3D-TOF磁共振血管成像诊断偏侧面肌痉挛的病因

目的　研究 3D TOF磁共振血管成像对偏侧面肌痉挛 (HFS)病因诊断的临床价值。方法　 3D TOF磁共振血管成像脑干薄层扫描 3 4例HFS患者和 3 5例对照 ,盲法诊断面神经根部解剖改变 ,两组对照分析。结果　 ( 1)HFS患者症状侧面神经根部受压迫 3 2侧 ( 94 % ) ,其中血管压迫 3 1侧( 91% ) ,肿瘤压迫 1侧 ( 3 % ) ;无症状侧受血管压迫 6侧 ( 18% ) ;对照组双侧受压迫 3侧 ( 4 % ) ,其中血管 2侧 ,肿瘤 1侧。 ( 2 )常见压迫血管分别为小脑前下动脉 11侧 ( 3 8% ) ,椎动脉单独或与小脑后下动脉联合 9侧 ( 2 9% ) ,小脑后下动脉 8侧 ( 2 5 % )。 ( 3 )面神经根部血管压迫发生HFS相对危险度的估计值为 3 7.2 8。 ( 4 )面肌痉挛侧面神经根部受血管压迫或包绕有 14侧 ( 4 5 % )。结论　研究提示 ,3D TOF磁共振血管成像为目前面肌痉挛病因诊断的最佳影像检查方法 ,HFS的主要病因为患侧面神经根部受血管压迫、包绕或与其紧密接触。     

面神经临床分段与面肌痉挛术中责任血管分布初探

目的介绍一种面神经临床分段方法并初步总结术中发现的责任血管的分布及特点,以帮助找到主要责任血管。方法回顾性分析连续123例面肌痉挛病例。将面神经分为中枢髓鞘段、髓鞘移行段和外周髓鞘段。血管压迫程度分为轻、中、重三型。术中打开小脑桥脑裂上、下支,探查责任血管的分布情况与压迫程度。结果所有病人均发现有血管压迫。中枢髓鞘段压迫98例,髓鞘移行段压迫15例,外周髓鞘段压迫10例;主要责任血管为小脑前下动脉68例,小脑后下动脉23例,椎基底动脉合并小脑前下动脉和/或小脑后下动脉共26例,其他6例;轻度压迫28例,中度压迫82例,重度压迫13例。术后112例痉挛立即消失,术后1年仅1例痉挛未完全消失;无死亡。2例轻、中度复发。结论打开小脑桥脑裂并根据面神经分段探查责任血管,重点为中枢髓鞘段,将有助于发现主要责任血管,避免遗漏,提高治愈率。     

面肌痉挛MVD入路与中血管神经复合体的解剖学研究

目的研究面肌痉挛微血管减压（MVD）经枕下外侧小脑绒球下方入路与桥小脑角（CPA）区中血管神经复合体的显微解剖学关系。方法应用15例经10％甲醛充分固定并灌有乳胶的国人成人头颅湿标本，经枕下外侧小脑绒球下方入路，在4～25倍手术显微镜下对CPA区中血管神经复合体逐层解剖，观察，测量及照相。同时，临床应用该入路治疗面肌痉挛9例。结果经枕下外侧小脑绒球下方入路可直接从尾侧方小脑绒球与舌咽神经之间的间隙显露面、听神经分离处，能够准确、安全、快捷到达CPA区相应的中血管神经复合体位置，充分暴露术野，达到完全减压效果。结论经枕下外侧小脑绒球下方入路是治疗面肌痉挛安全有效的方法，可以最大限度的减少术中损伤神经致术后颅神经麻痹等并发症。     

内窥镜辅助下显微神经血管减压术治疗 特发性偏侧面肌痉挛

目的为进一步提高神经血管减压术治疗特发性偏侧面肌痉挛的疗效。方法经乙状窦后小骨窗开颅加用内窥镜辅助显微血管减压术治疗特发性偏侧面肌痉挛３５例。结果术后３４例症状消失,１例症状减轻,３５例随访６个月至３年半,术后面肌抽搐消失者３３例,１例症状减轻,１例１年后复发。结论内窥镜可弥补手术显微镜的不足之处,减少组织损伤和判断错误,提高治疗效果。     

