April 1980

Friedreich's Ataxia With Acute Cardiomyopathy

Author Affiliations

From the Departments of Pediatric Neurology (Drs Berg and Kaplan), Pediatric Cardiology (Dr Molthan), and Pathology (Dr Jarrett), Good Samaritan Hospital, Phoenix, Ariz.

Am J Dis Child. 1980;134(4):390-393. doi:10.1001/archpedi.1980.04490010044015

• Friedreich's ataxia (FA) is a progressive, spinocerebellar degenerative disease. Onset is generally in the second decade of life, occurring as a neurologic degenerative process. Most, if not all, patients have an associated cardiomyopathy, which is frequently the cause of death. We studied two siblings who had FA with acute cardiomyopathy at 3 and 5 years of age, respectively, and in whom the classic nervous system signs developed, only later. The diagnosis of FA should be considered in patients of any age who have unexplained cardiomyopathy.

(Am J Dis Child 134:390-393, 1980)