Toronto Western Spasmodic Torticollis Rating Scale Research Articles

An Open-Label Phase 2a Study to Evaluate the Safety and Tolerability of Perampanel in Cervical Dystonia.

Cervical dystonia (CD) is the most common focal isolated dystonia. Preclinical studies report that AMPA-selective glutamate receptor antagonists improve dystonia. Perampanel is a clinically available, AMPA receptor antagonist that has shown efficacy and safety in epilepsy. To determine safety and tolerability of perampanel in CD. We performed a phase 2a, open-label, multicenter study to evaluate tolerability and safety of perampanel in CD. Included subjects had primary CD; those on botulinum toxin were 8 weeks post last injection. All subjects received perampanel 2 mg/day, titrated 2 mg weekly over 6 weeks, to maximum 12 mg/day; maintenance phase was 4 weeks, ending at week 10. Primary endpoints included tolerability, defined as ability to remain on perampanel for the maintenance period, at any dose level and safety, determined from adverse events (AEs) collected at each visit. Secondary exploratory endpoints included Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), quality of life (cervical dystonia impact profile [CIDP]-58) and Clinical Global Impression of change (CGI). CD participants (n=25) were recruited. Eight subjects withdrew; 4 because of AEs, 3 for other reasons and 1 lost to follow up. One subject tolerated 12 mg/day. Eight subjects (30.8%) tolerated 2 mg, whereas 19.2% tolerated 4 mg/day, and 15.4% tolerated 6 mg or 8 mg/day. All subjects experienced AEs. The most common AEs were dizziness, imbalance, and irritability. Exploratory endpoints of TWSTRS showed some improved pain scores and CIDP-58 improved sleep. Tolerability to perampanel was variable in CD subjects. Lower doses would be considered for future studies in this population.

Botulinum toxin type A versus anticholinergics for cervical dystonia.

This is an update of a Cochrane Review first published in 2005. Cervical dystonia is the most common form of focal dystonia and is a highly disabling movement disorder, characterised by involuntary, usually painful, head posturing. Currently, botulinum toxin type A (BtA) is considered the first line therapy for this condition. Before BtA, anticholinergics were the most widely accepted treatment. To compare the efficacy, safety, and tolerability of BtA versus anticholinergic drugs in adults with cervical dystonia. We searched the Cochrane Movement Disorders' Trials Register to June 2003, screened reference lists of articles and conference proceedings to September 2018, and searched CENTRAL, MEDLINE, and Embase, with no language restrictions, to July 2020. Double-blind, parallel, randomised trials (RCTs) of BtA versus anticholinergic drugs in adults with cervical dystonia. Two review authors independently assessed records, selected included studies, extracted data using a paper pro forma, and evaluated the risk of bias and quality of the evidence. We resolved disagreements by consensus or by consulting a third review author. If enough data had been available, we were to perform meta-analyses using a random-effects model for the comparison of BtA versus anticholinergic drugs to estimate pooled effects and corresponding 95% confidence intervals (95% CI). The primary efficacy outcome was improvement in cervical dystonia-specific impairment. The primary safety outcome was the proportion of participants with any adverse event. We included one RCT of moderate overall risk of bias (as multiple domains were at unclear risk of bias), which included 66 BtA-naive participants with cervical dystonia. Two doses of BtA (Dysport; week 0 and 8; mean dose 262 to 292 U) were compared with daily trihexyphenidyl (up to 24 mg daily). The trial was sponsored by the BtA producer. BtA reduced cervical dystonia severity by an average of 2.5 points (95% CI 0.68 to 4.32) on the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) severity subscale 12 weeks after injection, compared to trihexyphenidyl. More participants reported adverse events in the trihexyphenidyl treatment group (76 events), compared with the BtA group (31 events); however, the difference in dropouts due to adverse events was inconclusive between groups. There was a decreased risk of dry mouth, and memory problems with BtA, but the differences were inconclusive between groups for the other reported side effects (blurred vision, dizziness, depression, fatigue, pain at injection site, dysphagia, and neck weakness). We found very low-certainty evidence that BtA is more effective, better tolerated, and safer than trihexyphenidyl. We found no information on a dose-response relationship with BtA, differences between BtA formulations or different anticholinergics, the utility of electromyography-guided injections, or the duration of treatment effect.

Reappearance of Symptoms after GPi-DBS Discontinuation in Cervical Dystonia.

