A Cognitive Test Battery for Intellectual Disabilities

智力障碍认知测试电池

• 批准号: 9514197
• 负责人: DAVID R HESSL
• 金额: $ 60.88万
• 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 2020-09-29
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/9514197
• 关键词: Adult; Age; Animal Model; Animals; Attention; Behavior; Behavioral; Brain; Child; Clinical; Clinical Trials; Cognition; Cognitive; Cognitive deficits; Computers; Controlled Clinical Trials; Detection; Development; Developmental Disabilities; Dimensions; Disease; Down Syndrome; Episodic memory; Executive Dysfunction; FMR1; Floor; Fragile X Syndrome; Functional disorder; General Population; Goals; Gold; Growth; Human; Impairment; Individual; Intellectual functioning disability; Intelligence; Knockout Mice; Language; Letters; Longitudinal Studies; Measures; Memory; Names; Neurobiology; Neurodevelopmental Disorder; Neurosciences; Oral; Outcome; Outcome Measure; Participant; Pattern; Persons; Pharmacology; Phenotype; Population; Population Control; Prediabetes syndrome; Process; Property; Psychometrics; Reading; Research; Research Personnel; Short-Term Memory; Sorting - Cell Movement; Syndrome; Testing; Time; Translating; Translational Research; Translations; United States National Institutes of Health; Validation; Vocabulary; Work; cognitive development; cognitive function; cognitive testing; disability; executive function; improved; meetings; memory process; mouse Ts65Dn; mouse model; processing speed; public health relevance; response; standard measure; tool; translational neuroscience; treatment response; treatment trial; virtual

 DESCRIPTION (provided by applicant): There is a rapidly growing translational research effort to understand the brain mechanisms of dysfunction in neurodevelopmental disorders such as fragile X syndrome (FXS), Down syndrome (DS), and other conditions that cause intellectual disabilities (ID). Evidence from the animal models of these syndromic forms of disability documents that targeted treatments have strong potential to normalize the neurobiological, behavioral, and perhaps cognitive abnormalities associated with these conditions in humans. This groundbreaking work has led to many controlled clinical trials already underway, and many more now in the planning stages. However, virtually no developmentally appropriate, well-validated, sensitive and reliable cognitive measures suitable for tracking treatment response are available for individuals with ID. Indeed it could be argued that lack of timely development of such measures has significantly slowed translation of targeted treatments and could result in incorrect interpretation of such trial results, compromising development efforts for targeted treatments aimed at ID syndromes. In response to this urgent need, the primary aim of the proposed project is to validate a set of cognitive measures of executive function, processing speed, episodic memory, language, and attention for use as outcome measures for individuals with ID, with a primary initial focus on fragile X and Down syndromes. This cognitive battery was initially developed by the NIH Neuroscience Blueprint Toolbox consortium for the general population for ages of 3 to 89 years, and in the current project will be validated and modified as necessary for those with ID. Furthermore, a longitudinal study of these and other measures in participants over a two-year period will help to establish sensitivity to change. The proposed research will benefit a wide range of studies aiming to assess or improve specific domains of cognition and general intellectual functioning in persons with ID.

 描述（由申请人提供）：有一个快速增长的转化研究工作，以了解神经发育障碍，如脆性X综合征（FXS），唐氏综合征（DS）和其他条件，导致智力残疾（ID）功能障碍的大脑机制。来自这些综合征形式的残疾文件的动物模型的证据表明，有针对性的治疗具有很强的潜力，使与人类这些疾病相关的神经生物学，行为和认知异常正常化。这项开创性的工作已经导致许多对照临床试验正在进行中，还有更多的试验正在规划阶段。然而，几乎没有发育适当的，经过充分验证的，敏感和可靠的认知措施，适合跟踪治疗反应可用于个人ID。事实上，它可以说，缺乏及时发展的这些措施显着减缓翻译的目标治疗，并可能导致不正确的解释这样的试验结果，损害发展努力的目标治疗旨在ID综合征。为了满足这一迫切需求，拟议项目的主要目的是验证一套认知措施的执行功能，处理速度，情景记忆，语言和注意力作为个人ID的结果措施，主要的初始重点是脆性X和唐氏综合征。这种认知电池最初是由NIH神经科学蓝图联盟为3至89岁的普通人群开发的，在当前的项目中，将根据需要对ID患者进行验证和修改。此外，对这些和其他措施的纵向研究参与者在两年的时间内将有助于建立对变化的敏感性。拟议的研究将有利于广泛的研究，旨在评估或改善特定领域的认知和一般智力功能的人与ID。