Toronto Western Spasmodic Torticollis Rating Scale Research Articles

The course of cervical dystonia with head tremor during botulinum toxin type A treatment

Objective : to evaluate the efficacy and safety of botulinum toxin type A (BTA) injections into the neck muscles to reduce dystonic postures, head tremor, and pain syndrome in patients with cervical dystonia (CD) within the first 8 cycles of treatment. Patients and methods. The investigation included 76 patients (26 (34%) men and 50 (66%) women) with CD and dystonic head tremor, who were given BTA injections into the neck muscles for the first time. All the 76 patients received at least one cycle of BTA therapy. At the same type, 18 of these patients received 4 cycles of injections and 36 patients had 8 cycles. Injections were given when the symptoms of CD recurred or increased and the patient needed to be retreated. The interval between the injection cycles was arbitrary, but not less than 12 weeks. The doses of BTA agents per treatment cycle were as follows: Dysport was 400 to 1000 U, xeomin was 50 to 300 U, and Botox 200 to 300 U. The symptoms of CD were assessed using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and the Tsui scale before the first injection of BTA and injection cycles 4 and 8; the presence or absence of head tremor was recorded. Results. The TWSTRS severity of CD symptoms decreased from 38 [36; 41] to 30 [27; 33] scores by injection cycle 4 (p < 0.001) and to 26 [23; 27] scores by cycle 8 (p<0.01). The Tsui severity of CD reduced from 9.3 [9; 10] to 7.2 [7; 8] scores by injection cycle 4 (p<0.001) and to 6.7 [6; 7] scores by cycle 8. The Tsui tremor scores decreased from 1.9 [1.6; 2.1] to 1.4 [1.1; 1.6] scores by injection cycle 4 and to 1.1 [0.9;1.4] scores by cycle 8 (p<0.01). Tremor completely disappeared in 6 (11%) of patients by injection cycle 4 and in 6 (18%) patients by cycle 8. According to Section 3 of the TWSTRS, pain intensity was reduced from 9.9 [8.9; 11.0] to 5.0 [3.3; 6.6] scores by injection cycle 4 (p<0.001) and to 2.1 [0.7; 3.6] scores by cycle 8 (p < 0.01); pain regressed completely in 12 (41%) and 9 (64%) patients by cycles 4 and 8, respectively. Conclusion. BTA injections into the neck muscles are an effective treatment for CD symptoms, such as dystonic tremor, posture, and pain. Successive injection cycles have a cumulative effect that is more marked by injection cycle 8. It is appropriate to prescribe longer treatment cycles to patients with CD and head tremor. Dystonic head tremor, unlike dystonic postures, may regress fully after BTA injections

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.

Botulinum toxin type A versus botulinum toxin type B for cervical dystonia.

