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Deep brain stimulation in children: experience and technical pearls
Ellen L Air1, Jill L Ostrem, Terence D Sanger
1Department of Neurosurgery, University of California, San Francisco, CA 94143-0221, USA.
Insights
Deep brain stimulation (DBS) offers promising results for pediatric dystonia, particularly DYT1, but shows limited efficacy for neurodegeneration with brain iron accumulation (NBIA). Younger children experienced a higher risk of hardware infections during DBS procedures.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Movement Disorders
Background:
- Deep brain stimulation (DBS) is a recognized treatment for adult movement disorders.
- Limited data exists on the technical aspects, complications, and outcomes of DBS in pediatric populations.
- Understanding DBS in children is crucial for expanding treatment options for pediatric neurological conditions.
Purpose of the Study:
- To evaluate the safety and efficacy of deep brain stimulation (DBS) in children with various movement disorders.
- To document surgical techniques, complications, and neurological outcomes in pediatric DBS patients.
- To compare DBS outcomes in children with specific diagnoses (e.g., DYT1 dystonia, NBIA) to adult data.
Main Methods:
- Retrospective review of a prospectively established database of DBS implantations since 1998.
- Inclusion criteria: patients younger than 18 years at the time of DBS surgery.
- Data collected: diagnoses, surgical techniques (microelectrode-guided vs. image-guided), complications, and neurological function assessed by standard rating scales.
Main Results:
- Thirty-one pediatric patients (ages 4-17) underwent DBS for diagnoses including DYT1 dystonia, secondary dystonia, and NBIA.
- Surgical techniques evolved; younger children (<10 years) had a higher incidence of hardware infections.
- One-year outcomes showed significant improvement in DYT1 dystonia (75% movement score improvement), modest results for secondary dystonia, and poor outcomes for NBIA.
Conclusions:
- DBS in children with primary and secondary dystonias yields results comparable to adults, with excellent outcomes for DYT1 dystonia.
- Pediatric DBS for NBIA showed disappointing results, contrasting with adult data.
- Younger age (<10 years) is associated with an increased risk of hardware infections in pediatric DBS patients.
Object:
Deep brain stimulation (DBS) is an established technique for the treatment of several movement disorders in adults. However, the technical approach, complications, and results of DBS in children have not been well documented.
Methods:
A database of DBS implantations performed at a single institution, prospectively established in 1998, was reviewed for patients who received DBS prior to the age of 18. Diagnoses, surgical technique, and complications were noted. Outcomes were assessed using standard rating scales of neurological function.
Results:
Of 815 patients undergoing DBS implantation over a 12-year period, 31 were children (mean age at surgery 13.2 years old, range 4-17 years old). Diagnoses included the following: DYT1 primary dystonia (autosomal dominant, Tor1AΔGAG mutation, 10 cases), non-DYT1 primary dystonia (3 cases), secondary dystonia (11 cases), neurodegeneration with brain iron accumulation (NBIA, 3 cases), levodopa-responsive parkinsonism (2 cases), Lesch-Nyhan disease (1 case), and glutaric aciduria Type 1 (1 case). Six children ages 15-17 years old underwent awake microelectrode-guided surgery. For 25 children operated under general anesthesia, the surgical technique evolved from microelectrode-guided surgery to image-guided surgeries using real-time intraoperative MR imaging or CT for lead location confirmation. Complications included 5 hardware infections, all in children younger than 10 years old. At 1 year after implantation, patients with DYT1 dystonia had a mean improvement in the Burke-Fahn-Marsden Dystonia Rating Scale movement subscore of 75%, while those with secondary dystonia had only small improvements. Outcomes in the 3 children with NBIA were disappointing.
Conclusions:
Results of DBS in children with primary and secondary dystonias were similar to those in adults, with excellent results for DYT1 dystonia in children without fixed orthopedic deformity and much more modest results in secondary dystonia. In contrast to reported experience in adults with NBIA, these results in children with NBIA were poor. Infection risk was highest in the youngest patients.
