Yim, Shin-Young
(Department of Physical Medicine and Rehabilitation Ajou University School of Medicine)
,
Cho, Kye-Hee
(Department of Rehabilitation Medicine, Seoul National University College of Medicine Seoul National University Bundang Hospital)
,
Kim, Hyon-J.
(Department of Medical Genetics, Ajou University School of Medicine)
목 적: 윌리암스증후군 아동의 인지와 관련된 두 가지 특성을 검증하고자 하였다; 윌리암스증후군 아동은 언어성 지능이 동작성 지능 보다 유의하게 높다; 윌리암스증후군 아동은 시공간기능의 선택적 저하를 보인다. 대상 및 방법: 평균연령 $90.86{\pm}20.73$개월의 5명의 윌리암스 증후군 아동의 인지적 특성을 연령 및 성별이 유사한 12명의 프라더윌리증후군 혹은 다운증후군 아동의 인지적 특성과 비교, 분석하였다. 결 과: 윌리암스증후군 아동에서 언어성 지능 항목의 합은 $15.71{\pm}9.27$, 동작성 지능 항목의 합은 $14.29{\pm}7.50$으로, 언어성 지능과 동작성 지능 간의 유의한 차이를 보이지 않았다. 윌리암스증후군, 프라더윌리증후군, 다운증후군 아동들간의 언어성 지능의 유의한 차이는 관찰되지 않았다. 윌리암스증후군 아동에서 시공간지각과 관여된 세부항목의 선택적 저하는 관찰되지 않았으나, 물체조합 항목에서 프라더윌리증후군 아동에 비하여 유의한 기능의 저하를 보였다. 결 론: 윌리암스증후군 아동이 전체 지능에 비하여 상대적으로 높은 언어성 지능을 보인다는 일반적인 개념은 본 연구에서는 관찰되지 않았다. 동시에 윌리암스증후군 아동은 프라더윌리증후군이나 다운증후군 아동과의 비교 시에도 우수한 언어기능을 보이지 않았다. 그러나 윌리암스증후군 아동은 프라더윌리증후군 아동과 비교 시 현저히 낮은 시공간기능을 보였다.
목 적: 윌리암스증후군 아동의 인지와 관련된 두 가지 특성을 검증하고자 하였다; 윌리암스증후군 아동은 언어성 지능이 동작성 지능 보다 유의하게 높다; 윌리암스증후군 아동은 시공간기능의 선택적 저하를 보인다. 대상 및 방법: 평균연령 $90.86{\pm}20.73$개월의 5명의 윌리암스 증후군 아동의 인지적 특성을 연령 및 성별이 유사한 12명의 프라더윌리증후군 혹은 다운증후군 아동의 인지적 특성과 비교, 분석하였다. 결 과: 윌리암스증후군 아동에서 언어성 지능 항목의 합은 $15.71{\pm}9.27$, 동작성 지능 항목의 합은 $14.29{\pm}7.50$으로, 언어성 지능과 동작성 지능 간의 유의한 차이를 보이지 않았다. 윌리암스증후군, 프라더윌리증후군, 다운증후군 아동들간의 언어성 지능의 유의한 차이는 관찰되지 않았다. 윌리암스증후군 아동에서 시공간지각과 관여된 세부항목의 선택적 저하는 관찰되지 않았으나, 물체조합 항목에서 프라더윌리증후군 아동에 비하여 유의한 기능의 저하를 보였다. 결 론: 윌리암스증후군 아동이 전체 지능에 비하여 상대적으로 높은 언어성 지능을 보인다는 일반적인 개념은 본 연구에서는 관찰되지 않았다. 동시에 윌리암스증후군 아동은 프라더윌리증후군이나 다운증후군 아동과의 비교 시에도 우수한 언어기능을 보이지 않았다. 그러나 윌리암스증후군 아동은 프라더윌리증후군 아동과 비교 시 현저히 낮은 시공간기능을 보였다.
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? Mat...
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 $90.86{\pm}20.73$ 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 $15.71{\pm}9.27$ in verbal subtests and $14.29{\pm}7.50$ 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 ($2.29{\pm}0.95$) compared to that of PWS ($4.75{\pm}2.77$; 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.
