scholarly journals Secondary Dystonia in a Novel Mitochondriopathy Responsive to Deep Brain Stimulation Therapy

2020 ◽  
Vol 8 (1) ◽  
pp. 135-138
Author(s):  
Vibhash D. Sharma ◽  
Cathrin M. Buetefisch ◽  
Frances D. Kendall ◽  
Robert E. Gross ◽  
Mahlon R. DeLong ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Secondary Dystonia ◽  
Deep Brain

Deep brain stimulation for secondary dystonia: results in 8 patients

2009 ◽  
Vol 151 (5) ◽  
pp. 473-478 ◽  
Author(s):  
P. F. Katsakiori ◽  
Z. Kefalopoulou ◽  
E. Markaki ◽  
A. Paschali ◽  
J. Ellul ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Secondary Dystonia ◽  
Deep Brain

scholarly journals 218 Target Selection for Deep Brain Stimulation in Children with Secondary Dystonia Utilizing Temporary Depth Electrodes for Stimulation and Chronic Recording

Neurosurgery ◽  
2017 ◽  
Vol 64 (CN_suppl_1) ◽  
pp. 259-259
Author(s):  
Richard Aaron Robison ◽  
Diana Ferman ◽  
Mark A Liker ◽  
Terrence Sanger
Keyword(s):  
Deep Brain Stimulation ◽  
Beneficial Effect ◽  
Brain Stimulation ◽  
Single Unit ◽  
Response To Therapy ◽  
Rapid Improvement ◽  
Secondary Dystonia ◽  
Test Stimulation ◽  
Depth Electrodes ◽  
Deep Brain

Abstract INTRODUCTION The optimal target for deep brain stimulation (DBS) treatment in children with secondary dystonia is not known, and the target may vary depending on the etiology and anatomic distribution of injury in each child. We present a new technique for determining optimal neuro-anatomical targets in these patients. METHODS Up to ten depth electrodes are implanted in each child in multiple brain regions, including bilateral STN, GPi, and the VLa (Vo), VLp (Vim) and VPL nuclei of the thalamus. Each electrode had both high-impedance micro contacts to identify single unit firing and macro contacts for identifying local field potentials and for performing test stimulation. Children were monitored for up to one week with continuous recording from all electrodes and intermittent test stimulation at bilateral contact pairs. RESULTS >No single consistent pattern of abnormality was found. Most often, single-unit recording showed high firing rates in GPi, and dystonic movement correlated with activity VLa or VLp. The optimal stimulation target varied between children, with rapid improvement of dystonic postures during stimulation in either VLa, VLp or VPL. Stimulation of STN caused resolution of spasms during sleep in one child. Stimulation in GPi did not produce an immediate effect during the recording period, consistent with known latency of treatment effect. All of the children were implanted with up to 4 permanent stimulation leads connected to implanted pulse generators. Preliminary clinical observations show significant beneficial effect in all children. CONCLUSION This new method of DBS targeting identified targets that varied between children. Early response to therapy suggests a beneficial effect that exceeds what would be expected for GPi stimulation alone. This technique may increase the effectiveness of DBS in secondary dystonia and may allow application to a broader range of conditions in children not previously known to respond to stimulation.

Subthalamic nucleus deep brain stimulation for the treatment of secondary dystonia: A case series and review of literature

2017 ◽  
Vol 10 (4) ◽  
pp. 870-872 ◽  
Author(s):  
Jason Margolesky ◽  
Nathan Schoen ◽  
Walter Jermakowicz ◽  
Samir Sur ◽  
Iahn Cajigas ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Subthalamic Nucleus ◽  
Brain Stimulation ◽  
Case Series ◽  
Review Of Literature ◽  
Secondary Dystonia ◽  
Deep Brain

scholarly journals Deep brain stimulation in children and young adults with secondary dystonia: the Children's Hospital Los Angeles experience

2013 ◽  
Vol 35 (5) ◽  
pp. E7 ◽  
Author(s):  
Joffre E. Olaya ◽  
Eisha Christian ◽  
Diana Ferman ◽  
Quyen Luc ◽  
Mark D. Krieger ◽  
...  
Keyword(s):  
Young Adults ◽  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Secondary Dystonia ◽  
Benefit Evaluation ◽  
Generalized Dystonia ◽  
Children And Young Adults ◽  
The Mean ◽  
Deep Brain

Background Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. It can be classified as primary or secondary. There is no cure for dystonia and the goal of treatment is to provide a better quality of life for the patient. Surgical intervention is considered for patients in whom an adequate trial of medical treatment has failed. Deep brain stimulation (DBS), specifically of the globus pallidus interna (GPi), has been shown to be extremely effective in primary generalized dystonia. There is much less evidence for the use of DBS in patients with secondary dystonia. However, given the large number of patients with secondary dystonia, the significant burden on the patients and their families, and the potential for DBS to improve their functional status and comfort level, it is important to continue to investigate the use of DBS in the realm of secondary dystonia. Object The objective of this study is to review a series of cases involving patients with secondary dystonia who have been treated with pallidal DBS. Methods A retrospective review of 9 patients with secondary dystonia who received treatment with DBS between February 2011 and February 2013 was performed. Preoperative and postoperative videos were scored using the Barry-Albright Dystonia Scale (BADS) and Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) by a neurologist specializing in movement disorders. In addition, the patients' families completed a subjective questionnaire to assess the perceived benefit of DBS. Results The average age at DBS unit implantation was 15.1 years (range 6–20 years). The average time to follow-up for the BADS evaluation from battery implantation was 3.8 months (median 3 months). The average time to follow-up for the subjective benefit evaluation was 10.6 months (median 9.5 months). The mean BADS scores improved by 9% from 26.5 to 24 (p = 0.04), and the mean BFMDRS scores improved by 9.3% (p = 0.055). Of note, even in patients with minimal functional improvement, there seemed to be decreased contractures and spasms leading to improved comfort. There were no complications such as infections or hematoma in this case series. In the subjective benefit evaluation, 3 patients' families reported “good” benefit, 4 reported “minimal” benefit, and 1 reported no benefit. Conclusions These early results of GPi stimulation in a series of 9 patients suggest that DBS is useful in the treatment of secondary generalized dystonia in children and young adults. Objective improvements in BADS and BFMDRS scores are demonstrated in some patients with generalized secondary dystonia but not in others. Larger follow-up studies of DBS for secondary dystonia, focusing on patient age, history, etiology, and patterns of dystonia, are needed to learn which patients will respond best to DBS.

scholarly journals Deep brain stimulation in the treatment of secondary dystonia

2006 ◽  
Vol 119 (24) ◽  
pp. 2069-2074 ◽  
Author(s):  
Jian-guo ZHANG ◽  
Kai ZHANG ◽  
Zhong-cheng WANG ◽  
Ming GE ◽  
Yu MA
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Secondary Dystonia ◽  
Deep Brain

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.

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