Self-rated health status in spinocerebellar ataxia--results from a European multicenter study.
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.
Schmitz-Hübsch
Tanja
T
Department of Neurology, University Hospital of Bonn, Bonn, Germany. tanja.schmitz-huebsch@ukb.uni-bonn.de
Coudert
Mathieu
M
Giunti
Paola
P
Globas
Christoph
C
Baliko
Laszlo
L
Fancellu
Roberto
R
Mariotti
Caterina
C
Filla
Alessandro
A
Rakowicz
Maryla
M
Charles
Perrine
P
Ribai
Pascale
P
Szymanski
Sandra
S
Infante
Jon
J
van de Warrenburg
Bart P C
BP
Dürr
Alexandra
A
Timmann
Dagmar
D
Boesch
Sylvia
S
Rola
Rafal
R
Depondt
Chantal
C
Schöls
Ludger
L
Zdzienicka
Elszbieta
E
Kang
Jun-Suk
JS
Ratzka
Susanne
S
Kremer
Berry
B
Schulz
Jörg B
JB
Klopstock
Thomas
T
Melegh
Bela
B
du Montcel
Sophie Tezenas
ST
Klockgether
Thomas
T
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