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@ARTICLE{Hermle:282538,
author = {Hermle, Dominik and Schubert, Robin and Barallon, Pascal
and Ilg, Winfried and Schüle-Freyer, Rebecca and Reilmann,
Ralf and Synofzik, Matthis and Traschütz, Andreas},
title = {{D}igital {O}utcomes of {U}pper {L}imb {A}taxia {C}apture
{M}eaningful {L}ongitudinal {C}hange and {T}reatment
{R}esponse},
journal = {Movement disorders},
volume = {40},
number = {11},
issn = {0885-3185},
address = {New York, NY},
publisher = {Wiley},
reportid = {DZNE-2025-01301},
pages = {2486 - 2496},
year = {2025},
abstract = {Digital-motor outcomes promise better responsiveness than
clinician-reported outcomes in ataxia trials. However, their
patient meaningfulness and sensitivity to change remain to
be demonstrated, particularly in the upper limb
domain.Validation of quantitative motor (Q-Motor) assessment
for upper limb ataxia against patient-reported outcomes and
regarding sensitivity to both longitudinal and
treatment-induced change, the latter in n-of-1 treatment
settings.Single-center longitudinal assessment of finger
tapping, diadochokinesia, grip-lift, spiral drawing, and
target reaching in (1) 36 cross-genotype ataxia patients and
20 controls, validating digital measures for correlations
with patient-reported outcome measure (PROM)-ataxia, 2-weeks
test-retest reliability, and sensitivity to change within a
trial-relevant 1-year follow-up, anchored in Patient Global
Impression of Change (PGI-C); and (2) two patients with
spinocerebellar ataxia type 27B (SCA27B) on versus off
treatment with 4-aminopyridine.Twenty-four digital measures
correlated with the PROM-ataxia upper-limb composite (|ρ| =
0.4-0.7) and had excellent test-retest reliability (ICC =
0.91-0.99). Correlations to individual PROM-ataxia items
were specific for functional impairment the respective
measure was hypothesized to capture. Speed of finger tapping
and diadochokinesia, and smoothness of target reaching
(spectral arc length of movement in three dimensions
[SPARC3D]) captured 1-year progression in ataxia patients
(|rprb| = 0.38-0.51), and specifically in patients with
worsening PGI-C. Estimated sample sizes to detect
longitudinal change were lower for digital than clinical
outcomes (SPARC3D: n = 33, Scale for the Assessment and
Rating of Ataxia (SARA): n = 79, nine-hole peg-test: n =
214). Speed of diadochokinesia, stability of grip-lift, and
variability of target reaching captured treatment responses
to 4-aminopyridine in SCA27B, exceeding minimal detectable
and minimal important change.Digital upper limb measures
capture patient-meaningful 1-year longitudinal and
treatment-induced change, and are therefore promising
outcomes for upcoming ataxia trials. © 2025 The Author(s).
Movement Disorders published by Wiley Periodicals LLC on
behalf of International Parkinson and Movement Disorder
Society.},
keywords = {Humans / Male / Female / Middle Aged / Upper Extremity:
physiopathology / Longitudinal Studies / Adult / Aged /
Patient Reported Outcome Measures / Ataxia: physiopathology
/ Ataxia: drug therapy / Reproducibility of Results /
Treatment Outcome / Spinocerebellar Ataxias: physiopathology
/ Spinocerebellar Ataxias: drug therapy / Fingers:
physiopathology / ataxia; clinical outcome assessment;
digital motor outcome; patient meaningfulness; sensitivity
to change (Other)},
cin = {AG Gasser},
ddc = {610},
cid = {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:40888004},
pmc = {pmc:PMC12661631},
doi = {10.1002/mds.70012},
url = {https://pub.dzne.de/record/282538},
}