Hooper Stephen R, Hatton Deborah, Sideris John, Sullivan Kelly, Hammer Julie, Schaaf Jennifer, Mirrett Penny, Ornstein Peter A, Bailey Donald P
Department of Psychiatry, University of North Carolina School of Medicine, Chapel Hill, NC27599-7255, USA.
Neuropsychology. 2008 Jan;22(1):36-47. doi: 10.1037/0894-4105.22.1.36.
The performance of 54 boys with fragile X syndrome (FXS), ages 7 to 13 years, was compared to that of a group of typically developing boys who were matched on mental age (MA) and ethnicity across multiple measures of executive function (EF). Boys with FXS varied in their ability to complete EF measures, with only 25.9% being able to complete a set-shifting task and 94.4% being able to complete a memory for word span task. When compared to the control group, and controlling for MA and maternal education, boys with FXS showed significant deficits in inhibition, working memory, cognitive flexibility/set-shifting, and planning. No group differences were observed in processing speed. Mental age significantly impacted performance on working memory, set-shifting, planning, and processing speed tasks for both groups. In boys with FXS, MA significantly predicted performance on working memory and set-shifting tasks. Our findings suggest that deficits in EF in boys with FXS are not solely attributable to developmental delays but, rather, present as a true array of neurocognitive deficits.
研究比较了54名年龄在7至13岁的脆性X综合征（FXS）男孩与一组在心理年龄（MA）和种族上相匹配的发育正常男孩在多种执行功能（EF）测量指标上的表现。患有FXS的男孩在完成EF测量任务的能力上存在差异，只有25.9%的男孩能够完成一项任务转换任务，而94.4%的男孩能够完成一项词语记忆广度任务。与对照组相比，并控制MA和母亲教育程度后，患有FXS的男孩在抑制、工作记忆、认知灵活性/任务转换和计划方面表现出显著缺陷。在处理速度方面未观察到组间差异。心理年龄对两组的工作记忆、任务转换、计划和处理速度任务的表现均有显著影响。在患有FXS的男孩中，MA显著预测了工作记忆和任务转换任务的表现。我们的研究结果表明，患有FXS的男孩的EF缺陷并非仅仅归因于发育迟缓，而是表现为一系列真正的神经认知缺陷。
