-
Something wrong with this record ?
Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt-Jakob Disease: A Retrospective Study in 215 Autopsy Cases
N. Jankovska, R. Rusina, J. Keller, J. Kukal, M. Bruzova, E. Parobkova, T. Olejar, R. Matej
Language English Country Switzerland
Document type Journal Article
Grant support
00064165
Ministry of Health
00064190
Ministry of Health
NV19-04-00090
Ministry of Health
142120
Charles University
Q35/LF3
Charles University
NLK
Directory of Open Access Journals
from 2013
PubMed Central
from 2013
Europe PubMed Central
from 2013
ProQuest Central
from 2013-01-01
Open Access Digital Library
from 2013-01-01
ROAD: Directory of Open Access Scholarly Resources
from 2013
- Publication type
- Journal Article MeSH
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.
References provided by Crossref.org
- 000
- 00000naa a2200000 a 4500
- 001
- bmc22009774
- 003
- CZ-PrNML
- 005
- 20220425131547.0
- 007
- ta
- 008
- 220420s2022 sz f 000 0|eng||
- 009
- AR
- 024 7_
- $a 10.3390/biomedicines10030680 $2 doi
- 035 __
- $a (PubMed)35327482
- 040 __
- $a ABA008 $b cze $d ABA008 $e AACR2
- 041 0_
- $a eng
- 044 __
- $a sz
- 100 1_
- $a Jankovska, Nikol $u Department of Pathology and Molecular Medicine, Third Faculty of Medicine, Charles University and Thomayer University Hospital, 140 59 Prague, Czech Republic $1 https://orcid.org/0000000184222628
- 245 10
- $a Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt-Jakob Disease: A Retrospective Study in 215 Autopsy Cases / $c N. Jankovska, R. Rusina, J. Keller, J. Kukal, M. Bruzova, E. Parobkova, T. Olejar, R. Matej
- 520 9_
- $a 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.
- 655 _2
- $a časopisecké články $7 D016428
- 700 1_
- $a Rusina, Robert $u Department of Neurology, Third Faculty of Medicine, Charles University and Thomayer University Hospital, 140 59 Prague, Czech Republic
- 700 1_
- $a Keller, Jiri $u Department of Neurology, Third Faculty of Medicine, Charles University and University Hospital Kralovske Vinohrady, 100 34 Prague, Czech Republic $u Department of Radiology, Na Homolce Hospital, 150 00 Prague, Czech Republic $1 https://orcid.org/0000000267997840 $7 xx0145264
- 700 1_
- $a Kukal, Jaromir $u Faculty of Nuclear Sciences and Physical Engineering, Czech Technical University, 115 19 Prague, Czech Republic
- 700 1_
- $a Bruzova, Magdalena $u Department of Pathology and Molecular Medicine, Third Faculty of Medicine, Charles University and Thomayer University Hospital, 140 59 Prague, Czech Republic $1 https://orcid.org/0000000266037602
- 700 1_
- $a Parobkova, Eva $u Department of Pathology and Molecular Medicine, Third Faculty of Medicine, Charles University and Thomayer University Hospital, 140 59 Prague, Czech Republic
- 700 1_
- $a Olejar, Tomas $u Department of Pathology and Molecular Medicine, Third Faculty of Medicine, Charles University and Thomayer University Hospital, 140 59 Prague, Czech Republic $1 https://orcid.org/0000000207865029 $7 xx0242541
- 700 1_
- $a Matej, Radoslav $u Department of Pathology and Molecular Medicine, Third Faculty of Medicine, Charles University and Thomayer University Hospital, 140 59 Prague, Czech Republic $u Department of Pathology, First Faculty of Medicine, Charles University and General University Hospital, 128 00 Prague, Czech Republic $u Department of Pathology, Third Faculty of Medicine, Charles University and University Hospital Kralovske Vinohrady, 100 34 Prague, Czech Republic $1 https://orcid.org/0000000261526343
- 773 0_
- $w MED00205373 $t Biomedicines $x 2227-9059 $g Roč. 10, č. 3 (2022)
- 856 41
- $u https://pubmed.ncbi.nlm.nih.gov/35327482 $y Pubmed
- 910 __
- $a ABA008 $b sig $c sign $y - $z 0
- 990 __
- $a 20220420 $b ABA008
- 991 __
- $a 20220425131545 $b ABA008
- 999 __
- $a ind $b bmc $g 1784392 $s 1160972
- BAS __
- $a 3
- BAS __
- $a PreBMC
- BMC __
- $a 2022 $b 10 $c 3 $e 20220316 $i 2227-9059 $m Biomedicines $n Biomedicines $x MED00205373
- GRA __
- $a 00064165 $p Ministry of Health
- GRA __
- $a 00064190 $p Ministry of Health
- GRA __
- $a NV19-04-00090 $p Ministry of Health
- GRA __
- $a 142120 $p Charles University
- GRA __
- $a Q35/LF3 $p Charles University
- LZP __
- $a Pubmed-20220420
