Social cognition dysfunction in adolescents with 22q11.2 deletion syndrome (velo-cardio-facial syndrome): relationship with executive functioning and social competence/functioning.
BACKGROUND
Social difficulties are often noted among people with intellectual disabilities. Children and adults with 22q.11.2 deletion syndrome (22q11DS) often have poorer social competence as well as poorer performance on measures of executive and social-cognitive skills compared with typically developing young people. However, the relationship between social functioning and more basic processes of social cognition and executive functioning are not well understood in 22q11DS. The present study examined the relationship between social-cognitive measures of emotion attribution and theory of mind with executive functioning and their contribution to social competence in 22q11DS.


METHODS
The present cross-sectional study measured social cognition and executive performance of 24 adolescents with 22q11DS compared with 27 age-matched typically developing controls. Social cognition was tested using the emotion attribution task (EAT) and a picture sequencing task (PST), which tested mentalising (false-belief), sequencing, cause and effect, and inhibition. Executive functioning was assessed using computerised versions of the Tower of London task and working memory measures of spatial and non-spatial ability. Social competence was also assessed using the parent-reported Strengths and Difficulties Questionnaire.


RESULTS
Adolescents with 22q11DS showed impaired false-belief, emotion attribution and executive functioning compared with typically developing control participants. Poorer performance was reported on all story types in the PST, although, patterns of errors and response times across story types were similar in both groups. General sequencing ability was the strongest predictor of false-belief, and performance on the false-belief task predicted emotion attribution accuracy. Intellectual functioning, rather than theory of mind or executive functioning, predicted social competence in 22q11DS.


CONCLUSIONS
Performance on social-cognitive tasks of theory of mind indicate evidence of a general underlying dysfunction in 22q11DS that includes executive ability to understand cause and effect, to logically reason about social scenarios and also to inhibit responses to salient, but misleading cues. However, general intellectual ability is closely related to actual social competence suggesting that a generalised intellectual deficit coupled with more specific executive impairments may best explain poor social cognition in 22q11DS.
Centre for Translational Neuroscience and Mental Health Research, University of Newcastle, McAuley Centre, Waratah, NSW, 2298, Australia.|Hunter Medical Research Institute, Locked Bag 1, Hunter Region Mail Centre, NSW, 2310, Australia.|School of Psychology, University of Newcastle, Ourimbah, NSW, 2258, Australia.
L E
LE
Campbell
Centre for Translational Neuroscience and Mental Health Research, University of Newcastle, McAuley Centre, Waratah, NSW, 2298, Australia.|Brain & Mind Research Institute, University of Sydney, Camperdown, NSW, 2050, Australia.|Schizophrenia Research Institute, 405 Liverpool St Darlinghurst, NSW, 2010, Australia.
K L
KL
McCabe
School of Psychology, University of Newcastle, Ourimbah, NSW, 2258, Australia.
J L
JL
Melville
Centre for Translational Neuroscience and Mental Health Research, University of Newcastle, McAuley Centre, Waratah, NSW, 2298, Australia.|School of Psychology, University of Newcastle, Ourimbah, NSW, 2258, Australia.
P A
PA
Strutt
Centre for Translational Neuroscience and Mental Health Research, University of Newcastle, McAuley Centre, Waratah, NSW, 2298, Australia.|Hunter Medical Research Institute, Locked Bag 1, Hunter Region Mail Centre, NSW, 2310, Australia.|School of Medicine and Public Health, University of Newcastle, Callaghan, NSW, 2308, Australia.|Schizophrenia Research Institute, 405 Liverpool St Darlinghurst, NSW, 2010, Australia.
U
U
Schall
D000293:Adolescent; D002648:Child; D004062:DiGeorge Syndrome / Q000503:physiopathology*; D004644:Emotions / Q000502:physiology*; D056344:Executive Function / Q000502:physiology*; D005260:Female; D006801:Humans; D008297:Male; D012938:Social Perception; D066107:Social Skills; D056345:Theory of Mind / Q000502:physiology*; D055815:Young Adult
emotion attribution/recognition; executive function; intellectual disability; social cognition; theory of mind; velo-cardio-facial syndrome