Neurology
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Published online before print May 17, 2006, doi:10.1212/01.wnl.0000217913.37108.94)
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NEUROLOGY 2006;66:1944-1945
© 2006 American Academy of Neurology
Brief Communications

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.

Address correspondence and reprint requests to Dr. Hung Li, Institute of Molecular Biology, Academia Sinica, Taipei, Taiwan; e-mail: hungli{at}ccvax.sinica.edu.tw

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.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the June 27 issue to find the title link for this article.

This article was previously published in electronic format as an Expedited E-Pub on May 17, 2006, at www.neurology.org.

Disclosure: The authors report no conflicts of interest.

Received September 26, 2005. Accepted in final form February 24, 2006.






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