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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 25:1022-1027, June-July 2004
© 2004 American Society of Neuroradiology

PEDIATRICS

Peroxisomal Biogenesis Disorder: Comparison of Conventional MR Imaging with Diffusion-Weighted and Diffusion-Tensor Imaging Findings

Birgitta S. M. ter Rahe^a, Charles B. L. M. Majoie^a, Erik M. Akkerman^a, Gerard J. den Heeten^a, Bwee T. Poll-The^b and Peter G. Barth^b

^a Department of Radiology, Academic Medical Center, Amsterdam, the Netherlands
^b Department of Pediatric Neurology, Academic Medical Center, Amsterdam, the Netherlands

Address correspondence to Birgitta S. M. ter Rahe, MD, Department of Radiology, Academic Medical Center, P.O. Box 22660, 1100 DD Amsterdam, the Netherlands

BACKGROUND AND PURPOSE: Peroxisomal biogenesis disorders (PBDs) refer to a group of disorders of peroxisomal biogenesis causing neuronal migration disorder, delayed myelination, and demyelination. The aim of this study was to evaluate the added value of diffusion-weighted imaging (DWI) and diffusion tensor imaging (DTI) compared with that of conventional T2-weighted imaging in assessing the extent of white matter damage in patients with PBDs.

METHODS: Three patients (aged 12, 16, and 80 months) with PBD (type 1 protein targeting sequence [PTS1]) and three age-matched control subjects underwent MR imaging on a 1.5-T system. The protocol included axial T2-weighted, DWI, and DTI sequences. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) changes were calculated using regions of interest at several predefined white matter areas and compared with those of age-matched control subjects. Color-coded maps were obtained to visualize the range of FA values.

RESULTS: On the T2-weighted images, one patient revealed severe hypomyelination throughout the brain; the two other patients showed focal abnormal high-signal-intensity areas. All patients had significantly decreased FA values in white matter areas that appeared abnormal on the T2-weighted images. In two of the three patients, significant FA reduction was also found in normal-appearing white matter. The ADC values of the patients were significantly increased compared with those of the age-matched controls.

CONCLUSION: Although based on a small number of patients, our data suggest that DWI and DTI can be used to characterize and quantify white matter tract injury in patients with PBD-PTS1. Furthermore, our data suggest that these techniques have the potential to identify neurodegenerative changes not yet visible on T2-weighted images.