RESPIRATORY DYSFUNCTION IN MUSCULAR-DYSTROPHY AND OTHER MYOPATHIES

Respiratory muscle weakness occurs in a wide spectrum of acute and chr onic myopathic disorders, and may cause a restrictive ventilatory defe ct and life-threatening ventilatory failure. Therapeutic intervention is based on a thorough knowledge of the course of each disease and met iculous monitoring of respiratory function. Comprehensive management o f patients with myopathic disorders requires an understanding of the p athophysiology and specific manifestations of respiratory involvement in each disease.