Peroxisomal Biogenesis Disorder: Comparison of Conventional MR Imaging with Diffusion-Weighted and Diffusion-Tensor Imaging Findings
Birgitta S. M. ter Rahea,
Charles B. L. M. Majoiea,
Erik M. Akkermana,
Gerard J. den Heetena,
Bwee T. Poll-Theb and
Peter G. Barthb
a Department of Radiology, Academic Medical Center, Amsterdam, the Netherlands
b Department of Pediatric Neurology, Academic Medical Center, Amsterdam, the Netherlands
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FIG 1. MR images obtained in a 12-month-old boy with biopsy-proved PBD-PTS1 (A-D) and in an age-matched girl with mental retardation (E and F).
A and B, Axial fast spin-echo T2-weighted images (3500/90/1 [TR/TE/NEX]) obtained in a 12-month-old boy, showing diffuse signal intensity increase in the white matter on the level of the anterior limb and genu of the internal capsules and the corona radiata bilaterally, which is consistent with hypomyelination. Posterior limbs of the bilateral internal capsules show hypomyelination. Cortical dysplasia along the border of the right sylvian fissure is present, as can be seen in PBDs (arrows).
C and D, Color-coded FA maps, demonstrating decreased anisotropy in the white matter at the same levels in this patient as those in the control subject (E and F).
E and F, Color-coded FA maps of the age-matched control subject of the corresponding levels as in panels C and D.
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FIG 2. Images obtained in a 16-month-old girl with biopsy-proved PBD-PTS1 (A–D) and a control subject, an age-matched boy with mental retardation (E and F).
A and B, Axial fast spin-echo T2-weightedimages (3500/90/1), showing high signal intensity in the parietal white matter bilaterally, but normal signal intensity in the bilateral internal capsules, the corpus callosum, and the corona radiata bilaterally.
C and D, Color-coded FA maps, demonstrating decreased anisotropy in the NAWM and abnormal-appearing white matter at the same levels as A and B, as compared with findings in the control subjects (E and F).
E and F, Color-coded FA maps of the age-matched control of the corresponding areas to C and D.
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