williams syndrome group
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2016 ◽  
Vol 29 (4) ◽  
pp. 1189-1197 ◽  
Author(s):  
Karen J. Goldman ◽  
Cory Shulman ◽  
Yair Bar-Haim ◽  
Rany Abend ◽  
Jacob A. Burack

AbstractIndividuals with Williams syndrome and those with Down syndrome are both characterized by heightened social interest, although the manifestation is not always similar. Using a dot-probe task, we examined one possible source of difference: allocation of attention to facial expressions of emotion. Thirteen individuals with Williams syndrome (mean age = 19.2 years, range = 10–28.6), 20 with Down syndrome (mean age = 18.8 years, range = 12.1–26.3), and 19 typically developing children participated. The groups were matched for mental age (mean = 5.8 years). None of the groups displayed a bias to angry faces. The participants with Williams syndrome showed a selective bias toward happy faces, whereas the participants with Down syndrome behaved similarly to the typically developing participants with no such bias. Homogeneity in the direction of bias was markedly highest in the Williams syndrome group whose bias appeared to result from enhanced attention capture. They appeared to rapidly and selectively allocate attention toward positive facial expressions. The complexity of social approach behavior and the need to explore other aspects of cognition that may be implicated in this behavior in both syndromes is discussed.


2010 ◽  
Vol 115 (2) ◽  
pp. 172-189 ◽  
Author(s):  
Tricia A. Thornton-Wells ◽  
Christopher J. Cannistraci ◽  
Adam W. Anderson ◽  
Chai-Youn Kim ◽  
Mariam Eapen ◽  
...  

Abstract Williams syndrome is a genetic neurodevelopmental disorder with a distinctive phenotype, including cognitive–linguistic features, nonsocial anxiety, and a strong attraction to music. We performed functional MRI studies examining brain responses to musical and other types of auditory stimuli in young adults with Williams syndrome and typically developing controls. In Study 1, the Williams syndrome group exhibited unforeseen activations of the visual cortex to musical stimuli, and it was this novel finding that became the focus of two subsequent studies. Using retinotopy, color localizers, and additional sound conditions, we identified specific visual areas in subjects with Williams syndrome that were activated by both musical and nonmusical auditory stimuli. The results, similar to synesthetic-like experiences, have implications for cross-modal sensory processing in typical and atypical neurodevelopment.


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