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Facts and Features

Well, the first bit was true. But little did Ironside know that this rare disease would soon dominate his entire career. He had previously seen occasional cases of CJD, but there was something worrying about the recent up-tick in incidence, particularly because it seemed to be affecting young people. Sporadic CJD tends to strike the elderly, explains Ironside, and the disease progresses rapidly—onset until death can be a matter of months. By contrast, the younger patients were experiencing more protracted disease symptoms. What's more, the occurrence of this drawn-out, early-onset CJD was largely restricted to the UK—the country most affected by BSE.

It wasn't until Ironside performed the first autopsy work on the brains of these young patients that suspicions were consolidated, however. Sporadic CJD and the new variant of the disease had “a clear distinction of pathologies”, Ironside says. Further biochemical work showed the prion agents of BSE and vCJD were apparently identical. Presenting this evidence to the UK government's spongiform encephalopathy advisory committee, says Ironside, “is a day that is etched in my memory for better or for worse.” Conveying the news that, in all likelihood, this new illness was the result of BSE in humans “was quite stressful”, he recalls, because “of course, it had enormous implications for public health.” Formal proof that BSE and vCJD were caused by the same agent came later, as did estimates for the number of people in the UK potentially exposed to the deadly prion (approximately 1 in 10 000)—studies that he was also engaged in. Indeed, Ironside has been involved in all manner of studies on vCJD and other prion diseases ever since that fateful conversation with Will.

“Looking back I think I've been very fortunate”, says Ironside. “Identifying vCJD and then watching how that whole story unfolded has been a fascinating and enjoyable experience, scientifically”, he says, even though the burden of responsibility to the public and to patients has, at times, been difficult. It is a “most horrible neurological disease”, he adds.

So how did it feel, I ask, to be recognised for this and his other work with a CBE? When the letter arrived, “the envelope said, From the Office of the Prime Minister”, Ironside recalls, “and my first reaction was, oh my God, what have I done now, thinking it was something I'd said.” In the field of CJD, he explains, “I've learned from experience, you need to be very careful what you say because it can be latched onto and over-interpreted.” But even after reading the letter and realising he wasn't in trouble—quite the opposite—his modesty still presided. “I thought, surely this can't be right. It must be a send up.”

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Colin Martin, The Lancet Neurology
Heino Diringer, The Lancet
Susan Joiner et al., The Lancet
Adriano Aguzzi et al., The Lancet
Jacqui Wise, The BMJ
British Medical Journal Publishing Group, The BMJ
Robert W. Steele, MD, Medscape
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James Ironside: modest manner, mighty impact - The Lancet Neurology

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