This is an update of a Cochrane review first published in 2003. Cervical dystonia is the most common form of focal dystonia and is a disabling disorder characterised by painful involuntary head posturing. There are two available formulations of botulinum toxin, with botulinum toxin type A (BtA) usually considered the first line therapy for this condition. Botulinum toxin type B (BtB) is an alternative option, with no compelling theoretical reason why it might not be as- or even more effective - than BtA. To compare the efficacy, safety and tolerability of botulinum toxin type A (BtA) versus botulinum toxin type B (BtB) in people with cervical dystonia. To identify studies for this review we searched the Cochrane Movement Disorders Group Trials Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, Embase, reference lists of articles and conference proceedings. All elements of the search, with no language restrictions, were last run in October 2016. Double-blind, parallel, randomised, placebo-controlled trials (RCTs) comparing BtA versus BtB in adults with cervical dystonia. Two independent authors assessed records, selected included studies, extracted data using a paper pro forma, and evaluated the risk of bias. We resolved disagreements by consensus or by consulting a third author. We performed meta-analyses using the random-effects model, for the comparison BtA versus BtB to estimate pooled effects and corresponding 95% confidence intervals (95% CI). No prespecified subgroup analyses were carried out. The primary efficacy outcome was improvement on any validated symptomatic rating scale, and the primary safety outcome was the proportion of participants with adverse events. We included three RCTs, all new to this update, of very low to low methodological quality, with a total of 270 participants.Two studies exclusively enrolled participants with a known positive response to BtA treatment. This raises concerns of population enrichment, with a higher probability of benefit from BtA treatment. None of the trials were free of for-profit bias, nor did they provide information regarding registered study protocols. All trials evaluated the effect of a single Bt treatment session, and not repeated treatment sessions, using doses from 100 U to 250 U of BtA (all onabotulinumtoxinA, or Botox, formulations) and 5000 U to 10,000 U of BtB (rimabotulinumtoxinB, or Myobloc/Neurobloc).We found no difference between the two types of botulinum toxin in terms of overall efficacy, with a mean difference of -1.44 (95% CI -3.58 to 0.70) points lower on the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) for BtB-treated participants, measured at two to four weeks after injection. The proportion of participants with adverse events was also not different between BtA and BtB (BtB versus BtA risk ratio (RR) 1.40; 95% CI 1.00 to 1.96). However, when compared to BtA, treatment with BtB was associated with an increased risk of one adverse events of special interest, namely treatment-related sore throat/dry mouth (BtB versus BtA RR of 4.39; 95% CI 2.43 to 7.91). Treatment-related dysphagia (swallowing difficulties) was not different between BtA and BtB (RR 2.89; 95% CI 0.80 to 10.41). The two types of botulinum toxin were otherwise clinically non-distinguishable in all the remaining outcomes. The previous version of this review did not include any trials, since these were still ongoing at the time. Therefore, with this update we are able to change the conclusions of this review. There is low quality evidence that a single treatment session of BtA (specifically onabotulinumtoxinA) and a single treatment session of BtB (rimabotulinumtoxinB) are equally effective and safe in the treatment of adults with certain types of cervical dystonia. Treatment with BtB appears to present an increased risk of sore throat/dry mouth, compared to BtA. Overall, there is no clinical evidence from these single-treatment trials to support or contest the preferential use of one form of botulinum toxin over the other.

SP 2. Pallidal low frequency activity correlates with dystonic symptoms in patients with cervical dystonia

Question Low frequency (4–12 Hz) oscillations in the internal pallidum (GPi) have been shown to be correlated and coherent with dystonic EMG activity and have therefore been proposed as a pathophysiological signature in patients with dystonia. The aim of this study was to investigate the potential association between pallidal 4–12 Hz activity and symptom severity as quantified by the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) in patients with cervical dystonia. Methods 27 patients undergoing functional neurosurgery for therapeutic deep brain stimulation targeting the GPi were included in our study. Pallidal local field potentials were recorded bilaterally at rest from adjacent contact pairs (01, 12, 23) of the externalized leads before implantation of the impulse generator. All time-series data were taken to the frequency domain using fourier transform based methods. Resulting power spectra were normalized and visually inspected for peaks in the low-frequency ( θ : 4–12 Hz) and beta band ( β : 13–30 Hz). Additionally, inter-hemispheric coherence as a measure of spatial oscillatory connectivity has been analysed. Spearman’s correlations have been conducted to investigate a potential association between coherence and peak amplitude and preoperative dystonic symptom severity (TWSTRS) for each frequency band. Results All patients had distinct peaks in the low frequency band (4–12 Hz, mean: 6.6 Hz ± 0.26 S.E.M.) and in the beta band (13–30 Hz, mean: 17 Hz ± 0.68 S.E.M.). Both, averaged low frequency peak amplitude across available contact pairs and averaged inter-hemispheric low frequency band coherence correlated significantly with the patients’ dystonic symptom severity, as measured by preoperative TWSTRS (N = 27, Spearman’s ρ = 0.39, P = 0.02 and Spearman’s ρ = 0.54, P = 0.002 for peak amplitude and coherence respectively) and the correlation got better when only the highest peak amplitude was chosen in each electrode (N = 27, Spearman’s ρ = 0.47, P = 0.006). None of the correlations were significant for the beta band peak amplitudes, nor the beta band coherence. Conclusions The results of our study suggest that pallidal low frequency oscillations have a functional role in the pathophysiology of dystonia and may be a biomarker for dystonic symptom severity, which could potentially be used for adaptive deep brain stimulation.

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.

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.

Cervical dystonia and quality of life.

