Outcome analysis of deep brain stimulation for pediatric generalized dystonia

Abstract

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