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Kleine–Levin syndrome

kleine-levin syndrome, kleine levin syndrome symptoms
Kleine–Levin syndrome KLS, also known as Sleeping Beauty syndrome, is a rare sleep disorder characterized by persistent episodic hypersomnia and cognitive or mood changes Many patients also experience hyperphagia, hypersexuality and other symptoms Patients generally experience recurrent episodes of the condition for more than a decade Individual episodes generally last more than a week but less than a month The condition greatly affects the personal, professional, and social lives of sufferers, but symptoms spontaneously resolve and seldom cause permanent issues The severity of symptoms and the course of the syndrome vary between sufferers Patients commonly have about 20 episodes over about a decade Several months generally elapse between episodes The onset of the condition usually follows a viral infection; several different viruses have been observed to trigger KLS It is generally only diagnosed after similar conditions have been excluded; MRI, CT scans, lumbar puncture, and toxicology tests are used to rule out other possibilities The syndrome's mechanism is not known, but the thalamus is thought to possibly play a role Tomography has shown hypoperfusion in the brains of patients during episodes

KLS is very rare, occurring at a rate of one in a million, which limits research into genetic factors The condition primarily affects adolescent males, though females can also be affected and the age of onset varies There is no known cure, and there is little evidence supporting drug treatment Lithium has been reported to have limited effects in case reports, decreasing the length of episodes and duration between them in some patients Stimulants have been shown to promote wakefulness during episodes, but they do not counteract cognitive symptoms or decrease the duration of episodes The condition is named after Willi Kleine and Max Levin, who described cases of the disease in the early 20th century It was added to the International Classification of Sleep Disorders in 1990

Contents

  • 1 Symptoms
  • 2 Cause
  • 3 Diagnosis
  • 4 Treatment
    • 41 Prevention
  • 5 Prognosis
  • 6 Epidemiology
  • 7 History
  • 8 References
  • 9 Bibliography
  • 10 External links

Symptomsedit

Patients with Kleine–Levin syndrome KLS experience reoccurring feelings of excessive tiredness and prolonged sleep hypersomnia1 In most cases, patients sleep 15 to 21 hours a day during episodes2 Excessive appetite hyperphagia and unusual cravings are present in half to two thirds of cases234 About half of patients, mainly male patients, experience dramatically increased sexual urges hypersexuality53 Several other symptoms usually accompany the syndrome, including marked changes in mood and cognitive ability1 Derealization and severe apathy are present in at least 80 percent of cases6 About one third of patients experience hallucinations or delusions3 Depression and anxiety occur less commonly; one study found them in about 25 percent of patients6 Individuals usually cannot remember what happened during episodes2 Repetitive behaviors and headaches are commonly reported3 Some patients act very childlike during episodes,7 and communication skills and coordination sometimes suffer2 In some cases, the autonomic nervous system malfunctions as well1

Sleep studies of KLS show varying results based on the amount of time the patient is observed Slow wave sleep is often reduced at the beginning of episodes, and REM sleep is reduced near the end8 Conversely, REM sleep is often normal at the beginning, and slow wave sleep is often normal by the conclusion9 Stage two non-rapid eye movement sleep is often interrupted during KLS Studies also show that stage one and three non-rapid eye movement sleep become more efficient when the episodes end10 The Multiple Sleep Latency Test has yielded inconsistent results when given to KLS patients11 In many cases, hours are spent in a withdrawn sleep-like state while awake during episodes8 Most sleep studies have been performed while subject are near the end of their episodes9 Some patients experience brief insomnia and become very happy and talkative after the episode ends2

The first time a patient experiences KLS, it usually occurs along with symptoms that are similar to those of the flu or encephalitis In at least 75 percent of cases, symptoms occur after an airway infection or a fever Viruses observed before the development of the condition include Epstein-Barr virus, varicella zoster virus, herpes zoster virus, Influenza A virus subtypes, and adenovirus Several days after symptoms first occur, patients become very tired5 In cases that occur after an infection, KLS usually starts within three to five days for teenagers and fewer for children12 In other cases, alcohol consumption, head injury, or international travel precede symptoms510 Lifestyle habits, such as lack of sleep and stress, have also been proposed as possible triggers1 First episodes of KLS are preceded by a clear event in about 90 percent of cases4 Recurrences generally do not have clear triggers; only about 15 percent have a precipitating event13

