Facial muscle spasms: An Australian study

Purpose: A group of patients suffering from blepharospasm, hemifacial spasm and Meige's syndrome were surveyed to determine the delay from the onset of their condition until a correct diagnosis was reached, the attitudes of practitioners towards them and their condition, the effect of their condition on their lifestyle and the effects of different types of treatment on their conditions.
Methods: Questionnaires were offered to all patients with blepharospasm, hemifacial spasm and Meige's syndrome presenting to three ophthalmologists licensed to treat patients with botulinum toxin injections over a 12 month period.
Results: Patients consulted an average of 4.4 practitioners before a correct diagnosis was made and many waited a number of years before obtaining satisfactory treatment. Approximately two-thirds of all practitioners consulted were unaware of their condition. Ten per cent of patients reported a family history of similar conditions. Most patients received relief from their symptoms with treatment using injections of botulinum toxin. More than 55% of patients considered themselves to have psychological problems (usually relating to stress and trauma) that they associated with the onset of their condition.
Conclusions: Facial muscie dystonias are rare and patient experiences suggest that they are poorly appreciated in the medical community. From the time they first see a practitioner with symptoms of facial dystonia, patients typically wait 2 years and see four practitioners before a correct diagnosis is made. Stress may be a factor in the symptomatic onset of this condition. Many patients describe pain as part of the presenting symptomatology. Botulinum toxin seems to be effective in the management of facial spasm.     

“Squeezing Eyes.” A Clinical Pathological Conference

The case of a 55-year-old man with severe spasm of the upper and lower eyelids is presented. The differential diagnosis of essential blepharospasm is discussed, as are the various treatment modalities. The importance of careful ophthalmic and neurological examination is emphasized, as, in some instances, blepharospasm is associated with basal ganglia dysfunction.     

A型肉毒杆菌毒素治疗眼睑及面肌痉挛

目的：观察A型肉毒杆菌毒素治疗眼睑痉挛及面肌痉挛的效果。方法：对38例眼睑痉挛及18例面肌痉挛患者局部注射A型肉毒杆菌毒素。结果：治疗效果满意，痉挛症状缓解。药效作用时间：眼睑痉挛者为5－17周，平均9．5周；面肌痉挛者为10－24周，平均16周。无全身反应，局部副作用轻微，可重复注射。结论：用A型肉毒杆菌毒素治疗眼睑及面肌痉挛安全、有效，为首选药物。     

眼睑痉挛型Meige综合征的治疗进展

Meige综合征是一种节段性的颅颈部肌张力障碍性的锥体外系疾病,属于成人多动症的一种。发病原因不明,常见于中老年女性,多与抑郁、外伤、药物、手术等危险因素相关,眼睑痉挛是Meige综合征最早也是最常见的临床症状,尽管有自发缓解的可能,但亦有致盲风险,临床上多因对此病认识不足而延误治疗影响预后,随着其发病率的增长,尤其以眼睑痉挛就诊于眼科的人数增长,全面认识Meige综合征有利于提高眼科医师诊治本病的能力以及指导临床合理用药。本文就眼睑痉挛型Meige综合征的治疗进展予以综述,总结了药物、手术和中医疗法的利弊,以期提高眼科医师对此类疾病的诊治水平。     

Review and update of involuntary facial movement disorders presenting in the ophthalmological setting

We review the existing literature on the involuntary facial movement disorders—benign essential blepharospasm, apraxia of eyelid opening, hemifacial spasm, and aberrant facial nerve regeneration. The etiology of idiopathic blepharospasm, a disorder of the central nervous system, and hemifacial spasm, a condition involving the facial nerve of the peripheral nervous system, is markedly different. We discuss established methods of managing patients and highlight new approaches.     

