Abstract
Introduction: Deep brain stimulation (DBS) is a treatment option for refractory dystonia's motor symptoms, while its non-motor symptoms (NMS) have been less systematically assessed. We aimed to describe the effects of DBS on NMS in refractory generalized inherited/idiopathic dystonia prospectively.Methods: We evaluated patients before and 1 year after DBS surgery and applied the following scales: Burke–Fahn–Marsden Rating Scale (BFMRS), NMS Scale for Parkinson's Disease (NMSS-PD), Parkinson's Disease Questionnaire-8, short-form Brief Pain Inventory (BPI), Neuropathic Pain Symptom Inventory (NPSI), and short-form McGill Pain Questionnaire (MPQ).Results: Eleven patients (38.35 ± 11.30 years) underwent surgery, all with generalized dystonia. Motor BFMRS subscore was 64.36 ± 22.94 at baseline and 33.55 ± 17.44 1 year after DBS surgery (47.9% improvement, p = 0.003). NMSS-PD had a significant change 12 months after DBS, from 70.91 ± 59.07 to 37.18 ± 55.05 (47.5% improvement, p = 0.013). NMS changes were mainly driven by changes in the gastrointestinal (p = 0.041) and miscellaneous domains (p = 0.012). Seven patients reported chronic pain before DBS and four after it. BPI's severity and interference scores were 4.61 ± 2.84 and 4.12 ± 2.67, respectively, before surgery, and 2.79 ± 2.31 (0.00–6.25) and 1.12 ± 1.32 (0.00–3.00) after, reflecting a significant improvement (p = 0.043 and p = 0.028, respectively). NPSI score was 15.29 ± 13.94 before, while it was reduced to 2.29 ± 2.98 afterward (p = 0.028). MPQ's total score was 9.00 ± 3.32 before DBS, achieving 2.71 ± 2.93 after (p = 0.028).Conclusions: DBS improves NMS in generalized inherited/idiopathic dystonia, including chronic pain.
Highlights
Deep brain stimulation (DBS) is a treatment option for refractory dystonia’s motor symptoms, while its non-motor symptoms (NMS) have been less systematically assessed
Motor Burke–Fahn–Marsden Rating Scale (BFMRS) subscore was 64.36 ± 22.94 (31.00–102.00) at baseline and 33.55 ± 17.44 (9.00–58.50) 1 year after DBS surgery (p = 0.003, Table 1), which corresponds to a 47.9% improvement in motor symptoms related to dystonia (Figure 1)
The only differential scores that were significantly different from baseline and that had significant correlations were the Parkinson’s Disease Questionnaire-8 (PDQ8) and the Non-Motor Symptoms Scale for Parkinson’s Disease (NMSS-PD) scales (ρ = 0.740, p = 0.009, Supplementary Figure 1). These results suggest that after 12 months, quality of life (QoL) and NMS scores rise significantly and correlate, suggesting that DBS’s application improves NMS, which correlates with better QoL outcomes
Summary
Deep brain stimulation (DBS) is a treatment option for refractory dystonia’s motor symptoms, while its non-motor symptoms (NMS) have been less systematically assessed. We aimed to describe the effects of DBS on NMS in refractory generalized inherited/idiopathic dystonia prospectively. Generalized inherited/idiopathic dystonia is often refractory to pharmacological treatments. Deep brain stimulation (DBS) targeting the globus pallidus internus (GPi) or the subthalamic nucleus (STN) is already an established treatment, and beneficial motor outcomes have been extensively described [3,4,5,6]. Patients with generalized inherited/idiopathic dystonia usually have the best response to DBS treatment when compared with most other dystonia types, such as acquired forms of the disease [5]. Our main objective was to describe NMS outcomes after DBS surgery for refractory generalized inherited/idiopathic dystonia in a prospective exploratory study with a focus on chronic pain
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