Neurology
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Published online before print May 17, 2006, doi:10.1212/01.wnl.0000217913.37108.94)
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Received September 26, 2005
Accepted February 24, 2006

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

Raymond Yen-Yu Lo MD, Woei Cherng Shyu MD, PhD, and Hung Li PhD*

From the Department of Neurology (R.Y.-Y.L., W.C.S.), Neuro-Medical Scientific Center, Buddhist Tzu-Chi General Hospital, Tzu-Chi University, Hualien, Taiwan; and Institute of Molecular Biology (H.L.), Academia Sinica, Taipei, Taiwan; Institute of Biochemistry (H.L.), National Yang-Ming University, Taipei, Taiwan.


* To whom correspondence should be addressed. E-mail: hungli{at}ccvax.sinica.edu.tw.

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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