Second death in France in a laboratory working on prions

Creutzfeldt-Jakob disease killed a few days ago a retired research technician from the National Research Institute for Agriculture, Food and the Environment (Inrae), who had worked in Toulouse in contact of biological tissue infected with prions. This death sows consternation and concern in the scientific community working on these infectious agents. It follows the death, on June 17, 2019, of Emilie Jaumain, a 33-year-old laboratory technician, suffering from the same incurable neurodegenerative disease. The young woman is said to have contracted it in 2010, cutting herself while handling fragments of the brains of infected mice by prions, in another unit of INRAE, in Jouy-en-Josas.

Regarding the retiree from Toulouse, it will be necessary to determine whether she was the victim of a genetic or sporadic form of Creutzfeldt-Jakob disease, if the disease may have been caused by the ingestion of meat contaminated by the agent of encephalopathy. bovine spongiform (BSE, also called mad cow disease) or, as in the case of Emilie Jaumain, if accidental occupational exposure can be claimed. Prion diseases are caused by proteins taking an aberrant conformation, which gives them the property of replicating to form aggregates that are deleterious for neurons. There are around 150 cases per year in France, resulting in fatal degeneration of the central nervous system.

Read also An atypical case of variant Creutzfeldt-Jakob disease

Moratorium on research

A moratorium on research on the prion was announced on July 27, after INrae learned of the second suspected case. It was decided by the general management of the five organizations concerned by these activities – Anses, CEA, CNRS, Inrae and Inserm -, “Jointly and in agreement” with the supervisory ministries, those of research and agriculture. This new death raises again in an acute way the question of past and current precautions taken to protect people working on prions.

On October 26, during the ministerial health, safety and working conditions committee for higher education and research, all staff representatives asked Minister Frédérique Vidal “To finally take the measure of the gravity of the situation and to stop shirking its responsibilities”. They called in particular for an exhaustive census of all the people who worked on unconventional transmissible agents, including prions; an assessment of accidents during handling of prions since 1990, the disease being able to break out decades after contamination; a system for tracing the exposures of each agent concerned; a reflection on the implementation of a certification of the premises and an authorization of the personnel… Some union representatives also wonder what impact the race for scientific publications can have on the safety of the agents.

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