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Central and Peripheral Pathology of Kuru: Pathological Analysis of a Recent Case and Comparison with Other Forms of Human Prion Disease

Sebastian Brandner, Jerome Whitfield, Ken Boone, Anderson Puwa, Catherine O'Malley, Jacqueline M. Linehan, Susan Joiner, Francesco Scaravilli, Ian Calder, Michael P. Alpers, Jonathan D. F. Wadsworth and John Collinge
Philosophical Transactions: Biological Sciences
Vol. 363, No. 1510, The End of Kuru: 50 Years of Research into an Extraordinary Disease (Nov. 27, 2008), pp. 3755-3763
Published by: Royal Society
Stable URL: http://www.jstor.org/stable/20208828
Page Count: 9
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Central and Peripheral Pathology of Kuru: Pathological Analysis of a Recent Case and Comparison with Other Forms of Human Prion Disease
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Abstract

While the neuropathology of kuru is well defined, there are few data concerning the distribution of disease-related prion protein in peripheral tissues. Here we report the investigation of brain and peripheral tissues from a kuru patient who died in 2003. Neuropathological findings were compared with those in classical (sporadic and iatrogenic) Creutzfeldt-Jakob disease (CJD) and variant CJD (vCJD). The neuropathological findings of the kuru patient showed all the stereotypical changes that define kuru, with the occurrence of prominent PrP plaques throughout the brain. Lymphoreticular tissue showed no evidence of prion colonization, suggesting that the peripheral pathogenesis of kuru is similar to that seen in classical CJD rather than vCJD. These findings now strongly suggest that the characteristic peripheral pathogenesis of vCJD is determined by prion strain type alone rather than route of infection.

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