Outcome of pallidal deep brain stimulation for treating isolated orofacial dystonia

Abstract

Background Isolated orofacial dystonia is a rare segmental neurological disorder that affects the eye, mouth, face, and jaws. Current literature on pallidal surgery for orofacial dystonia is limited to case reports and small-scale studies. This study was to investigate clinical outcomes of deep brain stimulation (DBS) of the globus pallidus internus (GPi) in patients with isolated orofacial dystonia. Methods Thirty-six patients who underwent GPi DBS at Incheon St. Mary's Hospital, The Catholic University of Korea, between 2014 and 2019 were included in this study. Burke-Fahn-Marsden Dystonia Rating Scale, Unified Dystonia Rating Scale, and Global Dystonia Severity Rating Scale were retrospectively retrieved for analysis before surgery, at 6-month follow-up as short-term outcome, and at follow-up over 1 year (12 months to 69 months) as long-term results. Results Mean total BFMDRS-M scores at the three time points (baseline, 6 months, and over 1 year follow-up) were 11.6 +/- 4.9, 6.1 +/- 5.2 (50.3 +/- 29.9% improvement, p < 0.05), and 4.3 +/- 4.2 (65.0 +/- 24.2% improvement, p < 0.05), respectively. In terms of UDRS and GDS, improvement rates were 45.1% (p < 0.001) and 47.7% (p < 0.001) at 6 months, and 63.8% (p < 0.001) and 65.7% (p < 0.001) at over 1 year after surgery, respectively. Conclusions Bilateral GPi DBS in isolated orofacial dystonia can be effective if conservative treatment option fails. Its benefit is not only observed in a short term, but also maintained in a long-term follow-up.