Facial phantom model: a low-cost and safe tool for teaching botulinum toxin application in neurology residencies / Modelo de manequim facial: uma ferramenta segura e de baixo custo para o ensino da aplicação de toxina botulínica nas residências em neurologia

Abstract Background The application of botulinum toxin (BoNT) in the treatment of blepharospasm and hemifacial spasm (HS) is a well-established practice. However, neurology residency programs often rely on real patients for training, which has limitations in terms of patient availability and skill acquisition. Objective Assess the efficacy of a new facial phantom model for acquiring motor skills in BoNT application. Methods An anthropomorphic facial phantom model was developed in collaboration with a medical training simulator start-up. A group of seven neurologists and one ophthalmologist with expertise in BoNT application evaluated the model using an adapted learning object review instrument (LORI). The instrument assessed aspects such as: content quality, alignment of learning objectives, feedback and adaptation, motivation, presentation design, and accessibility. Results The facial phantom model received high scores in the LORI evaluation, with the highest ratings given to alignment with learning objectives and motivation. The model also scored well in terms of accessibility, content quality, and presentation design. However, feedback and adaptation received a lower score due to the static nature of the model. Conclusion The facial phantom model shows promise as a valuable tool for teaching and developing competence in BoNT application for HS and blepharospasm. The model reduces the reliance on real patients for training, providing a broader and safer learning experience for neurology residents. It also provides a realistic learning experience and offers portability, cost-effectiveness, and ease of manufacturing for use in various medical training scenarios. It is an effective and accessible tool for teaching BoNT application.

Resumo Antecedentes A aplicação de toxina botulínica (TxB) no tratamento do blefaroespasmo e do espasmo hemifacial (EH) é uma prática bem estabelecida. No entanto, os programas de residência em neurologia frequentemente dependem de pacientes reais para treinamento, o que apresenta limitações em termos de disponibilidade de pacientes e aquisição de habilidades. Objetivo Avaliar a eficácia de um novo modelo de manequim facial para aquisição de habilidades motoras na aplicação de TxB. Métodos Foi desenvolvido um modelo antropomórfico de manequim facial em coloboração com uma empresa de simuladores de treinamento médico. Um grupo constituído por sete neurologistas e um oftalmologista com experiência em aplicação de TxB avaliou o modelo utilizando um instrumento adaptado de revisão de objeto de aprendizagem (LORI). O instrumento analisou aspectos como: qualidade do conteúdo, alinhamento dos objetivos de aprendizagem, feedback e adaptação, motivação, concepção da apresentação e acessibilidade. Resultados O modelo de manequim facial obteve pontuações altas na avaliação do LORI com os maiores escores em alinhamento com os objetivos de aprendizagem e motivação. O modelo também obteve boas pontuações em termos de acessibilidade, qualidade do conteúdo e concepção da apresentação. No entanto, o item feedback e adaptação recebeu uma pontuação média mais baixa, devido à natureza estática. Conclusão O modelo manequim facial mostra-se promissor como uma EH e blefaroespasmo. O modelo reduz a dependência de pacientes reais para treinamento portátil, de baixo custo e de fácil fabricação para uso em diversos cenários de treinamento, proporcionando uma experiência de aprendizagem mais ampla e segura para residentes de neurologia. Além disso, fornece uma experiência de aprendizagem realista e oferece portabilidade, economia e facilidade de fabricação para uso em vários cenários de treinamento médico. É uma ferramenta eficaz e acessível para o ensino da aplicação de TxB.

Profile of patients with essential blepharospasm and hemifacial spasm in the two largest ophthalmology reference centers in Brazil / Perfil dos pacientes com blefaroespasmo essencial e espasmo hemifacial nos dois maiores centros de referência em oftalmologia no Brasil

ABSTRACT 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.

RESUMO Objetivo: Há poucos dados sobre o perfil de pacientes com blefaroespasmo essencial e espasmo hemifacial no Brasil. O objetivo deste estudo é avaliar de forma mais abrangente as características clínicas dos pacientes portadores dessas doenças acompanhados em dois centros de referência em oftalmologia no Brasil. Métodos: Pacientes com blefaroespasmo essencial e espasmo hemifacial, acompanhados nos Departamentos de Oftalmologia da Universidade Federal de São Paulo e da Universidade de São Paulo foram incluídos neste estudo. Além dos dados clínicos e demográficos, foram avaliados também a presença de eventos estressantes relacionados ao início dos sintomas (evento desencadeante), fatores agravantes, truques sensoriais, e outros fatores de melhora. Resultados: Cento e dois pacientes foram incluídos no estudo. A maior parte dos pacientes era do sexo feminino (67,7%). Blefaroespasmo essencial foi a condição mais frequente, observada em 51 (50%) dos pacientes. Espasmo hemifacial correspondia a 45% dos casos, enquanto 5% dos pacientes apresentavam a Síndrome de Meige. 63,5% dos pacientes associaram o início dos sintomas com um evento estressante prévio. 76,5% dos pacientes relataram fatores de melhora para os espasmos; 47% relataram truques sensoriais. Além disso, 87% dos pacientes relataram fatores de piora; estresse (51%) foi o mais frequente. Conclusão: Este estudo fornece informações a respeito das características clínicas dos pacientes com blefaroespasmo essencial e espasmo hemifacial acompanhados nos dois maiores centros de referência em oftalmologia do Brasil.

