Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “CATAPLEXY”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 523 records · Page 29Linked to original sources
PubMed1994

Night sleep does not predict day sleep in narcolepsy.

It has been suggested that the increased day sleep in narcolepsy-cataplexy is secondary to the known fragmentation and reduction of night sleep, there being no differences in 24 h sleep totals from normals. Twenty-two untreated patients underwent 24 h sleep wake recordings by ambulatory monitoring. Correlations were assessed by stepwise multiple regression between day sleep and night sleep measures. Almost no significant or near significant correlations emerged. It is concluded that day sleep in narcolepsy is based upon a different mechanism, perhaps a diurnal "subvigilance syndrome" of impaired arousal mechanisms.

Adult↗
PubMed1984

Narcolepsy: cholinergic receptor changes in an animal model.

An inbred colony of narcoleptic doberman pinschers has been analyzed for muscarinic receptor levels in 19 discrete brain regions. In comparison to age-matched controls, receptors were generally elevated in the brainstem and reduced in forebrain areas. No changes in receptor binding affinity were detected. The increased receptor levels found in the brainstem suggest that cholinoceptive neurons in this region are hypersensitive and may be involved in the initiation of cataplexy and other aspects of the narcolepsy syndrome.

Animals↗
PubMed1990

Subjective and polysomnographic characteristics of patients diagnosed with narcolepsy.

In order to better characterize the subjective and polysomnographic findings in patients with narcolepsy, a follow-up questionnaire was mailed to all patients diagnosed with the disorder at the Henry Ford Hospital Sleep Disorders and Research Center. The questionnaire inquired regarding the present, previous, and change in status for the constellation of narcolepsy symptoms. Memory problems, problems of daytime function, and nocturnal sleep disturbance were included among the questions related to the symptomatic constellation. By definition, all patients were symptomatic of daytime sleepiness and were diagnosed with narcolepsy only if there were two or more rapid eye movement (REM) onsets documented on the polysomnographic evaluation. A high percentage of patients reported nocturnal sleep disturbance, which was one of the symptoms with the latest reported onset. Retrospective comparison of questionnaire responses to the clinical polysomnography revealed significantly more sleep maintenance difficulties in the group of patients reporting this symptom on the questionnaire. Patients with disturbed nocturnal sleep reported taking more naps during the day, although the Multiple Sleep Latency Test (MSLT) failed to show differences in sleep latency. Interestingly, this group of patients was found to have a significantly higher number of sleep onset REM episodes on the MSLT. Finally, the findings are discussed as they compare to studies that required the presence of cataplexy as part of their inclusion criteria.

Adult↗
PubMed1996

Use of modafinil in the treatment of narcolepsy: a long term follow-up study.

One hundred and forty patients (104 male and 36 female) aged 42.26 +/- 19.19 (range = 8 to 79.5 years) with narcolepsy-cataplexy were given modafinil (200 to 400 mg) at the Montpellier sleep disorders center from 1984 onwards. The follow-up focused on the reduction of excessive daytime somnolence (EDS), side effects and duration of treatment. In order to determine if any clinical aspect of narcolepsy could be involved in modafinil discontinuation, patients were divided into two groups according to continued or interrupted treatment. When modafinil effect on EDS was evaluated according to a scale varying from 0 (no effect) to 3 (excellent effect), 64.1% of the subjects, scored good or excellent. The mean duration of treatment was 22.05 months +/- 24.9, ranging from 1 to 114 months. Dependency signs were never observed.

Adolescent↗
PubMed2004

Comparison of driving simulator performance and neuropsychological testing in narcolepsy.

