Desmin-storage disease and third degree atrioventricular block

Desmin storage myopathy is characterized by clinical myopathy, restrictive cardiomyopathy and atrioventricular conduction disturbances. Third degree atrioventricular block is usually preceded by clinical myopathy and/or restrictive cardiac myopathy. We report an unusual case of desmin storage disease presented with third degree atrioventricular block prior to development of skeletal myopathy and restrictive cardiomyopathy.