Hemifacial spasm, vertebrobasilar dolichoectasia and neurofibromatosis type 1

Hemifacial spasm (HFS) is usually produced by compression of the facial nerve by tortuous blood vessels at the root exit zone, including vertebrobasilar dolichoectasia (VBD). Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder with a variety of symptoms, affecting mainly the skin and nervous system. Cerebrovascular abnormalities are becoming a recognized complication of the disease and the most constantly described lesions are stenosis and occlusions affecting the internal carotid artery. VBD has rarely been associated with NF1. We report a 38-year-old female patient with HFS produced by VBD with NF1 presenting with other cerebrovascular abnormalities associated with this disease. We discuss the possible association between these three entities, assuming that a causal relationship may be established and that VBD is part of the spectrum of vascular abnormalities caused by NF1 in this patient.     

Hemifacial spasm resulting from vertebral artery dolichoectasia

A 32-year-old man presented with left hemifacial spasm. Neurophysiological findings revealed an absent ipsilateral R1 on blink reflex. An MRI showed a dolichoectatic left vertebral artery impinging on the root exit zone of the left facial nerve. Botulinum toxin infections relieved the manifestations of hemifacial spasm. This case demonstrates that MRI/MRA is an essential part of the work-up for hemifacial spasm, and shows that in accordance with the literature, vertebral dolichoectasia is an uncommon cause of hemifacial spasm.     

椎-基底动脉扩张延长症致面肌痉挛的手术治疗

目的 探讨椎-基底动脉扩张延长症（vertebrobasilar dolichoectasia,VBD）导致面肌痉挛的临床特点及手术方法。方法 回顾性分析7例VBD导致的面肌痉挛的临床资料,3例行常规微血管减压术,4例采用“双面胶悬吊技术”。结果 术后随访6个月~2年,7例均恢复良好;6例面肌抽搐症状完全消失,1例有部分残留;2例出现一过性面瘫,1例出现一过性听力下降,1例出现永久性听力下降;无复发、脑脊液漏、颅内感染、死亡。结论 VBD导致的面肌痉挛手术治疗难度大,并发症多,采用“双面胶悬吊技术”能达到较好的疗效。     

椎基底动脉扩张延长症的研究进展

椎基底动脉扩张延长症（Vertebrobasilar Dolichoectasia,VBD）是指椎基底动脉血管异常延长、扩张、迂曲或成角改变的血管畸形,发病较为罕见,总体发病率低于0．05％,目前正处于探索和研究阶段。该病于1986年由Smoker等正式定义,既往也曾命名为巨大延长扩张病、巨大基底动脉变异及梭形动脉瘤、动脉瘤样畸形、椎基系统迂曲等。由于其临床表现复杂多样,其致死及致残率较高,且起病隐匿,易造成漏诊、误诊。     

面肌痉挛

面肌痉挛（hemifacial spasm，HFS）是由于面神经过度兴奋引起的同侧面部肌肉非自主性痉挛收缩。通常情况下痉挛仅限于一侧．故义称为偏侧面肌痉挛。发病率为0．78／10万，女性发病率（14．50／10万）高于男性（7．40／10万）。亚洲人群患病率似乎高于欧美人群，但目前缺乏大宗病例的流行病学调查结果支持。面肌痉挛虽不危及生命，但可以对患造成心理负担，影响生活、工作和社交。笔仅就面肌痉挛的病因、电生理机制、临床特点、诊断和治疗进行综述。     

1例面肌痉挛合并椎基底动脉延长扩张症并文献复习

目的 报告1例面肌痉挛合并椎基底动脉延长扩张症（VBD）病例,提高对VBD的认识.方法 对1例合并VBD的面肌痉挛病人行手术治疗,并结合文献进行分析.结果 VBD是一种少见的血管异常性疾病,迂曲扩张的椎基底动脉压迫增加了面肌痉挛治疗的复杂性.结论 治疗面肌痉挛病人过程中应注意到其合并VBD的可能性,从而能够早期诊断和治疗.     

Hemifacial spasm in childhood

A nine-year-old girl presented with right hemifacial spasm (HFS) of 27 months duration. Stimulation of the right supraorbital nerve produced synkinesis of the ipsilateral facial muscles. Latency and amplitude of the blink reflex were equal bilaterally. Other investigations including computerized tomography (CT) were negative. Treatment with carbamazepine was not helpful. This is the third reported case of HFS in childhood.     

Hemifacial spasm in sleep

We conducted polygraphic studies during wakefulness and all-night sleep in 13 patients with cryptogenic and 3 with postparalytic hemifacial spasm. The movements decreased progressively with deepening sleep stages, reaching lowest values in REM sleep. The reduction was inversely related to the severity of movements during wakefulness. There was no relation between hemifacial spasm and mimic activity on the unaffected side. Central inhibitory processes may account for the partial decline in intensity of the movements in sleep.     

椎基底动脉延长扩张症165例临床特征分析

目的 探讨椎基底动脉延长扩张症(VBD)的临床特征.方法 回顾分析165例VBD患者的临床资料,并分析症状性与非症状性VBD的影响因素.结果 本组165例中男性105例,女性60例,平均年龄65±3岁.临床表现:后循环TIA61例,后循环梗死16例,脑干出血1例,前庭阵发症28例,三叉神经痛15例,面肌痉挛11例,无症状33例.CTA、MRA、DSA显示基底动脉不同程度的延长扩张、骑跨.VBD症状有无,与患者年龄、高血压病、糖尿病、吸烟、血管直径、延长扩张程度相关.结论 VBD临床表现复杂、多样;常见症状有卒中、压迫性症状和脑神经损害等,血管影像检查是重要的诊断依据.     

Hemifacial spasm in a child

Hemifacial spasm in a 10-year-old child is herein reported. Computed tomography and angiography were normal. Magnetic resonance imaging showed an anomalous vascular structure, probably a redundant loop arising from AICA or PICA, which was identified at surgery. The spasm, presumably due to vascular compression at the root entry zone of the right facial nerve, promptly and completely remitted after surgical decompression, without functional deficits. Atypical clinical features, as well as pre- and postoperative neurophysiological findings supporting the microvascular compression theory, are discussed.