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At least 667 records · Page 37Linked to original sources
PubMed2001

[Narcolepsy in a prepubertal boy].

We reported a 10-year-old boy with narcolepsy, the onset of which was at the age of 8 years and 6 months. The initial symptom was excessive daytime sleepiness, followed by cataplexy and disrupted nocturnal sleep. There was neither hallucination nor sleep paralysis. A daytime polysomnogram showed a sleep-onset rapid eye movement period (SOREMP), and human leukocyte antigen (HLA) analysis revealed HLA-DR2/DQB1 * 0602. Treatment with methylphenidate and clomipramine was effective; methylphenidate (30 mg/day) improved his wakefulness and alertness throughout the day, and clomipramine (20 mg/day) reduced the number of cataplexic episodes. Because of their abnormal behavior, prepubertal narcoleptic children may often be misdiagnosed as having epilepsy or an attention deficit hyperactivity disorder. Therefore, they need early diagnosis and treatment. Assistance should be provided to protect them from being labeled as lazy by their parents and school teachers.

Child↗
PubMedDate not supplied

[Narcolepsy in children].

INTRODUCTION: Narcolepsy is a neurological disorder characterized by excessive somnolence during the daytime, with recurrent, irresistible episodes of sleepiness. The complete forms are associated with cataplexy, hypnagogic hallucinations and sleep paralysis. The incidence reported in adults is 4 10/10,000. A considerable proportion of adults consider their disorder to have started before the age of 15 years. It is essential to have neurophysiological confirmation of the short period of the onset of REM sleep for diagnosis. CLINICAL CASE: An 11 year old boy with diurnal hypersomnolence, behavior disorder and weight gain. He was evaluated in the Sleep Disorder Unit by polysomnography and the multiple latency test, which confirmed the suspicion of narcolepsy. CONCLUSIONS: Narcolepsy is a disorder which starts during childhood and usually goes unnoticed or is erroneously diagnosed and treated. At the present time there are valid criteria for the identification and diagnosis of children with this disorder. Treatment of these patients should be orientated towards adaptation of the environment to the child and prevention of the psychosocial problems which may be caused by this disorder, in view of the poor response to the drugs used.

Central Nervous System Stimulants↗
PubMed2001

[Unwanted napping].

Three patients, one woman aged 43, and two men aged 32 and 51, suffered from excessive daytime sleepiness with different causes. The woman experienced nocturnal motor attacks of epileptic origin, responding to carbamazepine. The diagnosis was based on polysomnographic recordings combined with video monitoring. Narcolepsy was diagnosed in the 32-year-old man. He also suffered from cataplexy. The diagnosis was supported by a multiple sleep latency test and HLA-DR2 positivity. He was treated with clomipramine. In the 43-year-old man an obstructive sleep apnoea syndrome was diagnosed by polysomnographic recording. He was treated successfully with continuous positive airway pressure. These patients show that excessive daytime sleepiness is sometimes difficult to recognise and can be a potentially incapacitating symptom of a treatable underlying disorder. Diagnosis is made by careful history taking and through the use of different types of somnographic recordings.

Adult↗
PubMed2001

[Physiopathology of idiopathic hypersomnia. Current studies and new orientations].

In 1976 Bedrich Roth coined the term "idiopathic hypersomnia" and described two forms of the disease, one monosymptomatic, manifested only by excessive daytime sleepiness, and one polysymptomatic, characterized by excessive daytime sleepiness, nocturnal sleep of abnormally long duration and signs of "sleep drunkenness" on awakening. In comparison with that of narcolepsy, the pathophysiology of idiopathic hypersomnia remains poorly known. There are two main reasons for that: the absence of clinical and polysomnographic criteria pathognomonic or at least characteristic of the condition, as the cataplexies and the sleep onset REM periods of narcolepsy, and also the absence of a natural animal model comparable with the canine model of narcolepsy. The first investigations have stressed the frequent familial pattern of idiopathic hypersomnia. Later on biochemical assays have been performed in the CSF with results in favour of a dysfunction of noradrenergic systems. In the light of the two process model of sleep regulation in which sleep propensity is determined by a homeostatic process S and a circadian process C and of the later three-process model of regulation in which sleepiness/alertness are simulated by the combined action of a homeostatic process, a circadian process and sleep inertia, we suggest that idiopathic hypersomnia is not a pathological entity in itself, but rather the consequence of chronic sleep deprivation in very long sleepers.

