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At least 217 records · Page 12Linked to original sources
PubMed1999

Narcolepsy-cataplexy syndrome associated with DRB1*0806-DQB*0602 haplotype in a Caucasian patient.

Narcolepsy-Cataplexy (NC) is a neurological disorder associated with the human leukocyte antigen HLA DR2. This is a prerequisite for the disease in 95 to 98% of Caucasian patients. It has been demonstrated that the HLA DQB1*0602 allele is a better marker for narcolepsy than DRB1*1501 (DR2). We present a DR-negative and DQB1*0602-positive Caucasian Spanish patient with a very unusual genotype. A 20-year-old male presented with a 12-year history of excessive daytime sleepiness and sudden muscle weakness caused by laughter and disturbed nocturnal sleep. He had never presented hypnagogic hallucinations or sleep paralysis. The family history was negative. Physical and neurological examinations were normal. The Epworth Sleepiness Scale score was 21/24, The Ullanlinna Scale score was 20/40. The polysomnographic recording showed short sleep latency, increased percentage of stage 1 (St 1), increased number of body movements and decreased sleep efficiency index. MSLT data: mean sleep latency of 1 minute and three sleep onset rapid eye movement (REM) periods (SOREMPs). HLA phenotype: A1, A11; Cw5, Cw7; B44, B39; Bw4, Bw6; DR4, DR8; DR53; DQ6, DQ8 and at the gene level: DRB1*0402, DQB1*0302; DRB1*0806, DQB1*0602. The DRB1*0806 and DQB1*0602 genotype is very infrequent in NC and identical to one African-American case in the series by Mignot et al. (1997a), and to a Caucasian case in another series by Mignot et al. (1997b). This indicates the genetic heterogeneity of the NC.

Adult↗
PubMed1992

The more unusual sleep disturbances of childhood.

The sleep patterns of children often cause anxiety to their parents. Some disturbances are unusual, and therefore may cause diagnostic difficulties. Sleep walking and night terrors can be confused with epileptic seizures. The sudden sleep of narcolepsy can lead to false accusations, when in fact the episodes are beyond the child's control. The associated phenomena of cataplexy, hypnogogic hallucinations and sleep paralysis can be particularly alarming, especially if they occur in the absence of narcolepsy. The overlap between narcolepsy and the Kleine-Levin syndrome is confirmed. Although of a different nature the sleep apnoea syndrome is equally important from the point of view of diagnosis and treatment.

Child↗
PubMed1993

[Narcolepsy in horses].

Narcolepsy is an incurable non-progressive disease of the central nervous system. In humans, narcolepsy causes excessive drowsiness during the day (sometimes a sleep-attack occurs), cataplexy (sudden loss of muscle tone), hallucinations, and sleep paralysis. In the horse and other mammals cataplexy is the most frequently observed symptom. Excessive drowsiness can occur but is harder to observe. Cataplexy is caused by a fragmentation of the REM sleep. The etiology of narcolepsy is still subject to debate, partly because normal sleeping patterns are poorly understood. In humans and certain breeds of dogs a hereditary background has been demonstrated. In Shetland ponies the disease runs in certain families. The role of trauma and infection is the subject of debate. Cataplexy (which can be induced by physostigmine injection) confirms the diagnosis. Several drugs are available for the treatment of narcolepsy in humans. However there are a few data on the results of treatment of narcolepsy in the horse.

Animals↗
PubMed1996

Narcolepsy in children.

Childhood narcolepsy is frequently under-diagnosed. Hypersomnolence may not always be accompanied by cataplexy, sleep paralysis, or hypnagogic hallucinations in the early stages. Pathophysiologic considerations revolve around an altered central nervous system catecholamine-acetylcholine balance. Both idiopathic and symptomatic forms have been described. Serial polysomnography and multiple sleep latency tests may be required to establish a definitive diagnosis. The long-term management requires the provision of both pharmacological and non-pharmacological forms of therapy.

Adolescent↗
PubMed1987

Clinical and sleep laboratory study of narcoleptic symptoms in multiple sclerosis.

Seventy white patients with a diagnosis of MS and typed for their HLA-A, B, C, and DR were studied. A clinical interview and a questionnaire were used to evaluate the presence of narcoleptic symptoms. The prevalence of sleep attacks, cataplexy, and sleep paralysis was significantly elevated among these patients. However, no difference was seen between DR2 and non-DR2 subjects with regard to the incidence of narcoleptic symptoms. Nine DR2 patients complaining of both sleep attacks and cataplexy were studied in the sleep laboratory for five consecutive naps, but no polygraphic evidence of narcolepsy was found.

Adult↗
PubMed1979

Narcolepsy and automatic behavior: a case report.