The condition generally disrupts the social lives and academic or profession obligations of sufferers15 Some patients also gain weight during episodes5 The most severe cases cause a long-term impact on mood and cognitive attention5 In rare cases, patients experience long-term memory problems2

In patients with KLS, MRI and CT scans show normal brain morphology When SPECT is performed, hypoperfusion can often be observed in the brain,14 particularly in the thalamic and frontotemporal areas4 The hypoperfusion is significantly diminished between episodes15 Serum biology, c-reactive proteins and leptins, the hormonal pituitary axis, and protein in the cerebral spinal fluid CSF are normal in KLS patients16

Causeedit

It is not known what causes KLS, but several mechanisms have been proposed One possible explanation is hypothalamic or circadian dysfunction1 The thalamus probably plays a role in the out-of-control sleeping,17 and patients with diencephalic–hypothalamic dysfunction caused by tumors experience symptoms similar to those of KLS patients1 Specifically, the medial temporal regions of the thalamus may be involved,18 although examinations of KLS patients have not consistently found abnormalities in this area4 The temporal lobe also appears to play a role in the condition, possibly causing cognitive difficulties The apathy and disinhibition found in some KLS sufferers suggest that the condition may include frontal lobe dysfunction as well The involvement of the thalamus, temporal lobe, and frontal lobe of the brain suggests that there is a multifocal, localized encephalopathy There are also persistent subclinical abnormalities in KLS sufferers17

Another possible explanation concerns the metabolism of serotonin and dopamine An imbalance in the neurotransmitter pathways of these chemicals could play a role1 Viral infections have also been suggested as a possible cause Evidence for their role includes lesions found in autopsies1 CSF samples from KLS patients indicate that the condition has a different cause than influenza-associated encephalopathy12 Triggers of KLS may also affect the blood-brain barrier, which could play a role in the condition17 There is limited evidence of what role hypocretin may play, although it often influences hypersomnia12

Androgen might indirectly block melatonin receptors, possibly by mean of vasodilation, and cause cholinergic abnormalities in some cases of Kleine–Levin syndrome19

Because KLS occurs at a much higher rate in Jews and in some families, it is likely that there is some genetic component in addition to environmental factors17 Genetic studies hold promise for understanding the disease, but they have yielded inconsistent results16 and few patients are available for testing17

Epilepsy and depression do not appear to cause KLS The condition's rapid onset after infections indicates that the immune system is not to blame17

Diagnosisedit

KLS can be diagnosed when there is confusion, apathy, or derealization in addition to frequent bouts of extreme tiredness and prolonged sleep5 The earliest it can be diagnosed is the second episode, this is not common12 The condition is generally treated as a diagnosis of exclusion4 Because KLS is rare, other conditions with similar symptoms are usually considered first14

MRIs can determine if the symptoms are caused by certain brain disorders, stroke, and Multiple Sclerosis Lumbar puncture can determine if there is Encephalitis KLS must be differentiated from substance abuse by toxicology tests14 The use of Electroencephalography EEG can exclude temporal status epilepticus from consideration EEGs are normal in about 70% of KLS patients, but background slowing may sometimes be detected148 In addition, low-frequency high-amplitude waves can be observed during waking hours8

Initially, KLS appears similar to bipolar depression4 Patients with frontal-lobe syndromes and Klüver-Bucy syndrome also display similar symptoms, but these conditions can be differentiated by the presence of brain lesions14 KLS should also be distinguished from very rare cases of menstruation-caused hypersomnia3

Treatmentedit

Several drug therapies have been used on patients with KLS, but none of them have been subject to randomized controlled trials A 2013 Cochrane Review concluded that "No evidence indicates that pharmacological treatment for Kleine-Levin syndrome is effective and safe"1

In several cases, stimulants, including modafinil,4 have been reported to have a limited effect on patients, often alleviating sleepiness1 They can cause behavioral problems,16 but they may pose less issues if used in older patients with mild symptoms20 In some case reports, lithium has been reported to decrease the length of episodes and the severity of their symptoms and to increase the time between episodes1 It has been reported to be effective in about 25 to 60 percent of cases Its use carries the risk of side effects in the thyroid or kidneys Anti-psychotics and benzodiazepines can help alleviate psychotic and anxiety related symptoms, respectively16 Carbamazepine has been reported to be less effective than lithium but more effective than some drugs in its class17 Electroconvulsive therapy is not effective and worsens symptoms4

