原发性三叉神经痛的第三次伽玛刀治疗的初步经验报告

目的通过对三次伽玛刀治疗原发性三叉神经痛病例的长期随访,评价治疗的安全性,有效性及并发症发生情况。方法自1996年11月至2014年10月之间,5例难治性三叉神经痛患者均进行了3次伽玛刀治疗。初次治疗时:中位最大剂量为75Gy(70～80Gy),第二次治疗为70Gy(50～80Gy),第三次治疗为65Gy(50～70Gy)。中位靶区最高累积剂量为215Gy(175～230Gy)。结果第三次伽玛刀后中位随访66月(44～72个月)。疼痛完全缓解4例,部分缓解1例。前两次治疗平均疼痛复发时间26个月(12～48个月)。第三次伽玛刀后平均起效时间为1.5个月(1～4个月)。并发症情况:5例患者均出现面部麻木症状,其中2例为伽玛刀治疗前即出现,2例为第二次伽玛刀后出现,1例为第三次伽玛刀后出现。咬肌无力及颞肌萎缩2例,1例在第二次之治疗后出现,1例第三次伽玛刀后出现。结论三次伽玛刀治疗原发性三叉神经痛安全有效,并发症的发生率与二次治疗相似。     

旋转式伽玛刀治疗原发性三叉神经痛的初步研究

目的：探讨原发性三叉神经痛的旋转式伽玛刀治疗方法并分析其结果。方法：选择难治性原发性三叉神经痛患者45例，应用1．5TMR定位，以OUR－XGD治疗规划系统(r-TPS）作治疗规划，OUR－XGD旋转式伽玛刀进行治疗，治疗靶点三叉神经根入脑桥段，4mm准直器，1－2个等中心点，中心剂量75Gy,90Gy,100Gy三组，50％等剂量曲线覆盖靶点，脑干边缘剂量11．2-15.0Gy。结果：本组37例获随访，为期4－49个月。疼痛完全消失22例，明显缓解10例，轻度缓解3例，无效2例，复发4例，显效率86．5％，有效率94．6％，复发率10．8％，结论：复发症状较治疗前为轻，并发面部轻度麻木2例，面部麻木＋眼干涩感1例，张口稍困难1例，无死亡。结论：作为三叉神经痛的疗法，旋转式伽玛刀可以治疗各种类型的原发性三叉神经痛，且具有安全，高效和无创的优点，可作为长期药物治疗无效者的首选外科手段，75－80Gy是安全有效的治疗剂量，增大剂量至90Gy或更大可缩短平均起效时间，剂量达90Gy及以上时有效率可能升高但发生并发症的可能性也较大。     

伽玛刀治疗原发性三叉神经痛50例疗效分析

目的评价伽玛刀治疗原发性三叉神经痛的安全性,有效性和预后因素。方法原发性三叉神经痛50例接受了伽玛刀治疗,治疗部位位于三叉神经根近脑干侧,采用4mm准直器,双靶点治疗,周边剂量：35-40Gy;中心剂量：70-80Gy。结果随访：10-40个月（平均18个月）;术后疼痛缓解时间：1天～20个月（平均3．5个月）;总有效率为总有效率为90％（45／50）;面麻3例;并发症发生率为6％。结论伽玛刀治疗三叉神经痛安全有效,并发症少,在达到有效治疗剂量（70-90Gy）的同时,适当扩大照射体积可能提高疗效。     

伽玛刀治疗三叉神经痛照射剂量研究

目的 ;研究伽玛刀治疗原发性三叉神经痛的最佳照射剂量。方法选择2004年1月~2010年3月期间经保守治疗无效的原发性三叉神经痛患者进行伽玛刀治疗。根据不同的照射剂量随机分为高剂量组(80~85Gy)和低剂量组(70~75 Gy),治疗后定期随访,进行疗效分析。结果本组总计65例患者,随访60例,失访5例,随访率92.3%,随访期12~78个月,平均随访期36个月。对两组共60例患者进行了统计学分析,其中高剂量组25例,低剂量组35例。高剂量组1例无效,有效率96%,低剂量组2例无效,有效率94.3%,两组有效率无差异(P>0.05)。高剂量组出现面部麻木17例,并发症发生率68%,低剂量组出现面部麻木7例,并发症发生率20%,两组并发症发生率有显著差异(P<0.05)。高剂量组治疗后疼痛完全缓解时间为2.51个月,低剂量组治疗后疼痛完全缓解时间为2.67个月,两组疼痛缓解时间无差异(P>0.05)。结论伽玛刀治疗原发性三叉神经痛安全有效,70~75 Gy应作为最佳照射剂量。     

