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At least 451 records · Page 25Linked to original sources
PubMed2003

The orexin/hypocretin system: a critical regulator of neuroendocrine and autonomic function.

The hypocretins/orexins are hypothalamic peptides most recognized for their significant effects on feeding and arousal. Indeed, loss of the peptides results in a cataplexy quite similar to that observed canine models of human narcolepsy. However, neurons producing these peptides project to numerous brain sites known to be important in neuroendocrine regulation of pituitary function and autonomic centers as well. Results from numerous laboratories have suggested broad physiological roles for the hypocretins/orexins in neuroendocrine and autonomic regulation as a consequence of actions in the dorsal vagal complex, paraventricular nucleus, and pituitary. This review focuses upon evidence for potential physiologic roles for the peptides in these sites.

Animals↗
PubMed1999

Weak with laughter.

We found a marked reduction of H-reflex amplitude during laughter, suggesting that motor inhibition may underlie the feeling of being "weak with laughter". This effect may have a role in cataplexy, which is preferentially elicited by laughter.

Cataplexy↗
PubMedDate not supplied

The function of 'brain waves': a cybernetic model of electroencephalography.

Electroencephalograms recorded from sleeping subjects are reinterpreted, from first principles, to explain the relationship between the observed wave-forms and cerebral processes. The manifestations of narcolepsy, namely, sleep paralysis, hypnopompic hallucinations and cataplexy are consistent with a hypothesis that the disorder is the result of entrainment of cerebral waves, causing almost instantaneous descent into a REM sleep state.

Cataplexy↗
PubMed1996

Narcolepsy.

Narcolepsy is among the leading causes of excessive daytime sleepiness and is the most common neurologic cause. Its classic form--narcolepsy with cataplexy--is a distinct neurologic disease with characteristic clinical and paraclinical findings. The history, epidemiology, clinical picture, pathophysiology, cause, diagnosis, and treatment, both pharmacologic and nonpharmacologic, are discussed in detail.

Adult↗
PubMed2004

[Fake pearls... imperative day somnolence--a personal experience].

In this review, narcolepsy is described based on personal experience of the author with this disease, especially with imperative day-somnolence and cataplexy. Narcolepsy is frequently underestimated and repressed. The economical (disability) and ethical (traffic accidents) implications of the disease may be significant. Optimal treatment and changes of behaviour are important.

Accidents, Traffic↗
PubMed2001

MAO-A and COMT polymorphisms and gene effects in narcolepsy.

Narcolepsy presents one of the tightest associations with a specific HLA antigen (DQB1*0602) but there is strong evidence that non-HLA genes also confer susceptibility. Recent observations have implicated the hypocretin/orexin system in narcolepsy in both humans and animals. In addition, the implication of monoaminergic systems in the pathophysiology of narcolepsy is well established and a significant association between the monoamine oxydase-A (MAO-A) gene and human narcolepsy has recently provided a possible genetic link. We investigated polymorphisms of MAO-A and catechol-O-methyltransferase (COMT) in 97 Caucasians with well-defined narcolepsy-cataplexy and sought for genotypic effects on disease symptoms. No evidence of association between genotype or allele frequencies of both MAO-A or COMT gene and narcolepsy was found. However, a sexual dimorphism and a strong effect of COMT genotype on disease severity were found. Women narcoleptics with high COMT activity fell asleep twice as fast as those with low COMT activity during the multiple sleep latency test (MSLT) while the opposite was true for men. COMT genotype also strongly affected the presence of sleep paralysis and the number of REM sleep onsets during the MSLT. In agreement with well-documented pharmacological results in canine narcolepsy, this study reports the first genetic evidence for the critical involvement of the dopaminergic and/or noradrenergic systems in human narcolepsy.

Cataplexy↗
PubMed2001

Health-related quality of life in narcolepsy.

