Encefalitis de Rasmussen: diagnóstico y enfoque terapéutico / Rasmussen encephalitis: diagnostic and therapeutic approaches

Objetivo: describir el caso de una paciente escolar con diagnóstico de encefalitis de Rasmussen de presentación aguda y el enfoque terapéutico. Presentación: paciente femenina de 5 años y nueve meses que cursa con cuadro de evolución progresiva caracterizado por posturas distónicas que se inicia en pierna izquierda seguida de miembro superior ipsilateral asociadas con crisis focales con semiología de inicio opercular que la conducen a estatus epiléptico. La resonancia magnética cerebral evidencia cambios estructurales progresivos con atrofia hemisférica y del núcleo basal derechos, se inicia terapia antiepiléptica e inmunomoduladora con buena respuesta. Discusión: se realizó una revisión de la literatura sobre la presentación clínica, diagnóstico y mejores opciones terapéuticas. Conclusiones: se trata de una escolar con síndrome de Rasmussen en etapa aguda, con adecuada evolución clínica luego de inicio de inmunoterapia. En Colombia existen pocos reportes, nuestro propósito es realizar la presentación de un caso y revisar el enfoque diagnóstico y terapéutico.

Objective: to describe the case of a school-age patient aged 5 years 9 months diagnosed with acute Rasmussen encephalitis (RE), and its therapeutic approach. Case presentation: a girl aged 5 years 9 months presenting with progressive dystonic postures starting in the left lower limb with spread to the ipsilateral upper limb associated with focal seizures characterized by opercular epilepsy semiology which lead to status epilepticus. Cerebral magnetic resonance imaging revealed progressive structural changes with atrophy of the right cerebral hemisphere and basal nuclei. Anti-epileptic and immunmodulator therapy was initiated obtaining good response. Discussion: a review of the literature on RE clinical presentation, diagnosis and best treatment options was conducted. Conclusions: this is the case of a young girl with Rasmussen syndrome in the acute stage evidencing proper clinical progression after receiving immunotherapy. Few reports exist on this topic in Colombia. Our purpose was to present a case and review RE diagnostic and therapeutic approaches.

Proton magnetic resonance spectroscopy in epilepsy: key findings / Espectroscopia de prótons por ressonância magnética em epilepsia: principais achados / Espectroscopia de protones por resonancia magnética en epilepsia: principales hallazgos

Magnetic resonance spectroscopy (MRS) is a non-invasive technique useful both in research and neuroclini- cal evaluation. It relies on the same physical principles of magnetic resonance imaging providing information on chemical compounds in vivo. MRS uses the magnetic properties of several nuclei such as 13C, 31P and 19F, although the 1H is the most common due to its abundance and magnetic resonance signal sensitivity. Particularly in the last two decades, MRS has helped to better understand epilepsy and characterize its metabolic changes. In this review article, we aimed to point out the main contributions of MRS for epilepsy, focusing on proton magnetic resonance spectroscopy (1H-MRS).

A espectroscopia por ressonância magnética (ERM) é uma técnica não invasiva útil tanto em pesquisa quanto em avaliação neuroclínica. Baseia-se nos mesmos princípios físicos da ressonância magnética (RM) convencional, fornecendo informações sobre compostos químicos in vivo. A ERM usa as propriedades magnéticas de vários núcleos, como 13C, 31P e 19F, embora o 1H seja o mais utilizado devido a sua abundância e à sensibilidade do sinal de ressonância magnética. Especialmente nas duas últimas déca- das, a ERM tem ajudado a compreender melhor a epilepsia e a caracterizar suas alterações metabólicas. Nesse artigo de revisão, buscamos apontar as principais contribuições da ERM para a epilepsia, com foco em espectroscopia de prótons por ressonância magnética (1H-ERM).

La espectroscopia por resonancia magnética (ERM) es una técnica no invasiva utilizada en la investigación y en la evaluación neurológica clínica. Se basa en los mismos principios físicos de la resonancia magnética (RM) convencional, proporcionando informa- ción sobre compuestos químicos in vivo. Para este fin, la ERM utiliza las propiedades magnéticas de diversos núcleos tales como 13C, 19F y 31P. Sin embargo, el 1H es el más utilizado debido a su abundancia y la mayor sensibilidad de la señal de resonancia magnética. Especialmente en las últimas dos décadas, el uso de la ERM ha ayudado a comprender mejor la epilepsia y caracterizar sus cambios metabólicos. En este artículo de revisión tratamos de señalar las principales aportaciones de la ERM para la epilepsia, centrándonos en la espectroscopia de protones por resonancia magnética.

