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Published on: September 12, 2020
Tolerance of early pallidal stimulation in pediatric generalized dystonia
1Department of Stereotactic and Functional Neurosurgery, Kaizuka Hospital;
Insights
Deep brain stimulation (DBS) initially improved pediatric generalized dystonia in two patients with DYT1 mutations. However, symptom recurrence within a year suggests limitations in long-term efficacy, possibly due to brain maturation.
Area of Science:
- Neurology
- Neurosurgery
- Genetics
Background:
- Generalized dystonia is a movement disorder often associated with genetic mutations, such as the DYT1 mutation.
- Deep brain stimulation (DBS) targeting the globus pallidus internus (GPi) is a therapeutic option for severe dystonia.
Observation:
- Two pediatric patients with DYT1-positive generalized dystonia received chronic pallidal DBS, showing initial significant improvement.
- Symptomatic recurrence occurred within one year in both patients despite stimulation parameter adjustments.
- Subsequent surgical interventions, including lead revision and additional bilateral subthalamic nucleus DBS, did not restore therapeutic benefit.
Findings:
- Pallidal DBS demonstrates initial efficacy in pediatric generalized dystonia.
- Long-term therapeutic effect of pallidal DBS may be limited in pediatric patients.
- The developing pediatric brain's response to chronic electrical stimulation warrants further investigation.
Implications:
- Early pallidal DBS can be effective for pediatric generalized dystonia, but sustained benefit is not guaranteed.
- The plasticity of the preadolescent brain may influence the long-term outcomes of deep brain stimulation.
- Further research is needed to optimize DBS strategies for pediatric movement disorders.
Abstract:
The authors report on 2 cases of pediatric generalized dystonia with a DYT1 mutation; the patients, an 11-year-old girl and a 9-year-old boy, underwent chronic, pallidal deep brain stimulation (DBS) of the globus pallidus internus (GPi). The dystonic postures in both cases showed dramatic improvements with pallidal DBS, but each patient's symptoms gradually recurred within a year, irrespective of exhaustive readjustments of the stimulation settings. After the recurrence of the dystonic symptoms, the DBS leads were replaced within the GPi in one patient (Case 1) and additional DBS leads were implanted into the bilateral subthalamic nuclei in the other patient (Case 2). Neither measure produced any further clinical benefit, and the patient in Case 2 died of status dystonicus 2 days after reoperation. These findings suggest that early pallidal DBS for pediatric dystonia is indeed effective, although there are some cases in which its therapeutic effect is lost. One possible reason may be the ability of the preadolescent brain to tolerate chronic electrical stimuli during the active maturation process.