Narcolepsy is a chronic sleep disorder characterised by symptoms of excessive daytime sleepiness and cataplexy. The aim of this study was to describe the health-related quality of life of people with narcolepsy residing in the UK. The study comprised a postal survey of 500 members of the UK narcolepsy patient association, which included amongst other questions the UK Short Form 36 (SF-36), the Beck Depression Inventory (BDI), and the Ullanlinna Narcolepsy Scale (UNS). A total of 305 questionnaires were included in the final analysis. The results showed that the subjects had significantly lower median scores on all eight domains of the SF-36 than normative data, and scored particularly poorly for the domains of role physical, energy/vitality, and social functioning. The BDI indicated that 56.9% of subjects had some degree of depression. In addition, many individuals described limitations on their education, home, work and social life caused by their symptoms. There was little difference between the groups receiving different types of medication. This study is the largest of its type in the UK, although the limitations of using a sample from a patient association have been recognised. The results are consistent with studies of narcolepsy in other countries in demonstrating the extensive impact of this disorder on health-related quality of life.

Adolescent↗
PubMed1990

The influence of two behavioral regimens on the distribution of sleep and wakefulness in narcoleptic patients.

Thirty-two hours (night-day-night) of polygraphic recordings were performed on 14 patients with a diagnosis of narcolepsy-cataplexy. Half of the patients stayed in bed during the day, whereas the other half were seated at a table. Patients were free to nap whenever they wanted to. Patients under continuous bedrest slept 2-3 times more during the day than patients who were sitting at the table. Rapid-eye-movement (REM) sleep and slow-wave sleep (SWS, stages 3 and 4) were nearly absent during daytime sleep in the table group, but not in the bed group. The differential behavioral regimes during the day resulted in different amounts of SWS in the consecutive night sleep. Although SWS increased from the first to the second night in the table group, it decreased in the bed group. This result suggests that the presumably homeostatic regulation of SWS is intact in narcoleptic patients.

Adult↗
PubMed2001

Stimulant and anticataplectic effects of reboxetine in patients with narcolepsy: a pilot study.

STUDY OBJECTIVES: To investigate potential stimulant and anticataplectic effects of 10 mg reboxetine in patients diagnosed with narcolepsy. DESIGN: 12 patients were treated for a 2-week period with 10 mg reboxetine under open conditions. The dosage of reboxetine was gradually increased between Day 1 and Day 9. Outcome parameters consisted of nightime polysomnography (PSG), Multiple Sleep Latency Test (MSLT), Epworth Sleepiness Scale (ESS), Visual Analog Scale for Sleepiness (VAS), Ullanlinna Narcolepsy Scale (UNS), and the Beck Depression Inventory (BDI). SETTING: Sleep Disorders Clinic at a University Hospital. PATIENTS: 12 patients meeting ICSD-criteria for narcolepsy. INTERVENTIONS: Pharmacological treatment with reboxetine. RESULTS: Following treatment for two-weeks, a significant improvement in daytime sleepiness could be observed, as reflected by a mean decrease of 48.6% on the Epworth Sleepiness Scale and a mean increase of 54.7% in sleep latency on the MSLT. Furthermore, a significant reduction in the cataplexy subscore of the Ullanlinna Narcolepsy Scale and in REM-sleep was found. CONCLUSIONS: Our results suggest that reboxetine exerts stimulant and anticataplectic effects in narcolepsy. Contrary to previous thinking, by which stimulant action would require dopaminergic facilitation, noradrenergic mechanisms might be relevant to the control of wakefulness.

Adult↗
PubMed2001

Childhood narcolepsy in North China.

STUDY OBJECTIVES: The purpose is to report the results of an effort to diagnose children with narcolepsy in a pediatric referral clinic. DESIGN: Between September 1998 and December 1999, a program was implemented to emphasize recognition of childhood narcolepsy. Patients underwent brain computed tomography (CT) scan and magnetic resonance imaging (MRI) testing. All children received a MSLT test following a routine night's sleep, and serological HLA typing for HLA DR2. Three who reported occasional snoring also underwent nocturnal PSG prior to the MSLT. SETTING: N/A. PATIENTS OR PARTICIPANTS: N/A. INTERVENTIONS: N/A. MEASUREMENTS AND RESULTS: 29 (21 male, 8 female) children were identified with sleepiness and cataplexy. There was no evidence for brain functional or structural disease or for drug use. Sleep paralysis was elicited in 41%; hypnagogic hallucinations, in 59%. Psychosocial problems including emotional irritability and social isolation were present in 93% of the patients. Mean sleep latency on MSLT was 2.0+/-1.3 minutes; sleep-onset rapid eye movement (SOREM) occurred during 2/5 naps in 28 of 29 patients and 3/5 in 26/29 patients. The average number and latency of SOREM episodes were 4.2+/-0.9 episodes and 4.0+/-1.7 minutes, respectively. In those with snoring, a nocturnal PSG did not disclose sleep apneas/hypopneas. All patients but one were HLA DR2 positive. The estimated clinic incidence was 0.04%. CONCLUSIONS: A program for recognition in a referral neurology clinic combined with an availability of the MSLT and HLA testing resulted in the new identification in North China of a number of children with narcolepsy syndromes.

