Single-Electrode Deep Brain Stimulation of Bilateral Posterolateral Globus Pallidus Internus in Patients With Medically Resistant Lesch-Nyhan Syndrome.

Deep brain stimulation (DBS) targeting various locations within the globus pallidus internus (GPi) is emerging as a therapeutic option for patients with medically resistant Lesch-Nyhan syndrome. We report our institutional experience with single-electrode DBS in the bilateral posterolateral GPi as an effective method for reduction of both dystonia and self-injurious behavior. Two pediatric patients aged six and 14 years underwent implantation of bilateral singular DBS leads in the posterolateral GPi and were followed postoperatively through the programming process and symptomatic improvements. Caregivers reported that after DBS in the posterolateral GPi, these patients experienced decreased self-mutilation behavior and decreased dystonia.