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Characterization of Lifestyle in Spinocerebellar Ataxia Type 3 and Association with Disease Severity.
Hengel, Holger; Martus, Peter; Faber, Jennifer; Garcia-Moreno, Hector; Solanky, Nita; Giunti, Paola; Klockgether, Thomas; Reetz, Kathrin; van de Warrenburg, Bart P; Pereira de Almeida, Luís; Santana, Magda M; Januário, Cristina; Silva, Patrick; Thieme, Andreas; Infante, Jon; de Vries, Jeroen; Lima, Manuela; Ferreira, Ana F; Bushara, Khalaf; Jacobi, Heike; Onyike, Chiadi; Schmahmann, Jeremy D; Hübener-Schmid, Jeannette; Synofzik, Matthis; Schöls, Ludger.
Affiliation
  • Hengel H; Department of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.
  • Martus P; German Center for Neurodegenerative Diseases (DZNE), Tübingen, Germany.
  • Faber J; Institute of Clinical Epidemiology and Applied Biostatistics, University of Tübingen, Tübingen, Germany.
  • Garcia-Moreno H; German Center for Neurodegenerative Diseases (DZNE), Bonn, Germany.
  • Solanky N; Department of Neurology, University Hospital Bonn, Bonn, Germany.
  • Giunti P; Ataxia Centre, Department of Clinical and Movement Neurosciences, Queen Square Institute of Neurology, University College London (UCL), London, United Kingdom.
  • Klockgether T; Department of Neurogenetics, National Hospital for Neurology and Neurosurgery, University College London Hospitals (UCLH) National Health Service Foundation Trust, London, United Kingdom.
  • Reetz K; Ataxia Centre, Department of Clinical and Movement Neurosciences, Queen Square Institute of Neurology, University College London (UCL), London, United Kingdom.
  • van de Warrenburg BP; Department of Neurogenetics, National Hospital for Neurology and Neurosurgery, University College London Hospitals (UCLH) National Health Service Foundation Trust, London, United Kingdom.
  • Pereira de Almeida L; Ataxia Centre, Department of Clinical and Movement Neurosciences, Queen Square Institute of Neurology, University College London (UCL), London, United Kingdom.
  • Santana MM; Department of Neurogenetics, National Hospital for Neurology and Neurosurgery, University College London Hospitals (UCLH) National Health Service Foundation Trust, London, United Kingdom.
  • Januário C; German Center for Neurodegenerative Diseases (DZNE), Bonn, Germany.
  • Silva P; Department of Neurology, University Hospital Bonn, Bonn, Germany.
  • Thieme A; Department of Neurology, Rheinisch-Westfälische Technische Hochschule (RWTH) Aachen University, Aachen, Germany.
  • Infante J; Jülich Aachen Research Alliance (JARA) Brain Institute: Molecular Neuroscience and Neuroimaging, Forschungszentrum Jülich, Jülich, Germany.
  • de Vries J; Department of Neurology, Radboud University Medical Centre, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, The Netherlands.
  • Lima M; Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal.
  • Ferreira AF; Center for Innovation in Biomedicine and Biotechnology, University of Coimbra, Coimbra, Portugal.
  • Bushara K; Faculty of Pharmacy, University of Coimbra, Coimbra, Portugal.
  • Jacobi H; Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal.
  • Onyike C; Center for Innovation in Biomedicine and Biotechnology, University of Coimbra, Coimbra, Portugal.
  • Schmahmann JD; Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal.
  • Hübener-Schmid J; Center for Innovation in Biomedicine and Biotechnology, University of Coimbra, Coimbra, Portugal.
  • Synofzik M; Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal.
  • Schöls L; Center for Innovation in Biomedicine and Biotechnology, University of Coimbra, Coimbra, Portugal.
Mov Disord ; 37(2): 405-410, 2022 02.
Article in En | MEDLINE | ID: mdl-34713931
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.
Subject(s)
Key words

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Machado-Joseph Disease / Spinocerebellar Ataxias Type of study: Observational_studies / Risk_factors_studies Limits: Humans Language: En Journal: Mov Disord Journal subject: NEUROLOGIA Year: 2022 Document type: Article Affiliation country: Alemania

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Machado-Joseph Disease / Spinocerebellar Ataxias Type of study: Observational_studies / Risk_factors_studies Limits: Humans Language: En Journal: Mov Disord Journal subject: NEUROLOGIA Year: 2022 Document type: Article Affiliation country: Alemania