Tsui Score Research Articles

The role of tsui scale in the treatment of primary cervical dystonia

Objective: To evaluate the severity of patients with primary cervical dystonia and the effectiveness of treatment with botulinum toxin A (Dysport based on the Tsui scale Methods: We conducted a cross-sectional descriptive study of all patients with primary cervical dystonia treated with botulinum toxin A injections at Tam Anh General Hospital from November 2022 to April 2024. Patients were assessed in detail on the Tsui scale before and after treatment 4-6 weeks. Patients were considered to have improvement when the Tsui score after treatment decreased by at least 30% compared to before treatment. Results: There were 17 patients (12 men and 5 women) who met the criteria in our study. The Tsui score before treatment in the male patient group, complex disease, with tremor was higher than that in the female patient group, simple disease, without tremor (p< 0.001). 100% of patients responded to treatment with an average improvement rate of 0.56. The Tsui score after 4-6 weeks of injection (4.59 ± 2.87) improved significantly compared to before treatment (9.71 ± 4.34) (p< 0.001). Conclusion: Our research has demonstrated an enhancement in Tsui score following treatment with BoNT among patients with cervical dystonia. The Tsui score is an objective measure for evaluating treatment effectiveness and should be more widely used in clinical practice.

An analysis of the efficacy of botulinum neurotoxin type a in treating cervical dystonia.

The first-line treatment for cervical dystonia (CD) consists of repeated intramuscular injections of botulinum toxin (BoNT). However, the efficacy in some patients may be unsatisfactory and they may discontinue treatment. To examine the factors associated with the maximum rate of remission in patients with CD after initial botulinum neurotoxin type A (or botulinum toxin type A abbreviated as BTX-A or BoNT-A) treatment. Patients with CD who received BoNT-A injections were evaluated using the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and the Tsui scale, with follow-up endpoints lasting until the start of the second injection. Patients who did not receive a second injection of BoNT-A were followed up for at least 5 months. The maximum remission rates were determined using the lowest Tsui and TWSTRS total scores during the follow-up period. We obtained basic information about these patients such as age, gender, duration of disease, presence of additional disease, types of torticollis, presence of anxiety, depression, tremors, single-photon emission computed tomography (SPECT) findings, injected dose, and so on from their medical records. A total of 70 patients with CD participated in this study, with males comprising 35.7% (25 individuals) with an average age of 45 ± 14 years old. The duration of disease was an independent risk factor for determining whether a complete remission has been attained using the Tsui scale (odds ratio [OR] = 0.978, 95% confidence interval [CI]: 0.959-0.997, P= 0.026). The optimal cut-off point for predicting patients who were unable to achieve complete remission based on duration of disease was 7.5 months (AUG = 0.711). Patients with CD with additional disease had greater difficulty achieving complete remission than those with CD alone based on TWSTRS assessments (P= 0.049). During the study, approximately 17% of all participants reported experiencing adverse reactions that lasted between 1 to 3 weeks before disappearing. BoNT is an effective and safe method for treating CD. The maximum remission rates of patients after their first injections are influenced by the duration of their disease. Thus, treatment using BoNT injections must be administered as soon as possible.

Content overlap of 91 dystonia symptoms among the seven most commonly used cervical dystonia scales

IntroductionDystonia is a movement disorder characterized by sustained or intermittent muscle contractions. Cervical dystonia (CD) is the most common focal dystonia. There are several instruments assessing the symptoms of CD. However, different scales assess different features which may lead to poor patient evaluation.AimThe aim of the study was to evaluate the degree of overlap of most often used CD rating scales identified by the literature review.MethodsA thorough search of the Medline database was conducted in September 2021. Then the frequency of each scale was calculated, and 7 most common scales were included in the content overlap analysis using Jaccard index (0 – no overlap, 1 – full overlap).ResultsToronto Western Spasmodic Torticollis Rating Scale (TWSTRS), Tsui score, Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), Cervical Dystonia Impact Profile 58 (CDIP-58), Craniocervical Dystonia Questionnaire 24 (CDQ-24), Cervical Dystonia Severity Rating Scale (CDSS), Cervical Dystonia Severity Rating Scale (DDS) and The Dystonia Non-Motor Symptoms Questionnaire (DNMSQuest) were the most common scales. 91 CD symptoms were distinguished from 134 items used in the scales. The mean overlap among all scales was 0.17. 52 (62%) symptoms were examined by more than one scale. The CIDP-58 captured the highest number of symptoms (63.0%), while the CDSS captured the lowest number (8.0%). None of the symptoms were examined by seven instruments.ConclusionsThere was a very weak overlap among scales. High inconsistency between the scales may lead to highly different dystonia severity assessment in clinical practice. Thus, the instruments should be combined.

