Tratamiento efectivo de la toxina botulínica tipo A en el espasmo hemifacial, Guantánamo 2018-2019 / Botulinum toxin type A efficient treatment in patients with hemifacial spasm, Guantanamo 2018-2019

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.

Hemifacial Spasm Associated with Chiari Type I Malformation: Surgical Considerations and Case Report

Hemifacial spasm (HS) is a movement disorder characterized by paroxysmal and irregular contractions of the muscles innervated by the facial nerve. Chiari malformation type I (CM I) is a congenital disease characterized by caudal migration of the cerebellar tonsils, and surgical decompression of foramen magnum structures has been used for treatment. The association of HS with CM I is rare, and its pathophysiology and therapeutics are speculative. There are only a few cases reported in the literature concerning this association. The decompression of the posterior fossa for the treatment of CM I has been reported to relieve the symptoms of HS, suggesting a relation between these diseases. However, the possible complications of posterior fossa surgery cannot be underrated. We report the case of a 66-year-old patient, in ambulatory follow-up due to right HS, no longer responding to botulinum toxin treatment. Magnetic resonance imaging (MRI) of the skull revealed compression of the facial nerve and CM I. The patient underwent surgery for HS by neurovascular microdecompression of the facial nerve via right lateral suboccipital craniectomy, but presented significant clinical worsening in the postoperative period even though the cerebellum edema related to surgical manipulation was mild. Due to the clinical worsening, the patient underwent a median suboccipital craniectomy with decompression of the foramenmagnum structures. After this second surgery, the patient had progressive improvement and was discharged from the hospital for ambulatory care.

Asistencia Endoscópica en la Descompresión Microquirúrgica del Hemiespasmo Facial / Endoscopic Assistance in Microsurgical Decompression of Facial Hemiespasm

Introducción: El hemiespasmo facial primario (HFP) se produce por la hiperexcitabilidad del nervio facial y sus núcleos de origen como consecuencia de la compresión vascular. La cirugía de descompresión neurovascular se plantea como alternativa al tratamiento médico refractario. Objetivos: Presentar nuestra experiencia respecto a esta patología. Material y métodos: Se realizó una revisión retrospectiva de pacientes operados por HPF refractarios a tratamiento médico en nuestra institución en los últimos 5 años (periodo 2014-2019). Todos fueron intervenidos vía retrosigmoidea. Se evaluaron datos demográficos, evaluación prequirúrgica y evolución postoperatoria. Resultados: Se operaron 4 pacientes bajo técnica microquirúrgica asistido por endoscopía. Edad promedio 52 años (rango 41-61) con una relación femenino masculino 3:1. El 25% (n=1) presentaba paresia facial grado 2 (HB) en el prequirúrgico. No hubo cambios en cuanto al grado de paresia facial en el postoperatorio en ningún caso. Sólo un paciente registró caída leve en la audiometría postquirúrgica. El 75% (n=3) resolvieron el HFP. Conclusión: Si bien nuestra serie es acotada a un número reducido de pacientes, la cirugía descompresiva microvascular es efectiva como alternativa al tratamiento médico refractario del HFP.

Introduction: Primary hemifacial spasm (PHS) is defined as the hyper excitability of the nerve due to the compression of the facial nerve or its nuclei, most commonly by a vascular structure. The surgical indication for microvascular decompression is resistance to pharmacological treatment or severe adverse effects. Objectives: To present our experience in the surgical treatment of this pathology with this novel technique. Materials and methods: We retrospectively reviewed all patients (n=4) with a diagnosis of PHS, who underwent endoscope-assisted microvascular decompression surgery in our institution during the last 5 years. In all the cases, we choose the retrosigmoid approach. We evaluated demographic data, preoperative evaluation, and post-operative evolution. Results: Endoscope-assisted microvascular decompression was the surgical technique in all the patients. The median age was 52 years, the female-male ratio of 3:1. Only one patient presented a grade 2 facial palsy (House-Brackmann scale) in the preoperative evaluation; there were no significant changes in the post-operative evaluation in any patient. Only one patient experienced worsening in the post-operative audiometric follow-up. The 75% (n=3) of the patients solved the HFP after the surgical treatment. Conclusion: By taking into account our experience in this small case series, we can support the concept that endoscope-assisted microvascular decompression is as effective as the open surgical treatment of the PHS.

