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


Brain Structure and Function in MPS I, II, III, 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 head MRI’s on 3 Tesla scanners for volumetric analysis and for diffusion tensor imaging. The researchers are now in their fourth year of the longitudinal study. The researchers use MSI resources for longitudinal analysis of MRI data using Freesurfer and FSL programs, and recently started running surface-based statistical analysis using Avizo-generated surfaces. All these analyses require high computer processing capabilities.

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