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KMID : 1189120100070010045
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대한의학유전학회지
2010 Volume.7 No. 1 p.45 ~ p.52
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Cognitive Profile of Children with Williams Syndrome: Comparison with Children with Prader-Willi Syndrome and Down Syndrome
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Yim Shin-Young
Cho Kye-Hee
Kim Hyon-J.
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Abstract
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Purpose: The objectives were to examine following 2 questions related to cognitive profile for the children with Williams syndrome (WS); 1) Is there a significant advantage for verbal IQ over performance IQ in WS?; 2) Is there selective impairment in visuospatial ability in the children with WS?
Materials and Methods: Five children with WS with the age of 90.86±20.73 months were compared with 12 children with Prader-Willi syndrome (PWS) or Down syndrome (DS) with comparable age and IQ.
Results: All 5 children with WS showed intellectual disability whose mean scaled scores were 15.71± 9.27 in verbal subtests and 14.29±7.50 in performance subtests, which did not show significant difference. There was no significant difference in the total sum of scaled scores of verbal subtests among WS, PWS and DS. There was no selective impairment in subtests which represented visuospatial tasks for the children with WS. However, the scaled score of object assembly was significantly lower in WS (2.29±0.95) compared to that of PWS (4.75±2.77; P<0.05).
Conclusion: The general notion that the children with WS would be relatively strong in verbal function when compared with their overall cognitive function was not observed in this study. The verbal function of the children with WS was not better when compared to the children with DS or PWS. There was no selective impairment of visuospatial function in the children with WS at this age. However, the visuospatial function was significantly low in the children with WS only when compared to the children with PWS.
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KEYWORD
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Williams syndrome, Prader-Willi syndrome, Down syndrome, Mental retardation
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