Chapter

The Puzzle of Williams Syndrome

Barbara Landau and James E. Hoffman

in Spatial Representation

Published in print September 2012 | ISBN: 9780195385373
Published online January 2013 | e-ISBN: 9780199979189 | DOI: http://dx.doi.org/10.1093/acprof:oso/9780195385373.003.0001

Series: Oxford Series in Developmental Cognitive Neuroscience

The Puzzle of Williams Syndrome

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This chapter poses the central question of the book: How can the absence of approximately twenty-five genes in people with WS cause the unusual cognitive profile of severely impaired spatial cognition together with fluent and well-structured language? After presenting a description of this hallmark cognitive profile, the chapter argues that answering this question requires considering three more general issues. First, the complexity of the causal chain between genes and cognition requires that we clearly specify the target level of explanation—in this book, the chapter targets the level of cognition. Second, the chapter argues that the highly specialized nature of human spatial-cognitive architecture plays a crucial role in explaining any developmental outcomes. Third, the chapter argues for the importance of considering normal developmental trajectories in the emergence of spatial cognitive systems in people with WS. The chapter closes with a plan for the remaining chapters.

Keywords: Williams syndrome; spatial representation; language; development; genetic deficit; cognitive profile; block construction task; domain specialization; developmental disorders; phenotype

Chapter.  10407 words.  Illustrated.

Subjects: Cognitive Psychology

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