Considerations in deep brain stimulation (DBS) for pediatric secondary dystonia

Tsering, Deki; Tochen, Laura; Lavenstein, Bennett; Reddy, Srijaya K.; Granader, Yael; Keating, Robert F.; Oluigbo, Chima

doi:10.1007/s00381-017-3361-x

Cited by 24 publications

(28 citation statements)

References 25 publications

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“…Programming was mostly initiated between 1 day and 3 weeks postoperatively, although in cases of status dystonicus immediate programming was indicated …”

Section: Resultsmentioning

confidence: 99%

Deep brain stimulation for pantothenate kinase‐associated neurodegeneration: A meta‐analysis

et al. 2019

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Background Pantothenate kinase‐associated neurodegeneration is a rare autosomal‐recessive disorder, characterized by progressive neurodegeneration associated with brain iron accumulation. DBS has been trialed to treat related movement disorders, particularly dystonia. The objective of this study was to determine the outcome and safety of DBS for pantothenate kinase‐associated neurodegeneration. Methods We performed a meta‐analysis using independent participant data (n = 99) from 38 articles. Primary outcome was change in movement and disability scores of the Burke‐Fahn‐Marsden Dystonia Rating Scale 1 year postoperatively. Secondary outcomes were response rate and complications. Results Patients with classic‐type (n = 58) and atypical‐type (n = 15) pantothenate kinase‐associated neurodegeneration were operated on at a median age of 11 and 31 years, respectively (P < 0.001). GPi was primarily targeted (n = 87). Mean dystonia movement score improved 1 year following GPi‐DBS (‐26%; 95% confidence interval, ‐37% to ‐15%), particularly in atypical versus classic cases (‐45% vs ‐16%; P < 0.001). At least 30% improvement was observed in 34% of classic versus 73% of atypical cases (P = 0.04). Higher preoperative score and atypical type predicted larger improvement. GPi‐DBS improved dystonia disability score in atypical (‐31%; 95% confidence interval, ‐49% to ‐13%) but not classic (‐5%; 95% confidence interval, ‐17% to 8%) cases. Prevalence of surgical infections (6%) and hardware failure (7%) was similar to other dystonia etiologies. Two patients died within 3 months. There was insufficient data to describe outcome > 1 year following GPi‐DBS or with other DBS targets. Overall, small sample sizes limited generalizability. Conclusions This meta‐analysis provides level 4 evidence that GPi‐DBS for pantothenate kinase‐associated neurodegeneration may improve dystonia movement scores in classic type and atypical type and disability scores in atypical type 1 year postoperatively. © 2019 International Parkinson and Movement Disorder Society

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“…Programming was mostly initiated between 1 day and 3 weeks postoperatively, although in cases of status dystonicus immediate programming was indicated …”

Section: Resultsmentioning

confidence: 99%

Deep brain stimulation for pantothenate kinase‐associated neurodegeneration: A meta‐analysis

et al. 2019

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Section: History Of Pediatric Dbsmentioning

confidence: 99%

“…Frequency in the GPi is usually chosen at 120Hz to 185Hz, although some children can respond to 60Hz or 90Hz . Low‐frequency stimulation in the GPi may be more effective in primary than in secondary dystonia .…”

Section: Programmingmentioning

confidence: 99%

“…Currently, because of uncertain outcomes, the complexity of the procedure, and the risk of infection, DBS is usually reserved for children with significant functional limitations and no other options . With greater predictability of outcome, more reliable effect sizes, and increased simplicity of the procedure it will hopefully be possible to make DBS available to children with moderate symptoms.…”

Section: Future Directionsmentioning

confidence: 99%

“…Following the observation of nearly complete resolution of symptoms after pallidotomy or pallidal stimulation in certain primary dystonias, the GPi has become the standard target for treatment of secondary dystonia. 1,[4][5][6][22][23][24][25][26][27][28] Pallidotomy is less commonly used due to the possibility of mutism after bilateral procedures. 29 Since this does not seem to be a complication of DBS, stimulation has supplanted the earlier use of lesions.…”

Section: History Of Pediatric Dbsmentioning

confidence: 99%

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Deep brain stimulation for cerebral palsy: where are we now?

