Control of breathing in patients with neuromuscular diseases

We reviewed the studies devoted to investigate the control of breathing in patients with neuromuscular diseases. Neural respiratory drive has been assessed in terms of minute ventilation and mouth occlusion pressure (P0.1) in patients with several types of neuromuscular affections (amyotrophic lateral sclerosis, poliomyelitis and post-polio syndrome, Guillain Barré syndrome, muscular dystrophies, inflammatory muscle diseases). More recently we have used electromyographic activity of diaphragm to evaluate neural drive in patients with myasthenia gravis and muscular dystrophies. The results of these studies seem to indicate that patients with myasthenia gravis are not likely to have a decreased respiratory drive, the disorder in neuromuscular transmission being probably the reason for the apparent decrease in diaphragmatic neural activation during hypercapnic stimulation. Increase in neural afferent information from lung and/or rib cage might explain both the increased neural respiratory drive and the faster breathing observed in patients with neuromuscular disease.