Pallidal Stimulation for Dystonia

Neurosurgery ◽  
2004 ◽  
Vol 55 (6) ◽  
pp. 1361-1370 ◽  
Author(s):  
Martin Krause ◽  
Wolfgang Fogel ◽  
Manja Kloss ◽  
Dirk Rasche ◽  
Jens Volkmann ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Treatment Option ◽  
High Frequency ◽  
Dystonic Tremor ◽  
Tardive Dystonia ◽  
Pallidal Stimulation ◽  
Generalized Dystonia ◽  
Promising Treatment ◽  
Deep Brain

Abstract OBJECTIVE: High-frequency deep brain stimulation (DBS) of the globus pallidus internus (GPi) is a new and promising treatment option for severe dystonia. Yet only few studies have been published to date regarding this treatment. We present the results of DBS of the GPi in 17 patients with severe dystonia of different causes. METHODS: In our study, we included 10 patients with primary generalized dystonia, six patients with secondary generalized dystonia, and one patient with a severe dystonic cervical tremor. In all patients, DBS electrodes were implanted bilaterally within the GPi. Mean follow-up time was 36 months (range, 12–66 mo). Preoperative and postoperative evaluations (at least annually) were performed using the Burk-Fahn-Marsden scale. RESULTS: The best improvement was achieved in patients with DYT1-positive dystonia. Patients with DYT1-negative generalized dystonia showed inhomogeneous results. There was no significant change in patients with tardive dystonia. One case of Hallervorden-Spatz disease improved dramatically within the first 2 years. The improvement in the cervical dystonic tremor was disappointing, however. Three years after DBS implantation, we found a secondary worsening of symptoms in one patient with a DYT1-positive dystonia and in the patient with Hallervorden-Spatz disease. CONCLUSION: DBS of the GPi is a new and promising treatment option for dystonia. Secondary worsening may limit this therapy.

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.

Deep Brain Stimulation Targeting the Globus Pallidus Interna for Dystonic Tremor in the Setting of Generalized Dystonia

2020 ◽  
pp. 205-208
Author(s):  
Qiang Zhang ◽  
Teri R. Thomsen
Keyword(s):  
Deep Brain Stimulation ◽  
Globus Pallidus ◽  
Brain Stimulation ◽  
High Frequency ◽  
Pulse Width ◽  
Low Frequency ◽  
Good Control ◽  
Dystonic Tremor ◽  
Generalized Dystonia ◽  
Deep Brain

Intractable generalized dystonia can be treated by deep brain stimulation (DBS) targeting the globus pallidus interna (GPi). The GPi DBS parameters used for generalized dystonia are frequently different from those used for Parkinson disease, with low-frequency, high–pulse width stimulation being common. This chapter presents a patient with generalized dystonia and debilitating dystonic tremor involving the bilateral hands. He could not tolerate higher pulse width than 60 µsec, but high-frequency (130-Hz) stimulation achieved good control of the dystonic tremor as well as of the generalized dystonia. DBS therapy improved his qualify of life. For generalized dystonia and coexisting dystonic tremor, the tremor may in some cases respond to high-frequency GPi DBS.

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.

Long-term follow-up of bilateral subthalamic deep brain stimulation for refractory tardive dystonia

2017 ◽  
Vol 41 ◽  
pp. 58-65 ◽  
Author(s):  
Zheng-Dao Deng ◽  
Dian-you Li ◽  
Chen-cheng Zhang ◽  
Yi-Xin Pan ◽  
Jin Zhang ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Tardive Dystonia ◽  
Long Term Follow Up ◽  
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.

Lateralized effect of pallidal stimulation on self-mutilation in Lesch-Nyhan disease

2014 ◽  
Vol 14 (6) ◽  
pp. 594-597 ◽  
Author(s):  
Taylor J. Abel ◽  
Brian D. Dalm ◽  
Andrew J. Grossbach ◽  
Adam W. Jackson ◽  
Teri Thomsen ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
The Self ◽  
Lead Fracture ◽  
Self Mutilation ◽  
Pallidal Stimulation ◽  
Generalized Dystonia ◽  
Hypoxanthine Guanine Phosphoribosyltransferase ◽  
Deep Brain

Lesch-Nyhan disease (LND) is an X-linked hereditary disorder caused by a deficiency of hypoxanthine-guanine phosphoribosyltransferase. This syndrome is characterized by hyperuricemia, self-mutilation, cognitive impairment, and movement disorders such as spasticity and dystonia. The authors describe the case of a 15-year-old boy who underwent bilateral placement of globus pallidus internus (GPi) deep brain stimulation (DBS) electrodes for the treatment of generalized dystonia. His self-mutilating behavior gradually disappeared several weeks after the start of GPi stimulation. The dystonia and self-mutilating behavior returned on the left side only after a right lead fracture. This case is the first reported instance of LND treated with DBS in which the stimulation was interrupted and the self-mutilation returned in a lateralized fashion. The findings indicate that the neurobehavioral aspect of LND is lateralized and that contralateral GPi stimulation is responsible for lateralized improvement in self-injurious behavior.

