CARDIOPATHY IN FRIEDREICH'S ATAXIA (SPINAL FORM OF HEREDITARY SCLEROSIS)

Abstract

IT IS REMARKABLE that up to 1948 there had been no publication by American investigators on any aspect of the cardiopathology of Friedreich's ataxia (spinal form of hereditary sclerosis). At this time a report by Ellwood appeared in which he described 2 patients having Friedreich's disease, one of whom died seemingly of coronary occlusion and the other in congestive failure. Recently, a second report has appeared (Hejtmancik and others) in which descriptions of 2 patients with Friedreich's disease are presented, both having striking electrocardiographic changes and apical diastolic murmurs. REVIEW OF LITERATURE In Friedreich's original report (1863 a ) of 6 patients, one had experienced palpitation, dyspnea and paroxysmal auricular tachycardia, while 2 others had complained of frequent palpitation. On physical examination 5 were reported as showing no cardiac abnormality, while 1 had cardiomegaly and an apical systolic murmur. Postmortem examination of this patient revealed hypertrophy of the left and right