RESUMEN
BACKGROUND: Hemifacial spasm (HFS) is a debilitating disease characterized by involuntary tonic and clonic contractions of muscles innervated by the facial nerve. Botulinum toxin A (BTX-A) is the first-line option and the most effective medical treatment for HFS. The objective of this study was to evaluate the effect of BTX-A therapy on the physical and mental health of HFS patients. METHODS: Participants included 65 HFS patients and 65 matched healthy controls in the study. Cornell Medical Index (CMI) self-assessment questionnaire was used to detect the psychological health of all participants. Local injection of BTX-A was applied, and the Cohen hierarchical criteria were employed to stratify the degree of spasticity, further evaluating the efficacy of BTX-A before and two months after treatment in HFS patients. The HFS patients at two months post-treatment were re-evaluated by CMI self-assessment questionnaire, and the evaluated factors of these patients were compared with those of patients before treatment. RESULTS: The scores of somatization, depression, anxiety, inadaptation, sensitivity, anger, tension, M-R, and total scores in the HFS group were significantly higher than those in the control group (all P<0.05). Two months post-treatment, among 65 HFS patients who received with BTX-A treatment, 42 (64.6%) were completely relieved, 16 (24.6%) were significantly relieved, 7 (10.8%) were partially relieved, and 0 (0%) cases were invalid, and the total effective rate was 89.2%. Two months after BTX-A treatment, the scores of somatization, tension, anxiety, depression, sensitivity, M-R and total scores of patients with HFS were lower than those before treatment (all P<0.05). CONCLUSIONS: Patients with HFS are often accompanied by somatization, anger, inadaptation, sensitivity, anxiety, depression, and tension. BTX-A can not only alleviate the symptoms of HFS, but also improve the somatization, tension, anxiety, depression, and sensitivity.
Asunto(s)
Toxinas Botulínicas Tipo A , Espasmo Hemifacial , Fármacos Neuromusculares , Humanos , Espasmo Hemifacial/tratamiento farmacológico , Toxinas Botulínicas Tipo A/uso terapéutico , Femenino , Masculino , Persona de Mediana Edad , Fármacos Neuromusculares/uso terapéutico , Adulto , Salud Mental , Resultado del Tratamiento , Anciano , Encuestas y Cuestionarios , Ansiedad/tratamiento farmacológico , Depresión/tratamiento farmacológicoRESUMEN
The coincidence in a patient of Hemifacial Spasm and Trigeminal Neuralgia is not frequent. A case is presented with the objective of showing this association due to the abnormal activation of the Trigemino-Facial Reflex. A 55-year-old woman with an 8-year history of left-sided hemifacial spasm and typical trigeminal pain in the ipsilateral V1 and V2 territory. The physical examination shows spasms in the left hemiface, with reproduction of intense pain upon sensory stimulation of the skin on the forehead and upper dental arch. The MRI showed a vessel in intimate contact with the entrance area of ââthe left trigeminal nerve. A left retrosigmoid approach was performed. First, the entrance area of ââthe trigeminal nerve was accessed, finding a clear vascular conflict, which was isolated with Teflon. Then, the trajectory was changed and the exit zone of the facial nerve was accessed, and no type of vascular conflict was identified. The patient presented complete resolution of the Hemifacial Spasm and the associated trigeminal pain. The analysis of this case allows us to conclude that during microvascular decompression of the Facial Nerve, if frank proximal compression is not evident, the Trigeminofacial structural relationship must be taken into account, making it necessary to explore the Trigeminal Nerve.
RESUMEN
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.
RESUMEN
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.
RESUMEN
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.