scholarly journals Short circuit in deep brain stimulation

2012 ◽  
Vol 117 (5) ◽  
pp. 955-961 ◽  
Author(s):  
Kazuhiro Samura ◽  
Yasushi Miyagi ◽  
Tsuyoshi Okamoto ◽  
Takehito Hayami ◽  
Junji Kishimoto ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Short Circuit ◽  
Open Circuit ◽  
Symptom Improvement ◽  
Generalized Dystonia ◽  
Short Circuits ◽  
Insidious Development ◽  
Deep Brain

Object The authors undertook this study to investigate the incidence, cause, and clinical influence of short circuits in patients treated with deep brain stimulation (DBS). Methods After the incidental identification of a short circuit during routine follow-up, the authors initiated a policy at their institution of routinely evaluating both therapeutic impedance and system impendence at every outpatient DBS follow-up visit, irrespective of the presence of symptoms suggesting possible system malfunction. This study represents a report of their findings after 1 year of this policy. Results Implanted DBS leads exhibiting short circuits were identified in 7 patients (8.9% of the patients seen for outpatient follow-up examinations during the 12-month study period). The mean duration from DBS lead implantation to the discovery of the short circuit was 64.7 months. The symptoms revealing short circuits included the wearing off of therapeutic effect, apraxia of eyelid opening, or dysarthria in 6 patients with Parkinson disease (PD), and dystonia deterioration in 1 patient with generalized dystonia. All DBS leads with short circuits had been anchored to the cranium using titanium miniplates. Altering electrode settings resulted in clinical improvement in the 2 PD cases in which patients had specific symptoms of short circuits (2.5%) but not in the other 4 cases. The patient with dystonia underwent repositioning and replacement of a lead because the previous lead was located too anteriorly, but did not experience symptom improvement. Conclusions In contrast to the sudden loss of clinical efficacy of DBS caused by an open circuit, short circuits may arise due to a gradual decrease in impedance, causing the insidious development of neurological symptoms via limited or extended potential fields as well as shortened battery longevity. The incidence of short circuits in DBS may be higher than previously thought, especially in cases in which DBS leads are anchored with miniplates. The circuit impedance of DBS should be routinely checked, even after a long history of DBS therapy, especially in cases of miniplate anchoring.

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.

Long-term follow-up of deep brain stimulation for Meige syndrome

2010 ◽  
Vol 29 (2) ◽  
pp. E5 ◽  
Author(s):  
Mark K. Lyons ◽  
Barry D. Birch ◽  
Renee A. Hillman ◽  
Orland K. Boucher ◽  
Virgilio Gerald H. Evidente
Keyword(s):  
Deep Brain Stimulation ◽  
Parkinson Disease ◽  
Brain Stimulation ◽  
Long Term Results ◽  
Meige Syndrome ◽  
Pallidal Stimulation ◽  
Long Term Follow Up ◽  
Deep Brain

Object Meige syndrome is characterized by blepharospasm, cervical dystonia, and facial oromandibular dystonia. The medical treatment of this condition is largely unsuccessful over time and is a major source of decreased quality of life in those patients suffering from this disease. Recent advances in the application of deep brain stimulation (DBS) surgery techniques for many disorders have prompted several recent reports of DBS for medically refractory cases of Meige syndrome. While the etiology for this disorder is unknown, it is considered by many investigators to be a form of idiopathic torsion dystonia. Pallidal stimulation is widely considered to be effective for dystonia. Methods The authors report the long-term results of bilateral globus pallidus internus (GPi) or subthalamic nucleus (STN) stimulation in 3 patients with Meige syndrome and 1 patient with Parkinson disease and associated craniofacial dystonia treated at their center. Results Initial 12-month and long-term follow-up Burke-Fahn-Marsden scores were substantially improved in all 4 patients compared with preoperative scores. Conclusions Bilateral GPi DBS may be an effective and safe treatment for medically refractory Meige syndrome. The results are comparable with those reported in the literature. Sustained and long-term improvement in symptoms does appear to be reproducible across reports. The authors' patient with Parkinson disease and associated craniofacial dystonia syndrome undergoing bilateral STN DBS noted immediate and sustained improvement in his symptoms. Further study is required, but these results, along with the other reports, suggest that bilateral GPi DBS is an effective treatment for medically refractory Meige syndrome.

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