17 May 2022: Ironside - A

Prof Ironside was not a Raymond Burr doppelganger after all, but there was an air of Kevin Spacey around him. So maybe if the famous cop show returns, the new lead can be played by Kevin … no wait, strike that.

The Prof was on the research and development programme for CJD while also an academic at the University of Edinburgh. The usual review of his CV included both Scottish and English clinical and teaching roles. There had previously been a surveillance project for CJD in Oxford, but clearly with the incidence of BSE in the UK livestock during the 1990s, that new imperative led to a renewed focus. The Edinburgh project gained international support. Patients were identified through the established neuropathology and related networks, or even a person’s GP. Primarily, it was a case of flagging up unusual cases of dementia. A useful “backstop” in identifying possible cases was to look for death certificates mentioning CJD.

Another way of identifying people was to analyse the juices in and around the spinal cord and brain. The patient remained with their referring clinician, but Project staff saw people and families after obtaining consent to include them in, for example, epidemiological studies. Specialist nurses (National Care Coordinators) were also involved to provide support, including home visits. The Edinburgh Project passed on details to the blood collection services of anyone who came to their attention as a potential CJD risk. Equally, the blood services would refer in reverse. One of his roles was to provide a diagnosis of CJD, for example after death.

Building on the explanations about CJD introduced by Prof Collinge, Prof Ironside indicated that there is still much that is not known about vCJD. There remain to be small numbers of CJD deaths every year in the UK. It is clear that prions can stay in tissues for a long time, maybe several decades. It is a theoretical possibility for an infected person to remain asymptomatic, but it is only an inference from animal studies, mainly mice, who of course don’t live very long. Prof Ironside prefers not to refer to people as “carriers”, but as either symptomatic or asymptomatic. It is difficult to measure the incubation period, but maybe about 13 years could approximately be assumed. The prion protein gene is a common human gene most often found in the brain, but it differs into certain “genotypes” and genotypes seem to influence incubation periods. In terms of genotype, those with “mm” are the only people who have so far been discovered as susceptible. However, the currently available tests and diagnostic tools are not conclusive. It is further complicated as people get older since there is a greater incidence of dementia-type conditions in the elderly. There is currently a Lothian study to see if older people may have been missed from being identified as CJD patients.

(There was an early break to allow the technicians to fix a problem.)

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