The objective of this study was to assess quality of life (QoL) in a community-based sample of people with various forms of dystonia and to identify the factors that predict QoL in dystonia. QoL was assessed using two generic questionnaires: the Medical Outcomes Study Short-Form 36 (SF36) and the EuroQol questionnaire. A host of demographic, clinical, and psychosocial variables were measured to identify the best predictors of QoL in dystonia. A comparison of EuroQol and SF36 scores with the norms for the general UK population of similar age showed that people with dystonia had scores indicative of worse QoL on all domains, particularly those related to physical and social functioning. The impairment of QoL was seen in all age groups and was similar for men and women. Compared to the focal dystonia group, participants with generalized dystonia scored significantly worse on all QoL measures. Participants who were unemployed also scored significantly worse. There was also a trend for younger and separated/divorced participants to score worse on QoL measures. A stepwise regression analysis revealed that functional disability, body concept, and depression were important predictors of QoL in dystonia. Dystonia influences various aspects of QoL, particularly those related to physical and social functioning. The impairment of QoL was greater for participants with generalized dystonia, those who were unemployed, younger, and separated/divorced. Functional disability, body concept, and depression were the best predictors of QoL in dystonia. Efforts to improve health care for people with dystonia should not only focus on management of the movement disorder but also consider modifying functional disability and negative body concept and depression that contribute to poor QoL in this disorder.

In view of the steadily rising demand for treatment of dystonia with botulinum toxin (BT), a relatively expensive neurologic paralytic agent, an exploratory study was undertaken to assess the extent to which dystonia and BT treatment affect the quality of people's lives. One hundred thirty adults with a current diagnosis of dystonia completed two generic measures of health-related quality of life (HRQoL) at regular intervals over a minimum of 6 months. One hundred two participants were receiving regular injections of BT; 28 were not taking BT. The HRQoL instruments used were the EuroQol and the Short Form 36 health survey questionnaire (SF-36). Compared with general population samples, study participants reported greater impairment on all EuroQol and SF-36 dimensions and gave a lower rating to their own health status. Participants with nonfocal dystonia had significantly more problems with usual activities than participants with focal dystonia, and a higher number had problems with mobility and self-care. The groups reported similar levels of pain and emotional well-being. Small improvements in HRQoL were seen after the administration of BT, although few of these were statistically significant. The study results offer further psychometric evidence for the discriminant and construct validity of both the EuroQol and the SF-36.

Dystonia causes body disfigurement in the majority of those affected. Our aim was to test the hypothesis that low self-esteem resulting from the sense of disfigurement is an important component of self-reported depression in focal, segmental and generalized dystonia. Questionnaires to assess self-reported depression, self-esteem, body concept, disfigurement, disability and quality of life were completed by 329 community based dystonia patients. Moderate to severe depression was reported by 30 %. Self-reported depression had a strong somatic component, but patients also showed a specific concern with self-image. Extent of dystonia, body parts affected and marital status influenced self-reported depression in dystonia. Self esteem, body concept, disfigurement and quality of life emerged as factors which accounted for the variance of self-reported depression in dystonia. These results suggest that in dystonia, disfigurement, negative body concept, low self-esteem, and the impact of the disease on quality of life make important contributions to depression. However, longitudinal followup is required to firmly establish the direction of causality between depression and these psychosocial variables in dystonia.

The dystonias are a group of movement disorders arising from CNS dysfunction and characterised by involuntary and prolonged spasms of muscle contraction. Recently there has been increasing demand for treatment with botulinum toxin (BT), a relatively expensive neurological paralytic agent. As there has been no systematic assessment of patient benefit from BT, this study was undertaken to develop and test a methodology for assessing the cost utility of BT therapy for patients with dystonias. A generic health status instrument, the EuroQOL, was completed at regular intervals over at least 6 months by 130 patients with a current diagnosis of dystonia. A general population tariff was used to calculate quality-adjusted life-year (QALY) gains from BT treatment, and relevant cost data were obtained from patients and medical records. The cost-per-QALY estimates ranged considerably, depending on the type of dystonia, the duration of BT treatment, type of health-related quality-of-life (HR-QOL) tariff used and baseline characteristics of participants. The study findings reflect the general clinical impression of BT: that it can benefit patients with dystonia, but the benefit may be small compared with many treatments for other diseases. The nature of the disease and its cyclical treatment caused practical difficulties in recruiting participants, administering questionnaires and in estimating QALY gains.

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