Effect of inspiratory resistance and theophylline on respiratory muscle strength in patients with amyotrophic lateral sclerosis.

The effect of breathing through inspiratory flow resistive loads ranging between 4.5 and 27.0 cm H2O/L/s was assessed in eight patients with amyotrophic lateral sclerosis (ALS) and in eight control subjects. ALS patients developed respiratory muscle fatigue manifested by significant declines in negative inspiratory pressure (18.3%), vital capacity (7.2%), and peak inspiratory flow rate (5.5%). Control subjects did not fatigue with these resistances. In ALS patients, theophylline increased respiratory muscle strength after resistive breathing as manifested by an increase in negative inspiratory pressure (28.2%), vital capacity (10%), and peak inspiratory flow rate (11.8%). It is concluded that in patients with ALS, the already weakened respiratory muscles are easily fatigued. Furthermore, theophylline can strengthen loaded respiratory muscles in patients with ALS.