Objective: To evaluate the efficacy and complications of deep brain stimulation (DBS) of globus pallidus interna (GPi) in a mixed cohort of dystonia patients.

Background: Controlled trials have demonstrated remarkable improvement in medically refractory primary generalized dystonia following pallidal stimulation. We carried out this study to evaluate DBS outcomes in a mixed cohort of dystonia patients; a ‘real world’ practice setting in an academic medical center.

Methods: A retrospective chart review of all patients with medically refractory dystonia who underwent GPi DBS from 2007 to 2014 was done. Efficacy was determined based on pre-post DBS comparison of Burke-Fahn-Marsden Dystonia Rating Scales (BFMDRS). Statistical analysis was performed using JMP Pro version 11.

Results: A total of 17 patients, mean age 49 ± 18.6 years; 10 males were included in the study. 11 had primary generalized dystonia (4 out of these were DYT1 gene positive), 4 had cervical dystonia, and 2 had craniocervical dystonia/Meige Syndrome. Duration of disease at the time of surgery was 17.8 years (SD ± 13.4). Change in BMFDRS-Motor score from baseline to follow up after 6.4 SD ± 0.77 months was -21.5 (CI: 14.5-28.5) p value <.0001, while change in BFMDRS-Disability score was -6.6 (CI 4.5-8.7) p value <.0001 indicating a mean reduction of scores by 59.1% and 45.8% respectively. GPi was the target in all patients while one patient had a lead in VIM nucleus of the thalamus for dystonic tremor. Stimulation settings on last recorded visit were; median frequency of 135 Hz (range 110 to 185), amplitude of 3.8 V (range 2.7 to 4.5) and pulse width of 150 µsec (range 60 to 210) for right Gpi, while left Gpi parameters for frequency were 135 Hz (range 105 to 210), voltage of 3.6 (range 2.2 to 4.7) and pulse width of 150 µsec (range 60 to 210). Mean Clinical Global Impression scale (CGI-improvement scale 1-7) at follow up was 2 which represented ‘much improvement’ following the surgery. Two patients had hardware related infection and required hardware removal and reimplantation, two additional patients had intraoperative seizure but did not develop further seizures.

Conclusions: In this mixed cohort of dystonia patients in an academic medical center, DBS resulted in an overall reduction in motor and disability scores by 52.5% which is similar to previously reported large scale multicenter studies.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/dbs-outcomes-in-a-mixed-cohort-of-dystonia-a-single-center-experience/