Shaffer Rebecca C, Schmitt Lauren, John Thurman Angela, Abbeduto Leonard, Hong Michael, Pedapati Ernest, Dominick Kelli, Sweeney John, Erickson Craig
Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH 45229, USA.
Brain Sci. 2020 Jan 26;10(2):66. doi: 10.3390/brainsci10020066.
Language impairment is a core difficulty in fragile X syndrome (FXS), and yet standardized measures lack the sensitivity to assess developmental changes in the nature of these impairments. Expressive Language Sampling Narrative (ELS-N) has emerged as a promising new measure with research demonstrating its usefulness in a wide range of ages in developmental disabilities and typical development. We examined ELS-N results in FXS and age-matched typically developing (TD) controls along with cognitive, adaptive, and clinical measures. We found the groups differed significantly on all ELS-N variables. Cognitive abilities were related to lexical diversity, syntactic complexity, and unintelligibility for the FXS group, but only verbal abilities were related to syntactic complexity in TD. Autism spectrum disorder (ASD) symptomatology was related to less intelligibility in speech. Measures of hyperactivity were related to increased talkativeness and unintelligibility. In addition, FXS males in comparison to FXS females were more impaired in cognitive ability, ASD symptoms, hyperactivity, and anxiety. This study extends the previous ELS research, supporting its use in FXS research as a measure to characterize language abilities. It also demonstrates the relationships between ELS-N variables and measures of cognitive, adaptive, ASD symptoms, and clinical symptoms.
语言障碍是脆性X综合征（FXS）的一个核心难题，然而标准化测量方法缺乏评估这些障碍本质上发育变化的敏感性。叙事性表达语言抽样（ELS-N）已成为一种有前景的新测量方法，研究表明它在发育障碍和正常发育的广泛年龄段中都有用。我们研究了FXS患者以及年龄匹配的正常发育（TD）对照的ELS-N结果，同时还研究了认知、适应性和临床测量结果。我们发现两组在所有ELS-N变量上都有显著差异。对于FXS组，认知能力与词汇多样性、句法复杂性和可理解性相关，但在TD组中只有语言能力与句法复杂性相关。自闭症谱系障碍（ASD）症状与言语可理解性降低有关。多动测量指标与健谈和可理解性增加有关。此外，与FXS女性相比，FXS男性在认知能力、ASD症状、多动和焦虑方面受损更严重。本研究扩展了先前的ELS研究，支持将其用于FXS研究以表征语言能力。它还证明了ELS-N变量与认知、适应性、ASD症状和临床症状测量指标之间的关系。
