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

Published online by Cambridge University Press:  18 September 2015

J.B. Lamarche ,
M. Côté  and
B. Lemieux
J.B. Lamarche
Affiliation:
Centre Hospitalier Universitaire de Sherbrooke
M. Côté
Affiliation:
Centre Hospitalier Universitaire de Sherbrooke
B. Lemieux
Affiliation:
Centre Hospitalier Universitaire de Sherbrooke
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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 micronutrienls.

Type
Quebec Cooperative Study of Friedreich's Ataxia
Information
Canadian Journal of Neurological Sciences , Volume 7 , Issue 4 , November 1980 , pp. 389 - 396
Copyright
Copyright © Canadian Neurological Sciences Federation 1980

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