Transmissible Spongiform Encephalopathies, or Prion diseases, are fatal neurodegenerative diseases that affect both humans and animals. Examples include Creutzfeldt-Jakob disease (CJD) in humans, Bovine Spongiform Encephalopathy (BSE) in cattle and scrapie in sheep and goats. They are characterized by a long incubation period ranging from months to years, and a variable length clinical course, both determined by the particular infection and the species involved. Some 170,000 cattle in the UK have been diagnosed with BSE and have been destroyed. In the rest of Europe there have also been cases of BSE, though the numbers involved are much lower. The recent potential link between BSE and a new variant form of CJD has highlighted the importance of controlling both BSE and scrapie in Ireland, Control of the disease in both cattle and sheep is complicated by the long incubation period the lack of suitable tests and the general lack of detailed information in relation to the biology of The disease. Factors that must be considered in designing an effective control strategy include (1) better understanding of the epidemiology of the disease, (2) developing rapid and reliable tests for the disease, (3) introducing a suitable surveillance system for both BSE and scrapie, (4) minimizing the consequences for farmers that have BSE-infected animals, and (5) implementing and monitoring legislation to prohibit the spread of potentially infectious material. Each of these areas is discussed and it is proposed that the introduction of best available technology in both the diagnosis and surveillance of the national herd could form the basis for an effective program against animal priori diseases.
