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At least 145 records · Page 8Linked to original sources
PubMed1997

The alpha attenuation test: assessing excessive daytime sleepiness in narcolepsy-cataplexy.

Daytime sleep tendency was assessed in 10 drug-free patients with narcolepsy-cataplexy and 10 normals matched for age and gender. Following nocturnal polysomnography, the alpha attenuation test (AAT) and the multiple sleep latency test (MSLT) were administered during five sessions occurring at 2-hour intervals beginning at 0900 and 1000 hours, respectively. For the AAT, participants were polysomnographically recorded for 8 minutes while seated in an illuminated room with their eyes alternately opened and closed. Power spectral analyses of electroencephalograph (EEG) activity at 02-A1 (10 second epochs) were calculated using fast Fourier transformations (FFT) within the alpha frequency range (8-12 Hz) to obtain ratios of mean eyes-closed to mean eyes-open alpha power (i.e. the alpha attenuation coefficient, AAC). The narcoleptics were sleepier than the normals as indicated by a significantly smaller mean AAC and a significantly shorter mean latency to stage 1 on the MSLT. These findings suggest that the AAT may provide a quick and practical objective assessment of the excessive daytime sleepiness (EDS) associated with narcolepsy.

Adult↗
PubMed2001

Increased REM density in narcolepsy-cataplexy and the polysymptomatic form of idiopathic hypersomnia.

The present work is focused on REM sleep density in patients with primary hypersomnia in comparison with non-hypersomnia subjects. 28 unmedicated patients with narcolepsy-cataplexy (NC) and 10 unmedicated patients suffering from the polysymptomatic form of idiopathic hypersomnia (IH) and their age- and sex-matched controls were included in the study. The clinical diagnosis was confirmed by MSLT and nocturnal PSG, HLA typing was performed in a respective group of narcoleptic patients. Polygraphical recordings were visually scored with particular regard to the two most characteristic phasic features of REM sleep: the number of rapid eye movements (REMs) and chin muscle twitches (Tws) per minute. These events were evaluated according to recognized criteria; a closer look was taken at both their frequency and their distribution across all the nocturnal REM periods (REMPs). The following main differences between hypersomniac patients (of both groups examined) and healthy controls were found in terms of phasic activity: (I) REM density (expressed in REMs/min and Tws/min in each REM period) was significantly increased in the hypersomniac patients in comparison with the controls. (p>0.05).(II) The intra-night phasic activity distribution was found rising more conspicuously in the hypersomniacs than in the controls.

Adult↗
PubMed1986

Excessive daytime sleepiness and the pathophysiology of narcolepsy-cataplexy: a laboratory perspective.

The main disabling symptom of narcolepsy-cataplexy is shown to be the unrelenting excessive daytime sleepiness (EDS) based upon controlled studies of socioeconomic effects and the poor response to treatment. Objective performance deficits mainly involve tests of ability to sustain performance on repetitive boring tasks and are reversible by improved alertness. Physiologically, EDS is seen to represent relatively slow waxing and waning of alertness rather than punctate microsleeps. Evidence is provided for complex cerebral evoked potentials (P300, contingent negative variation) being very sensitive EDS measures comparable to the multiple sleep latency test (MSLT). EDS appears to have qualitatively somewhat different forms mainly reflecting pressure for REM sleep (REM sleepiness) or pressure for NREM sleep (NREM sleepiness), which have different effects on cerebral evoked potentials as well as subjective and objective (MSLT) differences. It is argued that in pathophysiological terms narcolepsy may best be considered a disease of state boundary control.

Cataplexy↗
PubMed1997

Extensive HLA class II studies in 58 non-DRB1*15 (DR2) narcoleptic patients with cataplexy.

Narcolepsy is a sleep disorder that has been shown to be tightly associated with HLA DR15 (DR2). In this study, 58 non-DR15 patients with narcolepsy-cataplexy were typed at the HLA DRB1, DQA1 and DQB1 loci. Subjects included both sporadic cases and narcoleptic probands from multiplex families. Additional markers studied in the class II region were the promoters of the DQA1 and DQB1 genes, two CA repeat polymorphisms (DQCAR and DQCARII) located between the DQA1 and DQB1 genes, three CA repeat markers (G51152, T16CAR and G411624R) located between DQB1 and DQB3 and polymorphisms at the DQB2 locus. Twenty-one (36%) of these 58 non-DR15 narcoleptic patients were DQA1*0102 and DQB1*0602, a DQ1 subtype normally associated with DRB1*15 in DR2-positive narcoleptic subjects. Additional microsatellite and DQA1 promoter diversity was found in some of these non-DR15 but DQB1*0602-positive haplotypes but the known allele specific codons of DQA1*0102 and DQB1*0602 were maintained in all 21 cases. The 37 non-DQA1*0102/DQB1*0602 subjects did not share any particular HLA DR or DQ alleles. We conclude that HLA DQA1*0102 and DQB1*0602 are the most likely primary candidate susceptibility genes for narcolepsy in the HLA class II region.

