Department of Psychology, University of Strathclyde, Graham Hills Building, 40 George Street, Glasgow G1 IQE, UK.
Neuropsychologia. 2010 Apr;48(5):1216-26. doi: 10.1016/j.neuropsychologia.2009.12.021. Epub 2009 Dec 21.
The present study investigated executive neuropsychological functioning in individuals with the neuro-developmental disorder Williams syndrome (WS) using a set of validated standardized neuropsychological tasks. Relatively few studies have examined frontal lobe related executive functions within the cognitive phenotype associated with the disorder. The present study compared participants with WS to typically developing participants who were individually matched for (1) chronological age and (2) verbal mental age (N=19 each group) on tasks of attention-set shifting, planning and working memory from the Cambridge Neuropsychological Test Automated Battery (CANTAB). To address the specificity of executive function impairment, non-executive tasks of delayed short-term memory and short-term memory span were also administered. Individuals with WS (mean age 18 years) showed impaired executive functioning on tasks of attention set-shifting, working memory, and planning. Non-executive deficits were also observed in short-term delayed memory and memory span. Neuropsychological impairments were correlated with a range of behavioural problems assessed using parent-rated Questionnaires. Overall, these findings point to the role of a range of executive function impairments in WS but further suggest that cognitive impairments extend beyond executive dysfunction.
本研究使用一系列经过验证的标准化神经心理学任务，调查了神经发育障碍威廉姆斯综合征（WS）个体的执行神经心理学功能。相对较少的研究在与该疾病相关的认知表型内检查了与额叶相关的执行功能。本研究将 WS 参与者与在（1）年龄和（2）语言心理年龄上与 WS 参与者相匹配的正常发育参与者进行了比较（每组 19 人），这些参与者来自剑桥神经心理学测试自动化电池（CANTAB）的注意力转换、计划和工作记忆任务。为了解决执行功能损伤的特异性问题，还进行了延迟短期记忆和短期记忆广度的非执行任务。WS 患者（平均年龄 18 岁）在注意力转换、工作记忆和计划任务中表现出执行功能障碍。在短期延迟记忆和记忆广度方面也观察到非执行缺陷。神经心理学损伤与使用父母评定问卷评估的一系列行为问题相关。总体而言，这些发现表明 WS 存在一系列执行功能障碍，但进一步表明认知损伤不仅限于执行功能障碍。
