The cardiomyopathy of Friedreich's ataxia morphological observations in 3 cases.
In the light of the recent finding of deposits of calcium salts and iron in myocardial cells in one case of Friedreich's ataxia, we have made a detailed morphological study of 3 new cases of this cardiomyopathy. Calcium deposits were not found in the muscle fibers but lipofuscin granules and deposits of iron were observed in our 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. There is a possibility that Friedreich's ataxia could be a neurocardiac degenerative disease with a membrane defect which could be related to defective metabolism of vitamin E or other micronutrients.
D000293:Adolescent; D000328:Adult; D002118:Calcium / Q000378:metabolism; D006332:Cardiomegaly / Q000150:complications / Q000473:pathology; D009202:Cardiomyopathies / Q000150:complications*; D003594:Cytoplasmic Granules / Q000378:metabolism; D005621:Friedreich Ataxia / Q000150:complications / Q000473:pathology*; D006651:Histocytochemistry; D006801:Humans; D007501:Iron / Q000378:metabolism; D008062:Lipofuscin / Q000378:metabolism; D008297:Male; D009206:Myocardium / Q000378:metabolism / Q000473:pathology*
D008062:Lipofuscin; D007501:Iron; D002118:Calcium