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@ARTICLE{JecmenicaLukic:153339,
author = {Jecmenica Lukic, Milica and Kurz, Carolin and Respondek,
Gesine and Grau-Rivera, Oriol and Compta, Yaroslau and
Gelpi, Ellen and Troakes, Claire and Barcelona Brain Bank
collaborative group, the MDS-endorsed PSP study group and
van Swieten, John C and Giese, Armin and Roeber, Sigrun and
Arzberger, Thomas and Höglinger, Günter and Grau-Rivera,
O. and Compta, Y. and Tolosa, E. and Martí, M. J. and
Valldeoriola, F. and Pagonabarraga, J. and Calopa, M. and
Bayès, A. and Hernandez, I. and Aguilar, M. and Genis, D.
and Fernandez, M. and Munoz-Garcia, C. and Antonell, A. and
Gelpi, E.},
title = {{C}opathology in {P}rogressive {S}upranuclear {P}alsy:
{D}oes {I}t {M}atter?},
journal = {Movement disorders},
volume = {35},
number = {6},
issn = {1531-8257},
address = {New York, NY},
publisher = {Wiley},
reportid = {DZNE-2020-01336},
pages = {984 - 993},
year = {2020},
abstract = {The influence of concomitant brain pathologies on the
progression rate in PSP is unclear.To analyze the frequency
and severity of copathologies and their impact on the
progression in PSP.We analyzed clinic-pathological features
of 101 PSP patients. Diagnoses and stages of copathologies
were established according to standardized criteria,
including Alzheimer's disease-related pathology,
argyrophilic grains, Lewy-related pathology, transactive
response DNA-binding protein 43 pathology, fused in sarcoma
pathology, cerebral amyloid angiopathy, and small vessel
disease. Demographic data and major clinical milestones
(frequency and latency to onset) were extracted from
patients' files.Only $8\%$ of 101 patients presented with
pure PSP pathology without any copathology. Alzheimer's
disease-related pathology was the most frequent $(84\%),$
followed by argyrophilic grains $(58\%),$ both occurring as
single copathology or in combination with other
proteinopathies or cerebrovascular disease. Lewy-related and
transactive response DNA-binding protein 43 copathology
occurred rarely $(8\%$ and $6\%,$ respectively). Fused in
sarcoma-positive cases were not found. While being common,
copathology was mostly mild in severity, with the exception
of frequently widespread argyrophilic grains. Small vessel
disease was also frequent $(65\%).$ Cerebral amyloid
angiopathy occurred only in the presence of Alzheimer's
disease-related changes $(25\%).$ The copathologies did not
have major impact on prevalence and time frame of major
disease milestones.In PSP, concomitant neurodegenerative
proteinopathies or cerebrovascular diseases are frequent,
but generally mild in severity. Our data confirmed that four
repeat tau is still the most relevant target for PSP,
whereas the impact of copathologies on progression rate
appears to be of less importance. This is relevant
information for the development of disease-modifying
therapies. © 2020 The Authors. Movement Disorders published
by Wiley Periodicals, Inc. on behalf of International
Parkinson and Movement Disorder Society.},
keywords = {Alzheimer Disease: epidemiology / Brain: metabolism /
Humans / Movement Disorders / Supranuclear Palsy,
Progressive: epidemiology / tau Proteins: metabolism},
cin = {AG Höglinger 2 / AG Höglinger 1 / München Pre 2020 /
Neuropathology / Brainbank},
ddc = {610},
cid = {I:(DE-2719)1111015 / I:(DE-2719)1110002 /
I:(DE-2719)6000016 / I:(DE-2719)1140013},
pnm = {344 - Clinical and Health Care Research (POF3-344)},
pid = {G:(DE-HGF)POF3-344},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:32125724},
doi = {10.1002/mds.28011},
url = {https://pub.dzne.de/record/153339},
}