Endoscope-assisted retrosigmoid approach in hemifacial spasm: our experience / Abordagem retrosigmóidea assistida por endoscopia em espasmo hemifacial: nossa experiência

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.

Microvascular Compressicaude by Slinging a Tortuous Vertebrobasilar Artery to the Petrous Dura in Hemifacial Spasm: Technical Note

Objective In cases of hemifacial spasm caused by a tortuous vertebrobasilar artery (TVBA), the traditional treatment technique involves Teflon (polytetrafluoroethylene), which can be ineffective and fraught with recurrence and neurological complications. In such cases, there are various techniques of arteriopexy using adhesive compositions, 'suspending loops' made of synthetic materials, dural or fascial flaps, surgical sutures passed around or through the vascular adventitia, as well as fenestrated aneurysmal clips. In the present paper, we describe a new technique of slinging the vertebral artery (VA) to the petrous dura for microvascular decompression (MVD) in a patient with hemifacial spasm caused by a TVBA. Method A 50-year-old taxi driver presented with a left-sided severe hemifacial spasm. A magnetic resonance imaging (MRI) scan of the brain showed a large tortuous left-sided vertebral artery impinging and compressing the exit/entry zone of the 7th and 8th nerve complex. After a craniotomy, a TVBAwas found impinging and compressing the entry zone of the 7th and 8th nerve complex. Arachnoid bands attaching the artery to the nerve complex and the pons were released by sharp microdissection. Through the upper part of the incision, a 2.5 1 cmtemporal fascia free flap was harvested. After the fixation of the free flap, a 6­0 prolene suture was passed through its length several times using the traditional Bengali sewing and stitching techniques to make embroidered quilts called Nakshi katha. The'prolenated' fascia was passed around the compressing portion of the VA. Both ends of the fascia were brought together and stitched to the posterior petrous dura to keep the TVBA away from the 7th and 8th nerves and the pons. Result The patient had no hemifacial spasm immediately after the recovery from the anesthesia. A postoperative MRI of the brain showed that the VA was away from the entry zone of the 7th and 8th nerves.

Blepharospasm with elevated anti-acetylcholine receptor antibody titer / Blefaroespasmo com título elevado de anticorpos antirreceptores de acetilcolina

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.

Uso de la toxina botulínica en pacientes con alteraciones espásticas palpebrales / Use of botulinum toxin in patients with palpebral spastic alterations

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)

Treatment of hemifacial spasm with botulinum toxin type a: effective, long lasting and well tolerated / Tratamento do espasmo hemi-facial com toxina botulínica tipo a foi efetivo, bem tolerado e com efeito sustentado

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.

Aplicaciones de la toxina botulínica en afecciones palpebrales / Uses of botulinum toxin in eyelid diseases

La toxina botulínica es un potente inhibidor neuromuscular altamente específico que produce una denervación química al bloquear la liberación de acetilcolina en la placa motora. Es sintetizada por Clostridium botulinum. Es un fármaco de alto valor terapéutico en las alteraciones de los anexos oculares; puede indicarse con muy buenos resultados en afecciones como el blefarospasmo primario, el espasmo hemifacial, el chalazión, el entropion espasmódico, la retracción palpebral, la ptosis de la ceja, la mioquimia palpebral y en el tratamiento de líneas de expresión facial, con muy buenos resultados estéticos. Se realizó una búsqueda bibliográfica y en Internet utilizando distintas bases de datos como Medline, Google, Bireme, PubMed.gov, así como artículos relevantes de la Academia americana de Oftalmología y Neurología con el objetivo de describir las aplicaciones de la neurotóxica botulínica en las afecciones de los anexos oculares(AU)

Botulinum toxin is a highly specific potent neuromuscular inhibitor that produces a chemical denervation when blocking the release of acetylcholine in the motor plaque. It is synthesized by clostridium botulinum. It is a drug with high therapeutic value to treat ocular adnexas and may be indicated for primary blepharospasm, hemifacial spasm, chalazion, spasmodic entropion, palpebral retraction, eye brow ptosis, palpebral myokymia and in treating expression lines of the face, all with very good esthetic results. Literature search was made in Internet by using databases such as Medline, Google, Bireme, PubMed.gov, as well as outstanding articles of the American Academy of Ophthalmology and Neurology with the objective of describing the uses of botulinum toxin for ocular adnexa diseases(AU)

Uso de toxina botulínica en espasmo hemifacial: a propósito de un caso / Hemifacial spasm and botulinum toxin: about a case

El espasmo Hemifacial (EHF) es un trastorno crónico del movimiento caracterizado por la contracción descontrolada, intermitente y espasmódica o tónica de los músculos de una de las hemicaras. La aplicación de toxina botulínica a través de la quimiodenervación produce parálisis muscular temporal y de forma reversible. Se presenta un caso clínico relacionado con EHF y la aplicación terapéutica de toxina botulínica tipo "A" con buenos resultados.

