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

J Comput Assist Tomogr. 2000 Mar-Apr;24(2):316-21. doi: 10.1097/00004728-200003000-00025.

Abstract

Purpose: 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.

Method: 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.

Conclusion: 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.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Behavior, Animal
  • Bone Marrow Transplantation
  • Demyelinating Diseases / diagnosis
  • Demyelinating Diseases / therapy
  • Disease Models, Animal
  • Disease Progression
  • Dogs
  • Feasibility Studies
  • Female
  • Leukodystrophy, Globoid Cell / diagnosis*
  • Leukodystrophy, Globoid Cell / therapy
  • Magnetic Resonance Imaging*
  • Male
  • Myelin Sheath / pathology
  • Predictive Value of Tests
  • Whole-Body Irradiation