Eficácia e segurança do Felbamato comparado a medicamentos disponíveis no SUS para o tratamento da epilepsia refratária em crianças e adultos: revisão rápida de evidências / Effectiveness and safety of Felbamate compared to drugs available in SUS for the treatment of refractory epilepsy in children and adults: rapid evidence review

Tecnologia: Felbamato. Indicação: Tratamento de epilepsia refratária. Pergunta: O Felbamato é mais eficaz e seguro comparado a anticonvulsivantes disponíveis no Sistema Único de Saúde (SUS) em pacientes com epilepsia refratária? Métodos: Revisão rápida de evidências (overview) de revisões sistemáticas, com levantamento bibliográfico realizado na base de dados PUBMED, utilizando estratégia estruturada de busca. A qualidade metodológica das revisões sistemáticas foi avaliada com AMSTAR-2 (Assessing the Methodological Quality of Systematic Reviews). Resultados: Foram selecionadas 2 revisões sistemáticas, que atendiam aos critérios de inclusão. Conclusão: O felbamato não demonstrou ser uma opção mais benéfica que os demais medicamentos disponíveis no SUS no tratamento de epilepsia refratária a medicamentos. Salienta-se que a maior parte das evidências eram de baixa certeza

Technology: Felbamate. Indication: Treatment of refractory epilepsy. Question: Is felbamate more effective and safer compared to anticonvulsants available in Brazilian Public Health System in patients with refractory epilepsy? Methods: A rapid review of evidence (overview) of systematic reviews, with bibliographic survey carried out in the PUBMED database, using a structured search strategy. The methodological quality of systematic reviews was assessed using AMSTAR-2 (Assessing the Methodological Quality of Systematic Reviews). Results: Two systematic reviews that met the inclusion criteria were selected. Conclusion: Felbamate did not prove to be a more beneficial option than the other drugs available in the Brazilian Public Health System in the treatment of drug-refractory epilepsy. It should be noted that most of the evidence was of low certainty

Experiencia de la neuromodulación en un hospital de alta complejidad / High Complexity Hospital ́s experience in neuromodulation

La neuromodulación es una práctica médica implementada desde hace más de cuatro décadas. En lo que respecta a la Neurocirugía, cumple un papel en el tratamiento de diversas patologías (Parkinson, distonías, epilepsia, etc.) y con un gran potencial para aplicarlas en otras (trastorno obsesivo compulsivo [TOC], dolor pélvico). Es por ello que, en los últimos años, se cuadruplicaron las inversiones de empresas biotecnológicas en este campo por la demanda y aplicación de la terapia. La neuromodulación abarca también otras especialidades, como por ejemplo Otorrinolaringología (ORL) en implantes cocleares, Cardiología con diversos modelos de marcapasos cardíacos, Endocrinología con bombas de infusión de medicamentos, Uroginecología en incontinencia, etcétera. Nuestra institución aplica en su práctica clínica todas estas técnicas, y cumple una función jerárquica como centro de referencia en educación y políticas sanitarias. Por estos aspectos, sumados a su infraestructura, personal profesional y enfoque sanitario, puede ser considerada como un Centro de Neuromodulación referente en la región. (AU)

Neuromodulation is a medical practice established for more than forty years. In the neurosurgical field it plays a role in the treatment of different diseases (Parkinson, Dystonia, Epilepsy, etc) and has a great potential to apply in other pathologies (Obsessive Compulsive Disorder, Pelvic pain). In the last years the biotechnological industry has quadrupled the investment in this field because of the demand and therapy application. Neuromodulation encompasses other specialities, for example otorhinolaryngology in cochlear implants, in cardiology with different models of pacemakers, endocrinology with implanted infusion pumps, urological gynecology in incontinence treatments, etc. Our institution applies all these techniques in its clinical practice, having a hierarchical role as a reference center in education and health policies. Due to these aspects, added to its infrastructure, professional staff and health approach, it can be considered as a reference Neuromodulation Center in the region. (AU)

Reducing VNS stimulation parameters: Is it safe?