Daytime sleepiness and cataplexy can increase automobile accident rates in narcolepsy. Several countries have produced guidelines for issuing a driving license. The aim of the study was to compare driving simulator performance and neuropsychological test results in narcolepsy in order to evaluate their predictive value regarding driving ability. Thirteen patients with narcolepsy (age: 41.5+/-12.9 years) and 10 healthy control patients (age: 55.1+/-7.8 years) were investigated. By computer-assisted neuropsychological testing, vigilance, alertness and divided attention were assessed. In a driving simulator patients and controls had to drive on a highway for 60 min (mean speed of 100 km/h). Different weather and daytime conditions and obstacles were presented. Epworth Sleepiness Scale-Scores were significantly raised (narcolepsy patients: 16.7+/-5.1, controls: 6.6+/-3.6, P < or = 0.001). The accident rate of the control patients increased (3.2+/-1.8 versus 1.3+/-1.5, P < or = 0.01). Significant differences in concentration lapses (e.g. tracking errors and deviation from speed limit) could not be revealed (9.8+/-3.5 versus 7.1+/-3.2, pns). Follow-up investigation in five patients after an optimising therapy could demonstrate the decrease in accidents due to concentration lapses (P < or = 0.05). Neuropsychological testing (expressed as percentage compared to a standardised control population) revealed deficits in alertness (32.3+/-28.6). Mean percentage scores of divided attention (56.9+/-25.4) and vigilance (58.7+/-26.8) were in a normal range. There was, however, a high inter-individual difference. There was no correlation between driving performance and neuropsychological test results or ESS Score. Neuropsychological test results did not significantly change in the follow-up. The difficulties encountered by the narcolepsy patient in remaining alert may account for sleep-related motor vehicle accidents. Driving simulator investigations are closely related to real traffic situations than isolated neuropsychological tests. At the present time the driving simulator seems to be a useful instrument judging driving ability especially in cases with ambiguous neuropsychological results.

Adult↗
PubMed2004

Syncope.

Syncope is a syndrome consisting of a relatively short period of temporary and self-limited loss of consciousness caused by transient diminution of blood flow to the brain (most often the result of systemic hypotension). Syncope comprises part of a subset of clinical conditions in which loss of consciousness is transient. Other conditions in this group, which are not syncope and should be clearly distinguished from syncope, include, for example, seizure disorders, posttraumatic loss of consciousness, and cataplexy. Recent surveys indicate that syncope accounts for approximately 1% of emergency department visits in Europe, although older reports from the United States placed this number closer to 3%. The reported prevalence of syncope in the population varies: 15% of children before the age of 18 years; 25% of a military population aged 17 to 26 years; 16% and 19%, respectively, in men and women aged 40 to 59 years; and up to 23% in a nursing home population older than 70 years. The highest frequency of syncope occurs in patients with cardiovascular comorbidity and older patients in institutional care settings. The causes of syncope are numerous and, not infrequently, multiple factors may contribute. The diagnostic evaluation is benefited by availability of a detailed medical history and reports of eyewitnesses. In this context, the physician must consider the classification of the causes of syncope, and address the most likely causes first. The principal groups of causes may be summarized as: (1) neurally mediated reflex syncope (eg, vasovagal faint, carotid sinus syndrome); (2) orthostatic (postural) syncope; (3) cardiac arrhythmias; (4) structural cardiac and pulmonary causes; and (5) cerebrovascular disorders (rare). In addition, conditions that may mimic syncope but are not true syncope (eg, psychogenic pseudosyncope) must be considered. Only after a definitive cause is established can appropriate treatment be initiated. In this regard, the syncope evaluation is facilitated by maintaining an organized diagnostic approach. The practitioner should avoid wasteful use of short-term ambulatory electrocardiographic recordings (eg, Holter monitors) and rarely positive neurologic tests (eg, electroencephelography, head magnetic resonance imaging/computed tomography) in the absence of head trauma or evident neurologic signs. In many medical centers the evaluation of patients with syncope is haphazard, and may be substantially enhanced by establishment of a multidisciplinary syncope evaluation unit or team.

Diagnosis, Differential↗
PubMed2005

How age influences the expression of narcolepsy.

OBJECTIVES: The aim of this study was to investigate the influence of age on the manifestation of narcolepsy symptoms and cognitive difficulties in patients with narcolepsy. METHODS: A total of 321 participants were included in the study: 157 were patients with narcolepsy from two Sleep Disorders Clinics and 164 were control participants. Narcoleptic patients were evaluated and diagnosed at the Sleep Disorders Clinic. All participants were interviewed by telephone using the Sleep-EVAL System. The interview comprised, among else, a detailed evaluation of narcolepsy symptoms and of cognitive difficulties. RESULTS: The first manifestation of the disease appeared early in life for most narcoleptic patients: 54.1% had their first symptom before the age of 20 years. Daytime sleepiness was the first symptom to appear in 65.5% of cases. In narcoleptics 60 years or older, cataplexy was more likely to be the first symptom to appear (47.4%) compared with other narcoleptic patients (21.4%; P<.05). Reported cognitive difficulties (attention-concentration, praxis, delay recall, orientation for persons, temporal orientation, and prospective memory) were higher in narcoleptic patients compared with the controls. The severity of daytime sleepiness and the presence of a major depressive disorder partly explained the cognitive difficulties. However, attention-concentration deficits and difficulties in prospective memory remained significant. Age was unrelated to cognitive difficulties in narcoleptics patients. CONCLUSIONS: The first manifestation of narcolepsy appears early in life. Reported cognitive difficulties are important in narcoleptic patients and are only partly explained by age, severity of daytime sleepiness, and major depressive disorder.

