Abstract Background Technetium-sestamibi single-photon emission computed tomography ( 99m Tc-MIBI SPECT) guidance has been shown to significantly improve the efficacy of botulinum toxin (BTX) injection in dystonic postural abnormalities. But its effect in parkinsonian postural abnormalities remains unknown. Methods Eligible PD patients with postural abnormalities all underwent 99m Tc-MIBI SPECT muscle metabolic imaging and baseline evaluation followed by SPECT-guided BTX injection. The primary efficacy outcome was the improvement in resting forward and lateral trunk flexion angle (RTF and RTL) at 1 month. Secondary efficacy outcomes included subjective efficacy, pain VAS score and PDQ-39 score. Adverse events (AEs) were also assessed. Results A total of 29 PD patients (14 males, 15 females) were analyzed with a mean age of 68.5 ± 8.9 years. The RTF increased (37.71 ± 16.82° vs. 40.17 ± 17.03°, Estimated Mean Difference = -2.46, 95% CI [-5.97, 1.06], p = 0.027) significantly while the RTL decreased (9.61 ± 8.78° vs. 8.28 ± 7.62°, Estimated Mean Difference = 1.34, 95% CI [-0.99, 3.67], p = 0.208) slightly at 1 month compared to baseline, though the difference was not significant. At 1 month, 4 patients (13.8%) experienced an improvement of more than 5° in RTF, 13 patients (44.8%) reported minimal to much subjective improvement and 10 patients (34.5%) saw an improvement of more than 1 point in the pain VAS score. Eleven AEs occurred, with no serious adverse events (SAEs). Related AEs mainly included transient worsening of postural abnormalities and low-back pain, which typically alleviated within 2 weeks to 2 months. Conclusions The 99m Tc-MIBI SPECT-guided BTX treatment for parkinsonian postural abnormalities demonstrated acceptable safety but was not as effective as expected. Future studies should focus on identifying potential candidates who shall benefit from this method. Clinical trials registration the Chinese Clinical Trial Registry, number ChiCTR2000039775, Nov. 8th, 2020.

OBJECTIVE:The Col-Cap classification for cervical dystonia (CD) has been proposed to optimize treatment strategies with botulinum toxin type A (BTX-A). Although this concept has been used for 15 years, the efficacy of BTX-A in various CD patterns remains unclear. The present study compared the efficacy of BTX-A, aided by single-photon emission computed tomography (SPECT) and electromyography (EMG), in various CD patterns. METHODS:CD patients who underwent SPECT scans to detect dystonic muscles were included. Candidate muscles were first selected based on clinical evaluation and abnormal radioactivity on SPECT imaging. All patients were injected under the guidance of EMG, and only candidate muscles with positive EMGs were injected. The efficacy of BTX-A was assessed via the subjective clinical improvement rate and the reduction in the Tsui score at four weeks after injection. RESULTS:The study enrolled 252 patients. The subjective improvement rates were 63.5%±20.6% for simple types and 59.9%± 22.2% for complex types, and this difference was not significant (p=0.247). The reduction rates of the Tsui score did not differ between the simple type (52.4%±24.2%) and the complex type (49.6%±23.6%, p=0.556). Moreover, clinical improvement rates did not significantly differ within each group of simple subtypes, complex subtypes, or patients with different types of tremors (p>0.05). CONCLUSION:The efficacy of BTX-A treatment was comparable in different CD patterns because of the appropriate selection of dystonic muscles via SPECT and EMG. Therefore, employing multifaceted approaches to identify dystonic muscles can yield favorable outcomes, even in complex cases.

