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Electromyography and Nerve Conduction Studies in Friedreich's Ataxia and Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS)

Published online by Cambridge University Press:  18 September 2015

J.P. Bouchard ,
A. Barbeau ,
R. Bouchard  and
R.W. Bouchard
J.P. Bouchard
Affiliation:
l'Hôpital de l'Enfant-Jésus, Quebec City and the Clinical Research Institute of Montreal
A. Barbeau
Affiliation:
l'Hôpital de l'Enfant-Jésus, Quebec City and the Clinical Research Institute of Montreal
R. Bouchard
Affiliation:
l'Hôpital de l'Enfant-Jésus, Quebec City and the Clinical Research Institute of Montreal
R.W. Bouchard
Affiliation:
l'Hôpital de l'Enfant-Jésus, Quebec City and the Clinical Research Institute of Montreal
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Summary

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Twenty four ataxie patients were investigated with electromyography and nerve conduction studies. They were divided in two groups according to the area they came from, the evolution of the disease, and the clinical signs. Group I patients from the Rimouski area displayed all the clinical and electrophysiological signs of Friedreich's ataxia. Group II comprised patients who presented with a new syndrome known as the autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Although the clinical evolution was better in the latter, there were more electromyographic signs of denervation and the motor conduction velocities were slower. Both groups showed identical and important abnormalities in sensory nerve conduction.

The results of electrophysiological studies in spastic ataxia have not been reported to our knowledge. They underline the place of spastic ataxia as distinct f rom Friedreich's ataxia, spastic paraplegia, and the known familial neuropathies.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 6 , Issue 2 , May 1979 , pp. 185 - 189
Copyright
Copyright © Canadian Neurological Sciences Federation 1979

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