James Ironside - Transcript Summary

James IronsideProfessor of Neuropathology, Edinburgh University

Interview location: His office at the Western General Hospital, Edinburgh
Interview date
: 3rd September, 2007

Key ThemesAlder Hey, Attributes of a Pathologist, Autopsy,  Legislation and RegulationMentors and Influences, Research versus Clinical Work


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Section 1

  • Discusses his path into medicine.  Started studying at Dundee University and says “it soon became clear to me that I was more interested in the mechanisms of disease, rather than in curing disease or treating the patients.”  Took an intercalated degree and spent the extra year doing pathology, and clearly enjoyed the detective work involved:  “You have to pick up as many clues from the story you're given, you have your list of suspects...and then you have to do tests to eliminate the various suspects, to end up with what is, hopefully, the truth.”
  • At that time autopsies were common practice, so he became skilled at dissection.  “That has stood me in good stead throughout my career.”  Comments that students today don’t have the same opportunities because of the dearth of autopsies, so the challenge as a lecturer is “to come up with better and more efficient ways of teaching them, or trying to instil in them the skills they need over a more compressed timeframe”.
  • Discusses briefly learning to cope with dissection. “You have to be able to step back or it's just impossible... A body is a miracle of nature, and if you approach it from that point of view then it is very interesting.”
  • Intrigued by the work of some of his colleagues, he decided to specialise in the neuropathology, which necessitated leaving Scotland for Sheffield, England.  Describes some important role models during his training and early career.  One thing he appreciated was being supported but allowed “to make my own mistakes”.
  • Describes the “shock” he felt in his first post, when a diagnosis he made resulted in the life-support being withdrawn from a patient.  “I think it's very important that we as pathologists remember that diagnosis is not made in a vacuum, or it's not something that exists only between you and the clinician. There's much wider impact, not just for the patient, but for the whole family.”  Mentions that today, working in multidisciplinary teams means that pathologists are less isolated.
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Section 2

  • Describes the everyday tasks of a young neuropathologist, and recalls the first cases he encountered of Creutzfeldt-Jakob disease (CJD), and the very rare but related Gerstmann-Straussler-Scheinker syndrome.  “I do remember looking down the microscope and seeing something just incredible – because this brain was full of amyloid plaques, full of plaques.  I thought, ‘I've never seen anything like it before!’  It was everywhere, including the cerebellum.”
  • To him this case exemplified the importance of “performing an autopsy in patients like this with neurological diseases which are undiagnosed...When a prion disease like that is diagnosed...it does have wider implications in terms of health and safety.”
  • Discusses the vital importance of “having an open mind and looking beyond the clinical expectation” when reaching a diagnosis.
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Section 3

  • Briefly describes the background to the dramatic problem that emerged in the mid 1980s of ‘mad cow disease’ (bovine spongiform encephalopathy, or BSE) and variant CJD.  Talks about leading figures in prion research and the setting up of the CJD surveillance project, which he joined on his move to Edinburgh in 1990.  “At that time it was sold to me as ‘a very rare but very interesting’ disease that probably wouldn't entail a great deal of extra work!”
  • Discusses the growing understanding of CJD in all its manifestations: “there was a lot happening and it was an exciting time, a lot of information to get your head round.” Describes some of the ongoing research – including a Europe-wide surveillance project.  International collaboration is essential to get enough cases for findings to be statistically significant.
  • Talks about the value of having tissue and organ archives, which they regularly consult.  “The best example is when we were involved in the identification of variant CJD.”  He goes on to describe the moment he was convinced that he was seeing something completely new. “Talk about detective work, it was really intense!... If you're going to claim that you've found a new disease that's related to BSE, that's no small claim and you want to make sure you've got it as right as you possibly can.”
  • Talks about setting up the National Retrospective Review to confirm their original hypothesis.  This, he points out, was “a good example of using the archive in a very constructive way to address an issue which was a real public health concern”.
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Section 4

  • Mentions the importance of pattern-recognition, and goes on to describe in more detail the pathology of variant CJD.  Discusses the need for painstaking collection of evidence and data to convince the scientific community of the case for a new variant.  Appears before the  Spongiform Encephalopathy Advisory Committee: “I was able to show them not just a visual depiction of what it looked like, but actually some hard data.”
  • Describes briefly some of the advances in technology.
  • Muses upon the crucial importance of pattern recognition for a pathologist, but also on the need to balance the instinctive response to an image with a more rigorous scientific approach.
  • Mentions in passing his love of art and music.
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Section 5

  • Describes the prion disease, kuru, found among tribespeople in the highlands of Papua New Guinea who practised ritualistic endocannibalism.  “What kuru has taught us is that you can have a long incubation period -- of 40 and 50 years -- for these diseases following oral exposure... The other thing that kuru has taught us is the influence of genetic variability.”
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Section 6

  • Goes into some detail about the legislation enacted after the Alder Hey controversy – “a dark hour for pathology” – and his role as a member of the Human Tissue Authority.
  • Draws attention to the implications of the new, more stringent legislation on tissue and organ retention: “suddenly there's going to be no archive... So my main motivation for joining the Human Tissue Authority was to try and make the implementation of the legislation as good as it could be for both the profession and indeed the public.”
  • Describes the wide range of skills and expertise – not just medical or legal – represented in the Human Tissue Authority, and the scope of topics covered.  “The magnitude of the task came as some surprise to all of us!”

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Section 7

  • Muses about how his daily contact with death has made him more aware of human frailty.  But, he says, “Other things bring you to think of the meaning of life and death.  Music is one of them.”
  • Expands upon the subject of mentors and role models.
  • Describes how he divides his time between the NHS, where he provides a diagnostic neuropathology service, and his work at the university, which includes continuing research on CJD in the UK and abroad.
  • Admits that there remain many puzzling aspects to transmissible spongiform encephalopathies:  “There’s no doubt that the prion protein is key to all these diseases, but whether it’s the only thing that’s important, we don’t know.”  Outlines some of the current hypotheses.  Describes a project to explore some of these, using stem cells, in collaboration with the university’s Centre for Regenerative Medicine.
  • Ends with the story of how he and his colleague, Bob Will, went to Geneva to make a presentation on variant CJD to the World Health Organization. “The whole thing took off; it became a global matter then.”


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