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

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.

Author(s):  
H.A. Eltahawy ◽  
J. Saint-Cyr ◽  
Y.Y. Poon ◽  
E. Moro ◽  
A.E. Lang ◽  
...  

Objective:Report on the clinical results following bilateral globus pallidus interna deep brain stimulation in four patients (one female and three males) with severe cervical dystonia, mean age 48 years (range 37-67).Methods:All four patients had failed extensive medical and botulinum toxin treatment. The mean duration of the disease was nine years (range 4-15 years). Patients were assessed pre and postoperatively using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Preoperatively, the mean TWSTRS total score was 43.2 (range 28-60.5). Posteroventral pallidal deep brain stimulators were inserted using MRI and microelectrode recording guidance. Last follow-up was 15 months for the four patients.Results:Mean reduction in the TWSTRS total scores at last follow- up was 73% (range 61- 85%). Improvement in pain occurred soon after deep brain stimulation surgery. Motor improvement was delayed and prolonged over several months. Frequent adjustment in the stimulation parameters was necessary in the first three months.Conclusion:Bilateral pallidal stimulation is effective in management of selected cases of intractable cervical dystonia.


2013 ◽  
Vol 12 (6) ◽  
pp. 582-587 ◽  
Author(s):  
Melita T. Petrossian ◽  
Lisa R. Paul ◽  
Trisha J. Multhaupt-Buell ◽  
Christine Eckhardt ◽  
Michael T. Hayes ◽  
...  

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.


Author(s):  
Assel Saryyeva ◽  
Hans Holger Capelle ◽  
Thomas Mehari Kinfe ◽  
Christoph Schrader ◽  
Joachim K. Krauss

<b><i>Introduction:</i></b> Deep brain stimulation (DBS) of the globus pallidus internus has become an accepted treatment for severe isolated idiopathic and inherited dystonia. Patients who had other forms of surgery earlier, such as radiofrequency lesioning or selective peripheral denervation, however, usually are not considered candidates for DBS. <b><i>Objective:</i></b> The aim of this study was to evaluate the long-term outcome of pallidal DBS in a rare subgroup of patients who had undergone both pallidotomy and selective peripheral denervation previously with a waning effect over the years. <b><i>Methods:</i></b> Pallidal DBS was performed according to a prospective study protocol in 2 patients with isolated idiopathic dystonia, and patients were followed for a period of at least 6 years. <b><i>Results:</i></b> Both patients benefitted from long-lasting amelioration of dystonia after pallidal DBS, which was comparable to that of patients who did not have previous surgeries. In a 62-year-old female with cervical dystonia both the Burke-Fahn-Marsden (BFM) and the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) motor scores were improved at follow-up 8 years after surgery (50 and 39%). In a 32-year-old male with generalized dystonia, the BFM motor and disability scores showed marked improvement at 6.5 years of follow-up (82 and 66%). <b><i>Conclusions:</i></b> Pallidal DBS can yield marked and long-lasting improvement in patients who underwent both pallidotomy and selective peripheral denervation earlier. Therefore, such patients, in general, should not be excluded from DBS.


2004 ◽  
Vol 17 (1) ◽  
pp. 32-38 ◽  
Author(s):  
Zelma H. T. Kiss ◽  
Kristina Doig ◽  
Michael Eliasziw ◽  
Ranjiit Ranawaya ◽  
Oksana Suchowersky

Object Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is beneficial for generalized dystonia and has been proposed as a treatment for cervical dystonia. The Canadian Stereotactic/Functional and Movement Disorders Groups designed a pilot project to investigate the following hypothesis: that bilateral DBS of the GPi will reduce the severity of cervical dystonia at 1 year of follow up, as scored in a blinded fashion by two neurologists using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Secondary outcome measures included pain and disability subscores of the TWSTRS, Short Form–36 quality of life index, and the Beck Depression Inventory. Methods Three patients have undergone surgery in Calgary with a followup duration of 7.4 ± 5.9 months (mean ± standard deviation). One patient underwent inadvertent ineffective stimulation for the first 3 months and did not experience a benefit until DBS programming was corrected. All three patients had rapid response to stimulation, with the muscles relaxing immediately and abnormal movements improving within days. Total TWSTRS scores improved by 79%, and severity subscores improved significantly, from 15.7 ± 2.1 to 7.7 ± 2.9 (paired ttest, p = 0.02). Pain and disability subscores improved from 25.5 ± 4.1 to 3.3 ± 3.1 (paired ttest, p = 0.002) and from 13.3 ± 4.9 to 3.3 ± 4.2 (paired ttest, p = 0.06), respectively. Conclusions Although it is too early to reach broad conclusions, this report of preliminary results confirms the efficacy of DBS of the GPi for cervical dystonia.


