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This 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.

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Lopez-Cuadrado, TAuthor

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March 15, 2022
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Survival Patterns of Human Prion Diseases in Spain, 1998-2018: Clinical Phenotypes and Etiological Clues

Publicated to:Frontiers in Neuroscience. 15 773727- - 2022-01-20 15(), DOI: 10.3389/fnins.2021.773727

Authors: Tejedor-Romero, Laura; Lopez-Cuadrado, Teresa; Almazan-Isla, Javier; Calero, Miguel; Garcia Lopez, Fernando J.; de Pedro-Cuesta, Jesus;

Affiliations

Carlos III Inst Hlth, Chron Dis Programme, Madrid, Spain - Author
Carlos III Inst Hlth, Natl Ctr Epidemiol, Dept Neurodegenerat Ageing & Mental Hlth, Madrid, Spain - Author
Consortium Biomed Res Neurodegenerat Dis CIBERNED, Madrid, Spain - Author
La Princesa Univ Teaching Hosp, Prevent Med Unit, Madrid, Spain - Author
Queen Sofia Fdn Alzheimer Ctr, Alzheimers Dis Res Unit, Fdn CIEN Ctr Invest Enfermedades Neurol, Madrid, Spain - Author
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Abstract

BackgroundHuman 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. MethodsSurvival 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. FindingsMedian 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. InterpretationTSE 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.

Keywords

clinical phenotypeshuman spongiform encephalopathiesprognostic factorssporadic creutzfeld-jakob diseaseClinical phenotypesCreutzfeldt-jakob-diseaseHuman spongiform encephalopathiesMortalityPrognostic factorsSporadic creutzfeld-jakob diseaseSurveillanceSurvival

Quality index

Bibliometric impact. Analysis of the contribution and dissemination channel

The work has been published in the journal Frontiers in Neuroscience due to its progression and the good impact it has achieved in recent years, according to the agency WoS (JCR), it has become a reference in its field. In the year of publication of the work, 2022, it was in position 94/272, thus managing to position itself as a Q2 (Segundo Cuartil), in the category Neurosciences. Notably, the journal is positioned en el Cuartil Q2 para la agencia Scopus (SJR) en la categoría Neuroscience (Miscellaneous).

From a relative perspective, and based on the normalized impact indicator calculated from the Field Citation Ratio (FCR) of the Dimensions source, it yields a value of: 2.03, which indicates that, compared to works in the same discipline and in the same year of publication, it ranks as a work cited above average. (source consulted: Dimensions Jul 2025)

Specifically, and according to different indexing agencies, this work has accumulated citations as of 2025-07-06, the following number of citations:

  • Scopus: 3

Impact and social visibility

From the perspective of influence or social adoption, and based on metrics associated with mentions and interactions provided by agencies specializing in calculating the so-called "Alternative or Social Metrics," we can highlight as of 2025-07-06:

  • The use, from an academic perspective evidenced by the Altmetric agency indicator referring to aggregations made by the personal bibliographic manager Mendeley, gives us a total of: 2.
  • The use of this contribution in bookmarks, code forks, additions to favorite lists for recurrent reading, as well as general views, indicates that someone is using the publication as a basis for their current work. This may be a notable indicator of future more formal and academic citations. This claim is supported by the result of the "Capture" indicator, which yields a total of: 3 (PlumX).

With a more dissemination-oriented intent and targeting more general audiences, we can observe other more global scores such as:

  • The Total Score from Altmetric: 10.75.
  • The number of mentions on the social network X (formerly Twitter): 3 (Altmetric).
  • The number of mentions in news outlets: 1 (Altmetric).

It is essential to present evidence supporting full alignment with institutional principles and guidelines on Open Science and the Conservation and Dissemination of Intellectual Heritage. A clear example of this is:

  • The work has been submitted to a journal whose editorial policy allows open Open Access publication.