ABSTRACTBackgroundDeep brain stimulation of the globus pallidus interna (GPi‐DBS) is a highly efficacious treatment for cervical dystonia. Typically, the treatment response is delayed, appearing and increasing even months after implantation. However, it is not known how fast the symptoms reappear and whether there is a long‐term therapeutic effect after the stimulation is discontinued.ObjectivesTo study symptom reappearance after switching GPi‐DBS off in cervical dystonia.MethodsTwelve patients with bilateral GPi‐DBS were included in the study. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was evaluated during the study with DBS stimulation on, after switching the stimulation off and 2 days after the stimulation was switched off. Presurgical symptom severity and best postsurgical response were extracted from the hospital records.ResultsAt the time of the investigation, GPi‐DBS was associated with 67 (SD 39)% symptom improvement of presurgical symptoms severity (P = 0.001). Symptom improvement decreased to 27 (53)% (P = 0.046) (n = 12) acutely after switching the stimulation off and was further reduced to 4 (56)% 2 days after discontinuation (P = 0.01) (n = 11), reaching the presurgical level (P = 0.42). In descriptive analyses, older age was associated with faster worsening of symptoms (P < 0.05). Presurgical symptoms severity, stimulation parameters or magnitude of treatment response did not predict symptom worsening. All but one patient tolerated 2 days DBS switched off.ConclusionsThe results provide novel information about the time frame and severity of symptom worsening after discontinuing GPi‐DBS in cervical dystonia. Symptoms partially reappear immediately after discontinuing GPi‐DBS and full presurgical symptom severity is reached within 2 days.

Clinical Improvement After Treatment With IncobotulinumtoxinA (XEOMIN®) in Patients With Cervical Dystonia Resistant to Botulinum Toxin Preparations Containing Complexing Proteins.

This study investigated the clinical long-term effect of incobotulinumtoxinA (incoBoNT/A) in 33 cervical dystonia (CD) patients who had developed partial secondary therapy failure (PSTF) under previous long-term botulinum toxin (BoNT) treatment. Patients were treated four times every 12 weeks with incoBoNT/A injections. Physicians assessed treatment efficacy using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) at the baseline visit, week 12 and 48. Patients rated quality of life of CD with the Craniocervical Dystonia Questionnaire (CDQ-24). Titres of neutralizing antibodies(NAB) were determined at start of the study and after 48 weeks. All patients had experienced significant and progressive worsening of symptoms in the last 6 months of previous BoNT treatment. Repeated incoBoNT/A injections resulted in a significant reduction in mean TWSTRS at week 12 and 48. Patients' rating of quality of life was highly correlated with TWSTRS but did not change significantly over 48 weeks. During the 48 weeks -period of incoBoNT/A treatment NAB titres decreased in 32.2%, did not change in 45.2%, and only increased in 22.6% of the patients. Thus, repeated treatment with the low dose of 200 MU incoBoNT/A over 48 weeks provided a beneficial clinical long-term effect in PSTF and did not booster titres of NAB.

Isolated Cervical Dystonia: Management and Barriers to Care.

Background: Cervical dystonia (CD) is a rare disorder, and health care providers might be unfamiliar with its presentation, thus leading to delay in the initial diagnosis. The lack of awareness displays the need to highlight the clinical features and treatment in cervical dystonia. In our cohort, we have identified an earlier age of onset in men, despite an overall preponderance of affected women.Objective: We aim to identify the prevalence, age of onset, spread, and treatment modalities of CD in the population. We also highlight the barriers which patients encounter related to diagnosis, follow-up, and treatment.Methods: We reviewed 149 CD patients who attended specialized Dystonia Clinics over a 14-year period. Dystonia severity was rated using the Burke-Fahn-Marsden (BFM), Tsui, and Toronto Western Spasmodic Torticollis Rating Scales (TWSTRS). Mood and quality of life were assessed using Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), and 36-Item Short Form Health Survey (SF-36).Results: CD patients were majority White (91.3%) and more commonly female (75.8%). Men had an earlier median age of onset, 40.5 years (p = 0.044). BAI revealed a mean score of 7.2 (±6.4, n = 50) indicating minimal anxiety while BDI revealed a mean score of 7.30 (±7.6, n = 50) indicating minimal depression. The only SF-36 subscales associated with CD severity were physical functioning (p = 0.040) pain (p = 0.008) and general health (p = 0.014).Conclusion: There appear to be gender differences in both the prevalence and age of onset of the disease. There was a 3-fold higher incidence in women than in men. CD patients of both sexes experience barriers to care, which can be reflected in their quality of life and time-to-diagnosis. In addition, males were less likely to experience an objective benefit with botulinum toxin treatment and more likely to discontinue care. Greater awareness of CD by health care providers is important to reduce the time-to-diagnosis.

