Objective: Gillette Children’s Specialty Healthcare is a non-profit independent hospital whose aim is to provide care to medically complicated patients with a special focus on cerebral palsy. As we see so many patients with severe secondary dystonia, a deep brain stimulation (DBS) program was created to try and provide functional betterment and palliation for patients in whom historically more conventional treatments (enteral pharmacotherapy, injected therapies, baclofen pumps, rhizotomy, others) have failed to provide benefit. We wish to share our experience with the first 16 consecutive patients implanted with DBS placed in the globus pallidus interna (GPi) nuclei.

Background: DBS of the globus pallidus interna (GPi) nuclei can treat primary and secondary dystonia in addition to other movement disorders.

Methods: Patients with severe dystonia were evaluated in a clinic staffed by Neurology, Rehabilitation Medicine, and Neurosurgery physicians. If the patient had disabling dystonia, was found to have trialed at least 2 medical interventions, not deemed to have a progressive neurologic disorder, and have appropriate intracranial anatomy on imaging, deep brain stimulation (DBS) was offered as an intervention. Candidates were required to be evaluated pre- and post-implantation at 12 month intervals using standard assessments including the Burke-Fahn Marsden (BFM) scale, the Canadian Occupational Performance Measure (COPM), the Melbourne Assessment, and Hypertonicity Assessment Test (HAT). Our first 16 patients ranged in age from 5-35 years of age (Mean 20 years old) with varying primary diagnoses.

Results: Of our initial 16 patients, 11 had follow up data showing benefit on at least one of the surveillance measures used. 1 died of causes unrelated to deep brain stimulation. 3 patients had infection leading to hardware removal, and 1 patient seemed to have continued progression of generalized dystonia despite treatment. Baseline and follow up data will be presented highlighting patients individually in regard to their age and underlying diagnosis.

Conclusions: Deep Brain stimulation for secondary dystonia continues to be a challenging therapy in regard to patient selection and anticipatory counseling. Despite this, it seems to benefit the majority of patients treated at our institution in various ways.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/deep-brain-stimulation-outcomes-for-dystonia-in-adult-and-pediatric-patients-at-gillette-childrens-specialty-healthcare/