Toronto Western Spasmodic Torticollis Rating Scale Scores Research Articles

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

BackgroundCervical 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.MethodsThis 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.ResultsTwenty 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.DiscussionNeu-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.

Reduced Neck Muscle Strength and Altered Muscle Mechanical Properties in Cervical Dystonia Following Botulinum Neurotoxin Injections: A Prospective Study.

ObjectiveTo evaluate changes in the strength and mechanical properties of neck muscles and disability in patients with cervical dystonia (CD) during a 12-week period following botulinum neurotoxin (BoNT) injections.MethodsEight patients with CD volunteered for this prospective clinical cohort study. Patients had received BoNT injections regularly in neck muscles at three-month intervals for several years. Maximal isometric neck strength was measured by a dynamometer, and the mechanical properties of the splenius capitis were evaluated using two myotonometers. Clinical assessment was performed using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) before and at 2, 4, 8, and 12 weeks after the BoNT injections.ResultsMean maximal isometric neck strength at two weeks after the BoNT injections decreased by 28% in extension, 25% in rotation of the affected side and 17% in flexion. At four weeks, muscle stiffness of the affected side decreased by 17% and tension decreased by 6%. At eight weeks, the muscle elasticity on the affected side increased by 12%. At two weeks after the BoNT injections, the TWSTRS-severity and TWSTRS-total scores decreased by 4.3 and 6.4, respectively. The strength, muscle mechanical properties and TWSTRS scores returned to baseline values at 12 weeks.ConclusionsAlthough maximal neck strength and muscle tone decreased after BoNT injections, the disability improved. The changes observed after BoNT injections were temporary and returned to pre-injection levels within twelve weeks. Despite having a possible negative effect on function and decreasing neck strength, the BoNT injections improved the patients reported disability.

Repetitive transcranial magnetic stimulation in cervical dystonia: effect of site and repetition in a randomized pilot trial.

Dystonia is characterized by abnormal posturing due to sustained muscle contraction, which leads to pain and significant disability. New therapeutic targets are needed in this disorder. The objective of this randomized, sham-controlled, blinded exploratory study is to identify a specific motor system target for non-invasive neuromodulation and to evaluate this target in terms of safety and tolerability in the cervical dystonia (CD) population. Eight CD subjects were given 15-minute sessions of low-frequency (0.2 Hz) repetitive transcranial magnetic stimulation (rTMS) over the primary motor cortex (MC), dorsal premotor cortex (dPM), supplementary motor area (SMA), anterior cingulate cortex (ACC) and a sham condition with each session separated by at least two days. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) score was rated in a blinded fashion immediately pre- and post-intervention. Secondary outcomes included physiology and tolerability ratings. The mean change in TWSTRS severity score by site was 0.25 ± 1.7 (ACC), -2.9 ± 3.4 (dPM), -3.0 ± 4.8 (MC), -0.5 ± 1.1 (SHAM), and -1.5 ± 3.2 (SMA) with negative numbers indicating improvement in symptom control. TWSTRS scores decreased from Session 1 (15.1 ± 5.1) to Session 5 (11.0 ± 7.6). The treatment was tolerable and safe. Physiology data were acquired on 6 of 8 subjects and showed no change over time. These results suggest rTMS can modulate CD symptoms. Both dPM and MC are areas to be targeted in further rTMS studies. The improvement in TWSTRS scores over time with multiple rTMS sessions deserves further evaluation.Trial RegistrationClinicalTrials.gov NCT01859247

Selective peripheral denervation for the treatment of spasmodic torticollis: long-term follow-up results from 648 patients.

