[The efficacy and safety of nerve loop blocking in the treatment of blepharospasm in Meige syndrome].

Objective: To evaluate the efficacy and safety of nerve loop blocking in the treatment of blepharospasm caused by Meige syndrome. Methods: It was a retrospective case series study. Patients with Meige syndrome characterized by blepharospasm or blepharospasm-oromandibular dystonia who underwent nerve loop blocking in the Ophthalmology Department of Henan No. 3 Provincial People's Hospital from April 2018 to January 2020 were included. Before and after surgery, blepharospasm was graded, and the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was used to score the symptoms of ocular and oral dystonia. The improvement rate of ocular spasm was calculated after surgery according to the scores. The postoperative complications were observed. The Wilcoxon rank test was used to compare the BFMDRS movement subscale scores before and after surgery. The independent sample Mann-Whitney U test was used to compare the improvement rates of eye spasm between male and female patients and between patients with and without combined oromandibular dystonia. Results: Among the 199 patients included, 64 (32.2%) were males, and 135 (67.8%) were females, aged 58 (51, 64) years (22-79 years). The postoperative follow-up period was 24 (21, 28) months. Preoperatively, blepharospasm was graded as grade 3 in 12 patients (6.0%) and grade 4 in 187 patients (94.0%), while the postoperative blepharospasm grades were grade 0 in 100 patients (50.3%), grade 1 in 64 patients (32.2%), grade 2 in 31 patients (15.6%), and grade 3 in 4 patients (2.0%). There was statistically significant difference in the BFMDRS scores of ocular dystonia before and after surgery [8.0 (8.0, 8.0) vs. 0.0 (0.0, 1.0); Z=-12.41, P<0.001]. The improvement rate of blepharospasm in all patients was 100% (87.5%, 100%), ranging from 43.8% to 100%, with no statistically significant difference between patients of different genders and between patients with and without combined oral dystonia (both P>0.05). Statistically significant difference existed in the scores of oral dystonia before and after surgery [2.0 (0.5, 4.5) vs. 1.0 (0.5, 2.0); Z=-4.38, P<0.001], with 25 of 65 patients (38.5%) having their oral symptoms improved. Postoperative complications included eyelid valgus (7.5%, 15/199), frontal numbness (100%, 199/199) and tearing (89.9%, 179/199). Conclusion: Nerve loop blocking is a relatively safe and effective method in the treatment of blepharospasm symptoms of Meige syndrome.

Suicidal ideation, hopelessness, and affective temperament in patients with blepharospasm.

OBJECTIVE: The aim of this study was to investigate the relationship between suicidal ideation and neurological, psychological, and psychiatric features in patients with blepharospasm (BSP). METHODS: We enrolled 70 BSP patients and 80 control subjects. All participants underwent a psychiatric and psychometric evaluation: Structured Clinical Interview, Clinical Global Impression, Hamilton Anxiety Rating Scale, Hamilton Depression Rating Scale, Columbia-Suicide Severity Rating Scale, Beck Hopelessness Scale, Temperament Evaluation of Memphis, Pisa, San Diego Auto-questionnaire. BSP severity was assessed using the Blepharospasm Severity Rating Scale. RESULTS: Suicidal ideation was reported in 18% of BSP patients and 6% had current suicidal ideation. 83% of BSP patients had severe hopelessness. BSP patients presented an increased sense of hopelessness (OR= 1.39, 95% CI = 1.13/1.70) and a pronounced depressive temperament (OR= 1.36, 95% CI = 1.12/1.65). Suicidal ideation in BSP patients correlated with psychiatric disorders (OR = 3.96, 95% CI = 1.23/12.74) and higher scores on the HAM-A (OR = 1.11, 95% CI = 1.02/1.20), HAM-D (OR = 1.18, 95% CI = 1.05/1.32), CGI (OR = 1.85, 95% CI = 1.18/2.90), TEMPS-A Cyclothymia (OR = 1.16, 95% CI = 1.02/1.31). CONCLUSION: Our findings suggest the presence of suicidal ideation and severe hopelessness in BSP patients.KEY POINTSBSP patients as compared to controls more frequently reported the presence of a psychiatric disorder and more severe anxiety and depressive symptoms, psychopathology on the CGI, suicidal ideation, and hopelessness.BSP patients with prevalent cyclothymic temperament had more severe suicidal ideation, suggesting an increased suicide risk most likely due to difficulties in psychological adaptation to changing environments, including the neurological disease.A psychiatric assessment is recommended for patients with this condition, with possible referral to a suicide prevention centre.

