Blepharospasm Disability Index Research Articles

BOTULINUM TOXIN A FOR BLEPHAROSPASM WITH DRY EYE: A SYSTEMATIC REVIEW

Introduction & Objectives : Essential blepharospasm is a neurological condition that is defined by uncontrolled and abnormal eyelid closure from a central cerebral disorder. Between 40%-85% of blepharospasm occurs in conjunction with dry eye symptoms. Botulinum neurotoxin (BoNT) injections are commonly used treatment for reducing spasms in blepharospasm. Furthermore, it is often suggested for dry eye therapy due to their ability to reduce lacrimal drainage. The purpose of this review is to assess the effectiveness of BoNT treatment for blepharospasm with dry eye symptoms.
 Methods : A literature research was conducted on online databases such as PubMed, Cochrane library and Google scholar. All studies of BoNT and dry eye with full-text journals published in English within the last 10 years were included. We assessed Tear Breakup Time (TBUT), Schirmer Test, and Blepharospasm Disability Index (BSDI).
 Results : 204 patients from 6 publications were analyzed. In five trials, the BoNT treatment statistically significant increased TBUT (p<0,5), with mean final TBUT 5,7 ±1.1 s. Furthermore, the Schirmer test also increased with the mean final score 6,29 ± 4,6 mm. The mean BSDI score dropped by 6,05 ± 3,7 in two studies.
 Conclusion : It is confirmed that BoNT injection is an effective treatment for essential blepharospasm with dry eye symptoms. Clinicians should be screened for dry eye in all blepharospasm patients prior to BoNT treatment.

Blepharospasm Secondary to Deep Brain Stimulation of the Subthalamic Nucleus in Parkinson Disease: Clinical Characteristics and Management Outcomes.

Deep brain stimulation of the subthalamic nucleus (STN-DBS) is an effective treatment for patients with motor symptoms of Parkinson disease but can be complicated by disabling blepharospasm and apraxia of eyelid opening (ALO). Currently, there is no clear consensus on optimal management, and addressing these issues is further hindered by systemic morbidity and resistance to treatments. We aim to describe the different phenotypes of these eyelid movement disorders, to report our management approach and patient responses to treatment. A retrospective case series of all patients with blepharospasm/ALO secondary to STN-DBS that were treated at a tertiary center between 2011 and 2020. Data collected included date of Parkinson diagnosis, date of DBS surgery, date of development of blepharospasm/ALO symptoms, STN-DBS stimulation settings, and treatment given. Patients' symptoms before and after treatment were measured using the blepharospasm disability index and Jankovic Rating Scale. Five patients were identified with eyelid movement disorders secondary to STN-DBS. All patients had moderate-to-severe symptoms at presentation. Four patients received periocular botulinum toxin injections. Three patients underwent surgery in the form of frontalis suspension or direct brow lift with or without upper lid blepharoplasty. All reported an improvement in symptoms following treatment. A multimodality, patient-specific approach is required in the treatment of blepharospasm/ALO secondary to STN-DBS. Botulinum toxin injections can be effective, but patients may require surgery if toxin treatment alone becomes ineffective. Tailoring treatment to individual needs can result in a measurable improvement in symptoms.

Blepharospasm: an analysis of typical medical practice and the effectiveness of therapy

