Shorter Disease Duration Correlates With Improved Long-term Deep Brain Stimulation Outcomes in Young-Onset DYT1 Dystonia

Neurosurgery ◽  
2012 ◽  
Vol 71 (2) ◽  
pp. 325-330 ◽  
Author(s):  
Leslie C. Markun ◽  
Philip A. Starr ◽  
Ellen L. Air ◽  
William J. Marks ◽  
Monica M. Volz ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Torsion Dystonia ◽  
Duration Of Disease ◽  
Primary Torsion ◽  
Primary Torsion Dystonia ◽  
Deep Brain

Abstract BACKGROUND: Treatment with deep brain stimulation (DBS) of the globus pallidus internus in children with DYT1 primary torsion dystonia is highly effective; however, individual response to stimulation is variable, and a greater understanding of predictors of long-term outcome is needed. OBJECTIVE: To report the long-term outcomes of subjects with young-onset DYT1 primary torsion dystonia treated with bilateral globus pallidus DBS. METHODS: Fourteen subjects (7 male, 7 female) treated consecutively from 2000 to 2010 at our center were included in this retrospective study. The Burke-Fahn-Marsden Dystonia Rating Scale was performed at baseline and at 1, 2, and up to 6 years postoperatively. RESULTS: Pallidal DBS was well tolerated and highly effective, with mean Burke-Fahn-Marsden Dystonia Rating Scale movement scores improving from baseline by 61.5% (P < .001) at 1 year, 64.4% (P < .001) at 2 years, and 70.3% (P < .001) at the final follow-up visit (mean, 32 months; range, 7–77 months). Disability scores also improved significantly. Multiple linear regression analysis revealed a significant influence of duration of disease as a predictor of percent improvement in Burke-Fahn-Marsden Dystonia Rating Scale movement score at long-term follow-up (duration of disease, P < .05). Subjects with fixed orthopedic deformities (4) had less improvement in these regions. Location of the active DBS electrode used at final follow-up visit was not predictive of clinical outcome. CONCLUSION: Our findings highlight the sustained benefit from DBS and the importance of early referral for DBS in children with medically refractory DYT1 primary torsion dystonia, which can lead to improved long-term benefits.

Bilateral Deep Brain Stimulation for Cervical Dystonia: Long-term Outcome in a Series of 10 Patients

Neurosurgery ◽  
2010 ◽  
Vol 67 (4) ◽  
pp. 957-963 ◽  
Author(s):  
Francesco Cacciola ◽  
Jibril Osman Farah ◽  
Paul R Eldridge ◽  
Patricia Byrne ◽  
Telekath K Varma
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Cervical Dystonia ◽  
Rating Scale ◽  
Spasmodic Torticollis ◽  
Long Term Outcome ◽  
Stimulation Parameters ◽  
Deep Brain

Abstract BACKGROUND: Bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) was shown to be effective in cervical dystonia refractory to medical treatment in several small short-term and 1 long-term follow-up series. Optimal stimulation parameters and their repercussions on the cost/benefit ratio still need to be established. OBJECTIVE: To report our long-term outcome with bilateral GPi deep brain stimulation in cervical dystonia. METHODS: The Toronto Western Spasmodic Torticollis Rating Scale was evaluated in 10 consecutive patients preoperatively and at last follow-up. The relationship of improvement in postural severity and pain was analyzed and stimulation parameters noted and compared with those in a similar series in the literature. RESULTS: The mean (standard deviation) follow-up was 37.6 (16.9) months. Improvement in the total Toronto Western Spasmodic Torticollis Rating Scale score as evaluated at latest follow-up was 68.1% (95% confidence interval: 51.5-84.6). In 4 patients, there was dissociation between posture severity and pain improvement. Prevalently bipolar stimulation settings and high pulse widths and amplitudes led to excellent results at the expense of battery life. CONCLUSION: Improvement in all 3 subscale scores of the Toronto Western Spasmodic Torticollis Rating Scale with bilateral GPi deep brain stimulation seems to be the rule. Refinement of stimulation parameters might have a significant impact on the cost/benefit ratio of the treatment. The dissociation of improvement in posture severity and pain provides tangible evidence of the complex nature of cervical dystonia and offers interesting insight into the complex functional organization of the GPi.

scholarly journals Longitudinal follow-up with VIM thalamic deep brain stimulation for dystonic or essential tremor

