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PubMed2005

[Clinical features of early-onset narcolepsy].

OBJECTIVE: To investigate the clinical features of early onset narcolepsy. METHODS: The clinical data of 105 consecutive patients with narcolepsy, 63 of which with an onset age of 9.7 +/- 3.1 on average and 42 with an onset age of 22.8 +/- 9.3 on average. Interrogation, physical examination, CT, and MRI were carried out. Polysomnography was conducted. Then the data were compared between these 2 groups. RESULTS: All 105 patients showed daytime sleepiness. The incidence rate of cataplexy was 92% in the early onset group, significantly higher than that of the late onset group (P = 0.023). There were no significant differences in the rates of sleep paralysis, hypnagogic hallucination, and disturbed nocturnal sleep. Multiple sleep latency test showed that the mean sleep latency of the early-onset group was 4.5 +/- 4.0 min, significantly shorter than that of the late onset group (7.0 +/- 5.7 min, P = 0.018); the REM sleep latency of the early onset group was 3.4 +/- 3.2 min, significantly shorter than that of the late onset group (4.8 +/- 2.2 min, P = 0.02). The number of REM sleep of the early onset group was 3.4 +/- 2.0, significantly more than that of the late onset group (2.5 +/- 1.9, P = 0.09). The apnea and hypopnea index of the late onset group was 9.2 +/- 16.5, significantly higher than that of the early onset group (1.9 +/- 6.3, P = 0.009). The 3 cases of narcolepsy with family history were all cases of early onset narcolepsy. CONCLUSION: Early onset narcolepsy patients have more severe daytime sleepiness and higher rate of cataplexy. The pathogenesis of early onset narcolepsy may be more closely associated with genetic factors.

Adolescent↗
PubMed2006

[Symptomatic hypersomnia due to orexin deficiency in hypothalamic lesions].

Narcolepsy is characterized by excessive daytime sleepiness (EDS), cataplexy and other abnormal manifestations of REM sleep. Recently, it was discovered that the pathophysiology of idiopathic narcolepsy-cataplexy is linked to orexin ligand deficiency in the brain and cerebrospinal fluid. Orexin neurons localize in the posterior hypothalamic area, which was previously described as "waking center" by von Economo in 1920s. Hypersomnia due to orexin ligand deficiency can also occur during the course of other neurological conditions, such as hypothalamic tumor, encephalopathy and demyelinating disorder (i.e. symptomatic hypersomnia). We experienced 8 pediatric cases with symptomatic hypersomnia. These cases were diagnosed as brain tumor (n = 2), head trauma (n = 1), encephalopathy (n = 1), demyelinating disorder (n = 3) and infarction (n = 1). Six pediatric cases with orexin measurements from the literatures were additionally included and total 14 cases were studied. Although it is difficult to rule out the comorbidity of idiopathic narcolepsy in some cases, a review of the case histories reveals numerous unquestionable cases of symptomatic hypersomnia. In these cases, the occurrences of the hypersomnia run parallel with the rise and fall of the causative diseases. Most of symptomatic hypersomnia cases show both extended nocturnal sleep time and EDS consisting of prolonged sleep episodes of NREM sleep. The features of nocturnal sleep and EDS in symptomatic hypersomnia are more similar to idiopathic hypersomnia than to narcolepsy.

Adolescent↗
PubMed1991

[A case of upper brainstem infarction developing symptomatic narcolepsy after the administration of anti-convulsant drugs].

A 52-year-old woman, who had ischemic infarction in the ventral upper brainstem due to subarachnoid hemorrhage in October 1986, had recurrent sleep and cataplexy attacks from May 1987. She was receiving valproate and phenytoin daily since 1986. The diagnosis of narcolepsy was made based on the clinical symptoms and EEG findings showing REM sleep during a sleep attack. Both sleep and cataplexy attack increased in parallel with an increase of the dose of anti-convulsant drugs and disappeared immediately after the discontinuation of the treatment. The findings that suggest that the administration of anti-convulsant drug as well as the brainstem vascular lesion was deeply involved in the development of narcolepsy in this case.

Brain Stem↗
PubMed1990

[A case of atonic partial seizure].

This report presented a 4-year-old girl who had atonic partial seizure of the right leg accompanied by impaired equilibrium. This patient had a generalized tonic-clonic seizures before, and had been on anticonvulsant medication. Mild cataplexy of the right leg and flail trunk while standing occurred abruptly. Based on clinical symptoms, physiological findings, and an electroencephalogram taken at the time of seizure, the cataplexy of the right leg was diagnosed as epileptic seizures. After the dosage of anticonvulsant drug was increased, all symptoms disappeared completely. Cases of atonic partial seizure have been reported only rarely. In our case, atonic partial seizure was associated with nonepileptic equilibrium impairment, probably due to cerebral cortex dysfunction. This is an extremely rare occurrence.

