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Research Abstracts Online
January 2009 - March 2010

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University of Minnesota Twin Cities
Medical School
Department of Pediatrics

PI: Elsa G. Shapiro

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 in a larger cohort of patients. The researchers also will track these patients yearly to map disease progression.

Group Member

Igor Nestrasil, Research Associate