Secondary Hemifacial Spasm Research Articles

Botulinum therapy for facial nerve disorders in Russia

Botulinum toxin type A (BTA) has taken a strong place in the rehabilitation of patients with impaired function of mimic muscles in both acute and late post-paralytic periods.Aim: to highlight the main stages of the development of botulinum therapy (BT) in the treatment of mimic muscles dysfunctions in Russia.Methods. Published data were searched in the electronic database Medline (PubMed) and eLibrary.Results. The role of BT in the treatment of mimic muscles dysfunctions, synkinesis (which can be interpreted clinically as secondary hemifacial spasm), lacrimation, dry eye syndrome, as well as the role of BT in the formation of protective ptosis, was analyzed. The stages of the BT formation in Russia as a method of restoring the function of mimic muscles by specialists of various profiles (neurologists, maxillofacial surgeons, rehabilitation specialists, etc.) are described.Conclusion. Currently, there is a BT school in Russia, which based on extensive clinical experience, developed algorithms and methods for administering BT to patients with facial nerve disorders of various etiologies.

Clinical Profile of Patients with Hemifacial Spasm at a Tertiary Eye Care Center in South India: A Retrospective Study.

To assess the incidence and clinical profile of hemifacial spasm (HFS) and the association between HFS and systemic diseases. This retrospective study was carried out on 85 patients with HFS, presenting at a tertiary eye care center in South India. Demographic and clinical details were recorded for all patients. Of these, the patients who had undergone magnetic resonance imaging (MRI) of the brain were analyzed for primary and secondary HFS. The mean age of the patients was 56.11 ± 12.51 years. The age at onset of HFS was 54.9 ± 12.7 years. The disease duration was 9.51 ± 7.28 years. Male:female ratio was 1:1.17. The right side was involved in 31 patients (36.47%) and the left side in 54 patients (63.52%). MRI was performed in 54 (63.52%) patients and showed neurovascular conflict in 22 (40.74%) patients and space-occupying lesions in 2 (3.70%) patients. Forty-nine (57.64%) patients had primary HFS, while five (5.88%) patients had secondary HFS due to old facial palsy in 3 and space-occupying lesions in two patients. Twenty (23.52%) patients received botulinum toxin A with a good response. Type of HFS had a significant association with hypertension (P = 0.046) while no significant association was present between laterality of HFS and systemic diseases (P > 0.05 each). Multivariate analysis showed a marginally significant association between type of HFS and hypertension (P = 0.057). Primary HFS was the main type of HFS with female dominance and predilection for the left side. Hypertension had a relationship with HFS that needs to be investigated further for its causal nature.

Hemifacial Spasm Caused by Cerebellar Arteriovenous Malformation and Spasm-Relief after Nidus Removal

Secondary hemifacial spasm (HFS) occurs by a conflict between the facial nerve and intracranial pathology, such as cerebellopontine angle tumor or vascular abnormality like arteriovenous malformation (AVM) or aneurysm. HFS caused by AVM is rare, and optimal combination of treatment options has not been established. AVM treatment with or without microvascular decompression (MVD) of the facial nerve was provided in the previous studies, and the outcomes in terms of spasm relief have been successful. Here, we report a case of HFS caused by cerebellar AVM. HFS symptom disappeared after AVM nidus removal without MVD of the facial nerve.

Eight years experience with botulinum toxin A and eyelid surgery in the treatment of hemifacial spasm