Crisis parciales como presentación atípica de aneurisma gigante / Partial seizures as atypical presentation giant aneurysm

Los aneurismas gigantes suelen manifestarse en forma de hemorragia subaracnoidea o cefalea. La presentación clínica de un aneurisma gigante intracraneal como epilepsia parcial es inusual, como lo indican los pocos casos recogidos en la literatura. Se presenta el caso de un aneurisma gigante intracraneal de arteria carótida interna derecha, que debuta con crisis focales del lóbulo temporal, manteniéndose sin otra manifestación a lo largo de años a pesar de las llamativas dimensiones.

Giant intracranial aneurysms are usually manifested as subarachnoid hemorrhage or headache. The clinical debut of a giant intracranial aneurysm as partial epilepsy is unusual and indicates the few cases reported in the literature. For giant aneurysm of an intracranial internal carotid artery occurs. Debuts with focal seizures of the temporal lobe, remaining without further demonstration over the years despite the striking dimensions.

Prevalência dos fatores instrínsecos e extrínsecos do processo de aprendizagem em crianças com epilepsia / Prevalence of intrinsic and extrinsic factors of the learning process in children with epilepsy

Objetivo levantar a prevalência dos fatores intrínsecos e extrínsecos que podem interferir no processo de aprendizagem em crianças com epilepsia. Métodos este estudo descritivo foi realizado no Ambulatório de Neurologia Infantil do Hospital de Pediatria Professor Heriberto Bezerra (HOSPED) da UFRN. A obtenção dos dados ocorreu durante setembro/2009 a março/2010 por meio da aplicação de um questionário com pais e cuidadores de crianças com epilepsia. A amostra foi constituída por 41 crianças, seguindo os seguintes critérios de inclusão: a) pais ou cuidadores de crianças com diagnóstico inequívoco de epilepsia atendidas no ambulatório do HOSPED; b) crianças com idades entre 3 e 12 anos; e c) pais ou responsáveis assinarem o termo de consentimento livre e esclarecido. Resultados 61% das crianças apresentaram diagnóstico de epilepsia pura. 59% tiveram sua primeira crise antes dos 03 anos de idade. 34% apresentavam crises do tipo generalizada. 51% apresentavam crises no período da pesquisa. 98% estavam em tratamento medicamentoso para controle das crises, sendo 55% monoterapia e 45% politerapia. 76% estavam inseridas na escola, sendo 50% em escolas públicas. 66% nunca repetiram o ano. 49% das crianças tiveram assiduidade escolar prejudicada em virtude das crises. 64% nunca foram excluídas da escola pelos professores devido a epilepsia e 85% dos pais afirmaram superproteger os filhos. Conclusão o estudo concluiu que, além da epilepsia, as crianças com essa patologia são também expostas a outros fatores, decorrentes da doença, que podem influenciar negativamente no processo de aprendizagem dessas crianças. .

Purpose to raise the prevalence of intrinsic and extrinsic factors of the learning process in children with epilepsy. Methods this descriptive study was conducted at the Clinic of Neurology Children’s Hospital of Pediatrics Professor Heriberto Bezerra, HOSPED – UFRN. Data collection occurred during the September/2009 to March/2010 through a questionnaire with parents and carers of children with epilepsy. The sample comprised 41 children, according to the following inclusion criteria: a) parents or caregivers of children with an unequivocal diagnosis of epilepsy seen at the outpatient clinic of HOSPED; b) children aged between 3 and 12 years; and c) parents or guardian sign the consent form free and clear. Results 61% of children were diagnosed with pure epilepsy. 59% had their first crisis before the age of 03. 34% presented generalized crisis type. 51% presented crisis during the survey period. 98% were on medications to control crisis, and from these children, 55% monotherapy and 45% polytherapy. 76% were at school, 50% inserted in public school. 66% never repeated the school year. 49% of children had school attendance affected because of the crisis. 64% have never been excluded from school by teachers because of epilepsy and 85% of parents affirmed to overprotect their children. Conclusion the study concluded that, in addition epilepsy, children with that pathology are also exposed to other factors, resulting from the disease, which may negatively affect these children learning process. .