Adolescent↗
PubMed2006

Low-carb diets, fasting and euphoria: Is there a link between ketosis and gamma-hydroxybutyrate (GHB)?

Anecdotal evidence links the initial phase of fasting or a low-carbohydrate diet with feelings of well-being and mild euphoria. These feelings have often been attributed to ketosis, the production of ketone bodies which can replace glucose as an energy source for the brain. One of these ketone bodies, beta-hydroxybutyrate (BHB), is an isomer of the notorious drug of abuse, GHB (gamma-hydroxybutyrate). GHB is also of interest in relation to its potential as a treatment for alcohol and opiate dependence and narcolepsy-associated cataplexy. Here I hypothesize that, the mild euphoria often noted with fasting or low-carbohydrate diets may be due to shared actions of BHB and GHB on the brain. Specifically, I propose that BHB, like GHB, induces mild euphoria by being a weak partial agonist for GABA(B) receptors. I outline several approaches that would test the hypothesis, including receptor binding studies in cultured cells, perception studies in trained rodents, and psychometric testing and functional magnetic resonance imaging in humans. These and other studies investigating whether BHB and GHB share common effects on brain chemistry and mood are timely and warranted, especially when considering their structural similarities and the popularity of ketogenic diets and GHB as a drug of abuse.

Brain↗
PubMed2006

Survival rates through time of hypocretin grafted neurons within their projection site.

Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness, inadvertent transitions from wakefulness to rapid eye movement sleep (so called "sleep-onset REMS period") and cataplexy (sudden bilateral skeletal muscle weakness during waking without impairment of consciousness). This disorder has been recently linked to a loss of hypocretin (HCRT) neurons making narcolepsy a neurodegenerative disease. Neuronal replacement could be used to reverse the symptoms of narcolepsy. Towards this end, we have recently reported that HCRT neurons from rat pups can survive when grafted into the pons of adult rats. Here, we investigate the time-course of survival of grafted HCRT neurons into the pons of adult rats. The HCRT neurons are present only in the lateral hypothalamus, and therefore suspension of cells from this region was derived from 8- to 10-day-old rat pups (donor), and grafted into the pons of adult (60 days old) host rats. Control rats received a transplant that consisted of cells from the cerebellum where no HCRT neurons are present. All adult host rats were sacrificed 1, 3, 6, 9, 12, 24, or 36 days after grafting. Immunohistochemistry was used to identify and count the presence of the HCRT grafted neurons in the target area. The tally of HCRT neurons present in the graft zone 1 day post-grafting was considered to be the baseline. From day 3 to 36 post-transplant there was a steady decline in the number of HCRT neurons. We also noted that on day 36, the HCRT neurons that survived in the pons had morphological features that were similar to mature HCRT neurons in the adult lateral hypothalamus, suggesting that these neurons might be functionally active. Control rats that received grafts of cerebellar tissue did not show HCRT neurons in the target area. These results demonstrate that there is a progressive decline in the number of transplanted neurons, but a significant percentage of HCRT neurons do survive until day 36. This study highlights the potential use of transplants as a therapeutical tool in order to treat narcolepsy.

Animals↗
PubMed2005

Increased serotonin receptor availability in human sleep: evidence from an [18F]MPPF PET study in narcolepsy.