Cervical dystonia is focal dystonia characterized by involuntary movement of the neck muscle, which leads to abnormal head posture. It can be accompanied with pain and tremor. In this study, we evaluated the presence of depression and anxiety in patients with cervical dystonia and the influence of dystonia symptoms on the quality of life. Psychiatric symptoms were evaluated by use of the Beck Depression Inventory (BDI-II) and Beck Anxiety Inventory. Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was used to evaluate the cervical dystonia symptoms. Quality of life was assessed by the craniocervical dystonia questionnaire (CDQ-24) and short form 36 health survey (SF-36). Nineteen patients were analyzed. Most of the patients had mild cervical dystonia (mean TWSTRS 23.89). Depression was present in 42.1% and anxiety in 57.9% of the patients. Disability due to cervical dystonia correlated with the occurrence of depression (ρ=0.534) and anxiety (r=0.652). Disability was found to significantly influence the stigma, emotional state, pain, daily activity, social life, physical function, and physical and mental disability. Pain influenced some aspects of body pain, physical function, and physical and mental disability. Being associated with disability and pain, cervical dystonia decreases the quality of life in many aspects. Disability also influenced depression and anxiety, which were present in half of study patients. In addition to follow up for cervical dystonia symptoms, patients with cervical dystonia should also be assessed for psychiatric symptoms on routine clinical check-ups. In addition to botulinum toxin, psychopharmaceuticals should be considered as a treatment option in these patients.

A mixed treatment comparison to compare the efficacy and safety of botulinum toxin treatments for cervical dystonia.

A systematic pair-wise comparison of all available botulinum toxin serotype A and B treatments for cervical dystonia (CD) was conducted, as direct head-to-head clinical trial comparisons are lacking. Five botulinum toxin products: Dysport® (abobotulinumtoxinA), Botox® (onabotulinumtoxinA), Xeomin® (incobotulinumtoxinA), Prosigne® (Chinese botulinum toxin serotype A) and Myobloc® (rimabotulinumtoxinB) have demonstrated efficacy for managing CD. A pair-wise efficacy and safety comparison was performed for all toxins based on literature-reported clinical outcomes. Multi-armed randomized controlled trials (RCTs) were identified for inclusion using a systematic literature review, and assessed for comparability based on patient population and efficacy outcome measures. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was selected as the efficacy outcome measurement for assessment. A mixed treatment comparison (MTC) was conducted using a Bayesian hierarchical model allowing indirect comparison of the interventions. Due to the limitation of available clinical data, this study only investigated the main effect of toxin treatments without explicitly considering potential confounding factors such as gender and formulation differences. There was reasonable agreement between the number of unconstrained data points, residual deviance and pair-wise results. This research suggests that all botulinum toxin serotype A and serotype B treatments were effective compared to placebo in treating CD, with the exception of Prosigne. Based on this MTC analysis, there is no significant efficacy difference between Dysport, Botox, Xeomin and Myobloc at week four post injection. Of the adverse events measured, neither dysphagia nor injection site pain was significantly greater in the treatment or placebo groups.

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.

Poster 303 Correlation between Tsui Tremor Scores and the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) in Clinical Practice

Poster 303 Correlation between Tsui Tremor Scores and the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) in Clinical Practice

Multimodal Physiotherapy as an Add-On Treatment to Botulinum Neurotoxin A-Therapy in Patients with Cervical Dystonia

<b>Background:</b> The treatment of choice for cervical dystonia comprises a local injection of botulinum neurotoxin type A (BoNT-A). Whether additional physiotherapy can achieve better results is not clearly documented in the literature. The present study aims to show possible effects of physiotherapy as an add-on treatment to therapy with botulinum neurotoxin type A in terms of pain, severity of dystonia, statics and function of the cervical spine as well as the quality of life in order to optimize the treatment of cervical dystonia. <b>Design/Methods:</b> This is a prospective, single-center, interventional study of a target patient population comprising 20 participants. The study is divided into 2 phases, with each phase lasting for 3 months. During the first phase, the usual BoNT-A will be applied. In addition, the baseline values will be collected and these assessments will be repeated at an interval of 6 weeks. In the second phase, participants will undergo a physiotherapy program consisting of two 45 min sessions per week in addition to the BoNT-A treatment. The assessments will be undertaken at the same intervals as in the first phase. <b>Analysis:</b> In order to determine the effect of the add-on physiotherapy program, the following parameters will be measured for each assessment: quality of life using the SF- 36 score and the range of motion determined by the ZEBRIS measurement system which will comprise the primary endpoints. Furthermore, for each assessment the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and electrical activity from the trapezius and the sternocleidomastoid muscles measured by a surface electromyography will be recorded. Descriptive statistical analysis will be performed for all of the collected data. Possible changes in the measured values will be compared to the baseline values over the 2 periods by means of the Wilcoxon-test at a significance level of p<0.05. For the statistical analysis the software SPSS 22.0 will be used. <b>Trial registration:</b> Deutsches Register Klinischer Studien: DRKS00006562

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.

