Abstract
Deep brain stimulation (DBS) for secondary (acquired, combined) dystonia does not reach the high degree of efficacy achieved in primary (genetic, isolated) dystonia. We hypothesize that this may be due to variability in the underlying injury, so that different children may require placement of electrodes in different regions of basal ganglia and thalamus. We describe a new targeting procedure in which temporary depth electrodes are placed at multiple possible targets in basal ganglia and thalamus, and probing for efficacy is performed using test stimulation and recording while children remain for one week in an inpatient Neuromodulation Monitoring Unit (NMU). Nine Children with severe secondary dystonia underwent the NMU targeting procedure. In all cases, 4 electrodes were implanted. We compared the results to 6 children who had previously had 4 electrodes implanted using standard intraoperative microelectrode targeting techniques. Results showed a significant benefit, with 80% of children with NMU targeting achieving greater than 5-point improvement on the Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS), compared with 50% of children using intraoperative targeting. NMU targeting improved BFMDRS by an average of 17.1 whereas intraoperative targeting improved by an average of 10.3. These preliminary results support the use of test stimulation and recording in a Neuromodulation Monitoring Unit (NMU) as a new technique with the potential to improve outcomes following DBS in children with secondary (acquired) dystonia. A larger sample size will be needed to confirm these results.
Highlights
Positive results of deep brain stimulation (DBS) in primary dystonia [1,2,3,4]suggest the potential of this procedure for benefit in children with cerebral palsy (CP) [5] and other causes of secondary dystonia [6]
While there is some commonality of injury in children with secondary dystonia, often due to hypoxic-ischemic injury to basal ganglia and lateral thalamus [11,12], variation in response to medication, functional neurosurgery, and therapy suggests significant heterogeneity of the processes leading to dystonia
6 children implanted with 4 electrodes using standard targeting procedures and intraoperative microelectrode recording (MER) are shown
Summary
Positive results of deep brain stimulation (DBS) in primary (genetic or isolated) dystonia [1,2,3,4]suggest the potential of this procedure for benefit in children with cerebral palsy (CP) [5] and other causes of secondary (acquired or combined) dystonia [6]. While there is some commonality of injury in children with secondary dystonia, often due to hypoxic-ischemic injury to basal ganglia and lateral thalamus [11,12], variation in response to medication, functional neurosurgery, and therapy suggests significant heterogeneity of the processes leading to dystonia. For this reason, we hypothesize that the optimal target for DBS is likely to vary between children, even when symptoms are similar
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