Data from animal studies suggest that serotonin release promotes wakefulness and suppresses REM sleep, but there are dangers in extrapolating these findings to humans. Binding of the radioligand [18F]MPPF to 5HT1A receptors is sensitive to levels of endogenous serotonin. In this study, we aimed to demonstrate changes in serotonin receptor availability in the human brain in wakefulness and sleep using [18F]MPPF and positron emission tomography. 14 subjects with narcolepsy cataplexy underwent [18F]MPPF PET scans in wakefulness and in sleep. Subjects who used the stimulant methylphenidate took their normal medication for the wake scan but omitted it prior to the sleep scan. The change in binding potential (BP) between the sleep and wake scans was examined using paired t test. Methylphenidate is thought to have little or no effect on serotonergic neurotransmission, and in order to confirm the absence of an effect on [18F]MPPF binding, a concurrent study was performed using a beta-microprobe technique to examine the effect of methylphenidate administration on [18F]MPPF binding in Sprague-Dawley rats. The human study showed a significant increase in [18F]MPPF binding in sleep compared to wakefulness in the whole brain and all regions of interest examined (temporal cortex, mesial temporal region and cingulate cortex). The beta-microprobe study confirmed that methylphenidate administration had no effect on [18F]MPPF binding. These findings indicate that serotonin receptor availability is increased in sleep compared to wakefulness in narcoleptic humans.

Adult↗
PubMed2005

Behavioral correlates of activity in identified hypocretin/orexin neurons.

Micropipette recording with juxtacellular Neurobiotin ejection, linked micropipette-microwire recording, and antidromic and orthodromic activation from the ventral tegmental area and locus coeruleus were used to identify hypocretin (Hcrt) cells in anesthetized rats and develop criteria for identification of these cells in unanesthetized, unrestrained animals. We found that Hcrt cells have broad action potentials with elongated later positive deflections that distinguish them from adjacent antidromically identified cells. They are relatively inactive in quiet waking but are transiently activated during sensory stimulation. Hcrt cells are silent in slow wave sleep and tonic periods of REM sleep, with occasional burst discharge in phasic REM. Hcrt cells discharge in active waking and have moderate and approximately equal levels of activity during grooming and eating and maximal activity during exploratory behavior. Our findings suggest that these cells are activated during emotional and sensorimotor conditions similar to those that trigger cataplexy in narcoleptic animals.

Action Potentials↗
PubMed2005

Dopaminergic-adrenergic interactions in the wake promoting mechanism of modafinil.

Adrenergic signaling regulates the timing of sleep states and sleep state-dependent changes in muscle tone. Recent studies indicate a possible role for noradrenergic transmission in the wake-promoting action of modafinil, a widely used agent for the treatment of excessive sleepiness. We now report that noradrenergic projections from the locus coeruleus to the forebrain are not necessary for the wake-promoting action of modafinil. The efficacy of modafinil was maintained after treatment of C57BL/6 mice with N-(2-chloroethyl)-N-ethyl 2-bromobenzylamine (DSP-4), which eliminates all noradrenaline transporter-bearing forebrain noradrenergic projections. However, the necessity for adrenergic receptors in the wake-promoting action of modafinil was demonstrated by the observation that the adrenergic antagonist terazosin suppressed the response to modafinil in DSP-4 treated mice. The wake-promoting efficacy of modafinil was also blunted by the dopamine autoreceptor agonist quinpirole. These findings implicate non-noradrenergic, dopamine-dependent adrenergic signaling in the wake-promoting mechanism of modafinil. The anatomical specificity of these dopaminergic-adrenergic interactions, which are present in forebrain areas that regulate sleep timing but not in brain stem areas that regulate sleep state-dependent changes in muscle tone, may explain why modafinil effectively treats excessive daytime sleepiness in narcolepsy but fails to prevent the loss of muscle tone that occurs in narcoleptic patients during cataplexy.

Adrenergic Agents↗
PubMed2006

Cyclic alternating pattern (CAP) alterations in narcolepsy.

