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Novel diffusion tensor imaging findings in Krabbe disease.

Andrea Poretti1, Avner Meoded1, Martin Bunge2

  • 1Section of Pediatric Neuroradiology, Division of Pediatric Radiology, The Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins School of Medicine, Baltimore, MD, USA.

European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society
|October 24, 2013
PubMed
Summary

Diffusion tensor imaging (DTI) reveals significant white matter damage in Krabbe disease. Radial diffusivity (RD) is the most sensitive DTI measure, indicating myelin injury and aiding early detection of demyelination.

Keywords:
Diffusion tensor imagingKrabbe diseaseMagnetic resonance imagingRadial diffusivity

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Area of Science:

  • Neuroimaging
  • Biomarkers
  • Lysosomal Storage Disorders

Background:

  • Krabbe disease is a severe lysosomal storage disorder impacting myelin.
  • Diffusion Tensor Imaging (DTI) offers quantitative insights into white matter integrity.
  • Radial diffusivity (RD) specifically detects myelin damage.

Observation:

  • This study analyzed DTI metrics, including axial diffusivity (AD) and RD, in two pediatric Krabbe disease patients.
  • Quantitative region of interest (ROI) DTI analysis compared patients to age- and gender-matched controls.
  • DTI data were acquired from patients at 6.25 years and 6.5 months of age.

Findings:

  • Krabbe disease patients showed negative fractional anisotropy (FA) and positive mean diffusivity (MD) and RD ratios across brain regions.
  • RD exhibited the most elevated variation ratios, particularly in the centrum semiovale, corpus callosum, and middle cerebellar peduncles.
  • Axial diffusivity (AD) ratios were minimal, close to zero, suggesting specific white matter damage patterns.

Implications:

  • DTI provides a quantitative method for assessing white matter damage in Krabbe disease.
  • Elevated RD strongly correlates with myelin pathology, aligning with Krabbe disease's primary demyelination.
  • This quantitative DTI approach may facilitate early detection of demyelination onset in Krabbe disease.