Benign Essential Blepharospasm Patients Research Articles

Combination of pretarsal and preseptal botulinum toxin injections in the treatment of blepharospasm: A prospective nonrandomized clinical trial

PurposeTo compare the efficacy of two botulinum toxin (BoNT) injection methods, pretarsal (PT) combined with preseptal (PS) injection (PT-PS) and conventional PT injection, in the treatment of benign essential blepharospasm (BEB). DesignProspective nonrandomized clinical trial. MethodsFrom January 2023 to April 2024, 95 BoNT injections into orbicularis oculi were performed in 45 BEB patients, including 52 PT-PS injections s and 43 PT injections. Jankovic Rating Scale (JRS) and Blepharospasm Disability Index (BSDI) were used to assess motor symptoms. The efficacy of two injection methods for BEB was compared in terms of latency to response (LTR), latency to the peak response (LPR), duration of peak response (DPR), duration of response (DOR), satisfaction degree, and possible complications. ResultsBoth injection methods significantly improved JRS and BSDI scores in patients with BEB. However, PT-PS injections showed a shorter LTR [(4.00(3.00,6.00) vs 5.00(4.00,7.00) days, p=0.024] and LPR [23.50(16.00,26.00) vs 26.00(20.00,30.00) days, p=0.040], a longer DPR [88.00(80.50,104.75) vs 75.00(65.00,92.00) days, p=0.003] and DOR [135.00(118.50,153.75) vs 121.00(107.00,135.00) days, p=0.003] than PT injections. Patients with PT-PS injections were more satisfied than those with PT injections [9.50(8.50,10.00) vs 8.00(7.50,9.00), p<0.001], and around 2/3 of patients were more willing to receive the combined injection method later. Among patients receiving PT-PS injections, only one case experienced ptosis, and there were no significant differences in other complications such as lacrimation, dry eyes, and blurred vision between the two injection methods. ConclusionsPT-PS injections of BoNT showed more advantages in the treatment of BEB than PT injections in terms of both their efficacy and patients’ satisfactions.

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.

Health-related quality of life of daily-life-affected benign essential blepharospasm: Multi-center observational study.

To compare Thais' health-related quality of life (HRQOL) and severity grading, efficacy and safety in daily-life-affected benign essential blepharospasm (BEB) patients at baseline and after Botulinum toxin type A (BTX-A) treatment. Prospective-observational study. BEB patients with Jankovic rating scale (JRS) at least 3 in both severity and frequency graded from 14 institutes nationwide were included from August 2020 to June 2021. Demographic data, HRQOL evaluated by the Thai version of EQ-5D-5L and NEI-VFQ-25 questionnaires, and severity grading score evaluated by Jankovic rating scale (JRS) at baseline, 1, and 3 months after the treatment were collected. The impact of the BTX-A injections and their complications were recorded. 184 daily-life-affected BEB patients were enrolled; 159 patients (86.4%) had complete data with a mean age of 61.40±10.09 years. About 88.05% were female, and 10.1% were newly diagnosed. Most of the patients had bilateral involvement (96.9%) and 12.6% had history of BEB-related accident. After BTX-A treatment, HRQOL improved significantly in 4 dimensions of EQ-5D-5L, except self-care. The EQ_VAS (mean±SD) was 64.54±19.27, 75.13±15.37, 73.8±15.85 (p<0.001) and EQ-5D-5L utility score was 0.748±0.23, 0.824±0.19 and 0.807±0.19 at baseline, 1, 3 months after treatment, respectively. From NEI-VFQ-25, HRQOL also improved in all dimensions, except eye pain. The JRS improved in all patients. Self-reported minor adverse events were 22.6%, which mostly resolved within the first month. Daily-life-affected BEB impacted HRQOL in most dimensions from both generic and visual-specific questionnaires. BTX-A treatment not only decreased disease severity, but also improved quality of life.

Serum Metabolomics of Benign Essential Blepharospasm Using Liquid Chromatography and Orbitrap Mass Spectrometry.