Síndrome opercular o síndrome perisilviano / Operculum syndrome or perisylvian syndrome

El interés de los autores es llamar la atención sobre el síndrome opercular, y estimular con ello su identificación en la práctica neuropediátrica. Se realizó una búsqueda en PubMed desde febrero de 2005 hasta septiembre de 2010, y se comentaron los artículos que, a consideración de los autores, mostraban los diferentes aspectos del concepto, historia, características clínicas, causas, así como del diagnóstico, evolución y pronóstico. El síndrome opercular puede ser de causa congénita o adquirida; en los adultos es más frecuente por infarto cerebral opercular bilateral, no así en los niños, en los que se puede presentar por diferentes causas, desde trastornos de la migración neuroblástica, hasta en la epilepsia. En niños epilépticos se debe estar atento a su evolución, ya que tanto por el tipo de epilepsia o síndrome epiléptico, como por la medicación antiepiléptica usada, puede presentarse este síndrome, teniendo una gran significación su identificación rápida y tratamiento adecuado

The interest of authors is to attract attention on operculum syndrome and thus to stimulate its identification in the neuropediatric practice. A search in PubMed from February, 2005 to September, 2010 was made commenting on papers that according authors showed the different features of concept, history, clinical features, causes, as well as diagnosis, evolution and prognosis. The operculum syndrome may be congenital or acquired; ion adults is more frequent by bilateral operculum cerebral infarction, but not in children in whom it may be present by different causes, from neuroblast migration to epilepsy. In the case of epileptic children it is necessary to pay attention to its course since due to the type of epilepsy or epileptic syndrome or due to antiepileptic drug used, this syndrome may be present, considering very much its fast identification and appropriate treatment

Tratamento das epilepsias parciais / Partial epilepsies treatment

As epilepsias parciais constituem a forma mais comum de epilepsia nos indivíduos adultos. As drogas antiepilépticas (DAEs) permanecem como a principal forma de tratamento para os pacientes com epilepsia. Apesar da importância da medicação um número elevado de pacientes permanece sob um regime terapêutico inapropriado ou até mesmo sem qualquer medicação. Existem várias medicações disponíveis para o tratamento das epilepsias. A escolha de uma medicação específica ou a associação entre DAEs deve ser particularizada o máximo possível. Neste artigo revisamos alguns aspectos como classificação, início das crises, idade, sexo, comorbidades, custo e posologia das DAEs e história medicamentosa com a perspectiva de auxiliar nesta individualização do tratamento. Algumas características das principais DAEs disponíveis também são discutidas. Estes aspectos podem auxiliar na criação de um perfil ajudando assim na escolha do regime terapêutico mais apropriado para cada indivíduo. Aspectos práticos como o manuseio dos efeitos adversos, monoterapia e politerapia também são abordados.

Partial epilepsies are the most common form of epilepsy in adult individuals. Antiepileptic drugs (AEDs) continue as the main form of treatment for patients with epilepsy. Regardless of the importance of the medication a high number of patients are under inappropriate or not receiving AEDs. There are several medications available for the treatment of epilepsy. The choice of a particular medication or association among AEDs may be individualized as much as possible. In this article some aspects such as classification, onset of the seizures, age, sex, associated medical conditions, cost and posology of AEDs and medical drug history are reviewed. Details of the available AEDs are also discussed. These points may help to create a profile helping the decision for the appropriate AED. Some practical issues like adverse reaction management, monotherapy and politherapy are also discussed.

Diferencias neurocognitivas entre las epilepsias parciales frontales y temporales en la infancia / Neurocognitive differences between frontal and temporal partial seizures in childhood

Introducción. Establecer las diferencias neurocognitivas entre las epilepsias pediátricas parciales del lóbulo frontal y del temporal. Métodos. En una investigación clínica de tipo transversal, descriptiva y prolectiva, se analizaron pacientes pediátricos con epilepsia parcial entre los 6 y 12 años de edad, de ambos sexos, en el Departamento de Neurología del Hospital Infantil de México Federico Gómez. Posterior a dividir a los pacientes en epilepsias parciales del lóbulo frontal y temporal, se aplicaron pruebas neurocognitivas, evaluación del cociente intelectual (CI), atención, memoria de trabajo, funciones ejecutivas y ejecución visuoespacial. Además, se evaluaron con resultados de electroencefalograma, neuroimagen y examen físico. Resultados. Se evaluaron 37 pacientes de ambos sexos (22 hombres, 15 mujeres) con epilepsia parcial del lóbulo frontal (17) y del lóbulo temporal (20). Las principales diferencias cognitivas entre estos dos tipos de epilepsia fueron: CI (promedio 82 en las epilepsias frontales y 97 en las epilepsias temporales) con mayor impacto en la memoria de trabajo y la ejecución vi-suoespacial en pequeños con epilepsia frontal. Los pacientes con epilepsia del lóbulo temporal presentaron mayores problemas en la atención de ejecución y de los test de memoria. Conclusiones. Los pacientes con epilepsia parcial del lóbulo frontal tienen mayor impacto sobre las habilidades neurocognitivas. Se considera muy importante esta evaluación con el fin de iniciar un soporte temprano con abordajes terapéuticos en este grupo de epilepsias, intentando revertir el impacto de crisis sobre las capacidades sociales y académicas.

