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PubMed2005

Narcolepsy and disorders of excessive somnolence.

Recent studies provide valid criteria that help differentiate idiopathic narcolepsy from other disorders of excessive daytime somnolence [3]. Research to date suggests that idiopathic narcolepsy might properly be considered a disorder of excessive sleepiness with dysfunctional REM-sleep mechanisms, clinically evidenced as cataplexy and electrophysiologically recognized as SOREMPs. Given these criteria, a diagnosis can generally be made using a combination of history, PSG, and MSLT. Traditionally, the medical treatment of idiopathic narcolepsy has centered on a two-drug regimen (stimulants for sleepiness and TCAs for cataplexy and auxiliary symptoms). Some newer medications are proving efficacious for sleepiness with minimal adverse effects, whereas others may provide a single-drug regimen that simultaneously addresses sleepiness and cataplexy [18]. New research has allowed some experts to hypothesize that idiopathic narcolepsy may be the result of a genetic predisposition to autoimmune disease [176]. It is possible that aberrant genetic coding of elements in the hypocretin/orexin systems allows a sensitivity to inducible and possibly virally mediated changes, which leave cells in the lateral hypothalamus susceptible to autoimmune attack [96]. As such, genetic screening of high-risk individuals might eventually rationalize the prophylactic use of immunosuppressants in some instances. In the future, for atypical cases(poorly responsive to therapy), genetic, CSF, and brain imaging studies, and possibly even neuronal transplantation may prove beneficial in the assessment and treatment of idiopathic narcolepsy.

Cataplexy↗
PubMed2005

Risks of high-dose stimulants in the treatment of disorders of excessive somnolence: a case-control study.

STUDY OBJECTIVES: To ascertain complications associated with high-dose stimulant therapy in patients with narcolepsy or idiopathic hypersomnia. DESIGN: Case-control, retrospective chart review. SETTING: Sleep center in an academic hospital. PATIENTS: 116 patients with narcolepsy or idiopathic hypersomnia were individually matched by sex, diagnosis, age of onset, and duration of follow-up from both onset and diagnosis. Members of the high-dose group (n = 58) had received at least 1 stimulant at a dosage > or = 120% of the maximum recommended by the American Academy of Sleep Medicine Standards of Practice Committee. The standard-dose control group (n = 58) had received stimulants at a dosage < or = 100% of the American Academy of Sleep Medicine guidelines. INTERVENTIONS: N/A. MEASUREMENTS AND RESULTS: The prevalence of psychosis (odds ratio = 12.0 [1.6-92.0]), alcohol or polysubstance misuse (odds ratio = 4.3 [1.2-15.2]), and psychiatric hospitalization (odds ratio = 3.2 [1.1-10.0]) was significantly increased in the high-dose group. More high-dose patients also experienced tachyarrhythmias (odds ratio = 3.3 [0.92-12.1] and anorexia or weight loss (odds ratio = 11.0 [1.4-85.2]). The frequency of physician-diagnosed depression, drug-seeking and suicide-related behaviors, hypertension, and cardiovascular disease did not differ significantly between the groups. CONCLUSIONS: This study demonstrated a significantly higher occurrence of psychosis, substance misuse, and psychiatric hospitalizations in patients using high-dose stimulants compared to those using standard doses. Tachyarrhythmias and anorexia or weight loss were also more common in this group as compared with controls. Clinicians should be very cautious in prescribing dosages that exceed maximum guidelines.

Adult↗
PubMed1986

HLA-DR2 and Dw2 in narcolepsy and in other disorders of excessive somnolence without cataplexy.

Studies on HLA antigens were conducted in several patient populations with the following findings: (a) All 135 Japanese narcoleptic patients, eight of whom were considered to have "symptomatic" narcolepsy, were found to be HLA-DR2 and HLA-DQw1 positive. All 17 members of a subgroup of the original population were also found to be HLA-Dw2-positive. It was concluded that HLA-DR2 is a prerequisite for the development of narcolepsy and that the diagnosis of narcolepsy can be excluded if HLA-DR2 or HLA-Dw2 is negative. The distinction between idiopathic and symptomatic narcolepsy needs to be reconsidered. (b) Haplotype studies in three families with narcoleptic members enabled detection of children at high risk for narcolepsy. (c) Of the 54 patients with disorders of excessive daytime sleepiness other than narcolepsy, those with essential hypersomnia (EHS) had a higher frequency of HLA-DR2; the others had a lower frequency. The DR2-positive EHS group could include members with an incomplete form of narcolepsy; the DR2-negative EHS group had disorders essentially different from narcolepsy, although both positive and negative groups manifested hypnagogic hallucinations, sleep paralysis, and sleep onset REMs. Two further studies were conducted in subgroups of the original narcoleptic population studied. In a subgroup of 30 patients who underwent lymphocyte subset studies, no T-cell abnormalities were detected; it is unlikely that an autoimmune mechanism is involved in the development of narcolepsy. In a subgroup of 33 narcoleptic patients, Southern's blot analysis of DNA using a DQ beta probe revealed three specific restriction fragments. Further studies are necessary to locate the DNA locus that carries the susceptibility gene for narcolepsy.

