Advanced Treatment for Dystonia

Author(s):  
Robertus M. A. de Bie ◽  
Susanne E. M. Ten Holter

Deep-brain stimulation is a last resort for the management of dystonia or dystonic movement disorders when oral or injectable therapies do not provide adequate relief to allow an acceptable quality of life. The underlying pathogenesis of dystonia is less well understood than in Parkinson’s disease, in which deep-brain stimulation is generally expected to provide effective and long-lasting benefit. Furthermore, it is likely that dystonia represents a number of different basal ganglia pathologies. The response of dystonia to deep-brain stimulation is typically delayed for up to weeks or months following a change in stimulation parameters. All of these factors make it more difficult to predict outcomes in dystonia following deep-brain stimulation, thus making patient selection all the more important.

2019 ◽  
Vol 4 (5) ◽  
pp. 825-841 ◽  
Author(s):  
C. K. Broadfoot ◽  
D. Abur ◽  
J. D. Hoffmeister ◽  
C. E. Stepp ◽  
M. R. Ciucci

Purpose Individuals with Parkinson disease (PD) present with complex and variable symptoms, with recent findings suggesting that the etiology of PD extends beyond the involvement of just the basal ganglia. These symptoms include significant impairments in the speech and swallowing domains, which can greatly affect quality of life and therefore require therapeutic attention. This research-based update reviews the neurophysiological basis for swallowing and speech changes in PD, the effectiveness of various types of treatments, and the implications for symptom evaluation and management. Conclusion The mechanisms responsible for swallowing and speech symptoms in PD remain largely unknown. Dopaminergic medication and deep brain stimulation do not provide consistent benefits for these symptoms, suggesting a nondopaminergic network is involved. Importantly, evidence suggests that symptoms of dysphagia and hypokinetic dysarthria may be early indications of PD, so it is critical to investigate the cause of these changes.


2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Florian Lange ◽  
Jonas Roothans ◽  
Tim Wichmann ◽  
Götz Gelbrich ◽  
Christoph Röser ◽  
...  

Abstract Introduction Deep brain stimulation of the internal globus pallidus is an effective treatment for dystonia. However, there is a large variability in clinical outcome with up to 25% non-responders even in highly selected primary dystonia patients. In a large cohort of patients we recently demonstrated that the variable clinical outcomes of pallidal DBS for dystonia may result to a large degree by the exact location and stimulation volume within the pallidal region. Here we test a novel approach of programing based on these insights: we first defined probabilistic maps of anti-dystonic effects by aggregating individual electrode locations and volumes of tissue activated of > 80 patients collected in a multicentre effort. We subsequently modified the algorithms to be able to test all possible stimulation settings of de novo patients in silico based on the expected clinical outcome and thus potentially predict the best possible stimulation parameters for the individual patients. Methods Within the framework of a BMBF-funded study, this concept of a computer-based prediction of optimal stimulation parameters for patients with dystonia will be tested in a randomized, controlled crossover study. The main parameter for clinical efficacy and primary endpoint is based on the blinded physician rating of dystonia severity reflected by Clinical Dystonia Rating Scales for both interventions (best clinical settings and model predicted settings) after 4 weeks of continuous stimulation. The primary endpoint is defined as “successful treatment with model predicted settings” (yes or no). The value is “yes” if the motor symptoms with model predicted settings are equal or better (tolerance 5% of absolute difference in percentages) to clinical settings. Secondary endpoints will include measures of quality of life, calculated energy consumption of the neurostimulation system and physician time for programming. Perspective We envision, that computer-guided deep brain stimulation programming in silico might provide optimal stimulation settings for patients with dystonia without the burden of months of programming sessions. The study protocol is designed to evaluate which programming method is more effective in controlling motor symptom severity and improving quality of life in dystonia (best clinical settings and model predicted settings). Trial registration Registered with ClinicalTrials.gov on Oct 27, 2021 (NCT05097001).


2009 ◽  
Author(s):  
Hunter Covert ◽  
Pennie S. Seibert ◽  
Caitlin C. Otto ◽  
Missy Coblentz ◽  
Nicole Whitener ◽  
...  

Neurology ◽  
2017 ◽  
Vol 89 (19) ◽  
pp. 1944-1950 ◽  
Author(s):  
Matthew A. Brodsky ◽  
Shannon Anderson ◽  
Charles Murchison ◽  
Mara Seier ◽  
Jennifer Wilhelm ◽  
...  