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.

AbobotulinumtoxinA using 2-mL dilution (500\u2009U/2-mL) maintains durable improvement across multiple treatment cycles

BackgroundCervical dystonia (CD), the most common focal dystonia, is a chronic neurological movement disorder characterized by sustained involuntary contractions of the neck muscles, leading to abnormal postures. AbobotulinumtoxinA (aboBoNT-A) was approved in the US initially as a 500 U per 1-mL dilution and subsequently, as a 500 U/2-mL dilution (or 250 U/mL), thereby providing clinicians with more flexible dosing options to better meet individual patient needs. The objective of this open-label extension study was to evaluate the longer term safety and efficacy of repeat treatments with aboBoNT-A using 2-mL dilutions in adults with cervical dystonia.MethodsPatients (N = 112) from a 12-week, double-blind lead-in study (NCT01753310) received up to three additional treatments of aboBoNT-A, with re-treatment every 12–16 weeks based on clinical judgment. Safety was assessed through treatment-emergent adverse events (TEAEs). The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) total and subscale scores were measured at day 1 of each treatment cycle (C), 4 weeks after each treatment, and 12 weeks after the third treatment. Descriptive statistics were used for all analyses.ResultsIn cycles 1, 2, 3, and 4, respectively, 35.7, 25.9, 30.2, and 22.8% of patients reported TEAEs. Dysphagia, muscular weakness, and neck pain were each reported by 10.7% of patients, over the full study duration. Mean TWSTRS total score decreased from 37.7 (SD 13.6 [C1, day 1]) to 30.1 (SD 12.8 [C3, week 12]). In each cycle, TWSTRS total and subscale scores decreased from day 1 to week 4 and increased between weeks 4 and 12, though the week 12 scores remained lower than day 1 scores.ConclusionExtended treatment of cervical dystonia with aboBoNT-A (up to 3 additional treatment cycles) using a 2-mL dilution is effective, with a positive risk-benefit profile.Trial registrationClinicalTrials.gov Identifier: NCT01753336. Registered 17 Dec 2012.

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.

Unilateral pallidotomy as a potential rescue therapy for cervical dystonia after unsatisfactory selective peripheral denervation.

Selective peripheral denervation (SPD) is a widely accepted surgery for medically refractory cervical dystonia (CD), but when SPD has failed, the available approaches are limited. The authors investigated the results from a cohort of CD patients treated with unilateral pallidotomy after unsatisfactory SPD. The authors retrospectively analyzed patients with primary CD who underwent unilateral pallidotomy after SPD between April 2007 and August 2019. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was used to evaluate symptom severity before surgery, 7 days postsurgery, 3 months postsurgery, and at the last follow-up. TWSTRS subscores for disability and pain and the 24-item Craniocervical Dystonia Questionnaire (CDQ-24) were used to assess quality of life. At a mean final follow-up of 5 years, TWSTRS severity subscores and total scores were significantly improved (n = 12, mean improvement 57.3% and 62.3%, respectively, p = 0.0022 and p = 0.0022), and 8 of 12 patients (66.7%) were characterized as responders (improvement ≥ 25%). Patients with rotation symptoms before pallidotomy showed greater improvement in TWSTRS severity subscores than those who did not (p = 0.049). The most common adverse event was mild upper-limb weakness (n = 3). Patients' quality of life was also improved. Unilateral pallidotomy seems to offer an effective and safe option for patients with CD who have otherwise experienced limited benefits from SPD.

Predictive factors for outcome of pallidal deep brain stimulation in cervical dystonia

