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PubMed1994

Selegiline in the treatment of narcolepsy.

We treated 17 narcolepsy patients in a placebo-controlled, double-blind, crossover trial with 10-, 20-, 30-, and 40-mg daily doses of selegiline, a monoamine oxidase inhibitor widely used in Parkinson's disease. There was a dose-dependent as well as a statistically and clinically significant improvement in narcoleptic symptoms and polygraphic measures. At 40 mg, there was a 36% reduction in the number of daytime sleep episodes and a 34% reduction in their duration (compared with placebo, mean values). The number of excessive sleepiness episodes decreased by 43%, and the duration decreased by 47%. The number of cataplectic attacks was reduced by 89%. On the multiple sleep latency test, the REM sleep latency increased from 5.0 to 13.3 minutes, and the number of sleep-onset REM periods decreased from 3.1 to 0.6. Sleep (S1) latency was not changed. No intolerable adverse events occurred. The effective dose range was 20 to 40 mg, requiring a low-tyramine diet, which was easy to maintain. In conclusion, selegiline alleviates both main symptoms of narcolepsy--the abnormal sleep tendency and cataplexy. Thus, treatment with selegiline makes it possible to avoid polypharmacy and to use a potent stimulant without known addiction risk.

Adolescent↗

PubMed1996

Pontine lesions in idiopathic narcolepsy.

Three patients had longstanding (37 to 50 years), highly disabling narcolepsy, poorly controlled by treatment. The clinical histories were typical, consisting of sleepiness, cataplexy, sleep paralysis, hypnagogic hallucinations, disturbed nocturnal sleep, and HLA-DR2 tissue typing. Polygraphic findings confirmed the diagnosis. Neurologic examination, spinal fluid, and evoked potentials were normal. On MRI scanning, all three patients showed overlapping bilateral and symmetric brainstem T2 hyperintensities circumscribed to the ventrolateral aspect of the midrostral pons. The nature of the lesions remains uncertain but their location corresponded to the pontine oral reticular formation, where the neuronal network generating REM sleep is located. This is the first report of MR signal abnormalities in patients with idiopathic narcolepsy and suggests a causal relationship between the disease and the central pontine lesions.

Aged↗

PubMed1998

Muscarinic cholinergic receptors in human narcolepsy: a PET study.

OBJECTIVES: To investigate the function of the muscarinic cholinergic receptor (mAchR) in narcolepsy and the effects of pharmacotherapy on mAchRs. BACKGROUND: Muscarinic neural transmission serves as the main executive system in REM sleep. Studies in canine narcolepsy reported an increase in mAchRs in the pons. METHODS: The mAchRs of 11 drug naive/free patients with narcolepsy and 21 normal controls were investigated using PET with [11C]N-methyl-4-piperidylbenzilate ([11C]NMPB). Measurements were done in the pons, thalamus, striatum, and cerebral cortex. Seven of the 11 patients also underwent additional PET scans after the alleviation of symptoms by pharmacotherapy. RESULTS: There were no differences in [11C]NMPB binding between the control and drug naive/free patients in all areas analyzed. At the time of on-medication PET scan, [11C]NMPB binding in the thalamus was decreased, but only to a small degree compared with that by anticholinergic drugs. CONCLUSION: The present results do not support the notion that the mAchR is the main site of action of pharmacotherapy in the marked clinical improvement of human cataplexy.

Adult↗

PubMed2006

Hypersomnias of central origin.

Hypersomnia related to CNS disorders can be due to a variety of conditions. In this review, we discuss the diagnosis and treatment of narcolepsy with and without cataplexy, idiopathic hypersomnia, recurrent hypersomnia, and related illnesses. Research has provided insight into the underlying etiologies of these disorders, such as the genetic influences on disease development and the fundamental role of hypocretins in narcolepsy. We define the current utility of diagnostic testing, including sleep studies, neuroimaging techniques, and laboratory investigations. New treatment options for hypersomnia are discussed.

