We studied 53 patients with proximal myopathy to determine at what level of muscle weakness hypercapnic respiratory failure is likely, and which tests of pulmonary function or respiratory muscle strength would best suggest this development. Respiratory muscle strength was determined from maximal static efforts and in half the patients, both inspiratory and expiratory muscle strengths were less than 50% of normal. In the 37 patients without lung disease respiratory muscle weakness was accompanied by significant decreases in vital capacity, total lung capacity, and maximum voluntary ventilation; by significant increases in residual volume and arterial carbon dioxide tension (Paco2); and greater likelihood of dependence on ventilators, atelectasis, and pneumonia. Hypercapnia was particularly likely when respiratory muscle strength was less than 30% of normal in uncomplicated myopathy, and when vital capacity was less than 55% of the predicted value in any patient.Myopathies which affect proximal limb muscles may also affect respiratory muscles;'-' but the relationships between the extent to which respiratory muscles are affected, abnormality of pulmonary function, and the onset of respiratory failure have not been defined. We addressed these questions in a group of 53 patients with various proximal myopathies. MethodsThe patients studied were adults with myopathy from the neurology and medicine services of Harlem Hospital Center and Columbia Presbyterian Medical Center in New York and the University of Virginia Hospital in Charlottesville. They were referred for evaluation of pulmonary or respiratory muscle function or both. They represent about one-third of all patients with a diagnosis of myopathy at these hospitals during the period of the study.Myopathy was diagnosed by conventional criteria

To assess the consequences to the human diaphragm of alterations in body weight and muscularity, we measured the mass, thickness, area, and length of diaphragm muscle at necropsy. Of 33 subjects who were clinically well until sudden death, 27 had sedentary occupations and normal weight (group N), while 6 were nonobese laborers whose average weight was 40% greater than normal (group M). Among 37 patients dying of more prolonged illness, 23 were of normal weight (group W), while 14 weighed 71% of normal (group U). Subjects with obesity, chronic pulmonary disease, or edema were excluded. Disease per se did not significantly affect diaphragm dimensions. However, in group M diaphragm muscle mass, thickness, area, and length were 165, 129, 125, and 117% of normal (P less than 0.005), whereas in group U the corresponding values were 57, 73, 77, and 83% (P less than 0.001). Thus alterations in body weight and muscularity profoundly affect diaphragm muscle mass, causing a nearly threefold variation between muscular normal subjects and underweight patients.

No abstract

To characterize the in vivo force-length relation of the human diaphragm, we related pressures during static inspiratory efforts (Pmus and Pdi, respiratory muscle and transdiaphragmatic pressures, respectively) to diaphragm lengths measured on chest X rays from 22 normal subjects. At total lung capacity, the intersection of diaphragm and chest wall contours corresponds to the anatomic junction of diaphragm and chest wall. This point is located by skeletal landmarks to reveal the entire diaphragm contour on films taken at lower lung volumes. To validate the X-ray measurements, corresponding diameters were measured on 32 normal diaphragms at necropsy. After correction for height and diaphragm position, in vivo and necropsy length estimates along the coronal section agreed within 9%. The diaphragm length-lung volume relation is curvilinear, with length increasing primarily in the portion of the diaphragm apposed to the chest wall. As length increases, Pmus and Pdi rise sharply then plateau, generally conforming to force-length behavior of isolated muscle. However, absence of a Pdi peak at presumed diaphragm resting length suggests that Pdi is submaximal during voluntary inspiratory effort.

The dynamics of voluntary cough were studied in healthy supine subjects during four successive infusions of d-tubocurarine (dTc) (0.05 mg/kg) to assess the effects of progressive expiratory muscle weakness on cough performance. Curarization produced a progressive decline in maximal static expiratory muscle strength (PEmax) measured at the mouth and in pleural pressures (Ppl) generated during coughing. Expiratory flow rates during coughing did not decrease except during the initial cough from total lung capacity with the last dTc dose (18% below control). This was associated with a decrease in end-inspiratory volume prior to coughing and with a marked decrease in Ppl to 30% of control. Although the decrease in flow rates was minimal compared with Ppl, flow patterns suggest that dynamic airway compression was reduced during these coughs. We conclude that the principal effect of the expiratory muscle weakness in curarized subjects is to reduce the cough-induced dynamic compression and linear velocity of airflow though the major intrathoracic airways.