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@ARTICLE{Respondek:157718,
author = {Respondek, Gesine and Höglinger, Günter},
title = {{D}escribe{PSP} and {P}ro{PSP}: {G}erman {M}ulticenter
{N}etworks for {S}tandardized {P}rospective {C}ollection of
{C}linical {D}ata, {I}maging {D}ata, and {B}iomaterials of
{P}atients {W}ith {P}rogressive {S}upranuclear {P}alsy.},
journal = {Frontiers in neurology},
volume = {12},
issn = {1664-2295},
address = {Lausanne},
publisher = {Frontiers Research Foundation},
reportid = {DZNE-2021-01175},
pages = {644064},
year = {2021},
abstract = {Background: The German research networks DescribePSP and
ProPSP prospectively collect comprehensive clinical data,
imaging data and biomaterials of patients with a clinical
diagnosis of progressive supranuclear palsy. Progressive
supranuclear palsy is a rare, adult-onset, neurodegenerative
disease with striking clinical heterogeneity. Since now,
prospective natural history data are largely lacking.
Clinical research into treatment strategies has been limited
due to delay in clinical diagnosis and lack of natural
history data on distinct clinical phenotypes. Methods: The
DescribePSP network is organized by the German Center for
Neurodegenerative Diseases. DescribePSP is embedded in a
larger network with parallel cohorts of other
neurodegenerative diseases and healthy controls. The
DescribePSP network is directly linked to other Describe
cohorts with other primary diagnoses of the
neurodegenerative and vascular disease spectrums and also to
an autopsy program for clinico-pathological correlation. The
ProPSP network is organized by the German Parkinson and
Movement Disorders Society. Both networks follow the same
core protocol for patient recruitment and collection of
data, imaging and biomaterials. Both networks host a
web-based data registry and a central biorepository.
Inclusion/exclusion criteria follow the 2017 Movement
Disorder Society criteria for the clinical diagnosis of
progressive supranuclear palsy. Results: Both networks
started recruitment of patients by the end of 2015. As of
November 2020, N = 354 and 269 patients were recruited into
the DescribePSP and the ProPSP studies, respectively, and N
= 131 and 87 patients received at least one follow-up visit.
Conclusions: The DescribePSP and ProPSP networks are ideal
resources for comprehensive natural history data of PSP,
including imaging data and biological samples. In contrast
to previous natural history studies, DescribePSP and ProPSP
include not only patients with Richardson's syndrome, but
also variant PSP phenotypes as well as patients at very
early disease stages, before a diagnosis of possible or
probable PSP can be made. This will allow for identification
and evaluation of early biomarkers for diagnosis, prognosis,
and progression.},
keywords = {biobank (Other) / corticobasal syndrome (Other) / disease
networks (Other) / natural history (Other) / progressive
supranuclear palsy (Other) / rare neurological disease
(Other)},
cin = {Clinical Research (Munich)},
ddc = {610},
cid = {I:(DE-2719)1111015},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
experiment = {EXP:(DE-2719)DESCRIBE-PSP-20160101},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:34113306},
pmc = {pmc:PMC8186498},
doi = {10.3389/fneur.2021.644064},
url = {https://pub.dzne.de/record/157718},
}