Outcomes of a regional variant of botulinum toxin type A in the treatment of essential blepharospasm and hemifacial spasms: A retrospective study

Purpose:The aim of this study was to report the outcomes of a regional variant of botulinum toxin type A (BtA) in essential blepharospasm and hemifacial spasm.Methods:The medical records of all patients with facial dystonias, who received at least one dose of BtA between May 2016 and April 2017 were retrospectively evaluated. The pre- and post-injection severity of symptoms, graded using the Jankovic rating system for essential blepharospasm and the Samsung Medical Center grading system for hemifacial spasm, the complications after each sitting, and the mean symptom-free interval were recorded. A correlation analysis was done to identify factors associated with longer symptom-free intervals. A P value < 0.05 was considered statistically significant.Results:The mean age at presentation was 56.62 ± 10.56 years. The mean duration of follow-up was 1.86 ± 2.06 years. The modal disease severity reduced from 5 to 0 in essential blepharospasm and from 2 to 0 in hemifacial spasm a week after injection of botulinum toxin. The mean symptom-free intervals with doses of 20, 22.5, 25, 30, and 50 units were 102.1 ± 44.7, 132.4 ± 35.3, 147.2 ± 61.6, 124.4 ± 55.1, and 142.4 ± 59.7 days, respectively. The commonest complication was lagophthalmos (26.3%; n = 20). Injections for primary dystonias were associated with longer disease-free intervals than those for secondary dystonias (P = 0.02). In nine sittings, the dose was increased for increased severity or presumed resistance, which resulted in a significant increase in the symptom-free interval (P = 0.004) without an increased incidence of complications (P = 0.48).Conclusion:BtA is safe and effective in the treatment of facial dystonias. The drug is more efficacious for primary facial dystonias.     

Use of high‐dose botulinum A toxin in benign essential blepharospasm: is too high too much?

BACKGROUND: Botulinum toxin (Botox) is the mainstay treatment for benign essential blepharospasm. Current treatment practice appears restricted by several reports demonstrating adverse effects and resistance to high-frequency, higher-dose therapy. This study aimed to explore whether high-dose, high-frequency treatments could be used without developing secondary resistance and without significant side-effects in patients refractory to conventional Botox doses. METHODS: From a cohort of 120 patients being treated with Botox therapy for benign essential blepharospasm and idiopathic hemifacial spasm, case notes from six patients were retrospectively examined. In these patients, therapy had exceeded the recommended 50 units per side for a duration greater than 12 months and at less than 3 monthly intervals. Patterns in subjective severity grading and percentage of improvement as well as reported side-effects were analysed. RESLUTS: All patients described greater than 60% improvement and 0-2 severity grading over a 3- to 15-year period with no evidence of secondary resistance. Side-effects were minor, transient and less frequently reported at higher doses. CONCLUSION: In a select group of patients, Botox therapy can be used effectively at doses higher than recommended over long periods with minimal side-effects and little evidence of secondary resistance.     

Efficacy of Carbamazepine Combined with Botulinum Toxin A in the Treatment of Blepharospasm and Hemifacial Spasm

 Purpose: To observe the efficacy of the combined treatment of carbamazepine and botulinum toxin A for blepharospasm and hemifacial spasm. Methods: Fifty-eight patients with either blepharospasm or hemifacial spasm were randomly divided into treatment and control groups. In the treatment group, 30 patients were administered with local intramuscular injections of botulinum toxin A and oral carbamazepine 100 mg/time,3 times/day for 60 days. Twenty-eight subjects in the control group underwent local intramuscular injections of botulinum toxin A only. Results: After combined treatment, the complete remission rate was 90%, which was significantly higher than that of the of the control group (67.9%, P<0.05,X2=4.733). However, no statistical significance was noted regarding the duration of therapeutic effects between the treatment group (range 14~40 weeks; 19.2 weeks on average) and control group (range 12~36 weeks; 18 weeks on average). Conclusion: The combined therapy of carbamazepine and topical injections of botulinum toxin A had increased efficacy in the treatment of blepharospasm or hemifacial spasm, but had no significant effect in terms of the duration of the therapeutic effect.     

BOTULINUM TOXIN IN OPHTHALMOLOGY

Alan B. Scott selected, researched and developed Type A Botulinum toxin for clinical use in ophthalmology. This unique drug has proved invaluable for treatment of a number of conditions which are difficult to treat in ophthalmology and in a variety of other disciplines. The indications, methods and problems of its use are described and the results of treatment of 133 patients are discussed. Up to December 1986 over 13 000 patients have been treated in a multicentre international trial without significant complications.     

