The general goals of the project are to, a) Investigate the nature of neuropsychological impairments in children with neurofibromatosis, type I (NF), a genetic disorder with a high incidence of academic impairment and neuropathology; b) Investigate the underlying neuroanatomical basis of these impairments, and c) Determine the added contribution of the diagnosis of a brain tumor to the cognitive status of children with NF. Research will be directed towards 3 specific aims: 1) Identify domains of cognitive impairment in children with NF and determine how these patterns are associated with deficits in learning and academic achievement. 2) Determine the underlying anatomical basis of cognitive impairment. 3) Document the added effect of a diagnosis of brain tumor on neuropsychological functioning. Three groups will be studied in a cross-sectional design at a single timepoint: Children with NF only; children with NF pius a brain tumor; and normal healthy children. Data will be obtained from all study participants from two sources: a) Neuropsychological: A comprehensive battery of tests assessing intellectual, academic achievement, and cognitive functioning; b) Anatomical: MRI scans will undergo quantitative morphometric analyses to determine the location and volume of areas of high signal intensity commonly found in children with NF and the volume of cerebral white matter. Children who are between 6 and 12 years old at the time of enrollment will be eligible for the study which will requires years to complete. Wherever possible, depending on the nature of the hypothesis being evaluated, analyses will be multivariate, taking into account the multiple outcome measures used to evaluate a specific hypothesis. Qualitative indices from the neuroimaging domain will be analyzed using appropriate methods for categorical data analysis to examine group differences. Quantitative indices will be analyzed using MANOVA. Evaluating the neuroanatomical basis for cognitive deficits in NF will be accomplished using multiple regression and correlation. Knowledge from this study will provide information about cognitive impairments in children in general and suggest avenues of research into their biological bases and possibilities for their remediation.

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Project (R01)
Project #
5R01NS031950-04
Application #
2735632
Study Section
Special Emphasis Panel (ZRG1-HUD-1 (01))
Program Officer
Broman, Sarah H
Project Start
1995-09-15
Project End
1999-11-30
Budget Start
1998-07-01
Budget End
1999-11-30
Support Year
4
Fiscal Year
1998
Total Cost
Indirect Cost
Name
University of Texas MD Anderson Cancer Center
Department
Pediatrics
Type
Other Domestic Higher Education
DUNS #
001910777
City
Houston
State
TX
Country
United States
Zip Code
77030
Moore, Bartlett D (2009) Potential influences on mathematical difficulties in children and adolescents with neurofibromatosis, type 1. Dev Disabil Res Rev 15:45-51
Billingsley, Rebecca L; Jackson, Edward F; Slopis, John M et al. (2004) Functional MRI of visual-spatial processing in neurofibromatosis, type I. Neuropsychologia 42:395-404
Billingsley, Rebecca L; Jackson, Edward F; Slopis, John M et al. (2003) Functional magnetic resonance imaging of phonologic processing in neurofibromatosis 1. J Child Neurol 18:731-40
Billingsley, Rebecca L; Slopis, John M; Swank, Paul R et al. (2003) Cortical morphology associated with language function in neurofibromatosis, type I. Brain Lang 85:125-39
Schrimsher, Gregory W; Billingsley, Rebecca L; Slopis, John M et al. (2003) Visual-spatial performance deficits in children with neurofibromatosis type-1. Am J Med Genet A 120A:326-30
Schrimsher, Gregory W; Billingsley, Rebecca L; Jackson, Edward F et al. (2002) Caudate nucleus volume asymmetry predicts attention-deficit hyperactivity disorder (ADHD) symptomatology in children. J Child Neurol 17:877-84
Billingsley, Rebecca L; Schrimsher, Gregory W; Jackson, Edward F et al. (2002) Significance of planum temporale and planum parietale morphologic features in neurofibromatosis type 1. Arch Neurol 59:616-22
Moore 3rd, B D; Slopis, J M; Jackson, E F et al. (2000) Brain volume in children with neurofibromatosis type 1: relation to neuropsychological status. Neurology 54:914-20
Kayl, A E; Moore 3rd, B D (2000) Behavioral phenotype of neurofibromatosis, type 1. Ment Retard Dev Disabil Res Rev 6:117-24
Kayl, A E; Moore 3rd, B D; Slopis, J M et al. (2000) Quantitative morphology of the corpus callosum in children with neurofibromatosis and attention-deficit hyperactivity disorder. J Child Neurol 15:90-6

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