Self-rated health status in spinocerebellar ataxia - Results from a European multicenter study

Tanja Schmitz-Hübsch, Mathieu Coudert, Paola Giunti, Christoph Globas, Laszlo Baliko, Roberto Fancellu, Caterina Mariotti, Alessandro Filla, Maryla Rakowicz, Perrine Charles, Pascale Ribai, Sandra Szymanski, Jon Infante, Bart P C Van De Warrenburg, Alexandra Dürr, Dagmar Timmann, Sylvia Boesch, Rafal Rola, Chantal Depondt, Ludger SchölsElszbieta Zdzienicka, Jun Suk Kang, Susanne Ratzka, Berry Kremer, Jörg B. Schulz, Thomas Klopstock, Bela Melegh, Sophie Tezenas Du Montcel, Thomas Klockgether

Research output: Contribution to journalArticle


Patient-based measures of subjective health status are increasingly used as outcome measures in interventional trials. We aimed to determine the variability and predictors of subjective health ratings in a possible target group for future interventions: the spinocerebellar ataxias (SCAs). A consecutive sample of 526 patients with otherwise unexplained progressive ataxia and genetic diagnoses of SCA1 (117), SCA2 (163), SCA3 (139), and SCA6 (107) were enrolled at 18 European referral centers. Subjective health status was assessed with a generic measure of health related quality of life, the EQ-5D (Euroqol) questionnaire. In addition, we performed a neurological examination and a screening questionnaire for affective disorders (patient health questionnaire). Patient-reported health status was compromised in patients of all genotypes (EQ-5D visual analogue scale (EQVAS) mean 61.45 ± 20.8). Specifically, problems were reported in the dimensions of mobility (86.9% of patients), usual activities (68%), pain/discomfort (49.4%), depression/ anxiety (46.4%), and self care (38.2%). Multivariate analysis revealed three independent predictors of subjective health status: ataxia severity, extent of noncerebellar involvement, and the presence of depressive syndrome. This model explained 30.5% of EQ-VAS variance in the whole sample and might be extrapolated to other SCA genotypes.

Original languageEnglish
Pages (from-to)587-595
Number of pages9
JournalMovement Disorders
Issue number5
Publication statusPublished - Apr 15 2010


  • Depression
  • EQ-5D
  • Quality of life
  • Spinocerebellar ataxia
  • Subjective health rating

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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    Schmitz-Hübsch, T., Coudert, M., Giunti, P., Globas, C., Baliko, L., Fancellu, R., Mariotti, C., Filla, A., Rakowicz, M., Charles, P., Ribai, P., Szymanski, S., Infante, J., Van De Warrenburg, B. P. C., Dürr, A., Timmann, D., Boesch, S., Rola, R., Depondt, C., ... Klockgether, T. (2010). Self-rated health status in spinocerebellar ataxia - Results from a European multicenter study. Movement Disorders, 25(5), 587-595.