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

Lo, Raymond Yen-Yu; Shyu, Woei Cherng; Li, Hung · Neurology · 2006

case_report · Level V

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Abstract

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.

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