Project description:BACKGROUND:Botulinum toxin injection chemodenervation is a well-established intervention for adult strabismus, and has also been recognised as an effective alternative to routine incisional surgery for paediatric disease. We aimed to investigate the temporal patterns of practice, indications and outcomes of chemodenervation for paediatric strabismus at national and tertiary centre level. METHODS:Retrospective study using routinely collected patient data: Hospital Episode Statistics (HES) data were used to identify children undergoing non-incisional strabismus procedures in England from 2007 to 2016. Single-centre retrospective data on children undergoing botulinum toxin injections (Dysport® 2.5 units/ 0.1ml) as an isolated intervention (not involving incisional procedures) was undertaken to identify indications and outcomes. Successful outcome was defined as deviation <11 prism dioptres (PD). RESULTS:Between 2007 and 2016, there was no increase in the proportion of childhood strabismus involving non-incisional procedures. Amongst 150 children undergoing chemodenervation for strabismus within the tertiary centre, the most common diagnoses were acute onset esotropia (n = 34), infantile esotropia (n = 16) and consecutive exotropia (n = 15). Median age at injection was 8.5 years (range 0.9-15 years), and median follow up 12 months (6 months-11 years). Success rates differed by diagnosis, from 66% (non or partially accommodative esotropia) to 0% (congenital cranial disorders). Adverse events were seen in 62/150, 41%, most commonly transient ptosis (39%, n = 58). Overcorrection was seen in 14/119, 13%. Mild subconjunctival haemorrhage (n = 2) was the only other adverse event. CONCLUSIONS:Botulinum toxin for childhood strabismus has an acceptable safety profile, and considerable potential therapeutic benefit. However, nationally there has been no increased uptake of chemodenervation non-incisional procedures. Further prospective studies are necessary to understand the predictors of outcome within the separate clinical subgroups, to guide clinical decision making.
Project description:The aim of this study is to review our longitudinal experience with onabotulinumtoxinA (onaBoNT-A) injections for medically refractory hand tremor. We performed a retrospective review of our database of patients treated with onaBoNT-A for hand tremor evaluated between 2010 and 2018 in at least 2 sessions with follow-up. The majority were injected into the forearm flexors (FF), although treatment was individualized. During the specified period, 91 patients (53 essential tremor, 31 dystonic tremor, 6 Parkinson's disease tremor, and 1 cerebellar outflow tremor) met our inclusion criteria. The mean age (SD) was 64.8 years (12.8), and mean duration of follow-up was 29.6 months (25.1) with mean of 7.7 (6.3) treatment visits. FF were injected in 89 (97.8%) patients, exclusively in 74 (81.3%), and 15 (16.5%) were injected in FF and other muscles. EMG guidance was used in 5 patients (5.5%). On a 0⁻4 "peak effect" rating scale (0 = no effect, 4 = marked improvement in severity and function), 80.2% and 85.7% of patients reported moderate or marked improvement (score 3 or 4) at their first and last follow-up visit, respectively. There was no statistically significant difference in the outcomes between first and last visit: average "peak effect" rating score (3.2 versus 3.4), "global" rating score (3.0 versus 3.2), latency of response (4.5 versus 3.8 days), and total duration of response (12.7 versus 12.8 weeks), except onaBoNT-A dose (65.0 versus 78.6 U/limb, p = 0.002). Of 1095 limb injections, there were 134 (12.2%) non-disabling and transient (mean 36 days) adverse events (132 limb weakness, 2 pain). OnaBoNT-A injections are safe and effective in the treatment of hand tremor.
Project description:BackgroundMany researchers have noticed that there is an increasing trend of Bielschowsky acquired comitant esotropia (ACE) in recent years related to excessive near work, but the exact pathogenesis and treatment methods have not been reported yet. Therefore, we aimed to characterize the clinical features of this ACE in adults and to evaluate the efficacy of botulinum toxin (BTX) injections in these patients.MethodsThis was a prospective consecutive case series of 47 patients with Bielschowsky ACE. BTX was injected bilaterally into the medial rectus muscle of 45 patients, and twenty-seven of them (27/45) completed 10 months of follow-up after their last injection. Angle of deviation, fusion, stereopsis, subjective assessment of diplopia were documented before and after BTX treatment, and repeated measures data were compared by the Wilcoxon signed-rank test or Analysis of variance. The relationship between BTX dosage and corrected esotropia was explored by the Regression analysis. Meanwhile, possible risk factors for ACE including time spent on near work, refraction error, patients' personality, glasses wearing habits and duration of symptoms were recorded and analyzed with General Linear Models.ResultsThe patients aged 32.32 ± 10.96 (range 15-53) years spent 8.34 ± 2.38 h on near work each day, and most myope habitually removed their glasses at near. Their chief complaint was distance diplopia, with more significant esotropia at distance (around 20 PD) than at near. This series of patients also exhibited perfectionist tendencies. However, most patients achieved orthophoria after BTX treatment, only with a mild residual esotropia (+ 3.96 ± 5.79 PD), which left them asymptomatic most of the time.ConclusionThis group of ACE patients was characterized by diplopia with more significant esotropia at distance. Besides excessive near-work, habitually removing myopic glasses and perfectionist tendencies may also contribute to this type of ACE. Fortunately, bilateral BTX injection safely and effectively reduced the esotropia with complete resolution of symptoms, especially for those treated at an early stage.
Project description:Primary lingual dystonia is a rare condition, especially when it is only induced by speaking. Trihexyphenidyl failed to improve the symptoms. Several case series have demonstrated the effectiveness of botulinum toxin injection for the management of focal lingual movement disorders. Only 1 case of botulinum toxin injection for primary lingual dystonia induced by speaking has been reported, but this treatment has limited effectiveness. Our patient was treated with botulinum toxin using a superficial approach for injection into the tongue with continuing excellent results. Lingual botulinum toxin injection is a fairly simple, safe and viable treatment option for lingual dystonia induced by speaking.
Project description:Dysphagia associated with upper esophageal sphincter (UES) dysfunction remarkably affects the quality of life of patients. UES injection of botulinum toxin is an effective treatment for dysphagia. In comparison with skeletal muscles of the limb and trunk, the UES is a special therapeutic target of botulinum toxin injection, owing to its several anatomical, physiological, and pathophysiological features. This review focuses on (1) the anatomy and function of the UES and the pathophysiology of UES dysfunction in dysphagia and why the entire UES rather than the cricopharyngeal muscle before/during botulinum toxin injection should be examined and targeted; (2) the therapeutic mechanisms of botulinum toxin for UES dysfunction, including the choice of injection sites, dose, and volume; (3) the strengths and weaknesses of guiding techniques, including electromyography, ultrasound, computed tomography, and balloon catheter dilation for botulinum toxin injection of the UES.
Project description:Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive disorder characterized by typical facial dysmorphism, generalized muscle stiffness, joint contracture, and skeletal abnormalities. This condition is caused by mutations in the heparan sulfate proteoglycan 2 (HSPG2) gene, which encodes perlecan, a component of the basement membrane. The management of patients with SJS primarily aims to alleviate symptoms related to muscle stiffness. In this report, we describe a male patient with SJS type 1A. Trio whole-exome sequencing identified a pathogenic mutation (NM_001291860.1: c.10897C>T; p.Arg3633Ter) and variants of unknown significance (NM_001291860.2: c.413+10G>T). The patient experienced difficulty in opening his eyes and mouth, which significantly limited his daily activities. Botulinum toxin A injection was administered and demonstrated significant clinical improvement after the treatment.