Explaining Selective Spatial Breakdown in Williams Syndrome: Four Principles of Normal Spatial Development and Why They Matter

This chapter describes people with Williams syndrome (WS) - a rare genetic deficit caused by missing a handful of genes - that are severely impaired in carrying out a class of tasks commonly called " visual-spatial construction" tasks. It proposes an explanation of the spatial impairment in WS that depends heavily on the understanding of normal spatial development. It also offers four principles underlying the normal development of human spatial representations. Then, it discusses the three salient characteristics of the WS spatial profile and documents these characteristics with evidence from studies in the labs. It presents an explanation within normal spatial development for why the WS profile is the way it is. Furthermore, it explains the relationships among genes, brains, and minds and how they can account for the spatial profile of WS in the context of normal human spatial representation and its development.