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Pathology of the Heart in Friedreich's Ataxia: Review of the Literature and Report of One Case

Published online by Cambridge University Press:  18 September 2015

G. Sanchez-Casis
Affiliation:
Centre Hospitaller Universitaire de Sherbrooke; and the Clinical Research Institute of Montreal
M. Cote
Affiliation:
Centre Hospitaller Universitaire de Sherbrooke; and the Clinical Research Institute of Montreal
A. Barbeau*
Affiliation:
Centre Hospitaller Universitaire de Sherbrooke; and the Clinical Research Institute of Montreal
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, Quebec, Canada H2W 1R7
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Summary:

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A single case of typical Friedreich's ataxia was analyzed for cardiac changes and compared to the findings from the literature. Macroscopically, there was a cardiomegaly with some degree of ventricular hypertrophy and probable mild dilatation of the auricles. The more important and constant histologic changes were myocardial fibrosis and degeneration of the cardiac muscle cells. Granular deposits of calcium salts and iron were found in the muscle cells. A cardiomyopathy hypertrophic in type and occasionally obstructive appears to be an integral part of Friedreich's ataxia.

Type
Quebec Cooperative Study of Friedreich's Ataxia
Copyright
Copyright © Canadian Neurological Sciences Federation 1976

References

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