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Krabbe disease treated with hematopoietic stem cell transplantation: serial assessment of anisotropy measurements--initial experience.

Publication ,  Journal Article
McGraw, P; Liang, L; Escolar, M; Mukundan, S; Kurtzberg, J; Provenzale, JM
Published in: Radiology
July 2005

PURPOSE: To prospectively compare diffusion-tensor magnetic resonance (MR) imaging anisotropy measurements of white matter (WM) regions in early and late treatment groups of Krabbe disease patients treated with stem cell transplantation. MATERIALS AND METHODS: The study was approved by the Institutional Review Board and was compliant with Health Insurance Portability and Accountability Act; informed consent was obtained from the families of all patients. Patients with early-onset Krabbe disease (four girls and three boys) underwent diffusion-tensor MR imaging before and after stem cell transplantation. Fractional anisotropy (FA) values from serial studies were compared in patients who underwent transplantation at less than 1 month (early group, two girls and one boy) and those who underwent transplantation at 5-8 months (late group, two girls and two boys). FA values were measured in the genu and splenium of the corpus callosum, the frontal WM, and the internal capsule; were compared with those of five age-matched children in the comparison group (normal MR images and no proved neurologic disease); and were expressed as a ratio. Images obtained after transplantation were evaluated at approximately 1 (n = 7), 2 (n = 6), 3 (n = 1), and 4 (n = 1) years. RESULTS: Before transplantation, mean FA ratios in the early group for all four WM regions ranged between 97% and 117%. At 1 year, mean FA ratios at all locations were either 92% or 93%. At 2 years after transplantation, mean FA ratios were between 83% and 92%. In one patient imaged at 3 years, the mean FA ratio was 97%; in another patient imaged at 4 years, the mean FA ratio was 77%. Before transplantation, mean FA ratios in the late group ranged between 55% and 74%. Mean FA ratios were between 37% and 50% at 1 year after transplantation and between 36% and 39% at 2 years. CONCLUSION: All patients had decreases in FA ratios over time. The early group had higher initial FA ratios and lower subsequent decreases, which may indicate amelioration of the dysmyelinating process.

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Published In

Radiology

DOI

ISSN

0033-8419

Publication Date

July 2005

Volume

236

Issue

1

Start / End Page

221 / 230

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Prospective Studies
  • Nuclear Medicine & Medical Imaging
  • Male
  • Leukodystrophy, Globoid Cell
  • Infant
  • Humans
  • Hematopoietic Stem Cell Transplantation
  • Female
  • Diffusion Magnetic Resonance Imaging
 

Citation

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McGraw, P., Liang, L., Escolar, M., Mukundan, S., Kurtzberg, J., & Provenzale, J. M. (2005). Krabbe disease treated with hematopoietic stem cell transplantation: serial assessment of anisotropy measurements--initial experience. Radiology, 236(1), 221–230. https://doi.org/10.1148/radiol.2353040716
McGraw, Peter, Luxia Liang, Maria Escolar, Srinivasan Mukundan, Joanne Kurtzberg, and James M. Provenzale. “Krabbe disease treated with hematopoietic stem cell transplantation: serial assessment of anisotropy measurements--initial experience.Radiology 236, no. 1 (July 2005): 221–30. https://doi.org/10.1148/radiol.2353040716.
McGraw P, Liang L, Escolar M, Mukundan S, Kurtzberg J, Provenzale JM. Krabbe disease treated with hematopoietic stem cell transplantation: serial assessment of anisotropy measurements--initial experience. Radiology. 2005 Jul;236(1):221–30.
McGraw, Peter, et al. “Krabbe disease treated with hematopoietic stem cell transplantation: serial assessment of anisotropy measurements--initial experience.Radiology, vol. 236, no. 1, July 2005, pp. 221–30. Pubmed, doi:10.1148/radiol.2353040716.
McGraw P, Liang L, Escolar M, Mukundan S, Kurtzberg J, Provenzale JM. Krabbe disease treated with hematopoietic stem cell transplantation: serial assessment of anisotropy measurements--initial experience. Radiology. 2005 Jul;236(1):221–230.
Journal cover image

Published In

Radiology

DOI

ISSN

0033-8419

Publication Date

July 2005

Volume

236

Issue

1

Start / End Page

221 / 230

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Prospective Studies
  • Nuclear Medicine & Medical Imaging
  • Male
  • Leukodystrophy, Globoid Cell
  • Infant
  • Humans
  • Hematopoietic Stem Cell Transplantation
  • Female
  • Diffusion Magnetic Resonance Imaging