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PubMed1980

Effects of nocturnal gamma-hydroxybutyrate on sleep/waking patterns in narcolepsy-cataplexy.

Continuous 48-hour polygraphic recordings of sleep/waking patterns were performed on 14 patients with narcolepsy-cataplexy before and after 7-10 days of treatment of their nocturnal sleep with gamma-hydroxybutyrate (GBH). GBH improved the quality of night sleep by increasing the amount of slow wave sleep, reducing stage I, increasing sleep efficiency (percentage of time in bed spent asleep), and reducing the number of periods of short sleep under 15 minutes. Also nighttime REM sleep was reduced in latency and became less fragmented. The daytime period contained less slow wave sleep and REM sleep, and fewer episodes of prolonged sleep. Patients experienced reduction or loss of daytime attacks of irresistible sleep, cataplectic attacks, and other auxiliary symptoms. Residual daytime drowsiness subsequently improved on low doses of methylphenidate. Tolerance did not develop and there were no serious toxic side-effects. Four of the patients had been refractory to previous combinations of antidepressants and high doses of stimulants.

Adult↗

PubMed1994

Daytime sleep inertia in narcolepsy-cataplexy.

Eight volunteers with narcolepsy-cataplexy participated in a study of scheduled naps and performance. Sleep inertia was examined following five "short" naps of 5% and a single "long" nap of 25% of total 24-hour sleep time as determined by prior sleep log data. Contrary to some subjective reports, short naps (mean duration of just under 30 minutes) were accompanied by sleep inertia in narcoleptics. As measured by the descending subtraction task, this sleep inertia was at times quite prolonged and lasted 20 minutes after waking from midday short naps, which ended on average at 1555 hours. In addition, sleep inertia, as measured by both the descending subtraction task and the four-choice reaction-time test, was evident throughout both afternoon and evening short naps; however, it was completely absent from reaction-time test results immediately following the single long nap, which ended on average at 1640 hours. Sleep inertia was maximum after slow-wave sleep arousals and was minimal or absent following the first short nap, which also contained the highest amount of rapid eye movement sleep of all naps.

Adult↗

PubMed1982

Narcolepsy-cataplexy. II. Psychosocial consequences and associated psychopathology.

The emotional and psychosocial correlates of narcolepsy were evaluated in 50 adults who had a current complaint of sleep attacks and cataplexy. The psychosocial consequences of narcolepsy were extensive; most patients reported that the condition seriously affected their interpersonal, marital, work, and social relationships. Many of these consequences were caused by others' misunderstanding of the symptoms of the disorder, which resulted in negative attitudes toward the patients. Th narcoleptic patients showed high levels of psychopathology compared with controls, but this difference is considered to be primarily a reaction to the disorder and its effects. The narcoleptic patients were overly concerned with emotional control, which appeared to lead to their generalized lack of expressiveness and tendency to build up emotional pressure. Based on these findings, recommendations are given for the psychosocial management of the narcoleptic patient.

Adolescent↗

PubMed2005

Narcolepsy increased L-PGDS (beta-trace) levels correlate with excessive daytime sleepiness but not with cataplexy.

OBJECTIVES: Alterations in the prostaglandin-D-system have been found in animal sleep experiments and disorders that present with hypersomnia or sleep disturbances. The recently demonstrated involvement of the leptomeningeal lipocalin-type prostaglandin-Dsynthase (L-PGDS) (beta-trace) in human physiological sleep encouraged us to investigate its role in the pathophysiology of narcolepsy. METHODS: In a pilot study, serum LPGDS and melatonin concentrations were assessed in 14 narcoleptic patients during undisturbed sleep and total sleep deprivation, compared with those from 14 healthy controls during undisturbed sleep. Excessive daytime sleepiness was measured by a standardized questionnaire (Epworth sleepiness scale, ESS). RESULTS: In narcoleptic patients, markedly increased baseline L-PGDS levels were significantly correlated with the ESS score, but not with the degree of cataplexy. Serum L-PGDS concentrations in patients as well as in controls followed a time-dependent fluctuation with evening increases, highest values during the night and in the morning. Compared with controls, patients exhibited significant/increased amplitude of circulating L-PGDS without any suppression by total sleep deprivation. CONCLUSION: These findings indicate that the prostaglandin-D-system contributes to the pathophysiology of narcolepsy, e. g. the regulation of excessive daytime sleepiness. Since it has been suggested that L-PGDS is also involved in neurodegenerative disorders, there may be a more specific role of the prostaglandin- D-system in narcoleptic aetiogenesis. Moreover, its linkage with the immune system as well as with human sleep regulation offers a direct access for investigating both systems.

