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AbstractPurposeMethodsResultsConclusionTo assess the retinal and choroidal microvascular changes in patients with benign essential blepharospasm (BEB) and to investigate the factors that may be effective on microvascularity.This study included patients with BEB and healthy controls. All participants underwent a comprehensive examination followed by optical coherence tomography angiography (OCTA). Macular vascular perfusion density (VPD), foveal avascular zone (FAZ), and subfoveal choroidal thickness (CT) were measured. The clinical findings of the BEB group and the administration of botulinum toxin A (BTx-A) prior to OCTA imaging were recorded retrospectively from the medical records.A total of 16 patients with BEB and 20 healthy controls were included in this study. VPD values in all quadrants of the superficial and deep macular capillary plexus were significantly different in the BEB group compared to the control group (each p-value <0.05). In the choriocapillaris layer, VPD was significantly higher in the superior, nasal, and temporal quadrants compared to the control group (each p-value <0.05). The BEB group exhibited significantly smaller superficial and deep FAZ values compared to the control group, consistent with the observed increase in vascular density (each p-value <0.05). CT was thinner in the BEB group, although not statistically significant (p = 0.138). No correlation was found between the total dose of BTx-A administered to the BEB group and OCTA parameters (each p-value >0.05)Benign essential blepharospasm may be associated with increased retinal vascular density in the superficial and deep capillary plexus and decreased FAZ area. [ABSTRACT FROM AUTHOR]

Background: This study investigates the effect of botulinum toxin A on lipid layer thickness (LLT) and blink dynamics in patients with benign essential blepharospasm (BEB) compared to dry eye disease (DED) patients. Methods: We reviewed the medical records of patients with dry eye disease (DED) and BEB treated with botulinum toxin A (BoT A) injections. Data on demographics, lipid layer thickness (LLT), meiboscore, and blink dynamics measured using a LipiView II interferometer before and 2 months after BoT A were collected. Results: Each 28 eyes from 28 patients with BEB and age- and sex-matched patients with DED were included. When comparing blink dynamics, complete blink rate was significantly higher in the pre-injection BEB group compared to the DED group (5.25 ± 4.32 times/20 s vs. 2.43 ± 2.82 times/20 s, p = 0.0055). In tear film lipid profiles analyzed in BEB patients at pre-injection and 2-month follow-up after injection, average LLT significantly increased after injection (72.4 ± 22.7 nm to 83.0 ± 22.2 nm, p = 0.0215). Diabetes and young age were associated factors influencing the LLT increase. Significant increase in LLT was observed post-injection, with specific demographic factors, including diabetes and age, associated with this improvement. Additionally, blink dynamics showed a decrease in complete blink rate post-injection. Conclusions: These findings suggest that BoT-A may alleviate BEB symptoms, but ophthalmologists should carefully interpret LLT and blink patterns in BEB patients due to potential overestimations of blink efficacy by the interferometer. [ABSTRACT FROM AUTHOR]

ABSTRACT Background Objectives Methods Results Conclusions Dystonia may respond to VMAT2 inhibition.Providing pilot data on the safety, tolerability, and efficacy of deutetrabenazine in non dopa‐responsive dystonia.Deutetrabenazine was titrated by adults with isolated dystonia. Primary study endpoints included the proportion who maintained the individual, maximum tolerated dose for 6 weeks, and how many titrated to 48 mg/day. Secondary endpoints included rates of QTc prolongation/arrhythmias, suicidality, excessive daytime sleepiness, cognitive decline, and drug‐induced parkinsonism. Exploratory endpoints for clinical efficacy were assessed.Among 15 participants, four (26.7%) withdrew early and six (40%) titrated to 48 mg/day. Common adverse events included fatigue and diarrhea. Secondary safety endpoints did not change significantly, but MDS‐UPDRS III scores worsened by ≥3 points in seven participants (46.7%). PGI‐C and the blinded CGI‐C and GDS improved in three women with blepharospasm.Most participants tolerated deutetrabenazine for 6 weeks, and those with blepharospasm may have benefitted from its use. [ABSTRACT FROM AUTHOR]

