Toronto Western Spasmodic Torticollis Rating Scale Scores Research Articles

MRI-Guided Focused Ultrasound in Cervical Dystonia

Introduction. MRI-guided focused ultrasound (MRgFUS) is approved for management of various movement disorders, primarily essential tremor and Parkinson’s disease (PD), with favorable long-term outcomes in numerous patients worldwide. However, few case studies describe the use of this modality for symptomatic treatment of dystonias that, as the third most common movement disorder, may be rather disabling.
 Objective: To improve outcomes in patients with cervical dystonia (CD) using MRgFUS.
 Materials and methods. We retrospectively analyzed 13 cases of various CD types managed with MRgFUS in single or multiple sessions. The mean age of the patients was 42 [39; 53] years. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was used to assess patients' statuses and severity of CD symptoms during therapy and the last available observation period. The targets included the pallidothalamic tract and the thalamic ventral oralis complex nucleus or their combination.
 Results. The mean follow-up period was 13.3 ± 3.4 months (July 2021 to April 2023). The mean CD severity sum score (TWSTRS score) was 22 [16; 25] before MRgFUS and 6 [4; 9] in the last observation. Therefore, we report 70.6% [55.6; 76.5] improvement (paired samples t-test p = 0.0025).
 Conclusion. Available data evidence that MRgFUS is efficient and sufficiently safe for symptomatic treatment in pharmacoresistant CD patients. A number of vital aspects of MRgFUS have to be specified in larger CD cohorts in the long-term follow-up.

GPi-DBS-induced brain metabolic activation in cervical dystonia

BackgroundDeep brain stimulation (DBS) of the globus pallidus interna (GPi) is a highly efficacious treatment for cervical dystonia, but its mechanism of action is not fully understood. Here, we investigate...

The effect of botulinum toxin on anxiety in cervical dystonia: A prospective, observational study

IntroductionAnxiety is present in 30–40% of patients with cervical dystonia (CD). It has been ascribed to a direct effect of the state of motor symptoms on related pain, disability, and disfigurement. Accordingly, any reported benefit of botulinum toxin (BoNT) on anxiety is thought to be secondary to its effect on the same. We sought to evaluate the distinctive impact of botulinum toxin (BoNT) on anxiety in cervical dystonia (CD). MethodsIn this prospective observational study, 60 participants with idiopathic isolated CD were recruited from clinic. We assessed motor and anxiety burden using Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) parts I-III and State-Trait Anxiety Inventory (STAI). Assessments were done at time of BoNT (baseline) and at 6 weeks post-injection. ResultsSTAI and motor severity TWSTRS scores poorly correlated at the baseline visit (rho = −0.30, p = 0.411). Both, motor TWSTRS (Mdifference = −1.46, p < 0.024) and STAI (Mdifference = −10.37, p = 0.007) improved from baseline to 6 weeks (peak effect). The change in motor TWSTRS poorly correlated with change in anxiety scores from baseline visit to 6 weeks (rho = −0.14, p > 0.999). Of these measures of anxiety, improvement in STAI-T had the largest effect size (rank biserial = 0.52). ConclusionBoNT improves both motor severity and anxiety in CD. Poor correlation between motor severity and anxiety at both the time of injection and during the time of peak effect, and improvement in trait anxiety suggests that BoNT has a direct beneficial effect on anxiety.

Characteristics and Treatment Response to OnabotulinumtoxinA of Patients From CD-PROBE With Anterocollis and Retrocollis (P12-11.005)

