Document Detail

The heart disease of Friedreich's ataxia: a clinical and electrocardiographic study of 115 patients, with an analysis of serial electrocardiographic changes in 30 cases.
MedLine Citation:
PMID:  6228949     Owner:  NLM     Status:  MEDLINE    
One hundred and fifteen patients with carefully defined Friedreich's ataxia were assessed clinically and electrocardiographically for evidence of heart disease. Cardiac symptoms, of which dyspnoea and palpitations were the most frequent, occurred in less than 30 per cent. Abnormalities on clinical examination were present in a similar proportion; harsh systolic murmurs, ventricular hypertrophy and added heart sounds were the commonest of these. Cardiac failure and persistent arrhythmias were rare and occurred late in the evolution of the neurological disease. Two patients presented with heart disease before developing neurological symptoms. Cardiac signs and symptoms were uncommon in patients without electrocardiographic abnormalities. About two-thirds of the cases had definitely abnormal ECG recordings. The characteristic finding was of widespread T-wave inversion with ventricular hypertrophy. Serial ECGs, recorded over periods of up to 32 years, were available in 30 cases and showed that abnormalities may develop in patients with Friedreich's ataxia at any time up until 20 years after the onset of neurological symptoms. In four patients initial ECG abnormalities had either improved or disappeared subsequently.
A E Harding; R L Hewer
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Quarterly journal of medicine     Volume:  52     ISSN:  0033-5622     ISO Abbreviation:  Q. J. Med.     Publication Date:  1983  
Date Detail:
Created Date:  1984-02-24     Completed Date:  1984-02-24     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0401027     Medline TA:  Q J Med     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  489-502     Citation Subset:  IM    
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MeSH Terms
Arrhythmias, Cardiac / etiology
Cardiomegaly / etiology
Dyspnea / etiology
Follow-Up Studies
Friedreich Ataxia / complications*,  physiopathology
Heart / physiopathology
Heart Diseases / etiology*
Middle Aged

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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