Introduction. Blepharospasm (BS) is a form of focal dystonia in which botulinum therapy (BT) has been used successfully, however, data on typical practice of patient management and the effectiveness of long-term BT in BS in our country are insufficient.Aim. To analyse the typical medical practice of management of patients with BS and the effectiveness of long-term BT.Materials and methods. A total of 50 patients with idiopathic BS, mean age 58 years (of which 37 were women) were included in the study. All patients were treated with botulinum toxin type A. Typical medical practice, erroneous diagnosis, time required to reach a correct diagnosis, severity of dystonia, and quality of life before BT and during a three-year prospective observation were analysed. The severity of dystonia was determined using the BS Disability Index (BSDI), and the quality of life was measured using the EQ-5D questionnaire.Results. The correct diagnosis was reached, on average, in 3 years. Upon onset of symptoms the majority of patients (84%) visited an ophthalmologist. Eye diseases and myasthenia gravis were the most common primary erroneous diagnosis in BS, which were treated with inadequate symptomatic therapy. After one-month BT, it was noted that the disability index decreased from an average of 13.6 ± 5.1 to 2.7 ± 2.3 scores (p < 0.001) according to BSDI and the quality of life increased from 59.9 ± 8.2 to 82.6 ± 6.4 scores (p < 0.001) according to the results of EQ-5D. Repeated BT injections (2–6 times a year) conducted for 3 years also resulted in essentially positive effect without significant adverse events. At the end of three years, the disability index decreased to 10.7 ± 4.7 scores, and the improvement in the quality of life reached 81.8 ± 5.9 scores according to the results of EQ-5D (p < 0.001).Conclusion. The low awareness of doctors, especially ophthalmologists, about the symptoms of BS and the widespread use of ineffective drugs was noted. The long-term regular (2–6 times a year) use of BT reduces the symptoms of the disease, improves the quality of life of patients and has no significant side effects.

Relations between nonmotor manifestations and motor disorders in patients with benign essential blepharospasm.

To evaluate the relations between nonmotor manifestations (dry eye, mood disorders, and sleep disturbance) and motor disorders in patients with benign essential blepharospasm (BEB), and to determine whether relieving motor disorders by botulinum neurotoxin can improve the nonmotor manifestations. In this prospective case series study, 123 BEB patients were enrolled for evaluations. Among them, 28 patients underwent botulinum neurotoxin therapy and attended another two postoperative visits at 1month and 3months. Motor severity was measured with Jankovic Rating Scale (JRS) and Blepharospasm Disability Index (BSDI). We assessed dry eye using OSDI questionnaire, Schirmer test, tear break-up time (TBUT), tear meniscus height, lipid layer thickness (LLT) and corneal fluorescence staining. Zung's Self-rating Anxiety and Depression Scale (SAS, SDS) and Pittsburgh Sleep Quality Index (PSQI) were for mood status and sleep quality evaluations. Patients with dry eye or mood disorders had higher JRS scores (5.78 ± 1.13, 5.97 ± 1.30) than those without (5.12 ± 1.40, 5.50 ± 1.16; P = 0.039, 0.019, respectively). BSDI values of patients with sleep disturbance (14.61 ± 4.71) was higher than those without (11.89 ± 5.44, P = 0.006). Correlations were found between JRS, BSDI and SAS, SDS, PSQI, OSDI, TBUT. Botulinum neurotoxin effectively relieved JRS, BSDI and improved PSQI, OSDI, TBUT, LLT (8.11 ± 5.81, 21.77 ± 15.76, 5.04 ± 2.15s, 79.61 ± 24.11nm) at the 1-month visit compared to baseline (9.75 ± 5.60, 33.58 ± 13.27, 4.14 ± 2.21s, 62.33 ± 22.01nm; P = 0.006, < 0.001, = 0.027, < 0.001, respectively). The BEB patients with dry eye, mood disorders, or sleep disturbance had more severe motor disorders. Motor severity was associated with the severity of the nonmotor manifestations. Relieving motor disorders by botulinum neurotoxin was effective in improving dry eye and sleep disturbance.

Outcomes of pallidal deep brain stimulation for treating pure blepharospasm

Objective: Blepharospasm (BSP) is a disease in which the closure rate of the bilateral eyelids increases, mainly due to involuntary contraction of the orbicularis oculi, procerus, and corrugator muscles. The objective of this study was to report postoperative outcomes after deep brain stimulation (DBS) in 10 cases of pure BSP after at least 12 months of follow-up.Methods: Ten patients with pure BSP who underwent bilateral globus pallidus interna (GPi) DBS at The Catholic University of Korea, Incheon St. Mary’s Hospital, between 2019 and 2021 were included. The Burke-Fahn-Marsden dystonia rating scale (BFMDRS), Blepharospasm Disability Index, and Jankovic Rating Scale were used for analysis before surgery, at 6 months of follow-up as short-term outcomes, and at follow-up over 1 year (12–37 months) as long-term results. Results: The median age of patients at surgery was 56.5 years (interquartile range [IQR], 50.5–65.8 years) and the median length of time from disease onset to the time of surgery was 58.0 months (IQR, 46.8–64.3 months). The median postoperative follow-up period was 22.5 months (IQR, 15.3–29.0 months). The median BFMDRS movement subscale scores at the three time points (preoperative baseline, 6 months, and over 1 year of follow-up) were 7.0 (IQR, 6.0–8.0), 4.5 (IQR, 3.9–6.0; 35.7% improvement, p&lt;0.001), and 3.8 (IQR, 2.8–5.3; 45.7% improvement, p=0.002), respectively.Conclusion: Bilateral GPi DBS for pure BSP can be effective if conservative treatment options fail. Its benefit is not only observed in the short term, but is also maintained during long-term follow-up.