Benign essential blepharospasm (BEB) is a form of focal dystonia that causes excessive involuntary spasms of the eyelids. Currently, the pathogenesis of BEB remains unclear. This study is aimed at investigating the serum metabolites profiles in patients with BEB and healthy control and to identify the mechanism and biomarkers of this disease. 30 patients with BEB and 33 healthy controls were recruited for this study. We conducted the quantitative and nontargeted metabolomics analysis of the serum samples from 63 subjects by using liquid chromatography and Orbitrap mass spectrometry (LC-Orbitrap MS). Multivariate statistical analysis was performed to detect and identify different metabolites between the two groups. The Kyoto Encyclopedia of Genes and Genomes (KEGG) and receiver operating characteristic (ROC) curve analysis of the altered metabolites were performed. A total of 134 metabolites were found and identified. The metabolites belonged to several metabolic pathways including phenylalanine metabolism, phenylalanine, tyrosine and tryptophan biosynthesis, arginine biosynthesis, linoleic acid metabolism, tryptophan metabolism, aminoacyl-tRNA biosynthesis, sphingolipid metabolism, glycosphingolipid biosynthesis, leucine and isoleucine biosynthesis, and vitamin B6 metabolism. Eight metabolites were identified as the potential biomarkers. These results demonstrated that serum metabolic profiling of BEB patients was significantly different from healthy controls based on LC-Orbitrap MS. Besides, metabolomics might provide useful information for a better understanding of BEB.

Tear meniscus, corneal topographic and aberrometric changes after botulinum toxin-a injection in patients with blepharospasm and hemifacial spasm.

To investigate the effect of botulinum neurotoxin-A (BTX-A) treatment on dry eye symptoms, tear meniscus, corneal topography and corneal aberrometry in patients with benign essential blepharospasm (BEB) and hemifacial spasm (HFS). This prospective study comprised of 6 patients with BEB and 20 patients with HFS. Tear meniscus height (TMH) and depth (TMD), tear break-up time (TBUT), corneal fluorescein staining score (CFSS), Schirmer I test, ocular surface disease index (OSDI) score, corneal topography [corneal power of flat axis (K1), corneal power of steep axis (K2), mean corneal power (Km), astigmatism and thinnest pachymetry] and anterior corneal aberrometry [spherical aberration (SA), vertical coma (vcoma), horizontal coma (hcoma), higher order root mean square (hRMS) and total RMS] were evaluated before BTX-A treatment, 3weeks after BTX-A treatment and 2months after BTX-A treatment. Six patients with BEB and 20 patients with HFS treated with BTX-A were evaluated in this study. Twenty contralateral spasm free eyes of 20 HFS patients were taken as control group. TMH and TMD were found to be significantly higher in eyes with spasm at both 3weeks and 2months after injection (TMH: 279.0 ± 123.2 at pretreatment, 380.5 ± 174.7 at third week and 317.0 ± 125.5 at second month p < 0.001 and p = 0.02, respectively), (TMD: 183.7 ± 59.7 at pretreatment, 235.7 ± 91.1 at third week and 209.8 ± 77.1 at second month p < 0.01 and p = 0.015, respectively). TBUT, CFSS, Schirmer I test values were similar (p > 0.05). OSDI scores decreased significantly from 29.6 ± 25.3 to 19.8 ± 20. p = 0.03 at third week and increased again by second month. K2 (43.9 ± 1.7 vs. 43.7 ± 1.6, p = 0.03) and astigmatism (0.8 ± 0.5 vs. 0.6 ± 0.4, p = 0.04) values were significantly lower at third week and increased again by second month. Pachymetry and aberrometric values did not change significantly. In the control group only Schirmer I test value decreased significantly at second month (10.5 ± 6.5 vs. 7.2 ± 5.6, p = 0.008), other parameters did not change. BTX-A injection increases tear meniscus and decrease symptoms related to dry eye disease in BEB and HFS patients. It decrease astigmatism and keratometry values, it does not cause a significant change in corneal aberrations. However the positive effects of BTX-A injection on ocular surface is temporary.