Hemifacial Spasm Due to Intracranial Tumor: An International Survey of Botulinum Toxin Investigators

Hemifacial spasm (HFS) due to intracranial mass lesions is rare. Most cases are thought to be due to compression of the facial nerve by small vessels near the root of the facial nerve. A survey was undertaken of all botulinum toxin investigators to determine the incidence of imaged mass lesions causing HFS. Responders contributed information on 1676 patients with HFS. Of this group, nine tumors were reported for an incidence of 0.54% of patients. However, of this group only 52.5% underwent computed tomography (CT) or magnetic resonance (MR) scanning so the incidence of tumor causing HFS could be as high as 1.0%. No one tumor type was predominant, and most patients were women older than 50 years of age. The incidence compares with another large series of HFS patients in which one tumor was found in 367 patients. The authors also report as an illustrative case a 26-year-old man with HFS due to a presumed lipoma of the cerebellopontine angle. This diagnosis can be made with increased certainty with MR scanning. If the incidence of unsuspected diagnostically significant mass lesions is 1 in 200 patients with HFS referred for botulinum toxin injection, the cost of detecting one such lesion would be $100,000 at an average imaging cost of $500 per MR imaging or CT examination. Although mass lesions are uncommon, any patient with HFS whose general clinical course could justify intervention should be considered for imaging studies to rule out treatable conditions other than vascular compression.     

Difference in response to botulinum toxin type A treatment between patients with benign essential blepharospasm and hemifacial spasm

Background: Botulinum toxin (BTX) is the first‐line treatment in managing benign essential blepharospasm (BEB) and hemifacial spasm (HFS). We wished to assess the difference in duration of effect and the number of BTX treatments required to treat patients with BEB and HFS. Methods: A prospective study of patients attending the BTX clinic in Manchester Royal Eye Hospital over 6 months. All treatments were administered by a single experienced ophthalmologist. A questionnaire was completed for each patient. In patients with BEB where the BTX was injected bilaterally, one side was randomized to compare with HFS patients. Patient demographics, cumulative dose of BTX, duration of BTX effect with patient satisfaction and the number of previous BTX injections were recorded. Results: Sixty‐four patients were included in the study. The mean age was 60.8 years. Among them, 30 patients had BEB and 34 had HFS. Patients with HFS received a lower mean dose of BTX than patients with BEB (12.23 units vs. 16.2 units). The patients with HFS had a longer duration of effect than patients with BEB, with fewer BTX treatments. Of all patients, 90% with HFS and BEB were satisfied with the effect of their last BTX injection. Three unsatisfied patients in the BEB group were referred on for surgical management of their disorder. Conclusions: We have shown that patients with BEB have a shorter duration of effect with BTX and require more frequent BTX treatments than patients with HFS, highlighting that facial dystonias in patients with BEB is more challenging to manage.     

Efficacy of lamellar division for correcting cicatricial lid entropion and its associated features unrectified by the tarsal fracture technique

OBJECTIVE : To determine the efficacy of lamellar division for correcting cicatricial lid entropion and its associated features unrectified by the tarsal fracture technique. METHODS : Fifty patients (92 lids) diagnosed as having cicatricial lid entropion were operated by the tarsal fracture technique. There was defective lid closure in 34/92 lids, irregular lid margin in 48/92 lids, distichiatic or metaplastic cilia in 28/92 lids and a history of previous entropion surgery in 34/92 lids. All patients were followed up for one year and the surgical failures at the end of this period were subjected to lamellar division. These subjects were further followed up for one more year./ RESULTS : An overall success rate of 28.26% (26/92 lids) was obtained with the tarsal fracture technique. A correction was achieved in all the 20 lids having cicatricial entropion without any associated features. However, this technique succeeded in only six of the remaining 72 lids (8.33%) with a history of previous surgery or the associated features stated above. Furthermore, it was not effective in correcting associated anomalies such as defective lid closure, irregular lid margin and distichiatic or metaplastic cilia. Reoperation using lamellar division gave good correction in 97% of the lids (64/66). CONCLUSIONS : Lamellar division is a better procedure for treating cicatricial lid entropion, especially in patients with associated complications or with a previous history of entropion surgery.     