Objective:To compare motor and nonmotor outcomes at 6 months of asleep deep brain stimulation (DBS) for Parkinson disease (PD) using intraoperative imaging guidance to confirm electrode placement vs awake DBS using microelectrode recording to confirm electrode placement.Methods:DBS candidates with PD referred to Oregon Health & Science University underwent asleep DBS with imaging guidance. Six-month outcomes were compared to those of patients who previously underwent awake DBS by the same surgeon and center. Assessments included an “off”-levodopa Unified Parkinson’s Disease Rating Scale (UPDRS) II and III, the 39-item Parkinson's Disease Questionnaire, motor diaries, and speech fluency.Results:Thirty participants underwent asleep DBS and 39 underwent awake DBS. No difference was observed in improvement of UPDRS III (+14.8 ± 8.9 vs +17.6 ± 12.3 points, p = 0.19) or UPDRS II (+9.3 ± 2.7 vs +7.4 ± 5.8 points, p = 0.16). Improvement in “on” time without dyskinesia was superior in asleep DBS (+6.4 ± 3.0 h/d vs +1.7 ± 1.2 h/d, p = 0.002). Quality of life scores improved in both groups (+18.8 ± 9.4 in awake, +8.9 ± 11.5 in asleep). Improvement in summary index (p = 0.004) and subscores for cognition (p = 0.011) and communication (p < 0.001) were superior in asleep DBS. Speech outcomes were superior in asleep DBS, both in category (+2.77 ± 4.3 points vs −6.31 ± 9.7 points (p = 0.0012) and phonemic fluency (+1.0 ± 8.2 points vs −5.5 ± 9.6 points, p = 0.038).Conclusions:Asleep DBS for PD improved motor outcomes over 6 months on par with or better than awake DBS, was superior with regard to speech fluency and quality of life, and should be an option considered for all patients who are candidates for this treatment.Clinicaltrials.gov identifier:NCT01703598.Classification of evidence:This study provides Class III evidence that for patients with PD undergoing DBS, asleep intraoperative CT imaging–guided implantation is not significantly different from awake microelectrode recording–guided implantation in improving motor outcomes at 6 months.


2018 ◽  
Vol 33 (7) ◽  
pp. 1160-1167 ◽  
Author(s):  
Elliot Hogg ◽  
Emmanuel During ◽  
Echo E. Tan ◽  
Kishore Athreya ◽  
Jonathan Eskenazi ◽  
...  

Neurology ◽  
2019 ◽  
Vol 92 (10) ◽  
pp. e1109-e1120 ◽  
Author(s):  
W.M. Michael Schuepbach ◽  
Lisa Tonder ◽  
Alfons Schnitzler ◽  
Paul Krack ◽  
Joern Rau ◽  
...  

ObjectiveTo investigate predictors for improvement of disease-specific quality of life (QOL) after deep brain stimulation (DBS) of the subthalamic nucleus (STN) for Parkinson disease (PD) with early motor complications.MethodsWe performed a secondary analysis of data from the previously published EARLYSTIM study, a prospective randomized trial comparing STN-DBS (n = 124) to best medical treatment (n = 127) after 2 years follow-up with disease-specific QOL (39-item Parkinson's Disease Questionnaire summary index [PDQ-39-SI]) as the primary endpoint. Linear regression analyses of the baseline characteristics age, disease duration, duration of motor complications, and disease severity measured at baseline with the Unified Parkinson’s Disease Rating Scale (UPDRS) (UPDRS-III “off” and “on” medications, UPDRS-IV) were conducted to determine predictors of change in PDQ-39-SI.ResultsPDQ-39-SI at baseline was correlated to the change in PDQ-39-SI after 24 months in both treatment groups (p < 0.05). The higher the baseline score (worse QOL) the larger the improvement in QOL after 24 months. No correlation was found for any of the other baseline characteristics analyzed in either treatment group.ConclusionImpaired QOL as subjectively evaluated by the patient is the most important predictor of benefit in patients with PD and early motor complications, fulfilling objective gold standard inclusion criteria for STN-DBS. Our results prompt systematically including evaluation of disease-specific QOL when selecting patients with PD for STN-DBS.Clinicaltrials.gov identifierNCT00354133.


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