Exploring the Interplay between the Clinical and Presumed Effect of Botulinum Injections for Cervical Dystonia: A Pilot Study.

Repetitive intramuscular injections of botulinum neurotoxin type A (BoNT/A) are the treatment of choice in patients with cervical dystonia (CD). As soon as BoNT therapy is initiated, the natural course of CD cannot be observed anymore. Nevertheless, the present study focuses on the "presumed" course of disease severity under the assumption that no BoNT therapy had been performed. The "experienced" benefit is compared with the "presumed" worsening. Twenty-seven BoNT/A long-term-treated CD patients were recruited. They had to assess the remaining severity of CD in percent of its severity at the start of BoNT therapy (RS-%). Then, they had to draw the course of severity from the onset of symptoms to the start of BoNT/A therapy (CoDB graph), as well as the course of severity from the start of BoNT/A therapy until the day of recruitment (CoDA graph). Then, they were instructed to presume the development of CD severity from the day of the start of BoNT/A therapy until the day of recruitment under the assumption that no BoNT/A therapy had been performed, and to assess the maximal severity they could presume in percent of the severity at the start of BoNT therapy (IS-%). Then, they had to draw the "presumed" development of CD severity (CoDI graph). The "experienced" change in disease severity and the "presumed" change since the start of BoNT/A therapy were compared and correlated with a variety of demographical and treatment-related data, including the actual severity of CD at the day of recruitment, which was assessed using the TSUI score and the actual dose per session (ADOSE). No CD patients expected an improvement without BoNT therapy. "Presumed" worsening ((IS-%)-100) was about 50% in the mean and did not correlate with the "experienced" benefit (100-(RS-%)). However, IS-% was significantly correlated with ATSUI and ADOSE. Obviously, CD patients have the opinion that their CD would have further progressed and worsened if no BoNT/A therapy had been performed. Thus, the total benefit of BoNT/A therapy for a patient with CD is a combination of the "experienced" benefit under BoNT/A therapy and the prevented worsening of CD that the patient expects to occur without BoNT/A therapy.

The Downside of Botulinum Injections for Anterocollis: A Case Series and a Review of the Literature.

This is a case series and a review of the literature of therapeutic outcomes of botulinum toxin (BT) injections for anterocollis. Data collected included gender, age, age at onset, muscles targeted, and doses injected. Routine forms were filled out during each visit: Patient Global Impression of Change, Clinician Global Impression of Severity, Tsui scale. The effect duration and side effects (SEs) of the previous treatment were noted. We described 4 patients (3 men, 13 visits) with anterocollis, as primary postural abnormality of the neck, emphasizing the therapeutic response to BT injection. Mean age at onset was 75.3 ± 7.0 years, age at first injection was 80.7 ± 3.5 years. The mean total dose per treatment was 290.0 ± 95.6 units. Patient Global Impression of Change with any grade of favorable effect was reported in 27.3% of the treatments. In objective assessment, Global Impression of Severity and Tsui scores did not show a consistent tendency of improvement. Neck weakness was prevalent in 18.2% of the visits of the anterocollis group while no other SEs were noted. We found 15 articles describing experience with BT for anterocollis in 67 patients (19 in deep and 48 in superficial neck muscles). This case series describes the poor outcome of BT treatment for anterocollis, with low efficacy and bothersome SE. Levator scapulae injection for anterocollis is not effective and is highly associated with head drop and should perhaps be abandoned. Injection to the longus colli might give some benefit in non-responders.

Does Ultrasound Guidance Improve the Effectiveness of Neurotoxin Injections in Patients with Cervical Dystonia? (A Prospective, Partially-Blinded, Clinical Study).

