Congenital muscular dystrophy of the Fukuyama type (FCMD) with severe myocardial fibrosis. A case report with postmortem angiography.

Autopsy findings of a 17-year-old Japanese male with the typical features of congenital muscular dystrophy of the Fukuyama type (FCMD) are presented. This case showed progressive muscular dystrophy and central nervous system malformation, accompanied by severe myocardial fibrosis. Large mitochondrial dense bodies were found in both the cardiac and skeletal muscle cells. Postmortem coronary angiography failed to reveal the presence of any obstructive lesions responsible for myocardial fibrosis. It was also demonstrated that abnormal vessels proliferating in an area of micropolygyria (MPG) in the cerebrum were branches of the cortical artery originating from the pia mater. The cerebral malformation was accompanied by basal skull deformities.