OBJECTIVE:This study aimed to assess the efficacy and safety of botulinum toxin type A (BTX-A) injection near the stylomastoid foramen for tinnitus in patients with hemifacial spasm (HFS). STUDY DESIGN:Retrospective study from 2011 to 2024. SETTING:Tongji Hospital affiliated to Tongji University in Shanghai, China. PATIENTS:Patients with HFS combined with tinnitus. INTERVENTION:BTX-A injection near the stylomastoid foramen. MAIN OUTCOME MEASURES:The efficacy of BTX-A was assessed based on subjective improvement reported by patients after 1 month. Adverse events were also documented. RESULTS:A total of 869 HFS patients were analyzed, including 122 with tinnitus. Among the 122 patients with tinnitus, 110 received BTX-A injections near the stylomastoid foramen, and complete follow-up data were available for 105 of them. The 3.75 U group showed a higher improvement rate (69.2 ± 14.3%) compared with the 2.5 U group (46.3 ± 25.0%, p < 0.05). The majority received a 5 U dose, with a significantly higher improvement rate (76.9 ± 15.1%) compared with the 2.5 U group ( p < 0.01). All reported adverse events were related to routine injection sites and were mild, requiring no special treatment. No adverse events related to BTX-A injection near the stylomastoid foramen were reported. CONCLUSION:In patients with HFS-associated tinnitus, additional injection of 5 U BTX-A near the stylomastoid foramen, targeting the stapedius muscle, proved to be an effective therapy with minimal adverse effects.

Dystonia is a genetically and phenotypically heterogeneous disorder that occurs in isolation (isolated dystonia) or in combination with other movement disorders. To determine the genetic spectrum in isolated dystonia, we enrolled 88 patients with isolated dystonia for whole-exome sequencing (WES). Seventeen mutations, including nine novel ones, were identified in 19 of the 88 patients, providing a 21.59% positive molecular diagnostic rate. Eleven distinct genes were involved, of which TOR1A and THAP1 accounted for 47.37% (9/19) of the positive cases. A novel missense variant, p.S225R in TOR1A, was found in a patient with adolescence-onset generalized dystonia. Cellular experiments revealed that p.S255R results in the abnormal aggregation of Torsin-1A encoding by TOR1A. In addition, we reviewed the clinical and genetic features of the isolated dystonia patients carrying TOR1A, THAP1, ANO3, and GNAL mutations in the Chinese population. Our results expand the genetic spectrum and clinical profiles of patients with isolated dystonia and demonstrate WES as an effective strategy for the molecular diagnosis of isolated dystonia.

Introduction:Retroform cervical dystonia (RCD), which includes retrocaput and retrocollis, is a rare form of cervical dystonia. Few reports have been published on RCD. The present study aimed to characterize the target muscles involved in RCD and the efficacy of botulinum toxin type A (BTX-A) injection.Methods:Patients with consecutive cervical dystonia with RCD as the most problematic feature were retrospectively analyzed over a 10-year period. Target muscles were screened and confirmed based on clinical evaluation, single-photon emission computed tomography, and electromyography. In addition, efficacy and adverse events following BTX-A injection in patients with RCD were evaluated.Results:A total of 34 patients with RCD were included, 18 of whom presented with retrocaput and 16 with retrocollis. The most frequently injected muscles in RCD were splenius capitis (SPCa, 97.1%) and semispinalis capitis (SSCa, 97.1%), followed by levator scapulae (LS, 50.0%), rectus capitis posterior major (RCPM, 47.1%), trapezius (TPZ, 41.2%), and sternocleidomastoid muscle (SCM, 41.2%). Besides cervical muscles, the erector spinae was also injected in 17.6% of patients. Most muscles were predominantly bilaterally injected. The injection schemes of retrocaput and retrocollis were similar, possibly because in patients with retrocollis, retrocaput was often combined. BTX-A injection achieved a satisfactory therapeutic effect in RCD, with an average symptom relief rate of 69.0 ± 16.7%. Mild dysphagia (17.6%) and posterior cervical muscle weakness (17.6%) were the most common adverse events.Conclusion:SPCa, SSCa, LS, RCPM, LS, and SCM were commonly and often bilaterally injected in RCD. Patients with RCD could achieve satisfactory symptom relief after BTX-A injection.