Introduction. Objective: The purpose of this clinical trial was to differentiate the neurocognitive performance between frontal and temporal seizures in pediatric epilepsy. This is an important issue related to measure the impact of the different type of seizures in the neurodevelopment of children with epilepsy. Methods. We analyzed patients with partial epilepsy between 6 and 12 years old, both genders, in the Neurology Department of the Hospital Infantil de Mexico. After classifying frontal and temporal epilepsies, neurocognitive, IQ, attention, working memory, executive functions and visuospatial performance tests were applied. Likewise, EEG, neuroimaging, social evaluation and physical examination were performed. Results. Thirty-seven patients both genders (22 males; 15 females) with frontal partial seizures (17) and temporal partial seizures (20) were evaluated. The main neurocognitive differences between these 2 types of epilepsy were IQ (mean 82 in frontal epilepsy and 97 in temporal epilepsy) a higher impact on working memory and visuospatial performance was observed in infants with frontal epilepsy. The patients with temporal epilepsy had more problems in executing attention and long memory tests. Conclusion. Frontal partial seizures had more impact on the neurocognitive abilities than temporal partial seizures in the studied patients. This observation should be taken into account for the early treatment of children with epilepsy.

Influencia de la medicación antiepiléptica sobre los resultados escolares de niños epilépticos

Con el objetivo de conocer la influencia de la medicación antiepiléptica sobre los resultados escolares se estudiaron 30 niños que padecen crisis epilépticas parciales y que asisten a escuelas primarias normales. En entrevista familiar se recogió: medicación antiepiléptica usada, dosis en sangre y resultados académicos del último curso escolar que se correlacionó con las variables estudiadas con el test de correlación múltiple. El 80 % (24 niños) recibió tratamiento en monoterapia y 6 (20 %) politerapia. La fenitoína correlacionó significativamente (p < 0,005) con los resultados escolares M. Existe relación de los niveles elevados de droga en sangre con los peores resultados académicos.

With the aim of knowing the influence of antiepileptic drug therapy on learning outcomes, 30 children with partial epileptic crisis that go to normal primary schools, were studied. These data were collected in family interviews: antiepileptic drug therapy used, dose in blood, and academic outcomes in the last school course correlated with the variables studied with the multiple correlation test. Twenty four children (80 %) received monotherapy treatment, and six (20 %) received politherapy. Phenytoin significantly correlated (p < 0.005) with M school outcomes. There is a relation of the increased drug levels in blood with the worst academic results.

Evaluación psicopedagógica de un grupo de niños epilépticos que asisten a escuelas primarias

Se estudiaron 30 niños que padecen crisis epilépticas parciales y que asisten a escuelas primarias normales, con el objetivo de conocer si presentan dificultades escolares y en qué asignaturas, para esto se realizó una evaluación psicopedagógica. Los resultados se compara-ron con un grupo control de 30 niños para lo cual se utilizó el test de comparación de medias. Se halló que 18 niños del grupo estudio (60 %) y 9 del grupo control (30 %) presentaron alguna dificultad en el cumplimiento de los objetivos del grado escolar, con una diferencia significativa entre los grupos de p < 0,001. Se concluye que las mayores dificultades escolares se encuentran en las áreas de Lectura y Español.

Thirty children with partial epileptic crisis that go to normal primary schools were studied, with the objective of knowing if they have learning difficulties, and in which subjects; with this purpose, a psychopedagogical assessment was carried out. Results were compared with a 30 children control group, and this was made through the mean comparison test. It was found that 18 children from the study group (60 %), and 9 from the control group (30 %) presented some difficulties in achieving the goals of the school grade with a significant difference between groups of p < 0,001. It is concluded that the highest learning difficulties are in the areas of Reading and Spanish.