Botulinum neurotoxin type-A (BoNT/A), which blocks quantal acetylcholine (ACh) release at the neuromuscular junction (NMJ), has demonstrated its efficacy in the symptomatic treatment of blepharospasm. In 3.89% of patients treated for blepharospasm at Tenon Hospital, BoNT/A was no longer effective in relieving the patient's symptoms, and a partial upper myectomy of the Orbicularis oculi muscle was performed. We used surgical waste samples from 14 patients treated with repeated injections of either abobotulinumtoxinA (Dysport®) or incobotulinumtoxinA (Xeomin®). These muscle fragments were compared to others from 4 normal subjects, naïve of BoNT/A. The morphological study was performed blinded to the BoNT/A treatment and between treated and control samples. Neuromuscular specimens analyzed by confocal laser scanning microscopy, using fluorescent staining and immune-labeling of presynaptic proteins, revealed that the pattern of innervation (e.g., polyneuronal and convergent innervation), the muscle nicotinic ACh receptors (nAChRs), and the NMJs exhibited marked differences in BoNT/A-treated muscles (regardless of the toxin clinically used), with respect to controls. BoNT/A-treated junctions exhibited profuse polyneuronal innervation in which 2–6 axons innervated 74.84% of single muscle fibers, while 99.47% of control junctions were mono-innervated. Another new finding was the stable convergent innervation, in which several motor axons end onto the same endplate. Morphological signs of synapse elimination included the presence of retraction bulbs in axons and nerve terminals and a reduced extension of postsynaptic nAChRs. These outcomes suggest that synapse elimination is altered and raise questions on the origin and factors contributing to the plasticity changes observed and the functioning of NMJs. [ABSTRACT FROM AUTHOR]

Dystonia is a neurological motor disorder characterised by involuntary and uncontrollable muscle contractions, tension, twisting, and tremors. The aim of the present study was to analyse the improvement in dystonic contractions in patients with dystonia following the use of a Hegab temporomandibular joint splint (HTS). The Fahn-Marsden Dystonia Movement Scale (DMS) and Disability Scale were used in the current study to evaluate dystonia. An HTS with a thickness ranging from 4 to 6 mm was used to treat the patients enrolled in the study. The final sample comprised 14 patients (10 female and four male) with mean (range) ages of 35.64 (18 to 55) years. Pre-treatment DMS ranged from 6.5 to 57 mean (SD) 18.21 (13.38). At the end of the study, DMS ranged from 0 to 15 mean (SD) 3.14 (3.86). Statistical analysis of the differences between pre-treatment and post-treatment DMS showed a significant decrease in DMS at the end of the treatment period (p = 0.0001). Regarding the disability scale, pre-treatment disability scale ranged from 7 to 18 mean (SD) 9.46 (3.02). At the end of the study, DMS ranged from 0 to 3 mean (SD) 1.46 (1.13). There was a statistically highly significant decrease in the Disability Scale at the end of the treatment period (p value ≤ 0.0001). This study suggests that the HTS can be considered an effective treatment modality for dystonia, as it significantly decreases both the DMS and the Disability scale. [ABSTRACT FROM AUTHOR]

Copyright of Arquivos Brasileiros de Oftalmologia is the property of Arquivos Brasileiros de Oftalmologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract Background This study investigates the effect of botulinum toxin A on lipid layer thickness (LLT) and blink dynamics in patients with benign essential blepharospasm (BEB) compared to dry eye disease (DED) patients. Methods We reviewed the medical records of patients with dry eye disease (DED) and BEB treated with botulinum toxin A (BoT A) injections. Data on demographics, lipid layer thickness (LLT), meiboscore, and blink dynamics measured using a LipiView II interferometer before and 2 months after BoT A were collected. Results Each 28 eyes from 28 patients with BEB and age- and sex-matched patients with DED were included. When comparing blink dynamics, complete blink rate was significantly higher in the pre-injection BEB group compared to the DED group (5.25 ± 4.32 times/20 s vs. 2.43 ± 2.82 times/20 s, p = 0.0055). In tear film lipid profiles analyzed in BEB patients at pre-injection and 2-month follow-up after injection, average LLT significantly increased after injection (72.4 ± 22.7 nm to 83.0 ± 22.2 nm, p = 0.0215). Diabetes and young age were associated factors influencing the LLT increase. Significant increase in LLT was observed post-injection, with specific demographic factors, including diabetes and age, associated with this improvement. Additionally, blink dynamics showed a decrease in complete blink rate post-injection. Conclusions These findings suggest that BoT-A may alleviate BEB symptoms, but ophthalmologists should carefully interpret LLT and blink patterns in BEB patients due to potential overestimations of blink efficacy by the interferometer.