PARRY ROMBERG SYNDROME - A RARE CASE REPORT

The purpose of this report is to present a rare case of Parrv-Romberg syndrome (PRS), also called "progressive hemifacial atrophy". This rare degenerative condition is characterized by atrophic changes affecting one side of the face. The etiology of these changes remains idiopathic. Definitive diagnosis of Parry-Romberg syndrome is based on thorough clinical and radiological examination. Treatment is usually conservative while others include using alloplastic implants to improve facial disfigurement was suggested to the patient.

Clinical comparison of modified postauricular transverse incision and traditional vertical incision for microvascular decompression in the treatment of hemifacial spasm / 中国医师进修杂志

Objective:To compare the effect of modified postauricular transverse incision and traditional vertical incision for microvascular decompression in the treatment of hemifacial spasm.Methods:Prospective study method was used. A total of 116 patients with hemifacial spasm in Handan Central Hospital from January 1, 2019 to January 1, 2020 were selected, and divided into two groups according to the admission order. Both groups underwent microvascular decompression; control group (57 cases) received traditional vertical incision, while treatment group (59 cases) received modified postauricular transverse incision. The brainstem auditory evoked potential (BAEP), pain degree, surgical indicators, facial aesthetic satisfaction and complications were compared between two groups.Results:After treatment, the BAEP of latency, wave interval and wave amplitude in the two groups increased compared with that before treatment, and the BAEP of latency, wave interval and wave amplitude in the treatment group were higher than those in the control group: (1.89 ± 0.15) ms vs. (1.62 ± 0.21) ms, (7.89 ± 0.15) ms vs. (6.25 ± 0.41) ms, (1.79 ± 0.19) ms vs. (1.54 ± 0.11) ms ( P<0.05). After treatment, the visual analogue score (VAS) of patients in the two groups decreased compared with that before treatment, and the VAS of patients in the treatment group was lower than that in the control group: (1.15 ± 0.27) points vs. (2.18 ± 0.24) points ( P<0.05). The operation time, intraoperative bleeding volume and postoperative scar length of patients in the treatment group were less than those in the control group: (60.41 ± 3.81) h vs. (76.87 ± 3.87) h, (30.18 ± 4.19) ml vs. (56.87 ± 4.15) ml and (4.18 ± 1.07) cm vs. (6.87 ± 1.05) cm ( P<0.05). The satisfaction rate of patients in the treatment group was higher than that in the control group: 91.53% (54/59) vs. 71.93% (41/57) ( P<0.05). The complication rate of patients in the treatment group was lower than that in the control group: 5.08% (3/59) vs. 21.05% (12/57) ( P<0.05). Conclusions:Compared with traditional vertical incision, the modified transverse incision for microvascular decompression in the treatment of hemifacial spasm can reduce intraoperative blood loss and postoperative scar area, enhance brainstem auditory evoked potential, and improve facial aesthetics, which is worthy of recommendation.

Application of preoperative and intraoperative electroneurophysiological examination for persons with hemifacial spasm / 中华物理医学与康复杂志

