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Evolution of Cardio-Pulmonary Involvement in Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

M. Cote ,
M. Bureau ,
C. Leger ,
J. Martin ,
H. Gattiker ,
M. Cimon ,
A. Larose  and
B. Lemieux
M. Cote
Affiliation:
The Centre Hospitalier Universitaire de Sherbrooke
M. Bureau
Affiliation:
The Centre Hospitalier Universitaire de Sherbrooke
C. Leger
Affiliation:
The Centre Hospitalier Universitaire de Sherbrooke
J. Martin
Affiliation:
The Centre Hospitalier Universitaire de Sherbrooke
H. Gattiker
Affiliation:
The Centre Hospitalier Universitaire de Sherbrooke
M. Cimon
Affiliation:
The Centre Hospitalier Universitaire de Sherbrooke
A. Larose
Affiliation:
The Centre Hospitalier Universitaire de Sherbrooke
B. Lemieux
Affiliation:
The Centre Hospitalier Universitaire de Sherbrooke
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Summary

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The evolution of 15patients initially evaluated during Phase One of the Quebec Cooperative Study of Friedreich's ataxia has been studied approximately three years later. It is concluded that the deterioration of cardio-pulmonary function in Friedreich's ataxia is multi-factorial. The neuromyopathy (or the underlying metabolic or cellular defect) appears to be the main contributing factor to the deterioration of cardio-pulmonary function, which is exacerbated by the scoliosis and varying severity of the cardiomyopathy.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 6 , Issue 2 , May 1979 , pp. 151 - 157
Copyright
Copyright © Canadian Neurological Sciences Federation 1979

References

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