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Second lab worker with deadly prion disease prompts research pause in France - Ars Technica
Jul 29, 2021 1 min, 46 secs

Five public research institutions in France announced a three-month moratorium on prion research this week, following a newly identified case of prion disease in a retired lab worker.

In 2019, another lab worker in the country died of a prion disease at the age of 33.

Prions are misfolded, misshapen forms of normal proteins, called prion proteins, which are commonly found in human and other animal cells.

When a misfolded prion enters the mix, it can corrupt the normal prion proteins around them, prompting them to misfold as well, clump together, and corrupt others. As the corruption ripples through the brain, it leads to brain tissue damage, eventually causing little holes to form.

The most common type of TSE in humans is Creutzfeldt-Jakob disease (CJD), which has two forms: "classic" and "variant." The classic form strikes about one person in a million in the US and other countries, and patients typically die within a year of the onset of symptoms.

In about 5 percent to 15 percent of cases, the disease is determined to be hereditary, linked to a family history of CJD or a mutation in a prion protein that's linked to misfolding.

People can contract variant CJD by eating prion-contaminated meat, which appeared to be the case in a large outbreak of BSE among cattle and variant CJD among people in the United Kingdom during the 1980s and 1990s.

Importantly, the classic and variant forms of CJD have distinct clinical and pathological features.

For one thing, classic CJD tends to afflict older adults (median age of death is 68), while the variant form tends to strike earlier (median age at death is 28).

Variant CJD was the clear cause of the 2019 prion disease in the young lab worker, named Émilie Jaumain.

According to a case report of her disease and death published in the New England Journal of Medicine last year, Jaumain first developed symptoms in November 2017, about 7.5 years after the accident.

However, the authors of the NEJM report noted that the last similar case of variant CJD in France died in 2014.

So far, little is known about the new case in France that prompted the moratorium this week.

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