The Generative Brain
French national surveillance network for Creutzfeldt-Jakob disease and related diseases (RNS-MCJ), coordinated by Sante publique France and Inserm with AP-HP and the Paris Brain Institute logo

French national surveillance network for Creutzfeldt-Jakob disease and related diseases (RNS-MCJ), coordinated by Sante publique France and Inserm with AP-HP and the Paris Brain Institute

France has run active national surveillance of human prion diseases since 1992, through a network of volunteer neurologists, laboratories and neuropathologists that Sante publique France, the national public health agency, now coordinates together with an Inserm research unit. The agency pages name it the national surveillance network for CJD and related diseases, abbreviated RNS-MCJ or RSN-MCJ. Clinical and research work sits at the Pitie-Salpetriere hospital in Paris: the AP-HP national CJD reference unit, the national reference centre for unconventional transmissible agents and the prion team at the Paris Brain Institute (Inserm U1127). Suspected CJD has been a notifiable disease in France since 19 September 1996. The network is part of the European EuroCJD surveillance network and classifies cases by its criteria. Its 25 year report counts 25,676 notified suspects and 2,907 prion disease cases from 1992 to 2016.

Key facts

  • Sante publique France describes the network as volunteer laboratories, neurologists and neuropathologists running CJD surveillance since 1992, strengthened by the agency from early 2000 into a formal national network that the agency coordinates under a three year agreement with Inserm; the coordinating Inserm unit is U1127, formerly U975.
  • Named members on the agency page are neurologists, the Inserm unit, the national reference centre for unconventional transmissible agents (CNR ATNC) with three parts (the Pitie-Salpetriere neuropathology and neurology department, which coordinates 22 regional neuropathology centres; the Lariboisiere biochemistry and molecular biology department, which leads a laboratory network; and an Inserm team on human prion diseases at Pitie-Salpetriere), the national CJD reference unit, the regional health agencies and Sante publique France.
  • Doctors and biologists who suspect CJD must report it without delay to the regional health agency, which passes it to Sante publique France, or directly to the network; a formal notification form (cerfa 12209*02) follows and is anonymised before it reaches the agency. Suspected CJD and other human TSEs have been notifiable since 19 September 1996.
  • Most suspected cases reach the network through biochemistry laboratories that test CSF for 14-3-3 protein; doctors can also notify the expert neurologists directly.
  • The stated aims are to find every human TSE case, especially variant CJD, classify cases by cause, estimate incidence, describe trends by place and time, detect clusters, and spot new forms early; the agency also works with the French blood service when a suspected patient was a recent blood donor.
  • From 1992 to 2016 the network received 25,676 notified suspects and diagnosed 2,907 probable or definite prion disease cases: 2,510 sporadic (86 percent), 240 genetic (8 percent) and 157 infectious, which were 116 after human growth hormone, 14 after dura mater grafts and 27 variant CJD.
  • Autopsy was done in 2,114 suspected patients, 70 percent of whom were confirmed as definite TSE; E200K is the most frequent PRNP mutation in France and D178N the second.
  • France recorded more growth hormone related and variant CJD than most European countries other than the UK, and both have declined; the authors still call for continued surveillance because of possible future variant CJD, chronic wasting disease in deer and rising sporadic counts.
  • A 2024 national report covering 2011 to 2019 counted 16,768 notified suspects and 1,449 classified TSE cases, including 3 variant CJD, 1 dura mater case and 3 growth hormone cases, and a mortality rate of 2.34 per million per year.
  • The agency data table, updated 31 July 2026, shows yearly suspects rising from 71 in 1992 to a peak of 2,303 in 2023, then 1,892 in 2025; total recorded CJD peaked at 173 in 2018 and was 89 in 2025.
  • The agency text gives 28 variant CJD deaths in France between 1992 and 2019, all methionine homozygous at PRNP codon 129, median age 36; the table adds one variant CJD death in 2021, and the 2023 paper counts 27 to 2016.
  • The French arm of the phase 2 doxycycline trial in CJD recruited through the French national referral system from January 2009 to January 2012, led from the AP-HP national CJD reference unit and the Paris Brain Institute, with Italian partners including ISS Rome and Carlo Besta.
  • The AP-HP national CJD reference unit and the CNR ATNC hold a yearly study day on prion diseases; the 10th, on 19 June 2025 at the Paris Brain Institute, was backed by Sante publique France, Inserm, CNRS, Sorbonne University and the Paris Brain Institute, and included talks on CJD cases found by brain bank screening and on RT-QuIC strain discrimination.
  • The Paris Brain Institute names the leader of its prion team as coordinator of the national reference centre for prion diseases and says the team works on CJD diagnostic tests and helped develop a blood test for variant CJD prions.
  • The Italian national registry lists Sante publique France among its external CJD links.

Timeline

  1. 1992Active national surveillance starts (1991 in the 2024 report).
  2. 1996Suspected CJD becomes a notifiable disease.
  3. 1997CSF 14-3-3 testing arrives, cited by the agency as one reason more cases were found.
  4. 2000Agency strengthens the volunteer system into a formal national network (early 2000).
  5. 2008European Euro and NeuroCJD networks reported merged under ECDC, per a French network document (search excerpt only).
  6. 2009French recruitment into the doxycycline trial opens (to 2012-01-10).
  7. 202325 year surveillance report 1992 to 2016 published in Eurosurveillance.
  8. 2024National epidemiology report for 2011 to 2019 published in BEH.
  9. 202510th prion disease study day at the Paris Brain Institute.
  10. 2026Agency CJD pages last updated.
  11. 2026Agency case table updated, with 2026 partial data.
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