Objective:To analyze the diagnostic value of preoperative electromyography and spasticity assessment for patients with hemifacial spasm, and to define a relationship between intraoperative electrophysiological examination and prognosis in order to provide help for clinical diagnosis and treatment.Methods:Thirty-one patients with hemifacial spasm were selected for the clinical spasticity scoring and divided into a general spasm group ( n=27) and a severe spasm group ( n=4). All received preoperative neurophysiological examination to record their twitch discharge, facial nerve conduction velocity (MCV), lateral spread (LSR) of the spasm, brainstem auditory evoked potential (BAEP), and blink reflex. Electrophysiological monitoring then recorded intraoperative LSR. According to whether the LSR disappeared or not, the patients were divided into the LSR disappearance group (of 15) and the LSR residual group (of 16), and facial muscle activity was recorded again one, three and six months after the operation. Results:Preoperative EMG examination of both groups showed positive LSR and that facial nerve MCV was within the normal range. There were, though, significant differences between the two groups in the twitching discharge by needle electromyography, blink reflex and preoperative BAEP. One week after the operation, one member of the residual group and 3 from the disappearance patients of the former and latter group had recovered in terms of LSR, with 3 and 7 cases significantly relieved, respectively. Two months later, the corresponding figures were 5 and 7, 3 and 6, respectively. Half of a year after the surgery, 5 from the residual group and 12 from the disappearance group had fully recovered in terms of LSR, while 9 and 2 cases were significantly relieved. Altogether, there were significant differences within the two groups in terms of recovery among all the time points, with significantly better recovery in the LSR disappearance group than the LSR residual group at 1 week after operation, while there were no significant differences between the two groups in recovery 3 and 6 months after their operation.Conclusions:Preoperative electromyography can provide objective assessments of the scope, severity, and facial nerve excitability of patients with hemifacial spasm. Real-time intraoperative electrophysiology monitoring can help surgeons to objectively assess the effect of decompression and to find and avoid nerve traction injury in surrounding areas quickly.

Mechanism of facial nerve nucleus excitability in patients with hemifacial spasm based on blink reflexes / 中华神经医学杂志

Objective:To further elucidate the pathogenesis of hemifacial spasm by analyzing blinking reflex characteristics.Methods:A total of 63 patients with hemifacial spasm (hemifacial spasm group) who underwent neuroelectrophysiological evaluation in Department of Neurological Electrophysiology, Guizhou Provincial People's Hospital from January 2021 to December 2022 were included as study subjects. Additionally, 58 patients with primary trigeminal neuralgia (trigeminal neuralgia group), 8 patients with post-facial paralysis associated exercise (post-facial paralysis associated exercise group), and 20 healthy volunteers (normal group) were selected as controls. Differences in facial nerve motor conduction velocity, complex muscle action potential latency, and blinking reflex characteristics including R1 latency, R1 amplitude, R2 initiation latency, R2 amplitude, R2 terminal latency, R2' initiation latency recorded on the affected side, R2' amplitude recorded on the affected side, and R2' terminal latency recorded on the affected side were collected and compared. Severity of hemifacial spasm was graded from grade 1 to grade 4 according to Samsung Medical Center scoring system; based on microvascular decompression findings regarding responsible blood vessels contacted with the facial nerve, patients were divided into one responsible blood vessel group and two or more responsible blood vessels group; trends or differences in incidences of increased/prolonged blink reflex indexes among all groups were analyzed.Results:No significant difference in facial nerve motor conduction velocity or complex muscle action potential latency was noted among the 4 groups ( P>0.05); the hemifacial spasm group had significantly higher R1 amplitude than the trigeminal neuralgia group and post-facial paralysis associated exercise group; additionally, the hemifacial spasm group had significantly higher R2 amplitude, R2' amplitude recorded on the affected side, R2 terminal latency, and R2' terminal latency recorded on the affected side compared with the other 3 groups ( P<0.05). Among patients with varying degrees of hemifacial spasm, increased incidences of increased R1 amplitude and prolonged R2 terminal latency were noted with increased spasm degrees, enjoying significant differences ( P<0.05). No significant differences in incidences of increased R1 amplitude, increased R2 amplitude, prolonged R2 terminal latency, increased R2' amplitude recorded on the affected side or prolonged R2' terminal latency recorded on the affected side were noted between one responsible blood vessel group and two or more responsible blood vessels group ( P>0.05). Conclusion:Increased R1 amplitude and prolonged R2 latency in patients with hemifacial spasm further substantiate the pathogenesis of hyperexcitability within facial nerve nucleus.

Correction of hemifacial microsomia using preformed titanium mesh combined with contralateral mandibular angle bone graft with CAD/CAM assistance / 口腔医学

@#Objective To explore the indications and clinical effects of simultaneous mandibular angleplasty on the unaffected side, performed using preformed titanium mesh combined with mandibular angle bone graft, for the treatment of hemifacial microsomia (HFM). Methods The study group included 11 patients between 2013 and 2021 with HFM who underwent simultaneous mandibular angle reduction of the unaffected side and augmentation of the affected side using resected bone scaffolds within the titanium mesh. A series of mandibular linear measurements such as ramus height(RH)and mandibular body length(BL), angular measurements, and asymmetry index combined with clinical photographs and postoperative complications were used to assess the therapeutic effect. Results Postoperatively, all patients were satisfied. Significant improvement in mandibular contour was shown, with no obvious complications. Postoperative data, such as BL and angular measurements, showed no statistically significant differences(P>0.05) between the affected and unaffected sides with improvement in symmetry, except for RH(P<0.05). The preoperative and postoperative asymmetry index were (17.11±4.56)% and (4.81±1.73)%, respectively, with significant improvement(P=0.002). Conclusion This approach provides satisfactory results and significantly improves facial asymmetries for unilateral HFM with hypertrophy of the contralateral mandibular angle.

