The complexity of central breathing disturbances during sleep has become increasingly obvious. They present as central sleep apnoeas (CSAs) and hypopnoeas, periodic breathing with apnoeas, or irregular breathing in patients with cardiovascular, other internal or neurological disorders, and can emerge under positive airway pressure treatment or opioid use, or at high altitude. As yet, there is insufficient knowledge on the clinical features, pathophysiological background and consecutive algorithms for stepped-care treatment. Most recently, it has been discussed intensively if CSA in heart failure is a "marker" of disease severity or a "mediator" of disease progression, and if and which type of positive airway pressure therapy is indicated. In addition, disturbances of respiratory drive or the translation of central impulses may result in hypoventilation, associated with cerebral or neuromuscular diseases, or severe diseases of lung or thorax. These statements report the results of an European Respiratory Society Task Force addressing actual diagnostic and therapeutic standards. The statements are based on a systematic review of the literature and a systematic two-step decision process. Although the Task Force does not make recommendations, it describes its current practice of treatment of CSA in heart failure and hypoventilation.

Quality of life in DMD is not correlated with physical impairment nor the need for noninvasive positive-pressure ventilation. The surprisingly high quality of life experienced by these severely disabled patients should be taken into consideration when therapeutic decisions are made.

BACKGROUND: Duchenne muscular dystrophy (DMD) leads to progressive impairment of muscle function, respiratory failure and premature death. Longitudinal data on the course of physical disability and respiratory function are sparse. OBJECTIVES: To prospectively assess physical impairment and disability, respiratory function and survival in DMD patients over several years in order to describe the course of the disease with current care. METHODS: In 43 patients with DMD, aged 5-35 years, yearly assessments of physical disability by the Duchenne muscular dystrophy physical Impairment and Dependence on care (DID) score ranging from 9 (no disability) to 80 (complete dependence), and forced vital capacity (FVC) were obtained over a mean +/-SD time interval of 5.4 +/-2.1 years. RESULTS: DID scores were correlated with age according to a hyperbolic function (f=85.3*age/(10.05+age), R=0.62, P<0.0001). FVC declined exponentially with age (f=139.1*exp(-0.08*age)), R=0.52, P<0.0001. Mean +/-SD age at which patients lost their ambulation was 9.4 +/-2.4 years and they became dependent on an electro-wheelchair at 14.6 +/-4.0 years. The age at beginning of assisted ventilation was 19.8 +/-3.9 years, Three patients deceased during the observation period. The estimated probability of survival to age 30 years was 85%, median survival was 35 years. CONCLUSIONS: Our detailed observations of the progression of physical disability, dependence on care and respiratory impairment in DMD patients from childhood to adult life is valuable for predicting the clinical course with current medical care. Compared to historical data, survival has considerably improved. Objectives: To prospectively assess physical impairment and disability, respiratory function and survival in DMD patients over several years in order to describe the course of the disease with current care. Methods:In 43 patients with DMD, aged 5-35 years, yearly assessments of physical disability by the Duchenne muscular dystrophy physical Impairment and Dependence on care (DID) score ranging from 9 (no disability) to 80 (complete dependence), and forced vital capacity (FVC) were obtained over a mean ±SD time interval of 5.4 ±2.1 years.

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Patients with restrictive lung disease are typically dyspneic and have an increase in overall respiratory center drive, as a result of increased lung elasticity. When we subjected healthy volunteers to external elastic loads, their variability of breathing was lessened. Accordingly, we hypothesized that patients with restrictive lung disease display decreased variability of breathing and, also, that decreased variability of breathing is related to dyspnea. Breathing pattern was measured nonobtrusively over 1 hour in 10 patients with restrictive lung disease and in 7 healthy subjects. On a separate occasion, dyspnea was measured while all subjects copied different tidal volumes and frequencies. Compared with healthy subjects, the random fraction of breath variability was reduced in patients with restrictive lung disease: 27 times for expiratory time, 12 times for tidal volume, and 6 times for inspiratory time (p < 0.01 in each instance). Conversely, the nonrandom, correlated fraction for tidal volume was increased almost 3-fold in the patients (p < 0.01). Small variations from average resting tidal volume caused marked increases in dyspnea in patients, and the relationship was parabolic (r2 = 0.97; p < 0.001). In conclusion, patients with restrictive lung disease adopt a tightly constrained breathing pattern, probably as a strategy for avoiding dyspnea.

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We evaluated the efficacy of two different continuous positive airway pressure devices with automatic mask pressure adjustment (autoCPAP) in comparison with fixed CPAP in treating obstructive sleep apnea syndrome in 29 patients. The mean (+/- SE) apnea-hypopnea index was 46 +/- 4 per hour and the Epworth score was 14.2 +/- 0.7. Patients were treated over three consecutive 1-month periods with three regimens in random order: an autoCPAP device responding to apnea-hypopnea and snoring, another autoCPAP device responding to snoring and changes in flow contour, and fixed CPAP at the 90th pressure percentile titrated by autoCPAP over 2 weeks. Allowed pressure in the autoCPAP mode was 4 to 15 cm H2O. At the end of each treatment period, symptoms, quality of life, vigilance, and nocturnal breathing disturbances were evaluated. All three treatment modalities improved symptoms, quality-of-life domains, and apnea-hypopnea index significantly and to a similar degree. Mean (+/- SE) maintenance-of-wakefulness time increased by 4.5 +/- 1.8, 6.0 +/- 1.5, and 6.1 +/- 1.4 minutes with DeVilbiss AutoAdjust LT, AutoSet T, and fixed-pressure CPAP, respectively (p<0.001 vs. baseline, p=not significant for comparisons among the three modalities). We conclude that both autoCPAP devices were equally effective as fixed-pressure CPAP in improving major outcomes during short-term therapy of sleep apnea.

NCT01527773 at http://www.clinicalTrials.gov.