• Korean Journal of Veterinary Research
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• v.53 no.2
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• pp.129-131
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• 2013

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.

• Sleep Medicine and Psychophysiology
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• v.17 no.2
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• pp.63-68
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• 2010

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.

• Sleep Medicine and Psychophysiology
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• v.18 no.1
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• pp.17-22
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• 2011

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.

• Korean Journal of Clinical Laboratory Science
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• v.37 no.3
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• pp.216-219
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• 2005

We report a case of narcolepsy. A 25-year-old man has had excessive daytime sleepiness of about 10 years durations. He awakens daily feeling exhausted and continually falls asleep during the day while engaged in such situation like reading and watching television. He has exhibited cataplexy, a sudden loss of muscular tone, brought on by emotion, usually laughter. Polysomnogram revealed increased sleep stage 1, 2 and decreased deep sleep. Multiple sleep latency test (MSLT) showed that sleep latency was 1.33 minutes and there were 3 noted sleep onset rapid eye movement (SOREM) on 5 trials. The epworth sleepiness scale (ESS) was 17/24. Typing of HLA haplotype that was positive for the $DQB1^{\ast}0602$ allele, and hypocretin-1 (orexin A) could not be detected in cerebrospinal fluid (CSF). Brain MRI showed normal image. We diagnosed his case as narcolepsy based on history of cataplexy, and three occurances of SOREM, and positive of HLA haplotype.

• Sleep Medicine and Psychophysiology
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• v.8 no.2
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• pp.107-112
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• 2001

Introduction: Narcolepsy, a sleep disorder characterized by excessive daytime sleepiness and cataplexy, is known to be closely associated with the human leukocyte antigen (HLA) DQB1*0602. Several studies have suggested that HLA-DQB1*0602 is strongly linked with narcolepsy-cataplexy. However, no studies have yet been made on whether HLA DQB1*0602 is associated with Korean patients with narcolepsy. This study was designed to investigate the frequency of HLA-DQB1*0602 of Korean patients with narcolepsy. Methods: Twenty patients were selected (mean age: $28.2{\pm}3.0$, 11 men and 9 women). The patients were confirmed to have narcolepsy by the overnight polysomnography and multiple sleep latency test (MSLT) in addition to their clinical history and symptoms at St. Vincent's Hospital and Korea University Hospital Sleep Disorders Clinic. Any subjects co-morbid with other hypersomnic sleep disorders such as sleep apnea or periodic limb movements during sleep were excluded. Clinical data was collected through a semi-structured interview for narcoleptic patients. All patients and 21 control did HLA typing for the presence of DQB1*0602. Results Obtained were as Follows: 1) Mean sleep latency was 2.4 (${\pm}2.0$ minutes) and mean frequency of sleep-onset REM period was 3.0 (${\pm}1.6$) by MSLT. 2) Characteristic symptoms of narcolepsy investigated were as follows: excessive daytime sleepiness (100%), cataplexy (100%), sleep paralysis (60%), hypnagogic hallucination (70%) and disrupted nocturnal sleep (75%). 3) Strong emotional expression such as laughing (80%) and joking (70%) triggered cataplexy which affects the knee and leg region (80%) and jaw region (30%). 4) HLA-DR2 was found in 90% of patients and 35% in controls. The frequency of HLA-DQB1*0602 in patients and controls was 90%, and 24%, respectively. Conclusions: These results, which exhibit high HLA-DQB1*0602 expression in Korean patients with narcolepsy, suggest that HLADQB1*0602 could be a strong genetic marker in narcolepsy.

