Bilateral hemifacial spasm: a series of 10 patients with literature review

Bilateral hemifacial spasm (HFS) is a rare movement disorder posing diagnostic doubt with other facial dyskinesias. In this report, we describe clinical, radiological and therapeutic features of 10 patients with bilateral HFS. The prevalence of bilateral HFS in our sample was of 2.6%. Clinical characteristics of our patients did not differ from the classic features of unilateral cases. The mean latency for contralateral facial nerve involvement was of 33.3 months. In only one case a vascular abnormality was seen. We conclude that bilateral HFS is rare and that clinical differentiation with other facial dyskinesias should be promptly made to introduce appropriate therapy.     

Hemifacial spasm caused by cross type vascular compression

Abstract

Objectives: The meatal segment of anterior inferior cerebellar artery usually crosses over the gap between cranial nerves VII and VIII, and may compress the cisternal portion (CP) of the facial nerve. This is defined as cross type compression, which is easy to be neglected and thus leads to poor outcome. Here our experience in treating patients of cross type hemifacial spasm (HFS) is reported.

Methods: Twenty-one patients of HFS due to cross type compression were treated with microvascular decompression (MVD) surgery with the aid of abnormal muscle response monitoring.

Results: In addition to cross type compression at CP, there were typical vascular compressions on the root exit zone and attached segment in 20 cases. After MVD surgery, 17 patients were cured, 3 patients achieved good resolution of spasm, and the other 1 patient got delayed resolution. Three patients had postoperative transient hearing loss and/or tinnitus.

Discussion: Even there are apparently typical vascular compressions at proximal portion of the facial nerve, the surgeon should be aware that cross type compression at the CP may co-exist. With the aid of abnormal muscle response (AMR) monitoring, MVD is efficient for patients due to cross type compression.     

以三叉神经痛和/或面肌痉挛起病的桥小脑角区肿瘤的手术治疗

目的探讨经枕下乙状窦后入路手术治疗以三叉神经痛和/或面肌痉挛起病的桥小脑角区肿瘤的临床疗效。方法回顾性分析2014年9月至2016年9月收治的21例以三叉神经痛和/或面肌痉挛起病的桥小脑角区肿瘤的临床资料,均采用枕下乙状窦后入路手术切除肿瘤,其中17例同期行微血管减压术。结果肿瘤全切除18例,近全切除3例。术后原有面部疼痛或不自主抽搐症状消失或明显减轻。术后随访3~27个月,临床症状和肿瘤均无复发。结论枕下乙状窦后入路手术治疗以三叉神经痛和/或面肌痉挛起病的桥小脑角区肿瘤的疗效确切,安全性高,并发症少,对于同时存在血管压迫的病例,可同期行微血管减压术治疗。     

Treatment with botulinum toxin improves the hyperexcitability of the facial motoneuron in patients with hemifacial spasm

Abstract

Botulinum toxin type A (BTX) injection into the orbicularis oculi muscle is an effective treatment for patients with hemifacial spasm (HFS). The objectives of this study were to investigate the effect of this treatment on HFS, in particular the associated hyperexcitability of the facial motor nucleus, and to discuss the potential mechanism of HFS. F waves in the mentalis muscle were examined before, 2 and 6 weeks after the BTX treatment of only the orbicularis oculi muscle in ten patients with HFS. F/M ratio, duration of F waves and frequency of F waves decreased significantly after the BTX treatment compared with those before the BTX treatment. These findings demonstrate that the excitability of the facial motonucleus decreases after BTX treatment of the orbicularis oculi muscle. From these results, we hypothesize that the trigeminal afferent input and the cortical control contribute to the hyperexcitability of the facial motor nucleus in patients with HFS. This warrants further investigation into the pathophysiology of HFS.     

Psychogenic hemifacial spasm

Facial spasms that distort facial expression are typically due to facial dystonia, tics, and hemifacial spasm (HFS). Psychogenic facial spasms, however, have not been well characterized. The authors sought to 1) determine prevalence of psychogenic facial spasm in patients referred for evaluation of HFS and 2) draw attention to clinical characteristics and potential diagnostic pitfalls. Among 210 consecutive patients referred for evaluation of HFS, 5 (2.4%) received diagnoses of psychogenic facial spasm. All patients were female; mean age was 34.6 years (range 26-45) and mean symptom duration 1.1 years (range 2 wk-2 yr). Onset was left-sided in 3 patients, and the lid was the initial site affected in 2 patients. This series of patients shows that facial spasms, although usually of neurovascular etiology, may be the initial or only manifestation of a psychogenic movement disorder, often associated with an underlying depression.     

