Abstract
A case of 11 year-old-boy, who has been diagnosed as Ullrich type of congenital muscular dystrophy, is presented. At 8 years of age, the diagnosis was established by the clinical picture characterized by “Akroatonien”, electromyogram, serum enzyme studies and the clinical course. After the follow-up period of three years and 6 months, he suffered from pneumonia, following which he died of severe continual insufficiency of the heart at the age of 11 years and 1 month.
The autopsy was carried out and the microscopic examination revealed the distinct dystrophic changes in the skeletal muscles and slightly degenerative lesions by anoxia in the central nervous system.