Model test study on treatment of Pruzansky type ⅡB and Ⅲ hemifacial microsomia with artificial condyle-mandibular distractor complex / 中国修复重建外科杂志

OBJECTIVE@#To preliminarily verify the effectiveness of self-designed artificial condyle-mandibular distraction (AC-MD) complex in the treatment of Pruzansky type ⅡB and Ⅲ hemifacial microsomia (HFM) through model test.@*METHODS@#Five children with Pruzansky type ⅡB and Ⅲ HFM who were treated with mandibular distraction osteogenesis (MDO) between December 2016 and December 2021 were selected as the subjects. There were 3 boys and 2 girls wih an average age of 8.4 years (range, 6-10 years). Virtual surgery and model test of AC-MD complex were performed according to preoperative skull CT of children. The model was obtained by three-dimensional (3D) printing according to the children's CT data at a ratio of 1∶1. The occlusal guide plate was designed and 3D printed according to the children's toothpaste model. The results of the model test and the virtual surgery were matched in three dimensions to calculate the error of the residual condyle on the affected side, and the model test was matched with the actual skull CT after MDO to measure and compare the inclination rotation of the mandible, the distance between the condylar of the healthy side and the residual condyle of the affected side, and the lengthening length of the mandible.@*RESULTS@#The error of residual condyle was (1.07±0.78) mm. The inclination rotation of the mandible, the distance between the condylar of the healthy side and the residual condyle of the affected side, and the lengthening length of the mandible after 3D printing model test were significantly larger than those after MDO ( P<0.05).@*CONCLUSION@#In the model test, the implantation of AC-MD complex can immediately rotate the mandible to the horizontal position and improve facial symmetry, and the residual condyle segment can be guided close to the articular fossa or the preset pseudoarticular position of the skull base after operation.

Intrauterine diagnosis and follow-up of a child with Goldenhar Syndrome: case report / Diagnóstico intrauterino e seguimento de uma criança com Síndrome de Goldenhar: relato de caso

Abstract Introduction: goldenhar syndrome is a rare congenital syndrome that affects the craniofacial morphogenesis. It is a complex syndrome, with heterogeneous presentation which the diagnosis can still be performed in the intrauterine through morphological ultrasound. Description: a case report of a 4-year-old male patient diagnosed with Goldenhar syndrome, along with its clinical presentation, diagnostic investigation and follow-up. Discussion: the follow-up on these patients remains a challenge, since it can affect different systems and with different presentations. The earlier the diagnosis is performed, the greater the patient's chances of having a favorable prognosis with multidisciplinary stimulation. The objective of this article is to contribute to the medical literature, in order to assist in the diagnosis and management of future cases.

Resumo Introdução: a síndrome de Goldenhar é uma síndrome congênita rara que afeta a morfogênese craniofacial. Trata-se de uma síndrome complexa, de apresentação heterogênea, cujo diagnóstico pode ser realizado ainda intra-útero através do ultrassom morfológico. Descrição: relato de caso de um paciente do sexo masculino de quatro anos, com diagnóstico de síndrome de Goldenhar, sua apresentação clínica, a investigação diagnóstica e seguimento. Discussão: o acompanhamento desses pacientes continua sendo um desafio, já que pode acometer diversos sistemas e com apresentação diversa. O diagnóstico e a estimulação multiprofissional precoce, podem levar a maiores chances de um prognóstico favorável. O objetivo deste trabalho é contribuir para a literatura médica, de forma a auxiliar no diagnóstico e conduta perante futuros casos.

