Variant Creutzfeldt-Jakob disease surveillance in Spain, 1993-2021

Variant Creutzfeldt-Jakob disease (vCJD) is a fatal transmissible prion disorder attributed primarily to the ingestion of meat from cattle infected with bovine spongiform encephalopathy (BSE), and it can also be transmitted through blood transfusions and occupational exposure in laboratories. As of July 2024, 233 deaths had been recorded in 12 countries, mostly in the United Kingdom (UK) and France.

This study aimed to describe the results of vCJD surveillance in Spain from 1993 to 2021 and the responses to those results.

Surveillance spanned the following three periods: 1993-2000, when neurologists and public health professionals created a network for reporting potential cases; 2001-2010, when diagnostic criteria were improved, and management guidelines were published; and 2011-2021, when reporting and post-mortem analyses decreased.

Five deaths due to vCJD were identified through post-mortem between 2005 and 2008 (ages ranging from 25 to 64 years). In two cases involving a mother and her son, their prion strain was similar to the strain found in UK humanized mouse models, suggesting a link to BSE. Three cases had recently undergone invasive dental procedures, one had undergone a fibroscopy, and another had donated blood several times. The majority of prevention measures were adopted with a delay.

Spain is the third most affected country by vCJD. Interruption of beef imports from the UK and control of the local BSE epizootic may have helped control vCJD in Spain since 2009. The increase in vCJD deaths through occupational exposures in laboratory work in other countries since 2016 should act as a warning signal for the surveillance of all forms of human transmissible spongiform encephalopathies.