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dc.contributor.authorTejedor-Romero, Laura 
dc.contributor.authorLopez-Cuadrado, Teresa 
dc.contributor.authorAlmazan-Isla, Javier 
dc.contributor.authorCalero, Miguel 
dc.contributor.authorGarcia Lopez, Fernando Jose 
dc.contributor.authorPedro-Cuesta, Jesus de
dc.identifier.citationFront Neurosci. 2022 Jan 20;15:773727.es_ES
dc.description.abstractBackground: Human transmissible spongiform encephalopathies (TSEs) are a group of fatal neurodegenerative disorders of short duration. There are few studies on TSE survival. This study sought to analyze the survival and related factors of a TSE patient cohort, based on a nationwide surveillance system in Spain. Methods: Survival analyses were performed on 1,530 cases diagnosed across the period 1998–2018 in Spain. We calculated median survival times and plotted survival curves using the Kaplan–Meier method for all cases and for sporadic TSE (sTSE) and genetic TSE (gTSE). Crude and adjusted Cox proportional hazard models were used to identify variables associated with shorter survival. Findings: Median age at onset decreased from the sporadic forms to gTSE and, lastly, to acquired TSE. Overall median and interquartile range (IQR) survival time was 5.2 (IQR, 3.0–11.7) months and 4.9 (IQR, 2.8–10.8) months in sporadic cases and 9 (IQR, 4.9 to over 12) months in genetic cases, p < 0.001. Male sex, older age at onset, presence of 14-3-3 protein, typical MRI, and MM and VV polymorphisms at codon 129 were associated with shorter survival. gTSE showed higher survival in crude comparisons but not after adjustment. Interpretation: TSE survival in Spain replicates both the magnitude of that shown and the TSE entity-specific population patterns observed in Western countries but differs from features described in Asian populations, such as the Japanese. The reduction in differences in survival between gTSE and sTSE on adjusting for covariates and international patterns might support the view that gTSE and sTSE share causal and pathophysiological features.es_ES
dc.description.sponsorshipThis work was basically funded by the Spain’s Ministry of Health and the Carlos III Health Institute (research grant no. PI08/0139) and Consortium for Biomedical Research in Neurodegenerative Diseases (CIBERNED) networks (QLRG3-CT-2002-81223). Role of funders: The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.es_ES
dc.publisherFrontiers Media es_ES
dc.relation.isversionofPublisher's versiones_ES
dc.subjectClinical phenotypeses_ES
dc.subjectHuman spongiform encephalopathieses_ES
dc.subjectPrognostic factorses_ES
dc.subjectSporadic Creutzfeld-Jakob diseasees_ES
dc.titleSurvival Patterns of Human Prion Diseases in Spain, 1998-2018: Clinical Phenotypes and Etiological Clueses_ES
dc.rights.licenseAtribución 4.0 Internacional*
dc.contributor.funderCentro de Investigación Biomédica en Red - CIBERNED (Enfermedades Neurodegenerativas)es_ES
dc.contributor.funderInstituto de Salud Carlos III es_ES
dc.contributor.funderMinisterio de Sanidad (España)es_ES
dc.identifier.journalFrontiers in Neurosciencees_ES
dc.repisalud.centroISCIII::Centro Nacional de Epidemologíaes_ES
dc.repisalud.centroISCIII::Unidad Funcional de Investigación de Enfermedades Crónicas (UFIEC)es_ES

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