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

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

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

Rapid-eye-movement (REM) sleep behavior (RBD) is a form of REM sleep motor dyscontrol characterized by complex, vigorous, and frequently violent behaviors without atonia during the REM sleep. The motor dyscontrol may include not only cataplexy and sleep paralysis but alto periodic limb movements during REM and non-REM sleep. We examined two patients with charateristic episodes of behavioral manifestations during the REM sleep as well as with other sleep disorders such as narcolepsy and periodic limb movement disorder. The one patina was an 18 year-old man who had childhood-onset RBD associated with narcolepsy since 10 years old. The polysomnographic studies showed excessive augmentation of chin EMG and 6 attacks of violent behavior during REM sleep. He also complained of cataplexic symptomes. Multiple sleep latency tests (MSLT) showed four sleep onset REMs and two episodes of violent behavior during the REM sleep. The other patient was a 74 year-old man who complained of violent behaviors during the REM sleep and polysomnographic studies showed excessive augmentation of chin EMG during the REM sleep and periodic leg movements for 24min. And 14sec. During the sleep. We report two patients with RBD which were associated with narcolepsy, and periodic limb movememt disorder irrespectively, suggesting that RBD, narcolepsy and periodic limb movement disorder could occur coincidently and be understood as a motor dyscontrol during REM sleep.
Adolescent ; Aged ; Cataplexy ; Child ; Chin ; Extremities ; Humans ; Leg ; Mental Disorders* ; Narcolepsy* ; Nocturnal Myoclonus Syndrome* ; REM Sleep Behavior Disorder ; Sleep Wake Disorders ; Sleep Paralysis ; Sleep, REM*

Parasomnia overlap disorder is characterized by coexisting rapid eye movement (REM) sleep behavior disorder and non-REM parasomnia. We report herein an 8-year-old boy with REM sleep behavior disorder, sleep talking, and confusional arousal. Polysomnography revealed REM sleep without atonia, and arousal disorder. Neurological examination revealed bilateral ptosis, lateral gaze palsy, facial palsy, vertical nystagmus, and dysmetria. A pontine glioblastoma was found on brain magnetic resonance imaging, which could have been responsible for his neurologic deficit and sleep problem.
Arousal ; Brain ; Cerebellar Ataxia ; Child ; Facial Paralysis ; Glioblastoma ; Humans ; Magnetic Resonance Imaging ; Neurologic Examination ; Neurologic Manifestations ; Nystagmus, Pathologic ; Paralysis ; Parasomnias ; Polysomnography ; REM Sleep Behavior Disorder ; Sleep Arousal Disorders ; Sleep, REM ; Sleep-Wake Transition Disorders

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

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.
Adult ; Cataplexy ; Executive Function ; Hallucinations ; Humans ; Intelligence ; Korea ; Memory ; Narcolepsy ; Sleep Paralysis ; Sleep Wake Disorders ; Verbal Learning ; Wisconsin

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 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

PURPOSE: Narcolepsy is a specific neurologic syndrome consisting of excessive daytime drowsiness, cataplexy, and the additional phenomena of sleep paralysis and intense dreamlike hallucinations at sleep-wake transitions. We are reporting a patient with narcolepsy whose repeated self-injury caused traumatic eyeball perforation. METHODS: A 26-year-old male with narcolepsy injured himself, which resulted in eyeball perforation in his both eyes. We diagnosed him as traumatic corneal laceration, traumatic cataract, and vitreous opacity due to lens material in vitreous in both eyes. RESULTS: After improvement of narcoleptic symptom and healing of corneal perforation, we performed cataract extraction and pars plana vitrectomy under local anesthesia. During operation, we found preretinal gliosis at superior portion of optic disc and subretinal hemorrhage in the right eye. We observed successful improvement in visual acuity through intraocular lens implantation.
Adult ; Anesthesia, Local ; Cataplexy ; Cataract ; Cataract Extraction ; Corneal Perforation ; Gliosis ; Hallucinations ; Hemorrhage ; Humans ; Lacerations ; Lens Implantation, Intraocular ; Male ; Narcolepsy ; Sleep Paralysis ; Sleep Stages ; Visual Acuity ; Vitrectomy