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.

抽象的 这个R01项目的总体目标（“智力残疾的认知测试电池”）是开发 并验证评估电池以衡量具有智力和知识分子的人的认知结果 临床试验中的发育障碍（IDD）。为了实现这一目标，在过去的7年中 汇编数据，为这个独特和挑战的人群提供适当的住宿，以及 仔细检查心理测量特性（包括可行性，可靠性，有效性和对 更改NIH工具箱认知电池（NIHTB-CB），这是一套基于iPad的和规范的测试集 测量流体推理（处理速度，工作记忆，情节记忆，抑制性控制和 儿童的注意力和认知灵活性）和结晶的语言（接受词汇和阅读） 和患有IDD的年轻人，包括患有脆弱X综合征（FXS），唐氏综合症（DS），自闭症和 特发性/其他IDD。本管理补充申请的目的是要求 额外的资金将数据收集扩展到具有IDD的老年人，包括DS和FXS。 IDD的老化 被理解为，通常由于可用的限制而被排除在研究之外 评估工具。该补充剂将允许收集NIHTB-CB数据，标准化智商和自适应 来自ABC降低综合征联盟的行为数据和措施来自100名成人的核心 参与者（50名DS，50个有FXS； 26岁及以上的年龄在年轻人和老年人群体中分层） 作为与ABC-DS组合作的生物标志物表征的血液样本。基于ABC- DC痴呆评估，我们计划包括20种痴呆症，以允许检查潜在的敏感性 NIHTB-CB与痴呆相关的认知变化。我们与成人的扩展数据收集将下降 在项目目标1和3的范围内，重点是针对低功能人员优化电池 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 从老化的人到确定是否需要修改，是否需要新测试 开发，以及是否显示出与衰老相关的认知发现检测的潜力 神经变性，就像在某些DS参与者中一样。因为DS中的衰老与早期的高风险有关 开发阿尔茨海默氏病（AD），开发和验证可能用于的认知测试很重要 跟踪疾病过程和对干预的反应。老年人的认知和神经退行性 FX在很大程度上被忽略了，与广告相关的生物标志物异常和认知能力下降的任务证据 在某些患者中。来自FXS队列的补充数据收集将有助于确定是否更大， 更广泛的纵向研究是合理的，可以描述该人群的自然历史并开放 他们参与其他研究的门。从这个衰老组收集的补充NIHTB-CB数据 将提供有关其未来自然史或治疗研究中其潜在效用的关键初步信息。