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Oculomotor and Vestibular Findings in Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay

Published online by Cambridge University Press:  18 September 2015

J. Dionne ,
G. Wright ,
H. Barber ,
R. Bouchard  and
J. P. Bouchard
J. Dionne
Affiliation:
Le Centre Hospitalier de l'Université Laval, the Dizziness Unit of Sunnybrook Medical Centre, Department of Otolaryngology, University of Toronto and I’Hôpital de I’Enfant-Jésus, Québec
G. Wright
Affiliation:
Le Centre Hospitalier de l'Université Laval, the Dizziness Unit of Sunnybrook Medical Centre, Department of Otolaryngology, University of Toronto and I’Hôpital de I’Enfant-Jésus, Québec
H. Barber
Affiliation:
Le Centre Hospitalier de l'Université Laval, the Dizziness Unit of Sunnybrook Medical Centre, Department of Otolaryngology, University of Toronto and I’Hôpital de I’Enfant-Jésus, Québec
R. Bouchard
Affiliation:
Le Centre Hospitalier de l'Université Laval, the Dizziness Unit of Sunnybrook Medical Centre, Department of Otolaryngology, University of Toronto and I’Hôpital de I’Enfant-Jésus, Québec
J. P. Bouchard
Affiliation:
Le Centre Hospitalier de l'Université Laval, the Dizziness Unit of Sunnybrook Medical Centre, Department of Otolaryngology, University of Toronto and I’Hôpital de I’Enfant-Jésus, Québec
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Summary

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Electronystagmographic recordings were made of oculomotor and vestibular function in II patients with autósomat recessive spastic ataxia of Charlevoix-Saguenay. All had horizontal gaze nystagmus, marked impairment of smooth ocular pursuit and optokinetic nystagmus, and defective fixation suppression of caloric nystagmus. Many had saccadic dysmetria. but saccade velocity was probably unaffected. Abnormallies pointing to brainstem disturbance were sparse. The findings are thought to indicate mainly diffuse cerebellar disease, with particular involvement of vermis and vestibulo-cerebellum.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 6 , Issue 2 , May 1979 , pp. 177 - 184
Copyright
Copyright © Canadian Neurological Sciences Federation 1979

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