Profile of patients with essential blepharospasm and hemifacial spasm in the two largest ophthalmology reference centers in Brazil.

PURPOSE: Information is scarce regarding the comprehensive profile of patients with essential blepharospasm and hemifacial spasm in Brazil. The present study aimed to assess the clinical features of patients with these conditions, followed up in two reference centers in Brazil. METHODS: The study included patients with essential blepharospasm and hemifacial spasm, followed up at the Departments of Ophthalmology at Universidade Federal de São Paulo and Universidade de São Paulo. Apart from demographic and clinical features, past stressful events related to the first symptoms (triggering event), aggravating factors, sensory tricks, and other ameliorating factors for the eyelid spasms were assessed. RESULTS: A total of 102 patients were included in this study. Most patients were female (67.7%). Essential blepharospasm was the most frequent movement disorder [51/102 patients (50%)], followed by hemifacial spasm (45%) and Meige's syndrome (5%). In 63.5% of the patients, the onset of the disorder was associated with a past stressful event. Ameliorating factors were reported by 76.5% of patients; 47% of patients reported sensory tricks. In addition, 87% of the patients reported the presence of an aggravating factor for the spasms; stress (51%) was the most frequent. CONCLUSION: Our study provides information regarding the clinical features of patients treated in the two largest ophthalmology reference centers in Brazil.

Prospective, Multicenter Clinical Study of Microvascular Decompression for Hemifacial Spasm.

BACKGROUND: Microvascular decompression (MVD) is the most effective procedure for hemifacial spasm (HFS). MVD results from nonspecialized or low-volume institutes are not always reliable. Most studies on MVD for HFS are retrospective and single centered; to the best of our knowledge, no prospective, multicenter studies exist. OBJECTIVE: To evaluate short- and long-term outcomes and complications in patients who underwent MVD for HFS in specialized Japanese institutions, in this multicenter, prospective, cohort study. METHODS: Included patients had undergone MVD for HFS in study centers between April 2012 and March 2015. Patients' postoperative grade of involuntary movements and complications were recorded postoperatively at 7 d (short-term) and at 1 (mid-term) and 3 (long-term) yr. RESULTS: A total of 486 patients (150 men, 336 women; mean age 53.9 yr with 181 patients over 60 yr) were enrolled during the study period. Neuromonitoring was used in 96.3% of the cases. The complete cure rate of symptom relief, mortality rate, and complication rate at short-term follow-up were 70.6%, 0%, and 15%, respectively. The long-term follow-up was completed by 463 patients (95.3%); the complete cure rate of symptom relief and complication rate were 87.1% and 3.0%, respectively. CONCLUSION: Our study revealed that under expert guidance and intraoperative neuromonitoring, the long-term curative effect rate of MVD for HFS is high, while complications are uncommon and usually transient. Our results indicate that MVD is an effective and safe treatment for patients with HFS, including elderly patients.

Role of neuroimaging in cases of primary and secondary hemifacial spasm.

Purpose: The objective of this study was to analyze the role of neuroimaging and documenting various intracranial pathologies in primary and secondary hemifacial spasm. Methods: This retrospective study included patients with HFS who had undergone neuroimaging. The demographic profile, onset, progression, neuroimaging findings, and types of HFS were documented and analyzed. Results: A total of 202 patients (male = 110, female = 92) were included. The mean age of the study population was 51.81 ± 11.76 years. The right side was involved in 104 patients, the left side was involved in 97 patients and bilateral involvement was observed in one patient. Primary HFS: secondary HFS was 9.6:1. The mean age of onset of the spasms in the primary HFS group was 49.26 ± 8.35 years and in secondary HFS was 43.13 ± 12.12 years respectively. The anterior inferior cerebellar artery was the major vessel causing neurovascular conflict in primary HFS (n = 55). Facial nerve palsy was the most common cause (n = 13) of secondary HFS followed by cerebellopontine angle (CPA) tumors. Conclusion: The hemifacial spasm occurs mostly in the fifth decade of life. Primary HFS is more prevalent than secondary HFS. Clinical distinction between them is difficult. Neuroimaging is essential to detect the conflicting vasculature in cases of primary HFS and pathologies like CPA tumor, cyst, and aneurysms in cases of secondary HFS.

