Heart disease in Friedreich's ataxia: observation of a case for half a century

Autor: Hideyuki Haebara, Hisayoshi Fujiwara, Shotaro Kato, Chuichi Kawai, Masayuki Takashima
Rok vydání: 2000
Předmět:
Zdroj: Japanese circulation journal. 64(3)
ISSN: 0047-1828
Popis: 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 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.
Databáze: OpenAIRE