Department of Psychology and Human Development, UCL Institute of Education, UK.
Department of Psychology, Ulster University, Coleraine, UK.
Res Dev Disabil. 2020 May;100:103609. doi: 10.1016/j.ridd.2020.103609. Epub 2020 Feb 19.
The current systematic review is the first to systematically explore and synthesis research to date on mathematical abilities in Williams syndrome (WS), a rare genetic disorder that results in an uneven cognitive profile. As mathematical development is complex and relies on both domain-specific and domain-general abilities, it is currently not clear what mathematical abilities have been examined in WS and also what the current gaps in this research area are.
A total of 27 studies across 22 publications were identified through a systematic review search process.
Overall, all mathematical abilities, except for simple counting and subitizing abilities, were reported to be impaired but in line with overall mental-age abilities. However, the literature to date has not established the underlying causes of these mathematical difficulties in WS. Some studies suggested that mathematical abilities in WS follow an atypical developmental pathway with a greater reliance on verbal abilities than in typical development but coupled with impaired understanding of counting and knowledge of the number system more broadly. However, most included studies used different assessments of mathematical skills and there is a lack of studies that have examined more than one particular aspect of mathematical development within the same study. In addition, studies have often included large age ranges and small participant samples, despite the known large individual variability in WS.
Although we know mathematical abilities in WS are impaired, this area is under-researched and there is a lack of longitudinal studies that provide insight into the cognitive mechanisms that underpin mathematical development in WS. Therefore, there is a lack of an evidence-base to inform interventions or educational practice.
本系统评价是首次对威廉姆斯综合征（WS）的数学能力研究进行系统探索和综合，WS 是一种罕见的遗传性疾病，导致认知能力不均衡。由于数学发展复杂，依赖于特定领域和非特定领域的能力，目前尚不清楚 WS 中已经检查了哪些数学能力，以及该研究领域目前存在哪些差距。
通过系统评价搜索过程共确定了 27 项来自 22 项出版物的研究。
总体而言，除了简单的计数和数量估计能力外，所有的数学能力都被报告为受损，但与整体智力年龄能力相符。然而，迄今为止的文献尚未确定 WS 中这些数学困难的根本原因。一些研究表明，WS 中的数学能力遵循一种非典型的发展途径，与典型发展相比，对语言能力的依赖更大，但同时对计数的理解和对数字系统的知识更广泛地受损。然而，大多数纳入的研究使用了不同的数学技能评估方法，而且缺乏在同一研究中检查数学发展的一个以上特定方面的研究。此外，尽管 WS 中个体间的差异很大，但研究通常包括较大的年龄范围和较小的参与者样本。
尽管我们知道 WS 中的数学能力受损，但该领域的研究不足，缺乏提供对 WS 中数学发展的认知机制见解的纵向研究。因此，缺乏干预或教育实践的证据基础。
