Creutzfeldt–Jakob disease

Abstract

Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of neurodegenerative conditions that are transmissible, progressive and uniformly fatal. They occur in both humans and animals. Prion disease came to prominence after the development of bovine spongiform encephalopathy (BSE) in the 1980s and, consequently, variant Creutzfeldt–Jakob disease (vCJD) in the 1990s. BSE and vCJD have had far reaching implications for the UK economy but especially for the farming and healthcare sectors. With the advent of BSE, prices for beef originating in the UK fell dramatically and this proved crippling to much of the UK farming community. Although the peak of the vCJD epidemic appears to have passed, prevalence studies suggest that around 1 in 3000 UK adults may be infected with vCJD prions (http://www.hpa. org.uk/hpr/archives/2011/news3611.htm#cjd). As there is currently no treatment for vCJD, considerable effort has been put into disease prevention. Difficulties in the diagnosis of vCJD, lack of treatment, and inability of conventional sterilization to completely remove prion protein from surgical instruments have also resulted in significant changes in healthcare delivery. Although the implications of vCJD for the delivery of anaesthesia are limited, it is important that anaesthetists understand how to identify patients at high risk of vCJD and are aware of the relevant precautionary measures that must be taken.