Survival Patterns of Human Prion Diseases in Spain, 1998–2018: Clinical Phenotypes and Etiological Clues
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...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2022-01-01
|
| Series: | Frontiers in Neuroscience |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fnins.2021.773727/full |
| _version_ | 1818744490036297728 |
|---|---|
| author | Laura Tejedor-Romero Laura Tejedor-Romero Teresa López-Cuadrado Javier Almazán-Isla Javier Almazán-Isla Miguel Calero Miguel Calero Miguel Calero Fernando J. García López Fernando J. García López Jesús de Pedro-Cuesta Jesús de Pedro-Cuesta |
| author_facet | Laura Tejedor-Romero Laura Tejedor-Romero Teresa López-Cuadrado Javier Almazán-Isla Javier Almazán-Isla Miguel Calero Miguel Calero Miguel Calero Fernando J. García López Fernando J. García López Jesús de Pedro-Cuesta Jesús de Pedro-Cuesta |
| author_sort | Laura Tejedor-Romero |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-18T02:45:08Z |
| format | Article |
| id | doaj.art-c232f454131d45a9848dcb85ec52092a |
| institution | Directory Open Access Journal |
| issn | 1662-453X |
| language | English |
| last_indexed | 2024-12-18T02:45:08Z |
| publishDate | 2022-01-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neuroscience |
| spelling | doaj.art-c232f454131d45a9848dcb85ec52092a2022-12-21T21:23:34ZengFrontiers Media S.A.Frontiers in Neuroscience1662-453X2022-01-011510.3389/fnins.2021.773727773727Survival Patterns of Human Prion Diseases in Spain, 1998–2018: Clinical Phenotypes and Etiological CluesLaura Tejedor-Romero0Laura Tejedor-Romero1Teresa López-Cuadrado2Javier Almazán-Isla3Javier Almazán-Isla4Miguel Calero5Miguel Calero6Miguel Calero7Fernando J. García López8Fernando J. García López9Jesús de Pedro-Cuesta10Jesús de Pedro-Cuesta11Department of Neurodegeneration, Ageing and Mental Health, National Centre for Epidemiology, Carlos III Institute of Health, Madrid, SpainPreventive Medicine Unit, La Princesa University Teaching Hospital, Madrid, SpainDepartment of Neurodegeneration, Ageing and Mental Health, National Centre for Epidemiology, Carlos III Institute of Health, Madrid, SpainDepartment of Neurodegeneration, Ageing and Mental Health, National Centre for Epidemiology, Carlos III Institute of Health, Madrid, SpainConsortium for Biomedical Research in Neurodegenerative Diseases (CIBERNED), Madrid, SpainConsortium for Biomedical Research in Neurodegenerative Diseases (CIBERNED), Madrid, SpainAlzheimer’s Disease Research Unit, Fundación CIEN (Centro de Investigación de Enfermedades Neurológicas), Queen Sofia Foundation Alzheimer Centre, Madrid, SpainChronic Disease Programme, Carlos III Institute of Health, Madrid, SpainDepartment of Neurodegeneration, Ageing and Mental Health, National Centre for Epidemiology, Carlos III Institute of Health, Madrid, SpainConsortium for Biomedical Research in Neurodegenerative Diseases (CIBERNED), Madrid, SpainDepartment of Neurodegeneration, Ageing and Mental Health, National Centre for Epidemiology, Carlos III Institute of Health, Madrid, SpainConsortium for Biomedical Research in Neurodegenerative Diseases (CIBERNED), Madrid, SpainBackgroundHuman 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.https://www.frontiersin.org/articles/10.3389/fnins.2021.773727/fullsurvivalhuman spongiform encephalopathiesprognostic factorsclinical phenotypessporadic Creutzfeld-Jakob disease |
| spellingShingle | Laura Tejedor-Romero Laura Tejedor-Romero Teresa López-Cuadrado Javier Almazán-Isla Javier Almazán-Isla Miguel Calero Miguel Calero Miguel Calero Fernando J. García López Fernando J. García López Jesús de Pedro-Cuesta Jesús de Pedro-Cuesta Survival Patterns of Human Prion Diseases in Spain, 1998–2018: Clinical Phenotypes and Etiological Clues Frontiers in Neuroscience survival human spongiform encephalopathies prognostic factors clinical phenotypes sporadic Creutzfeld-Jakob disease |
| title | Survival Patterns of Human Prion Diseases in Spain, 1998–2018: Clinical Phenotypes and Etiological Clues |
| title_full | Survival Patterns of Human Prion Diseases in Spain, 1998–2018: Clinical Phenotypes and Etiological Clues |
| title_fullStr | Survival Patterns of Human Prion Diseases in Spain, 1998–2018: Clinical Phenotypes and Etiological Clues |
| title_full_unstemmed | Survival Patterns of Human Prion Diseases in Spain, 1998–2018: Clinical Phenotypes and Etiological Clues |
| title_short | Survival Patterns of Human Prion Diseases in Spain, 1998–2018: Clinical Phenotypes and Etiological Clues |
| title_sort | survival patterns of human prion diseases in spain 1998 2018 clinical phenotypes and etiological clues |
| topic | survival human spongiform encephalopathies prognostic factors clinical phenotypes sporadic Creutzfeld-Jakob disease |
| url | https://www.frontiersin.org/articles/10.3389/fnins.2021.773727/full |
| work_keys_str_mv | AT lauratejedorromero survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues AT lauratejedorromero survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues AT teresalopezcuadrado survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues AT javieralmazanisla survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues AT javieralmazanisla survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues AT miguelcalero survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues AT miguelcalero survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues AT miguelcalero survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues AT fernandojgarcialopez survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues AT fernandojgarcialopez survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues AT jesusdepedrocuesta survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues AT jesusdepedrocuesta survivalpatternsofhumanpriondiseasesinspain19982018clinicalphenotypesandetiologicalclues |