Characterization of Lifestyle in Spinocerebellar Ataxia Type 3 and Association with Disease Severity

ABSTRACT Background Lifestyle could influence the course of hereditary ataxias, but representative data are missing. Objective The objective of this study was to characterize lifestyle in spinocerebellar ataxia type 3 (SCA3) and investigate possible associations with disease parameters. Methods In a...

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Published inMovement disorders Vol. 37; no. 2; pp. 405 - 410
Main Authors Hengel, Holger, Martus, Peter, Faber, Jennifer, Garcia‐Moreno, Hector, Solanky, Nita, Giunti, Paola, Klockgether, Thomas, Reetz, Kathrin, Warrenburg, Bart P., Pereira de Almeida, Luís, Santana, Magda M., Januário, Cristina, Silva, Patrick, Thieme, Andreas, Infante, Jon, Vries, Jeroen, Lima, Manuela, Ferreira, Ana F., Bushara, Khalaf, Jacobi, Heike, Onyike, Chiadi, Schmahmann, Jeremy D., Hübener‐Schmid, Jeannette, Synofzik, Matthis, Schöls, Ludger
Format Journal Article
LanguageEnglish
Published Hoboken, USA John Wiley & Sons, Inc 01.02.2022
Wiley Subscription Services, Inc
Subjects
Alcohol
Ataxia
Body mass index
Exercise
Humans
Life Style
lifestyle
Lifestyles
Machado-Joseph Disease
Movement disorders
Physical activity
Physical therapy
Prospective Studies
SCA3
Severity of Illness Index
Smoking
Spinocerebellar Ataxias - complications
Spinocerebellar Ataxias - epidemiology
alcohol
SCA3
body mass index
lifestyle
physical activity
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Summary:ABSTRACT Background Lifestyle could influence the course of hereditary ataxias, but representative data are missing. Objective The objective of this study was to characterize lifestyle in spinocerebellar ataxia type 3 (SCA3) and investigate possible associations with disease parameters. Methods In a prospective cohort study, data on smoking, alcohol consumption, physical activity, physiotherapy, and body mass index (BMI) were collected from 243 patients with SCA3 and 119 controls and tested for associations with age of onset, disease severity, and progression. Results Compared with controls, patients with SCA3 were less active and consumed less alcohol. Less physical activity and alcohol abstinence were associated with more severe disease, but not with progression rates or age of onset. Smoking, BMI, or physiotherapy did not correlate with disease parameters. Conclusion Differences in lifestyle factors of patients with SCA3 and controls as well as associations of lifestyle factors with disease severity are likely driven by the influence of symptoms on behavior. No association between lifestyle and disease progression was detected. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society
Bibliography:H.H., P.M., J.F., N.S., P.G., K.R., L.P.A., M.M.S., C.J., P.S., A.T., J.I., J.d.V., K.B., H.J., C.O., J.D.S., J.H.‐S., and M.S. have nothing to report. H G.‐M. reports the EU Joint Programme–Neurodegenerative Disease Research (JPND) project, supported through the following funding organization under the aegis of JPND: Medical Research Council. Grant from CureSCA3. T.K. reports JPND project, supported through the following funding organization under the aegis of JPND: Federal Ministry of Education and Research (funding codes 01ED1602A/B). B.v.W. reports research support from ZonMW (Grant 733051066). M.L. and A.F.F. report the JPND project, supported through the following funding organization under the aegis of JPND: FCT (JPCOFUND/0002/2015). L.S. reports the JPND project, supported through the following funding organization under the aegis of JPND: Federal Ministry of Education and Research (funding codes 01ED1602A/B).
This publication is an outcome of the European Spinocerebellar ataxia type 3/Machado‐Joseph disease initiative (ESMI), an EU Joint Programme–Neurodegenerative Disease Research (JPND) project (see
Relevant conflicts of interest/financial disclosures
www.jpnd.eu
Funding agencies
The project is supported through the following funding organizations under the aegis of JPND: Germany, Federal Ministry of Education and Research (funding codes 01ED1602A/B); The Netherlands, The Netherlands Organisation for Health Research and Development; Portugal, Foundation for Science and Technology (FCT); United Kingdom, Medical Research Council. This project has received funding from the European Union's Horizon 2020 research and innovation program under Grant 643417. At the US sites, this work was in part supported by the National Ataxia Foundation and the National Institute of Neurological Disorders and Stroke Grant R01 NS080816. P.G. is supported by the National Institute for Health Research University College London Hospitals (UCLH) Biomedical Research Centre. P.G. receives also support from the North Thames Clinical Research Network (CRN). P.G. and H.G.M. work at University College London Hospitals/University College London, which receives a proportion of funding from the Department of Health's National Institute for Health Research Biomedical Research Centres funding scheme. P.G. received funding from CureSCA3 in support of H.G.M.'s work. This work was moreover supported, in part, by the Deutsche Forschungsgemeinschaft (German Research Foundation) No. 441409627, as part of the Progression chart of Spastic ataxias (PROSPAX) consortium under the frame of the European Joint Programme on Rare Diseases (EJP RD), under the EJP RD COFUND‐EJP N° 825575 (to M.S., B.v.W,) and Grant 779257 “Solve‐RD” from the Horizon 2020 research and innovation program to M.S.
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ISSN:0885-3185
1531-8257
DOI:10.1002/mds.28844