Cataplexy↗
PubMed1998

"Cataplexy" and muscle ultrasound abnormalities in Coffin-Lowry syndrome.

The Coffin-Lowry syndrome is a rare cause of mental retardation recognised by its distinctive facial and digital features. We have observed an unusual, non-epileptic, cataplexy-like phenomenon in three subjects with the syndrome and we speculate that this feature may go unrecognised. We also provide evidence of neuromuscular dysfunction as part of the phenotype by showing abnormalities on muscle ultrasound in four gene carriers.

Cataplexy↗
PubMed1977

Symptomatic cataplexy.

The case is described of a man who developed attacks of cataplexy, narcolepsy, and sleep paralysis because of microglioma which infiltrated the walls and floor of the IIIrd ventricle and the upper brain stem. The mechanisms by which the pathology is related to the symptoms are discussed.

Adult↗
PubMed1996

A comparison of idiopathic hypersomnia and narcolepsy-cataplexy using self report measures and sleep diary data.

Eighteen patients with idiopathic hypersomnia (IH) were compared with 50 patients with the narcoleptic syndrome of cataplexy and daytime sleepiness (NLS) using self report questionnaires and a diary of sleep/wake patterns. The IH group reported more consolidated nocturnal sleep, a lower propensity to nap, greater refreshment after naps, and a greater improvement in excessive daytime sleepiness since onset than the NLS group. In IH, the onset of excessive daytime sleepiness was predominantly associated with familial inheritance or a viral illness. Two variable--number of reported awakenings during nocturnal sleep and the reported change in sleepiness since onset--provided maximum discrimination between the IH and NLS groups. Confusional arousals, extended naps or nocturnal sleep, autonomic nervous system dysfunction, low ratings of medication effectiveness, or side effects of medication were not associated differentially with either IH or NLS.

Cataplexy↗
PubMed2006

Narcolepsy with cataplexy in early childhood.

Narcolepsy is a rare neurologic sleep disorder with morbidity associated with functional impairment and frequent delay in diagnosis. Symptoms typically manifest in adolescence or early adulthood, but diagnosis of narcolepsy has been reported in early childhood. Diagnosis rates are as low as 50% of the total population of patients with narcolepsy and are delayed as much as 10 years after disease onset due to inadequate patient-physician communication and/or misdiagnosis. I present the complexity of diagnosing narcolepsy in early childhood in a patient with cataplexy that started soon after independent ambulation at age 10 months.

Adolescent↗
PubMed2006

Narcolepsy-cataplexy associated with precocious puberty.

In children, narcolepsy may be the symptom of a brain lesion or genetic disease. The authors report two cases with severe narcolepsy-cataplexy emerging in childhood in close temporal association with obesity and precocious puberty.

Aspartic Acid↗
PubMed1984

Transient cataplexy after removal of a craniopharyngioma.

We studied a patient with cataplexy secondary to a surgical lesion that involved the perichiasmal hypothalamus. We believe that this lesion interfered with the hypothalamic mechanism for timing sleep and wakefulness, whereas the pontine mechanism for generating sleep cycles remained relatively intact.

Adolescent↗
PubMed2003

From club drug to orphan drug: sodium oxybate (Xyrem) for the treatment of cataplexy.

Narcolepsy, a rare disease with a prevalence of 0.05% in the general population, affects an estimated 140,000 patients in the United States. Patients have been able to lead fuller personal and professional lives since the Food and Drug Administration approved sodium oxybate (Xyrem) in 2002 for treatment of cataplexy in patients with narcolepsy. Previously, gamma-hydroxybutyrate (GHB), the active ingredient of sodium oxybate, had been a substance of abuse, most notoriously as a date-rape drug. Public Law 106-172, the date-rape prohibition act enacted in 2000, was modified to allow the drug to be legally administered for medical purposes. Because of the apprehension regarding the risk of possible drug diversion after the approval of sodium oxybate and concerns about safety, the Xyrem Risk Management Program was created. This program has been successful in satisfying the needs of patients and physicians while ensuring responsible distribution of the drug.

Administration, Oral↗
PubMed1999

REM sleep enhancement and behavioral cataplexy following orexin (hypocretin)-II receptor antisense perfusion in the pontine reticular formation.

