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Report of a Case

Roger A. Brumback, MD; Bernard J. Panner, MD; William J. Kingston, MD

Arch Neurol. 1986;43(2):189-192.

Abstract
• Cardiac abnormalities are a characteristic feature of the autosomal, recessively inherited, spinocerebellar degeneration known as Friedreich's ataxia. We report the pathologic changes in the heart of a 27-year-old woman with Friedreich's ataxia, including ventricular subendocardial fibroelastosis, occlusion of the coronary sinus ostium, individual myofiber loss, myofiber disarray, and markedly enlarged, hyperchromatic myofiber nuclei. A common pathogenetic mechanism may underlie both cardiac and neurological abnormalities.

Author Affiliations
From the Departments of Pathology and Laboratory Medicine (Drs Brumback and Panner) and Neurology (Dr Kingston), University of Rochester Medical Center, Rochester, NY.

Footnotes
Accepted for publication Jan 8, 1985.

Reprint requests to Box 626, University of Rochester Medical Center, Rochester, NY 14642 (Dr Brumback).