Validation of a short disease specific quality of life scale for hemifacial spasm: correlation with SF-36

BACKGROUND: A short, practical, and validated quality of life (QoL) scale for hemifacial spasm (HFS) is not currently available. OBJECTIVES: To examine the reliability and validity of a short self-rating scale (HFS-7) by comparing HFS patients with healthy controls. We also evaluated the correlation of HFS-7 with the physical and mental domains of SF-36, a generic QoL scale. METHODS: Seven self-rating items (HFS-7) were administered to HFS patients and healthy controls. In addition, HFS patients answered the SF-36 questionnaire. The validity and reliability of HFS-7 were analysed and correlation between HFS-7 and SF-36 examined. RESULTS: A total of 178 subjects were enrolled in the study, including 85 HFS patients with mean age of 54.8 (SD 11.0) years, of whom 52 (61.2%) were women, and 93 controls with mean age of 51.4 (SD 10.0) years, of whom 59 (63.4%) were women. The test-retest intraclass correlation coefficient for the seven items was between 0.75 and 0.90 and Cronbach's coefficient of reliability for the HFS-7 scale was 0.88. Every item in HFS-7 discriminated between disease and controls (p<0.0001). The HFS-7 summary index correlated with the SF-36 summary score (Spearman's correlation r = -0.28, p = 0.009), in particular the mental health summary score (r = -0.416, p<0.0001) and the emotional domain (r = -0.466, p<0.00001). CONCLUSION: HFS-7 could prove useful as a simple clinical tool to assess and monitor QoL measures in HFS patients.     

Factors affecting the quality of life in hemifacial spasm patients

Background and purposeHemifacial spasm (HFS), a movement disorder manifested by unilateral spasms of the muscles innervated by the facial nerve, interferes with social life in about 90% of patients, causing social isolation and depression and having a significant impact on the quality of life. The aim of the study was to assess factors affecting the quality of life in patients with HFS in respect of influence of the severity of depression symptoms and botulinum toxin type A (BTX-A) therapy.Material and methodsEighty-five out of 129 patients included in the HFS database of the Movement Disorders Out-patient Clinic, Department of Neurology, University Hospital, Cracow who fulfilled the inclusion criteria and had no exclusion criteria (suffering from concomitant movement disorders, other severe chronic diseases or cognitive impairment) were studied. Demographic and clinical data (age at onset, disease duration and accompanying symptoms) were collected. Severity of HFS was assessed by the five-point clinical scale and seven-point Clinical Global Impression scale. Quality of life was assessed with the HFS-36 questionnaire and severity of depressive symptoms was evaluated with the Beck Depression Inventory. HFS-36 was performed twice, before BTX-A injection and two weeks later.ResultsThe mean global score of HFS-36 was 47 ± 31 (maximum: 140 pts). Decreased HFS-36 score resulted from divergent deterioration in all subscales included in the questionnaire. Independent risk factors of deterioration in HFS-36 were increased severity of HFS and depressive symptoms as well as accompanying trismus. The HFS-36 score depended on the number and type of accompanying symptoms as well. Botulinum toxin type A therapy led to a significant improvement of HFS-36, particularly high in patients with multiple (> 4) HFS-related symptoms.ConclusionsThe HFS-36 score depends mostly on severity of HFS, depressive symptoms and occurrence of accompanying trismus. It improves after BTX-A treatment.     

Repeat microvascular decompression for hemifacial spasm

OBJECTIVE: To report our experience with repeat microvascular decompression (MVD) for hemifacial spasm (HFS) in patients who have failed their first operation. METHODS: The authors describe 41 redo MVDs for HFS in 36 patients performed over a 3 year period. Seven patients underwent early re-operation after an aborted seventh nerve decompression. Eight patients underwent early re-operation for clinical failure. Eighteen patients underwent late re-operation for spasm recurrence long after their original MVD. Eight MVDs were performed on patients who had already undergone at least two prior operations. RESULTS: Twenty four patients experienced complete spasm resolution (70.6%), eight patients had near total resolution (23.5%), and two patients failed re-operation (5.9%). Two patients were lost to follow up (5.6%). A favourable outcome was reported by 82.4% of patients at a mean follow up interval of 18 months. A total of 91.7% of patients 50 years of age or younger were cured at follow up versus 59.1% of patients older than 50 (p = 0.04). Patients undergoing early re-operation were significantly more likely to be cured or improved than patients undergoing late re-operation (p = 0.03). CONCLUSIONS: Repeat MVD for HFS is effective in experienced hands. Younger patients respond better to repeat MVD. Late repeat MVD for HFS is a reasonable treatment option, although results are less favourable than for early re-operation.     

