dc.description.abstract | Prion diseases are a family of rare and fatal progressive neurogenerative diseases,
characterised by incubation periods up to several months or years, spongiform changes seen
as neuronal loss, and no inflammatory response, commonly seen in more frequent viral and
bacterial diseases, are visible in blood or cerebrospinal fluid
The most known prion diseases are chronic wasting disease (CWD) in cervids, scrapie in
sheep and goat, and bovine spongiform encephalopathy (BSE) in cattle. Creutzfeldt-Jakob
disease (CJD) and the variant form (vCJD) and Kuru are examples of prion diseases occurring
in humans.
CWD is a fatal, neurodegenerative prion disease diagnosed in the family of Cervidae,
including captive and free-ranging deer, elk, moose and reindeer in North America, South
Korea, and recently in Scandinavia.
CWD is one of the few TSEs considered highly infectious and are believed to be horizontally
transmitted.
In March 2016, CWD was for the first time diagnosed in Europe. A female reindeer from the
wild reindeer area called “Nordfjella region zone 1” died during an attempt to chemically
immobilise wild reindeer in Norway. In May and June, the same year, CWD was diagnosed in
two Eurasian moose approx. 300 km. north of the first confirmed case, in Trøndelag county in
central Norway and was for the first time discovered in red deer in Norway, October 2017 in
the municipality of Gjemnes in mid-western Norway.
Classical scrapie is a fatal, neurodegenerative prion disease affecting ovine and caprine
species. The disease is known for almost 300 years, as the first case reported was in Great
Britain in 1732 in sheep. Scrapie was for the first time diagnosed in Norway in 1958 in two
rams imported from England, and Norwegian bred sheep in 1981. | en_US |