BACKGROUND AND PURPOSE: Narcolepsy is a sleep disorder with clinical symptoms attributed to a reduced activation of the arousal system. Cyclic alternating pattern (CAP) is the expression of rhythmic arousability during non-rapid eye movement (NREM) sleep. CAP parameters, arousals and conventional sleep measures were studied in narcoleptic patients with cataplexy. PATIENTS AND METHODS: Data were collected from all-night polysomnographic (PSG) recordings and the multiple sleep latency test (MSLT) on the intervening day of 25 drug-naive patients (10 males and 15 females; mean age: 34+/-16 years) after adaptation and exclusion of other sleep disorders. A group of 25 age- and gender-matched normal sleepers were selected as controls. Each PSG recording was subdivided into sleep cycles. Analysis of CAP included classification of A phases into subtypes A1, A2, and A3. RESULTS: There was an increase in sleep period time mainly due to an increased wake time after sleep onset. REM latency was sharply reduced. The percentage of NREM sleep was slightly reduced and the balance between light sleep (S1+S2) and deep sleep (S3+S4) showed a curtailment of the former, while deep sleep was slightly increased. Excluding sleep cycles with sleep onset REM periods (SOREMPs), the duration of ordered sleep cycles was not different between narcoleptics and controls. The two groups showed similar values of arousal index, while CAP time, CAP rate, number of CAP cycles and of phase A subtypes (in particular subtypes A1) were significantly reduced in narcoleptic patients. CONCLUSIONS: The reduced periods of CAP in narcoleptic NREM sleep could be the electroencephalographic (EEG) expression of a generally reduced arousability or an increased strength of sleep-promoting forces in the balance between sleep and arousal systems. This can explain some of the clinical correlates of the disorder, i.e. excessive sleepiness, short sleep latency and impaired attentive performances, even without any sign of arousal-induced sleep fragmentation.

Adolescent↗
PubMed1997

FFT analysis of EEG during stage 2-to-REM transitions in narcoleptic patients and normal sleepers.

The EEG in REM sleep is markedly different from the EEG in non-REM sleep; however, very little research has been conducted analyzing the transition from NREM to REM sleep. The purpose of this study was to describe the changes in EEG power spectra that coincide with significant physiological events throughout the transition from stage 2 to REM sleep. Furthermore, a comparison of the stage 2 to REM transition between narcoleptic patients and normal sleepers was conducted. Five female and five male patients diagnosed with narcolepsy-cataplexy and 10 normal sleepers matched for age and gender participated. Analyses were based on the consecutive appearance of 3 common physiological indicators of REM sleep: First, a decrease in tonic submental EMG activity; second, the appearance of saw tooth waves; and third, the appearance of rapid eye movements. Systematic changes in EEG power density were evident for delta, theta, alpha and sigma frequencies, across the stage 2-REM transition. These changes appeared to be relatively continuous throughout this transition rather than changing dramatically following the onset of any one of the 3 primary physiological indicators of REM sleep. Furthermore, the transition from stage 2 to stage REM appeared to be similar for narcoleptic patients and normal sleepers.

Adult↗
PubMed1998

Sleep latency on the maintenance of wakefulness test (MWT) for 530 patients with narcolepsy while free of psychoactive drugs.

OBJECTIVES: To compare maintenance of wakefulness test (MWT) data gathered at baseline in the course of two, multicenter studies on the therapeutic efficacy of modafinil with published MWT norms. METHODS: The MWT is a procedure that uses electrophysiological measures to determine the ability to remain awake while sitting in a quiet, darkened room. The test consists of 4 20 min trials conducted 4 times at 2 h intervals commencing 2 h after awakening from a night of sleep. MWT data were gathered at baseline in the course of two, multicenter studies on the therapeutic efficacy of modafinil. Subjects were 17-68 year old men (n = 239) and women (n = 291) diagnosed with narcolepsy according to the International Classification of Sleep Disorders (ICSD). All patients were free of psychoactive medication for a minimum of 14 days. RESULTS: Mean MWT sleep latency was 6.0 +/- 4.8 min. However, the mean for the first MWT trial was 7.0 min which was longer that the means for the following 3 trials (5.8, 5.6 and 5.7 min, respectively). The 4 distributions of the individual MWT trials were similar and adequately summarized by the distribution of the average MWT sleep latency. As a group, patients with narcolepsy were less able to remain awake than normals; only 8 of 530 (1.5%) patients were able to remain awake on 4 20 min MWT trials compared with 35 of 64 (54.7%) normals in another study. However, using a mean MWT sleep latency of 12 min (the 5th percentile for normals) as the lowest cut-point for normalcy, 15% of patients with narcolepsy appeared to have an unimpaired ability to remain awake. CONCLUSIONS: The diagnosis of narcolepsy did not always predict inability to remain awake on the MWT. Age, gender and the duration of illness did not predict ability to remain awake. Patients with severe cataplexy and other ancillary symptoms were least able to remain awake on MWT trials. Patients who used tobacco and caffeine moderately had the lowest MWT sleep latencies relative to patients with heavy and light use.