RESUMEN
Objetivo Evaluar la presencia de epiteliopatía en limpiaparabrisas en pacientes con blefaroespasmo o espasmo hemifacial antes del tratamiento habitual con toxina botulínica y 4 semanas después. Métodos Estudio prospectivo compuesto por 31 ojos de 20 pacientes con diagnóstico neurológico de espasmo hemifacial (9 ojos de 9 pacientes) y blefaroespasmo esencial (22 ojos de 11 pacientes). Se evaluaron antes y 4 semanas después de la infiltración con toxina botulínica diversos parámetros de superficie ocular con el cuestionario OSDI, test de Schirmer, tiempo de rotura lagrimal y tinciones de fluoresceína y verde de lisamina valoradas con el test de Oxford y el grado de afectación del limpiaparabrisas palpebral. Resultados El 100% de los pacientes presentaron afectación del limpiaparabrisas palpebral antes (30% grado leve y 70% moderado) y después del tratamiento con toxina (100% grado leve). El 75% de los pacientes presentaron un OSDI normal-leve antes del tratamiento; después del tratamiento fue del 80%. El tiempo de rotura lagrimal fue de 7,2±0,2 s antes y de 7,5±0,7 s después del tratamiento. El test de Schirmer fue de 11,4±5,5 y 12,5±5,5mm antes y después del tratamiento. El test de Oxford resultó patológico inicialmente en el 69,3% de los pacientes; tras 4 semanas solo fue patológico en el 54%. Conclusión La epiteliopatía en limpiaparabrisas está presente en el 100% de los pacientes con blefaroespasmo o espasmo hemifacial. El principal mecanismo fisiopatológico que la desencadena en estos pacientes es el aumento en el coeficiente de fricción, ya que el volumen y la estabilidad lagrimal son normales (AU)
Objective To evaluate the presence of wiper epitheliopathy in patients with blepharospasm and/or hemifacial spasm before and 4 weeks after routine treatment with botulinum toxin. Methods Prospective study comprising 31 eyes of 20 patients with neurological diagnosis of hemifacial spasm (9 eyes of 9 patients) and essential blepharospasm (22 eyes of 11 patients). Various ocular surface parameters were assessed before and 4 weeks after infiltration with botulinum toxin using the OSDI questionnaire, Schirmer's test, tear break-up time, fluorescein and lissamine green staining assessed with the Oxford test and the degree of involvement of the palpebral wiper. Results 100% of the patients had palpebral wiper involvement before (30% mild and 70% moderate) and after toxin treatment (100% mild). 75% of patients had mild-normal OSDI before treatment, after treatment it was 80%. The tear break-up time was 7.2±0.2 sg before and 7.5±0.7 sg after treatment. Schirmer's test was 11.4±5.5 and 12.5±5.5mm before and after treatment. The Oxford test was initially pathological in 69.3% of patients, after 4 weeks it was pathological in only 54%. Conclusion Wiper epitheliopathy is present in 100% of patients with blepharospasm and/or hemifacial spasm. The main pathophysiological mechanism that triggers it in these patients is the increase in the coefficient of friction, as tear volume and stability are norma (AU)
Asunto(s)
Humanos , Masculino , Femenino , Persona de Mediana Edad , Blefaroespasmo/complicaciones , Blefaroespasmo/tratamiento farmacológico , Toxinas Botulínicas Tipo A/uso terapéutico , Espasmo Hemifacial/complicaciones , Espasmo Hemifacial/tratamiento farmacológico , Índice de Severidad de la Enfermedad , Estudios Longitudinales , Estudios ProspectivosRESUMEN
OBJECTIVE: To evaluate the presence of wiper epitheliopathy in patients with blepharospasm and/or hemifacial spasm before and 4 weeks after routine treatment with botulinum toxin. METHODS: Prospective study comprising 31 eyes of 20 patients with neurological diagnosis of hemifacial spasm (9 eyes of 9 patients) and essential blepharospasm (22 eyes of 11 patients). Various ocular surface parameters were assessed before and 4 weeks after infiltration with botulinum toxin using the OSDI questionnaire, Schirmer's test, tear break-up time (BUT), fluorescein and lissamine green staining assessed with the Oxford test and the degree of involvement of the palpebral wiper. RESULTS: 100% of the patients had palpebral wiper involvement before (30% mild and 70% moderate) and after toxin treatment (100% mild). 75% of patients had mild-normal OSDI before treatment, after treatment it was 80%. The BUT was 7.2⯱â¯0.2â¯sg before and 7.5⯱â¯0.7â¯sg after treatment. Schirmer's test was 11.4⯱â¯5.5 and 12.5⯱â¯5.5â¯mm before and after treatment. The Oxford test was initially pathological in 69.3% of patients, after 4 weeks it was pathological in only 54%. CONCLUSION: Wiper epitheliopathy is present in 100% of patients with blepharospasm and/or hemifacial spasm. The main pathophysiological mechanism that triggers it in these patients is the increase in the coefficient of friction, as tear volume and stability are normal.
Asunto(s)
Blefaroespasmo , Toxinas Botulínicas Tipo A , Espasmo Hemifacial , Blefaroespasmo/complicaciones , Blefaroespasmo/tratamiento farmacológico , Toxinas Botulínicas Tipo A/uso terapéutico , Párpados , Espasmo Hemifacial/complicaciones , Espasmo Hemifacial/tratamiento farmacológico , Humanos , Estudios ProspectivosRESUMEN
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.
RESUMEN
Resumen El espasmo hemifacial se caracteriza por contracciones tonicoclónicas en los músculos inervados por el nervio facial, causado por la compresión del nervio facial por una arteria, generalmente la arteria cerebelosa anteroinferior o venas del ángulo pontocerebeloso. Suelen aparecer espontáneamente, debido a la tensión emocional, fatiga, y disminuyen durante el reposo. Se presenta entre los 13 a 77 años con una duración de los síntomas aproximadamente de ocho años. La inyección local de toxina botulínica puede ser efectiva en el tratamiento, y la descompresión microvascular está reservada para casos refractarios al tratamiento médico, con resolución completa entre el 85 al 93%. En este artículo, se resumen las principales características anatomo-cliÌnicas, fisiopatológicas, y una descripción detallada de la descompresión microvascular como mejor opción terapéutica.
