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Equivocal expression of emotions in children with Prader-Willi syndrome: what are the consequences for emotional abilities and social adjustment?
Orphanet Journal of Rare Diseases ( IF 3.7 ) Pub Date : 2020-02-21 , DOI: 10.1186/s13023-020-1333-9
Nawelle Famelart 1, 2 , Gwenaelle Diene 3 , Sophie Çabal-Berthoumieu 3 , Mélanie Glattard 3 , Catherine Molinas 3 , Michèle Guidetti 1 , Maithe Tauber 3, 4
Affiliation  

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.

中文翻译:

Prader-Willi综合征患儿情绪的模棱两可表达:对情绪能力和社会适应的影响是什么?

背景技术患有普拉德-威利综合症(PWS)的人在社交适应中遇到很大的困难,这可以通过情感能力的紊乱来解释。但是,有关PWS患者情绪功能的最新知识尚不完善。尤其是,尽管它们是社会适应的基础,但他们的情感表达能力从未得到过研究。此外,运动和认知障碍-PWS的特征-可能进一步削弱这些能力。方法为了探讨PWS儿童的表达能力,对25名5至10岁的PWS儿童进行了以下评估:1)他们对有趣的视频剪辑的面部表情反应; 2)他们按需产生面部和身体的能力。表达喜悦,愤怒,恐惧和悲伤。将他们的生产与两组具有典型发育状况的儿童的生产进行了比较,按年龄和发育年龄与PWS儿童相匹配。分析着重于儿童作品中与目标情感和非目标情感相关的表达模式的比例。结果结果表明,PWS患儿的面部和身体情感表达特别难以解释,涉及不同情感模式的明显混合。此外,据观察,PWS儿童要求产生的情绪特别差且模棱两可。结论据我们所知,这项研究是第一个强调PWS儿童情绪表达中存在特殊性的研究。
更新日期:2020-04-22
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