Advances in screening test development for transmissible spongiform encephalopathies

The blood of patients with transmissible spongiform encephalopathy or prion disease can no longer be considered free of infectivity. There have been two recent reports of highly probable transfusion-associated iatrogenic variant Creutzfeldt-Jakob disease infections, and there is supporting experimental evidence of scrapie transmission by the transfusion of blood from sheep with naturally occurring disease. In the absence of a preclinical diagnostic test for transmissible spongiform encephalopathy, the main precautionary measures undertaken by blood agencies employ donor exclusion criteria, ensuring that the number of any further iatrogenic cases will be small. The development of a sensitive, specific and reliable diagnostic test is urgently needed for early identification of infected individuals in order to ensure the safety of blood supplies. During the past 5 years, significant progress has been made in improving the sensitivity and specificity of tests using brain and lymphoreticular tissues to identify Creutzfeldt-Jakob disease-infected individuals. However, the quest for a blood test is still in its infancy and requires extensive further research.