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Deep Brain Stimulation for Refractory Status Dystonicus in Children: Multicenter Case Series and Systematic Review
Lindsey M Vogt1, Han Yan1,2, Brendan Santyr3,4
1Division of Neurology, Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Deep brain stimulation (DBS) effectively treats pediatric status dystonicus (SD), resolving symptoms in most patients within weeks. This intervention shows potential for improved survival compared to pharmacotherapy alone.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Medicine
Background:
- Status dystonicus (SD) is a severe, life-threatening condition in children.
- Current treatments for pediatric SD have limited efficacy and significant side effects.
- Deep brain stimulation (DBS) is an emerging therapeutic option for refractory SD.
Purpose of the Study:
- To evaluate the workflow, outcomes, and complications of DBS for pediatric SD.
- To systematically review existing literature and present a multicenter case series.
- To identify optimal DBS targets and stimulation parameters for pediatric SD.
Main Methods:
- Systematic review of 77 published pediatric SD cases treated with DBS.
- Multicenter case series of 8 pediatric SD patients treated with DBS.
- Voxelwise mapping of stimulated tissue in relation to dystonia improvement.
Main Results:
- DBS resolved SD in 8/8 institutional cases and 73/74 review cases, typically within 2-4 weeks.
- Globus pallidus pars interna (GPi) was the primary DBS target.
- Mean dystonia improvement was 32% (institutional) and 51% (review).
- Mortality was 4% in the review, lower than pharmacotherapy (10-12.5%).
Conclusions:
- DBS is a feasible and effective treatment for refractory pediatric SD.
- DBS offers potential for improved survival in pediatric SD patients.
- Increased awareness and timely implementation of DBS are crucial for pediatric SD management.
Objective:
We sought to better understand the workflow, outcomes, and complications of deep brain stimulation (DBS) for pediatric status dystonicus (SD). We present a systematic review, alongside a multicenter case series of pediatric patients with SD treated with DBS.
Methods:
We collected individual data regarding treatment, stimulation parameters, and dystonia severity for a multicenter case series (n = 8) and all previously published cases (n = 77). Data for case series were used to create probabilistic voxelwise maps of stimulated tissue associated with dystonia improvement.
Results:
In our institutional series, DBS was implanted a mean of 25 days after SD onset. Programming began a mean of 1.6 days after surgery. All 8 patients in our case series and 73 of 74 reported patients in the systematic review had resolution of their SD with DBS, most within 2 to 4 weeks of surgery. Mean follow-up for patients in the case series was 16 months. DBS target for all patients in the case series and 68 of 77 in our systematic review was the globus pallidus pars interna (GPi). In our case series, stimulation of the posterior-ventrolateral GPi was associated with improved dystonia. Mean dystonia improvement was 32% and 51% in our institutional series and systematic review, respectively. Mortality was 4% in the review, which is lower than reported for treatment with pharmacotherapy alone (10-12.5%).
Interpretation:
DBS is a feasible intervention with potential to reverse refractory pediatric SD and improve survival. More work is needed to increase awareness of DBS in this setting, so that it can be implemented in a timely manner. ANN NEUROL 2023.
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