labeled, highly specific antibody to ATXN3 and recombinant human ATXN3 purified to homogeneity, serving as standard to determine absolute amounts of ATXN3 and observed considerable variation of normal and mATXN3 in human peripheral blood mononuclear cells (PBMC). Between 0.5 and 3 nanograms ATXN3were present per microgram protein (Supplementary Fig. 1). The well-known inverse correlation between CAG repeat length and age at onset was reflected in our cohort, suggesting that the cohort was representative despite the small number. No clear cut correlation with age, sex, or the severity of ataxia (scale for the assessment and rating of ataxia score) became apparent with absolute amounts of normal or mATXN3; similar to what has been observed recently (data not shown). Independent of the absolute amount, however, the ratio of normal to mATXN3 correlated with the age at onset of motor symptoms (ie, the more mATXN3 relative to normal ATXN3 was present in PBMC, the earlier the age at onset (Fig. 1). This phenomenon points to the importance of the individual relative proportion of expanded, dysfunctional protein, and fits with the idea of a toxic gain of function of mATXN3. As we observed no clear cut correlation with age and little variation with repeated sampling, measurement ofATXN3 in PBMC appears as a feasible tool to determine the amount of both normal and mATXN3 in an individual over time and therefore, to evaluate the effect of compounds or molecular tools, which supposedly lower neuronal ATXN3 expression in a clinical trial. It will be interesting to evaluate a putative change of ATXN3 expression in PBMC after systemic (or intrathecal) application of a canonical drug or antisenseoligonucleotides, because we are concerned that the amount of ATXN3 in cerebrospinal fluid (CSF) might be to low to measure, even with a highly specific and sensitive assay. Whether the relative amounts of ATXN3 will develop into a useful biomarker for SCA3 (ie, whether this ratio has a prognostic value in individuals at risk before the onset of obvious motor signs) remains to be determined in larger longitudinal cohorts.

Torticaput is the most common primary form of cervical dystonia (CD). Obliquus capitis inferior (OCI) plays a major role in ipsilateral rotation of the head. The present study aimed to use single-photon emission computed tomography (SPECT/CT) to determine the involvement of OCI in torticaput and in torticaput associated with no–no tremor. We retrospectively analyzed the SPECT/CT images of 60 patients with torticaput as the main abnormal posture and ranked the affected muscles. The affected muscles in patients with no–no tremor were also ranked. The correlation between the radioactivity of OCI and the thickness of OCI measured by ultrasonography was analyzed. The agreement between SPECT/CT and electromyography in detecting OCI was also analyzed. After sternocleidomastoid muscle (81.7%), OCI was the second most affected muscle (70.0%) in torticaput, followed by splenius capitis (63.3%). In 23 patients with no–no tremor, OCI (78.3%) and sternocleidomastoid muscle (78.3%) were the most frequently affected muscles, followed by splenius capitis (69.6%). Furthermore, bilateral muscle involvement was commonly seen in patients with no–no tremor, especially for OCI (12/23) and sternocleidomastoid muscle (11/23). A positive correlation was found between the radioactivity and thickness of OCI (r = 0.330, P < 0.001). The total agreement rate between SPECT/CT and electromyography in the diagnosis of OCI excitement was 94.0%, with kappa value = 0.866 (P < 0.001). OCI plays a critical role in torticaput and no–no tremor. SPECT/CT could be a practical tool to help clinicians detect abnormally excited OCI.

Sensory ataxic neuropathy, dysarthria, and ophthalmoparesis (SANDO) is a rare mitochondrial disorder associated with mutations in the POLG gene, which encodes the DNA polymerase gamma catalytic subunit. A few POLG-related SANDO cases have been reported, but the genotype–phenotype correlation remains unclear. Here, we report a patient with SANDO carrying two novel missense variants (c.2543G>C, p.G848A and c.452 T>C, p.L151P) in POLG. We also reviewed previously reported cases to systematically evaluate the clinical and genetic features of POLG-related SANDO. A total of 35 distinct variants in the coding region of POLG were identified in 63 patients with SANDO. The most frequent variant was the p.A467T variant, followed by the p.W748S variant. The clinical spectrum of SANDO is heterogeneous. No clear correlation has been observed between the mutation types and clinical phenotypes. Our findings expand the mutational spectrum of POLG and contribute to clinical management and genetic counseling for POLG-related SANDO.