Abstract Hemifacial spasm is characterized by tonic-clonic contractions of the muscles innervated by the facial nerve. It is caused by compression of the facial nerve by a blood vessel, which usually is the anterior inferior cerebellar artery or the veins of the cerebello-pontine angle. It typically appears spontaneously, caused by emotional tension and fatigue, and it's reduced during rest. Likewise, it presents between the ages of 13 and 77 years, with a median duration of symptoms of eight years. Treatment with local botulinum toxin injection can be effective, and the Microvascular decompression is reserved for cases that are refractory to medical treatment, resulting in full spasm resolution in 85 to 93% of patients. This article summarizes the main anatomic-clinical and physio-pathological characteristics of hemifacial spasms. Additionally, a detailed description of microvascular decompression as the best therapeutic option is described in detail.
RESUMEN
RESUMEN Introducción: No se encuentran antecedentes de investigaciones que aborden resultados de la aplicación de toxina botulínica tipo A en pacientes con espasmo hemifacial en la provincia Guantánamo. Objetivo: Evaluar los resultados terapéuticos de la aplicación de toxina botulínica tipo A en pacientes con espasmo hemifacial atendidos en consulta de Neurología. Método: Se realizó un estudio descriptivo longitudinal de 21 pacientes atendidos en servicio de Neurología con espasmo hemifacial y tratamiento con toxina botulínica tipo A, desde enero 2018 hasta enero 2019. Las variables estudiadas fueron: edad, sexo, hemicara afectada, periodo de latencia, duración, desaparición del efecto clínico de toxina botulínica tipo A, y porciento de incapacidad funcional en pacientes al aplicar la escala de discapacidad antes y después de la aplicación del tratamiento. Resultados: Predominó el sexo femenino con 57,1%, edad mayor de 55 años con 57,14 %, lado afectado predominante el izquierdo con 57,14 %, el periodo de latencia de la toxina botulínica tipo A fue entre el tercer y décimo día con 14 pacientes para un 66,67 %, el tiempo máximo de duración del tratamiento fue de uno a dos meses con un 57,14 %, y la desaparición del efecto terapéutico de la toxina fue entre el tercer y cuarto mes con un 52,38%. Luego de la aplicación de la escala de evaluación clínica del espasmo hemifacial se evidenció disminución del porcentaje de pacientes con incapacidad funcional. Conclusiones: La toxina botulínica tipo A es efectiva en el tratamiento del espasmo hemifacial.
ABSTRACT Introduction: No research records were founded dressing the results of the application of botulinum toxin type A in patients with hemifacial spasm in Guantanamo province. Objective: to evaluate the therapeutic results of the application of botulinum toxin type A in patients with hemifacial spasm treated in a Neurology consultation. Method: a descriptive and longitudinal study was carried out in 21 patients attended in Neurology service with hemifacial spasm and botulinum toxin type A treatment, fromJanuary 2018 to January 2019. The variables studied were: age, gender, hemiface affected, latency period, duration, disappearance of the clinical effects of botulinum toxin type A, and percentage of functional disability in patients when applying the disability scale before and after the treatment. Results: Female cases predominated (57.1%), agesover 55 years (57.14 %), the most common affected side of the face was the left, with 57.14 %, the latency period of botulinum toxin type A was between the third to the tenth day in 14 of the patients (66.67 %), the maximum time of duration of treatment ranged from one to two months (57.14 %), and the disappearance of the therapeutic effect of the toxin in the patients was between the third to the fourth month (52.38%). After the application of the clinical assessment scale of hemifacial spasm, a decrease in the percentage of patients with functional disability was evidenced. Conclusions: Botulinum toxin type A is effective in the treatment of hemifacial spasm.
Asunto(s)
Humanos , Resultado del Tratamiento , Toxinas Botulínicas Tipo A/uso terapéutico , Espasmo Hemifacial/terapia , Epidemiología Descriptiva , Estudios LongitudinalesRESUMEN
Abstract Introduction: The use of surgical decompression of facial hemispasm due to the loop in the internal auditory canal is not always accepted due to the risk related to the surgical procedure. Currently a new surgical technique allows surgeons to work in safer conditions. Objective: To report the results with endoscope-assisted retrosigmoid approach for facial nerve microvascular decompression in hemifacial spasm due to neurovascular conflict. The surgical technique is described. Methods: We carried out a prospective study in a tertiary referral center observing 12 (5 male, 7 female) patients, mean age 57.5 years (range 49-71) affected by hemifacial spasm, that underwent to an endoscope assisted retrosigmoid approach for microvascular decompression. We evaluated intra-operative findings, postoperative HFS resolution and complication rates. Results: Hemifacial spasm resolution was noticed in 9/12 (75%) cases within 24 h after surgery and in 12/12 (100%) subjects within 45 days. A significant (p < 0.001) correlation between preoperative historical duration of hemifacial spasm and postoperative recovery timing was recorded. Only 1 patient had a complication (meningitis), which resolved after intravenous antibiotics with no sequelae. No cases of cerebrospinal fluid leak, facial palsy or hearing impairment were recorded. Hemifacial spasm recurrence was noticed in the only subject where the neurovascular conflict was due to a vein within the internal auditory canal. Conclusions: The endoscope assisted retrosigmoid approach technique offers an optimal visualization of the neurovascular conflict thorough a minimally invasive approach, thus allowing an accurate decompression of the facial nerve with low complication rates. Due to the less invasive nature, the procedure should be considered in functional surgery of the cerebellar pontine angle as hemifacial spasm treatment, specially when the procedure is performed by an otolaryngologist.
