The shadow

Blepharospasm, the most frequent feature of cranial dystonia, and hemifacial spasm are two involuntary movement disorders that affect facial muscles. The cause of blepharospasm and other forms of cranial dystonia is not known. Hemifacial spasm is usually due to compression of the seventh cranial nerve at its exit from the brain stem. Cranial dystonia may result in severe disability. Hemifacial spasm tends to be much less disabling but may cause considerable distress and embarrassment. Patients affected with these disorders are often mistakenly considered to have psychiatric problems. Although the two disorders are quite distinct pathophysiologically, therapy with botulinum toxin has proven very effective in both. We review the clinical features, proposed pathophysiologic features, differential diagnosis and treatment, including the use of botulinum toxin, of cranial dystonia and hemifacial spasm.     

Microsurgical Vascular Decompression for Trigeminal Neuralgia and Hemifacial Spasm

Vascular cross-compression of cranial nerves has been proposed as the cause of cranial neuropathies, including trigeminal neuralgia and hemifacial spasm. Over the last decade we have used microsurgical vascular decompression to treat these two disorders. Results in 50 patients treated for trigeminal neuralgia have been excellent in 42, good in 5 and poor in 2; and 1 patient was cured after a second operation. Results in 22 patients treated for hemifacial spasm have been excellent in 18, good in 2 and fair in 1. One patient died. There were no late recurrences of symptoms.The pathophysiological mechanisms of trigeminal neuralgia and hemifacial spasm remain unknown.     

微血管减压术治疗面肌痉挛术后相关并发症的临床分析

目的:总结面肌痉挛患者的术后并发症的发生情况并分析其原因。方法:回顾性分析了1548名在我院行微血管减压术治疗面肌痉挛的患者的临床资料,所有患者接受电话随访或者门诊随访,随访时间均超过2年,总结其临床疗效及术后并发症的发生情况。结果:微血管减压术后痊愈率及明显缓解率分别为92.5%及4.2%。249名(16.09%)患者出现不同的并发症,其中最常见的并发症为面瘫及术后手术区域皮肤感觉障碍,无死亡及重大并发症患者。听力损害发生率为3.5%。其他并发症包括脑脊液漏、后组颅神经损伤、外展神经损伤、颅内出血等。结论:微血管减压术是治疗面肌痉挛的安全有效操作,以手术区域感觉障碍及迟发性面瘫是主要的并发症,持久性的或者严重的并发症比较少见。     

A 型肉毒毒素治疗半侧面肌痉挛的临床疗效评价

目的：观察A型肉毒毒素对面肌痉挛患者的痉挛程度、抑郁症状和焦虑症状的改善。方法：对58例面肌痉挛患者进行局部注射A型肉毒毒素。在治疗前后对痉挛程度改善情况进行评定以及用汉密顿焦虑量表（HAMA）、汉密顿抑郁量表（HAMD）对焦虑状态、抑郁状态进行评分,并对药物的副作用进行观察。结果：A型肉毒毒素明显改善面肌痉挛患者的痉挛程度,治疗后2周的HAMA、HA肋评分较治疗前明显下降,且差异具有统计学意义（P〈0．01）。结论：局部注射A型肉毒毒素可迅速缓解或消除面肌痉挛患者肌肉痉挛及相关的抑郁和焦虑状态,提高患者的生活质量。     

A型肉毒毒素治疗偏侧面肌痉挛和头颈部肌张力障碍的疗效观察

为评估Ａ型肉毒毒素治疗偏侧面肌痉挛和头颈部肌张力障碍的疗效,本文对４２例偏侧面肌痉挛及３４例头颈部肌张力障碍（后者包括１８例眼睑痉挛,１２例Ｍｅｉｇｅ氏病、４例痉挛性斜颈）病人进行Ａ型肉毒毒素肌肉多点注射治疗,并治疗前后的病情分级对比。结果表明,Ａ型肉毒毒素治疗有效率为１００％,疗效持续８～２６周,可重复注射,并再次取得疗效。部分病人局部出现轻度肌无力,数周后均可恢复,无全身毒副作用。结论认为Ａ型肉毒毒素能治疗偏侧面肌痉挛和头颈部局限性肌张力障碍,不失为一种有效安全简便易行的治疗手段。     