Hemifacial (EHF) spasm is a chronic movement disorder characterized by uncontrolled, intermittent and spasmodic contraction of the muscles or tone of one of the hemicaras. The application of botulinum toxin through chemodenervation produces temporary muscular paralysis and reversibly. a clinical case involving EHF and the therapeutic application of botulinum toxin type "A" with good results is presented.

Descompresión microvascular en espasmo hemifacial: reporte de 13 casos y revisión de la literatura / Microvascular decompression in hemifacial spasm: report of 13 cases and review of the literature

Objetivo: el propósito del presente trabajo es presentar los resultados de 13 pacientes con diagnóstico de espasmo hemifacial (EHF), en los cuales se realizó una descompresión microvascular (DMV). Material y Método: Desde Junio de 2005 a Mayo de 2014, 13 pacientes con diagnóstico de EHF fueron intervenidos quirúrgicamente, realizando una DMV. Se evaluó: edad, sexo, tiempo de evolución de la sintomatología, hallazgos intraoperatorios y resultados postoperatorios. Resultados: De los 13 pacientes intervenidos, 7 fueron mujeres y 6 varones. La media de edad fue de 53 años. El tiempo medio entre el inicio de la sintomatología y la intervención quirúrgica osciló entre 3 y 9 años. En todos los casos el EHF era típico, uno de ellos con neuralgia trigeminal concomitante, observándose en todos compresión neurovascular intraoperatoria. Por orden decreciente de frecuencia la causa de la compresión fue arteria cerebelosa anteroinferior, arteria cerebelosa posteroinferior, arteria dolicomega basilar y arteria dolicomega vertebral. El seguimiento postoperatorio fue en promedio de 24 meses. El 62% presentó desaparición postquirúrgica inmediata de la sintomatología preoperatoria, el 30% desaparición tras un período de 3 semanas a 2 meses (8% con mejoría parcial), y en el 8% no hubo mejoría. En cuanto a las complicaciones postoperatorias: 3 pacientes presentaron paresia facial II-III en la escala de House-Brackman (se recuperaron en un período de 6 meses), y 1 paciente presentó fístula de líquido cefalorraquídeo. Ninguno de los pacientes de la serie presentaron hipoacusia transitorio o permanente. Conclusión: La DMV como tratamiento del EHF es un procedimiento efectivo y seguro, que permite la resolución completa de la patología en la mayoría de los casos.

Objective: the aim of this study is to describe the results of 13 patients with facial hemispasm, treated with microvascular decompression.Method: Between June 2005 and May 2014, 13 patients with facial hemispasm were operated, underwent microvascular decompression. The age, sex, duration of symptoms before surgery, and surgical finds, were all evaluated. In addition, postoperative results were also analyzed. Results: 7 patients were women and 6 were men. The average age of the patients was 53 years. The average time between onset of symptoms and surgery ranged from 3 to 9 years. In all cases the facial hemispasm was typical, one with concomitant trigeminal neuralgia, observed in all neurovascular compression intraoperative. In decreasing order of frequency, the cause of compression was anterior inferior cerebellar artery, posterior inferior cerebellar artery, dolicomega basilar artery and dolicomega vertebral artery. The average time of postoperative follow-up after the surgery was 24 months. Complete relief from spasm occurred in 62%; 30% disappearance after 3 weeks-2 months (8% partial) and in 8% had no improvement. Regarding postoperative complications: 3 patients had facial paresis II-III in House-Brackman scale and 1 patient presented CSF leak. None of the patients in the serie had hearing loss or deafness. Conclusion: The microvascular decompression for facial hemispasm is a safe an effective procedure, which allows complete resolution of the disease in most cases.

