Profiling Fragile X Syndrome in males: Strengths and weaknesses in cognitive abilities

被引：60
作者：
Van der Molen, M. J. W. [1 ]
Huizinga, M. [1 ]
Huizenga, H. M. [1 ]
Ridderinkhof, K. R. [1 ]
Van der Molen, M. W. [1 ]
Hamel, B. J. C. [2 ]
Curfs, L. M. G. [3 ,4 ]
Ramakers, G. J. A. [1 ]
机构：
[1] Univ Amsterdam, Dept Dev Psychol, NL-1018 WB Amsterdam, Netherlands
[2] Univ Nijmegen, Dept Clin Genet, Nijmegen, Netherlands
[3] Maastricht Univ, GROW Sch Oncol & Dev Biol, Maastricht, Netherlands
[4] Acad Hosp Maastricht, Dept Clin Genet, Maastricht, Netherlands
来源：
RESEARCH IN DEVELOPMENTAL DISABILITIES | 2010年 / 31卷 / 02期
关键词：
Fragile X Syndrome; Cognitive profile; Model-based cluster analysis; Subgroups; Executive control; MENTAL-RETARDATION; WORKING-MEMORY; YOUNG MALES; FLUENCY PERFORMANCE; EXECUTIVE FUNCTIONS; RHO-PROTEINS; FRONTAL-LOBE; CGG REPEAT; ATTENTION; CHILDREN;
D O I：
10.1016/j.ridd.2009.10.013
中图分类号：
G76 [特殊教育];
学科分类号：
040109 ;
摘要：
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. (C) 2009 Elsevier Ltd. All rights reserved.
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收藏
页码：426 / 439
页数：14
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