AIM: To investigate the relationship between benign essential blepharospasm (BEB) symptoms and depression/anxiety/sleep disorder in a prospective manner and to determine whether treatment the BEB with botulinum toxin type A (BoNT/A) can impact psychological symptoms. METHODS: This prospective interventional case series recruited 61 adults with evidence of BEB. Patients were administered the Jankovic Rating Scale (JRS), the Blepharospasm Disability Index (BSDI), Personal Health Questionnaire Depression Scale (PHQ-8), Generalized Anxiety Disorder 7-item scale (GAD-7) and the Athens insomnia scale (AIS) to evaluate the severity of BEB symptoms, depression, anxiety and sleep disorder before and 1wk, 1, 3mo after the BoNTA treatment. Statistical analysis was performed to assess the relationships between changes in the survey scores. RESULTS: The mean score for JRS, BSDI, PHQ-8, and GAD-7 improved significantly (P

Chemodenervation by botulinum neurotoxin (BoNT) has significantly evolved over years since Alan Scott first described its use in 1970s for treatment of strabismus. It has been used for a wide variety of diseases. It has surpassed other treatment options to become the first line of therapy among oculoplasty surgeons for facial dystonias and as an aesthetic agent. The effect of BoNT is temporary but as a well-maintained therapeutic effect even after repeated injections. The side effects and complications are very rare, and it is often temporary. In this article, we review the pharmacology, indications in ophthalmic plastic surgery, techniques, and adverse effects of BoNT.

This retrospective study examined the clinical outcomes and complications in 6 cases of compound Meige's syndrome, presenting with blepharospasm and masticatory muscle spasm, following treatment with CT-guided radiofrequency ablation targeting bilateral facial nerves and mandibular branches of the trigeminal nerve. After the operation, the symptoms of eyelid spasm and masticatory muscle spasm resolved, but mild facial paralysis and numbness of mandibular nerve innervation persisted. Follow-up for 4–28 months showed that the symptoms of facial paralysis resolved within 2–5 (3.17 ± 0.94) months after the operation, whereas the numbness in the mandibular region persisted, accompanied by a decrease in masticatory function. During the follow-up period, none of the 6 patients experienced a recurrence of Meige's syndrome. These findings suggest that CT-guided radiofrequency ablation of the bilateral facial nerve and mandibular branches of the trigeminal nerve may offer a promising approach to treating compound Meige's syndrome. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the effect of botulinum toxin-A (BTX-A) treatment on corneal topography, ocular biometry and keratometry in patients with benign essential blepharospasm (BEB) and hemifacial spasm (HFS). Methods: This study comprised 66 eyes of 33 patients with BEB and 5 eyes of 5 patients with HFS who underwent BTX-A injections consecutively. Refractive error values, tear break-up time (TBUT), corneal topography [corneal power of flat axis (K1) and steep axis (K2), mean corneal power (Km), corneal astigmatism (K2–K1)] and ocular optical biometry [axial length (AL), anterior chamber depth (ACD)] were recorded before BTX-A treatment and 1 month after BTX-A treatment. The researchers calculated the expected emmetropic intraocular lens power (emm-IOL) using the SRK-T, Holladay, Hoffer-Q and Haigis formulas at each examination. Results: K1 (43.48 ± 2.02 vs. 43.57 ± 2.08, p = 0.036), Km (43.91 ± 1.99 vs. 43.99 ± 2.06, p = 0.024) and ACD (3.22 (2.77–3.76) vs. 3.41 (2.99–4.02), p < 0.001) values were found to be significantly higher. The expected emm-IOL according to the SRK-T (21.04 ± 1.6 vs. 20.93 ± 1.6, p = 0.048), Holladay (21.05 ± 1.6 vs. 20.91 ± 1.62, p = 0.037) and Hoffer-Q (21.08 ± 1.65 vs. 20.94 ± 1.68, p = 0.038) decreased significantly. The expected emm-IOL according to the Haigis formula slightly decreased, but it was not significant (p = 0.386). Additionally, TBUT was found to be significantly lower (p < 0.001) after BTX-A injection. Other parameters were not statistically significant (p > 0.05). Conclusions: Our study is the first in the literature to compare optic biometry data and intraocular lens power calculation formulas before and after BTX-A injection in eyes with BEB and HFS. BTX-A injection could play an important role in changing the keratometric and ACD values. It should be considered that IOL power calculations that might be unpredictable due to blepharospasm, so repeated measurements and especially measurements after releasing the spasm with BTX-A injections, are necessary in BEB and HFS. [ABSTRACT FROM AUTHOR]

