Publication Type Academic Article
Authors Kumar M, Nasrallah I, Kim S, Ittyerah R, Pickup S, Li J, Parente M, Wolfe J, Poptani H
Journal J Neuropathol Exp Neurol
Volume 73
Issue 1
Pagination 39-49
Date Published 01/01/2014
ISSN 1554-6578
Keywords Brain, Diffusion Tensor Imaging, Disease Models, Animal, Mucopolysaccharidosis VII
Abstract High-resolution microscopic magnetic resonance imaging (μMRI) and diffusion tensor imaging (DTI) were performed to characterize brain structural abnormalities in a mouse model of mucopolysaccharidosis type VII (MPS VII). Microscopic magnetic resonance imaging demonstrated a decrease in the volume of anterior commissure and corpus callosum and a slight increase in the volume of the hippocampus in MPS VII versus wild-type mice. Diffusion tensor imaging indices were analyzed in gray and white matter. In vivo and ex vivo DTI demonstrated significantly reduced fractional anisotropy in the anterior commissure, corpus callosum, external capsule, and hippocampus in MPS VII versus control brains. Significantly increased mean diffusivity was also found in the anterior commissure and corpus callosum from ex vivo DTI. Significantly reduced linear anisotropy was observed from the hippocampus from in vivo DTI, whereas significantly decreased planar anisotropy and spherical anisotropy were observed in the external capsule from only ex vivo DTI. There were corresponding morphologic differences in the brains of MPS VII mice by hematoxylin and eosin staining. Luxol fast blue staining demonstrated less intense staining of the corpus callosum and external capsule; myelin abnormalities in the corpus callosum were also demonstrated quantitatively in toluidine blue-stained sections and confirmed by electron microscopy. These results demonstrate the potential for μMRI and DTI for quantitative assessment of brain pathology in murine models of brain diseases.
DOI 10.1097/NEN.0000000000000023
PubMed ID 24335527
PubMed Central ID PMC4120119
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