[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.

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.

[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.

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)

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.

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.

Blefaroespasmo essencial: revisão da literatura / Essential blepharospasm: literature review

O blefaroespasmo essencial é uma distonia focal caracterizada por contrações involuntárias, espasmódicas e bilaterais dos músculos orbicular da pálpebra, corrugador, prócerus e depressor do supercílio. Trata-se de uma doença rara, cujo diagnóstico é frequentemente tardio, o que permite a progressão dos sintomas até quadros de cegueira funcional. Nesse estudo, por meio de revisão da literatura, descreve-se as principais características da doença, incluindo os seguintes aspectos: histórico, epidemiologia, etiologia, quadro clí­nico, diagnóstico diferencial e tratamento.

Essential blepharospasm is a focal distonia characterized by involuntary, spasmodic, bilateral contractions of eyelid protractors. This is a rare disease, which diagnosis is frequently late and symptoms may progress until functional blindness. In this article, we perform a review and describe the most important features of the disease, including historical aspects, epidemiology, etiology, clinical findings, differential diagnosis and treatment.

[What to do in cases of inadequate effectiveness of botulinum toxin for the treatment of eyelid cramping?]. / Was tun bei unzureichender Wirkung von Botulinumtoxin in der Behandlung von Lidkrämpfen?

In 5-15% of individuals suffering essential blepharospasm, treatment with botulinum toxin is insufficient. In these cases the following measures can be applied: increase the dose, increase or vary the injection sites, change the botulinum toxin medication, use additional oral medication, or perform surgery. Certain measures can also be carried out by the patient. In cases of hemifacial spasm, inadequate results of botulinum toxin injections are even more unusual. But for these cases, additional oral medication or the neurosurgical Janetta operation can be used.

Assessment of blepharospasm surgery using an improved disability scale: study of 138 patients.

PURPOSE: To assess a new scale for grading functional disability in a series of patients operated on for blepharospasm resistant to treatment by botulinum toxin (BT) injections. METHODS: The bicentric study included 138 patients: 80 (58%) with essential blepharospasm; 46 (33%) with apraxia of eyelid opening; and 12 (9%) with intermediate forms. They had undergone orbicularis myectomy (92 cases, 67%), frontalis suspension (31 cases, 22%), or both (15 cases, 11%). Functional disability was assessed by using an original scale covering 6 daily life activities, each graded using 5 levels from 0 (no disability) to 4 (activity impossible). The score obtained was related to the total of activities actually performed to produce a functional disability score (FDS) from 0 to 100. Each patient's FDS was prospectively determined, before surgery and 3 months after surgery (M3). In patients who, after surgery, required a further course of BT injections, the FDS was assessed immediately before the third injection. RESULTS: The mean FDS fell significantly, from 78 +/- 15 (standard deviation) before surgery to 45 +/- 21 at M3 (p < 0.01%), whatever the clinical form of essential blepharospasm or surgical procedure. The mean postoperative FDS was significantly lower in the 62 patients (45%) weaned off BT than in the unweaned group, respectively (31 +/- 17 versus 56 +/- 18; p < 0.01). In the latter group, the mean FDS after BT reinjection (34 +/- 19) fell to a level comparable with that of the weaned patients. CONCLUSIONS: This accurate, easy-to-use scale makes it possible to quantify in patients with blepharospasm functional improvement as the result of surgery and, where applicable, of BT reinjection.

Is acupuncture an useful tool for pain-treatment in ophthalmology?

Pain that does not respond to conventional treatment procedures makes it necessary to look for alternative methods. Acupuncture is an ancient procedure with empirical effects on pain. Previous studies established the increased output of messengers at neuronal junctions in spinal cord and hypothalamic locations, especially of endorphins which inhibit the perception of pain. We treated several painful symptoms with acupuncture and evaluated the outcome of the treatment. Patients with various kinds of therapy-refractory pain and patients in whom conventional treatment methods could not be applied were included in the study. The diagnoses included glaucoma. Tolosa-Hunt-Syndrome, ophthalmic migraine, blepharospasm, and dry eyes. In one case acupuncture was used for analgesia during surgery. Acupuncture was performed with sterile disposable needles, at points known to have an empirical analgesic effect. The stimulation was adapted to the patient's individual needs. VAS assessments before and after acupuncture were compared. The t-test was used for statistical evaluation. Acupuncture had no side effects, but reduced pain to a variable extent. Especially in cases of severe pain and in surgery, very effective pain reduction was achieved. In general, pain was significantly reduced in all patients by the use of acupuncture. A statistically significant effect was noted (p < 0.05). Further studies should be conducted to demonstrate the specific effect in larger patient populations. Monitoring neurotransmitter activity will possibly help to illustrate the effect.

Blepharospasm: past, present, and future.