<h3>Objective:</h3> To describes baseline characteristics and treatment response of these cervical dystonia (CD) subtypes. <h3>Background:</h3> CD-PROBE included patients with anterocollis (AC) and retrocollis (RC) treated with onabotulinumtoxinA (onabotA). <h3>Design/Methods:</h3> CD PROBE was an observational, prospective study designed to identify real-world outcomes in patients with CD after onabotA treatment; patients from 82 US sites with CD diagnosis who were candidates for onabotA therapy, new to treatment, or not previously treated in a clinical trial in ≥16 weeks, and completed 3 onabotA treatments. Treatment benefit was measured using the Patient’s Global Impression of Change (PGI-C), Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), Cervical Dystonia Impact Profile (CDIP-58), Clinician’s Global Impression of Change (CGI-C), and physician assessments of CD severity. <h3>Results:</h3> Patients with AC (n=59) and RC (n=55) were older and more disabled, with a shorter time to diagnosis and treatment, than patients with laterocollis (LC) or torticollis (TC). From PGI-C responses, 58.4% of RC vs 47.8% of AC patients reported being much/very much improved by onabotA post-injection 3 (final visit). CGI-C responses similarly improved over this timeframe; 82.6% of RC and 60.9% of AC patients reporting much/very much improved. The mean total TWSTRS score decreased for AC and RC patients from injection 1 (46 [AC]; 40 [RC]) to final visit (36 [AC]; 32 [RC]); CDIP-58 scores also decreased over time. The proportion of patients with physician-assessed severe CD decreased from injection 1 (28.8% [AC]; 21.8% [RC]) to final visit (21.7% [AC]; 4.3% [RC]); most patients shifted to lower symptom severity. OnabotA dose generally increased per visit, with AC patients having lowest doses (153.5–195.4U) and RC patients the highest (184.0–213.4U). Adverse event of dysphagia reported in 4 AC and 7 RC patients. No new safety signals were identified. <h3>Conclusions:</h3> CD PROBE data indicates real-world treatment with onabotA relieves CD symptoms in patients with anterocollis and retrocollis. <b>Disclosure:</b> Dr. Dashtipour has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Amneal, Abbvie, Acorda, Acadia, Ipsen, Supernus, Sunovion, Neurocrine, Teva. Dr. Dashtipour has received personal compensation in the range of $100,000-$499,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amneal, Abbvie, Acadia, Supernus. Dr. Dashtipour has received personal compensation in the range of $100,000-$499,999 for serving on a Speakers Bureau for Amneal, Acorda, Abbvie, Ipsen, Teva, Neurocrine, SupernusPD, sunovion . Dr. Jankovic has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Revance. Dr. Jankovic has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Revance, Allergan. The institution of Dr. Jankovic has received research support from Baylor College of Medicine. Dr. Jankovic has received research support from Abbvie. The institution of Dr. Jankovic has received research support from Abbvie. Dr. Charles has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Supernus. Dr. Charles has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Revance. Dr. Charles has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merz. Dr. Charles has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Newronika. Dr. Charles has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ipsen. Dr. Charles has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Iota. Dr. Charles has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Abbott. Dr. Charles has received personal compensation in the range of $500-$4,999 for serving as a Consultant for AbbVie. Dr. Charles has stock in Arena Therapeutics. The institution of Dr. Charles has received research support from Pharma 2 B. The institution of Dr. Charles has received research support from Intec. The institution of Dr. Charles has received research support from Merz. The institution of Dr. Charles has received research support from Novartis. The institution of Dr. Charles has received research support from Aeon. The institution of Dr. Charles has received research support from Impax. Dr. Mehta has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Adamas Pharma. Dr. Mehta has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Abbott. Dr. Mehta has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for CNS Ratings. Marc Schwartz has nothing to disclose. Dr. Sadeghi has received personal compensation for serving as an employee of AbbVie. Dr. Sadeghi has stock in Abbvie/Abbott.

Management of cervical dystonia through Ayurveda - A case report

Abstract Spasmodic torticollis is a rare condition characterized by involuntary movement of the head. The pain often becomes the primary reason for the patient to seek medical attention, with the patient almost indifferent to the dystonic movements. Prakupita Vata (aggravated Vata) causes various kinds of diseases of Vata called Vata Vyadhi (diseases of vitiated Vata) and treatments such as Snehana (oleation therapy), Swedana (sudation therapy), and Shodhana (internal detoxification) are advised. The patient complaining of pain, stiffness of the neck, and tremors gradually progressing to hyperextension of the neck was clinically diagnosed as Dhatu Kshaya Vata Vikara and treated on the lines of Vata Vyadhi (diseases of vitiated Vata). Shodhana (internal detoxification) such as Nasya and Basti, in combination with external Panchakarma procedures and internal medicines including Rasayana (rejuvenation), were administered. Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was assessed for the outcome of the treatment. The patient showed good improvement in pain, stiffness, tremors, and neck retention time. TWSTRS score had reduced from 66.25 to 29 and is presented here as a case study.