Ependymoma of the fourth ventricle presenting with hemifacial spasm. Report of a case.

According to Gardner's hypothesis (1962) later confirmed by Jannetta (1982, 1985), hemifacial spasm can usually be related to a "vascular conflict" which takes place inside the cerebellopontine angle (CPA). Occasionally, the causative lesion can be identified as a mass encasing the facial nerve at its root exit zone (REZ) from the brain stem. The hemifacial spasm has been rarely reported in presence of a contralateral CPA mass ("false localising sign"). Hemifacial spasm in patients with masses in anatomical regions other than the CPA has to be considered exceptional. The case of an adult man harboring an ependymoma of the fourth ventricle whose only neurological sign was a left hemifacial spasm is reported. The rarity of such a condition prompted us to review the literature. Particular attention has been paid to the possible pathogenetic mechanisms and their therapeutic implications.     

Enterogenous cyst as a rare cause of hemifacial spasm

Hemifacial spasm (HFS) is most frequently caused by vascular compression of the facial nerve at the root exit zone of the brain stem, but there are other rare causes. An enterogenous cyst (EC) is a rare congenital lesion which occurs rarely in the cerebellopontine angle. The authors report a patient with HFS from an EC and review unusual causes of HFS.     

Distinguishing features of psychogenic (functional) versus organic hemifacial spasm

Hemifacial spasm (HFS) is one of the most common presentations in patients with cranial psychogenic (functional) movement disorders (PMD). Medical records and videos of patients with PMD and HFS were reviewed to identify those with psychogenic HFS and to compare the phenomenology of psychogenic HFS with organic HFS. We identified 18 (9.8%) patients with psychogenic HFS from a cohort of 184 patients with PMDs. There were 14 (78%) women and 4 men, with a mean age at onset of 33 ± 13.5 years. These were compared with 37 consecutive patients with organic (primary) HFS. Patients with psychogenic HFS were significantly younger and had more frequently tonic muscle contractions, bilateral asynchronous hemifacial involvement, isolated lower facial involvement, downward deviation of the mouth’s angle, and lack of the “other Babinski sign” compared to those with organic HFS. Other features such as ipsilateral downward movements of the eyebrow; associated tremor, dystonia and hemi-masticatory spasms were more frequently observed in patients with psychogenic HFS but these differences did not reach statistical significance. Lack of other Babinski sign and tonic muscle contractions showed the highest sensitivity (1.00 and 0.87, respectively), whereas downward mouth’s angle deviation showed the highest specificity (1.00) for the diagnosis of psychogenic HFS. Besides other features such as suggestibility, distractibility, periods of unexplained improvements observed in most patients with PMDs, several clinical features, such as tonic muscle contractions, downward mouth’s angle deviation, predominant lower facial and bilateral involvement, may be helpful in distinguishing psychogenic from organic HFS.     

Young onset hemifacial spasm

INTRODUCTION: Hemifacial spasm (HFS) frequently affects middle aged individuals and the clinical features and etiology have been well reported. However, there is limited data on the exact pathogenesis in young-onset HFS. If age is a major determinant of the etiology or influences the presentation of HFS, there may be clinical differences between the young and elderly HFS patients. OBJECTIVES: We determined the prevalence, clinical and imaging features of young-onset HFS (age of onsetor=65 years) HFS patients. METHODS: We examined consecutive patients clinically diagnosed with HFS in a tertiary referral center. The clinical (demographics, clinical presentation, severity of HFS, associated medical conditions and other variables) and imaging findings of young onset patients and old onset patients were tabulated and compared. RESULTS: Amongst 230 consecutive HFS patients, 15 (6.5%) were young-onset HFS and 50 (21.7%) were old-onset HFS. In the young-onset HFS, the mean age of onset of symptoms was 26.5+/-6.5 (6-30) years, with 80% women and 75.0% of young onset HFS having neurovascular compression (NVC) of the root exit zone (REZ) of the facial nerve on the ipsilateral side; 86.7% had initial onset of twitching in the upper eyelids that later progressed to the lower facial muscles. While the prevalence of hypertension, diabetes mellitus and other associated vascular disorders in late onset HFS was higher than in young onset groups, the clinical features and frequency of NVC of the facial REZ between the two groups were similar. CONCLUSIONS: We demonstrated a 6.5% frequency of young-onset HFS in our cohort of HFS and their clinical presentation was similar to the old onset patients. Genetic, anatomic or other unidentified factors may contribute to NVC in young-onset HFS.     

