[Steinert's syndrome and the myocardium. Total gene expression by the myocardium]

Abstract

As revealed by a very thorough cardiological study of Steinert's disease in 13 cases, it would appear that the incidence of cardiac involvement observed in this disease is not merely frequent; it is, in fact, usual, systematic, and forms an integral part of the pathological picture in the same way as peripheral muscular dystrophy. One is aware of the diagnostic interest of this fact in the juvenile or abortive forms of the disease. The cardiac involvement is an indication of the complete expression of the gene towards striated muscle tissue, whether skeletal or myocardial. Finally, it opens new perspectives on the prophylactic attitude to be adopted for such patients; regular and systematic cardiological checks, moderation in the prescription of digitalis and anti-arythmia drugs, implantation of a cardiac pacemaker before an auriculo-ventricular syncopal block occurs.