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Research Review: Williams syndrome: a critical review of the cognitive, behavioral, and neuroanatomical phenotype
"... This review critically examines the research findings which characterize the cognitive, behavioral, and neuroanatomical features of Williams syndrome (WS). This article analyzes 178 published studies in the WS literature covering the following areas: 1) General intelligence, 2) Language skills, 3) ..."
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This review critically examines the research findings which characterize the cognitive, behavioral, and neuroanatomical features of Williams syndrome (WS). This article analyzes 178 published studies in the WS literature covering the following areas: 1) General intelligence, 2) Language skills, 3) Visuospatial and face processing skills, 4) Behavior patterns and hypersociability, 5) Musical abilities, and 6) Brain structure and function. We identify methodological issues relating to small sample size, use and type of control groups, and multiple measures of task performance. Previously described 'peaks' within the cognitive profile are closely examined to assess their veracity. This review highlights the need for methodologically sound studies that utilize multiple comparison groups, developmental trajectories, and longitudinal analyses to examine the WS phenotype, as well as those that link brain structure and function to the cognitive and behavioral phenotype of WS individuals.
RUNNING HEAD: Looking at Movies and Cartoons Looking at Movies and Cartoons: Eye-tracking evidence from Williams syndrome and Autism
"... Background: Autism and Williams syndrome (WS) are neuro-developmental disorders associated with distinct social phenotypes. Whilst individuals with autism show a lack of interest in socially important cues, individuals with WS often show increased interest in socially relevant information. Methods: ..."
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Background: Autism and Williams syndrome (WS) are neuro-developmental disorders associated with distinct social phenotypes. Whilst individuals with autism show a lack of interest in socially important cues, individuals with WS often show increased interest in socially relevant information. Methods: The current eye-tracking study explores how individuals with WS and autism preferentially attend to social scenes and movie extracts containing human actors and cartoon characters. The proportion of gaze time spent fixating on faces, bodies and the scene background was investigated. Results: Whilst individuals with autism preferentially attended to characters ’ faces for less time than was typical, individuals with WS attended to the same regions for longer than typical. For individuals with autism atypical gaze behaviours extended across human actor and cartoon images or movies but for WS atypicalities were restricted to human actors. Conclusions: The reported gaze behaviours provide experimental evidence of the divergent social interests associated with autism and WS.
PAPER Development of motion processing in children with autism
"... Abstract Recent findings suggest that children with autism may be impaired in the perception of biological motion from moving point-light displays. Some children with autism also have abnormally high motion coherence thresholds. In the current study we tested a group of children with autism and a g ..."
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Abstract Recent findings suggest that children with autism may be impaired in the perception of biological motion from moving point-light displays. Some children with autism also have abnormally high motion coherence thresholds. In the current study we tested a group of children with autism and a group of typically developing children aged 5 to 12 years of age on several motion perception tasks, in order to establish the specificity of the biological motion deficit in relation to other visual discrimination skills. The first task required the recognition of biological from scrambled motion. Three quasi-psychophysical tasks then established individual thresholds for the detection of biological motion in dynamic noise, of motion coherence and of form-from-motion. Lastly, individual thresholds for a task of static perception -contour integration (Gabor displays) were also obtained. Compared to controls, children with autism were particularly impaired in processing biological motion in relation to any developmental measure (chronological or mental age). In contrast, there was some developmental overlap in ability to process other types of visual motion between typically developing children and the children with autism, and evidence of developmental change in both groups. Finally, Gabor display thresholds appeared to develop typically in children with autism.
Orientation perception
, 2009
"... This article appeared in a journal published by Elsevier. The attached copy is furnished to the author for internal non-commercial research and education use, including for instruction at the authors institution and sharing with colleagues. Other uses, including reproduction and distribution, or sel ..."
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This article appeared in a journal published by Elsevier. The attached copy is furnished to the author for internal non-commercial research and education use, including for instruction at the authors institution and sharing with colleagues. Other uses, including reproduction and distribution, or selling or licensing copies, or posting to personal, institutional or third party websites are prohibited. In most cases authors are permitted to post their version of the article (e.g. in Word or Tex form) to their personal website or institutional repository. Authors requiring further information regarding Elsevier’s archiving and manuscript policies are encouraged to visit:
Mathematical skill in individuals with Williams syndrome: Evidence from a standardized mathematics battery
, 2007
"... Williams syndrome (WS) is a developmental disorder associated with relatively spared verbal skills and severe visuospatial deficits. It has also been reported that individuals with WS are impaired at mathematics. We examined mathematical skills in persons with WS using the second edition of the Test ..."
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Williams syndrome (WS) is a developmental disorder associated with relatively spared verbal skills and severe visuospatial deficits. It has also been reported that individuals with WS are impaired at mathematics. We examined mathematical skills in persons with WS using the second edition of the Test of Early Mathematical Ability (TEMA-2), which measures a wide range of skills. We administered the TEMA-2 to 14 individuals with WS and 14 children matched individually for mental-age on the matrices subtest of the Kaufman Brief Intelligence Test. There were no differences between groups on the overall scores on the TEMA-2. However, an item-by-item anal-ysis revealed group differences. Participants with WS performed more poorly than controls when reporting which of two numbers was closest to a target number, a task thought to utilize a mental number line subserved by the parietal lobe, consistent with previous evidence showing parietal abnormalities in people with WS. In contrast, people with WS performed better than the control group at reading num-bers, suggesting that verbal math skills may be comparatively strong in WS. These findings add to evidence that components of math-ematical knowledge may be differentially damaged in developmental disorders.