Neurology ◽  
2020 ◽  
Vol 94 (10) ◽  
pp. e1073-e1084 ◽  
Author(s):  
Takashi Tsuboi ◽  
Zakia Jabarkheel ◽  
Pamela R. Zeilman ◽  
Matthew J. Barabas ◽  
Kelly D. Foote ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Essential Tremor ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Statistical Significance ◽  
Ventral Intermediate Nucleus ◽  
Deep Brain

ObjectiveTo assess longitudinal tremor outcomes with ventral intermediate nucleus deep brain stimulation (VIM DBS) in patients with dystonic tremor (DT) and to compare with DBS outcomes in essential tremor (ET).MethodsWe retrospectively investigated VIM DBS outcomes for 163 patients followed at our center diagnosed with either DT or ET. The Fahn-Tolosa-Marin tremor rating scale (TRS) was used to assess change in tremor and activities of daily living (ADL) at 6 months, 1 year, 2–3 years, 4–5 years, and ≥6 years after surgery.ResultsTwenty-six patients with DT and 97 patients with ET were analyzed. Compared to preoperative baseline, there were significant improvements in TRS motor up to 4–5 years (52.2%; p = 0.032) but this did not reach statistical significance at ≥6 years (46.0%, p = 0.063) in DT, which was comparable to the outcomes in ET. While the improvements in the upper extremity tremor, head tremor, and axial tremor were also comparable between DT and ET throughout the follow-up, the ADL improvements in DT were lost at 2–3 years follow-up.ConclusionOverall, tremor control with VIM DBS in DT and ET was comparable and remained sustained at long term likely related to intervention at the final common node in the pathologic tremor network. However, the long-term ADL improvements in DT were not sustained, possibly due to inadequate control of concomitant dystonia symptoms. These findings from a large cohort of DT indicate that VIM targeting is reasonable if the tremor is considerably more disabling than the dystonic features.Classification of evidenceThis study provides Class IV evidence that VIM DBS improves tremor in patients with DT or ET.

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.

Sixty hertz pallidal deep brain stimulation for primary torsion dystonia

Neurology ◽  
2007 ◽  
Vol 69 (7) ◽  
pp. 681-688 ◽  
Author(s):  
R. L. Alterman ◽  
J. Miravite ◽  
D. Weisz ◽  
J. L. Shils ◽  
S. B. Bressman ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Torsion Dystonia ◽  
Pallidal Deep Brain Stimulation ◽  
Primary Torsion ◽  
Primary Torsion Dystonia ◽  
Deep Brain

Thalamic deep brain stimulation for tremor in Parkinson disease, essential tremor, and dystonia

Neurology ◽  
2017 ◽  
Vol 89 (13) ◽  
pp. 1416-1423 ◽  
Author(s):  
Rubens Gisbert Cury ◽  
Valerie Fraix ◽  
Anna Castrioto ◽  
Maricely Ambar Pérez Fernández ◽  
Paul Krack ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Parkinson Disease ◽  
Essential Tremor ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Dystonic Tremor ◽  
Over Time ◽  
Deep Brain

Objective:To report on the long-term outcomes of deep brain stimulation (DBS) of the thalamic ventral intermediate nucleus (VIM) in Parkinson disease (PD), essential tremor (ET), and dystonic tremor.Methods:One hundred fifty-nine patients with PD, ET, and dystonia underwent VIM DBS due to refractory tremor at the Grenoble University Hospital. The primary outcome was a change in the tremor scores at 1 year after surgery and at the latest follow-up (21 years). Secondary outcomes included the relationship between tremor score reduction over time and the active contact position. Tremor scores (Unified Parkinson's Disease Rating Scale-III, items 20 and 21; Fahn, Tolosa, Marin Tremor Rating Scale) and the coordinates of the active contacts were recorded.Results:Ninety-eight patients were included. Patients with PD and ET had sustained improvement in tremor with VIM stimulation (mean improvement, 70% and 66% at 1 year; 63% and 48% beyond 10 years, respectively; p < 0.05). There was no significant loss of stimulation benefit over time (p > 0.05). Patients with dystonia exhibited a moderate response at 1-year follow-up (41% tremor improvement, p = 0.027), which was not sustained after 5 years (30% improvement, p = 0.109). The more dorsal active contacts' coordinates in the right lead were related to a better outcome 1 year after surgery (p = 0.029). During the whole follow-up, forty-eight patients (49%) experienced minor side effects, whereas 2 (2.0%) had serious events (brain hemorrhage and infection).Conclusions:VIM DBS is an effective long-term (beyond 10 years) treatment for tremor in PD and ET. Effects on dystonic tremor were modest and transient.Classification of evidence:This provides Class IV evidence. It is an observational study.