Cerebral Cortex↗
PubMed1985

Narcolepsy. Clinical features and aetiology.

Narcolepsy is not a rare disease. Age of onset varies from childhood to the 5th decade. Evidence for a genetic basis stems from the overall rate of narcolepsy and/or disorder of excessive somnolence among first degree relatives. The clinical features include overwhelming episodes of sleep, excessive daytime somnolence, hypnagogic hallucinations, disturbed nocturnal sleep; manifestations of dissociated REM sleep inhibitory process, cataplexy and sleep paralysis; and a special polygraphic pattern: the sleep onset REM episode. Not all symptoms are necessarily present at the onset or even during the course of narcolepsy. Excessive daytime somnolence never disappears completely while other symptoms may. Narcolepsy is a disabling condition. Its aetiology is still poorly understood but the use of natural animal models, namely dogs and horses, has been an important contribution in the areas of genetic, pharmacological and direct neurochemical analysis. Treatment of excessive daytime somnolence is still primarily based upon CNS stimulants while treatment of cataplexy and other related symptoms rests on chlorimipramine. However, new treatments are being tested, which could be of significant value.

Adolescent↗
PubMed1996

[Narcolepsy].

Narcolepsy is a rather unknown but not exceptional condition. Its prevalence, 2 to 6/10000, is ranging among this of multiple sclerosis. Narcolepsy is remarkable for clinical, polygraphic and immunogenetic features which make it a kind of model of disorders of alertness. It was first described in 1877. It has recently benefited from consistent pathophysiological progresses, which have been facilitated by the discovery of a natural canine model. The two main symptoms are irresistible and refreshing episodes of sleep and cataplexy a loss of muscle tone emotionally triggered. Polygraphically the sleep onset REM period is the major feature. Immunogenetically the condition is remarkable for an almost 100% association with HLA DR2-DQ1. Narcolepsy is a debilitating, chronic condition. Its treatment is threefold including stimulants against excessive daytime sleepiness and irresistible episodes of sleep, modafinil a new compound with awakening properties has just been introduced, antidepressants against cataplexy and associated symptoms and hypnotics against disrupted sleep.

Adolescent↗
PubMed1998

[Narcolepsy].

Narcolepsy is a disorder of sleep with uncontrolled sleepiness and cataplexy. Its prevalence in Japanese is estimated to be 0.16-0.59%. The handicap of narcoleptic patients is even larger than that of epileptic patients. The etiology is still unknown, however, a tight association between specific HLA (DR15 and QQ6) or its genotypes (DRB1*1501, DRB5*0101, DQB1*0602, DQA1*0102) and narcolepsy was recently established. This finding raises the possibility that narcolepsy might be an immunological disorder. At present, any concrete evidence supporting this possibility is lacking. Methylphenidate and pemoline are used for controlling excessive daytime sleepiness. Some tricyclic antidepressant such as clomipramine and imipramine are effective in controlling cataplexy, sleep paralysis and hypnagogic hallucination.

Antidepressive Agents, Tricyclic↗
PubMed1998

[Wake disorders. I. Primary wake disorders].

