2026-08-05T11:45:36Zhttps://repisalud.isciii.es/rest/oai/requestoai: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
Repisalud
author
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
funder
Ministerio de Sanidad (España)
funder
Instituto de Salud Carlos III
funder
Unión Europea. Comisión Europea
funder
Fundación Centro De Investigación De Enfermedades Neurológicas
funder
Centro de Investigación Biomédica en Red - CIBERNED (Enfermedades Neurodegenerativas)
2022-04-06T10:51:08Z
2022-04-06T10:51:08Z
2021-12
Prion. 2021;15(1):94-106.
10.1080/19336896.2021.1933873
1933-690X
Prion
34120571
http://hdl.handle.net/20.500.12105/13930
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.
eng
Transmissible spongiform encephalopathies
Creutzfeldt-jakob disease
Epidemiology
Prion proteins
Registries
Surveillance
Spain
Human prion disease surveillance in Spain, 1993-2018: an overview
research article
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HumanPrionDiseaseSurveillance_2021.pdf
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HumanPrionDiseaseSurveillance_2021.pdf.txt