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*

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

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

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

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

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

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

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

OBJECTIVES: Narcolepsy with cataplexy is a rare chronic sleep disorder characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, and hypnagogic/hypnopompic hallucinations. The aims of the present study were comparing the health-related quality of life (HR-QOL) of patients with type I and type II narcolepy patients, and determining the factors that influence the HR-QOL in narcolepsy patients. METHODS: All patients performed night polysomnography (PSG) and multiple sleep latency test (MSLT). HR-QOL and the severity of subjective symptoms were evaluated using various questionnaires, including the Korean versions of the Medical Outcome Study Short Form-36, the Pittsburg Sleep Quality Index-Korean version, the Korean version Epworth Sleepiness Scale, and the Korean version Beck Depression Inventory-2. RESULTS: We enrolled 21 type I narcolepsy patients and 27 type II patients. Type I patients had short rapid eye movement (REM) latency on night PSG and more sleep onset REM periods on MSLT. The total score of HR-QOL was worse in patients with type I narcolepsy than in the type II narcolepsy patients. There was association between the severities of excessive daytime sleepiness, depression and the degree of worsening of QOL. CSF hypocretin level had no correlation with the scores of HR-QOL. CONCLUSIONS: These findings demonstrate that type I narcolepsy patients are sleepier, depressive, and have more burden on the HR-QOL. And the impairment in QOL of narcolepsy patients is related to the degree of excessive daytime and depressive mood.
Cataplexy ; Depression ; Hallucinations ; Humans ; Narcolepsy* ; Outcome Assessment (Health Care) ; Polysomnography ; Quality of Life* ; Sleep Paralysis ; Sleep Wake Disorders ; Sleep, REM