scholarly journals Successful Pallidal Deep Brain Stimulation Treatment in a Case of Generalized Dystonia due to a Novel ANO3 Mutation

2019 ◽  
Vol 2019 ◽  
pp. 1-2 ◽  
Author(s):  
Lizl Lasky ◽  
Lindsay Bliss ◽  
Christos Sidiropoulos
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Case Series ◽  
Long Term Outcomes ◽  
Pallidal Stimulation ◽  
Generalized Dystonia ◽  
Pallidal Deep Brain Stimulation ◽  
Deep Brain

Background. Dystonia is a ubiquitous syndrome, with a growing number of genes being continually identified. Mutations in the anoctamin-3 gene have been described to cause dystonia but the management and long-term outcomes are still largely unknown. Methods. We present here a long term, longitudinal follow up of a patient with generalized dystonia, who was treated with bilateral pallidal deep brain stimulation and was found to harbor a mutation in the anoctamin-3 gene. Results. Ongoing adjustment of stimulation settings and medications led to good and sustained dystonia control; however the patient did suffer short term relapses, manifested as dystonic crisis, which necessitated inpatient admission. Conclusion. This only the second patient to be reported with pallidal stimulation and an anoctamin-3 gene mutation. Long term outcomes seem to be favorable but larger case series are needed to confirm our findings.

scholarly journals Pallidal Deep Brain Stimulation in DYT6 Dystonia: Clinical Outcome and Predictive Factors for Motor Improvement

2019 ◽  
Vol 8 (12) ◽  
pp. 2163 ◽  
Author(s):  
Annika Danielsson ◽  
Miryam Carecchio ◽  
Laura Cif ◽  
Anne Koy ◽  
Jean-Pierre Lin ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Clinical Outcome ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Case Series ◽  
Long Term Follow Up ◽  
Pallidal Deep Brain Stimulation ◽  
Deep Brain

Pallidal deep brain stimulation is an established treatment in dystonia. Available data on the effect in DYT-THAP1 dystonia (also known as DYT6 dystonia) are scarce and long-term follow-up studies are lacking. In this retrospective, multicenter follow-up case series of medical records of such patients, the clinical outcome of pallidal deep brain stimulation in DYT-THAP1 dystonia, was evaluated. The Burke Fahn Marsden Dystonia Rating Scale served as an outcome measure. Nine females and 5 males were enrolled, with a median follow-up of 4 years and 10 months after implant. All benefited from surgery: dystonia severity was reduced by a median of 58% (IQR 31-62, p = 0.001) at last follow-up, as assessed by the Burke Fahn Marsden movement subscale. In the majority of individuals, there was no improvement of speech or swallowing, and overall, the effect was greater in the trunk and limbs as compared to the cranio-cervical and orolaryngeal regions. No correlation was found between disease duration before surgery, age at surgery, or preoperative disease burden and the outcome of deep brain stimulation. Device- and therapy-related side-effects were few. Accordingly, pallidal deep brain stimulation should be considered in clinically impairing and pharmaco-resistant DYT-THAP1 dystonia. The method is safe and effective, both short- and long-term.

scholarly journals Pallidal deep brain stimulation for dystonia: a case series

2013 ◽  
Vol 12 (6) ◽  
pp. 582-587 ◽  
Author(s):  
Melita T. Petrossian ◽  
Lisa R. Paul ◽  
Trisha J. Multhaupt-Buell ◽  
Christine Eckhardt ◽  
Michael T. Hayes ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Early Onset ◽  
Rating Scale ◽  
Case Series ◽  
Long Term Outcome ◽  
Pallidal Deep Brain Stimulation ◽  
Deep Brain

Object Pallidal deep brain stimulation (DBS) is a treatment option for those with early-onset dystonia. However, there are limited data on long-term outcome and treatment complications. The authors report on the short- and long-term effects of pallidal DBS in a cohort of patients with early-onset dystonia. Methods Fourteen consecutive pediatric patients with early-onset dystonia were systematically evaluated and treated. The duration of follow-up ranged from 16 to 84 months. Results There were no immediate postoperative complications. At last follow-up, 12 of the 14 patients displayed a significant decline in the Burke-Fahn-Marsden Dystonia Rating Scale motor subscale score, with an average decrease of 62% ± 8.4%. The most common hardware complication was lead fracture (14.3%). Conclusions These data provide further evidence that DBS is a safe and effective treatment for those with earlyonset dystonia.