Medical cannabis oil for benign essential blepharospasm: a prospective, randomized controlled pilot study.

To examine the efficacy and safety of medical cannabis in benign essential blepharospasm (BEB). This is a prospective, double-blind, placebo-controlled study. All consecutive adult BEB patients who had been treated with BTX-A injectionswithout success between 3/2019 and 2/2020 were recruited. The study patients were randomly allocated into a treatment and a control (placebo) group in a 1:1 ratio. The treatment group used cannabis drops and the control group used cannabis oil drops during the first 6weeks of the study, and both groups were treated with the medical cannabis drops during the second 6weeks. The cannabis dose was gradually increased for each patient depending upon effect and tolerability. Three patients were included in each group(treatment andcontrol groups). The mean duration of spasm attack during the first 6weeks was 4.29min in the treatment group and 73.9min in the placebo group (P < 0.01). During the last 6weeks, the treatment group used an average of 6.27 drops and the placebo group used an average of 5.36 drops (P = 0.478). There were 61 spasm events in the treatment group and 94 spasm events in the placebo group (P = 0.05). The mean duration of spasm attack was 1.77 and 8.96min, respectively (P < 0.01). The side effects were mild, and they included general fatigue, dry mouth, and insomnia. Medical cannabis can be an effective and safe treatment for BEB as a second line after BTX-A injections when used for 3months. No significant ocular or systemic side effects was associated with the treatment.

Benign essential blepharospasm in Latvia. Clinical analysis

The aim of this study was to analyze the clinical features of benign essential blepharospasm (BEB) in Latvian patients. To our knowledge, this is the first study describing clinical features of BEB patients in Latvia.

Efficacy of Repetitive Transcranial Magnetic Stimulation Combined with Botulinum Toxin Type A for Benign Essential Blepharospasm Patients Accompanied by Anxiety and Depression.

ObjectiveTo evaluate the improvement of motor, anxiety, and depression in patients with blepharospasm with the use of botulinum toxin type A (BTX-A) and repetitive transcranial magnetic stimulation (rTMS).MethodsA total of 63 BEB patients accompanied by anxiety/depression were enrolled, among which 28 patients were treated with the injection of botulinum toxin type A (BTX-A) alone, while 35 patients were treated with BTX-A injection combined with rTMS. All patients were followed up for 6 months, and the overall efficacy was evaluated.ResultsBTX-A alone treatment and combined rTMS treatment could both significantly improve the symptoms of patients, and the effective rate was 92.86% and 94.29%, respectively. The duration of efficacy was significantly longer in the combined rTMS treatment group (16.89±3.39 weeks) than in BTX-A treatment group (13.04±3.48 weeks). After treatment, SDS score of BTX-A treatment group and combined rTMS treatment group was 49.69±7.90 and 49.46±6.73, respectively, and there was no significant difference between the two treatment groups; SAS score of BTX-A treatment group and combined rTMS treatment group was 53.88±7.34 and 48.79±6.62, respectively, and there was significant difference between the two treatment groups.ConclusionCompared to BTX-A alone treatment, BTX-A combined with rTMS can effectively improve the effect of BTX-A, prolong the duration of blepharospasm relief, and significantly reduce depression and anxiety in patients with BEB.