Treatment outcome correlates with knowledge of disease in hemifacial spasm

OBJECTIVES: Hemifacial spasm (HFS), a potentially disabling facial condition affects quality of life (QOL) and botulinum toxin is an effective treatment. No studies have examined whether a better level of knowledge of the disease would lead to an improved quality of life and treatment response in HFS. We examined the relationship between knowledge of disease with improvement in QOL following botulinum toxin treatment in HFS patients. PATIENTS AND METHODS: A total of 106 HFS patients (mean age of 56.8+/-9.9 years) were prospectively included. A baseline knowledge questionnaire and a validated disease-specific quality of life scale (HFS-7) were administered before and after botulinum toxin treatment. RESULTS: A better educational level was an independent predictor of high knowledge of HFS (p=0.02). Multivariate analysis using improvement in HFS-7 (total and subscore) as outcomes, and adjusting for age, gender, education, severity and duration of HFS, showed that high knowledge was predictive of a bigger improvement in HFS-7 total (p=0.03) and HFS-7 subscore (p=0.03). CONCLUSIONS: HFS patients with high knowledge of disease reported better improvement in QOL following botulinum toxin treatment. Better educational efforts will augment current medical and surgical treatments in improving QOL in HFS. Our findings could potentially be extended to many other medical conditions.     

神经电生理监测异常肌反应在面肌痉挛术中的作用

目的 评价异常肌反应（AMR）监测在面肌痉挛微血管减压术（MVD）中的应用价值。方法 2011年4月至2013年7月收治的88例面肌痉挛患者,均行MVD治疗,采用神经电生理监测AMR波指导手术。结果 术前所有患者均监测到AMR波,压迫面神经的血管减压后76例（86.4%）AMR波消失,12例（13.7%）AMR波未消失。术后随访结果6个月,AMR波消失的76例患者中,75例面肌痉挛消失,1例痉挛程度较术前明显缓解;AMR波未消失的12例患者,8例面肌痉挛消失,3例较术前明显缓解,1例无效。结论 面肌痉挛患者MVD术中监测AMR波有助于鉴别责任血管、评价手术减压效果、判断手术后患者的预后。     

显微血管减压术治疗面肌痉挛的症状转归

目的观察面肌痉挛显微血管减压术后的症状变化规律。方法 2006年11月~2007年7月104例面肌痉挛患者接受了异常肌反应电生理监测下的显微血管减压术,随访术后疗效,分析术后症状的演变规律。结果术后随访3.2~4年,14例失访。在资料完整的90例患者中,随访期内82例治愈,2例症状明显改善,4例无效,2例复发。在治愈的82例患者中:57例术后即刻治愈;25例出现延迟治愈,症状消失时间为术后3天~1.5年。有78例(95.1%)在术后半年内症状消失。结论显微血管减压术后,面肌痉挛症状整体呈进行性改善的趋势,症状消失时间主要集中在术后半年内。     

面肌痉挛微血管减压再手术病例的选择与疗效

目的探讨微血管减压(microvascular decompression,MVD)手术治疗面肌痉挛手术失败或复发再手术病例的选择和疗效。方法 2001年11月至2010年5月期间,在首次手术失败或复发的面肌痉挛患者中,根据其要求手术的愿望,结合术前影像学和首次手术录像、记录等资料综合判断后,选择了8例首次手术失败和20例术后复发患者进行MVD再手术治疗。结果经过平均34月的随访,仅1例患者术后症状无明显改善。其余27例(96.4%)患者术后症状缓解程度达75%以上,其中21例(75.0%)术后症状完全消失。24例(85.7%)患者术后自我疗效评价达到"优"和"良"。所有患者术后无严重病残和死亡发生,仅一例术后发生HBS 3级面瘫。结论选择合适的病人、恰当的手术时机、耐心仔细的显微操作,是面肌痉挛微血管减压再手术取得良好疗效的保证。     