Long-term outcomes of pallidal deep brain stimulation in X-linked dystonia parkinsonism (XDP): Up to 84 months follow-up and review of literature

2019 ◽  
Vol 60 ◽  
pp. 81-86 ◽  
Author(s):  
Joshua Emmanuel E. Abejero ◽  
Roland Dominic G. Jamora ◽  
Theodor S. Vesagas ◽  
Rosalia A. Teleg ◽  
Raymond L. Rosales ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Review Of Literature ◽  
Long Term Outcomes ◽  
Pallidal Deep Brain Stimulation ◽  
Deep Brain

scholarly journals Deep brain stimulation treatment in dystonia: a bibliometric analysis

2020 ◽  
Vol 78 (9) ◽  
pp. 586-592
Author(s):  
Clarice LISTIK ◽  
Eduardo LISTIK ◽  
Rubens Gisbert CURY ◽  
Egberto Reis BARBOSA ◽  
Manoel Jacobsen TEIXEIRA ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Bibliometric Analysis ◽  
Brain Stimulation ◽  
The United States ◽  
Scientific Journals ◽  
Long Term Outcomes ◽  
Generalized Dystonia ◽  
Research Domain ◽  
Deep Brain

ABSTRACT Background: Dystonia is a heterogeneous disorder that, when refractory to medical treatment, may have a favorable response to deep brain stimulation (DBS). A practical way to have an overview of a research domain is through a bibliometric analysis, as it makes it more accessible for researchers and others outside the field to have an idea of its directions and needs. Objective: To analyze the 100 most cited articles in the use of DBS for dystonia treatment in the last 30 years. Methods: The research protocol was performed in June 2019 in Elsevier’s Scopus database, by retrieving the most cited articles regarding DBS in dystonia. We analyzed authors, year of publication, country, affiliation, and targets of DBS. Results: Articles are mainly published in Movement Disorders (19%), Journal of Neurosurgery (9%), and Neurology (9%). European countries offer significant contributions (57% of our sample). France (192.5 citations/paper) and Germany (144.1 citations/paper) have the highest citation rates of all countries. The United States contributes with 31% of the articles, with 129.8 citations/paper. The publications are focused on General outcomes (46%), followed by Long-term outcomes (12.5%), and Complications (11%), and the leading type of dystonia researched is idiopathic or inherited, isolated, segmental or generalized dystonia, with 27% of articles and 204.3 citations/paper. Conclusions: DBS in dystonia research is mainly published in a handful of scientific journals and focused on the outcomes of the surgery in idiopathic or inherited, isolated, segmental or generalized dystonia, and with globus pallidus internus as the main DBS target.

scholarly journals Unilateral thalamic and pallidal deep brain stimulation for idiopathic hemidystonia: results of individual and combined stimulations. Case report

2017 ◽  
Vol 43 (1) ◽  
pp. E2 ◽  
Author(s):  
Victor Goulenko ◽  
Paulo Luiz da Costa Cruz ◽  
Paulo Niemeyer Filho
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Multiple Target ◽  
Motor Pathways ◽  
Severity Rating ◽  
Pallidal Stimulation ◽  
Pallidal Deep Brain Stimulation ◽  
Deep Brain

Pallidal stimulation has been the usual surgical treatment for dystonia in the last decades. The continuous investigation of the physiopathology and the motor pathways involved leads to the search for complementary targets to improve results. The authors present the case of a 37-year-old woman who had suffered from idiopathic hemidystonia with hyperkinetic and hypokinetic movements for 11 years, and who was treated with deep brain stimulation. A brief literature review is also provided. The globus pallidus internus and the ventral intermediate/ventral oral posterior complex of the thalamus were stimulated separately and simultaneously for 3 months and compared using the Burke-Fahn-Marsden Dystonia Rating Scale and the Global Dystonia Severity Rating Scale, with a 3.5-year follow-up. The synergism of multiple-target stimulation resulted in a complete improvement of the mixed dystonic symptoms.

