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Abstract #1297

Diffusion tensor imaging to assess gray and white matter microstructural brain abnormalities in a feline model of alpha-mannosidosis

Manoj Kumar 1 , Jeff T Duda 1 , Sea-Young Yoon 2 , Jessica Bagel 3 , Patricia ODonnell 3 , Charles Vite 3 , Stephen Pickup 1 , James C Gee 1 , John H Wolfe 2 , and Harish Poptani 1

1 Radiology, University of Pennsylvania, Philadelphia, Pennsylvania, United States, 2 Research Institute of the Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States, 3 School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA, United States, Pennsylvania, United States

Alpha-mannosidosis is an autosomal recessively inherited lysosomal storage disorder, caused by deficiency of the enzyme alpha mannosidase. We performed ex-vivo diffusion tensor imaging (DTI) and in vivo DTI on AMD affected and wild type cat brains to assess brain abnormalities. A multi-atlas approach was used to generate a brain template and process the ex vivo DTI data. The probabilistic label method was used to measure fractional anisotropy (FA) and mean diffusivity (MD) values from gray and white matters from whole brain. Region of interest based DTI data analysis was also from ex vivo and in vivo DTI from six gray and five white matter regions. Significantly reduced FA was observed from both the ex vivo and in vivo DTI data from all the white matter in AMD affected cats may be suggestive of gliosis or demyelination. Some of the analyzed gray matter also demonstrates significantly reduced FA in AMD affected cats.

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