Report from the Prion Surveillance Study, May 2018

The annual report from the Prion Surveillance Study has been published.  

The Prion Surveillance Study has been running for over 10 years since it began in 2006. The study aims to find out whether there is evidence of the abnormal prion protein that is associated with variant Creutzfeldt-Jakob Disease (vCJD) in the blood and body tissues of primary immunodeficiency patients exposed to UK-sourced immunoglobulin between 1996 and 2000. vCJD is a very rare disease that causes degeneration of nerve cells in the brain and spinal cord.

Approximately 175 primary immunodeficiency patients are thought to have been exposed to UK-sourced immunoglobulin between 1996 and 2000. Of these patients, 79 from 16 immunology centres across Britain have been involved in the study.

There is no blood test that can reliably tell us if someone is infected with vCJD before they develop symptoms of disease. All study participants have agreed to donate blood for storage for future testing when such a test becomes available.


To date, no primary immunodeficiency patients have shown symptoms of prion disease, nor is there any evidence of prion infection in the tissues tested. These results are reassuring but it is very important that we continue to monitor patients over the long term because we know that prion disease may take many decades to develop after exposure.

If you would like to find out more, please contact the Prion Surveillance Study research nurse, Mrs Kudzai Karekwaivanane, and they will be happy to help. Telephone: 0131 537 2128 / 07464 677118.

Posted July 2018