Central ocular motor abnormalities in Duane's retraction syndrome

Duane's retraction syndrome (DRS) is a congenital eye movement disorder characterized by marked limitation or absence of abduction, variable limitation of adduction, and narrowing of the palpebral fissure with retraction of the globe on attempted adduction. We have recently recorded and quantitated ocular motility in five patients with unilateral DRS. In all patients, abduction of the affected eye was greatly limited, whereas adduction was limited, whereas adduction was limited to a lesser degree. Abnormalities in saccadic velocities were found in both the affected eye and the sound eye. Results of testing of the vestibulo-ocular reflex, optokinetic nystagmus, and optokinetic afternystagmus showed notable asymmetry. Our results suggest that DRS is produced by a primary brainstem abnormality involving premotor structures.