Objective measures of the efficacy of noninvasive positive-pressure ventilation in amyotrophic lateral sclerosis

The impact of noninvasive positive-pressure ventilation (NIPPV) on pulmonar y function studies, quality of life, and survival was assessed in patients with amyotrophic lateral sclerosis. NIPPV did not change the rate of declin e of the forced vital capacity (FVC) and forced expiratory Volume in the fi rst second (FEV1) (2.31 and 2.09 percent-predicted points per month, respec tively), NIPPV resulted in a drop of FEV, by 5.94 percent-predicted points (P = 0.07), and of maximal inspiratory pressure by 6.33 percent-predicted p oints (P = 0.11). The change in FEV, and FVC pre- and postintervention corr elated with the corresponding change in maximal inspiratory pressure. Fatig ue and mastery scores were improved by NIPPV. Median survivals in patients intolerant and tolerant of NIPPV were 5 and 20 months, respectively (P = 0. 002). Although NIPPV has no impact on the rate of decline of lung function and may have deleterious effects on spirometric measures, it may improve qu ality of life and survival. (C) 2001 John Wiley & Sons, Inc.