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.

[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.

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.

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.

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.

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.

Intractable Blepharospasm Treated with Bilateral Pallidal Deep Brain Stimulation.

BACKGROUND: Blepharospasm can be present as an isolated dystonia or in conjunction with other forms of cranial dystonia, causing significant disability. CASE REPORT: We report a case of a 69-year-old male with craniocervical dystonia, manifesting primarily as incapacitating blepharospasm refractory to medical treatments. He underwent bilateral globus pallidus (GP) deep brain stimulation (DBS) with complete resolution of his blepharospasm and sustained benefit at 12 months postoperatively. DISCUSSION: This case illustrates successful treatment of blepharospasm with pallidal stimulation. GP-DBS should be considered a reasonable therapeutic option for intractable blepharospasm.

Uso de la toxina botulínica en pacientes con alteraciones espásticas palpebrales / Use of botulinum toxin in patients with palpebral spastic alterations

Objetivo: evaluar los resultados terapéuticos de la aplicación de toxina botulínica A en pacientes con alteraciones espásticas palpebrales. Métodos: se realizó un estudio observacional, descriptivo, longitudinal y prospectivo en 18 pacientes con estas afecciones, seleccionados en la consulta de Oculoplastia del Instituto Cubano de Oftalmología Ramón Pando Ferrer en el período comprendido entre enero y diciembre del año 2016. Se describió la muestra según la edad, el sexo, el color de la piel, los antecedentes patológicos personales, las afecciones palpebrales y las limitaciones en tareas de la vida diaria. Se evaluó en el posoperatorio el tiempo de desaparición de los síntomas, la duración del efecto de la inyección de toxina botulínica, las complicaciones inmediatas y las mediatas. Resultados: el 70,6 por ciento estuvo comprendido en el rango de edad mayor de 60 años, y el 52,9 por ciento fueron mujeres. La piel blanca constituyó el 58,8 por ciento de la muestra. La afección que se presentó con mayor frecuencia fue el blefaroespasmo (47,1 por ciento). Todos los pacientes presentaron limitaciones para realizar tareas de la vida diaria. El 100 por ciento presentó dolor en el sitio de la inyección. En el 94,1 por ciento de ellos hubo desaparición de los movimientos involuntarios entre el cuarto y el quinto día posterior a la aplicación de xeomeen y en el 88,2 por ciento demoró la reaparición de los síntomas entre cuatro y seis meses. Conclusiones: la toxina botulínica es efectiva en la corrección de las afecciones palpebrales espásticas con desaparición de los síntomas en un período de tiempo entre cuatro y cinco días, con una rápida incorporación del paciente a la vida laboral y social por cuatro a seis meses(AU)

Objective: to evaluate the therapeutic results of the application of toxin botulínica A in patients with palpebral spastic alterations. Methods: an observational, descriptive, longitudinal and prospective study was performed on 18 patients with these conditions, who were selected at the Oculoplasty Service of Ramón Pando Ferrer Cuban Institute of Ophthalmology in the period from January to December 2016. The analyzed variables were age, sex, race, personal pathological history, eyelid disorders and restricted daily life. In the postoperative period, length of time for symptom relief, duration of the effect of botulinum toxin injection, and immediate and mediate complications were assessed. Results: in the group, 70,6 percent were included in the over 60 y age group and 52,9 percent were women. Caucasians accounted for 58,8 percent of the sample. The most frequent condition was blepharospasm (47,1 percent). All patients had limitations to perform daily life tasks. In 94,1 percent of the patients, involuntary movements disappeared between the 4th and the 5th day after the administration of Xeomeen and in 88,2 percent, the symptoms delayed to appear again from 4 to 6 months. Conclusions: botulinum toxin A is an effective alternative to correct palpebral spastic conditions, with symptoms disappearing in four to five days, and quick reincorporation of the patient to working and social life for 4 to 6 months(AU)

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.

Use of Alleviating Maneuvers for Periocular Facial Dystonias.

IMPORTANCE: 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. OBJECTIVE: To determine whether the use of alleviating maneuvers for benign essential blepharospasm or hemifacial spasm correlates with disease severity or botulinum toxin treatment. DESIGN, SETTING, AND PARTICIPANTS: 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. MAIN OUTCOMES AND MEASURES: Prevalence and type of alleviating maneuvers used for blepharospasm and hemifacial spasm, dystonia severity, and dose and frequency of botulinum toxin injections. RESULTS: 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). CONCLUSIONS AND RELEVANCE: 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.

Pallidal stimulation for medically intractable blepharospasm.

Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is an established procedure to improve generalised, segmental craniofacial dystonia. However, no studies have addressed the applicability of DBS for blepharospasm as a focal craniofacial dystonia. A 52-year-old man developed medically intractable involuntary eye closure. Because the abnormal movement was observed exclusively in the eyelids, he was diagnosed not with Meige's syndrome but with blepharospasm as a focal craniofacial dystonia. He underwent stereotactic surgery under general anaesthesia for bilateral GPi-DBS. Continuous GPi stimulation almost completely abolished the blepharospasm. 15 months after the operation, his preoperative scores on the Burke-Fahn-Marsden Dystonia Rating Scale (=8 points) decreased to 1 (87.5% improvement). The present study demonstrates the applicability of GPi-DBS for treating blepharospasm presenting as focal dystonia. Further studies with accumulated case series are needed to confirm the effect of DBS on blepharospasm and other focal craniofacial dystonias.