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.

We investigated the effect of pallidal deep brain stimulation (GPi-DBS) in dystonia on cognition, mood, and quality of life and also assessed if DYT1 gene status influenced cognitive outcome following GPi-DBS. Fourteen patients with primary generalized dystonia (PGD) were assessed, measuring their estimated premorbid and current IQ, memory for words and faces, and working memory, language, executive function, and sustained attention, one month before and one year or more after surgery. Changes in mood and behaviour and quality of life were also assessed. There was a significant improvement of dystonia with GPi-DBS (69 % improvement in Burke-Fahn-Marsden score, p < 0.0001). Performance on five cognitive tests either improved or declined at post-surgical follow-up. Calculation of a reliable change index suggested that deterioration in sustained attention on the PASAT was the only reliable change (worse after surgery) in cognition with GPi-DBS. DYT1 gene status did not influence cognitive outcome following GPi-DBS. Depression, anxiety and apathy were not significantly altered, and ratings of health status on the EQ5D remained unchanged. In our sample, GPi-DBS was only associated with an isolated deficit on a test of sustained attention, confirming that GPi-DBS in PGD is clinically effective and safe, without adverse effects on the main domains of cognitive function. The dissociation between GPi-DBS improving dystonia, but not having a significant positive impact on the patients’ QoL, warrants further investigation.

Deep brain stimulation of the subthalamic nucleus is an effective treatment for the motor symptoms of Parkinson’s disease. Although a range of psychiatric and behavioural problems have been documented following deep brain stimulation, the short-term effects of subthalamic nucleus stimulation on patients’ mood have only been investigated in a few studies. Our aim was to compare self-reported mood in Parkinson’s patients with deep brain stimulation of the subthalamic nucleus ON versus OFF. Twenty-three Parkinson’s patients with bilateral deep brain stimulation of the subthalamic nucleus and 11 unoperated Parkinson’s patients completed a mood visual analogue scale twice. Operated patients were tested with deep brain stimulation of the subthalamic nucleus both ON and OFF. All were assessed on medication. The operated Parkinson’s group reported feeling significantly better coordinated, stronger and more contented with deep brain stimulation ON compared to OFF. Fourteen of the 16 mood scales changed in a positive direction when deep brain stimulation of the subthalamic nucleus was ON. When changes in motor scores were taken into account, the operated patients still reported feeling better-coordinated, but also less gregarious with stimulation ON. Unoperated Parkinson’s patients showed no differences on any of these measures between their two ratings. Short-term changes in deep brain stimulation of the subthalamic nucleus have a small and mostly positive effect on mood, which may be partly related to improvements in motor symptoms. The implications for day-to-day management of patients with deep brain stimulation of the subthalamic nucleus are discussed.

Problems with shifting attentional set and concurrent performance of tasks are key cognitive deficits in Parkinson's disease (PD). Our aim was to examine the effects of deep brain stimulation of the subthalamic nucleus on tests of set shifting and dual task performance in patients with PD. Twelve patients with PD were assessed on tests of set shifting and on dual task performance with subthalamic nucleus (STN) stimulation switched on and off in a counterbalanced order. All patients obtained a clinical benefit from deep brain stimulation (DBS) of the STN. STN stimulation significantly improved set shifting. The effect of DBS on dual task performance was not significant. Change in measures of set shifting was significantly associated with the change in the motor symptoms of PD with DBS. The improved set shifting with DBS of the STN in PD supports the critical role of the striato-frontal circuits in this cognitive function.