Auditory brain-stem responses in Marcus Gunn ptosis

The auditory brain-stem response (ABR) can detect anomalies both in the auditory pathways and in structures adjacent to these pathways. Patients with Duaneś retraction syndrome, associated with hypoplasia of the abducens nerve in the brain-stem, and patients with hemifacial spasm, due to compression of the facial nerve in the brain-stem, have been found to have abnormal ABRs. Marcus Gunn ptosis with ‘jaw winking’ is considered to be due to misconnection of oculomotor, trigeminal and other cranial nerves. Suspecting that perhaps some ‘jaw-winking’ phenomena may be due to detectable brain-stem anomalies we tested 7 patients with Marcus Gunn ptosis. Three of the patients demonstrated abnormal ABRs indicative of pontine pathology.     

Botulinum toxin A in clinical medicine

The results of the treatment with botulinum toxin A injection of 234 patients with blepharospasm and 73 with hemifacial spasm were reviewed. Visual function improved in the majority of patients with blepharospasm, and the improvement was sustained for up to 40 sets of injections. Mid and lower facial movements were also reduced in a minority of patients. However, a sub-group with pre-tarsal blepharospasm or persistent levator inhibition after treatment had a poor response. An average 75% reduction in abnormal movements was seen in cases of hemifacial spasm. Side effects of the treatment were usually mild and short lived.     

显微血管减压术对面肌痉挛患者生活质量的影响

目的:研究显微血管减压术对面肌痉挛(HFS Hemifacial Spasm)患者生活质量的影响。方法:对2014年至2016年底在哈医大一院神经外科四科接受显微血管减压手术(MVD Mcrovascular Decompression)治疗的40名面肌痉挛患者进行生活质量量表(QOL Quality of Life)评分,对患者的疗效及治疗前后生活质量的变化情况进行统计学分析。结果:治疗后,MVD的明显缓解率为95%,手术前后不同生活质量项目评分的差异具有统计学意义(P<0.05),术前QOL量表评分总值为21.83±2.01,术后总值为2.15±0.8,术后QOL量表评分总值较术前显著降低(P<0.05)。结论:显微血管减压手术对面肌痉挛患者的症状缓解效果显著,同时可以明显改善患者的生活质量,特别是对患者内心窘迫的改善最为明显。     

Contralateral injections of botulinum A toxin for the treatment of hemifacial spasm to achieve increased facial symmetry.

Six patients noted facial asymmetry after botulinum toxin injection for hemifacial spasm. Each patient was injected on the side contralateral to the spasms with 10 to 15 IU over the zygomatic major and minor muscles. Each patient noted improvement in facial symmetry in the resting position and dynamic facial movements. Five of the six patients desired this approach with subsequent injections. This injection method variation proved helpful in the managing of hemifacial weakness created by botulinum A toxin for this condition.     

Contributions from the auditory nerve to the brain-stem auditory evoked potentials (BAEPs): results of intracranial recording in man

Intraoperative recordings obtained from electrodes placed on the scalp (vertex and earlobe or ear canal) in response to click stimulation were compared with recordings made directly from the auditory nerve in patients undergoing microvascular decompression (MVD) operations to relieve hemifacial spasm (HFS) and disabling positional vertigo (DPV). The results support earlier findings that show that the auditory nerve is the generator of both peak I and peak II in man, and that it is the intracranial portion of the auditory nerve that generates peak II. The results indicate that the second negative peak in the potentials recorded from the earlobe is generated by the auditory nerve where it passes through the porus acusticus into the skull cavity, and that the proximal portion of the intracranial portion of the auditory nerve generates a positive peak in the potentials that are recorded from the vertex. This peak appears with a latency that is slightly longer than that of the second negative peak in the potentials recorded from the earlobe (or ear canal). The second negative peak in the recording from the ear canal and the positive peak in the vertex recording contribute to peak II in the differentially recorded BAEP. Since our results indicate that the difference in the latency of the second negative peak in the recording from the earlobe and that of the positive peak in the vertex recording represents the neural travel time in the intracranial portion of the auditory nerve, this measure may be valuable in the differential diagnosis of eighth nerve disorders such as vascular compression syndrome.     