Resumo Introdução: O uso de descompressão cirúrgica do espasmo hemifacial devido ao loop no canal auditivo interno nem sempre é aceito devido ao risco relacionado ao procedimento cirúrgico. Atualmente, uma nova técnica cirúrgica permite trabalhar em condições seguras. Objetivo: Relatar os resultados que obtivemos com a abordagem retrosigmóidea assistida por endoscopia para a descompressão microvascular do nervo facial em casos de espasmo hemifacial devido a conflito neurovascular. A técnica cirúrgica é descrita. Método: Realizamos um estudo prospectivo em um centro de referência terciária observando 12 pacientes (5M, 7F), com média de idade de 57,5 (intervalo 49-71) anos com espasmo hemifacial submetidos a uma abordagem retrosigmóide assistida por endoscopia para descompressão microvascular. Foram avaliados os achados intraoperatórios, a resolução pós-operatória do espasmo hemifacial e as taxas de complicações. Resultados: A resolução do espasmo hemifacial foi observada em 9/12 (75%) dos casos nas 24 horas após a cirurgia e em 12/12 (100%) dos indivíduos até 45 dias. Uma correlação significativa (p < 0,001) entre a duração do histórico pré-operatório de espasmo hemifacial e o tempo de recuperação pós-operatório foi registrado. Apenas um paciente apresentou uma complicação (meningite), que foi resolvida após administração de antibióticos por via intravenosa sem sequelas. Nenhum caso fístula liquórica, paralisia facial ou deficiência auditiva foi registrado. A recorrência do espasmo hemifacial foi observada em único indivíduo em quem o conflito neurovascular foi causado por um vaso no interior do canal auditivo interno. Conclusões: A técnica da abordagem retrosigmóidea assistida por endoscopia oferece uma ótima visualização do conflito neurovascular através de uma abordagem minimamente invasiva, permite assim uma descompressão precisa do nervo facial com baixas taxas de complicações. Por ser menos invasivo, o procedimento deve ser considerado na cirurgia funcional do ângulo pontocerebelar como tratamento de espasmo hemifacial, especialmente quando o procedimento é feito por um otorrinolaringologista.
Asunto(s)
Humanos , Masculino , Femenino , Persona de Mediana Edad , Anciano , Espasmo Hemifacial/cirugía , Endoscopía/métodos , Cirugía para Descompresión Microvascular/métodos , Microcirugia/métodos , Estudios Prospectivos , Resultado del TratamientoRESUMEN
OBJECTIVE: To propose guidelines for the diagnosis and treatment of facial dystonia prepared by a group of experts in orbit and oculoplastics from the Iberoamerican Oculoplastic Society. MATERIAL AND METHODS: An interactive discussion between the expert panel and those attending the 6th Iberoamerican Society of Oculoplastics Congress, which took place at the Hospital Nuestra Señora de la Luz in Mexico City on 22 October 2018, providing their personal experience based on evidence for diagnosis and treatment of facial dystonia. Around 200 ophthalmologists specialised in oculoplastics from North, Central and South America, Spain, and Portugal were involved. Discussion was focused on the following themes: pathophysiology, diagnosis, medical management, and surgical management. CONCLUSIONS: Facial dystonia diagnosis is clinical; therefore, image studies are rarely needed. The ophthalmologist is generally the first physician to be consulted, and is able to be the treating physician, with the exception of specific cases of hemifacial spasm where management with neurosurgery may be beneficial. Botulinum toxin is the treatment of choice. Treatment with oral neuroleptics and myectomy of the orbicularis oculi muscle are reserved for refractory cases, since these do not have an adequate clinical response as first choice treatments. Persistent use of botulinum toxin does not modify the natural course of the disease.
