Relative sparing of extraocular muscles in myotonic dystrophy: an electrooculographic study

We studied 40 patients with myotonic dystrophy (MD) to investigate whether saccadic eye movement (SEM) abnormalities have a central or peripheral origin. SEMs were recorded by electrooculography and analyzed by a computer system. Six patients were followed up to 2-7 years. Slow SEMs were present in 70% of patients, while saccadic latency and accuracy were within normal ranges. Peak saccadic velocity (PSV) did not correlate with disease duration and muscular disability, and showed a significant reduction only in 1 patient during the follow-up. Muscular disability correlated significantly with age and disease duration and worsened in 4 patients over time. The doll's head maneuver elicited vestibular compensatory eye movements with high velocities. These findings suggest that the extraocular muscles are at least partially spared in MD and that supranuclear structures, most likely the burst cells in brainstem reticular formation, may contribute to the slowing of SEMs.