Therapeutic Efficacy and Prediction of 18F-FDG PET/CT-Assisted Botulinum Toxin Therapy in Patients With Idiopathic Cervical Dystonia.

This study aimed to investigate the therapeutic efficacy of 18F-FDG PET/CT-assisted botulinum toxin (BTX) injection therapy and predictive PET findings in relation to a good response in patients with idiopathic cervical dystonia (ICD). A total of 78 patients was enrolled from November 2007 to July 2018. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) score was determined at baseline and 4 weeks after BTX injection guided by electromyography and PET/CT. The number of hypermetabolic muscles, the highest SUVmax among hypermetabolic muscles, and the total SUVmax of hypermetabolic muscles were evaluated as pretreatment PET parameters. A good response was defined as a reduction rate ≥30% and a point decrease ≥15 of the TWSTRS total score. Half of the subjects showed a good response. Good responders had significantly higher baseline TWSTRS scores than poor responders (total score, P < 0.001; severity, P < 0.001; disability, P < 0.001; pain, P = 0.026). Good responders also had significantly higher numbers of hypermetabolic muscles and BTX-injected hypermetabolic muscles (P < 0.001, both). In multivariable analysis, the baseline TWSTRS disability subscale score and the number of BTX-injected hypermetabolic muscles were significant predictors for good response (P = 0.001 and P = 0.028). The aforementioned 3 PET parameters were positively correlated with the baseline TWSTRS scores. In addition, PET/CT well detected dystonic deep cervical muscles. FDG PET/CT-assisted BTX injection therapy showed good therapeutic efficacy in ICD patients. The numbers of hypermetabolic cervical muscles and BTX-injected hypermetabolic muscles may be helpful in predicting a good response.

The Long-Term Efficacy, Prognostic Factors, Safety, and Hospitalization Costs Following Denervation and Myotomy of the Affected Muscles and Deep Brain Stimulation in 94 Patients with Spasmodic Torticollis.

The surgical methods for treating spasmodic torticollis include the denervation and myotomy (DAM) of the affected muscles and deep brain stimulation (DBS). This study reports on the long-term efficacy, prognostic factors, safety, and hospitalization costs following these two procedures. We collected data from 94 patients with spasmodic torticollis, of whom 41 and 53 were treated with DAM and DBS, respectively, from June 2008 to December 2020 at the Chinese People’s Liberation Army General Hospital. We used the Tsui scale and the global outcome score of the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) to evaluate the preoperative and postoperative clinical conditions in all patients. We also determined the costs of hospitalization, prognostic factors, and serious adverse events following the two surgical procedures. The mean follow-up time was 68.83 months (range = 13–116). Both resection surgery and DBS showed good results in terms of Tsui (Z = −5.103, p = 0.000; Z = −6.210, p = 0.000) and TWSTRS scores (t = 8.762, p = 0.000; Z = −6.308, p = 0.000). Compared with the DAM group, the preoperative (47.71, range 24–67.25) and postoperative (18.57, range 0–53) TWSTRS scores in the DBS group were significantly higher (Z = −3.161, p = 0.002). We found no correlation between prognostic factors and patient age, gender, or disease duration for either surgical procedure. However, prognostic factors were related to the length of the postoperative follow-up period in the DBS surgery group (Z = −2.068, p = 0.039; Z = −3.287, p = 0.001). The mean hospitalization cost in the DBS group was 6.85 times that found in the resection group (Z = −8.284, p = 0.000). The total complication rate was 4.26%. We found both resection surgery and DBS showed good results in the patients with spasmodic torticollis. Compared with DAM, DBS had a greater improvement in TWSTRS score; however, it was more expensive. Prognostic factors were related to the length of the postoperative follow-up period in patients who underwent DBS surgery.

