Translation, adaptation, and cross-cultural validation of the CDQ-24 scale in patients with primary blepharospasm under botulinum toxin treatment, pilot test.

BACKGROUND: Benign essential blepharospasm (BEB) is the most common adult-onset focal facial dystonia and its treatment of choice is periodic application of botulinum toxin (BtA). It has a higher incidence in middle and late adulthood, especially in women between 40 and 60 years of age. OBJECTIVE: To carry out the translation and cross-cultural adaptation of the CDQ24 questionnaire in its Spanish version in patients diagnosed with BEB who have been treated with BtA in an ophthalmologic center in Bogotá - Colombia. MATERIALS AND METHODS: Pilot test of validation study and adaptation of a scale assembled in a prospective cohort of the CDQ24 instrument to Spanish in adult patients with primary blepharospasm treated with botulinum toxin in Bogota, Colombia. RESULTS: We obtained a sample of 26 patients to whom the instrument was applied after translation and retranslation of the original document, composed of 19 (73%) women with a median age of 64.5 years; the average time to answer the survey was 4.93 min. The internal consistency of the scale evaluated by Cronbach's Alpha had a total score of 0.78. Criterion validity between the CDQ24 scale and the WHOQOL-BREF quality of life scale was determined by determining correlation between the Emotional Well-Being and Phsychological domains of both scales. CONCLUSIONS: The translation and cross-cultural adaptation of the CDQ-24 scale into Spanish allowed the applicability of the instrument to the Spanish-speaking population during the pilot test, which allows us to continue the relevant studies in the study population.

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

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

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

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

ABSTRACT Purpose: Information is scarce regarding the comprehensive profile of patients with essential blepharospasm and hemifacial spasm in Brazil. The present study aimed to assess the clinical features of patients with these conditions, followed up in two reference centers in Brazil. Methods: The study included patients with essential blepharospasm and hemifacial spasm, followed up at the Departments of Ophthalmology at Universidade Federal de São Paulo and Universidade de São Paulo. Apart from demographic and clinical features, past stressful events related to the first symptoms (triggering event), aggravating factors, sensory tricks, and other ameliorating factors for the eyelid spasms were assessed. Results: A total of 102 patients were included in this study. Most patients were female (67.7%). Essential blepharospasm was the most frequent movement disorder [51/102 patients (50%)], followed by hemifacial spasm (45%) and Meige's syndrome (5%). In 63.5% of the patients, the onset of the disorder was associated with a past stressful event. Ameliorating factors were reported by 76.5% of patients; 47% of patients reported sensory tricks. In addition, 87% of the patients reported the presence of an aggravating factor for the spasms; stress (51%) was the most frequent. Conclusion: Our study provides information regarding the clinical features of patients treated in the two largest ophthalmology reference centers in Brazil.

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

Blepharospasm Patients after Botulinum Toxin - Sleep Approach.

Background Blepharospasm is a focal dystonia that affects the orbicularis oculi muscles. The interest in nonmotor symptoms is due to their impact on quality of life. Objective We evaluated the frequency of sleep disorders and circadian rhythm in a sample of Brazilian blepharospasm patients. Methods A total of 51 patients, who met the clinical criteria for blepharospasm, evaluated by 2 specialists in movement disorders, were recruited from the outpatient clinic for movement disorders of two reference centers in the city of São Paulo: Universidade Federal de São Paulo and Hospital do Servidor Público do Estado de São Paulo. The selected 13 patients were evaluated from 13 days before to 13 days after using botulinum toxin. They were interviewed, underwent physical examination and actigraphy, and completed sleep diaries. Results After using botulinum toxin, the group that reported sleep improvement exhibited a 50% decrease in sleep latency. There was no change in restless leg syndrome or circadian rhythm. Patients who reported no sleep improvement after using botulinum toxin presented poorer synchronization of the light-dark cycle. Conclusion Blepharospasm patients have poor sleep quality. About 50% of the patients had sleep improvement after using botulinum toxin. The synchronization of the light-dark cycle should be influenced by this finding.