Arousal↗
PubMed2002

Pineal gland hormone and idiopathic scoliosis: possible effect of melatonin on sleep-related postural mechanisms.

Experimental and clinical evidences indicate that endocrine mechanisms, particularly involving the pineal gland, exert a role in the development of postural deficits leading to the occurrence of idiopatic scoliosis (IS). In particular, experiments performed in bipedal animals have shown that removal of the pineal gland, which secretes melatonin (M), induced a scoliosis, and that in such preparations, administration of this hormone prevented the development of this deformity (cf. 131). It appears also that adolescents with IS showed a reduced level of serum M with respect to age-related control subjects. The possible mechanisms involved in the M regulation of the tonic contraction of the axial musculature have been discussed. It is known that the pineal gland is implicated in the control of circadian rhythms, including the sleep-waking cycle, and that during this cycle there are prominent changes in postural activity, which affect not only the limbs, but also the axial musculature. These changes are characterized by a decrease followed by a suppression of postural activity, which occur particularly during transition from wakefulness to synchronized sleep and, more prominently, to rapid eye movement (REM) sleep. Episodes of postural atonia may also occur during the cataplectic episodes, which are typical of narcolepsy. Cholinergic and/or cholinoceptive neurons located in the dorsal pontine reticular formation (pRF) and the related medullary inhibitory reticulospinal (RS) system, intervene in the suppression of posture during REM sleep, as well as during the cataplectic episodes which occur in narcolepsy. These structures are under the modulatory (inhibitory) influence of the dorsomedial and the dorsolateral pontine tegmentum, where serotoninergic raphe nuclei (RN) neurons and noradrenergic locus coeruleus (LC) neurons are located. We postulated that M may act not only on the circadian pacemaker, but also directly on the pontine tegmental structures involved in the regulation of posture during the animal states indicated above. This hypothesis is supported by the facts that: 1) the dorsal pRF may contain specific binding sites for M; 2) this structure is particularly sensitive to M in adolescents, as well as in adult subjects affected by narcoleptic disturbances leading to cataplexy; 3) M increases the release of serotonin (5-HT), a neurotransmitter which enhances the postural tone by acting on the dorsal pRF: on the other hand, deficits in M levels may lower the activity of the serotoninergic raphe system, thus leading to a decrease or suppression of postural activity similar to that occurring either during REM sleep or during the cataplectic episodes typical of narcoleptic patients; 4) IS patients may show episodes of sleep apnea, a phenomenon which has been attibuted to a reduced tonic contraction of primary and accessory respiratory muscles during REM, resulting from a reduced release of 5-HT at dorsal pontine level. It has been postulated that, if the reduced M and 5-HT levels are subliminal to produce a complete suppression of posture under the conditions reported above, the reduced postural tone, which results from this condition may lead to the development of IS, due to hypotonia which affects the axial musculature. M secretion could be regulated not only by the activity of the serotoninergic raphe neurons projecting to the pineal gland, but probably also by the activity of noradrenergic LC neurons. It is likely that the development of IS, which results from a reduced level of M and 5-HT, may occur provided that the noradrenergic LC inhibition of the pontine structures is impaired. Such impairment could depend upon genetic factors, similar to those postulated to play a role in narcolepsy. In conclusion, the possibility exists that an impaired activity of brain monoaminergic systems may lead to disfunction in the production of M, which is apparently an important factor in the etiopathogenesis of IS.

Animals↗
PubMed2002

[Three cases of metastatic meningeal carcinomatosis from lung cancer].

We report three cases of meningeal carcinomatosis that metastasized from lung cancer. The patients were men of 73, 65 and 77 years old. The histological type was adenocarcinoma in all cases. At the time of emergence of neurological symptoms such as nausea, headache and cataplexy, enhanced CT of the brain did not disclose brain metastasis. Although brain MRI failed to detect abnormal meningeal findings in cases 1 and 2, meningeal carcinomatosis was diagnosed by cerebrospinal fluid cytology in all three cases. As for treatment, in case 1, methotrexate and prednisolone were administered intrathecally, while the optimum supportive care was given in cases 2 and 3. Because it is difficult to detect meningeal carcinomatosis by brain CT and MRI alone, careful neurological observation and cerebrospinal fluid cytology are necessary for its diagnosis.

Adenocarcinoma↗
PubMed1997

[Neurophysiology and analysis of the phenomenon of catalepsy].