Aim: The aim of this study was to evaluate the efficacy of ultrasound guidance (US) in the treatment of cervical dystonia (CD) with botulinum neurotoxin type A (BoNT-A) injections in comparison to anatomical landmarks (AL). To date, US is routinely used in many centers, but others deny its usefulness. Materials and Methods: Thirty-five patients (12 males, 23 females) with a clinical diagnosis of CD were included in the study. Intramuscular administration of BoNT-A was performed using either US guidance, or with AL, in two separate therapeutic sessions. The efficacy of BoNT-A administration was assessed with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), Tsui modified scale, Craniocervical Dystonia Questionnaire (CDQ-24) and Clinical Global Impression—Improvement scale (CGI-I). Additionally, patients at therapeutic sessions were digitally recorded and evaluated by two blinded and independent raters. Results: A significant decrease in total TWSTRS, severity subscale TWSTRS, Tsui score, and CDQ-24 was found in both the AL and US group; however, in the TWSTRS disability and pain subscales, a significant decrease was found only in the US group. Moreover, US guided treatment also resulted in a greater decrease in TWSTRS, Tsui score and CDQ-24 compared to anatomical landmarks use only. Conclusions: US guidance might be helpful in improving the results of BoNT-A injections in cervical dystonia, reducing associated pain and disability; however, more studies are needed to evaluate its clinical efficacy.

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

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

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

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

The Use of High Initial Doses of Botulinum Toxin Therapy for Cervical Dystonia Is a Risk Factor for Neutralizing Antibody Formation-A Monocentric Cross-Sectional Pilot Study.

Background and Objectives: The present study aims to analyze the complex patient/treating physician interaction at onset of botulinum toxin (BoNT) therapy in patients with idiopathic cervical dystonia (CD) and the influence of high initial doses on long-term outcomes. Materials and Methods: A total of 74 CD patients with well-documented courses of BoNT treatment were consecutively recruited after written informed consent. Patients had to rate the amount of improvement of CD in percent of severity of CD at onset of BoNT therapy. They had to draw the course of disease severity (CoD) of CD from the onset of symptoms until the onset of BoNT therapy and from the onset of BoNT therapy until recruitment. The remaining severity of CD was estimated by the treating physician using the TSUI score. Demographic- and treatment-related data were extracted from the charts of the patients. Seventeen patients with suspected secondary treatment failure (STF) were tested for the presence of antibodies. Results: Depending on the CoD before BoNT therapy, three patient subgroups could be distinguished: rapid onset, continuous onset and delayed onset groups. Time to BoNT therapy, increase in dose and improvement were significantly different between these three groups. In the rapid onset group, with the highest initial doses, the best improvement was reported, but the highest number of patients with an STF and with neutralizing antibodies was also observed. Conclusion: The use of high initial doses in the BoNT therapy of CD is associated with a rapid response and quick success; however, it leads to an elevated risk for the development of a secondary treatment failure and induction of neutralizing antibodies.

Efficacy of single-photon emission computed tomography aided botulinum toxin injection in cervical dystonia: A double-blind, randomized study

BackgroundAlthough single-photon emission computed tomography (SPECT/CT) could help to predetermine dystonic muscles in patients with cervical dystonia (CD), its efficacy in aiding botulinum toxin injection is undetermined. This randomized, double-blinded study aimed to assess the efficacy of SPECT/CT aided botulinum toxin injection in CD. MethodsPatients were randomized into study group (candidate muscles selected by SPECT/CT and clinical evaluation) or control group (clinical evaluation). Follow-ups were done at two weeks (T1), one (T2), three (T3) and six months (T4). The primary outcomes included symptom improvement assessed using Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and Tsui score at T2. ResultsA total of 122 patients were enrolled and 108 patients accomplished the study. For primary outcomes, the study group had significantly better symptom improvement at T2 (TWSTRS: β, −4.86 [95%CI -9.40 to −0.32; P = 0.036]; Tsui: β, −1.65 [95%CI -2.77 to −0.54; P = 0.004]). For secondary outcomes, the study group also showed better outcomes at T1 (TWSTRS: β, −6.33 [95%CI -10.17 to −2.49; P = 0.001]; Tsui: β, −1.42 [95%CI -2.48 to −0.37; P = 0.008]) and T3 (TWSTRS: β, −6.05 [95%CI -11.09 to −1.01; P = 0.019]; Tsui: β, −1.24 [95%CI -2.40 to −0.08; P = 0.037]). The interval of re-injection was significantly longer in the study group than the control group (159.1 ± 28.6 versus 141.8 ± 51.0 days, P = 0.032). ConclusionsSPECT/CT could improve the efficacy of botulinum toxin in CD. It could become a useful tool to aid botulinum toxin injection.

