Remediating functional activation in the face perception network in autism and 22q11.2 deletion syndrome

Introduction: Vis-à-Vis (VAV), an online enrichment program targeting skills that underlie social abilities (looking at the eye region of faces, emotion comprehension & recognition and visuo-spatial working memory) was administered to children and adolescents with autism spectrum disorder (ASD), 22q11.2 deletion syndrome (22q11DS)) and developmental delay (DD).  Differential functional activation throughout the brain and in regions related to face processing were measured before and after remediation. Methods: Participants with ASD (N=16), 22q11DS (N=12), and DD (N=9) completed a functional localizer task consisting of pictures of faces and tools before undertaking the 12-week VAV program (PreR). The same fMRI task was administered immediately after VAV, at post-remediation (PostR), and again three months later (Break). A group of healthy controls (N=10) was scanned, but not given VAV, to provide a baseline comparison for patients’ results. Functional neuroimaging data was analysed for category-specific brain activity. Results: The ASD and the 22q11DS groups both showed atypical activation to faces at PreR compared to controls. Whereas activation in 22q11DS at PostR and Break resembled that of controls in bilateral superior temporal gyrus, insula, bilateral precentral gyrus, right lingual gyrus and right middle frontal cortex. Unlike the 22q11DS group, the ASD group’s activation did not resemble that of controls at PostR or Break. Significant clusters in the ASD group were limited to left insula and right lingual gyrus at PostR, and right superior frontal gyrus at Break. Nevertheless, a significant relationship between increased BOLD response between PreR and PostR in the right fusiform gyrus and improvement on the VAV games teaching participants to focus on the eyes was observed in both groups. This relationship was not present in the DD group. Conclusions: These results support hypo-activation in response to faces in ASD and 22q11DS and differential cerebral activation in face processing networks following cognitive remediation.