Central Nervous System↗

PubMed2003

Pharmacological management of narcolepsy.

Narcolepsy is a disabling disorder characterised by excessive daytime sleepiness, cataplexy, hypnagogic hallucinations, sleep paralysis and disturbed nocturnal sleep. Traditionally, a wide variety of substances are used in the symptomatic pharmacological treatment of narcolepsy. Several generally more-tolerated substances have been added to the therapeutic repertoire after extensive testing in large patient populations during recent years. This review addresses the state-of-the-art knowledge about the pharmacological treatment of narcolepsy along with the personal view of the authors. The recent discovery that narcolepsy is caused by deficient hypocretin (orexin) transmission opens a perspective on causal therapy.

Antidepressive Agents, Tricyclic↗

PubMed2003

Pharmacogenomics in the treatment of narcolepsy.

Narcolepsy is a neurological disorder characterized by excessive daytime sleepiness and cataplexy. Available treatments of narcolepsy include stimulants and antidepressants but the recent discovery of orexin/hypocretin deficiency in narcolepsy opens up new perspectives. Narcolepsy is a complex disorder involving genetic, immune and environmental factors. Although only a strong association is found with the HLA DQB1*0602 gene, other genetic susceptibility factors might be involved. Among these, the functional polymorphism of the catechol-O-methyltransferase (COMT) gene is critically involved in the severity of narcolepsy and in the response to the stimulant modafinil. Other pharmacogenetic targets include the orexinergic, noradrenergic and possibly the serotonergic pathways.

Carrier Proteins↗

PubMed2006

The psychosocial problems of children with narcolepsy and those with excessive daytime sleepiness of uncertain origin.

BACKGROUND: Narcolepsy is a predominantly rapid eye movement sleep disorder with onset usually in the second decade but often in earlier childhood. Classically it is characterized by combinations of excessive sleepiness especially sleep attacks, cataplexy, hypnagogic hallucinations, and sleep paralysis. The psychosocial effects of this lifelong condition are not well documented, especially in children. This study aims to describe the psychosocial profile of a large group of children with narcolepsy compared with other excessively sleepy children and controls. METHODS: We used an international cross-sectional questionnaire survey of children aged from 4 to 18 years who had received from a physician a diagnosis of narcolepsy compared with age- and gender-matched controls. Assessments were made of behavior, mood, quality of life, and educational aspects. RESULTS: Recruited children were separated into those who met conventional criteria for narcolepsy (n = 42) and those whose primary complaint was excessive daytime sleepiness without definite additional features of narcolepsy (excessive daytime sleepiness group; n = 18). Compared with controls, children with narcolepsy and also those with excessive daytime sleepiness alone showed significantly higher rates of behavioral problems and depression. Again, to a significant extent, their quality of life was poorer and they had more educational problems. The children with narcolepsy and the excessive daytime sleepiness group were indistinguishable from each other on these measures. CONCLUSIONS: A range of psychosocial problems can be identified in children with narcolepsy. The origins of these problems are unclear. The similar profiles of difficulties in the narcolepsy and excessive daytime sleepiness groups suggest that excessive sleepiness is the main cause. Clinicians and others responsible for the care of such children need to be mindful of the importance of early detection, intervention, and, ideally, the prevention of these problems.

Adolescent↗

PubMed1991

Narcolepsy in childhood and adolescence.

The objective of this study was to evaluate clinical, polysomnographic and the multiple sleep latency test (MSLT) features in young narcoleptics. We evaluated 14 patients with mean age of 13.6 years old (ranging from 6 to 18 years); 11 were males and 3 females. Daytime sleepiness was the main complaint in all cases and started at the ages of 6 to 17 years. Cataplexy was described in 10 cases and it was considered mild to moderate in all but one case. Sleep paralysis was present in 6 cases and hypnagogic hallucinations in 7 cases. The main polysomnographic characteristics were the short sleep latency in 9 cases and the sudden onset of REM periods in 7 cases. The MSLT showed short or borderline sleep latencies in 13 cases, with a mean of 4.9 min; 2 or more REM periods were present in 13 cases. Clinical, polysomnographic and MSLT characteristics in the age bracket focused were remarkably similar to those of adult narcoleptics suggesting the stability of these physiopathological markers.