• Sleep Medicine and Psychophysiology
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• v.12 no.2
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• pp.133-138
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• 2005

Objectives: Narcolepsy is characterized by excessive sleepiness, cataplexy, sleep paralysis and hypnagogic hallucination. As there have been few researches on narcolepsy symptomatology in adolescents, we examined gender differences and prevalence of narcolepsy tetrad among students attending high school. Methods: Total 20,407 subjects, ages 14-19 years filled out Ullanlinna Narcolepsy Scale (UNS). Subjects whose UNS scores were equal to or more than 14 were interviewed by telephone using semi-structured questionnaire. Variables included questions to evaluate tetrad of narcolepsy. Results: UNS scores were higher in female than male ($11.1{\pm}5.2$ vs. $9.6{\pm}4.5$, p<0.001). Subjects scoring the UNS equal to or more than 14 were 4,535 (22.2% of all the participants), more frequently observed in female than in male (p<0.001). Excessive daytime sleepiness, cataplexy-like symptoms, sleep paralysis and hypnagogic hallucination in subjects of UNS ${\geq}14$ were significantly higher in female subjects than male ones. However, no significant gender difference was observed in the frequencies of severe sleep attack and cataplexy-like symptoms. Sleep paralysis was most frequently reported during sleep. There was significant correlation between sleep paralysis and hypnagogic hallucination (r=0.235, p<0.01). Conclusions: Our findings were that female adolescents complained more frequently narcolepsy symptoms than male subjects. Female adolescents might be more sensitive than male ones to physical complaints such as sleepiness or muscle weakness.

• Sleep Medicine and Psychophysiology
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• v.18 no.1
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• pp.40-44
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• 2011

Narcolepsy is a sleep disorder, which is characterized by excessive daytime sleepiness (EDS) that is typically associated with cataplexy, sleep fragmentation and other REM sleep-related phenomenon such as sleep paralysis and hypnagogic hallucination. Narcoleptic symptoms can be developed from various medical or neurological disorders. A 17-year-old male patient admitted for the evaluation of EDS which started three-month ago. He slept more than 18 hours a day with cataplexy and hypnagogic hallucination. He was obese with body mass index (BMI) of 30.4 kg/$m^2$. After admission he was newly diagnosed to the thyrotoxicosis. T3 391.2 ng/dL (60-181), free T4 4.38 ng/dL (0.89-1.76), TSH <0.01 ${\mu}IU$/mL (0.35-5.5) were measured. His pulse rate ranged 70-90 beats per minute and blood pressure ranged 150/100-120/70 mmHg. Polysomnography revealed many fragmentations in sleep with many positional changes (81 times/h). Sleep onset latency was 33.5 min, sleep efficiency was 47.9%, and REM latency from sleep onset was delayed to 153.6 min. REM sleep percent was increased to 27.1%. Periodic limb movement index was 13.4/h. In the multiple sleep latency test (MSLT), average sleep latency was 0.4 min and there were noted 3 SOREMPs (Sleep Onset REM sleep period) on 5 trials. We couldn't discriminate the obvious sleep-wake pattern in the actigraph and his HLA DQB1 $^*0602$ type was negative. His thyroid function improved following treatment with methimazole and propranolol. Vital sign maintained within normal range. Cataplexy was controlled with venlafaxine 75 mg. Subjective night sleep continuity and PLMS were improved with clonazepam 0.5 mg, but the EDS were partially improved with modafinil 200-400 mg. Thyrotoxicosis might give confounding role when we were evaluating the EDS, though sleep fragmentation was one of the major symptoms of narcolepsy, but enormous amount of it made us think of the influence of thyroid hormone. The loss of sleep-wake cycle, limited improvement of EDS to the stimulant treatmen, and the cataplexy not supported by HLA DQB1 $^*0602$ should be answered further. We still should rule out idiopathic hypersomnia and measuring CSF hypocretin level would be helpful.

• Sleep Medicine and Psychophysiology
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• v.3 no.1
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• pp.47-55
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• 1996

