Biomedical subjects
C Guilleminault
Publications and source records attributed to C Guilleminault.
Cardiac abnormalities in myotonic dystrophy. Electrophysiologic and histopathologic studies.
Eight young adult male patients with myotonic dystrophy, mean age 26 years, underwent 24-hour Holter electrocardiographic monitoring and intracardiac electrophysiologic study. Right ventricular endomyocardial biopsies were performed at the end of the electrophysiologic study in five of them. The atrial to His[A-H] interval was 155 msec in one case and less than or equal to 55 msec in all patients. Twenty-four hour Holter electrocardiographic monitoring demonstrated more than 4 premature ventricular contractions per minute in two patients and marked cyclical sinus arrhythmia during sleep in two others. Electron microscopic analysis of the endomyocardial biopsy specimens disclosed no prominent sarcoplasmic reticulum abnormalities but prominent I bands compared to previously obtained controls. Myofibrillar degeneration was seen in all cases and was associated with abnormal mitochondria in two. Cardiac abnormalities can be detected very early in the evolution of myotonic dystrophy, even prior to the onset of cardiac symptoms. The reported abnormalities appear closely related to the pathologic process affecting other skeletal muscles.
REM sleep episodes during the Multple Sleep Latency Test in narcoleptic patients.
Forty narcoleptic patients were given the Multiple Sleep Latency Test, consisting of 20 min opportunities to sleep offered at 10.00, 12.00, 14.00, 16.00 and 18.00 o'clock. Eleven patients had 2 episodes of REM sleep, 5 had 3, 11 had 4, and 13 had 5 before they were awakened. Fourteen control subjects given similar opportunities to sleep (reported in a companion article (Richardson et al. 1978)) had no REM sleep episodes. For the 10.00-18.00 o'clock opportunities respectively, there were 32, 29, 30, 28 and 27 REM sleep episodes. We conclude that this procedure can provide physicians with data useful in the diagnosis of narcolepsy.
Cholinergic mechanisms and cataplexy in dogs.
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Hypnotic efficacy of temazepam: a long-term sleep laboratory evaluation.
1 Temazepam was evaluated in a strictly defined insomniac patient population under sleep laboratory conditions. Two protocols were used: a short-term (26-night) and a long-term (54-night) protocol evaluated the efficacy of the drug administered at night at 15 mg (short-term study) and 30 mg (long-term study), respectively. 2 Temazepam seemed to be both safe and effective at doses of 15 and 30 mg with up to 5 weeks of ingestion. 3 Suppression of slow wave sleep was observed at the high dose, but no suppression of REM sleep, found in studies with other benzodiazepines, was noted. 4 No evidence was found for development of tolerance or rebound effects.
Mixed and obstructive sleep apnea and near miss for sudden infant death syndrome: 2. Comparison of near miss and normal control infants by age.
Twenty-nine full-term near miss for sudden infant death syndrome (SIDS) and 30 normal control infants underwent 24-hour polygraphic monitoring. Several types of respiratory events during sleep (eg, central, mixed, and obstructive apnea, periodic breathing) were defined and tabulated. Analysis of these respiratory variables and comparison of groups of near miss and control infants indicated that between 3 weeks and 4 1/2 months of age only one variable was consistently different at a statistically significant level: the number of mixed and obstructive apnea greater than 3 seconds during total sleep time. This study also showed an increase in mixed and obstructive respiratory events during sleep at 6 weeks of age in control as well as in near miss infants.
250 years ago: tribute to a new discipline (1729--1979).
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Obstructive sleep apnea and near miss for SIDS: I. Report of an infant with sudden death.
An infant girl followed up from birth to death at the University Medical Center had ""congenital stridor'' and a ""near miss for SIDS'' event at 3 months of age. As part of an ongoing SIDS research project, she underwent 24-hour polygraphic monitoring at 21 weeks of age. Sudden infant death occurred within 30 hours after the polygraphic study. Polygraphic data obtained from this infant are compared with those from control infants and other infants with near miss for SIDS who were of similar ages. The number of mixed and obstructive respiratory events during sleep was abnormally high on the infant's recording. Histologic findings, involving particularly the midline structures of the brain stem, are discussed.
Neurologic abnormalities in near miss for sudden infant death syndrome infants.