Narcolepsy is characterized by excessive daytime sleepiness and cataplexy, which may be accompanied by hypnogogic or hypnopompic hallucinations and sleep paralysis. Automatic behavior is a relatively newly recognized symptom of the narcolepsy syndrome. This case report describes a particularly troublesome sort of automatic behavior--shoplifting--in a narcoleptic patient. It illustrates how a sleep-laboratory evaluation was used to confirm the diagnosis of narcolepsy and considers aspects of the treatment of the problem.

Automatism↗
PubMed1990

Narcolepsy in a young Chinese child.

Narcolepsy is characterized by narcoleptic sleep attacks, cataplexy, sleep paralysis and hypnagogic hallucinations. It is very rare in children. A 7-year old Chinese boy presenting with typical narcoleptic symptoms is reported. Diagnosis of narcolepsy usually relies on the clinical picture, the presence of sleep-onset REM periods on nocturnal polysomnograph and the Multiple Sleep Latency Test. The strong association with HLA-DR2 may help in diagnosis, especially in children.

Child↗
PubMed2006

Survey of sleep problems amongst Singapore children in a psychiatric setting.

BACKGROUND: Sleep problems are common among children and range from transient ones to chronic problems like snoring, somnambulism and bedwetting. Sleep problems in turn impact on children's health, learning, school performance, quality of life and are often closely related to mental health problems. Population based surveys have also revealed a strong association between sleep problems and behavioral and emotional symptoms in children. The objectives of our study were to estimate the prevalence of sleep problems in children and adolescents attending psychiatric services in Singapore and to identify the correlates of sleep problems in this population. METHODS: A total of 490 parents/guardians accompanying their children to the child guidance clinic consented to complete a questionnaire, which was used to collect both sociodemographic data and the frequency of sleep problems. These included sleep starts, confusional arousal, sleep talking, bruxism, sleep walking, sleep terrors, nightmares, sleep paralysis and nocturnal enuresis. Psychiatric diagnosis was determined from case record survey of the participating subjects. RESULTS: About 62.2% (95% CI 57.8%-66.6%) of the children suffered from at least one problem. Girls were significantly more likely to suffer from sleep problems when compared with boys (chi(2)=8.5, P<0.005). The significant predictors for sleep problems were gender, diagnosis of developmental disorders and a family history of sleep problems. CONCLUSIONS: The study highlights the need for child and adolescent psychiatrists to inquire about sleep problems since sleep disturbances of children are frequent and may not be self-reported.

Child↗
PubMed2006

Parasomnias.

Parasomnias are undesirable behaviors that arise from sleep but are not fully under voluntary control. Parasomnias are grouped broadly according to whether they arise from non-rapid eye movement (NREM) or rapid eye movement (REM) sleep. NREM parasomnias are disorders of arousal that occur along a continuum of behavioral, affective, and autonomic activation. REM-related parasomnias include REM sleep behavior disorder, sleep paralysis, and nightmare disorder. Parasomnias can often be managed successfully using behavioral and pharmacologic therapies.

Arousal↗
PubMedDate not supplied

[Narcolepsy in children].

INTRODUCTION: Narcolepsy is a neurological disorder characterized by excessive somnolence during the daytime, with recurrent, irresistible episodes of sleepiness. The complete forms are associated with cataplexy, hypnagogic hallucinations and sleep paralysis. The incidence reported in adults is 4 10/10,000. A considerable proportion of adults consider their disorder to have started before the age of 15 years. It is essential to have neurophysiological confirmation of the short period of the onset of REM sleep for diagnosis. CLINICAL CASE: An 11 year old boy with diurnal hypersomnolence, behavior disorder and weight gain. He was evaluated in the Sleep Disorder Unit by polysomnography and the multiple latency test, which confirmed the suspicion of narcolepsy. CONCLUSIONS: Narcolepsy is a disorder which starts during childhood and usually goes unnoticed or is erroneously diagnosed and treated. At the present time there are valid criteria for the identification and diagnosis of children with this disorder. Treatment of these patients should be orientated towards adaptation of the environment to the child and prevention of the psychosocial problems which may be caused by this disorder, in view of the poor response to the drugs used.

Central Nervous System Stimulants↗
PubMed1992

The treatment of sleep disorders.