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 $90.86{\pm}20.73$ 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 $15.71{\pm}9.27$ in verbal subtests and $14.29{\pm}7.50$ 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 ($2.29{\pm}0.95$) compared to that of PWS ($4.75{\pm}2.77$; 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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가설 설정
1) Is there a significant advantage of verbal IQ over performance IQ in children with WS? and 2) Is there a selective impairment in visuospatial ability in children with WS?
2) Is there a selective impairment in visuospatial ability in children with WS?
제안 방법
Each of the verbal and the performance scales has 6 subtests. Among these 12 subtests, 6 subtests are included in both the K-WISC-III and the K-WPPSI; information, comprehension and arithmetic in verbal subtests; object assembly, block design and picture completion in performance subtests. The raw scores of each subtest of K-WISC-III/K-WPPSI were converted into the scaled scores which had 10 as mean and 3 as standard deviation in order to compare subtests and to obtain the intelligence quotient.
Therefore, we used scaled scores for statistical analysis instead of IQ scores. The data on the total scaled score of 6 verbal subtests, the total score of 6 performance subtests, and the scaled scores of 6 subtests which were included in both the K-WISC-III and the K-WPPSI, were collected for analysis.
대상 데이터
PWS was confirmed by the FISH, microsatellite analysis and/or methylation-specific polymerase chain reaction. Among 18 children with PWS, 5 children who were tested with either K-WISC-III or K-WPPSI were included, and one child among them had the test done three times and one child went through the test twice. Therefore, 8 tests taken by 5 children with PWS were included in this study.
236). Among the 13 children, 5 children who were included in this study were tested with either Korean Wechsler Intelligence Scale for Children-III (K-WISCIII) or Korean Wechsler Preschool and Primary Scale of Intelligence (K-WPPSI). Since remaining 8 children were too young to take the above 2 tests, they took Bailey Scales of Infant Development-II or Kauffman Battery for Children for the evaluation of cognitive function, therefore, they were not included in this study.
From August 1994 to July 2006, 13 children were diagnosed as WS at the laboratory of Department of Medical Genetics, Ajou University Medical Center. WS were confirmed by the positive fluorescent in situ hybridization test (FISH) for ELN (elastin) at chromosome 7q11.
From August 1994 to July 2006, 124 children with DS visited the Department of Physical Medicine and Rehabilitation, Ajou University Medical Center. The majority of children with DS were too young to take K-WISC-III or K-WPPSI, and 7 children who took one of the above psychological tests were included in this study. They were 3 boys and 4 girls with the age of 91.
The subjects of WS were 2 boys and 3 girls with the age of 90.86±20.73 months (range; 68-123 months old; Table 1).
Among 18 children with PWS, 5 children who were tested with either K-WISC-III or K-WPPSI were included, and one child among them had the test done three times and one child went through the test twice. Therefore, 8 tests taken by 5 children with PWS were included in this study. They were 4 boys and one girl with the age of 81.
We retrospectively reviewed the psychological reports (K-WISC-III, K-WPPSI) of these 17 children. Twenty two tests for 17 children were done by the same experienced psychologist from August 1994 to July 2006.
데이터처리
ANOVA tests for the comparison of cognitive profiles for the children with WS, PWS and DS and independent t-test for the comparison of scaled scores between verbal subtests and performance subtest within each syndrome were conducted.
성능/효과
; 3) In terms of visuospatial function, PWS is known to be proficient in putting together jigsaw puzzles27, 28), whereas DS is known to have no deficiency or proficiency in visuospatial ability.
All 5 children with WS showed intellectual disability of mean scaled scores 15.71±9.27 in verbal subtests and 14.29±7.50 in performance subtests, showing no significant difference (P >0.05; Fig. 1).
However, the comparison of psychological profiles according to syndrome showed significant difference in the scaled score of object assembly among 3 syndromes (Fig. 3), and the post hoc test revealed that the scaled score of object assembly was significantly lower in WS (2.29±0.95) than that of PWS (4.75±2.77; P <0.05).
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