Objective: Describe our 2-year experience establishing a pediatric deep brain stimulation (pDBS) service in a tertiary hospital and report our outcomes.

Background: DBS is a humanitarian device exemption for drug-resistant childhood onset dystonia with a high recognized evidence of efficacy.

Method: We set up a multidisciplinary team and established pDBS protocols for evaluation and treatment. Patients were older than 6 years old, weighted above 15kg, had a diagnosis of pharmacoresistant segmental or generalized dystonia, and were impaired for activities of daily living. A preoperative brain MRI stereotactic study with T1, T2 and DTI sequences was performed. Clinical status was recorded through scales (BFM-DRS, UMRS and NeuroQL). On the surgery day, we assembled a stereotaxic frame under general anesthesia and we took a head CT scan. We fused brain image (CT-MRI) and planned electrode trajectory using imaging software. In a one-staged approach, we implanted bidirectional electrodes in the globus pallidus internus (GPi). We used a multichannel microelectrode recording, intraoperative stimulation test and intermittent photic stimulation to choose the optimal trajectory and target. The extensions were placed under the skin behind the ear and connected to the implantable pulse generator (IPG) at the abdomen. Three days later, we performed a stimulation test to select the best electrode contact and started therapy.

Results: GPi-DBS was performed on 17 patients at a mean age of 14(8-20)years. Dystonia was isolated or combined with myoclonus and/or neuropsychiatric features. Etiology was genetic (n=12, TOR1A, GLB1, SGCE, GNAO1, ATP8A2, GCDH), idiopathic (n=3, negative exome) or acquired (n=2, cerebral palsy). We chose the central planning trajectory in 50% of cases showing good accuracy (X=0,26±0,6, Y=0,65±0,78, Z=0,56±0,75). After 13(4-24) months of follow-up, we observed a global improvement in motor (60%) and disability (40%) according to rating scales. Better results (42-94% improvement) were seen in isolated dystonia (SGCE, TOR1A, GNAO1) and idiopathic forms. Three patients had mild complications (atrophic scar, skin wound infection, broken extension).

Conclusion: Pediatric DBS is a patient-centered therapy and its choice must rely on a well-established and expert multidisciplinary team. In our sample GPi-DBS was effective and safe. Predictive factors for a good response were clinical features, etiology, disease duration and lead placement accuracy.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/setting-up-a-pediatric-deep-brain-stimulation-unit/