Selective peripheral denervation (SPD) is currently the primary surgical treatment for spasmodic torticollis (ST). Our objective here is to report on the outcome of patients treated with this procedure for ST in our department. Between June 1995 and June 2013, 648 patients underwent SPD for ST. We included 293 women (45.2 %) and 355 men (54.8 %) with a mean age of 41.1 years (range, 8-74 years) at the onset of dystonia. Surgery was performed at a mean of 3.6 years (range, 1-32 years) after onset of symptoms. Data on clinical presentation, radiological studies, operation tragedy, clinical outcomes and complications were analysed retrospectively. For evaluation of clinical outcomes, patients' responses were assessed using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Results were obtained from all 648 patients with a follow-up period ranging from 11 months to 154 months (mean, 33.4 months). The mean preoperative TWSTRS score was 54.7 ± 18.3 points (range, 39-67 points), which decreased to 31.1 ± 11.6 points postoperatively (range, 1-67 points); a significant improvement was observed between preoperative and postoperative TWSTRS evaluation; the clinical improvement of TWSTRS was 73.5 ± 11.9 %. In addition, no deaths and serious complications occurred in this cohort of patients. SPD is an effective surgical method for patients with ST. This procedure should be recommended if conservative therapy does not offer satisfactory relief of symptoms.

Effectiveness of selective peripheral denervation in combination with pallidal deep brain stimulation for the treatment of cervical dystonia.

Selective peripheral denervation (SPD) and deep brain stimulation of the globus pallidus (GPi-DBS) are available surgical options for patients with medically refractory cervical dystonia (CD). There are few data available concerning whether patients who have unsatisfactory treatment effects after primary surgery benefit from a different type of subsequent surgery. The aim of this study was to assess whether combining these surgical procedures (SPD plus GPi-DBS) was effective in patients with unsatisfactory treatment effects after their initial surgery. Forty-one patients with medically refractory idiopathic CD underwent SPD and/or GPi-DBS. Patients who were dissatisfied with their primary surgery (SPD or GPi DBS) elected to subsequently undergo a different type of surgery. These patients were assessed with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). SPD alone and GPi-DBS alone were performed in 16 and 21 patients, respectively. Four patients had unsatisfactory treatment effects after the initial surgery and subsequently underwent another type of surgery. Among them, two patients with persistent dystonia after SPD subsequently underwent GPi-DBS, and two other patients who had insufficient treatment effects following GPi-DBS were subsequently treated with SPD. All of these patients experienced sustained improvement from the combined surgical procedures according to the TWSTRS score during a long-term follow-up of 12-90 months. Patients with unsatisfactory treatment effects after an SPD or GPi-DBS experienced improvement from subsequently undergoing other types of surgery. Therefore, combined surgical procedures are additional surgical options with good outcomes in the treatment of patients with residual symptoms after their initial surgery.

Assessing the burden of illness from cervical dystonia using the Toronto Western Spasmodic Torticollis Rating Scale scores and health utility: a meta-analysis of baseline patient-level clinical trial data

Purpose:This study aimed to explore the burden of illness associated with cervical dystonia (CD), including possible demographic and humanistic correlates of baseline disease severity.Methods:The analysis involved the five multinational randomized, placebo-controlled clinical trials that had evaluated the efficacy and safety of Dysport® in patients with CD, including assessment using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Patient-level TWSTRS scores from the individual studies were meta-analysed to estimate disease severity at baseline. One of the studies had reported Short Form-36 (SF-36) Health Survey quality-of-life measures, and these data were used to investigate whether the severity of CD was associated with humanistic outcomes, as measured by health utility. A generalized regression model was then applied to explore potential correlation between TWSTRS scores and utilities.Results:The estimated pooled mean baseline severity of CD in clinical trial entrants, as measured by TWSTRS score, was 43.23 (95% CI = 39.31–47.15). In general, disease severity was significantly greater in patients aged over 40 years (compared to the reference group aged 18–30 years). However, there was no correlation between disease severity and other demographic characteristics (e.g., weight, height, gender). Higher TWSTRS scores correlated with worse health-related quality of life as perceived by patients and was reflected in health utility (R2 = 0.133).Conclusions:This study was able to define TWSTRS scores in patients with CD in terms of associated utility. This approach could help in capturing the disease’s burden through measures that are more tangible than TWSTRS scores to patients, carers, clinicians, and healthcare payers.

Microvascular decompression surgery is effective for the laterocollis subtype of spasmodic torticollis: a long-term follow-up result.

