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Electroencephalographic Findings in Friedreich's Ataxia and Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS)

Published online by Cambridge University Press:  18 September 2015

R.W. Bouchard ,
J.P. Bouchard ,
R. Bouchard  and
A. Barbeau
R.W. Bouchard
Affiliation:
l'Hôpital de l'Enfant-Jésus, Quebec City and the Clinical Research Institute of Montreal
J.P. Bouchard
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
A. Barbeau
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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Electroencephalographie studies have heen done in two groups of hereditary ataxia: a group hearing the classical features of Friedreich's ataxia and a group clinically different described as autosomal recessive spastic ataxia of Charlevoix-Saguenay (A RSA CS). The qualitative anomalies observed in the two Ķroups were similar and were comparable with the data reported in the literature. However, the main difference between the two groups is the greater incidence of EEG abnormalities in the A RSA CS group, which suggests more involvement of the cortical and subcortical structures. This is reinforced by the lower I.Q. performance in the latter patients. Some comments are made about focal EEG findings, behavior and I.Q. In general, EEG was not considered a valuable instrument for diagnosis since no qualitative electric pattern could be identified. With regard to prognosis, EEG cannot be used as a criterion, since there is no relation between the degree of anomalies and the severity of the disease and since EEG does not worsen with the progression of the disease.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 6 , Issue 2 , May 1979 , pp. 191 - 194
Copyright
Copyright © Canadian Neurological Sciences Federation 1979

References

Andermann, E., Remillard, G. M., Goyer, C., Blitzer, L., Andermann, F. and Barbeau, A. (1976). Genetic and family studies in Friedreich's ataxia. Can. J. Neurol. Sci., 3, 287–301.CrossRefGoogle ScholarPubMed
Badiu, G. and Popescu-Tismana, G. (1968). Some electroencephalogrphic features of spino-cerebellar degeneration. Confín. Neurol., 30, 261–271.CrossRefGoogle Scholar
Bouchard, J. P., Barbeau, A., Bouchard, R. and Bouchard, R. W. (1978). Autosomal recessive spastic ataxia of Charlevoix-Saguenay. Can. J. Neurol. Sci., 5, 61–69.Google Scholar
Crighel, E. and Jonasescu, H. (1962). Electroencephalographic findings in two families with Friedreich's ataxia. Confín. Neurol., 22, 28–39.CrossRefGoogle ScholarPubMed
Davies, D. L. (1949a). The intelligence of patients with Friedreich's ataxia. J. Neurol. neurosurg. Psychiat., 12, 34–38.Google ScholarPubMed
Davies, D. L. (1949b). Psychiatric changes associated with Friedreich's ataxia. J. Neurol. Neurosurg. Psychiat.. 12, 246–250.Google ScholarPubMed
Guillain, G., Bertrand, I., Godet, J. and Grüner, J. (1942). L'électroencépha-logramme dans la maladie de Friedreich. C. R. Soc. Bio!., 136, 494–495.Google Scholar
Kissel, P., Arnould, G., Hartemann, P., Dureux, J. and Debry, G. (1955). L'électroencéphalogramme dans la maladie de Friedreich. Rev. Neurol., 93, 761–764.Google Scholar
Remillard, G., Andermann, F., Blitzer, L. and Andermann, E. (1976). Electroencephalographic findings in Friedreich's ataxia. Can. J. Neurol. Sci., 3, 309–312.CrossRefGoogle ScholarPubMed