Measurement Properties of Clinical Scales Rating the Severity of Blepharospasm: A Multicenter Observational Study.

Several scales have been proposed to clinically evaluate the Motor Severity of Blepharospasm (BSP) but information about their measurement properties as a multicenter instrument is limited. To compare the measurement properties of four clinical scales in rating the severity of BSP in a large sample of patients from multiple sites. The Burke-Fahn-Marsden Scale (BFMS), the Global Dystonia Severity Rating Scale (GDRS), the Jankovic Rating Scale (JRS), and the Blepharospasm Severity Rating Scale (BSRS) were administered to 211 patients across 10 sites who were also requested to self-complete the Blepharospasm Disability Index (BDI). Measurement properties to be assessed included inter-/intra-observer agreement, item-to-total correlation, internal consistency, floor and ceiling effect, convergent/discriminant validity, and adherence to the distribution of BDI. The BFMS had unsatisfactory measurement properties, the GDRS had acceptable reliability but other properties could not be completely testable; the JRS had satisfactory measurement properties but the scale did not accurately reflect the distribution of disability parameter (BDI) in the sample, and the BSRS had satisfactory measurement properties and also showed the best adherence to the distribution of BDI in the assessed sample. The comparison of the measurement properties of four rating scales to assess the motor state of the BSP in a large sample of patients from multiple sites showed that the GDRS should be used to simultaneously assess BSP and dystonia in other body parts, while the JRS (easier to use) and BSRS (better to discriminate severity) should be used to assess BSP alone.

A dual dopaminergic therapy with L-3,4-dihydroxyphenylalanine and chlorpromazine for the treatment of blepharospasm, a focal dystonia: Possible implications for striosomal D1 signaling

Impairment of balanced activity between dopamine D1 and D2 receptor functions in the striatum, particularly in striatal functional subdivisions (i.e., striosome and matrix compartments), has been proposed to underlie dystonia genesis. This study was undertaken to examine the therapeutic effect of dual dopaminergic modulation with L-3,4-dihydroxyphenylalanine (L-DOPA) and chlorpromazine (CPZ) in patients with blepharospasm, a focal dystonia. For this purpose, Dopacol tablets™ (L-DOPA 50 mg plus carbidopa 5 mg) and Wintermin™ (CPZ phenolphthalinate 180 mg/g) were used. Clinical evaluations were performed before and after an 8-week drug treatment interval using the Visual Analog Scale (VAS), Blepharospasm Disability Index (BSDI), modified VAS (mVAS), and Jankovic Rating Scale (JRS). The data were analyzed using non-parametric statistics. Results showed that in patients (n = 7) with blepharospasm, dystonia symptoms were significantly alleviated by the administration of both Dopacol tablets™ (one tablet × 3/day) and CPZ (5 mg × 3/day), as determined using the VAS, BSDI, mVAS, and JRS. In contrast, there was no improvement of dystonia symptoms in patients (n = 7) who ingested Dopacol tablets™ (one tablet × 3/day) alone, nor in those (n = 7) who ingested CPZ (5 mg × 3/day) alone. Thus, dual pharmacotherapy with L-DOPA and CPZ can exert a therapeutic effect on blepharospasm, suggesting that dystonia symptoms can be attenuated through dopaminergic modulation with inducing an increase in striatal D1-signals. Since dopamine D1 receptors are heavily enriched in the striosome compartment in the “human” striatum, our results also suggest that striosomal loss of D1-signaling may be important in the pathogenesis of dystonia.

