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The Executive Function Account of Repetitive Behavior: Evidence From Rubinstein-Taybi Syndrome. 重复行为的执行功能解释:来自Rubinstein-Taybi综合征的证据。
IF 2.1 4区 医学 Q2 Medicine Pub Date : 2023-01-01 DOI: 10.1352/1944-7558-128.1.49
Jane Waite, Sarah R Beck, Laurie Powis, Chris Oliver

In this study, we focus on Rubinstein-Taybi syndrome (RTS) to explore the associations between executive function deficits and repetitive behaviors. Thirty individuals with RTS completed direct assessments of inhibition, working memory and set-shifting. Informants completed repetitive behavior and executive function questionnaires. Repetitive questions were associated with poorer inhibition and working memory. Stereotypy was associated with poorer inhibition. Adherence to routines was associated with poorer set-shifting, but only on the parental report measure. No other associations were evident. There is evidence of an association between specific repetitive behaviors and executive functioning in RTS, suggesting executive dysfunction may underpin behavioral difference in RTS. The findings point towards specific associations that are of interest for further research across populations in which repetitive behaviors are present.

本研究以Rubinstein-Taybi综合征(RTS)为研究对象,探讨执行功能缺陷与重复行为之间的关系。30名RTS患者完成了抑制、工作记忆和场景转移的直接评估。被调查者完成了重复行为和执行功能问卷。重复的问题与较差的抑制和工作记忆有关。刻板印象与较差的抑制能力有关。坚持常规与较差的设置转换有关,但仅在父母报告的衡量标准上。没有其他明显的联系。有证据表明,RTS中的特定重复行为与执行功能之间存在关联,这表明执行功能障碍可能是RTS行为差异的基础。这些发现指出了一些特定的关联,这些关联对于在存在重复行为的人群中进行进一步研究很有意义。
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引用次数: 1
FMR1 CGG Repeats and Stress Influence Self-Reported Cognitive Functioning in Mothers. FMR1 CGG重复和压力影响母亲自我报告的认知功能。
IF 2.1 4区 医学 Q2 Medicine Pub Date : 2023-01-01 DOI: 10.1352/1944-7558-128.1.1
Nell Maltman, Leann Smith DaWalt, Jinkuk Hong, Mei Wang Baker, Elizabeth M Berry-Kravis, Murray H Brilliant, Marsha Mailick

Variation in the FMR1 gene may affect aspects of cognition, such as executive function and memory. Environmental factors, such as stress, may also negatively impact cognitive functioning. Participants included 1,053 mothers of children with and without developmental disabilities. Participants completed self-report measures of executive function, memory, and stress (i.e., life events, parenting status), and provided DNA to determine CGG repeat length (ranging from 7 to 192 CGGs). Stress exposure significantly predicted greater self-reported difficulties in executive function and the likelihood of memory problems. Cubic CGG effects independently predicted executive function and memory difficulties, suggesting effects of both genetic variation and environmental stress exposure on cognitive functioning.

FMR1基因的变异可能影响认知的各个方面,如执行功能和记忆。环境因素,如压力,也可能对认知功能产生负面影响。参与者包括1053名有或没有发育障碍儿童的母亲。参与者完成了执行功能、记忆和压力(即生活事件、养育状况)的自我报告测量,并提供DNA来确定CGG重复长度(范围从7到192个CGG)。压力暴露显著预示着更大的自我报告的执行功能困难和记忆问题的可能性。立方CGG效应独立预测执行功能和记忆困难,表明遗传变异和环境应激暴露对认知功能都有影响。
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引用次数: 4
A Personal Narrative Intervention for Adults With Autism and Intellectual Disability. 成人自闭症和智力障碍的个人叙事干预。
IF 2.1 4区 医学 Q2 Medicine Pub Date : 2023-01-01 DOI: 10.1352/1944-7558-128.1.21
Nicole L Birri, Christina R Carnahan, Carla Schmidt, Pamela Williamson

Due to the unique social cognitive profiles of individuals with autism spectrum disorder (ASD) with and without intellectual disability (ID) sharing coherent and complex personal narratives can be challenging. To address these challenges research has focused on teaching macrostructure components using visual supports and repeated opportunities to practice. Despite success by young children with ASD and ID, the application of this instruction for adults with ASD with and without ID is still largely unknown. An ABAB single case withdrawal design was used to determine the effects of a personal narrative intervention to teach macrostructure within participant-generated personal narratives. Results indicate all participants demonstrated more coherent and complex personal narratives with the intervention. The results and implications for practice are discussed.