Evaluation of outcome of different neurosurgical modalities in management of cervical dystonia

BackgroundCervical dystonia is the most common form of focal dystonia and is managed by multiple modalities including repeated botulinum toxin injections, in addition to medical treatment with anticholinergics, muscle relaxants, and physiotherapy. However, surgical interventions could be beneficial in otherwise refractory patients. This study aims to report our experience in the neurosurgical management of cervical dystonia and evaluate patient outcomes using reliable outcome scores for the assessment of patients with cervical dystonia and possible complications. This case series study was conducted on 19 patients with cervical dystonia of different etiologies who underwent surgical management [ten patients underwent selective peripheral denervation, five patients underwent pallidotomy, and four patients underwent bilateral globus pallidus internus (GPi) deep brain stimulation (DBS)] in the period between July 2018 and June 2021 at Ain Shams University Hospitals, Cairo, Egypt. With the assessment of surgical outcomes using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and the Tsui scale 6 months postoperatively.ResultsSurgical management of patients with cervical dystonia of either primary or secondary etiology was associated with significant improvement in head and neck postures after 6 months without major complications associated with the different surgical procedures. The mean improvement in total TWSTRS and Tsui scores were 51.2% and 64.8%, respectively, compared with preoperative scores, while the mean improvement in the TWSTRS subscales (severity, disability, and pain) were 40.2%, 66.9%, and 58.3%, respectively.ConclusionCervical dystonia patients in whom non-surgical options have failed to alleviate their symptoms can be managed surgically leading to significant improvements with minimal adverse effects. However, surgical treatment should be tailored according to several factors including but not limited to the etiology, pattern of dystonic activity, and comorbidities. Therefore, management should be tailored to achieve long-term improvement with minimal risk of complications.

P.0132 Selective lesions of medial septal cholinergic and gabaergic neurons differentially affect spatial memory and various neurotransmitter markers in the hippocampus

To investigate the characteristics of oropharyngeal dysphagia in adults with dyskinetic cerebral palsy (DCP) and cervical dystonia (CD).Exploratory observational cross-sectional study.University hospital.Seventeen patients with DCP (8 men, 9 women; age, 45.7±6.3y) enrolled in a randomized controlled trial on the effects of botulinum toxin injection on CD.Not applicable.Baseline clinical assessments and videofluoroscopic swallowing studies (VFSSs) were conducted. VFSS findings were evaluated using the videofluoroscopic dysphagia scale (VDS) and the penetration-aspiration scale (PAS). The Gross Motor Function Classification System (GMFCS) and Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores were also assessed. Relationships between outcomes were evaluated using Spearman’s rank correlation.The clinical assessment revealed abnormalities in chewing (n=10, 58.8%), tongue movement (n=10, 58.8%), and laryngeal elevation (n=8, 47.1%). The most common abnormality on the VDS was inadequate mastication (n=13, 76.5%), followed by premature bolus loss, vallecular residue, and penetration/aspiration (all: n=10, 58.8%). A maximum PAS score of 8 was observed in 8 of 17 patients (47.1%). Total and pharyngeal VDS scores were significantly correlated with TWSTRS scores (ρ=0.543, P=.024 and ρ=0.539, P=.026, respectively); the VDS oral score did not correlate with the TWSTRS score (ρ=0.446, P=.073). There was no significant correlation between VDS score and GMFCS level (ρ=0.212, P=.414).This preliminary observational study presents the characteristics of oropharyngeal dysphagia in adults with DCP and CD. Pharyngeal stage difficulties were negatively correlated with severity of CD, but not with GMFCS level. Screening for dysphagia may be recommended in adults with DCP and severe CD.