Narcolepsy is characterized by sleep attack with excessive daytime sleepiness(EDS), cataplexy, sleep paralysis, and hypnagogic hallucination. Paradoxically, narcoleptics tend to complain of frequent arousals and shallow sleep during the night time despite their excessive sleepiness. However, nocturnal sleep fragmentation in narcoleptics is relatively ignored in treatment strategies, compared with sleep attack/EDS and cataplexy. In our paper, we attempted to investigate further on the poor nocturnal sleep in narcoleptics and to discuss possible treatment interventions. Out of consecutively seen patients at Seoul National University Sleep Disorders Clinic and Division of Sleep Studies, we recruited 57 patients, clinically assessed as having sleep attack and/or EDS. Nocturnal polysomnography and multiple sleep latency test(MSLT) were done in each of the subjects. We selected 19 subjects finally diagnosed as narcolepsy(mean age $26.0{\pm}18.3$ years, 16 men and 3 women) for this study, depending on the nocturnal polysomnographic and MSLT findings as well as clinical history and symptomatology. Any subject co-morbid with other hypersomnic sleep disorders such as sleep apnea or periodic limb movements during sleep was excluded. Sleep staging was done using Rechtschaffen and Kales criteria. Sleep parameters were calculated using PSDENT program(Stanford Sleep Clinic, version 1.2) and were compared with the age-matched normal values provided in the program. In narcoleptics, compared with the normal controls, total wake time was found to be significantly increased with significantly decreased sleep efficiency(p<.01, p<.05, respectively), despite no difference of sleep period time and total sleep time between the two groups. Stage 2 sleep%(p<.05), slow wave sleep%(p<.05), and REM sleep%(p<.01) were found to be significantly decreased in narcoleptics compared with normal controls, accompanied by the significant increase of stage 1 sleep%(p<.01). Age showed negative correlation with slow wave sleep%(p<.05). The findings in the present study indicate significant fragmentation of nocturnal sleep in narcoleptics. Reduction of REM sleep% and the total number of REM sleep periods suggests the disturbance of nocturnal REM sleep distribution in narcoleptics. No significant correlations between nocturnal polysomnographic and MSLT variables in narcoleptics suggest that nocturnal sleep disturbance in narcoleptics may be dealt with, in itself, in diagnosing and managing narcolepsy. With the objective demonstration of qualitative and quantitative characteristics of nocturnal and daytime sleep in narcoleptics, we suggest that more attention be paid to the nocturnal sleep fragmentation in narcoleptics and that appropriate treatment interventions such as active drug therapy and/or circadian rhythm-oriented sleep hygiene education be applied as needed.

• Journal of agricultural medicine and community health
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• v.38 no.2
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• pp.97-107
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• 2013

Objective: This study aimed to evaluate attention, memory and executive function in patients with narcolepsy. Methods: This study included 23 narcoleptic patients whose diagnosis were confirmed by the International Classification of Sleep Disorders(ICSD) at Chonnam National University Hospital Sleep Disorders Clinic or an other hospital in Korea, from 2005 to 2008, as well as 23 normal controls. All participants were given an IQ test for Korean-Wechsler Adult Intelligence Scale and several neuropsychological function tests (the d2 test for attention function, the Rey Complex Figure Test for nonverbal memory, the Korean-California Verbal Learning Test [K-CVLT] for verbal memory, and the Wisconsin Card Sorting Test for executive function). Clinical features of narcoleptic patients, including the frequency of excessive daytime sleepiness, cataplexy, sleep paralysis and hypnagogic hallucination, were investigated by a structured clinical interview administered by a neuropsychiatist. Excessive daytime sleepiness was evaluated by the Epworth sleepiness scale. Results: Characteristic symptoms of narcolepsy observed in this study included excessive daytime sleepiness (n=23, 100.0%), cataplexy (n=19, 82.6%), hypnagogic hallucination (n=5, 21.7%) and sleep paralysis (n=12, 52.2%). In nocturnal polysomnographic findings, stage 2 sleep and REM latency were found to be significantly decreased in narcoleptic patients compared with the control group, and were accompanied by significant increases in stage 1 sleep. Narcoleptic patients had lower scores than the control group on total number, Total Number-Total Error, Concentration Performance and Fluctuation Rate on the d2 test, which measures attention. Also, there were significant differences between the performance of patient and control groups on the B list of the K-CVLT, which measures verbal memory. Conclusion: Narcoleptic patients showed decreased attention and verbal memory performance compared to the control group; however, in many areas, narcoleptic patients still demonstrated normal cognitive function.

• Sleep Medicine and Psychophysiology
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• v.4 no.1
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• pp.89-95
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• 1997

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 idiopathic 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.