神经电生理监测面神经三叉神经微血管减压术的临床效果

目的：探讨术中使用神经电生理监测实施面神经、三叉神经微血管减压术的临床效果。方法面肌抽搐10例，三叉神经痛13例患者经药物或其他注射治疗方法疗效不佳而选择行神经电生理监测下行微血管减压术。结果术后随访3个月，面肌抽搐10例中9例术后抽搐消失，1例症状缓解；三叉神经痛13例中11例术后止痛，2例疼痛明显减轻；术后均无并发症。结论神经电生理监测下微血管减压术可避免神经功能损伤，减轻术后并发症，是目前最常用、最安全能够达到终生治愈的方式。     

异常肌反应监测在面肌痉挛显微血管减压术中的应用

目的探讨术中异常肌反应在原发性面肌痉挛微血管减压术中的价值及评估患者预后疗效的作用。方法回顾性分析我科2015年1月至4月收治的41例患者进行术中异常肌电反应监测(AMR),根据术中AMR波有无消失对术中减压效果进行评判,并随访1个月,将术中AMR波消失组与未消失组治愈率进行对比。结果41例患者中手术结束时AMR波未消失11例,术后1个月随访,5例停止抽搐,6例面部仍间断抽搐,其中2例未见明显减轻,治愈率为45.5%;AMR波消失30例,术后1个月随访,26例停止抽搐,4例仍间断抽搐,但较术前明显减轻,治愈率为86.7%。AMR波消失组的治愈率明显高于未消失组,两组间比较有显著差异(P0.05)。结论面肌痉挛微血管减压术中行异常肌反应监测能够评估患者预后,提高近期治愈率,降低术后延迟治愈的发生率。     

射波刀治疗原发性三叉神经痛初步经验(附15例报道)

目的 总结射波刀治疗原发性三叉神经痛治疗效果和初步经验,评价射波刀的治疗作用.方法 作者总结了2007年6月～2008年12月射波刀治疗的原发性三叉神经痛患者15例,靶点选择在三叉神经根入桥脑段前3.0mm处,大小5.0 mm×3.0mm～4.0mm×3.75mm～5.0mm为照射区域;采用5mm准直器,650mm源轴距(SAD),边缘剂量60～65Gy,中心剂量66.66～84.21Gy.结果 本组15例病人随访时间为6～18个月,平均一年以上,依据Brisman的疗效判定标准,治愈和疗效显著者13例,症状缓解期1～7天,症状消失期1个月;无效者2例,未见并发症.结论 本组病例经随访6～18月证实射波刀治疗三叉神经痛具有可靠疗效,与伽玛刀相比,具有无需麻醉、无创、无框架、精度高以及非等中心、非共面、非聚焦照射等优点.靶点的精确定位是本项成功治疗的关键之一.     

伽玛刀治疗颅内软骨肉瘤四例报道及文献回顾

目的 探讨伽玛刀放射外科治疗颅内软骨肉瘤的临床疗效. 方法 回顾性分析天津医科大学第二医院神经外科伽玛刀中心自2004年11月至2012年1月收治的4例软骨肉瘤患者诊治过程.4例患者均行手术切除并经病理证实,其中男1例,女3例;年龄26～36岁,平均31.3岁.肿瘤体积0.6～25.4 cm3,平均13.8 cm3;边缘剂量15～20 Gy,平均17.3 Gy;中心剂量30～40 Gy,平均35 Gy;靶点数4～19个,平均10个.本组随访时间5～28个月,平均14.5个月. 结果 1例左侧乳突及颈静脉孔区患者及1例鞍区患者治疗后分别随访19个月、9个月,肿瘤未见增大,生存良好;1例右侧眶颅沟通患者随访28个月后复发,后两次行手术治疗,效果不佳,于伽玛刀治疗后56个月死亡;1例左侧鞍旁患者治疗后5个月肿瘤复发,伴脑积水,随后失访.4例均未出现任何与伽玛刀治疗相关的并发症. 结论 伽玛刀治疗定位精确度高,疗效可靠且对周围组织损伤小,可作为术后残留软骨肉瘤辅助治疗方法.     

