PubMed2003
We investigated the role of executive and spatial representational processes in impaired performance of block construction tasks by children with Williams syndrome (WS), a rare genetic defect that results in severely impaired spatial cognition. In Experiment 1, we examined performance in two kinds of block construction tasks, Simple Puzzles, in which block faces contained a single color, and Complex, in which some block faces contained an arrangement of two colors. WS and control children were comparable in their ability to solve simple puzzles, and showed similar eye-fixation patterns, suggesting that basic executive processes were intact. However, WS children were severely impaired in their ability to solve complex puzzles. In these puzzles, WS children fixated the complex puzzle models and checked their partial solutions less often than normal children, but they were comparable in their ability to detect errors in their copies and almost exclusively made repairs to copies that were, in fact, incorrect. We conjecture that the abnormal fixation patterns were a consequence of impoverished spatial representations, rather than a cause of it. This conjecture was tested in Experiment 2, where we examined children's capacity to match and place individual blocks without engaging the complex executive processes required to carry out a complete puzzle solution. We found serious deficiency among WS children in both aspects of spatial representation. Moreover, estimates of the errors in representing the identity and location of model blocks derived from Experiment 2 provided a good account of the observed errors in the block construction task of Experiment 1.