A Review of Tolerance and Safety Profiles of Long-Term Botulinum Neurotoxin Type A in Asian Patients with Hemifacial Spasm and Benign Essential Blepharospasm

ABSTRACT This article reports the tolerance and long-term safety profiles of botulinum neurotoxin type A among Asian patients with benign essential blepharospasm (BEB) and hemifacial spasm (HFS). We performed a retrospective review of clinical documents and procedure records of consecutive BEB and HFS patients receiving onabotulinum toxin A (Botox) treatment in our clinic over the past 20 years. We reviewed the information of 105 patients diagnosed with BEB (n = 31) and HFS (n = 74). All of the patients were Asian. The mean age of disease onset was 59 (range 37–80) years old for BEB and 61 (range 31–83) for HFS. The mean follow up was 84 (range 12–240) months and the mean number of sessions per patient was 19 (range 1–61). The botulinum toxin dose per session increased significantly in both BEB (16.5 versus 21.6 units, p < .05) and HFS (22.6 versus 26.9 units, p < .05) patients after a mean of 18 sessions; however, the onset time, effective duration and subjective treatment outcome were similar over time in both BEB and HFS patients. At least one local complication was reported among 26% and 41% of patients with BEB and HFS respectively, with ptosis (32%) being most frequent.

Screening Gene Mutations in Chinese Patients With Benign Essential Blepharospasm.

Objective: This study aimed to screen gene mutations in Chinese patients with benign essential blepharospasm (BEB) to understand its etiology.Methods: Twenty BEB patients diagnosed by clinical manifestations between April 2015 and October 2015 were enrolled. All the cases were investigated by questionnaires about general conditions, social behavioral factors, environmental factors, psychological factors, genetic factors, and previous diseases. In each patient, a total of 151 genes related to movement disorders were analyzed by second-generation sequencing.Results: Two patients had a family history of BEB, and they had SYNE1 and Cdkn1A-interacting zinc finger protein 1 (CIZ1) mutation, respectively. We found the SYNE1 mutation in seven patients, the CIZ1 mutation in two patients, the CACNA1A mutation in two patients, the LRRK2 mutation in two patients, and the FUS mutation in two patients. The C10orf2, TPP1, SLC1A3, PNKD, EIF4G1, SETX, PRRT2, SPTBN2, and TTBK2 mutations were found in only one patient, respectively, while not any mutation in the 151 genes were found in two patients. Some patients had mutations in two genes.Conclusion: Genetic factors, especially SYNE1 and CIZ1 mutations, contribute to the etiology of BEB.

Increased pupillary constriction velocity in benign essential blepharospasm associated with photophobia.

We evaluated whether the pupillary light reflex is altered in benign essential blepharospasm patients. Twenty-three patients with benign essential blepharospasm, 47 with reflex blepharospasm, and 29 dry eye disease controls were included. Pupillary light reflex-related parameters were measured under mesopic (10 lux) and photopic illuminance (200 lux) using an infrared pupillometer. Additionally, we assessed photophobia grade, eyelid function, and dry eye disease-related parameters. There were no differences in age, sex predominance, or dry eye disease-related parameters among the three groups, or in photophobia grade and eyelid function between benign essential blepharospasm and reflex groups. Constriction velocity and maximum constriction velocity in the mesopic condition were significantly greater in the benign essential blepharospasm group (3.26 ± 0.56 and 5.27 ± 0.90 mm/s) than in reflex (2.86 ± 0.62 and 4.59 ± 1.00 mm/s) or dry eye disease groups (2.96 ± 0.46 and 4.72 ± 0.67 mm/s). Constriction velocity and maximum constriction velocity in the mesopic condition positively correlated with photophobia grade (r = 0.525 and 0.617, P = 0.025 and 0.006) in the benign essential blepharospasm group. Pupillary light reflex may be related to the pathophysiology of benign essential blepharospasm with photophobia. Further studies are required to reveal connections among pupillary light reflex, photophobia, and focal dystonia in benign essential blepharospasm patients with photophobia.

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.

Botulinum toxin relieves anxiety and depression in patients with hemifacial spasm and blepharospasm.