To investigate causes, associations, and results of treatment with blepharospasm, 1,653 patients were evaluated by extensive questionnaires to study blepharospasm and long-term results of treatment with the full myectomy operation, botulinum-A toxin, drug therapy, and help from the Benign Essential Blepharospasm Research Foundation (BEBRF). The percent of patients improved by the BEBRF was 90%, full myectomy 88%, botulinum-A toxin 86%, and drug therapy 43%. The patient acceptance rate for the BEBRF was 96%, full myectomy 82%, botulinum-A toxin 95%, and drug therapy 57%. Blepharospasm is multifactorial in origin and manifestation. A vicious cycle and defective circuit theory to explain in origin and direct treatment rather than a defective specific locus is presented. All four forms of therapy evaluated are useful and must be tailored to the patient's needs. Mattie Lou Koster and the BEBRF have helped blepharospasm sufferers more than any other modality, and all patients should be informed of this support group. The full myectomy is reserved for botulinum-A toxin failures, and the limited myectomy is an excellent adjunct to botulinum-A toxin.

Necrotising fasciitis as a complication of botulinum toxin injection.

PURPOSE: To highlight the need for early diagnosis and treatment of the rare condition of necrotising fasciitis as a complication of botulinum toxin injection, and to illustrate that injections in immunocompromised patients carry a rare but serious risk. RESULTS AND METHODS: A case report is presented of an 80-year-old woman suffering from blepharospasm and chronic myeloid leukaemia, who developed necrotising fasciitis 3 days after a botulinum toxin injection. CONCLUSIONS: Chronic debilitating processes such as diabetes, alcoholism and polymyositis have been suggested as predisposing factors in the development of necrotising fasciitis. We believe this is the first reported case of necrotising fasciitis occurring secondary to a botulinum toxin injection. The fact that this infection extended through the fascial planes and led to the death of muscle was, probably, because an inoculum was introduced directly into the muscle at the time of botulinum toxin treatment. This may have led to its deep spread and difficulty in debriding the area. Chronic myeloid leukaemia does not in itself cause significant immunosuppression, but our patient was on anti-proliferative treatment and had a low leucocyte count, which may have been a predisposing factor in this case.

Preventing ptosis after botulinum treatment.

In a series of 33 blepharospasm patients who had the side effect of ptosis following therapeutic botulinum toxin type A (Botox: Allergan, Inc., Irvine, CA, U.S.A.) injection, we administered 41 injections of human botulinum immune globulin (IG) following injections of the toxin to test the dosage and timing of IG injection and its effectiveness in limiting or avoiding ptosis. An IG dose of 3.2 x 10(-3) international units (IU) per unit of Botox was effective in blocking toxin effect when injected into the same tissue site within 4 hours. An IG dose of 1.6 x 10(-2) to 3.2 x 10(-2) into the levator of the eye having more frequent ptosis in 19 patients reduced the incidence of ptosis to 11%. The fellow (control) eye had a ptosis incidence of 37%. No orbital hemorrhage or other adverse effect occurred from the IG or its injection.

Blefaroespasmo: tratamento com toxina botulinica / Blepharospasm: treatment with botulinum toxin

O blefaroespasmo é uma forma de distonia focal caracterizada por contraçöes involuntárias e repetitivas do musculo orbicular dos olhos. Quatorze casos de blefaroespasmo foram submetidos a injeçöes de toxina botulinica tipo A. Dos 14 casos estudados, seis eram do sexo masculino e oito do sexo feminino, sendo que 50 por cento dos pacientes apresentavam a forma isolada do BE e em outros 50 por cento a distonia estendia-se a outros territorios musculares na regiäo cervical ou cranial. A media de idade era de 58 anos (variando de 33 a 76 anos) e a duraçäo média da doenba de 7,3 anos (com extremos de 1 a 25 anos). Todos haviam sido tratados com drogas de acao antidistonica com resultados insatisfatorios. Cinco pacientes haviam sido submetidos a neurectomia superseletiva do nervo facial, com melhora transitoria. Em todos os pacientes do grupo a resposta a toxina botulinica, avaliada atraves de escalas especificas, foi considerada boa. O periodo de latencia para o inicio da resposta foi em media de tres dias (com faixa de 0 a 7 dias)...

Tratamiento de la distonia focal con toxina botulinica / Treatment of focal dystonia with botulinic toxin

Dystonia is a neurologic disease characterized by involuntary repetitive muscular contractions which frequently causes torsion, spasmodic movements or abnormal postures. Pharmacologic and surgical treatment had been unsatisfactory in the majority of the cases. Intramuscular botulinic toxin application in focal dystonia disorders, achieve relief in approximately 90% of the cases by quimiodenervation. We included 35 patients with focal dystonia treated between November 1994 through June 1996: 14 facial hemispasm, nine blepharospasm, five cervical dystonia, two writer's cramps, one oromandibular dystonia and four patients with mixed focal dystonia. Improvement between 50-90% was observed in 32/34 (94%) of the patients; one case (2.8%) with therapeutic failure and one case (2.8%) never returned to quantify the response. Actually, botulinic toxin is the first choice treatment of focal dystonia and other diseases related to involuntary muscle spasm