Adolescent↗

PubMed1997

Narcolepsy and depression.

Narcolepsy main symptoms include excessive daytime sleepiness and cataplexy. Its chronic course is accompanied by psychosocial impairment added to the difficulties and side effects of stimulants and tricyclics long term use. Depressive complaints are occasionally reported. The aim of this paper was to evaluate objectively the possibility of depression in a sample of 12 narcoleptics (7F; 5M), with mean age of 53 years (12 years SD), using the Beck Depression Inventory (BDI) and the Hamilton Rating Scale for Depression (HAM-D). The results showed absence of depressive disorder in 75.0% of the cases according to BDI (or 58.3% according to HAM-D). The remaining patients had mild depression (only one patient presented major depression). The findings showed no correlation between narcolepsy and major depression.

Adult↗

PubMed2006

gamma-Hydroxybutyrate/sodium oxybate: neurobiology, and impact on sleep and wakefulness.

gamma-Hydroxybutyrate (GHB) is an endogenous short chain fatty acid and a, mostly oral, pharmacological compound that has been utilised in a variety of ways. Endogenously, GHB is synthesised locally within the CNS, mostly from its parent compound GABA. Sodium oxybate is the sodium salt of GHB and is used for the exogenous oral administration of GHB. It is likely that supraphysiological concentrations of GHB from exogenous administration produce qualitatively different neuronal actions than those produced by endogenous GHB concentrations. Evidence suggests a role for GHB as a neuromodulator/neurotransmitter. Under endogenous conditions and concentrations, and depending on the cell group affected, GHB may increase or decrease neuronal activity by inhibiting the release of neurotransmitters that are co-localised with GHB. After exogenous administration, most of the observed behavioural effects appear to be mediated via the activity of GHB at GABA(B) receptors, as long as the concentration is sufficient to elicit binding, which does not happen at endogenous concentrations. Endogenous and exogenous GHB is rapidly and completely converted into CO(2) and H(2)O through the tricarboxylic acid cycle (Krebs cycle). Sodium oxybate has been observed to modulate sleep in nonclinical study participants, and sleep and wakefulness in clinical populations, including groups with insomnia, fibromyalgia and narcolepsy. In narcolepsy, sodium oxybate has shown dose-related effects on various properties of sleep, including increases in slow-wave sleep duration and delta power, and a reduced number of night-time awakenings. Furthermore, multiple measures of daytime sleepiness and cataplexy demonstrated consistent short- and long-term improvement in response to night-time sodium oxybate therapy. The most common reported adverse events include dose-related headache, nausea, dizziness and somnolence.

Animals↗

PubMed1999

Multiple sclerosis presenting as catatonia.

OBJECTIVE: Catatonic disorder due to general medical condition must be excluded in psychiatric patients presenting with this movement disorder. This report emphasizes the association of catatonia with multiple sclerosis. METHOD: A patient with catatonia, psychotic depression, and the subsequent diagnosis of multiple sclerosis is described and the literature reviewed. RESULTS: Mutism, immobility, cataplexy, waxy flexibility, and other aspects of catatonia occur in multiple sclerosis, usually as a consequence of a severe mood disorder and extensive cerebral demyelination. These symptoms may be the presenting manifestations of multiple sclerosis. CONCLUSIONS: A high index of suspicion for neurological disease is indicated in patients with new-onset catatonia. Neuroimaging and other studies may reveal underlying demyelination requiring specific therapy.

Adult↗

PubMedDate not supplied

Narcolepsy with tinnitus aura: interpretation.