Globus pallidus deep brain stimulation for adult-onset axial dystonia.

Generalized dystonia, both primary and secondary forms, and axial dystonias such as tardive dystonia, and idiopathic cervical dystonia are responsive to globus pallidus interna (GPi) DBS. There is a paucity of investigations probing the impact of DBS on adult-onset axial dystonia. We assessed the efficacy of GPi DBS in four patients with rare adult-onset axial dystonia. Primary outcome measure was improvement in the motor component of the Burke-Fahn-Marsden (BFM) rating scale. Secondary outcome measures were quality of life as determined by the SF-36 questionnaire, time to achieve best possible benefit and DBS parameters that accounted for the best response. In patients with prominent concomitant cervical dystonia we also used the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). GPi DBS improved BFM scores by 87.63±11.46%. Improvement in total severity scale of TWSTRS was 71.5±12.7%. Quality of life also remarkably improved as evidenced by 109.38±82.97 and 7.05±21.48% percent change in psychometrically-based physical component summary (PCS), and a mental component summary (MCS) score respectively. GPi DBS is a very effective treatment for adult-onset axial dystonia. Considering its refractoriness to medical therapy and significant impact on quality of life DBS should be considered for this disorder.

Should We Consider Deep Brain Stimulation for Cervical Dystonia

A growing number of cases of cervical dystonia resistant to medications and botulinum toxin are presenting to clinicians for advanced management. This study was a randomized, sham-controlled, manufacturer-funded trial of bilateral globus pallidus deep brain stimulation (DBS) in this population (an investigational use not specifically approved by the FDA). Participants had to have at least a 3-year disease duration and a Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) severity score of ≥15 and be free …

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.

The physical, social and emotional aspects are the most affected in the quality of life of the patients with cervical dystonia.

Describe the functional, clinical and quality of life (QoL) profiles in patients with cervical dystonia (CD) with residual effect or without effect of botulinum toxin (BTX), as well as verify the existence of correlation between the level of motor impairment, pain and QoL. Seventy patients were assessed through the Craniocervical dystonia questionnaire-24 (CDQ-24) and the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). The greater the disability, pain and severity of dystonia, the worse the QoL (p<0.0001). Greater severity relates to greater disability (p<0.0001). Pain was present in 84% of the sample, being source of disability in 41%. The most frequent complaints were: difficulty in keeping up with professional and personal demands (74.3%), feeling uneasy in public (72.9%), hindered by pain (68.6%), depressed, annoyed or bitter (47.1%), lonely or isolated (32.9%). The physical, social and emotional aspects are the most affected in the QoL of these patients.

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.

Effects of two weeks of cerebellar theta burst stimulation in cervical dystonia patients.

Dystonia is generally regarded as a disorder of the basal ganglia and their efferent connections to the thalamus and brainstem, but an important role of cerebellar-thalamo-cortical (CTC) circuits in the pathophysiology of dystonia has been invoked. Here in a sham controlled trial, we tested the effects of two-weeks of cerebellar continuous theta burst stimulation (cTBS) in a sample of cervical dystonia (CD) patients. Clinical evaluations were performed by administering the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). We used TMS to measure the inhibitory connectivity between the cerebellum and the contralateral motor cortex (cerebellar brain inhibition [CBI]), and the excitability of the contralateral primary motor cortex assessing intracortical inhibition (SICI), intracortical facilitation (ICF) and cortical silent period (CSP). Paired associative stimulation (PAS) was tested to evaluate the level and the topographical specificity of cortical plasticity, which is abnormally enhanced and non-focal in CD patients. Two weeks of cerebellar stimulation resulted in a small but significant clinical improvement as measured by the TWSTRS of approximately 15%. Cerebellar stimulation modified the CBI circuits and reduced the heterotopic PAS potentiation, leading to a normal pattern of topographic specific induced plasticity. These data provide novel evidence CTC circuits could be a potential target to partially control some dystonic symptoms in patients with cervical dystonia.