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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

References

Andermann, E., Remillard, G., Goyer, C., Blitzer, L., Andermann, F. and Barbeau, A. (1976). Genetic and family studies in Friedreich's ataxia. Cañad. J. Neurol. Sci., 3, 287–301.CrossRefGoogle ScholarPubMed
Andermann, F. (1976). Nicolaus Friedreich and degenerative atrophy of the posterior columns of the spinal cord. Canad. J. Neurol. Sci., 3, 275–277.CrossRefGoogle ScholarPubMed
Baloh, R. W., Konrad, H. R. and Hon-Rubia, V. (1975). Vestibulo-ocular function in patients with cerebellar atrophy. Neurology (Minn.), 25, 160–168.CrossRefGoogle ScholarPubMed
Barbeau, A. (1978). Friedreich's ataxia 1978 — an overview. Canad. J. Neurol. Sci., 5, 161–165.CrossRefGoogle ScholarPubMed
Dale, R. T., Dirby, A. W. and Jampel, R. S. (1978). Square wave jerks in Friedreich's ataxia. Am. J. Ophthalmol., 85, 400–406.CrossRefGoogle ScholarPubMed
Dell'osso, L. F., Troost, B. T. and Daroff, R. B. (1975). Macro square wave jerks. Neurology (Minn.), 25, 975–979.CrossRefGoogle ScholarPubMed
Dichgans, J. (1977). Optokinetic nystagmus as dependent on the retinal periphery via the vestibular nucleus. In: Control of gaze by brain stem neurons. Developments in Neuroscience. Vol. I, (Baker, R. and Berthoz, A., eds.) pp. 261–266.Google Scholar
Geoffroy, G., Barbeau, A., Breton, G., Lemieux, B., Leger, C., Aube, M., Bouchard, J. P. (1976). Clinical description and roentgenologic evaluation of patients with Friedreich's ataxia. Canad. J. Neurol. Sci., 3, 279–286.CrossRefGoogle ScholarPubMed
Gorman, W. F. and Brock, S. (1950). Periodic alternating nystagmus in Friedreich's ataxia. Am. J. Ophthalmol., 33, 860–864.CrossRefGoogle ScholarPubMed
Hassul, M., Daniels, P.D. and Kimm, J. (1976). Effects of bilateral flocculectomy on the vestibulo-ocular reflex in the chinchilla. Brain Research, 118, 339–343.CrossRefGoogle ScholarPubMed
Hood, J. D., Kayan, A. and Leech, J. (1973). Rebound nystagmus. Brain, 96, 507–526.CrossRefGoogle ScholarPubMed
Kato, L., Kimura, Y., Aoyagi, M., Mizukoshi, K. and Kawasaki, T. (1977). Visual suppression of caloric nystagmus in normal individuals. Acta Otolaryngol., 83, 245–251.CrossRefGoogle ScholarPubMed
Kreindler, A., Poilici, I. and Crighel, E. (1963). Das Symptom des “erschwerten Seitenblicks” bei heredodgen-erativen spinocerebellaren Erkrankungen. Nervenarzt, 34, 504–506.Google Scholar
Miles, F. A. and Fuller, J. H. (1975). Visual tracking and the primate flocculus. Science, 189, 1000–1002.CrossRefGoogle ScholarPubMed
Mizukoshi, K., Fabian, P. and Stahle, J. (1977). Optokinetic test comprising both acceleration and constant velocity stimulation (ACV-OKN test). Acta Otolaryngol., 84, 155–165.CrossRefGoogle ScholarPubMed
Monday, L. A., LemieuxB., ST. B., ST.Vincent, H. and Barbeau, A. (1978). Clinical and electronystagmographic findings in Friedreich's ataxia. Cañad. J. Neurol. Sci., 5, 71–73.CrossRefGoogle ScholarPubMed
Morissette, Y., Abel, S. M. and Barber, H. O. (1974). Optokinetic nystagmus in otoneurological diagnosis. Canad. J. Otolaryngol., 3, 348–362.Google Scholar
Nemet, P. and Ron, S. (1977). Cerebellar rôle in smooth pursuit movement. Docum. Ophthalmol., 43, 101–107.CrossRefGoogle ScholarPubMed
Optican, L. and Robinson, D. A. (1978). Cerebellar dependent adaptation of the saccadic system. Abstracts. Second Congress of the International Society of Neuro-Ophthalmology, Washington, Maryland.Google Scholar
Ritchie, L. (1976). Effects of cerebellar lesions on saccadic eye movements. J. Neurophysiol., 39, 1246–1256.CrossRefGoogle ScholarPubMed
Robinson, D. A. (1974). The effects of cere-bellectomy on the cat's vestibulo-ocular integrator. Brain Research, 71, 195–207.CrossRefGoogle Scholar
Robinson, D. A. (1977). Vestibular and optokinetic symbiosis: and example of explaining by modelling. In: Control of gaze by brain stem neurons. Developments iń Neuroscience. Elsevier/North Holland Biomedicai Press, Vol. I. (Baker, R. and Berthoz, A., eds.) pp 49–58.Google Scholar
Ron, S. and Nemet, P. (1977). The cerebellum involvement in the generation of saccades. Docum. Ophthalmol., 43, 109–114.CrossRefGoogle ScholarPubMed
Spiller, W. G. (1910). Friedreich's ataxia. J. Nerv. Ment. Dis., 37, 411–435.CrossRefGoogle Scholar
Takemori, S. and Cohen, B. (1974). Loss of visual suppression of vestibular nystagmus after flocculus lesions. Brain Research, 72, 213–224.CrossRefGoogle ScholarPubMed
Troost, B. T. and Daroff, R. B. (1977). The ocular motor defects in progressive supranuclear palsy. Ann. Neurol., 2, 397–403.CrossRefGoogle ScholarPubMed
Troost, B. T., Dell'osso, L. F. and Daroff, R. B. (1976). Effects of visual pursuit deficit on the human vestibulo-ocular reflex. Neurology (Minn.), 26, 352–353.Google Scholar
Troost, B. T., Weber, R. B. and Daroff, R. B. (1974). Hypometric saccades. Am. J. Ophthalmol., 78, 1002–1005.CrossRefGoogle ScholarPubMed
Tyrer, J. H. (1975). Friedreich's ataxia. In: Handbook of Clinical Neurology. North Holland Press., Vol. 21. (Vinken, P. J. and Bruyn, E. W., eds.) pp 319–364.Google Scholar
Walsh, F. B. and Hoyt, W. F. (1969). Clinical Neuro-Ophthalmology. Third Edition. The Wiliams and Wilkis Co., Vol. I. pp 919–922.Google Scholar
Whyte, J. M. (1898). Four cases of Friedreich's ataxia with a critical digest of recent literature on the subject. Brain, 21, 72–136.Google Scholar
Young, L. R. (1971). Pursuit eye tracking movements. In: The control of eye movements. Academic Press. (Bach-Y-Rita, P., Collins, C. C. and Hyde, J. E., eds.) pp 429–443,Google Scholar
Zee, D. S. (1977). Suppression of vestibular nystagmus. Ann. Neurol., 1, 207.CrossRefGoogle ScholarPubMed
Zee, D. S., Yee, R. D., Cogan, D. G., Robinson, D. A. and Engel, W. K. (1976). Ocular motor abnormalities in hereditary cerebellar ataxia. Brain, 99, 207–234.CrossRefGoogle ScholarPubMed