Asynchronous blepharospasm, facial and cervical dystonia, and bilateral asynchronous hemifacial spasm.

We present a patient with a facial movement disorder that has characteristics of both blepharospasm and bilateral asynchronous hemifacial spasm. Because of the increased incidence of blepharospasm in patients with hemifacial spasm, our patient's clinical presentation is probably not a chance occurrence, but rather a manifestation of some predisposition for these two movement disorders. This unusual constellation of signs and symptoms challenges the current diagnostic criteria and suggests that some of these facial movement disorders may lie on a spectrum, rather than represent distinct entities.     

Botulinum toxin in the treatment of blepharospasm and hemifacial spasm

Summary Blepharospasm and hemifacial spasm are the two most common craniofacial movement disorders. Blepharospasm is a syndrome characterized by excessive or continuous eye closure related to overactivity of the orbicularis oculi and adjacent muscles bilaterally. Hemifacial spasm is a peripherally-induced movement disorder typically caused by vascular compression of cranial nerve VII (CN VII) leading to involuntary unilateral contractions of muscles used in facial expression. Treatment options for both conditions include medications, botulinum toxin, and various surgical interventions. This article summarizes the existing medical literature which indicates that botulinum toxin is the treatment of choice for blepharospasm and hemifacial spasm. Correspondence: Christopher Kenney, Parkinson’s Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, 6550 Fannin, Suite 1801, Houston, Texas 77030, USA     

Hemifacial spasm: Clinical findings and treatment

Hemifacial spasm (HFS) is a peripherally induced movement disorder characterized by involuntary, unilateral, intermittent, irregular, tonic or clonic contractions of muscles innervated by the ipsilateral facial nerve. We reviewed the clinical features and response to different treatments in 158 patients (61% women) with HFS evaluated at our Movement Disorders Clinic. The mean age at onset was 48.5 ± 14.1 years (range: 15–87) and the mean duration of symptoms was 11.4 ± 8.5 (range: 0.5–53) years. The left side was affected in 56% instances; 5 patients had bilateral HFS. The lower lid was the most common site of the initial involvement followed by cheek and perioral region. Involuntary eye closure which interfered with vision and social embarrassment were the most common complaints. HFS was associated with trigeminal neuralgia in 5.1% of the cases and 5.7% had prior history of Bell's palsy. Although vascular abnormalities, facial nerve injury, and intracranial tumor were responsible for symptoms in some patients, most patients had no apparent etiology. Botulinum toxin type A(BTX-A)injections, used in 110 patients, provided marked to moderate improvement in 95% of patients. Seven of the 25 (28%) patients who had microvascular decompression reported permanent complications and the HFS recurred in 5 (20%). Although occasionally troublesome, HFS is generally a benign disorder that can be treated effectively with either BTX-A or microvascular decompression. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21: 1740–1747, 1998     

Health-related quality of life in blepharospasm or hemifacial spasm

OBJECTIVES: Health-related quality of life (HRQL) has become an important outcome criterion of medical interventions, but was hardly studied in patients with facial spasms. MATERIALS AND METHODS: Patients with blepharospasm or hemifacial spasm treated with botulinum toxin were included. A healthy control group sociodemografically matched to patients was established. Instruments applied included the SF-36 (global HRQL), the NEI-VFQ (disease-specific HRQL) and the Blepharospasm Rating/Disability Scale, the latter to patients only. RESULTS: Thirty-one patients with blepharospasm and 21 patients with hemifacial spasm took part. The Blepharospasm Rating/Disability Scale revealed minor functional limitations, but identified some symptoms to be improved. In both patient groups global (both SF-36 Component Summaries) and disease-specific (eight of 12 subscales) HRQL were significantly impaired compared with controls. CONCLUSION: HRQL should be integrated as core outcome criterion in treatment of patients with facial spasms. Assessment by a generic and a disease-specific instrument is recommended.     

AAEM case report #21: hemifacial spasm: preoperative diagnosis and intraoperative management

A 75-year-old man developed progressive involuntary hemifacial spasm. Electrophysiologic evidence of abnormal cross-transmission between neurons of the facial nerve was demonstrated. Electrodiagnostic studies were used to confirm the diagnosis preoperatively and determine the adequacy of vascular decompression of the facial nerve intraoperatively.     

Blink reflex recovery curves in blepharospasm,torticollis spasmodica,and hemifacial spasm

R1 and R2 blink reflex responses to single and paired stimuli were investigated in 23 control subjects: 21 patients with blepharospasm (BSP), 20 patients with torticollis spasmodica (TS), and 23 with hemifacial spasm (HFS). For paired stimuli, we compared measurements of area and peak responses at two and three times R2 threshold. R1 and R2 indices were calculated as the average of the recovery values at 0.5-, 0.3-, and 0.21-s interstimulus intervals to test individual patients. Peak amplitude measurements at three times R2 threshold were optimal. The R2 index was abnormal in 67% of BSP patients, 37% of TS patients, and 50% of HFS patients on the affected side and 20% on the unaffected side. A normal R2 index in one third of patients with BSP may indicate that different pathophysiological mechanisms are involved in this type of focal dystonia. © 1996 John Wiley & Sons, Inc.     

