Images in Neurology |

Early Abnormality of Diffusion-Weighted Magnetic Resonance Imaging Followed by Brain Atrophy in a Case of Gerstmann-Sträussler-Scheinker Disease

Shiro Yamamoto, MD; Makoto Kinoshita, MD; Satoshi Furukawa, MD; Koji Kajiyama, MD, PhD
Arch Neurol. 2007;64(3):450-451. doi:10.1001/archneur.64.3.450.
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A 72-year-old man presented with a 1-year history of progressive bilateral limb weakness, aphasia, and apathy. Diffusion-weighted magnetic resonance imaging demonstrated hyperintense signal change in the frontal, temporal, occipital, and parietal cortical gyri of the bilateral hemisphere (Figure A) although computed tomographic scan showed no abnormalities (Figure B). The results from cerebrospinal fluid examination were normal except for the elevation of neuron-specific enolase levels (47ng/mL); analysis for 14-3-3 protein was also positive. Prion protein gene analysis revealed a mutation of proline to leucine at codon 102.

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Diffusion-weighted magnetic imaging (A) revealed hyperintensity in the cortical gyri of the bilateral hemisphere although computed tomographic scan (B) showed no abnormality. Computed tomographic scans performed 2 (C) and 8 months later (D) indicated remarkable progression of cortical atrophy and subcortical hypodense lesions.

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