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Abstract #0907

Diffusion Tensor Imaging for Assessment of Brain Structural Abnormalities in the Mucopolysaccharidosis VII Murine Model

Manoj Kumar1, Ilya M. Nasrallah1, Sungheon Kim2, Ranjit P. Ittyerah1, John H. Wolfe3, 4, Stephen Pickup1, Harish Poptani1

1Radiology, University of Pennsylvania, Philadelphia, PA, United States; 2Radiology, New York University, NY, United States; 3Pathobiology and Pediatrics, University of Pennsylvania, Philadelphia, United States; 4Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, United States

In-vivo and Ex-vivo diffusion tensor imaging (DTI) was performed on 22 week old MPS VII (n=6) and wild-type littermates (n=5) to assess abnormalities in the white matter areas of MPS VII mice. DTI data was processed and region of interest (ROI) analysis was performed from different gray and white matter regions of the brain using DTI studio software. Significantly reduced fractional anisotropy (FA) in corpus callosum and external capsule was observed in MPS VII affected mice as compared to wild type indicating reduced myelination. Luxol fast blue stains from these regions confirmed the presence of less compact and myelinated fibers in MPS VII brains. This preliminary data demonstrates the utility of DTI in observing microstructural abnormalities in brain of MPS VII mice. The study may be helpful in understanding the pathology of MPS VII and in assessing therapeutic response in these mice using DTI as a surrogate imaging biomarkers.