由于自闭症谱系障碍(ASD)患者有或没有智力残疾(ID)的独特社会认知特征,分享连贯和复杂的个人叙述可能具有挑战性。为了应对这些挑战,研究集中在使用视觉支持和重复练习的机会来教授宏观结构组件。尽管有ASD和ID的幼儿取得了成功,但这种指导在有或没有ID的ASD成人中的应用仍然很大程度上是未知的。采用ABAB单例退出设计来确定个人叙事干预在参与者生成的个人叙事中教授宏观结构的效果。结果表明,所有参与者都表现出更连贯和复杂的个人叙述。讨论了研究结果及其对实践的启示。
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引用次数: 0
Résumés en Français. 英文摘要。
IF 2.1 4区 医学 Q2 Medicine Pub Date : 2022-12-01 DOI: 10.1352/1944-7558-122.3.282
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引用次数: 0
Examining Phenotypic Differences in Gaze Avoidance Between Autism Spectrum Disorder and Fragile X Syndrome. 自闭症谱系障碍和脆性X综合征凝视回避的表型差异研究。
IF 2.1 4区 医学 Q2 Medicine Pub Date : 2022-11-01 DOI: 10.1352/1944-7558-127.6.435
Ellen H Wilkinson, Tobias C Britton, Scott S Hall

We examined potential phenotypic differences in eye gaze avoidance exhibited by boys with autism spectrum disorder (ASD) and boys with fragile X syndrome (FXS). In Study 1, the Eye Contact Avoidance Scale (ECAS) was administered to caregivers of boys aged 7-18 years with FXS (n = 148), ASD (n = 168), and mixed developmental disabilities (MDD; n = 128). In Study 2, subsets of boys with FXS (n = 31) and boys with ASD (n = 25) received a brief behavioral treatment probe to improve eye contact. Results showed that boys with FXS obtained significantly higher scores on the ECAS compared to boys with ASD and MDD. Exposure to the brief behavioral treatment probe resulted in significant decreases in scores for boys with FXS, but not for boys with ASD.

我们研究了自闭症谱系障碍(ASD)男孩和脆性X染色体综合征(FXS)男孩在眼睛凝视回避方面的潜在表型差异。研究1对7-18岁FXS (n = 148)、ASD (n = 168)、混合性发育障碍(MDD;N = 128)。在研究2中,患有FXS的男孩(n = 31)和患有ASD的男孩(n = 25)接受了简短的行为治疗探针,以改善眼神交流。结果显示,FXS男孩的ECAS得分明显高于ASD和MDD男孩。暴露于简短的行为治疗探针导致FXS男孩的得分显著下降,但对ASD男孩没有影响。
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引用次数: 2
Public Stigmatization of People With Intellectual Disability During the COVID-19 Pandemic. 2019冠状病毒病大流行期间智障人士的公众污名化
IF 2.1 4区 医学 Q2 Medicine Pub Date : 2022-11-01 DOI: 10.1352/1944-7558-127.6.485
Maria R Dekker, Alexander H C Hendriks, Noud Frielink, Petri J C M Embregts

This study aimed to examine the level of discrimination against people with intellectual disability during COVID-19, and assessed stereotypes, levels of familiarity with people with intellectual disability, and personal experiences with COVID-19 as potential correlates. A cross-sectional study was conducted using a large sample from the Dutch population (n = 1,797). Salient stereotype factors of people with intellectual disability were "friendly" and "in need of help," but not "give nuisance." Those respondents who were unfamiliar with people with intellectual disability in real life demonstrated higher levels of discrimination, perceiving them as more of a nuisance and as being less in need of help, in comparison to those who were more familiar. People with intellectual disability were judged by an ambivalent set of stereotypes during the COVID-19 pandemic that were in line with pre-COVID-19 findings and as such seemed to be fairly persistent and robust. There is a pressing need to both raise awareness of stereotypes towards and discrimination against people with intellectual disability via advocacy and education, and to facilitate positive encounters.

