Social Responsiveness and Psychosocial Functioning in Adults with Prader-Willi Syndrome

Although various studies have investigated symptoms of autism spectrum disorder (ASD) in Prader-Willi syndrome (PWS), little is known about the consequences of these symptoms, especially in psychosocial function. We aimed to explore ASD symptoms in adults with PWS with special attention to psychosocial functionality. This cross-sectional study included 26 adults (15 women) with PWS who attended a reference unit for rare diseases. Participants' primary caregivers completed the Social Responsiveness Scale (SRS), and clinicians assessed multidimensional functioning with the Personal and Social Performance Scale (PSP). Impaired social responsiveness was identified in 20 (76.9%) participants, and manifest to marked difficulties in social functioning were identified in 13 (50%). Participants with impaired social responsiveness (SRS >= 60) had significantly worse scores in functionality measured with the PSP (U = 12.5; p = 0.009) and with three of the four PSP main areas. Moreover, scores for the Social Cognition domain of the SRS correlated positively with the Socially useful activities (p < 0.05) and Personal and social relationships (p < 0.01) main areas of the PSP. These results suggest that difficulties in social skills should be assessed in all psychosocial evaluations of patients with PWS.

Automated summary

This study explored symptoms of autism spectrum disorder (ASD) in adults with Prader-Willi syndrome (PWS), focusing on their psychosocial functionality. The results showed that impaired social responsiveness was common in participants with PWS and had a significant impact on their overall functionality, particularly in social and personal relationships. The study highlights the importance of assessing social skills in psychosocial evaluations of PWS patients.