KLS patients generally do not need to be admitted to hospitals It is recommended that caregivers reassure them and encourage them to maintain sleep hygiene16 It may also be necessary for patients to be prevented from putting themselves in dangerous situations, such as driving20

Preventionedit

Lithium is the only drug that appears to have a preventive effect In two studies of more than 100 patients, lithium helped prevent recurrence of symptoms in 20% to 40% of cases The recommended blood level of lithium for KLS patients is 08-12 mEq/ml It is not known if other mood stabilizers have an effect on the condition20 Anti-depressants do not prevent recurrence1

Prognosisedit

The frequency of KLS episodes can vary from attacks one week in length occurring twice a year to dozens of episodes that follow each other in close succession5 The median duration of KLS episodes is about ten days, but some last several weeks or months A study of 108 patients found an average of 19 episodes over the duration of the disease5 Another study found a median of 35 months between episodes18 Outside of episodes, there is no disturbance in patients' sleep patterns and they are generally asymptomatic52 Patients do not experience the same symptoms in each episode3

About 80 percent of patients are adolescents when they first experience KLS On some occasions though, its first occurrence comes in childhood or adulthood5 In most adolescent-onset patients, symptoms cease by the time they are 30 years old A French study of 108 patients found a median duration of 13 years,5 but a review of 186 cases found a median duration of 8 years18 Unusually young or old patients and those who experience hypersexuality tend to have a more severe course Patients who initially have frequent attacks generally see the disease cease earlier than others5 The condition spontaneously resolves,5 and the patient is considered to be cured if there have been no symptoms for six years4

Epidemiologyedit

Population-based studies of KLS have not been performed Its prevalence is about 1 case per million people4 In France, KLS has a prevalence of 15 per million people It occurs most frequently among Jews in the US and Israel First-degree relatives of people who have suffered from the syndrome are much more likely than the general population to suffer from it, although only in about one percent of cases do family members contract it About 70 to 90 percent of patients are male Patients with the syndrome are more likely than the general population to have genetic disorders, and about a third of people with the syndrome encountered some form of birth difficulty21 In a study of 186 older patients, about ten percent had preexisting psychiatric issues2 One study found that about ten percent of patients had a neurological condition before KLS developed4 The condition does not appear to occur most frequently in one season7

Historyedit

In 1815 there was a report of a young man who showed excessive appetite and prolonged sleep after experiencing a fever; this may have been an early description of the condition1 Another case with similar symptoms was described by Brierre de Boismont in 186218

Five patients with symptoms of persistent sleepiness were described in detail in 1925 by Willi Kleine, a neurologist from Frankfurt This report was followed four years later by details of a similar case by New York-based psychiatrist Max Levin In 1935, Levin published information about several more cases, including one described by Kleine Levin noted that some patients displayed an intense appetite in addition to their persistent tiredness MacDonald Critchley, who first wrote about the condition in 1942,18 described 11 cases he had examined and reviewed 15 other published cases in a 1962 publication In the report, which included patients he had examined in the Royal Navy during World War II,4 he observed that irritability and depersonalization often occurred while patients were awake He named the condition Kleine–Levin syndrome and noted four common traits: hypersexuality, adolescent onset, spontaneous resolution, and compulsive eating18 He believed that the condition only affected males, but later studies showed some female patients22 In the 1970s, several psychoanalytic and psychodynamic explanations for the condition were proposed23 In 1980, a Hawaiian–Caucasian family was found in which nine family members suffered from the condition18

Diagnostic criteria for KLS was established by Schmidt in 1990, and the International Classification of Sleep Disorders further refined them that year1 KLS is classified as a sleep disorder,8 specifically one of recurrent hypersomnia4 Before 2005, hyperphagia and hypersexuality were thought to occur in all cases That was changed with the guidelines published that year, which noted that they did not always occur2

