Pallidal Deep Brain Stimulation for Primary Dystonia in Children

Haridas, Abilash; Tagliati, Michele; Osborn, Irene P.; Isaias, Ioannis U.; Gologorsky, Yakov; Bressman, Susan; Weisz, Donald J.; Alterman, Ron L.

doi:10.1227/neu.0b013e3182077396

Cited by 63 publications

(56 citation statements)

References 22 publications

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“…Marks et al reported a 17% infection rate for children with dystonic CP, 23 and Haridas et al reported a 14% infection rate for children with primary dystonia. 12 Children with dystonic CP, who are severely disabled and often wheelchair bound, may have a high risk of perioperative infectious complications. The high infection rates may simply be the result of small sample sizes, but it is imperative to identify factors that make this pediatric population more susceptible and establish better infection prophylaxis protocols.…”

Section: Infectionsmentioning

confidence: 99%

“…5 The posteroventrolateral globus pallidus internus (GPi) has become a well-established target for deep brain stimulation (DBS) in both primary and secondary dystonia. 23,35 While primary dystonia has been shown to respond robustly to pallidal stimulation in both adults and children, 1,2,6,12,21,29,39 secondary dystonia, especially that related to CP, appears to be less responsive. 1,2,9,21,23,27,30 The inherent difficulty in evaluating the effect of DBS within this heterogeneous population is that the literature is sparse and fraught with case series that include incomplete data, involve the inconsistent use of dystonia rating scales that may not be sensitive to the subtleties of secondary dystonia, and consist of patients of different age groups suffering from various forms of secondary dystonia.…”

mentioning

confidence: 99%

“…22 This discrepancy was not observed on BADS scoring, as both groups improved similarly (CP 19.3% vs DYT1 27.6%), and neither group showed improvement on the BFMDRS-D scores. Marks and colleagues, as well as others, 1,6,8,12,24 have advocated for early intervention in both groups before musculoskeletal contractures limit the therapeutic response. 22 Olaya et al recently reported their experience with DBS in children and young adults with secondary dystonia.…”

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Deep brain stimulation for the treatment of childhood dystonic cerebral palsy

Keen¹,

Przekop

Olaya

et al. 2014

PED

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Object Deep brain stimulation (DBS) for dystonic cerebral palsy (CP) has rarely been reported, and its efficacy, though modest when compared with that for primary dystonia, remains unclear, especially in the pediatric population. The authors present a small series of children with dystonic CP who underwent bilateral pallidal DBS, to evaluate the treatment's efficacy and safety in the pediatric dystonic CP population. Methods The authors conducted a retrospective review of patients (under the age of 18 years) with dystonic CP who had undergone DBS of the bilateral globus pallidus internus between 2010 and 2012. Two of the authors independently assessed outcomes using the Barry-Albright Dystonia Scale (BADS) and the Burke-Fahn-Marsden Dystonia Rating Scale–movement (BFMDRS-M). Results Five children were diagnosed with dystonic CP due to insults occurring before the age of 1 year. Mean age at surgery was 11 years (range 8–17 years), and the mean follow-up was 26.6 months (range 2–42 months). The mean target position was 20.6 mm lateral to the midcommissural point. The mean preoperative and postoperative BADS scores were 23.8 ± 4.9 (range 18.5–29.0) and 20.0 ± 5.5 (range 14.5–28.0), respectively, with a mean overall percent improvement of 16.0% (p = 0.14). The mean preoperative and postoperative BFMDRS-M scores were 73.3 ± 26.6 (range 38.5–102.0) and 52.4 ± 21.5 (range 34.0–80.0), respectively, with a mean overall percent improvement of 28.5% (p = 0.10). Those stimulated at least 23 months (4 patients) improved 18.3% (p = 0.14) on the BADS and 30.5% (p = 0.07) on the BFMDRS-M. The percentage improvement per body region yielded conflicting results between rating scales; however, BFMDRS-M scores for speech showed some of the greatest improvements. Two patients required hardware removal (1 complete system, 1 unilateral electrode) within 4 months after implantation because of infections that resolved with antibiotics. Conclusions All postoperative dystonia rating scale scores improved with pallidal stimulation, and the greatest improvements occurred in those stimulated the longest. The results were modest but comparable to findings in other similar series. Deep brain stimulation remains a viable treatment option for childhood dystonic CP, although young children may have an increased risk of infection. Of particular note, improvements in the BFMDRS-M subscores for speech were comparable to those for other muscle groups, a finding not previously reported.