Surgical Outcome of Microvascular Decompression for Hemifacial Spasm: Symptom Control and Quality of Life / Resultado Cirúrgico da Descompressão Microvascular no Espasmo Hemifacial: Controle de Sintomas e Qualidade de Vida

Introduction Hemifacial spasm (HFS) is characterized by a segmental myoclonus of the face muscles innervated by the ipsilateral facial nerve. The accepted pathophysiology of HFS suggests that it is a disease process of the nerve root entry zone associated with any neuro-vascular conflict. Aim Review the surgical results and outcome regarding spasm control, post-operative quality of life and morbidity of microvascular decompression (MVD) for HFS from a Brazilian neurosurgical team. Method An observational investigation was conducted with data collection from patients with hemifacial spasm treated with MVD from January 2000 to December 2015 in two different centers in the West of São Paulo State, Brazil. Results A total of 152 patients underwent MVD for the treatment of HFS, ninety-eight (64.5%) female. Eighty-seven (57.2%) patients presented right-side spasms. The most common offending vessel was the posterior inferior cerebellar artery (PICA) with 78 (51.3%) patients. According to clinical presentation, an amount of 144 (94.7%) patients presented total control of symptoms after 36 months of follow-up. Regarding quality of life, a total of 125 (82.2%) patients referred normal quality of life after MVD for HFS and 121 (96.8%) from then were able to return to work or previous occupation. Permanent facial paresis / palsy was observed in 6 (3.6%) patients. There was no surgical mortality. Conclusion MVD for the treatment of HFS is a safe and efficacious surgical procedure to control spasm. Neurosurgeons experience, adequate patient selection and good anatomical knowledge are fundamental to success of the treatment.

Introdução O espasmo hemifacial (EHF) é caracterizado por mioclonia segmentar dos músculos da face inervados pelo nervo facial ipsilateral. A fisiopatologia aceita da EHF sugere que é um processo da doença da zona de entrada da raiz nervosa associada a conflito neuro-vascular. Objetivo Revisar os resultados e desfechos cirúrgicos em relação ao controle de espasmo, a qualidade de vida pós-operatória e a morbidade da descompressão microvascular (DMV) para EHF de uma equipe de neurocirurgia brasileira. Método Realizada investigação observacional com coleta de dados de pacientes com espasmo hemifacial tratados com DMV entre janeiro de 2000 a dezembro de 2015, em dois diferentes centros do Oeste do Estado de São Paulo, Brasil. Resultados Um total de 152 pacientes foram submetidos a DMV, noventa e oito (64,5%) do sexo feminino. Oitenta e sete (57,2%) pacientes apresentavam espasmos no lado direito. O conflito mais comum foi com a artéria cerebelar inferior posterior (PICA) em 78 (51,3%) pacientes. Um total de 144 (94,7%) pacientes apresentou controle total dos sintomas após 36 meses de acompanhamento. Em relação à qualidade de vida, 125 (82,2%) pacientes referiram qualidade de vida normal após a MVD para HFS e 121 (96,8%) puderam retornar ao trabalho/ocupação anterior. Paresia/paralisia facial permanentes foram observadas em 6 (3,6%) pacientes. Não houve mortalidade cirúrgica. Conclusão DMV para o tratamento da EHF é um procedimento cirúrgico seguro e eficaz para o controle do espasmo. A experiência dos neurocirurgiões, a seleção adequada dos pacientes e o bom conhecimento anatômico são fundamentais para o sucesso do tratamento.

Hemifacial Hypertrophy – Report of 2 Cases

Hemifacial hyperplasia (HH) is a rare congenital condition involving enlargement of one or more tissues of the face. The treatment is surgically challenging and requires expertise. This manuscript aims to report two similar appearing HH but warranting different surgical treatment. A 19?year?old female and a 14?year?old boy presented with right facial asymmetry since birth and sought correction of the same. Surgical treatment was planned. Based on clinical history, diagnosis and imaging, HH was diagnosed. The first case was entirely a soft tissue abnormality that was treated with debulking while the second case had involvement of facial bones, necessitating surgical recontouring. The facial asymmetry was addressed. Healing was uneventful. Though the aesthetical concern and appearance of the two cases of HH were same, the treatment vastly differed. This was based on the source of asymmetry. Proper diagnosis and informed decision are a key for successful surgical outcome.

Advances in diagnosis and treatment of primary hemifacial spasm / 中华全科医师杂志

Primary hemifacial spasm is a motor disorder of facial muscles related to facial nerve. During the attack, the facial muscles present irregular and involuntary clonus, which can be induced or aggravated by emotional excitement, mental tension and random facial movement, seriously affecting daily work and life. The pathogenesis, diagnosis, differential diagnosis and treatment of the primary hemifacial spasm have been studied extensively in recent years. This article reviews the progress in these aspects.