Neurovascular free-muscle transfer to treat facial paralysis associated with hemifacial microsomia

Despite the wide spectrum of hemifacial microsomia manifestations, treatment mainly focuses on mandible and ear abnormalities, rather than on facial paralysis. In fact, the surgical treatment of facial paralysis associated with hemifacial microsomia is quite underdeveloped, because the degree of paralysis is frequently incomplete or partial. Timing and type of surgery are also difficult to determine. Neurovascular free-muscle transfer is now a standard procedure for the dynamic smile reconstruction of longstanding facial paralysis. This type of strategy has considerable potential in the treatment of facial paralysis in patients with hemifacial microsomia. We present here our experience with neurovascular free-muscle transfer for smile reconstruction in eight patients with facial paralysis associated with hemifacial microsomia. The age of the patients at the time of surgery ranged from 7 to 28 years old, (average, 13.9 years). Six were male patients and two were female patients. The two-stage method combining gracilis muscle transfer with cross-face nerve grafting was performed in three patients, whereas the one-stage transfer of the latissimus dorsi muscle was performed in five. To construct a natural or near-natural smile, the muscles were transferred into the paralyzed cheek in all except one patient, in whom the latissimus dorsi muscle was transferred into the sublabial area to reconstruct a paralyzed lower lip. A dermal flap segment vascularized with perforating vessels from the latissimus dorsi muscle was simultaneously inserted into the underdeveloped cheek for soft-tissue augmentation in this patient. Muscle contraction was evident in all patients between 4 and 8 months after muscle transfer. Our present series revealed that neurovascular free-muscle transfer is a good option not only for smile reconstruction but also for restoration of the facial contours of patients with hemifacial microsomia. Compared with the two-stage method combining gracilis muscle transfer with cross-face nerve grafting, the one-stage method using the latissimus dorsi muscle has some advantages, including a one-stage operation, a shorter recovery period, and the absence of sequelae that occur after harvesting a sural nerve.     

Electrophysiologic identification and evaluation of stylohyoid and posterior digastricus muscle complex.

PURPOSE: To identify the function of stylohyoid and posterior digastricus (STH-PD) muscle complex by the EMG techniques. METHODS: Unaffected sides of the faces of 30 patients with facial paralysis or hemifacial spasm were investigated. A concentric needle electrode was inserted to the STH-PD muscle complex and another concentric needle electrode was inserted to the orbicularis oris (OO) muscle. Simultaneous recording were obtained from two muscles using electrical stimulation (ES) (in 25 cases) and magnetic coil stimulation (MS) (in 15 cases); and both in 10 cases. Afterwards, the function of STH-PD was studied such as whistling, lip pursing, swallowing, jaw opening and closing. RESULTS: (1) The motor latency of compound muscle action potential (CMAP) of the STH-PD muscle was shorter than that of OO. (2) When the facial nerve was stimulated more distally than the stylomastoid foramen, the CMAP elicited from the STH-PD muscle complex immediately disappeared. (3) Ipsilateral MS was able to elicit the motor evoked potential (MEP) from STH-PD either at intracranially (half of cases) or at the extracranially. While OO muscle was always stimulated intracranially by MS. (4) The STH-PD muscle complex could not be basically recruited by the mimicry except lip pursing. The main recruitment were provided by swallowing and jaw opening. Cortical MS were facilitated during swallowing (5) Late reflex responses appeared in the STH-PD muscle complex during infraorbital-trigeminal and facial nerve ES. CONCLUSION: The STH-PD muscle complex is identified electrophysiologically. Although it is innervated by the facial nerve, its functions are mainly related with jaw opening and oropharyngeal swallowing. However, it is activated by the lip pursing.     