Asunto(s)
Trastornos Distónicos/diagnóstico , Trastornos Distónicos/tratamiento farmacológico , Cara , Antipsicóticos/uso terapéutico , Blefaroespasmo/diagnóstico , Blefaroespasmo/tratamiento farmacológico , Toxinas Botulínicas/uso terapéutico , Técnicas de Diagnóstico Oftalmológico , Manejo de la Enfermedad , Trastornos Distónicos/fisiopatología , Trastornos Distónicos/cirugía , Espasmo Hemifacial/diagnóstico , Espasmo Hemifacial/tratamiento farmacológico , Espasmo Hemifacial/cirugía , Humanos , Cirugía para Descompresión Microvascular , Músculo Esquelético/cirugía , PronósticoRESUMEN
INTRODUCTION: The use of surgical decompression of facial hemispasm due to the loop in the internal auditory canal is not always accepted due to the risk related to the surgical procedure. Currently a new surgical technique allows surgeons to work in safer conditions. OBJECTIVE: To report the results with endoscope-assisted retrosigmoid approach for facial nerve microvascular decompression in hemifacial spasm due to neurovascular conflict. The surgical technique is described. METHODS: We carried out a prospective study in a tertiary referral center observing 12 (5 male, 7 female) patients, mean age 57.5 years (range 49-71) affected by hemifacial spasm, that underwent to an endoscope assisted retrosigmoid approach for microvascular decompression. We evaluated intra-operative findings, postoperative HFS resolution and complication rates. RESULTS: Hemifacial spasm resolution was noticed in 9/12 (75%) cases within 24h after surgery and in 12/12 (100%) subjects within 45 days. A significant (p<0.001) correlation between preoperative historical duration of hemifacial spasm and postoperative recovery timing was recorded. Only 1 patient had a complication (meningitis), which resolved after intravenous antibiotics with no sequelae. No cases of cerebrospinal fluid leak, facial palsy or hearing impairment were recorded. Hemifacial spasm recurrence was noticed in the only subject where the neurovascular conflict was due to a vein within the internal auditory canal. CONCLUSIONS: The endoscope assisted retrosigmoid approach technique offers an optimal visualization of the neurovascular conflict thorough a minimally invasive approach, thus allowing an accurate decompression of the facial nerve with low complication rates. Due to the less invasive nature, the procedure should be considered in functional surgery of the cerebellar pontine angle as hemifacial spasm treatment, specially when the procedure is performed by an otolaryngologist.
Asunto(s)
Endoscopía/métodos , Espasmo Hemifacial/cirugía , Microcirugia/métodos , Cirugía para Descompresión Microvascular/métodos , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Resultado del TratamientoRESUMEN
ABSTRACT Objective: To determine whether serum levels of anti-acetylcholine receptor antibody (anti-AChR-Abs) are related to clinical parameters of blepharospasm (BSP). Methods: Eighty-three adults with BSP, 60 outpatients with hemifacial spasm (HFS) and 58 controls were recruited. Personal history, demographic factors, response to botulinum toxin type A (BoNT-A) and other neurological conditions were recorded. Anti-AChR-Abs levels were quantified using an enzyme-linked immunosorbent assay. Results: The anti-AChR Abs levels were 0.237 ± 0.022 optical density units in the BSP group, which was significantly different from the HFS group (0.160 ± 0.064) and control group (0.126 ± 0.038). The anti-AChR Abs level was correlated with age and the duration of response to the BoNT-A injection. Conclusion: Patients with BSP had an elevated anti-AChR Abs titer, which suggests that dysimmunity plays a role in the onset of BSP. An increased anti-AChR Abs titer may be a predictor for poor response to BoNT-A in BSP.
RESUMO Objetivo: Determinar se os níveis séricos do anticorpo antirreceptor de acetilcolina (anti-AChR-Abs) estão relacionados aos parâmetros clínicos do blefaroespasmo (BSP). Métodos: Fora recrutados 83 adultos com BSP, 60 pacientes ambulatoriais com espasmo hemifacial (HFS) e 58 controles. Foi aplicado um questionário para registrar história pessoal, fatores demográficos, resposta à toxina botulínica tipo A (BoNT-A) e outras condições neurológicas. Os níveis de anti-AChR-Abs foram quantificados usando um ensaio imunoenzimático. Resultados: O nível de anti-AChR-Abs foi de 0,237 ± 0,022 unidades de densidade óptica (OD) no grupo BSP, significativamente diferente em comparação com o grupo HFS (0,160 ± 0,064) e o grupo controle (0,126 ± 0,038). O nível de anti-AChR-Abs se correlacionou com a idade e a duração da resposta à injeção de BoNT-A. Conclusão: Pacientes com BSP apresentaram títulos elevados de anti-AChR-Abs, o que sugere que a desimunidade desempenha um papel no surgimento de BSP. O aumento do título de anti-AChR-Abs pode ser um preditor de resposta insuficiente à BoNT-A em BSP.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Autoanticuerpos/sangre , Blefaroespasmo/sangre , Receptores Colinérgicos/inmunología , Espasmo Hemifacial/sangre , Valores de Referencia , Blefaroespasmo/fisiopatología , Blefaroespasmo/tratamiento farmacológico , Ensayo de Inmunoadsorción Enzimática , Estudios de Casos y Controles , Factores Sexuales , Análisis de Varianza , Factores de Edad , Toxinas Botulínicas Tipo A/uso terapéutico , Espasmo Hemifacial/fisiopatología , Espasmo Hemifacial/tratamiento farmacológico , Electromiografía , Fármacos Neuromusculares/uso terapéuticoRESUMEN
ABSTRACT Professor Charcot had several pupils in his famous neurological service at the Salpêtrière Hospital in Paris, France. Among them, Édouard Brissaud was one of Charcot's favorite pupils, temporarily becoming his successor after Charcot's death. Brissaud's neurological contributions were significant, including the description of hemifacial spasm, "geste antagoniste" in dystonia, pseudobulbar affect, post-traumatic stress disorder, the Brissaud-Sicard syndrome, and Brissaud's sign. Additionally, Brissaud was the first to suggest that Parkinson's disease pathology could be related to the substantia nigra.
