Narcolepsy is a serious, lifelong, disabling disorder characterized by a tetrad of symptoms including excessive daytime sleepiness, cataplexy, hypnagogic hallucination, and sleep paralysis. We experienced a 14 year old boy with a narcolepsy diagnosed by typical clinical symptoms above mentioned and multiple sleep latency test. A brief review of the related literatures was also made.
Adolescent ; Cataplexy ; Hallucinations ; Humans ; Male ; Narcolepsy* ; Sleep Paralysis

Humans ; Cataplexy/drug therapy* ; Sleep, REM ; Dopamine ; Narcolepsy/drug therapy*

We report two patients with frequent isolated sleep paralysis (SP). They have neither cataplexy nor daytime sleepiness, and there was no HLA DR2 or DQ1 association. We elicited one episode of sleep paralysis from a patient by a sleep interruption schedule. The polysomnographic recording during SP showed abundant alpha rhythm with persistent atonia, which indicate a concurrence of REM-atonia and wakefulness. The frequency of SP was markedly reduced by a use of clomipramine
Alpha Rhythm ; Appointments and Schedules ; Cataplexy ; Clomipramine ; Humans ; Polysomnography ; Sleep Paralysis* ; Sleep, REM ; Wakefulness

Childhood narcolepsy is one of the underdiagnosed diseases even the first symptoms often appear in childhood. Making diagnosis through history taking is not always easy because the symptoms of childhood narcolepsy are different from those of adulthood. Diagnostic laboratory tests such as sleep studies, tests for human leukocyte antigens, cerebrospinal fluid hypocretin measurement should be considered when the child has excessive daytime sleepiness without cataplexy. Treatment approach should be start as early as possible to avoid secondary academic, emotional difficulties. Both pharmacological and non-pharmacological management, and close cooperation between parents and school teachers should be maintained. In the near future, childhood narcolepsy can be a key to understand the pathogenesis of narcolepsy.
Cataplexy ; Child ; HLA Antigens ; Humans ; Intracellular Signaling Peptides and Proteins ; Narcolepsy ; Neuropeptides ; Parents ; Orexins

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

A 5-year-old, castrated male, Maltese was presented with history of acute flaccid paralysis. The dog was presented with sudden loss of muscle tone and involuntary movements of hind limbs. Neurologic examination revealed reduced postural reaction in the bilateral hind limbs. MRI of brain showed moderate hydrocephalus, but other examination results were normal. Based on the characteristic episodes and examination results, canine cataplexy was suspected. Treatment was initiated with clomipramine as cataplexy control. Clinical signs resolved with 3-month medication. This case demonstrates therapeutic diagnosis of cataplexy. To the author's knowledge, this is the first report of cataplexy treating with clomipramine.
Animals ; Brain ; Cataplexy ; Clomipramine ; Dogs ; Dyskinesias ; Extremities ; Humans ; Hydrocephalus ; Male ; Muscles ; Narcolepsy ; Neurologic Examination ; Paralysis

INTRODUCTION: Excessive daytime sleepiness and cataplexy are key features of narcolepsy. Modafinil is psychostimulant used in the treatment of narcolepsy. In this study, we evaluated effects of modafinil on nocturnal sleep structure and sleep latency in multiple sleep latency test and clinical features. METHODS: Twelve narcoleptic patients (7 male, age: 22.9 +/- 2.6 yrs) were participated in the study. All of them had done nocturnal polysomnography (nPSG), multiple sleep latency test (MSLT), clinical symptoms scales and have repeated same procedure after taking 200 mg of modafinil. We have done linear mixed model analysis to describe effects of group, medication and nap time on these measures. RESULTS: Modafinil did not affect clinical scales except PSQI which had been reduced after medication. In this study, Modafinil reduced total sleep time, sleep efficiency and increased wake after sleep onset and percent of arousal during sleep in nocturnal polysomnography and prolonged mean sleep latency in multiple sleep latency tests in both group. DISCUSSION: Modafinil has stimulant effect of central nervous system but its effect on night sleep is less than other psychostimulants such as methylphenidate. We ascertained that modafinil affected total sleep time, sleep efficiency and percent of wake during sleep but did not effect on sleep structure. Modafinil was effective in the management of day time sleepiness. Modafinil can enhance alertness of control group without day time sleepiness.
Arousal ; Cataplexy ; Central Nervous System ; Humans ; Male ; Methylphenidate ; Narcolepsy* ; Polysomnography ; Weights and Measures

Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, hypnagogic hallucinations and disrupted nocturnal sleep. We report a patient with a diagnosis of schizophrenia who was subsequently diagnosed as narcolepsy. A case of a 22-year-old woman with concomitant narcolepsy and psychotic symptoms was discussed from a perspective of the diagnosis and therapeutic challenges, particularly on pharmacological treatment dealing with issues raised by their coexistence.
Cataplexy ; Diagnosis ; Female ; Hallucinations ; Humans ; Narcolepsy ; Schizophrenia ; Sleep Paralysis ; Young Adult

Narcolepsy with cataplexy (NC) is associated with hypocretin deficiency, and is thought to be an autoimmunity condition. The mean age at onset is estimated to be in the early 20s. Recent papers have addressed the response to immunotherapies in NC, with challenging results. We report a case of late-onset NC in a patient who did not benefit from early intravenous high-dose immunoglobulin (IVIg) therapy. This is the first reported attempt at using IVIg to treat an NC patient in Korea.
Autoimmunity ; Cataplexy ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Immunotherapy ; Intracellular Signaling Peptides and Proteins ; Korea ; Narcolepsy ; Neuropeptides ; Orexins

OBJECTIVES: Narcolepsy is a sleep disorder, characterized by excessive daytime sleepiness, cataplexy, sleep paralysis and hypnagogic hallucination. Among these symptoms, cataplexy is one of the most pathognomonic symptoms in narcolepsy. This study was designed to investigate the clinical features, frequency of DQB1*0602 and CSF hypocretin levels in Korean narcoleptics with cataplexy to compare with those who have not cataplexy. METHODS: From August 2003 to July 2005, we selected 72 patients who have narcolepsy confirmed by nocturnal polysomnography and multiple sleep latency test (MSLT) as well as their history and clinical symptoms at Sleep Disorders Clinic of St. Vincent's Hospital, Catholic University of Korea. Patients were divided into 56 cataplexy-positive group (narcolepsy with cataplexy group) and 12 cataplexy-negative group (narcolepsy without cataplexy group). HLA typing was done in all patients for the presence of DQB1*0602, and patients received spinal tapping to measure the level of CSF hypocretin. Clinical variables were examined by semi-structured interview for narcolepsy patients. RESULTS: 1) In cataplexy-positive group, compared with cataplexy-negative group, the frequency of HLA-DQB1*0602 was found to be significantly increased (50 subjects, 89.3% vs. 8 subjects, 50.0%)(p=0.000). 2) In 48 out of 56 cataplexy-positive patients (85.7%), hypocretin levels were decreased (< or =110 pg/ml) or were below the detection limit of assay (<40 pg/ml). However, only 6 out of 16 cataplexy-negative patients (37.5%) exhibited decreased hyopcretin level. The difference between two groups were statistically significant (p=0.000). 3) Cataplexy-positive group, compared to cataplexy-negative group, reported more frequent hypnagogic hallucinations (36 subjects, 64.3% vs. 4 subjects, 25.0%)(p=0.005). However, there were no significant differences in frequency or severity of daytime sleepiness, sleep paralysis and demographic data. 4. In nocturnal polysomnography and MSLT findings, there were no significant differences in all sleep parameters between two groups. CONCLUSION: Higher frequency of HLA-DQB1*0602, and lower hypocretin levels in cataplexy-positive groups than catapelxy-negatives suggest that narcoleptics with cataplexy might be a etiologically different disease entity from narcoleptics without cataplexy. Additionally, Current criteria prevail for the diagnosis of narcolepsy need to be reclassified according to the presence of cataplexy or not.
Cataplexy* ; Diagnosis ; Hallucinations ; Histocompatibility Testing ; Humans ; Korea ; Limit of Detection ; Narcolepsy* ; Polysomnography ; Sleep Wake Disorders ; Sleep Paralysis ; Spinal Puncture ; Orexins