面肌痉挛微血管减压术后持续性异常肌反应对预后的影响

目的 探讨异常肌反应（AMR）在面肌痉挛（HFS）微血管减压术（MVD）后持续存在的意义。方法 回顾性分析2012年2月至2017年2月MVD治疗的168例HFS的临床资料。术中进行电生理监测记录AMR波,其中162例监测到AMR波,术后130例AMR波形消失（AMR波消失组）,32例波形未消失（AMR波未消失组）。结果 AMR波消失组术后即刻治愈率（90.8%,118/130）明显高于AMR波未消失组（46.9%,15/32;P<0.05）。两组术后并发症发生率无统计学差异（3.8% vs. 3.8%;P>0.05）。AMR波消失组128例获得随访,随访时间13~71个月,平均41.2个月;AMR波未消失32均获得随访,随访时间15~70个月,平均43.6个月。AMR波消失组随访治愈率（94.5%）与AMR波未消失组（93.8%）无统计学差异（P>0.05）。结论 HFS病人MVD中,AMR监测对判断面神经减压是否充分具有一定的指导价值;术后AMR波形持续存在并不意味着预后不佳。     

微血管减压术治疗面肌痉挛复发原因分析

目的 探讨微血管减压术治疗面肌痉挛后症状复发的原因。方法 对19例经微血管减压术治疗后症状消失1年以上，痉挛又复出现的患者行二次手术。术中发现所有病例均因第一次手术时减压棉片放置位置不当和/或大小不适，致责任血管复位而重新压迫面神经根部。二次手术中将责任血管游离后推移离开面神经根部，用Teflon棉进行固定。结果 二次手术后18例症状消失，1例减轻。第二次术后所有病例经1．2～7年随访，平均3．8年，除1例仍有轻度痉挛外，余症状均消失。结论 微血管减压术是治疗面肌痉挛的有效方法，准确判断责任血管并实施有效的减压是提高手术效果、减少症状复发的关键。     

Endoscopic assistance in microvascular decompression of cranial nerves

Microvascular decompression (MVD) is an effective method for treatment of trigeminal neuralgia (TN), hemifacial spasm (HFS), glossopharyngeal neuralgia (GPN). The aim of this study was to assess the role of endoscopic assistance in MVD for the treatment of cranial neuropathies. Since 2009 till 2011 133 patients with cranial neuropathies were treated by MVD in Burdenko Neurosurgical Institute, Moscow. In 22 patients (11 patients with HFS, 10 patients with TN, 1 with GPN) endoscopic assistance was applied during the MVD. We used minimally invasive retrosigmoid approach in a unilateral position. Cerebellopontine angle was explored by 30-degree or 70-degree telescope to visualize the root entry zone of trigeminal, facial or glossopharyngeal nerves and to locate the neurovascular conflict. In 9 patients with HFS and in 1 patient with TN and in another patient with GPN endoscopy discovered offending vessels that were not visible through the microscope. In all cases endoscope was used to exclude another site of compression and to verify decompression and to identify position of teflon and offending vessel after MVD. Immediately after the surgery excellent outcome was observed in 10 patients with HFS (89%), one patient was reoperated 1.5 years after first operation with positive effect. Relief of pain in early postoperative period was observed in patients with TN and GPN. There were no major complications and postoperative mortality in our series. Endoscopic assistance is very effective and helpful technique in MVD of cranial nerves, especially in cases with HFS. In this study the use of the endoscope allowed to identify the site of compression and to confirm the position of teflon after MVD.     