[Hemifacial spasm. Etiology and management]. / Spasmes hémifaciaux. Étiologies et conduite à tenir.

INTRODUCTION: Hemifacial spasm (HFS) is an involuntary contracture of the facial muscles innervated by the ipsilateral facial nerve. We studied the etiology of these HFS. MATERIALS AND METHODS: This retrospective study included 233 patients with HFS who came to the ophthalmologist for quarterly botulinum neurotoxin A injection. Of these, we analyzed the 198 patients for whom MRI scans were performed. We recorded patient clinical data and clarified the etiology of their HFS. RESULTS: The 198 patients (62.6% women) had a mean age of 55.7±14years. An etiology was found in 52.5% of cases. In 34.5% of HFS, MRI revealed vascular compression where the facial nerve emerged from the brainstem. We specify the arteries involved. Brain tumors accounted for 1.5% of cases. MRI was normal in 64.5% of cases. In these secondary cases of HFS, we found 8.5% peripheral facial palsy, 4% post-traumatic HFS and 4% secondary to an eye injury. Stress was found in 17% of patients. DISCUSSION AND CONCLUSION: This study illustrates the need for MRI with attention to the posterior fossa in the work-up of HFS in order to identify primary HFS associated with vascular compression of the facial nerve and to rule out a rare but serious posterior fossa tumor. The treatment of HFS is based on quarterly injections of botulinum neurotoxin/A (NTBo/A), the three brands of which have market approval. The injection pattern and frequency is customized according to the results. In cases of insufficient response to injections of NTBo/A, neurosurgical microvascular decompression may be considered for cases of primary HFS.

The Epidemiology, Cause, and Prognosis of Painful Tic Convulsif Syndrome: An Individual Patient Data Analysis of 192 Cases.

BACKGROUND: Characterized by the coexistence of trigeminal neuralgia and ipsilateral hemifacial spasm (HFS), painful tic convulsif (PTC) is a rare entity that has not yet been systematically studied. OBJECTIVE: To systematically explore the epidemiology, cause, prognosis, and prognosis predictors of PTC. METHODS: We searched PubMed, Web of Science, and the Cochrane Library for relevant studies published between establishment of the library and July 1, 2020. Information on demographics, causes, specific interventions, and intervention outcomes was extracted. We first performed descriptive analysis of demographics, causes, and surgical outcomes of PTC. Univariate and multivariate regression methods were used to explore potential prognosis predictors. Further, a 2-step meta-analysis method was used to validate the identified factors. RESULTS: Overall, 57 reports including 192 cases with PTC were included in the analysis. The median age of patients with PTC is 54 years (range, 44-62 years), with more patients being female (P < 0.001), initiated as HFS (P = 0.005), and being affected with left side (P = 0.045). The vertebrobasilar artery contributes to >65% of the causes of single vascular compression for PTC. Anterior inferior cerebellar artery/posterior inferior cerebellar artery involvement (odds ratio, 4.050; 95% confidence interval, 1.091-15.031) and older age (P = 0.008) predict freedom from symptoms and recurrence after microvascular decompression, respectively. CONCLUSIONS: PTC occurs more in middle-aged women between 40 and 60 years old, initiates as HFS, and affects the left side. Vertebrobasilar artery compression is the most common single cause of PTC. Microvascular decompression effectively treated PTC, with a cure rate >80%. Anterior inferior cerebellar artery/posterior inferior cerebellar artery involvement predicts successful surgery and older age predicts recurrence.

Microvascular decompression in patients with hemifacial spasm.

