Objective: The primary objectives were to characterize the referral of patients with dystonia receiving deep brain stimulation (DBS) therapy seeking specialized management for their DBS-related issues and the impressions of the overseeing neurologists following the initial evaluations. With up to 2 years of clinic follow-ups, we also investigated whether the patients’ initial concerns for the referral were addressed and what intervention strategies were implemented.

Background: DBS of the globus pallidus interna has become an established therapy for medically refractory dystonia, but programming it can be very challenging. This requires movement disorders physicians to be familiar with the treatment modality and the strategies to troubleshoot problems relating to suboptimal efficacy and therapy-related adverse effects.

Method: We conducted a retrospective chart review of patients with dystonia referred to the DBS Troubleshooting Clinic at the University of Florida from October 2019 to December 2022. Patient demographics, the clinical features of their dystonia, their DBS-related complaints, their DBS parameters and responses, and the assessments and plans of the DBS providers at the initial and follow-up visits were recorded. Descriptive statistics were calculated.

Results: Ten patients (3 male, 7 female) were identified. The mean (SD) age at the initial visit to the Troubleshooting Clinic was 38.8 (22.3) years. Four patients had documented UDRS with a mean (SD) of 29 (22.15), 1 had TWSTRS of 19, and 1 had BFMDRS of 13. Seven patients had positive genetic results: 3 TOR1A, 2 SGCE, 1 CTNNB1, 1 GCDH, and 1 PKND. Eight patients underwent bilateral implantations; 2 had unilateral DBS. Six patients had undergone DBS revision surgeries before visiting our clinic.

Seven patients had suboptimally placed leads, and 8 were receiving stimulation with suboptimal settings. Additionally, 2 had over-expectations toward the anticipated benefit of DBS. The battery of 1 patient was depleted. By 2 years, 40% of the patients had their complaints addressed, with a mean time to resolution of 10 months. Three of the 4 patients required revision surgeries, while 1 showed improvement following a bipolar stimulation configuration.

Conclusion: Troubleshooting DBS for dystonia benefits from a systematic approach which may include lead revision and/or programming optimization.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-characteristics-and-management-of-patients-with-dystonia-referred-for-the-troubleshooting-of-deep-brain-stimulation-therapy-a-single-center-experience/