title: Self-rated health status in spinocerebellar ataxia--results from a European multicenter study.
abstract: 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 (EQ-VAS) 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.
authors.affiliation: Department of Neurology, University Hospital of Bonn, Bonn, Germany. tanja.schmitz-huebsch@ukb.uni-bonn.de
authors.forename: Tanja
authors.initials: T
authors.lastname: Schmitz-Hübsch
authors.forename: Mathieu
authors.initials: M
authors.lastname: Coudert
authors.forename: Paola
authors.initials: P
authors.lastname: Giunti
authors.forename: Christoph
authors.initials: C
authors.lastname: Globas
authors.forename: Laszlo
authors.initials: L
authors.lastname: Baliko
authors.forename: Roberto
authors.initials: R
authors.lastname: Fancellu
authors.forename: Caterina
authors.initials: C
authors.lastname: Mariotti
authors.forename: Alessandro
authors.initials: A
authors.lastname: Filla
authors.forename: Maryla
authors.initials: M
authors.lastname: Rakowicz
authors.forename: Perrine
authors.initials: P
authors.lastname: Charles
authors.forename: Pascale
authors.initials: P
authors.lastname: Ribai
authors.forename: Sandra
authors.initials: S
authors.lastname: Szymanski
authors.forename: Jon
authors.initials: J
authors.lastname: Infante
authors.forename: Bart P C
authors.initials: BP
authors.lastname: van de Warrenburg
authors.forename: Alexandra
authors.initials: A
authors.lastname: Dürr
authors.forename: Dagmar
authors.initials: D
authors.lastname: Timmann
authors.forename: Sylvia
authors.initials: S
authors.lastname: Boesch
authors.forename: Rafal
authors.initials: R
authors.lastname: Rola
authors.forename: Chantal
authors.initials: C
authors.lastname: Depondt
authors.forename: Ludger
authors.initials: L
authors.lastname: Schöls
authors.forename: Elszbieta
authors.initials: E
authors.lastname: Zdzienicka
authors.forename: Jun-Suk
authors.initials: JS
authors.lastname: Kang
authors.forename: Susanne
authors.initials: S
authors.lastname: Ratzka
authors.forename: Berry
authors.initials: B
authors.lastname: Kremer
authors.forename: Jörg B
authors.initials: JB
authors.lastname: Schulz
authors.forename: Thomas
authors.initials: T
authors.lastname: Klopstock
authors.forename: Bela
authors.initials: B
authors.lastname: Melegh
authors.forename: Sophie Tezenas
authors.initials: ST
authors.lastname: du Montcel
authors.forename: Thomas
authors.initials: T
authors.lastname: Klockgether
mesh_terms: D000328:Adult; D000368:Aged; D001007:Anxiety / Q000175:diagnosis / Q000209:etiology; D004644:Emotions / Q000502:physiology*; D005060:Europe / Q000453:epidemiology; D005260:Female; D006304:Health Status; D006801:Humans; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D010147:Pain Measurement; D011788:Quality of Life; D012720:Severity of Illness Index; D020754:Spinocerebellar Ataxias / Q000145:classification / Q000150:complications / Q000175:diagnosis* / Q000523:psychology*; D018709:Statistics, Nonparametric; D011795:Surveys and Questionnaires