Diagnosis, disease course, and management of patients with Kleine-Levin syndrome

@article{Arnulf2012DiagnosisDC,
  title={Diagnosis, disease course, and management of patients with Kleine-Levin syndrome},
  author={Isabelle Arnulf and Thomas J. Rico and Emmanuel Mignot},
  journal={The Lancet Neurology},
  year={2012},
  volume={11},
  pages={918-928}
}
Kleine-Levin syndrome is a rare sleep disorder that mainly affects adolescents and is characterised by relapsing-remitting episodes of severe hypersomnia, cognitive impairment, apathy, derealisation, and psychiatric and behavioural disturbances. Boys are more frequently affected than girls. Just over half of patients have hyperphagia, are hypersexual (mainly boys), or have depressed mood (mainly girls), and 30% become anxious, delusional, and have hallucinations. Although some symptoms are… 
Kleine-Levin syndrome: A neuropsychiatric disorder.
TLDR
Patients' cognitive and psychiatric status should be regularly checked during asymptomatic periods, as 20-40% develop long-term mild cognitive impairment or mood disorders.
Kleine-Levin Syndrome: An Overview and Relevance to Nursing Practice.
TLDR
Kleine-Levin syndrome is a neuropsychiatric sleep disorder primarily affecting adolescent males and has been shown to impact activities of daily living, usurping an adolescent of his/her social relations with peers, experiences, and time.
Kleine-Levin Syndrome.
TLDR
Stimulants are poorly beneficial during episodes, whereas lithium and valproate help reducing the episodes frequency and duration, and brain functional imaging is often abnormal.
Lithium treatment of Kleine-Levin syndrome
TLDR
Early manifestation of Kleine-Levin syndrome, combined with hypersexuality during symptomatic phases, seems to be a predictor for a long course of the disease, which can last from 1 to 27 years.
Feeling unreal: a functional imaging study in patients with Kleine-Levin syndrome.
TLDR
Because the parieto-temporal junction is involved in cross-modal association between somatosensory (body knowledge), auditory and visual information, the robust hypoperfusions and correlations observed in this area may underlie the striking derealization reported by patients during episodes.
Kleine–Levin syndrome with comorbid iron deficiency anemia
TLDR
The treatment of IDA along with modafinil decreased the severity of symptoms and shortened the hospital stay during episodes and might be the first case report of KLS with comorbid IDA.
L'enfant et l'adolescent hypersomniaque : diagnostic et prise en charge Hypersomnia in childhood and adolescence : diagnosis and management
Narcolepsy with cataplexy is a rare but long-lasting and disabling disorder with onset often in childhood. Excessive daytime sleepiness, uncontrollable sleep attacks with per- iods of hyperactivity,
Between Neurology and Psychiatry, a Difficult Preliminary Diagnosis ofKleine-levin Syndrome: Case-report of a Young Girl
Kleine-Levin syndrome appears suddenly, sometimes without a triggering factor or sometimes being precipitated by an infection (38.2%), head trauma (9%) or acute consumption of alcohol (5.4%) [2-5].
Kleine–Levin syndrome elicited by encephalopathy with reversible splenial lesion
TLDR
A 13‐year‐old girl who presented with drowsiness triggered by influenza A as the first episode was diagnosed with Kleine–Levin syndrome, a rare sleep disorder characterized by repetitive episodes of hypersomnia between asymptomatic periods.
Emerging psychiatric disorders in Kleine‐Levin syndrome
TLDR
Female sex, longer disease course, longer time incapacitated and more frequent psychiatric symptoms during episodes and no family or personal history of psychiatric disorders were associated with emerging psychiatric disorders.
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References

SHOWING 1-10 OF 89 REFERENCES
The Kleine-Levin syndrome. Report of a case and review of the literature.
TLDR
It is important that the diagnosis is suspected early, especially in adolescent males who present with recurrent episodes of somnolence, increased appetite, and abnormal behavior, since it most often represents a benign and self-limited entity and does not warrant extensive investigations or treatment.
The Kleine-Levin syndrome - effects of treatment with lithium -.
TLDR
In KLS with a high frequency of episodes and severe behavioural changes lithium may become a treatment option, and statistical modelling showed that the risk for a relapsing episode under maintenance of lithium drops per months of therapy from 100 % to 93 %, and furthermore that the Maintenance of lithium shortens the mean duration of episodes to 19 %.
Síndrome de Kleine-Levin: aportaciÓn diagnÓstica de la SPECT cerebral
TLDR
The findings from the brain SPECT confirmed a non dominant hypoperfusion of the frontal lobe, which could result from a phenomenon of diaschisis brought about by a diencephalic dysfunction.
Kleine-Levin syndrome: a systematic review of 186 cases in the literature.
TLDR
In conclusion, KLS is a unique disease which may be more severe in female and secondary cases, and lithium (but not carbamazepine or other antiepileptics) had a higher reported response rate for stopping relapses when compared to medical abstention.
Familial Recurrent Hypersomnia: Two Siblings with Kleine-Levin Syndrome and Menstrual-Related Hypersomnia
TLDR
The male sibling with Kleine-Levin syndrome was easily controlled with carbamazepine in low doses, but his sister could be only efficaciously treated with oral contraceptives, suggesting that hormonal mechanisms could be implicated in the phenotypical expression of this sleep disorder.
Kleine Levin syndrome (KLS) in young females.
TLDR
In 4 patients, hypersomnolence was accompanied by hyperphagia and hypersexuality, while in the remaining 4, recurrent hypersomnia was the only symptom, and all patients enjoyed good health, with no evidence of behavioral or endocrine dysfunction.
Monozygotic twins concordant for Kleine-Levin syndrome
TLDR
Observations suggest that Kleine-Levin syndrome may be due to genetic and autoimmune processes, although etiologic relationship to specific HLA type remains controversial.
A case of PANDAS with Kleine-Levin type periodic hypersomnia.
We report on an 11-year-old girl, presenting with clinical features suggesting both pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) and Kleine-Levin
SPECT findings in the Kleine-Levin syndrome.
TLDR
Hypoperfusion of the thalamus is a consistent finding during the symptomatic period, but perfusion abnormalities may persist even during the asymptomatic period.
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