Bilateral pallidal stimulation improves cervical dystonia for more than a decade

IntroductionDeep brain stimulation (DBS) is an effective treatment in medically resistant cervical dystonia (CD) with a documented therapeutic effect. Long term outcome beyond a decade, however, has not been studied systematically. MethodsTo investigate the impact of pallidal DBS beyond 10 years in CD we followed a series of five consecutive patients with severe medication-resistant CD. Severity of head and neck deviation, disability, and pain related to dystonia were assessed by the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) in the frame of a prospective study. The primary endpoint of this study was a change in the TWSTRS total score. Secondary endpoints were changes in the subscores of the TWSTRS. ResultsThe mean follow-up time was 11.5 years (range 10–12.8). Comparing baseline and the last follow-up, CD improved by 53% on the total TWSTRS score, by 54.1% on the severity score, and by 70.1% on the disability score, while pain did not improve significantly. Improvement was stable over time. Patients with a tonic pattern of CD responded less to DBS than patients with a phasic pattern. DBS had no significant effect on mood and cognition. Two patients underwent electrode revisions. One patient had an infection of the proximal cable two years after surgery. ConclusionsChronic bilateral pallidal stimulation improves severity of dystonia and disability over more than a decade in treatment resistant CD. Results may vary among individual patients.

Cumulative effects of long-term treatment with abobotulinumtoxinA in cervical dystonia: Findings from a prospective, observational study

ObjectivePatients with cervical dystonia (CD) require regular injections of botulinum neurotoxin type A (BoNT-A) to maintain treatment effectiveness. In this secondary analysis of a large routine-practice study, we assessed the response to abobotulinumtoxinA over repeated cycles and compared the responses of patients previously naïve to BoNT treatment to those already treated with BoNT-A. MethodsINTEREST IN CD2 was a prospective, 3-year, observational study following CD patients treated with BoNT-A. Patients were assessed at each injection visit using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and Tsui scale; patient satisfaction with symptom control (previous cycle) was recorded. ResultsThis analysis included 466 patients treated with abobotulinumtoxinA over 3 years. TWSTRS-Total scores continually decreased; the mean ± SD reduction from baseline to 36 months was −7.4 ± 11.9. BoNT-naïve patients tended to show greater reductions in TWSTRS scores than already treated patients, particularly in the first 6 months. Incremental improvements were also seen in Tsui-tremor scores. Overall levels of patient satisfaction at peak effect (84.1–91.2%) were consistently higher than at the end of cycle (47.2–53.2%). The most frequently reported adverse events were dysphagia (6.8%) and muscular weakness (3.7%). ConclusionsThese data support the long-term effectiveness and safety of abobotulinumtoxinA in a real-world setting.

A pooled meta-analysis of GPi and STN deep brain stimulation outcomes for cervical dystonia.

To analyze deep brain stimulation (DBS) outcomes in patients with cervical dystonia (CD), the relationships between motor and disability/pain outcomes, and the differences in outcomes between globus pallidus internus (GPi) and subthalamic nucleus (STN) DBS, and to identify potential outcome predictors. A systematic literature search identified individual patient data of CD patients who underwent DBS and whose outcomes were assessed with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Then, we performed a pooled meta-analysis on this cohort. A review of 39 papers yielded 208 patients with individual TWSTRS scores and demographic information. At a mean follow-up period of 23.3months after either GPi or STN DBS, the TWSTRS total (58.8%), severity (53.9%), disability (61.3%), and pain (46.6%) scores significantly improved compared to baseline status (all p < 0.001). There were no significant outcome differences between short-term (< 23.3months) and long-term (≥ 23.3months). The TWSTRS outcomes after GPi and STN DBS were comparable, whereas these two targets showed different adverse effect profiles. The rates of responders to DBS according to the TWSTRS total and severity (defined as ≥ 25% improvement) were both 89%. Regression analyses demonstrated motor benefits associated with disability improvement more than pain relief (R2 = 0.345 and 0.195, respectively). No clinically meaningful predictors for DBS outcomes were identified. DBS improves motor symptoms, disability, and pain in CD patients and may provide sustained benefits over 2years. GPi and STN appear to be equally effective targets with different adverse effect profiles.

Unilateral pallidotomy in the treatment of cervical dystonia: a retrospective observational study.