Smartphone and custom-made software to assess the effect of botulinum toxin in essential blepharospasm: Initial results.

BACKGROUND AND PURPOSE: It is challenging to assess essential blepharospasm (EB) patients objectively because they exhibit chaotic patterns of abnormal eyelid movements. Previously employed objective approaches used systems with low levels of accuracy systems or were too complex to be applied in routine clinical practice. We aimed to develop a practical tool using a smartphone camera and custom-made software to objectively assess the therapeutic effects of botulinum toxin in blepharospasm patients. METHODS: Thirty-four patients with EB were evaluated before and 15 days after receiving onabotulinumtoxinA injections. The control group was composed of 19 age-matched healthy individuals. A smartphone (iPhone 6 S; Apple) was used to record spontaneous eyelid movements for 3 min, after which eyelid movement frequency was analysed using custom-made software. RESULTS: Before treatment, eyelid movement frequency was significantly higher in the EB group (21.55 ± 13.30 movements/min) compared to the control group (8.26 ± 8.89 eyelid movements/min; p < 0.001). The frequency of spontaneous eyelid movements was significantly reduced after treatment in the EB patients (8.46 ± 6.32 eyelid movements/min; p < 0.001). After treatment, no statistically significant difference in eyelid movement rate was observed between the EB patients and the control group (p = 0.32). CONCLUSIONS: Assessment of the spontaneous eyelid movements obtained with the smartphone camera and analysed with the custom-made software enabled us to objectively measure the therapeutic effects of botulinum toxin in patients with blepharospasm. Further refinement of this system could enable customized and fine adjustments to botulinum toxin doses based on each patient's needs.

Apraxia of Eyelid Opening and Blepharospasm in Two Spinocerebellar Ataxia Type 3 Patients.

BACKGROUND: Neuroophthalmological phenotypical particularities of SCA3. PHENOMENOLOGY: Eyelid opening apraxia and asymmetrical blepharospasm. EDUCATIONAL VALUE: To illustrate the phenomenology for purposes of education.

Real-World Dosing of OnabotulinumtoxinA and IncobotulinumtoxinA for Cervical Dystonia and Blepharospasm: Results from TRUDOSE and TRUDOSE II.

The real-world use of onabotulinumtoxinA and incobotulinumtoxinA for cervical dystonia and blepharospasm treatment was assessed in two separate retrospective studies using identical protocols (TRUDOSE and TRUDOSE II). The studies were conducted in Mexico, Norway, and United Kingdom and designed to evaluate dose utilization of the two botulinum toxins in clinical practice. Eighty-three patients treated with both onabotulinumtoxinA and incobotulinumtoxinA for ≥2 years for each botulinum toxin were included, (52, cervical dystonia; 31, blepharospasm). All patients switched from onabotulinumtoxinA to incobotulinumtoxinA for administrative/financial reasons. A range of dose ratios (incobotulinumtoxinA to onabotulinumtoxinA) was reported; with the majority of dose ratios being >1. The mean dose ratio was >1 regardless of the study site or underlying clinical condition. The inter-injection interval was significantly longer for onabotulinumtoxinA versus incobotulinumtoxinA when assessed for all patients (15.5 vs. 14.3 weeks; p = 0.006), resulting in fewer onabotulinumtoxinA treatments over the study time period. Consistent with product labeling, no single fixed-dose ratio exists between incobotulinumtoxinA and onabotulinumtoxinA. The dosage of each should be individualized based on patient needs and used as per product labeling. These real-world utilization data may have pharmacoeconomic implications.

