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PubMed1975

Methysergide in the treatment of narcolepsy.

Five patients with narcolepsy (four with the allied symptom of cataplexy) were treated with the serotonin antagonist methysergide. All patients had as good control of their sleep attacks while on methysergide therapy as on a control period of dextroamphetamine therapy. The cataplexy was less well controlled by methysergide than by dextroamphetamine, but improved when compared to a period without medication. Two patients developed severe calf claudication while on methysergide.

Adult↗
PubMed1979

Comparison of interwave latencies of brain stem auditory evoked responses in narcoleptics, primary insomniacs and normal controls.

A study of brain stem auditory evoked responses (BAER) was carried out in 10 narcoleptics, 10 primary insomniacs and 10 normal controls to determine if a neurophysiologic abnormality could be detected in these primary sleep disorders. The mean interpeak conduction times of Wave I-III, III-V and Iv were compared between the following groups: normal controls awake and in monitored sleep; narcoleptics awake and in monitored sleep, normal controls awake and narcoleptics awake; normal controls awake and insomniacs awake; narcoleptics awake and insomniacs awake; narcoleptics with cataplexy (n = 6) awake and narcoleptics without cataplexy (n = 4) awake. No significant differences were found which suggests that these sleep disorders represent dysfunctions which do not involve brain stem structures subserving the BAER.

Adult↗
PubMed2006

Semantic priming effect during REM-sleep inertia in patients with narcolepsy.

Patients with narcolepsy-cataplexy (NC) present excessive daytime sleepiness (EDS), cataplexy and an altered architecture of nocturnal sleep, with frequent episodes of REM-sleep at sleep onset (SOREM-sleep). This altered organization of nocturnal sleep may be accompanied by some differences in the functioning of the cognitive processes involved in the access, organization and consolidation of information during sleep. This study attempts to ascertain whether the activation of semantic memory during REM-sleep, as measured using a technique of semantic priming (namely, the facilitation of the activation of strongly-related rather than weakly-related and, overall, unrelated pairs of prime-target words) is different in NC patients compared to normal subjects. A lexical decision task (LDT) was carried out twice in wakefulness (at 10a.m. and after a 24h interval) and twice in the period of sleep inertia following awakening from SOREM and 4th-cycle REM-sleep on 12 NC patients and from 1st- and 4th-cycle REM-sleep on 12 matched controls. Reaction time (RT) to target words, taken as a measure of the semantic priming effect, proved to be longer (a) in NC patients than in control subjects; (b) in the period of REM-sleep inertia than in wakefulness; (c) in the first rather than the second session; and (d) for unrelated compared to weakly-related and, overall, strongly-related prime-target pairs. RT in post-REM-sleep sessions was less impaired, compared to waking sessions, and less dependent on the associative strength of prime-target pairs in NC patients than in normal subjects. Finally, RT of NC patients, although longer than that of normal subjects in waking sessions, significantly improved in the second session, as a consequence of either the amount of exercise or the consolidation advantage provided by REM-sleep for the procedural components of the task. The whole picture suggests a greater effectiveness of the activation of semantic memory during (SO)REM-sleep in NC patients rather than in normal subjects, and overall for the organization of new and unexpected relationships (such as those between unrelated pairs) between items of information.

Adult↗
PubMed2006

Association of HLA-DR and -DQ genes with narcolepsy in Koreans: comparison with two control groups, randomly selected subjects and DRB1*1501-DQB1*0602--positive subjects.

The purpose of this study was to investigate the association of human leukocyte antigen (HLA) class II alleles with narcolepsy-cataplexy susceptibility in Koreans. The distribution of HLA-DRB1 and -DQB1 alleles and presence or absence of DRB3/4/5 alleles were examined in 60 narcoleptic patients with clear-cut cataplexy, and the results were compared with two groups of healthy controls: 200 randomly selected controls and 144 DRB1*1501-DQB1*0602 positive controls. All of the narcoleptic patients were DRB1*1501 and DQB1*0602 positive, and their frequencies were significantly higher in patients than in random controls (100% vs 17.0%, p(c) = 2.3 x 10(-30), OR = 583.96; 100% vs 16.5%, p(c) = 3.9 x 10(-31), OR = 605.00). The HLA association in Koreans was as tight as that reported in Japanese. Several DRB1 (*0101, *0405, *0901) and DQB1 alleles (*0303, *0401, *0501, *0601, *0604) were found to have weak protective effects against narcolepsy. DRB4 showed strong protective effect, and this was also significant when compared with DRB1*1501-DQB1*0602 positive controls (18.3% vs 44.4%, p(c) = 0.001, OR = 0.28). DRB3 (OR = 1.86) and DQB1*0301 (OR = 2.45) were found to have weak predisposing effect, when compared with DRB1*1501-DQB1*0602 positive controls. The protective effect of DRB4 has to be further studied in other populations.