ObjectivePrimary cervical dystonia (CD) is characterized by abnormal contractions of neck muscles. Globus pallidus internus deep brain stimulation (GPi-DBS) is recognized as an effective therapy for patients with refractory CD, but the prognostic factors need further research. Our study investigated the predictive factors of clinical outcomes in CD patients who underwent GPi-DBS. Patients and methodsPatients (n = 23) who underwent GPi-DBS at Chinese PLA General Hospital from March 2012 to April 2018 were included in our analysis. Their scores of Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and Tsui were acquired at baseline and at the last follow-up visit. Percent improvement in these scores were compared between the categorical variables. Correlations between outcomes and continuous demographic and clinical variables were calculated. ResultsPatients showed significant improvement in TWSTRS total (55.71 %), severity (48.75 %), disability (57.05 %), pain (63.67 %) scores and total Tsui score (46.07 %, all P ﹤0.001). Follow-up duration was positively correlated with percent improvement in TWSTRS total (rho = 0.594, P = 0.003), severity (rho = 0.581, P = 0.004) and disability (rho = 0.470, P = 0.023) scores. No significant differences in the outcomes were found between any pair of the categorical variables. ConclusionsFollow-up duration was the only independent factor correlated to the outcomes of GPi-DBS for CD patients. However, follow-up duration is an indefinite factor prior to surgery, thus further studies are needed before the final conclusions of prognostic factors are established.

Assessment of hand functions in patients with idiopathic cervical dystonia.

Cervical dystonia (CD) is the most common form of focal dystonia characterized by involuntary contractions of the neck muscles, causing abnormal rotation of the head into specific directions. Studies report that idiopathic dystonia is a developmental disorder of the sensorimotor circuits, involving both the cortico-striatal and thalamo-cortical pathways. It is also suggested that enhanced cortical plasticity extends beyond the clinically affected region and may also be detected in the unaffected upper limbs of the patient with CD. In the present study, we aimed at exploring if patients with CD had hand motor dysfunctions. Forty patients with idiopathic CD and 40 healthy controls were included in this study. Dystonic symptoms were assessed by means of The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Stanford Health Assessment Questionnaire (HAQ) was used to assess functional status. Quality of life (QoL) was assessed by using the Medical Outcomes Study Short Form 36-Item Health Survey (SF 36). Grip strength was assessed by using hand dynamometers. Tip pinch, lateral pinch and chuck pinch of the hand were assessed by using a pinchmeter. Fingertip dexterity and hand coordination was assessed using Purdue Pegboard. Duruoz Hand Index (DHI) was used for the assessment of hand functions. There were no significant differences between the groups in grip and pinch strengths of hands and fingers. As to the fingertip dexterity, patients with CD had a mean Pin 1 and Pin 2 test score of 10.6±2.8 and 10.8±3.2 respectively and a mean assembling test score of 5.2±2.0. These results were significantly worse than those of the healthy controls. As to the SF 36 sub-scores, there were significant differences between the groups in all SF 36 sub-scores (p<.001). This study indicates that patients with CD suffer a deteriorated fine motor coordination of hands without dystonic involvement of upper extremities. Furthermore, lower SF 36 scores in patients with CD suggest poorer health-related quality of life.

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.

Impact of abobotulinumtoxinA on the clinical features of cervical dystonia in routine practice

IntroductionThe efficacy and safety of abobotulinumtoxinA in the management of cervical dystonia has been established in randomized, controlled trials that use a selected trial population. In this meta-analysis of observational data, we evaluated the real-life effectiveness of abobotulinumtoxinA as delivered in routine clinical practice. MethodsMeta-analysis of patient-level data for adult patients with cervical dystonia treated with abobotulinumtoxinA from three prospective, multicenter, observational studies (NCT01314365, NCT00833196 and NCT01753349). ResultsWe report data for patients treated with abobotulinumtoxinA over one injection cycle at 181 neurology centers in 35 countries. CD clinical features as assessed by Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) Total scores (N = 920) significantly reduced by a mean [95%CI] of −12.9 [−13.9, −11.8] points at Week 4 (N = 449) and −3.2 [−3.8, −2.7] points at the end of the injection cycle (N = 890). All three TWSTRS domains (symptom severity, disability and pain) contributed to the overall improvement. Patients were generally content with symptom control at peak effect of the treatment cycle, with 86% reporting overall satisfaction. ConclusionFindings from this meta-analysis of observational studies confirm the effectiveness of abobotulinumtoxinA in routine practice. Despite inclusion of a broader population sample, the magnitude of improvements observed is consistent with that seen in the pivotal, randomized controlled trials.

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.

