Long-Term Outcome of Idiopathic and Acquired Dystonia After Pallidal Deep Brain Stimulation: A Case Series

BACKGROUND Among dystonia patients receiving globus pallidus internus (GPi) deep brain stimulation (DBS), long-term outcomes remain to be established. OBJECTIVE To report the long-term outcome of GPi DBS in a patient cohort with idiopathic and acquired dystonia. METHODS In this long-term follow-up cohort, there were 4 patients with idiopathic dystonia and 2 patients with acquired dystonia. The Burk-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was used to evaluate 6 consecutive patients preoperatively and at 6 months, 12 months, and the last follow-up. The relationship between etiology and clinical improvement was analyzed. Stimulation parameters were evaluated for similarities and differences among these patients. RESULTS The mean follow-up of our cohort was 65.3 months (median 40.5 months). The average improvement in BFMDRS (mean ± SEM) was 56% ± 7.6, 67% ± 6.8 and 66% ± 9.7 at 6 months, 12 months, and last follow-up, respectively. There was greater improvement during long-term follow-up in the 4 patients with idiopathic dystonia than in the 2 patients with acquired dystonia. The 2 most ventral electrodes (contact 0 and 1) were activated in all 11 leads in this cohort. The average stimulation intensity, pulse width and frequency were 2.0 ± 0.24 mA, 252 ± 43 µs, and 99 ± 6.0 Hz, respectively. CONCLUSION Isolated dystonia, either monogenetic or idiopathic, usually responds better to GPi DBS than does acquired dystonia. Selection of patients by dystonia etiology, accurate placement of DBS leads in GPi targets, and proper stimulation programming are crucial to achieve better long-term outcomes.

Improvement of Non-motor Symptoms and Quality of Life After Deep Brain Stimulation for Refractory Dystonia: A 1-Year Follow-Up

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.

Psychological Traits and Behavioural Influences in Patients with Dystonia—An Observational Cohort Study in a Romanian Neurology Department

(1) Background: Emerging evidence indicates that non-motor symptoms significantly influence the quality of life in dystonic patients. Therefore, it is essential to evaluate their psychological characteristics and personality traits. (2) Methods: Subjects with idiopathic dystonia and a matched control group were enrolled in this prospective observational cohort study. Inclusion criteria for patient group included idiopathic dystonia diagnosis, evolution exceeding 1 year, and signed informed consent. Inclusion criteria for the control group included lack of neurological comorbidities and signed informed consent. All subjects completed the DECAS Personality Inventory along with an additional form of demographic factors. Data (including descriptive statistics and univariate and multivariate analysis) were analyzed with SPSS. (3) Results: In total, 95 participants were included, of which 57 were in the patient group. Females prevailed (80%), and the mean age was 54.64 ± 12.8 years. The most frequent clinical features of dystonia were focal distribution (71.9%) and progressive disease course (94.73%). The patients underwent regular treatment with botulinum toxin (85.95%). In addition, patients with dystonia obtained significantly higher openness scores than controls, even after adjusting for possible confounders (p = 0.006). Personality traits were also different between the two groups, with patients more often being fantasists (p = 0.007), experimenters (p = 0.022), sophists (p = 0.040), seldom acceptors (p = 0.022), and pragmatics (p = 0.022) than control subjects. (4) Conclusion: Dystonic patients tend to have different personality profiles compared to control subjects, which should be taken into consideration by the treating neurologist.