AIM: To report the therapeutic outcomes of botulinum toxin A (Botox) and eyelid surgery in patients with hemifacial spasm (HFS). METHODS: Patients’ images and medical notes were retrospectively reviewed with subsequent analysis of both the therapeutic outcomes and complications of Botox injections. RESULTS: The information of 76 patients (female=58) with HFS who received a minimum of 4 Botox injections were included. The mean follow-up interval was 83±50 (20-112)mo with an average of 16±10 (4-34) injections. The peak incidence was between 55 and 64 years and the average age of onset was 66±11 (32-85)y. Up to 23% of patients with HFS had aberrant vascular structures (right=8, left=7) in close relationship to the facial nerve (MRI=14, CT=1), where the vertebral artery (n=6) was the most involved vessel followed by the anterior inferior cerebellum artery (n=5). Patients with primary HFS had a shorter effective duration (2.5 vs 3.1mo, P<0.05), a longer onset time (4.1 vs 3.8d, P=0.739) and a lower Subjective Spasm Alleviation score (SSAs; 1.7 vs 1.9, P=0.179) than those with secondary HFS. Twelve of the 19 patients with preexisting eyelid diseases underwent surgical correction including upper blepharoplasty (n=12), limited myectomy (n=7), browplasty (n=7) and advancement of levator aponeurosis (n=5). Five (41.7%) of those with surgical correction and suboptimal response to Botox showed improvement 6mo after surgery [onset time (P=0.0256), effective duration (P=0.374) and SSAs (P=0.0161)]. Those 12 patients with eyelid surgery had a lower complication rate than those without eyelid surgery (23% vs 42%, P≤0.05). CONCLUSION: Botox is an effective and safe treatment for HFS. Patients with primary HFS had a less favorable therapeutic outcome with Botox than those with secondary HFS. Eyelid surgery for patients with concurrent eyelid diseases can augment the effect of subsequent Botox injections by improving patient satisfaction and reducing complication rate.

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.

Mechanism underlying cranial nerve rhizopathy

The cranial nerve rhizophathy, commonly presented with trigeminal neuralgia (TN) or hemifacial spasm (HFS), is a sort of hyperexcitability disorders with higher incidence in senior Asian. In this paper, a novel hypothesis on the pathogenesis is proposed and with which some clinical phenomena are explained.In those with crowded cerebellopontine angle in anatomy, the cranial nerve root and surrounding vessel are getting closer and closer to each other with aging and finally the neurovascular conflict happens. As the interfacial friction associated with pulse, the nerve incurs demyelination. Since this pathological change develops to a certain degree, some transmembrane proteins emerge from the nerve due to a series of signaling pathway mediated by inflammatory cytokines. Among them, voltage-gated (Nav1.3) and mechanosensitive (Piezo2) ion channels may play the important role. With pulsatile compressions, the Piezo2 drives the resting potential toward depolarization forming a state of subthreshold membrane potential oscillation. Under this condition, just an appropriate pressure can make the membrane potential easy to reach threshold and activate the sodium channel, eventually generating conductible action potentials from the axon. When these ectopic action potentials propagate to the central nerve system, an illusion of sharp pain is perceived; while to the nerve-muscle junctions, an attack of irregular muscle constriction occurs.This hypothesis can well explain the symptomatic manifestation of paroxysmal attacks aroused by emotions. When we get nervous or excited, our heart rate and blood pressure alter correspondingly, which may give rise to “a just right pressure” – with specific frequency, amplitude and angle – impacting the suffered nerve to reach the threshold of impulse ignition. After a successful microvascular decompression surgery, the trigger is gone (there is no compression anymore) and the symptom is alleviated. While the postoperative recurrence could be attributable to Teflon granuloma development if had been placed improperly – for this nerve root has been susceptible no matter to arteries or to neoplasms. Besides, it may illustrate the clinical phenomenon that secondary TN or HFS cases are seldom caused by schwannoma: with a proliferative sheath, the nerve root is actually insulated. By contrast, not all neurovascular contacts can lead to the onset: it demands an exclusive extent of demyelination firstly.