The objective of this study was to assess the efficacy of unilateral pallidotomy in patients with asymmetrical cervical dystonia. This study retrospectively included 25 consecutive patients with asymmetrical cervical dystonia refractory to botulinum toxin injections, who underwent unilateral pallidotomy between January 2015 and April 2017. Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores were evaluated preoperatively and 1 week, 3 months, and 6 months postoperatively. The clinical responses were defined as good responders, exhibiting > 50% improvement in the TWSTRS score at 6 months postsurgery, or poor responders, exhibiting < 50% improvement in TWSTRS scores at 6 months postsurgery. Twelve and 9 patients showed right- and left-side rotation, respectively; 1 and 3 patients had right- and left-side laterocollis, respectively. The mean age of onset and duration of the disease were 40.2 ± 13.9 and 8.9 ± 10.9 years, respectively. Mean TWSTRS scores were 38.4 ± 12.6 (p < 0.001), 17.3 ± 12.4 (p < 0.001), 19.5 ± 13.4 (p < 0.001), and 20.0 ± 14.7 (p < 0.001), preoperatively and 1 week, 3 months, and 6 months postoperatively, respectively. Fourteen patients (56%) demonstrated > 50% improvement in their TWSTRS total score (mean improvement of TWSTRS total score = 70.5%) 6 months postsurgically. Furthermore, preoperative TWSTRS severity score was a prognostic factor (odds ratio 1.37, 95% confidence interval 1.06-1.78, p = 0.003). These results suggest that unilateral pallidotomy is an acceptable treatment option for asymmetrical cervical dystonia. Further investigations with a larger number of cases and longer follow-up period are required to confirm these data.

Clinical outcomes of pallidal deep brain stimulation for dystonia implanted using intraoperative MRI.

Lead placement for deep brain stimulation (DBS) using intraoperative MRI (iMRI) relies solely on real-time intraoperative neuroimaging to guide electrode placement, without microelectrode recording (MER) or electrical stimulation. There is limited information, however, on outcomes after iMRI-guided DBS for dystonia. The authors evaluated clinical outcomes and targeting accuracy in patients with dystonia who underwent lead placement using an iMRI targeting platform. Patients with dystonia undergoing iMRI-guided lead placement in the globus pallidus pars internus (GPi) were identified. Patients with a prior ablative or MER-guided procedure were excluded from clinical outcomes analysis. Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) scores and Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores were assessed preoperatively and at 6 and 12 months postoperatively. Other measures analyzed include lead accuracy, complications/adverse events, and stimulation parameters. A total of 60 leads were implanted in 30 patients. Stereotactic lead accuracy in the axial plane was 0.93 ± 0.12 mm from the intended target. Nineteen patients (idiopathic focal, n = 7; idiopathic segmental, n = 5; DYT1, n = 1; tardive, n = 2; other secondary, n = 4) were included in clinical outcomes analysis. The mean improvement in BFMDRS score was 51.9% ± 9.7% at 6 months and 63.4% ± 8.0% at 1 year. TWSTRS scores in patients with predominant cervical dystonia (n = 13) improved by 53.3% ± 10.5% at 6 months and 67.6% ± 9.0% at 1 year. Serious complications occurred in 6 patients (20%), involving 8 of 60 implanted leads (13.3%). The rate of serious complications across all patients undergoing iMRI-guided DBS at the authors' institution was further reviewed, including an additional 53 patients undergoing GPi-DBS for Parkinson disease. In this expanded cohort, serious complications occurred in 11 patients (13.3%) involving 15 leads (10.1%). Intraoperative MRI-guided lead placement in patients with dystonia showed improvement in clinical outcomes comparable to previously reported results using awake MER-guided lead placement. The accuracy of lead placement was high, and the procedure was well tolerated in the majority of patients. However, a number of patients experienced serious adverse events that were attributable to the introduction of a novel technique into a busy neurosurgical practice, and which led to the revision of protocols, product inserts, and on-site training.