Hyper- and hypo-connectivity in sensorimotor network of drug-naïve patients with cervical dystonia

BackgroundCervical dystonia (CD) is the most common form of focal dystonia with involuntary movements and postures of the head. The pathogenesis and neural mechanisms underlying CD have not been fully elucidated. MethodsTwenty-seven newly drug-naïve patients with CD and 21 healthy controls (HCs) were recruited with clinical assessment and resting-state functional magnetic resonance imaging (rs-fMRI) scanning. Severity of CD was measured by Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and Tsui scores. Whole-brain voxel-wise intrinsic connectivity (IC) and seed-based functional connectivity (FC) analyses were performed for detection of changes in the CD group relative to HCs, controlling for age, gender, and global time series correlation, followed by correlation analyses of IC, seed-based FC and clinically relevant features, respectively. ResultsIn comparison with HCs, CD patients showed significantly increased IC measurement in the anterior part of the left supramarginal gyrus and extended to the inferior left postcentral gyrus (AL-SMG/IL-PCG). With this cluster as a seed, decreased FC was found in the right precentral and postcentral gyrus. Moreover, the regional IC value in the AL-SMG/IL-PCG was significantly positively correlated with TWSTRS-1 (severity) score, and significantly negatively correlated with the associated seed-based FC strength. ConclusionsOur results showed signs of both hyper- and hypo-connectivity in bilateral regions of the sensorimotor network related to CD. The imbalance of functional connectivity (both hyper- and hypo-) may hint both overloading and disrupted somatosensory or sensorimotor integration dysfunction within the sensorimotor network underlying the pathophysiology of CD, thus providing a network target for future therapies.

The Impact of SARS-CoV-2 Pandemic Lockdown on a Botulinum Toxin Outpatient Clinic in Germany.

Botulinum neurotoxin type A (BoNT/A) injections have to be administered repeatedly to achieve a rather stable, high level of improvement. This study aimed to take a look at changes in the daily routine of a BoNT/A outpatient clinic due to the SARS-CoV-2 pandemic lockdown, analyze the impact of SARS-CoV-2-induced re-injection delay on outcomes in patients with cervical dystonia (CD) (n = 36) and four other disease entities (n = 58), and study the influence of covariables, including previous injections and doses. For the present observational study, the first 100 patients who were scheduled to have an appointment between April 20 and May 18 during the partial lockdown and also had been treated regularly before the lockdown were recruited. Clinical and demographical characteristics and treatment-related data from the previous visits were extracted from charts. Time delay, symptom severity assessment, and TSUI score (if applicable) were gathered at the first coronavirus pandemic lockdown emergency visit for each patient. Of the 94 patients who could come to the clinic, 48 reported a delay and 44 reported worsening during the delay. Delays ranged from 1 to 63 days, the mean delay was 23 days, and the mean worsening was 26% compared to the previous visit. A significant correlation was found between the duration of the delay and the patient’s rating of worsening (PwP). In CD patients, the physician´s rating of CD worsening by the TSUI score (ATUSI-PTSUI) was significantly correlated with general worsening (DwP) and the TSUI at the last visit (PTSUI). A small delay of a few weeks led to a similar worsening of symptoms in CD and all other disease entities and to relapse on a higher level of severity. This relapse can only be compensated by continuous treatment up to at least 1 year until patients reach the same level of treatment efficacy as that before the SARS-CoV-2 pandemic.

Brain connectivity abnormalities and treatment-induced restorations in patients with cervical dystonia.

The relationship between brain abnormalities and phenotypic characteristics in cervical dystonia (CD) patients has not been fully established, and little is known about the neuroplastic changes induced by botulinum toxin type A (BoNT-A) treatment. Ninety-two CD patients presenting with rotational torticollis and 45 healthy controls from our database were retrospectively screened. After clinical assessment, the 92 patients underwent baseline magnetic resonance imaging (MRI) followed by a single-dose injection of BoNT-A. Four weeks later, 76 out of the 92 patients were re-evaluated with the Tsui scale for dystonia severity, and 33 out of 76 patients completed post-treatment MRI scanning. Data-driven global brain connectivity and regional homogeneity in tandem with seed-based connectivity analyses were used to examine the functional abnormalities in CD and longitudinal circuit alterations that scaled with clinical response to BoNT-A. Multiple regression models were employed for the prediction analysis of treatment efficacy. Cervical dystonia patients exhibited elevated baseline connectivity of the right postcentral gyrus with the left dorsomedial prefrontal cortex and right caudate nucleus, which was associated with their symptom severity. BoNT-A reduced excessive functional connectivity between the sensorimotor cortex and right superior frontal gyrus, which was significantly correlated with changes in Tsui score. Moreover, pre-treatment regional homogeneity of the left middle frontal gyrus was linearly related to varied response to treatment. Our findings unravel dissociable connectivity of the sensorimotor cortex underlying the pathology of CD and central effects of BoNT-A therapy. Furthermore, baseline regional homogeneity with the left middle frontal gyrus may represent a potential evidence-based marker of patient stratification for BoNT-A therapy in CD.