The impact of the reclusion on patients with blepharospasm during the COVID19 pandemic

BackgroundBlepharospasm (BS) is a focal dystonia that can be treated successfully with Botulinum toxin (BoNT). During the reclusion due to the Covid 19 pandemic many patients missed the scheduled treatment. ObjectivesAim of the study is to evaluate Level of Disability (LoD) related to BS during the lockdown period. MethodsLoD was assessed by an adapted version of Blepharospasm Disability Index (4iBSDI) during reclusion (T1), and three months after the first injection following the lock down phase (T2). 4iBSDI scores were compared between T1 and T2, a correlation between the change of LoD in the two periods (t-delta) and patients’ clinical data was analyzed. ResultsLoD was not modified between the two periods in most of the patients and it was reduced at T1 in almost one third of the participants. No correlation between t-delta and clinical data was found. ConclusionsLoD did not increase during the lock down period in most of BS patients although BoNT treatment was suspended. Environmental and psychosocial factors may contribute to determine the LoD due to BS.

P 3 Mental rotation performance in patients with focal dystonia is associated with their cognitive state

Introduction: Mental rotation (MR) of body parts is based on the imagination of actual movements. It is assumed to require activation of brain areas responsible for planning and execution of movements. From previous studies with partly contradictory results, it is still unclear whether patients (pts) with idiopathic focal dystonia have an impairment of MR of body parts. We investigated MR of body parts and non-corporal objects in pts with cervical dystonia (CD) and blepharospasm (BS) and assessed potential confounders of MR performance like cognitive deficits. Patients and Methods: 23 pts with CD and 23 healthy subjects as well as 21 pts with BS and 19 pts with hemifacial spasm (HS) were compared. Matched groups did not differ significantly regarding gender distribution, age, or duration of schooling. Besides handedness, dexterity and general reaction time, cognitive status was assessed using the Montreal Cognitive Assessment (MoCA). Severity of diseases was evaluated by using the Toronto Western Spasmodic Torticollis Rating Scale, Blepharospasm severity scale, Blepharospasm disability index and Jancovic rating scale. In the MR test, photographs of body parts (head, hand, or foot) and other objects such as cars were presented on a screen. Images of the head had a black mark on either the right or left eye, the car had a black mark on either the right or left headlight. The images were presented at six different angles rotated around their own axis. Subjects were asked to select by keystroke whether the right or left hand/foot/headlight was marked. Both speed and correctness of responses were evaluated. For statistical analysis, mixed ANOVA and Spearman-Rho correlation coefficient were applied. Results: In the MR test, pts with CD showed a significantly longer reaction time (RT) compared to healthy controls for both body parts and non-corporal objects (p=0.003, p=0.027). There was no significant difference concerning accuracy except for cars (p=0.048). Furthermore, pts with CD achieved a significantly lower score in the MoCA test compared to controls (p<0.001). Negative correlations were found between MoCA results and RTs in the MR test for all items (hand: r=-0.434, p<0.001, foot: r=-0.419, p<0.001, head: r=-0.363, p=0.001, cars: r=-0.425, p<0.001), whereas there was no correlation between severity of diseases and RTs. No significant differences were observed between pts with B and HS. Conclusion: In our study, prolonged RTs in pts with CD in the MR task are not limited to images of body parts. Rather, we found a significant association of MR performance with cognitive fitness. However, it remains unclear whether increased RTs in pts result from cognitive deficits or from dystonia itself. The former would be supported by the lack of difference in the MR task between BS and HS, whose MoCA results did not differ. Future studies should investigate MR performance in dystonia pts and subjects with comparable cognitive state.

