Anxiety-like behavior in Rett syndrome: characteristics and assessment by anxiety scales
Anxiety-like behavior in Rett syndrome: characteristics and assessment by anxiety scales
复制标题
DOI：
10.1186/s11689-015-9127-4
复制
发表时间：
2015-09-15
影响因子：
4.9
通讯作者：
Kaufmann, Walter E.
中科院分区：
文献类型：
--
作者：
Barnes, Katherine V.;Coughlin, Francesca R.;Kaufmann, Walter E.
背景：Rett综合征（RTT）是一种严重的神经发育障碍，以语言和运动技能退化、认知障碍和频繁癫痫发作为特征。虽然诊断标准侧重于沟通、运动障碍和手部刻板印象，但行为异常是RTT表型中普遍存在的致残成分。在这些问题行为中，焦虑是一个突出的症状。虽然Rett综合征行为问卷（RSBQ）的引入代表了该领域的重大进步，但尚未有使用RSBQ或其他标准化测量方法系统表征焦虑行为的报道。方法：本研究检查了74名患有RTT的女孩样本的焦虑行为概况，重点是确定在这一人群中具有最佳心理测量特性的工具。父母评定的RSBQ、焦虑、抑郁和情绪量表（ADAMS）和异常行为量表-社区（ABC-C）这两种以前用于神经发育障碍儿童的工具，在得分谱、与年龄和临床严重程度的关系、信度、并发效度和功能影响方面进行了分析。后者是通过回归分析Vineland适应行为量表-第二版（Vineland- ii）和儿童健康问卷（CHQ）确定的，CHQ是在RTT中验证的生活质量测量。结果：我们发现，相对于RSBQ、ADAMS和ABC-C测量的其他行为结构，焦虑亚量表的得分处于中间水平。年龄不影响得分，一般焦虑的严重程度与临床严重程度呈负相关。我们证明了焦虑相关分量表的内部一致性是最高的。ADAMS分量表的重测信度和量表内信度较高。通过量表间的相关关系来衡量收敛效度和区别效度，其中社会焦虑子量表表现出最好的特征。其中，只有ADAMS社交回避与生活质量相关。结论：我们得出结论，焦虑样行为是RTT行为表型的一个重要组成部分，主要影响神经功能损害较轻的儿童，并具有功能性后果。基于标准化仪器的现有数据，ADAMS，特别是其社会回避子量表具有最佳的心理测量特性和功能相关性，使其适合临床和研究
Background: Rett syndrome (RTT) is a severe neurodevelopmental disorder characterized by regression of language and motor skills, cognitive impairment, and frequent seizures. Although the diagnostic criteria focus on communication, motor impairments, and hand stereotypies, behavioral abnormalities are a prevalent and disabling component of the RTT phenotype. Among these problematic behaviors, anxiety is a prominent symptom. While the introduction of the Rett Syndrome Behavioral Questionnaire (RSBQ) represented a major advancement in the field, no systematic characterization of anxious behavior using the RSBQ or other standardized measures has been reported.Methods: This study examined the profiles of anxious behavior in a sample of 74 girls with RTT, with a focus on identifying the instrument with the best psychometric properties in this population. The parent-rated RSBQ, Anxiety, Depression, and Mood Scale (ADAMS), and Aberrant Behavior Checklist-Community (ABC-C), two instruments previously employed in children with neurodevelopmental disorders, were analyzed in terms of score profiles, relationship with age and clinical severity, reliability, concurrent validity, and functional implications. The latter were determined by regression analyses with the Vineland Adaptive Behavior Scales-Second Edition (Vineland-II) and the Child Health Questionnaire (CHQ), a quality of life measure validated in RTT.Results: We found that scores on anxiety subscales were intermediate in range with respect to other behavioral constructs measured by the RSBQ, ADAMS, and ABC-C. Age did not affect scores, and severity of general anxiety was inversely correlated with clinical severity. We demonstrated that the internal consistency of the anxiety-related subscales were among the highest. Test-retest and intra-rater reliability was superior for the ADAMS subscales. Convergent and discriminant validity were measured by inter-scale correlations, which showed the best profile for the social anxiety subscales. Of these, only the ADAMS Social Avoidance showed correlation with quality of life.Conclusions: We conclude that anxiety-like behavior is a prominent component of RTT's behavioral phenotype, which affects predominantly children with less severe neurologic impairment and has functional consequences. Based on available data on standardized instruments, the ADAMS and in particular its Social Avoidance subscale has the best psychometric properties and functional correlates that make it suitable for clinical and research