title: Working memory impairment in people with Williams syndrome: effects of delay, task and stimuli.
abstract: Williams syndrome (WS) is a neurodevelopmental disorder associated with impaired visuospatial representations subserved by the dorsal stream and relatively strong object recognition abilities subserved by the ventral stream. There is conflicting evidence on whether this uneven pattern in WS extends to working memory (WM). The present studies provide a new perspective, testing WM for a single stimulus using a delayed recognition paradigm in individuals with WS and typically developing children matched for mental age (MA matches). In three experiments, participants judged whether a second stimulus 'matched' an initial sample, either in location or identity. We first examined memory for faces, houses and locations using a 5s delay (Experiment 1) and a 2s delay (Experiment 2). We then tested memory for human faces, houses, cat faces, and shoes with a 2s delay using a new set of stimuli that were better controlled for expression, hairline and orientation (Experiment 3). With the 5s delay (Experiment 1), the WS group was impaired overall compared to MA matches. While participants with WS tended to perform more poorly than MA matches with the 2s delay, they also exhibited an uneven profile compared to MA matches. Face recognition was relatively preserved in WS with friendly faces (Experiment 2) but not when the faces had a neutral expression and were less natural looking (Experiment 3). Experiment 3 indicated that memory for object identity was relatively stronger than memory for location in WS. These findings reveal an overall WM impairment in WS that can be overcome under some conditions. Abnormalities in the parietal lobe/dorsal stream in WS may damage not only the representation of spatial location but may also impact WM for visual stimuli more generally.
authors.affiliation: Department of Psychiatry, University of Pittsburgh Medical Center, Laboratory of Neurocognitive Development, 112 Loeffler Building, 121 Meyran Avenue, Pittsburgh, PA 15213, USA. ohearnk@upmc.edu
authors.forename: Kirsten
authors.initials: K
authors.lastname: O'Hearn
authors.forename: Susan
authors.initials: S
authors.lastname: Courtney
authors.forename: Whitney
authors.initials: W
authors.lastname: Street
authors.forename: Barbara
authors.initials: B
authors.lastname: Landau
mesh_terms: D000293:Adolescent; D000328:Adult; D000704:Analysis of Variance; D002648:Child; D002675:Child, Preschool; D005145:Face; D005149:Facial Expression; D005260:Female; D006801:Humans; D008297:Male; D008569:Memory Disorders / Q000523:psychology*; D008570:Memory, Short-Term; D008875:Middle Aged; D009483:Neuropsychological Tests; D010364:Pattern Recognition, Visual; D013028:Space Perception; D013647:Task Performance and Analysis; D013997:Time Factors; D018980:Williams Syndrome / Q000523:psychology*; D055815:Young Adult