title: Object recognition with severe spatial deficits in Williams syndrome: sparing and breakdown.
abstract: Williams syndrome (WS) is a rare genetic disorder that results in severe visual-spatial cognitive deficits coupled with relative sparing in language, face recognition, and certain aspects of motion processing. Here, we look for evidence for sparing or impairment in another cognitive system-object recognition. Children with WS, normal mental-age (MA) and chronological age-matched (CA) children, and normal adults viewed pictures of a large range of objects briefly presented under various conditions of degradation, including canonical and unusual orientations, and clear or blurred contours. Objects were shown as either full-color views (Experiment 1) or line drawings (Experiment 2). Across both experiments, WS and MA children performed similarly in all conditions while CA children performed better than both WS group and MA groups with unusual views. This advantage, however, was eliminated when images were also blurred. The error types and relative difficulty of different objects were similar across all participant groups. The results indicate selective sparing of basic mechanisms of object recognition in WS, together with developmental delay or arrest in recognition of objects from unusual viewpoints. These findings are consistent with the growing literature on brain abnormalities in WS which points to selective impairment in the parietal areas of the brain. As a whole, the results lend further support to the growing literature on the functional separability of object recognition mechanisms from other spatial functions, and raise intriguing questions about the link between genetic deficits and cognition.
authors.affiliation: Department of Cognitive Science, Krieger Hall, Johns Hopkins University, Baltimore, MD 21218, USA. landau@cogsci.jhu.edu
authors.forename: Barbara
authors.initials: B
authors.lastname: Landau
authors.forename: James E
authors.initials: JE
authors.lastname: Hoffman
authors.forename: Nicole
authors.initials: N
authors.lastname: Kurz
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D005145:Face; D005260:Female; D006801:Humans; D008297:Male; D010468:Perceptual Disorders / Q000175:diagnosis* / Q000209:etiology*; D021641:Recognition, Psychology; D013028:Space Perception; D014796:Visual Perception; D018980:Williams Syndrome / Q000150:complications*