Impaired behavioral and neural representation of scenes in Williams syndrome

Katrina Ferrara, Barbara Landau, Soojin Park

Research output: Contribution to journalArticlepeer-review

4 Citations (Scopus)


Boundaries are crucial to our representation of the geometric shape of scenes, which can be used to reorient in space. Behavioral research has shown that children and adults share exquisite sensitivity to a defining feature of a boundary: its vertical extent. Imaging studies have shown that this boundary property is represented in the parahippocampal place area (PPA) among typically developed (TD) adults. Here, we show that sensitivity to the vertical extent of scene boundaries is impaired at both the behavioral and neural level in people with Williams syndrome (WS), a genetic deficit that results in severely impaired spatial functions. Behavioral reorientation was tested in three boundary conditions: a flat Mat, a 5 cm high Curb, and full Walls. Adults with WS could reorient in a rectangular space defined by Wall boundaries, but not Curb or Mat boundaries. In contrast, TD age-matched controls could reorient by all three boundary types and TD 4-year-olds could reorient by either Wall or Curb boundaries. Using fMRI, we find that the WS behavioral deficit is echoed in their neural representation of boundaries. While TD age-matched controls showed distinct neural responses to scenes depicting Mat, Curb, and Wall boundaries in the PPA, people with WS showed only a distinction between the Wall and Mat or Curb, but no distinction between the Mat and Curb. Taken together, these results reveal a close coupling between the representation of boundaries as they are used in behavioral reorientation and neural encoding, suggesting that damage to this key element of spatial representation may have a genetic foundation.

Original languageEnglish
Pages (from-to)264-276
Number of pages13
Publication statusPublished - 2019 Dec

Bibliographical note

Funding Information:
This research was supported by an Integrative Graduate Education and Research Traineeship through the National Science Foundation ( DGE 0549379 to K. Ferrara), a T32 Postdoctoral Research Fellowship through the National Institutes of Health ( 5T32 HD 046388 to K. Ferrara), by a grant from the National Institutes of Health ( NIH R01 EY026042 to S. Park), by funds to B. Landau as a George Bergeron Visiting Scholar, and a grant from National Research Foundation of Korea ( MSIP-2019028919 ) to S. Park. Appendix A

Publisher Copyright:
© 2019 Elsevier Ltd

All Science Journal Classification (ASJC) codes

  • Neuropsychology and Physiological Psychology
  • Experimental and Cognitive Psychology
  • Cognitive Neuroscience


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