Deep brain stimulation in children: experience and technical pearls

2011 ◽  
Vol 8 (6) ◽  
pp. 566-574 ◽  
Author(s):  
Ellen L. Air ◽  
Jill L. Ostrem ◽  
Terence D. Sanger ◽  
Philip A. Starr
Keyword(s):  
Deep Brain Stimulation ◽  
Surgical Technique ◽  
Brain Stimulation ◽  
Rating Scales ◽  
Rating Scale ◽  
Glutaric Aciduria Type 1 ◽  
Secondary Dystonia ◽  
Guided Surgery ◽  
Primary Dystonia ◽  
Deep Brain

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.

Clinical phenotypes associated with outcomes following deep brain stimulation for childhood dystonia

2019 ◽  
Vol 24 (4) ◽  
pp. 442-450 ◽  
Author(s):  
Jetan H. Badhiwala ◽  
Brij Karmur ◽  
Lior M. Elkaim ◽  
Naif M. Alotaibi ◽  
Benjamin R. Morgan ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Latent Variables ◽  
Rating Scale ◽  
Significant Heterogeneity ◽  
Secondary Dystonia ◽  
Primary Dystonia ◽  
Relative Change ◽  
Deep Brain

OBJECTIVEAlthough deep brain stimulation (DBS) is an accepted treatment for childhood dystonia, there is significant heterogeneity in treatment response and few data are available to identify ideal surgical candidates.METHODSData were derived from a systematic review and individual patient data meta-analysis of DBS for dystonia in children that was previously published. Outcomes were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale for movement (BFMDRS-M) and for disability (BFMDRS-D). The authors used partial least squares, bootstrapping, and permutation statistics to extract patterns of contributions of specific preoperative characteristics to relationship with distinct outcomes, in all patients and in patients with primary and secondary dystonia separately.RESULTSOf 301 children undergoing DBS for dystonia, 167 had primary dystonia, 125 secondary dystonia, and 9 myoclonus dystonia. Three dissociable preoperative phenotypes (latent variables) were identified and associated with the following: 1) BFMDRS-M at last follow-up; 2) relative change in BFMDRS-M score; and 3) relative change in BFMDRS-D score. The phenotype of patients with secondary dystonia, with a high BFMDRS-M score and truncal involvement, undergoing DBS at a younger age, was associated with a worse postoperative BFMDRS-M score. Children with primary dystonia involving the trunk had greater improvement in BFMDRS-M and -D scores. Those with primary dystonia of shorter duration and proportion of life with disease, undergoing globus pallidus DBS, had greater improvements in BFMDRS-D scores at long-term follow-up.CONCLUSIONSIn a comprehensive, data-driven, multivariate analysis of DBS for childhood dystonia, the authors identified novel and dissociable patient phenotypes associated with distinct outcomes. The findings of this report may inform surgical candidacy for DBS.

Primary Dystonia Is More Responsive than Secondary Dystonia to Pallidal Interventions: Outcome after Pallidotomy or Pallidal Deep Brain Stimulation

Neurosurgery ◽  
2004 ◽  
Vol 54 (3) ◽  
pp. 613-621 ◽  
Author(s):  
Hazem A. Eltahawy ◽  
Jean Saint-Cyr ◽  
Nir Giladi ◽  
Anthony E. Lang ◽  
Andres M. Lozano
Keyword(s):  
Magnetic Resonance Imaging ◽  
Deep Brain Stimulation ◽  
Magnetic Resonance ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Resonance Imaging ◽  
Secondary Dystonia ◽  
Primary Dystonia ◽  
Pallidal Deep Brain Stimulation ◽  
Deep Brain

Abstract OBJECTIVE The response of patients with dystonia to pallidal procedures is not well understood. In this study, we assessed the postoperative outcome of patients with primary and secondary dystonia undergoing pallidotomy or pallidal deep brain stimulation. METHODS Fifteen patients with dystonia had pallidal surgery (lesions or deep brain stimulation). These included nine patients with primary dystonia (generalized and cervical dystonias) and six with secondary dystonia (generalized, segmental, and hemidystonias). There were nine male patients and six female patients. The mean age at onset was 21 years for primary dystonia and 18 years for secondary dystonia. The primary outcome measure was a Global Outcome Scale score for dystonia at 6 months after surgery. Other outcome measures were the Burke-Fahn-Marsden Dystonia Rating Scale and Toronto Western Spasmodic Torticollis Rating Scale scores. RESULTS The mean Global Outcome Scale score at 6 months for patients with primary dystonia was 3 (improvement in both movement disorder and function). In contrast, patients with secondary dystonia had a mean score of 0.83 (mild or no improvement in movement disorder with no functional improvement). All patients with primary dystonia had normal brains by magnetic resonance imaging, whereas five of six patients with secondary dystonia had basal ganglia abnormalities on their magnetic resonance imaging scans. CONCLUSION This study indicates that primary dystonia responds much better than secondary dystonia to pallidal procedures. We could not distinguish a difference in efficacy between pallidotomy and pallidal deep brain stimulation. The presence of basal ganglia abnormalities on the preoperative magnetic resonance imaging scan is an indicator of a lesser response to pallidal interventions for dystonia.

scholarly journals Deep Brain Stimulation for Obsessive-Compulsive Disorder: Real World Experience Post-FDA-Humanitarian Use Device Approval