Spasmodic torticollis (ST) is characterized by sustained, involuntary, and painful spasms of specific muscle (s), which results into abnormal posture of the neck and head. Although various treatments for ST have been introduced, none of them shows absolute effectiveness. Earlier research from our department showed that microvascular decompression (MVD) surgery is effective in the short-term for ST patients with confirmed accessory nerve compression. However, the long-term outcome of MVD remains unknown. Twelve ST patients with confirmed accessory nerve compression received MVD surgery of their accessory nerves. We utilized the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) to evaluate the long-term outcome (5.4 ± 0.87 years). The MVD lowered total TWSTRS scores by 42.8 % in all ST patients. This result, however, only counted for moderate relief. Interestingly, we observed that the laterocollis (LC) subtypes of ST (n = 3) obtained a higher TWSTRS score improvement (86.9 ± 6.2 %), compared to that of the non-LC (28.1 ± 12 %) (P =0.0001). Additionally, the disability (92.7 ± 2 %) subscale score in the LC subtypes had the most prominent improvement compared to the pain (88.1 ± 5.1 %) and severity (81.3 ± 10.5 %). In the cases of confirmed accessory nerve compression, the MVD could be considered as a treatment alternative for ST, especially for the LC subtypes.

A randomized double-blind crossover trial comparing subthalamic and pallidal deep brain stimulation for dystonia

The authors' aim was to compare the subthalamic nucleus (STN) with the globus pallidus internus (GPi) as a stimulation target for deep brain stimulation (DBS) for medically refractory dystonia. In a prospective double-blind crossover study, electrodes were bilaterally implanted in the STN and GPi of 12 patients with focal, multifocal, or generalized dystonia. Each patient was randomly selected to undergo initial bilateral stimulation of either the STN or the GPi for 6 months, followed by bilateral stimulation of the other nucleus for another 6 months. Preoperative and postoperative ratings were assessed by using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and video recordings. Quality of life was evaluated by using questionnaires (36-item Short Form Health Survey). Supplemental Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores were assessed for patients with focal dystonia (torticollis) by examining the video recordings. On average for all patients, DBS improved the BFMDRS movement scores (p < 0.05) and quality of life physical scores (p < 0.01). After stimulation of the STN, the mean 6-month improvement in BFMDRS movement score was 13.8 points; after stimulation of the GPi, this improvement was 9.1 points (p = 0.08). Quality of life did not differ significantly regardless of which nucleus was stimulated. All 12 patients accepted 6 months of stimulation of the STN, but only 7 accepted 6 months of stimulation of the GPi. Among those who rejected stimulation of the GPi, 3 accepted concomitant stimulation of both the STN and GPi for 6 months, resulting in improved quality of life physical and mental scores and BFMDRS movement scores. Among the 4 patients who were rated according to TWSTRS, after 6 months of stimulation of both the STN and GPi, TWSTRS scores improved by 4.7% after stimulation of the GPi and 50.8% after stimulation of the STN (p = 0.08). The STN seems to be a well-accepted, safe, and promising stimulation target in the treatment of dystonia, but further studies are necessary before the optimal target can be concluded. Simultaneous stimulation of the STN and GPi should be further investigated. Clinical trial registration no.: KF 01-110/01 (Committees on Biomedical Research Ethics of the Capital Region of Denmark).

Long Disease Duration Interferes With Therapeutic Effect of Globus Pallidus Internus Pallidal Stimulation in Primary Cervical Dystonia

We retrospectively investigated the correlation between disease duration and the therapeutic effect of globus pallidus internus (GPi) stimulation in patients with primary cervical dystonia (CD). Eight patients with CD unresponsive to medical treatments underwent bilateral GPi deep brain stimulation (DBS). They were followed for 63.5 ± 38.2 months (mean ± standard deviation) and were assessed before and at 1, 12, 24, and 36 months after surgery and at their final visit to our outpatient clinic using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Univariate analysis was performed to identify factors that affected their postoperative TWSTRS score. At last follow-up, disease severity and the degree of disability and pain on the TWSTRS were significantly improved by 70.2%, 76.1%, and 87.1%, respectively (p < 0.05, Wilcoxon signed-rank test). Neither age nor preoperative CD severity was predictive of postoperative improvement; however, the disease duration affected their reduction rate of TWSTRS severity score at each time point investigated (p < 0.05). Bilateral GPi-DBS is an effective long-term therapy in patients with CD. The delivery of GPi stimulation in the earlier course of CD may yield greater postoperative improvement.

