부산대학교 도서관

As a subset of genetic abnormalities, subtelomeric deletions have been found in 7–10 of individuals with mental retardation MR. One subtelomeric deletion, WolfHirschhorn syndrome WHS, causes mild to severe MR, but the cognitivebehavioral features of individuals with WHS have not been studied systematically. To that end, we administered a comprehensive cognitivebehavioral battery to 12 children with WHS, ages 4–17 years, who also had some expressive language. Using the StanfordBinet 4th Edition, we found cognitive deficits ranged from mild to severe, with mean IQ  44.1. Interviewing parents with the Vineland Adaptive Behavior Scales, we found mean adaptive behavior score DQ  37.3, with females exhibiting slightly higher scores than males. Cognitive profiles indicated relative strengths in Verbal and Quantitative Reasoning. Adaptive behavior profiles noted significant relative strengths in the Socialization Domain. These cognitivebehavioral profiles differed from children with other subtelomeric deletion syndromes, 2q37 or 8p23. Attention deficits and hyperactivity ADHD were observed in 712 58 of the children we tested. One child attained a score on the Child Autism Rating Scale CARS suggestive of mild autism. We conclude that different genetic disorders, which cause MR, produce diverse cognitivebehavioral profiles. Consequently, cognitivebehavioral profiles of children with MR need to be assessed more comprehensively. © 2008 WileyLiss, Inc.