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OBJECTIVE: Focal Cortical Dysplasias (FCDs) represent a common architectural cortical disorder underlying drug-resistant focal epilepsy. So far, studies aimed at evaluating whether age at surgery is a factor influencing surgical outcome are lacking, so that data on the comparison between patients harboring Type II FCD operated at younger age and those operated at adult age are still scarce. We compared presurgical clinical features and surgical outcomes of patients with histopathologically diagnosed Type II FCD undergoing surgery at an earlier age with those operated after 20 years of age. METHODS: We retrospectively analyzed 1660 consecutive patients operated at the "Claudio Munari" Epilepsy Surgery Centre. There were 289 patients (17.4%) with a neuropathological diagnosis of Type II FCD. We included two different groups of patients, the first one including patients operated on at less than 6years, the second sharing the same seizure onset age but with delayed surgery, carried out after the age of 20. Seizure characteristics and, neuropsychological and postoperative seizure outcomes were evaluated by study group. RESULTS: Forty patients underwent surgery before the age of 6 and 66 patients after the age of 20. Surgical outcome was favorable in the whole population (72.6% were classified in Engel's Class Ia+Ic), independently from age at surgery. In the children group, 32 patients were classified in Class I, including 30 (75%) children in classes Ia and Ic. In the adult group, 53 belonged to Class I of whom 47 (71%) were in classes Ia and Ic. The percentage of permanent complications, the surgical outcomes, and AED withdrawal did not significantly differ by study group. CONCLUSION: Our results indicate that there is no difference between the groups, suggesting that outcome depends mainly on the histological findings and not on timing of surgery.
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Epilepsia Resistente a Medicamentos/psicologia , Epilepsia Resistente a Medicamentos/cirurgia , Epilepsia/psicologia , Epilepsia/cirurgia , Malformações do Desenvolvimento Cortical do Grupo I/psicologia , Malformações do Desenvolvimento Cortical do Grupo I/cirurgia , Adulto , Criança , Pré-Escolar , Epilepsia Resistente a Medicamentos/epidemiologia , Epilepsia/epidemiologia , Feminino , Seguimentos , Humanos , Masculino , Malformações do Desenvolvimento Cortical/epidemiologia , Malformações do Desenvolvimento Cortical/psicologia , Malformações do Desenvolvimento Cortical/cirurgia , Malformações do Desenvolvimento Cortical do Grupo I/epidemiologia , Testes Neuropsicológicos , Período Pós-Operatório , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVE: To design and validate a scale to evaluate the quality of life in children and adolescents with epilepsy. METHODS: Scale validation, multicentered, three-phase study. We did a literature review for the construction of the instrument, and a validation of appearance, construct, criterion, and reproducibility. We evaluated the scale among the patients that consulted at the Liga Central contra la Epilepsia and the Fundación Hospital de La Misericordia (Bogotá, Colombia) between 2014 and 2015. RESULTS: The resulting questionnaire has 4 domains, with 18-26 items according to age groups (0-3, 4-10 and 11-17 years old) and a Likert scale score from 1 to 5. The comparison with CAVE and QOLIE AD 48 was adequate (Pearson correlation coefficient between 0.713 and 0.837 according to age groups: intraclass correlation coefficient between 0.664 and 0.817.) Internal consistency was adequate (Cronbach's alpha between 0.791 and 0.809). Test-retest assessment was good, with Spearman's coefficient between 0.99 and 1.00. The time to fill out the scale ranged between 3.5 and 6.8 min. SIGNIFICANCE: We designed and validated a quality-of-life scale in Spanish for children and adolescents with epilepsy, which is easy and quick to fill and has excellent reliability and validity parameters.