Ophthalmological and neurological manifestations of a dog with intracranial anaplastic ependymoma. Case report / Manifestações oftalmológicas e neurológicas em um cão com ependimoma anaplásico intracranial: relato de caso

The objective is to report ependymoma associated with uveitis in a four-month-old male boxer crossbreed. The dog initially presented in both eyes conjunctival hyperemia, blepharospasm, projection of the nictitating membrane, and photophobia, characteristic of uveitis.Traumatic uveitis was rejected. Six days after onset of ophthalmic symptoms, vomiting, incoordination, and behavior changes occurred. Neurological symptoms and eyebrow contraction suggesting head pain and brain injury were investigated by computed tomography (CT) nine days after, when the dog showed spasms of the limbs and neck followed by respiratory arrest with reversal and stabilization. The scans revealed extensive amorphic neoformation in the diencephalon, midbrain, and within the right lateral ventricle, along with sinistral displacement of the cerebral sickle. The animal was euthanized, and necropsy of the head revealed hydrocephalus and an intracerebral tumor mass consistent with the CT imaging. Histopathological evaluation by hematoxylin and eosin staining revealed tissue alterations in several CNS segments, showing several pseudorosettes in the neuropil, mitosis, and a high degree of cell atypia, indicating ependymoma. Inflammatory, hemorrhagic, and necrotic tissue lesions were observed in the brainstem and cerebellum due to compression by tumor tissue and hydrocephalus. The neoplasia was phenotyped by Immunohistochemistry (IHC), and tested positive for the tumoral markers vimentin and glial fibrillary acid protein, confirming intracranial anaplastic ependymoma. Behavior changes and neurological signs resulted from vascular, inflammatory, and degenerative processes in the neuropil caused by neoplasm compression and invasion of brain tissue. Although dogs with ependymoma often present with neurological disease, in the present case, blepharospasm was the first symptom noticed by the owner, and it persisted until euthanasia. According to the literature, and confirmed in the evolution of the current case, the symptoms are related to tumor location and extent and to secondary lesions due to tumor expansion. Clinical symptomatology and complementary laboratory testing, CT, necropsy, histology, and IHC characterized ependymoma, a rare condition in young dogs.

Objetiva-se relatar um caso de ependimoma anaplásico associado a uveíte em cão macho de quatro meses de idade, mestiço Boxer. O cão apresentou, inicialmente, hiperemia conjuntival, blefaroespasmo, projeção da membrana nictitante e fotofobia bilaterais, sinais característicos de uveíte. Uveíte por causa traumática foi descartada. Seis dias após o início dos sintomas oftálmicos ocorreram vômito, incoordenação motora e alterações de comportamento. Sinais neurológicos e contração das sobrancelhas sugeriram algia na região cefálica. Injúria cerebral foi investigada por tomografia computadorizada (TC) nove dias após o início dos sintomas, quando o cão demonstrou espasmos dos membros e pescoço, seguido por parada respiratória com reversão e estabilização. A TC revelou extensa e amórfica neoformação no diencéfalo, mesencéfalo, e dentro do ventrículo lateral direito, com deslocamento à esquerda da foice cerebral. O animal foi eutanasiado e a necropsia revelou hidrocefalia e uma massa tumoral intracerebral condizente com a imagem da TC. A avaliação histopatológica por coloração hematoxilina e eosina, revelou alterações teciduais em várias áreas do Sistema Nervoso Central (SNC), mostrando várias pseudorosetas no neurópilo, mitose, e um elevado grau de atipia celular, indicando ependimoma.Lesões teciduais inflamatórias, hemorrágicas e necróticas foram observadas no tronco encefálico e cerebelo, devido à compressão do tumor e à hidrocefalia. Amostras da neoplasia foram submetidas à análise imuno-histoquímica (IHQ), sendo positiva a expressão dos marcadores tumorais vimentina e proteína glial fibrilar ácida, confirmando ependimoma anaplásico intracranial. As mudanças comportamentais e sinais neurológicos resultaram do processo vascular, inflamatório e degenerativo no neurópilo, causados pela compressão e invasão do tecido cerebral pela neoplasia. Embora cães com ependimoma frequentemente apresentem manifestações neurológicas, no presente caso, blefaroespasmo foi o primeiro sinal clínico observado pelo proprietário, e persistiu até a eutanásia do paciente. Os sinais neurológicos estão relacionados à localização do tumor e extensão das lesões secundárias devidas à sua expansão. Sintomatologia clínica e testes laboratoriais complementares, TC, necropsia, histologia e IHQ caracterizaram ependimoma, que é raro em animais jovens.