Adolescent↗
PubMed2000

A validity study of Ullanlinna Narcolepsy Scale in Hong Kong Chinese.

OBJECTIVE: To validate the Chinese version of Ullanlinna Narcolepsy Scale (CUNS). METHODS: A total of 234 subjects [163 male (69.7%) and 71 female (30.3%)] including 17 patients with narcolepsy, 21 normal controls and 196 patients with various sleep and psychiatric disorders were studied. The diagnoses of these patients were independently ascertained with sleep laboratory confirmation whenever indicated. All the subjects were interviewed through the telephone by a trained lay interviewer who was blind to the diagnosis. The questionnaire included demographic information, sleep habits and CUNS. RESULTS: Narcoleptic patients had a significantly higher CUNS score (18.6+/-4.7; 95% confidence interval (CI) 16.2-21.0) and differentiated well from all the other groups (F(6,227)=28.4, P<0.001). The CUNS has a satisfactory internal consistency with Cronbach's alpha of 0.75. The principal component analysis with varimax rotation revealed two factors, namely sleepiness and cataplexy factors, which accounted for 45.5% of the total variance. The best cut-off point for the CUNS scale was found to be at 13/14 with high specificity (93.5%), sensitivity (94.1%), negative predictive value (NPV, 99.5%) and modest positive predictive value (PPV, 53.3%). The AUC of receiver operating characteristic (ROC) analysis was 0.97 (95% CI 0.95-0.99). CONCLUSIONS: The CUNS was validated with satisfactory psychometric properties. The cross-cultural validation of UNS scale suggested that it could be used across the ethnic groups.

Adolescent↗
PubMed2002

Prevalence of restless legs syndrome and periodic limb movement disorder in the general population.

BACKGROUND: Periodic limb movement disorder (PLMD) and restless legs syndrome (RLS) are two sleep disorders characterized by abnormal leg movements and are responsible for deterioration in sleep quality. However, the prevalence of these disorders is not well known in the general population. This study aims to document the prevalence of RLS and PLMD in the general population and to identify factors associated with these conditions. METHODS: Cross-sectional studies were performed in the UK, Germany, Italy, Portugal and Spain. Overall, 18,980 subjects aged 15 to 100 years old representative of the general population of these five European countries underwent telephone interviews with the Sleep-EVAL system. A section of the questionnaire assessed leg symptoms during sleep. The diagnoses of PLMD and RLS were based on the minimal criteria provided by the International Classification of Sleep Disorders. RESULTS: The prevalence of PLMD was 3.9% and RLS was 5.5%. RLS and PLMD were higher in women than in men. The prevalence of RLS significantly increased with age. In multivariate models, being a woman, the presence of musculoskeletal disease, heart disease, obstructive sleep apnea syndrome, cataplexy, doing physical activities close to bedtime and the presence of a mental disorder were significantly associated with both disorders. Factors specific to PLMD were: being a shift or night worker, snoring, daily coffee intake, use of hypnotics and stress. Factors solely associated with RLS were: advanced age, obesity, hypertension, loud snoring, drinking at least three alcoholic beverages per day, smoking more than 20 cigarettes per day and use of SSRI. CONCLUSIONS: PLMD and RLS are prevalent in the general population. Both conditions are associated with several physical and mental disorders and may negatively impact sleep. Greater recognition of these sleep disorders is needed.

Adolescent↗
PubMed2004

Neurologic disorders masquerading as pediatric sleep problems.

Neurologic disorders may present or masquerade as pediatric sleep problems and fool the pediatrician, which may delay diagnosis and treatment. Many of the sleep problems in children with neurologic disorders arise directly from primary dysfunction or delayed maturation of their sleep-wake regulation systems. It is important to realize that nocturnal frontal lobe seizures or cluster headaches can be mistaken for night terrors, and craniopharyngiomas or myotonic dystrophy may present as narcolepsy-cataplexy. Hypothalamic dysfunction may explain not only the impaired circadian rhythm disorders in children with profound mental retardation but also excessive sleepiness and hyperphagia in Prader-Willi and Kleine-Levin syndromes. Intellectually challenged children perform better, learn more, and are better behaved with sufficient restorative sleep.

Circadian Rhythm↗