Investigating demyelination in the brain in a canine model of globoid cell leukodystrophy (Krabbe disease) using magnetization transfer contrast: preliminary results.
OBJECTIVE
This study was designed to examine the use of quantitative magnetization transfer imaging (MTI) in naturally occurring globoid cell leukodystrophy (GLD) in the Cairn terrier.


METHODS
A model of GLD was established via a breeding colony, and a total of seven animals were studied with MTI, including two dogs with GLD, one of which underwent whole-body irradiation (725 cGy) and bone marrow transplantation from a genotypically normal littermate. The remaining dogs served as untreated, irradiated, and unirradiated controls.


RESULTS
Region-of-interest (ROI) analysis of the MTI showed a decrease in MT ratio (MTR) in the internal capsule of the untreated/affected dog compared with age-matched controls but revealed similar results in the two other study animals. On MT contour plotting, inside-to-out gradients of MTR mimicked the demyelination pathology of the disease in the untreated/affected dog.


CONCLUSIONS
MT contour plotting demonstrated patterns of MT abnormality in the untreated/affected dog that were consistent with histopathology, establishing a clear relationship between pathology-proven demyelination and MTR as well as a striking contrast to the patterns of radiation damage.
McGowan
J C
JC
Department of Radiology, School of Medicine, University of Pennsylvania, Philadelphia 19104, USA. jmcgowan@seas.upenn.edu
Haskins
M
M
Wenger
D A
DA
Vite
C
C
D000818:Animals; D001522:Behavior, Animal; D016026:Bone Marrow Transplantation; D003711:Demyelinating Diseases / Q000175:diagnosis / Q000628:therapy; D004195:Disease Models, Animal; D018450:Disease Progression; D004285:Dogs; D005240:Feasibility Studies; D005260:Female; D007965:Leukodystrophy, Globoid Cell / Q000175:diagnosis* / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D009186:Myelin Sheath / Q000473:pathology; D011237:Predictive Value of Tests; D014916:Whole-Body Irradiation