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《Williams 综合征的语言技能与社会交际能力比较》

A comparison between linguistic skills and socio-communicative abilities in Williams syndrome.

机构信息

Child Neuropsychiatry Unit, Neuroscience Department, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

出版信息

J Intellect Disabil Res. 2017 Sep;61(9):866-876. doi: 10.1111/jir.12401. Epub 2017 Jul 26.

Abstract

BACKGROUND

Individuals with Williams syndrome (WS) show a disharmonic linguistic profile with a clear pattern of strengths and weaknesses. Despite their sociable nature, atypical socio-communicative abilities and deficits in communication and relationship with others have been found.

AIM

The aim of the present study was to investigate whether linguistic skills (LS) were in line with the pragmatic and social use of language and the cognitive development of 32 individuals with WS (18 boys and 14 girls) with a mean chronological age of 12.3 (±4.4) years. To examine the relationship between language and mental age (MA) at different ages, the issue was investigated in children and adolescents/young adults with WS.

METHOD

Measures of LS, including lexical and morphosyntactic competences, and adaptive socio-communicative abilities (ASCA), pertaining to the use of language in daily living social context, were compared with the MA of participants. In a second step, participants with WS were split into two subgroups based on age, and the relationship between LS, ASCA and MA was studied.

RESULTS

Although expressive and receptive LS were generally found to be in line with or better than would be expected for MA, specific deficits in receptive ASCA were documented. LS and ASCA appeared to have a different evolution during the different time windows considered.

CONCLUSIONS

Our results underlined the importance of assessing linguistic abilities in the context of adaptive functioning, to guide educational and rehabilitative strategies for individuals with WS.

摘要

背景

威廉姆斯综合征(WS)患者的语言模式存在不平衡,表现出明显的优势和劣势。尽管他们性格外向,但在社会交往能力方面存在异常,并且在沟通和与他人的关系方面存在缺陷。

目的

本研究旨在调查 32 名 WS 患者(18 名男孩和 14 名女孩)的语言技能(LS)是否与语用和社会语言使用以及认知发展相匹配,这些患者的平均年龄为 12.3(±4.4)岁。为了研究语言与不同年龄的心理年龄(MA)之间的关系,我们对 WS 儿童和青少年/年轻成人进行了研究。

方法

使用 LS 评估工具,包括词汇和形态句法能力,以及适应社会交际能力(ASCA),评估日常生活中的语言使用和社会交际能力,与参与者的 MA 进行比较。在第二步中,根据年龄将 WS 患者分为两个亚组,并研究 LS、ASCA 和 MA 之间的关系。

结果

尽管表达性和接受性 LS 通常与 MA 相符或优于 MA,但在接受性 ASCA 方面存在特定缺陷。LS 和 ASCA 在不同的时间段内似乎有不同的发展。

结论

我们的研究结果强调了在适应功能背景下评估语言能力的重要性,以便为 WS 患者制定教育和康复策略。

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