Movement disorders such as cervical dystonia, blepharospasm, and hemifacial spasm negatively impact the quality of life of people living with these conditions. Botulinum toxin (BoNT) injections are commonly used to treat these disorders. We sought to describe patient characteristics, BoNT utilization, and potential adverse events (AEs) among patients with cervical dystonia, blepharospasm, and hemifacial spasm using Optum's de-identified Clinformatics® Data Mart Database. Patients were required to have a diagnosis of the specific condition plus evidence of treatment with BoNT between 8/1/2010 and 5/31/2022. Cervical dystonia patients were commonly females (76%) and aged 45 and older (78%); both blepharospasm and hemifacial spasm patients were commonly females (both 69%) and aged 65 and older (61% and 56%, respectively). Anticholinergics were commonly used (65–82% across cohorts), as were peripheral muscle relaxants for cervical dystonia patients specifically (31%). The median number of injections per year was 2 with the median weeks between injections being between 13 and 15. Of the AEs evaluated, dyspnea was identified frequently across all the cohorts (14–20%). The findings were similar for different BoNT formulations. More research is needed to thoroughly describe BoNT utilization, such as the doses injected, and to optimize treatment for patients with these conditions. [ABSTRACT FROM AUTHOR]

Background: Prior studies have indicated that female individuals outnumber male individuals for certain types of dystonia. Few studies have addressed factors impacting these sex differences or their potential biological mechanisms. Objectives: To evaluate factors underlying sex differences in the dystonias and explore potential mechanisms for these differences. Methods: Data from individuals with various types of dystonia were analyzed in relation to sex. Data came from two different sources. One source was the Dystonia Coalition database, which contains predominantly idiopathic adult‐onset focal and segmental dystonias. The second source was the MDSGene database, which contains predominantly early‐onset monogenic dystonias. Results: The 3222 individuals from the Dystonia Coalition included 71% female participants and 29% male participants for an overall female‐to‐male ratio (F:M) of 2.4. This ratio varied according to body region affected and whether dystonia was task‐specific. The female predominance was age‐dependent. Sex did not have a significant impact on co‐existing tremor, geste antagoniste, depression or anxiety. In the 1377 individuals from the MDSGene database, female participants outnumbered male participants for some genes (GNAL, GCH1, and ANO3) but not for other genes (THAP1, TH, and TOR1A). Conclusions: These results are in keeping with prior studies that have indicated female individuals outnumber male individuals for both adult‐onset idiopathic and early onset monogenic dystonias. These results extend prior observations by revealing that sex ratios depend on the type of dystonia, age, and underlying genetics. [ABSTRACT FROM AUTHOR]

Apraxia of eyelid opening (AEO) is a non-paralytic motor abnormality characterized by difficulty in eyelid opening bilaterally. This is due to the inability of voluntary opening of the eyes in the absence of visible contraction of the orbicularis oculi despite sustained willful frontalis contraction. We present you a case of a 60-year-old woman with AEO associated with benign essential blepharospasm who suffered marked limitation in daily activities due to functional blindness. She was treated with botulinum toxin type A injections following which she had adequate eyelid opening in both eyes during 1 week follow up. It is essential for physicians and patients to be aware of this condition for proper diagnosis and treatment.

