A Cognitive Test Battery for Intellectual Disabilities

智力障碍认知测试电池

• 批准号: 10456432
• 负责人: DAVID R HESSL
• 金额: $ 77.47万
• 依托单位: UNIVERSITY OF CALIFORNIA AT DAVIS
• 依托单位国家: 美国
• 财政年份: 2014
• 资助国家: 美国
• 起止时间: 2014-09-22 至 2025-09-29
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/10456432
• 关键词: Adaptive Behaviors; Administrative Supplement; Adolescent; Adult; Affect; Age; Aging; Alzheimer' s Disease; Assessment tool; Attention; Attention deficit hyperactivity disorder; Behavior; Biological Markers; Blood specimen; Child; Clinical Trials; Cognition; Cognitive; Collaborations; Collection; Concept Formation; Crystallization; Data; Data Collection; Dementia; Deterioration; Development; Disease; Double-Blind Method; Down Syndrome; Elderly; Episodic memory; Executive Dysfunction; Fragile X Syndrome; Funding; Future; Goals; Growth; Impaired cognition; Individual; Infant; Intellectual functioning disability; Intervention; Knowledge; Language; Language Disorders; Liquid substance; Longitudinal Studies; Measurement; Measures; Memory; Modification; Natural History; Nerve Degeneration; Outcome; Participant; Patients; Performance; Persons; Pharmaceutical Preparations; Phenotype; Placebo Effect; Placebos; Population; Process; Property; Psychometrics; Randomized; Reading; Research; Research Personnel; Risk; Ritalin; Sampling; Severities; Short-Term Memory; Standardization; Subgroup; Syndrome; Testing; Time; Toddler; United States National Institutes of Health; Validation; Validity and Reliability; Vocabulary; Work; autism spectrum disorder; base; clinical outcome measures; cognitive change; cognitive testing; cohort; comparative; data standards; executive function; experience; falls; feasibility testing; flexibility; high risk; improved; intervention effect; mathematical ability; mental age; population based; primary outcome; processing speed; response; young adult

Abstract The overarching goal of this R01 project (“A Cognitive Test Battery for Intellectual Disabilities”) is to develop and validate an assessment battery to measure cognitive outcomes of individuals with intellectual and developmental disability (IDD) in clinical trials. To achieve this goal, over the past 7 years we have been compiling data, making appropriate accommodations for this unique and challenging-to-test population, and carefully examining the psychometric properties (including feasibility, reliability, validity, and sensitivity to change) of the NIH Toolbox Cognitive Battery (NIHTB-CB), an iPad-based and norm-referenced set of tests measuring Fluid Reasoning (Processing Speed, Working Memory, Episodic Memory, Inhibitory Control and Attention, and Cognitive Flexibility) and Crystallized Language (Receptive Vocabulary and Reading) in children and youth with IDD, including those with fragile X syndrome (FXS), Down syndrome (DS), autism and idiopathic/other IDDs. The purpose of the present Administrative Supplement application is to request additional funds to extend data collection to older adults with IDD, including DS and FXS. Aging in IDDs is understudied, and older individuals are often excluded from research in part due to limitations in available assessment tools. The supplement will allow collection of NIHTB-CB data, standardized IQ and adaptive behavior data and measures from the ABC-Down Syndrome consortium harmonized core from 100 adult participants (50 with DS, 50 with FXS; ages 26 and up, stratified across young and older adult groups), as well as blood samples for biomarker characterization in collaboration with the ABC-DS group. Based upon the ABC- DC dementia assessment, we plan to include 20 with dementia to allow examination the potential sensitivity of the NIHTB-CB to dementia-related cognitive changes. Our extended data collection with adults will fall primarily within the scope of project Aims 1 and 3, which focus on optimization of the battery for lower functioning persons and sensitivity of the battery to developmental changes. In particular, we will examine the newly collected data from aging persons to determine whether modifications may be necessary, whether new tests may need to be developed, and whether it shows potential for detecting deterioration in cognition associated with aging or neurodegeneration, as in some participants with DS. As aging in DS is associated with high risk of early developing Alzheimer’s disease (AD), it is important to develop and validate cognitive tests that may be used to track the disease process and response to intervention. Cognition and neurodegeneration in older adults with FXS have been largely ignored, despite evidence of AD-related biomarker abnormalities and cognitive decline in some patients. The supplemental data collection from the FXS cohort will help to determine whether larger, more extensive longitudinal studies are justified to describe the natural history of this population and open the door for their participation in other research. The supplemental NIHTB-CB data collected from this aging group will provide critical preliminary information about its potential utility in future natural history or treatment studies.

摘要 这个R 01项目（“智力残疾者认知测试组合”）的首要目标是开发 并验证一个评估组合，以衡量具有智力和 发育障碍（IDD）的临床试验。为了实现这一目标，在过去的7年里，我们一直在 汇编数据，为这一独特的、具有挑战性的测试人群提供适当的便利， 仔细检查心理测量特性（包括可行性，可靠性，有效性和敏感性）， 美国国立卫生研究院认知电池（NIHTB-CB），一个基于iPad和正常参考的测试集 测量流体推理（处理速度，工作记忆，情景记忆，抑制控制和 注意力和认知灵活性）和儿童的结晶语言（接受性词汇和阅读） 和青年缺碘症患者，包括脆性X综合征（FXS）、唐氏综合征（DS）、自闭症和 特发性/其他IDDs。本行政补充申请的目的是要求 额外的资金，以扩大数据收集，以老年人IDD，包括DS和FXS。缺碘症的老龄化 研究不足，老年人往往被排除在研究之外，部分原因是由于可用的限制。 评估工具。该补充将允许收集NIHTB-CB数据、标准化IQ和自适应 行为数据和措施，从ABC-唐氏综合征联盟协调核心，从100名成年人 参与者（50名DS患者，50名FXS患者;年龄26岁及以上，在年轻和老年成人组中分层），以及 与ABC-DS小组合作，作为生物标志物表征的血液样本。根据ABC- DC痴呆评估，我们计划纳入20名痴呆患者，以允许检查潜在的敏感性， NIHTB-CB与痴呆相关的认知变化。我们对成年人的扩展数据收集将主要集中在 在项目目标1和3的范围内，重点是为功能较低的人优化电池 以及电池对发育变化的敏感性。特别是，我们将检查新收集的数据， 从老年人，以确定是否有必要修改，是否需要新的测试， 开发，以及它是否显示出检测与衰老相关的认知退化的潜力， 神经退行性变，如在一些参与者与DS。由于DS中的衰老与早期 发展阿尔茨海默病（AD），重要的是要开发和验证认知测试，可用于 跟踪疾病进程和干预反应。老年人的认知和神经退行性变 尽管有证据表明AD相关生物标志物异常和认知能力下降，但FXS在很大程度上被忽视了 在一些患者中。从FXS队列收集的补充数据将有助于确定是否更大， 更广泛的纵向研究是合理的，以描述这一人口的自然历史，并打开 为他们参与其他研究提供了机会。从该老化组收集的补充NIHTB-CB数据 将提供关于其在未来自然史或治疗研究中的潜在效用的关键初步信息。