Pallidal Deep Brain Stimulation in Primary Cervical Dystonia with Phasic Type : Clinical Outcome and Postoperative Course

The purpose of this study was to analyze in detail the relationship between outcome and time course of effect in medically refractory primary cervical dystonia (CD) with phasic type that was treated by bilateral globus pallidus internus (Gpi) deep brain stimulation (DBS). Six patients underwent bilateral implantation of DBS into the Gpi under the guide of microelectrode recording and were followed for 18.7 +/- 11.1 months. The mean duration of the CD was 5.8 +/- 3.4 years. The mean age at time of surgery was 54.2 +/- 10.2 years. Patients were evaluated with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and relief scale using patient self-reporting. The TWSTRS total scores improved by 64.5%, 65.5%, 75.8%, and 76.0% at 3, 6, 12 months, and at the last available follow-up after surgery, respectively. Statistically significant improvements in the TWSTRS scores were observed 3 months after surgery (p = 0.028) with gradual improvement up to 12 months after surgery, thereafter, the improvement was sustained. However, there was no statistically significant difference between the scores at 3 and 12 months. Subjective improvement reported averaged 81.7 +/- 6.8% at last follow-up. Mild dysarthria, the most frequent adverse event, occurred in 3 patients. Our results show that the bilateral Gpi-DBS can offer a significant therapeutic effect from 3 months postoperatively in patients with primary CD with phasic type, without significant side effects.

Efficacy of Dysport ® (Botulinum toxin type A) for the treatment of cervical dystonia: A multicenter, randomized, double-blind, placebo-controlled phase III study

Single, intramuscular (IM) doses of Dysport were used in the treatment of cervical dystonia (CD). Dysport 500 unit (U) or placebo was used, with injection sites and dose decided at baseline. Efficacy assessments include Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) total and subscale scores, visual analog scale (VAS) for pain, subject/investigator's VAS for symptom assessments, investigator's global assessment of pain and symptoms at week 12 and SF-36 QOL scores at week 8. Mean TWSTRS scores improved markedly for Dysport compared to placebo at week 4 and significantly at weeks 8 and 12. Adjusted least squares mean (SE) TWSTRS total score improved by 15.6 Dysport vs. 6.7 placebo. TWSTRS total score maintained continued improvement at weeks 8 (14.7 vs. 5.9) and 12 (9.1 vs. 4.9) for Dysport and placebo, respectively. VAS for pain was always lower with Dysport, with greater reduction in VAS pain score at weeks 4 and 8 (−17.7±24.4 and −15.8±30.9, respectively) and less at week 12 (−7.9±27.5), similar trends are seen for TWSTRS pain subscale scores. For Dysport, SF-36 summary score at week 8 and proportion of treatment successes at week 12 were also significantly better compared to placebo. Most adverse events were mild or moderate in nature and typical of the known safety profile of Dysport in CD. The study demonstrates the superior efficacy and benefit, compared to placebo, of a single, IM 500 U Dysport dose in the treatment of CD. Supported by Ipsen Ltd.

Subthalamic nucleus deep brain stimulation for severe idiopathic dystonia: impact on severity, neuropsychological status, and quality of life

Medically refractory dystonia has recently been treated using deep brain stimulation (DBS) targeting the globus pallidus internus (GPI). Outcomes have varied depending on the features of the dystonia. There has been limited literature regarding outcomes for refractory dystonia following DBS of the subthalamic nucleus (STN). Four patients with medically refractory, predominantly cervical dystonia underwent STN DBS. Intraoperative assessments with the patients in a state of general anesthesia were performed to determine the extent of fixed deformities that might predict outcome. Patients were rated using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) preoperatively and 3 and 12 months following surgery by a rater blinded to the study. Mean changes and standard errors of the mean in scores were calculated for each subscore of the two scales. Scores were also analyzed using analysis of variance and probability values were generated. Neuropsychological assessments and quality of life ratings using the 36-Item Short Form Health Survey (SF-36) were evaluated longitudinally. Significant improvements were seen in motor (p = 0.04), disability (p = 0.02), and total TWSTRS scores (p = 0.03). Better outcomes were seen in those patients who did not have fixed deformities. There was marked improvement in the mental component score of the SF-36. Neuropsychological function was not definitively impacted as a result of the surgery. Deep brain stimulation of the STN is a novel target for dystonia and may be an alternative to GPI DBS. Further studies need to be performed to confirm these conclusions and to determine optimal candidates and stimulation parameters.