scholarly journals Deep Brain Stimulation in DYT1 Dystonia

Neurosurgery ◽  
2013 ◽  
Vol 73 (1) ◽  
pp. 86-93 ◽  
Author(s):  
Fedor Panov ◽  
Yakov Gologorsky ◽  
Grayson Connors ◽  
Michele Tagliati ◽  
Joan Miravite ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Symptom Severity ◽  
Rating Scale ◽  
Long Term Results ◽  
Long Term Effects ◽  
Dyt1 Dystonia ◽  
Deep Brain

Abstract BACKGROUND: Globus Pallidus Interna (GPi) deep brain stimulation (DBS) is an effective treatment for DYT1-associated dystonia, but long-term results are lacking. OBJECTIVE: To evaluate the long-term effects of GPi DBS in patients with DYT1 dystonia. METHODS: A retrospective chart review (cohort study) of 47 consecutive DYT1+ patients treated by a single surgical team over a 10-year period and followed for up to 96 months (mean, 46 months) was performed. Symptom severity was quantified with the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) motor (M) and disability (D) sub-scores. RESULTS: As measured with the BFMDRS (M), symptom severity was reduced to less than 20% of baseline after 2 years of DBS therapy (P = .001). The disability scores were reduced to &lt;30% of baseline (P = .001). Symptomatic improvement was durable throughout available follow-up. Sixty-one percent of patients had discontinued all dystonia-related medications at their last follow-up. Ninety-one percent had discontinued at least 1 class of medication. Infections requiring removal and later reimplantation of hardware occurred in 4 of 47 patients (8.5%). Hardware malfunction including lead fractures occurred in 4 of 47 cases (8.5%). Lead revision to address poor clinical response was performed in 2 of 92 implanted leads (2.2%). CONCLUSION: GPi DBS is an effective therapy for DYT1-associated torsion dystonia. Statistically significant efficacy is maintained for up to 7 years. Neurologic complications are rare, but long-term hardware-related complications can be significant.

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 benefits in quality of life after unilateral thalamic deep brain stimulation for essential tremor

2012 ◽  
Vol 117 (1) ◽  
pp. 156-161 ◽  
Author(s):  
Jules M. Nazzaro ◽  
Rajesh Pahwa ◽  
Kelly E. Lyons
Keyword(s):  
Quality Of Life ◽  
Deep Brain Stimulation ◽  
Essential Tremor ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Well Being ◽  
Deep Brain

Object The goal of this study was to evaluate short- and long-term benefits in quality of life (QOL) after unilateral deep brain stimulation (DBS) for essential tremor (ET). Methods Patients who received unilateral DBS of the ventral intermediate nucleus of the thalamus between 1997 and 2010 and who had at least 1 follow-up evaluation at least 1 year after surgery were included. Their QOL was assessed with the Parkinson Disease Questionnaire-39 (PDQ-39), and ET was measured with the Fahn-Tolosa-Marin tremor rating scale (TRS) prior to surgery and then postoperatively with the stimulation in the on mode. Results Ninety-one patients (78 at 1 year; 42 at 2–7 years [mean 4 years]; and 22 at > 7–12 years [mean 9 years]) were included in the analysis. The TRS total, targeted tremor, and activities of daily living (ADL) scores were significantly improved compared with presurgical scores up to 12 years. The PDQ-39 ADL, emotional well-being, stigma, and total scores were significantly improved up to 7 years after surgery compared with presurgical scores. At the longest follow-up, only the PDQ-39 stigma score was significantly improved, and the PDQ-39 mobility score was significantly worsened. Conclusions Unilateral thalamic stimulation significantly reduces ET and improves ADL scores for up to 12 years after surgery, as measured by the TRS. The PDQ-39 total score and the domains of ADL, emotional well-being, and stigma were significantly improved up to 7 years. Although scores were improved compared with presurgery, other than stigma, these benefits did not remain significant at the longest (up to 12 years) follow-up, probably related in part to changes due to aging and comorbidities.

