Diaphragm function and alveolar hypoventilation.

A syndrome of alveolar hypoventilation has been identified in a group of patients with bilateral diaphragm paralysis. Eight patients were studied in whom diaphragm weakness had been suggested by paradoxical (inward) movement of the abdominal wall on inspiration, of whom seven had evidence of a generalized neuromuscular disorder. Diaphragm function was assessed quantitatively by measurement of the change in transdiaphragmatic pressure during a maximum inspiration (deltaPDi). In five patients, deltaPDi was zero and in the others ranged from 2-6 cm H2O (normal greater than 25 cm H2O) indicating paralysis and severe weakness in the respective groups. Fluoroscopy of the diaphragm was found to give misleading results, and the resons for this are considered. Vital capacity ranged from 65-30 per cent of the predicted normal in the upright posture, typically falling by about a half in the supine posture. Alveolar hypoventilation was present in five patients when supine and in six when asleep, the deterioration in blood gases associated with sleep generally being much greater in these patients than in normal subjects. Respiratory rate was significantly greater than age-matched controls. The ventilatory response to CO2 was impaired. The PCO2 could be brought to normal levels by voluntary hyperventilation, and the unreliability of voluntary respiratory manoeuvres of this kind as indices of ventilatory reserve is emphasized. Alveolar hypoventilation was associated with disturbed sleep, morning headache and day-time fatigue. Symptomatic benefit was achieved by the use of a cuirass respirator at night.