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Case Report
BRAIN

Mitochondrial Encephalomyopathy: Comparison of Conventional MR Imaging with Diffusion-Weighted and Diffusion Tensor Imaging: Case Report

Charles B. Majoiea, Erik M. Akkermana, Christian Blankb, Peter G. Barthb, Bwee Tien Poll-Theb and G.J. den Heetena

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

Address reprint requests to Charles B. Majoie, MD, PhD, Department of Radiology, Academic Medical Center, P.O. Box 22660, 1100 DD, Amsterdam, the Netherlands

Summary: Conventional MR imaging, MR spectroscopy, diffusion-weighted imaging, and diffusion tensor imaging were performed in a 5-month-old male patient with mitochondrial encephalomyopathy. On conventional T2-weighted MR images, symmetric, confluent high signal intensity was found in the temporoparietal white matter. A large lactate peak and decreased N-acetylaspartate were found in this region on proton MR spectroscopic images. Diffusion-weighted imaging showed increased apparent diffusion coefficient, representing vasogenic edema. Diffusion tensor imaging revealed decreased anisotropy, consistent with injury to the oligodendro-axonal unit. A muscle biopsy specimen revealed an isolated complex III enzyme respiratory chain deficiency. Diffusion-weighted and diffusion tensor imaging are valuable techniques for the characterization of hyperintense lesions on T2-weighted MR images in cases of mitochondrial encephalomyopathy.




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B. S. M. ter Rahe, C. B. L. M. Majoie, E. M. Akkerman, G. J. den Heeten, B. T. Poll-The, and P. G. Barth
Peroxisomal Biogenesis Disorder: Comparison of Conventional MR Imaging with Diffusion-Weighted and Diffusion-Tensor Imaging Findings
AJNR Am. J. Neuroradiol., June 1, 2004; 25(6): 1022 - 1027.
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