Variant Creutzfeldt-Jakob disease and the risks from blood transfusion (page 1 of 4)
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Whilst the incidence of clinical cases of variant Creutzfeldt-Jakob disease (vCJD) has declined in recent years, concern has increased over the possibility of an epidemic arising through blood transmission. Here we review important aspects of the disease and its epidemiology and consider their implications for the risks from blood transfusion.
vCJD
vCJD is one of a family of neurodegenerative diseases known as Transmissible Spongiform Encephalopathies (TSEs). The oldest known disease of this type is scrapie, which affects sheep and has been endemic in most parts of the world for over 200 years. Bovine Spongiform Encephalopathy (BSE) or ‘mad cow disease’ also belongs to this family of diseases. Human forms of the disease include CJD, Gerstmann-Straussler-Scheinker disease (GSS), fatal familial insomnia and kuru. The latter was first discovered in the 1950s among the Fore population in Papua New Guinea, with the epidemic resulting in approximately 2,500 deaths from 1957 onwards. The disease was transmitted through cannabilistic consumption of, or contact with, human tissues during funeral rites. Sporadic cases of CJD occur worldwide with an annual incidence of approximately one case per million of the population, whilst inherited forms of the disease are even rarer (approximately 10% of all cases of CJD). Transmissible Spongiform Encephalopathies are characterised by spongiform changes in the brain, neuronal loss and amyloid plaque formation, which occur with the accumulation of an abnormal protease-resistant prion in the brain. A particular characteristic shared by these diseases is a long and variable incubation period, typically of many years, during which there is little or no detectable immune response to the pathogen.
Epidemiology
vCJD was first identified in the UK in 1996 and was distinguished from classical CJD by its different clinical presentation and neuropathology.1 Through March 2006 there have been 154 deaths from vCJD in the UK with a further six probable cases remaining alive. Elsewhere cases have been reported in Ireland, France, Italy, United States, Canada, Saudi Arabia, Portugal and Japan.