Adolescent↗

PubMed2003

Rasmussen's syndrome and new-onset narcolepsy, cataplexy, and epilepsy in an adult.

We report a case of new-onset seizures and narcolepsy in a previously healthy 40-year-old man. He developed severe daytime somnolence and cataplexy over the course of a few months. Brain MRI was normal, and polysomnography with multiple sleep latency testing confirmed a diagnosis of narcolepsy. His HLA haplotype is DQB1*0602 and cerebrospinal fluid analysis showed no detectable hypocretin. Approximately 18 months later, he developed complex partial seizures. Further MRI showed a progressively enlarging lesion involving the left frontotemporal and insular areas. Pathology from a partial resection was consistent with Rasmussen's syndrome. Evaluation for tumor, infectious, and paraneoplastic etiologies was negative. There was no further progression of the residual lesion on serial MRI. Although the pathophysiologic bases of narcolepsy and Rasmussen's syndrome are unknown, they may have an autoimmune basis. This unique case of both disorders in a single patient suggests the possibility of a common underlying disease process.

Adult↗

PubMed2002

Gelastic syncope mistaken for cataplexy.

Laughter is a complex human behavior that has an emotional and a physical component. We present a patient who suffered syncope as a result of intense laughter, and hypothesize that this is analogous to other types of Valsalva-induced syncope. We emphasize the clinical characteristics that differentiate gelastic syncope from cataplexy.

Journal Article↗

PubMed1993

Scheduled naps in the management of daytime sleepiness in narcolepsy-cataplexy.

A repeated testing paradigm was used to assess the efficacy for the management of daytime sleepiness in narcolepsy-cataplexy of single long, multiple short and no-nap sleep/wake schedule conditions, with total sleep per 24 hours held constant. Eight narcoleptic subjects participated and followed each experimental schedule for two consecutive days, the second of which served as a test day during which simultaneous electroencephalogram (EEG) polygraphic recordings were made. Performance tests reported here include a grammatical transformation test and a four-choice reaction time test. A single long nap placed 180 degrees out-of-phase with the nocturnal midsleep time improved sustained performance over the no-nap condition. Reaction time performance was significantly improved in the long nap condition over the no-nap condition. Time-of-day analyses found that the greatest improvement was in the afternoon and evening. By contrast, the grammatical transformation test results suffered under the napping compared to no-nap schedules, suggesting that continuity of wakefulness and/or a long nocturnal sleep period may be important for this test. In addition, unscheduled sleep episodes tended to occur earlier in the day than the period of maximum afternoon sleep tendency seen in normal subjects. Two napping strategies are suggested for further study.

Adult↗

PubMed2005

Emerging therapies in narcolepsy-cataplexy.

In the past, narcolepsy was primarily treated using amphetamine-like stimulants and tricyclic antidepressants. Newer and novel agents, such as the wake-promoting compound modafinil and more selective reuptake inhibitors targeting the adrenergic, dopaminergic, and/or serotoninergic reuptake sites (ie, venlafaxine, atomoxetine) are better-tolerated available alternatives. The development of these agents, together with sodium oxybate (a slow-wave sleep-enhancing agent that consolidates nocturnal sleep, reduces cataplexy, and improves sleepiness), has led to improved functioning and quality of life for many patients with the disorder. However, these treatments are all symptomatically based and do not target hypocretin, a major neurotransmitter involved in the pathophysiology of narcolepsy. In this review, we discuss emerging therapies in the area of narcolepsy. These include novel antidepressant or anticataplectic, wake-promoting, and hypnotic compounds. We also report on novel strategies designed to compensate for hypocretin deficiency and on the use of immunosupression at the time of narcolepsy onset.

Genetic Therapy↗

PubMed2004

Expression of a poly-glutamine-ataxin-3 transgene in orexin neurons induces narcolepsy-cataplexy in the rat.

