Abstract
An autopsy case of 10 months old male infant with typical type II glycogenosis was reported. In addition to routine histopathological and histochemical studies on skeletal muscle biopsy and specimens obtained at autopsy, histochemical analyses of the enzyme activities, electromicroscopic studies on muscle biopsy, and cardiac muscle and liver obtained at autopsy were employed. In our case, His bundle electrogram was performed and histopathologically the shortening and some degenerations were found in the atrioventricular junction, especially in His bundle and central fibrous body.