Objective: The objective of this study was to perform a long-term evaluation of the efficacy and safety of treatment with botulinum toxin A (BoNT-A) in patients with blepharospasm (BS) and hemifacial spasm (HFS) from January 2007 to December 2019. Methods: In each application of BoNT-A, the date of treatment, number of units applied, and time elapsed since the previous application were recorded. Outcome data was: mean latency of the clinical effect, mean duration of the clinical effect, mean improvement on Jankovic rating scale, side effects were self-reported, and evaluated 2 weeks after injection, including non-responding patients to BoNT-A for two consecutive sessions. The comparison between the first and last dose of BoNT-A was analyzed by Student's t-test, for which a value of p < 0.05 was considered statistically significant. Results: A total of 136 patients were analyzed; 60 had BS, 76 had HFS, and 75% were female. The duration between onset and referral for BoNT-A treatment was 18 ± 3 months, and the mean age at the time of the first therapeutic injection was 50 ± 12 years. The mean dose per session was 16 ± 4 for BS and 36 ± 12 for HFS. The therapeutic interval for injections was 4.4 ± 1 month. The mean latency of the clinical effect was 8 ± 3 days, the mean duration of the clinical effect was 112 ± 9 days, and the mean improvement on the Jankovic scale was 2 ± 1 points. Side effects were observed in 9 patients (6.6%), that is, ptosis (7 patients) and hematoma (2 patients). Conclusions: BoNT-A is a safe and effective long-term treatment for BS and HFS. [ABSTRACT FROM AUTHOR]

Botulinum toxin (BoNT) has been known for over 50 years. It has conquered many areas of medicine and has become indispensable in contemporary medicine. Now, BoNT is used to treat at least 26 conditions in six medical specialties. Although the use of BoNT began in strabology, it became the gold standard for many ophthalmologic pathologies. The present review of the literature focuses on the use of BoNT in ophthalmology and treatment of the following conditions: blepharospasm, facial hemispasm, facial palsy, spastic entropion, strabismus, endocrine orbitopathy, convergence spasm, and facial trauma. We conclude that nearly half a century of experience in utilizing BoNT in ophthalmology ensured a satisfactory level of effectiveness and safety for patients with many pathologies. Areas of future research include the application of BoNT in new selected indications, the development of the route of application without injections, and the development of long‑acting BoNT forms for patients who require repeated long‑term treatment. [ABSTRACT FROM AUTHOR]

Copyright of Vlaams Diergeneeskundig Tijdschrift is the property of Ghent University, Faculty of Veterinary Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Botulinum toxin is one of the most potent neurotoxins, but when injected into an overactive muscle, it can transiently alleviate an involuntary movement, such as dystonia. The primary aim of this article is to provide a comprehensive review of the various forms of dystonia observed in patients with Parkinson's disease who can benefit from a therapeutic trial of botulinum toxin. Although most of these indications are not supported by randomized controlled clinical trials and, therefore, not approved by the Food and Drug Administration, there are many open-label trials supporting a large body of empirical experience testifying to the benefits of botulinum toxin treatment in these conditions. [ABSTRACT FROM AUTHOR]

Background: Blepharospasm is treated with botulinum toxin, but obtaining satisfactory results is sometimes challenging. Objective: The aim is to conduct an exploratory trial of oral dipraglurant for blepharospasm. Methods: This study was an exploratory, phase 2a, randomized, double-blind, placebo-controlled trial of 15 participants who were assigned to receive a placebo or dipraglurant (50 or 100 mg) and assessed over 2 days, 1 and 2 hours following dosing. Outcome measures included multiple scales rated by clinicians or participants, digital video, and a wearable sensor. Results: Dipraglurant was well tolerated, with no obvious impact on any of the measurement outcomes. Power analyses suggested fewer subjects would be required for studies using a within-subject versus independent group design, especially for certain measures. Some outcome measures appeared more suitable than others. Conclusion: Although dipraglurant appeared well tolerated, it did not produce a trend for clinical benefit. The results provide valuable information for planning further trials in blepharospasm. © 2024 International Parkinson and Movement Disorder Society. [ABSTRACT FROM AUTHOR]