A patient-initiated treatment model for blepharospasm and hemifacial spasm: a randomized controlled trial

BackgroundTo test, in a two-arm, single center, superiority, randomized controlled trial, the effectiveness of and costs associated with a patient-initiated treatment model for people with hemifacial spasm (HFS) and blepharospasm (BEB) in comparison to usual care.MethodsOne hundred and thirty patients with HFS or BEB, aged 18 years or over, were recruited from a nurse-led botulinum toxin type A clinic at an eye hospital in the United Kingdom (UK), completed baseline measures and were randomized (1:1). The intervention group determined their own botulinum toxin type A (BoNT/A) treatment schedule during the trial period (9 months) and received an information leaflet with a “hotline” number to book an appointment. Usual care appointments were scheduled by treating clinicians. Data analysts were blind to study group. The primary outcomes were disease severity and functional disability, as measured by the Jankovic Rating Scale and Blepharospasm Disability Index, respectively. Secondary outcomes included quality of life, anxiety and depression, satisfaction with care, confidence in the service, economic costs and employment days lost.ResultsSixty-five patients were randomized to each group. The intervention demonstrated no statistically significant difference to usual care for any of primary outcomes. On secondary outcomes the levels of anxiety differed significantly (F2, 142.39 = 1.65, p = 0.02), with the intervention arm exhibiting a decrease and the control arm an increase (Hedges’ g = − 0.26 [99% CI -0.83, 0.32]). No other statistically significant differences were found for secondary outcomes. Overall healthcare costs and costs to the patient were on average £198.95 less (95% CI -£256.76, £654.67; p = 0.10) per participant for those in the intervention compared to usual care, although this finding was not significant.ConclusionsWe did not observe differences between the patient-initiated treatment model and usual care for people with BEB or HFS, on any primary outcome measure, quality of life, or depression. The patient-initiated treatment model may, however, have the potential to save healthcare costs and reduce anxiety. Patients using this new model were also equally as satisfied in the service and confident in their care as those receiving treatment as usual.Trial registrationClinicaltrials.gov ID NCT02577224, 16th October 2015.

Comprehensive Korean Medicine Treatment for Benign Essential Blepharospasm in a Patient Responding Poorly to Botulinum Toxin Treatment

Benign essential blepharospasm (BEB) is a condition/disease which involves involuntary muscle contractions causing the eye to repetitively close, making it impossible for the patient to perform daily activities. This study reports Korean medicine treatment of a rare case BEB in a 34-year-old male patient whose symptoms showed minimal improvement following botulinum toxin injection (the standard treatment). Acupuncture, pharmacopuncture, and herbal medicine treatment for 11 days resulted in clinical improvement for all symptoms as assessed by using the Jankovic Rating Scale, Blepharospasm Disability Index, numerical rating scale, duration of spasms lasting more than 1 hour per day, and number of spasms lasting less than 1 minute per day. This case report suggests that comprehensive Korean medicine treatment could be a treatment option for BEB in patients who do not respond well to botulinum toxin injection.

THE EFFECT OF BOTULINUM TOXIN TYPE A IN PATIENTS WITH ESSENTIAL BLEPHAROSPASM.

The aim of this study was to determine the effect of botulinum toxin type A and satisfaction in patients with essential blepharospasm. The study included 12 subjects suffering from essential blepharospasm who received therapy with botulinum toxin type A injections. Respondents were given a Blepharospasm Disability Index (BSDI) survey before and three weeks after the procedure with questions related to quality of life. They were also given a Jankovic Rating Scale with questions related to the severity and frequency of symptoms. The overall level of difficulties was reduced, meaning the quality of life increased after the application of botulinum toxin, and the study found that the increase was statistically significant (p = 0.003). The severity of symptoms after the application of botulinum toxin was reduced, which meant that there was a decrease in the severity of the difficulties, and the study found a statistically significant difference (p=0.003). The frequency of symptoms was reduced after botulinum toxin administration, and the study revealed a statistically significant difference (p = 0.012). From the results of this study, we can determine that the use of botulinum toxin type A increases the quality of life and that the severity and frequency of symptoms are statistically significantly reduced.

