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@ARTICLE{Thierfelder:163303,
author = {Thierfelder, Annika and Seemann, Jens and John, Natalie and
Harmuth, Florian and Giese, Martin and Schüle, Rebecca and
Schöls, Ludger and Timmann, Dagmar and Synofzik, Matthis
and Ilg, Winfried},
title = {{R}eal-{L}ife {T}urning {M}ovements {C}apture {S}ubtle
{L}ongitudinal and {P}reataxic {C}hanges in {C}erebellar
{A}taxia.},
journal = {Movement disorders},
volume = {37},
number = {5},
issn = {1531-8257},
address = {New York, NY},
publisher = {Wiley},
reportid = {DZNE-2022-00083},
pages = {1047-1058},
year = {2022},
note = {(CC BY-NC-ND 4.0)},
abstract = {Clinical and regulatory acceptance of upcoming molecular
treatments in degenerative ataxias might greatly benefit
from ecologically valid endpoints that capture change in
ataxia severity in patients' real life.This longitudinal
study aimed to unravel quantitative motor biomarkers in
degenerative ataxias in real-life turning movements that are
sensitive for changes both longitudinally and at the
preataxic stage.Combined cross-sectional (n = 30) and
longitudinal (n = 14, 1-year interval) observational study
in degenerative cerebellar disease (including eight
preataxic mutation carriers) compared to 23 healthy
controls. Turning movements were assessed by three body-worn
inertial sensors in three conditions: (1) instructed
laboratory assessment, (2) supervised free walking, and (3)
unsupervised real-life movements.Measures that quantified
dynamic balance during turning-lateral velocity change (LVC)
and outward acceleration-but not general turning measures
such as speed, allowed differentiating ataxic against
healthy subjects in real life (effect size δ = 0.68), with
LVC also differentiating preataxic against healthy subjects
(δ = 0.53). LVC was highly correlated with clinical ataxia
severity (scale for the assessment and rating of ataxia
[SARA] score, effect size ρ = 0.79) and patient reported
balance confidence (activity-specific balance confidence
scale [ABC] score, ρ = 0.66). Moreover, LVC in real
life-but not general turning measures or the SARA
score-allowed detecting significant longitudinal change in
1-year follow-up with high effect size (rprb =
0.66).Measures of turning allow capturing specific changes
of dynamic balance in degenerative ataxia in real life, with
high sensitivity to longitudinal differences in ataxia
severity and to the preataxic stage. They thus present
promising ecologically valid motor biomarkers, even in the
highly treatment-relevant early stages of degenerative
cerebellar disease. © 2022 The Authors. Movement Disorders
published by Wiley Periodicals LLC on behalf of
International Parkinson and Movement Disorder Society.},
keywords = {Ataxia / Biomarkers / Cerebellar Ataxia / Cross-Sectional
Studies / Humans / Longitudinal Studies / Spinocerebellar
Ataxias: genetics / cerebellar ataxia (Other) / motor
biomarker (Other) / real-life walking (Other) / turning
(Other) / wearable sensors (Other)},
cin = {AG Maetzler / AG Gasser 1},
ddc = {610},
cid = {I:(DE-2719)5000024 / I:(DE-2719)1210000},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:35067979},
doi = {10.1002/mds.28930},
url = {https://pub.dzne.de/record/163303},
}