RESUMO Professor Charcot teve vários discípulos em seu famoso serviço de neurologia no hospital Salpêtrière, em Paris, França. Dentre eles, Édouard Brissaud foi um dos pupilos favoritos de Charcot. Após a morte de Charcot, em 1893, Brissaud foi o seu sucessor temporário. Suas contribuições neurológicas foram muito expressivas, incluindo a descrição do espasmo hemifacial, do "geste antagoniste", da paralisia pseudobulbar, do transtorno de estresse pós-traumático, a síndrome de Brissaud-Sicard, e também do sinal de Brissaud. Além disso, Brissaud, sugeriu pela primeira vez no mundo, que a patologia da doença de Parkinson poderia estar relacionada à substância negra.
Asunto(s)
Historia del Siglo XIX , Historia del Siglo XX , Neurología/historia , FranciaRESUMEN
Resumen Objetivo: Evaluar los resultados terapéuticos de la aplicación de la toxina botulínica tipo A en pacientes con espasmo hemifacial atendidos en una consulta de Neurología de trastornos de movimiento en Cuba. Métodos: Se determinaron variables temporales: latencia, duración del efecto máximo y desaparición del efecto terapéutico de la toxina botulínica a 35 pacientes con espasmo hemifacial. Fueron evaluados el deterioro funcional, gravedad, frecuencia y efectos adversos. Resultados: Hubo predominio de mujeres (88,6%) con esta enfermedad. El tiempo de latencia más frecuente resultó ser 48 hs, la duración del efecto máximo hasta los 60 días y la desaparición del efecto terapéutico hasta 3 meses. El deterioro funcional mejoró entre la consulta inicial y los 15 meses de evolución, independientemente del tiempo de presentación de los síntomas y causa del espasmo hemifacial. También, la gravedad y frecuencia mejoraron. Las dosis de toxina botulinica aunmentó significativamente durante el seguimiento de los pacientes. Solo se observaron pacientes con debilidad muscular facial y ptosis parpebral. Conclusiones: Debido al favorable comportamiento de las variables temporales, la mejoría del deterioro funcional evolutivo, y los pocos efectos adversos observados se pudiera sugerir que las dosis de toxina botulínica en el tratamiento del espasmo hemifacial parecen ser eficaces y seguras.
Abstract Objective: To evaluate botulinum toxin A therapy in patients with hemifacial spasm attended in a Neurology Service in Cuba. Patients and methods: 35 patients with hemifacial spasm were treated with botulinum toxin A during 15 months. Some parameters of botulinum toxin were collected including doses, latency, maximum duration and total duration of improvement. Clinical data were determined, such as functional status, gravity, frequency and adverse events. Results: There was a high percentage of female with hemifacial spasm. Most patients showed a latency of 48 hs, maximum duration until 60 days and total duration of improvement of 3 months. Functional status decreased through time, independently of hemifacial spasm onset and its origin. Parameters related to functional status, as gravity and frequency also diminished significantly their categories. Botulinum toxin doses were statistically increased over this treatment, independently of any patient conditions. Only eight patients showed adverse events (facial muscle weakness and ptosis). Conclusions: Recovery of functional status, and low rate of adverse events observed during botulinum toxin treatment might suggest that this therapy seems to be effective and low-risk under our conditions.
