Heart Disease in Friedreich's Ataxia : Observation of a Case for Half a Century

概要

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A case of Friedreich's ataxia was followed for 47 years, beginning in 1930; this patient had an abnormal electrocardiogram (flat or inverted T waves in leads IIandIII with prolonged QT interval) from the very beginning of the onset of neurological symptoms. Cardiac and neurological disturbances progressed slowly but steadily, and the patient died suddenly at the age of 67. The autopsy revealed typical findings of Friedreich's ataxia and hypertrophic cardiomyopathy with thickened left ventricular wall and myocardial fiber didsarray. To the authors' knowledge, this is the longest continuous follow-up study of Friedreich's ataxia, and it will provide invaluable information on the natural history and development of the cardiac and neurological disorders in this condition.
社団法人日本循環器学会の論文
2000-02-20