Comparison of botulinum toxin serotypes A and B for the treatment of cervical dystonia

To directly compare two serotypes of botulinum toxin (BoNTA and BoNTB) in cervical dystonia (CD) using a randomized, double-blind, parallel-arm study design. Subjects with CD who had a previous response from BoNTA were randomly assigned to BoNTA or BoNTB and evaluated in a blinded fashion at baseline, 4 weeks, 8 weeks, and 2-week intervals thereafter until loss of 80% of clinical effect or completion of 20 weeks of observation. CD severity was measured with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), and adverse events were assessed by structured interview. Statistical analysis included Wilcoxon rank sum test, log rank tests, and Kaplan-Meier survival curves for duration of effect. A total of 139 subjects (BoNTA, n = 74; BoNTB, n = 65) were randomized at 19 study sites. Improvement in TWSTRS score was found at 4 weeks after injection and did not differ between serotypes. Dysphagia and dry mouth were more frequent with BoNTB (dysphagia: BoNTA 19% vs BoNTB 48%, p = 0.0005; dry mouth (BoNTA 41% vs BoNTB 80%, p < 0.0001). In clinical responders, BoNT A had a modestly longer duration of benefit (BoNTA 14 weeks, BoNTB 12.1 weeks, p = 0.033). Both serotypes of botulinum toxin (BoNTA and BoNTB) had equivalent benefit in subjects with cervical dystonia at 4 weeks. BoNTA had fewer adverse events and a marginally longer duration of effect in subjects showing a clinical response.

Treatment of cervical dystonia and focal hand dystonia by high cervical continuously infused intrathecal baclofen: A report of 2 cases

Dykstra DD, Mendez A, Chappuis D, Baxter T, DesLauriers L, Stuckey M. Treatment of cervical dystonia and focal hand dystonia by high cervical continuously infused intrathecal baclofen: a report of 2 cases. Arch Phys Med Rehabil 2005;86:830–3. We describe 2 patients, one with cervical dystonia (CD) combined with focal hand dystonia (writer’s cramp) and another with idiopathic CD, who were unresponsive to oral medications and became resistant to botulinum toxin type A and B injections. Both patients were successfully treated with high cervical (C1–3) continuously infused intrathecal baclofen (ITB). Neck range of motion (ROM) was measured by using a 3-dimensional electromagnetic cervical ROM system. Pain, disability, and severity were assessed by using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). The patient with CD and writer’s cramp did well on a continuous baclofen dose of 186.1μg/d. Her total TWSTRS score improved significantly, her electromagnetic measurements showed an increased in total neck flexion and extension, and her handwriting improved. Unfortunately, this patient (a heavy smoker) developed small cell carcinoma of the lung and died 9 months after her pump was placed. Total TWSTRS score and electromagnetic measurements also significantly improved after pump implant in the patient with CD. He continues to do well on a periodic bolus dose using a combination of 50μg of baclofen and 25μg of hydromorphone (Dilaudid) every 4 hours. Our findings suggest the potential usefulness of this therapy in other patients with focal dystonia. To our knowledge, this is the first reported successful treatment of CD and CD combined with writer’s cramp with high cervical continuously infused ITB.

The Canadian multicenter trial of pallidal deep brain stimulation for cervical dystonia: preliminary results in three patients

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. Three patients have undergone surgery in Calgary with a follow-up 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 t-test, p = 0.02). Pain and disability subscores improved from 25.5 +/- 4.1 to 3.3 +/- 3.1 (paired t-test, p = 0.002) and from 13.3 +/- 4.9 to 3.3 +/- 4.2 (paired t-test, p = 0.06), respectively. 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.