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PrPd accumulation in organs of ARQ/ARQ sheep experimentally infected with BSE by peripheral routes

100%

Lezmi S. , Ronzon F. , Bencsik A. , Bedin A. , Calavas D. , Richard Y. , Simon S. , Grassi J. , Baron T.

nr 2

399-405

To study the pathogenesis of bovine spongiform encephalopathy infection in small ruminants, two Lacaune sheep with the AA136RR154QQ171 and one with the AA136RR154RR171 genotype for the prion protein, were inoculated with a brain homogenate from a French cattle BSE case by peripheral routes. Sheep with the ARQ/ARQ genotype are considered as susceptible to prion diseases contrary to those with the ARR/ARR genotype. The accumulation of disease-associated prion protein (PrPd) was analysed by biochemical and immunohistochemical methods. No PrPd accumulation was detected in samples from the ARR/ARR sheep 2 years post inoculation. In the two ARQ/ARQ sheep that had scrapie-like clinical symptoms, PrPd was found in the central, sympathetic and enteric nervous systems and in lymphoid organs. Remarkably, PrPd was also detected in some muscle types as well as in all peripheral nerves that had not been reported previously thus revealing a widespread distribution of BSE-associated PrPd in sheep tissues.

Neuropathology of variant Creutzfeldt-Jakob disease

88%

Ironside J. W. , Head M. W. , McCardle L. , Knight R.

tom 62

nr 3

Rapidly progressing dementia as a manifestation of the Creutzfeldt-Jakob disease: an analysis of two cases

75%

Seweryn Bartosz S. , Natalia Leksa N. , Dominika Uberman-Kluz D. , Artur Szymczak A. , Marek Biesiadecki M. , Sabina Galiniak S.

nr 1

89-93

Introduction. Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disease of the central nervous system which is caused by an infectious protein called prion. Multiple forms of CJD have been classified including sporadic (more than 90% cases), familial, iatrogenic and variant type of disease. CJD, especially in its early stages, is a highly challenging illness to diagnose. Aim. Article aims to present cases of Creutzfeldt-Jackob disease with early symptoms of rapidly progressing dementia at the initial stage of CJD. Description of the cases. This paper describes two cases of patients with suspected CJD with a history of rapidly progressive dementia admitted to the Department of Neurology, MSWiA Hospital in Rzeszów. Conclusion. Despite the fact that CJD is an incurable illness and there is no cure guaranteeing recovery, it is important to make the right diagnosis. Assay of 14-3-3 protein in cerebrospinal fluid is a sensitive and specific marker which is helpful in the diagnosis of CJD. The only relevant method of correctly confirming a diagnosis of this disease is by performing a brain biopsy.

Prion disease and Alzheimer's disease: pathogenic overlap

75%

Castellani R. J. , Perry G. , Smith M. A.

nr 1

Molecular biology of prions

63%

Weissmann C. , Enari M. , Klohn P. C. , Rossi D. , Flechsig E.

tom 62

nr 3