异常肌反应检测对面肌痉挛诊断及鉴别诊断的意义

目的评估异常肌反应(abnormal muscle response,AMR)检测在面肌痉挛(hemifacial spasm,HFS)诊断及鉴别诊断中的价值。方法选取因眼轮匝肌痉挛就诊于作者医院的50例患者,其中41例最终经临床诊断为HFS,7例为眼睑痉挛(blepharospasm,BSP),2例为特发性眼睑-口下颌肌张力障碍(Meige综合征)。回顾性分析以上三组患者面肌的AMR检测结果,检测方法为刺激面神经下颌缘后,记录同侧眼轮匝肌的AMR检测结果。分析这三种疾病的AMR阳性率,如三组间比较差异有统计学意义(P0.05),则进一步分析HFS患侧组AMR阳性率分别与其余两组比较差异是否有统计学意义;并根据HFS患者及非HFS患者AMR的检测结果,计算出AMR诊断HFS的灵敏度、特异度、阳性预测值、阴性预测值及约登指数。结果在41例HFS患者中患侧面肌AMR阳性率为95.12%(39例),眼睑痉挛组、Meige综合征组中AMR阳性率均为0%(0例),三组间AMR阳性率比较差异有统计学意义(P0.05);HFS患侧组分别与其余两组比较,AMR阳性率均高于其他两组,差异均有统计学意义(均P0.0167)。应用AMR检测来诊断HFS,灵敏度为95.12%,特异度为100%,阳性预测值为100%,阴性预测值为95.35%,约登指数为0.95%。结论 AMR检测对HFS的诊断及鉴别诊断可能具有重要参考价值。     

Botulinum toxin: State of the art

Botulinun neurotoxin (BoNT) has emerged as one of the most multipurpose therapeutic agents in modern medicine with more clinical applications than any other drug currently on the market. Initially developed in the treatment of strabismus and neurologic movement disorders, the use of botulinun neurotoxin has been expanding during the past 3 decades to include the treatment of a variety of ophthalmologic, gastrointestinal, urologic, orthopedic, dermatologic, dental, secretory, painful, cosmetic, and other conditions. In addition to onabotulinumtoxinA (Botox), abobotulinumtoxinA (Dysport), incobotulinumtoxinA (Xeomin), and RimabotulinumtoxinB (Myobloc or NeuroBloc) there are other novel botulinun neurotoxin products currently in development. With a better understanding of the cellular mechanisms of botulinun neurotoxin and advances in biotechnology, future botulinun neurotoxin products will likely be even more effective and customized to the specific indication and tailored to the needs of the patients. © 2017 International Parkinson and Movement Disorder Society     

Botulinum toxin treatment in patients with focal dystonia and hemifacial spasm. A multicenter study of the Italian Movement Disorder Group

In six Centers belonging to the Italian Movement Disorder Study Group, the efficacy of botulinum toxin treatment was evaluated in an open collaborative study in 251 patients with focal dystonia and hemifacial spasm. The percentage of functional improvement ranged from 66% to 81% in patients with blepharospasm, from 40% to 51% in patients with spasmodic torticollis and from 73% to 81% in those with hemifacial spasm. Good results were also obtained in patients with oromandibular dystonia, laryngeal dystonia and writer's cramp. Side effects were mild and transient. Local botulinum toxin injection is the first choice symptomatic treatment in focal dystonia and hemifacial spasm.

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Sommario In 6 centri facenti parte del Gruppo Italiano per lo Studio dei Disturbi del Movimento è stata valutata l'efficacia della somministrazione di tossina botulinica A in 251 pazienti affetti da distonia focale e da spasmo del facciale. Nei pazienti con blefarospasmo, la percentuale media di miglioramento osservata è compresa tra il 66 e l'81%, mentre nei pazienti con torcicollo varia tra il 40% e il 51%. Nei pazienti affetti da spasmo del facciale la percentuale media di miglioramento è compresa tra il 73% e l'81%. Buoni risultati sono stati ottenuti anche nella terapia di distonie focali meno frequenti, come la distonia oromandibolare e laringea e il crampo dello scrivano. Gli effetti collaterali osservati sono risultati generalmente lievi, locali e transitori. Lo studio conferma quindi l'utilità della tossina botulinica nella terapia sintomatica delle distonie focali e nello spasmo del facciale.

     

The management of blepharospasm and hemifacial spasm

Summary The aetiology of blepharospasm and hemifacial spasm is different, but both produce involuntary eye closure and facial movements which do not respond to systemic drug treatment. The introduction of therapeutic focal muscle weakening with botulinum toxin injections in the early 1980s appeared to offer great promise in the management of these conditions. In this paper the results of botulinum toxin treatment of 234 patients with blepharospasm and 73 patients with hemifacial spasm over a 7-year period have been analysed. Most patients receive sustained benefit from repeated injections whilst side-effects become less frequent. A clinically recognisable subgroup of patients with blepharospasm respond poorly and may be better treated surgically.     