Subjective versus objective symptom intensities ratings in cervical dystonia and hemifacial spasm across a botulinum neurotoxin cycle.

BackgroundSubjective symptom complaints often do not match the expert’s ratings in focal dystonia. Nonetheless, perceived symptom intensities drive compliance and outcome of botulinum neurotoxin (BoNT) treatment.MethodsPerception of symptom development across a BoNT cycle was obtained in 21 cervical dystonia (CD) and 15 hemifacial spasm (HFS) patients at four time points during a BoNT cycle. Subjective assessments were recorded by means of a quality‐of‐life questionnaire and a patient diary containing items related to subjective severity of disease, mood, pain, social impairment, and quality of life. Medical investigation used the Tsui score and TWSTRS, and a HFS rating score, respectively.ResultsIn both patient groups, subjective intensities were strongly associated with psychological variables. Only in CD did objective assessment moderately correlate with subjective ratings solely at the beginning and the end of the BoNT cycle. Overall, the beneficial effects of BoNT treatment were only loosely associated with subjective experiences in both groups.ConclusionThe emotional situation should be assessed regularly in patients undergoing BoNT therapy.

Targeting pain in the long-term treatment of cervical dystonia with botulinum toxin A

Aims Cervical pain is one of the most common non-motor symptoms of cervical dystonia (CD) and affects from 54.6% to 88.9% of patients. To date, minority of studies investigated the relevance of pain in a long-term botulinum toxin (BoNT) therapy of CD. The aim of the study was to define an impact of cervical pain on the disease severity and disability, as well as to assess antinociceptive BoNT efficacy in a long-term treatment of CD. Materials and methods In this case-control study, CD patients who received stable doses of BoNT for at least 3 years were assessed with the use of validated scales. Participants were divided into two groups depending on the occurrence of CD-related pain. Results We examined 50 participants who received a mean of 24 injection cycles (6–51) of BoNT during a mean treatment period of 10.3 years (3.0–23.5). Participants with cervical pain (68.0%) were characterized by higher scores in all scales used in this study: TWSTRS severity (p = 0.030), disability (p < 0.001), total (p < 0.001) and TSUI score (p = 0.046). Pain reduction following BoNT injection lasted longer than muscle relaxation in 85.3% of patients. Pain improvement between first and last BoNT injection cycle was reported by 76.5% of patients with CD-related pain. Conclusions The presence of cervical pain in CD may increase the severity of muscular symptoms and disease-related disability. BoNT has a noticeable antinociceptive effect in the long-term treatment of CD.

Disease Progression of Idiopathic Cervical Dystonia in Spite of Improvement After Botulinum Toxin Therapy.

Aim of the Study: To demonstrate general progression of symptoms in cervical dystonia (CD) on the one hand and improvement of some special symptoms on the other hand after botulinum toxin (BoNT) therapy.Methods: 74 patients with idiopathic CD under continuous treatment in a BoNT outpatient department with at least three injections, completed a short questionnaire. They were asked whether pain, increased muscle tone and tension, reduced mobility of the head, abnormal head position, head tremor, or other symptoms had been present at the onset of BoNT-therapy and which symptoms were present at the time of recruitment. Patients had to rate actual severity of CD in percent of the severity of CD at the onset of BoNT-therapy. The TSUI score was determined by the treating physician. Blood samples were taken to analyze induction of neutralizing antibodies.Results: Mean improvement of CD reported by the patients and scored by the physician was about 50%. The frequency of all symptoms increased with duration of therapy. The symptom most frequently improved was abnormal head position. The longer the time span between onset of symptoms and onset of BoNT-therapy was, the higher was the actual TSUI score and the lower the improvement reported. Twelve patients had positive antibody tests.Conclusions: Patients experience a progression of CD, but recognize improvement of abnormal head position due to BoNT-therapy. The longer patients have been without BoNT- therapy, the poorer is the long-term outcome independent on duration of BoNT treatment. Therefore BoNT-therapy should be initiated as early as possible.

Transient Improvement after Switch to Low Doses of RimabotulinumtoxinB in Patients Resistant to AbobotulinumtoxinA.

