DESMIN MYOPATHY INVOLVING CARDIAC, SKELETAL, AND VASCULAR SMOOTH-MUSCLE - REPORT OF A CASE WITH IMMUNOELECTRON MICROSCOPY

Desmin myopathy is a rare idiopathic disorder characterized by abnorma l aggregates of desmin-type intermediate filaments, which affects card iac and skeletal muscle, and rarely the intestinal smooth muscle. We r eport a 42-year-old woman with atrial fibrillation and progressive res trictive cardiomyopathy, Left ventricular biopsy cardiac explant, and subsequent autopsy study of skeletal muscle revealed cytoplasmic granu lo-filamentous inclusions that were continuous with Z-lines and were i mmunoreactive for desmin filaments both at the light immunohistochemic al and electron microscopic level. In addition, we report the presence of characteristic inclusions within the smooth muscle of intramural c oronary blood vessels. This is the first description of desmin inclusi ons within vascular smooth muscle, and underscores the systemic nature of this rare myopathy. Copyright (C) 1998 by W.B. Saunders Company.