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探索 Sotos 综合征中的近似数量系统:来自点比较任务的启示。

Exploring the approximate number system in Sotos syndrome: insights from a dot comparison task.

机构信息

Department of Psychology, The University of Sheffield, Sheffield, UK.

Department of Psychology and Human Development, UCL Institute of Education, London, UK.

出版信息

J Intellect Disabil Res. 2019 Aug;63(8):917-925. doi: 10.1111/jir.12604. Epub 2019 Feb 7.

DOI:10.1111/jir.12604
PMID:30734385
Abstract

BACKGROUND

Sotos syndrome is a congenital overgrowth condition associated with intellectual disability and an uneven cognitive profile. Previous research has established that individuals with Sotos syndrome have relatively poor mathematical ability, but domain-specific numeracy skills have not been explored within this population. This study investigated the approximate number system (ANS) in Sotos syndrome.

METHOD

A dot comparison task was administered to 20 participants with Sotos syndrome (mean age in years = 18.43, SD = 9.29). Performance was compared to a chronological agematched typically developing control group (n = 25) and a mental age-matched Williams syndrome group (n = 24).

RESULTS

The Sotos group did not display an ANS deficit overall when compared to chronological agematched control participants. However, for trials where the size of the individual dots and the envelope area were negatively correlated with the total number of dots (incongruent trials), the Sotos group were less accurate than the typically developing group but more accurate than the Williams syndrome group, suggesting an inhibitory control deficit. Better accuracy on incongruent trials, but not congruent trials, was associated with higher quantitative reasoning ability for participants with Sotos syndrome.

CONCLUSION

Overall, the findings suggest that ANS acuity is not impaired in Sotos syndrome but that numerical difficulties may be associated with an inhibitory control deficit for individuals with Sotos syndrome.

摘要

背景

Sotos 综合征是一种与智力障碍和认知不平衡相关的先天性过度生长疾病。先前的研究已经证实,Sotos 综合征患者的数学能力相对较差,但在该人群中尚未探索特定于领域的计算技能。本研究调查了 Sotos 综合征的近似数量系统 (ANS)。

方法

对 20 名 Sotos 综合征患者(平均年龄为 18.43 岁,标准差为 9.29 岁)进行了点比较任务。将表现与年龄匹配的典型发育对照组(n=25)和精神年龄匹配的威廉姆斯综合征组(n=24)进行比较。

结果

与年龄匹配的对照组相比,Sotos 组在整体上并未显示出 ANS 缺陷。然而,对于个别点的大小和信封面积与总点数呈负相关的试验(不一致试验),Sotos 组的准确性低于典型发育组,但高于威廉姆斯综合征组,这表明存在抑制控制缺陷。对于 Sotos 综合征患者,在不一致试验上的准确性更好,而不是在一致试验上的准确性,与更高的定量推理能力相关。

结论

总体而言,这些发现表明 Sotos 综合征中的 ANS 敏锐度没有受损,但数值困难可能与 Sotos 综合征患者的抑制控制缺陷有关。

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引用本文的文献

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A Further Characterisation of the Neuropsychological Profile, Social Perception, and Academic Skills in Sotos Syndrome.Sotos综合征的神经心理学特征、社会认知及学术技能的进一步表征
J Intellect Disabil Res. 2025 Aug;69(8):664-674. doi: 10.1111/jir.13250. Epub 2025 May 15.
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Online and Face-to-Face Performance on Two Cognitive Tasks in Children With Williams Syndrome.威廉姆斯综合征患儿在两项认知任务中的线上与面对面表现
Front Psychol. 2021 Feb 4;11:594465. doi: 10.3389/fpsyg.2020.594465. eCollection 2020.