Department of Neuroscience, Bambino Gesù Children's Hospital, Piazza Sant'Onofrio 4, I-00165 Rome, Italy.
Res Dev Disabil. 2013 May;34(5):1770-80. doi: 10.1016/j.ridd.2013.01.024. Epub 2013 Mar 15.
Executive functions are a set of high cognitive abilities that control and regulate other functions and behaviors and are crucial for successful adaptation. Deficits in executive functions are frequently described in developmental disorders, which are characterized by disadaptive behavior. However, executive functions are not widely examined in individuals with intellectual disability. The present study is aimed at evaluating the etiological specificity hypotheses pertaining to executive functions by comparing individuals with intellectual disability of different etiology, as Williams syndrome and Down syndrome, on different aspects of executive functions. To this aim a battery evaluating attention, short-term and working memory, planning, categorization, shifting and inhibition, was administered to 15 children, adolescents and adults with Williams syndrome, to 15 children, adolescents and adults with Down syndrome and to 16 mental-age-matched typically developing children. The two groups with intellectual disability showed impairment in a set of executive functions, as auditory sustained attention, visual selective attention, visual categorization and working memory, and preserved visual sustained attention, auditory selective attention and visual inhibition. However, a distinctive profile has been found between the two syndromic groups on other executive functions. While participants with Down syndrome were poor in shifting and verbal aspects of memory and inhibition, those with Williams syndrome were poor in planning. The specific weakness and straights on executive functions may support the etiological specificity hypothesis accounting for distinctive cognitive development syndrome-specific.
执行功能是一组高级认知能力,可控制和调节其他功能和行为,对成功适应至关重要。在发育障碍中经常描述执行功能缺陷,其特征是适应不良行为。然而,在智力障碍个体中,执行功能并未广泛研究。本研究旨在通过比较不同病因(如威廉姆斯综合征和唐氏综合征)的智力障碍个体,评估与执行功能相关的病因特异性假说,以不同的执行功能方面进行评估。为此,对 15 名患有威廉姆斯综合征的儿童、青少年和成年人,15 名患有唐氏综合征的儿童、青少年和成年人以及 16 名智力年龄匹配的正常发育儿童进行了一系列评估注意力、短期和工作记忆、计划、分类、转移和抑制的测试。两组智力障碍个体在一系列执行功能方面存在缺陷,如听觉持续注意力、视觉选择性注意力、视觉分类和工作记忆,而视觉持续注意力、听觉选择性注意力和视觉抑制则保持完好。然而,在其他执行功能方面,两个综合征组之间存在独特的表现。唐氏综合征患者在转换和记忆的言语方面以及抑制方面表现较差,而威廉姆斯综合征患者则在计划方面表现较差。执行功能的特定弱点和强项可能支持病因特异性假说,解释特定的认知发展综合征特异性。