Adolescent↗
PubMed2006

Prazosin increases immobility episodes in taiep rats without changes in the properties of alpha1 receptors.

The taiep rat is a myelin mutant in which immobility episodes (IEs) can be induced in adult males by gripping. EEG recordings during gripping-induced IEs show a rapid eye movement (REM) sleep-like pattern, similar to that reported for narcolepsy-cataplexy suggesting that IEs represent a disorder of REM-sleep. An alpha(2) adrenoceptor agonist increases gripping-induced IEs, whereas alpha(2) antagonists decrease these. We have studied the effect of prazosin on IEs and the levels of alpha(1) adrenoceptors were evaluated in cerebro-cortical homogenates of taiep and control rats. Systemic administration of prazosin results in a significant increase in both the frequency and duration of gripping-induced IEs. Our results show that cerebro-cortical tissue is not an adequate candidate for the expression of cataplexy-like symptoms, but prazosin, an alpha(1) antagonist, is a potent inducer of gripping-induced immobility episodes in taiep rats.

Adrenergic alpha-1 Receptor Antagonists↗
PubMed2004

Factors associated with a delay in the diagnosis of narcolepsy.

BACKGROUND: There can be a long interval from the onset of symptoms before a diagnosis of narcolepsy is made. There are no multivariate analyses reported in the literature of factors that may contribute to this delay. The aims of this study were to describe the delay in diagnosis of people with narcolepsy living in the UK and to identify associated factors. METHODS: The study comprised a postal survey of 500 members of the Narcolepsy Association UK, which included questions regarding age of onset of symptoms, year of diagnosis and subject demographics. Cox's proportional hazards regression was performed. RESULTS: A total of 313 questionnaires were returned of which 219 had been completed sufficiently for analysis. The interval between symptom onset and diagnosis ranged from within 1 to 61 years with a median of 10.5 years. Multivariate analysis showed that the presence of cataplexy as one of the initial symptoms and a more recent year of symptom onset were the only factors associated with time to diagnosis. CONCLUSIONS: We have confirmed that the diagnosis of narcolepsy can be delayed for many years particularly when cataplexy is absent initially. The delay in diagnosis in the UK appears to be decreasing, probably through greater doctor and patient awareness of the clinical manifestations of narcolepsy.

Adolescent↗
PubMed2001

Narcolepsy: new understanding of irresistible sleep.

Recently, low levels of a newly identified neuropeptide, hypocretin 1, were described in the cerebrospinal fluid of patients with narcolepsy. This neurochemical finding furthers our understanding of this enigmatic sleep disorder typically characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, and hypnagogic hallucinations. Narcolepsy appears to be fundamentally related to abnormally regulated rapid eye movement sleep. The diagnosis of this disorder remains challenging because of multiple other conditions that can cause daytime sleepiness and the difficulties in recognizing cataplexy based on patient report. The role of hypocretins in narcolepsy is unclear but intriguing because the cell bodies are restricted to the lateral hypothalamus, a brain region long associated with sleep regulation, with neuronal widespread projections to areas including the locus ceruleus, ventral tegmental area, amygdala, and dorsal raphe. Hypocretins potentially modulate the activity of monoamines and acetylcholine, and therefore their absence leads to the multiple symptoms of narcolepsy. This article reviews the current understanding of the diagnosis and treatment of narcolepsy and discusses the possible implications of the hypocretin discovery.

Animals↗
PubMed1989

Treatment of narcolepsy with L-tyrosine: double-blind placebo-controlled trial.

A randomised, double-blind, placebo-controlled study of L-tyrosine was done in ten subjects with narcolepsy and cataplexy. Of twenty-eight visual analogue scales rating mood and arousal, the subjects' ratings in the tyrosine treatment (9 g daily) and placebo periods differed significantly for only three (less tired, less drowsy, more alert). Ratings of daytime drowsiness, cataplexy, sleep paralysis, night-time sleep, overall clinical response, and measurements of multiple sleep latency and tests of speed and attention did not differ significantly between tyrosine and placebo periods. Dietary supplementation with tyrosine 9 g daily for 4 weeks seems to have a mild stimulant action on the central nervous system but this effect is not clinically significant in the treatment of the narcoleptic syndrome.

Adult↗
PubMed1998

The clinical diagnosis of the narcoleptic syndrome.

