Kleine-Levin syndrome is a rare disorder which usually affects adolescent males and is characterized by periodic hypersomnia, hyperphagia and abnormal behavior. This is an unexplained clinical syndrome for which several etiologies have been entertained with no standard treatment is yet available. A 18-year old woman began suffering from recurrent hypersomnia, hyperphagia, and behavioral disturbances such as irritability, derealization, and amnesia. She was normal between the episodes and diagnosed as Kleine-Levin syndrome. In the course of about two years she had 11 episodes and the mean interval between the episodes was 52.8+/-16.7 days. After application of amantadine, there were two mild episodes and then she had no episodes for more than 6 months. This case suggests the possible role of amantadine in the treatment of Kleine-Levin syndrome.
Adolescent ; Amantadine* ; Amnesia ; Depersonalization ; Disorders of Excessive Somnolence ; Female* ; Humans ; Hyperphagia ; Kleine-Levin Syndrome* ; Male

Kleine-Levin syndrome (KLS) is a rare disorder and is diagnosed by recurrent episodes of hypersomnia, hyperphagia, and neurobehavioral dysfunctions. We present a case of a male 19 year-old with seven episodes of the above symptoms. All episodes occurred after a respiratory infectious illness and spontaneously resolved after a few days or a few weeks. A polysomnography showed prolonged sleep latency and decreased REM sleep (14.6%). A multiple sleep latency test revealed a slightly short mean sleep latency (8min 7sec) and three sleep-onset REM episodes in a series of four sleep latency tests across a one day period. The electrophysiological features of previously reported were reviewed.
Disorders of Excessive Somnolence ; Humans ; Hyperphagia ; Kleine-Levin Syndrome* ; Male ; Polysomnography ; Sleep, REM ; Young Adult

Kleine-Levin syndrome (KLS) is characterized by recurring episodes of hypersomnia, megaphagia, and abnormal behavior. We report two cases of KLS. Two boys, aged 18 (case 1) and 17 (case 2), had recurrent episodes of hyper-somnolence with compulsive eating or drinking and hypersexuality for several years. HLA-DR typing was HLA-DR3 and 13 in case 1 and HLA-DR4 and 10 in case 2. Case 1 showed hypersomnia with early onset of REM sleep on MSLT and frequent frontal intermittent rhythmic delta activity on EEG. Both cases showed no abnormalities on brain MRI. HLA-DR typing facilitates differentiation between KLS and narcolepsy by the absence of HLA-DR2.
Brain ; Disorders of Excessive Somnolence ; Drinking ; Eating ; Electroencephalography ; HLA-DR Antigens ; HLA-DR2 Antigen ; HLA-DR3 Antigen ; HLA-DR4 Antigen ; Kleine-Levin Syndrome* ; Magnetic Resonance Imaging ; Narcolepsy ; Sleep, REM

BACKGROUNDKleine-Levin syndrome (KLS) is a rare sleep disorder characterized by recurrent episodes of hypersomnia. Polysomnographic (PSG) researches of KLS have been reported only in few publications in the past decades. This study aimed to investigate the characteristics of PSG of KLS.

METHODSThis study, which was conducted from March 2010 to July 2014, included seven patients diagnosed with KLS in the Sleep and Wake Disorder Center of Huashan Hospital, Fudan University (Shanghai, China). PSG and multiple sleep latency tests (MSLT) were performed during their episodes and the results were evaluated.

RESULTSFive of the seven patients were males. The mean age at KLS onset was 15.6 ± 3.6 years. The number of episodes ranged from 2 to 7. The duration of episodes lasted from 4 to 11 days. The sleep architecture and proportion were normal in most of the patients. The average value of mean sleep latency was 6.9 ± 4.1 min. No sleep-onset rapid eye movement (SOREM) was detected in three of the patients, whereas one patient experienced one period of SOREM, and such episodes occurred twice in other two patients.

CONCLUSIONSWe found that sleep architecture and proportion were normal in most KLS patients. However, the results of PSG and MSLT had no specificity for KLS patients.

Adolescent ; Child ; China ; Female ; Humans ; Kleine-Levin Syndrome ; diagnosis ; physiopathology ; Male ; Polysomnography ; methods ; Retrospective Studies ; Sleep Wake Disorders ; physiopathology ; Sleep, REM ; physiology