Article

The Cerebellum

, Volume 9, Issue 2, pp 232-239

First online:

Falls in Spinocerebellar Ataxias: Results of the EuroSCA Fall Study

  • Ella M. R. FonteynAffiliated withDepartment of Neurology (935), Radboud University Nijmegen Medical Centre
  • , Tanja Schmitz-HübschAffiliated withDepartment of Neurology, University Hospital of Bonn
  • , Carla C. VerstappenAffiliated withDepartment of Neurology, Canisius Wilhelmina Hospital
  • , Laslo BalikoAffiliated withDepartment of Neurology and Stroke, County Hospital
  • , Bastiaan R. BloemAffiliated withDepartment of Neurology (935), Radboud University Nijmegen Medical Centre
  • , Silvia BoeschAffiliated withDepartment of Neurology, University of Innsbruck
  • , Lisa BunnAffiliated withDepartment of Molecular Neuroscience, Institute of Neurology
  • , Perrine CharlesAffiliated withINSERM, UMR_S679 Neurologie and Thérapeutic Expérimentele, Ap-HP, Hôpital de la Pitié-SalpêtrièreDépartment of Genetics, Cytogenetics and Embryology, Salpetriere Hospital
  • , Alexandra DürrAffiliated withINSERM, UMR_S679 Neurologie and Thérapeutic Expérimentele, Ap-HP, Hôpital de la Pitié-SalpêtrièreDépartment of Genetics, Cytogenetics and Embryology, Salpetriere Hospital
    • , Allesandro FillaAffiliated withDepartment of Neurology, University of Naples
    • , Paola GiuntiAffiliated withDepartment of Molecular Neuroscience, Institute of Neurology
    • , Christoph GlobasAffiliated withDepartment of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen
    • , Thomas KlockgetherAffiliated withDepartment of Neurology, University Hospital of Bonn
    • , Bela MeleghAffiliated withDepartment of Medical Genetics and Child Development, University of Pécs
    • , Massimo PandolfoAffiliated withDepartment of Neurology, Hôpital Erasme, Université Libre de Bruxelles
    • , Anna De RosaAffiliated withDepartment of Neurology, University of Naples
    • , Ludger SchölsAffiliated withDepartment of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen
    • , Dagmar TimmannAffiliated withDepartment of Neurology, University of Duisburg-Essen
    • , Marten MunnekeAffiliated withDepartment of Neurology (935), Radboud University Nijmegen Medical Centre
    • , Berry P. H. KremerAffiliated withDepartment of Neurology, University Medical Centre Groningen
    • , Bart P. C. van de WarrenburgAffiliated withDepartment of Neurology (935), Radboud University Nijmegen Medical CentreDonders Institute for Brain, Cognition and Behaviour, Centre for Neuroscience Email author 

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Abstract

To investigate the frequency, details, and consequences of falls in patients with autosomal dominant spinocerebellar ataxias (SCAs) and to derive specific disease-related risk factors that are associated with an increased fall frequency. Two hundred twenty-eight patients with SCA1, SCA2, SCA3, or SCA6, recruited from the EuroSCA natural history study, completed a fall questionnaire that assessed the frequency, consequences, and several details of falls in the previous 12 months. Relevant disease characteristics were retrieved from the EuroSCA registry. The database of the natural history study provided the ataxia severity scores as well as the number and nature of non-ataxia symptoms. Patients (73.6%) reported at least one fall in the preceding 12 months. There was a high rate of fall-related injuries (74%). Factors that were associated with a higher fall frequency included: disease duration, severity of ataxia, the presence of pyramidal symptoms, the total number of non-ataxia symptoms, and the genotype SCA3. Factors associated with a lower fall frequency were: the presence of extrapyramidal symptoms (more specifically dystonia of the lower limbs) and the genotype SCA2. The total number of non-ataxia symptoms and longer disease duration were independently associated with a higher fall frequency in a logistic regression analysis, while the presence of extrapyramidal symptoms was independently associated with a lower fall frequency. Our findings indicate that, in addition to more obvious factors that are associated with frequent falls, such as disease duration and ataxia severity, non-ataxia manifestations in SCA play a major role in the fall etiology of these patients.

Keywords

Ataxia SCA Falls Pyramidal Injuries