Respiratory dysfunction in muscular dystrophy and other myopathies.

Respiratory muscle weakness occurs in a wide spectrum of acute and chronic myopathic disorders, and may cause a restrictive ventilatory defect and life-threatening ventilatory failure. Therapeutic intervention is based on a thorough knowledge of the course of each disease and meticulous monitoring of respiratory function. Comprehensive management of patients with myopathic disorders requires an understanding of the pathophysiology and specific manifestations of respiratory involvement in each disease.