1/14. Respiratory control and respiratory sensation in a patient with a ganglioglioma within the dorsocaudal brain stem.We encountered a young woman with severe central sleep apnea caused by a medullary glioma located slightly dorsal to and to the right of the midline, a region not generally associated with CO(2) chemosensitivity. The patient had normal spirometric readings, lung volumes, diffusing capacity, maximal inspiratory pressure, and alveolar-arterial oxygen difference. While awake, she displayed marked irregularity in her breathing pattern; her end-tidal CO(2) (FET(CO(2))) ranged from 5.3 to 10.9%. During voluntary hyperpnea, she could quickly reduce her FET(CO(2)) to 4.2%, but her PCO(2) did not change after administration of acetazolamide or progesterone. Like patients with congenital central hypoventilation syndrome (CCHS), our patient had a relatively intact ventilatory response to exercise; her PCO(2) was high at the start of exercise and increased slightly thereafter. In contrast to CCHS patients, however, our patient had an intact hypoxic ventilatory response (DeltaVE/ DeltaSa(O(2)) = -0.37 L/min/Sa(O(2))). In further contrast to CCHS patients, our patient had a very short breathholding time and described a sensation of air hunger as the factor limiting her breathholding ability. Her heart rate and blood pressure responses to the valsalva maneuver were normal.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/14. Primary pulmonary hypertension with central sleep apnea: sudden death after bilevel positive airway pressure therapy.An obese 23-year-old man with sleep-disordered breathing and primary pulmonary hypertension (PPH) had been administered oral beraprost sodium, anticoagulant warfarin, and home oxygen therapy, at another hospital as treatment for the PPH, but he had not experienced any symptomatic improvement. The patient had a body mass index of 32.4kg/m2, and complained of fatigue, shortness of breath on exertion, excessive daytime sleepiness, and snoring. Arterial blood gas analysis showed a PaO2 and a PaCO2 of 70.9 and 31.2mmHg, respectively. A polysomnographic study revealed central sleep apnea with an apnea-hypopnea index (AHI) of 29.7episodes/h. The patient showed improvement of daytime sleepiness after starting nocturnal nasal bilevel positive airway pressure (BiPAP) therapy for the central sleep apnea, but his pulmonary hypertension, measured in the daytime, worsened. The patient died suddenly while walking to the bathroom in the morning 1 month after initiation of BiPAP therapy. It is necessary to consider the possibility of sudden death when nasal BiPAP therapy is given to a PPH patient with central sleep apnea.- - - - - - - - - - ranking = 2.5keywords = pressure (Clic here for more details about this article) |
3/14. Peculiar respiratory response observed during sleep-onset REM sleep of an infant with Ondine's curse.We treated an infant with congenital central hypoventilation syndrome ("Ondine's curse"). She was cyanotic and given ventilatory support at the first hour after birth. An investigation of sleep state and respiration performed at the age of 3 months led to this diagnosis. hypoventilation persisted in all sleep stages, with the most severely reduced tidal volumes occurring during delta-wave sleep (stages 3 and 4). In addition, severe secondary reduction in tidal volumes occurred in sleep-onset REM sleep. This phenomenon was absent in non sleep-onset REM sleep. At 4 months of age, her respiratory treatment was successfully converted to positive-pressure ventilation via a nasal mask, thus avoiding tracheotomy. This transition to noninvasive ventilatory support dramatically improved her quality of life during wakefulness. This report may be a clue to discuss the function of sleep-onset REM sleep seen in the early stage of life and suggests that nasal mask ventilation is a viable option in selected cases with congenital central hypoventilation syndrome (CCHS).- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
