TY - JOUR
T1 - Social Responsiveness and Psychosocial Functioning in Adults with Prader-Willi Syndrome
AU - Fernández-Lafitte, Meritxell
AU - Cobo, Jesus
AU - Coronas, Ramon
AU - Parra Uribe, Isabel
AU - Oliva, Joan Carles
AU - Àlvarez, Aida
AU - Esteba-Castillo, Susanna
AU - Giménez-Palop, Olga
AU - Palao, Diego
AU - Caixàs i Pedragós, Assumpta
PY - 2022
Y1 - 2022
N2 - 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.
AB - 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.
KW - Prader-Willi syndrome
KW - Autism
KW - Autism spectrum disorders
KW - Social responsiveness
KW - Social function
KW - Functionality
KW - Function
U2 - 10.3390/jcm11051433
DO - 10.3390/jcm11051433
M3 - Article
C2 - 35268524
SN - 2077-0383
VL - 11
JO - Journal of Clinical Medicine
JF - Journal of Clinical Medicine
ER -