Developments in the treatment of benign essential blepharospasm.

PURPOSE OF REVIEW: To review new developments in the medical and surgical treatment options for benign essential blepharospasm (BEB). RECENT FINDINGS: Botulinum toxin injections remain the mainstay treatment for BEB with several formulations currently commercially available. Reports in the medical literature support photochromatic modulation for the symptoms of photophobia, as well as oral medications and surgical myectomy for control of the motor signs of eyelid protractor spasm. SUMMARY: Although there remains no cure for BEB, several treatment options are available to effectively manage the signs and symptoms of the condition.

[Botulinum Toxin - New Developments in Ophthalmology]. / Botulinumtoxintherapie ­ neue Entwicklungen in der Augenheilkunde.

Botulinum toxin is recognised as the gold standard for the treatment of essential blepharospasm and hemifacial spasm, which is similar in effect in synkinesis after facial nerve palsy. The injection intervals can be adjusted according to the patients' needs and be shortened for up to six weeks in cases of eyelid cramping. Newer indications for the use of botulinum toxin in ophthalmology include eyelid retraction in Graves' disease, induction of protective ptosis and treatment of crocodile tears syndrome after facial nerve palsy. In future, botulinum toxin may be used in depression (facial feedback), facial injuries and for protection of facial glands against irradiation injury.

Blepharospasm 40 years later.

Forty years ago, C.D. Marsden proposed that blepharospasm should be considered a form of adult-onset focal dystonia. In the present paper, we provide a comprehensive overview of the findings regarding blepharospasm reported in the past 40 years. Although prolonged spasms of the orbicularis oculi muscles remain the clinical hallmark of blepharospasm, patients with blepharospasm may be characterized by various types of involuntary activation of periocular muscles. In addition to motor features, blepharospasm patients may also have nonmotor manifestations, including psychiatric, mild cognitive, and sensory disturbances. The various motor and nonmotor symptoms are not present in all patients, suggesting that blepharospasm is phenomenologically a heterogeneous condition. This emphasizes the need for tools for severity assessment that take into account both motor and nonmotor manifestations. The cause of blepharospasm remains elusive, but several lines of evidence indicate that blepharospasm is a multifactorial condition in which one, or several, as yet unknown genes together with epigenetic and environmental factors combine to reach the threshold of the disease. Although blepharospasm was originally believed to be solely a basal ganglia disorder, neurophysiological and neuroimaging evidence point to anatomical and functional involvement of several brain regions. The contribution of multiple areas has led to the hypothesis that blepharospasm should be considered as a network disorder, and this might reflect the varying occurrence of motor and nonmotor manifestations in blepharospasm patients. Despite advances in the aetiology and pathophysiology, treatment remains symptomatic. © 2017 International Parkinson and Movement Disorder Society.

Blepharospasm in children and adolescents.

PURPOSE: Benign essential blepharospasm (BEB) generally is considered a disorder of adults; however, it rarely can present in childhood or adolescence. The main purpose of this study was to determine the prevalence of BEB in children and adolescents. Our research question was whether blepharospasm is seen in children or adolescents as well as in the adult population. METHODS: We conducted a retrospective chart review at the University of Utah and Johns Hopkins University. We reviewed our databases for diagnoses of blepharospasm and tic disorder over the past 10 years in patients of all ages. Charts then were reviewed to confirm the diagnosis, and a questionnaire was sent to subjects whose blepharospasm had apparently begun before age 20 years. RESULTS: We identified 26 patients diagnosed with eyelid spasms that had begun while under the age of 20. We confirmed BEB in four of these cases. Of these individuals, all had developed symptoms in adolescence or before and all were still symptomatic but had noted improvement in the severity and frequency of their symptoms. CONCLUSIONS: Although rare, BEB can develop in the first decade of life, producing symptoms and signs that are similar to adults, with persistence into adulthood.

Effect of upper eyelid myectomy on subsequent chemodenervation in the management of benign essential blepharospasm.

