Narcolepsy is characterized by excessive daytime sleepiness, cataplexy, sleep paralysis and hypnagogic hallucinations. Only a few studies have focused on non-rapid eye movement (NREM) and REM parasomnias in narcolepsy. We report a narcolepsy without cataplexy patient presenting parasomnia as an initial symptom. A 18-year-old boy was admitted to hospital for abnormal behavior of sitting up during sleep over 2 years. He had a symptom of lethargy without cataplexy and subjective excessive daytime sleepiness, but his family found him often asleep during daytime. He underwent 3 times of polysomnography (PSG) including 1 multiple sleep latency test (MSLT) after the last PSG. The last PSG showed 1 episode of abrupt sitting. Three sleep REM onset period was observed in MSLT which was not detect in PSG. Parasomnia as an initial symptom of narcolepsy is a rare clinical entity. The MSLT may be useful in the evaluation of patients with parasomnia and unexplained hypersomnia.
Adolescent ; Cataplexy ; Disorders of Excessive Somnolence ; Eye Movements ; Hallucinations ; Humans ; Lethargy ; Male ; Narcolepsy ; Parasomnias ; Polysomnography ; Sleep Arousal Disorders ; Sleep Paralysis

Narcolepsy is a central neurologic system disease. It begins early in life with disabling symptoms including excessive daytime sleepiness, cataplexy, sleep paralysis, hypnagogic hallucination and nocturnal sleep fragmentation. Patient with typical symptoms of narcolepsy is diagnosed by objective data from nocturnal polysomnography and multiple sleep latency tests. Narcolepsy is controlled with various medications. Nowadays, modafinil with favorable side effects profiles compared with traditional stimulant is mainly used. Gamma hydroxyl butyrate is effective in cataplexy. Cataplexy is also controlled with antidepressant such as Venlafaxine, SSRI, and TCA. As the knowledge of pathophysiology of narcolepsy expands, new treatment including immunological method, application of hypocretin and histamine systems have been tried.
Benzhydryl Compounds ; Butyrates ; Cataplexy ; Cyclohexanols ; Disorders of Excessive Somnolence ; Hallucinations ; Histamine ; Humans ; Intracellular Signaling Peptides and Proteins ; Narcolepsy ; Neuropeptides ; Polysomnography ; Sleep Deprivation ; Sleep Paralysis ; Orexins ; Venlafaxine Hydrochloride

The authors studied 5 cases of idiopathic CNS hypersomnia who visited Division of Sleep Studies, Seoul National University Hospital in 1995. Detailed medical history was taken and nocturnal polysomnography(NPSG), multiple sleep latency test(MSLT)and human leukocyte antigen(HLA) typing were performed. Neither cataplexy nor hypnagogic hallucination was reported in all cases and in NPSGs, there were tendencies of increased sleep period time and decreased slow wave sleep time. In MSLT, all the subjects showed average sleep latencies less than 8 minutes without sleep-onset rapid eye movement period(SOREMP). In HLA typing, some correlation between idiopathis CNS hypersomnia and HLA DR4 was observed. in contrast to previous reports, overall treatment response with methylphenidate was remarkable. Therefore, the authors suggest that patients suspected of idiopathic CNS hypersomnia be actively evaluated and treated with rather optimistic perspective.
Cataplexy ; Central Nervous System* ; Disorders of Excessive Somnolence* ; Hallucinations ; Histocompatibility Testing ; Humans ; Leukocytes ; Methylphenidate ; Seoul ; Sleep, REM

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

No abstract available.
Disorders of Excessive Somnolence* ; Pituitary Neoplasms*

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

Narcolepsy is chronic devastating disease that characterized by excessive daytime sleepiness, cataplexy, which often precipitated by intense emotion or excitement, hypnagogic, or hypnapompic hallucinations, sleep paralysis and nocturnal disrupted sleep. In child onset narcolepsy, the presentations of narcolepsy can be very variable, making misdiagnosis as seizure disorders or delaying diagnosis as much as several years after disease onset. For the diagnosis of narcolepsy, overnight polysomnography(PSG) and multiple sleep latency test(MSLT) should be evaluated. Test for Cerebrospinal fluid hypocretin(orexin) concentration and human leukocyte antigens(HLA) would be great helpful to confirm the narcolepsy with cataplexy even in early stage of disease in children. The mainstays of treatment are that reducing the excessive daytime sleepiness, preventing the intrusion of the REM related phenomena including cataplexy and consolidating the nighttime sleep. Central nervous system stimulators such as methylphenidate or amphetamine decrease excessive daytime sleepiness and tricyclic antidepressant(TCA) or selective serotonin reuptake inhibitors(SSRI) can prevent cataplexy. Recently, new therapeutic agents such as modafinil and sodium oxybate are emerging in clinical practice with much effectiveness. Counseling for poor school performance, social isolation and depression should be provided. Early diagnosis and treatment can greatly improve the quality of life. Awareness of excessive daytime sleepiness in children or adolescent will allow pediatricians to effectively identify hypersomnia such as narcolepsy.
Adolescent ; Amphetamine ; Benzhydryl Compounds ; Cataplexy ; Central Nervous System ; Child ; Counseling ; Depression ; Diagnostic Errors ; Disorders of Excessive Somnolence ; Early Diagnosis ; Epilepsy ; Hallucinations ; Humans ; Leukocytes ; Methylphenidate ; Narcolepsy ; Quality of Life ; Serotonin ; Sleep Paralysis ; Social Isolation ; Sodium Oxybate

We report a 26 year-old female who initially presented with hypersomnia and visual disturbance with preceding upper respiratory infection. She was diagnosed as neuromyelitis optica spectrum disorder (NMOSD) with the presence of anti-AQP4 antibody. Eight months later, she experienced nausea and vomiting refractory to conventional therapies, which was proved correlated with a lesion of area postrema on brain magnetic resonance imaging. These might be significant clinical manifestations in NMOSD and may widen the clinical spectrum of the disease.
Area Postrema ; Brain* ; Disorders of Excessive Somnolence ; Female ; Humans ; Magnetic Resonance Imaging ; Narcolepsy* ; Nausea ; Neuromyelitis Optica* ; Vomiting*

No abstract available.
Disorders of Excessive Somnolence ; Humans ; Metoclopramide ; Parkinsonian Disorders