A. Objective The primary objectives of the Neurobehavioral Evaluation Core (NEC) are (a) to provide sophisticated neurobehavioral/ neuropsychological research support to IDDRC projects, and (b) to develop/validate new measures and methods for this purpose. Scientific advancement of our understanding of complex neurobehavioral disorders of development is greatly facilitated by our ability to define reliably and precisely developmental and neurobehavioral phenotypes. Measurement of neurobehavioral and cognitive variables is crucial in studies of both disease and normal development and over the past 5 years, the NEC has supported 38 IDDRC projects resulting in 54 peer-reviewed publications.
The aims of these studies have focused on developmental, cognitive, and behavioral outcomes in children who have a variety of neurogenetic, neurodevelopmental, or acquired neurological disorders that affect cognition and behavior. In addition to a rigorous study of the underlying medical condition (e.g. urea cycle disorders or epilepsy. Table 1: Project#9,13), these investigations aim to describe the impact on the child's neurocognitive and neurobehavioral development. Newer neuropsychological tests and methods allow a more in depth focus into cognitive developmental factors that include attention, executive function, speed of processing, and memory. In addition, the explosion of the developmental neuropsychological knowledge base linking underlying neurological developmental disorders with behavioral and cognitive development has further fueled the need for pediatric research to include sophisticated models of neurobehavioral and/or neuropsychological function. In turn, this requires the continued development of new methods to measure neuropsychological functioning. The primary goal of the NEC is to improve access to state of the art measures and to enhance the overall quality of neurobehavioral research being conducted throughout the IDDRC. The NEC supports and enhances studies that can benefit from the examination of neuropsychological and neurobehavioral variables, enhancing our understanding of the functional outcomes of medical and neurological diseases and disorders. The Core is able to bring state of the art methods and measures to these studies, improving efficiency by providing access to expert personnel and/or testing materials that maximize cost effectiveness. The NEC provides (1) access to an extensive battery of neuropsychological testing services, (2) consultation to IDDRC investigators on the proper design, use and interpretation of neurobehavioral/neuropsychological measures in research studies, (3) training of investigators and their staff in the use of these measures, and (4) assistance in the administration of neurobehavioral tasks. This increased sophistication in neurobehavioral assessment allows investigators to assess specific, yet complex, neuropsychological processes. These methods can be joined with studies exploring etiologic factors at the genetic level (utilizing the Molecular Genetics Core) and neuropathophysiologic level (using the Neuroimaging Core). As our Core is also involved in developing novel test materials, especially those focusing on executive function, memory and processing speed, the Core benefits the entire IDDRC network. Several issues were raised regarding the NEC in the 2006 renewal that have been explicitly addressed. These were: i) time allocation and prioritizing services;ii) specifics regarding collaboration with other Cores, and iii) testing provided in other languages than English. Time Allocation As a new Core established in 2006, the NEC has actively worked to establish its role and presence with a variety of research protocols. In order to deliver the array of services, especially direct testing support, we prioritize studies in terms of the particular study's needs and Core resources. Each study is evaluated with respect to its needs from the Core. The first service option is study consultation. The next level of service involves training staff on specific neuropsychological tests or methods. Some studies may need greater direct service, requiring the third service option, which is assisting with data collection. Finally, some studies, such as a K award with fewer resources, will receive the fourth service option, a relatively greater amount of direct service. A study with greater resources (R01 or center grant) would receive less direct service and more consultative/training service. Studies with lower service needs (e.g., multi-center study with a low number and/or sporadic recruitment of study participants, such as the Cool Kids protocol. Table 1: Project#37) would also receive direct service, assuming no direct study staff to perform the testing is available. Finally, projects that require a significant and ongoing direct involvement of the NEC staff provide support within the grant for this involvement, allowing the NEC to expand its staff to provide this service (e.g. Table 1, Proj. 9). Collaboration with Other Cores There is a strong collaboration between the NEC and a number of the other IDDRC Cores. NEC develops activation task protocols with the NI Core and works closely with the BI Core in validating new studies. Many of the genetically based projects involve close collaboration between the GP Core and NEC to establish genotype/phenotype relationships. In many of these studies, key clinical outcomes involve neurobehavioral or neuropsychological performance, necessitating reliable and valid measures. These collaborations have markedly advanced since the establishment of the NEC 4 years ago. Testing in Other Languages This is clearly a challenging issue in all of research as many of the standard neuropsychological tests have limited validated foreign language translations. In our DC population, the principal second language is Spanish. The NEC has purchased and maintained Spanish translations for a variety of tests and protocols. In addition, there is the availability of a Spanish-English bilingual neuropsychologist within the Division of Pediatric Neuropsychology for consultation on these issues when necessary. For verbal interviews in a foreign language, a translation service can be accessed. Nevertheless, valid neuropsychological/cognitive assessment of non-English speakers remains a challenge for many IDDRC studies, and the NEC plays an important role in educating investigators about the challenges and advising them on their options. For some studies, the only realistic option is to require English fluency.

National Institute of Health (NIH)
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Center Core Grants (P30)
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Special Emphasis Panel (ZHD1-DSR-Y)
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Children's Research Institute
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Washington, Stuart D; Gordon, Evan M; Brar, Jasmit et al. (2014) Dysmaturation of the default mode network in autism. Hum Brain Mapp 35:1284-96
Berl, Madison M; Zimmaro, Lauren A; Khan, Omar I et al. (2014) Characterization of atypical language activation patterns in focal epilepsy. Ann Neurol 75:33-42
Hammond, Timothy R; Gadea, Ana; Dupree, Jeff et al. (2014) Astrocyte-derived endothelin-1 inhibits remyelination through notch activation. Neuron 81:588-602
Gallo, Vittorio (2014) Lethal migration: the bradykinin story. J Physiol 592:4805-6
Helman, Guy; Pacheco-Colón, Ileana; Gropman, Andrea L (2014) The urea cycle disorders. Semin Neurol 34:341-9
Evans, Tanya M; Flowers, D Lynn; Napoliello, Eileen M et al. (2014) The functional anatomy of single-digit arithmetic in children with developmental dyslexia. Neuroimage 101:644-52
Henriques-Pons, Andrea; Yu, Qing; Rayavarapu, Sree et al. (2014) Role of Toll-like receptors in the pathogenesis of dystrophin-deficient skeletal and heart muscle. Hum Mol Genet 23:2604-17
Agematsu, Kota; Korotcova, Ludmila; Scafidi, Joseph et al. (2014) Effects of preoperative hypoxia on white matter injury associated with cardiopulmonary bypass in a rodent hypoxic and brain slice model. Pediatr Res 75:618-25
Defour, Aurelia; Sreetama, S C; Jaiswal, Jyoti K (2014) Imaging cell membrane injury and subcellular processes involved in repair. J Vis Exp :
Croft, Louise J; Baldeweg, Torsten; Sepeta, Leigh et al. (2014) Vulnerability of the ventral language network in children with focal epilepsy. Brain 137:2245-57

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