本研究旨在调查2019冠状病毒病期间对智障人士的歧视程度,并评估了刻板印象、对智障人士的熟悉程度以及与COVID-19相关的个人经历。横断面研究从荷兰人口中抽取了大量样本(n = 1797)。对智障人士的显著刻板印象因素是“友好”和“需要帮助”,而不是“讨厌”。与那些更熟悉的人相比,那些在现实生活中不熟悉智障人士的受访者表现出更高程度的歧视,认为他们更讨厌,更不需要帮助。在2019冠状病毒病大流行期间,人们对智障人士的评价是一套矛盾的刻板印象,这些刻板印象与2019冠状病毒病之前的调查结果一致,因此似乎相当持久和强大。迫切需要通过宣传和教育提高人们对对智力残疾者的陈规定型观念和歧视的认识,并促进积极的接触。
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引用次数: 2
Mental State Language Development in Children With Down Syndrome Versus Typical Development. 唐氏综合症儿童的精神状态语言发展与典型发展。
IF 2.1 4区 医学 Q2 Medicine Pub Date : 2022-11-01 DOI: 10.1352/1944-7558-127.6.495
Marie Moore Channell, Linnea E Sandstrom, Danielle Harvey

This study compared mental state language (talk about emotions, thoughts, intentions, etc.) used by 6- to 11-year-old children with Down syndrome (DS) to a younger typically developing (TD) comparison group matched by nonverbal cognition. We aimed to determine (1) whether mental state language use is delayed in DS relative to developmental expectations, and (2) if there are differences between groups in the association between mental state language and developmental factors (emotion knowledge, expressive language). Rate of mental state language use was significantly lower in the group with DS, but the number of different mental state terms was not significantly different. Nuanced patterns of similarity and difference emerged between groups regarding the association between mental state language and other developmental factors.

本研究比较了6- 11岁唐氏综合症(DS)儿童和较年轻的典型发育(TD)儿童使用的心理状态语言(谈论情绪、思想、意图等)与非语言认知相匹配的对照组。我们的目的是确定(1)相对于发展期望,残疾儿童的心理状态语言使用是否延迟;(2)心理状态语言与发展因素(情感知识、表达语言)的关联是否在群体之间存在差异。心理状态语言使用率显著低于心理障碍组,但不同心理状态术语的使用数量无显著差异。关于心理状态、语言和其他发展因素之间的关系,不同群体之间出现了细微的相似和差异模式。
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引用次数: 1
Using a Delphi Process to Update the Nisonger Child Behavior Rating Form. 用德尔菲法更新大儿童行为评定表。
IF 2.1 4区 医学 Q2 Medicine Pub Date : 2022-11-01 DOI: 10.1352/1944-7558-127.6.455
Kelsey B Shively, Marc J Tassé

The Nisonger Child Behavior Rating Form (NCBRF) was developed to specifically screen psychopathology and problem behavior in children and adolescents with IDD. This study aimed to update the NCBRF to the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5) and screen for the most prevalent psychopathologies that affect children and adolescents with IDD. The authors re-aligned the existing items to fit within a DSM-5 framework, and then used the Delphi method with a panel of experts in IDD to evaluate the NCBRF items. The revision process included deleting and revising existing items and formulating new items. We obtained a final item pool after three iterations. The Delphi process and resulting item pool are described in this article.

