title: Cognitive heterogeneity in Williams syndrome.
abstract: This study used the Woodcock-Johnson Tests of Cognitive Ability-Revised to investigate a wide range of cognitive abilities in people with Williams syndrome (WS). It involved a comparatively large sample of 31 people with WS, but took a case-series approach. The study addressed the widespread claims of a characteristic "WS cognitive profile" by looking for heterogeneity rather than homogeneity. People with WS showed a variety of preserved (significantly above mental age [MA]), expected (at MA), and significantly impaired (significantly below MA) levels of functioning. Such results provide clear evidence for heterogeneity in cognitive functions within WS. We found the most homogeneity on a test of phonological processing and a test of phonological short-term memory, with half of the WS sample performing at MA levels on these tests. Interestingly, no WS individual showed a weakness on a test of nonverbal reasoning, and only one WS individual showed a weakness on a test of verbal comprehension. In addition, we found that strengths on analysis-synthesis and verbal analogies occurred only for WS individuals with an MA less than 5.5 years (our sample median MA); people with an MA greater than 5.5 years performed at MA level on these 2 tests. Results also provided preliminary evidence for distinct subgroups of WS people based on their cognitive strengths and weaknesses on a broad range of cognitive functions. On the basis of the findings, caution should be made in declaring a single cognitive profile that is characteristic of all individuals with WS. Just as there is heterogeneity in genetic and physical anomalies within WS, not all WS individuals share the same cognitive strengths and weaknesses. Also, not all WS individuals show the profile of a strength in verbal abilities and a weakness in spatial functions.
authors.affiliation: Macquarie Centre for Cognitive Science, Macquarie University, Sydney, Australia. mporter@maccs.mq.edu.au
authors.forename: Melanie A
authors.initials: MA
authors.lastname: Porter
authors.forename: Max
authors.initials: M
authors.lastname: Coltheart
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003071:Cognition / Q000145:classification / Q000502:physiology*; D003072:Cognition Disorders / Q000145:classification / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007360:Intelligence / Q000502:physiology; D007361:Intelligence Tests; D008297:Male; D008570:Memory, Short-Term / Q000502:physiology; D009483:Neuropsychological Tests / Q000706:statistics & numerical data; D010465:Perception / Q000502:physiology; D025341:Principal Component Analysis; D011597:Psychomotor Performance / Q000502:physiology; D014706:Verbal Learning / Q000502:physiology; D018980:Williams Syndrome / Q000145:classification / Q000503:physiopathology*