Management of Meige’s syndrome by facial and trigeminal nerve combing followed by transplantation of autologous adipose-derived mesenchymal stem cells: a prospective nonrandomized controlled study

Increasing attention has recently been focused on reducing abnormal neuroexcitability in patients with Meige’s syndrome using nerve combing surgery. However, nerve injury caused by nerve combing is of critical concern. Animal studies have shown that stem cells can repair cranial nerves; autologous adipose-derived mesenchymal stem cells have been proved to be safe and effective in clinical trials. A total of 38 patients with Meige’s syndrome were enrolled in this prospective nonrandomized controlled study and divided into a non–stem cell group (n = 30) and a stem cell group (n = 8). Patients in the non-stem cell group underwent facial and trigeminal nerve combing only; patients in the stem cell group underwent adipose-derived mesenchymal stem cell implantation after facial and trigeminal nerve combing. A blepharospasm disability index score was used to evaluate effectiveness of the surgery, and a House–Brackmann grade was used to evaluate facial nerve injury. These data were recorded before the operation and at 7 days, 3 months, and 6 months after the operation. The overall improvement percentage of blepharospasm was 93% at 6-month follow-up in the non-stem cell group. A greater number of nerve combing events during the operation led to better outcomes but increased risk of facial paralysis. Patients in the stem cell group had better facial nerve function at the 6-month follow-up (House–Brackmann grade, P = 0.003) and better blepharospasm improvement at 3 and 6 months than those in the non–stem cell group (blepharospasm disability index score, P = 0.003 and P < 0.001, respectively). Cerebrospinal fluid protein analysis showed that levels of several cytokines were significantly increased after adipose-derived mesenchymal stem cell transplantation, including interleukin-6 (P < 0.01) and interferon gamma-induced protein 10 (P < 0.0001) and the growth factors insulin-like growth factor-1 (P < 0.0001), insulin-like growth factor-binding protein-1 (P < 0.0001), growth/differentiation factor-15 (P < 0.001), and angiopoietin-like 4 (P < 0.001). Facial and trigeminal nerve combing combined with adipose-derived mesenchymal stem cell transplantation is a safe and effective remedy to improve recovery from Meige’s syndrome.

Impact of Upper Eyelid Surgery on Symptom Severity and Frequency in Benign Essential Blepharospasm.

Objective To assess the impact of periocular surgery, other than orbicularis stripping, on the severity and frequency of blepharospasm symptoms.Methods Consecutive patients with benign essential blepharospasm (BEB) who underwent eyelid/eyebrow surgery with the aim of improving symptoms were retrospectively reviewed over a 5-year period. Patients who had completed the Jankovic Rating Scale (JRS) and Blepharospasm Disability Index (BDI) pre- and at least 3 months postoperatively were included.Results Twenty-four patients were included. JRS scores significantly improved from 7.0 preoperatively to 4.1 postoperatively (p < 0.001), and BDI scores significantly improved from 18.4 preoperatively to 12.7 postoperatively (p < 0.001); the mean percentage improvements were 41% and 30%, respectively. Patients were followed for a median of 24 months postoperatively.Conclusion Periocular surgery significantly reduced BEB symptoms in the majority (83%) of patients by an average of 33% and may therefore be offered for suitable patients. An important minority (17%) of patients experienced symptom worsening.

Botulinum Toxin in Management of Benign Essential Blepharospasm

The objective of this study is to evaluate effectiveness of botulinum toxin injection in treatment of benign essential blepharospasm. It is a prospective interventional study conducted in Ispahani Islamia Eye Institute and Hospital within the period of June 2018 to May 2020. In this study 102 patients, suffering from benign essential blepharospasm, were included. After taking standard precaution, botulinum toxin injection was injected at 5 to 6 sites of each eye with 2.5 units per site. Patients were followed up routinely on the following day, after 7 day, then after 1 month and 3 months. 65.68% patients are of 50 years or more with mean age 54.91 years. 35.29% patients are male and 64.70% are female. 62.74% patient had bilateral blepharospasm while 37.25% patient presented with unilateral symptoms. Useful effect of injection appeared within 48 hours in all patients. According to Blepharospasm disability index, 13.72% patient had minor blepharospasm (mean BSDI score 1to 2), 34.31% patient had moderate (mean BSDI score >2 to 3) and 51.96% patient had severe blepharospasm (>3 to 4). Baseline mean BSDI score before injection of 102 patients was 2.62. 7 days after injection, the score reduced to 1.46 and after 1month, score was 0.95. Minor transient side effect like ptosis, dry eye and lagophthalmos was found in very few patients. The useful effect of injection lasted for 3-4 months in majority of patients. This study explored Botulinum toxin as the first line treatment of benign essential blepharospasm,however, there are few limitations of this agent, including short-term effect, repeated application, and high cost.

