Psychology Department, Macquarie University, Sydney, New South Wales, Australia.
J Intellect Disabil Res. 2021 Aug;65(8):784-794. doi: 10.1111/jir.12860. Epub 2021 Jun 7.
There is very little research on the cognitive profile of young children with Williams syndrome (WS).
The present study utilised the Differential Ability Scales - Second Edition to examine the early cognitive abilities of 22 young children with WS (aged 3.98 to 7.70 years, 10 male and 12 female participants).
Overall, IQ ranged from 38 (severely impaired) to 81.00 (low average). Consistent with Mervis et al. who looked at an older sample, over half (59.08%) of our young WS sample showed a significant and abnormal weakness in spatial ability relative to verbal ability. Moreover, 81.82% showed a significant and clinically unusual weakness in spatial ability relative to nonverbal reasoning ability. At the subtest level, only 4.55% of our sample showed a significant strength in naming vocabulary compared with verbal comprehension, while 13.64% showed a significant weakness in naming vocabulary relative to verbal comprehension.
The results of the present study show cognitive heterogeneity, consistent with the literature on older children and adults with WS. There were variable levels of intellect and variable patterns of cognitive strength and weakness across both index and subtest scores. Findings highlight the need for individual assessment and management of young children with WS but also indicate that for the majority of WS individuals spatial skills are indeed an area of significant and abnormal weakness and should be a focus for early intervention.
目前针对威廉姆斯综合征（Williams syndrome，WS）患儿认知特征的研究较少。
本研究采用《韦氏儿童智力量表第四版》（Differential Ability Scales - Second Edition）评估 22 名年龄在 3.98 岁至 7.70 岁（男 10 名，女 12 名）的 WS 患儿的早期认知能力。
总体而言，智商范围从 38（严重受损）到 81.00（中下）。与 Mervis 等人研究的年龄较大的样本一致，超过一半（59.08%）的 WS 患儿在空间能力上存在显著而异常的弱于言语能力的情况。此外，81.82%的患儿在空间能力上显著弱于非言语推理能力，表现出明显的临床异常。在分测验水平上，只有 4.55%的患儿在命名词汇方面显著强于言语理解，而 13.64%的患儿在命名词汇方面显著弱于言语理解。
本研究结果显示认知存在异质性，与针对年长儿童和 WS 成人的文献一致。在指数和分测验得分方面，存在不同程度的智力水平和认知强弱模式。研究结果强调了对 WS 患儿进行个体化评估和管理的必要性，但也表明，对于大多数 WS 个体而言，空间技能确实是一个显著且异常薄弱的领域，应成为早期干预的重点。