Hemifacial spasm

Hemifacial spasm (HFS), or facial hemispasm, is a paroxysmal hyperkinetic disorder involving muscles innervated by the facial nerve, mainly on the one hand. The development of HFS is based on neurovascular conflict. However, it is impossible to explain the clinical manifestations of HFS only by nerve compression. Both peripheral and central mechanisms are involved in the generation of HFS, with the formation of ephaptic transmission, antidromic excitation, primary or secondary hyper-excitability of the nuclear and supranuclear level of innervation. Two treatment methods are pathogenetically justified: microvascular decompression (MVD) and botulinum toxin (BTX) injections. The effectiveness of MVD is 95.37% with full or partial recovery. The recurrence rate does not exceed 2.4%. Facial nerve paralysis (2.7-22.5%) and hearing loss (1.9-20%) are the most common complications of treatment with the use of the MVD for HFS with partial or complete cure in most cases. Botulinum toxin injection chemo-denervation is a first-line treatment of primary and secondary HFS. HFS is an officially registered indication for the drug dysport (abobotulotoxin) (ABO) in the Russian Federation. Total doses of ABO ranged from 25 to 150 units for one side depending on the severity of the clinical manifestations. Studies demonstrate the statistically significant benefits of HFC treatment with ABO. ABO is generally well-tolerated. Adverse events (up to 3.6%) are transient and include ptosis, lacrimation, blurred vision, double vision, dry eyes and weak facial muscles.

Magnetic resonance imaging findings of hemifasial spasm

Hemifacial Spasm (HFS) is irregular, painless, clonic withdrawal of one half of the face. In addition to medical treatment, surgical treatment is applied according to the underlying cause. magnetic resonance imaging (MRI) is essential to show the offending vessel at the origin of HFS and the absence of other causes of nerve compression. Twenty-five patients with HFS (17 female, 8 male) were evaluated in our clinic. The mean age of the patients was 56.4 years (32-78). While MRI of 17 patients was unremarkable, 4 patients had basilar artery dolichoectasia, 2 patients had basilar artery indentation, 1 patient had cerebellopontin tumour and 1 patient had cerebellopontine arachnoid cyst. The patient with cerebellopontin mass lesion had undergone operation and 12 patients were treated with botulinum toxin. Imaging has first to exclude a secondary hemifacial spasm and secondly to search for and characterize the responsible neurovascular conflict, including arterial dolichoectasia or mass lesions.

Surgical Treatment of Secondary Hemifacial Spasm: Long-Term Follow-Up

Hemifacial spasm (HFS) is generally caused by the root exit zone of the facial nerve compressed by an overlying arterial loop. HFS can also be caused by various types of tumor, aneurysm, or arteriovenous malformation. We retrospectively analyzed patients to evaluate possible differences in the demographic and clinical features between primary and secondary HFS. A retrospective study of 3140 cases of HFS treated in our department between January 2009 and June 2016. Among the 3140 total cases, 26 patients had secondary HFS. The 26 tumors of secondary HFS included 11 meningiomas, 8 epidermoid cysts, and 7 vestibular schwannomas. Compared to those with idiopathic HFS, those patients with tumor-induced HFS were significantly younger (P < 0.05). Secondary HFS tended to have responsible vessels, and were observed in 20 (76.92%) of these 26 patients. The long-term effective rate of operation was 84% in the secondary HFS group and was 96.45% in the primary HFS group (P < 0.05); the incidence rate of complication was 12% in the secondary HFS group and was 3.06% in the primary HFS group (P < 0.05). In cases of secondary HFS, facial nerves of most patients were compressed by blood vessels, so microvascular decompression after tumor resection plays an important role. We should examine the entire nerve root for possible vascular compression.

Imaging of hemifacial spasm

Almost all primary hemifacial spasms are associated with one or more neurovascular conflicts, most often at the root exit zone in the immediate vicinity of the brainstem. Imaging has first to exclude a secondary hemifacial spasm and secondly to search for and characterize the responsible neurovascular conflict(s). Magnetic resonance imaging should include high-resolution anatomical hyper T2-weighted sequences and magnetic resonance angiography by using 1.5 or even better 3 Tesla magnets. The most frequent vascular compressions are from the anterior-inferior cerebellar artery, the posterior-inferior cerebellar artery and the vertebrobasilar artery; venous conflicts are very rare. Conflicts are often multiple; also, the same vessel may compress the facial nerve in two places. Also, conflicts may be aided by particular anatomical circumstances, including arterial dolichoectasia, posterior fossa with a small volume or bony malformations.

Clinical features and response to botulinum toxin in primary and secondary hemifacial spasm.