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Epilepsia , Qualidade de Vida , Adolescente , Criança , Pré-Escolar , Humanos , Lactente , Recém-Nascido , Psicometria , Reprodutibilidade dos Testes , Inquéritos e QuestionáriosRESUMO
A lack of neurologists in Latin America forces primary health care providers to manage epilepsy. With the main goal of improving diagnostic and therapeutic management of patients with epilepsy through training of physicians in the primary health care level, the International League Against Epilepsy Education Commission (2013-2017) created a low-cost, regional, virtual course. The course, set-up in Moodle platform, was structured in eight modules, each lasting for a week. Teaching was based on written didactic material, videos, and interactive discussions, both in Spanish and Portuguese. Topics included epidemiology, diagnosis, classification, treatment, prognosis, social issues, and epilepsy policies. Each course was limited to 50 participants and priority was given to general practitioners. Certification was given to those approving the final examination. Since 2015, five courses have been developed, involving 143 participants from 17 countries and 21 tutors. Of the participants, 61% worked in primary health care services. A total of 129 participants (90%) completed the course, and 110 submitted the final examination with an approval rate of 95%. From 85 participants completing the course evaluation, 98% would recommend the course to other colleagues, and 99% showed interest in taking other similar courses. High self-confidence for the management of patients with epilepsy increased from 21% at baseline to 73% after the course. The online course on epilepsy for primary care physicians in Latin America was shown to be a cost-effective course, with good retention and excellent approval rates. Our current challenges include periodic updating, complete self-sustainability, and exploring different strategies to reach our target audience more effectively.
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Instrução por Computador , Epilepsia/diagnóstico , Epilepsia/terapia , Atenção Primária à Saúde/economia , Análise Custo-Benefício , Humanos , América Latina , Papel do Médico , Estados UnidosRESUMO
Abstract Introduction: Language development is a complex process that may be considered as an evolutionay trait in human beings; it is possible to undetstand said process by evaluating the contribution of sensory systems and the events that frame critical periods of development. Objective: To conduct a literature on how auditory, visual and proprioceptive information integration occurs in language development, as well as the role of social interaction in this process. Materals and methods: The MeSH terms "Language Development"; "Visual Perception"; "Hearing"; and "Proprioception" were used, limiting the main search to articles written in English, Spanish and Portuguese. The databases consulted were Medline and EMBASE. Results: Auditory information is the first sensory stimulus to consider because, during the first year of life, the infant recognizes and discriminates environmental signals that correspond to language, followed by a peak in its acquisition and, subsequently, by a stage of maximum linguistic discrimination. Visual information allows correlating language with images since it acts as the substrate for the designation and understanding of words, as well as for interpretation and imitation of the emotional component in gesticulation. Proprioceptive information provides feedback on motor performance patterns used in language production. Conclusion: This perspective offers new points of view for treating and managing deviations in language development.
Resumen Introducción. El desarrollo del lenguaje es un proceso complejo considerado como marcador evolutivo del ser humano y puede ser comprendido a partir de la contribución de los sistemas sensoriales y de los eventos que ocurren en periodos críticos del desarrollo. Objetivo. Realizar una revisión de cómo se da la integración de la información auditiva, visual y propioceptiva y cómo se refleja en el desarrollo del lenguaje, destacando el papel de la interacción social como contexto que favorece este proceso. Materiales y métodos. Se utilizaron los términos MeSH "Language Development"; "Visual Perception"; "Hearing"; y "Proprioception en las bases de datos MEDLINE y Embase, limitando la búsqueda principal a artículos escritos en inglés, español y portugués. Resultados. El punto de partida lo constituye la información auditiva, la cual, en el primer año de vida, permite la discriminación de los elementos del ambiente que corresponden al lenguaje; luego un pico en su adquisición y posteriormente una etapa de máxima discriminación lingüística. La información visual proporciona la correspondencia del lenguaje en imágenes, sustrato de nominación y comprensión de palabras, además de la interpretación e imitación del componente emocional en la gesticulación. La información propioceptiva ofrece la retroalimentación de los patrones de ejecución motora empleados en la producción del lenguaje. Conclusión. El estudio del desarrollo lenguaje desde la integración sensorial ofrece nuevas perspectivas para el abordaje e intervención de sus desviaciones.
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ABSTRACT Introduction: Super-refractory status epilepticus (SRSE) is a pathology that affects the neuronal environment depending on the types of seizure and their duration. Case presentation. This paper presents the case of a 7-year old child presenting with super-refractory status epilepticus and multifocal seizures. Metabolic, structural, infectious, toxicological and autoimmune causes were discarded, while different anticonvulsive agents were administered without any clinical improvement; seizures were controlled 6 weeks after admission to ICU. A 12-year follow-up was performed, during which time the patient presented recurrent status epilepticus with autonomic seizures and progressive cognitive decline. Discussion: This type of status epilepticus is part of the syndrome known as Febrile Infection-Related Epilepsy Syndrome (FIRES), a possibly autoimmune form of epileptic encephalopathy that is refractory to acute and chronic management. There is no report in the literature that includes long term follow-up, therefore, there is no actual consensus about the appropriate management of the chronic phase of the disease. Conclusion: FIRES must be considered as one of the possible etiologies of super-refractory status epilepticus, so early management strategies (like ketogenic diet) can be used in order to achieve control of the critically ill patient, control long term seizures and improve cognitive outcomes, having as the final result a positive impact on the quality of life of the patient.
