A Second Person in France May Have Caught Deadly Prion Disease From Lab Exposure

Public research labs in France are temporarily halting their work into prions, after at least two employees are believed to have contracted a rare but universally fatal prion brain disease. One woman has since died, almost certainly after having been exposed during a lab accident in 2010, while the second is reportedly still alive, and it is not yet confirmed whether her illness was caused by lab exposure.

Prions are a type of protein commonly found in the brain. They seem to serve some important but still unclear natural function. But prions can also turn into a misfolded form of themselves, one that slowly turns other “normal” prions around them rogue, too. Over time, this cascading effect spreads throughout the brain and destroys it, leaving behind telltale sponge-like holes that can be spotted under a microscope. Prion diseases are very rare, but there are no available treatments, and people usually die within months to a year of the onset of symptoms, which typically include dementia and motor impairment.

The first case, now known to be Émilie Jaumain, began experiencing symptoms in late 2017. She was suspected to have variant-Creutzfeldt-Jakob disease (vCJD), a form of CJD spread through exposure to prions from other animals. The first cases of vCJD were discovered in the 1990s, after people ate meat from tainted cows and then developed a prion disease, which was given the morbid nickname of mad cow disease. The diagnosis was confirmed in a brain autopsy following Jaumain’s death 19 months later.

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