Influence of Eye Movements on Rett Stereotypies: Evidence Suggesting a Stage-Specific Regression

The influence of eye movements on hand use and pervasive stereotypies in Rett syndrome was studied, to better understand the developmental abnormality in this disorder. Nine patients in the post-regression phase were offered objects. As expected, the girls looked at the objects but usually did not reach for them. Nor were their characteristic hand stereotypies altered by such looking. But the occurrence of stereotypies was promoted by overall shifts in gaze, ie, eye movements in any direction. This finding may be relevant to the late-infancy loss of voluntary hand use in Rett syndrome associated with onset of pervasive stereotypies. It suggests a stage-specific regression to a level characteristic of normal 3-month-old infants who do not reach and grasp, but whose hand clasping and mouthing may be triggered by the perceptual consequences of exploratory eye movements and shifts in gaze. (J Child Neurol 1998;13:158-162).