4/14. Familial adult onset primary alveolar hypoventilation syndrome.A 41-year-old man fell into type II respiratory failure after catching a cold, and became dependent on a respirator. Chest radiography showed no abnormalities and the hyperventilation test showed improved arterial blood gas findings. His sleep study showed marked nocturnal desaturation due to hypopnea and apnea with a decrease of thoracic and abdominal movement during sleep. Therefore, we diagnosed him as primary alveolar hypoventilation syndrome (PAH). Seven years previously, his 2-year elder sister had suffered from similar respiratory failure during her second pregnancy and had been diagnosed as PAH. While myopathy was suspected in both cases, attenuation of muscle strength was slight and it appeared not to be the main cause of alveolar hypoventilation. Since medication was not effective in each case, they underwent non-invasive positive pressure ventilation (NIPPV). While sustained mild hypercapnia remained during the daytime, it improved their respiratory failure. To our knowledge, this is the first study of familial adult onset PAH.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
5/14. Congestive heart failure and continuous positive airway pressure therapy: support of a new modality for improving the prognosis and survival of patients with advanced congestive heart failure.Nasal continuous positive airway pressure therapy has recently been studied as a nonpharmacologic adjunct to congestive heart failure therapy. In patients with congestive heart failure, it was reported that continuous positive airway pressure therapy for the condition cheyne-stokes respiration with central sleep apnea led to long-term improvements in cardiac function and alleviation of heart failure symptoms. cheyne-stokes respiration with central sleep apnea is a frequent breathing disorder well described in patients with congestive heart failure, and is an associated risk factor for increased cardiovascular morbidity and mortality. These apneas cause an increase in sympathetic nervous system activity, which would maintain afterload at a high level or tend to increase it with time, leading to further compromise of ejection fraction. continuous positive airway pressure appears to benefit the failing heart by increasing intrathoracic pressure, which is believed to cause an increase in cardiac output by decreasing the pressure gradient across the heart wall and allowing the left-ventricular end diastolic volume to decrease, thereby reducing the afterload. This beneficial "resting" of the heart has been documented to increase left-ventricular ejection fraction, increase cardiac index, improve inspiratory muscle strength, lower blood pressure and heart rate, decrease plasma and overnight urinary levels of norepinephrine, lower levels of atrial natriuretic peptide and endothelin-1, and increase heart rate variability. Other benefits include improvement in new york Heart association functional class status and improvement in dyspnea.- - - - - - - - - - ranking = 5keywords = pressure (Clic here for more details about this article) |
6/14. Rigid spine syndrome with chronic respiratory failure.Rigid spine syndrome (RSS) is a rare myopathic ailment characterized by mild axial and proximal muscle weakness. muscle contraction in these patients causes limitation of neck and trunk flexion, scoliosis, and mild joint deformity. We report the case of a 30-year-old man with RSS who presented with severe restrictive ventilatory defect (forced vital capacity, 1.53 L, 39% of predicted), mild scoliosis (Cobb's angle 12), proximal muscle weakness and stiff back. creatine phosphokinase was 986 IU/L. Muscle biopsy of the right vastus lateralis revealed increased variation in muscle fiber diameter, proliferation of endomysium, and type I fiber atrophy. Round and whorled fibers indicating myopathic change were found on sections stained with nicotinamide adenine dinucleotide dehydrogenase and succinate dehydrogenase. RSS was diagnosed based on myopathic findings and clinical presentation. Nocturnal chronic respiratory failure and fragmented sleep developed. He was treated with continuous positive airway pressure and had recovered his normal daily activity by 1-year follow-up. We suggest that patients with RSS should be assessed for possible ventilatory failure; treatment with nasal continuous positive airway pressure or bilevel continuous positive airway pressure therapy should be considered.- - - - - - - - - - ranking = 1.5keywords = pressure (Clic here for more details about this article) |
