NEUROBEHAVIORAL EVALUATION CORE

A.目标 在2001年对我们的MRDDRC申请的评论中，研究部分指出（第5页）： 增加到中心，以便在赠款开发的早期阶段为这一领域提供更好的投入。在 针对这一建议，我们建议开发神经行为评估核心（NEC）， 为调查人员提供这种专业知识。NEC的主要目标是提供先进的 神经行为和神经心理学研究支持MRDDRC项目，并开发或验证 为此目的的新方法我们对复杂神经行为的理解的科学进步 我们能够可靠而精确地定义发育障碍， 和行为表型。 行为和认知变量的测量在疾病和发育研究中至关重要， 组成了我们的民主与发展改革委员会。在过去的3-5年里， 以行为和/或心理社会为重点的MRDDRC项目。这些研究的目的是 发育，认知和行为结果的儿童谁有各种神经遗传， 神经发育或影响认知和认知的获得性障碍。除了严格研究 基础疾病（如尿素循环障碍或癫痫），这些研究旨在描述 对孩子认知和行为发展的影响。二十到三十年前，这种类型的 研究可能集中在发展的广泛方面，例如这些人的智力水平。 孩子较新的方法可以更深入地关注诸如注意力， 执行功能和记忆子过程。发展神经心理学的大爆发 将潜在的神经发育和障碍与行为和认知联系起来的知识库 发展进一步推动了儿科研究的需要，包括复杂的模型， 神经行为和神经心理功能。 从历史上看，许多研究可能受益于神经心理学的检查， 神经行为变量没有包括这样的评估，因为：1）有限的知识， 现有措施; 2）专家人员和/或采购的研究项目预算内的成本限制 缺乏测试材料;以及3）缺乏熟练进行这些评估的人员。的 国家教育委员会的主要目标是改善获得适当措施的机会，并提高 神经行为研究正在整个MRDDRC进行。 在NEC内部，我们建议：1）提供广泛的神经心理测试电池 服务; 2）开发和管理神经成像协议的神经行为任务，包括fMRI项目 通过神经影像学核心; 3）向MRDDRC研究者提供关于适当设计的咨询， 在研究中使用神经行为和神经心理测量; 4）培训研究人员， 他们的工作人员在使用这些措施。随着技术的进步，调查人员将能够评估 神经行为结果的几个水平，例如，特定的神经心理过程，如记忆或 执行功能，以及生活质量功能状态。这将能够与研究相结合， 在遗传水平上探索致病因素（利用分子遗传学，蛋白质组学和生物化学 分析核心）和神经病理生理水平（使用神经影像核心）。

A. OBJECTIVES In the critique of our MRDDRC application in 2001 the study section stated (p.5): behavioral expertise will need to be added to the Center to provide better input into this area at an early stage of grant development. In response to this suggestion we propose to develop the Neurobehavioral Evaluation Core (NEC) that will provide investigators with this expertise. The primary objective of the NEC is to provide sophisticated neurobehavioral and neuropsychological research support to MRDDRC projects, and to develop or validate new methods for this purpose. Scientific advancement of our understanding of complex neurobehavioral disorders of development is facilitated greatly by our ability to reliably and precisely define the developmental and behavioral phenotype. Measurement of behavioral and cognitive variables is crucial in studies of disease and development that comprise our MRDDRC. Over the past 3-5 years, there has been a substantial increase in the number of MRDDRC projects with a behavioral and/or psychosocial focus. The aims of these studies have been on the developmental, cognitive, and behavioral outcomes in children who have a variety of neurogenetic, neurodevelopmental, or acquired disorders that affect cognition and behaivor. In addition to a rigorous study of the underlying medical condition (e.g. urea cycle disorders or epilepsy), these investigations aim to describe the impact on the child's cognitive and behavioral development. Twenty to thirty years ago, this type of research might have focused on the broad aspects of development, such as the level of intelligence of these children. Newer methods allow a far more in depth focus into such critical developmental factors as attention, executive function, and memory subprocesses. The explosion of developmental neuropsychological knowledge base linking underlying neurological development and disorder with behavioral and cognitive development has further fueled the need for pediatric research to include sophisticated models of neurobehavioral and neuropsychological function. Historically, many studies that could have potentially benefited from examination of neuropsychological and neurobehavioral variables have not included such assessments because of: 1) limited knowledge regarding available measures; 2) cost-constraints within research project budgets for expert personnel and/or purchase of testing materials; and 3) lack of access to personnel skilled in the performance of these assessments. The primary goal of the NEC is to improve access to appropriate measures and to enhance the overall quality of neurobehavioral research being conducted throughout the MRDDRC. Within the NEC, we propose to: 1) provide access to an extensive battery of neuropsychological testing services; 2) develop and administer neurobehavioral tasks for neuroimaging protocols, including fMRI projects through the Neuroimaging Core; 3) provide consultation to MRDDRC investigators on the proper design and use of neurobehavioral and neuropsychological measures in research studies; and 4) train investigators and their staff in the use of these measures. With this increased sophistication investigators will be able to assess several levels of neurobehavioral outcome, e.g., specific neuropsychological processes, such as memory or executive function, together with quality of life functional status. This will be able to be coupled with studies exploring etiologic factors at the genetic level (utilizing the Molecular Genetics, Proteomics and Biochemical Analysis Core) and neuropathophysiologic level (using the Neuroimaging Core).