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oai:repisalud.isciii.es:20.500.12105/139302025-06-23T16:41:13Zcom_20.500.12105_2052com_20.500.12105_2051col_20.500.12105_19608col_20.500.12105_19617

00925njm 22002777a 4500 dc Pedro-Cuesta, Jesus de author Almazan-Isla, Javier author Tejedor Romero, Laura author Ruiz-Tovar, Maria author Avellanal, Fuencisla author Rábano, Alberto author Calero, Miguel author Garcia Lopez, Fernando Jose author Spanish CJD Surveillance Group author 2021-12 In Spain, human transmissible spongiform encephalopathies (TSEs) have been undergoing continuous surveillance for over 25 years. In 1995, the system was launched as an EU Concerted Action, with EU surveillance network procedures being incorporated from 2002 onwards. The aim of this report was to describe performance and outcomes of this surveillance system across the period 1993-2018. Neurology and public health specialists from every region reported cases to a central hub at the Carlos III Health Institute, Madrid. In all, eight accidentally transmitted cases and five definite variant Creutzfeldt-Jakob disease (vCJD) patients were reported. All vCJD cases were diagnosed between 2005 and 2008. Two of these were family/dietary-related and spatially linked to a third. Yearly incidence of sporadic CJD per million was 1.25 across the period 1998-2018, and displayed a north-south gradient with the highest incidence in La Rioja, Navarre and the Basque Country. Genetic TSEs were observed to be clustered in the Basque Country, with a 4-fold incidence over the national rate. A total of 120 (5.6%) non-TSE sporadic, conformational, rapidly progressing neurodegenerative and vascular brain disorders were reported as suspect CJD. We conclude that TSEs in Spain displayed geographically uneven, stable medium incidences for the sporadic and genetic forms, a temporal and spatial family cluster for vCJD, and decreasing numbers for dura-mater-associated forms. The vCJD surveillance, framed within the EU network, might require continuing to cover all prion disorders. There is need for further strategic surveillance research focusing on case definition of rapid-course, conformational encephalopathies and surgical risk. Prion. 2021;15(1):94-106. 10.1080/19336896.2021.1933873 1933-690X Prion 34120571 http://hdl.handle.net/20.500.12105/13930 Transmissible spongiform encephalopathies Creutzfeldt-jakob disease Epidemiology Prion proteins Registries Surveillance Spain Human prion disease surveillance in Spain, 1993-2018: an overview