Neurophysiological mechanisms of the photogenic catalepsy (the "animal hypnosis"), genetic catalepsy, and cataplexy are discussed. The data obtained demonstrates a significance of the brainstem structures suppressing motor activity and the muscle tone in these conditions. Motor disorders associated with the general immobility are discussed from the standpoint of the evolutionary theory.

Adaptation, Physiological↗
PubMed2003

Excitation of ventral tegmental area dopaminergic and nondopaminergic neurons by orexins/hypocretins.

Orexins/hypocretins are involved in mechanisms of emotional arousal and short-term regulation of feeding. The dense projection of orexin neurons from the lateral hypothalamus to mesocorticolimbic dopaminergic neurons in the ventral tegmental area (VTA) is likely to be important in both of these processes. We used single-unit extracellular and whole-cell patch-clamp recordings to examine the effects of orexins (A and B) and melanin-concentrating hormone (MCH) on neurons in this region. Orexins caused an increase in firing frequency (EC(50) 78 nm), burst firing, or no change in firing in different groups of A10 dopamine neurons. Neurons showing oscillatory firing in response to orexins had smaller afterhyperpolarizations than the other groups of dopamine neurons. Orexins (100 nm) also increased the firing frequency of nondopaminergic neurons in the VTA. In the presence of tetrodotoxin (0.5 microm), orexins depolarized both dopaminergic and nondopaminergic neurons, indicating a direct postsynaptic effect. Unlike the orexins, MCH did not affect the firing of either group of neurons. Single-cell PCR experiments showed that orexin receptors were expressed in both dopaminergic and nondopaminergic neurons and that the calcium binding protein calbindin was only expressed in neurons, which also expressed orexin receptors. In narcolepsy, in which the orexin system is disrupted, dysfunction of the orexin modulation of VTA neurons may be important in triggering attacks of cataplexy.

Action Potentials↗
PubMed2003

Voxel-based morphometry in hypocretin-deficient narcolepsy.

STUDY OBJECTIVES: Recent studies suggest that narcolepsy is caused by degeneration of hypocretin (orexin) producing neurons. To find evidence for this hypothesis, we aimed to detect structural changes in the hypothalamus and/or hypocretin projection areas of patients with narcolepsy. DESIGN: We used voxel-based morphometry (VBM), an unbiased MRI morphometric method with a high sensitivity for subtle changes in gray and white matter volumes. SETTING: Image acquisition was carried out in the department of Radiology at Leiden University Medical Center; image post-processing was performed in the Wellcome Department of Cognitive Neurology, London. PARTICIPANTS: Fifteen narcoleptic patients were studied, all having cataplexy and typical findings on Multiple Sleep Latency Testing. All patients were HLA-DQB1*0602 positive and hypocretin-1 deficient. The control group consisted of 15 age and sex matched healthy subjects. MEASUREMENTS AND RESULTS: We found no differences in global gray or white matter volumes between patients and controls. Furthermore, regional gray or white matter volumes in the hypothalamus and hypocretin projection areas did not differ between patients and controls. CONCLUSIONS: VBM failed to show structural changes in the brains of patients with narcolepsy. This suggests that narcolepsy either is associated with microscopic changes undetectable by VBM or that functional abnormalities of hypocretin neurons are not associated with structural correlates.

Adult↗
PubMedDate not supplied

[Rat behavior in the "open field" next day after haloperidol injection: dependence on experimental conditions].

Wistar rats were injected with haloperidol (3.5 mg/kg) that resulted in a high level of cataplexy. Next day after haloperidol injection rat behavior was studied in the open field. The animals were divided in two groups. The first group of animals was tested in the daylight without additional illumination of the open-field chamber. The second group was tested in a darkened room with additional intense illumination of the open-field center with a 60W bulb. The testing time was 240 s. The high level of the open-field locomotor activity in the first group was attributed to anxiety. The low level of locomotor activity in the second group was qualified as depressive state.

Animals↗
PubMed2003

[A six year-old case of narcolepsy].

We report here a 6-year-old boy with narcolepsy. The diagnostic criteria were met by the clinical symptoms including excessive daytime sleepiness and cataplexy, and by the results of overnight polysomnography (PSG), multiple sleep latency test (MSLT), and human leukocyte antigen (HLA). PSG showed increased ratio of sleep stages 1 and 2 due to frequent awakening. All the five test session of MSLT showed a sleep onset REM period. HLA typing was positive for DRB1* 1501 and DQB1* 0602. Though the present case had very early onset, all the clinical symptoms and results of sleep studies met the criteria of narcolepsy. The CSF orexin level was far below the lower limit of the control values. It is very useful to measure CSF orexin for the diagnosis of early onset narcolepsy.

