Bilateral deep brain stimulation (DBS) of globus pallidus internus (GPi) for the treatment of benign hereditary chorea and other childhood onset choreas: a single-center experience.

Abstract

Purpose: Chorea is a clinical sign characterized by involuntary, rapid, unpredictable, and irregular muscle movements that can affect various parts of the body. It can be seen in various medical conditions, both neurological and systemic, of genetic and acquired etiology. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has been used to treat various types of chorea. The aim of this study was to evaluate the efficacy of GPi DBS for chorea in pediatric patients.

Methods: The authors undertook a single-center retrospective study of all pediatric patients who underwent DBS in the period from July 2017 to April 2024 to identify those presenting with chorea.

Results: Three patients with chorea underwent bilateral posteroventral GPi DBS without surgical complications. The mean age at operation was 14.2 years (range: 1.5 years), and the mean follow-up was 49 months (range: 15 months). Two of the 3 patients experienced a positive effect on chorea with an improvement in functional status. In one patient, the pre- and postop Gross Motor Function Classification System (GMFCS) score was 4, while his Burke-Fahn-Marsden Dystonia Scale (BFMDS) score improved from 102/20 pre- to 53.5/20 postop. In the other patient the GMFCS score improved from 4 preop to 3 postop. His preop BFMDS score was not available, postop it was 83/120. In the patient who did not experience a positive effect on chorea the pre- and postop GMFCS score was 4, her BFMDS score was 84.5/120 pre- and 100/120 postop.

Conclusion: Bilateral GPi DBS can be safely administered to pediatric patients with choreiform movement disorders, and it could be an effective treatment option for managing chorea in certain patients.