Protocolo de aplicação bilateral de toxina botulínica tipo A para evitar assimetria no tratamento de espasmo hemifacial / Protocol for bilateral application of botulinum toxin type A to avoid asymmetry during treatment of hemifacial spasms

Introdução: O espasmo hemifacial (EHF) caracteriza-se por movimento tônico-clônico involuntário da musculatura de uma hemiface. O tratamento tem sido realizado com aplicação de toxina botulínica (TxB). A aplicação unilateral classicamente descrita resulta em assimetria semelhante à paralisia facial. O objetivo desse trabalho foi normatizar o tratamento do EHF bilateralmente com TxB, a fim de prevenir a ocorrência de assimetria facial iatrogênica. Método: Foram analisadas 66 aplicações em 15 pacientes, seguindo o protocolo do serviço para paralisia facial, acrescentado de pontos pré-tarsais no lado com EHF. Foi feita dose complementar na reavaliação após 15 dias nos pacientes que apresentavam algum grau residual de espasmo ou assimetria, buscando-se a dose necessária para alcançar controle satisfatório do espasmo sem causar assimetria facial. Resultados: A dose média total foi 20,2 U do lado não acometido e 28,4 U do lado acometido, totalizando 48,6 U por aplicação. Houve diferença significante entre as hemifaces na dose para os músculos zigomático, orbicular da boca e orbicular dos olhos. Conclusões: A técnica proposta de aplicação bilateral de TxB controlou adequadamente o EHF e evitou assimetria iatrogênica. Como regra geral, a aplicação deve ser feita na proporção de 1:1,5 U no orbicular dos olhos (porção lateral) e 1:2 U no orbicular da boca. Nos demais músculos, a dose nos dois lados deve ser a mesma, realizando-se dose de reforço em 15 dias caso permaneça algum grau de espasmo. O único local com pontos exclusivos do lado acometido é a região pré-tarsal do músculo orbicular do olho.

Introduction: Hemifacial spasm (HFS) is characterized by the involuntary tonic-clonic movement of the muscles of the hemiface. It is usually treated with botulinum toxin (BTX). The classically described unilateral application of BTX results in an asymmetry similar to facial paralysis. The aim of this study was to standardize the treatment of HFS by applying BTX bilaterally to prevent the occurrence of iatrogenic facial asymmetry. Methods: The outcomes of 66 applications in 15 patients were analyzed according to the protocol of the facial paralysis service, to which pretarsal sites were added on the HFS side. On reassessment 15 days later, a complementary dose was administered to patients who exhibited some residual degree of spasm or asymmetry with the aim of determining the dose required to achieve satisfactory spasm control without causing facial asymmetry. Results: The total mean dose was 20.2 U at the contralateral side and 28.4 U at the spasm side (a total dose of 48.6 U per application). There was a significant difference between the doses applied to the zygomaticus, orbicularis oris, and orbicular oculi muscles on each hemiface . Conclusions: The proposed bilateral BTX application technique was effective in controlling HFS and prevented iatrogenic asymmetry. In general, application should be performed at a ratio of 1:1.5 U in the orbicularis oculi (lateral portion) and 1:2 U in the orbicularis oris. In the remaining muscles, the same dose should be administered on both sides and an additional dose can be applied 15 days later if some degree of spasm is present. The pretarsal region of the orbicularis oculi muscle is the only area for which BTX application on the healthy side is unnecessary.

Misdiagnosis of hemifacial spasm is a frequent event in the primary care setting / Erro diagnostico do espasmo hemifacial e ocorrencia frequente em unidades primarias de atencao a saude

Primary hemifacial spasm (HFS) is characterized by irregular and involuntary contraction of the muscles innervated by the ipsilateral facial nerve. Treatment controls symptoms and improves quality of life (QoL). Objective : Evaluate the initial diagnosis and treatment of HFS prior to referral to a tertiary center. Method : We interviewed through a standard questionnaire 66 patients currently followed in our center. Results : Mean age: 64.19±11.6 years, mean age of symptoms onset: 51.9±12.5 years, male/female ratio of 1:3. None of the patients had a correct diagnosis in their primary care evaluation. Medication was prescribed to 56.8%. Mean time from symptom onset to botulinum toxin treatment: 4.34 ±7.1 years, with a 95% satisfaction. Thirty percent presented social embarrassment due to HFS. Conclusion : Despite its relatively straightforward diagnosis, all patients had an incorrect diagnosis and treatment on their first evaluation. HFS brings social impairment and the delay in adequate treatment negatively impacts QoL. .

