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Oculomotor Abnormalities in Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

T.H. Kirkham ,
D. Guitton ,
A. Katsarkas ,
L.B. Kline  and
E. Andermann
T.H. Kirkham
Affiliation:
McGill University Departments of Neurology, Neurosurgery, Opthalmology, Otolaryn-gology and Neurogenetics, the Montreal Neurological Hospital and Institute and the Royal Victoria Hospital, Montreal, Quebec, Canada
D. Guitton
Affiliation:
McGill University Departments of Neurology, Neurosurgery, Opthalmology, Otolaryn-gology and Neurogenetics, the Montreal Neurological Hospital and Institute and the Royal Victoria Hospital, Montreal, Quebec, Canada
A. Katsarkas
Affiliation:
McGill University Departments of Neurology, Neurosurgery, Opthalmology, Otolaryn-gology and Neurogenetics, the Montreal Neurological Hospital and Institute and the Royal Victoria Hospital, Montreal, Quebec, Canada
L.B. Kline
Affiliation:
McGill University Departments of Neurology, Neurosurgery, Opthalmology, Otolaryn-gology and Neurogenetics, the Montreal Neurological Hospital and Institute and the Royal Victoria Hospital, Montreal, Quebec, Canada
E. Andermann
Affiliation:
McGill University Departments of Neurology, Neurosurgery, Opthalmology, Otolaryn-gology and Neurogenetics, the Montreal Neurological Hospital and Institute and the Royal Victoria Hospital, Montreal, Quebec, Canada
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A clinical neuro-opthalmo-logical and electro-oculographic study was made on fourteen patients with Friedreich's ataxia. None had evidence of optic nerve dysfunction. No patient complained of oscillopsia although all had ocular motor deficits of varying degrees, which appeared to be related to the severity of the general manifestations of the disease. The defects comprised square wave jerks, jerky pursuit with inability to maintain eccentric gaze resulting in gaze paretic nystagmus and rebound nystagmus. There was failure to suppress by fixation the vestíbulo-ocular reflex. The slow phase velocity of caloric nystagmus was always of reduced velocity. There was inability to augment the slow phase velocity of optokinetic nystagmus with increasing stimulus velocity. Abnormalities of the saccadic system were manifest particularly as hypermetria. These signs in combination are suggestive of disease involving the cere be I lar flocculus and vermis or their brain stem connections. No abnormalities were found in 17 parents or siblings.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 6 , Issue 2 , May 1979 , pp. 167 - 172
Copyright
Copyright © Canadian Neurological Sciences Federation 1979

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