Study indicates the number of people in the UK who could be incubating vCJD

The Journal of Pathology recently published a paper entitled Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. The study looked at 12,674 stored appendix and tonsil samples, and found that 3 of them showed evidence of the prion protein associated with variant Creutzfeldt-Jakob Disease (vCJD).

In response to the paper, Professor Pat Troop, Chief Executive of the Health Protection Agency, said ‘We welcome this research, which provides important data for estimating the number of people in the UK who may be incubating CJD. When the Department of Health and Medical Research Council steering group considered these results they recommended that a much larger study should be undertaken. The Health Protection Agency recently began this large-scale project to collect 100,000 samples of leftover tonsil tissue removed during routine operations. Once anonymised, these specimens will be tested for the presence of the abnormal prion that causes CJD, and the larger scale of the study should provide better estimates of the number of people who may be affected”

Creutzfeldt-Jakob Disease (CJD) is a brain disorder characterized by memory loss, jerky movements, gait disorder, rigid posture, and seizures due to a rapid loss of cerebral cells caused by transmissible proteins called prions. The disease is correctly diagnosed in anywhere from one to two people per million and it usually appears in mid-life with an average disease onset age of 50.

The prion that is believed to cause Creutzfeldt-Jakob exhibits an amino acid sequence and configuration which makes it insoluble in water, while the normal protein is highly soluble. So, as the numbers of defective prion proteins propagate and increase exponentially, the process leads to a huge load of insoluble prions in affected cells. This load of proteins disrupts cell function and causes cell death. Once the prion is transmitted, the defective proteins invade the brain like a forest fire and the patient dies within a few months (a few patients live for about 1-2 years). The defective protein can be transmitted by human growth hormone products, corneal grafts or dural grafts (acquired form) or it can be inherited (hereditary form) or appear for the first time in the patient (sporadic form). In the latter two forms the defective protein is not transmitted from an external source but already exists in the genes of the individual.

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