A neurological condition called dystonia results in abnormal, uncontrollable postures or movements because of sporadic or continuous muscular spasms. Several varieties of dystonia can impact people of all ages, leading to severe impairment and a decreased standard of living. The discovery of genes causing variations of single or mixed dystonia has improved our understanding of the disease's etiology. Genetic dystonias are linked to several genes, including pathogenic variations of VPS16, TOR1A, THAP1, GNAL, and ANO3. Diagnosis of dystonia is primarily based on clinical symptoms, which can be challenging due to overlapping symptoms with other neurological conditions, such as Parkinson's disease. This review aims to summarize recent advances in the genetic origins and management of focal dystonia. [ABSTRACT FROM AUTHOR]

Background: Cognitive dysfunction has been reported in idiopathic adult‐onset dystonia (IAOD), but whether this is a primary or secondary component of the disorder remains uncertain. Objective: Here, we aimed to analyze the key domains of abnormal cognitive performance in IAOD and whether this is associated with motor or mood changes. Methods: Article selection for our critical review was guided by PRISMA guidelines (mesh terms "dystonia" and "cognitive," publication period: 2000–2022). Only peer‐reviewed, English‐language original case–control studies involving patients with IAOD who were not exposed to dopamine‐ or acetylcholine‐modulating agents and validated cognitive assessments were included. Results: Abstract screening ultimately yielded 22 articles for full‐text review and data extraction. A greater proportion of studies (17 of 22, 82%) reported abnormal cognitive performance in IAOD. Most of these studies focused on blepharospasm (BSP) and cervical dystonia (10 and 14, respectively). Most studies reporting cognitive impairment (11 of 17) identified multidomain impairment in cognition. Executive functions were the domain most frequently explored (14 of 22 studies), 79% of which detected worse performance in people with dystonia. Results related to other domains were inconclusive. Cognitive abnormalities were independent of motor symptoms in most studies (7 of 12) that explored this relationship and independent of mood status in all 8 that investigated this. Conclusions: Within IAOD, cognitive dysfunction (in particular, executive dysfunction) has been documented mainly in BSP and cervical dystonia. More comprehensive testing is warranted to assess abnormalities in other domains and in other forms of IAOD, as well as to evaluate longitudinal progression of cognitive disturbances in this condition. [ABSTRACT FROM AUTHOR]

Copyright of Türkiye Klinikleri Journal of Ophthalmology is the property of Turkiye Klinikleri and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: In preceding work, a deleterious REEP4 variant [GRCh38/hg38, NC_000008.11:g.22140245G>A, NM_025232.4:c.109C>T, p.Arg37Trp] was found to co-segregate with blepharospasm (BSP) in a large African-American pedigree. Other REEP4 variants have been reported in genetic screening studies of dystonia. The REEP4 paralogs, REEP1 and REEP2, are associated with spastic paraplegia. The causal contributions of REEP4 variants to dystonia and other neurological disorders remains indecisive. Methods: Sanger sequencing was used to screen subjects (N = 307) with BSP and BSP-plus dystonia affecting additional anatomical segments (BSP+) phenotypes for variants in REEP4. In silico tools were used to examine the deleteriousness of reported (ClinVar) and previously published REEP4 variants. Results: No highly deleterious variant was identified in coding or contiguous splice site regions of REEP4 in our cohort of 307 subjects. In silico analysis identified numerous deleterious REEP4 variants in published screening studies of dystonia and several highly deleterious single nucleotide REEP4 variants in ClinVar. Conclusion: Highly deleterious REEP4 variants are rare in BSP and BSP+ phenotypes. [ABSTRACT FROM AUTHOR]

Background The application of botulinum toxin (BoNT) in the treatment of blepharospasm and hemifacial spasm (HS) is a well-established practice. However, neurology residency programs often rely on real patients for training, which has limitations in terms of patient availability and skill acquisition.