Hemifacial Spasm: A Neurosurgical Perspective

Hemifacial spasm (HFS) is characterized by tonic clonic contractions of the muscles innervated by the ipsilateral facial nerve. Compression of the facial nerve by an ectatic vessel is widely recognized as the most common underlying etiology. HFS needs to be differentiated from other causes of facial spasms, such as facial tic, ocular myokymia, and blepharospasm. To understand the overall craniofacial abnormalities and to perform the optimal surgical procedures for HFS, we are to review the prevalence, pathophysiology, differential diagnosis, details of each treatment modality, usefulness of brainstem auditory evoked potentials monitoring, debates on the facial EMG, clinical course, and complications from the literature published from 1995 to the present time.     

Interaction between the blink reflex and the abnormal muscle response in patients with hemifacial spasm: results of intraoperative recordings

Patients with hemifacial spasm (HFS) have an abnormal muscle response (AMR) that can be elicited by stimulating one branch of the facial nerve and recording electromyographically from muscles innervated by other branches of the facial nerve. In addition, the R1 component of the blink reflex can be elicited from the affected side in patients with HFS who are undergoing microvascular decompression (MVD) operations under inhalation anesthesia. A synkinetic component of the blink reflex response that corresponds to the R1 component can be recorded from the mentalis muscle. In the present study we show that the blink reflex elicited by electrical stimulation of the supraorbital nerve can suppress the AMR elicited by electrical stimulation of the temporal branch of the facial nerve in patients with HFS when the interval between stimulation of the supraorbital nerve and stimulation of the temporal branch of the facial nerve (interstimulus interval, ISI) is such that the blink reflex response would appear later than the AMR if they had been elicited independently. Within a short range of ISIs the two responses suppress each other partially or totally. We find evidence that the suppression of the AMR is the result of an interaction in the facial motonucleus. We believe that the results of the present study support the hypothesis that the facial motonucleus is hyperactive in patients with HFS, and we suggest that the AMR is a result of backfiring from the facial motonucleus and that it may thus be an exaggerated F-response.     

Direct correlation between the facial nerve nucleus and hemifacial seizures associated with a gangliocytoma of the floor of the fourth ventricle: a case report

A dysplastic neuronal lesion of the floor of the fourth ventricle (DNFFV) causes hemifacial seizures (HFS) from early infancy. However, it is still controversial whether HFS is generated by the facial nerve nucleus or cerebellar cortex. In this study, we confirm a direct correlation between the rhythmic activities in the DNFFV and HFS using intraoperative electroencephalography (EEG) and electromyography (EMG) monitoring. Our results support the theory that a DNFFV provokes ipsilateral HFS via the facial nerve nucleus.     

Hemifacial spasm as clinical presentation of intracranial meningiomas. Report of three cases and review of the literature

Hemifacial spasm (HFS) is a clinical entity consisting of brief clonic jerking movements of the facial musculature, beginning in the orbicularis oculi with downward spreading to other facial muscles. Apart from vascular loop compression at the root exit zone of the facial nerve, other causes of HFS are rare. It is exceptional as a form of presentation of intracranial meningiomas We report three cases of patients with meningiomas who presented with HFS, either as an isolated sign or associated with symptoms of rise intracranial pressure or focal deficit. We review the literature and discuss the possible physiopathological mechanisms responsible for this association.     

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.     

Microvascular decompression surgery: surgical principles and technical nuances based on 4000 cases

Abstract

Background:

As an etiological treatment of trigeminal neuralgia (TN) and hemifacial spasm (HFS), microvascular decompression (MVD) has been popularized around the world. However, as a functional operation in the cerebellopontine angle (CPA), this process can be risky and the postoperative outcomes might not be good enough sometimes.

Objective:

In order to obtain a better result with less complication, this surgery should be further addressed.

Methods:

With experience of more than 4000 MVDs, we have gained knowledge about the operative technique. Through abundant intraoperative photos, each step of the procedure was demonstrated in detail and the surgical strategy was focused.

Results:

The principle of MVD is to separate the nerve-vessel confliction rather than isolate it with prostheses. A prompt identification of the conflict site is important, which hinges on a good exposure. A satisfactory working space can be established by an appropriate positioning of the patient's head and a proper craniectomy as well as a rational approach. A sharp dissection of arachnoids leads to a maximal visualization of the entire intracranial course of the nerve root. All the vessels contacting the trigeminal or facial nerve should be treated. Intraoperative electrophysiological mentoring is helpful to distinguish the offending artery for hemifacial cases.