Primary wake disorders encompass various conditions of excessive daytime sleepiness and/or increased nighttime sleep, of unknown origin beginning most often in adolescence and of chronic or recurrent natural history. The best known of these conditions is narcolepsy associating two major clinical features, irresistible episodes of sleep, sleep onset REM periods and an almost constant association with HLA DR2-DQ1. The prevalence of the condition is close to the one of multiple sclerosis but positive diagnosis requires most often over 10 years to be made. The treatment of excessive daytime sleepiness has recently benefited from a new non-amphetamine awakening compound, modafinil, active in 60 to 70 p. 100 of the cases. The treatment of cataplexy still relies on antidepressants, tricyclics or selective serotonin reuptake blockers. Major advances in pathophysiology and pathogeny have been obtained through a natural model of the disease, canine narcolepsy. Pharmacological studies point to the importance of alpha-1 b adrenergic mechanisms in cataplexy, while dopaminergic systems seem more involved in excessive daytime sleepiness. As concerns genetics, the HLA DQB1*0602 gene predisposes to narcolepsy. In the canine model it is mirrored by an autosomal recessive gene showing a strong homology with the human immunoglobulin gene mu-switch. Familial studies have shown that besides typical phenotypes, attenuated forms of the condition characterized by isolated recurrent daytime naps and/or lapses into sleep do exist. In addition one or several other genes may be involved. Narcolepsy is multifactorial, including one or several genes as well as environmental factors. Idiopathic hypersomnia is noted for very long night sleep, difficulty waking up and more or less constant excessive daytime sleepiness. In contrast with narcolepsy sleep in not refreshing. There is no polysomnographic or immunogenetic special feature. Idiopathic hypersomnia is 10 times less frequent than narcolepsy. It is often overdiagnosed due to insufficient knowledge of other causes of excessive daytime sleepiness such as the upper airway resistance syndrome. Modafinil is also of great value in the treatment of idiopathic hypersomnia. In the absence of an animal model, pathophysiology and pathogeny are still poorly understood. Even rarer is the Kleine-Levin syndrome which is easily distinguishable through its recurrent character and its tendency to progressively disappear. It mainly occurs in early adolescent males. Its main features are episodes of sleep of a week duration recurring at a several months' interval along with disturbances of alimentary and sexual behavior. There is no satisfactory treatment of hypersomniac episodes. On the other hand a prophylactic treatment with carbamazepine or lithium may be active. Pathophysiology remains unsettled in spite of some evidence of a hypothalamic functional disturbance.

Animals↗
PubMed2003

Family study of affective spectrum disorder.

BACKGROUND: Affective spectrum disorder (ASD) represents a group of psychiatric and medical conditions, each known to respond to several chemical families of antidepressant medications and hence possibly linked by common heritable abnormalities. Forms of ASD include major depressive disorder (MDD), attention-deficit/hyperactivity disorder, bulimia nervosa, cataplexy, dysthymic disorder, fibromyalgia, generalized anxiety disorder, irritable bowel syndrome, migraine, obsessive-compulsive disorder, panic disorder, posttraumatic stress disorder, premenstrual dysphoric disorder, and social phobia. Two predictions of the ASD hypothesis were tested: that ASD, taken as a single entity, would aggregate in families and that MDD would coaggregate with other forms of ASD in families. METHODS: Probands with and without MDD, together with their first-degree relatives, were interviewed using the Structured Clinical Interview for DSM-IV and a supplemental interview for other forms of ASD. The familial aggregation and coaggregation of disorders were analyzed using proband predictive logistic regression models, including a novel bivariate model for the presence or absence of each of 2 disorders in a relative as predicted by the presence or absence of each of 2 disorders in the associated proband. RESULTS: In the 178 interviewed relatives of 64 probands with MDD and 152 relatives of 58 probands without MDD, the estimated odds ratio (95% confidence interval) for the familial aggregation of ASD as a whole was 2.5 (1.4-4.3; P =.001) and for the familial coaggregation of MDD with at least one other form of ASD was 1.9 (1.1-3.2; P =.02). CONCLUSIONS: Affective spectrum disorder aggregates strongly in families, and MDD displays a significant familial coaggregation with other forms of ASD, taken collectively. These results suggest that forms of ASD may share heritable pathophysiologic features.

Anxiety Disorders↗
PubMed1998

Narcolepsy in prepubertal children.

Narcolepsy was diagnosed in 51 children (29 boys). The age range was 2.1 to 11.8 years (mean, 7.9 +/- 3.1 years). A mean of three referrals was made before narcolepsy was considered. In 10 children, cataplexy was the presenting symptom. Thirty-eight children acknowledged sleep paralysis and 30 acknowledged hypnagogic hallucinations. All children had sleep studies; 31 exhibited rapid eye movement at sleep onset. The mean sleep latency was 1.5 minutes +/- 39 seconds on the Multiple Sleep Latency Test. All children had at least two sleep-onset rapid eye movement sleep episodes in this test. Forty-six children were HLA class II-positive for DQw6, and 45 were also positive for DRw15. Thirty (65%) families refused referrals to support and counseling groups. Teachers often refused to acknowledge a medical problem. During follow-up, all children presented at least once with depressive symptoms in reaction to their syndrome. Narcolepsy should be considered when evaluating children with behavioral and depressive symptoms.

Attitude to Health↗
PubMed2006

alpha2 adrenoceptors are involved in the regulation of the gripping-induced immobility episodes in taiep rats.