2021 ◽  
Vol 12 ◽  
Author(s):  
Lora Kahn ◽  
Brianne Sutton ◽  
Helena R. Winston ◽  
Aviva Abosch ◽  
John A. Thompson ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Obsessive Compulsive Disorder ◽  
Brain Stimulation ◽  
Rating Scales ◽  
Rating Scale ◽  
Case Series ◽  
Autism Spectrum ◽  
Obsessive Compulsive ◽  
Compulsive Disorder ◽  
Deep Brain

Background: While case series have established the efficacy of deep brain stimulation (DBS) in treating obsessive-compulsive disorder (OCD), it has been our experience that few OCD patients present without comorbidities that affect outcomes associated with DBS treatment. Here we present our experience with DBS therapy for OCD in patients who all have comorbid disease, together with the results of our programming strategies.Methods: For this case series, we assessed five patients who underwent ventral capsule/ventral striatum (VC/VS) DBS for OCD between 2015 and 2019 at the University of Colorado Hospital. Every patient in this cohort exhibited comorbidities, including substance use disorders, eating disorder, tic disorder, and autism spectrum disorder. We conducted an IRB-approved, retrospective study of programming modifications and treatment response over the course of DBS therapy.Results: In addition to patients' subjective reports of improvement, we observed significant improvement in the Yale-Brown Obsessive-Compulsive Scale (44%), the Montgomery-Asberg Depression Rating Scale (53%), the Quality of Life Enjoyment and Satisfaction Questionnaire (27%), and the Hamilton Anxiety Rating scales (34.9%) following DBS. With respect to co-morbid disease, there was a significant improvement in a patient with tic disorder's Total Tic Severity Score (TTSS) (p = 0.005).Conclusions: DBS remains an efficacious tool for the treatment of OCD, even in patients with significant comorbidities in whom DBS has not previously been investigated. Efficacious treatment results not only from the accurate placement of the electrodes by the surgeon but also from programming by the psychiatrist.

scholarly journals Deep Brain Stimulation for Generalized Dystonia from Secondary Carnitine Deficiency: A Case Report and Literature Review.

2021 ◽  
Author(s):  
Yixuan Zhang ◽  
Laura Sperry ◽  
Michelle Chan ◽  
Suma Shankar ◽  
Norika Malhado-Chang ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Literature Review ◽  
Organic Acid ◽  
Brain Stimulation ◽  
Metabolic Disorder ◽  
Carnitine Deficiency ◽  
Secondary Dystonia ◽  
Generalized Seizures ◽  
Primary Dystonia ◽  
Deep Brain

Abstract BACKGROUND: Carnitine deficiencies result from a metabolic disorder of fatty acid β-oxidation and may lead to organic acidemia, which are thought to be associated with dystonia, epilepsy, autism and developmental delay. Pharmacotherapy has been the dominant therapy, while many refractory patients still require other treatment. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has been found to be effective for medically refractory primary dystonia and now it has been proposed to be used for secondary dystonia from mitochondrial metabolic disorder. OBJECTIVE: To investigate the efficacy and safety of DBS treatment in secondary dystonia from organic acid metabolic disorder. METHODS: We present a patient born with secondary carnitine deficiency who had the onset of generalized seizures at age 4.5 months and developed torsion dystonia at age 14. Multiple medical therapies failed to adequately control her symptoms, therefore she received GPi DBS at age 26 years. In addition, we performed a literature review of this therapy in the treatment of organic acid metabolic disorder. RESULTS: Our patient’s dystonia resolved without side effects post-DBS surgery, but intermittent spastic symptoms along with severe pain in her lower extremity persist. Concerning the 8 cases from our literature review, 7 received GPi DBS, and had improvement in motor symptoms. Overall, DBS efficacy was lower than in treatment of primary dystonia. One patient with methylmalonic acidemia received STN DBS and had marked improvement in dystonia and reduction in pain afterwards. CONCLUSION: DBS has become an effective therapy in refractory secondary dystonia from organic acid metabolic disorder. More prospective studies are needed to determine the eligibility and efficacy of this surgical therapy in these cases.

Deep brain stimulation for the treatment of childhood dystonic cerebral palsy

2014 ◽  
Vol 14 (6) ◽  
pp. 585-593 ◽  
Author(s):  
Joseph R. Keen ◽  
Allison Przekop ◽  
Joffre E. Olaya ◽  
Alexander Zouros ◽  
Frank P. K. Hsu
Keyword(s):  
Cerebral Palsy ◽  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Rating Scales ◽  
Rating Scale ◽  
Pallidal Stimulation ◽  
Increased Risk ◽  
Percent Improvement ◽  
The Mean ◽  
Deep Brain

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.

Mapping efficacious deep brain stimulation for pediatric dystonia

2021 ◽  
pp. 1-11
Author(s):  
Ailish Coblentz ◽  
Gavin J. B. Elias ◽  
Alexandre Boutet ◽  
Jurgen Germann ◽  
Musleh Algarni ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Clinical Outcome ◽  
Brain Stimulation ◽  
Pediatric Patients ◽  
Rating Scale ◽  
Structural Connectivity ◽  
Response To Treatment ◽  
Internal Globus Pallidus ◽  
Probabilistic Map ◽  
Deep Brain

OBJECTIVEThe 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.METHODSA 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.RESULTSA 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.CONCLUSIONSThis 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.

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