Minimal clinically important change in patients with cervical dystonia: Results from the CD PROBE study

ObjectiveTo determine the minimal clinically important change (MCIC) on Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores using data from Cervical Dystonia Patient Registry for Observation of OnabotulinumtoxinA Efficacy (CD PROBE), which captured real-world practices and outcomes. MethodsChanges in the baseline TWSTRS scores (point and percentage changes) were compared to changes in the Patient and Clinician Global Impression of Change (PGIC and CGIC) ratings. Using logistic regression, the discrimination of the model was determined. ResultsAmong the 479 patients who completed all TWSTRS assessments, the mean TWSTRS Total score significantly decreased from baseline (39.2) to the final visit (27.1) (P < .0001). TWSTRS Total score point changes that compared with PGIC assessments “very much improved,” “much improved” or better, and “minimally improved” or better were −11, −9, and −8, respectively, and were similar to previously published changes (ie, a decrease of ≥10 points). TWSTRS Total score data met indicators of good cutoffs for discrimination of the model including ≥70% percentage of outcomes correctly classified when compared with PGIC ratings. The TWSTRS Total score mapped to PGIC and CGIC ratings better than any TWSTRS subscale score. ConclusionsThe MCIC for improvement was ≥8 points based on mean TWSTRS Total scores in patients with cervical dystonia when compared against the patient-based evaluation of benefit (PGIC).

Deep Brain Stimulation Versus Peripheral Denervation for Cervical Dystonia: A Systematic Review and Meta-Analysis

Cervical dystonia is a disabling medical condition that drastically decreases quality of life. Surgical treatment consists of peripheral nerve denervation procedures with or without myectomies or deep brain stimulation (DBS). The current objective was to compare the efficacy of peripheral denervation versus DBS in improving the severity of cervical dystonia through a systematic review and meta-analysis. A search of PubMed, MEDLINE, EMBASE, and Web of Science electronic databases was conducted in accordance with PRISMA guidelines. Preoperative and postoperative Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) total scores were used to generate standardized mean differences and 95% confidence intervals (CIs), which were combined in a random-effects model. Both mean percentage and absolute reduction in TWSTRS scores were calculated. Absolute reduction was used for forest plots. Eighteen studies met the inclusion criteria, comprising 870 patients with 180 (21%) undergoing DBS and 690 (79%) undergoing peripheral denervation procedures. The mean follow-up time was 31.5 months (range, 12-38 months). In assessing the efficacy of each intervention, forest plots revealed significant absolute reduction in total postoperative TWSTRS scores for both peripheral denervation (standardized mean difference 1.54; 95% CI 1.42-1.66) and DBS (standardized mean difference 2.07; 95% CI 1.43-2.71). On subgroup analysis, DBS therapy was significantly associated with improvement in postoperative TWSTRS severity (standardized mean difference 2.08; 95% CI 1.66-2.50) and disability (standardized mean difference 2.12; 95% CI 1.57-2.68) but not pain (standardized mean difference 1.18; 95% CI 0.80-1.55). Both peripheral denervation and DBS are associated with a significant reduction in absolute TWSTRS total score, with no significant difference in the magnitude of reduction observed between the 2 treatments. Further comparative data are needed to better evaluate the long-term results of both interventions.

Oropharyngeal Dysphagia in Adults With Dyskinetic Cerebral Palsy and Cervical Dystonia: A Preliminary Study