Orexin (hypocretin)-containing neurons of the hypothalamus project to brainstem sites that are involved in the neural control of REM sleep, including the locus coeruleus, the dorsal raphe nucleus, the cholinergic zone of the mesopontine tegmentum, and the pontine reticular formation (PRF). Orexin knockout mice exhibit narcolepsy/cataplexy, and a mutant and defective gene for the orexin type II receptor is present in dogs with an inherited form of narcolepsy/cataplexy. However, the physiological systems mediating these effects have not been described. We reasoned that, since the effector neurons for the majority of REM sleep signs, including muscle atonia, were located in the PRF, this region was likely implicated in the production of these orexin-related abnormalities. To test this possibility, we used microdialysis perfusion of orexin type II receptor antisense in the PRF of rats. Ten to 24 hours after antisense perfusion, REM sleep increased two- to three-fold during both the light period (quiescent phase) and the dark period (active phase), and infrared video showed episodes of behavioral cataplexy. Moreover, preliminary data indicated no REM-related effects following perfusion with nonsense DNA, or when perfusion sites were outside the PRF. More work is needed to provide precise localization of the most effective site of orexin-induced inhibition of REM sleep phenomena.

Animals↗
PubMed2000

[Hypocretin (orexin) deficiency in narcolepsy-cataplexy].

A mutation in the HCRT locus was proved in 18-yrs old male suffering from narcolepsy-cataplexy. He has demonstrated cataplectic attacks (brief spells of head dropping provoked by laughter) as well as imperative sleep in spells of several minutes up to one hour since the age of six months. He has suffered from severe bulimia since five years; later hypnagogic hallucinations, sleep paralysis and unquiet nocturnal sleep accompanied by periodic limb movements appeared. Symptoms are partially controlled with methylphenidate and either imipramine, clomipramine or fluoxetine. Periodic leg movements poorly responded to L-DOPA and clonazepam treatment. He is HLA-DQB1*0602 negative. Repeated MSLT (over 16 years followed-up period) showed extremely short latency with predominant SOREMPs and also nocturnal PSG recordings revealed fragmented sleep with SOREMPs. This case report demonstrates that hypocretin (orexin) mutations in human can produce the full narcolepsy phenotype and validates data recently reported in dog and mouse models suggesting a role for hypocretin (orexin) in the pathophysiology of narcolepsy and the regulation of REM sleep.

Adolescent↗
PubMedDate not supplied

Childhood onset of narcolepsy-cataplexy syndrome in Turkey: clinical and genetic study.

Narcolepsy is a disabling sleep disorder characterized by excessive daytime sleepiness and abnormal manifestations of rapid eye movement (REM) sleep including cataplexy, sleep paralysis and hypnagogic hallucinations. It is known to be complex disorder in which both genetic predisposition and environmental factors play a role. In humans, susceptibility to narcolepsy is tightly associated with a specific HLA allele, DQB1*0602. In this report, we took advantage of the ongoing genetic study in Turkish narcoleptic patients to document clinical and genetic data of eight patients whose onset of symptoms were in the childhood period.

Adolescent↗
PubMed2002

A female with Coffin-Lowry syndrome and "cataplexy".

Coffin-Lowry syndrome (CLS) is an X-linked semidominant condition, caused by mutations in the gene encoding the ribosomal protein S6 kinase-2 (RSK-2), a growth factor regulating protein kinase, which is mapped to Xp 22.2. The syndrome is mainly seen in males. It is manifested by moderate to severe mental retardation and characteristic facial, hand and skeletal malformations. We present a female patient with fully manifested CLS, confirmed by molecular analysis, who experienced daily drop episodes, diagnosed as "cataplexy". The episodes were precipitated by emotional or auditory stimuli and were significantly reduced, by selective serotonine re-uptake inhibitors.

Adolescent↗
PubMed1989

Treatment of cataplexy in a dog with narcolepsy.

A 1-year-old female Rottweiler with a history of narcolepsy and cataplexy lost weight and became worse when given cholinergic agents and/or prednisolone over a 12-day period. The dog was then treated with imipramine HCl, and has been almost clinically normal for 2 years.

Animals↗
PubMedDate not supplied

[Use of substances modifying serotonin metabolism in the treatment of narco-cataplexy (considerations on 5 cases)].

The AA., after a critical analysis of the new proposed treatments for the narco-cataplexy, report their experience about the therapeutic possibilities of this syndrome with particular manipulations of cerebral serotonin (5-HT). In 5 patients they tried to act on the two sides of the sleeping-waking cycle by using a diurnal administration of Methysergide (5-HT inhibitor) and an evening loading of L-Tryptophan combined with Benserazide (dopa-decarboxilase inhibitor). The immediate and long term results on the nercoleptic as well as cataplectic symptoms can be considered good. Conversely treatments with Clomipramine alone exerted poor beneficial effects in these patients. The AA. discuss the physiopathogenetic mechanisms which are thought to be involved in this disease.

Adult↗