ObjectiveTo explore the efficacy of botulinum toxin type A (BTX-A) therapy in relieving anxiety and depression in patients with hemifacial spasm (HFS) and benign essential blepharospasm (BEB).Patients and methodNinety idiopathic HFS patients and 90 BEB patients were enrolled. The anxiety and depression status were evaluated by self-rating anxiety scale (SAS) and self-rating depression scale (SDS), respectively, before and after the injection of BTX-A.ResultsBefore treatment, the SAS and SDS scores of HFS patients were 41.25±6.35 and 42.25±7.57, respectively. The SAS scores were 40.17±8.36 for males and 43.56±6.10 for females (P=0.031). The SDS scores were 40.25±6.46 for males and 45.48±7.31 for females (P=0.008). After treatment, the SAS and SDS scores were 30.12±4.35 and 30.58±4.89, respectively. There was a significant difference in the SAS and SDS scores before and after treatment. Before treatment, the SAS scores of male and female BEB patients were 56.45±8.75 and 60.89±9.11, respectively, and the SDS scores of male and female BEB patients were 57.90±7.93 and 60.12±8.35, respectively. After treatment, the SAS score was 38.17±3.67 and the SDS score was 38.12±4.15, with a significant difference in before and after treatment scores.ConclusionIn HFS and BEB, especially in female patients, there is an association with anxiety and depression. BTX-A can improve the symptoms of anxiety and depression.

Increase lipid tear thickness after botulinum neurotoxin A injection in patients with blepharospasm and hemifacial spasm

The aim of this study was to investigate changes in the tear film lipid layer thickness (LLT) and aqueous tear production after botulinum neurotoxin A (BoNT) injection in patients with benign essential blepharospasm (BEB) and hemifacial spasm (HFS). Eleven and six patients with BEB and HFS, respectively, who received BoNT injection were consecutively enrolled in this prospective study. The blepharospasm disability index (BSDI), blink pattern, dry eye symptoms, Schirmer test 1 findings, LLT, eyelid performance, and corneal integrity were evaluated before and after treatment. Both BEB and HSF patients experienced remarkable relief from spasms and ocular discomfort after BoNT injection. LLT, the partial blink rate, the snap-back time, the lid distraction distance, and lateral canthal laxity were significantly increased at 1 month after treatment. There were no significant changes in Schirmer test 1 findings and meibomian gland dropout. Our findings suggest that LLT, a decisive factor for tear film stability, significantly increases at 1 month after BoNT injection for BEB and HFS. A decrease in BSDI and an increase in the snap-back time may contribute to the increase in LLT; this mechanism is probably responsible for the relief from dryness after BoNT injection in patients with facial movement disorders.

Effect of Botulinum Toxin A Treatment on Eyelid Pressure in Eyes with Blepharospasm

ABSTRACTObjective: To determine the effect of botulinum toxin A (BTX-A) on the eyelid pressure in patients with benign essential blepharospasm (BEB).Methods: Twenty normal volunteers (10 men, 10 women; average age 59.7 ± 11.3 years) and 33 patients (12 men, 21 women; average age 61.1 ± 14.7 years) with BEB were studied. The upper and lower eyelid pressures were measured with a blepharo-tensiometer in the normal subjects (N group). The eyelid pressures and intraocular pressures (IOP) were measured before and after the BTX-A injections in the BEB patients (B group).Results: The mean eyelid pressure in the N group was 31.0 ± 6.8 mmHg for the upper eyelid and 29.9 ± 6.5 mmHg for the lower eyelid (P > 0.05). The mean eyelid pressure in the B group before treatment was 35.3 ± 7.0 mmHg for the upper eyelid and 37.8 ± 6.6 mmHg for the lower eyelid. The eyelid pressure in the B group was significantly higher than in the N group for the upper and lower eyelids (both P < 0.001). The eyelid pressure was significantly decreased after BTX-A treatment for the upper (29.9 ± 7.5 mmHg) and the lower (32.8 ± 7.0 mmHg) eyelids (both P < 0.001). The mean IOP was 15.1 ± 2.9 mmHg before, and it significantly decreased to 14.5 ± 2.8 mmHg (P = 0.020) after the BTX-A injections. The IOP was significantly correlated with the lower eyelid pressure (P = 0.0435), but not with the upper eyelid pressure (P = 0.175).Conclusion: The eyelid pressure was higher in the patients with BEB. The eyelid pressure and the IOP were significantly reduced after the BTX-A injections. In addition, the IOPs were significantly correlated with the eyelid pressure of the lower eyelid.

