Fragile X syndrome in young females: Influence of executive function on the neurocognitive profile and adaptive behavior

Abstract

Introduction: The aim of this study is to describe the relationship between executive function (EF) and performance in different areas of the neurocognitive profile in young girls with Fragile-X-Syndrome (FXS).

Method: A neuropsychological assessment was carried out to 40 female participants aged 7-16 years (26 FXS, 14 control group).

Results: Regarding intellectual ability, in the group of girls with FXS 3.84 % of the participants obtained IQ scores in the range of moderate ID (IQ 35-40 to 49), 46.15 % in the range of mild ID (IQ 50-70), 38.46 % in the borderline range (IQ 70-85), and 11.53 % within the average range (IQ > 85). EF was found to have a greater influence on adaptive behavior, arithmetic ability, theory of mind, leadership, social integration, social competence, and anxiety/shyness in the group with FXS.

Conclusions: In girls with FXS, EF showed a greater influence on adaptive behavior, arithmetic ability, and social domain.

Keywords: Adaptive behavior; Executive function; Females; Fragile-X-Syndrome; Neurocognitive profile.