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PubMed1996

Pediatric brain stem gliomas with the predominant symptom of sleep apnea.

Two children complaining of sleep apnea presented with brain stem gliomas. In the early stage of their illness, neurological disorders were too subtle to be recognized as significant by the physicians or to be noted by the parents. Case 1 experienced an episode of unsteady gait and weakness in the bilateral arms, at the age of 5. When it recurred after 7 years of remission, the predominant symptom was sleep apnea. Case 2 exhibited nasality of speech as the earliest sign of this illness very early in his life, presumably 5 years before the diagnosis of brain stem glioma. A slight sleep apnea which developed afterwards did not draw attention of the physicians because no neurological signs other than paralyses of the bilateral soft palates were present. MRIs of the both cases revealed diffuse, infiltrating lesions in the pons, the medulla oblongata and the upper cervical spinal cord. Both cases shared some features: (1) diagnostic delay of several years from the first symptom; (2) the main lesion in the medulla oblongata, where important structures for respiratory control are identified; (3) infiltrative growth patterns in the MRI of the tumor, which might account for the uncommon clinical courses.

Blood Gas Analysis↗
PubMed1996

Sleep-related breathing disorders in patients with multiple system atrophy and vocal fold palsy.

We performed sleep studies in eight patients with multiple system atrophy (MSA) and three patients with peripheral bilateral vocal fold palsy (PBVFP) and investigated stenosis of the upper airway tract during sleep in MSA patients with vocal fold palsy. Among the eight MSA patients in this study, five had definite glottic snoring and two others were suspected of having glottic snoring. Of the PBVFP patients, two had glottic snoring. Three of 11 patients died, and two of the three deaths occurred during sleep. Glottic snoring indicated a high degree of negative esophageal pressure. High negative esophageal pressure demonstrates severe narrowing of the upper airway tract. Therefore, glottic snoring should be considered a risk factor for sudden death in sleep. Repeated laryngoscopic examination is useful in evaluating the progressive process of vocal fold palsy while awake, but this examination performed only while awake is not enough to evaluate narrowing of the upper airway during sleep. Sleep studies that include the measurement of esophageal pressure can be very useful in evaluating the severity of narrowing in the upper airway tract. It is suspected that sudden nocturnal death in MSA patients is caused not only by abnormal respiration resulting from impairment of the respiratory center, but also by glottic obstruction caused by sputum or by edema of the vocal folds. We recommend treatment of respiratory disorders when loud laryngeal snoring occurs in patients with MSA, even if they do not complain of dyspnea while awake.

Adult↗
PubMedDate not supplied

[Cognition, emotion, and behavior. Neuropsychosomatisms and non-neurological paroxysms].

INTRODUCTION: We present neuropsychosomatic disorders diagnosed and treated during the period 1994-1997. PATIENTS AND METHODS: A total of 83 cases, 24 boys and 59 girls, were selected according to suspected diagnosis. Their ages were in relationship with the psychosomatic disorder. This 83 cases is 10% of neuropediatric assistance in the period. A protocol was designed for disclosing any organic pathology. The psychopedagogic method is based on the PASS theory of intelligence and emotion processing theory of masquerade behavior. The success was defined after a period of, at least, two years of follow-up. RESULTS: Cephalalgia was the most frequent diagnosis. Language and learning difficulties, attention deficit disorder and pseudo-epilepsy were also frequent. Other diagnoses were: amblyopia, paralysis, pseudo-autism, tic, sphincter disorder, vertigo, mutism and sleep disorder. DISCUSSION: Concerning differential diagnosis, it must be emphasized that complex partial epilepsy of frontal lobe can mimic psychosomatics disorder as short, less than one minute, automatism. Partial epilepsy of temporal lobe may also mimic psychosomatic disorder but epilepsy does not respond to psychopedagogic remediation. Tumor and migraine must be also disclosed in case of cephalalgia but they do not respond to psychopedagogic remediation. Neurological scientific bases of emotion processing theory are widely explained. CONCLUSION: Cognition and emotion are functions of the central nervous system so they are competency of neuropediatrician. On the other hand, it is convenient for neuropediatrician to know about behavioral analysis in order to improve diagnosis and economical cost.

Adolescent↗
PubMed1987

[Effects of normobaric oxygen on rat hepatocytes].