PURPOSE: To study blood pressure alterations after microvascular decompression (MVD) surgery in patients with hemifacial spasm (HFS). METHODS: A retrospective study was performed to review HFS patients who received MVD surgery between January 2014 and December 2016. Vessels that were considered to be responsible for HFS were determined by reviewing the brain magnetic resonance imaging, magnetic resonance angiography, and surgical video. Blood pressure measurements were performed 1 day before (preoperative) and 7 days after (postoperative) the MVD surgery. Pre- and postoperative blood pressure measurements were compared. RESULTS: A total of 374 patients were included in the study, with 118 (31.6%) male patients, age 53.8 ± 9.9 years old, and 141 (37.7%) patients with hypertension. Systolic blood pressure had statistically significant decrease in patients with (134.5 ± 8.2-132.6 ± 9.1 mmHg, p = .01) or without (125.6 ± 9.1-123.8 ± 10.0 mmHg, p = .01) hypertension. Diastolic blood pressure only had statistically significant decrease in patients with hypertension (83.0 ± 5.8-82.0 ± 6.5 mmHg, p = .04). Analyses in all the study patients and in the subgroup of patients with hypertension showed that more statistically significant blood pressure reductions were observed when left-side vessel or vertebrobasilar artery was involved. CONCLUSION: In patients with HFS, MVD not only decreased blood pressure in patients with hypertension but also affected blood pressure in patients without hypertension. Blood pressure reductions were more prominent when left-side vessel or vertebrobasilar artery was involved.

Geographic Disparities in the Surgical Management of Hemifacial Spasm in Canada.

BACKGROUND: We aimed to interrogate the Canadian Institute for Health Information (CIHI) database in order to determine the geographic distribution and outcomes of microvascular decompression (MVD) for the treatment of hemifacial spasm (HFS). METHODS: The CIHI database was searched from 2004 to 2017 for relevant diagnostic and procedure codes. A new database was populated with the following categories: year, institution, province, number of interventions per year, and mean length of stay. Descriptive statistics were generated. Provincial utilization rates of MVD for HFS were calculated. RESULTS: During the period 2004-17, we identified 671 MVDs performed for HFS at 20 centers across Canada. During the study period, 286 MVDs (42.6%) were performed at one center in Manitoba. Another 131 (19.5%) and 72 (10.7%) were performed at one center in British Columbia and Ontario, respectively. The remaining 182 (27.1%) MVDs for HFS were performed at 17 centers across the rest of Canada where the mean number of cases performed per year was 1.1 (SD=0.9, range 0.2-2.9). When out-of-province cases were re-allocated to place of residence, the adjusted provincial utilization of MVD for HFS ranged between 0.5 and 6.1 patients per million per year. CONCLUSIONS: Microvascular decompression for HFS is performed relatively rarely and there is a tremendous geographic variation in utilization across Canada. Although most of these surgeries are performed by a few surgeons, more than half of Canadian centers perform an average of less than 1 case per year. Further examination of the impact of these discrepancies appears warranted.

Disparités géographiques dans la prise en charge chirurgicale du spasme hémifacial au Canada. CONTEXTE: Nous avons voulu interroger la base de données de l'Institut canadien d'information sur la santé (ICIS) afin de déterminer la répartition géographique et les résultats de la décompression vasculaire microchirurgicale (DVMC) dans le traitement du spasme hémifacial. MÉTHODES: Notre recherche a porté sur les années 2004 à 2017 et visait, au moyen de la base de données de l'ICIS, à repérer des diagnostics et des codes d'acte pertinents. Une nouvelle base de données a été ensuite alimentée en fonction des catégories suivantes : l'année de l'intervention, l'établissement concerné, la province, le nombre d'interventions par année et la durée moyenne de séjour des patients. C'est à partir de cette nouvelle base de données que des statistiques descriptives ont été produites. À cet égard, nous avons calculé les taux provinciaux d'utilisation de la DVMC pour traiter le spasme hémifacial. RÉSULTATS: Au cours de la période allant de 2004 à 2017, nous avons identifié 671 interventions de DVMC dans 20 établissements de santé situés partout au Canada. Durant cette période d'étude, 286 interventions de DVMC (42,6 %) ont été réalisées dans un seul établissement du Manitoba tandis que 131 autres (19,5 %) et 72 autres (10,7 %) ont été respectivement réalisées dans un seul établissement de la Colombie-Britannique et de l'Ontario. Quant aux 182 (27,1 %) autres interventions, elles ont été réalisées dans 17 établissements du reste du pays où le nombre moyen d'interventions par année était de 1,1 (σ = 0,9; étendue = 0,2 ­ 2,9). Une fois réassignés les cas de patients provenant d'autres provinces, et ce, en fonction de leurs lieux de résidence, l'utilisation ajustée par province de la DVMC variait, sur une base annuelle, de 0,5 à 6,1 patients par million. CONCLUSIONS: Les interventions de DVMC pour traiter le spasme hémifacial sont réalisées peu fréquemment. Il existe aussi une énorme variation géographique au Canada quant à l'utilisation de cette technique chirurgicale. Bien que la plupart de ces interventions soient effectuées par quelques chirurgiens, plus de la moitié des établissements de santé canadiens concernés effectuent en moyenne moins d'une intervention par année. Un examen plus approfondi de l'impact de ces écarts semble ainsi se justifier.