Conclusion:

MVD is an effective treatment for the patient with TN or HFS. Immediate relief can be achieved by an experienced neurosurgeon with good knowledge of regional anatomy. A safe surgery is the tenet of MVD, and accordingly, no single step of the procedure should be ignored.     

Hemifacial spasm associated with epidermoid tumors of the cerebellopontine angle

Hemifacial spasm (HFS) is rarely due to serious compressive lesions, such as tumors, aneurysms, or vascular malformations, located in the cerebellopontine angle. Because of the interesting association of HFS with epidermoid tumors, we reviewed the records of all patients with HFS and all patients with intracranial epidermoid tumors seen from January 1975 to December 1986. Of the 18 patients who had epidermoid tumors of the cerebellopontine angle, 3 (17%) had a facial movement disorder that resembled HFS at sometime during their illness. There were 429 patients who had HFS with no obvious serious compressive lesion of the facial nerve. Therefore, HFS was associated with epidermoid tumor in 0.7% of cases. All 3 patients developed other findings due to involvement of adjacent neural structures. Patients with HFS have a low probability of having a serious compressive lesion, but those with atypical features should be evaluated for cerebellopontine angle masses such as epidermoid tumors.     

Hemifacial spasm and facial myokymia: electrophysiological findings

In two patients, one with clinical hemifacial spasm (HFS) and one with clinical facial myokymia, the spontaneous electromyographic findings consisted of regular burst activity: each burst composed of high frequency repetitive motor unit potentials. These bursts fired synchronously in multiple ipsilateral facial muscles in both patients and from a contralateral facial muscle in the patient with HFS. In addition, the patient with HFS exhibited crossed evoked responses on the affected side with stimulation of the facial and supraorbital nerves on the unaffected side. It is concluded that the current electrophysiological distinctions between HFS and facial myokymia may be unsupportable and that facial nuclear hyperactivity with activation of bilateral facial nuclear connections plays a role in the pathophysiology of HFS.     

Efficacy of levetiracetam in primary hemifacial spasm

Hemifacial spasm (HFS) is a peripherally-induced movement disorder characterized by the involuntary, unilateral, intermittent, irregular, tonic or clonic contractions of muscles innervated by the ipsilateral facial nerve. Kindling-like hyperactivity of the facial nucleus induced by constant stimulation of compressing artery is considered as the predominant mechanism underlying the pathogenesis of HFS. As a treatment for HFS, microsurgical decompression and botulinum toxin injection have been shown to be highly successful. Anticonvulsant drugs relieve HFS in some patients; however, the use of such drugs is limited owing to their side effects, predominantly in elderly patients. We experienced two elderly HFS patients who exhibited a marked response to levetiracetam (LEV) without side effects. Although the exact underlying pharmacological mechanism remains unknown, we assume anti-kindling effect as one of the important pharmacological mechanism underlying the effect of LEV against HFS. Moreover, LEV is considered to be suitable for use in elderly patients because of its good tolerability. In addition, the lack of hepatic induction or inhibition makes it an easy and safe drug when used in addition to other anticonvulsants. Although the long-term benefit remains unknown, LEV may represent an alternative treatment for elderly HFS patients who are unable to undergo or decline surgical intervention and/or botulinum toxin injections or are intolerant to other anticonvulsants.     

Unusual causes of hemifacial spasm

Hemifacial spasm (HFS) has been defined as consisting of brief clonic jerking movements of the facial musculature, beginning in the orbicularis oculi with downward spreading to other facial muscles. HFS, perhaps the most common of the abnormal involuntary facial movements, has been classically ascribed to vascular loop compression at the root exit zone of the facial nerve. Causes other than such vascular loops are rare in the medical literature. Here we present three case studies in which the phenomenology of the HFS was atypical in onset and evolution. Using these three patients as introduction to the topic, we reviewed the literature of all cases of HFS with causes other than the vascular loop. In these three cases, HFS was caused by (1) a parotid gland tumor, (2) a cerebellopontine angle meningioma, and (3) an acoustic schwannoma. We also discuss the radiological findings as well as possible differences in the genesis of HFS and phenomenology in such cases and present recommendations on how to evaluate these patients.     

Ephaptic transmission in hemifacial spasm: a single-fiber EMG study

In two patients with hemifacial spasm (HFS), single-fiber EMG recordings in facial muscles demonstrated low jitter in the late responses produced by stimulation of peripheral branches to other facial muscles. Surgical decompression of the facial nerve in one patient was followed by clinical improvement and disappearance of the abnormal late responses. These observations are consistent with the hypothesis that there is ephaptic transmission among peripheral branches of the facial nerve at the site of compression in HFS.