Ophthalmological and neurological manifestations of a dog with intracranial anaplastic ependymoma. Case report / Manifestações oftalmológicas e neurológicas em um cão com ependimoma anaplásico intracranial: relato de caso

The objective is to report ependymoma associated with uveitis in a four-month-old male boxer crossbreed. The dog initially presented in both eyes conjunctival hyperemia, blepharospasm, projection of the nictitating membrane, and photophobia, characteristic of uveitis.Traumatic uveitis was rejected. Six days after onset of ophthalmic symptoms, vomiting, incoordination, and behavior changes occurred. Neurological symptoms and eyebrow contraction suggesting head pain and brain injury were investigated by computed tomography (CT) nine days after, when the dog showed spasms of the limbs and neck followed by respiratory arrest with reversal and stabilization. The scans revealed extensive amorphic neoformation in the diencephalon, midbrain, and within the right lateral ventricle, along with sinistral displacement of the cerebral sickle. The animal was euthanized, and necropsy of the head revealed hydrocephalus and an intracerebral tumor mass consistent with the CT imaging. Histopathological evaluation by hematoxylin and eosin staining revealed tissue alterations in several CNS segments, showing several pseudorosettes in the neuropil, mitosis, and a high degree of cell atypia, indicating ependymoma. Inflammatory, hemorrhagic, and necrotic tissue lesions were observed in the brainstem and cerebellum due to compression by tumor tissue and hydrocephalus. The neoplasia was phenotyped by Immunohistochemistry (IHC), and tested positive for the tumoral markers vimentin and glial fibrillary acid protein, confirming intracranial anaplastic ependymoma. Behavior changes and neurological signs resulted from vascular, inflammatory, and degenerative processes in the neuropil caused by neoplasm compression and invasion of brain tissue. Although dogs with ependymoma often present with neurological disease, in the present case, blepharospasm was the first symptom noticed by the owner, and it persisted until euthanasia. According to the literature, and confirmed in the evolution of the current case, the symptoms are related to tumor location and extent and to secondary lesions due to tumor expansion. Clinical symptomatology and complementary laboratory testing, CT, necropsy, histology, and IHC characterized ependymoma, a rare condition in young dogs.(AU)

Objetiva-se relatar um caso de ependimoma anaplásico associado a uveíte em cão macho de quatro meses de idade, mestiço Boxer. O cão apresentou, inicialmente, hiperemia conjuntival, blefaroespasmo, projeção da membrana nictitante e fotofobia bilaterais, sinais característicos de uveíte. Uveíte por causa traumática foi descartada. Seis dias após o início dos sintomas oftálmicos ocorreram vômito, incoordenação motora e alterações de comportamento. Sinais neurológicos e contração das sobrancelhas sugeriram algia na região cefálica. Injúria cerebral foi investigada por tomografia computadorizada (TC) nove dias após o início dos sintomas, quando o cão demonstrou espasmos dos membros e pescoço, seguido por parada respiratória com reversão e estabilização. A TC revelou extensa e amórfica neoformação no diencéfalo, mesencéfalo, e dentro do ventrículo lateral direito, com deslocamento à esquerda da foice cerebral. O animal foi eutanasiado e a necropsia revelou hidrocefalia e uma massa tumoral intracerebral condizente com a imagem da TC. A avaliação histopatológica por coloração hematoxilina e eosina, revelou alterações teciduais em várias áreas do Sistema Nervoso Central (SNC), mostrando várias pseudorosetas no neurópilo, mitose, e um elevado grau de atipia celular, indicando ependimoma.Lesões teciduais inflamatórias, hemorrágicas e necróticas foram observadas no tronco encefálico e cerebelo, devido à compressão do tumor e à hidrocefalia. Amostras da neoplasia foram submetidas à análise imuno-histoquímica (IHQ), sendo positiva a expressão dos marcadores tumorais vimentina e proteína glial fibrilar ácida, confirmando ependimoma anaplásico intracranial. As mudanças comportamentais e sinais neurológicos resultaram do processo vascular, inflamatório e degenerativo no neurópilo, causados pela compressão e invasão do tecido cerebral pela neoplasia. Embora cães com ependimoma frequentemente apresentem manifestações neurológicas, no presente caso, blefaroespasmo foi o primeiro sinal clínico observado pelo proprietário, e persistiu até a eutanásia do paciente. Os sinais neurológicos estão relacionados à localização do tumor e extensão das lesões secundárias devidas à sua expansão. Sintomatologia clínica e testes laboratoriais complementares, TC, necropsia, histologia e IHQ caracterizaram ependimoma, que é raro em animais jovens.(AU)

