Novel diffusion tensor imaging findings in Krabbe disease |
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Affiliation: | 1. Section 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;2. Department of Radiology, Faculty of Medicine, University of Manitoba, Winnipeg, Manitoba, Canada;3. Kennedy Krieger Institute, Baltimore, MD, USA;4. Department of Neurology, The Johns Hopkins School of Medicine, Baltimore, MD, USA;5. Department of Diagnostic Imaging, Health Sciences Centre, Winnipeg, Manitoba, Canada;6. Section of Pediatric Neurology, Department of Pediatrics and Child Health, Faculty of Medicine, University of Manitoba, Winnipeg, Manitoba, Canada;1. Division of Child and Adolescent Neurology, Mayo Clinic, Rochester, Minnesota;2. Department of Radiology, Mayo Clinic, Rochester, Minnesota;1. Department of Pediatric Neurology, Central Hospital, Aichi Human Service Center, Kasugai, Aichi, Japan;2. Department of Pediatrics, Okazaki City Hospital, Okazaki, Aichi, Japan;3. Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan;4. Department of Pediatrics, Anjo Kosei Hospital, Anjo, Aichi, Japan;5. Department of Pediatrics and Neonatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi, Japan;6. Department of Pediatrics, Juntendo University Faculty of Medicine, Bunkyo-ku, Tokyo, Japan;7. Department of Pediatrics, Aichi Medical University, Nagakute, Aichi, Japan;1. Division of Neonatal-Perinatal Medicine, The Carman and Ann Adams Department of Pediatrics, Wayne State University School of Medicine, Detroit Medical Center, Children''s Hospital of Michigan, Detroit, Michigan;2. Department of Pediatric Radiology, Wayne State University School of Medicine, Detroit Medical Center, Children''s Hospital of Michigan, Detroit, Michigan;1. Department of Neurology, Mayo Clinic, Rochester, Minnesota;2. Department of Medical Genetics, Mayo Clinic, Rochester, Minnesota;3. Department of Pediatrics, Mayo Clinic, Rochester, Minnesota |
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Abstract: | BackgroundKrabbe disease is a lysosomal disorder that primarily affects myelin. Diffusion tensor imaging (DTI) provides quantitative information about the white matter organization and integrity. Radial diffusivity (RD) reflects myelin injury selectively.PurposeTo report on quantitative DTI findings (including axial diffusivity (AD) and RD, not previously reported) in two children with Krabbe disease compared to controls.MethodsA quantitative region of interest (ROI) based DTI analysis was performed for the patients and age- and gender-matched controls. Fractional anisotropy (FA), mean diffusivity, AD and RD values as well as variation ratios between the patients' and controls' values were calculated for nine brain regions.ResultsTwo boys with Krabbe disease were included in this study. DTI data were acquired at the ages of 6.25 years and 6.5 months. For all regions, FA ratios were negative, while RD and MD ratios positive. The most elevated variation ratios were found for RD. Variation ratios were greater in the centrum semiovale, corpus callosum, and middle cerebellar peduncles than in other anatomical regions, especially in the older patient in comparison with the younger patient. The AD ratios, however, were much lower and close to zero.ConclusionsDTI allows a quantitative evaluation of white matter damage in Krabbe disease. RD seems to be the most sensitive DTI parameter in agreement with the histopathological findings in Krabbe disease, a primary myelin disorder. This may be important in the early detection of the onset of demyelination. |
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Keywords: | Krabbe disease Magnetic resonance imaging Diffusion tensor imaging Radial diffusivity |
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