Structural Brain Changes in Blepharospasm: A Cortical Thickness and Diffusion Tensor Imaging Study.

White matter abnormalities in blepharospasm (BSP) have been evaluated using conventional intra-voxel metrics, and changes in patterns of cortical thickness in BSP remain controversial. We aimed to determine whether local diffusion homogeneity, an inter-voxel diffusivity metric, could be valuable in detecting white matter abnormalities for BSP; whether these changes are related to disease features; and whether cortical thickness changes occur in BSP patients. Diffusion tensor and structural magnetic resonance imaging were collected for 29 patients with BSP and 30 healthy controls. Intergroup diffusion differences were compared using tract-based spatial statistics analysis and measures of cortical thickness were obtained. The relationship among cortical thickness, diffusion metric in significantly different regions, and behavioral measures were further assessed. There were no significant differences in cortical thickness and fractional anisotropy between the groups. Local diffusion homogeneity was higher in BSP patients than controls, primarily in the left superior longitudinal fasciculus, corpus callosum, left posterior corona radiata, and left posterior thalamic radiata (P < 0.05, family-wise error corrected). The local diffusion homogeneity values in these regions were positively correlated with the Jankovic rating scale (rs = 0.416, P = 0.031) and BSP disability index (rs = 0.453, P = 0.018) in BSP patients. These results suggest that intra- and inter-voxel diffusive parameters are differentially sensitive to detecting BSP-related white matter abnormalities and that local diffusion homogeneity might be useful in assessing disability in BSP patients.

IncobotulinumtoxinA for the Treatment of Blepharospasm in Toxin-Naïve Subjects: A Multi-Center, Double-Blind, Randomized, Placebo-Controlled Trial.

This study aimed to assess the efficacy/safety of incobotulinumtoxinA (Xeomin®, Merz Pharmaceuticals GmbH) in botulinum neurotoxin-naïve subjects with blepharospasm. Botulinum neurotoxin-naïve subjects (≥ 12months without botulinum neurotoxin treatment for blepharospasm) received single-dose incobotulinumtoxinA 50 U, 25U, or placebo. Subjects were followed for 6-20weeks (main period). Qualified subjects entered an open-label extension period and received another incobotulinumtoxinA injection (≤ 70 U). The primary efficacy variable was change from baseline in the Jankovic Rating Scale (JRS) severity subscore at the main period of week 6. Other efficacy variables included changes in the Blepharospasm Disability Index score and JRS frequency subscore and sumscore. Adverse events were monitored. Sixty-one subjects were randomized (main period: incobotulinumtoxinA 50 U, n = 19; incobotulinumtoxinA 25 U, n = 22; placebo, n = 20); 39 entered the open-label extension period (9, 14, and 16 subjects from the incobotulinumtoxinA 50 U, incobotulinumtoxinA 25 U, and placebo groups [main period], respectively, changed to open-label extension period dosing). A statistically significantly greater reduction in JRS severity subscore was reported for subjects receiving incobotulinumtoxinA 50 U versus placebo (ANCOVA, least square mean difference: -1.2, p = 0.0004). Subjects receiving incobotulinumtoxinA experienced improvements in other efficacy variables versus baseline and/or placebo. Sustained clinical improvements and low adverse event rates (22.2-42.1%) were observed. This is the second placebo-controlled, double-blind study that demonstrates favorable efficacy/safety of incobotulinumtoxinA in subjects with blepharospasm. IncobotulinumtoxinA is the first botulinum neurotoxin that could fulfill the American Academy of Neurology criteria for a Level A recommendation for blepharospasm.Trial registration ClinicalTrials.gov identifier, NCT01896895.