Exposure of adult rats to oxygen (100%) during 55 hrs decreases their sleeping time induced by pentobarbital and doesn't modify their paralysis time provoked by zoxazolamine. At 48 and 55 hrs, the hepatic cytochrome P-450 decreases significantly. Morphological study showed tissular changes suggestive of hepatic hemodynamic perturbations, which are probably responsible for the functional modifications.

Animals↗
PubMed1995

Anosognosia in parietal lobe syndrome.

Patients with right parietal lesions often deny their paralysis (anosognosia), but do they have "tacit" knowledge of their paralysis? I devised three novel tests to explore this. First, the patients were given a choice between a bimanual task (e.g., tying shoe laces) vs a unimanual one (e.g., threading a bolt). They chose the former on 17 of 18 trials and, surprisingly, showed no frustration or learning despite repeated failed attempts. I conclude that they have no tacit knowledge of paralysis (or, if such knowledge exists, it is not available for this particular task). Second, I used a "virtual reality box" to convey the optical illusion to the patient that she was moving her paralyzed left hand up and down to the rhythm of a metronome, and yet she showed no sign of surprise. Third, I irrigated patient BM's left ear canal with cold water, a procedure that is known to shift that patient's spatial frame of reference by stimulating the vestibular system. Surprisingly, this allowed her "repressed" memory of the paralysis to come to the surface; she said she had been paralyzed continuously for several days. I suggest that the vestibular stimulation produces these remarkable effects by mimicking REM sleep. These patients also employ a whole arsenal of grossly exaggerated Freudian "defense mechanisms" to account for their paralysis. To explain this, I propose that in normal individuals the left hemisphere ordinarily deals with small, local anomalies by trying to impose consistency but, when the anomaly exceeds threshold, an interaction with the right hemisphere forces a "paradigm shift." A failure of this process, in patients with right hemisphere damage, might partially account for anosognosia. Finally, I present a new conceptual framework that may help link several psychological and neurological phenomena such as Freudian defense mechanisms, vestibular stimulation, anosognosia, memory repression, visual illusions, anterograde amnesia, REM sleep, dreaming, and humor.

Adaptation, Psychological↗
PubMed1996

Sleep and neuromuscular disorders.

Polysomnographic evaluation in the sleep laboratory is recommended for patients with neuromuscular disorders who develop symptoms and sign of sleep-wake abnormality or nocturnal respiratory failure. Nocturnal sleep-related ventilatory alterations may occur in disproportion to the severity of the neuromuscular disorder. Diaphragmatic paralysis occurring in the context of a neuromuscular disorder is often an overlooked complication. Failure to thrive, daytime tiredness, and incapacitating fatigue may be the result of a potentially correctable sleep-related abnormality and not due to relentless progression of the neuromuscular condition. Application of CPAP and BiPAP and administration of supplemental oxygen are relatively simple, noninvasive, ambulatory, therapeutic maneuvers that may correct sleep-related ventilatory alterations in patients with neuromuscular disorders.

Amyotrophic Lateral Sclerosis↗
PubMed2005

Sleep and neuromuscular disorders.

Nocturnal sleep-related ventilatory alterations may occur in dis-proportion to the severity of the neuromuscular disorder. Diaphragm paralysis occurring with a neuromuscular disorder is an overlooked complication. Failure to thrive, daytime tiredness, and incapacitating fatigue may be the result of a correctable sleep-related abnormality, not the result of relentless progression of a neuromuscular condition. Polysomnographic evaluation is recommended for patients who have neuromuscular disorder who develop symptoms and signs of sleep-wake abnormality or nocturnal respiratory failure. Application of noninvasive positive airway ventilation and, in some cases, administration of supple-mental oxygen may improve quality of life and prolong survival of patients who have neuromuscular disorder.

Amyotrophic Lateral Sclerosis↗
PubMed1973

Electrophysiological investigations of cephalic tetanus.

Electrophysiological studies of a patient with cephalic tetanus, manifested by trismus and unilateral facial palsy, revealed that the trismus resulted from strictly unilateral masseter spasm which stopped during sleep, a normal silent period in the involved masseter muscles, initially prolonged distal latency of the paretic orbicularis muscles, and abnormal continuous muscle activity in the paretic facial muscles which stopped during sleep and after facial nerve block. We conclulde that the facial paralysis was probably secondary to peripheral nerve dysfunction and the continuous muscle potentials represented enhanced activity of the nucleus. An intensity spectrum of the inhibitory defect in tetanus is postulated.

