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Nerve Conduction Studies and Electromyography in Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

J.M. Peyronnard
Affiliation:
Hôpital Hôtel-Dieu de Montréal; The Hôpital de l'Enfant Jésus, Quebec City; The Centre Hospitalier de l'Universitô de Sherbrooke, and the Clinical Research Institute of Montreal
L. Lapointe
Affiliation:
Hôpital Hôtel-Dieu de Montréal; The Hôpital de l'Enfant Jésus, Quebec City; The Centre Hospitalier de l'Universitô de Sherbrooke, and the Clinical Research Institute of Montreal
J.P. Bouchard
Affiliation:
Hôpital Hôtel-Dieu de Montréal; The Hôpital de l'Enfant Jésus, Quebec City; The Centre Hospitalier de l'Universitô de Sherbrooke, and the Clinical Research Institute of Montreal
A. Lamontagne
Affiliation:
Hôpital Hôtel-Dieu de Montréal; The Hôpital de l'Enfant Jésus, Quebec City; The Centre Hospitalier de l'Universitô de Sherbrooke, and the Clinical Research Institute of Montreal
B. Lemieux
Affiliation:
Hôpital Hôtel-Dieu de Montréal; The Hôpital de l'Enfant Jésus, Quebec City; The Centre Hospitalier de l'Universitô de Sherbrooke, and the Clinical Research Institute of Montreal
A. Barbeau*
Affiliation:
Hôpital Hôtel-Dieu de Montréal; The Hôpital de l'Enfant Jésus, Quebec City; The Centre Hospitalier de l'Universitô de Sherbrooke, and the Clinical Research Institute of Montreal
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, H2W 1R7, Quebec, Canada
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Summary:

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Twenty-six of 50 patients were investigated with nerve conduction studies and electromyography using a standard protocol and were compared to the findings in 50 normal control subjects. Almost all cases of typical Friedreich's ataxia had absent sensory action potentials (SAP) in the digital (92%) or sural (96%) nerves. The others had markedly decreased S.A.P's. In these same patients motor conduction auvelocities were either normal or only slightly decreased. In the second, atypical group of 9 patients, the motor conduction velocities were considerably decreased.

Because of the absence of sensory action potentials in Friedreich's ataxia, and that the absence was noted in our very mild cases, it is proposed that this measure be used to facilitate early diagnosis.

Type
Quebec Cooperative Study of Friedreich's Ataxia
Copyright
Copyright © Canadian Neurological Sciences Federation 1976

References

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