Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Birmingham, UK.
School of Psychology, College of Health and Life Sciences, Aston University, Birmingham, UK.
J Intellect Disabil Res. 2021 Jun;65(6):601-607. doi: 10.1111/jir.12829. Epub 2021 Mar 10.
Cornelia de Lange syndrsome (CdLS) is a rare genetic syndrome with notable impaired expressive communication characterised by reduced spoken language. We examined gesture use to refine the description of expressive communication impairments in CdLS.
During conversations, we compared gesture use in people with CdLS to peers with Down syndrome (DS) matched for receptive language and adaptive ability, and typically developing (TD) individuals of similar chronological age.
As anticipated the DS and CdLS groups used fewer words during conversation than TD peers (P < .001). However, the CdLS group used twice the number of gestures per 100 words compared with the DS and TD groups (P = .003).
Individuals with CdLS have a significantly higher gesture rate than expected given their level of intellectual disability and chronological age. This result indicates the cause of reduced use of spoken language does not extend to all forms of expressive communication.
Cornelia de Lange 综合征（CdLS）是一种罕见的遗传性综合征，其显著特征是表达性沟通受损，表现为口语语言能力降低。我们研究了手势的使用情况，以更准确地描述 CdLS 患者的表达性沟通障碍。
在对话过程中，我们比较了 CdLS 患者与接受语言和适应能力相匹配的唐氏综合征（DS）患者以及具有相似年龄的典型发育（TD）个体的手势使用情况。
正如预期的那样，DS 和 CdLS 组在对话中使用的单词比 TD 同龄人少（P<0.001）。然而，与 DS 和 TD 组相比，CdLS 组每 100 个单词使用的手势数量是其两倍（P=0.003）。
考虑到 CdLS 患者的智力残疾和年龄，他们的手势使用率明显高于预期。这一结果表明，口语语言使用减少的原因并非扩展到所有形式的表达性沟通。