Action Potentials↗
PubMed1980

[Structure of nocturnal sleep in akinesia of many years duration and artificial pulmonary ventilation].

The polygram of night sleep was studied in 4 patients, who due to a lesion to the CNS and peripheral nervous system were in a state of long-term akinesia and artificial lung ventilation. The cyclic organization of sleep and its transition from stage to stage, as a whole corresponded with those in norm. However, there was a deficit of delta-sleep, differently expressed in each patient with a general tendency in all patients to a reduction of sleep spindles. The relationship between delta-sleep spindles and motor activity is discussed.

Adult↗
PubMed1992

Effects of body movement restraint on cardiac response to auditory stimulation in sleeping infants.

When exposed to an unfamiliar and sudden noise, infant rodents may show an abrupt bradycardia, as part of a "fear-paralysis response". The response is enhanced by body movements restraint. To investigate if this reaction is seen in humans, 15 normal infants with a median age of 12 weeks were studied polygraphically. They were exposed to a 100-dB (A) white noise, while sleeping in REM sleep. Each infant was studied in both "unrestrained" and "restrained" conditions. Restraint of body movement was obtained by means of sand bags and tightly-binding bed sheets. During movement restraint, the infants had a significantly greater and earlier decrease in heart rate compared with during the unrestrained condition. No infant had a minimal heart rate less than 95 beats/min. The present observation raises questions about the possible unfavorable effects of tightly wrapped bed sheets around sleeping infants.

Acoustic Stimulation↗
PubMed1992

Restructuring of sleep and reversal of REM-induced supraspinal hypotonia of respiratory muscles following bilateral phrenicotomy.

The long-term effect of diaphragm paralysis on respiratory system function is still not clear. We monitored changes in breathing pattern and the sleep/wake cycle in a dog before and after bilateral phrenicotomy. The post-operative observation extended over 6 months. It was noted that minute ventilation increased during wakefulness and non-REM sleep in the initial 4-6 weeks (compared to pre-surgery period), but decreased during REM sleep, mainly due to inhibition of chest wall and abdominal muscles. These episodes resulted in hypoxemia and frequent arousals. Following this period, there was a restructuring of REM sleep, increasing the frequency of REM sleep and reducing the duration of each REM sleep episode. In addition, the enhanced activity of parasternal and abdominal muscles was persistently seen during REM sleep. These changes in breathing and sleep provided stable ventilation during sleep. We conclude that bilateral phrenicotomy restructures breathing and alters sleep/wake cycle to prevent nocturnal hypoxemia. The mechanisms underlying these changes may reflect plasticity in the control of breathing and REM sleep.

Animals↗
PubMed2000

Long-term effect of bilateral plication of the diaphragm.

STUDY OBJECTIVES: To assess the feasibility and clinical outcome of bilateral plication of the diaphragm in patients with bilateral diaphragmatic paralysis (BDP) caused by neuralgic amyotrophy (NA), a mononeuritis of the phrenic nerves. DESIGN: Prospective, case-control study over a 1-year period. SETTING: A university hospital in The Netherlands. PATIENTS: Six patients who presented with BDP caused by NA. METHODS: The diagnosis of BDP was based on the absence of muscle response after cervical magnetic stimulation of both phrenic nerves. Three patients did not undergo surgery but were observed for a period of 2 years, and the other three patients underwent a limited lateral thoracotomy at the eighth intercostal space. Plication was performed by U-stitches until the diaphragm was as tight as possible. Vital capacity (VC) and arterial blood gas was measured during follow-up. RESULTS: One month postoperatively, mean VC measured in the supine position was significantly improved by 17%, and this effect was sustained for 12 months. Arterial PO(2) increased by 45%. VC and blood gas levels did not improve in the three patients that were only observed during the 2-year period. All three surgical patients could sleep in the supine position after the operation. CONCLUSION: Bilateral plication of the diaphragm for NA-induced paralysis results in improvement of ventilation and blood gas exchange, allowing patients to sleep in the supine position without dyspnea.

Aged↗