Social Cognition in Adolescent Girls With Fragile X Syndrome

Abstract

This study aimed to characterize social cognition, executive functions (EFs), and everyday social functioning in adolescent girls with fragile X syndrome, and identify relationships among these variables. Participants were 20 girls with FXS and 20 typically developing peers. Results showed significant between-groups differences in social cognition, accounted for by differences in IQ and language. Within the FXS group, IQ and language were related to social cognition; parent-reported social functioning was related to language and EFs; and self-reported social functioning was generally good and not related to cognitive or social cognition variables. Results suggest that intervention might focus on managing language and cognitive contributions to social functioning, rather than social cognition, and underscore the importance of considering parent and adolescent perspectives. (120 words)

Childhood Predictors of Young Adult Social Functioning in 22q11.2 Deletion Syndrome

“…These findings did not support our hypothesis and were inconsistent with previous cross-sectional 22q11.2DS studies (Campbell et al, 2015), longitudinal studies in ADHD (Diamantopoulou et al, 2007; Rinsky & Hinshaw, 2011) and schizophrenia (Sánchez et al, 2009) and cross-sectional studies in schizophrenia (Rocca et al, 2009), ASD (Gilotty et al, 2002), Turner syndrome (Lepage et al, 2013) and Fragile X syndrome (Turkstra et al, 2014). Unlike the non-significant 22q11.2DS and community control findings, childhood planning abilities was a significant predictor of self-reported social functioning in the sibling group; better planning abilities prospectively predicted higher social functioning.…”

“…Full scale IQ (Butcher et al, 2012; Campbell et al, 2015) and working memory (Campbell et al, 2015) was previously noted to be associated with social outcomes in 22q11.2DS cross-sectional studies. In the non-22q11.2DS literature, executive functioning skills including response inhibition, cognitive flexibility, working memory and planning have been documented to be associated with social functioning (Diamantopoulou, Rydell, Thorell, & Bohlin, 2007; Gilotty, Kenworthy, Sirian, Black, & Wagner, 2002; Lepage, Dunkin, Hong, & Reiss, 2013; Rinsky & Hinshaw, 2011; Rocca et al, 2009; Sánchez et al, 2009; Turkstra, Abbeduto, & Meulenbroek, 2014). Accordingly, we hypothesize that childhood Full Scale IQ (Specific Aim 3a) will significantly predict young adult social functioning in 22q11.2DS and executive functioning (Specific Aim 3b) will significantly predict young adult social functioning in all 3 groups.…”

Intellectual functioning and behavioural features associated with mosaicism in fragile X syndrome

“…Specifically, girls with FXS ( n = 20; VIQ > 70) asked fewer questions overall, fewer questions that logically flowed from the previous utterance, and spoke more repetitious phrases compared with typically developing peers; factors that would contribute to poorer quality of social overtures in an ADOS-2 assessment. Turkstra and colleagues [77] also demonstrated that the gap in performance on social cognition tasks for females with FXS compared with typically developing peers is likely attributed to general cognitive functions such as language and IQ. This phenomenon is likely further inflated in males with FXS, given their more impaired verbal abilities.…”

Signaling of noncomprehension in communication breakdowns in fragile X syndrome, Down syndrome, and autism spectrum disorder

“…Similarly, compared with age-matched peers, females with FXS took longer to initiate conversation (Lesniak-Karpiak, Mazzocco, & Ross, 2003) and asked fewer questions to maintain a topic (Mazzocco et al, 2006) in two other investigations. On a standardized test of expressive language abilities, Turkstra, Abbeduto, and Meulenbroek (2014) reported that adolescent females with FXS scored significantly lower overall than age-matched females with TD. In addition, 11 of the 20 girls with FXS had below-average scores on a pragmatic subtest.…”