Espasmo hemifacial primário é caracterizado pela contração irregular ou involuntária dos músculos inervados pelo nervo facial ipsilateral. O tratamento é eficaz para controlar sintomas e melhorar a qualidade de vida. Objetivo : Avaliar diagnóstico e tratamento do espasmo hemifacial primário feitos antes do encaminhamento ao centro terciário. Método : Foram coletados retrospectivamente dados de 66 pacientes atualmente acompanhados no nosso serviço através de entrevista padronizada. Resultados : Média de idade: 64,19±11,6 anos; média de idade no início dos sintomas: 51,9±12,5 anos; razão homem/mulher de 1:3. Nenhum dos pacientes foi corretamente diagnosticado na primeira avaliação. Foram prescritos medicamentos para 56,8%. O tempo médio entre início dos sintomas e o tratamento com toxina botulínica foi 4,34±7,1 anos; 95% ficaram satisfeitos com o tratamento; 30% tinham constrangimento social. Conclusão : Embora seja uma condição de relativa facilidade diagnóstica, todos os pacientes tiveram diagnóstico e tratamento incorretos na primeira avaliação. Espasmo hemifacial primário traz constrangimento social, agravado pelo atraso no tratamento adequado. .

Descompresion neurovascular en espasmo del nervio facial. Fisiopatologia y diagnostico / Neurovascular decompression in hemifacial spasm. Physiopathology and diagnosis

El espasmo hemifacial es una enfermedad rara, con una incidencia de menos de 1 en 100.000 habitantes en los Estados Unidos;y esta caracterizada por contracciones involuntarias de los músculos en una lado de la cara. Es un síndrome de apariciónespontánea y progresiva con contracciones intermitentes de los músculos que se ocupan de la expresión facial. El espasmohemifacial tiene un efecto cosmético y psicológico importante en el paciente, y en los casos mas avanzados la visión puede estarafectada. En nuestra experiencia el espasmo hemifacial primario es una enfermedad que comienza generalmente entre los 41y 60 años con un promedio de 50 años. Sin embargo en las diferentes series de la literatura la edad de aparición puede variarentre 18 y 77 años. La mayoría de los pacientes son del sexo femenino con una incidencia de 2.4 mujeres sobre hombres. El ladomas afectado de la cara es el lado izquierdo; la razón por la cual existe esta asimetría de movimientos involuntarios no estamuy clara. Se ha sugerido que la predominancia del lado izquierdo puede ser el reflejo de una predisposición embriológica enrelación a la posición del nervio facial y el vaso compresivo en la zona de entrada del nervio en el lado izquierdo. El espasmohemifacial bilateral es muy raro.

Avaliação da fenda palpebral após aplicação de toxina botulínica tipo A em pacientes com distonias faciais / Evaluation of palpebral fissure after botulinum toxin type A injection in patients with facial dystonias

OBJETIVO: Avaliar a medida da fenda palpebral em pacientes com blefaroespasmo essencial benigno (BEB) e espasmo hemifacial (EHF) após a aplicação periocular de toxina botulínica tipo A. MÉTODOS: Foram estudados pacientes portadores de BEB e EHF submetidos à injeção periocular de toxina botulínica tipo A pela técnica inner orbital de aplicação. Os pacientes foram fotografados em PPO antes da aplicação e catorze dias depois dela. A fenda palpebral foi mensurada nestas imagens por meio de processamento computadorizado de imagens, utilizando o programa ImageJ. As alterações da fenda palpebral foram observadas comparando-se as medidas obtidas no pré e pós-aplicação. RESULTADOS: Comparando-se as imagens obtidas com o programa ImageJ, houve aumento estatisticamente significante (p<0,001) da fenda palpebral em 14 olhos (51,8%) após a aplicação de injeção periocular da toxina botulínica e nenhuma das imagens analisadas apresentou diminuição da fenda palpebral. CONCLUSÃO: No presente estudo, os pacientes portadores de distonias faciais apresentaram aumento de fenda palpebral estatisticamente significante após aplicação periocular de toxina botulínica tipo A.