Alleles↗
PubMed1981

Sleep and its disorders.

The advances in research on sleep an biological rhythms have recently been applied to the diagnosis and treatment of sleep disorders. A new clinical specialty has developed with the establishment of sleep disorder centers and a diagnostic classification of sleep and arousal disorders. This new nosological approach has evolved from an extensive base of new scientific information concerning descriptive polygraphic and analysis of clinical case series. Four major categories have been defined: (a) disorders of initiating and maintaining sleep (insomnias), (b) disorders of excessive somnolence, (c) disorders of the sleep-wake schedule, and (d) dysfunctions associated with sleep. Within this comprehensive classification certain major pathophysiological advances are described for the "insomnias." These include polysomnographic identification of altered sleep stage patterns in the major effective illnesses, insomnias related to hypnotic drugs and alcohol, sleep disturbances associated with sleep-induced respiratory impairment, and sleep-related periodic movements during sleep (nocturnal myoclonus). Excessive daytime somnolence is primarily associated with the hypersomnia sleep-apnea syndrome and with narcolepsy. The relationship between biological rhythms (chronobiology) and disorders of the human sleep-wake schedules is very actively investigated. The recognition that sleep length, internal organization, and timing within neurophysiological circadian time-keeping systems has lead to better diagnosis of these sleep-wake disorders and new chronotherapeutic regimens. Finally, increasing identification and description of "parasomnias," i.e. dysfunctions associated with sleep, has led sleep research into important new areas that are of general physiological interest. It is now clear that sleep disorders medicine has become a new scientific and clinical discipline in its own right.

Arousal↗
PubMed2003

REM behavior disorder and excessive daytime somnolence in Machado-Joseph disease (SCA-3).

We reported previously that behavior suggestive of rapid eye movement sleep behavior disorder (RBD) was markedly increased in a small population of SCA-3 patients. We, therefore, asked patients and nonpatient attendees at an SCA-3 annual clinic to complete a questionnaire soliciting RBD-like behavior. Our results support the previous observation that RBD-like behaviors are significantly increased in SCA-3.

Adult↗
PubMed1990

Disorder of excessive daytime somnolence: a case series of 1,000 patients.

Symptoms of excessive daytime somnolence range from mild to severe. In mild cases, there may be minimal interference with normal daytime function. The hypersomnia can be disabling. When severe the patient finds it difficult to remain awake at times when physically inactive. Excessive daytime somnolence is the chief complaint of the majority of our adult patients. In this paper, we present the findings for 1,000 consecutive patients (755 males and 245 females) who were seen at the Humana Hospital Audubon Sleep Disorders Center. Patients ranged in age from 15 to 83. All patients had a sleep history, medical history and physical, psychological evaluation, polysomnographic evaluation, and other laboratory tests as indicated. Obstructive sleep apnea syndrome was the most prevalent diagnosis for males (84.2%) and females (59.6%). It accounted for over three-fourths of all diagnoses. Hypersomnia secondary to a psychiatric disorder was the next most frequent diagnosis overall (6.1%). A psychiatric disorder was second for females and third for males. Narcolepsy was diagnosed for 5.8% of all patients. This was the second most prevalent diagnosis for males and third for females. Eighteen males (47.4% of all males with a diagnosis of narcolepsy) and 9 females (45.0%) had cataplexy. Nocturnal myoclonus was the primary diagnosis in 2.5% of all patients with excessive daytime somnolence. An additional 49 patients with sleep apnea syndrome and 18 patients with narcolepsy also had periodic leg movements during sleep. A diagnosis of obstructive sleep apnea and narcolepsy was made for 1.3% of patients. The narcolepsy component of this diagnosis was typically made only after the obstructive sleep apnea had been resolved (eg, nasal CPAP, tracheostomy).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1981

Disorders of excessive daytime somnolence: polygraphic and clinical data for 100 patients.

