Progressive weakness of the respiratory
muscles is one of the major problems in
the management of patients with muscular
dystrophy and can be at least partially prevented
by training of respiratory muscles.3,4 The decreasing
pulmonary and chest wall compliance
observed in neuromuscular diseases increases the
mechanical load sustained at each breath by
the impaired respiratory muscles. This increased
load leads to fatigue and eventual respiratory
failure, which is the leading cause of death in
these patients.5–10 Primary pulmonary insufficiency
and failure are associated with severe
general disability marking the ultimate stage of
the disease.