Treatment Of Generalized Dystonia Research Articles

Outcome analysis of deep brain stimulation for pediatric generalized dystonia

Objective To explore the feasibility and efficacy of deep brain stimulation (DBS) in the treatment of generalized dystonia in children. Methods A retrospective study was designed to investigate the clinical data of 12 children with generalized dystonia. All of them were treated with DBS at Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences from August 2015 to July 2018. Genetic etiology testing showed that 5 cases were type DYT-1, 1 case was type DYT-6, and 6 cases were idiopathic generalized dystonia. All children underwent head susceptibility-weighted imaging (SWI) to locate the target before surgery. The clinical efficacy of the children was assessed using the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). In addition, 12 adult patients with generalized dystonia with matched gender, genotype were selected from our database. We compared and analyzed the difference in anatomy of the internal globus pallidus (GPi) between children and adults to evaluate the feasibility of operation. Results All patients were successfully implanted with electrodes without surgery-related complications. The average duration of follow-up was (19.8±9.6) months (12-47 months). The median BFMDRS scores at 3 months and 1 year after surgery were 15.5 (10-50) and 12.5 (4-48) respectively, which, compared with the preoperative score of 49.0(28-103), were significantly different (both P 8 years old was (75.5±4.7)mm (70.0-82.8 mm), which was not statistically different from that in the adults group (P=0.190). Conclusions Preliminary findings suggest that DBS could improve clinical symptoms without serious complications for the treatment of pediatric generalized dystonia with clear etiological causes. The anatomical location of the GPi in children >8 years old is not significantly different from that in adults. However, the long-term prognosis and incidence of complications such as electrode displacement still require more follow-up studies. Key words: Dystonia; Child; Deep brain stimulation; Generalized

Treatment of generalized dystonia by deep brain stimulation

Objective To evaluate the efficacy of deep brain stimulation in treatment of generalized dystonia. Methods A retrospective analysis was conducted on 20 patients who were implanted with a PINS device at Department of Neurosurgery, Peking Union Medical College Hospital from July 2013 to September 2016. Among them, 13 cases were primary generalized dystonia and 7 cases had secondary generalized dystonia. The scores of Burke-Fahn-Marsden dystonia movement/disability were documented at baseline and at 3, 6, 12 months of follow-up. The rate of improvement was assessed at 12 months postoperatively to evaluate the effectiveness of the PINS DBS system. Comparison was conducted regarding the improvement of scores between groups with different targets and parameters of stimulation. Results Compared with preoperative state, the improvement rates of median BFMDRS-M (Burke-Fahn-Marsden Dystonia Rating Scale-Movement) scores were 86.1% and 43.9% in patients with primary and secondary systemic dystonia at 12 months post surgery, respectively. The improvement rates of median BFMDRS-D (Burke-Fahn-Marsden Dystonia Rating Scale-Disability) scores were 66.7% and 44.4%, respectively, and there was significant difference between pre- and post-operative states (both P 0.05). However, the stimulation parameters of STN-DBS group were significantly lower than those of GPi-DBS group (all P<0.05) in terms of voltage, frequency and amplitude. The follow-up lasted 12-48 months. Only 2 cases developed dyskinesia as the adverse event which resolved or obtained improvement by the end of the study. No adverse effects were classified as severe. Conclusions The deep brain stimulation system domestically manufactured could significantly improve the symptoms of generalized dystonia and demonstrates safety and effectiveness. Key words: Deep drain stimulation; Generalized dystonia; Treatment outcome