A consecutive series of 100 sleep apnea free patients with the complaint of excessive daytime somnolence (EDS) were evaluated; data from medical histories, physical examination, personality inventories, and polysomnography [nocturnal polysomnography (NPSG) and daytime multiple sleep latency testing (MSLT)] were tabulated. Significant differences were found between narcoleptic and non-narcoleptic patients in a number of parameters, including EDS severity, mean sleep latency on MSLT, sleep latency on NPSG, latency to REM sleep at night, number of REM sleep at night, number of REM sleep segments throughout the night, the total number of nocturnal myoclonic jerks (as well as the number occurring per hour of NREM and REM sleep), and the number of arousals and wake periods preceded by a myoclonic jerk. Significant differences in sleep latency during MSLT and NPSG testing were found between different EDS diagnostic groups of non-narcoleptic patients. The majority of patients in the MSLT group with long sleep latencies were in the diagnostic groups of EDS associated with psychophysiological and/or psychiatric problems or with drug abuse; patients with a diagnosis of idiopathic central nervous system hypersomnia or EDS associated with disturbed nocturnal sleep formed the majority of the MSLT group with short sleep latencies. The non-narcoleptic patients in a MSLT group with short sleep latencies had significantly shorter sleep latencies at night, more sleep cycles, higher sleep efficiency, and earlier REM sleep than patients with long sleep latencies.

Adolescent↗
PubMed2005

Odds, prevalence and predictors of sleep problems in school-age normal children.

The objectives of the study were to describe the prevalence, odds, and predictors of 36 paediatric sleep behaviours and describe their coexistence in a school-age normal population. The design was community-based questionnaire survey of sleep-wake patterns, sleep environment, and 36 sleep behaviours indicative of six sleep disorder-subscales using the Health-Behaviour Questionnaire. A caregivers' report of 3045 children aged 6-13 years in Belgium constituted the participants. Prevalence of each sleep behaviour was calculated. Log-linear modelling within and between the sleep disorder-subscales was used to screen for coexistence. The effect size of selected night-time parameters to the likelihood of sleep behaviours and disorder-subscale was expressed as odds ratios via logit regression analysis. Significant differences in sleep-wake patterns were found between weekday and weekend. Ranking by odds showed that: (1) sleep problems such as 'tired when waking up', 'repetitive limb movements', 'going to bed reluctantly', and 'sleep paralysis' and; (2) the disorder-subscale 'excessive somnolence' are common in children. Coexistences within and between disorder-subscales of sleep problems are evident in a school-age, normal population. These results suggest that disorders of excessive somnolence (DES) are highly prevalent in a non-clinical sample of school-age children. Furthermore, sleep-onset latency and a noisy, not well-darkened room are predictive towards the odds for exhibiting sleep problems and disorders. It is advocated that more information on the importance of good sleep-wake hygiene should reach parents and children.

Adolescent↗
PubMed1990

Classification of sleep disorders.

This review discusses recent developments in classifying the sleep disorders. Several classification systems are presented, and the Diagnostic Classification of the Sleep and Arousal Disorders (DCSAD), published by the Association of Sleep Disorders Centers in 1979, is used as the basis for reviewing their differences. The four main sections of the DCSAD are presented: the disorders of initiating and maintaining sleep, the disorders of excessive somnolence, the sleep-wake schedule disorders, and the dysfunctions associated with sleep, sleep stages, or partial arousals. Disorders discovered since the publication of the DCSAD are mentioned along with the new nomenclature of the International Classification of Sleep Disorders, due to be published in 1990. References are given to the original clinical descriptions and nomenclature.

Disorders of Excessive Somnolence↗
PubMed1987

Neuropsychological dysfunction in sleep apnea.

To evaluate the effect of intermittent hypoxemia on neuropsychological functioning, neuropsychological tests were administered to 14 sleep apnea patients, a control group of 10 patients with other disorders of excessive somnolence, and another control group of 14 healthy volunteers. The sleep disorder groups were matched on two measures of sleepiness. It was found that sleep apnea patients performed significantly worse than both controls on 7 of 14 neuropsychological measures and on a rating of global neuropsychological impairment. The overall level of performance reflected only moderate impairment. Within the sleep apnea group, hypoxemia severity was significantly correlated with deficits on measures of motor and perceptual-organizational ability.