RESUMEN Introducción: El estado epiléptico superrefractario (EES) es una patología con importante morbimortalidad que afecta el ambiente neuronal según el tipo y duración de las crisis. Presentación del caso: Se presenta el caso de un escolar con estado epiléptico superrefractario y crisis multifocales. Se descartaron causas metabólicas, estructurales, infecciosas, toxicológicas y autoinmunes y se utilizaron diferentes manejos anticonvulsivantes sin respuesta, lográndose control de las crisis 6 semanas después del ingreso a UCI. Se realizó un seguimiento de 12 años, periodo en el que el paciente presentó múltiples recaídas del estado epiléptico asociadas a la presencia de epilepsia refractaria con múltiples tipos de crisis, en su mayoría vegetativas; además se dio involución cognitiva. Discusión: Esta forma de estado epiléptico corresponde al síndrome de estado epiléptico facilitado por fiebre (FIRES), entidad de posible origen inmunológico conocida por ser refractaria al tratamiento agudo y al manejo crónico de la epilepsia y que se presenta como secuela. Su evolución no se ha descrito a largo plazo y por tanto no hay consenso sobre el manejo en la fase crónica. Conclusión: Es importante considerar esta etiología en estado epiléptico superrefractario para utilizar de forma temprana diferentes estrategias terapéuticas, como la dieta cetogénica, que permitan, por un lado, controlar su condición crítica y las crisis epilépticas a largo plazo y, por el otro, mejorar el pronóstico cognitivo, logrando así un impacto en la calidad de vida.
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Humanos , Epilepsia , Criança , Febre , Epilepsia Resistente a MedicamentosRESUMO
Abstract Based on the importance of a comprehensive professional training, this research aims at observing and describing the learning process achieved through the hidden curriculum of students enrolled in the Neuropediatrics Specialization Program at Universidad Nacional de Colombia in 2012 y 2013. A qualitative study to explore students' training, transmission of attitudes, values and ethical aspects was performed through the implementation of a semi-structured interview and a focal group. For this group of students, learning through the hidden curriculum is real and educational, allows having a better approach to patients and acquiring appropriate tools for a successful job performance. Nevertheless, it is still insufficient to address ethical dilemmas such as delivery of bad news and patients' deaths. Thus, complementing this type of learning with explicit training on bioethics is required.
Resumen Basándose en la importancia de la formación integral del profesional, se pretendió observar y describir el aprendizaje logrado a través del currículo oculto en los estudiantes matriculados en la especialidad en Neuropediatría de la Universidad Nacional de Colombia en 2012 y 2013. A través de la aplicación de una entrevista semiestructurada y de un grupo focal, se realizó un estudio cualitativo que exploró aspectos de la formación, la transmisión de actitudes y valores y los aspectos éticos de los estudiantes. Para este grupo de alumnas el aprendizaje a través del currículo oculto es real y formativo, les permite una mejor aproximación a sus pacientes y les da las herramientas para un desempeño profesional exitoso; sin embargo, es aún insuficiente para resolver dilemas éticos como enfrentar la explicación de malas noticias y el momento del final de la vida. Se requiere entonces complementar este aprendizaje con enseñanzas explícitas en el área de la bioética.
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El estado epiléptico es una condición frecuente en la población general, siendo los extremos de la vida los más susceptibles de presentarlo y dado que es una condición que amenaza la vida del paciente, requiere de medidas organizadas pero agresivas para atacarlo y de este modo prevenir daño neurológico, complicaciones sistémicas y la muerte. Se requiere difundir el conocimiento al respecto de esta patología para mejorar la atención y el pronóstico de los niños que se presentan al servicio de urgencias, por lo cual es importante conocer la fisiopatología subyacente para enfilar adecuadamente los esfuerzos en la práctica diaria. Tradicionalmente se ha usado medicamentos de primera línea como benzodiacepinas y fenitoina pero actualmente en nuestro medio se cuenta con un mayor arsenal terapéutico que permite al clínico escoger la mejor opción para cada paciente en particular, incluso en casos de estado epiléptico refractario. [Espitia OM, Buch MF, Uscátegui AM. Estado epiléptico en niños. MedUNAB 2010; 13:22-30].
