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男性脆性 X 综合征分析:认知能力的优势与不足。

Profiling Fragile X Syndrome in males: strengths and weaknesses in cognitive abilities.

机构信息

Department of Developmental Psychology, University of Amsterdam, The Netherlands.

出版信息

Res Dev Disabil. 2010 Mar-Apr;31(2):426-39. doi: 10.1016/j.ridd.2009.10.013. Epub 2009 Nov 24.

DOI:10.1016/j.ridd.2009.10.013
PMID:19939624
Abstract

The present study examined the cognitive profile in Fragile X Syndrome (FXS) males, and investigated whether cognitive profiles are similar for FXS males at different levels of intellectual functioning. Cognitive abilities in non-verbal, verbal, memory and executive functioning domains were contrasted to both a non-verbal and verbal mental age reference. Model-based cluster analyses revealed three distinct subgroups which differed in level of functioning, but showed similar cognitive profiles. Results showed that cognitive performance is particularly weak on measures of reasoning- and performal abilities confined to abstract item content, but relatively strong on measures of visuo-perceptual recognition and vocabulary. Further, a significant weakness was found for verbal short-term memory. Finally, these results indicated that the choice of an appropriate reference is critically important in examining cognitive profiles. The pattern of findings that emerged from the current cognitive profiling of FXS males was interpreted to suggest a fundamental deficit in executive control.

摘要

本研究考察了脆性 X 综合征(FXS)男性的认知特征,并探讨了不同智力水平的 FXS 男性的认知特征是否相似。非言语、言语、记忆和执行功能领域的认知能力与非言语和言语智力年龄参照进行了对比。基于模型的聚类分析揭示了三个不同的亚组,这些亚组在功能水平上存在差异,但表现出相似的认知特征。结果表明,推理和执行能力的测量结果特别薄弱,仅限于抽象项目内容,而在视知觉识别和词汇方面的测量结果相对较强。此外,还发现言语短期记忆存在显著的缺陷。最后,这些结果表明,在检查认知特征时,选择适当的参照是至关重要的。从当前 FXS 男性的认知分析中得出的发现模式被解释为表明执行控制存在根本缺陷。

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