7/14. Early use of Nasal-BiPAP in two infants with Congenital Central hypoventilation syndrome.AIM: To reduce the problems caused by prolonged artificial ventilation in babies with Congenital Central hypoventilation syndrome (CCHS). methods: Two term infants with CCHS, weighing 4030 g and 3100 g, respectively, at the beginning of treatment and aged 53 and 31 d, respectively, were successfully ventilated with a Nasal Bilevel Positive Airway Pressure (N-BiPAP) device. RESULTS: In the first patient the tcPO2 recordings (mean /- SD) during sleep were 46 /- 12 mmHg before using N-BiPAP and 58 /- 13 mmHg after using the device, while those for tcPCO2 were 75 /- 9 mmHg and 49 /- 11 mmHg, respectively. In the second patient tcPO2 during sleep was 42 /- 3 mmHg before, and 55 /- 5 after N-BiPAP, and for tcPCO2 the recordings were 119 /- 24 mmHg and 55 /- 6 mmHg, respectively, showing a significant improvement. One infant had persistent gastro-oesophageal reflux, and frontal skin abrasion caused by the face mask. Nevertheless, these complications did not necessitate the discontinuation of N-BiPAP ventilation, thus precluding prolonged use of intubation and tracheotomy. CONCLUSION: In infants with CCHS, early use of non-invasive, positive-pressure ventilation with N-BiPAP, in association with careful monitoring, can decrease problems caused by prolonged intubation and tracheotomy.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
8/14. Noninvasive interface for transtracheal mechanical ventilation for patients with severe cervical spine deformity.This article describes a custom-molded patient-to-ventilator interface that permits mechanical ventilation via a tracheostomy site for patients for whom tracheostomy tubes can not be used because of severe cervical deformity. The nocturnal delivery of intermittent positive-pressure ventilation resulted in normalization of oxyhemoglobin saturation and end-tidal CO2 and alleviation of symptoms of alveolar hypoventilation. Thermoplastic patient-ventilator tubing interfaces can be constructed to permit ventilatory assistance for patients with tracheostomies who can not use tracheostomy tubes.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
9/14. Noninvasive ventilatory strategies in the management of a newborn infant and three children with congenital central hypoventilation syndrome.Four children with congenital central hypoventilation syndrome (CCHS) treated with noninvasive techniques of ventilation are presented. Two infants (one in the newborn period) were treated with nasal mask bilevel positive airway pressure (BiPAP), and then both were transitioned to negative pressure chamber ventilation at several years of age because of possible midface hypoplasia. Tracheostomies were not performed. Two older children were transitioned from mechanical ventilation via tracheostomy to nasal mask BiPAP, and then in one case to negative pressure chamber ventilation, and in the other to phrenic nerve pacing. Their tracheostomies were removed.- - - - - - - - - - ranking = 1.5keywords = pressure (Clic here for more details about this article) |
10/14. anterior spinal artery syndrome complicated by the ondine curse.BACKGROUND: Anterior spinal artery (ASA) syndrome results in motor palsy and dissociated sensory loss below the level of the lesion, accompanied by bladder dysfunction. When the cervical spine is involved, breathing disorders may be observed. OBJECTIVE: To describe the polysomnographic findings in a patient with cervical ASA syndrome complicated by a sleep breathing disorder. SETTING: Unit of neurology at a sleep center.Patient A 30-year-old man had an ischemic lesion that affected the anterior cervical spinal cord (C2-C6) bilaterally because of an ASA thrombosis. He developed ASA syndrome associated with respiratory impairment during sleep. RESULTS: The polysomnographic study during sleep showed a severe sleep disruption caused by continuous central apneas that appeared immediately after falling asleep. Treatment by intermittent positive pressure ventilation normalized the respiratory pattern and sleep architecture. CONCLUSIONS: The sleep breathing pattern was compatible with central alveolar hypoventilation due to automatic breathing control failure caused by a lesion of the reticulospinal pathway, which normally activates ventilatory muscles during sleep. This autonomic sleep breathing impairment resembles that found as a complication in patients who undergo spinothalamic tract cervical cordotomy for intractable pain. This surgical complication is known as the Ondine curse.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
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