Abstract
Prognosis and cause of death were studied in 80 patients with myotonic dystrophy in Japan. The subjects consisted of 48 male and 32 female patients. Ten of them were congenital form and the rest were adult form. Average age at death was 12. 6 years in the congenital form, 55.1 years in the adult form and 49.6 years in all cases. In an, analysis of cause of death, more than half of the patients died of pulmonary infection or respiratory failure. Most of the cases in this group were in the terminal stage of the disease, and the deterioration was preceded by aspiration of food. Ten percent of the cases died by suffocation with food or sputum, and the other 10 percent died of cardiac failure or arrhythmia. Thirteen percent died of coincidental disorders such as malignant tumors, cerebral hemorrhage or hepatic failure. Sudden death were observed in 10 percent of the cases. It was noteworthy that this group included the cases who were relatively young and were not much disabled. Proper care to avoid aspiration of food was considered to be very important in the terminal stage of this disease. A manual of periodical examination should be framed to prevent sudden death.
