Department of Psychological Sciences, Purdue University, 703 Third Street, West Lafayette, IN, 47907, USA.
J Autism Dev Disord. 2019 Apr;49(4):1717-1726. doi: 10.1007/s10803-018-3853-x.
Children with neurogenetic syndromes (NGS) experience comorbid challenging behaviors and psychopathology. We examined challenging behaviors in 86 toddlers and preschoolers across three NGS [Angelman syndrome (AS), Prader-Willi syndrome (PWS), and Williams syndrome (WS)] and 43 low-risk controls (LRC), using the Child Behavior Checklist for Ages 1½-5. Challenging behavior profiles differed across NGS, with generally elevated behaviors in AS and WS, but not PWS, relative to LRC. Withdrawn and autism spectrum symptoms were particularly elevated in AS. Although several profiles were similar to those previously reported in older children and adults, we also observed inconsistencies that suggest non-linear developmental patterns of challenging behaviors. These findings underscore the importance of characterizing early challenging behaviors to inform atypical phenotypic development and targeted intervention.
患有神经遗传综合征 (NGS) 的儿童伴有共病的挑战性行为和精神病理学问题。我们使用儿童行为检查表(1.5-5 岁),对三个 NGS(天使综合征 (AS)、普拉德-威利综合征 (PWS) 和威廉姆斯综合征 (WS))的 86 名幼儿和学龄前儿童以及 43 名低风险对照组 (LRC) 的挑战性行为进行了检查。NGS 之间的挑战性行为特征不同,AS 和 WS 组的行为普遍升高,而 PWS 组则没有。与 LRC 相比,AS 组的退缩和自闭症谱系症状特别升高。虽然有几个特征与以前在年龄较大的儿童和成人中报告的特征相似,但我们也观察到不一致的情况,表明挑战性行为的非线性格局。这些发现强调了描述早期挑战性行为的重要性,以告知非典型表型发育和针对性干预。