An objective tool to measure the effect of botulinum toxin in blepharospasm and hemifacial spasm.

BACKGROUND AND PURPOSE: It is challenging to assess patients with blepharospasm (BSP) and hemifacial spasm (HFS) as these patients exhibit a wide range of amplitudes of eyelid movements. In order to quantify these movements, a mathematical algorithm, i.e. Fast Fourier Transform, can be employed to convert the signal from the time domain to the frequency domain. The result of this quantification represents the energy generated during the eyelid movements. In order to objectively assess the therapeutic effects of botulinum toxin (BoNT) in these patients, we evaluated the energy generated by the upper eyelid during spontaneous eyelid movements before and after treatment. METHODS: A total of 39 patients with BSP and HFS were evaluated before and 30 days after receiving onabotulinum toxin A injections. A high-speed camera and micro light-emitting diodes were used to register the spontaneous eyelid movements. The result of the quantification obtained using Fast Fourier Transform permitted assessment of the activity associated with the eyelid movements. RESULTS: We studied 78 eyelids. The total energy generated during spontaneous eyelid movements was significantly reduced after treatment in the patients with BSP (P = 0.0018) and on the affected side in the patients with HFS (P = 0.0058). CONCLUSIONS: The assessment of the energy generated by the upper eyelid during spontaneous eyelid movements enabled us to measure the therapeutic effects of BoNT in patients with these conditions. The use of this system could enable customized and fine adjustments to BoNT doses based on each patient's needs.

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)

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)

Brief considerations on the dispensation profile of the botulinum toxin type A by the Brazilian Unified Health System for treatment of dystonias: Datasus data.

Botulinum toxin injections are the most effective approach for the treatment of focal dystonia. Despite growing demand and clinical indications over the years, there are few reports or publications of its use and benefit to patients seen at the Sistema Único de Saúde - SUS (Unified Health System). Analyzing the Datasus data (Unified Health System Information Department of Brazilian Ministry of Health), it was noticed that in Brazil the percentage of dystonic patient benefited from this procedure is still low. We therefore suggest some strategies to increase the dispensation of the toxin by the Brazilian Unified Health system for the dystonic patients.

White Matter Microstructure in Idiopathic Craniocervical Dystonia.

BACKGROUND: Dystonias are hyperkinetic movement disorders characterized by involuntary muscle contractions resulting in abnormal torsional movements and postures. Recent neuroimaging studies in idiopathic craniocervical dystonia (CCD) have uncovered the involvement of multiple areas, including cortical ones. Our goal was to evaluate white matter (WM) microstructure in subjects with CCD using diffusion tensor imaging (DTI) analysis. METHODS: We compared 40 patients with 40 healthy controls. Patients were then divided into subgroups: cervical dystonia, blepharospasm, blepharospasm + oromandibular dystonia, blepharospasm + oromandibular dystonia + cervical dystonia, using tract-based spatial statistics. We performed a region of interest-based analysis and tractography as confirmatory tests. RESULTS: There was no significant difference in the mean fractional anisotropy (FA) and mean diffusivity (MD) between the groups in any analysis. DISCUSSION: The lack of DTI changes in CCD suggests that the WM tracts are not primarily affected.

