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Longitudinal Evoked Potential Studies in Hereditary Ataxias

Published online by Cambridge University Press:  18 September 2015

M.J. Taylor ,
W.Y. Chan-Lui  and
W.J. Logan
M.J. Taylor*
Affiliation:
Division of Neurology, Departments of Paediatrics and Medicine, The Hospital for Sick Children. University of Toronto, Toronto, Canada
W.Y. Chan-Lui
Affiliation:
Division of Neurology, Departments of Paediatrics and Medicine, The Hospital for Sick Children. University of Toronto, Toronto, Canada
W.J. Logan
Affiliation:
Division of Neurology, Departments of Paediatrics and Medicine, The Hospital for Sick Children. University of Toronto, Toronto, Canada
*
Division of Neurology, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8
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Abstract:

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We studied multimodal evoked potentials (EPs) longitudinally in a series of children with Friedreich’s ataxia and ataxia telangiectasia to determine both their diagnostic utility and their correlation with clinical regression.

The auditory brainstem responses (ABRs) were abnormal only in the children with Friedreich’s ataxia. The abnormality seen in these patients was a rostral-caudal loss of the ABR waves. The visual EPs (VEPs) were abnormal in many of the patients; those with ataxia telangiectasia had unusually low amplitude or absent VEPs, occasionally with increased latencies, whereas those with Friedreich’s ataxia had normal amplitude VEPs, often at increased latencies. The somatosensory EPs were usually of increased latency or absent in these patients. Unlike the ABR and VEPs, they did not serve to differentiate the groups.

Changes in the EPs appeared to reflect clinical deterioration; patients with little change in their EPs over several years were regressing very slowly, whereas others had rapid deterioration in both EPs and clinical status. We suggest that the EPs are diagnostically of value in degenerative ataxias and may be of value in monitoring these patients and their response to therapy.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 12 , Issue 2 , May 1985 , pp. 100 - 105
Copyright
Copyright © Canadian Neurological Sciences Federation 1985

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