Department of Psychology, University of Wisconsin-Madison, 1202 W. Johnson Street, Madison, WI, 53706, USA,
J Neurodev Disord. 2011 Dec;3(4):335-47. doi: 10.1007/s11689-011-9095-2. Epub 2011 Oct 13.
Fragile X syndrome (FXS) is the most common inherited cause of intellectual disability. Although language delays are frequently observed in FXS, neither the longitudinal course of language development nor its cognitive predictors are well understood. The present study investigated whether phonological and working memory skills are predictive of growth in vocabulary and syntax in individuals with FXS during adolescence. Forty-four individuals with FXS (mean age = 12.61 years) completed assessments of phonological memory (nonword repetition and forward digit recall), verbal working memory (backward digit recall), vocabulary, syntax, and nonverbal cognition. Vocabulary and syntax skills were reassessed at a 2-year follow-up. In a series of analyses that controlled for nonverbal cognitive ability and severity of autism symptoms, the relative contributions of phonological and working memory to language change over time were investigated. These relationships were examined separately for boys and girls. In boys with FXS, phonological memory significantly predicted gains in vocabulary and syntax skills. Further, verbal working memory was uniquely associated with vocabulary gains among boys. In girls with FXS, phonological and working memory skills showed no relationship with language change across the 2-year time period. Our findings indicate that, for adolescent boys with FXS, acquisition of vocabulary and syntax may be constrained by the ability to maintain and manipulate phonological representations online. Implications for the identification and treatment of language disorders in this population are discussed. The present study is the first to identify specific cognitive mechanisms contributing to language growth over time in individuals with FXS.
脆性 X 综合征(FXS)是最常见的遗传性智力障碍病因。尽管 FXS 患者常伴有语言发育迟缓,但语言发展的纵向过程及其认知预测因素仍不清楚。本研究旨在探讨在青少年时期,语音和工作记忆技能是否可以预测 FXS 个体词汇和语法的增长。44 名 FXS 患者(平均年龄=12.61 岁)完成了语音记忆(非言语重复和顺背数字)、言语工作记忆(倒背数字)、词汇、语法和非言语认知的评估。在 2 年的随访中重新评估了词汇和语法技能。在一系列控制了非言语认知能力和自闭症症状严重程度的分析中,研究了语音和工作记忆对语言随时间变化的相对贡献。分别对男孩和女孩进行了这些关系的检查。在 FXS 男孩中,语音记忆显著预测了词汇和语法技能的提高。此外,言语工作记忆与男孩的词汇增长具有独特的相关性。在 FXS 女孩中,语音和工作记忆技能与 2 年时间内的语言变化没有关系。我们的研究结果表明,对于患有 FXS 的青少年男孩来说,词汇和语法的习得可能受到在线维持和处理语音表示的能力的限制。讨论了这些发现对该人群语言障碍识别和治疗的意义。本研究首次确定了特定的认知机制,这些机制有助于 FXS 个体随时间增长的语言发展。