Hemifacial spasm (HFS) is a hyperkinetic movement disorder of adults. The condition is usually primary, but may be secondary to facial nerve damage, and responds well to botulinum toxin therapy. To study the demographic and clinical features of primary and secondary HFS and assess its response to botulinum toxin therapy. A retrospective study of 40 patients of primary (33) and secondary (7) HFS, who attended our botulinum toxin clinic over the last 18 months, was conducted. We collected the data including the demographic profile, clinical features of primary and secondary HFS, severity measured by Likert scale, etiology, details of magnetic resonance imaging (MRI) of the brain, and a detailed account of the botulinum toxin injection. Thirty-three out of the 40 patients had primary HFS. The mean age at presentation was 48.4 ± 12.6 years whereas the mean duration of symptoms was 57.4 ± 39.3 (6-144) months. The onset of spasm on the upper part of face was seen in 31 patients (77.5%). An ipsilateral ear click was found in 17 (42.5%) patients. Mild spasms were seen in 18 patients, whereas 12 patients had severe spasms. The severity of spasm was lower in patients with secondary HFS. Five out of 22 magnetic resonance imaging (MRI) results showed an abnormality. The mean dosage of botulinum toxin was 21.86 ± 4.19 units in primary spasm and 17 ± 4.16 units in secondary spasm (P =0.018). The mean duration of response was 112.6 ± 55.7 days (median: 90 days; range: 30-240 days) in primary spasm and 135 ± 63.6 days (median: 135 days; range: 90-180 days) in secondary spasm (P = 0.29). Ipsilateral auditory clicks are fairly common in primary and secondary HFS. The patients with secondary HFS have a milder intensity of disease and they may have the onset of spasm from the upper part of face. Both primary as well as secondary HFS patients respond well to botulinum toxin injection; however, the adverse effects are more likely to occur in secondary HFS, and therefore, these patients require lower dosages of toxin.

Secondary or Symptomatic Hemifacial Spasm caused by a Tumor of Cerebellopontine Angle Case Report and Review of the Literature

Background & Importance: Primary hemifacial spasm is usually caused by microvascular compression of the facial nerve at its root exit zone at brainstem without any space-occupying pathology. Secondary or so-called symptomatic hemifacial spasm has an additional underlying pathology e.g. tumors leading to the microvascular compression. We review and discuss the pathophysiology and the literature of secondary hemifacial spasm. Case Presentation: We report on a 23 year-old man with a secondary right-sided hemifacial spasm in association with an ipsilateral epidermoid tumor of the cerbello-pontine angle. Conclusion: Intraoperatively, no offending vessel was observed near the root exit zone of the facial nerve. Microsurgical tumor removal relieved the patient also from his hemifacial spasm.

Two Cases of Secondary Hemifacial Spasm: Pathophysiology and Management.

Dear Editor, Classical hemifacial spasm (HFS) has been attributed to an atraumatic pulsatile vascular compression of the facial nerve leading to hyperactivity within the central components of the nerve, alleviated via mobilization of the culprit vessel [1]. In a small subset of patients the cause may be attributed to lesions involving the facial nerve, most commonly benign cerebellopontine angle (CPA) tumors [2,3]. In such secondary HFS the clinical manifestation is usually a tonic facial contraction rather than the intermittent twitching characteristic of common HFS [4,5]. There may, however, also be rare patients with a common presentation of HFS but with an associated CPA tumor [6,7]; the role of neurovascular compression (NVC) in these cases is uncertain. We present 2 cases of secondary HFS, both related to benign CPA tumors.

Hemifacial Spasm and Neurovascular Compression

Hemifacial spasm (HFS) is characterized by involuntary unilateral contractions of the muscles innervated by the ipsilateral facial nerve, usually starting around the eyes before progressing inferiorly to the cheek, mouth, and neck. Its prevalence is 9.8 per 100,000 persons with an average age of onset of 44 years. The accepted pathophysiology of HFS suggests that it is a disease process of the nerve root entry zone of the facial nerve. HFS can be divided into two types: primary and secondary. Primary HFS is triggered by vascular compression whereas secondary HFS comprises all other causes of facial nerve damage. Clinical examination and imaging modalities such as electromyography (EMG) and magnetic resonance imaging (MRI) are useful to differentiate HFS from other facial movement disorders and for intraoperative planning. The standard medical management for HFS is botulinum neurotoxin (BoNT) injections, which provides low-risk but limited symptomatic relief. The only curative treatment for HFS is microvascular decompression (MVD), a surgical intervention that provides lasting symptomatic relief by reducing compression of the facial nerve root. With a low rate of complications such as hearing loss, MVD remains the treatment of choice for HFS patients as intraoperative technique and monitoring continue to improve.