scholarly journals Surgical Management for Dystonia: Efficacy of Deep Brain Stimulation in the Long Term

2021 ◽  
Vol 13 (3) ◽  
pp. 371-386
Author(s):  
Walaa A. Kamel ◽  
Pritam Majumdar ◽  
Georgios Matis ◽  
Albert J. Fenoy ◽  
Shankar Balakrishnan ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Statistical Significance ◽  
Longitudinal Change ◽  
Effective Treatment Option ◽  
Post Surgery ◽  
Deep Brain

Introduction: Dystonia is a movement disorder substantially affecting the quality of life. Botulinum Neurotoxin (BoNT) is used intramuscularly as a treatment for dystonia; however, not all dystonia patients respond to this treatment. Deep brain stimulation (DBS) is an established treatment for Parkinson’s disease (PD) and essential tremor, but it can help in dystonia as well. Objectives: We studied a total of 67 dystonia patients who were treated with DBS over a period of 7 years to find out the long-term efficacy of DBS in those patients. First, we calculated patient improvement in post-surgery follow-up programs using the Global Dystonia Severity scale (GDS) and Burke–Fahn–Marsden dystonia rating scale (BFMDRS). Secondly, we analyzed the scales scores to see if there was any statistical significance. Methods: In our study we analyzed patients with ages from 38 to 78 years with dystonia who underwent DBS surgery between January 2014 and December 2020 in four different centers (India, Kuwait, Egypt, and Turkey). The motor response to DBS surgery was retrospectively measured for each patient during every follow-up visit using the GDS and the BFMDRS scales. Results: Five to 7 years post-DBS, the mean reduction in the GDS score was 30 ± 1.0 and for the BFMDRS score 26 ± 1.0. The longitudinal change in scores at 12 and 24 months post-op was also significant with mean reductions in GDS and BFMDRS scores of 68 ± 1.0 and 56 ± 1.0, respectively. The p-values were <0.05 for our post-DBS dystonia patients. Conclusions: This study illustrates DBS is an established, effective treatment option for patients with different dystonias, such as generalized, cervical, and various brain pathology-induced dystonias. Although symptoms are not completely eliminated, continuous improvements are noticed throughout the post-stimulation time frame.

Pallidal Deep Brain Stimulation for Primary Dystonia in Children

Neurosurgery ◽  
2011 ◽  
Vol 68 (3) ◽  
pp. 738-743 ◽  
Author(s):  
Abilash Haridas ◽  
Michele Tagliati ◽  
Irene Osborn ◽  
Ioannis Isaias ◽  
Yakov Gologorsky ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Clinical Improvement ◽  
Brain Stimulation ◽  
Functional Disability ◽  
Rating Scale ◽  
Retrospective Chart Review ◽  
Consecutive Series ◽  
Internal Globus Pallidus ◽  
Deep Brain

Abstract BACKGROUND: Deep brain stimulation (DBS) at the internal globus pallidus (GPi) has replaced ablative procedures for the treatment of primary generalized dystonia (PGD) because it is adjustable, reversible, and yields robust clinical improvement that appears to be long lasting. OBJECTIVE: To describe the long-term responses to pallidal DBS of a consecutive series of 22 pediatric patients with PGD. METHODS: Retrospective chart review of 22 consecutive PGD patients, ≤21 years of age treated by one DBS team over an 8-year period. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was used to evaluate symptom severity and functional disability, pre- and post-operatively. Adverse events and medication changes were also noted. RESULTS: The median follow-up was 2 years (range, 1-8 years). All 22 patients reached 1-year follow-up; 14 reached 2 years, and 11 reached 3 years. The BFMDRS motor subscores were improved 84%, 93%, and 94% (median) at these time points. These motor responses were matched by equivalent improvements in function, and the response to DBS resulted in significant reductions in oral and intrathecal medication requirements after 12 and 24 months of stimulation. There were no hemorrhages or neurological complications related to surgery and no adverse effects from stimulation. Significant hardware-related complications were noted, in particular, infection (14%), which delayed clinical improvement. CONCLUSION: Pallidal DBS is a safe and effective treatment for PGD in patients &lt;21 years of age. The improvement appears durable. Improvement in device design should reduce hardware-related complications over time.

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