Hemifacial microsomia: use of the OMENS-Plus classification at the Royal Children's Hospital of Melbourne

Hemifacial microsomia is the most common facial congenital disability after cleft lip and palate, but as yet its pathogenesis remains unknown. Clinical classification systems have evolved over the last 30 years from those classifying only single components of the disorder, to those classifying according to the combination of deformities, to the most recent systems that grade each anatomical component separately, such as the Orbit, Mandible, Ear, Nerve, and Soft tissue (OMENS) system. The aim of the present study was to review the classification of patients with hemifacial microsomia treated by the Melbourne Craniofacial Unit at the Royal Children's Hospital using the OMENS-Plus system of classification and to correlate the findings with data from other centers. Records of patients treated by the craniofacial unit were reviewed and included in the study if adequate clinical records, photographs, and radiographs (anteroposterior, lateral, basal cephalometry, panoramic views) were available. The data were entered into a database file developed for this purpose. Seventy-one patients were identified from the hospital database, of which six were excluded because of incomplete data. Of the 65 patients, there were 31 (48 percent) with right-sided microsomia, 25 (38 percent) with left-sided microsomia, and nine (14 percent) with bilateral microsomia, with an overall male-to-female ratio of 1.2:1. The majority of patients had a normal orbit (77 percent), mildly hypoplastic mandibular ramus-condyle with functioning temporomandibular joint (57 percent with type M1 or M2a), normal facial nerve (76 percent), and mild soft-tissue hypoplasia (73 percent). There was a similar proportion of patients with mild ear anomalies (53 percent with grade 0 or 1) compared with those with more severe anomalies (47 percent with grade 2 or 3). Correlative analysis demonstrated a slight but positive correlation between the severity gradings of the five individual components. The correlation was lowest between the grading of the nerve and ear and that of the mandible and nerve. The data demonstrate the phenotypic variability of hemifacial microsomia and suggest a degree of relationship among the components of hemifacial microsomia. The OMENS-Plus system has provided a major advancement in the classification of hemifacial microsomia. The authors suggest refinements to the grading of the orbit and nerve components.     

Hemifacial deficiency induced by a shift in dominance of the mouse mutation far: a possible genetic model for hemifacial microsomia

Hemifacial deficiency appeared in 10% of juvenile mice when BALB/cGaBc mice carrying the recessive lethal mutation far were crossed with ICR/Bc. The hemifacial deficiency increased to 15-20% after one backcross to ICR/Bc and then remained at that level for 11 additional generations of backcrossing of far into ICR/Bc. Neither the ICR/Bc strain nor BALB/cGaBc (+/far) produces hemifacial deficiency. Genetic and anatomical studies of adults and fetuses showed that the hemifacial deficiency was due to +/far in the ICR/Bc strain genome; that is, far becomes an incomplete dominant in the ICR/Bc strain background. The hemifacial deficiency (38% of +/far) is probably caused by premature synostosis of the maxilla and premaxilla, observable on day 16 of gestation. An additional 20% of +/far in ICR/Bc have cleft palate and die at birth. Most +/far in both strains have a hidden anomaly, bilateral splitting of the maxillary branch of the trigeminal nerve. far/far homozygotes of both strain backgrounds have a syndrome of severe bilateral deficiency of the derivatives of the maxillary prominence. In human pedigrees, where the equivalents of the dominance modifiers in BALB/cGaBc and ICR/Bc would segregate within families, it would be difficult to recognize that sporadic hemifacial deficiency and severe bilateral maxillary deficiency were due to the same gene. We suggest that human bilateral and unilateral abnormalities of tissue derived from the first branchial arch should be analyzed with the awareness that, in mice, at least, the two kinds of syndrome are due to the same mutant gene.     

Altered Spontaneous Brain Activity in Patients with Hemifacial Spasm: A Resting-State Functional MRI Study

Resting-state functional magnetic resonance imaging (fMRI) has been used to detect the alterations of spontaneous neuronal activity in various neurological and neuropsychiatric diseases, but rarely in hemifacial spasm (HFS), a nervous system disorder. We used resting-state fMRI with regional homogeneity (ReHo) analysis to investigate changes in spontaneous brain activity of patients with HFS and to determine the relationship of these functional changes with clinical features. Thirty patients with HFS and 33 age-, sex-, and education-matched healthy controls were included in this study. Compared with controls, HFS patients had significantly decreased ReHo values in left middle frontal gyrus (MFG), left medial cingulate cortex (MCC), left lingual gyrus, right superior temporal gyrus (STG) and right precuneus; and increased ReHo values in left precentral gyrus, anterior cingulate cortex (ACC), right brainstem, and right cerebellum. Furthermore, the mean ReHo value in brainstem showed a positive correlation with the spasm severity (r = 0.404, p = 0.027), and the mean ReHo value in MFG was inversely related with spasm severity in HFS group (r = -0.398, p = 0.028). This study reveals that HFS is associated with abnormal spontaneous brain activity in brain regions most involved in motor control and blinking movement. The disturbances of spontaneous brain activity reflected by ReHo measurements may provide insights into the neurological pathophysiology of HFS.     