面肌痉挛微血管减压术后并发症的 相关危险因素分析

目的 探讨面肌痉挛微血管减压术后并发症的相关危险因素。方法 对2012年至2014年治疗的148例面肌痉挛的临床资料进行回顾性分析。统计分析术后出现并发症的种类、发生率及转归,并就术前的一般情况、病程、侧别、面肌痉挛严重程度、骨窗前缘距颞骨岩部内侧面的距离、责任血管的类型及与神经根的关系、蛛网膜的增厚程度、硬膜下操作时间与术后出现并发症的相关性进行Logistic回归分析。结果 148例中,面肌痉挛术后1年缓解率为92.3%,术后均未出现严重并发症,术后2周内出现耳鸣28例（18.9%）、听力下降15例（10.1%）、面瘫12例（8.1%）、发热伴颈项强直10例（6.8%）、颅内感染2例（1.4%）。术后12个月随访,并发症发生情况为听力障碍3例、面瘫2例。骨窗前缘距颞骨岩部内侧面的距离>2 cm（OR=3.167,P=0.038）、责任血管发出穿支血管包绕神经出脑干区（OR=1.674,P=0.027）、分离血管时间>30 min（OR=1.563,P=0.042）与术后并发症的发生有显著相关性。结论 面瘫和听力下降为面肌痉挛术后早期容易出现的并发症,其发生原因主要与术中责任血管的分离困难,增加血管痉挛有关,术中听神经的操作损伤也与术后耳鸣及听力下降等并发症相关。     

Delayed effects of the microvascular decompression on hemifacial spasm: a retrospective study of 131 consecutive operated cases

We reviewed 131 consecutive cases operated for hemifacial spasm (HFS) by the same surgeon between January 1983 and April 1999. Microvascular decompression (MVD) was performed via lateral suboccipital approach. Post-operative follow-up ranged from 1.5 to 10 years (average 34 months). The final outcome divided into three categories, excellent (total recovery) in 120 cases (91.6%), partial (> 75% recovery) in 4 cases (3.1%), and unchanged or recurrent in 7 cases (5.3%). Only 2 cases were re-operated, and final outcome of both was excellent. Based on these data, we aimed to determine a period of the final judgement of MVD effect and the causative factors of delayed effects on HFS retrospectively. There were 102 complete recovered cases without hemifacial paralysis; immediate recovery from HFS was observed in 78 cases (76.5%), after 1 month in nine cases, 1-3 months in 5 cases, 3-6 months in 3 cases, 6-10 months in 2 cases, and 10-12 months in 5 cases. Thus, most cases were completely recovered within one year of observation. On the other hand, there was no statistically significant difference between immediate and delayed relief cases in clinical histories or operative observations. Therefore, our results suggest that the final judgement of the MVD effect could be made at least one year after surgery.     

Delayed effects of the microvascular decompression on hemifacial spasm: A retrospective study of 131 consecutive operated cases

Abstract

We reviewed 131 consecutive cases operated for hemifacial spasm (HFS) by the same surgeon between January 1983 and April 1999. Microvascular decompression (MVD) was performed via lateral suboccipital approach. Post-operative follow-up ranged from 1.5 to 10 years (average 34 months). The final outcome divided into three categories, excellent (total recovery) in 120 cases (91.6%), partial ( > 75% recovery) in 4 cases (3.1%), and unchanged or recurrent in 7 cases (5.3%). Only 2 cases were re-operated, and final outcome of both was excellent. Based on these data, we aimed to determine a period of the final judgement of MVD effect and the causative factors of delayed effects on HFS retrospectively. There were102 complete recovered cases without hemifacial paralysis; immediate recovery from HFS was observed in 78 cases (76.5%), after 1 month in nine cases, 1-3 months in 5 cases, 3-6 months in 3 cases, 6-10 months in 2 cases, and 10-12 months in 5 cases. Thus, most cases were completely recovered within one year of observation. On the other hand, there was no statistically significant difference between immediate and delayed relief cases in clinical histories or operative observations. Therefore, our results suggest that the final judgement of the MVD effect could be made at least one year after surgery. [Neurol Res 2002; 24: 296-300]     

显微血管减压术治疗三叉神经痛和面肌痉挛的技术差别(附37例报告)

