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  • Title: Long-duration sCJD with PRNP codon 129 methionine homozygosity and cerebral cortical plaques.
    Author: Lo RY, Shyu WC, Li H.
    Journal: Neurology; 2006 Jun 27; 66(12):1944-5. PubMed ID: 16707728.
    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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