In 1989 Holmgren et al. (Holmgren et al. 1989 Lab Anim Sci 39:226-228) described a new mutant rat that developed a progressive motor disturbance during its lifespan. The syndrome is characterized by a tremor in the hind limbs followed by ataxia, episodes of tonic immobility, epilepsy, and paralysis. The acronym of these symptoms (taiep) became the name of this autosomic, recessive mutant rat. The taiep rats are neurological mutant animals with a hypomyelination, followed by a progressive demyelination process. At 7-8 months of age, taiep rats develop immobility episodes (IEs) characterized by a cortical desynchronization, associated with the theta rhythm in the hippocampus and changes of the nucal electromyogram (EMG), whose pattern is like rapid-eye-movement (REM) sleep. These rats also show an altered sleep pattern with an equal REM sleep distribution. This study analyzed therole of alpha(2) adrenoceptors in the expression of gripping-induced IEs in 8-month-old male taiep rats. The alpha(2) adrenoceptor agonists clonidine and xylacine increased the frequency of gripping-induced IEs whereas the alpha(2) antagonists yohimbine and idazoxandecreased or prevented such episodes. These findings correlate with the pharmacological observations in narcoleptic dogs and humans in which alpha(2) adrenergic mechanisms are involved in the modulation of cataplexy. Unexpectedly, the repetitive administration of clonidine resulted in jumping behavior, indicative of phasic activation of extensor musculature. Taken together, our results show that alpha(2) adrenoceptors are involved in the modulation in gripping-induced IEs and after the administration of several doses of clonidine produced phasic motor activation.

Adrenergic alpha-2 Receptor Agonists↗
PubMed1984

Behavioral treatment of excessive daytime sleepiness in an elderly woman with multiple medical problems.

The case is described of a 61-year-old woman who complained of excessive day-time sleepiness, periodic cataplexy, and nocturnal urinary frequency, in the context of several other medical problems. Behavioral treatment consisted of training in progressive muscle relaxation and fluid restriction to facilitate sleep maintenance, and the use of a rubber band which was snapped against the wrist to facilitate daytime arousal. Following this self-management program, improvements were noted in the following dependent measures: sleep attacks at home; sleep attacks while driving; cataplectic episodes; nocturnal awakenings, and nocturnal urine output. These improvements were maintained at 6- and 12-month follow-up. The integration of medical and behavioral strategies was highlighted.

Behavior Therapy↗
PubMed1978

Sleep respiratory arrhythmias in control subjects, narcoleptics and non-cataplectic hypersomniacs.

Normal subjects may present central-type apneas or periodic respiration during sleep (stages I and II and paradoxical sleep). The importance of these respiratory disorders increases with age. Hypersomniac patients can manifest either similar or more significant sleep respiratory disorders than normal subjects. The presence of cataplexy or obesity does not permit the prediction of the existence of respiratory arrhythmias or of their type. Sleep respiratory arrhythmias of central type are not likely to cause hypersomnia; however, an aggravating role may be played by obstructive apneas.

Adult↗
PubMed1987

Rapid eye movements, muscle twitches and sawtooth waves in the sleep of narcoleptic patients and controls.

Seventeen unmedicated patients with narcolepsy-cataplexy and 17 age- and sex-matched controls were recorded polygraphically for 3 consecutive nights. Rapid eye movements (REMs), m. mentalis twitches and sawtooth waves in the EEG were visually scored. REM and twitch densities during REM sleep were significantly higher in the patients than in the controls. The distribution pattern of REMs and twitches was altered in the patients: twitch density peaked in the first REM period and density of REMs showed an even distribution across all the REM periods of the night. In the controls both REM and twitch density increased from the first to the second REM period. We therefore assume that in the narcoleptics phasic activity of REM sleep is disinhibited. Densities of REMs, twitches and sawtooth waves did not correlate with one another in patients and controls. They appear to be independently regulated. The REM periods of the patients contained 3 times as many waking epochs as those of the controls. This suggests that in narcolepsy the transition REM/waking is selectively facilitated. The REM/NREM ratio of twitch and sawtooth wave densities was the same in patients and controls.

Adult↗
PubMed1993

Sleep paralysis.

Sleep paralysis is a common condition with a prevalence of 5-62%. Although most affected people have single or infrequent episodes, sleep paralysis may be recurrent, or occur in association with the narcoleptic syndrome. In a study of 22 subjects with frequent sleep paralysis and also excessive daytime sleepiness, episodes continued for between 5 and 35 years. In contrast to subjects with the narcoleptic syndrome, these patients did not have cataplexy, daytime sleepiness and insomnia were less severe, and there was no HLA DR2(15) or DQ1(6) association. Sleep paralysis was familial in 19 of these subjects. A non-HLA linked genetic factor, in addition to environmental factors, may thus predispose to sleep paralysis.