A comparative study of primary and secondary hemifacial spasm

BACKGROUND: Hemifacial spasm (HFS) is a common movement disorder. OBJECTIVE: To evaluate possible differences in the demographic and clinical features between primary and secondary HFS. DESIGN: In-person interview using a standardized questionnaire to collect demographic and clinical data. SETTING: A multicenter study that included patients with HFS attending 3 Italian academic centers.Patients Two hundred fourteen patients with HFS. MAIN OUTCOME MEASURE: A complete neurological examination assessed the current muscle distribution of spasm and the presence of synkinetic movements between upper and lower facial muscles. RESULTS: The study sample comprised 214 patients with HFS, 81 men and 133 women, having a mean +/- SD age of 65.9 +/- 12.3 years; 164 patients were classified as having primary HFS and 50 patients (48 postparalytic and 2 symptomatic cases) were classified as having secondary HFS. Patients with primary and those with secondary HFS had similar mean +/- SD ages at onset (54.9 +/- 13.5 vs 57.0 +/- 12.8 years), male-female ratios (63:101 vs 18:32), right-sided-left-sided HFS (77:86 [1 bilateral] vs 21:28 [1 bilateral]), and frequencies of familial cases (2.9% vs 2.0%), respectively. Most patients (65.0%) with primary HFS had initial symptoms of periocular muscle contractions alone and had subsequent involvement of the lower facial muscles. Most patients (72.0%) with secondary HFS reported initial involvement of the upper and lower facial muscles simultaneously. Signs of synkinesis were present in primary (43.3%) and secondary (58.0%) HFS. CONCLUSIONS: Patients with primary and those with secondary HFS share common demographic and clinical features, including sex distribution, age at onset, affected side of HFS, synkinesis, and rarity of familial cases. Signs of synkinesis were present in significant proportions of patients with primary or secondary HFS. The 2 forms differed in clinical presentation.     

Placebo-controlled double-blind cross-over study of botulinum A toxin in hemifacial spasm

The evaluation of the efficacy of botulinum A toxin injection for hemifacial spasm has never previously been done in a double-blind study in spite of its use as a treatment. We thus conducted a double-blind cross-over study of botulinum A toxin use in hemifacial spasm in 55 patients at Siriraj Hospital, Mahidol University, Bangkok, Thailand. Thirteen patients decided to withdraw from the study due to a lack of efficacy, all of them were subsequently found to be in the saline injection group. The remaining 42 patients, in the botulinum A toxin injection (30 mouse units) group, reported the responses as: excellent (34 patients; 80.95%), moderate patients; 2.38%). In contrast, when given the saline injection they reported no excellent outcome, 1 patient (2.38%) with moderate improvement, 5 patients (11.90%) with mild improvement and, 36 patients (85.71%) with no response. Side effects of botulinum toxin injections were found in 14.29% of patients compared with 9.5% of the saline injection group. The side effects of botulinum toxin injection were mild transient facial weakness (7.14%), local pain (4.76%) and excessive lacrimation (2.38%).

We concluded that botulinum A toxin injection was a simple and effective out-patient treatment for the management of hemifacial spasm.     

Baclofen in hemifacial spasm

A 58-year-old woman with long-standing hemifacial spasm experienced dramatic relief of symptomatology while receiving baclofen (Lioresal) in a daily dosage of 37.5 mg. Clinical response was noted within 48 h and she remained asymptomatic during 12 months of treatment. No toxic or side-effects were observed. Controlled studies are needed to establish the role of baclofen in hemifacial spasm.     

Baclofen in hemifacial spasm

Six patients with idiopathic hemifacial spasm refractory to the usually employed medications were successfully treated with baclofen. Adverse side effects were found in only 1 patient (memory loss) which was controlled with administration of piracetam. We suggest that there is an association between stress and certain cases of hemifacial spasm and that such stress related cases may respond positively to baclofen.     

Electrophysiological study of ephaptic axono-axonal responses in hemifacial spasm

One of the classic features of hemifacial spasm (HFS) is spread of the blink reflex responses to muscles other than the orbicularis oculi. The pathophysiological mechanisms underlying the generation of such abnormal responses include lateral spread of activity between neighboring fibers of the facial nerve and hyperexcitability of facial motoneurons. In this report we present evidence for another mechanism that can contribute to the generation of responses in lower facial muscles resembling the R1 response of the blink reflex. In 13 HFS patients, we studied the responses induced in orbicularis oris by electrical stimuli applied at various sites between the supraorbital and zygomatic areas. We identified responses with two different components: an early and very stable component, with an onset latency ranging from 10.5 to 14.8 ms, and a more irregular longer-latency component. Displacement of the stimulation site away from the supraorbital nerve and towards the extracranial origin of the facial nerve caused a progressive shortening of response latency. These features indicate that, in our patients, the shortest latency component of the orbicularis oris response was likely generated by antidromic conduction in facial nerve motor axons followed by axono-axonal activation of the fibers innervating the lower facial muscles. Our results suggest that motor axono-axonal responses are generated by stimulation of facial nerve terminals in HFS.     