Evaluation value of abnormal muscle response monitoring in efficacy of microvascular decompression in primary hemifacial spasm / 中华神经医学杂志

Objective:To explore the evaluation value of abnormal muscle response (AMR) monitoring in efficacy of microvascular decompression in primary hemifacial spasm.Methods:A retrospective study was performed. Sixty-four patients with primary hemifacial spasm, admitted to our hospital from April 2019 to December 2020, were chosen. All patients underwent intraoperative AMR monitoring and were divided into AMR complete-disappeared group, AMR significant-changed group and AMR not significant-changed group according to the monitoring results. The relief of spasticity symptoms among the three groups was observed one week and one year after surgery. The specificity and sensitivity of AMR monitoring in evaluating the efficacy, the relations between intraoperative AMR changes and postoperative efficacy after microvascular decompression were analyzed.Results:The specificity and sensitivity of AMR monitoring in predicting spasmodic relief after microvascular decompression were 89.7% and 66.7%, respectively, at one week, and 86.7% and 50.0%, respectively, at one year. There were statistical differences in clinical remission rate one week after microvascular decompression among AMR complete-disappeared group ( n=46), AMR significant-changed group ( n=8) and AMR not significant-changed group ( n=10, P<0.05); there were no significant differences in clinical remission rate one year after microvascular decompression among the three groups ( P>0.05). There was no significant difference in clinical remission rate one week and one year after microvascular decompression between patients having AMR complete disappearance before Teflon and patients having AMR complete disappearance after Teflon ( P>0.05). Conclusion:AMR monitoring has high specificity but modest sensitivity in predicting spasmodic relief after microvascular decompression; intraoperative AMR disappearance is associated with short-term efficacy, but it is not a reliable indicator for long-term efficacy; the time of disappearance of intraoperative AMR has no guiding significance in judging the efficacy of patients with primary hemifacial spasm.

Espasmo hemifacial: experiencia en un centro terciario

Resumen El espasmo hemifacial consiste en un infrecuente trastorno del movimiento que afecta a la musculatura inervada por el nervio facial, en especial la de la hemicara superior. Existen dos formas clínicas, una primaria en la que la causa subyacente está representada por un conflicto neurovascular, y una secundaria, en la que la alteración del nervio facial es producida por algún tipo de lesión ocupante de espacio. Resulta de especial interés para el otorrinolaringólogo conocer esta entidad y ser capaz de diferenciar ambas formas clínicas dado el diferente enfoque terapéutico que pueden requerir. Aportamos una visión general de esta patología repasando su epidemiología y fisiopatología, además, de nuestra experiencia en forma de una serie de cuatro casos que ilustran las variadas formas de presentación de esta entidad, así como los signos y síntomas de alarma que pueden ayudar a realizar un correcto diagnóstico y manejo.

Abstract Hemifacial spasm consists of an unfrecuent movement disorder involving facial muscles, especially those from the upper half of the face. Two different clinical presentations are described. Primary hemifacial spasm is defined by a neurovascular conflict, and secondary occurs when facial nerve is damaged by a space occupying lesion. It is of special interest for the otorhinolaryngologist to get to know about this condition and to be able to acknowledge its clinical presentations due to the different therapeutical approach that may be needed for each of them. We contribute with a general vision of this entity reviewing its epidemiology and patophysiology. Furthermore, we show our experience by sharing a four-case series which we believe to illustrate the different ways of presentation as well as the alarm signs and symptoms that may be helpful in order to accomplish an accurate diagnose and treatment.

Clinical and imaging characteristics of 107 patients with linear morphea: a retrospective analysis / 中华皮肤科杂志

Objective:To analyze clinical, laboratory and imaging characteristics of different subtypes of linear morphea (LM) , and to propose an appropriate approach to the diagnosis and severity assessment of LM.Methods:Clinical data were collected from patients with clinically and/or pathologically confirmed LM in Department of Dermatology, West China Hospital, Sichuan University from January 2018 to December 2019, and retrospectively analyzed.Results:A total of 107 patients with LM were enrolled into this study, including 63 with LM of the limbs/trunk, 22 with morphea en coup de sabre, 11 with progressive hemifacial atrophy and 11 with eosinophilic fasciitis. Disease severity was evaluated by using the modified localized scleroderma skin severity index (mLoSSI) and localized scleroderma skin damage index (LoSDI) scores in 88 patients, with the mLoSSI scores ranging from 0 to 51 points, and the LoSDI scores ranging from 0 to 40 points. Routine blood examination of 10 patients with eosinophilic fasciitis showed increased eosinophil counts in 4 patients. Thirteen (14.8%) of 88 patients with LM were positive for antinuclear antibody, with titers of ≥ 1∶320. Magnetic resonance imaging (MRI) examination showed ipsilateral cerebral hemisphere atrophy and contralateral white matter hyperintensity on T2-weighted images in 2 out of 4 patients with progressive hemifacial atrophy, myofascial thickening in 26 out of 28 patients with LM of the limbs/trunk (92.9%) , subcutaneous septal and myofascial thickening in all 11 patients with eosinophilic fasciitis.Conclusions:The preliminary assessment of disease activity, severity and prognosis of LM can be made by mLoSSI and LoSDI. MRI examination is recommended for patients with clinical signs of involvement of subcutaneous structures.

