Investigating demyelination in the brain in a canine model of globoid cellleukodystrophy (Krabbe disease) using magnetization transfer contrast: Preliminary results

Purpose: This study was designed to examine the use of quantitative magneti zation transfer imaging (MTI) in naturally occurring globoid cell leukodyst rophy (GLD) in the Cairn terrier. Method: A model of GLD was established via a breeding colony, and a total o f seven animals were studied with MTI, including two dogs with GLD, one of which. underwent whole-body irradiation (725 cGy) and bone marrow transplan tation from a genotypically normal littermate. The remaining dogs served as untreated, irradiated, and unirradiated controls. Results: Region-of-interest (ROT) analysis of the MTI: showed a decrease in NT ratio (MTR) in the internal capsule of the untreated/affected dog compa red with age-matched controls but revealed similar results in the two other study animals. On MT contour plotting, inside-to-out gradients of MTR mimi cked the demyelination pathology of the disease in the untreated/affected d og. Conclusion: MT contour plotting demonstrated patterns of MT abnormality in the untreated/affected dog that were consistent with histopathology, establ ishing a clear relationship between pathology-proven demyelination and MTR as well as a striking contrast to the patterns of radiation damage.