Abstract
Chuichi Kawai, MD; Shotaro Kato, MD à ; Masayuki Takashima, MD; Hisayoshi Fujiwara, MD*; Hideyuki Haebara, MD** A case of Friedreich's ataxia was followed for 47 years, beginning in 1930; this patient had an abnormal electro-cardiogram (flat or inverted T waves in leads II and III 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 disarray. 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.
(Jpn Circ J 2000; 64: 229 -236)

Key Words: Friedreich's ataxia; Hypertrophic cardiomyopathy; Myocardial fiber disarray; Natural history

Mailing address: Dr Chuichi Kawai, (Professor Emeritus, Kyoto University), Nishiiru Karasuma, Shimochojamachi-dori, Kamikyo-ku, Kyoto 602-8002, Japan