Walsh & Hoyt: Clinical Features of Nystagmus with Lesions Affecting the Visual Pathways

Congenital or acquired retinal disorders causing blindness, such as Lebers congenital amaurosis, lead to continuous jerk nystagmus with components in all three planes, which changes direction over the course of seconds or minutes. The drifting ""null point"" the eye position at which nystagmus changes direction probably reflects inability to calibrate the ocular motor system, and it has also been reported after experimental cerebellectomy. This nystagmus often shows the increasing-velocity waveform that was once thought to be specific for congenital nystagmus. Recent developments in gene therapy for retinal disorders suggest that if vision can be restored, nystagmus will be suppressed.