As part of the Sudden Death Research Project at Stanford University School of Medicine, we have performed systematic neurologic examination of 41 near miss infants, 7 normal siblings of babies who died a sudden infant death, and 21 normal control infants. Many infants were examined longitudinally until 2 years of age. We found that near miss infants under 3 months of age had consistent abnormalities of muscle tone, particularly shoulder hypotonia. These abnormalities disappeared with maturation, but only 60% of older infants who had suffered near miss episodes of neurologically and developmentally normal. In addition, only one of the seven apparently normal siblings had an entirely normal neurologic examination when first seen. Thus, infants who present with near miss episodes may have an underlying central nervous system abnormality or may acquire some abnormality as a result of the episode.
Obstructive sleep apnea: electromyographic and fiberoptic studies.
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Why should we study the infant 'near miss for Sudden Infant Death'?
The use of 'near miss for Sudden Infant Death syndrome' infants as a model toward understanding the Sudden Infant Death syndrome has been questioned. Although there are numerous problems in delineating this patient population and defining events occurring during sleep, continuous polygraphic monitoring demonstrates potentially life-threatening events.
Respiratory and hemodynamic study during wakefulness and sleep in myotonic dystrophy.
Six young male patients with grade I (mild) myotonic dystrophy and a complaint of excessive daytime sleepiness were studied during wakefulness and sleep. Pulmonary function tests during wakefulness showed evidence of mild abnormality related to respiratory muscle weakness. During sleep, some patients developed a sleep apnea syndrome with high sleep Apnea Indices. There was no relation between hypoxic and hypercapnic ventilatory responses during wakefulness and sleep Apnea Indices. But hypoxemia and hypercapnia worsened considerably during REM sleep. Myotonic dystrophy patients with sleep apnea presented increased pulmonary and systemic arterial pressures during sleep. It was also during sleep that arrhythmias were observed.
Baclofen trial in six myotonic dystrophy patients.
Six young adult patients with grade I myotonic dystrophy and a complaint of daytime somnolence underwent 36-hour polygraphic monitoring, dynamometric and electromyographic studies before and under baclofen (60 mg/daily). Patients with the most severe daytime sleepiness had pathologic Sleep Apnea Indexes. After 6 weeks' ingestion of baclofen, no subjective or objective effect on patient symptomatology was found.
Tracheostomy and hemodynamic changes in sleep-inducing apnea.
Because pulmonary hypertension and systemic hypertension occur during sleep-induced obstructive apnea, six patients underwent overnight hemodynamic monitoring before and after tracheostomy. Variables studied included heart rate, pulmonary artery pressure, femoral artery pressure, and arterial oxygen tension (Po2). After tracheostomy, significant reductions were noted during sleep in mean pulmonary artery pressure from 45 +/- 6 mm Hg (mean +/- SEM) to 22 +/- 2 mm Hg (P less than 0.05) and in mean femoral artery pressure from 137 +/- 6 mm Hg to 97 +/- 3 mm Hg (P less than 0.005). There was also a significant increase for the group in arterial Po2 recorded during the apneic episodes from 38 +/- 3 mm Hg before tracheostomy to 71 +/- 2 mm Hg (P less than 0.001) after tracheostomy. We conclude that tracheostomy improves the hemodynamic abnormalities and hypoxemia that occur during sleep in patients with sleep-induced obstructive apnea.
State of the art. Sleep and control of breathing.
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Twenty-four-hour recording in REM-narcoleptics with special reference to nocturnal sleep disruption.
Twenty narcoleptic patients and ten age-matched normals were polygraphically monitored for 58 consecutive hours. All subjects were on regimented sleep (hours between 2230 and 0700). Group A (11 patients and 10 normals) had enforced wakefulness during the day whereas Group B (9 patients) were permitted to sleep (mean = 2 1/2 hr.). On day 2, all subjects were permitted to sleep for 15-min periods every 2 hr. In narcoloptics, sleep recordings demonstrated a reduction of sleep latency, an increase of stage 1, and a decrease in stages 3 and 4 compared to normals, but total REM time and percentage of REM sleep were similar. Groups A and B showed no difference in the incidence of nocturnal awakenings. REM cyclic periodicity was larger in narcoleptics who also demonstrated a REM-sleep fragmentation. This fragmentation became more pronounced as time passed, with several shifts from REM to wakefulness and stage 1. Narcoleptics present REM onset sleep period but also show an inability to remain in REM sleep.
Advances in the pathology of sleep.
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