Primary Progressive Aphasia (disorder)
|
0.300 |
Biomarker
|
disease |
BEFREE |
There were no significant differences between clinical syndromes (PPA subtypes), the main clinical forms of dementia (frontotemporal dementia and AD), or the expected pathological groups (frontotemporal lobar degeneration-tau [FTLD-tau], FTLD-TDP43, and AD).
|
31640103 |
2019 |
Primary Progressive Aphasia (disorder)
|
0.300 |
Biomarker
|
disease |
BEFREE |
Seventy-three presenile subjects were included: Amyloid/Tau group (33 Alzheimer's disease with confirmed cerebrospinal fluid [CSF] biomarkers), probable TDP-43 group (7 semantic variant progressive primary aphasia, 5 GRN and 2 C9orf72 mutation carriers) and 26 healthy controls.
|
29935415 |
2018 |
Primary Progressive Aphasia (disorder)
|
0.300 |
Biomarker
|
disease |
BEFREE |
This study investigated the presence of combined pathologies in a large cohort of autopsies that show a primary pathologic diagnosis of phosphorylated 43-kDa TAR DNA-binding protein (FTLD-TDP), the majority of which portrayed clinical phenotypes consistent with primary progressive aphasia or behavioral variant frontotemporal dementia (bvFTD).
|
29584904 |
2018 |
Primary Progressive Aphasia (disorder)
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
Antibodies to phospho-TDP-43, NeuN (neuronal nuclei), and HLA-DR were used to visualize inclusions, neurons, and activated microglia in paraffin-embedded tissue sections from 4 participants with PPA: 2 of the agrammatic and 2 of the logopenic subtype.
|
26791154 |
2016 |
Primary Progressive Aphasia (disorder)
|
0.300 |
Biomarker
|
disease |
BEFREE |
Our results show that while pathogenic variants within the most common dementia genes were rarely associated with PPA, these were found almost exclusively in GRN and C9orf72, suggesting that PPA is more TDP43- than tau-related in our series.
|
30599136 |
2019 |
Primary Progressive Aphasia (disorder)
|
0.300 |
Biomarker
|
disease |
BEFREE |
Activated microglia and TDP-43 inclusions were visualized in whole-hemisphere brain sections using immunohistochemical methods from five participants with PPA-TDP.
|
29665116 |
2018 |
Primary Progressive Aphasia (disorder)
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
In an autopsy cohort of PPA (FTLD-TDP = 13, FTLD-Tau = 14), we analyzed laterality and regional distribution of postmortem pathology, quantified using a validated digital histopathological approach, in available brain tissue from up to 8 cortical regions bilaterally.
|
30851133 |
2019 |
Primary Progressive Aphasia (disorder)
|
0.300 |
Biomarker
|
disease |
BEFREE |
Brain sections from 5 PPA participants with postmortem diagnoses of frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP) were immunohistochemically stained using an antibody to phosphorylated TDP-43 and quantitatively examined for regional and hemispheric distribution using unbiased stereology.
|
30753613 |
2019 |
Primary Progressive Aphasia (disorder)
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
All prior TDP-43 type C cases from the UCL FTD cohort (n=25) had a semantic variant PPA (svPPA) phenotype, with all having a younger age at onset and longer disease duration than the nfvPPA case.
|
31747857 |
2020 |
Primary Progressive Aphasia (disorder)
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
To quantitatively examine the regional densities and hemispheric distribution of the 43-kDa transactive response DNA-binding protein (TDP-43) inclusions, neurons, and activated microglia in a left-handed patient with right hemisphere language dominance and logopenic-variant primary progressive aphasia (PPA).
|
29305438 |
2018 |
Primary Progressive Aphasia (disorder)
|
0.300 |
Biomarker
|
disease |
BEFREE |
These results demonstrate a phenotypically concordant distribution of abnormal TDP-43 inclusions in primary progressive aphasia (PPA).
|
20479359 |
2010 |
Primary Progressive Aphasia (disorder)
|
0.300 |
Biomarker
|
disease |
BEFREE |
Autopsy data revealed Alzheimer disease pathology as the most common pathologic diagnosis in lvPPA (76%), frontotemporal lobar degeneration-TDP-43 in svPPA (80%), and frontotemporal lobar degeneration-TDP-43/tau in nfvPPA (64%).
|
30255971 |
2018 |
Primary Progressive Aphasia (disorder)
|
0.300 |
Biomarker
|
disease |
MGD |
|
|
|