目的为了提高手术安全性和疗效,减少术后并发症,探讨显微血管减压术治疗三叉神经痛和面肌痉挛的方法和技术细节差别。方法回顾性分析37例显微血管减压术手术患者,其中三叉神经痛15例,面肌痉挛22例,分析术中体位,切口,骨窗,责任血管压迫等细节,观察二者术后疗效。结果三叉神经痛患者术后疼痛立即完全缓解14例,1例延迟缓解。所有面肌痉挛患者痉挛症状术后即刻消失,无严重并发症。随访半年~2年,1例三叉神经痛患者复发,所有面肌痉挛患者未见复发。结论显微血管减压术是治疗三叉神经痛和面肌痉挛的有效治疗方法,但在术中体位、切口、骨窗、责任血管压迫等具体操作细节方面有细微差别,了解这些差别有助于增加手术安全性,取得更好的疗效。     

Prognostic value of intra-operative abnormal muscle response monitoring during microvascular decompression for long-term outcome of hemifacial spasm

The reliability of intra-operative abnormal muscle response (AMR) monitoring as an indicator of post-operative outcome in patients with hemifacial spasm (HFS) is under debate. The primary aim of this study was to evaluate the correlation between intra-operative AMR changes and long-term post-operative outcome. We monitored intra-operative AMR during microvascular decompression (MVD) in consecutive patients with HFS (n = 104). Patients in this study were divided into two groups based on whether their AMR disappeared or persisted following MVD. Ninety patients were followed-up, and the mean duration from surgery to final follow-up examination was 3.7 years. Fourteen patients were lost to follow-up. AMR disappeared during surgery for 80 patients; of these, 74 achieved complete resolution of HFS, five had persistent HFS, and one patient developed a recurrence of HFS. Of the 10 patients with persistent AMR despite effective MVD, eight patients achieved complete resolution, one patient had persistent HFS, and one developed recurrent HFS. The long-term clinical outcome of HFS after MVD did not significantly correlate with intra-operative AMR changes (p = 0.791). Therefore, we suggest that intra-operative AMR monitoring may not be a reliable indicator of long-term post-operative outcome for HFS.     

Efficacy of the Disappearance of Lateral Spread Response before and after Microvascular Decompression for Predicting the Long-Term Results of Hemifacial Spasm Over Two Years

Objective

The purpose of this large prospective study is to assess the association between the disappearance of the lateral spread response (LSR) before and after microvascular decompression (MVD) and clinical long term results over two years following hemifacial spasm (HFS) treatment.

Methods

Continuous intra-operative monitoring during MVD was performed in 244 consecutive patients with HFS. Patients with persistent LSR after decompression (n=22, 9.0%), without LSR from the start of the surgery (n=4, 1.7%), and with re-operation (n=15, 6.1%) and follow-up loss (n=4, 1.7%) were excluded. For the statistical analysis, patients were categorized into two groups according to the disappearance of their LSR before or after MVD.

Results

Intra-operatively, the LSR was checked during facial electromyogram monitoring in 199 (81.5%) of the 244 patients. The mean follow-up duration was 40.9±6.9 months (range 25-51 months) in all the patients. Among them, the LSR disappeared after the decompression (Group A) in 128 (64.3%) patients; but in the remaining 71 (35.6%) patients, the LSR disappeared before the decompression (Group B). In the post-operative follow-up visits over more than one year, there were significant differences between the clinical outcomes of the two groups (p<0.05).

Conclusion

It was observed that the long-term clinical outcomes of the intra-operative LSR disappearance before and after MVD were correlated. Thus, this factor may be considered a prognostic factor of HFS after MVD.     

Delayed Cranial Nerve Palsy after Microvascular Decompression for Hemifacial Spasm

Objective

Microvascular decompression (MVD) for hemifacial spasm (HFS) is a safe and effective treatment with favorable outcomes. The purpose of this study was to evaluate the incidence of delayed cranirve ( VI, VII, and VIII ) palsy following MVD and its clinical courses.

