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

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.

Download Full-text

Pallidal deep brain stimulation for dystonia: a case series

Journal of Neurosurgery Pediatrics ◽

10.3171/2013.8.peds13134 ◽

2013 ◽

Vol 12 (6) ◽

pp. 582-587 ◽

Cited By ~ 13

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.

Download Full-text

Long-term follow-up of pallidal Deep Brain Stimulation in teenagers with refractory Tourette syndrome and comorbid psychiatric disorders: About three cases

European Journal of Paediatric Neurology ◽

10.1016/j.ejpn.2016.06.005 ◽

2017 ◽

Vol 21 (1) ◽

pp. 214-217 ◽

Cited By ~ 6

Author(s):

P.-A. Hauseux ◽

F. Cyprien ◽

L. Cif ◽

V. Gonzalez ◽

J.-P. Boulenger ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Psychiatric Disorders ◽

Tourette Syndrome ◽

Brain Stimulation ◽

Comorbid Psychiatric Disorders ◽

Long Term Follow Up ◽

Pallidal Deep Brain Stimulation ◽

Deep Brain

Download Full-text

Long-term follow-up of pallidal deep brain stimulation in two cases of Huntington's disease

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp.2009.202903 ◽

2010 ◽

Vol 82 (3) ◽

pp. 272-277 ◽

Cited By ~ 41

Author(s):

G. A. Kang ◽

S. Heath ◽

J. Rothlind ◽

P. A. Starr

Keyword(s):

Deep Brain Stimulation ◽

Huntington's Disease ◽

Huntington’S Disease ◽

Brain Stimulation ◽

Long Term Follow Up ◽

Pallidal Deep Brain Stimulation ◽

Deep Brain

Download Full-text

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

Case Reports in Neurological Medicine ◽

10.1155/2019/3154653 ◽

2019 ◽

Vol 2019 ◽

pp. 1-2 ◽

Cited By ~ 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.

Download Full-text

P7.20 Bilateral, pallidal deep-brain stimulation in primary and non-primary dystonia: a prospective long-term follow-up study

Clinical Neurophysiology ◽

10.1016/s1388-2457(11)60330-1 ◽

2011 ◽

Vol 122 ◽

pp. S94

Author(s):

L. Romito ◽

G. Zorzi ◽

M. Ciceri ◽

A. Franzini ◽

N. Nardocci ◽

...

Keyword(s):

Deep Brain Stimulation ◽

Brain Stimulation ◽

Follow Up Study ◽

Long Term Follow Up ◽

Primary Dystonia ◽

Pallidal Deep Brain Stimulation ◽

Deep Brain

Download Full-text

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

Neurosurgery ◽

10.1227/neu.0b013e3181ec49c7 ◽

2010 ◽

Vol 67 (4) ◽

pp. 957-963 ◽

Cited By ~ 39

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.

Download Full-text

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

Neurosurgical FOCUS ◽

10.3171/2017.4.focus17134 ◽

2017 ◽

Vol 43 (1) ◽

pp. E2 ◽

Cited By ~ 1

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.

Download Full-text

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

Neurology ◽

10.1212/wnl.0000000000008875 ◽

2020 ◽

Vol 94 (10) ◽

pp. e1073-e1084 ◽

Cited By ~ 6

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.

Download Full-text