Causes of variations in the pathway of the basilar and vertebral arteries

Artery loops at the root exit zones of cerebral nerves are regarded as causes of certain diseases, e.g. trigeminal neuralgia or hemifacial spasm. The factors, which may cause such loops and displacements of arteries, however, are still not known sufficiently. In order to find out more about such causes, 60 corpses were examined. We recorded the variations in the positions of vertebral and basilar arteries and correlated them with the respective age at the time of death. We found that those showing atypical artery positions and loops were generally of older age. We further examined possible influences of blood flow factors on variations of artery positions. Our sample indicated such influence of flow factors on displacements of basilar artery, but they seemed to be of lesser importance than the effect of ageing.     

Auditory evoked blink reflex and posterior auricular muscle response: Observations in patients with HFS and PFS

Our goal was to investigate the characteristics of the auditory brainstem reflexes in patients with hemifacial spasm (HFS) and postparalytic facial syndrome (PFS).The spasm activities and responses by supraorbital and auditory stimuli were recorded from the orbicularis oculi, the posterior auricular and the mentalis muscles in 27 HFS and 21 PFS patients. The results were compared with those of 20 controls.Blink reflex (BR) was obtained by supraorbital stimulation in normal controls and on both sides of HFS and PFS patients whereas sound evoked bilateral auditory blink reflex (ABR) in 96.3%, 90.5% and 100%, respectively. Both BR and ABR showed synkinetic spread on symptomatic sides in all patients. The posterior auricular muscle response (PAMR) was observed bilaterally in 59.3%, 42.9% and 75.0% of groups, respectively. However, there was no synkinetic spread of PAMR.Since PAMR does not show synkinetic spread even in the presence of synkinetic spread of ABR and BR, we may suggest that a distal origin may be responsible of the synkinetic spread, or PAM is probably governed by a smaller nucleus in the brainstem. Thus it may be speculated that its excitability is insufficient to stimulate the ABR nucleus, whereas the reverse process is possible.     

Histochemical study of the facial nerve and of several muscles in Papio papio suffering from facial spasm]

Facial nerve neurectomy were performed in the baboon (Papio papio). Histochemical investigations showed usual criteria of such lesions. Therefore a loose of glycogene within the Type I muscular fibers was observed in animals with a former facial spasm.     

Axonal degeneration of peripheral facial nerve in a patient with progressive hemifacial atrophy.

We report a case of a 23-year-old woman with progressive hemifacial atrophy. She showed an atrophic change on the left side of her face for 8 years. A skin biopsy obtained from the lesion revealed the fibrotic changes in the deep dermis and adipose tissue with infiltrations of lymphocytes and plasma cells. She underwent the augmentation using a deepithelialized anteromedial thigh flap with endoscopic assistance. A specimen of the peripheral facial nerve taken from the region adjacent to the skin lesion during the operation showed atrophy of neurofibers with vacuole degeneration. On an electron microscopic examination, a high degree of degeneration of myelinated and unmyelinated axons was observed. These findings may provide direct evidence that atrophic changes of nerve fibers are closely related with the pathology of this disease.     

Botulinum toxin: a treatment for facial asymmetry caused by facial nerve paralysis

Injury to the frontal or other facial nerve branches can result in an asymmetry that can be very distressful to both patient and surgeon. This is especially true following cosmetic procedures such as rhytidectomy. We propose a means to create temporary symmetry while awaiting the possible return of nerve function. Botulinum neurotoxin causes a muscle paralysis lasting for approximately 3 months, and it is well established as the preferred treatment for blepharospasm. A case is presented in which botulinum toxin type A was injected into the opposite functioning frontalis muscle of a patient with unilateral frontal nerve paralysis. The patient experienced satisfactory relief of the asymmetry caused by onesided forehead wrinkling and brow elevation. Botulinum toxin therapy should be considered for both temporary and permanent facial asymmetries due to facial nerve paralysis as well as spasm.