Department of Psychiatry and Forensic Medicine, Facultat de Medicina. Universitat Autònoma de Barcelona, Barcelona, Spain.
Division of Neuropediatrics, Corporación Sanitaria Parc Taulí, Sabadell, Spain.
Am J Med Genet A. 2021 May;185(5):1448-1460. doi: 10.1002/ajmg.a.62130. Epub 2021 Feb 18.
Fragile X syndrome (FXS) is the leading cause of inherited intellectual disability. The objective of this research is to analyze the relationship between linguistic functions and performance of the following neuropsychological functions: executive, quantitative reasoning, social perception, behavior, social skills, and adaptive behavior. A neuropsychological and behavioral evaluations were carried out with a group of 26 girls with FXS, and 14 girls without FXS as a control group, using standardized tests. The two groups were homogeneous in age and IQ. Significant differences were found between groups in the relationship between some language processes: inhibition, auditory working memory, cognitive flexibility, level of social adaptation, self-direction, conceptual adaptation, academic skills, leadership ability, theory of mind, and arithmetic. In the group of girls with FXS, it was found that different aspects of language influence some of the executive functions evaluated, in addition to some specific aspects of social perception, adaptive behavior, and quantitative reasoning, in different ways. Future research should incorporate the study of the influence of other cognitive variables such as visual perception and executive function on behavioral, social, and adaptive aspects to know the real influence of all the cognitive variables on the behavior of girls with FXS.
脆性 X 综合征 (FXS) 是遗传性智力障碍的主要原因。本研究旨在分析语言功能与以下神经心理学功能表现之间的关系:执行功能、定量推理、社会感知、行为、社交技能和适应行为。对一组 26 名患有 FXS 的女孩和 14 名无 FXS 的女孩进行了神经心理学和行为评估,使用了标准化测试。两组在年龄和智商方面具有同质性。在一些语言过程的关系上,两组之间存在显著差异:抑制、听觉工作记忆、认知灵活性、社会适应水平、自我指导、概念适应、学业技能、领导能力、心理理论和算术。在患有 FXS 的女孩组中,发现语言的不同方面以不同的方式影响评估的一些执行功能,以及一些特定的社会感知、适应行为和定量推理方面。未来的研究应该纳入对其他认知变量(如视觉感知和执行功能)对行为、社会和适应方面的影响的研究,以了解所有认知变量对患有 FXS 的女孩行为的真正影响。