Paying attention to communicative faces is essential for our understanding of the social world. Indeed, faces provide observers rich and complex information about the identity (gender, age, etc), the socio-emotional state (eye-brows movements, eye-gaze) and the linguistic message (auditory speech sounds/mouth movements) of our social partners. The aim of this research project is to understand how infants with Prader-Willi Syndrome perceive communicative faces from their first months of life. We hypothesize that the general lack of alertness observed at birth might prevent infants with PWS to benefit from communicative faces as much as normal-developing babies. Later on in their life, these early impairments coud contribute to their immature social and emotional behaviors. This project will allow us to characterize these abilities and then, more importantly, to develop adapted rehabilitation methods, intervening since the first month of life, to stem the development of further social and cognitive impairments later on. Crucially, this study will be the first building block to develop new therapeutic approaches for the treatment of socio-cognitive symptoms associated with PWS. This research could thus directly impact the social life of individuals with PWS.
Research Outcomes: Public Summary
The present study referring to the facial exploration yielded fresh insights into face processing in PWS, showing that patients have impaired face and emotion recognition a deficit that is partly related to atypical eye/face exploration. If they shift their gaze to the mouth region, patients with PWS potentially encounter difficulty understanding and deciphering social cues or extracting all the information needed for appropriate everyday social exchanges. It also revealed a difference between the two main genetic subtypes, suggesting that patients with UPD behave like the ASD population (gaze predominantly on the mouth region of a face). It is therefore crucial to distinguish between the two subtypes as early as possible, so that rehabilitation has the maximum impact on social communication abilities. This, of course, presupposes early determination of the genetic subtype as part of routine diagnosis. Moreover, dynamic presentation allowed us to concluded that in PWS, as in ASD, increasing the social content leads to social impairments. In our clinical practice, we find that patients with PWS communicate better when there are only a few people present. When there are more than two people, they adopt an ASD-like behavior, avoiding eye contact.
Research Outcomes: Publications
A study of voice and non-voice processing in Prader-Willi syndrome. Strenilkov, K., Debladis, J., Salles, J., Valette, M., Mantoulan, C., Thuilleaux, D., Laurier, V., Molinas, C., Barone, P. & Tauber, M. Orphanet J Rare Dis 15, 22 (2020).
Face processing and exploration of social signals in Prader-Willi syndrome: a genetic signature. Debladis, J., Valette, M., Strenilkov, K. Mantoulan, C., Thuilleaux, D., Laurier, V., Molinas, C., Barone, P., Tauber, M. Orphanet J Rare Dis 14, 262 (2019) doi:10.1186/s13023-019-1221-3.
Pascal Barone, PhD
University of Toulouse, France