Sanger

2019

Develop Med Child Neuro

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DBS Deep brain stimulation GPiInternal globus pallidusCerebral palsy (CP) is a complex disorder and children frequently have multiple impairments. Dystonia is a particularly frustrating impairment that interferes with rehabilitation and function and is difficult to treat. Of the available treatments, deep brain stimulation (DBS) has emerged as an option with the potential for large effect size in a subgroup of children. While brain stimulation has been used in CP for more than 40 years, modern devices and targeting methods are improving both the safety and efficacy of the procedure. Successful use of DBS depends on appropriate selection of patients, identification of effective neuroanatomical targets in each patient, careful neurosurgical procedure, and detailed follow-up evaluation and programming. The use of functional neurosurgery for neuromodulation in CP remains a technology in its infancy, but improving experience and knowledge are likely to make this one of the safest and most effective interventions for children with moderate-to-severe motor disorders. This review summarizes the current procedures for patient and target selection, and surgical implantation of DBS electrodes for CP. The history of DBS and future directions when used in secondary dystonia are also examined.

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Deep brain stimulation and intrathecal/intraventricular baclofen for glutaric aciduria type 1: A scoping review, individual patient data analysis, and clinical trials review

Shlobin

Hofmann

Keating

et al. 2023

J of Inher Metab Disea

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Glutaric aciduria type 1 (GA1) is an autosomal recessive disease frequently leading to dystonia. Deep brain stimulation (DBS), intrathecal baclofen (ITB), and intraventricular baclofen (IVB) are the current interventional treatment options for refractory dystonia. We performed a scoping review, individual patient data (IPD) analysis, and clinical trials review to summarize the existing literature on these interventions in this population, characterize outcomes, and suggest directions for future investigation. PubMed, Embase, and Scopus were searched following PRISMA guidelines. IPD were extracted from studies providing IPD for GA1 patients. http://clinicaltrials.gov was reviewed. Of 139 articles, 7 studies with 10 patients were included. In study‐level data, 2/4 (50.0%) DBS studies found no improvement in dystonia and 3/3 (100%) on baclofen found decreased dystonia and enteral medication regimen. In the IPD analysis, four studies with 5 patients (2 IVB, 2 DBS, 1 ITB) were included. The average percent reduction in dystonia was 29.9% ± 32.5% (median:18%, IQR:18%–29.2%). Function improved in 4 (80.0%) patients. All patients with reported changes in enteral dystonia‐related medication regimen (3/3, 100%) reported reduction in medication usage. No patients (0%) had perioperative complications. Mean follow‐up length was 14.8 ± 12.2 months. No interventional clinical trials were found. ITB, IVB, and DBS represent present neuromodulatory approaches for the treatment of GA1. ITB and IVB reduce dystonia, while DBS has a heterogeneous effect. ITB and IVB improved function and reduced enteral medication regimens. These findings must be viewed with caution considering limited data and a serious risk of bias. Further large‐scale studies are necessary to determine indications for ITB, IVB, and DBS and elucidate treatment algorithms.

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Considerations in deep brain stimulation (DBS) for pediatric secondary dystonia

Abstract: Pallidal DBS should be considered among children with functionally debilitating, medication-resistant secondary dystonia. Patients without fixed skeletal deformities who have experienced a short duration of symptoms are most likely to benefit from this intervention.

Cited by 24 publications

References 25 publications

Deep brain stimulation for pantothenate kinase‐associated neurodegeneration: A meta‐analysis

Deep brain stimulation for pantothenate kinase‐associated neurodegeneration: A meta‐analysis

Deep brain stimulation for cerebral palsy: where are we now?

Deep brain stimulation and intrathecal/intraventricular baclofen for glutaric aciduria type 1: A scoping review, individual patient data analysis, and clinical trials review

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