Adolescent↗
PubMed2003

Decreased cerebrospinal fluid hypocretin-1 levels near the onset of narcolepsy in 2 prepubertal children.

We present 2 cases of narcolepsy with prepubertal onset. Although excessive daytime sleepiness and cataplexy had appeared early in both patients, the presence of sleep-onset rapid eye movement periods was detected several months after the onset of hypersomnia. The levels of hypocretin in the cerebrospinal fluid were reduced when measured 3 weeks (Patient 1) and 2 months (Patient 2) after the appearance of hypersomnia, before the presence of sleep-onset rapid eye movement periods was confirmed. Because the symptoms of narcolepsy in children are often obscure and easily mistaken as other diseases, and the electrophysiologic studies may not be specific in the early stage, the definite diagnosis tends to be delayed. Measurement of hypocretin-1 levels in the cerebrospinal fluid is useful for the early diagnosis of narcolepsy with prepubertal onset.

Brain↗
PubMed2006

Altered skin-temperature regulation in narcolepsy relates to sleep propensity.

STUDY OBJECTIVES: In healthy subjects, sleep propensity increases when the distal skin temperature increases relative to the proximal skin temperature. This increase results from increased blood flow in the skin of the extremities and is, among other factors, controlled by the hypothalamic circadian clock, as is sleep. Because narcolepsy is characterized by hypothalamic alterations, we studied skin temperature in narcoleptic patients in relation to their characteristically increased sleep propensity during the day. DESIGN: Distal and proximal skin temperature and their gradient (DPG) were measured during a Multiple Sleep Latency Test. This allowed temperature to be studied during wakefulness, at sleep onset and during sleep. SETTING: Tertiary narcolepsy referral center in a university hospital. PATIENTS: Fifteen unmedicated narcolepsy patients with cataplexy and 15 controls. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: In subjects in the waking state, DPG was higher in narcoleptics than in controls throughout the day (time by group interaction, p < .0001), due to increased distal skin temperature and decreased proximal skin temperature. The increase in DPG was related to a shorter subsequent sleep-onset latency (p = .02). Once asleep, narcoleptics maintained their elevated distal skin temperature and DPG (p < .0001), whereas proximal skin temperature increased to reach normal levels. CONCLUSIONS: This is the first demonstration of a dramatic alteration of daytime skin temperature control in narcolepsy. Even awake narcoleptic patients showed a DPG higher than that which healthy controls achieve when asleep. This observation suggests that hypocretin deficiency in narcolepsy affects skin-temperature regulation and invites further examination. Skin-temperature control might ultimately even have therapeutic implications for the alleviation of narcoleptic symptoms.

Adult↗
PubMed1985

Diagnosis of narcolepsy using the multiple sleep latency test: analysis of current laboratory criteria.

Multiple sleep latency tests (MSLT) performed on 144 patients with excessive daytime somnolence were examined for the diagnostic reliability of a short sleep latency (SL less than 5 min) and the presence of sleep-onset REM periods (SOREMPs). Based on clinical criteria, 61 patients (42%) were diagnosed as having narcolepsy. Thirty-five narcoleptic patients and five nonnarcoleptic patients exhibited a mean SL less than 5 min, yielding a sensitivity of 57% and a specificity of 94% for this criterion for pathological drowsiness. The occurrence of two or more SOREMPs was found in 52 narcoleptic patients but in only one nonnarcoleptic patient (sensitivity of 84% and specificity of 99%). Those narcoleptic patients with cataplexy demonstrated a shorter SL and more frequent SOREMPs than their noncataplectic counterparts. It was concluded that the MSLT is a highly reliable laboratory tool for the confirmation of the diagnosis of narcolepsy based on the SOREMP criterion. The criterion value for SL in pathological drowsiness may depend on laboratory conditions as well as the patient population selected.

