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. 2020 Feb 21;15(1):55.
doi: 10.1186/s13023-020-1333-9.

Equivocal expression of emotions in children with Prader-Willi syndrome: what are the consequences for emotional abilities and social adjustment?

Nawelle Famelart  1   2 , Gwenaelle Diene  3 , Sophie Çabal-Berthoumieu  3 , Mélanie Glattard  3 , Catherine Molinas  3 , Michèle Guidetti  4 , Maithe Tauber  3   5
Affiliations

Equivocal expression of emotions in children with Prader-Willi syndrome: what are the consequences for emotional abilities and social adjustment?

Nawelle Famelart et al. Orphanet J Rare Dis. .

Abstract

Background: People 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.

Method: To 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.

Results: The 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.

Conclusions: As 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.

Keywords: Children; Emotion expressions; Prader-Willi syndrome; Social adaptation.

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Conflict of interest statement

All the authors report no potential conflicts of interest with this work. The authors have no financial relationships relevant to this article to disclose.

Figures

Fig. 1
Fig. 1
Developmental model of emotion competencies
Fig. 2
Fig. 2
Mean proportion of AUs from each emotion pattern in the children’s facial reactions (EMOrea task). Legend. Upper part of the figure: target emotion; lower part: untargeted emotions
Fig. 3
Fig. 3
Mean proportion of AUs in the happiness condition (EMOmim task). Legend. Proportion of AUs from each emotion pattern in the children’s facial and bodily productions. Upper part of the figure: target emotion; lower part: untargeted emotions
Fig. 4
Fig. 4
Mean proportion of AUs in the anger condition (EMOmim task). Legend. Proportion of AUs from each emotion pattern in the children’s facial and bodily productions. Upper part of the figure: target emotion; lower part: untargeted emotion
Fig. 5
Fig. 5
Mean proportion of AUs in the sadness condition (EMOmim task). Legend. Proportion of AUs from each emotion pattern in the children’s facial and bodily productions. Upper part of the figure: target emotion; lower part: untargeted emotion
Fig. 6
Fig. 6
Mean proportion of AUs in the fear condition (EMOmim task). Legend. Proportion of AUs from each emotion pattern in the children’s facial and bodily productions. Upper part of the figure: target emotion; lower part: untargeted emotion
See this image and copyright information in PMC

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