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Section: Infectionsmentioning

confidence: 99%

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confidence: 99%

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confidence: 99%

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Deep brain stimulation for the treatment of childhood dystonic cerebral palsy

Keen¹,

Przekop

Olaya

et al. 2014

PED

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“…1,4,6,8,18,21 Clinical outcome is critically dependent on exact lead placement in the posterior (motor) area of the internal pallidum, 26,28 but surgical methods to achieve excellent lead placement have not been standardized. In adults, the traditional method for DBS lead implantation involves physiological localization while patients are awake.…”

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confidence: 99%

Interventional MRI–guided deep brain stimulation in pediatric dystonia: first experience with the ClearPoint system

Starr

Markun

Larson

et al. 2014

PED

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Object The placement of deep brain stimulation (DBS) leads in adults is traditionally performed using physiological confirmation of lead location in the awake patient. Most children are unable to tolerate awake surgery, which poses a challenge for intraoperative confirmation of lead location. The authors have developed an interventional MRI (iMRI)–guided procedure to allow for real-time anatomical imaging, with the goal of achieving very accurate lead placement in patients who are under general anesthesia. Methods Six pediatric patients with primary dystonia were prospectively enrolled. Patients were candidates for surgery if they had marked disability and medical therapy had been ineffective. Five patients had the DYT1 mutation, and mean age at surgery was 11.0 ± 2.8 years. Patients underwent bilateral globus pallidus internus (GPi, n = 5) or sub-thalamic nucleus (STN, n = 1) DBS. The leads were implanted using a novel skull-mounted aiming device in conjunction with dedicated software (ClearPoint system), used within a 1.5-T diagnostic MRI unit in a radiology suite, without physiological testing. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was used at baseline, 6 months, and 12 months postoperatively. Further measures included lead placement accuracy, quality of life, adverse events, and stimulation settings. Results A single brain penetration was used for placement of all 12 leads. The mean difference (± SD) between the intended target location and the actual lead location, in the axial plane passing through the intended target, was 0.6 ± 0.5 mm, and the mean surgical time (leads only) was 190 ± 26 minutes. The mean percent improvement in the BFMDRS movement scores was 86.1% ± 12.5% at 6 months (n = 6, p = 0.028) and 87.6% ± 19.2% at 12 months (p = 0.028). The mean stimulation settings at 12 months were 3.0 V, 83 μsec, 135 Hz for GPi DBS, and 2.1 V, 60 μsec, 145 Hz for STN DBS). There were no serious adverse events. Conclusions Interventional MRI–guided DBS using the ClearPoint system was extremely accurate, provided real-time confirmation of DBS placement, and could be used in any diagnostic MRI suite. Clinical outcomes for pediatric dystonia are comparable with the best reported results using traditional frame-based stereotaxy. Clinical trial registration no.: NCT00792532 (ClinicalTrials.gov).

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“…166 Haridas and colleagues followed with a case series of 22 patients with primary dystonia who had 94% median improvement in their functional scores with a decrease in their oral and intrathecal medications. 76 More recently, this same group published their experience with 47 DYT1 patients who received pallidal DBS over 10 years with symptom reduction to less than 20% of baseline. In addition, 61% of their patients discontinued all dystonia-related medications after surgery.…”

Section: Treatment Of Dystoniamentioning

confidence: 99%

Deep brain stimulation: a mechanistic and clinical update

Karas

Mikell

Christian

et al. 2013

FOC

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Deep brain stimulation (DBS), the practice of placing electrodes deep into the brain to stimulate subcortical structures with electrical current, has been increasing as a neurosurgical procedure over the past 15 years. Originally a treatment for essential tremor, DBS is now used and under investigation across a wide spectrum of neurological and psychiatric disorders. In addition to applying electrical stimulation for clinical symptomatic relief, the electrodes implanted can also be used to record local electrical activity in the brain, making DBS a useful research tool. Human single-neuron recordings and local field potentials are now often recorded intraoperatively as electrodes are implanted. Thus, the increasing scope of DBS clinical applications is being matched by an increase in investigational use, leading to a rapidly evolving understanding of cortical and subcortical neurocircuitry. In this review, the authors discuss recent innovations in the clinical use of DBS, both in approved indications as well as in indications under investigation. Deep brain stimulation as an investigational tool is also reviewed, paying special attention to evolving models of basal ganglia and cortical function in health and disease. Finally, the authors look to the future across several indications, highlighting gaps in knowledge and possible future directions of DBS treatment.

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Pallidal Deep Brain Stimulation for Primary Dystonia in Children

Abstract: Pallidal DBS is a safe and effective treatment for PGD in patients <21 years of age. The improvement appears durable. Improvement in device design should reduce hardware-related complications over time.

Cited by 63 publications

References 22 publications

Deep brain stimulation for the treatment of childhood dystonic cerebral palsy

Deep brain stimulation for the treatment of childhood dystonic cerebral palsy

Interventional MRI–guided deep brain stimulation in pediatric dystonia: first experience with the ClearPoint system

Deep brain stimulation: a mechanistic and clinical update

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