Deep Brain Stimulation for Dystonia

2020 ◽  
pp. 185-194
Author(s):  
Mitesh Lotia
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Short Term ◽  
Gradual Onset ◽  
Primary Dystonia ◽  
Relative Scarcity ◽  
Pallidal Deep Brain Stimulation ◽  
Deep Brain

The past two decades have revealed substantial benefits of bilateral pallidal deep brain stimulation (DBS) in patients with medication-refractory primary dystonia. There is a growing body of evidence now describing not only short-term but also long-term benefits up to 10 years following DBS. These benefits are often sustained, requiring minimal long-term modification. Pallidal programming for dystonia may be complex owing to the gradual onset of benefits and often delayed development of side effects. There is a relative scarcity of evidence-based recommendations for standardized programming methods. This chapter reviews essential factors to consider for appropriate patient selection and discusses strategies for initial and follow-up programming. Finally, the chapter describes the potential short-term and long-term adverse effects, while considering various strategies to mitigate them.

Disease Stabilization of DYT1-Positive Primary Generalized Dystonia With Deep Brain Stimulation of the Globus Pallidus Interna: A 15-Year Follow-up

2017 ◽  
Vol 14 (5) ◽  
pp. E53-E57 ◽  
Author(s):  
Derrick A Dupre ◽  
Raj Nangunoori ◽  
Sravanthi Koduri ◽  
Cindy Angle ◽  
Diane Cantella ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Globus Pallidus ◽  
Brain Stimulation ◽  
Disease Stabilization ◽  
Long Term Follow Up ◽  
Generalized Dystonia ◽  
The Right ◽  
Deep Brain

Abstract BACKGROUND AND IMPORTANCE Primary generalized dystonia (PGD) is a genetic form of dystonia that frequently displays pharmacological resistance and progresses quickly after onset. Deep brain stimulation (DBS) has been used successfully to treat refractory dystonia, specifically globus pallidus interna (GPi) DBS for DYT1-positive PGD patients. Long-term follow-up of the safety and efficacy falls short of the longevity seen in other diseases treated with DBS. CASE PRESENTATION A male patient presented for neurosurgical evaluation with scapular winging, hand contractures, and violent truncal spasms, which forced him to be bedridden. After failing conservative therapy, the 18-yr-old patient was implanted with bilateral GPi-DBS. DBS parameter adjustments were made primarily within the first 3 yr after implantation, with nominal changes thereafter. Initial settings were contact of 3 + 0−, amplitude of 4.9 V, frequency of 185 Hz, and pulse width of 270 μsec on the left and 3 + 0−, 2.8 V, 185 Hz, and 120 μsec on the right. Current settings are 3 + 2 + 1−, 5.2 V, 130 Hz, 330 μsec on the left and 3 + 0−, 3.5 V, 185 Hz, and 180 μsec on the right and have been relatively unchanged in the past 4 yr. Unified dystonia rating scale scores reveal a significant decrease in dystonic symptoms. CONCLUSION While prior reports have shown that GPi-DBS is effective for dystonia, this is the first with 15 yr of long-term follow-up showing disease stabilization, suggesting that stimulation is efficacious and can potentially prevent disease progression. This report reaffirms previous reports that recommend early surgical intervention before the onset of permanent musculoskeletal deficits.

Deep Brain Stimulation for Disabling “Outflow” Tremors

2020 ◽  
pp. 229-232
Author(s):  
Danielle S. Shpiner ◽  
Sagari Bette ◽  
Corneliu C. Luca
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Case Series ◽  
Long Term Outcomes ◽  
Significant Disability ◽  
Large Case Series ◽  
Clinically Significant ◽  
Site Of Stimulation ◽  
Deep Brain

“Outflow” tremors, sometimes referred to in the literature by a variety of terms (outflow, rubral, midbrain, Holmes), are frequently associated with significant disability and are notoriously unresponsive or only partially responsive to medical treatment. Deep brain stimulation (DBS) has been successfully used in patients with outflow tremors; however, long-term outcomes are not well-documented because large case series are hard to assemble. This chapter describes a patient with disabling outflow tremors secondary to multiple sclerosis, treated with unilateral thalamic DBS, who achieved excellent tremor control with initial programming but shortly afterward developed rebound tremor. Intensive programming involved alternating the site of stimulation by using different combinations of contacts; this was a successful strategy for maintaining a clinically significant reduction in tremor. Disease progression, worsening of tremor, habituation, and loss of efficacy are known problems with some tremors, and their management can be challenging. The pathophysiology of worsening tremor after DBS is discussed in the chapter, as are potential programming strategies to manage this problem.

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