Clinical Features of Benign Essential Blepharospasm in Korean Patients.

PurposeTo analyze the clinical features of benign essential blepharospasm in Korean patients.MethodsPatients diagnosed with benign essential blepharospasm in Kim's Eye Hospital from November 2014 to December 2016 were evaluated using a clinical examination and questionnaire. The questionnaire reviewed personal medical history, demographic factors, risk factors for blepharospasm development, and relieving and aggravating factors.ResultsOf the 101 patients enrolled, 78 (77.2%) were women. The mean age was 64.9 years old. Hypertension was the most common medical disorder (42.6%), followed by diabetes mellitus. The majority of the patients were non-smokers (83.2%) and drank less than a cup of a caffeinated beverage a day (30.7%). Fifty-seven percent of patients reported no stressful events immediately prior to symptom development. Fatigue and stress were aggravating factors in more than 55% of patients; rest was the most common relieving factor (35.6%).ConclusionsHere, we report the clinical features of benign essential blepharospasm in Korean patients for the first time. The results were consistent with previous reports showing that the majority of benign essential blepharospasm patients are women and non-smokers. In contrast to previous reports though, fatigue and stress were aggravating factors, and the most common relieving factor was rest. No stressful events had immediately preceded the development of blepharospasm in 57.4% of patients. This report may aid in treating and counseling patients with benign essential blepharospasm.

Grey Matter Microstructural Integrity Alterations in Blepharospasm Are Partially Reversed by Botulinum Neurotoxin Therapy.

ObjectiveBenign Essential Blepharospasm (BEB) and hemifacial spasm (HFS) are the most common hyperkinetic movement disorders of facial muscles. Although similar in clinical presentation different pathophysiological mechanisms are assumed. Botulinum Neurotoxin (BoNT) is a standard evidence-based treatment for both conditions. In this study we aimed to assess grey matter microstructural differences between these two groups of patients and compared them with healthy controls. In patients we furthermore tracked the longitudinal morphometric changes associated with BoNT therapy. We hypothesized microstructural differences between the groups at the time point of maximum symptoms representation and distinct longitudinal grey matter dynamics with symptom improvement.MethodsCross-sectional and longitudinal analyses of 3T 3D-T1 MRI images from BEB, HFS patients prior to and one month after BoNT therapy and from a group of age and sex matched healthy controls. Cortical thickness as extracted from Freesurfer was assessed as parameter of microstructural integrity.ResultsBoNT therapy markedly improved motor symptoms in patients with BEB and HFS. Significant differences of grey matter integrity have been found between the two patients groups. The BEB group showed lower cortical thickness at baseline in the frontal-rostral, supramarginal and temporal regions compared to patients with HFS. In this group BoNT treatment was associated with a cortical thinning in the primary motor cortex and the pre-supplementary motor area (pre-SMA). Contrary patients with HFS showed no longitudinal CT changes. A decreased cortical thickness was attested bilaterally in the temporal poles and in the right superior frontal region in BEB patients in comparison to HC. Patients in the HFS group presented a decreased CT in the left lingual gyrus and temporal pole.ConclusionsAlthough patients with BEB and HFS present clinically with involuntary movements of facial muscles, they exhibited differences in cortical thickness. While BoNT therapy was equally effective in both groups, widespread changes of cortical morphology occurred only in BEB patients. We demonstrated specific disease- and therapy-dependent structural changes induced by BoNT in the studied hyperkinetic conditions.

Comparison of Two Botulinum Neurotoxin A Injection Patterns with or without the Medial Lower Eyelid in the Treatment of Blepharospasm.