PURPOSE: To evaluate the measurement of palpebral fissure in patients with facial dystonias before and after periocular injection with botulinum toxin type A. METHODS: We studied patients with benign essential blepharospasm and hemifacial spasm underwent periocular injection of botulinum toxin type A by the inner orbital technique of application. Patients were photographed 14 days before and after application. The palpebral fissure was measured in these images by means of computerized image processing using the program ImageJ. The palpebral fissure changes were observed by comparing the measurements obtained before and after application. RESULTS: Comparing the images obtained with the program ImageJ, there was a statistically significant increase (p <0.001) of the palpebral fissure in 14 eyes (51,8%) after the application of periocular injection of botulinum toxin and the images analyzed showed no decrease of the palpebral fissure. CONCLUSION: In this study, patients with facial dystonias showed increased palpebral fissure periocular statistically significant after application of botulinum toxin type A.

Resultados tardios da descompressão microvascular do nervo facial pela artéria vertebral / Long-term outcomes of facial nerve microvascular decompression by vertebral artery

Espasmos faciais constituem afecção rara, caracterizada por contrações assimétricas, involuntárias e paroxísticas da musculatura facial. Frequentemente são de acometimento unilateral e levam a situação desconfortável e prejudicial à qualidade de vida dos pacientes. Na maioria das vezes, o quadro inicia-se gradualmente no músculo orbicular dos olhos, seguindo-se por acometimento do andar inferior da face ipsilateralmente. Os espasmos são causados por compressão vascular, mais comumente provocada pela presença da artéria cerebelosa póstero-inferior (PICA) ou da artéria cerebelosa antero-inferior (AICA) (sendo mais raro o envolvimento da artéria vertebral) sobre a raiz do nervo facial, na REZ (Root Entry Zone). Injeção de toxina botulínica no músculo facial envolvido é uma opção terapêutica medicamentosa, no entanto possui benefícios transitórios ao longo da terapia. A descompressão microvascular é um tratamento efetivo, com baixas taxas de complicação. Descreve-se o caso de um paciente de 65 anos portador de contrações involuntárias e esporádicas da musculatura orbicular do olho esquerdo de caráter insidioso e progressivo. Optou-se por microcirurgia descompressiva com interposição de teflon entre artéria vertebral esquerda e o nervo facial na fossa posterior, com significativa melhora clínica, e regressão progressiva dos espasmos até sua completa remissão.

Facial spasm is a rare disease characterized by asymmetriccontractions and involuntary paroxysmal activity offacial muscles. It is often unilateral disease and leadsto an uncomfortable and injurious situation that affectspatients’ quality of life. Most often, it begins gradually inthe orbicularis oculi muscle, followed by involvement of theipsilateral inferior face . The spasms are usually causedby PICA and AICA vascular compression (vertebral arterycompression is more rare) of the facial nerve root, in itsentry zone. Botulinum toxin injection in the involved facialmuscle is a treatment option, however has transient benefitsduring the therapy. Microvascular decompression is aneffective treatment, with low complication rates. We reporta case of a 65-year-old patient with sporadic involuntarycontractions of left orbicular muscle , with an insidious andprogressive character. Microsurgical decompression withTeflon interposition between the left vertebral artery and thefacial nerve in the posterior fossa was performed. There wasa significant clinical improvement, with progressive decreaseof the spasms until complete remission.

Toxina botulínica tipo a: Experiencia con 2.000 aplicaciones / Botulinum toxin type a: An experience with 2.000 injections

Los múltiples usos terapéuticos de la toxina botulínica tipo A son una herramienta importante para el abordaje de algunos trastornos del movimiento. Damos a conocer la experiencia, resultados y conclusiones obtenidas en el servicio de neurología del Hospital de San José de Bogotá DC, al completar más de 2.000 aplicaciones en distonías, espasticidad y cefalea. En espasmo hemifacial la mejoría varió entre 60 y 80%, en blefaroespasmo 70 y 90%, en distonía cervical con patrones básicos 70-80% y en complejas (dos o más patrones de movimiento) de 50 a 60%. En espasticidad la mejoría se evidenció por aumento en los rangos de movimientos medidos con goniómetro antes y después de la aplicación, siendo variable de acuerdo con el tiempo de evolución, severidad y patrón de la espasticidad, haciendo seguimiento con la escala de sistema de clasificación de la función motora global (GMFCS). En cefalea crónica diaria hubo remisión total del dolor en 60%, disminución marcada en 30% y sin mejoría el 10%. Conclusión: La administración de toxina botulínica es segura y debe ser un proceso dinámico ajustado a cada paciente, el efecto terapéutico se evidencia a partir del segundo día de la aplicación con una expresión máxima a los 14 días, la duración del efecto oscila entre 2 y 6 meses con una media de 2.8. Los efectos adversos desaparecen en los siguientes treinta días de la aplicación...