Crotoxin in humans: analysis of the effects on extraocular and facial muscles / Crotoxina em humanos: estudo da ação em músculos extraoculares e faciais

PURPOSE: Crotoxin is the main neurotoxin of South American rattlesnake Crotalus durissus terrificus. The neurotoxic action is characterized by a presynaptic blockade. The purpose of this research is to assess the ability of crotoxin to induce temporary paralysis of extraocular and facial muscles in humans. METHODS: Doses of crotoxin used ranged from 2 to 5 units (U), each unit corresponding to one LD50. We first applied 2U of crotoxin in one of the extraocular muscles of 3 amaurotic individuals to be submitted to ocular evisceration. In the second stage, we applied crotoxin in 12 extraocular muscles of 9 patients with strabismic amblyopia. In the last stage, crotoxin was used in the treatment of blepharospasm in another 3 patients. RESULTS: No patient showed any systemic side effect or change in vision or any eye structure problem after the procedure. The only local side effects observed were slight conjunctival hyperemia, which recovered spontaneously. In 2 patients there was no change in ocular deviation after 2U crotoxin application. Limitation of the muscle action was observed in 8 of the 12 applications. The change in ocular deviation after application of 2U of crotoxin (9 injections) was in average 15.7 prism diopters (PD). When the dose was 4U (2 applications) the change was in average 37.5 PD and a single application of 5U produced a change of 16 PD in ocular deviation. This effect lasted from 1 to 3 months. Two of the 3 patients with blepharospasm had the hemifacial spasm improved with crotoxin, which returned after 2 months. CONCLUSIONS: This study provides data suggesting that crotoxin may be a useful new therapeutic option for the treatment of strabismus and blepharospasm. We expect that with further studies crotoxin could be an option for many other medical areas.

OBJETIVO: A crotoxina é a principal neurotoxina da cascavel sul-americana Crotalus durissus terrificus e sua ação neurotóxica caracteriza-se por um bloqueio pré-sináptico. O objetivo da pesquisa é avaliar a capacidade da crotoxina em induzir paralisia transitória de músculos extraoculares e faciais em seres humanos. MÉTODOS: As doses utilizadas de crotoxina foram de 2 a 5 unidades (U), sendo que cada unidade correspondia a uma DL-50. Na primeira etapa, aplicou-se 2U de crotoxina em músculos extraoculares de 3 indivíduos amauróticos, candidatos à evisceração. Na segunda etapa, realizaram-se 12 aplicações de crotoxina em músculos extraoculares de 9 indivíduos estrábicos e amblíopes. Na terceira e última etapa, utilizou-se a crotoxina para o tratamento do blefaroespasmo essencial em 3 indivíduos. RESULTADOS: Nenhum paciente demonstrou qualquer efeito sistêmico ou alteração da visão ou de qualquer estrutura ocular. O único efeito local adverso foi hiperemia conjuntival, que melhorou espontaneamente. Em 2 pacientes não houve alteração do desvio ocular após a aplicação de 2U de crotoxina. Observou-se em 8 das 12 aplicações, limitação do movimento ocular no campo de ação do músculo aplicado. A diminuição do desvio ocular com 2U crotoxina (9 aplicações) foi em média de 15,7 dioptrias prismáticas (DP); na dosagem de 4U (2 aplicações) foi em média de 37,5 DP e na única aplicação de 5U, obteve-se redução de 16 DP no desvio ocular. A alteração do alinhamento ocular manteve-se por 1 a 3 meses. Dois dos 3 pacientes portadores de blefaroespasmo apresentaram melhora dos espasmos hemifacias, os quais voltaram após 2 meses. CONCLUSÕES: Através dos resultados observados neste estudo, acreditamos que a crotoxina possa ser útil no tratamento do estrabismo e do blefaroespasmo. Novos estudos precisam ser realizados para confirmar a eficácia e a segurança da crotoxina como opção terapêutica para diversas áreas da medicina que atualmente utilizam a toxina botulínica.