Narcolepsy is clinically associated with cataplexy, sleep paralysis and hypnagogic hallucinations. It is treated by reassurance (that there is no physical disease) and by stimulants such as ephedrine and amphetamine on an intermittent basis. The special tricyclic antidepressant clomipramine is also used, and mono-amine oxidase inhibitors (MAOIs) are useful in theory. Obstructive sleep apnoea is an important and often unrecognised cause of daytime somnolence. It is treated by weight reduction (pickwickian syndrome), hormones, or recently, with continuous positive pressure apparatus. Night terrors (pavor nocturnus) and sleepwalking typically occur during deep sleep (stage 3 and 4 throughout the episode) in children. In a night terror the child sits up with a scream, with eyes open, but inaccessible. He eventually falls asleep calmly. Sleepwalking, too, shows the features of inaccessibility and subsequent amnesia for the episode. Both conditions are normally treated with reassurance (to the parents) but may occasionally warrant benzodiazepines. Enuresis usually occurs in non-rapid eye movement (NREM) sleep, especially stages 3 and 4. The reason for the efficacy of tricyclic antidepressants is not precisely known. Delirium tremens (DT) is treated as a rebound excess of REM sleep, with benzodiazepines and other drugs. It is the withdrawal syndrome (with or without major seizures) to the barbiturate-alcohol group of drugs, which includes alcohol, chloral, paraldehyde, glutethimide, methylprylone, ethchlorvynol, meprobamate and meprobamate-diphenhydramine. Insomnia may be treated by the above drugs, by analgesics, antidepressants, major tranquillisers (neuroleptics) and miscellaneous other compounds. For the majority of patients, however, the most suitable group seems to be the benzodiazepines. The benzodiazepines are much safer than their predecessors, in both acute and chronic usage.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2006

REM alpha rhythm: diagnostic for narcolepsy?

SUMMARY: A young man, in whom narcolepsy was subsequently diagnosed, had the simultaneous onset of quadriparesis and a rapid eye movement (REM)-sleep polysomnographic pattern. During this REM-sleep pattern, a waking alpha EEG rhythm, appearing when he was asked to close his eyes, immediately attenuated when he was instructed to open his eyes, after which the REM-sleep pattern persisted. The juxtaposition of REM sleep patterns and reactive alpha rhythms are likely unique to sleep paralysis and may prove valuable in diagnosing narcolepsy.

Adolescent↗
PubMedDate not supplied

[Prevalence of sleep disorders in Madrid, Spain].

INTRODUCTION: Sleep disorders are among the clinical entities with a great health and socioeconomic impact. So far, in Spain there are isolated epidemiologic data, lacking from studies that cover the full spectrum of sleep disorders in the general adult population. METHODS: A sample of 1,500 adult subjects from Madrid were interviewed during the first semester of 1990, using a clinical questionnaire including sociodemographic data, habits, sleep disorders, and information regarding somatic and psychiatric pathology. A detailed analysis of the influence of sex, age and occupational status on the prevalence of the different disorders is presented. RESULTS: 1,131 persons (75.4%) completed the interview. 22.8% (95% confidence interval, CI: 20.4-25.4) referred at least one sleep difficulty, 11.3% (9.5-13.3) of the sample complained of insomnia, this being more common in women, older individuals and unemployed. Daytime sleepiness was reported by 11.6% (9.8-13.7) of the sample, and 3.2% (2.2-4.4) referred hypersomnia, with sleep attacks and sleep paralysis being rarely found. Regarding parasomnias, 12.3% (10.4-14.4) had nightmares, with the prevalence of sonambulism, night terrors and enuresis being near 1%. Daily snoring was present in 11.9% (10.1-14.0) of the sample, whereas 1.1% (0.6-2.0) reported respiratory pauses during the night. CONCLUSIONS: A high prevalence of sleep disorders is estimated in the adult population of the city of Madrid, Spain. Our data are in general similar to those found in other countries, and differences found are discussed.

Adolescent↗
PubMed1988

Neurochemical perspectives of the narcoleptic syndrome.

Narcolepsy has been defined as a disorder of excessive sleep often associated with cataplexy, sleep paralysis and hypnagogic hallucinations. Although the pathophysiology of the narcoleptic syndrome is not well understood, derangement in the functions of CNS catecholamines and serotonin (5-HT) have been implicated. In the present paper we summarize evidence to suggest a role for the endogenous opioids in the regulation of normal sleep and in the pathophysiology of the narcoleptic syndrome.

Humans↗
PubMed2005

Interobserver reliability of ICSD-R minimal diagnostic criteria for the parasomnias.

