Quantitative Analysis of Diffusion Tensor Imaging Data in Serial Assessment of Krabbe Disease

Krabbe disease is a rare autosomal recessive pediatric white matter (WM) disorder that is due to deficiency of a specific enzyme, beta-galactocerebrosidase. This report reviews our experience with use of diffusion tensor imaging (DTI) in serial assessment of WM changes in Krabbe disease following stem cell transplantation. DTI appears to be a sensitive means to monitor effects of stem cell transplantation on WM development in Krabbe disease. The group of early transplantation infants was clearly distinguishable from the group of late transplantation infants based on anisotropy measurements. Good correlation also was seen between neurodevelopmental scores and anisotropy measurements. The work described here in Krabbe disease may serve as a model for application of DTI to other therapies in various WM disorders such as multiple sclerosis and dysmyelinating disorders of childhood.