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Wiley Interdiscip Rev Cogn Sci. 2013 Nov;4(6):693-706. doi: 10.1002/wcs.1258. Epub 2013 Sep 30.

Space and language in Williams syndrome: insights from typical development.

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1
Department of Cognitive Science, Johns Hopkins University, Baltimore, MD, USA.

Abstract

One of the holy grails of cognitive science is to understand the causal chain that links genes and cognition. Genetic syndromes accompanied by cognitive effects offer natural experiments that can uniquely inform our understanding of this chain. In this article, we discuss the case of Williams syndrome (WS), which is characterized by a set of missing genes on chromosome 7q11.23, and presents with a unique cognitive profile that includes severe spatial impairment along with strikingly fluent and well-structured language. An early inference from this profile was the idea that a small group of genes could directly target one cognitive system while leaving others unaffected. Recent evidence shows that this inference fails. First, the profile within the spatial domain is varied, with relative strength in some aspects of spatial representation but severe impairment in others. Second, some aspects of language may fail to develop fully, raising the question of how to compare the resilience and fragility of the two key cognitive domains in this syndrome. Third, much research on the profile fails to place findings in the context of typical developmental trajectories. We explore these points and propose a new hypothesis that explains the unusual WS cognitive profile by considering normal mechanisms of cognitive development that undergo change on an extremely prolonged timetable. This hypothesis places the elements of the WS cognitive profile in a new light, refocuses the discussion of the gene-cognition causal chain for WS and other disorders, and more generally, underlines the importance of understanding cognitive structure in both typical and atypical development. WIREs Cogn Sci 2013, 4:693-703. doi: 10.1002/wcs.1258 Conflict of interest: The authors have declared no conflicts of interest for this article. For further resources related to this article, please visit the WIREs website.

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