Introduction Vagal nerve stimulation (VNS) is an adjuvant therapy used in the treatment of patients with refractory epilepsy who are not candidates for resective surgery or who have limited results after surgical procedures. Currently, there is enough evidence to support its use in patients with various types of epilepsy. Therefore, the present study was conducted to explore the possibility of optimizing therapy by reducing the consumption of the system's battery. Methods The prospective and double-blind analysis consisted in the evaluation of 6 patients submitted to VNS implantation for 3 months, followed by adjustment of the stimulation settings and continuity of follow-up for another month. The standard protocol was replaced by another with a frequency value of 20 Hz instead of 30 Hz to increase battery life. The safety of this procedure was evaluated through the assessment of two main variables: seizures and side effects. Results The stimulation at 20 Hz showed 68% reduction in the incidence of seizures (p»0.054) as well as low incidence of side effects. Conclusion The present study suggests that the reduction of the stimulation frequency from 30 to 20 Hz is a safe procedure, and it does not compromise the effectiveness of therapy.

Efficacy and safety of vagus nerve stimulation in the treatment of refractory epilepsy / 中南大学学报(医学版)

OBJECTIVES@#Vagus nerve stimulation (VNS) is a neuromodulative therapeutic technique for patients with drug-resistant epilepsy who are not suitable for resection or who have experienced a failed resection. This study aims to explore the efficacy and safety of VNS in patients with refractory epilepsy, and to analyze the influential factors for the efficacy.@*METHODS@#A retrospective review of clinical data were conducted for 35 patients, who were treated for refractory epilepsy through VNS surgery in the Department of Neurosurgery, Xiangya Hospital, Central South University from April 2016 to August 2019. All patients were analyzed in terms of the clinical and follow-up data.@*RESULTS@#After a mean follow-up of 26 months (6-47 months), outcome was as follows: 7 patients were MuHugh class I, 13 patients were MuHugh class II, 8 patients were MuHugh class III, and 7 patients were MuHugh class IV-V. The total efficacy rate in the short duration group was significantly higher than that in the long duration group (77.8% vs 50.0%, @*CONCLUSIONS@#VNS is a safe and effective option in treating patients with refractory epilepsy, especially for those with short duration.

Mutación de protocadherina 19 (PC-DH19), en paciente con epilepsia refractaria / Mutation of protocadherin 19 (PCDH19), in patients with refractory epilepsy

Paciente de 4 años de edad, con epilepsia de difícil manejo, cuya etiología se atribuye a patología autoinmune y que finalmente se diagnostica una mutación de protocadherina (PCDH19). Se discute la fisiopatología, características clínicas, exámenes y los posibles tratamientos.

Four-year-old patient with intractable epilepsy, whose etiology is attributed to autoimmune pathology and who is eventually diagnosed with a protocadherin mutation (PCDH19). Pathophysiology, clinical characteristics, examinations and possible treatments are discussed.

Estado epiléptico refractario en UCIP. Experiencia en un hospital de la Provincia de Buenos Aires / Refractory status epilepticus in the PICU. Experience at a hospital in the Province of Buenos Aires

Introducción: El estado epiléptico (EE) es la emergencia neurológica más frecuente en pediatría. Los pacientes que no responden al tratamiento estándar con dosis adecuadas de benzodiacepinas seguido de una droga antiepiléptica aceptable son definidos como Estado epiléptico Refractario (ER). Objetivo: caracterizar la población de niños con EE que ingresan a UCIP y determinar qué factores son predictores de refractariedad en esta población. Métodos: Estudio de casos y controles, retrospectivo. Población: niños con EE internados en UCIP desde Febrero 2015 a Febrero 2017. Casos: Estado epiléptico Refractario (ER). Controles: Estado epiléptico No Refractario (ENR). Se calculó el Odds Ratio (OR) individual para las distintas variables en Med Calc. Resultados: Se internaron 35 pacientes de los cuales 12 fueron casos y 23 controles. Hubo fiebre en 77% de los pacientes. En el total de niños estudiados hubo 11% con antecedente de convulsión febril, 11% con antecedente de epilepsia y 9% con antecedente de malformación del SNC. Los niños con antecedente de convulsión febril tuvieron 2,5 veces mayor riesgo de ER (OR: 2,58; IC 95%: 1,17-5,68). Los niños con EE que tenían antecedentes de enfermedad neurológica previa presentaron riesgo de ER 2,6 veces mayor que el grupo control (OR 2,60; IC 95%: 1,24-5,42). Discusión: Dado el aumento en la mortalidad de los pacientes con ER sería importante disponer de más herramientas para predecir este desenlace e iniciar tratamiento oportuno. Resultaría útil entrenar a los padres de niños con antecedente de convulsión febril en la aplicación de medicación antiepiléptica prehospitalaria, esto podría prevenir la farmacorresistencia, el daño neurológico y las complicaciones que acarrea el ingreso a UCIP. (AU)

