scholarly journals The effect of unilateral thalamic deep brain stimulation on the vocal dysfunction in a patient with spasmodic dysphonia: interrogating cerebellar and pallidal neural circuits

2018 ◽  
Vol 128 (2) ◽  
pp. 575-582 ◽  
Author(s):  
Anujan Poologaindran ◽  
Zurab Ivanishvili ◽  
Murray D. Morrison ◽  
Linda A. Rammage ◽  
Mini K. Sandhu ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Neural Circuits ◽  
Rating Scale ◽  
Low Voltage ◽  
Spasmodic Dysphonia ◽  
High Frequency Stimulation ◽  
Vocal Function ◽  
Positive Effects ◽  
Deep Brain

Spasmodic dysphonia (SD) is a neurological disorder of the voice where a patient's ability to speak is compromised due to involuntary contractions of the intrinsic laryngeal muscles. Since the 1980s, SD has been treated with botulinum toxin A (BTX) injections into the throat. This therapy is limited by the delayed-onset of benefits, wearing-off effects, and repeated injections required every 3 months. In a patient with essential tremor (ET) and coincident SD, the authors set out to quantify the effects of thalamic deep brain stimulation (DBS) on vocal function while investigating the underlying motor thalamic circuitry.A 79-year-old right-handed woman with ET and coincident adductor SD was referred to our neurosurgical team. While primarily treating her limb tremor, the authors studied the effects of unilateral, thalamic DBS on vocal function using the Unified Spasmodic Dysphonia Rating Scale (USDRS) and voice-related quality of life (VRQOL). Since dystonia is increasingly being considered a multinodal network disorder, an anterior trajectory into the left thalamus was deliberately chosen such that the proximal contacts of the electrode were in the ventral oralis anterior (Voa) nucleus (pallidal outflow) and the distal contacts were in the ventral intermediate (Vim) nucleus (cerebellar outflow). In addition to assessing on/off unilateral thalamic Vim stimulation on voice, the authors experimentally assessed low-voltage unilateral Vim, Voa, or multitarget stimulation in a prospective, randomized, doubled-blinded manner. The evaluators were experienced at rating SD and were familiar with the vocal tremor of ET. A Wilcoxon signed-rank test was used to study the pre- and posttreatment effect of DBS on voice.Unilateral left thalamic Vim stimulation (DBS on) significantly improved SD vocal dysfunction compared with no stimulation (DBS off), as measured by the USDRS (p < 0.01) and VRQOL (p < 0.01). In the experimental interrogation, both low-voltage Vim (p < 0.01) and multitarget Vim + Voa (p < 0.01) stimulation were significantly superior to low-voltage Voa stimulation.For the first time, the effects of high-frequency stimulation of different neural circuits in SD have been quantified. Unexpectedly, focused Voa (pallidal outflow) stimulation was inferior to Vim (cerebellar outflow) stimulation despite the classification of SD as a dystonia. While only a single case, scattered reports exist on the positive effects of thalamic DBS on dysphonia. A Phase 1 pilot trial (DEBUSSY; clinical trial no. NCT02558634, clinicaltrials.gov) is underway at the authors' center to evaluate the safety and preliminary efficacy of DBS in SD. The authors hope that this current report stimulates neurosurgeons to investigate this new indication for DBS.

scholarly journals Thalamic Deep Brain Stimulation Ameliorates Mixed and Abductor Spasmodic Dysphonia: Case Reports and Proof of Concept

2021 ◽  
Vol 2 (3) ◽  
Author(s):  
C Michael Honey ◽  
Michael G Hart ◽  
Linda A Rammage ◽  
Murray D Morrison ◽  
Amanda Hu ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Case Reports ◽  
Vocal Folds ◽  
Spasmodic Dysphonia ◽  
Proof Of Concept ◽  
Deep Brain