PURPOSE: Some patients with severe benign essential blepharospasm (BEB) become clinically refractory to chemodenervation with botulinum toxin. In these patients, surgical myectomy is an effective additive treatment for the management of this disabling condition. The purpose of this study is to determine how myectomy for BEB alters subsequent botulinum toxin administration. METHODS: A retrospective chart review of 27 patients undergoing surgical myectomy for the treatment of refractory BEB was conducted. The frequency and dosage of botulinum toxin were compared between preoperative and postoperative treatments. Paired Student t tests were used to determine significance (α = 0.05). RESULTS: The mean interval between botulinum treatments was 10.1 weeks preoperatively and 15.7 weeks postoperatively (p < 0.001). The mean quantity of botulinum toxin administered was 68 units prior to myectomy and 64.9 units postoperatively (p = 0.227). When comparing the quantity of botulinum toxin injected into the upper eyelid region only (site of myectomy), there was a significant decrease in the dose administered preoperatively and postoperatively (46.8 units vs. 39.9 units, p = 0.00310). CONCLUSIONS: Upper eyelid myectomy is an effective treatment modality for BEB in those patients who are refractory to botulinum toxin injections. Myectomy for BEB decreases the morbidity, botulinum toxin treatment frequency, and long-term expense associated with this disabling condition.

Microlesion Effect Induced by Electrode Implantation in the Posteroventral Globus Pallidus Interna for Severe Dystonic Tics.

Background: Tourette syndrome (TS) is a neurologic condition characterized by motor and phonic tics. Dystonic tics, including blepharospasm, are considered atypical or unusual in severe TS. Case Report: We report a severe case of TS with facial dystonic tics resembling blepharospasm in which the microlesion effect and a sustained therapeutic effect was observed with bilateral globus pallidus interna (GPi) deep brain stimulation (DBS). Discussion: Bilateral GPi DBS can be beneficial for blepharospasm-like tics and severe symptoms of TS. The improvements seen can be explained by the microlesion effect induced by DBS lead placement in the GPi.

Long-term depression-like plasticity of the blink reflex for the treatment of blepharospasm.

Our previous work showed a beneficial therapeutic effect on blepharospasm using slow repetitive transcranial magnetic stimulation, which produces a long-term depression (LTD)-like effect. High-frequency supraorbital electrical stimulation, asynchronous with the R2 component of the blink reflex, can also induce LTD-like effects on the blink reflex circuit in healthy subjects. Patients with blepharospasm have reduced inhibition of their blink recovery curves; therefore, a LTD-like intervention might normalize the blink reflex recovery (BRR) and have a favorable therapeutic effect. This is a randomized, sham-controlled, observer-blinded prospective study. In 14 blepharospasm patients, we evaluated the effects of high-frequency supraorbital stimulation on three separate treatment days. We applied 28 trains of nine stimuli, 400 Hz, either before or after the R2 or used sham stimulation. The primary outcome was the blink rate, number of spasms rated by a blinded physician and patient rating before, immediately after and 1 hour after stimulation while resting, reading, and talking; secondary outcome was the BRR. Stimulation "before" and "after" the R2 both showed a similar improvement as sham stimulation in physician rating, but patients felt significantly better with the before condition. Improvement in recovery of the blink reflex was noted only in the before condition. Clinical symptoms differed in the three baseline conditions (resting, reading, and talking). Stimulation before R2 increased inhibition in trigeminal blink reflex circuits in blepharospasm toward normal values and produced subjective, but not objective, improvement. Inhibition of the blink reflex pathway by itself appeared to be insufficient for a useful therapeutic effect.

Tardive Blepharospasm May Respond to Bilateral Pallidal Deep Brain Stimulation.

Background: To date, there have been no reports of tardive blepharospasm being treated with deep brain stimulation (DBS), though there have been two reports of focal blepharospasm responding favorably to bilateral pallidal DBS. Case: A 34 year old man with tardive blepharospasm that was refractory to oral medications as well as botulinum toxin types A and B underwent bilateral pallidal DBS under general anesthesia. He had significant improvement of his severe blepharospasm by one and half months post-DBS which was sustained at last follow-up 30 months post-DBS. The best programming parameters included pulse widths of 90-100 µsec, frequencies of 140-150 Hz, and stimulating the ventral contacts in each side. Conclusion: Our case represents the first report of medically refractory tardive blepharospasm responding favorably to bilateral pallidal DBS.

Electrical activation of the orbicularis oculi muscle does not increase the effectiveness of botulinum toxin type A in patients with blepharospasm.