Meige syndrome is a rare neurological disease characterized by segmental dystonia, specifically blepharospasm and oromandibular dystonia. These symptoms are often accompanied by complex movements of the eyelids, lower facial muscles, mandible, and neck muscles. Bilateral blepharospasm is the most common feature of this disease. In this case report, we present the successful treatment of refractory blepharospasm in a 72-year-old woman with Meige syndrome via 2 incisions resulting from myectomy and in situ surgery. [ABSTRACT FROM AUTHOR]

Copyright of Bosphorus Medical Journal / Boğaziçi Tıp Dergisi is the property of KARE Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Botulinum toxin is one of the most potent neurotoxins, but when injected into an overactive muscle, it can transiently alleviate an involuntary movement, such as dystonia. The primary aim of this article is to provide a comprehensive review of the various forms of dystonia observed in patients with Parkinson’s disease who can benefit from a therapeutic trial of botulinum toxin. Although most of these indications are not supported by randomized controlled clinical trials and, therefore, not approved by the Food and Drug Administration, there are many open-label trials supporting a large body of empirical experience testifying to the benefits of botulinum toxin treatment in these conditions.

Blepharospasm is a focal dystonia characterized by involuntary tetanic contractions of the orbicularis oculi muscle, which can lead to functional blindness and loss of independent living ability in severe cases. It usually occurs in adults, with a higher incidence rate in women than in men. The etiology and pathogenesis of this disease have not been elucidated to date, but it is traditionally believed to be related to the basal ganglia. Studies have also shown that this is related to the decreased activity of inhibitory neurons in the cerebral cortex caused by environmental factors and genetic predisposition. Increasingly, studies have focused on the imbalance in the regulation of neurotransmitters, including dopamine, serotonin, and acetylcholine, in blepharospasm. The onset of the disease is insidious, and the misdiagnosis rate is high based on history and clinicalmanifestations. This article reviews the etiology, epidemiological features, and pathogenesis of blepharospasm, to improve understanding of the disease by neurologists and ophthalmologists. [ABSTRACT FROM AUTHOR]

Copyright of Arquivos de Neuro-Psiquiatria is the property of Thieme Medical Publishing Inc. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: The aim of this clinical study was to assess the impact of a non-invasive selective blue-filtering photochromic lens coating Crizal Prevencia on the treatment and response of benign essential blepharospasm (BEB). Patients and methods: Twenty-four patients were recruited in the outpatient clinic of the Medical University of Vienna in a randomized, double-blind, cross-over study design. Blink frequencies were assessed in patients with BEB before and 14 days after intervention with either a filtering ophthalmic lens or a placebo lens, respectively. Outcome parameters include sub-group analysis of a blink frequency under six different conditions: three photopic conditions, one resting condition, one reading condition, and one video game condition. Results: From 24 recruited patients, 15 patients were available for final analysis. Comparing the optical blue filtering lens to placebo, showed a reduced blink frequency in specific subtests, but not compared to baseline. Discussion: In conclusion, optical filtering glasses might have a beneficial effect on BEB and provide a non-invasive therapeutic add-on option, in addition to botulinum neurotoxin therapy, for patients with BEB and should necessarily be further investigated in a multicenter setting, resulting in larger sample sizes to gain valid information about the effect of photochromic blue filter glasses in BEB. Clinical trial registration: https://drks.de/search/en/trial/DRKS00032135, DRKS00032135. [ABSTRACT FROM AUTHOR]

Aim: Hyperkinetic facial disorders include Benign Essential Blepharospasm (BEB) and Hemifacial Spasm (HFS), which involve involuntary facial muscle contractions. Botulinum toxin (BTX) is essential for relieving symptoms. The Coronavirus-19 (COVID-19) pandemic-related restrictions have led to delays in receiving BTX treatment. This study aims to assess the impact of BTX treatment delays in patients with hyperkinetic facial disorders. Materials and Methods: This study retrospectively examined the data of 84 patients (68 HFS and 16 BEB) whose BTX appointments were delayed due to COVID-19 restrictions. Patient status was evaluated using Jankovic disability rating scale (JRS) scores, and administered BTX doses were documented and compared before and after lockdown. Results: After the BTX treatment delay, the median JRS scores significantly increased from 2 before the lockdown to 3 after the lockdown (p<0.001). Furthermore, there was a noticeable increase in the average BTX treatment dose after the lockdown, from 35 units to 37.5 units (p<0.001). Conclusion: Longer intervals between injections may increase disease severity and the effective BTX doses in patients with HFS and BEB. The COVID-19 pandemic has highlighted the importance of timely BTX therapy in managing hyperkinetic facial disorders. [ABSTRACT FROM AUTHOR]

