The cardiomyopathy of Friedreich's ataxia morphological observations in 3 cases

Can J Neurol Sci. 1980 Nov;7(4):389-96. doi: 10.1017/s0317167100022927.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Calcium / metabolism
  • Cardiomegaly / complications
  • Cardiomegaly / pathology
  • Cardiomyopathies / complications*
  • Cytoplasmic Granules / metabolism
  • Friedreich Ataxia / complications
  • Friedreich Ataxia / pathology*
  • Histocytochemistry
  • Humans
  • Iron / metabolism
  • Lipofuscin / metabolism
  • Male
  • Myocardium / metabolism
  • Myocardium / pathology*


  • Lipofuscin
  • Iron
  • Calcium