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@ARTICLE{Germani:276156,
author = {Germani, Massimiliano and Rebollo Mesa, Irene and Buchanan,
Tim J and De Bruyn, Steven and Gasalla, Teresa and Van
Tricht, Hans Lieve G and Ewen, Colin and Golbe, Lawrence I
and Boxer, Adam and Höglinger, Günter},
title = {{C}ombined {A}ssessment of {F}unction and {S}urvival to
{D}emonstrate the {E}ffect of {T}reatment on {P}rogressive
{S}upranuclear {P}alsy.},
journal = {Movement disorders},
volume = {40},
number = {1},
issn = {0885-3185},
address = {New York, NY},
publisher = {Wiley},
reportid = {DZNE-2025-00228},
pages = {97 - 107},
year = {2025},
note = {This research was funded by UCB. G.H. was supported by the
European Joint Programme on Rare Diseases (Improve-PSP) and
the Deutsche Forschungsgemeinschaft DFG, (German Research
Foundation) under Germany's Excellence Strategy within the
framework of the Munich Cluster for Systems Neurology (EXC
2145 SyNergy ID 390857198).},
abstract = {Progressive supranuclear palsy (PSP) is a rare and fatal
neurodegenerative disorder for which there are currently no
disease-modifying treatments. Recent trials of potential
therapies had durations of 12 months, which may be
insufficient because of nonrandom missingness due to death.
Longer durations, incorporating PSP Rating Scale and
survival, can reduce the potential for type II error.
Selecting efficacy measures more sensitive to disease
modification may facilitate identification of treatment
effect.The objective of this study was to evaluate the
simulated phase 3 PSP trial assessing the effect of
disease-modifying intervention on a novel combined primary
endpoint comprising function (PSP Rating Scale) and
survival, the Combined Assessment of Function and Survival
(CAFS), and to determine operating characteristics of the
CAFS.To simulate PSP progression in the trial population, we
developed models of PSP Rating Scale and survival using data
from published clinical studies. These models were used to
define operating characteristics of the CAFS for use in a
phase 3 trial.The sample size determined (N = 384; 1:1
randomization) would provide $>80\%$ power to detect
significant treatment effects on the CAFS compared with
placebo. The CAFS provides good operating characteristics
and increased power to detect moderate treatment effects on
the PSP Rating Scale. We propose a trial design allowing
potential detection of treatment effects at a preplanned
interim analysis after participants complete 12 months of
treatment, with assessment of effects of treatment (≤24
months) on survival.Use of the CAFS could provide a
comprehensive and robust estimate of the clinical benefit of
future therapies. © 2024 UCB. Movement Disorders published
by Wiley Periodicals LLC on behalf of International
Parkinson and Movement Disorder Society.},
keywords = {Supranuclear Palsy, Progressive: drug therapy /
Supranuclear Palsy, Progressive: physiopathology / Humans /
Disease Progression / Male / Female / Treatment Outcome /
Severity of Illness Index / Aged / clinical rating scale
(Other) / item response theory (Other) / joint rank test
(Other) / neurodegenerative disease (Other) / progressive
supranuclear palsy (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},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39470015},
pmc = {pmc:PMC11752988},
doi = {10.1002/mds.30027},
url = {https://pub.dzne.de/record/276156},
}