Introduction - Transmissible subacute spongiform encephalopathies (TSSEs) have been subject to epidemiological surveillance in France and Europe since the early 1990s. The observation of cases of iatrogenic Creutzfeldt-Jakob disease (CJD), the emergence of bovine spongiform encephalopathy (BSE), and its probable transmission to numerous species were the impetus for this surveillance, which remains justified by the emergence of variant CJD (vCJD) in 1996. State of Knowledge - In France, epidemiological surveillance is currently coordinated by the InVS and organized around the reporting of cases to the Inserm-U708 unit by clinicians, either directly or through a request for the detection of the 14-3-3 protein in cerebrospinal fluid. Each suspected case is followed up until a final diagnosis of probability or certainty is obtained. Thanks to the efficiency of the French neuropathology network, more than half of patients who die with suspected CJD undergo autopsy. Diagnostic criteria allow for comparing the incidence of different forms of the disease across countries conducting epidemiological surveillance. Among these different forms, sporadic CJD, of unknown cause, is by far the most common, accounting for more than 80% of cases. Iatrogenic CJD cases currently observed are due to dura mater transplants or treatment with human growth hormone, which took place in the 1980s. Genetic forms, which are autosomal dominant, account for 10% of TSE cases and are linked to various mutations or insertions in the PRNP gene encoding the PrP protein. Finally, vCJD, linked to BSE, is a new form of CJD identified as early as 1994 in the United Kingdom, which is the country most affected by this disease. Outlook and Conclusions - Active epidemiological surveillance of CJD remains relevant in France, primarily due to the continued occurrence of iatrogenic CJD cases linked to treatment with human growth hormone. It is important in France and the rest of the world due to the emergence of post-transfusion vCJD cases and the continued possibility of vCJD cases in individuals with the valine-valine or methionine-valine genotype at codon 129 of the PRNP gene.