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Selective Impairment of Dorsal Stream Functions in Children with Williams Syndrome

 Daniel D. Dilks, Barbara Landau, James E. Hoffman and Jill D. Siegfried
  
 

Abstract:
Evidence suggests that visual processing is divided into two pathways: the 'ventral stream' that is specialized for object recognition (the "what" system) and the 'dorsal stream' that is specialized for the visual guidance of action (the "how" system). We investigated the possible dissociation of the two cortical visual systems in children with Williams syndrome (WS), a rare genetic disorder that results in a unique cognitive profile including impaired spatial cognition, together with relatively spared language. Following Milner & Goodale (1995), we compared performance in an action task (Posting) to that in a perceptual task (Matching). In the Posting task, children were instructed to post a card into a slot oriented to 0, 45, 90, or 135 degrees. In the Matching task, they judged when a card was aligned such that it would fit into the slot. The results of Experiment 1 showed breakdown in the Posting task among WS children relative to normally developing mental age-matched children. The results of Experiment 2, in which a 5 second delay was imposed between viewing the slot and Posting or Matching, showed additional and disproportionate breakdown in Posting among WS children. These results are consistent with the hypothesized division of labor in the visual system, and the selective impairment of the action system in Williams syndrome.

 
 


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