro-Junior, A. C. Felicio, Y. K. Fu, S. Tufik, R. A. Bressan. Lower dopamine transporter density in an asymptomatic patient with Kleine-Levin syndrome. Acta Neurol Scand, 2008, May, 117(5): 370-373.
  8. ^ I. Arnulf, L. Lin, N. Gadoth, J. File, M. Lecendreux, P. Franco, J. Zeitzer, B. Lo, J. H. Faraco, E. Mignot. Kleine-Levin syndrome: a systematic study of 108 patients. Ann Neurol, 2008 Apr; 63(4): 482-493.
  9. ^ I. Arnulf, L. Lin, N. Gadoth, J. File, M. Lecendreux, P. Franco, J. Zeitzer, B. Lo, J. H. Faraco, E. Mignot. Kleine-Levin syndrome: A systematic study of 108 patients. Ann Neurol, 2008 Apr; 63(4): 482-493.
  10. ^ I. Arnulf, J. M. Zeitzer, J. File, N. Farber, E. Mignot. Kleine-Levin syndrome: a systematic review of 186 cases in the literature. Brain, 2005 Dec; 128(Pt 12): 2763-2776.
  11. ^ A. M. Landtblom, N. Dige, K. Schwerdt, P. Säfström, G. Granérus. Short-term memory dysfunction in Kleine-Levin syndrome. Acta Neurol Scand, 2003, Nov, 108(5): 363-367.
  12. ^ C. H. Schenck, I. Arnulf, M. W. Mahowald. Sleep and sex: What can go wrong? A review of the literature on sleep related disorders and abnormal sexual behaviors and experiences. Sleep, 2007, Jun 1, 30(6): 683-702.
  13. ^ L. P. Justo, H. M. Calil, S. A. Prado-Bolognani, M. Muszkat. Kleine-Levin syndrome: Interface between neurology and psychiatry. Arq Neuropsiquiatr, 2007, Mar, 65(1): 150-152.
  14. ^ A. S. BaHammam, M. O. GadElRab, S.M. Owais, K. Alswat, K. D. Hamam. Clinical characteristics and HLA typing of a family with Kleine-Levin syndrome. Sleep Med, 2008 Jul; 9(5): 575-578.
  15. ^ W. M. Fisher MD. Telephone INTERVIEW. 6 Nov, 2008.
  16. ^ Kleine-Levin Syndrome Information Page: National Institute of Neurological Disorders and Stroke (NINDS)
  17. ^ F. Muratori, N. Bertini, G. Masi. Efficacy of lithium treatment in Kleine-Levin syndrome. Eur Psychiatry, 2002, Jul, 17(4): 232-233.
  18. ^ I. Arnulf, J. M. Zeitzer, J. File, N. Farber, E. Mignot. Kleine-Levin syndrome: A systematic review of 186 cases in the literature. Brain, 2005 Dec; 128(Pt 12): 2763-2776.
  19. ^ C. H. Schenck, I. Arnulf, M. W. Mahowald. Sleep and sex: What can go wrong? A review of the literature on sleep related disorders and abnormal sexual behaviors and experiences. Sleep, 2007, Jun 1, 30(6): 683-702.

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