Specialization in Spatial Cognition: Evidence from Williams Syndrome

Barbara Landau
Department of Cognitive Science
Johns Hopkins University

Williams syndrome is a genetic deficit caused by a micro-deletion on chromosome 7. Individuals with this syndrome exhibit an unusual cognitive profile which has captured the attention and imagination of cognitive scientists, neuroscientists, and developmentalists. This profile includes severely impaired spatial cognition together with relatively spared language. Understanding this intriguing cognitive profile is likely to be a complex undertaking, in part because each cognitive domain (space, language) is inherently complex and multi-faceted. In order to address this challenge, I suggest a strategy of "divide and conquer", in which we examine the spatial cognitive impairment by looking at different sub-domains which have already been established by studies of normal and brain-damaged adults.

I will present evidence from our studies of object recognition and identification, perception of biological motion, navigation, and spatial language (in particular, the language of motion events). This evidence shows a strong degree of sparing in these sub-domains of spatial cognition. Importantly, where there are deficits, these do not appear to be characterized by qualitatively different kinds of (i.e. abnormal) cognitive structures; rather, they appear to reflect normally structured systems that can appear compromised due to fragile visual-spatial memory. I discuss the implications of these findings, and of our research strategy, for understanding normal cognitive architecture and breakdown under different genetic deficits.


Cognitive Neuroscience Colloquium: Friday December 7, 2001 1:30-3:00 BSBE 2-101