Adult↗
PubMed2002

[Psychic disorders and somnolence].

Hallucinations (mainly visual), psychosis and excessive daytime sleepiness are potential side-effects of dopaminergic treatment. They may require a reduction or suppression of dopaminergic agonists, and the prescription of atypical neuroleptic agents or vigilance-enhancing drugs. The recent description of narcolepsy-like sleep onset in rapid eye movement sleep periods synchronous with hypnagogic hallucinations in patients with dopaminergic-induced psychosis or excessive daytime sleepiness, suggests that the mesodiencephalic lesions may predispose to the psychic effects of dopaminergic treatment. Disease-related mood disorders, sexual compulsions, gambling or levodopa addiction may also be amplified by the antiparkinsonian treatment. These complications illustrate the neuro-psychic aspect of Parkinson's disease: psychic troubles may result from a subtle balance between the direct effects of drugs, the pre-morbid pathological personality and the cortical and subcortical lesions.

Disorders of Excessive Somnolence↗
PubMedDate not supplied

Sleep-related myoclonus in rheumatic pain modulation disorder (fibrositis syndrome) and in excessive daytime somnolence.

We describe a new syndrome, Rheumatic Pain Modulation Disorder (RPMD) ("fibrositis syndrome") with sleep-related myoclonus (involuntary periodic leg movements). Measures of sleepiness, fatigue and pain, before and after sleep, and aspects of sleep of nine subjects (Ss) with RPMD and sleep-related myoclonus were compared to nine subjects with excessive daytime somnolence and sleep-related myoclonus. In eight of the RPMD with sleep-related myoclonus and three of those with daytime sleepiness, an alpha (7.5-11 Hz) EEG Non-Rapid Eye Movement sleep disorder was demonstrated. The RPMD with sleep-related myoclonus group contained a greater number of women, more pain, morning fatigue, and disturbances in sleep (more stage changes and alpha EEG sleep prior to leg myoclonus); but in comparison to the sleep-related myoclonus, daytime somnolent group, there were no differences in evening and morning sleepiness, number of limb movements, movement arousals, awakenings after sleep onset, sleep duration, and percent sleep stages.

Adult↗
PubMed1980

Sleep disturbances in a series of elderly patients: polysomnographic findings.

A group of 27 elderly patients with complaints of either chronic insomnia or excessive daytime sleepiness were studied in the Sleep Evaluation Center of Western Psychiatric Institute and Clinic during the period January 1977-June 1979. On the basis of anamnestic data from patients and bedroom partners, together with polysomnographic findings, sleep disturbances were classified according to the nosology of the Association of Sleep Disorders Centers. Of the 27 patients, 19 had disorders of initiating or maintaining sleep (DIMS), 7 had disorders of excessive somnolence (DOES), and 1 had parasomnia (episodic nocturnal wandering). Of the 19 DIMS patients, two-thirds had either a primary affective disorder (depression) or a persistent psychophysiologic disturbance. Of the 7 DOES patients, 6 had a primary sleep disorder such as a sleep apnea syndrome or narcolepsy-cataplexy. Additional electroencephalographic sleep data are presented on elderly patients with primary nonpsychotic depression. The latency of rapid eye movements (REM) in the depressed patients was shorter (p less than 0.05) than in patients with a persistent psychophysiologic disturbance. The percentage of REM sleep was significantly elevated (p less than 0.05) in the depressed group, and intermittent wakefulness was decreased (p less than 0.01). The causes of sleep disturbance in the elderly are both heterogeneous and complex. The need for accurate differential diagnosis and a multiaxial approach is stressed.

Aged↗
PubMed1984

Absence spells. Hyperventilation syndrome as a previously unrecognized cause.

Absence spells in adults have been recognized in association with disorders of excessive somnolence, transient ischemia of the temporal lobes, and seizure disorders. A 66-year-old man who presented with a history of absence spells for more than 20 years is described. After diagnosis of a hyperventilation syndrome without an associated seizure disorder, educational and behavioral therapy without the use of medication has produced a long, continuing remission of these spells. The hyperventilation syndrome continues to present in many ways, often without recognition by physicians for prolonged periods. The case presented exemplifies this problem and may be the first report of absence spells caused by hyperventilation.

Aged↗