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Brain, Vol. 113, No. 2, 463-473, 1990
© 1990 Oxford University Press


research-article

DISORDERS OF EYE MOVEMENT IN MYOTONIC DYSTROPHY

J. P. TER BRUGGEN1, L. A. K. BASTIAENSEN3, C. C. TYSSEN1,2, and G. GIELEN2

1Department of Neurology Tilburg, The Netherlands 2Department of Neurophysiology Tilburg, The Netherlands 3Department of Ophthalmology, St Elisabeth and Maria Hospitals Tilburg, The Netherlands

Correspondence to: Correspondence to: Dr C. C. Tyssen, Department of Neurology, st Elisabeth Hospital, PO Box 90151, 5000 LC Tilburg, The Netherlands.

Horizontal saccades and smooth pursuit eye movements were studied in 26 patients with myotonic dystrophy. Clinical neuro-ophthalmological investigations in 1 patient revealed an inability to achieve a full range of eye movements. Electro-oculography showed a significant decrease of the maximum velocity of the visually-guided saccades in 83% of the patients. Smooth pursuit eye movements were not significantly different from age-matched controls. Visual evoked potential (VEP) latencies (P100) were significantly prolonged compared with controls in 64% of the patients. The saccadic latency of the visually-guided saccades was correlated with the prolonged VEP latencies, indicating that lesions in the primary visual pathways probably contribute to the oculomotor dysfunction. The isolated decrease of the maximum velocity of the saccades in combination with EMG findinsgs favours a peripheral (dystrophic) pathophysiological mechanism.

Received July 1, 1988. Revised April 25, 1989. Accepted May 24, 1989.


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