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Scientific report on the analysis of the 2‐year compulsory intensified monitoring of atypical scrapie

on the Wiley Online Library


Note: The Protocol is available under ‘ Supporting Information’.


The European Commission asked EFSA whether the scientific data on the 2‐year intensified monitoring in atypical scrapie (AS) outbreaks (2013–2020) provide any evidence on the contagiousness of AS, and whether they added any new knowledge on the epidemiology of AS. An ad hoc data set from intensified monitoring in 22 countries with index case/s of AS in sheep and/or goats (742 flocks from 20 countries, 76 herds from 11 countries) was analysed. No secondary cases were confirmed in goat herds, while 35 secondary cases were confirmed in 28 sheep flocks from eight countries. The results of the calculated design prevalence and of a model simulation indicated that the intensified monitoring had limited ability to detect AS, with no difference between countries with or without secondary cases. A regression model showed an increased, but not statistically significant, prevalence (adjusted by surveillance stream) of secondary cases in infected flocks compared with that of index cases in the non‐infected flocks (general population). A simulation model of within‐flock transmission, comparing a contagious (i.e. transmissible between animals under natural conditions) with a non‐contagious scenario, produced a better fit of the observed data with the non‐contagious scenario, in which each sheep in a flock had the same probability of developing AS in the first year of life. Based on the analyses performed, and considering uncertainties and data limitations, it was concluded that there is no new evidence that AS can be transmitted between animals under natural conditions, and it is considered more likely (subjective probability range 50–66%) that AS is a non‐contagious, rather than a contagious disease. The analysis of the data of the EU intensified monitoring in atypical scrapie infected flocks/herds confirmed some of the known epidemiological features of AS but identified that major knowledge gaps still remain.