BACKGROUND Receptive and expressive vocabulary in adult and adolescent males with fragile X syndrome (FXS) have been shown as significantly lower than their chronological age; however, receptive vocabulary has been considered a strength relative to mental age. This has not been formally examined, however, and data are needed to compare receptive vocabulary with other language skills and with mental age in individuals with FXS. This is especially important as vocabulary measures are sometimes used as a proxy to estimate language ability. METHODS This preliminary study examined receptive vocabulary, global language, and cognitive skills in 42 adults (33 males and 9 females) with FXS as a portion of the baseline evaluation prior to randomization in a clinical trial of ampakine CX516. The battery of standardized tests addressed receptive vocabulary with the Peabody Picture Vocabulary Test, Third Edition (PPVT-III), receptive and expressive language (termed henceforth as global language) via the Preschool Language Scale, Fourth Edition or the Clinical Evaluation of Language Fundamentals, Third Edition, and non-verbal cognition via the Stanford-Binet Intelligence Scales, Fourth Edition (SB-IV). RESULTS Results showed (1) significantly higher receptive vocabulary than global language, (2) significantly better receptive vocabulary than non-verbal cognition, (3) equivalent non-verbal cognition and global language, and (4) severity of autism symptomatology was not correlated to receptive vocabulary or global language once non-verbal cognition was removed as factor. The scores from the PPVT-III did not represent the global language skills in our sample of adults with FXS. CONCLUSIONS Findings from this investigation strongly suggest that the PPVT-III should not be used as a screening tool for language levels or cognitive function in clinical studies since the scores from the PPVT-III were not representative of global language or non-verbal cognitive skills in adults with intellectual disabilities. This finding is critical in order to understand how to evaluate, as well as to treat, language in individuals with FXS. Development of efficient and appropriate tools to measure language, cognition, and behavior in individuals with FXS is essential.

中文翻译：

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成人易碎X综合征（FXS）的词汇理解。

背景技术已经表明，患有脆性X综合征（FXS）的成年和青春期男性的接受和表达词汇量明显低于其年龄。但是，接受词汇被认为是相对于心理年龄的一种力量。但是，尚未对此进行正式检查，并且需要数据来比较具有FXS的个人的接受性词汇与其他语言技能和心理年龄。这一点尤其重要，因为词汇量有时会用作评估语言能力的替代指标。方法这项初步研究在接受安帕金CX516临床试验随机化之前，对FXS作为基线评估的一部分的42名成年人（33名男性和9名女性）的接受词汇，整体语言和认知技能进行了检查。一系列标准化测试通过Peabody图片词汇测试，第3版（PPVT-III），通过学前语言量表，第4版或《语言基础知识临床评估》，对接受和表达语言（此后称为全球语言）进行处理，第三版，以及通过Stanford-Binet智力量表，第四版（SB-IV）进行的非语言认知。结果结果显示：（1）接受词汇比整体语言明显高；（2）接受词汇比非语言认知大得多；（3）等效的非语言认知和整体语言；（4）自闭症症状的严重程度与相关性不相关一旦去除非语言认知作为因素，就可以接受词汇或全球性语言。在我们的FXS成人样本中，PPVT-III的分数并不代表全球语言技能。结论这项调查的结果强烈表明，PPVT-III不应该用作临床研究中语言水平或认知功能的筛查工具，因为PPVT-III的分数不能代表全球语言或非语言认知技能。智障成人。为了了解如何评估和治疗FXS个体的语言，这一发现至关重要。开发有效和适当的工具来衡量具有FXS的个人的语言，认知和行为至关重要。结论这项调查的结果强烈表明，PPVT-III不应该用作临床研究中语言水平或认知功能的筛查工具，因为PPVT-III的分数不能代表全球语言或非语言认知技能。智障成人。为了了解如何评估和治疗FXS个体的语言，这一发现至关重要。开发有效和适当的工具来衡量具有FXS的个人的语言，认知和行为至关重要。结论这项调查的结果强烈表明，PPVT-III不应该用作临床研究中语言水平或认知功能的筛查工具，因为PPVT-III的分数不能代表全球语言或非语言认知技能。智障成人。为了了解如何评估和治疗FXS个体的语言，这一发现至关重要。开发有效和适当的工具来衡量具有FXS的个人的语言，认知和行为至关重要。以及在具有FXS的个人中对待语言。开发有效和适当的工具来测量具有FXS的个人的语言，认知和行为至关重要。以及在具有FXS的个人中对待语言。开发有效和适当的工具来衡量具有FXS的个人的语言，认知和行为至关重要。