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@ARTICLE{Hengel:165522,
author = {Hengel, Holger and Martus, Peter and Faber, Jennifer and
Giunit, Paola and Garcia-Moreno, Hector and Solanky, Nita
and Klockgether, Thomas and Reetz, Kathrin and van de
Warrenburg, Bart P and Santana, Magda M and Silva, Patrick
and Cunha, Maria Ines and de Almeida, Luís Pereira and
Timmann, Dagmar and Infante, Jon and de Vries, Jeroen and
Lima, Manuela and Pires, Paula and Bushara, Khalaf and
Jacobi, Heike and Onyike, Chiadi and Schmahmann, Jeremy D
and Hübener-Schmid, Jeannette and Synofzik, Matthis and
Schöls, Ludger},
collaboration = {Initiative, European Spinocerebellar Ataxia
Type-3/Machado-Joseph Disease},
title = {{T}he frequency of non-motor symptoms in {SCA}3 and their
association with disease severity and lifestyle factors.},
journal = {Journal of neurology},
volume = {270},
number = {2},
issn = {0340-5354},
address = {Berlin},
publisher = {Springer},
reportid = {DZNE-2022-01668},
pages = {944-952},
year = {2023},
note = {ISSN 1432-1459 not unique: **2 hits**.},
abstract = {Non-motor symptoms (NMS) are a substantial burden for
patients with SCA3. There are limited data on their
frequency, and their relation with disease severity and
activities of daily living is not clear. In addition,
lifestyle may either influence or be affected by the
occurrence of NMS.To characterize NMS in SCA3 and
investigate possible associations with disease severity and
lifestyle factors.In a prospective cohort study, we
performed a cross-sectional analysis of NMS in 227 SCA3
patients, 42 pre-ataxic mutation carriers, and 112 controls
and tested for associations with SARA score, activities of
daily living, and the lifestyle factors alcohol consumption,
smoking and physical activity.Sleep disturbance, restless
legs syndrome, mild cognitive impairment, depression,
bladder dysfunction and pallhypesthesia were frequent among
SCA3 patients, while mainly absent in pre-ataxic mutation
carriers. Except for restless legs syndrome, NMS correlated
significantly with disease severity and activities of daily
living. Alcohol abstinence was associated with bladder
dysfunction. Patients with higher physical activity showed
less cognitive impairment and fewer depressive symptoms, but
these differences were not significant.This study revealed a
clear association between disease severity and NMS, likely
driven by the progression of the widespread
neurodegenerative process. Associations between lifestyle
and NMS can probably be attributed to the influence of NMS
on lifestyle.},
keywords = {Humans / Restless Legs Syndrome: epidemiology / Prospective
Studies / Cross-Sectional Studies / Activities of Daily
Living / Patient Acuity / Life Style / Lifestyle (Other) /
Non-motor symptoms (Other) / Physical activity (Other) /
SCA3 (Other)},
cin = {AG Schöls / AG Gasser 1 / Patient studies, Bonn / AG
Klockgether},
ddc = {610},
cid = {I:(DE-2719)5000005 / I:(DE-2719)1210000 /
I:(DE-2719)1011101 / I:(DE-2719)1011001},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pmc = {pmc:PMC9886646},
pubmed = {pmid:36324033},
doi = {10.1007/s00415-022-11441-z},
url = {https://pub.dzne.de/record/165522},
}