OBJECTIVE: To estimate the interobserver reliability (IR) of the minimal diagnostic criteria for the parasomnias provided in the International Classification of Sleep Disorders Revised (ICSD-R). METHODS: Fifty consecutive subjects underwent a structured interview by three doctors based on the ICSD-R minimal criteria for the diagnosis of 13 parasomnias at any time in life. IR was calculated by Kappa statistics and interpreted according to conventional categories. RESULTS: In the group of Arousal Disorders, IR was "substantial" (kappa 0.74) for confusional arousals, "slight" (kappa 0.36) for sleepwalking, "fair" (kappa -0.02) for sleep terrors. In the group of Wake-Sleep Transition Disorders, IR was "substantial" to "almost perfect", but "moderate" for sleep starts (kappa 0.41). In the group of Parasomnias usually associated with REM Sleep, IR was "substantial" (kappa 0.69) for sleep paralysis, "moderate" (kappa 0.46) for RBD, "fair" (kappa 0.25) for nightmares. In the group of Other Parasomnias, IR was "substantial" to "almost perfect" (kappa between 0.73 and 0.93). CONCLUSIONS: When the clinical diagnosis of parasomnias is based on the ICSD-R: 1) the majority of Arousal Disorders and REM sleep parasomnias showed only a "fair" to "moderate" IR; 2) all of the other parasomnias showed a "substantial" to "almost perfect" IR. Nosological entities with unsatisfactory IR share complex motor phenomenology associated with a mental state difficult for the patient to define. The source of disagreement probably lies in the difficulty in interpreting patients' reports. For these parasomnias IR must be verified and possibly improved with training.

Adolescent↗
PubMed1994

Posttraumatic narcolepsy in mild to moderate closed head injury.

Scattered reports exist describing secondary narcolepsy that develops following disease or organic insult to the brain. The present study is concerned with one particular type of secondary narcolepsy, posttraumatic narcolepsy, which we define as "narcolepsy that develops following a head injury in a previously asymptomatic individual". We obtained data on nine patients who had been previously diagnosed with mild to moderate closed head injury and who had unresolved sleep complaints. All patients presented with complaints of excessive daytime somnolence and/or sleep attacks. Patients also presented with a mix of cataplexy, hypnagogic hallucinations and/or sleep paralysis. All patients had undergone previous neurological and/or neuropsychological evaluation and testing, with seven of the nine patients having the Halstead-Reitan Neuropsychological Test Battery. Human leucocyte antigen (HLA) tissue typing was obtained in six of the nine cases. The standard protocol was utilized, consisting of overnight polysomnography with a Multiple Sleep Latency Test (MSLT) the following day. All patients' histories were negative for narcolepsy or any other significant sleep disorders prior to the head injury. The results of the neuropsychological testing indicated that all patients fell within the mild to moderate impairment range. The results of overnight polysomnography and MSLT data, along with patient histories, indicated a diagnosis of narcolepsy in all cases. The results of HLA typing indicated that three patients were DR2 positive, two were DR4 positive and one was DQW1 positive. We conclude that narcolepsy may be "dormant" and that, in cases genetically at risk, even a minor injury to the central nervous system can cause that person to become symptomatic.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMedDate not supplied

Narcolepsy. Signs, symptoms, differential diagnosis, and management.

Narcolepsy is a chronic neurologic disorder characterized by excessive daytime sleepiness and cataplexy and less often by hypnagogic hallucinations and sleep paralysis. While patients report excessive daytime sleepiness and cataplexy as the more frequent symptoms of this condition, excessive daytime sleepiness is generally believed to be the most debilitating. Narcolepsy often is undiagnosed or misdiagnosed for a variety of reasons. Although confirmation of an initial diagnosis requires monitoring of physiologic variables conducted at a sleep center by specialists, the primary care physician has a critical role in the identification and management of this incurable affliction. This article provides recommendations for the diagnosis and management of narcolepsy. The cataplexy associated with narcolepsy can be managed with tricyclic antidepressants. The excessive sleepiness is managed with stimulants but newer agents, such as modafinil, which will be marketed as Provigil, and selegiline hydrochloride, with fewer adverse effects and less abuse potential, may offer means of promoting daytime wakefulness. Groups such as the National Sleep Foundation, Washington, DC, and the Narcolepsy Network, Cincinnati, Ohio, can provide patients with needed support and information.

Diagnosis, Differential↗
PubMed1993

[Parasomnias].

Parasomnias are frequent. They usually represent either the exaggeration of a physiological phenomenon (e.g. sleep starts) or a non-disturbing, idiopathic and usually benign sleep disorder (e.g. sleep talking and bruxism), which need only counseling and improvement of sleep hygiene. However, occasionally parasomnias are of clinical relevance. They can cause insomnia or hypersomnia (e.g. 'myoclonus nocturnus'), psychosocial stress (e.g. sleep-related enuresis and sleep walking) and injuries to oneself and others (e.g. REM-parasomnia). Finally, they can be symptomatic of neurological and medical disorders (e.g. sleep paralysis and 'myoclonus nocturnus'). In these cases special investigations including video-polysomnography can establish a correct diagnosis and allow a specific treatment.

Bruxism↗