Mutations in VPS16 have been identified to be responsible for generalized dystonia. We screened VPS16 variants in 53 unrelated subjects with isolated dystonia via whole-exome sequencing. A novel pathogenic frameshift mutation p.R643fs* was found in a patient with early-onset multifocal dystonia with prominent oromandibular and bulbar involvement. Our findings expanded the spectrum of VPS16-related dystonia and suggested that mutations in VPS16 should be considered in patients with progressive early-onset dystonia.

目的 调查国内医生对特发性肌张力障碍的认知现状,从医务人员角度分析特发性肌张力障碍延误诊治的相关因素,探讨影响医生规范、持续开展针对性治疗的主要原因.方法 选自2019年10月至2020年3月通过中国肌张力障碍联盟、中国神经科学学会神经毒素分会成员单位相关科室的医生进行随机横断面调查,问卷内容包括医生的基本情况、诊治特发性肌张力障碍的经验、对相关诊疗规范及区域专科设置的了解情况、开展肉毒毒素治疗过程中遭遇的困难等.通过线上匿名方式填写,数据导入SPSS 21.0统计软件进行建库分析.结果 共收集有效问卷311份,来自可能诊治过特发性肌张力障碍患者的医生.绝大多数调查对象来自三级医院,60.1％的医生为神经内科医生,其中43.7％的医生专攻运动障碍病亚专业.参与调查的所有医生几乎都诊治过特发性肌张力障碍的患者,其中最常诊治的肌张力障碍类型是眼睑痉挛,有75.2％的医生正在开展肉毒毒素治疗肌张力障碍诊疗工作.区域设置运动障碍专科对于医生了解肌张力障碍诊疗规范(P<0.001)及肉毒毒素注射技术(P<0.05)具有显著影响.25.1％的医生因"未经培训、技术有难度"难以初次开展肉毒毒素治疗,影响医生持续开展肉毒毒素治疗的主要因素为收费低廉、药占比高.结论 国内医生对特发性肌张力障碍的认知水平有待提高,设置运动障碍专科可显著提高当地医生对肌张力障碍诊疗规范及肉毒毒素的了解程度,技术瓶颈、收费低廉、药占比高等是限制医生无法规律开展肉毒毒素治疗的主要原因.

Background: Although 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. Methods: Patients 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. Results: A 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: beta,-4.86 [95%CI-9.40 to-0.32; P = 0.036]; Tsui: beta,-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: beta,-6.33 [95%CI-10.17 to-2.49; P = 0.001]; Tsui: beta,-1.42 [95%CI-2.48 to-0.37; P = 0.008]) and T3 (TWSTRS: beta,-6.05 [95%CI-11.09 to-1.01; P = 0.019]; Tsui: beta,-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). Conclusions: SPECT/CT could improve the efficacy of botulinum toxin in CD. It could become a useful tool to aid botulinum toxin injection.

BACKGROUND: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.METHODS: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.RESULTS: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.CONCLUSIONS: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.

INTRODUCTION:Dystonia is a movement disorder with high clinical and genetic heterogeneity. Mutations in Anoctamin-3 (ANO3) gene have been reported to cause dystonia 24 (DYT24). This study aims to clarify the spectrum and frequency of ANO3 rare variants in Chinese populations with primary dystonia and understand the clinical and genetic features of DYT24.METHODS:Sanger sequencing was used to screen all exons and exon-intron boundaries of ANO3 for rare variants in 115 primary dystonia patients. The clinical manifestations of patients with ANO3 variants in our study and previously reported literatures were further characterized.RESULTS:Four distinct variants of ANO3 (c.1127A > G, c.1235 T > A, c.1531-3T > C, c.-11G > T) were identified in six unrelated individuals. Combined with our work and literature review, a total of 35 different rare variants distributed in ANO3 were identified in 62 dystonia patients. The predominant phenotype is cranio-cervical dystonia and more than half of patients develop head/limb tremor. Most of patients presented with isolated dystonia whereas few of them showed combined dystonia. The age of onset ranged from 1 to 69 years and peaked in late adulthood, while for generalized dystonia it peaked in a young age. Half of patients with generalized dystonia experienced deep brain stimulation (DBS). And all of them showed improvement of dystonia by DBS.CONCLUSIONS:This study confirms a relatively high frequency of rare ANO3 variants in Chinese patients with dystonia and indicates that the late adulthood-onset, cranio-cervical dystonia seems to be an important feature of the ANO3 phenotype. Further functional studies are warranted to understand the role of ANO3 in dystonia.