Brainstem auditory evoked potentials combined with electrocochleogram in microvascular decompression / 中华神经医学杂志

Objective:To investigate the value of brainstem auditory evoked potential (BAEPs) combined with cochlear electrogram (ECochG) monitoring in the protection of auditory function during microvascular decompression (MVD) for patients with facial spasm (HFS).Methods:Clinical data of 908 patients with HFS who received MVD treatment in our hospital from January 2018 to December 2020 were retrospectively analyzed. The patients were divided into BAEPs group ( n=309), ECochG group ( n=301) and BAEPs+ECochG group ( n=298) according to the different methods of auditory nerve function monitoring. Waveform extraction rate, mean extraction time, amplitude, latency, intraoperative warning effect of 3 monitoring methods, as well as hearing status immediately after surgery and during follow-up were compared in patents from the 3 groups. Results:(1) The overall waveform extraction rate in ECochG group and BAEPs+ECochG group was significantly higher than that in BAEPs group, and the average waveform extraction time in ECochG group and BAEPs+ECochG group was significantly shorter than that in BAEPs group ( P<0.05). The amplitude of compound action potential (CAP) wave in ECochG group was significantly higher than that of V wave in BAEPs group, and the latency of CAP wave was also significantly earlier than that of V wave ( P< 0.05). (2) A total of 48 patients of the 288 patients in the BAEPs group showed warning signs; a total of 73 of the 292 patients in the ECochG group showed warning signs; and a total of 65 of the 292 patients in the BAEPs+ECochG group showed warning signs. (3) There was significant difference in hearing grading (American Association of Otolaryngology Head and Neck Surgery [AAO-HNS] grading) among the 3 groups immediately after surgery ( H=18.041, P=0.000), and the average rank suggested that the hearing of patients in the BAEPs+ECochG group was superior to the other two groups. All patients were followed up for an average of 15 months (ranged 3-24 months); there was still a significant difference in AAO-HNS grading among the 3 groups ( H=29.625, P=0.000), and the hearing of patients in the BAEPs+ECochG group was still superior to the other two groups. Conclusion:The combined application of ECochG and BAEPs monitoring can reflect the changes of intraoperative hearing impairment comprehensively, accurately and timely, which is of great significance for the protection of auditory function in HFS patients during MVD.

Excitability of facial nerve motor nucleus in patients with hemifacial spasm / 中华神经医学杂志

Objective:To study whether the excitability of facial nerve motor nucleus is increased in patients with facial spasm to further study the mechanism of facial spasm.Methods:Thirty patients with HFS underwent MVD in our hospital from April 2018 and March 2019 were chosen in our study. The amplitude and threshold voltage of facial nerve motor evoked potential (FNMEP) of the affected side and healthy side were monitored and compared by using monopulse stimulation and multi-pulse stimulation. The changes of FNMEP amplitude in patients before and after sevoflurane inhalation were monitored.Results:Stable facial motor evoked potential could be stimulated by monopulse in 26 patients (89.7%) on the affected side, while only 5 patients (17.2%) on the healthy side, showing statistical difference between the two sides ( P<0.05); there was no significant difference in FNMEP amplitude between the affected side and the healthy side ([207.2±62.1] μV vs. [180.2±55.0] μV, P>0.05); the average threshold voltage of neuromotor evoked potential on the affected side was (140.3±26.8) V, which was significantly lower than that in the healthy side ([177.0±23.2] V, P<0.05). The amplitude of FNMEP on the affected side was (207.2±62.1) μV, (133.0±36.5) μV, and (70.4±40.2) μV, respectively, before sevoflurane inhalation, and at 0.5 fold of minimun alveolar concentration (MAC) and 1.0 fold of MAC concentrations, with significant differences ( P<0.05). Conclusion:In patients with hemifacial spasm, there is increased excitability of facial nerve motor nucleus on the affected side, which to a certain extent supports the central theory of hemifacial spasm pathogenesis.

