Department of Psychological Sciences, Case Western Reserve University, 11220 Bellflower Road, Mather Memorial Building, Cleveland, OH, 44106, USA.
J Autism Dev Disord. 2019 Nov;49(11):4441-4454. doi: 10.1007/s10803-019-04152-4.
Children with Prader-Willi syndrome (PWS) and autism spectrum disorder (ASD) present with challenges in social cognitive ability, Research comparing PWS to ASD is important given the implication of 15q11-q13 region in the biology of autism. However, recent findings question the accuracy of relying solely on parent report in behavioral characterization. Thus, this study examined social cognition in an observable pretend play task and by parent report in 50 preschool children (ages 3-5) with PWS, by subtype, compared to ASD. Behaviorally, the paternal deletion subtype expressed overall higher functioning, whereas the maternal uniparental disomy subtype performed more similarly to the ASD group. Results are the first to show deficits in social cognitive ability early in development. The severity and differences in deficits between PWS subtypes are important in informing early intervention efforts.
患有普拉德-威利综合征（PWS）和自闭症谱系障碍（ASD）的儿童在社会认知能力方面存在挑战。鉴于 15q11-q13 区域在自闭症生物学中的作用，比较 PWS 与 ASD 的研究非常重要。然而，最近的发现质疑仅依靠父母报告进行行为特征描述的准确性。因此，本研究在可观察的假装游戏任务中以及通过父母报告，在 50 名患有 PWS 的学龄前儿童（3-5 岁）中，按亚型与 ASD 进行了比较，以检查社会认知能力。行为上，父系缺失亚型表现出更高的整体功能，而母系单亲二体性亚型与 ASD 组的表现更为相似。研究结果首次表明，在早期发育阶段，社会认知能力存在缺陷。PWS 亚型之间缺陷的严重程度和差异对于告知早期干预工作非常重要。
