Social-Affective Bases of Word Learning in Fragile X Syndrome and Autism

脆性 X 综合征和自闭症词汇学习的社会情感基础

• 批准号: 7780329
• 负责人: LEONARD J. ABBEDUTO
• 金额: $ 55.64万
• 依托单位: UNIVERSITY OF WISCONSIN-MADISON
• 依托单位国家: 美国
• 财政年份: 2008
• 资助国家: 美国
• 起止时间: 2008-03-08 至 2013-02-28
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7780329
• 关键词: Accounting; Address; Affective; Anxiety; Area; Autistic Disorder; Behavior; Behavioral; Biological; Characteristics; Child; Cognition; Cognitive; Communication; Comorbidity; Cues; Data; Development; Diagnostic; Dimensions; Disease; Environment; Eye; FMR1; FMR1 Gene; FMRP; Female; Fragile X Syndrome; Gene Silencing; Genes; Impairment; Individual; Individual Differences; Inherited; Institutes; Intervention; Language; Language Delays; Language Development; Learning; Linear Regressions; Linguistics; Literature; Masks; Matched Group; Measures; Mediating; Mental Retardation; Methylation; Mind; Modeling; Mosaicism; Mutation; Nature; Neonatal Screening; Neurocognitive; Outcome; Performance; Phenotype; Probability; Process; Process Measure; Psychopathology; Relative (related person); Research; Role; Severities; Subgroup; Symptoms; Syndrome; Testing; Time; Variant; Vocabulary; Wisconsin; X Chromosome; base; boys; comparison group; dyadic interaction; emotional reaction; experience; gaze; insight; interest; joint attention; lexical; male; meetings; mental age; novel; programs; psychologic; social; social reciprocity; success

DESCRIPTION (provided by applicant): Although fragile X syndrome (FXS) is a single-gene (i.e., FMR1) disorder associated with a characteristic behavioral phenotype, there is considerable within-syndrome variability in the severity of affectedness and the profile of neurocognitive impairments and co-morbid conditions displayed. About 25-30% of people with FXS also meet diagnostic criteria for autism (AUT), with the remainder displaying autistic-like behavior below the diagnostic threshold. In this project, we focus on understanding within-syndrome variability in language development in relation to AUT status among boys with FXS. We propose to use a well-established paradigm (i.e., novel word learning) to study the ways in which children with FXS (with and without AUT) use social- affective cues to learn new words (Specific Aim 1); how such learning processes impact later language outcomes (Specific Aim 2); and how differences in AUT status and symptoms and anxiety, as well as in the environment and the FMR1 gene, contribute to within-syndrome variation in these processes (Specific Aim 3). Four groups of boys will participate, with the groups matched on nonverbal mental age: FXS only; co-morbid FXS and AUT; idiopathic AUT; and typically developing. At Time 1, each boy will participate in three word- learning studies, each focused on the use of a different type of social-affective cue. Assessments of AUT symptoms, anxiety, cognition, and language will also be conducted. Maternal synchrony, a dyadic interaction strategy known to support language development, and FMRP levels will also be measured. 1.5 years later, each boy's language will again be assessed. ANOVA, multiple linear regression, and path analysis will be used to compare the performance of the groups in the word learning tasks, to examine the relationship between Time 1 word learning and later language, and to examine the concurrent relationships among word learning, autism symptom domains, anxiety, maternal synchrony, and FMR1 variation. The project will be conducted at the Waisman Center (Wisconsin) and the M.I.N.D. Institute (CA). The project is unique in its inclusion of these three atypical groups and its focus on the processes underlying language problems.

描述（由申请人提供）：尽管脆性X综合征（FXS）是一种单基因（即，FMR 1）障碍与特征性行为表型相关，在受影响的严重程度和所显示的神经认知障碍和共病状况方面存在相当大的综合征内变异性。大约25-30%的FXS患者也符合自闭症（AUT）的诊断标准，其余的人表现出低于诊断阈值的自闭症样行为。在这个项目中，我们专注于理解与FXS男孩AUT状态相关的语言发展中的综合征内变异性。我们建议使用一个完善的范例（即，新单词学习）来研究FXS儿童（有和没有AUT）使用社会情感线索来学习新单词（具体目标1）;这些学习过程如何影响后来的语言结果（具体目标2）;以及AUT状态和症状和焦虑的差异，以及环境和FMR 1基因的差异如何导致这些过程中的综合征内变异（具体目标3）。四组男孩将参与，各组在非语言心理年龄上匹配：仅FXS;共病FXS和AUT;特发性AUT;和典型发展。在时间1，每个男孩将参加三个单词学习的研究，每一个集中在使用不同类型的社会情感线索。还将对AUT症状、焦虑、认知和语言进行评估。母亲的同步性，一个二元的互动策略，支持语言的发展，和FMRP水平也将被测量。1.5几年后，每个男孩的语言将再次进行评估。将使用ANOVA、多元线性回归和路径分析来比较各组在单词学习任务中的表现，以检查时间1单词学习与以后语言之间的关系，并检查单词学习、自闭症症状域、焦虑、母体同步性和FMR 1变异之间的并发关系。该项目将在韦斯曼中心（威斯康星州）和麻省理工学院进行。研究所（CA）。该项目的独特之处在于它包含了这三个非典型群体，并专注于语言问题的潜在过程。