Mapping efficacious deep brain stimulation for pediatric dystonia

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  • 1 Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto;
  • | 2 University Health Network, Toronto;
  • | 3 Joint Department of Medical Imaging, University of Toronto;
  • | 4 Edmond J. Safra Program in Parkinson’s Disease, Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital, University Health Network, Division of Neurology, University of Toronto;
  • | 5 Department of Neurosurgery, The Hospital for Sick Children, Toronto;
  • | 6 Faculty of Medicine, University of Ottawa;
  • | 7 Krembil Brain Institute, Toronto; and
  • | 8 Center for Advancing Neurotechnological Innovation to Application (CRANIA), Toronto, Ontario, Canada
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OBJECTIVE

The objective of this study was to report the authors’ experience with deep brain stimulation (DBS) of the internal globus pallidus (GPi) as a treatment for pediatric dystonia, and to elucidate substrates underlying clinical outcome using state-of-the-art neuroimaging techniques.

METHODS

A retrospective analysis was conducted in 11 pediatric patients (6 girls and 5 boys, mean age 12 ± 4 years) with medically refractory dystonia who underwent GPi-DBS implantation between June 2009 and September 2017. Using pre- and postoperative MRI, volumes of tissue activated were modeled and weighted by clinical outcome to identify brain regions associated with clinical outcome. Functional and structural networks associated with clinical benefits were also determined using large-scale normative data sets.

RESULTS

A total of 21 implanted leads were analyzed in 11 patients. The average follow-up duration was 19 ± 20 months (median 5 months). Using a 7-point clinical rating scale, 10 patients showed response to treatment, as defined by scores < 3. The mean improvement in the Burke-Fahn-Marsden Dystonia Rating Scale motor score was 40% ± 23%. The probabilistic map of efficacy showed that the voxel cluster most associated with clinical improvement was located at the posterior aspect of the GPi, comparatively posterior and superior to the coordinates of the classic GPi target. Strong functional and structural connectivity was evident between the probabilistic map and areas such as the precentral and postcentral gyri, parietooccipital cortex, and brainstem.

CONCLUSIONS

This study reported on a series of pediatric patients with dystonia in whom GPi-DBS resulted in variable clinical benefit and described a clinically favorable stimulation site for this cohort, as well as its structural and functional connectivity. This information could be valuable for improving surgical planning, simplifying programming, and further informing disease pathophysiology.

ABBREVIATIONS

BFMDRS = Burke-Fahn-Marsden Dystonia Rating Scale; DBS = deep brain stimulation; GPe = external globus pallidus; GPi = internal globus pallidus; MNI = Montreal Neurological Institute; rsfMRI = resting-state functional MRI; VTA = volume of tissue activated.
Figure from Coblentz et al. (pp 346–356).

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Contributor Notes

Correspondence Alfonso Fasano: Toronto Western Hospital, Toronto, ON, Canada. alfonso.fasano@uhn.ca.

INCLUDE WHEN CITING Published online January 1, 2021; DOI: 10.3171/2020.7.PEDS20322.

Disclosures Dr. Oliveira reports receiving funding for travel from Medtronic. Dr. Kalia reports being a consultant to Medtronic. Dr. Lozano reports being a consultant to Boston Scientific, Medtronic, Abbott, and Insightec; and being an employee of Functional Neuromodulation. Dr. Fasano reports being a consultant to, and receiving honoraria from, Abbott, Boston Scientific, and Medtronic.

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