Facial synkinesis can be present in both primary and postparalytic hemifacial spasm (HFS). The present retrospective study aimed to summarize the clinical features of synkinesis and explore an appropriate botulinum toxin A (BoNT-A) injection strategy to manage the synkinesis accompanying HFS. Video recordings of 234 patients with primary and postparalytic HFSs were analyzed. Improvements in the severity of spasm and synkinesis owing to BoNT-A treatment were monitored and compared among 36 primary and 12 postparalytic HFS patients with synkinesis and completed follow-up records. BoNT-A was injected into the voluntary facial region (VFR), the synkinetic facial region (SFR), or both VFR and SFR, and the efficacy of these strategies was evaluated and analyzed. Oral-ocular synkinesis in the primary group (32.8%) and ocular-oral synkinesis in the postparalytic group (81.0%) showed the highest incidence. Patients in both the primary and postparalytic groups exhibited a tremendous alleviation of spasm (97.2% vs. 91.7%, P > 0.05) following BoNT-A treatment. In both groups, coinjection and SFR injection were commonly used and effective in treatment of ocular and oral synkinesis, while VFR was frequently used but ineffective for frontal synkinesis. In addition, the improper muscle selection surrounding the mouth corner resulted in pattern change and treatment failure of oral synkinesis. Synkinesis mostly affected the ocular and oral regions. BoNT-A, via treatment of SFR, is effective against synkinesis accompanying HFS.

Botulinum neurotoxin (BoNT) has become a powerful therapeutic tool, and is extensively used in aesthetic medicine and in the treatment of neurological disorders. However, its duration of effect is limited, mainly owing to nerve sprouting. Inhibition of nerve sprouting to prolong the effective duration of BoNT is therefore of great clinical interest. However, appropriate interventional strategies to accomplish this are currently unavailable. In this study, we determined the role of the neurogenic regulator agrin in BoNT type A (BoNT/A)-induced nerve sprouting in a rat model. We then determined whether agrin could be used as an interventional target for prolonging the duration of effect of BoNT/A, and made a preliminary study of the upstream and downstream regulatory mechanisms by which agrin could influence the effective duration of BoNT/A. Our results showed that agrin was involved in the regulation of BoNT/A-induced nerve sprouting, and blocking of agrin function with anti-agrin antibody temporarily could delay muscle strength recovery and prolong the duration of BoNT/A effect. Moreover, agrin influenced the duration of BoNT/A effect by regulating downstream myogenic muscle-specific receptor tyrosine kinase (MuSK), and was simultaneously regulated by upstream miR-144. In conclusion, agrin could regulate BoNT/A-induced nerve sprouting through miR-144-agrin-MuSK signaling; it influences the effective duration of BoNT/A, and could find clinical application as an interventional target for prolonging the effect of BoNT/A.