Abstract BACKGROUND AND IMPORTANCE Spasmodic dysphonia (SD) is a dystonia of the vocal folds causing difficulty with speech. A recent randomized controlled trial showed that thalamic deep brain stimulation (DBS) was safe and could improve this condition in the most common subtype—adductor SD. We investigated if thalamic DBS could also improve the other subtypes of abductor SD and mixed SD. These prospective blinded trials of 1 were designed to assess the safety of thalamic DBS in mixed and abductor SD and to quantify the magnitude of any benefit from unilateral or bilateral thalamic stimulation. CLINICAL PRESENTATION One patient with mixed SD and one patient with abductor SD received bilateral thalamic DBS. After optimizing their DBS settings for vocal improvement, they were blinded and prospectively randomized to receive 1 mo of left, right, both, or neither hemisphere stimulation. Outcome was assessed by a speech language pathologist, blinded to the settings, rating voice recordings with the Unified Spasmodic Dysphonia Rating Scale, and by patient self-reported quality-of-life questionnaires. Additional outcomes included scores of mood and cognition. There were no complications. Both patients reported a subjective improvement of their voice and quality of life with blinded left thalamic DBS. The quality of their voice was also objectively rated as improved with blinded left thalamic DBS. CONCLUSION This small proof-of-concept study suggests that left thalamic DBS can improve the quality of voice and quality of life of patients with mixed SD and abductor SD.

scholarly journals Is deep brain stimulation useful in Lance–Adams syndrome?

2021 ◽  
Vol 26 (3) ◽  
pp. 617-620
Author(s):  
Gülşah Öztürk ◽  
İrem Taşçı ◽  
Mustafa Yavuz Samanci ◽  
Selçuk Peker
Keyword(s):  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Cardiopulmonary Arrest ◽  
Disease Onset ◽  
Underlying Mechanism ◽  
High Frequency Stimulation ◽  
Frequency Stimulation ◽  
Deep Brain

Lance–Adams syndrome (LAS) is a chronic post-hypoxic myoclonus that occurs after successful cardiopulmonary resuscitation. Although many drugs are available to treat this condition, the underlying mechanism of the disease is yet to be understood. Deep brain stimulation (DBS) has been attempted and proven to be partially successful in treating LAS in several cases. Here, we present a 40-year-old woman who developed myoclonus subsequent to cardiopulmonary arrest (CPA) that occurred after her first cesarean delivery at the age of 26 years. The patient underwent implantation of bilateral globus pallidus interna (GPi)-DBS about 14 years after disease onset. Regarding Unified Myoclonus Rating Scale (UMRS), 8% and 20% improvements were observed in action and resting myoclonus, respectively, with high-frequency stimulation as a result of the 3-year follow-up study. In this case, neuromodulation therapy applied 14 years after hypoxia-causing LAS was not sufficiently beneficial.

Mapping efficacious deep brain stimulation for pediatric dystonia

2021 ◽  
pp. 1-11
Author(s):  
Ailish Coblentz ◽  
Gavin J. B. Elias ◽  
Alexandre Boutet ◽  
Jurgen Germann ◽  
Musleh Algarni ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Clinical Outcome ◽  
Brain Stimulation ◽  
Pediatric Patients ◽  
Rating Scale ◽  
Structural Connectivity ◽  
Response To Treatment ◽  
Internal Globus Pallidus ◽  
Probabilistic Map ◽  
Deep Brain

OBJECTIVEThe objective of this study was to report the authors’ experience with deep brain stimulation (DBS) of the internal globus pallidus (GPi) as a treatment for pediatric dystonia, and to elucidate substrates underlying clinical outcome using state-of-the-art neuroimaging techniques.METHODSA retrospective analysis was conducted in 11 pediatric patients (6 girls and 5 boys, mean age 12 ± 4 years) with medically refractory dystonia who underwent GPi-DBS implantation between June 2009 and September 2017. Using pre- and postoperative MRI, volumes of tissue activated were modeled and weighted by clinical outcome to identify brain regions associated with clinical outcome. Functional and structural networks associated with clinical benefits were also determined using large-scale normative data sets.RESULTSA total of 21 implanted leads were analyzed in 11 patients. The average follow-up duration was 19 ± 20 months (median 5 months). Using a 7-point clinical rating scale, 10 patients showed response to treatment, as defined by scores < 3. The mean improvement in the Burke-Fahn-Marsden Dystonia Rating Scale motor score was 40% ± 23%. The probabilistic map of efficacy showed that the voxel cluster most associated with clinical improvement was located at the posterior aspect of the GPi, comparatively posterior and superior to the coordinates of the classic GPi target. Strong functional and structural connectivity was evident between the probabilistic map and areas such as the precentral and postcentral gyri, parietooccipital cortex, and brainstem.CONCLUSIONSThis study reported on a series of pediatric patients with dystonia in whom GPi-DBS resulted in variable clinical benefit and described a clinically favorable stimulation site for this cohort, as well as its structural and functional connectivity. This information could be valuable for improving surgical planning, simplifying programming, and further informing disease pathophysiology.

