Objective: To evaluate clinical outcomes of deep brain stimulation (DBS) of globus pallidus internus (GPi) or subthalamic nucleus (STN) for isolated cranial or cranio-cervical dystonia.

Background: Isolated cranial or cranio-cervical dystonia (Meige syndrome) is characterized by combination of blepharospasm and dystonia of lower face, jaw and sometimes neck muscles. DBS of GPi for Meige syndrome has previously only been described in case-reports or small case-series. Here we report the largest series of patients with isolated cranial or cranio-cervical dystonia treated with either bilateral GPi or STN DBS.

Methods: We retrospectively assessed outcomes of all patients with isolated cranial or cranio-cervical dystonia who were treated with bilateral DBS at our center between 2002-2015. A total of 24 patients (13 with STN DBS, 11 with GPi DBS) were identified. Dystonia severity was assessed by comparing the total and individual sub-item scores of the Burke-Fahn-Mardsen Dystonia Rating Scale (BFMDRS) preoperatively and at short-term (3-6 month) and long-term (12-36 month) follow up. Disability scales, medications, programming settings, active contact location and surgical adverse events were also evaluated.

Results: Mean duration of symptoms prior to DBS surgery was 10±8.5 years and mean age was 57±11.5 years. The BFMDRS total score improved from mean 22.9±2.2 pre-operatively to 7.5±1.2 or by 62% at short-term follow up (p<0.001) and to 7.6±1.5 or by 60% at long-term follow up (p<0.001). The BFMDRS sub-scores for both eyes and mouth improved from mean of 9.1±0.8 pre-operatively to 2.9±0.6 or by 67% at short-term follow (p<0.001) and to 2.7±0.5 or by 60% at long-term follow up (p<0.001). The BFMDRS disability score improved from 5.8±0.7 pre-operatively to 2.7±0.4 or by 40% at short-term follow up (p<0.001) and to 3.1±0.5 or by 37% at long-term follow up (p<0.002). There was no significant difference between GPi and STN groups for BFMDRS motor scores at short or long-term follow up (p=0.96 and p=0.85, respectively) or disability scores at short or long-term follow (p=0.39 and p=0.18, respectively).

Conclusions: Both GPi and STN DBS appear to be effective for treatment of isolated cranial and cranio-cervical dystonia, with sustained benefit over the long term. No difference in outcomes between GPi and STN DBS was seen but the study was limited by low sample size to detect small differences.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/clinical-outcomes-of-pallidal-and-subthalamic-nucleus-deep-brain-stimulation-in-isolated-cranial-and-cranio-cervical-dystonia/