RESUMEN
Objetivo: evaluar los resultados terapéuticos de la aplicación de toxina botulínica A en pacientes con alteraciones espásticas palpebrales. Métodos: se realizó un estudio observacional, descriptivo, longitudinal y prospectivo en 18 pacientes con estas afecciones, seleccionados en la consulta de Oculoplastia del Instituto Cubano de Oftalmología Ramón Pando Ferrer en el período comprendido entre enero y diciembre del año 2016. Se describió la muestra según la edad, el sexo, el color de la piel, los antecedentes patológicos personales, las afecciones palpebrales y las limitaciones en tareas de la vida diaria. Se evaluó en el posoperatorio el tiempo de desaparición de los síntomas, la duración del efecto de la inyección de toxina botulínica, las complicaciones inmediatas y las mediatas. Resultados: el 70,6 por ciento estuvo comprendido en el rango de edad mayor de 60 años, y el 52,9 por ciento fueron mujeres. La piel blanca constituyó el 58,8 por ciento de la muestra. La afección que se presentó con mayor frecuencia fue el blefaroespasmo (47,1 por ciento). Todos los pacientes presentaron limitaciones para realizar tareas de la vida diaria. El 100 por ciento presentó dolor en el sitio de la inyección. En el 94,1 por ciento de ellos hubo desaparición de los movimientos involuntarios entre el cuarto y el quinto día posterior a la aplicación de xeomeen y en el 88,2 por ciento demoró la reaparición de los síntomas entre cuatro y seis meses. Conclusiones: la toxina botulínica es efectiva en la corrección de las afecciones palpebrales espásticas con desaparición de los síntomas en un período de tiempo entre cuatro y cinco días, con una rápida incorporación del paciente a la vida laboral y social por cuatro a seis meses(AU)
Objective: to evaluate the therapeutic results of the application of toxin botulínica A in patients with palpebral spastic alterations. Methods: an observational, descriptive, longitudinal and prospective study was performed on 18 patients with these conditions, who were selected at the Oculoplasty Service of Ramón Pando Ferrer Cuban Institute of Ophthalmology in the period from January to December 2016. The analyzed variables were age, sex, race, personal pathological history, eyelid disorders and restricted daily life. In the postoperative period, length of time for symptom relief, duration of the effect of botulinum toxin injection, and immediate and mediate complications were assessed. Results: in the group, 70,6 percent were included in the over 60 y age group and 52,9 percent were women. Caucasians accounted for 58,8 percent of the sample. The most frequent condition was blepharospasm (47,1 percent). All patients had limitations to perform daily life tasks. In 94,1 percent of the patients, involuntary movements disappeared between the 4th and the 5th day after the administration of Xeomeen and in 88,2 percent, the symptoms delayed to appear again from 4 to 6 months. Conclusions: botulinum toxin A is an effective alternative to correct palpebral spastic conditions, with symptoms disappearing in four to five days, and quick reincorporation of the patient to working and social life for 4 to 6 months(AU)
Asunto(s)
Humanos , Femenino , Blefaroespasmo/terapia , Toxinas Botulínicas Tipo A/uso terapéutico , Espasmo Hemifacial/terapia , Glándulas Tarsales/lesiones , Epidemiología Descriptiva , Estudios Longitudinales , Estudio Observacional , Estudios ProspectivosRESUMEN
Objetivo: evaluar los resultados terapéuticos de la aplicación de toxina botulínica A en pacientes con alteraciones espásticas palpebrales. Métodos: se realizó un estudio observacional, descriptivo, longitudinal y prospectivo en 18 pacientes con estas afecciones, seleccionados en la consulta de Oculoplastia del Instituto Cubano de Oftalmología Ramón Pando Ferrer en el período comprendido entre enero y diciembre del año 2016. Se describió la muestra según la edad, el sexo, el color de la piel, los antecedentes patológicos personales, las afecciones palpebrales y las limitaciones en tareas de la vida diaria. Se evaluó en el posoperatorio el tiempo de desaparición de los síntomas, la duración del efecto de la inyección de toxina botulínica, las complicaciones inmediatas y las mediatas. Resultados: el 70,6 por ciento estuvo comprendido en el rango de edad mayor de 60 años, y el 52,9 por ciento fueron mujeres. La piel blanca constituyó el 58,8 por ciento de la muestra. La afección que se presentó con mayor frecuencia fue el blefaroespasmo (47,1 por ciento). Todos los pacientes presentaron limitaciones para realizar tareas de la vida diaria. El 100 por ciento presentó dolor en el sitio de la inyección. En el 94,1 por ciento de ellos hubo desaparición de los movimientos involuntarios entre el cuarto y el quinto día posterior a la aplicación de xeomeen y en el 88,2 por ciento demoró la reaparición de los síntomas entre cuatro y seis meses. Conclusiones: la toxina botulínica es efectiva en la corrección de las afecciones palpebrales espásticas con desaparición de los síntomas en un período de tiempo entre cuatro y cinco días, con una rápida incorporación del paciente a la vida laboral y social por cuatro a seis meses(AU)
