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Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt–Jakob Disease: A Retrospective Study in 215 Autopsy Cases

Creutzfeldt–Jakob disease (CJD), the most common human prion disorder, may occur as “pure” neurodegeneration with isolated prion deposits in the brain tissue; however, comorbid cases with different concomitant neurodegenerative diseases have been reported. This retrospective study examined correlati...

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Autores principales: Jankovska, Nikol, Rusina, Robert, Keller, Jiri, Kukal, Jaromir, Bruzova, Magdalena, Parobkova, Eva, Olejar, Tomas, Matej, Radoslav
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8944998/
https://www.ncbi.nlm.nih.gov/pubmed/35327482
http://dx.doi.org/10.3390/biomedicines10030680
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author Jankovska, Nikol
Rusina, Robert
Keller, Jiri
Kukal, Jaromir
Bruzova, Magdalena
Parobkova, Eva
Olejar, Tomas
Matej, Radoslav
author_facet Jankovska, Nikol
Rusina, Robert
Keller, Jiri
Kukal, Jaromir
Bruzova, Magdalena
Parobkova, Eva
Olejar, Tomas
Matej, Radoslav
author_sort Jankovska, Nikol
collection PubMed
description Creutzfeldt–Jakob disease (CJD), the most common human prion disorder, may occur as “pure” neurodegeneration with isolated prion deposits in the brain tissue; however, comorbid cases with different concomitant neurodegenerative diseases have been reported. This retrospective study examined correlations of clinical, neuropathological, molecular-genetic, immunological, and neuroimaging biomarkers in pure and comorbid CJD. A total of 215 patients have been diagnosed with CJD during the last ten years by the Czech National Center for Prion Disorder Surveillance. Data were collected from all patients with respect to diagnostic criteria for probable CJD, including clinical description, EEG, MRI, and CSF findings. A detailed neuropathological analysis uncovered that only 11.16% were “pure” CJD, while 62.79% had comorbid tauopathy, 20.47% had Alzheimer’s disease, 3.26% had frontotemporal lobar degeneration, and 2.33% had synucleinopathy. The comorbid subgroup analysis revealed that tauopathy was linked to putaminal hyperintensity on MRIs, and AD mainly impacted the age of onset, hippocampal atrophy on MRIs, and beta-amyloid levels in the CSF. The retrospective data analysis found a surprisingly high proportion of comorbid neuropathologies; only 11% of cases were verified as “pure” CJD, i.e., lacking hallmarks of other neurodegenerations. Comorbid neuropathologies can impact disease manifestation and can complicate the clinical diagnosis of CJD.
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spelling pubmed-89449982022-03-25 Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt–Jakob Disease: A Retrospective Study in 215 Autopsy Cases Jankovska, Nikol Rusina, Robert Keller, Jiri Kukal, Jaromir Bruzova, Magdalena Parobkova, Eva Olejar, Tomas Matej, Radoslav Biomedicines Article Creutzfeldt–Jakob disease (CJD), the most common human prion disorder, may occur as “pure” neurodegeneration with isolated prion deposits in the brain tissue; however, comorbid cases with different concomitant neurodegenerative diseases have been reported. This retrospective study examined correlations of clinical, neuropathological, molecular-genetic, immunological, and neuroimaging biomarkers in pure and comorbid CJD. A total of 215 patients have been diagnosed with CJD during the last ten years by the Czech National Center for Prion Disorder Surveillance. Data were collected from all patients with respect to diagnostic criteria for probable CJD, including clinical description, EEG, MRI, and CSF findings. A detailed neuropathological analysis uncovered that only 11.16% were “pure” CJD, while 62.79% had comorbid tauopathy, 20.47% had Alzheimer’s disease, 3.26% had frontotemporal lobar degeneration, and 2.33% had synucleinopathy. The comorbid subgroup analysis revealed that tauopathy was linked to putaminal hyperintensity on MRIs, and AD mainly impacted the age of onset, hippocampal atrophy on MRIs, and beta-amyloid levels in the CSF. The retrospective data analysis found a surprisingly high proportion of comorbid neuropathologies; only 11% of cases were verified as “pure” CJD, i.e., lacking hallmarks of other neurodegenerations. Comorbid neuropathologies can impact disease manifestation and can complicate the clinical diagnosis of CJD. MDPI 2022-03-16 /pmc/articles/PMC8944998/ /pubmed/35327482 http://dx.doi.org/10.3390/biomedicines10030680 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Jankovska, Nikol
Rusina, Robert
Keller, Jiri
Kukal, Jaromir
Bruzova, Magdalena
Parobkova, Eva
Olejar, Tomas
Matej, Radoslav
Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt–Jakob Disease: A Retrospective Study in 215 Autopsy Cases
title Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt–Jakob Disease: A Retrospective Study in 215 Autopsy Cases
title_full Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt–Jakob Disease: A Retrospective Study in 215 Autopsy Cases
title_fullStr Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt–Jakob Disease: A Retrospective Study in 215 Autopsy Cases
title_full_unstemmed Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt–Jakob Disease: A Retrospective Study in 215 Autopsy Cases
title_short Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt–Jakob Disease: A Retrospective Study in 215 Autopsy Cases
title_sort biomarkers analysis and clinical manifestations in comorbid creutzfeldt–jakob disease: a retrospective study in 215 autopsy cases
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8944998/
https://www.ncbi.nlm.nih.gov/pubmed/35327482
http://dx.doi.org/10.3390/biomedicines10030680
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