儿童原发性颅骨巨大尤文氏肉瘤（附1例报告并文献复习）

目的探讨原发性颅骨尤文氏肉瘤的诊断、治疗和预后。方法报告1例经病理检查证实的颅骨尤文氏肉瘤．并介绍其病理、临床及影像学特点。结果患者入院后行显微手术，全切肿瘤并保持上矢状窦通畅；术后进行4周放疗，总剂量为40Gy；术后随访8个月未见肿瘤复发。结论原发性颅骨尤交氏肉瘤是一种多发生于儿童和青年的罕见颅骨肿瘤：主要临床表现为头部包块、颅内压增高及肿瘤压迫所致神经系统定位症状；病理学上以小圆细胞为主要结构；影像学检查缺乏特异性：确诊主要依据病理学检查；治疗采用手术切除加放疗和化疗可明显改善患者的预后。     

伽玛刀治疗126例原发性三叉神经痛的疗效及相关因素分析

目的研究伽玛刀治疗原发性三叉神经痛的疗效及影响疗效的相关因素。方法选择2004年1月～2010年1月,126例经药物和其他保守治疗无效的原发性三叉神经痛患者接受伽玛刀治疗,并进行了长期随访,对疗效及影响疗效的相关因素进行评价。同时把116例随访资料完整的患者,根据靶中心剂量的不同分成高低两个组,高剂量组53例,中心剂量80Gy;低剂量组63例,中心剂量75Gy,通过统计学处理分析对比高低两个剂量组在有效率及并发症等方面的差异性。结果本组126例患者,116例获得完整随访资料,失访10例,随访率92%。随访期12～76个月,平均随访38个月。治疗总有效率96%,并发症发生率31%,复发率12%。高低两个剂量组有效率无差异(P>0.05);并发症发生率有显著差异(P<0.05)。结论伽玛刀治疗原发性三叉神经痛75Gy中心照射剂量有效率高,并发症低,应作为最佳参考照射剂量;伽玛刀治疗原发性三叉神经痛术前MRI定位中,患侧三叉神经根的显示情况会直接影响伽玛刀治疗效果,且复发率高,应作为伽玛刀治疗相对禁忌证;从治疗效果和安全角度来讲,伽玛刀可以作为原发性三叉神经痛的主要治疗方法。     

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.     

Role of hypofractionated stereotactic radiotherapy in treatment of skull base meningiomas

The objective of this paper is to examine the efficacy and safety of hypofractionated stereotactic radiotherapy (SRT) in the treatment of skull base meningiomas. Thirty-eight patients were treated with a median prescribed dose of 37.5 Gy in 15 fractions to the 80% isodose. Median follow-up was 47 months. Ten males and 28 females of median age 55.5 years were followed. SRT was the primary treatment in 15 patients, adjuvant in 10 and given for recurrence in 14 patients. On clinical follow-up 27 patients are unchanged and in six their symptoms have resolved. One patient had symptomatic deterioration and four patients have developed new symptoms. No patients have radiological evidence of progression. Our data suggest that conventional hypofractionated radiotherapy schemes for benign CNS disease may be useful in conjunction with stereotactic techniques. Such schemes are attractive in terms of resource allocation and where tumour size or cranial nerve tolerance is of concern.     