Epidemiology of benign essential blepharospasm: A nationwide population-based retrospective study in Taiwan.

IMPORTANCE: This study provides a nationwide, population-based data on the incidence of benign essential blepharospasm in Asian adults. BACKGROUND: To describe the incidence, patient demographics, and risk factors associated with benign essential blepharospasm. DESIGN: Population-based retrospective study. PARTICIPANTS AND SAMPLES: A total of 1325 patients with benign essential blepharospasm were identified. METHODS: Patients with diagnosis of blepharopsasm between January 2000 and December 2013 were sampled using the Longitudinal Health Insurance Database 2000. Secondary blepharospasm that may be related to neurological, trauma, and ocular surface disease were excluded. MAIN OUTCOME MEASURED: Multivariate conditional logistic regression was used to estimate the odds ratios for potential risk factors of benign essential blepharospasm. RESULTS: The mean annual incidence was 0.10‰ (0.07‰ for males, and 0.12‰ for females). The peak incidence was in the 50 to 59-year-old age group (0.19‰). People living in urban regions have more risk of developing blepharospasm comparing to people living in less urban regions (p <0.01). White-collar workers also have higher chance of having blepharospasm (p<0.001). Significant difference between control group and case group in hyperlipidemia (p <0.001), sleep disorders (p <0.001), mental disorders (depression, anxiety, obsessive compulsive disorder) (p <0.001), dry eye-related diseases (dry eye, Sjögren's syndrome) (p <0.001), Parkinson's disease (p <0.004), and rosacea (p <0.021) were also identified. CONCLUSIONS AND RELEVANCE: Higher level of urbanization, white-collar work, sleep disorders, mental health diseases, dry eye-related diseases, Parkinsonism, and rosacea are possible risk factors for benign essential blepharospasm.

Outcome of Biomedical Glue Sling Technique in Microvascular Decompression for Hemifacial Spasm Involving the Vertebral Artery.

BACKGROUND: Microvascular decompression (MVD) is the most useful treatment for hemifacial spasm (HFS). In cases of vertebral artery (VA) compression of the facial nerve, MVD is often difficult. In this study, we compared the outcome of the biomedical glue sling technique with the traditional technique in MVD for HFS involving the VA. METHODS: A retrospective study of patients with HFS treated by MVD was conducted between January 2013 and December 2015. A total of 327 patients with VA-associated HFS underwent their first MVD at our institution. Among them, the traditional technique was performed in 153 patients and the biomedical glue sling technique was performed in 174 patients. We measured effectiveness at 1 day, 7 days, 1 month, 3 months, and 1 year after MVD surgery. RESULTS: In the traditional technique group, the effective rates of MVD were 89.54%, 88.89%, 89.40%, 88.44%, and 86.71%, and the incidence rates of complication were 5.23%, 4.58%, 3.97%, 2.72%, and 0.70%. In the biomedical glue sling technique group, the effective rates of operation were 96.55%, 96.55%, 97.66%, 95.86%, and 95.76% (P < 0.05), and the incidence rates of complication were 8.62%, 8.62%, 7.60%, 4.73%, and 2.42% (P > 0.05). CONCLUSION: When the HFS were associated with the VA, the effective rate of biomedical glue sling technique of MVD was higher than the traditional technique, and there was no statistical difference between the 2 groups about the incidence of complication.

