Essential Blepharospasm and Hemifacial Spasm

Patients with benign essential blepharospasm or hemifacial spasm are known to use botulinum toxin injections and alleviating maneuvers to help control their symptoms. The clinical correlates between the use of botulinum toxin injections and the use of alleviating maneuvers are not well established. To determine whether the use of alleviating maneuvers for benign essential blepharospasm or hemifacial spasm correlates with disease severity or botulinum toxin treatment. A prospective cross-sectional observational study (designed in September 2013) of 74 patients with benign essential blepharospasm and 56 patients with hemifacial spasm who were consecutively recruited from adnexal clinics at Moorfields Eye Hospital (January-June 2014) to complete a questionnaire and undergo a clinical review. Data analysis was performed in December 2015. Prevalence and type of alleviating maneuvers used for blepharospasm and hemifacial spasm, dystonia severity, and dose and frequency of botulinum toxin injections. Of the 74 patients with blepharospasm, 39 (52.7%) used alleviating maneuvers (mean [SD] age, 70.4 [9.1] years); of the 56 patients with hemifacial spasm, 25 (44.6%) used alleviating maneuvers (mean [SD] age, 66.5 [12.7] years). The most commonly used maneuver was the touching of facial areas (35 of 64 patients [54.7%]); other maneuvers included covering the eyes (6 of 64 patients [9.4%]), singing (5 of 64 patients [7.8%]), and yawning (5 of 64 patients [7.8%]). Patients with blepharospasm who used alleviating maneuvers scored higher on the Jankovic Rating Scale (median score, 5 vs 4; Hodges-Lehmann median difference, 1 [95% CI, 0-2]; P = .01) and the Blepharospasm Disability Index severity score (median score, 11 vs 4; Hodges-Lehmann median difference, 4 [95% CI, 1-7]; P = .01) than patients with blepharospasm who did not use alleviating maneuvers. Patients with hemifacial spasm who used alleviating maneuvers scored higher on the 7-item Hemifacial Spasm Quality of Life scale (median score, 7 vs 3; Hodges-Lehmann median difference, 4 [95% CI, 1-7]; P = .01) and the SMC Severity Grading Scale (median score, 2 vs 2; Hodges-Lehmann median difference, 0 [95% CI, 0-1]; P = .03) than patients with hemifacial spasm who did not use alleviating maneuver. The severity of dystonia correlated with botulinum toxin treatment for patients with blepharospasm (r = 0.23; P = .049) and patients with hemifacial spasm (r = 0.45; P = .001). There was no difference found in botulinum toxin treatment between patients who used alleviating maneuvers and those who did not, in either the blepharospasm group (150 vs 125 units; Hodges-Lehmann median difference, 20 units [95% CI, -10 to 70 units]; P = .15) or the hemifacial spasm group (58 vs 60 units; Hodges-Lehmann median difference, 0 units [95% CI, -15 to 20 units]; P = .83). Half of the patients with periocular facial dystonias used alleviating maneuvers. Their use was associated with more severe disease but not with increased use of botulinum toxin. This may help to guide future therapies, such as advice on maneuver augmentation or tailored devices.

To identify risk factors associated with benign essential blepharospasm (BEB) with reference to hemifacial spasm (HFS). Persons with BEB and HFS experience similar physical symptoms, yet the two disorders have different etiologies. Patients with BEB (n = 159) or HFS (n = 91) were identified from two large neuro-ophthalmology clinics. Demographic, medical, behavioral, and psychological characteristics were obtained from chart review and a telephonic survey questionnaire. The average age of BEB and HFS was 66 years. Most patients in both groups were retired, white, and female. BEB patients were more than two times as likely to meet the diagnostic criteria for generalized anxiety disorder than HFS patients (odds ratio, 2.13; 95% confidence interval, 1.22-3.72). There was no difference between the two groups regarding demographics, smoking, a family history of dystonia, Parkinson disease, Bell palsy, Tourette disorder, obsessive compulsive symptoms, history of head trauma, alcohol use, or caffeine consumption. As compared to HFS, BEB was significantly more often associated with generalized anxiety disorder. Given the similarity of other clinical features of these two disorders, it is reasonable to conclude that anxiety is a cause not a consequence of BEB. Contrary to previous studies, BEB was not associated with obsessive-compulsive symptoms, head trauma, Parkinson disease, Bell palsy, Tourette disorder, or lack of smoking.

Effects of botulinum toxin type a on quality of life assessed with the WHOQOL-BREF in hemifacial spasm and blepharospasm

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). 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. 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). 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.