BACKGROUND: Our primary aim in this study was to determine whether electrically induced activation of the injected muscle increases effectiveness of botulinum type A toxin (BonT-A) in patients with blepharospasm (BPS). The second aim was to assess the safety of BonT-A by investigating whether BonT-A injection alters the excitability of blink reflex circuits in the brainstem. METHODS: Twenty-three patients with BPS received BonT-A (Botox) injected bilaterally into the orbicularis oculi muscle at a standard dose. In 18 patients, electrically induced muscle activation of the orbicularis oculi muscle on one side was performed for 60 min (4 Hz frequency) in a single session, immediately after BonT-A injection and in five patients for 60 min once a day for five consecutive days. The severity of BPS was assessed clinically with the BPS score. Compound muscle action potential (cMAPs) from the orbicularis oculi muscles were measured bilaterally. The blink reflex recovery cycle was studied at interstimulus intervals of 250 and 500 ms. Participants underwent clinical and neurophysiological assessment before BonT-A injection (T0) and 2 weeks thereafter (T1). RESULTS: Compound muscle action potential amplitude significantly decreased at T1 but did not differ between stimulated and non-stimulated orbicularis oculi in the two groups. BonT-A injection left the blink reflex recovery cycle tested on the stimulated and non-stimulated sides unchanged. CONCLUSIONS: In patients with BPS, the electrically induced muscle activation neither increases the effectiveness of BonT-A nor produces larger electrophysiological peripheral effects. The lack of BonT-A-induced changes in the blink reflex recovery cycle provides evidence that BonT-A therapy is safe in patients with BPS.

FL-41 tint improves blink frequency, light sensitivity, and functional limitations in patients with benign essential blepharospasm.

OBJECTIVE: The objective of these 2 studies was to assess the efficacy of FL-41-tinted lenses in the treatment of benign essential blepharospasm (BEB). DESIGN: A randomized crossover study and a randomized crossover case-control study. PARTICIPANTS: The first study included 30 subjects with BEB. The second study included 26 subjects with BEB and 26 controls. METHODS: For the first study, subjects were randomized to wear either FL-41 or gray-tinted lenses for 2 weeks. After a 2-week washout period, the other lens was worn for 2 weeks. Questionnaires were completed at baseline, after the first lens, and after the second lens. In the second study, surface electromyography (EMG) was used to measure blink frequency, duration, and force while subjects read and wore FL-41, rose, or gray-tinted lenses. MAIN OUTCOME MEASURES: Questionnaires were used to assess perceptions of light sensitivity and the effect of light sensitivity on activities of daily living (ADL). EMG was used to measure blink frequency, duration, and force. RESULTS: Most participants observed improvement while wearing both FL-41 and gray-tinted lenses. FL-41-tinted lenses provided superior improvement in the areas of reading, fluorescent light sensitivity, overall light sensitivity, blepharospasm frequency, and blepharospasm severity. FL-41 lenses reduced mean blink rate compared with both rose and gray-tinted lenses, and reduced eyelid contraction force compared with rose-tinted lenses. CONCLUSIONS: FL-41 lenses provided both subjective and objective benefit to subjects with BEB. Physicians should consider recommending this noninvasive and inexpensive lens tint to patients with BEB. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Stimulation-induced parkinsonism after posteroventral deep brain stimulation of the globus pallidus internus for craniocervical dystonia.

The authors report on a patient with craniocervical dystonia who was treated with bilateral GPi stimulation, with excellent improvement in dystonia but at the cost of stimulation-induced, reversible parkinsonism. Stimulation through ventral contacts resulted in maximal relief of craniocervical dystonia but induced considerable hypophonia, bradykinesia, rigidity, freezing, and impaired postural reflexes. Stimulation through dorsal contacts alleviated parkinsonism, but resulted in the return of dystonia. No stimulation parameters could alleviate the dystonia without inducing parkinsonism over the course of his 4-year follow-up.

A trial of a mechanical device for the treatment of blepharospasm.