Biomarkers↗
PubMed2003

[Excessive daytime sleepiness and low CSF orexin-A/hypocretin-I levels in a patient with probable progressive supranuclear palsy].

We report a 74-year-old woman with excessive daytime sleepiness (EDS) who were diagnosed as probable progressive supranuclear palsy (PSP). Her EDS mimicked narcolepsy without cataplexy, because multiple sleep latency tests showed short latencies, human leukocyte antigen testing was positive for DR2/DQB1, and orexin A (hypocretin-I) concentration in her cerebrospinal fluid was undetectable. In PSP, neurofibrillary tangles appears in the hypothalamus, neuronal loss and gliosis are seen in a number of pontine and mesencephalic tegmental nuclei, substantia nigra, locus caeruleus. These neuropathological changes of PSP may cause decreased pre- or post-synaptic hypothalamic orexin neurotransmission because orexin neurons are located in the hypothalamus and project widely to the forebrain and the brain stem. In our patient, the treatment with methylphenidate HCl was effective on EDS.

Aged↗
PubMed1992

Evaluation of daytime sleepiness.

In summary, the evaluation of the tired patient requires an awareness of the various meanings of tiredness. Furthermore, it is important to differentiate normal sleepiness that is a product of circadian rhythm variation in vigilance from pathologic sleepiness. Sleepiness that results from faulty habits, e.g., altered sleep scheduling, drugs, or sleep restriction, can be readily discerned with the aid of a sleep-wake diary. Because subjective sleepiness is often unappreciated, especially in patients with sleep apnea, methods that rely on self-ratings of the severity of sleepiness, e.g., visual analogue scale, 10-cm line, or SSS may not coincide with performance tasks, observer assessments, or such physiologic methods as the MSLT. Less commonly employed neurophysiologic methods include pupillometry and averaged evoked potentials. On the other hand, the MSLT is commonly used for the detection of physiologic sleepiness. Moreover, it is helpful in evaluating response to treatment. A variation of the MSLT, the MWT, which instructs the individual to remain awake, does not discriminate between sleep onset times for wakefulness and the MSLT for sleepiness in normal subjects. The MWT may be useful for the assessment of treatment responses for excessive daytime sleepiness, e.g., narcolepsy, and for determining the frequency of daytime sleep episodes. The differences that have been observed between behavioral measures and physiologic measures of sleepiness suggest that these techniques assess different aspects of sleepiness. HLA typing (DR2, DQw1) has been shown to be a useful method for corroborating narcolepsy-cataplexy, but the antigens are neither specific for the disorder nor for sleepiness alone.

Diagnosis, Differential↗
PubMed1992

The treatment of sleep disorders.

Narcolepsy is clinically associated with cataplexy, sleep paralysis and hypnagogic hallucinations. It is treated by reassurance (that there is no physical disease) and by stimulants such as ephedrine and amphetamine on an intermittent basis. The special tricyclic antidepressant clomipramine is also used, and mono-amine oxidase inhibitors (MAOIs) are useful in theory. Obstructive sleep apnoea is an important and often unrecognised cause of daytime somnolence. It is treated by weight reduction (pickwickian syndrome), hormones, or recently, with continuous positive pressure apparatus. Night terrors (pavor nocturnus) and sleepwalking typically occur during deep sleep (stage 3 and 4 throughout the episode) in children. In a night terror the child sits up with a scream, with eyes open, but inaccessible. He eventually falls asleep calmly. Sleepwalking, too, shows the features of inaccessibility and subsequent amnesia for the episode. Both conditions are normally treated with reassurance (to the parents) but may occasionally warrant benzodiazepines. Enuresis usually occurs in non-rapid eye movement (NREM) sleep, especially stages 3 and 4. The reason for the efficacy of tricyclic antidepressants is not precisely known. Delirium tremens (DT) is treated as a rebound excess of REM sleep, with benzodiazepines and other drugs. It is the withdrawal syndrome (with or without major seizures) to the barbiturate-alcohol group of drugs, which includes alcohol, chloral, paraldehyde, glutethimide, methylprylone, ethchlorvynol, meprobamate and meprobamate-diphenhydramine. Insomnia may be treated by the above drugs, by analgesics, antidepressants, major tranquillisers (neuroleptics) and miscellaneous other compounds. For the majority of patients, however, the most suitable group seems to be the benzodiazepines. The benzodiazepines are much safer than their predecessors, in both acute and chronic usage.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2006

[Narcolepsy--new implications of molecular biology].