面肌痉挛显微血管减压术的诱发肌电图监测和评估

目的:探讨面神经诱发肌电图在显微血管减压(MVD)术中、术后对面肌痉挛治疗效果的监测和评估。方法:对26例典型面肌痉挛病人、探讨在MVD术前、术中、术后,经皮刺激痉挛侧(手术侧)面神经下颌缘支,记录诱发同侧眼轮匝肌肌电位(MD-OC反应)变化,并以正常侧作对照检查。结果:26例术前均记录到痉挛侧MD-OC反应,在术中操作不同阶段,21例均见MD-OC反应消失。术后1周～3个月随访复查21例中,12例痉挛完全消失,MD-OC反应不复存在,9例症状明显或部分减轻,但有4例再记录到MD-OC反应。另5例术中,术后均记录到MD-OC反应,症状未有改善。结论:面神经诱发肌电图运用于MVD术中监测和预后判断,可改善和提高面肌痉挛的治愈率。     

微血管减压术治疗面肌痉挛术后迟发性面瘫临床分析

目的 探讨微血管减压术治疗面肌痉挛所发生的术后迟发性面瘫的病因、预防及处理措施.方法 回顾性分析潍坊医学院附属医院及清华大学玉泉医院微血管减压术治疗面肌痉挛716 例术后出现迟发性面瘫的情况.结果 术后出现迟发面瘫31 例,其中Ⅱ级8 例,Ⅲ级17 例,Ⅳ级5 例,Ⅴ级1 例.随访1 ～4 个月,平均2.5 个月,痊愈25 例,好转5 例,无明显变化1 例.结论 产生迟发性面瘫的原因很多,以血管因素和手术操作为主要原因,但预后较好.一旦出现迟发性面瘫,早期积极处理可望获得良好结果.     

继发性面肌痉挛(附5例分析)

目的探讨继发性面肌痉挛的病因及手术治疗。方法回顾性分析2000年10月．2007年7月采用手术治疗的977例面肌痉挛病例中的5例（0．5％）继发性者。其中3例为桥小脑角胆脂瘤，术中除将肿瘤切除减压外，还行显微血管减压术；1例为听神经瘤．行肿瘤切除；另外1例双侧面肌痉挛病人为右上矢状窦旁前中1/3交界处脑膜瘤，行常规开颅肿瘤切除。结果5例病人术后面肌痉挛均消失，分别随访1、15、37、40、79个月，治愈率100％。术后并发症包括：暂时性单纯耳鸣1例；轻度面瘫并听力下降1例：无菌性脑膜炎1例，出院时均治愈。结论继发性面肌痉挛多由桥小脑角生长较广泛的胆脂瘤引起：除行颅后窝显微手术切除肿瘤外，如发现动脉通过面神经出脑干区，还应行责任动脉显微血管减压术，方能彻底减压。     

面肌诱发肌电图监测下面肌痉挛微血管减压术

目的 探讨面肌诱发肌电图监测下，微血管减压治疗面肌痉挛的有效方法。方法21例术中以面肌诱发肌电图监测寻找责任血管，粗大成袢的责任血管以宽带涤纶条抬起固定于硬膜上，术中用罂粟碱生理盐水冲洗神经根部血管及术野。结果17例术后面肌抽搐立即停止，4例6～61d停止。结论微血管减压术中运用面肌诱发肌电图可明确判断责任血管，证实充分减压，评估预后；粗大成袢的责任血管抬起固定可有效地预防复发；术中罂粟碱应用，有利于减少术后并发症的发生。     

Treatment of idiopathic hemifacial spasm with botulinum toxin

Twelve patients with idiopathic hemifacial spasm received treatment with botulinum toxin A over a period of 18 months. Of 76 treatments given, most (94.7%) led to successful relief of eyelid spasms and all treatments were successful for perioral and lower facial muscle spasms. An average dose of 9.3 units of toxin per session was given to produce a mean interval of relief of 10.8 weeks. Blepharoptosis was the only ocular side effect; it was mild, reversible and occurred in 2 patients. However, lower facial palsy was frequent (9 patients); it was mild to moderate in severity but only partially reversible in 8 patients. Dosage for lower facial muscles should therefore be reduced.     

Botulinum toxin therapy of hemifacial spasm: comparing different therapeutic preparations

Hemifacial spasm (HFS) is characterized by involuntary irregular clonic or tonic movements of the muscles innervated by cranial nerve VII on one side of the face, and is most often a result of vascular compression of the facial nerve at the root exit zone (Muscle and Nerve 1998; 21 :1740). Disability associated with this disorder ranges from social embarrassment to interference with vision resulting from involuntary eye closure. Treatment of HFS most often involves botulinum toxin injections, but may also include medications and surgery. We describe treatment with the three types of botulinum toxin currently commercially available – Botox®, Dysport® and Myobloc®/NeuroBloc®.