CYTOPLASMIC BODY MYOPATHY WITH HYPERTROPHIC CARDIOMYOPATHY

A patient with cytoplasmic body myopathy presented muscle hypotonia fr om birth and developed progressive muscular atrophy and weakness, scol iosis, contracture of joints and cardiorespiratory failure. At the age of 17, he died of heart failure. Post mortem examination revealed sev ere hypertrophy of cardiac walls and generalized muscular atrophy. Mic roscopic examination showed many cytoplasmic bodies in skeletal muscle fibers and myofiber disarray in myocardium. No cases of cytoplasmic b ody myopathy with hypertrophic cardiomyopathy have been reported previ ously. It is suggested that the Z-line component is related to the for mation of the cytoplasmic body in skeletal muscle and disarray in the cardiac muscle.