目的 探讨A型肉毒毒素(botulinum toxin type A,BoNT-A)治疗原发性及面瘫后面肌痉挛患者痉挛及伴随联带运动的疗效差异.方法 选取2009年1月-2018年7月具有联带运动的面瘫后面肌痉挛患者12例,按照1∶2配比性别无差异且具有联带运动的原发性面肌痉挛患者24例,分析2组BoNT-A治疗后痉挛严重程度以及伴随联带运动的改善差异.结果 面瘫后及原发性面肌痉挛患者在起效时间[(4.38±2.17) vs.(4.67±4.14)d],维持时间[(3.83±2.94) vs.(4.96±2.48)d],注射剂量[(31.35±12.30) vs.(26.98±8.75) U]方面无明显差异(P＞0.05).原发性面肌痉挛组患者满意度显著较高(84.79±19.81 vs.64.17±31.54,P＜0.05).2组痉挛改善有效率均高于90％,组间无明显差异(91.67％ vs.95.83％,P＞0.05).2组联带运动均有改善,但无明显差异(83.33％ vs.58.33％,P＞0.05).5个自主动作相关联带运动改善方面仅发现原发性面肌痉挛患者噘嘴引起联带运动的改善有明显差异(P＜0.05).结论 BoNT-A可以显著改善原发性与面瘫后面肌痉挛患者的痉挛严重程度,在联带运动严重程度改善方面有一定疗效.原发性面肌痉挛患者噘嘴引起的联带运动在BoNT-A治疗后显著改善.

This study aimed to evaluate the usefulness of [99mTc]sestamibi ([99mTc]MIBI) single photon emission computed tomography (SPECT)/X-ray computed tomography (CT) imaging for the identification of dystonic muscles in primary cervical dystonia (PCD) patients who underwent botulinum neurotoxin type A (BoNT-A) therapy. Thirty-six patients with PCD and 10 healthy subjects (control group) who underwent [99mTc]MIBI SPECT/CT were enrolled. The image characteristics of dystonic muscles and normal muscles were evaluated. Muscle/background ratio (MBR) of six representative muscles was calculated for dystonic muscles in PCD group and normal muscles in control group. In PCD patients, target muscles injected with BoNT-A were selected by clinical evaluations and the results of needle electromyography (EMG) were considered as the gold standard. The sensitivity, specificity, and diagnostic efficacy of SPECT/CT were obtained from the receiver operator characteristic (ROC) curve. Twenty-four PCD patients were included in our study eventually, because three PCD patients whose follow-up were lost and 9 PCD patients whose maximum reduction of Tsui scale scores was < 80 % were ruled out. Normal muscles of healthy subjects showed mild symmetrical radioactivity distribution, while in PCD patients, [99mTc]MIBI uptake in dystonic muscles abnormally increased. The mean MBRs of dystonic muscles were significantly higher than those of normal muscles. The sensitivity, specificity, and area under the curve (AUC) of SPECT/CT were 93.2 %, 88.5 %, and 0.908, respectively. Our study indicated that [99mTc]MIBI SPECT/CT may be a useful method for identifying dystonic muscles and a guide to BoNT-A therapy in PCD patients.

Introduction 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 99m technetium-sestamibi single-photon emission computed tomography in identifying target muscles in cervical dystonia. Methods In the study group (n = 18), target muscles were selected according to clinical evaluation combined with 99m 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. Results 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. Conclusion 99m 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.

Botulinum toxin (BT) is the treatment of choice for hemifacial spasm (HFS). When BT is injected into the affected side, patients may experience increased facial asymmetry. We wanted to evaluate in a prospective, randomised, placebo-controlled study whether bilateral BT injections may reduce this facial asymmetry. For this, we treated 19 HFS patients with unilateral and 24 with bilateral BT therapy using CBTX-A (Lanzhou Biological Products Institute, Lanzhou, China). BT doses on the affected side were standard doses, on the non-affected side they were one-third of those. Facial asymmetry was studied with the Sunnybrook facial grading system (SFGS), the Facial Clinimetric Evaluation Scale (FaCE), the Symmetry Scale for Hemifacial Spasm (SSHS) and a self-assessment scale. As shown in SFGS and SSHS, bilateral BT therapy reduces facial asymmetry, whilst unilateral BT therapy increases it. Both effects are more pronounced during voluntary facial movements than at rest. BT effect delay, BT effect duration, adverse effect frequency and severity were not affected. FaCE total score, some of its subscores and the self-assessment scale did not show an effect. Bilateral BT therapy may improve the outcome of BT therapy for HFS without producing additional adverse effects. This strategy, however, raises drug costs (by about a third). Using even higher doses in the non-affected side may intensify the improvement even further. Future studies may also monitor the patient's quality of life and the naïve public's overall perception of the patient's facial expression.