ObjectivesTo investigate the characteristics of oropharyngeal dysphagia in adults with dyskinetic cerebral palsy (DCP) and cervical dystonia (CD). DesignExploratory observational cross-sectional study. SettingUniversity hospital. ParticipantsSeventeen patients with DCP (8 men, 9 women; age, 45.7±6.3y) enrolled in a randomized controlled trial on the effects of botulinum toxin injection on CD. InterventionsNot applicable. Main Outcome MeasuresBaseline clinical assessments and videofluoroscopic swallowing studies (VFSSs) were conducted. VFSS findings were evaluated using the videofluoroscopic dysphagia scale (VDS) and the penetration-aspiration scale (PAS). The Gross Motor Function Classification System (GMFCS) and Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores were also assessed. Relationships between outcomes were evaluated using Spearman’s rank correlation. ResultsThe clinical assessment revealed abnormalities in chewing (n=10, 58.8%), tongue movement (n=10, 58.8%), and laryngeal elevation (n=8, 47.1%). The most common abnormality on the VDS was inadequate mastication (n=13, 76.5%), followed by premature bolus loss, vallecular residue, and penetration/aspiration (all: n=10, 58.8%). A maximum PAS score of 8 was observed in 8 of 17 patients (47.1%). Total and pharyngeal VDS scores were significantly correlated with TWSTRS scores (ρ=0.543, P=.024 and ρ=0.539, P=.026, respectively); the VDS oral score did not correlate with the TWSTRS score (ρ=0.446, P=.073). There was no significant correlation between VDS score and GMFCS level (ρ=0.212, P=.414). ConclusionsThis preliminary observational study presents the characteristics of oropharyngeal dysphagia in adults with DCP and CD. Pharyngeal stage difficulties were negatively correlated with severity of CD, but not with GMFCS level. Screening for dysphagia may be recommended in adults with DCP and severe CD.

Neuropsychological consequences of pallidal deep brain stimulation altering brain networks

The purpose of this study was to evaluate postoperative changes in the neuropsychological function of cervical dystonia patients who had undergone deep brain stimulation (DBS) of the internal segment of the globus pallidus (GPi) and to investigate how DBS affects neuropsychological function by altering the neural networks of the brain. In 12 cervical dystonia patients, the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was used to measure the preoperative and postoperative status of cervical dystonia, and the Seoul Neuropsychological Screening Battery was used to gather neuropsychological data. The data were analyzed using a Wilcoxon signed-rank test. The average improvement in the TWSTRS score at the time of the postoperative neuropsychological battery was 56.1 ± 26.8%. In the neuropsychological battery, inhibitory control, as evaluated by the Stroop test, was significantly decreased after GPi DBS. The average pre-/postoperative Stroop test word and color reading correct response score were 107.9/99.2 (P = 0.043) and 85.3/75.8 (P = 0.032), respectively. The observed neuropsychological consequence of GPi DBS in this study, i.e., decreased inhibitory control, implies that electrical stimulation of the GPi may alter brain networks via the centromedian-parafascicular nuclear complex, suppressing the inhibitory control function of the prefrontal cortex.

Subthalamic nucleus deep brain stimulation in isolated dystonia: A 3-year follow-up study.

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). 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.gov NCT00773604). 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). This study offers support for long-term tolerability and sustained effectiveness of STN DBS in the treatment of severe forms of isolated dystonia. This study provides Class IV evidence that STN DBS decreases long-term dystonia severity in patients with medically refractory isolated dystonia.

Impact of Neu-botulinumtoxinA on the Severity and Quality of Life of Cervical Dystonia Patients

<strong>Background:</strong> Cervical dystonia (CD) is a debilitating neurological disorder that may gravely affect a patient’s quality of life (QoL). Botulinum toxin treatment has been approved as a first-line treatment for this condition. This study aims to look at the efficacy and impact on the QoL of neu-botulinumtoxinA, a newer and cheaper botulinum toxin type A, in patients with CD. <strong>Methods:</strong> This is a prospective, open-label, single-arm study. CD patients were recruited and evaluated for severity of CD using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), and for QoL using the Craniocervical Dystonia Questionnaire (CDQ-24), and the 36-item Short Form Health Survey questionnaire (SF-36) at baseline and 6 weeks after injection. <strong>Results:</strong> Twenty patients were recruited. Significant improvement was shown in part 1 and total TWSTRS score and total CDQ-24 scores. Analysis of individual items of the TWSTRS scale showed significant improvement in rotation, duration of CD, and work ability. Significant improvements in the QoL were also seen in some items of the stigma, emotional wellbeing, and energy/fatigue domains of the CDQ-24 and SF-36 questionnaires. <strong>Discussion:</strong> Neu-botulinumtoxinA is efficacious in treating CD symptoms and improving QoL of patients with CD. A larger, double-blinded study is needed to study the extent of improvements.