Introduction: Status epilepticus (SE) is the most common neurologic emergency in children. Patients that do not respond to standard treatment with adequate doses of benzodiazepines followed by an acceptable antiepileptic drug are defined as having refractory status epilepticus (RSE). Objective: To characterize the population of children with SE admitted to the PICU and to determine predictive factors for refractoriness in this population. Methods: A retrospective case-control study was conducted. Population: Children with SE admitted to the PICU between February 2015 and February 2017. Cases: Refractory status pilepticus (RSE). Controls: Non-refractory status epilepticus (NRSE). Individual Odds Ratio (OR) was calculated for different variables using Med Calc. Results: 35 patients were admitted of whom 12 were cases and 23 controls. Overall, 77% of the patients had fever. Of all the children, 11% had a history of febrile seizures, 11% had history of epilepsy and 9% had a CNS malformation. Children with a history of febrile seizures had a 2.5-fold higher risk of developing RSE (OR: 2.58; 95% CI: 1.17-5.68). Children with SE that had a history of neurologic disease had a 2.6-fold higher risk of developing RSE than controls (OR 2.60; 95% CI: 1.24-5.42). Discussion: Given the increased mortality in children with RSE, availability of tools to predict this outcome in order to initiate early treatment is important. It would be useful to train the parents of children with a history of febrile seizures in the prehospital administration of antiepileptic drugs as this may prevent pharmaco-resistance, neurologic damage, and complication related to PICU admission (AU)

El equipo multidisciplinario en la aplicación clinica de la dieta cetogénica / Multidisciplinary teamwork in the clinical application of the ketogenic diet

La dieta cetogénica se considera un tratamiento eficaz y bien tolerado para la epilepsia refractaria. Nuestro trabajo suministra información práctica sobre el uso de la dieta cetogénica, realizando una revisión acerca de las indicaciones clásicas y las nuevas que permiten ampliar el uso de la misma. Detallamos cómo implementar la dieta, los controles médicos en el seguimiento y la forma de actuar en situaciones especiales, tales como la anestesia o la enfermedad aguda. Se enfatiza la i mportancia del trabajo de los profesionales de las distintas especialidades y la familia como equipo, detallando los roles que ocupa cada uno en este interesante pero laborioso tratamiento.

The ketogenic diet is an established, effective and well-tolerated treatment in refractory epilepsy. This paper provides practical information on the administration of ketogenic diet. It goes over the standardized protocols and the new ones that allow to broaden the scope regarding the clinical management of the ketogenic diet. It addresses issues such as the implementation, counseling and follow-up as well as the application and how to proceed in special situations such as anesthesia or acute illness. Finally, the aim of this paper is to highlight the relevance of the multidisciplinary teamwork and the family support throughout this laborious but interesting treatment.

The role of vagus nerve stimulation in refractory epilepsy / O papel da estimulação do nervo vago na epilepsia refratária

ABSTRACT Vagus nerve stimulation is an adjunctive therapy used to treat patients with refractory epilepsy who are not candidates for resective surgery or had poor results after surgical procedures. Its mechanism of action is not yet fully comprehended but it possibly involves modulation of the locus coeruleus, thalamus and limbic circuit through noradrenergic and serotonergic projections. There is sufficient evidence to support its use in patients with focal epilepsy and other seizure types. However, it should be recognized that improvement is not immediate and increases over time. The majority of adverse events is stimulation-related, temporary and decreases after adjustment of settings. Future perspectives to improve efficacy and reduce side effects, such as different approaches to increase battery life, transcutaneous stimulation and identification of prognostic factors, should be further investigated.

RESUMO A estimulação vagal é uma terapia paliativa utilizada no tratamento de pacientes com epilepsia refratária que não são candidatos à cirurgia ressectiva ou naqueles com evolução insatisfatória após o procedimento cirúrgico. Seu mecanismo de ação ainda não foi completamente elucidado mas possivelmente envolve a modulação do locus coeruleus, tálamo e circuito límbico através de projeções noradrenérgicas e serotoninérgicas. Atualmente há evidência suficiente para corroborar o uso desta terapia em pacientes com epilepsia focal e outros tipos de crise, com resultados que, apesar de não imediatos, melhoram progressivamente no longo prazo. Os eventos adversos são, em sua maioria, relacionados à estimulação e auto-limitados. Perspectivas futuras para aumentar a eficácia e reduzir os efeitos colaterais como a utilização de baterias com maior durabilidade, estimulação transcutânea e identificação de fatores prognósticos devem ser investigadas.