This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Neurofibromatosis Type 1 (NF-1) is one of the most common genetic disorders affecting the nervous system. Individuals with NF-1 manifest pigmentary abnormalities as well as benign and malignant tumors. Although NF-1 commonly is regarded as an inherited predisposition to cancer syndrome, some of the most prominent features of this disorder are not attributable directly to tumor formation. As an example, cognitive dysfunction or learning disabilities occur in up to 40% of children with NF-1 - six to seven times the incidence seen in the general population. Not only do these children have cognitive difficulties, but they also suffer from behavioral disturbances and a large percentage have attention deficit/hyperactivity disorder. The etiology for these learning disabilities and behavioral disturbances is unclear. With the advent of neuroimaging, there has been some movement towards research in understanding the neurobiology of learning disabilities in NF-1. However, this body of literature is still very small with only a handful of studies using state of the art Magnetic Resonance Imaging (MRI), and many using small samples which were poorly matched for age and gender. Using state of the art image processing tools, MRI scans will be collected on school-aged children with NF-1. Quantitative measures of brain structure will then be compared to those of healthy controls. In addition, cognitive and behavioral measures will be obtained using neuropsychological tests on both groups. Finally, the relationship between regional brain structure and brain function, quantitative measures from the MRI scans will be correlated with performance on the cognitive tests.
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