Grammatical Development in Boys with Fragile X Syndrome and Autism

患有脆性 X 综合征和自闭症的男孩的语法发展

• 批准号: 8443403
• 负责人: Audra Marie Sterling
• 金额: $ 14.11万
• 依托单位: UNIVERSITY OF WISCONSIN-MADISON
• 依托单位国家: 美国
• 财政年份: 2011
• 资助国家: 美国
• 起止时间: 2011-04-22 至 2015-09-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8443403
• 关键词: Accounting; Adult; Age; Autistic Disorder; Behavior; Canis familiaris; Child; Clinical assessments; Cognition; Cognitive; Communication; Communication impairment; Control Groups; DSM-IV; Data; Development; Developmental Disabilities; Diagnosis; Diagnostic; Etiology; Fragile X Syndrome; Future; Hearing; Impairment; Individual; Inherited; Intellectual functioning disability; Intervention; Investigation; Knowledge; Language; Language Delays; Language Development; Language Disorders; Linguistics; Measures; Methods; Mission; National Institute on Deafness and Other Communication Disorders; Nature; Participant; Pattern; Persons; Phenotype; Population; Procedures; Public Health; Reporting; Research; Rice; Sampling; Speech; Staging; Structure; Symptoms; System; Testing; United States National Institutes of Health; Walking; Work; base; boys; clinical practice; design; improved; insight; interest; male; meetings; neurodevelopment; phonology; programs; public health relevance; relating to nervous system; specific language impairment; theories

DESCRIPTION (provided by applicant): Fragile X syndrome is the leading inherited cause of intellectual disability, with the majority of males (95%) having IQs below 70. Most males with fragile X display autistic-like behaviors, and 25-30% meet the DSM-IV criteria for an autism co-diagnosis. Males with comorbid fragile X and autism are typically reported to have more severe overall impairments in cognition and receptive/expressive language. However, the language phenotype within fragile X only and comorbid fragile X and autism is extremely variable across individuals. Moreover, the impact of autism on the language phenotype of fragile X is not clear. The purpose of the proposed project is to determine if there is a grammatical deficit in FXS and in autism, while examining the consequences of comorbid autism on language in fragile X. Research on language development in developmental disabilities to date has been descriptive in nature, as opposed to theory-driven. There is however, a rich theory-driven line of research on grammatical development in children with language impairments. The extended optional infinitive account (EOI) posits that children with specific language impairment seem to get "stuck" in an optional infinitive stage in which they treat the use of certain grammatical morphemes as optional, despite their obligatory status in the adult grammar. Preliminary evidence suggests that children with fragile X and children with autism may demonstrate a deficit in at least one aspect of grammatical development (i.e., finiteness marking; past tense: he walked) independent of nonverbal IQ. This project will advance preliminary work by examining all finiteness markers (BE/DO), as well as non-finiteness grammatical morphemes (i.e., plural -s, possessive -s, present progressive -ing). This investigation will inform whether boys with fragile X show a grammatical profile similar to SLI, or if their language is characterized by global difficulty with all grammatical morphemes. In addition, the impact of co-morbid autism on grammatical development will be examined by comparing boys with fragile X with and without autism and boys with idiopathic autism. Additionally, the proposed study will examine the most effective method of assessing grammar in boys with FXS and boys with autism. Sixty-three boys between the ages of 9-16 years will participate in this study: boys with FXS only, boys with FXS and autism, and boys with idiopathic autism. Participants will complete standardized tests, a language sample, autism diagnostic measures, and a sentence imitation task. These results will inform the phenotypes of fragile X and autism and provide insights into the nature of variability in language impairments across different etiological conditions. The information from the proposed studies will inform (1) hypotheses regarding neural constraints on language development as well as (2) clinical assessment and intervention procedures. This study lays the groundwork for future studies needed to assess additional aspects of the linguistic system.

描述（由申请人提供）：脆弱的X综合征是智障的主要遗传原因，大多数男性（95％）的智商低于70。大多数具有脆弱X的男性表现出自闭症的行为，而25-30％符合自闭症诊断的DSM-IV标准。据报道，患有合并症的X和自闭症的男性在认知和接受/表达语言方面具有更严重的总体障碍。但是，仅易碎X中的语言表型和合并症易碎的X和自闭症在各个个体之间都是极大的。此外，自闭症对脆弱X语言表型的影响尚不清楚。拟议项目的目的是确定FXS和自闭症中是否存在语法缺陷，同时研究了合并症自闭症对脆弱X语言的后果。迄今为止，对发展障碍的语言发展的研究本质上是描述性的，与理论驱动相对应。但是，有关于语言障碍儿童的语法发展的丰富理论驱动研究线。扩展的可选无限账户（EOI）认为，具有特定语言障碍的儿童似乎在可选的不定阶段被“卡住”，尽管他们在成人语法中具有强制性的状态，但他们将使用某些语法形式视为可选的。初步证据表明，患有X和自闭症儿童的儿童可能在语法发展的至少一个方面（即有限标记；过去时：他走路）与非语言智商无关。该项目将通过检查所有有限标记（BE/do）以及非专业语法语言（即复数-s，所有格，所有占有-s，当前的渐进-ING）来推进初步工作。这项调查将告知患有易碎X的男孩是否表现出类似于SLI的语法特征，还是他们的语言具有所有语法词素的全球困难。此外，将通过比较患有和没有自闭症的男孩以及患有特发性自闭症的男孩的男孩来检查共临界自闭症对语法发展的影响。此外，拟议的研究将研究FXS和自闭症男孩的男孩评估语法的最有效方法。 9-16岁之间的63个男孩将参加这项研究：仅有FXS的男孩，有FXS和自闭症的男孩以及患有特发性自闭症的男孩。参与者将完成标准化的测试，语言样本，自闭症诊断措施和句子模仿任务。这些结果将为脆弱的X和自闭症的表型提供信息，并提供有关不同病因条件语言障碍的可变性性质的见解。拟议研究的信息将告知（1）关于语言发展的神经限制以及（2）临床评估和干预程序的假设。这项研究为未来的研究奠定了基础，以评估语言系统的其他方面。