Atypical/Nor98 scrapie in the Basque Country: a case report of eight outbreaks.

Ana B Rodríguez-Martínez,Joseba M Garrido,Mariví Geijo,Ramón A Juste,Sonia Maza,Esmeralda Minguijón,Leyre Benedicto,Nieves Gómez,Sylvie L Benestad

doi:10.1186/1746-6148-6-17

Ana B Rodríguez-Martínez, Joseba M Garrido + Show 7 more

Open Access

https://doi.org/10.1186/1746-6148-6-17

Copy DOI

Abstract

BackgroundSince 2002, an active surveillance program for transmissible spongiform encephalopathy in small ruminants in European Union countries allowed identification of a considerable number of atypical cases with similarities to the previously identified atypical scrapie cases termed Nor98.Case presentationHere we report molecular and neuropathological features of eight atypical/Nor98 scrapie cases detected between 2002 and 2009. Significant features of the affected sheep included: their relatively high ages (mean age 7.9 years, range between 4.3 and 12.8), their breed (all Latxa) and their PRNP genotypes (AFRQ/ALRQ, ALRR/ALRQ, AFRQ/AFRQ, AFRQ/AHQ, ALRQ/ALRH, ALRQ/ALRQ). All the sheep were confirmed as atypical scrapie by immunohistochemistry and immunoblotting. Two cases presented more PrP immunolabelling in cerebral cortex than in cerebellum.ConclusionsThis work indicates that atypical scrapie constitutes the most common small ruminant transmissible spongiform encephalopathy form in Latxa sheep in the Spanish Basque Country. Moreover, a new genotype (ALRQ/ALRH) was found associated to atypical scrapie.

Highlights

IntroductionSince 2002, an active surveillance program for transmissible spongiform encephalopathy in small ruminants in European Union countries allowed identification of a considerable number of atypical cases with similarities to the previously identified atypical scrapie cases termed Nor
Since 2002, an active surveillance program for transmissible spongiform encephalopathy in small ruminants in European Union countries allowed identification of a considerable number of atypical cases with similarities to the previously identified atypical scrapie cases termed Nor98.Case presentation: Here we report molecular and neuropathological features of eight atypical/Nor98 scrapie cases detected between 2002 and 2009
This work indicates that atypical scrapie constitutes the most common small ruminant transmissible spongiform encephalopathy form in Latxa sheep in the Spanish Basque Country

Summary

Introduction

Since 2002, an active surveillance program for transmissible spongiform encephalopathy in small ruminants in European Union countries allowed identification of a considerable number of atypical cases with similarities to the previously identified atypical scrapie cases termed Nor. Case presentation: Here we report molecular and neuropathological features of eight atypical/Nor scrapie cases detected between 2002 and 2009. Since 2002, an active surveillance program for Transmissible Spongiform Encephalopathy (TSE) in small ruminants has been implemented in European Union countries. As a result of this program, an atypical type of scrapie different from classical scrapie (CS) and similar if not identical to Nor identified in Norway [1] was detected in most of the European countries. CS is the traditional form of TSE affecting small ruminants, which was first detected in England around 1730 and thereafter in Germany and France [1,2,3].

Methods

Findings

Discussion

Conclusion