The sleep disorder narcolepsy has been linked to loss of hypothalamic neurons producing the orexin (hypocretin) neuropeptides. Here, we report the generation of transgenic rats expressing a human ataxin-3 fragment with an elongated polyglutamyl stretch under control of the human prepro-orexin promoter (orexin/ataxin-3 rats). At 17 weeks of age, the transgenic rats exhibited postnatal loss of orexin-positive neurons in the lateral hypothalamus, and orexin-containing projections were essentially undetectable. The loss of orexin production resulted in the expression of a phenotype with fragmented vigilance states, a decreased latency to rapid eye movement (REM) sleep and increased REM sleep time during the dark active phase. Wakefulness time was also reduced during the dark phase, and this effect was concentrated at the photoperiod boundaries. Direct transitions from wakefulness to REM sleep, a defining characteristic of narcolepsy, occurred frequently. Brief episodes of muscle atonia and postural collapse resembling cataplexy were also noted while rats maintained the electroencephalographic characteristics of wakefulness. These findings indicate that the orexin/ataxin-3 transgenic rat could provide a useful model of human narcolepsy.

Animals↗

PubMed1998

Impaired circadian waking arousal in narcolepsy-cataplexy.

The 24-hour sleep/wake distributions of untreated patients with narcolepsy-cataplexy and matched normal habitual nappers were compared using home ambulatory monitoring. Subjects followed their usual sleep patterns including, for the habitual nappers, a self-selected daytime nap. There were no differences in 24-hour totals of sleep between groups other than a small increase in SWS in narcolepsy. Narcolepsy showed greater amounts of day sleep (stages 2, SWS, REM and total sleep) and less night sleep (stage 2, total sleep). Data were collapsed into 5 min epochs and entered into a matrix. The data in the two groups were then "wrapped" (re-aligned) around the 24 hours with phase 0 as each of the times of: evening sleep onset, onset of SWS, mid-point of night sleep and moment of morning awakening. In habitual nappers alignment beginning at morning wake-up produced the highest amplitude, least temporal dispersion and greatest kurtosis of daytime sleep (naps). The 24-hour sleep/wake distribution curves of both subject groups (data aligned at morning wake-up) based on collapsed data into 5 min bins then underwent curve fitting using 15th order polynomial regression. As with visual analyses of the raw data, the curve fits confirmed that the peak in daytime sleep propensity in narcoleptics was earlier by about 40 (2.66 hours). It was concluded that decreased daytime amplitude of a circadian arousal system was the most parsimonious explanation for the increased amount, broader temporal distribution and relative phase advance of day sleep in narcolepsy and that, as well, such a mechanism could explain a number of other features of the disease.

Chronobiology Disorders↗

PubMed1999

Narcolepsy-cataplexy syndrome associated with DRB10806-DQB0602 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↗

PubMed2002

[Nacrolepsy manifesting initially as cataplexy and sleep paralysis: usefulness of CSF hypocretin-1 examination for early diagnosis].

We report a 24-year-old man with narcolepsy initially suffered from cataplexy and sleep paralysis. From May 2000, at age 23 he experienced two kinds of recurrent episodes of weakness without altered consciousness; one was provoked by emotion and excitement, the other occurred spontaneously on onset of sleep without hallucination. He denied having daytime sleepiness and did not experience hypnagogic hallucinations. In July 2000, at our hospital he received the first medical examinations, of which physical and neurological results were unremarkable. A magnetic resonance imaging scan of the brain also gave unremarkable results. The initial diagnosis was epilepsy, and anti-convulsant drugs were begun in August 2000. The weakness episodes were not lessened by the treatment with carbamazepine, sodium valproate or clonazepam, and he was admitted to our clinic in April 2001 for further examinations. Human leukocyte antigen testing was positive for DR15 (DR2) and DQ6 (DQ1). The routine electroencephalographam detected no epileptic discharge or paradoxical alpha blocking. A polysomnogram showed a sleep onset REM sleep period and sleep fragmentation, but there was no apnea or periodic leg movements. A multiple sleep latency test showed a mean sleep latency of 1.8 min and REM sleep in three of five naps. These findings suggested probable narcolepsy, so we examined the hypocretin-1 (orexin A) concentration in his cerebrospinal fluid (CSF). It was below the detection limit of the assay (< 40 pg/mL). The final diagnosis in April 2001 was narcolepsy. Making an initial diagnosis of incomplete or atypical narcolepsy is difficult for clinicians. A delay in diagnosis, however, may produce personal and social problems for narcoleptic patients. We believe that an examination of CSF hypocretin-1 aids in the early diagnosis of narcolepsy.

Adult↗

PubMed1953