The Current Status of Microvascular Decompression for the Treatment of Hemifacial Spasm in Japan: An Analysis of 2907 Patients Using the Japanese Diagnosis Procedure Combination Database.

Microvascular decompression (MVD) is widely used as a safe and effective treatment for hemifacial spasm (HFS). However, the extent of application of this therapeutic method and its outcomes in Japan are currently unclear. To address these questions, we analyzed the utilization of MVD for the treatment of HFS during the 33- month period from July 2010 to March 2013. We conducted an analysis on data contained in the Diagnosis Procedure Combination database in Japan. This analysis showed that MVD was used for the treatment of HFS in 2907 cases (men, 916; women, 1991) with 2.2 times more women treated than men. MVD for HFS was most frequently performed in women aged 50 to 69 years; however, most men were aged between 40 and 59 years at the time of the procedure. The numbers of procedures performed per 100,000 population/year were 0.83 overall in Japan, with the numbers larger in prefectures with larger populations. Regarding discharge outcomes, the mortality rate was 0.1%. The mean length of hospital stay in patients undergoing MVD for HFS was 14.7 days. This analysis provides preliminary information regarding the trends in the performance of MVD for the treatment of HFS in Japan. Further studies on other registries that contain data obtained by standardized assessment methods and that include long-term outcomes and postoperative complications are required.

Five-Year Retrospective Review of Cases with Benign Essential Blepharospasm and Hemifacial Spasm Presenting in a Tertiary Eye Care Center in North India.

Retrospective analysis of epidemiological and clinical characteristics of patients diagnosed with benign essential blepharospasm and hemifacial spasm who reported to the oculoplasty clinic of a tertiary eye care center in north India between January 2010 and April 2015 was carried out. Dry eye, as well as all the local factors that can cause blepharospasm or hemifacial spasm, was ruled out. Systemic evaluation was done to rule out any neurological disorder. A detailed history was taken to rule out any associated psychiatric disorders as well as use of any medication which could be responsible for dystonic movements. In every patient of hemifacial spasm, magnetic resonance imaging (MRI) of the brain was done for any facial nerve compression or tumor involving posterior fossa. Botulinum type A injections were given after assessing their requirements on the basis of guidelines given by Jankovic et al.

Revisiting the link between hypertension and hemifacial spasm.

The relationship between hypertension and hemifacial spasm (HFS) has been debated. Microvascular decompression surgery is effective in some HFS patients with uncontrolled hypertension. To address current gaps in knowledge, we conducted a meta-analysis of case-control studies that have examined the prevalence of hypertension in HFS patients compared to non-HFS controls. We also evaluated the implications and limitations of the pooled studies. We identified 62 studies from PubMed, The Cochrane Library, Web of Science and Scholar.google.com and six studies that fit our inclusion criteria were included. A random-effects model was used to derive the pooled estimate of the Odds Ratio. The data was plotted on a Forest plot. A pooled analysis involving 51585 subjects, 549 cases, 720 neurological controls and 50316 controls from the general population, showed that HFS patients had a higher chance of developing hypertension (OR = 1.72, 95% CI = (1.12, 2.31), p-value <0.001). The prevalence of hypertension was higher in HFS patients as compared to non-HFS patients. This meta-analysis highlights a positive correlation between hypertension and HFS. Blood pressure should be closely monitored during the follow-up of HFS patients. Preliminary links between ventrolateral medullary (VLM) compression and HFS should be further evaluated in future studies.

Benign essential blepharospasm: risk factors with reference to hemifacial spasm.

BACKGROUND: To identify risk factors associated with benign essential blepharospasm (BEB) with reference to hemifacial spasm (HFS). Persons with BEB and HFS experience similar physical symptoms, yet the two disorders have different etiologies. METHODS: Patients with BEB (n = 159) or HFS (n = 91) were identified from two large neuro-ophthalmology clinics. Demographic, medical, behavioral, and psychological characteristics were obtained from chart review and a telephonic survey questionnaire. RESULTS: The average age of BEB and HFS was 66 years. Most patients in both groups were retired, white, and female. BEB patients were more than two times as likely to meet the diagnostic criteria for generalized anxiety disorder than HFS patients (odds ratio, 2.13; 95% confidence interval, 1.22-3.72). There was no difference between the two groups regarding demographics, smoking, a family history of dystonia, Parkinson disease, Bell palsy, Tourette disorder, obsessive compulsive symptoms, history of head trauma, alcohol use, or caffeine consumption. CONCLUSIONS: As compared to HFS, BEB was significantly more often associated with generalized anxiety disorder. Given the similarity of other clinical features of these two disorders, it is reasonable to conclude that anxiety is a cause not a consequence of BEB. Contrary to previous studies, BEB was not associated with obsessive-compulsive symptoms, head trauma, Parkinson disease, Bell palsy, Tourette disorder, or lack of smoking.

