THE HEART-DISEASE OF FRIEDREICH ATAXIA - A CLINICAL AND ELECTROCARDIOGRAPHIC STUDY OF 115 PATIENTS, WITH AN ANALYSIS OF SERIAL ELECTROCARDIOGRAPHIC CHANGES IN 30 CASES

  • 1 January 1983
    • journal article
    • research article
    • Vol. 52  (208) , 489-502
Abstract
Patients (15) with carefully defined Friedreich''s ataxia were assessed clinically and by ECG for evidence of heart disease. Cardiac symptoms, of which dyspnea and palpitations were the most frequent, occurred in < 30%. Abnormalities on clinical examination were present in a similar proportion; harsh systolic murmurs, ventricular hypertrophy and added heart sounds were the commonest of these. Cardiac failure and persistent arrhythmias were rare and occurred late in the evolution of the neurological disease. Two patients presented with heart disease before developing neurological symptoms. Cardiac signs and symptoms were uncommon in patients without ECG abnormalities. Of the cases, .apprx. 2/3 had definitely abnormal ECG recordings. The characteristic finding was of widespread T-wave inversion with ventricular hypertrophy. Serial ECG, recorded over periods of up to 32 yr, were available in 30 cases and showed that abormalities may develop in patients with Friedreich''s ataxia at any time up until 20 yr after the onset of neurological symptoms. In 4 patients initial ECG abnormalities had either improved or disappeared subsequently.

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