Manejo a nivel primario de cuerpo extraño en ojo / Manage in primary care of foreign bodies in the eye

Los cuerpos extraños en ojo es quizás la forma más común de presentación del trauma ocular. En la consulta de emergencias es usual observar traumas oculares de los cuales la mayoría son asociados con cuerpos extraños en cornea o a nivel tarsal. Dada la dificultad de acceso de algunas zonas del país a los servicios especializados en oftalmología, en el Servicio de Emergencias de Valverde Vega nació la necesidad de redactar esta actualización con lo más reciente en manejo de cuerpos extraños, con el fin de brindar la mejor atención a los usuarios con este tipo de patologías. Durante este proceso hemos concluido que el manejo ha variado en relación a la enseñanza básica del médico general en las escuelas de medicina.

Probably the most frequent presentation of ocular trauma is foreign bodies in the eye; it is a usual consultation in the emergency service, and most are associated to foreign bodies in the cornea or at the tarsus. SincetheaccesstoophthalmologicconsultationinCostaRica is limited, the Emergency Service in Valverde Vega needed to update procedures for a correct management of foreign bodies in the eye, in order to give the best attention possible to those pathologies. During this process we concluded that the management of the foreign bodies has evolved from the basic training the physicians had in medical school.

Clinical features and treatment with botulinum toxin in blepharospasm: a 17-year experience / Característica clínicas e tratamento com toxina botulínica em blefaroespasmo: experiência de 17 anos

OBJECTIVE: It was to analyze clinical aspects of patients with blepharospasm, including outcomes of botulinum toxin treatment. Additionally, clinical characteristics of isolated blepharospasm were compared to those of blepharospasm plus other movement disorders. METHODS: Clinical data recorded during 17 years were reviewed. The variables included age, gender, age of onset, past medical history, head trauma, smoking history, family history of dystonia, severity, duration of botulinum toxin relief and adverse effects. RESULTS: A total of 125 patients were included and 75.2% were female. The mean age of onset was 54.3 years; 89.6% of the individuals started with contractions in eye region, and 39.2% of them spread to lower face or neck. Isolated blepharospasm group was compared with blepharospasm-plus group for demographic and clinical features, and therapeutic outcomes, without significant differences. Botulinum toxin treatment improved the severity of contractions (p=0.01) with low rate of side effects (14%). CONCLUSIONS: Both groups - isolated blepharospasm and blepharospasm-plus - shared similar results concerning epidemiology, clinical features and therapeutic response to botulinum toxin.

OBJETIVOS: Analisar as características clínicas de pacientes com blefaroespasmo e os resultados do tratamento com toxina botulínica. Além disso, os pacientes foram divididos em dois grupos, blefaroespasmo isolado e blefaroespasmo associado a outros distúrbios do movimento, os quais foram comparados quanto a características clínicas e terapêuticas. MÉTODOS: Foram revisados prontuários dos últimos 17 anos. As variáveis consideradas foram idade, sexo, idade de início dos sintomas, antecedentes pessoais, história prévia de trauma crânio-encefálico, tabagismo, história familiar de distonia, gravidade do blefaroespasmo, duração do efeito da toxina botulínica e efeitos adversos. RESULTADOS: Foram incluídos 125 pacientes, dos quais 75,2% eram do sexo feminino. A média de idade do início dos sintomas era 54,3 anos; 89,6% dos pacientes tiveram início dos sintomas na região ocular e em 39,2% dos casos houve disseminação para face ou pescoço. Não houve diferenças significativas entre os grupos blefaroespasmo isolado versus blefaroespasmo-plus. O tratamento com toxina botulínica proporcionou melhora dos sintomas (p=0,01) com baixa incidência de efeitos adversos (14%). CONCLUSÕES: Ambos os grupos - blefaroespasmo isolado e blefaroespasmo-plus - apresentaram as mesmas características clínicas, epidemiológicas e resposta terapêutica à toxina botulínica.