Neurology ◽  
2007 ◽  
Vol 68 (6) ◽  
pp. 457-459 ◽  
Author(s):  
S. W. Hung ◽  
C. Hamani ◽  
A. M. Lozano ◽  
Y-Y W. Poon ◽  
P. Piboolnurak ◽  
...  

Neurology ◽  
2020 ◽  
Vol 94 (10) ◽  
pp. e1073-e1084 ◽  
Author(s):  
Takashi Tsuboi ◽  
Zakia Jabarkheel ◽  
Pamela R. Zeilman ◽  
Matthew J. Barabas ◽  
Kelly D. Foote ◽  
...  

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.


Neurology ◽  
2016 ◽  
Vol 88 (1) ◽  
pp. 25-35 ◽  
Author(s):  
Jill L. Ostrem ◽  
Marta San Luciano ◽  
Kristen A. Dodenhoff ◽  
Nathan Ziman ◽  
Leslie C. Markun ◽  
...  

Objective:To report long-term safety and efficacy outcomes of a large cohort of patients with medically refractory isolated dystonia treated with subthalamic nucleus (STN) deep brain stimulation (DBS).Methods:Twenty patients (12 male, 8 female; mean age 49 ± 16.3 years) with medically refractory isolated dystonia were studied (14 were followed for 36 months). The primary endpoints were change in Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) motor score and Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) total score at 36 months compared to preoperative baseline. Multiple secondary outcomes were also assessed (ClinicalTrials.govNCT00773604).Results:Eighteen of 20 patients showed improvement 12 months after STN DBS with sustained benefit persisting for 3 years (n = 14). At 36 months, BFMDRS motor scores improved 70.4% from a mean 17.9 ± 8.5 to 5.3 ± 5.6 (p = 0.0002) and total TWSTRS scores improved 66.6% from a mean 41.0 ± 18.9 to 13.7 ± 17.9 (p = 0.0002). Improvement at 36 months was equivalent to that seen at 6 months. Disability and quality of life measures were also improved. Three hardware-related and 24 stimulation-related nonserious adverse events occurred between years 1 and 3 (including 4 patients with dyskinesia).Conclusions:This study offers support for long-term tolerability and sustained effectiveness of STN DBS in the treatment of severe forms of isolated dystonia.Classification of evidence:This study provides Class IV evidence that STN DBS decreases long-term dystonia severity in patients with medically refractory isolated dystonia.


2010 ◽  
Vol 112 (6) ◽  
pp. 1271-1276 ◽  
Author(s):  
Kai Zhang ◽  
Sanjay Bhatia ◽  
Michael Y. Oh ◽  
David Cohen ◽  
Cindy Angle ◽  
...  

Object Deep brain stimulation (DBS) of the ventral intermediate nucleus of the thalamus (VIM) has proven to be efficacious in the treatment of essential tremor (ET). The authors report on long-term follow-up of a series of patients treated at 1 institution by 1 neurosurgeon. Methods Thirty-four patients with ET received unilateral or bilateral VIM DBS. The tremor and handwriting components of the Fahn-Tolosa-Marin clinical tremor rating scale were assessed pre- and postoperatively. Visual analog scale scores for overall patient satisfaction and tremor control were recorded. Stimulation parameters at different intervals after surgery were also recorded. Results The average follow-up period was 56.9 months. The average tremor score improved from 3.27 preoperatively to 0.64 postoperatively (on stimulation; p < 0.001) and the average handwriting score improved from 2.94 to 0.89 (p < 0.001). The average visual analog scale score for overall satisfaction was 8.12 and for tremor control was 1.43. Overall, there was an 80.4% improvement in tremor and 69.7% improvement in handwriting. In 12 patients both tremor and handwriting scores were compared between 57.3 months and 90.7 months after surgery and no significant changes were discovered. Comparison of stimulation parameters at onset and at 1–3, 3–5, 5–7, and > 7 years after surgery showed significant differences, with a gradual increase in stimulation parameters within 5 years after surgery. The overall hardware-related complication rate was 23.5%. Conclusions Deep brain stimulation of the VIM is an efficient and safe treatment for ET. Tremor and handwriting improvements in long-term follow-up are stable. The patients' perception of their outcome is quite good. However, tolerance may develop in some patients requiring changes in stimulation parameters.


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 ◽  
...  

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 &lt; .001) at 1 year, 64.4% (P &lt; .001) at 2 years, and 70.3% (P &lt; .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 &lt; .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.


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