The many faces of hemifacial spasm: Differential diagnosis of unilateral facial spasms

Hemifacial spasm is defined as unilateral, involuntary, irregular clonic or tonic movement of muscles innervated by the seventh cranial nerve. Most frequently attributed to vascular loop compression at the root exit zone of the facial nerve, there are many other etiologies of unilateral facial movements that must be considered in the differential diagnosis of hemifacial spasm. The primary purpose of this review is to draw attention to the marked heterogeneity of unilateral facial spasms and to focus on clinical characteristics of mimickers of hemifacial spasm and on atypical presentations of nonvascular cases. In addition to a comprehensive review of the literature on hemifacial spasm, medical records and videos of consecutive patients referred to the Movement Disorders Clinic at Baylor College of Medicine for hemifacial spasm between 2000 and 2010 were reviewed, and videos of illustrative cases were edited. Among 215 patients referred for evaluation of hemifacial spasm, 133 (62%) were classified as primary or idiopathic hemifacial spasm (presumably caused by vascular compression of the ipsilateral facial nerve), and 4 (2%) had hereditary hemifacial spasm. Secondary causes were found in 40 patients (19%) and included Bell's palsy (n = 23, 11%), facial nerve injury (n = 13, 6%), demyelination (n = 2), and brain vascular insults (n = 2). There were an additional 38 patients (18%) with hemifacial spasm mimickers classified as psychogenic, tics, dystonia, myoclonus, and hemimasticatory spasm. We concluded that although most cases of hemifacial spasm are idiopathic and probably caused by vascular compression of the facial nerve, other etiologies should be considered in the differential diagnosis, particularly if there are atypical features. © 2011 Movement Disorder Society     

No clinical or neurophysiological evidence of botulinum toxin diffusion to non-injected muscles in patients with hemifacial spasm

Botulinum toxin injected into a muscle may diffuse to nearby muscles thus producing unwanted effects. In patients with hemifacial spasm, we evaluated clinically and neurophysiologically, whether botulinum toxin type A (BoNT-A) diffuses from the injection site (orbicularis oculi) to untreated muscles (orbicularis oris from the affected side and orbicularis oculi and oris from the unaffected side). We studied 38 patients with idiopathic hemifacial spasm. Botulinum toxin was injected into the affected orbicularis oculi muscle alone (at 3 standardized sites) at a clinically effective dose. Patients were studied before (T0) and 3-4 weeks after treatment (T1). We evaluated the clinical effects of botulinum toxin and muscle strength in the affected and unaffected muscles. We also assessed the peak-to-peak amplitude compound muscle action potential (CMAP) recorded from the orbicularis oculi and orbicularis oris muscles on both sides after supramaximal electrical stimulation of the facial nerve at the stylomastoid foramen. In all patients, botulinum toxin treatment reduced muscle spasms in the injected orbicularis oculi muscle and induced no muscle weakness in the other facial muscles. The CMAP amplitude significantly decreased in the injected orbicularis oculi muscle, but remained unchanged in the other facial muscles (orbicularis oris muscle on the affected side and contra-lateral unaffected muscles). In conclusion, in patients with hemifacial spasm, botulinum toxin, at a clinically effective dose, induces no clinical signs of diffusion and does not reduce the CMAP size in the nearby untreated orbicularis oris or contralateral facial muscles.     

Electrophysiological study on hemifacial spasm--usefulness in the etiological diagnosis and pathophysiological mechanism

Electrophysiological studies were performed in 30 patients with idiopathic hemifacial spasm (idiopathic HFS), who underwent microvascular decompression with abolishment of spasm, and 10 patients with symptomatic hemifacial spasm (symptomatic HFS) secondary to Bell's palsy. (1) The maximum firing rate of abnormal discharges recorded from the orbicularis oris muscle during spasm in patients with idiopathic and with symptomatic HFS, and that of discharges recorded on the intact side during voluntary contraction in idiopathic HFS patients measured 181 +/- 71 Hz, 68.4 +/- 36.9 Hz, 56.3 +/- 21.8 Hz, respectively. Thus, the maximum firing rate of the discharges during spasm in idiopathic HFS patients was exceedingly higher than that in symptomatic HFS patients. (2) Electroneurography, performed to evaluate quantitatively degeneration of the facial nerve, revealed that the ENoG value (90.2 +/- 16.5%) in idiopathic HFS patients were higher than that (57.6 +/- 26.8%) in symptomatic HFS patients. (3) In blink reflex examined, synkinetic potentials (S1, S2), synchronous to the potentials consisting of the early (R 1) and late component (R 2) in the orbicularis oculi muscle, were recorded from the orbicularis oris muscle on the affected side in all patients with idiopathic and with symptomatic HFS. In sequential recording of blink reflex potentials (R 1, R 2) and synkinetic potentials (S 1, S 2), the recording pattern of synkinetic potentials was divided into variable and constant type. In the variable type, synkinetic potentials appeared unsteadily and the difference in latency between R 1 and S 1 was varied. In the constant type, synkinetic potentials appeared steadily and the difference in latency between the two was not varied.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

