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

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023884%3A_____%2F22%3A00009392" target="_blank" >RIV/00023884:_____/22:00009392 - isvavai.cz</a>

Nalezeny alternativní kódy

RIV/00064173:_____/22:43923227 RIV/00064165:_____/22:10442399 RIV/00216208:11110/22:10442399 RIV/00216208:11120/22:43923227 a 2 dalších

Výsledek na webu

<a href="https://www.mdpi.com/2227-9059/10/3/680" target="_blank" >https://www.mdpi.com/2227-9059/10/3/680</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.3390/biomedicines10030680" target="_blank" >10.3390/biomedicines10030680</a>

Jazyk výsledku

angličtina

Název v původním jazyce

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

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

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

Popis výsledku anglicky

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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30210 - Clinical neurology

Projekt

<a href="/cs/project/NV19-04-00090" target="_blank" >NV19-04-00090: Překrývání neurodegenerativních demencí a jejich klinickopatologické korelace: prospektivně-retrospektivní multicentrická studie</a><br>

Návaznosti

N - Vyzkumna aktivita podporovana z neverejnych zdroju

Rok uplatnění

2022

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Biomedicines

ISSN

2227-9059

e-ISSN

—

Svazek periodika

10

Číslo periodika v rámci svazku

3

Stát vydavatele periodika

CH - Švýcarská konfederace

Počet stran výsledku

14

Strana od-do

—

Kód UT WoS článku

000775872000001

EID výsledku v databázi Scopus

2-s2.0-85129860953