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dc.contributor.authorPedro-Cuesta, Jesus de 
dc.contributor.authorAlmazan-Isla, Javier 
dc.contributor.authorTejedor-Romero, Laura 
dc.contributor.authorRuiz-Tovar, María 
dc.contributor.authorAvellanal, Fuencisla 
dc.contributor.authorRábano, Alberto
dc.contributor.authorCalero, Miguel 
dc.contributor.authorGarcia Lopez, Fernando Jose 
dc.contributor.authorSpanish CJD Surveillance Group
dc.date.accessioned2022-04-06T10:51:08Z
dc.date.available2022-04-06T10:51:08Z
dc.date.issued2021-12
dc.identifier.citationPrion. 2021;15(1):94-106.es_ES
dc.identifier.urihttp://hdl.handle.net/20.500.12105/13930
dc.description.abstractIn 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.es_ES
dc.description.sponsorshipThis work was basically funded by the Spain’s Ministry of Health and the Carlos III Health Institute. Additional funding was obtained from the EU Research Commission, Concerted Actions (BIOMED2 Contract No. BMH4-CT97-2216, QLRG3-CT-2002-81223), EU Joint Program – Neurodegenerative Disease Research (JPND – DEMTEST, Spanish Health Research Fund, FIS PI11/03021 and PI12/00045), EUROCJD (funded by DG SANCO, 2003201), research grants by the Carlos III Health Institute (PI08/0139), NEUROPRION (an EU Network of Excellence funded by Framework 6 Programme, FOOD CT 2004 056579), the Spanish Centro de Investigaciones en Enfermedades Neurológicas (CIEN C03-06), and Consortium for Biomedical Research in Neurodegenerative Diseases (CIBERNED) networks.es_ES
dc.language.isoenges_ES
dc.publisherTaylor & Francis es_ES
dc.relation.isversionofPublisher's versiones_ES
dc.rights.urihttp://creativecommons.org/licenses/by-nc/4.0/*
dc.subjectTransmissible spongiform encephalopathieses_ES
dc.subjectCreutzfeldt-jakob diseasees_ES
dc.subjectEpidemiologyes_ES
dc.subjectPrion proteinses_ES
dc.subjectRegistrieses_ES
dc.subjectSurveillancees_ES
dc.subjectSpaines_ES
dc.titleHuman prion disease surveillance in Spain, 1993-2018: an overviewes_ES
dc.typeArtículoes_ES
dc.rights.licenseAtribución-NoComercial 4.0 Internacional*
dc.identifier.pubmedID34120571es_ES
dc.format.volume15es_ES
dc.format.number1es_ES
dc.format.page94-106es_ES
dc.identifier.doi10.1080/19336896.2021.1933873es_ES
dc.contributor.funderMinisterio de Sanidad (España)es_ES
dc.contributor.funderInstituto de Salud Carlos III es_ES
dc.contributor.funderUnión Europea. Comisión Europeaes_ES
dc.contributor.funderFundación Centro De Investigación De Enfermedades Neurológicases_ES
dc.contributor.funderCentro de Investigación Biomédica en Red - CIBERNED (Enfermedades Neurodegenerativas)es_ES
dc.description.peerreviewedes_ES
dc.identifier.e-issn1933-690Xes_ES
dc.identifier.journalPriones_ES
dc.repisalud.centroISCIII::Centro Nacional de Epidemologíaes_ES
dc.repisalud.centroISCIII::Unidad Funcional de Investigación de Enfermedades Crónicas (UFIEC)es_ES
dc.repisalud.institucionISCIIIes_ES
dc.relation.projectIDinfo:eu-repo/grantAgreement/EC/QLRG3-CT-2002-81223es_ES
dc.relation.projectIDinfo:eu-repo/grantAgreement/EC/BMH4-CT97-2216es_ES
dc.relation.projectIDinfo:eu-repo/grantAgreement/EC/CT 2004 056579es_ES
dc.relation.projectIDinfo:eu-repo/grantAgreement/EC/2003201es_ES
dc.rights.accessRightsinfo:eu-repo/semantics/openAccesses_ES
dc.relation.projectFECYTinfo:eu-repo/grantAgreement/ES/CIEN C03-06es_ES
dc.relation.projectFISinfo:eu-repo/grantAgreement/ES/PI11/03021es_ES
dc.relation.projectFISinfo:eu-repo/grantAgreement/ES/PI12/00045es_ES
dc.relation.projectFISinfo:eu-repo/grantAgreement/ES/PI08/0139es_ES


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