Intraoperative Facial Electromyography and Brainstem Auditory Evoked Potential Findings in Microvascular Decompression for Hemifacial Spasm: Correlation with Postoperative Delayed Facial Palsy

Background: Delayed facial palsy (DFP) after microvascular decompression (MVD) in patients with hemifacial spasm (HFS) is not uncommon, but the cause remains unknown. Objectives: To assess whether intraoperative electromyography (EMG) and brainstem auditory evoked potential (BAEP) can predict DFP after MVD. Methods: Between September 2009 and February 2011 we examined 86 patients, 9 of whom (10.4%) developed DFP after MVD on the same side. All patients underwent MVD and were followed-up for a median period of 13 months (range 6–22). We retrospectively examined intraoperative facial EMG and BAEP findings using our MVD patients’ registry. We excluded secondary HFS and immediate postoperative facial palsy after MVD in this study. We assessed the prevalence and clinical characteristics of DFP and compared EMG and BAEP findings between DFP and non-DFP groups. Results: All patients recovered completely, with a mean time to recovery of 37.8 days (range 22–57). There were no significant differences between DFP and non-DFP patients in terms of the amplitude and latency of intraoperative EMG and BAEP. Conclusion: The usefulness of intraoperative facial EMG and BAEP is limited and cannot predict DFP after MVD for HFS. We speculate that DFP after MVD is not associated with permanent nerve damage according to the EMG findings.

Hemifacial Spasm due to Compression of the Posterior Inferior Cerebellar Artery Aneurysm Treated with Botulinum Toxin Type-A: A Case Report

A 79-year-old female presented with five months history of progressive involuntary twitching movement on left face. Brain MR imaging revealed a heterogeneous T2 hyperintense lesion at left cerebellopontine angle. CT angiography showed a partially thrombosed saccular aneurysm of left PICA (posterior inferior cerebellar artery). The patient was treated with Botulinum toxin type A and almost total relief of symptoms was noticed during one month followup. Botulinium toxin injection is an effective symptomatic treatment option in nonsurgical secondary hemifacial spasm (HFS) cases.

A Comparative Study of Primary and Secondary Hemifacial Spasm

Hemifacial spasm (HFS) is a common movement disorder. To evaluate possible differences in the demographic and clinical features between primary and secondary HFS. In-person interview using a standardized questionnaire to collect demographic and clinical data. A multicenter study that included patients with HFS attending 3 Italian academic centers. Patients Two hundred fourteen patients with HFS. A complete neurological examination assessed the current muscle distribution of spasm and the presence of synkinetic movements between upper and lower facial muscles. 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. 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.

特発性顔面けいれんの経験 手術と電顕所見について

Eight patients with idiopathic hemifacial spasm (HFS) were treated by surgery. Five of them (1985-1986) were treated with a total facial nerve decompression through a transmastoid and middle fossa approach combined with retrolabyrinthine microvascular decompression (MVD). A small amount of facial nerve tissue was taken from various portions in the temporal bone for electronmicroscopic evaluation in these cases. The other three patients (1990-1991) were treated by MVD through a suboccipital approach. One patient needed revision surgery, the other seven had an excellent clinical course without any complications. A definite vascular compression of the facial nerve at the root entry zone was identified in six patient. The ultrastructural findings suggested that the histological changes in the nerve tissue itself were not so severe as in Bell's palsy which needs decompression surgery. We concluded that MVD surgery by an experienced surgeon is the best treatment for HFS provided we can exclude secondary HFS.