Headlines - Mortality from Creutzfeldt-Jakob Disease (CJD)

Prion diseases, or transmissible subacute spongiform encephalopathies (TSEs), are neurodegenerative disorders affecting both humans and animals, characterized by their transmissibility. In addition to being transmissible, TSE are diseases characterized by their rarity, long incubation period, fatal course without remission, and the absence of any detectable inflammatory or immune response. This condition received significant media attention in connection with cases linked to the use of growth hormone and cases linked to mad cow disease (variant Creutzfeldt-Jakob disease or vCJD).

According to data updated as of April 30, 2012, by the InVS, 25 confirmed or probable cases of vCJD have been identified in France, all of whom have died to date. These 25 deceased cases share the following characteristics: they died in 1996 (1 case), 2000 (1 case), 2001 (1 case), 2002 (3 cases), 2004 (2 cases), 2005 (6 cases), 2006 (6 cases), 2007 (3 cases), and 2009 (2 cases). The group consists of 12 men and 13 women. The median age at death was 37 years (ranging from 19 to 58 years). Among them, 7 resided in the Île-de-France region and 18 in other parts of the country. These data are highly dynamic due to the complexity of the subject and the network, as well as the time required to confirm cases. However, they do provide insights into the impact of mad cow disease, which caused significant concern in the 1990s.

This number is low compared to the number of suspected cases reported, which has risen gradually since 1992 (the year the CJD epidemiological surveillance network was established) to reach 1,614 and 1,609 in 2010 and 2011 in France. Of these suspected cases, only a portion were confirmed as CJD cases (sporadic, iatrogenic growth hormone, other iatrogenic CJD, genetic, definite or probable vCJD in deceased patients, probable vCJD in living patients), numbering 155 and 80, respectively. Burgundy and Franche-Comté are affected by this disease. Between 2005 and 2009, there were 6,575 reports of suspected CJD in France, including 660 confirmed cases of CJD. In Burgundy, during the same period, there were 244 reports of suspected CJD, including 15 confirmed cases, and in Franche-Comté, there were 194 reports of suspected CJD, including 13 confirmed cases.