A fifty-five year old man was admitted to our ward for evaluation of narcoleptic-cataplectic attacks which were signaled by a "hissing sound like high pressure steam leaking out of a pipe." This tinnitus was exclusively localized to the right ear. It started about sixty-to-ninety seconds before the cataplectic episode (affecting mostly the jaw muscles) and lasted through part of it as it became progressively louder toward the end and ceased abruptly. It is postulated that, along with the collapse of tonic musculature of the mandible, a decrease of proprioceptive tone also affected the tensor tympani muscle leaving the middle ear vulnerable to internally generated noise. We further postulate that tinnitus was finally arrested by the generation of phasic bursts of contraction of the tensor tympani and/or the stapedius muscle at a later stage of cataplexy.

Electroencephalography↗

PubMed1988

Neurochemical perspectives of the narcoleptic syndrome.

Narcolepsy has been defined as a disorder of excessive sleep often associated with cataplexy, sleep paralysis and hypnagogic hallucinations. Although the pathophysiology of the narcoleptic syndrome is not well understood, derangement in the functions of CNS catecholamines and serotonin (5-HT) have been implicated. In the present paper we summarize evidence to suggest a role for the endogenous opioids in the regulation of normal sleep and in the pathophysiology of the narcoleptic syndrome.

Humans↗

PubMed1999

Narcolepsy and other causes of excessive daytime sleepiness.

Narcolepsy is a chronic disorder characterized by excessive daytime sleepiness, cataplexy, and other auxiliary symptoms. An interview can ascertain specific symptomatology, whereas a polysomnogram can reveal distinct clinical features. The clinical and laboratory evaluation together enable an accurate diagnosis of narcolepsy. This diagnosis includes a wide spectrum of symptom combinations. Treatment of narcolepsy should include the empathic guidance of a sleep clinician, an emphasis on sleep hygiene, and in many cases pharmacotherapy.

Circadian Rhythm↗

PubMed1999

Anesthesia considerations for patients with narcolepsy.

Narcolepsy is a derangement of the normal sleep-wakefulness rhythms. Originally, narcolepsy was thought to be a form of epilepsy; however, with the development and subsequent refinement of the electroencephalograph, this notion is no longer accepted. The disorder is characterized by inappropriate intrusions of rapid eye movement sleep into the wakeful state and multiple disruptions of the sleep cycles. Narcolepsy usually has its onset anytime between the ages of 10 years and 50 years, with the greatest majority of patients first reporting noticeable symptoms between the ages of 15 and 35 years. Patients with narcolepsy may exhibit excessive daytime sleepiness, cataplexy, hypnogogic and/or hypnopompic hallucinations, and sleep paralysis. The cause of narcolepsy is presently unknown. Recent research has identified a possible genetic contribution via chromosome 6, but some form of environmental influence appears to be necessary for the disorder to be manifested. There is no cure for narcolepsy; however, symptomatic relief may be achieved through a trial-and-error combination of amphetamines and tricyclic antidepressants. The nature of narcolepsy often forces the individual to undergo some rather dramatic lifestyle changes and can lead to the development of other associate disorders, such as depression and obesity.

Anesthesia↗

PubMed1999

Modafinil: a second look. Confirmed efficacy in narcolepsy.

(1) Modafinil, a psychostimulant, has been available in France for the treatment of narcolepsy and idiopathic hypersomnia since 1994. (2) The initial clinical file was inadequate to judge its possible clinical value. (3) In narcolepsy three new short-term clinical trials have compared modafinil with a placebo (nearly 500 patients treated with modafinil). (4) These trials show that the effects of modafinil on daytime drowsiness are only moderate (average reduction of one unwanted sleep episode a day) but are subjectively better than those of a placebo. In all, 84% of patients preferred the modafinil period to the placebo period during a cross-over trial. In contrast, modafinil does not improve cataplexy. (5) No new trials have been done in idiopathic hypersomnia. (6) The effects of modafinil persist for at least four months according to a double-blind placebo-controlled trial. (7) The safety profile of modafinil appears to be similar to that of non amphetamine psychostimulants. (8) There is currently no evidence of dependence leading to withdrawal symptoms after abrupt treatment cessation, or of a risk of abuse.

