School of Psychology, University of Birmingham, Edgbaston, Birmingham, B15 2TT, UK.
Griffith University, PO Box 3370, South Brisbane Qld 4101, Sidon Street, South Bank, QLD, Australia.
J Autism Dev Disord. 2018 Jan;48(1):326-331. doi: 10.1007/s10803-017-3319-6.
This study describes the profile of repetitive behaviour in individuals with Williams syndrome, utilising cross-syndrome comparisons with people with Prader-Willi and Down syndromes. The Repetitive Behaviour Questionnaire was administered to caregivers of adults with Williams (n = 96), Prader-Willi (n = 103) and Down (n = 78) syndromes. There were few group differences, although participants with Williams syndrome were more likely to show body stereotypies. Individuals with Williams syndrome also showed more hoarding and less tidying behaviours than those with Down syndrome. IQ and adaptive ability were negatively associated with repetitive questioning in people with Williams syndrome. The profile of repetitive behaviour amongst individuals with Williams syndrome was similar to the comparison syndromes. The cognitive mechanisms underlying these behaviours in genetic syndromes warrant further investigation.
本研究利用与普拉德-威利综合征和唐氏综合征患者的跨综合征比较，描述了威廉姆斯综合征个体重复行为的特征。使用重复行为问卷对威廉姆斯（n = 96）、普拉德-威利（n = 103）和唐氏（n = 78）综合征患者的照顾者进行了评估。尽管威廉姆斯综合征患者更有可能出现身体刻板行为，但各小组之间几乎没有差异。与唐氏综合征患者相比，威廉姆斯综合征患者也表现出更多的囤积行为和更少的整理行为。在威廉姆斯综合征患者中，智商和适应能力与重复提问呈负相关。威廉姆斯综合征个体的重复行为特征与对照综合征相似。遗传综合征中这些行为的认知机制值得进一步研究。