Case-control study of anxiety symptoms in hemifacial spasm.

In a case-control study, we evaluated symptoms in nine different psychological domains in hemifacial spasm (HFS; using the Symptom Checklist-90R [SCL-90R]) and found the anxiety score to be significantly greater in HFS compared to healthy controls in both the univariate (P = 0.004) and multivariate analysis (adjusted for sex, age, marital status, and educational level; P = 0.002). Similar findings were obtained when comparison was made with an independent group of outpatient controls. Compared to outpatient controls, the HFS patients had a higher mean Hamilton Anxiety Rating Score (HAM-A; 10.0 +/- 8.0 [range, 0 to 28] vs. 5.0 +/- 5.0 [range, 0 to 25]; P = 0.004), and 19.5% had HAM-A score of 18 or above compared to 3.8% in controls (P = 0.02). Among the HFS patients, the mean anxiety score in SCL-90R was significantly higher in those defined with mild to severe anxiety under HAM-A compared to those without anxiety (74.0 +/- 6.0 vs. 48.0 +/- 13.0) (P < 0.0005). There was good correlation of the anxiety score with the HAM-A in HFS patients (r = 0.915; P < 0.0001). HFS patients with anxiety reported significant improvement of their symptoms (mean HAM-A score 19.0 +/- 5.0 vs. 11.0 +/- 6.0; P = 0.001) following appropriate management. As stress and anxiety can aggravate HFS, diagnosis and early management of anxiety symptoms can improve quality of life in these patients.     

面肌痉挛发病机制的实验研究

目的 通过建立面肌痉挛的动物模型,结合电生理检测技术,探讨其发病机制。方法 ２８只新西兰大白兔随机分为４组：面神经脱髓鞘并血管压迫组（模型组）１３只;单一面神经血管压迫组５只;单一面神经脱髓鞘组５只;假手术对照组５只。术后第３、６周检测并比较各组动物的异常肌反应（ＡＭＲ）和Ｆ波变化。结果 术后第３周,４组动物均未诱发出ＡＭＲ,模型组有１０只动物于术后第６周诱发出典型的ＡＭＲ;模型组动物的Ｆ／Ｍ波幅比率、Ｆ波持续时间及其诱发频率均明显高于其他各组动物（Ｐ〈０．０１）,但各组动物的Ｆ波潜伏期差异无显著性意义（Ｐ〉０．０５）。结论 面神经运动核兴奋性的增高可能是发生面肌痉挛的主要病理生理基础。     

Differences in improvement between patients with idiopathic versus neurovascular hemifacial spasm after botulinum toxin treatment.

This study was performed to investigate the differences in response to botulinum toxin treatment between patients with idiopathic versus neurovascular hemifacial spasm. A total of 69 patients with hemifacial spasm were investigated prospectively with cranial magnetic resonance imaging and magnetic resonance angiography. Neurovascular contact was found in 23 patients. All patients were assessed with a severity scale and a disease awareness scale. After treatment, the patients with idiopathic hemifacial spasm improved significantly in terms of both severity and awareness scores, but the patients with neurovascular hemifacial spasm improved only in the awareness scores. In conclusion, patients with idiopathic hemifacial spasm experienced a greater improvement after treatment with botulinum toxin than did patients with neurovascular hemifacial spasm.     

Primary hemifacial spasm: a neurophysiological study.

A series of 53 cases of primary hemifacial spasm have been evaluated by means of blink reflexes and their results compared with a normal control group. Reflex responses were obtained by percutaneous electrical stimulus of both the supraorbital nerve (trigemino-facial reflex), and the facial nerve at the stylo-mastoid region (facio-facial reflex). The R2 response was considered abnormal when its latency was shortened (hyperactivity) or delayed (hypoactivity). Thirty-six out of 53 cases with primary hemifacial spasm showed abnormal responses, with a combination of facial nerve impairment (delayed R2 in the facio-facial reflex) and trigeminal-facial hyperactivity (shortened R2 in the trigemino-facial reflex). Five cases showed hyperactivity in both the trigemino-facial reflex and the facio-facial reflex reflexes. These results suggest a state of hyperexcitability, probably at the level of the facial nucleus, combined with a peripheral facial nerve involvement in a high proportion of patients with primary hemifacial spasm.