Botulinum toxin type B (BoNT/B) has been recommended as an alternative for patients who have become resistant to botulinum toxin type A (BoNT/A). This study aimed to compare the clinical effect, within a patient, of four injections with low doses of rimabotulinumtoxinB with the effect of the preceding abobotulinumtoxinA (aboBoNT/A) injections. In 17 patients with cervical dystonia (CD) who had become resistant to aboBoNT/A, the clinical effect of the first four rimabotulinumtoxinB (rimaBoNT/B) injections was compared to the effect of the first four aboBoNT/A injections using a global assessment scale and the TSUI score. After the first two BoNT/B injections, all 17 patients responded well and to a similar extent as to the first two BoNT/A injections, but with more side effects such as dry mouth and constipation. After the next BoNT/B injection, the improvement started to decline. The response to the fourth BoNT/B injection was significant (p < 0.048) lower than the fourth BoNT/A injection. Only three patients developed a complete secondary treatment failure (CSTF) and five patients a partial secondary treatment failure (PSTF) after four BoNT/B injections. In nine patients, the usual response persisted. With the use of low rimaBoNT/B doses, the induction of CSTF and PSTF to BoNT/B could not be avoided but was delayed in comparison to the use of higher doses. In contrast to aboBoNT/A injections, PSTF and CSTF occurred much earlier, although low doses of rimaBoNT/B had been applied.

The cerebellum in idiopathic cervical dystonia: A specific pattern of structural abnormalities?

IntroductionIn recent years, cerebellar abnormalities have gained increasing attention as possible physiopathological substratum of idiopathic cervical dystonia (ICD), but a consistent pattern of cerebellar structural modifications has not yet been established. We systematically investigated the presence of volumetric alterations of cerebellar gray (GM) and white matter (WM) in ICD patients, as well as their clinical relevance. MethodsIn this two-centers prospective cross-sectional study, from May 2013 to December 2017, 27 patients with ICD and 27 age- and sex-comparable healthy controls underwent brain MRI including 3D T1-weighted sequences for volumetric analyses. Between-group differences in terms of gray matter and cerebellar peduncles volumes were investigated using both region of interest (ROI)-based and voxel-based approaches using the SUIT tool (SPM12), and significant volumetric changes were correlated with clinical impairment (as measured with the Tsui score) and presence of tremor. ResultsICD patients showed significant volumetric reduction of cerebellar GM in the anterior lobe and lobule VI, resulting from both ROI-based (p ≤ 0.009) and voxel-based (p ≤ 0.04) analyses, while small clusters of reduced WM volume were found in the right cerebellum and left midbrain (p = 0.04), along with reduced volume of the bilateral superior (p = 0.04) and middle (p = 0.03) cerebellar peduncles. Furthermore, higher middle cerebellar peduncles volume was associated with the presence of tremor (p = 0.04). ConclusionOur data show evidence of a specific pattern of cerebellar structural abnormalities in ICD patients, with volume loss mainly involving cortical GM regions related to the somatotopic representation of the affected body parts and, to a lesser extent, cerebellar peduncles.

The efficacy of single-photon emission computed tomography in identifying dystonic muscles in cervical dystonia.

The key point for botulinum toxin type A injection in treating cervical dystonia is to accurately identify dystonic muscles. This study aimed to evaluate the efficacy of technetium-sestamibi single-photon emission computed tomography in identifying target muscles in cervical dystonia. In the study group (n = 18), target muscles were selected according to clinical evaluation combined with technetium-sestamibi single-photon emission computed tomography, while in the control group (n = 18), target muscles were selected by clinical evaluation alone. All patients were followed-up at 2 weeks, 1, 3 and 6 months after botulinum toxin type A injection. The primary outcomes were the reduction rates in Toronto Western Spasmodic Torticollis Rating Scale and Tsui score at 1 month. Although the reduction rates in Toronto Western Spasmodic Torticollis Rating Scale and Tsui scores were not different between the two groups at 2 weeks and 1 month, the reduction rates in both scores were significantly higher in the study group at 3 and 6 months. The number of patients receiving re-injection within 6 months was significantly lower in the study group. Also, the re-injection interval was significantly longer in the study group. In the study group, more deep cervical muscles were injected, which concerns especially semispinalis capitis, longissimus capitis, and obliques capitis inferior muscles. technetium-sestamibi single-photon emission computed tomography is a useful method for screening target muscles in cervical dystonia. It helps clinicians draw a 'blueprint' for the distribution of dystonic muscles before botulinum toxin type A injection.

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.