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Long-duration sCJD with PRNP codon 129 methionine homozygosity and cerebral cortical plaques.

Lo RY, Shyu WC, Li H

Department of Neurology, Neuro-Medical Scientific Center, Buddhist Tzu-Chi General Hospital, Tzu-Chi University, Hualien, Taiwan.

The authors investigated a 40-year-old woman who presented with ataxia and dementia with little progression for over 40 months. The results of a CSF 14-3-3 protein and EEG study did not reveal major abnormalities. Brain MRI showed increased signal intensity over the occipital cortex in diffusion-weighted imaging. To our knowledge, this is the longest MM-type sporadic Creutzfeldt-Jakob disease case with cortical kuru-type plaques.

Published 27 June 2006 in Neurology, 66(12): 1944-5.
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