Heart disease in Friedreich's ataxia

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Abstract
Severity of the myocardiopathy associated with Friedreich''s ataxia seems to parallel the neurologic deficit. In no case reviewed did onset of cardiac symptoms precede that of neurologic symptoms and in only one did the onsets appear to be simultaneous. Subjective cardiac symptoms reflect poorly both the onset and the presence of myocardiopathy. Physical examination most often is unrevealing except when arrhythmia is present. Blood pressure characteristically is not elevated. The condition is a progressive one. Cardiac enlargement is found in less than one-fourth (22.6%) of cases and, when present, occurs late in the course of the illness. Ecg remains the one reliable means of diagnosis. Similarities in patterns of ecg changes, observed in sisters in this case series and noted by others, reflects a familial tendency. The most plausible theory of etiology is that both neurologic and cardiac abnormalities are manifestations of a pleiotrophic genetically determined biochemical disorder. Similar cardiac disorders, occurring in other neurologic heredodegenerative diseases, may perhaps occur on the same basis.

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