Benzhydryl Compounds↗

PubMed2000

Synkinetic blepharoclonus.

OBJECTIVES: To analyze the clinical data and test results collected in a group of patients exhibiting eyelid-closure blepharoclonus (BLC) on clinical neurologic examination. MATERIALS AND METHODS: Thirty-five patients were referred for neurologic evaluation for reasons other than BLC. Clinical electrophysiologic evaluations, including cranial nerve testing and electromyograms, were done according to standards. All patients had neuroimaging studies, including brain magnetic resonance imaging and head computerized tomography, or both, and many had electroencephalograms. Additional tests were done based on the patient's symptoms or reasons for referral. RESULTS: Eight patients had reflex BLC. Two cases were precipitated by vertical gaze; one of these patients had hereditary palmoplantar keratoderma and cataplexy, and the other patient had Ehlers-Danlos syndrome and familial BLC. Other precipitants included speech in four cases, postural changes in two cases, and light stimulation in one case. Two patients had generalized myoclonus independent of their BLC, two patients had a history of sleep myoclonus, and several patients had BLC-associated facial myoclonus. One patient had BLC-associated myoclonus of the right shoulder. Synkinetic cranial movements were detected in 11 patients (four oculofacial, three oculopterygoid, one oculolingual, two dual cases, and one case of imitation synkinesis.) Three patients had familial BLC, seven patients had congenital developmental disorders, six patients had synkinetic tremors, and six patients had restless feet. Some indication of peripheral neuropathy was evident in eight patients. CONCLUSIONS: Eyelid-closure BLC is an underrecognized, sporadic or familial, mostly benign, chronic eyelid-movement disorder that may be associated with tremors, myoclonus, cranial synkinesis, and restless feet. Reflex mechanisms may be identified in some patients. Gaze-induced BLC seems to have the greatest clinical relevance. In the current series, there were no examples of posttraumatic BLC, multiple sclerosis, hydrocephalus, or blepharospasm conditions previously reported to be associated with BLC. No electroencephalographic abnormalities were recorded during BLC, ruling out eyelid-closure epilepsy.

Adolescent↗

PubMed1998

Increased dopaminergic transmission mediates the wake-promoting effects of CNS stimulants.

Amphetamine-like stimulants are commonly used to treat sleepiness in narcolepsy. These compounds have little effect on rapid eye movement (REM) sleep-related symptoms such as cataplexy, and antidepressants (monoamine uptake inhibitors) are usually required to treat these symptoms. Although amphetamine-like stimulants and antidepressants enhance monoaminergic transmission, these compounds are non-selective for each monoamine, and the exact mechanisms mediating how these compounds induce wakefulness and modulate REM sleep are not known. In order to evaluate the relative importance of dopaminergic and noradrenergic transmission in the mediation of these effects, five dopamine (DA) uptake inhibitors (mazindol, GBR-12909, bupropion, nomifensine and amineptine), two norepinephrine (NE) uptake inhibitors (nisoxetine and desipramine), d-amphetamine, and modafinil, a non-amphetamine stimulant, were tested in control and narcoleptic canines. All stimulants and dopaminergic uptake inhibitors were found to dose-dependently increase wakefulness in control and narcoleptic animals. The in vivo potencies of DA uptake inhibitors and modafinil on wake significantly correlated with their in vitro affinities to the DA and not the NE transporter. DA uptake inhibitors also moderately reduced REM sleep, but this effect was most likely secondary to slow wave sleep (SWS) suppression, since selective DA uptake inhibitors reduced both REM sleep and SWS proportionally. In contrast, selective NE uptake inhibitors had little effect on wakefulness, but potently reduced REM sleep. These results suggest that presynaptic activation of DA transmission is critical for the pharmacological control of wakefulness, while that of the NE system is critical for REM sleep regulation. Our results also suggest that presynaptic activation of DA transmission is a key pharmacological property mediating the wake-promoting effects of currently available CNS stimulants.

Animals↗