Status epilepticus is a frequent emergency in general population, being the extreme ages the most susceptible, and since it is a potentially life threatening condition, requires an organized plan and aggressive measures to attack it and then prevent neuronal damage, systemic complications and even death. It is required to spread the knowledge about this pathology to improve attention and prognosis in emergency room settings, thus it is important to know the underlying physiopathology to line up the efforts in daily practice. Traditionally it has been used first line drugs as benzodiazepines and phenytoin however actually there are some other options that let the physician to choose the better one for each particular patient, even in the refractory status epilepticus. [Espitia OM, Buch MF, Uscátegui AM. Status epilepticus in children. MedUNAB 2010; 13:22-30].
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Estado Epiléptico , Convulsões , Criança , EpilepsiaRESUMO
Síndrome de West y epilepsia focal. En el primer caso la evidencia es comparable con la descrita para esteroides, sin embargo la incidencia de efectos colaterales es menor. Es de elección, con adecuado nivel de evidencia, en el manejo de epilepsia causada por esclerosis tuberosa. Se puede recomendar en el manejo de epilepsia focal, vigilando la aparición de crisis paradójicas.
We present a review regard the effectiveness of vigabatrine in the treatment of childhood epilepsy, emphasizing on West syndrome and focal epilepsy. In the first case, evidence is comparable with corticosteroids but with lesser side effects. In epilepsy caused by tuberous sclerosis Vigabatrine is the drug of choice. Vigabatrine can be recommended in the treatment of focal epilepsy monitoring the occurrence of paradoxical crisis.
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Humanos , Efetividade , Epilepsia , Vigabatrina , NeurologiaRESUMO
Se presenta una revisión de la literatura acerca de la presencia de alteración del campo visual asociada al uso de vigabatrín como antiepiléptico. Considerando las precauciones de prescripción derivadas de los efectos visuales de vigabatrin, presentamos una revisión no sistemática que muestra los principales datos respecto a prevalencia, factores de riesgo, fisiopatología y seguimiento de esta alteración. La prevalencia de aparición de la retinopatía es variable según las series revisadas, y los factores de riesgo como uso de dosis máxima, dosis acumulada y edad no están claramente demostrados, por lo que se considera a éste un efecto idiosincrático. Se recomienda la vigilancia oftalmológica y campimétrica periódica.
A review of the literature about the presence of visual field disturbance associated with the use of vigabatrin as an antiepileptic. Taking into account that there exists warnings to prescribe vigabatrine derived from visual defects, we present a non - systematic review that describe the main aspects regard to prevalence, risk factors, pathophisiology and following of this side effect. Prevalence of occurrence of retinopathy varies according to differentseries, and risk factors such as use of maximum dose, cumulative dose and age, so that this effect is considered idiosyncratic. Periodic ophthalmologic supervision is recommended.
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Humanos , Anticonvulsivantes , Epilepsia , Vigabatrina , NeurologiaRESUMO
Se realiza una discusión sobre los proyectos de ley en curso y próximos a constituirse en leyes del estado, que modificarán en un futuro próximo el ejercicio profesional del médico y por ende del neurólogo infantil en el país. Estos proyectos incluyen reformas a la ley 100, buscando ampliar coberturas de aseguramiento, mayor participación en los organismos de planeación y control, incentivos a la investigación y gestión en salud y mayor posibilidad de contratación de IPS independientes, sean estas privadas o públicas, evitando monopolio de las EPS. También se analiza la reglamentación del ejercicio del recurso humano en salud, incluyendo vigilancia, control, recertificación de profesionales, observatorio laboral, recomendaciones sobre formación, reglamentación de sitios de práctica, incluyendo hospitales universitarios y recomendaciones sobre un manual de tarifas para el personal de salud. El conocimiento de esta legislación nos permitirá estar preparados como profesionales y como agremiaciones para estos cambios y así poder intervenir activamente en estos procesos de cambio, buscando en estos el beneficio común tanto para el gremio como para los pacientes y los demás actores del sistema general de seguridad social.