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

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

A 71-year-old male initially presented with vocal cord palsy and underwent tracheostomy. After thorough examination, urogenital dysfunction, orthostatic hypotension, and Parkinsonism were found, which led to the diagnosis of multiple system atrophy (MSA). After the tracheostomy, bi-level positive airway pressure ventilation was required during the night due to nocturnal hypoxemia. Night-time hypoxemia is related to central sleep apnea, which is one of the manifestations of MSA. This is the first case of MSA manifested by bilateral vocal cord palsy as an initial sign in Korea. This case supports the notion that MSA should be taken into consideration when vocal cord paralysis is observed.
Aged ; Airway Obstruction ; Anoxia ; Diagnosis ; Humans ; Hypotension, Orthostatic ; Korea ; Male ; Multiple System Atrophy* ; Parkinsonian Disorders ; Sleep Apnea, Central ; Tracheostomy ; Ventilation ; Vocal Cord Paralysis*

A 71-year-old male initially presented with vocal cord palsy and underwent tracheostomy. After thorough examination, urogenital dysfunction, orthostatic hypotension, and Parkinsonism were found, which led to the diagnosis of multiple system atrophy (MSA). After the tracheostomy, bi-level positive airway pressure ventilation was required during the night due to nocturnal hypoxemia. Night-time hypoxemia is related to central sleep apnea, which is one of the manifestations of MSA. This is the first case of MSA manifested by bilateral vocal cord palsy as an initial sign in Korea. This case supports the notion that MSA should be taken into consideration when vocal cord paralysis is observed.
Aged ; Airway Obstruction ; Anoxia ; Diagnosis ; Humans ; Hypotension, Orthostatic ; Korea ; Male ; Multiple System Atrophy ; Parkinsonian Disorders ; Sleep Apnea, Central ; Tracheostomy ; Ventilation ; Vocal Cord Paralysis

Experiences of needling techniques of Professor LI Ya- fang is introduced in this article. Gentle and superficial insertion is adopted by Professor LI in clinic. Emphases are put on the qi regulation function, needling sensation to the affected region and insertion with both hands, especially the function of the left hand as pressing hand. The gentle and superficial insertion should be done as the follows: hold the needle with the right hand, press gently along the running course of meridians with the left hand to promote qi circulation, hard pressing should be applied at acupoints to disperse the local qi and blood, insert the needle gently and quickly into the subcutaneous region with the right hand, and stop the insertion when patient has the needling sensation. While the fast needling is characterized with shallow insertion and swift manipulation: the left hand of the manipulator should press first along the running course of the meridian, and fix the local skin, hold the needle with the right hand and insert the needle quickly into the acupoint. Withdrawal of the needle should be done immediately after the reinforcing and reducing manipulations. Professor LI is accomplished in qi regulation. It is held by him that regulating qi circulation is essence of acupuncture, letting the patient get the needling sensation is the most important task of needling. Lifting, thrusting and rotation manipulations should be applied to do reinforcing or reducing. The tissue around the tip of the needle should not be too contracted or too relaxed, and the resistance should not be too strong or too weak. The feeling of the insertion hand of the practitioner should not be too smooth or too hesitant. Needle should be inserted into the skin quickly at the moment of hard pressing by the left hand. And then, slow rotation and gentle lifting and thrusting can be applied to promote the needling sensation like electric current pass through and to reach the affected region along the running course of meridians.
Acupuncture Therapy ; instrumentation ; methods ; Adult ; Facial Paralysis ; therapy ; Female ; Humans ; Infant ; Male ; Middle Aged ; Sleep Wake Disorders ; therapy

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 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/m2. 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 microIU/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 treatment, 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.
Adolescent ; Benzhydryl Compounds ; Blood Pressure ; Body Mass Index ; Cataplexy ; Clonazepam ; Cyclohexanols ; Extremities ; Hallucinations ; Heart Rate ; HLA-DQ beta-Chains ; Humans ; Hypersomnolence, Idiopathic ; Intracellular Signaling Peptides and Proteins ; Male ; Methimazole ; Narcolepsy ; Nervous System Diseases ; Neuropeptides ; Polysomnography ; Propranolol ; Reference Values ; Sleep Deprivation ; Sleep Paralysis ; Sleep, REM ; Thyroid Gland ; Thyrotoxicosis ; Vital Signs ; Orexins ; Venlafaxine Hydrochloride

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

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

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