Visual information process in Williams syndrome: intact motion detection accompanied by typical visuospatial dysfunctions.

摘要

It has been suggested that visuospatial cognitive disabilities seen in children with Williams syndrome (WMS) are related to a dysfunction of the dorsal stream in the visual information analysis system. We investigated whether visual motion detection is also impaired in WMS because it is one of the main functions of the dorsal stream. Using various psychophysical examinations and magnetoencephalography, we studied a child with WMS who had the typical features of the syndrome. We found profound impairments in the visuospatial cognitions, as previously reported in WMS. In contrast, he had normal ability for the direction discrimination of coherent motion on a background of randomly moving dots, and he perceived apparent motion as do normal children. Furthermore, the latencies of both responses to the coherent and incoherent motions as measured by magnetoencephalography were within the mean +/- 2 SD among normal adults and the estimated origins were near the human homologue of V5/MT (visual area 5/middle temporal area). The results indicate that the visuospatial cognitive deficits in WMS can occur without impairment of the visual motion detection. We consider that the deficits are caused by a restricted dysfunction of the neural groups for position and three-dimensional form perceptions in the dorsal stream of the visual system, though other possibilities are not excluded.