We aimed to study sub therapeutic (to reduce intensity of discomfort) and safe dose administration of BoTn/A in certain dystonic neurological movement disorders and Post herpetic Neuralgia (PHN) during pregnancy. Total 7 cases studied [(Cervical dystonia =1), (Blepharospasm =2), (Hemi facial spasm=1) and (PHN=3)].BoTn/A (dysport – SPEYWOOD) was diluted in 10 ml of normal saline. Arbitrary 200 to 500 units was administerd depending upon clinical assessment. The patients followed at 1, 3, 4, 12 and 16 weeks. The fetal well being was assessed after delivery and maximum follow-up to 1 and half year period.Botulinum toxin type a is used as a therapeutic agent in various hyper active movement disorders such as cervical dystonia, blepharospasm, hemi facial spasm and intractable neuropathic pain such as post herpetic neuralgia (PHN). It is an exotoxin produced by clostridium botulinum. It prevents the release of acetylcholine thereby preventing the muscle contraction and reducing pain transmission. The mean age was 35 years. The severity of spasm assessed on intensity, range and frequency of contraction of muscles. In PHN pain intensity measured on VAS score. There was a significant subjective improvement in the spasm in cervical dystonia, blepharo spasm, &amp; hemifacial spasm. The VAS score reduced significantly in all three cases. The fetuses born were healthy and no congenital malformation observed. The children were followed by an average one year were found healthy. We observed that BoTn/A decreased subjective discomfort in Dystonia, spasm and in the intensity of neuropathic pain. It is safer to administer during pregnancy particularly in third trimester as the fetus born were healthy. The oral medications such as antiepileptic are not without side effects. Therefore, we conclude that it is safer to administer during pregnancy particularly in third trimester. However, larger study is needed.

&lt;p&gt;Background: Blepharospasm (BSP) and hemifacial spasm (HFS) are both facial hyperkinesia however BSP is thought to be caused by maladaptation in multiple brain regions in contrast to the peripherally induced cause in HFS. Plausible coexisting pathophysiologies between these two distinct diseases have been proposed.&lt;/p&gt;&lt;p&gt;Objectives: In this study, we compared brain resting state functional connectivity (rsFC) and quantitative thermal test (QTT) results between patients with BSP, HFS and heathy controls (HCs).&lt;/p&gt;&lt;p&gt;Methods: This study enrolled 12 patients with BSP, 11 patients with HFS, and 15 HCs. All subjects received serial neuropsychiatric evaluations, questionnaires determining disease severity and functional impairment, QTT, and resting state functional MRI. Image data were acquired using seed-based analyses using the CONN toolbox.&lt;/p&gt;&lt;p&gt;Results: A higher cold detection threshold was found in the BSP and HFS patients compared to the HCs. The BSP and HFS patients had higher rsFC between the anterior cerebellum network and left occipital regions compared to the HCs. In all subjects, impaired cold detection threshold in the QTT of lower extremities had a correlation with higher rsFC between the anterior cerebellar network and left lingual gyrus. Compared to the HCs, increased rsFC in right postcentral gyrus in the BSP patients and decreased rsFC in the right amygdala and frontal orbital cortex in the HFS subjects were revealed when the anterior cerebellar network was used as seed.&lt;/p&gt;&lt;p&gt;Conclusions: Dysfunction of sensory processing detected by the QTT is found in the BSP and HSP patients. Altered functional connectivity between the anterior cerebellar network and left occipital region, especially the Brodmann area 19, may indicate the possibility of shared pathophysiology among BSP, HFS, and impaired cold detection threshold. Further large-scale longitudinal study is needed for testing this theory in the future.&lt;/p&gt;

Background: Blepharospasm (BSP) and hemifacial spasm (HFS) are both facial hyperkinesia however BSP is thought to be caused by maladaptation in multiple brain regions in contrast to the peripherally induced cause in HFS. Plausible coexisting pathophysiologies between these two distinct diseases have been proposed.Objectives: In this study, we compared brain resting state functional connectivity (rsFC) and quantitative thermal test (QTT) results between patients with BSP, HFS and heathy controls (HCs).Methods: This study enrolled 12 patients with BSP, 11 patients with HFS, and 15 HCs. All subjects received serial neuropsychiatric evaluations, questionnaires determining disease severity and functional impairment, QTT, and resting state functional MRI. Image data were acquired using seed-based analyses using the CONN toolbox.Results: A higher cold detection threshold was found in the BSP and HFS patients compared to the HCs. The BSP and HFS patients had higher rsFC between the anterior cerebellum network and left occipital regions compared to the HCs. In all subjects, impaired cold detection threshold in the QTT of lower extremities had a correlation with higher rsFC between the anterior cerebellar network and left lingual gyrus. Compared to the HCs, increased rsFC in right postcentral gyrus in the BSP patients and decreased rsFC in the right amygdala and frontal orbital cortex in the HFS subjects were revealed when the anterior cerebellar network was used as seed.Conclusions: Dysfunction of sensory processing detected by the QTT is found in the BSP and HSP patients. Altered functional connectivity between the anterior cerebellar network and left occipital region, especially the Brodmann area 19, may indicate the possibility of shared pathophysiology among BSP, HFS, and impaired cold detection threshold. Further large-scale longitudinal study is needed for testing this theory in the future.