Sleep-wake habits and control of postural muscle tone were investigated by self-report questionnaire in 183 subjects considered to have the narcoleptic syndrome, 62 subjects with hypersomnia and 10 with obstructive sleep apnoea. Results were compared with those in a group of 188 control subjects with normal sleep wake habits. Excessive daytime sleepiness, determined by the Epworth Sleepiness Scale (ESS), was five times greater in the narcoleptic syndrome than in control subjects (score range 0-24, mean scores +/-SD 19.6+/-3.0; and 4.5+/-3.3 respectively; P<0.001). The propensity to cataplexy, as determined by a rating scale developed to estimate the likelihood of loss of postural tone in response to sudden emotional stimuli, including laughter, was 10 times greater in narcoleptic syndrome than in control subjects (postural atonia total score range 0-600; mean + SD 334+/-122 and 28+/-45, respectively; P<0.001). Narcoleptics had more disturbances of night sleep than controls with episodes of muscle jerking, sleep walking, sleep talking and sleep terrors, as well as sleep paralysis, and higher insomnia self-rating scores. Sleep latency from bedtime to sleep-onset time was shorter in narcoleptics than controls. The hypersomniac group of 62 subjects was heterogeneous. Subsequent investigation showed that 18 subjects (29%) had idiopathic hypersomnia, four (6%) 'incomplete' narcolepsy without cataplexy and 10 (16%) hypersomnia accompanying a mood disorder. The mean ESS scores in this group and in subjects with obstructive sleep apnoea were comparable to those of the narcoleptic syndrome subject group. Mean postural atonia scores were similar to those of control subjects.

Adult↗
PubMed1994

Modafinil in diurnal sleepiness. A study of 123 patients.

The efficacy of modafinil, a psychostimulant drug acting on postsynaptic alpha-1 adrenergic receptors, as a treatment for excessive daytime sleepiness was studied in 123 patients. Subjects included 94 narcoleptic patients (76 with cataplexy), 23 hypersomnia patients and 6 patients with disrupted nocturnal sleep (DNS) and excessive daytime sleepiness (EDS). Clinical efficacy of the treatment on each symptom (EDS, cataplexy and DNS) was evaluated on a four-point scale from excellent to absent. The effectiveness of modafinil as a treatment for EDS was excellent in 17% of all patients, good in 63%, fair in 17% and absent in 3%. The incidence of side effects was rather low (14 of 123 patients) and most of them disappeared (11 patients) when doses were reduced.

Adolescent↗
PubMed2003

Effects of IV and ICV hypocretin-1 (orexin A) in hypocretin receptor-2 gene mutated narcoleptic dogs and IV hypocretin-1 replacement therapy in a hypocretin-ligand-deficient narcoleptic dog.

STUDY OBJECTIVES: Using two different canine models of narcolepsy, we evaluated the therapeutic effects of hypocretin-1 on cataplexy and sleep. MEASUREMENTS AND RESULTS: Intracerebroventricular administration of hypocretin-1 (10 and 30 nmol per dog) but not intravenous administration (up to 6 microg/kg) induced significant wakefulness in control dogs. However, hypocretin-1 had no effect on cataplexy or wakefulness in hypocretin receptor-2 gene (Hcrtr2) mutated narcoleptic Dobermans. Only very high intravenously doses of hypocretin-1 (96-384 microg/kg) penetrated the brain, to produce a short-lasting anticataplectic effect in a hypocretin-ligand-deficient animal. CONCLUSIONS: Hypocretin-1 administration, by central and systemic routes, does not improve narcoleptic symptoms in Hcrtr2 mutated Dobermans. Systemic hypocretin-1 hardly crosses the blood-brain barrier to produce therapeutic effects. The development of more centrally penetrable and longer lasting hypocretin analogs will be needed to further explore this therapeutic pathway in humans.

Animals↗
PubMed1986

Sleep fragmentation in canine narcolepsy.

Genetically narcoleptic dogs were recorded continuously for 24 h to examine their sleep-wake patterns and to evaluate the extent of sleep fragmentation. Three narcoleptic and three control dogs from each of two affected breeds (Labrador retrievers and Doberman pinschers) were surgically implanted with electrodes for recording standard sleep parameters. Recordings were scored in 30-s epochs for the states of active waking, drowsiness, light sleep, deep slow wave sleep, REM sleep, and cataplexy. All affected dogs displayed marked fragmentation and disruption of the sleep-wake cycle characterized by repeated awakenings, frequent shifts in sleep stages, numerous attacks of cataplexy occurring from active waking, and a disturbance of the normal REM-NREM periodicity. This sleep disruption was reflected in significantly greater numbers of episodes of each behavioral state as well as in a 38% increase in the total number of all states. These results demonstrate a severe disturbance of the normal sleep pattern in canine narcoleptics. The possibility of a general dysfunction of circadian organization is discussed.

