% IMPORTANT: The following is UTF-8 encoded. This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.
@ARTICLE{Siderowf:257678,
author = {Siderowf, Andrew and Concha-Marambio, Luis and Lafontant,
David-Erick and Farris, Carly M and Ma, Yihua and Urenia,
Paula A and Nguyen, Hieu and Alcalay, Roy N and Chahine,
Lana M and Foroud, Tatiana and Galasko, Douglas and
Kieburtz, Karl and Merchant, Kalpana and Mollenhauer, Brit
and Poston, Kathleen L and Seibyl, John and Simuni, Tanya
and Tanner, Caroline M and Weintraub, Daniel and Videnovic,
Aleksandar and Choi, Seung Ho and Kurth, Ryan and
Caspell-Garcia, Chelsea and Coffey, Christopher S and
Frasier, Mark and Oliveira, Luis M A and Hutten, Samantha J
and Sherer, Todd and Marek, Kenneth and Soto, Claudio},
collaboration = {Initiative, Parkinson's Progression Markers},
title = {{A}ssessment of heterogeneity among participants in the
{P}arkinson's {P}rogression {M}arkers {I}nitiative cohort
using α-synuclein seed amplification: a cross-sectional
study.},
journal = {The lancet / Neurology},
volume = {22},
number = {5},
issn = {1474-4422},
address = {London},
publisher = {Lancet Publ. Group},
reportid = {DZNE-2023-00475},
pages = {407 - 417},
year = {2023},
abstract = {Emerging evidence shows that α-synuclein seed
amplification assays (SAAs) have the potential to
differentiate people with Parkinson's disease from healthy
controls. We used the well characterised, multicentre
Parkinson's Progression Markers Initiative (PPMI) cohort to
further assess the diagnostic performance of the
α-synuclein SAA and to examine whether the assay identifies
heterogeneity among patients and enables the early
identification of at-risk groups.This cross-sectional
analysis is based on assessments done at enrolment for PPMI
participants (including people with sporadic Parkinson's
disease from LRRK2 and GBA variants, healthy controls,
prodromal individuals with either rapid eye movement sleep
behaviour disorder (RBD) or hyposmia, and non-manifesting
carriers of LRRK2 and GBA variants) from 33 participating
academic neurology outpatient practices worldwide (in
Austria, Canada, France, Germany, Greece, Israel, Italy, the
Netherlands, Norway, Spain, the UK, and the USA).
α-synuclein SAA analysis of CSF was performed using
previously described methods. We assessed the sensitivity
and specificity of the α-synuclein SAA in participants with
Parkinson's disease and healthy controls, including
subgroups based on genetic and clinical features. We
established the frequency of positive α-synuclein SAA
results in prodromal participants (RBD and hyposmia) and
non-manifesting carriers of genetic variants associated with
Parkinson's disease, and compared α-synuclein SAA to
clinical measures and other biomarkers. We used odds ratio
estimates with $95\%$ CIs to measure the association between
α-synuclein SAA status and categorical measures, and
two-sample $95\%$ CIs from the resampling method to assess
differences in medians between α-synuclein SAA positive and
negative participants for continuous measures. A linear
regression model was used to control for potential
confounders such as age and sex.This analysis included 1123
participants who were enrolled between July 7, 2010, and
July 4, 2019. Of these, 545 had Parkinson's disease, 163
were healthy controls, 54 were participants with scans
without evidence of dopaminergic deficit, 51 were prodromal
participants, and 310 were non-manifesting carriers.
Sensitivity for Parkinson's disease was $87·7\%$ $(95\%$ CI
84·9-90·5), and specificity for healthy controls was
$96·3\%$ (93·4-99·2). The sensitivity of the α-synuclein
SAA in sporadic Parkinson's disease with the typical
olfactory deficit was $98·6\%$ (96·4-99·4). The
proportion of positive α-synuclein SAA was lower than this
figure in subgroups including LRRK2 Parkinson's disease
$(67·5\%$ [59·2-75·8]) and participants with sporadic
Parkinson's disease without olfactory deficit $(78·3\%$
[69·8-86·7]). Participants with LRRK2 variant and normal
olfaction had an even lower α-synuclein SAA positivity rate
$(34·7\%$ [21·4-48·0]). Among prodromal and at-risk
groups, 44 $(86\%)$ of 51 of participants with RBD or
hyposmia had positive α-synuclein SAA (16 of 18 with
hyposmia, and 28 of 33 with RBD). 25 $(8\%)$ of 310
non-manifesting carriers (14 of 159 $[9\%]$ LRRK2 and 11 of
151 $[7\%]$ GBA) were positive.This study represents the
largest analysis so far of the α-synuclein SAA for the
biochemical diagnosis of Parkinson's disease. Our results
show that the assay classifies people with Parkinson's
disease with high sensitivity and specificity, provides
information about molecular heterogeneity, and detects
prodromal individuals before diagnosis. These findings
suggest a crucial role for the α-synuclein SAA in
therapeutic development, both to identify pathologically
defined subgroups of people with Parkinson's disease and to
establish biomarker-defined at-risk cohorts.PPMI is funded
by the Michael J Fox Foundation for Parkinson's Research and
funding partners, including: Abbvie, AcureX, Aligning
Science Across Parkinson's, Amathus Therapeutics, Avid
Radiopharmaceuticals, Bial Biotech, Biohaven, Biogen,
BioLegend, Bristol-Myers Squibb, Calico Labs, Celgene,
Cerevel, Coave, DaCapo Brainscience, 4D Pharma, Denali,
Edmond J Safra Foundation, Eli Lilly, GE Healthcare,
Genentech, GlaxoSmithKline, Golub Capital, Insitro, Janssen
Neuroscience, Lundbeck, Merck, Meso Scale Discovery,
Neurocrine Biosciences, Prevail Therapeutics, Roche, Sanofi
Genzyme, Servier, Takeda, Teva, UCB, VanquaBio, Verily,
Voyager Therapeutics, and Yumanity.},
keywords = {Humans / alpha-Synuclein: genetics / Parkinson Disease:
diagnosis / Parkinson Disease: genetics / Cross-Sectional
Studies / Anosmia / REM Sleep Behavior Disorder / Biomarkers
/ alpha-Synuclein (NLM Chemicals) / Biomarkers (NLM
Chemicals)},
cin = {AG Fischer},
ddc = {610},
cid = {I:(DE-2719)1410002},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pmc = {pmc:PMC10627170},
pubmed = {pmid:37059509},
doi = {10.1016/S1474-4422(23)00109-6},
url = {https://pub.dzne.de/record/257678},
}