Oculopharyngeal dystrophy: Ultrastructure of muscles distant from the primary myopathy

Abstract

The pectoral and psoas muscles from a 72-year-old man afflicted with oculopharyngeal muscular dystrophy were processed at autopsy for electron microscopy. The ultrastructural analyses of the pectoral muscle showed myofibrils which exhibited Z line streaming and a general breakdown in the organization of the sarcomere. In addition, some of the myofibrils displayed sites of degeneration at the center of the A band. The changes in the psoas muscles which are distant from the primary myopathic loci of oculopharyngeal muscular dystrophy were more extensive than that seen in the pectoral muscle. Control tissues showed intact myofibrils and little postmortem alteration.