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脆性X综合征和威廉姆斯综合征成人的实用特征

Pragmatic Profiles of Adults with Fragile X Syndrome and Williams Syndrome.

作者信息

Diez-Itza Eliseo, Viejo Aitana, Fernández-Urquiza Maite

机构信息

LOGIN Research Group, University of Oviedo, 33003 Oviedo, Spain.

出版信息

Brain Sci. 2022 Mar 13;12(3):385. doi: 10.3390/brainsci12030385.

DOI:10.3390/brainsci12030385
PMID:35326341
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8946534/
Abstract

Linguistic phenotypes of individuals with Fragile X (FXS) and Williams (WS) syndromes exhibit various degrees of pragmatic impairment, involving difficulties in social communication and in adapting to conversational principles. The goal of the present study was to explore syndrome-specific pragmatic profiles of adults with FXS and WS based on the assessment of the observance of Gricean maxims of conversation. The participants were 12 Spanish-speaking adults (6 FXS/6 WS), without a diagnosis of ASD, whose extensive naturalistic conversations (71,859 words) were transcribed and coded with the CHILDES/TALKBANK tools and the PREP-CORP pragmatic protocol. Violations of the maxims of conversation were analyzed, and indexes of cooperation and conversational response were obtained. Both groups showed reduced verbal production and repetitive dysfluencies; prominent features in the FXS profile were higher proportion of non-contingent language, perseverations of topic and form, and impulsive conversational responses; in the WS profile, salient characteristics were higher proportion of tangential utterances, reformulations, and conversational responses reflecting overly literal interpretation. Pragmatic profiles of violation of conversational maxims reflect specific communication skills impaired in adults with FXS and WS and raise the need for assessment and intervention methods that specifically address their social communication abilities.

摘要

脆性X综合征(FXS)和威廉姆斯综合征(WS)患者的语言表型表现出不同程度的语用障碍,包括社交沟通困难和难以适应会话原则。本研究的目的是基于对格赖斯会话准则遵守情况的评估,探索患有FXS和WS的成年人特定综合征的语用特征。参与者为12名讲西班牙语的成年人(6名FXS患者/6名WS患者),未被诊断为自闭症谱系障碍(ASD),他们的大量自然对话(71,859个单词)使用CHILDES/TALKBANK工具和PREP-CORP语用协议进行转录和编码。分析了违反会话准则的情况,并获得了合作和会话反应指标。两组均表现出言语产出减少和重复性言语不流畅;FXS特征中的突出特点是非偶然性语言比例较高、话题和形式的持续、以及冲动性会话反应;在WS特征中,显著特征是离题话语、重新表述以及反映过度字面解释的会话反应比例较高。违反会话准则的语用特征反映了患有FXS和WS的成年人受损的特定沟通技能,并提出了对专门针对其社交沟通能力的评估和干预方法的需求。

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