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.

scholarly journals Pediatric Deep Brain Stimulation Using Awake Recording and Stimulation for Target Selection in an Inpatient Neuromodulation Monitoring Unit

2018 ◽  
Vol 8 (7) ◽  
pp. 135 ◽  
Author(s):  
Terence D. Sanger ◽  
Mark Liker ◽  
Enrique Arguelles ◽  
Ruta Deshpande ◽  
Arash Maskooki ◽  
...  
Keyword(s):  
Deep Brain Stimulation ◽  
Basal Ganglia ◽  
Brain Stimulation ◽  
Rating Scale ◽  
Target Selection ◽  
Test Stimulation ◽  
Depth Electrodes ◽  
A New Technique ◽  
High Degree ◽  
Deep Brain

Deep brain stimulation (DBS) for secondary (acquired, combined) dystonia does not reach the high degree of efficacy achieved in primary (genetic, isolated) dystonia. We hypothesize that this may be due to variability in the underlying injury, so that different children may require placement of electrodes in different regions of basal ganglia and thalamus. We describe a new targeting procedure in which temporary depth electrodes are placed at multiple possible targets in basal ganglia and thalamus, and probing for efficacy is performed using test stimulation and recording while children remain for one week in an inpatient Neuromodulation Monitoring Unit (NMU). Nine Children with severe secondary dystonia underwent the NMU targeting procedure. In all cases, 4 electrodes were implanted. We compared the results to 6 children who had previously had 4 electrodes implanted using standard intraoperative microelectrode targeting techniques. Results showed a significant benefit, with 80% of children with NMU targeting achieving greater than 5-point improvement on the Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS), compared with 50% of children using intraoperative targeting. NMU targeting improved BFMDRS by an average of 17.1 whereas intraoperative targeting improved by an average of 10.3. These preliminary results support the use of test stimulation and recording in a Neuromodulation Monitoring Unit (NMU) as a new technique with the potential to improve outcomes following DBS in children with secondary (acquired) dystonia. A larger sample size will be needed to confirm these results.

Psychiatric neurosurgery

2018 ◽  
pp. 135-184
Author(s):  
Walter Glannon
Keyword(s):  
Clinical Trials ◽  
Deep Brain Stimulation ◽  
Brain Stimulation ◽  
Neural Circuits ◽  
Therapeutic Potential ◽  
Genetic Material ◽  
Research Subjects ◽  
Ethical Justification ◽  
The Brain ◽  
Deep Brain

This chapter discusses functional neurosurgery designed to modulate dysfunctional neural circuits mediating sensorimotor, cognitive, emotional, and volitional capacities. The chapter assesses the comparative benefits and risks of neural ablation and deep brain stimulation as the two most invasive forms of neuromodulation. It discusses the question of whether individuals with a severe or moderately severe psychiatric disorder have enough cognitive and emotional capacity to weigh reasons for and against ablation or deep brain stimulation and give informed consent to undergo it. The chapter also discusses the obligations of investigators conducting these trials to research subjects. In addition, it examines the medical and ethical justification for a sham control arm in psychiatric neurosurgery clinical trials. It considers the therapeutic potential of optogenetics as a novel form of neuromodulation. The fact that this technique manipulates both genetic material and neural circuits and has been tested only in animal models makes it unclear what its benefit–risk ratio would be. The chapter concludes with a brief discussion of the potential of neuromodulation to stimulate endogenous repair and growth mechanisms in the brain.

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