Risk factors for severe hearing impairment after microvascular decompression for treatment of hemifacial spasm / 中华神经医学杂志

Objective:To study the risk factors for ipsilateral severe hearing impairment in patients with hemifacial spasm (HFS) after microvascular decompression (MVD).Methods:MVD was performed in 3700 patients with HFS, admitted to our hospital from October 2007 to August 2020; according to the existence of ipsilateral severe hearing impairment, these patients were divided into severe hearing impairment group and non-severe hearing impairment group. The clinical data of these patients were compared. Multivariate linear regression analysis was used to determine the independent influencing factors for ipsilateral severe hearing impairment.Results:Forty-five patients (1.2%) had ipsilateral severe hearing impairment after MVD; no one got recovery of hearing impairment during the follow-up period (0.6-11.8 years, 6.3 years in average). As compared with those in the non-severe hearing impairment group, patients in the severe hearing impairment group had significantly older age, significantly higher percentages of male patients, and patients with left HFS, hypertension, and diabetes mellitus, statistically higher percentage of patients having small posterior fossa volume, arachnoid thickening and adhesion, and vertebral artery compression, significantly lower percentage of patients with anterior inferior cerebellar artery compression, significantly higher percentage of patients with arteriosclerosis of offending arteries and difficult decompression ( P<0.05). Multivariate linear regression analysis revealed that hypertension, vertebral artery compression, arteriosclerosis of offending artery and difficult decompression were independent risk factors for severe hearing impairment in patients with HFS after MVD. Conclusion:It's difficult to get recovery for severe hearing impairment in patients with HFS after MVD; this complication is much common in patients with hypertension, vertebral artery compression, arteriosclerosis of offending artery or difficult decompression.

Feasibility of using surface electromyography for the detection of abnormal muscle response in patients with hemifacial spasm / 西安交通大学学报(医学版)

【Objective】 To investigate the feasibility of using surface electromyography （SEMG） for the detection of abnormal muscle response （AMR） in patients with hemifacial spasm （HFS）. 【Methods】 We retrospectively reviewed the clinical data of HFS patients who underwent microvascular decompression （MVD） in our hospital between June 2019 and December 2020. Patients who received both surface electrode （preoperative） and needle electrode （intraoperative） detection of AMR were included. SEMG recorded from two stimulation-recording sites， namely， zygomatic-mentalis and mandibular marginal-orbicularis oculi， was selected for analyzing the characteristics of AMR. The positive rates of AMR detected by these two kinds of electrodes were comprehensively compared. 【Results】 Totally 77 patients were included in this study. When detected with surface electrodes， the positive rate， latency and amplitude of AMR recorded at zygomatic-mentalis oculi were 90.9% （70/77）， （10.87±1.86） ms and （202.8±47.4） μV， and at mandibular marginal-orbicularis oculi were 92.2% （71/77）， （10.41±1.83） ms and （211.1±54.1） μV， respectively. AMR was detected in 74 patients （96.1%） with surface electrodes. There was no significant difference in positive rate， latency and amplitude of AMR between these two stimulation-recording methods. When detected with needle electrodes， the positive rate of AMR recorded at zygomatic-mentalis oculi was 98.7% （76/77）， which was significantly higher than the rate 89.6% （69/77） recorded at mandibular marginal-orbicularis oculi （P=0.016）. The latency and amplitude of AMR recorded at zygomatic-mentalis were （10.63±1.39） ms and （83.5±27.2） μV， and at mandibular marginal-orbicularis oculi were （10.31±1.18） ms and （58.6±21.4） μV. There was no significant difference in latency between the two stimulation-recording methods， but the amplitude recorded at mandibular marginal-orbicularis oculi was significantly lower （P=0.041）. AMR was detected in 76 patients （98.7%） with needle electrodes. There was no significant difference in the detection rate of AMR between surface electrodes and needle electrodes （P=0.500）， the results were moderately consistent （Kappa=0.490， P<0.001）. 【Conclusion】 The detection efficiency of surface electrodes for AMR is similar to that of needle electrode. With its non-invasive characteristic， the surface electrode can be routinely used for electrophysiological evaluation of HFS.

Needle-embedding treatment for hemifacial spasm: research progress / 中国针灸

The articles regarding needle-embedding treatment for hemifacial spasm published before September 30, 2019 were searched from SinoMed, Wanfang, CNKI, VIP and PubMed database, and were analyzed and summarized from treatment methods, acupoint selection, stage differentiation and action mechanism. As a result, 45 Chinese articles were obtained. The needle-embedding treatment was divided into intradermal needling and acupoint thread-embedding; the top five acupoints were Sibai (ST 2), Taiyang (EX-HN 5), Dicang (ST 4), Jiache (ST 6) and spasm trigger points. The basic research of needle-embedding treatment for hemifacial spasm is weak, and the literature regarding stage differentiation is insufficient, which are in need of further study.