To objectively measure the blink rate in patients with blepharospasm managed by botulinum toxin type-A injections. In this prospective, non-interventional case series, the complete blink rates of subjects were measured before incobotulinumtoxina injection and at follow-up within 4 weeks using slow-motion video-taping. Additionally, subjects graded the frequency of blinking, the severity of light-sensitivity, and the severity and frequency of dry eye symptoms on a categorical visual analog scale. The results are reported as median (range). Ten subjects were enrolled, with nine females. The total duration of treatment was 70 (5–116) months with total of 27.5 (2–51) injections. The subjects were grouped as short-time (<52w) or long-time (>52w) treatments. The median age, follow-up time, and injected doses were 73.5 (49–81) years, 21 (14–28) days, and 38 (8–47) units, respectively, with no significant difference between groups. The total complete blinks per minute before incobotulinumtoxina injection was 39 (23–64) which decreased to 18.5 (1–60) at follow-up (p = 0.004). The average change in complete blink rate was −67.4 ± 23.7% in long-time and −45.2 ± 31.2% in short-time groups (mean ± SD, p = 0.01). The total self-graded frequency of blinking and light-sensitivity decreased significantly at follow-up (p = 0.004, p = 0.047, respectively). Similar patterns of subject reported grades were seen in both groups. Videotaping is a low-cost method for objective measurement of blink rate in blepharospasm patients after incobotulinumtoxina injection. There was a significant reduction in blink rate after incobotulinumtoxina injections with higher percentage of change in the long-time treatment group. Incobotulinumtoxina injection also significantly improves subjective photophobia. [ABSTRACT FROM AUTHOR]

Background: There are several widely used clinical rating scales for documenting the severity and distribution of various types of dystonia. Objectives: The goal of this study was to evaluate the performance of the most commonly used scales in a large group of adults with the most common types of isolated dystonia. Methods: Global Dystonia Rating Scale (GDRS) and the Burke‐Fahn‐Marsden Dystonia Rating Scale (BFM) scores were obtained for 3067 participants. Most had focal or segmental dystonia, with smaller numbers of multifocal or generalized dystonia. These scales were also compared for 209 adults with cervical dystonia that had Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) scores and 210 adults with blepharospasm that had Blepharospasm Severity Scale (BSRS) scores. Results: There were strong correlations between the GDRS and BFM total scores (r = 0.79) and moderate correlations for their sub scores (r > 0.5). Scores for both scales showed positive skew, with an overabundance of low scores. BFM sub‐scores were not normally distributed, due to artifacts caused by the provoking factor. Relevant sub‐scores of the GDRS and BFM also showed moderate correlations with the TWSTRS (r > 0.5) for cervical dystonia and the BSRS (r > 0.5) for blepharospasm. Conclusions: The BFM is more widely used than the GDRS, but these results suggest the GDRS may be preferable for focal and segmental dystonias. The overabundance of very low scores for both scales highlights challenges associated with discriminating very mild dystonia from other abnormal movements or variants of normal behavior. [ABSTRACT FROM AUTHOR]

Purpose: 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. Methods: 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 1 month and 3 months. 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. Results: 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 ± 2.15 s, 79.61 ± 24.11 nm) at the 1-month visit compared to baseline (9.75 ± 5.60, 33.58 ± 13.27, 4.14 ± 2.21 s, 62.33 ± 22.01 nm; P = 0.006, < 0.001, = 0.027, < 0.001, respectively). Conclusions: 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. [ABSTRACT FROM AUTHOR]