Friedreich's ataxia with acute cardiomyopathy

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...

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Bibliographic Details
Published inAmerican journal of diseases of children (1960) Vol. 134; no. 4; p. 390
Main Authors Berg, R A, Kaplan, A M, Jarrett, P B, Molthan, M E
Format Journal Article
LanguageEnglish
Published United States 01.04.1980
Subjects
Child
Electrocardiography
Female
Friedreich Ataxia - complications
Friedreich Ataxia - diagnosis
Friedreich Ataxia - pathology
Heart Failure - etiology
Humans
Male
Myocardium - pathology
Spinal Cord - pathology
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Summary: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.
ISSN:0002-922X
DOI:10.1001/archpedi.1980.04490010044015