[Surgical removal of unilateral vestibular schwannomas after failed Gamma Knife radiosurgery]. / Exérèse microchirurgicale des schwannomes vestibulaires unilatéraux préalablement traites par radiochirurgie Gamma Knife.

One of the primary criticismes of vestibular schwannoma (VS) radiosurgery is that the risk of surgical morbidity is increased for patients whose tumor progresses after the procedures. We reviewed the French experience of operated patients after failed Gamma Knife radiosurgery. From July 1992 to January 2002, 25 out of the 1000 treated patients underwent another treatment procedure for a gamma knife failure. Excluding the NF2 patients, 21 patients have been operated and the present study shows the data collected for 20 of them. In order to analyze the difficulties observed during the surgery, a questionnaire was filled by the surgeons. The mean interval between radiosurgery and removal was 36 Months, from 10 to 83 Months. The mean increase in Volume was 559% (37 to 3036%, median 160%). Evolution of the Koos grading was found from 8 grade II, 10 grade III et 2 grade IV to 10 grade III and 10 grade IV. Patients have been operated for radiological tumor growth in 7 cases and for clinico-radiological evolution in 13 cases. In 9 cases, the surgeon considered that he had to face unusual difficulties mainly because of adhesion of the tumor to neurovascular structures. Tumor removal was total in 14 cases, near total in 4 cases and subtotal in 2 cases. One case of venous infarction was noticed at the second day following surgery responsible of hemiparesis and aphasia that gradually recovered. At last follow-up examination, facial nerve was normal (House and Brackmann grade I and II) in 10 cases while it was a grade III in 7 cases and grade IV and V in 3 cases. We recommend that the decision for surgical removal of growing vestibular schwannoma after Gamma Knife treatment should be done after a sufficiently long follow-up period. Our results show that the quality of removal and of facial nerve preservation might be impaired by radiosurgery in half of cases. However these results do not support a change in our policy of radiosurgical treatment of small to medium size vestibular schwannoma.

[Radiosurgery of vestibular schwannomas after microsurgery and combined radio-microsurgery]. / Radiochirurgie Gamma Knife après microchirurgie des schwannomes vestibulaires et stratégie combinée radio-microchirurgicale.

Significant morbidity is expected after microsurgery for recurrent or growing residual vestibular schwannoma (VS). In order to avoid this procedure, radiosurgery appeared as an interesting alternative. The purpose of this study is to analyze the results of Gamma Knife radiosurgery in this indication. Between July 1992 and January 2002, 60 patients (including 12 NF2 patients) underwent radiosurgical treatment after one or more attempts of surgical resection (including 27 growing remnant VS and 19 recurrent VS) and could be followed with enough informations, out of 103 patients (10% of the VS population treated during the same period). Mean interval between surgical removal and radiosurgery was 71.5 Months (1.8-127.8 Months). Technical difficulties during the procedure were observed in the 12 cases, mainly due to problems in identifying the target. Median follow-up was 51.6 Months. Four out of 58 patients (7%, confidence interval: 1.9-16.7) were diagnosed as failure. Statistical study failed to find significant parameters influencing failure. Facial and trigeminal nerves were not impaired while one case of severe bulbopontine radio-induced injury leading to a lower cranial nerve deficit was observed. These results show that, in spite of additional difficulties to treat these patients with radiosurgery, this treatment is efficient with acceptable morbidity and can avoid another microsurgical procedure. The strategy of planned combined micro-and radiosurgical treatment of large VS deserves additional investigations to be validated.