“In Vivo Assessment of Neurodegeneration in Niemann-Pick Type C Mice by Quantitative T2 Mapping and Diffusion Tensor Imaging”, by John W. Totenhagen, Silvia Lope-Piedrafita, Ivan A. Borbon, Eriko S. Yoshimaru, Robert P. Erickson, and Theodore P. Trouard; Journal of Magnetic Resonance Imaging 35 (2012), 528-536. doi: 10.1002/jmri.22837
NIEMANN-PICK TYPE C (NPC) disease is a rare genetic neurodegenerative disease with no cure and few effective options for treatment. Diagnosis is most often made during childhood with symptoms progressively worsening and leading to death prior to adulthood. The purpose of this work was to quantitatively and noninvasively assess neurological disease progression in a mouse model of NPC disease by measuring white matter status with magnetic resonance imaging (MRI) techniques of T2 mapping and diffusion tensor imaging (DTI) at multiple timepoints. The parameters obtained from these measurements were compared to histological measures of myelination. The findings of this study suggest that quantitative MRI measurements may be useful in vivo biomarkers for future studies of disease progression and proposed treatments in NPC disease models.