肉毒杆菌毒素A治疗眼睑及面肌痉挛远期疗效观察

目的 探讨肉毒杆菌毒素Ａ（ｂｏｔｕｌｉｎｕｍ Ａ ｔｏｘｉｎ,ＢＴＸＡ）治疗眼睑及面部肌肉痉挛的远期疗效。方法 对９２例眼睑及面部肌肉痉挛患者用ＢＴＸＡ注射治疗,随诊观察其远期疗效。结果 ＢＴＸＡ注射后１～４ｄ痉挛逐渐缓解,维持１６ｗｋ左右后症状复发。复发多由眼睑痉挛开始,面部痉挛缓解时间长,可达２４ｗｋ。注射后可见表情麻木、上睑下垂、睑裂闭合不全等并发症,但均较轻,且２ｗｉ后多能自行恢复。结论     

Botulinum toxin in ophthalmology

Since its introduction into clinical medicine in 1980, botulinum toxin has become a major therapeutic drug with applications valuable to many medical sub-specialties. Its use was spearheaded in ophthalmology where its potential applications have expanded to cover a broad range of visually related disorders. These include dystonic movement disorders, strabismus, nystagmus, headache syndromes such as migraine, lacrimal hypersecretion syndromes, eyelid retraction, spastic entropion, compressive optic neuropathy, and, more recently, periorbital aesthetic uses. Botulinum toxin is a potent neurotoxin that blocks the release of acetylcholine at the neuromuscular junction of cholinergic nerves. When used appropriately it will weaken the force of muscular contraction, or inhibit glandular secretion. Recovery occurs over 3 to 4 months from nerve terminal sprouting and regeneration of inactivated proteins necessary for degranualtion of acetylcholine vesicles. Complications are related to chemodenervation of adjacent muscle groups, injection technique, and immunological mechanisms.     

眶周肌肉切除术与A型肉毒毒素治疗特发性眼睑痉挛的选择与评估

目的：探讨眶周肌肉切除术与A型肉毒毒素治疗眼睑痉挛的选择依据。
　　方法：回顾性分析2012-03/2014-05来我科诊治的特发性眼睑痉挛患者100例,根据眼睑痉挛的程度将其分成0~Ⅳ级,按分级分为两组：A 组为眼睑痉挛I~Ⅲ级患者60例,行A型肉毒毒素治疗;B组为眼睑痉挛>Ⅲ~Ⅳ级患者40例,采用眶周肌肉切除术治疗。术后随访3~24 mo。结果：A组60例患者行A型肉毒毒素注射后,57例患者眼睑痉挛分级为0级,完全矫正,2例注射后残留部分痉挛,但分级均明显下降,1例无效。 B组40例患者中有25例患者行肉毒毒素治疗已耐受,属复发性并伴有眉下垂和上睑下垂症状,均改行眶周肌肉切除术,患者行眶周肌肉切除术后,30例患者痉挛分级为0级,7例为I级,3例为Ⅱ级。
　　结论：根据眼睑痉挛的临床分级,正确选择特发性眼睑痉挛的治疗方法可提高治疗的有效性和安全性。     

A型肉毒毒素治疗半侧面肌痉挛

目的:探讨A型肉毒毒素治疗半侧面肌痉挛疗效。方法:局部注射A型肉毒毒素,注射前后观察,复发后重新注射。结果:121例痉挛患者,IV级痉挛18例,III级痉挛55例,II级痉挛47例,I级痉挛1例。完全缓解111例,部分缓解10例。结论:A型肉毒毒素是治疗半侧面肌痉挛有效、完全、方便的治疗药物。     

150例眼睑痉挛及面肌痉挛的非手术治疗

目的分析非手术方法治疗眼睑痉挛和面肌痉挛的效果。方法统计150例眼睑及面肌痉挛患者。将痉挛时间低于6个月的50例患者分为两组,一组采取局部冰敷的方法治疗（25例）,另一组不做任何处理（25例）;对100例痉挛时间大于6个月的患者应用A型肉毒素局部注射治疗。结果25例患者冰敷1—2周后,其中23例症状完全消失,2例痉挛减轻：25例不做处理的．15例于1～2个月后症状自行消失．7例痉挛减轻,3例发展为持续性眼睑痉挛;痉挛时间大于6个月的100例患者A型肉毒素局部注射后,98例患者症状完全缓解或明显缓解．2例患者治疗无效。所有注射A型肉毒素的患者均未出现全身毒副作用。结论局部冰敷对于早期眼睑痉挛可能起到抑制或减轻症状的作用,A型肉毒素局部注射对于持续时间较久的眼睑或面肌痉挛为一安全、有效、可靠的治疗手段并易于推广与普及。