BACKGROUND: Idiopathic blepharospasm (IB) is a rare but well-characterised adult onset focal dystonia that may cause severe visual disability. The most effective treatment is with periodic injections of botulinum toxin (BTX) into the pre-tarsal and/or pre-septal orbicularis oculi muscles bilaterally. However, even with treatment, practical visual function often remains compromised. A subset of IB sufferers find that eye opening improves with a focal unilateral digital pressure usually on a specific point on the temple. This is known as a 'sensory trick'. We have developed a spectacle mounted device ('Pressop') to apply continuous individually localised focal pressure on the temple to mimic the effect of finger pressure. The aim of the study was to determine if the 'sensory trick' could be replicated by Pressop and if the interval between BTX treatments could thereby be extended. SUBJECTS/METHODS: Study participants had three clinic visits-an initial screening assessment, a visit 2 weeks before the next injection was due when the device was fitted, and one 2 weeks later to assess the response to Pressop. A CDQ 24 and device-specific feedback questionnaire were completed and comparison photographs were taken. Repeat BTX injections were administered at the third visit. RESULTS: Of 58 patients with typical IB recruited to the trial, 39 reported an effective focal finger pressure sensory trick. 56 completed the trial, more than 50% of whom reported some benefit using Pressop; 18% had substantial improvement, sustained for up to 5 years. Improvement could occur in those without an effective sensory trick, therefore there was no significant correlation between using a sensory trick and benefiting from 'Pressop'. There was a trend towards the responders having greater improvement in CDQ24 total score than non-responders but this was not statistically significant. CONCLUSIONS: We recommend a trial of this simple safe device as a means of augmenting visual function in all IB patients.

Activating the levator to elevate the eyelid.

PURPOSE: To demonstrate in an animal model the feasibility of elevating the eyelid in a functionally useful manner by chronically stimulating the levator palpebrae superioris (LPS) muscle with an implanted electrode. METHODS: Five rabbits were implanted with electrodes designed to stimulate the nerve innervating the LPS near its entry to the muscle. Bipolar platinum electrodes in a silicone rubber envelope with silicone-sleeved, PTFE-coated platinum lead wires were used to provide long-term stimulation with bipolar square-wave pulse trains of 0.18-0.80 mA and 200 Hz at a duty cycle of 8 seconds on and 2 seconds off. Explanted electrodes were examined for damage, and stimulated tissues were evaluated for abnormalities by light microscopy. RESULTS: We achieved mean lid elevation of 1.6 mm, approaching the diameter of the light-adapted pupil, with 0.5 mA stimulus. Stimulus currents below 1.0 mA produced no signs of discomfort. Three animals with which we attempted daily stimulation, averaged 16.1 hours per week. Experiments lasted 22 weeks on average. Lid lifting with a well-implanted platinum electrodes was stable, with no apparent tissue or electrode damage after as long as 29.1 weeks. CONCLUSIONS: Stable, functionally useful eyelid lifting was achieved with stimulation currents that caused no apparent discomfort or damage to muscles or nerves. A simple, discrete bipolar electrode was effective and survivable.

Treatment Effects of Botulinum Toxin Injection and Acupuncture on Blepharospasm Assessed by the Change in Lower Eyelid Tension.

Objective: To investigate the treatment effects of botulinum toxin-A (BTA) injection and acupuncture on blepharospasm (BP) evaluated by the change in lower eyelid tension (LET). Methods: A series of 30 patients (male: 8, female: 22) aged between 37 and 83 years (63.80 ± 10.96 yrs) who met the eligibility criteria of BP were recruited in this study, who were randomly assigned to BTA injection group (BTA group, n = 15) and acupuncture treatment group (Acupuncture group, n = 15). BTA injections were administered to the patients in BTA group while patients in acupuncture group received the acupuncture treatment. The LET was measured by a tensiometer in both groups at baseline and at post-treatment. Results: A significant decrease in LETs over 8 weeks was found in acupuncture group (812.76 ± 193.95 Pa at baseline, 549.69 ± 150.04 Pa at 4 weeks, and 510.96 ± 150.66 Pa at 8weeks, respectively; F = 31.127, p << 0.001). There was a significant decrease in LET from 858.61 ± 190.54 Pa at baseline to 414.45 ± 63.38 Pa at 2 weeks after treatment (Z = -4.542, p << 0.01) in BTA group. At the endpoint of the study, a significant difference in LET was found between the acupuncture group (301.80 ± 181.77 Pa) and the BTA group (444.16 ± 193.44 Pa) (t = -2.077, p = 0.047). Conclusions: BP patients have an increased LET. Both BTA and acupuncture are effective in decreasing the LET. Close monitoring of LET holds promise in planning the treatment strategy for Blepharospasm.