The Cardiomyopathy of Friedreich's Ataxia Morphological Observations in 3 Cases

Abstract

Deposits of Ca salts and Fe in myocardial cells in 1 case of Friedreich''s ataxia, were recently found. A detailed morphological study of 3 new cases of this cardiomyopathy is conducted. Ca deposits were not found in the muscle fibers, but lipofuscin granules and deposits of Fe were observed in the 3 cases. In addition to the usual findings of interstitial fibrosis, hypertrophy and degeneration of myocardial fibers, foci of segmental active muscle necrosis were constantly present. Friedreich''s ataxia may be a neurocardiac degenerative disease with a membrane defect which could be related to defective metabolism of vitamin E or other micronutrients.