Methods

Between January 1998 and December 2009, 1354 patients underwent MVD for HFS at our institution. Of them, 100 patients (7.4%) experienced delayed facial palsy (DFP), one developed sixth nerve palsy, and one patient had delayed hearing loss.

Results

DFP occurred between postoperative day number 2 and 23 (average 11 days). Ninety-two patients (92%) completely recovered; however, House-Brackmann grade II facial weakness remained in eight other patients (8%). The time to recovery averaged 64 days (range, 16 days to 9 months). Delayed isolated sixth nerve palsy recovered spontaneously without any medical or surgical treatment after 8 weeks, while delayed hearing loss did not improve.

Conclusion

Delayed cranial nerve (VI, VII, and VIII) palsies can occur following uncomplicated MVD for HFS. DFP is not an unusual complication after MVD, and prognosis is fairly good. Delayed sixth nerve palsy and delayed hearing loss are extremely rare complications after MVD for HFS. We should consider the possibility of development of these complications during the follow up for MVD.     

显微血管减压术治疗面肌痉挛51例临床分析

目的 探讨显微血管减压术治疗特发性面肌痉挛疗效.方法 回顾分析显微血管减压术治疗51例面肌痉挛的临床资料,术中确认责任血管并减压.结果 本组均能见到面神经出脑干处有动脉血管压迫,其中AICA26例(51.0%),PICA16例(31.3%),VA4例(7.8%),复合型5例(9.8%).43例(84.4%)术后症状完全缓解,8例术后减轻(15.6%).其中4例(7.8%)3周内停止抽搐,3例(5.8%)3月后抽搐停止,1例(2%)一直未完全缓解.随访1年以上,复发1例(2.0%).结论 显微血管减压是面肌痉挛的首选方法,术中确认责任血管,充分减压和防止副损伤是手术成功的关键.     

The Efficacy and Safety of Microvascular Decompression for Hemifacial Spasm in Elderly Patients

Objective

The purpose of this study was to examine the efficacy and safety of microvascular decompression (MVD) for hemifacial spasm (HFS) in elderly patients.

Methods

Between 1997 and June 2008, 1,174 patients had undergone MVD for HFS at our institute. Among these, 53 patients were older than 65 years. We retrospectively reviewed and compared the complication and the cure rates of these patients with those of younger patients.

Results

There were 38 females and 15 males. The mean duration of symptoms of HFS of these patients was 94.6 months (range, 12-360 months), compared with 67.2 months (range, 3-360 months) in the younger group. The overall cure rate in elderly patients who underwent MVD for HFS during this period was 96.2%. Permanent cranial nerve dysfunctions, such as hearing loss and facial palsy, were seen in 2 patients (3.8%, 2/53) in the elderly group and 19 patients (1.7%, 19/1121) in the younger group. The difference in permanent cranial nerve dysfunction between the two groups was not statistically significant. There was no operative mortality in either group.

Conclusion

Microvascular decompression is the most effective surgical modality available for the treatment of HFS. Results of this study indicate that such technique can be performed in the elderly without higher rates of morbidity or mortality. Any patient with HFS, whose general health is acceptable for undergoing general anesthesia, should be considered as a candidate for MVD.     

绒球下入路微血管减压术治疗面肌痉挛疗效分析

目的 探讨绒球下入路微血管减压术（MVD）治疗面肌痉挛（HFS）的安全性和有效性。方法 回顾性分析2020年7月至2021年6月采用绒球下入路MVD治疗的233例HFS的临床资料。结果 术中发现单一血管压迫166例（71.2%）,其中小脑前下动脉142例;多支血管压迫67例（28.8%）,其中椎动脉参与53例。术后1、3、6、12个月有效率分别为95.3%、94.8%、95.3%、94.8%。术后1年复发率为1.7%。术后发生并发症26例（11.2%）,其中无菌性脑膜炎10例,脑脊液鼻漏3例,迟发性面瘫6例,后组颅神经麻痹4例,听力损伤2例,小脑梗死1例。结论 绒球下入路MVD治疗HFS是一种安全的、有效的手术方法,可充分显露面神经根出脑干区,同时减少对小脑的牵拉,降低听力损伤风险。