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Evoked Potential Studies in Friedreich's Ataxia and Progressive Early Onset Cerebellar Ataxia

Published online by Cambridge University Press:  18 September 2015

M. Vanasse ,
L. Garcia-Larrea ,
Ph. Neuschwander ,
P. Trouillas  and
F. Mauguière
M. Vanasse*
Affiliation:
Laboratoire de Neurophysiologie Sensorielle, Faculté de Médecine Lyon-Nord, EEG Department, Hôpital Neurologique, Lyon, France
L. Garcia-Larrea
Affiliation:
Laboratoire de Neurophysiologie Sensorielle, Faculté de Médecine Lyon-Nord, EEG Department, Hôpital Neurologique, Lyon, France
Ph. Neuschwander
Affiliation:
Division of Neurology, Hôpital de l'Antiquaille, Lyon, France
P. Trouillas
Affiliation:
Division of Neurology, Hôpital de l'Antiquaille, Lyon, France
F. Mauguière
Affiliation:
Laboratoire de Neurophysiologie Sensorielle, Faculté de Médecine Lyon-Nord, EEG Department, Hôpital Neurologique, Lyon, France
*
Laboratoire d'électrophysiologie médicale, Hôpital St. Justine, 3175 Côte de Ste Catherine, Montréal, Québec, Canada H3T 1C9
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Abstract:

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We recorded somatosensory evoked potentials (SEP) in 15 patients affected by Friedreich's ataxia (FA) and in 9 patients with progressive early onset cerebellar ataxia (PEOCA). Brainstem auditory evoked potentials (BAEP) were also recorded in 14 FA patients and in five PEOCA patients. SEP results showed clear differences between groups of FA, evidence of peripheral involvement was seen in all patients, with absence of the N9 potential or a major reduction of its amplitude. In patients in whom central responses could be recorded, conduction velocity was normal or near normal up to the brainstem but was reduced from brainstem to cerebral cortex. Four patients with PEOCA had SEP abnormalities similar to those seen in FA. In the five other patients, the amplitude and latency of N9 were normal but conduction velocity was reduced from brainstem to cerebral cortex. In FA, BAEP were abnormal in all patients with a disease duration of four years or more but were normal in four of the five PEOCA patients. Systematic evoked potential recording is useful in the investigation of hereditary ataxias.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 15 , Issue 3 , August 1988 , pp. 292 - 298
Copyright
Copyright © Canadian Neurological Sciences Federation 1988

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