Functional blindness: Blepharospasm with apraxia of eyelid opening

Purpose: The purpose was to evaluate the effectiveness of protractor myectomy surgery in patients with severe blepharospasm whose daily life is severely impaired. Materials and Methods: Records of fifty patients with benign, essential blepharospasm were examined retrospectively and seven were included in the study. All patients underwent a complete ophthalmological and neurological examination. The severity and frequency of spasm was determined by Jankovic Rating Scale, ranging from 0 to 4, and six daily activities were determined by Blepharospasm Disability Index. The primary indication for protractor myectomy was poor response to serial botulinum toxin injections. The main outcome measure was based on whether the spasms were fully healed or the need for additional botox application. Results: Fourteen eyes of seven patients with significant functional disability were included in the study. Excessive blinking and visual disability were the most common symptoms seen in all patients, and all had previously used one or more medications. Myectomy surgery was performed in all patients who were resistant to serial botulinum toxin injections. Of the 14 eyelids, 85.7% showed resolution of spasms and no longer required botulinum toxin treatment following surgery. One patient with keratitis sequelae and dry eye was given botulinum 1 month after the surgery. One patient with residual blepharospasm in the lower eyelid underwent lower eyelid myectomy and blepharoplasty. Transient periorbital edema, hematoma, and echymosis developed following surgery. The patients were followed up for 2 years, and excellent cosmetic result with no contracture was observed in all patients. Conclusion: Protractor myectomy is an effective treatment modality in cases of blepharospasm with severe functional disability.

The first UK national blepharospasm patient and public involvement day; identifying priorities

ABSTRACT Purpose We describe the planning and outcomes of the first ‘Blepharospasm Day’ in the UK. Blepharospasm is a distressing condition for patients and carers. Our ‘patient and public involvement’ event aimed to: cultivate a more informed patient group via active dialogue, help clinicians more effectively prioritise research and to facilitate peer-to-peer support for affected patients and public. Design A national one-day event was organised by the oculoplastics department at Moorfields Eye Hospital. The event was divided into informative lectures delivered by professionals and a patient panel, during which patients shared their experiences and expectations. Methods Data were collected from a variety of sources including: an interactive voting “LiveWall” poster, a pre-event questionnaire; “living with Blepharospasm”, transcripts from patient panel discussions; and a feedback questionnaire. Results The event was well-received with 100% of respondents rating it good or excellent. Four research themes were identified: “aetiology”, “alternative treatments”, “faster, more accurate diagnosis”, and “symptom control”. Delegates’ self-reported knowledge of blepharospasm increased significantly after the event. Limitations of the BdSI severity-assessment tool were noted with 22% of respondents failing to utilise it appropriately. Conclusion Through our innovative “Blepharospasm Day”, patient’s priorities for research were identified, delegates understanding of blepharospasm increased and an independent blepharospasm patients-representatives’ group was established; a first in the UK. Furthermore, short-fallings identified in the BdSI tool highlight the need for better severity-assessment tools. We demonstrate the benefits of the ‘patient and public involvement’ approach in the management of complex conditions such as blepharospasm. Abbreviations PPI: Patient and public involvement; SLV-PSP: sight loss and vision sector – priority setting partnership; BRC: Biomedical Research Centre; NIHR: National Institute for Health Research; BsDI: Blepharospasm Disability Index

Sensory Trick Frames: A New Device for Blepharospasm Patients.

ObjectiveTo determine whether the use of unique customized spectacles provided with modified side arms may be helpful in reducing benign essential blepharospasm (BEB) in patients describing periocular sensory tricks (ST).MethodsA prospective descriptive study of patients with BEB with positive periocular or temporal region ST phenomenon response under the care of the Botox Clinic at Moorfields Eye Hospital, London, UK. Nine consecutive patients with BEB describing ST were recruited, and the disease frequency and severity were assessed with the Jankovic Rating Scale (JRS) and the Blepharospasm Disability Index (BSDI) before and after the use of the sensory trick frames (STF).ResultsA reduction in the score was noted in both severity (p = 0.0115) and frequency patterns (p = 0.0117) in the JRS in patients using the STF. A significant reduction of the BSDI score was also observed (p = 0.0314).ConclusionAll the patients selected and fitted with the STF had a reduction in spasms and related symptoms. This new device may be helpful in some selected BEB patients who previously responded positively to periocular pressure alleviating maneuvers.