Botulinum toxin type A is used extensively for the management of motility disorders constituting an important therapeutic tool. This report shows the experience, results and conclusions obtained at the neurology service of Hospital de San José de Bogotá DC, through 2.000 botulinum toxin injections to treat dystonia, spasticity and migraine. Hemifacial spasm improved between 60 and 80%, blepharospasm between 70 and 90%, cervical dystonia between 70 and 80%, and complex dystonia (deterioration of two or more movement patterns) from 50 to 60%. Improvement in spasticity was evidenced by increased range of motion measured with a goniometer before and after the injection. Spasticity improvement varied according to the rate of progression, severity and spasticity pattern. Follow-up was conducted using the gross motor function classification system (GMFCS). Total remission of pain was achieved in 60 % of patients with chronic daily headaches, was significantly reduced in 30% and did not improve in 10%. Conclusions: botulinum toxin injection appears to be safe and must be a dynamic process adjusted to each patient needs. Therapeutic effects are evidenced on the second day after injection and maximum expression is seen on day 14. Duration of effect ranges from 2 to 6 months with a media of 2.8. Adverse effects fade about 30 days after injection...

Aberrações ópticas de alta ordem em pacientes com distonias faciais tratados com toxina botulínica / Ocular wavefront aberrations in patients with facial dystonia treated with botulinum toxin

OBJETIVO: Avaliar as aberrações ópticas de alta ordem em pacientes com distonias faciais tratados com toxina botulínica tipo A. MÉTODOS: Pacientes com diagnóstico clínico de espasmo hemifacial ou blefaroespasmo essencial em atividade foram submetidos ao exame biomicroscópico e à análise de frente de ondas através do aberrômetro Alcon LADARvision®, sob midríase medicamentosa. A seguir, foram tratados com injeções de toxina botulínica tipo A. Após um mês, a análise de frente de ondas foi repetida da mesma forma e pelo mesmo oftalmologista. As aberrações de alta ordem foram comparadas antes e após o tratamento. O teste T pareado foi utilizado para comparar os valores numéricos antes e após o tratamento. RESULTADOS: Foram incluídos no estudo um total de 11 pacientes, 6 com blefaroespasmo essencial (54,5 por cento) e 5 com espasmo hemifacial (45,5 por cento). Nos pacientes com espasmo hemifacial foram analisados apenas o lado acometido, totalizando 17 olhos com espasmo. A idade variou de 50 a 72 anos, com média de 65,9 ± 8,2 anos. Oito pacientes eram do sexo feminino (72,7 por cento), sendo a relação masculino/feminino de 1:2,6. A média do "root mean square" (RMS) das aberrações de alta ordem foi 0,68 antes e 0,63 após um mês do tratamento (p=0,01). A média da aberração esférica foi de 0,23 e 0,17 antes e após o tratamento respectivamente (p=0,01). Não houve diferenças estatisticamente significantes nos demais tipos de aberrações de alta ordem após o tratamento (p>0,05). CONCLUSÃO: O tratamento com toxina botulínica A pode diminuir as aberrações esféricas em pacientes com distonias faciais.

PURPOSE: To analyze the ocular wavefront aberrations in patients with facial dystonia treated with botulinum toxin A. METHODS: Patients with benign essential blepharospasm and hemifacial spasm in activity underwent slit lamp examination and bilateral wavefront analysis under pharmacologic mydriasis using Alcon LADARvision® wavefront aberrometry system. After that, all patients were treated with botulinum toxin A injections performed by the same ophthalmologist. After one month, the wavefront analysis was performed in the same way and by the same examiner. The main outcome measure was the change in ocular wavefront aberrations. Paired T-test was used to compare pre and post-injection numeric wavefront values. RESULTS: From a total of 11 patients enrolled in this study, 6 (54.5 percent) had essential blepharospasm and 5 (45.5 percent) had hemifacial spasm. The fellow eyes of patients with hemifacial spasm were not included, totalizing 17 eyes with spasm. Eight patients were female (72.7 percent) and three were male (27.3 percent), the male:female ratio was 1:2.6. The age ranged from 50 to 72 years old with a mean of 65.9 ± 8.2 years. The mean of high order root mean square (RMS) wavefront aberrations was 0.68 before and 0.63 one month after the treatment (p=0.01). Before the treatment, the mean of spherical aberration was 0.23 and decreased to 0.17 one month after the treatment (p=0.01). There was no significant difference in the other higher-order aberrations before and after the treatment (p>0.05). CONCLUSION: The treatment with botulium toxin may decrease spherical aberrations in patients with facial dystonia.