MR 3D FLASH-WE技术对特发性面肌痉挛显微血管减压的术前诊断价值

目的探讨MR 3D FLASH-WE序列对特发性面肌痉挛显微血管减压术(MVD)术前的诊断价值。方法回顾性分析采用MVD治疗49例特发性面肌痉挛病人的临床资料,术前均行MR 3D FLASH-WE序列扫描,判断面神经根部血管受压情况。结果 MRI显示病侧面神经根部有血管接触征象42例,可疑接触4例,无接触3例。术前MRI检查与术中发现责任血管完全一致42例;MRI显示为单根责任血管,而术中证实责任血管为多根动脉4例;MRI未见责任血管,但术中发现2例;术前MRI和术中均未发现责任血管1例。该序列判断责任血管与术中所见的符合率达87.5%。结论 MR 3D FLASH-WE技术对面肌痉挛病人MVD术前判断责任血管有较高的价值。     

Successful Treatment of Infantile Fibrosarcoma Spinal Metastasis by Chemotherapy and Stereotactic Hypofractionated Radiotherapy

We report a case of infantile fibrosarcoma in an 8-month-old boy manifested as a right-sided lower leg mass. Repeated local recurrence and distant metastasis were noted during the following three-year period. Whole body fluoro-deoxyglucose positron emission tomography scan revealed an asymptomatic metastasis involving the fourth lumbar vertebrae. The patient received chemotherapy (VAC regimen) with Cyberknife® stereotactic hypofractionated radiotherapy (26 Gy; 4 fractions). This treatment reduced tumor size by 23% without acute radiation toxicity even after 33 months. This case suggests that combining chemotherapy and this form of radiotherapy may be safe and effective against childhood spinal metastasis.     

Familial hemifacial spasm: report of cases and review of literature.

We describe clinical characteristics of 10 patients (five families) with familial hemifacial spasm, with reviews of 13 patients hitherto reported in the literature. There is no clear difference in clinical manifestations between sporadic and familial hemifacial spasms. There is no definite inheritance pattern, but may be autosomal dominant with low penetrance. The ages of onset of familial hemifacial spasm are variable, but occasionally can occur at early years of life. There is a left-side predominance with respect to the affected side of cases with familial hemifacial spasm. Similar to sporadic hemifacial spasm, vascular decompression was effective, suggesting that vascular compression is involved in generating hemifacial spasm even in the familial cases. Familial hemifacial spasm may not be a rare disorder, but may possibly be overlooked. Clarifying the role of genetic susceptibility in pathophysiological mechanisms underlying hemifacial spasm is an important approach toward better understanding of the pathogenesis of cranial rhizopathies.     

Remission of hemifacial spasm after proximal occlusion of vertebrobasilar dissecting aneurysm with coils: case report.

Intracranial aneurysm is a rare cause of hemifacial spasm and most of the previously reported cases are treated with surgical microvascular decompression. Authors report a case of hemifacial spasm caused by a dissecting aneurysm located at the vertebrobasilar junction which improved after endovascular obliteration of the affected vertebral artery with coils.The patient was a 69-year-old man with 20 months' history of left hemifacial spasm. A vertebral angiogram showed an irregular dilatation of the right vertebral artery associated with aneurysmal dilatation at the vertebrobasilar junction. Endovascular obliteration of the abnormally dilated right vertebral artery proximal to the vertebrobasilar junction was performed. The hemifacial spasm gradually improved after the embolisation and disappeared 6 months later. Endovascular proximal obliteration of the vertebral artery may have changed the hemodynamic force inside the aneurysm and eliminated the vascular compression at the root exit zone of the facial nerve.