Cataplexy↗
PubMed2005

NREM sleep alterations in narcolepsy/cataplexy.

OBJECTIVE: NREM sleep patterns of narcoleptic patients with cataplexy were studied, focusing on their sleep 'microstructure', by analyzing the cyclic alternating pattern (CAP). METHODS: Forty-nine HLA DQB1*0602-positive patients with narcolepsy/cataplexy (32 men and 17 women, aged 18-46 years) were included together with 37 age-matched normal controls. Each subject underwent one polysomnographic night recording after an adaptation night. Sleep stages were scored following standard criteria and CAP A phases were detected and classified into 3 subtypes (A1, A2, and A3). Power spectra for frequencies between 0.5 and 25 Hz were obtained for each CAP condition, separately in sleep stage 2 and SWS. RESULTS: Narcoleptic patients displayed reduced total CAP rate. A selective reduction in the number of A1 subtypes/hour and a reduced A3 index were found in narcoleptics who had also a smaller average number of CAP sequences. Narcoleptic patients had higher power spectra for fast frequencies mostly during SWS, while REM sleep power spectra showed significantly higher power density for frequency bins 0.5-1.5, 8.5-9.5, and 17.5-25 Hz. Similarly, CAP A1 subtypes and NCAP epochs during SWS displayed significantly higher power density for fast frequency bins. CONCLUSIONS: The main finding of this study is that the occurrence of the A1 CAP subtypes is impaired during NREM sleep in narcoleptic patients. Thus, narcolepsy seems to be accompanied not only by alterations of REM but also NREM sleep which is subtly but significantly impaired, as reflected by CAP and the corresponding EEG spectral analysis. SIGNIFICANCE: Our findings might indicate that in narcolepsy very-slow oscillation processes less effective than normal might be present, with a subtly impaired capability of grouping the other sleep EEG activities; this aspect deserves further insight in order to obtain a better understanding of its functional meaning.

Adult↗
PubMed2004

Health-related quality of life in Italian patients with narcolepsy: the SF-36 health survey.

OBJECTIVE: To evaluate the psychometric performances of the 36-item short form (SF-36), a tool measuring health-related quality of life (HRQoL) in patients with narcolepsy or other sleep disorders, presenting excessive daytime sleepiness (EDS) as the main symptom. To compare the HRQoL of patients with narcolepsy with the Italian norm. To disclose the clinical predictors influencing the HRQoL. PATIENTS AND METHODS: One hundred and eight adults with narcolepsy, 115 with obstructive sleep apnea syndrome (OSAS) and 42 with idiopathic hypersomnia (IH) enrolled for a multicentric cross-section study employing self-administered questionnaires investigating the HRQoL (SF-36), EDS and cataplexy. A psychometric analysis and the standardized scoring of the 8 scales of the SF-36 were performed. The correlation between SF-36 scales and clinical features of narcolepsy was measured by a multiple linear regression analysis. RESULTS: SF-36 satisfied the recommended psychometric standards. Patients with narcolepsy had all domains except Bodily Pain lower than the Italian norm. Some of the variance of Physical Functioning (R(2) 0.07), Role functioning-Physical (R(2) 0.31), General Health (R(2) 0.15), Vitality (R(2) 0.10), Social Functioning (R(2) 0.21) and Role functioning-Emotional (R(2) 0.15) was explained both by EDS (inverse correlation) and disease duration (direct correlation). CONCLUSIONS: The SF-36 form is a reliable outcome measure for hypersomniac disorders. Narcolepsy seriously impacts patients' HRQoL in terms of physical and mental well-being and impairs physical and emotional functions, restricting social life. EDS is the main symptom determining this impairment. However, disease duration plays a role in counterbalancing this feature, probably by means of adaptation.

Activities of Daily Living↗
PubMed2002

Narcolepsy.

Narcolepsy is a chronic disorder of EDS. All patients experience EDS. Other symptoms include cataplexy, sleep paralysis, hypnagogic hallucinations, and disrupted nocturnal sleep. Treatment, usually with stimulants and low-doses of antidepressant medications, can dramatically improve the patient's quality of life. Although only advanced practice nurses may be actively involved in the diagnosis and treatment of this disorder, all nurses can encourage their patients who complain of EDS to consult a specialist in sleep disorders medicine, provide emotional support after diagnosis, and educate patients and their families about narcolepsy and its treatment.

Behavior Therapy↗