The aim of this study was to evaluate the efficacy of two botulinum toxin A (BoNT-A) injection patterns with or without the medial lower eyelid (MLE) in treating benign essential blepharospasm (BEB) and influencing lacrimal drainage. Two different injection patterns of BoNT-A were randomly applied to 98 eyes of 49 BEB patients: MLE Group received a full injection pattern of 5 sites and non-MLE Group received a MLE waived injection pattern of 4 sites. Tear breakup time (BUT), Schirmer I test, lagophthalmos height, and lower lid tear meniscus height (TMH) were measured and Jankovic Rating Scale (JRS) was surveyed before injection and at 1 week, 1 month, and 3 months after injection. The symptom of BEB was relieved in both groups as suggested by JRS scores at all time points after injection, and MLE Group came up with a better JRS score at 3 months. The increases of Schirmer I test value and TMH in MLE Group were higher than those in non-MLE Group at 1 week after injection. This study shows that the MLE-involved full injection pattern is a better choice for patients with BEB. It has longer-lasting effects in relieving BEB symptoms and better efficacy in reducing lacrimal drainage. Clinical Trials registration number is NCT02327728.

The Influence of Benign Essential Blepharospasm on Dry Eye Disease and Ocular Inflammation

To study the influence of blepharospasm on dry eye disease by analyzing the clinical features, tear cytokine, and treatment response of patients with dry eye disease accompanied by benign essential blepharospasm. Prospective case series study. Forty adults with a diagnosis of benign essential blepharospasm (BEB) and dry eye disease (DED) were consecutively recruited. Forty subjects with dry eye disease only and 40 healthy adults were recruited as eligible controls. A tear specimen was collected from all participants for cytokine analysis. The patients with benign essential blepharospasm were treated with botulinum neurotoxin type A. The main outcome measures were the following: (1) Ocular Surface Disease Index (OSDI) questionnaire; (2) clinical features, including tear break-up time (BUT), Schirmer І test, and fluorescein staining; (3) conjunctival impression cytology; and (4) multiplex cytokine immunobead assay. The symptoms of DED+ BEB patients were significantly different from those of DED controls and healthy controls. Cytokine analysis in tear fluid also showed that tumor necrosis factor-α, interleukin (IL)-1β, IL-6, IL-2, IL-17, and vascular endothelial growth factor levels were significantly increased in DED+ BEB patients. In treatment, botulinum neurotoxin type A injection effectively relieved blepharospasm in all of the DED+ BEB patients. Moreover, in this group of patients, OSDI decreased significantly after the botulinum neurotoxin type A injection, and BUT was increased as well. BEB may participate in the progress of inflammation in DED+ BEB patients. Botulinum neurotoxin type A injections could effectively relieve the symptoms of DED+ BEB patients and improve their ocular surface condition.

A Resting State Functional Magnetic Resonance Imaging Study of Patients With Benign Essential Blepharospasm

Benign essential blepharospasm (BEB) is a neurologic disorder characterized by an adult-onset focal dystonia that causes involuntary blinking and eyelid spasms. The pathophysiology of BEB patients remains unclear. This study investigated intrinsic low-frequency fluctuation in BEB patients during resting state functional magnetic resonance imaging (fMRI). The study included 9 patients with BEB (mean age, 61.7 years; range, 52-66 years), in whom the average duration of symptoms was 2.7 ± 1.8 years, and another 9 subjects from an age- and sex-matched control group. Resting state fMRI was performed in both the patients with BEB and the normal controls. Voxel-based analysis was used to characterize the alteration of amplitude of low-frequency fluctuation (ALFF) in both patients with BEB and the normal controls. The whole brain analysis indicated that in comparison with the normal control group, there was a significantly increased ALFF in the left putamen, pallidum, insular lobe, and medial prefrontal cortex and a significantly decreased ALFF in the bilateral somatosensory regions, thalami, cerebellum, and medial and posterior cingulate cortex. The present study suggests that both an abnormal default mode network and corticostriatopallidothalamic loop may play a role in the pathophysiology of BEB.