Adolescent↗
PubMed1986

Viloxazine hydrochloride in narcolepsy: a preliminary report.

Twenty-three narcoleptic subjects participated in a single-blind drug study with placebo-viloxazine hydrochloride (100 mg/day). One woman discontinued the study because of nausea and headaches during the treatment period. The remaining 22 subjects were polygraphically monitored during baseline, The remaining 22 subjects were polygraphically monitored during baseline, placebo intake, viloxazine treatment, and placebo intake following 48 h of drug withdrawal. Symptoms and side effects were evaluated subjectively and objectively. Except for the subject who discontinued the study, viloxazine was well tolerated, particularly in elderly subjects. The drug had an inhibitory effect on REM sleep, cataplexy, and other auxiliary symptoms. While there is no objective evidence that it heightens alertness, patients reported fewer sleep attacks.

Adult↗
PubMed2001

Narcolepsy: clinical features, new pathophysiologic insights, and future perspectives.

Narcolepsy is characterized by excessive daytime sleepiness and abnormal manifestations of rapid eye movement sleep such as cataplexy. The authors review the clinical features of narcolepsy, including epidemiology, symptoms, diagnosis, and treatment, in detail. Recent findings show that a loss of hypocretin-producing neurons lies at the root of the signs and symptoms of narcolepsy. The authors review the current state of knowledge on hypocretin anatomy, physiology, and function with special emphasis on the research regarding the hypocretin deficiency in narcolepsy, which may also explain associated features of the disorder, such as obesity. Lastly, they discuss some future perspectives for research into the pathophysiology of sleep/wake disorders, and the potential impact of the established hypocretin deficiency on the diagnosis and treatment of narcolepsy.

Age of Onset↗
PubMed2002

Hypocretin/orexin and sleep: implications for the pathophysiology and diagnosis of narcolepsy.

PURPOSE OF REVIEW: Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness and cataplexy. The discovery that the majority of human patients lack the hypothalamic neuropeptide hypocretin-1 has initiated a large body of new research. RECENT FINDINGS: Several studies on cerebrospinal fluid hypocretin-1 levels in narcolepsy and in various other sleep disorders have shown that hypocretin deficiency is both highly sensitive and specific for narcolepsy/cataplexy. Importantly, 15% of narcoleptic patients show low hypocretin levels, despite a negative multiple sleep latency test. Besides regulating sleep, the hypocretin system is involved in the regulation of energy balance, autonomic function and several neuroendocrine ensembles. Consequently, up to one-third of patients are obese (body mass index > 30). Furthermore, serum leptin levels are decreased in both nonoverweight and obese patients. The new rodent models for narcolepsy may aid in the further characterization of these endocrinological abnormalities. Finally, there is increasing insight into the physiological role of the hypocretin system in the regulation of sleep and wakefulness. SUMMARY: Hypocretin measurements may now be applied as a new diagnostic tool, providing the results are interpreted within the clinical context. In the clinical care of narcoleptic patients, attention should be paid to the obesity that frequently accompanies the disorder. In the future, hypocretin agonists may become available. Further characterization of animal models for narcolepsy will undoubtedly increase our insight into the pathophysiology of the disorder.

Animals↗
PubMed1996

Narcolepsy in a 2-year-old boy.

This report describes the prospective follow-up of a 2-year-old child with narcolepsy. The initial clinical diagnosis was confirmed by the onset of cataplexy when the child was 5 years old. He does not have the HLA genotype characteristic of narcolepsy or an identifiable brain lesion, although he has megalencephaly and mild learning difficulties. Treatment with dietary tyrosine supplements who are to young to use stimulants safely. The morbidity of narcolepsy is high and early diagnosis is therefore important, as treatment is effective.

Cataplexy↗
PubMed1978

Genetic study of narcoleptic syndrome.

In this family study of the narcoleptic syndrome, 52% of the probands had an affected first degree relative, 41.9% of the sibs of those probands with an affected parent were similarly affected, 33.3% of the parents of 2 affected sibs and 41.2% of the children (after a correction for age) had narcolepsy, cataplexy, or both.

Age Factors↗