Objective: to evaluate the therapeutic results of the application of toxin botulínica A in patients with palpebral spastic alterations. Methods: an observational, descriptive, longitudinal and prospective study was performed on 18 patients with these conditions, who were selected at the Oculoplasty Service of Ramón Pando Ferrer Cuban Institute of Ophthalmology in the period from January to December 2016. The analyzed variables were age, sex, race, personal pathological history, eyelid disorders and restricted daily life. In the postoperative period, length of time for symptom relief, duration of the effect of botulinum toxin injection, and immediate and mediate complications were assessed. Results: in the group, 70,6 percent were included in the over 60 y age group and 52,9 percent were women. Caucasians accounted for 58,8 percent of the sample. The most frequent condition was blepharospasm (47,1 percent). All patients had limitations to perform daily life tasks. In 94,1 percent of the patients, involuntary movements disappeared between the 4th and the 5th day after the administration of Xeomeen and in 88,2 percent, the symptoms delayed to appear again from 4 to 6 months. Conclusions: botulinum toxin A is an effective alternative to correct palpebral spastic conditions, with symptoms disappearing in four to five days, and quick reincorporation of the patient to working and social life for 4 to 6 months(AU)
Asunto(s)
Humanos , Femenino , Blefaroespasmo/terapia , Toxinas Botulínicas Tipo A/uso terapéutico , Espasmo Hemifacial/terapia , Glándulas Tarsales/lesiones , Epidemiología Descriptiva , Estudios Longitudinales , Estudio Observacional , Estudios ProspectivosRESUMEN
ABSTRACT Hemifacial spasm (HFS) is a common movement disorder characterized by involuntary tonic or clonic contractions of the muscles innervated by the facial nerve. Objective To evaluate the long-term effect of botulinum toxin type A (BTX-A) in the treatment of HFS. Methods A retrospective analysis of patients treated at the Movement Disorders Outpatient Clinic in the Neurology Service, Hospital de Clínicas, Federal University of Paraná, Curitiba, from 2009 to 2013 was carried out. A total of 550 BTX-A injections were administered to 100 HFS patients. Results Mean duration of improvement following each injection session was 3.1 months, mean latency to detection of improvement was 7.1 days and mean success rate was 94.7%. Patients were evaluated at an interval of 5.8 months after each application. Adverse effects, which were mostly minor, were observed in 37% of the patients at least once during follow-up. The most frequent was ptosis (35.1%). Conclusion Treatment of HFS with BTX-A was effective, sustainable and safe and had minimal, well-tolerated side effects.
RESUMO O espasmo hemifacial (EHF) é um distúrbio do movimento comum, caracterizado pela presença de contrações musculares tônicas ou clônicas dos músculos inervados pelo nervo facial. Objetivo Avaliar o efeito em longo prazo do uso da toxina botulínica tipo A (TXB) no tratamento do espasmo hemifacial (EHF). Métodos Foi realizada uma análise retrospectiva de pacientes do Ambulatório de Distúrbios do Movimento do departamento de Neurologia do Hospital das Clínicas da Universidade Federal do Paraná entre 2009 e 2013. Foram administradas 550 doses em 100 portadores de EHF. Resultados A duração média de efeito foi de 3,14 meses, com latência de 7,10 dias e a taxa de sucesso foi de 94,73%. Os pacientes foram reavaliados pelo médico em um intervalo de 5,76 meses após cada aplicação. Efeitos adversos transitórios, em sua maioria menores, foram observados em 37% dos pacientes ao menos uma vez durante o seguimento e o mais freqüente foi ptose palpebral (35,14%). Conclusão O tratamento do EHF com TXB-A mostrou-se eficaz, sustentado e seguro, com efeitos colaterais mínimos e bem tolerados.
Asunto(s)
Humanos , Masculino , Femenino , Persona de Mediana Edad , Toxinas Botulínicas Tipo A/uso terapéutico , Espasmo Hemifacial/tratamiento farmacológico , Fármacos Neuromusculares/uso terapéutico , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: The coexistence of hemifacial spasm and trigeminal neuralgia, a clinical entity known as painful tic convulsive, was first described in 1910. It is an uncommon condition that is worthy of interest in neurosurgical practice, because of its common pathophysiology mechanism: Neuro-vascular compression in most of the cases. OBJECTIVE: To present 2 cases of painful tic convulsive that received treatment at our institution, and to give a brief review of the existing literature related to this. The benefits of micro-surgical decompression and the most common medical therapy used (botulin toxin) are also presented. CLINICAL CASES: Two cases of typical painful tic convulsive are described, showing representative slices of magnetic resonance imaging corresponding to the aetiology of each case, as well as a description of the surgical technique employed in our institution. The immediate relief of symptomatology, and the clinical condition at one-year follow-up in each case is described. A brief review of the literature on this condition is presented. CONCLUSION: This very rare neurological entity represents less than 1% of rhizopathies and in a large proportion of cases it is caused by vascular compression, attributed to an aberrant dolichoectatic course of the vertebro-basilar complex. The standard modality of treatment is micro-vascular surgical decompression, which has shown greater effectiveness and control of symptoms in the long-term. However medical treatment, which includes percutaneous infiltration of botulinum toxin, has produced similar results at medium-term in the control of each individual clinical manifestation, but it must be considered as an alternative in the choice of treatment.