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Research Abstracts Online
January 2008 - March 2009

University of Minnesota Twin Cities
Medical School
Department of Pediatrics

PI: Elsa G. Shapiro
Co-PI: Lawrence Charnas

Brain Structure and Function in MPS I, II, and VI

The purpose of this study is to better understand the brain pathophysiology that results in cognitive and behavioral abnormality or decline in mucopolysaccharidoses (MPSs). MPSs are a group of inherited metabolic diseases in which a defective or missing enzyme results in the accumulation of harmful amounts of mucopolysaccharides (complex sugar molecules) in cells and tissues. There are six different types of MPS disorders with varying degrees of severity and a characteristic phenotype. This study includes measures of cognitive ability, attention, memory, and visual-spatial skills as well as acquisition of high-field (3 Tesla) images for volumetric analysis and for diffusion tensor imaging and calculation of fractional anisotropy (FA) values. Preliminary analysis of this data suggests specificity for hippocampal/memory impairment. Poor FA correlates with slow reaction time and poor attention, presumably due to poor white matter connectivity in the brain. However, these preliminary results need to be confirmed including reanalysis of both volumes and FA.

This project uses the FreeSurfer program to generate volumetric data of mesiotemporal structures (hippocampus, amygdala), and other cortical and subcortical regions. This software tool requires high computing performance, thus necessitating the use of MSI resources.

Group Members

Igor Nestrasil, Research Associate
Kathleen Thomas, Faculty Collaborator