Narcolepsy is a common but underdiagnosed sleep disorder characterized by excessive daytime sleepiness and abnormal manifestations of REM sleep: cataplexy, sleep paralysis and hypnagogogic hallucinations. Sleep onset REM periods (SOREMPs) are diagnostic. Within the last five years the pathogenesis has become clearer: narcolepsy is associated with almost total absence of the neuropeptides hypocretin-1 and -2 in the hypothalamus and CSF. A low level of CSF hypocretin is now recognized as a new diagnostic tool. This review provides updated knowledge of narcolepsy.

Adolescent↗
PubMed2006

Fatigue in systemic lupus erythematosus: contributions of disordered sleep, sleepiness, and depression.

OBJECTIVE: To clarify the role of sleep disorders, sleepiness, and depression in patients with systemic lupus erythematosus (SLE) who complain of disabling tiredness. METHODS: Patients with SLE (31 women, 4 men) with disabling tiredness were evaluated with the Epworth Sleepiness Scale (ESS) and overnight polysomnography, followed by daytime multiple sleep latency tests (MSLT) and the Beck Depression Inventory (BDI). Their polysomnography was compared with 17 healthy, asymptomatic controls. RESULTS: Polysomnography of the patients in comparison with healthy controls showed impaired sleep efficiency (p < 0.02), high arousal frequencies (p < 0.01), increased stage 1 sleep (p < 0.02), decreased stage 3/4 slow-wave sleep (p < 0.02), and a high percentage (77% of patients) with increased alpha-EEG non-REM sleep. In 23% of patients periodic limb movement (PLM) disorder was observed (mean PLM index 31.1 +/- 15); 26% of patients had obstructive sleep apnea (mean apnea/hypopnea index 19.3 +/- 10), and one patient had narcolepsy-cataplexy. Remarkably, 51% of patients were excessively sleepy on both the ESS and MSLT (mean sleep latency < 10 min). This excessive daytime sleepiness was not related to sleep restriction. There was no association between sleepiness and SLE disease features such as neuropsychiatric SLE, medications, fibromyalgia, or disease activity. As a whole, the study group reported mild to moderate depression (mean BDI = 15.8 +/- 9.9). Within the group, the sleepy patients had lower BDI scores than the non-sleepy patients (p < 0.02), and fewer of the sleepy patients were depressed (p < 0.04). CONCLUSION: Primary sleep disorders, sleepiness, and depression are common in tired SLE patients. Tiredness in SLE that is the result of excessive daytime sleepiness can be distinguished from tiredness of depression. Such distinctions will help identify appropriate treatment for tired patients with SLE.

Adult↗
PubMed1990

Worldwide use of clomipramine.

Clomipramine, a preferential inhibitor of 5-hydroxytryptamine uptake, has proven effective in the management of depression, resistant depression, and obsessive compulsive disorder. Investigators have also reported benefits of this medication in patients with phobia, panic disorder, chronic pain, Gilles de la Tourette's syndrome, premature ejaculation, anorexia nervosa, cataplexy, and enuresis. In double-blind studies of patients with depression, clomipramine has been significantly more effective than placebo and equivalent to standard tricyclics. Clomipramine is particularly well suited for the treatment of resistant depression, for which its efficacy may be enhanced by combination therapy with tryptophan and/or lithium. In at least 12 double-blind comparative trials, clomipramine has exhibited significant benefit in patients with obsessive compulsive disorder, this efficacy not being limited to patients with an associated depressive illness. In the United States, clomipramine is approved only for the treatment of obsessive compulsive disorder.

Anxiety Disorders↗
PubMed1990

Narcolepsy in a young Chinese child.

Narcolepsy is characterized by narcoleptic sleep attacks, cataplexy, sleep paralysis and hypnagogic hallucinations. It is very rare in children. A 7-year old Chinese boy presenting with typical narcoleptic symptoms is reported. Diagnosis of narcolepsy usually relies on the clinical picture, the presence of sleep-onset REM periods on nocturnal polysomnograph and the Multiple Sleep Latency Test. The strong association with HLA-DR2 may help in diagnosis, especially in children.

Child↗