尼松格儿童行为评定表(NCBRF)是专门用于筛查儿童和青少年缺乏症的精神病理和问题行为。本研究旨在将NCBRF更新为精神疾病诊断与统计手册第5版(DSM-5),并筛选影响儿童和青少年IDD的最普遍的精神病理。作者将现有条目重新排列以适应DSM-5框架,然后与IDD专家组一起使用德尔菲法对NCBRF条目进行评估。修订过程包括删除和修订现有项目和制定新项目。经过三次迭代,我们获得了最终的项池。本文描述了Delphi过程和结果项池。
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引用次数: 0
NIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome. 美国国立卫生研究院工具箱认知电池在威廉姆斯综合征患者中的可行性。
IF 2.1 4区 医学 Q2 Medicine Pub Date : 2022-11-01 DOI: 10.1352/1944-7558-127.6.473
Emma E Condy, Lindsey Becker, Cristan Farmer, Aaron J Kaat, Colby Chlebowski, Beth A Kozel, Audrey Thurm

The NIH Toolbox Cognition Battery (NIHTB-CB) was developed for epidemiological and longitudinal studies across a wide age span. Such a tool may be useful for intervention trials in conditions characterized by intellectual disability (ID), such as Williams syndrome (WS). Three NIHTB-CB tasks, including two executive functioning (Flanker, Dimensional Change Card Sort) and one episodic memory (Picture Sequence Memory) task, were given to 47 individuals with WS, ages 4 to 50, to evaluate feasibility (i.e., proportion of valid administrations) in this population. Findings indicated that NIHTB-CB tests showed good feasibility. Flanker and DCCS age-corrected scores were negatively correlated with age and showed floor effects, indicating these scores may not be useful for quantifying performance on these NIHTB-CB tests in ID.

美国国立卫生研究院工具箱认知电池(NIHTB-CB)是为流行病学和纵向研究而开发的,跨越广泛的年龄跨度。这种工具可能对以智力残疾(ID)为特征的干预试验有用,例如威廉姆斯综合征(WS)。对47名年龄在4 - 50岁的WS患者进行三项NIHTB-CB任务,包括两项执行功能(侧卫,维度变化卡片排序)和一项情景记忆(图片序列记忆)任务,以评估该人群的可行性(即有效给药的比例)。结果表明,NIHTB-CB试验具有良好的可行性。Flanker和DCCS年龄校正分数与年龄呈负相关,并表现出最低效应,表明这些分数可能不适用于量化ID在这些NIHTB-CB测试中的表现。
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引用次数: 0
Working Memory Training in Youth With Autism, Fragile X, and Intellectual Disability: A Pilot Study. 自闭症、脆性X和智力残疾青年的工作记忆训练:一项试点研究。
IF 2.1 4区 医学 Q2 Medicine Pub Date : 2022-09-01 DOI: 10.1352/1944-7558-127.5.369
Catrina A Calub, Songpoom Benyakorn, Shuai Sun, Ana-Maria Iosif, Lauren H Boyle, Marjorie Solomon, David Hessl, Julie B Schweitzer

This pilot study sought to identify potential markers of improvement from pre-post treatment in response to computerized working memory (WM) training for youth (ages 8-18) with autism spectrum disorder (ASD) and comorbid intellectual disability (ID) in a single arm, pre-post design. Participants included 26 children with ASD and 18 with comorbid ASD and fragile X syndrome (ASD+FXS). Analyses were adjusted for age and IQ. The ASD group demonstrated greater improvement on WM training relative to the ASD+FXS group. Participants improved on WM and far transfer outcomes, however, there were no significant group differences in improvement except for repetitive behavior. Higher hyperactivity/impulsivity ratings predicted lower performance on visuospatial WM. Findings suggest cognitive training may be beneficial for youth with ASD and ID, warranting further exploration.

这项试点研究试图在一项单臂、前后设计中,为患有自闭症谱系障碍(ASD)和共病智力残疾(ID)的青少年(8-18岁)确定针对计算机化工作记忆(WM)训练的治疗前后改善的潜在标志物。参与者包括26名ASD儿童和18名患有ASD和脆性X综合征(ASD+FXS)的儿童。分析根据年龄和智商进行了调整。与ASD+FXS组相比,ASD组在WM训练方面表现出更大的改善。参与者在WM和远迁移结果方面有所改善,然而,除了重复行为外,在改善方面没有显著的组间差异。较高的多动/冲动评分预测视觉空间WM的表现较低。研究结果表明,认知训练可能对患有ASD和ID的年轻人有益,值得进一步探索。
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引用次数: 0
期刊
Ajidd-American Journal on Intellectual and Developmental Disabilities
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