53. Is the facial nerve injured by a vascular compression in hemifacial spasm? Stimulating single-fiber EMG study

Patient-reported outcomes following a break in ophthalmic botulinum toxin therapy during the COVID-19 pandemic

This study was a Phase IV, prospective, one arm, non-comparative open trial, to investigate the efficacy and safety of Dysport (Botulinum toxin type A) in patients with idiopathic blepharospasm or hemifacial spasm. During the treatment period, patients were evaluated at baseline (week 0), week 6, and week 8, 10, or 12. Thirty two women and 16 men completed the whole course of the study. The therapeutic efficacy of Dysport became evident from 1.5 to 15 days (mean+/- SD, 6.1 +/- 2.9 days). The maximal effect appeared 12.2+/-5.0 days later. Injection of Dysport achieved 72.9 (13.0% amelioration in the spasm symptom. Dysport significantly improved the following functions, such as reading, watching TV, house work, working, driving and outing alone. At the twelfth week after Dysport injection, it was still effective in relieving blepharospasm or hemifacial spasm. The most frequent adverse event was ptosis, which was noted in 9 cases and represented 18.7% of total patients. Other adverse events were very mild, although lagophthalmos and dry eyes occurred in some patients, but none manifested any corneal complications. In conclusion, Dysport injection appears to be a safe, and effective procedure - accompanied only by minor, and transit adverse events.

Epidemiological and clinical analyses of hemifacial spasm (HFS) are presented and pertinent literature is reviewed. A group of 61 patients with HFS treated with botulinum toxin (BT) was studied, and a structured questionnaire took into account: age at onset (range: 18–77, mean: 51.1 years) and age at time of initiation of treatment (range: 22–82, mean: 58.1 years), sex distribution (showing a female predominance: 70%), side distribution (showing a predominant involvement of the left side of the face: 67%), initiation and evolution of HFS, personal and family history, symptoms associated with HFS, and response to treatment given before BT. Data on age, sex, and side distributions on these 61 patients were compared to those obtained in a group of 50 patients with HFS who underwent operation to perform vascular decompression. All of the parameters that were analyzed in patients with HFS were compared to those obtained in patients with blepharospasm (BSP). Thus, 57 patients with BSP treated with BT were age-matched with a similar number of the patients with HFS (belonging to the group of 61 patients).

Blepharospasm, dry eye and extractable nuclear antigen antibodies

OBJETIVO: Avaliar os custos e a qualidade de vida dos pacientes com blefaroespasmo essencial e espasmo hemifacial, submetidos à terapia com a toxina botulínica-A. MÉTODOS: Vinte e oito pacientes, sendo 16 com blefaroespasmo essencial e 12 com espasmo hemifacial, tratados com toxina botulínica-A, foram avaliados por meio de dois instrumentos: o SF-36 e a Escala de Disfunção de Blefaroespasmo. Os custos foram calculados segundo o número de unidades da toxina utilizadas por cada paciente no período de um ano. RESULTADOS: O custo total direto foi estimado em 1081,62 reais por ano (±89,39) para blefaroespasmo essencial e 618,06 reais por ano (±60,06) para espasmo hemifacial, para tratamento ambulatorial (p<0,001). Antes do tratamento, todos os domínios do SF-36 foram significantemente piores em ambas as doenças. Sob tratamento efetivo com toxina botulínica-A, os aumentos nos índices do SF-36 evidenciaram mudanças nas taxas de saúde geral da população em estudo, com diferença estatisticamente significante entre as respostas pré e pós-tratamento (p<0,05). Relacionando-se o SF-36 com Escala de Disfunção de Blefaroespasmo, foram observadas nos casos de blefaroespasmo essencial, relações significantes positivas (capacidade funcional, estado geral, aspecto emocional e aspectos sociais), ao passo que nos pacientes com espasmo hemifacial não foi encontrada nenhuma correlação. CONCLUSÃO: Este estudo demonstra notável impacto negativo das doenças na qualidade de vida desses pacientes, e uma significante melhora em ambas as doenças após tratamento com toxina botulínica-A. Além disso, os dados fornecem subsídios para justificar o alto custo da aquisição da toxina botulínica-A, devido a seus consideráveis benefícios para a qualidade de vida.

Personality traits associated with blepharospasm: A comparison with healthy subjects, patients with facial hemispasm and patients with hyperhidrosis

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.