DBS-GPi shows significant enhancements in motor functionality and quality of life in children with myoclonus dystonia, indicating promising pediatric applications.
Deep brain stimulation of the globus pallidus internus (DBS-GPi) has shown efficacy in adult patients with SGCE-related myoclonus dystonia. However, evidence regarding its impact in pediatric populations is limited. The aim was to evaluate motor and non-motor outcomes following DBS-GPi intervention in children and adolescents with SGCE-MD. Ten patients (mean age 12.8 ± 3.4 years) underwent DBS-GPi. Blinded experts rated patients with the Unified Myoclonus, Burke-Fahn-Marsden, Writer's cramp and Gait Dystonia rating scales. Psychiatric and quality-of-life outcomes were evaluated using the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition criteria and Quality of Life in Neurological Disorders. Significant improvements were observed in myoclonus (68.1%), generalized dystonia (63.2%), and dystonia during writing (48.1%), walking (70.3%) and running (44.2%) after 3.8 ± 2.4 years of the surgery. Psychiatric symptoms remained stable, while quality-of-life assessments revealed reductions in anxiety, fatigue, and stigma (P < 0.05). DBS-GPi in children with SGCE-myoclonus dystonia mitigates long-term disability, potentially enhancing academic, social, and professional prospects.
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Salazar‐Villacorta et al. (2025) studied this question.
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