The goal of this study was to show whether a correlation exists between the
intensity of specific respiratory muscle training and the improvement of s
trength and endurance in inspiratory musculature in patients with neuromusc
ular disorders (NMD). Sixteen patients with NMD (13 with Duchenne muscular
dystrophy and 3 with spinal muscular atrophy) performed inspiratory muscle
training (IMT) at home with a special training apparatus for 9 months. Maxi
mal inspiratory mouth pressure (PIMAX) and 12s-maximum voluntary ventilatio
n (12s-MVV) test served as parameters for inspiratory muscle strength and e
ndurance, respectively. In patients whose inspiratory vital capacity (VCin)
declined by less than 10% during the year before training began (n = 10),
a significant positive correlation was found between the number of successf
ully completed strength and endurance exercises and the improvement of PIMA
X (P < 0.05) and 12s-MVV (P < 0.05). In patients whose VCin-decline exceede
d 10% (n = 6), indicating more progressive respiratory system involvement o
f the disease, no significant correlation between the improvement of PIMAX
and 12s-MVV and the intensity of training was found. In patients with NMD,
the effects of IMT-runs are dose-dependent, provided that the respiratory s
ystem involvement of the disease is only slowly progressive. (C) 2000 John
Wiley & Sons, Inc.