Abstract
BackgroundPeople with Prader-Willi Syndrome (PWS) experience great difficulties in social adaptation that could be explained by disturbances in emotional competencies. However, current knowledge about the emotional functioning of people with PWS is incomplete. In particular, despite being the foundation of social adaptation, their emotional expression abilities have never been investigated. In addition, motor and cognitive difficulties - characteristic of PWS - could further impair these abilities.MethodTo explore the expression abilities of children with PWS, twenty-five children with PWS aged 5 to 10 years were assessed for 1) their emotional facial reactions to a funny video-clip and 2) their ability to produce on demand the facial and bodily expressions of joy, anger, fear and sadness. Their productions were compared to those of two groups of children with typical development, matched to PWS children by chronological age and by developmental age. The analyses focused on the proportion of expressive patterns relating to the target emotion and to untargeted emotions in the children’s productions.ResultsThe results showed that the facial and bodily emotional expressions of children with PWS were particularly difficult to interpret, involving a pronounced mixture of different emotional patterns. In addition, it was observed that the emotions produced on demand by PWS children were particularly poor and equivocal.ConclusionsAs far as we know, this study is the first to highlight the existence of particularities in the expression of emotions in PWS children. These results shed new light on emotional dysfunction in PWS and consequently on the adaptive abilities of those affected in daily life.
Highlights
The expression of facial or bodily emotions is an ability that plays a major role in the establishment of interpersonal relationships and in social adaptation
As far as we know, this study is the first to highlight the existence of particularities in the expression of emotions in Prader-Willi Syndrome (PWS) children
In view of the multiple disorders related to this syndrome, we suggest that children suffering from PWS exhibit particularities in the expression of their emotions that contribute to their difficulties in emotional recognition, comprehension, regulation skills and social adjustment
Summary
The expression of facial or bodily emotions is an ability that plays a major role in the establishment of interpersonal relationships and in social adaptation. Prader-Willi syndrome (PWS) is a rare genetic disease (birth incidence rate of 1: 20,000 to 1: 25,000) related to the loss of expression of some paternal inherited genes on chromosome 15 region q11–13. This leads to significant dysfunction of the hypothalamic-pituitary hormonal and neurohormonal system. People with Prader-Willi Syndrome (PWS) experience great difficulties in social adaptation that could be explained by disturbances in emotional competencies. Despite being the foundation of social adaptation, their emotional expression abilities have never been investigated. Motor and cognitive difficulties characteristic of PWS - could further impair these abilities
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