Referencesedit

  1. ^ a b c d e f g h i j k l m n o Oliveira, Conti & Prado 2013
  2. ^ a b c d e f g h i Arnulf, Rico & Mignot 2012, p 920
  3. ^ a b c d e f Arnulf, Rico & Mignot 2012, p 921
  4. ^ a b c d e f g h i j k l m Ramdurg 2010
  5. ^ a b c d e f g h i j k l m n Arnulf, Rico & Mignot 2012, p 919
  6. ^ a b Huang, Lakkis & Guilleminault 2010, p 558
  7. ^ a b Billiard et al 2011
  8. ^ a b c d e Arnulf, Rico & Mignot 2012, p 923
  9. ^ a b Huang, Lakkis & Guilleminault 2010, p 559
  10. ^ a b Gupta, Lahan & Srivastava 2011
  11. ^ Frenette & Kushida 2009, p 364
  12. ^ a b c d Kodaira & Yamamoto 2012
  13. ^ Frenette & Kushida 2009, p 363
  14. ^ a b c d e Arnulf, Rico & Mignot 2012, p 922
  15. ^ Huang, Lakkis & Guilleminault 2010, p 560
  16. ^ a b c d e Arnulf, Rico & Mignot 2012, p 924
  17. ^ a b c d e f g Arnulf, Rico & Mignot 2012, p 925
  18. ^ a b c d e f g Pearce 2008
  19. ^ Brown 1993
  20. ^ a b c Mignot 2012
  21. ^ Arnulf, Rico & Mignot 2012, p 918
  22. ^ Huang, Lakkis & Guilleminault 2010, p 557
  23. ^ Arnulf et al 2005

Bibliographyedit

  • Arnulf, Isabelle; Rico, Thomas; Mignot, Emmanuel 2012 "Diagnosis, Disease Course, and Management of Patients with Kleine-Levin Syndrome" The Lancet Neurology 11 10: 918–28 doi:101016/S1474-44221270187-4 PMID 22995695 
  • Arnulf, Isabelle; Zeitzer, J M; Farber, N; Mignot, Emmanuel 2005 "Kleine–Levin Syndrome: a Systematic Review of 186 Cases in the Literature" Brain 128 12: 2763–76 doi:101093/brain/awh620 PMID 16230322 
  • Billiard, Michel; Jaussent, Isabelle; Dauvilliers, Yves; Besset, Alain 2011 "Recurrent Hypersomnia: a Review of 339 Cases" Sleep Medicine Reviews 15 4: 247–57 doi:101016/jsmrv201008001 PMID 20970360 
  • Brown, DW 1993 "Abnormal fluctuations of acetylcholine and serotonin" Medical Hypotheses 40 5: 309–310 doi:101016/0306-98779390011-e PMID 8350780 
  • Frenette, Eric; Kushida, Clete 2009 "Primary Hypersomnias of Central Origin" Seminars in Neurology 29 4: 354–67 doi:101055/s-0029-1237114 PMID 19742411 
  • Gupta, Ravi; Lahan, Vivekananda; Srivastava, Malini 2011 "Kleine-Levin Syndrome and Idiopathic Hypersomnia: Spectrum Disorders" Indian Journal of Psychological Medicine 33 2: 194–98 doi:104103/0253-717692048 PMC 3271500  PMID 22345850 
  • Huang, Yu-Shu; Lakkis, Clair; Guilleminault, Christian 2010 "Kleine-Levin Syndrome: Current Status" Medical Clinics of North America 94 3: 557–62 doi:101016/jmcna201002011 PMID 20451032 
  • Kodaira, Minori; Yamamoto, Kanji 2012 "First Attack of Kleine-Levin Syndrome Triggered by Influenza B Mimicking Influenza-associated Encephalopathy" Internal Medicine 51 12: 1605–8 doi:102169/internalmedicine517051 PMID 22728499 
  • Mignot, Emmanuel 2012 "A Practical Guide to the Therapy of Narcolepsy and Hypersomnia Syndromes" Neurotherapeutics 9 4: 739–52 doi:101007/s13311-012-0150-9 PMC 3480574  PMID 23065655 
  • Oliveira, Marcio; Conti, Cristiane; Prado, Gilmar 2013 "Pharmacological Treatment for Kleine-Levin Syndrome" Cochrane Database of Systematic Reviews 8: CD006685 doi:101002/14651858CD006685pub3 PMID 23945927 
  • Pearce, J M 2008 "Kleine–Levin Syndrome: History and Brief Review" European Neurology 60 4: 212–4 doi:101159/000148694 PMID 18667831 
  • Ramdurg, Santosh 2010 "Kleine–Levin Syndrome: Etiology, Diagnosis, and Treatment" Annals of Indian Academy of Neurology 13 4: 241–6 doi:104103/0972-232774185 PMC 3021925  PMID 21264130 

External linksedit

  • The KLS Foundation
  • KLS Support UK
  • KLS Life
  • Kleine-Levin syndrome | Mayo Clinic Connect
  • kleine_levin at NINDS

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