Análise da ceratometria corneana em pacientes portadores de espasmo hemifacial / Analysis of corneal keratometry in patients with hemifacial spasm

OBJETIVO: Determinar a variação da ceratometria em olhos de pacientes portadores de espasmo hemifacial submetidos a tratamento com toxina botulínica. MÉTODOS: Foram incluídos 18 pacientes portadores de espasmo hemifacial que foram submetidos ao exame oftalmológico completo, além da topografia corneana e Orbscan previamente à aplicação de toxina botulínica tipo A e 1 mês após o procedimento. RESULTADOS: Não houve diferença estatisticamente significante entre a ceratometria encontrada pela topografia corneana ou diferença no BFS ("best fit sphere") anterior e posterior e índice de Roush avaliados pelo Orbscan nos olhos acometidos pelo espasmo em relação ao olho contralateral quando comparados antes e após o procedimento. CONCLUSÃO: Neste estudo, não encontramos diferença entre olhos com espasmo hemifacial e olhos sem essa condição quando avaliados a ceratometria, o índice de Roush e o BFS corneano antes e após tratamento.

PURPOSE: To determine the corneal keratometric variation in patients' eyes with unilateral hemifacial spasm which underwent treatment with botulinum toxin and set against control group. METHODS: Eighteen patients with hemifacial spasm were submitted to complete ophthalmologic exam, corneal topography and Orbscan previously botulinum toxin application and one month afterwards. They were evaluated for keratometry, BFS (anterior and posterior) and Roush value. RESULTS: There were no statistical differences between eyes with hemi facial spasm and normal eyes related to keratometric values by topography and in the parameters evaluated by Orbscan as Roush and best fit sphere (anterior or posterior) prior to or after treatment with botulinum toxin. CONCLUSION: This study has not shown statistical difference between the corneal curvatures when compared eyes with hemi facial spasm with the opposite normal eyes.

Ritidoplastia em face espástica: relato de caso / Rhytidoplasty in patient with hemifacial spasm: case report

O espasmo hemifacial (EH) é um distúrbio do movimento caracterizado por contrações involuntárias dos músculos inervados pelo nervo facial, a descompressão microvascular(DMV) é considerada tratamento definitivo, com baixas taxas de recidiva. Os casos refratários tornam-se um desafio para o cirurgião plástico. Relato do Caso: MSP, sexo feminino, 53 anos, portadora de EH, refratária à DMV, e com queixas de flacidez dos dois terços inferiores da face. Foi submetida à ritidoplastia, neurectomias e miectomias seletivas. Obteve-se ausência completa dos espasmos, manutenção da mímica facial e rejuvenescimento facial. A ritidoplastia cérvico-facial associada a neurectomias e miectomias mostrou-se eficaz no tratamento do EH.

The peripheral facial palsy is a disease that can leave significant aesthetical and functional sequelae in patients. According to medical literature, Bell’s palsy (BP) is the most common cause, representing from 50 to 80% of the cases. However, it must be considered as an exclusion diagnosis. A slightly higher prevalence is estimated in women and its incidence is bimodal with peaks in the third and eighth life decades. Most patients recover within weeks, though a worsening degree may be common in the first 48 hours. Treatment must be performed to prevent complications and the medical procedures are not universally accepted. The following have been mentioned as BP risk factors: arterial hypertension, mellitus diabetes, pregnancy, puerperium and infection caused by type I herpesvirus. Although there are several BP associated risk factors, regarding face-lifting surgery there is only one reported case in medical literature. Case Report: In the present study, the authors describe the second case of Bell’s palsy after face-lifting surgery.