Treatment of severe refractory dystonic tremor associated with cervical dystonia by bilateral deep brain stimulation: A case series report

ObjectivesThe aim of the present study was to present a case series of 3 patients with longstanding, severe, debilitating dystonic head tremor (DT) coexistence with cervical dystonia (CD) treated successfully by bilateral deep brain stimulation (DBS). Pharmacological treatment including benzodiazepines and botulinum toxin injections employed to overactive muscles have failed to adequately control dystonic jerking movements of the head and neck. Patients and methodsAll patients were diagnosed with DT who accompanied CD. Two patients underwent bilateral implantation of DBS leads into the posteroventrolateral segment of the globus pallidus internus (GPi). 1 patient received combination of implantation of left lead in the nucleus ventralis intermedius of the thalamus (Vim) and the bilateral implantation of DBS leads in the GPi. All surgeries were uneventful. The formal preoperative objective assessment included Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and the motor score of Tremor Rating Scale (TRS). The postoperative TWSTRS, TRS assessments were done at 3, 6 months and 12 months postoperatively up to 48 months after surgery. ResultsAt the 12 months postoperative follow-up visit, the severity, disability and pain scores of TWSTRS were improved by 56 %, 52 % and 38,5 % respectively. The TRS improved by 66 % at 12 months when compared to baseline TRS score. One patient developed seroma over the implanted internal pulse generator and had some wound healing problems which solved without sequel. There were no hardware-related complications over follow-up period. ConclusionOur experience gathered in 3 patients indicates that bilateral DBS can be an effective treatment for disabling, pharmacological refractory tremulous CD.

ANXIETY AND DEPRESSIVE DISORDERS IN PATIENTS WITH CRANIOCERVICAL DYSTONIA: CLINICAL AND ANAMNESTIC FEATURES AND MODIFYING ROLE OF BOTULINUM TOXIN THERAPY

The study included 54 patients with cranio-cervical dystonia, 38 (70.4%) of whom were women, 16 (29.6%) - men, mean age 49,8 ± 11,3 years. 32 patients were diagnosed with cervical dystonia, groups with blepharospasm and Meige’s syndrome included 11 patients respectively. All patients were screened by «Unified Dystonia Rating Scale» and those who had cervical dystonia also by «Toronto western spasmodic torticollis rating scale (TWSTRS)». Psychoemotional state in these patients was examined by Beck depression inventory (BDI) and Hamilton’s anxiety rating scale (HAM-A). Depression and anxiety occurrence and severity was evaluated, impact of different factors including Botulinum toxin treatment was observed. Results of the study confirmed that anxiety and depressive disorders are associated with craniocervical dystonia. Sex, localization of dystonia, disease duration, and satisfaction with the treatment do not affect significantly severity of anxiety and depression. Regular injections of Botulinum toxin significantly decrease severity of psychoemotional disorders in these patients.

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.

Management of cervical dystonia with dry needling

Introduction Cervical dystonia is characterized by involuntary muscle contractions of the neck and abnormal head positions that affect daily life activities. Botulinum toxin treatment continues to be the first choice treatment for most types of focal dystonia. Unfortunately, botulinum toxins are expensive and have limitations. Dry needling has been used widely to relieve muscle spasms and restore its length. Case report A 34 years-old man developed severe axial neck dystonia gradually. His symptoms began approximately 6 months earlier with painless pulsing in his neck followed by involuntary neck movement and rotated of the neck with spasm. There was severe right head rotation and laterocollis with mild retrocollis. Head CT-scan was normal but cervical spine x ray showed retrolisthesis and narrowing intervertebral disc gap of C4-C5 and C6-C7. We used the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) to monitor the progress of dry needling in this patient. Before dry needling was performed, the TWSTRS was 47 and after 5 times dry needling in one week interval, the score became 2 and persisted at 6 months reassessment. Dry needling was done on the myotendinous junction and/or muscle belly of ipsilateral sternocleidomastoideus muscle, spinalis capitis muscle and upper trapezius muscle. Conclusions Few treatment options are available and dry needling may be useful in facilitating a rapid reduction in disability and severity. However, management using dry needling technique may require further assessment and re-needling after 3–6 months.

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).

Torticaput versus Torticollis: Clinical Effects with Modified Classification and Muscle Selection.

BackgroundSeveral different subtypes are distinguished in cervical dystonia, depending on their different levels of movement. In simple rotation, classified as torticollis spasmodicus, we now differentiate between torticollis and torticaput dependent on whether only the head or the neck is turned. The new classification system permits for different injection schemes.Case reportsIn a retrospective study of 22 patients, we examined whether modifying the injected muscles leads to improvement in the results as evaluated in the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). The results showed that both injection schemes do in fact lead to improvements while differentiating between caput and collum has significantly better effects.DiscussionDue to our results we recommend the classification differentiating between torticollis and torticaput type.