You may have read the harrowing story of Émilie Jaumain on my blog or elsewhere. It is a tragic and infamous case, many have covered it. But there is another deeply unsettling case that may have happened, another story of fatal infection by prions–fatal is a pleonasm, when prions enter the body the mortality is 100%. This one comes from Spain, and the use of hypothetical is compulsory, as the details are still scarce…
I’ll summarise what we know, thanks to an article published in El Pais in October 2023, a Spanish newspaper (link at the bottom).
In November 2020, an unnamed researcher at a Spanish university begins feeling unwell and takes a sick leave. He has joined less than 3 years before, at the helm of a team working on prions. Disturbingly, his sickness is consistent with the symptoms of Creutzfeldt-Jakob disease, a fatal prion disease.
Late 2020 brings another chilling revelation, in a -80°C freezer used by the sick researcher’s team. A fortuitous check reveals a drawer storing thousands of unauthorised samples from patients and animals with Creutzfeldt-Jakob disease and other neurodegenerative disorders. There are no records nor labels warning of the content of the drawer. The whole laboratory is immediately closed and decontaminated.
In 2022, the researcher dies, aged 45. The cause of death remains undisclosed. A question gnaws in the minds of most: are the samples infectious?
This question has an answer. But it comes more than 2 years after the discovery of the unauthorised samples. The samples are sent to a specialised centre only in December 2022. The answer arrives in March 2023, but it is disclosed to the deceased researcher’s coworkers only in July 2023. And it is blood-freezing: Yes, some of the unauthorised samples are indeed infectious.
And this is the end of what we know. We are left with uncertainties, doubts, questions, and with the hypotheses, educated guesses, speculations that our minds can conjure up to fill the many gaps.
The following are the three main questions that I have, accompanied by some thoughts of mine.
- Did the unnamed researcher die of Creutzfeldt-Jakob disease? No official answers exist. A speculation based on scientific data: once the symptoms are apparent, prion diseases are fatal in months, a timeframe consistent with the researcher’s death.
- If so, did the researcher become infected upon exposure to some unauthorised samples at his most recent lab? The incubation period of prion diseases typically stretches far longer than his 3-year tenure in Spain. Fatal contamination during his time studying prions in another European laboratory cannot be ruled out.
- Most crucially: were members of his team exposed to prions? Coworkers were unaware of the presence of infectious samples. Fortunately, transmission of a prion disease is not easy: it only occurs via inoculation or ingestion of prion-contaminated material and there are no recorded occupational accidents of this sort in the laboratory. However, contamination only takes a minor cut…
It’s now almost 5 years since the fortuitous discovery of the unauthorised infectious samples in a freezer drawer… Will we ever have an update?
For further reading: the article in El Pais.
WritinGenomics 
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