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Deep brain stimulation in children with dystonia: experience from a tertiary care center
Partha S Ghosh1, Andre G Machado, Milind Deogaonkar
1Pediatric Neurology Center, Children's Hospital, Cleveland Clinic, Cleveland, Ohio 44195, USA.
Insights
Deep brain stimulation (DBS) of the globus pallidus internus (GPi) effectively improved movement and disability in children with primary dystonia. The therapy showed modest benefits for secondary dystonia, with manageable side effects.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Medicine
Background:
- Dystonia is a movement disorder characterized by involuntary muscle contractions.
- Globus pallidus internus deep brain stimulation (GPi DBS) is a therapeutic option for movement disorders.
Purpose of the Study:
- To evaluate the efficacy and safety of GPi DBS in pediatric patients diagnosed with dystonia.
Main Methods:
- Retrospective chart review of patients aged 21 years or younger who underwent GPi DBS.
- Assessment of outcomes using the Burke-Fahn-Marsden Dystonia Rating (BFMDR) movement and disability scales pre- and post-intervention.
Main Results:
- Significant improvements in BFMDR movement and disability scores were observed in six patients with primary dystonia.
- Modest improvements were noted in two patients with secondary dystonia.
- Two patients experienced hardware-related issues, and one patient had an infection.
Conclusions:
- GPi DBS demonstrates efficacy and safety in pediatric patients with primary dystonia.
- The therapy is also effective in selected cases of secondary dystonia in children.
Objective:
To investigate the efficacy and safety of deep brain stimulation (DBS) of the globus pallidus internus (GPi) in children with dystonia.
Methods:
Retrospective chart review of patients (≤21 years) with dystonia who underwent GPi DBS. Outcome measures were assessed by the Burke-Fahn-Marsden Dystonia Rating (BFMDR) movement and disability scales pre- and post-DBS.
Results:
Eight patients underwent DBS; mean age of onset was 7.5 ± 4.8 years (7 were male). Mean age at DBS was 14.1 ± 4.6 years. Etiology of dystonia was primary in 6 patients and secondary in 2. There was significant improvement of BFMDR movement as well as BFMDR disability scales in 6 patients with primary dystonia with modest improvement in those scales in 2 patients with secondary dystonia. Hardware-related problems were observed in 2 and infection was noted in 1.
Conclusions:
GPi DBS is an effective and safe therapy in pediatric patients with primary as well as selected cases of secondary dystonia.