Animals↗
PubMed1986

Nocturnal sleep of narcoleptic patients: revisited.

Narcoleptic patients were compared to idiopathic hypersomniac patients and REM hypersomnia patients with regard to nocturnal sleep disruption. Results showed specificity of the narcoleptic sleep pattern and a possible correlation between REM fragmentation and cataplexy. Patients with and without periodic movements in sleep (PMS) were compared and no difference was found in their nocturnal sleep pattern, suggesting that PMS is not the major determinant of sleep disruption previously described in narcoleptic patients. Finally, nocturnal sleep disruption was treated with gamma-hydroxybutyrate (GHB) and results further indicate a possible link between REM fragmentation and cataplexy.

Cataplexy↗
PubMed1986

Therapeutic effects of mazindol on narcolepsy.

Mazindol, a new anorexiant, was administered at a daily dose of 0.5-4 mg to 10 narcoleptic subjects aged 21-63 years. All the patients suffered from sleep attacks and one or more of the REM-related symptoms. Eight patients received only mazindol, and two patients received mazindol simultaneously with clomipramine or flurazepam. Sleep attacks were reduced in nine patients, and cataplexy was also markedly reduced in four patients. Mild adverse reactions were reported in six patients: two patients complained of headache, four of nocturnal sleep disturbance, and two of reduced appetite. Most side effects disappeared spontaneously or after dose reduction, and none of the patients had to stop medication. The results suggest that mazindol is effective not only for sleep attacks but also for cataplexy. It is recommended as a treatment for mild cases of narcolepsy.

Adult↗
PubMed1996

Symptom description and management in narcolepsy.

Narcolepsy is a neurologic condition that is chronic and lifelong. The study reported describes the experience of living with the most prevalent symptoms of narcolepsy and the management strategies employed for those symptoms by persons with narcolepsy. Descriptions of living with excessive daytime sleepiness, sleep attacks, and cataplexy are given. Nonpharmacologic strategies for symptom management were described by 63.2% of the study participants for cataplexy, 54.5% for sleep attacks, and 35.5% for excessive daytime sleepiness. Specific management and coping strategies are provided.

Adaptation, Psychological↗
PubMed1988

Narcolepsy and HLA in the Japanese.

Narcolepsy is characterized by excessive daytime sleepiness and cataplexy. It had been reported that 100% of Japanese and Caucasian narcoleptic patients are HLA-DR2 and HLA-DQw1 positive; last year, however, exceptionally rare cases of DR2-negative narcolepsy were reported. Conversely, we tested 190 Japanese narcoleptic patients, and all were still DR2 and DQw1 positive. Among several symptoms in narcoleptic patients, HLA-DR2 showed the strongest association with cataplexy. Exclusive association has been demonstrated between narcolepsy and a specific band of EcoRI-digested DQ beta fragments. In this paper Taq I-digested genomic DNAs were hybridized with a DQ alpha probe, and five specific bands were observed to associate strongly with particular HLA-DR or D specificities. Interestingly, the 6.0-kb band was found in 100% of 28 narcoleptic patients and in 54% of DR2-positive normal control subjects. For the time being, HLA-Dw2 is the better marker of the HLA-associating narcoleptic gene than is DR2, DQw1, 2.4-kb band of EcoRI-digested DQ beta, and 6.0-kb band of Taq I digested DQ alpha, because the frequency of DW2 is the lowest in normal control subjects.

Blotting, Southern↗
PubMed2003

Hypocretin (orexin) deficiency in narcolepsy and primary hypersomnia.

The discovery that hypocretins are involved in narcolepsy, a disorder associated with excessive daytime sleepiness, cataplexy, and unusually rapid transitions to rapid eye movement sleep, opens a new field of investigation in the area of disorders of sleep and activation. Hypocretin-1 (hcrt-1) and hypocretin-2 (hcrt-2) (also called orexin-A and orexin-B) are newly discovered neuropeptides processed from a common precursor. Hypocretin containing cells are located exclusively in the lateral hypothalamus, with widespread projections within the central nervous system. The role of the hypocretin system in other disorders causing excessive daytime sleepiness is more uncertain. This study reports the findings of a prospective study measuring cerebrospinal fluid concentrations of hypocretin-1 and hypocretin-2 in HLA DQB1*0602 positive narcolepsy with cataplexy, monosymptomatic narcolepsy, and primary hypersomnia. The results confirmed the previous observations, that hcrt-1 is deficient in narcolepsy and for the first time report very low levels of hcrt-1 in primary hypersomnia. It is also reported for the first time that there is a generalised defect in hcrt-2 transmission in all three of these clinical entities compared with controls.

Adult↗