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@ARTICLE{Bernhardt:279350,
author = {Bernhardt, Alexander Maximilian and Rodríguez-Baz, Íñigo
and Aldecoa, Iban and Arranz, Javier and Arriola-Infante,
José Enrique and Maure-Blesa, Lucia and Carmona-Iragui,
Maria and Longen, Sebastian and Trossbach, Svenja Verena and
Giese, Armin and Matthias, Torsten and Benejam, Bessy and
Videla, Laura and Del Hoyo Soriano, Laura and Barroeta,
Isabel and Sanjuan, Aída and Fernández, Susana and
Vaqué-Alcázar, Lídia and Rozalem Aranha, Mateus and
Morcillo-Nieto, Alejandra O and Nübling, Georg and
Wagemann, Olivia and Stockbauer, Anna and Tondo, Mireia and
Bejanin, Alexandre and Lleó, Alberto and Alcolea, Daniel
and Molina-Porcel, Laura and Fortea, Juan and Levin,
Johannes},
title = {{A}lpha-synuclein co-pathology in {D}own
syndrome-associated {A}lzheimer's disease.},
journal = {Alzheimer's and dementia},
volume = {21},
number = {6},
issn = {1552-5260},
address = {Hoboken, NJ},
publisher = {Wiley},
reportid = {DZNE-2025-00727},
pages = {e70342},
year = {2025},
abstract = {Alpha-synuclein (αSyn) seed amplification assay (SAA)
enables in vivo study of αSyn but remains underexplored in
Down syndrome-associated Alzheimer's disease (DSAD).We
analyzed αSyn-SAA in cerebrospinal fluid (CSF) from 270
adults with Down syndrome, from the Down Alzheimer Barcelona
Neuroimaging Initiative and from the AD21 cohort from the
Department of Neurology at the University Hospital, Ludwig
Maximilian University of Munich, Germany. Neuropathological
examinations were conducted in 19 brain donors (five with
ante mortem CSF). Participants were classified as
asymptomatic or symptomatic (prodromal/dementia) Alzheimer's
disease (AD). CSF Aβ1-42/1-40, CSF and plasma p-Tau181, and
neurofilament light chain (NfL) levels were measured.
Neuropathological evaluations assessed AD neuropathological
changes and Lewy body pathology (LBP).ΑSyn-SAA was positive
in $9.2\%$ of cases, independent of age or cognitive status.
Symptomatic αSyn-positive cases exhibited higher plasma NfL
levels than αSyn-negative cases (31 vs 21 pg/mL, p =
0.027). LBP was observed in $47\%$ of necropsies. The
individual with severe neocortical LBP was
αSyn-SAA-positive.These findings highlight LBP prevalence
in DSAD but suggest current SAA may fail to detect limited
αSyn deposition.αSyn-SAA positivity in DSAD is $9.2\%,$
similar to ADAD but lower than sporadic AD. Misfolded αSyn
was detectable from early ages in individuals with DS.
Positivity rates did not vary with age or clinical status in
DS. Plasma NfL levels are higher in symptomatic αSyn-SAA
positive versus negative cases. CSF αSyn seeding activity
was associated with high neocortical LBP at necropsy.},
keywords = {Humans / Alzheimer Disease: pathology / Alzheimer Disease:
cerebrospinal fluid / alpha-Synuclein: cerebrospinal fluid /
Down Syndrome: pathology / Down Syndrome: complications /
Down Syndrome: cerebrospinal fluid / Female / Male / Middle
Aged / Aged / Amyloid beta-Peptides: cerebrospinal fluid /
Brain: pathology / Biomarkers: cerebrospinal fluid / tau
Proteins: cerebrospinal fluid / tau Proteins: blood / Adult
/ Neurofilament Proteins: cerebrospinal fluid /
Neurofilament Proteins: blood / Alzheimer's disease (Other)
/ Down syndrome (Other) / Lewy body pathology (Other) /
biomarker (Other) / neuropathology (Other) / seed
amplification assay (Other) / α‐synuclein (Other) /
alpha-Synuclein (NLM Chemicals) / Amyloid beta-Peptides (NLM
Chemicals) / Biomarkers (NLM Chemicals) / tau Proteins (NLM
Chemicals) / Neurofilament Proteins (NLM Chemicals)},
cin = {Clinical Research (Munich) / AG Levin / AG Simons},
ddc = {610},
cid = {I:(DE-2719)1111015 / I:(DE-2719)1111016 /
I:(DE-2719)1110008},
pnm = {353 - Clinical and Health Care Research (POF4-353) / 351 -
Brain Function (POF4-351)},
pid = {G:(DE-HGF)POF4-353 / G:(DE-HGF)POF4-351},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:40528443},
pmc = {pmc:PMC12173960},
doi = {10.1002/alz.70342},
url = {https://pub.dzne.de/record/279350},
}