Evolución clínica, cognitiva y calidad de vida posquirúrgica en un caso con síndrome de Lennox Gastaut / Clinical and Cognitive Evolution and Postoperative Quality of Life in a Case with Lennox Gastaut Syndrome

Introducción: El síndrome de Lennox Gastaut se considera una encefalopatía epiléptica. Las anomalías epileptiformes en este síndrome contribuyen a la discapacidad intelectual gradual, a las comorbilidades psiquiátricas y alteraciones conductuales. En la práctica clínica aparecen atipicidades del síndrome, con focalización funcional cuyo tratamiento constituye un desafío. Objetivo: Describir la evolución clínica, cognitiva y calidad de vida en un caso con síndrome de Lennox Gastaut antes, y después del tratamiento quirúrgico. Presentación del caso: Paciente masculino de 16 años con síndrome de Lennox Gastaut. Se revisó la historia clínica y se tomaron en consideración, los resultados del video-electroencefalograma, de la resonancia magnética nuclear y de la tomografía computarizada por emisión de fotón único. Se evaluó, además, el proceder quirúrgico, la evaluación clínica y neuropsicológica. Se realizó una descripción cualitativa de la evolución del paciente a los 6 meses y al año de la intervención quirúrgica. Conclusiones: el paciente con síndrome de Lennox Gastaut presentó una evolución favorable después del tratamiento quirúrgico, lo cual se reflejó en una disminución en la frecuencia de las crisis. mejoría cognitiva, conductual y mejor calidad de vida(AU)

Introduction: Lennox Gastaut syndrome is considered an epileptic encephalopathy. Epileptiform abnormalities in this syndrome contribute to gradual intellectual disability, psychiatric comorbidities and behavioral disturbances. In clinical practice, atypicalities of the syndrome appear with functional focalization whose treatment constitutes a challenge. Objective: To describe the clinical and cognitive evolution and quality of life in a case with Lennox Gastaut syndrome before and after surgical treatment. Case presentation: A 16-year-old male patient with Lennox Gastaut syndrome. The clinical history was reviewed and the results of the video-electroencephalogram, nuclear magnetic resonance and single photon emission computed tomography were taken into consideration. The surgical procedure, clinical and neuropsychological evaluation were also evaluated. A qualitative description of the patient's evolution past 6 months and one year after surgery was prepared. Conclusions: the patient with Lennox Gastaut syndrome has a favorable evolution after surgical treatment, which is reflected in a decrease in seizure frequency, cognitive and behavioral improvement and better quality of life(AU)

Impacto psicosocial del desarrollo científico-técnico en el estudio de la epilepsia

Introducción: Se ha planteado que no existe otra enfermedad asociada a tantos problemas sociales como lo es la epilepsia. Objetivo: Describir el impacto social del desarrollo científico-técnico en el estudio de la epilepsia. Método: Se realizó una revisión narrativa a partir del estudio documental de varias fuentes bibliográficas encontradas en base de datos electrónicas. Los principales criterios de búsqueda fueron: artículos publicados en los últimos 10 años sobre impacto psicosocial del desarrollo científico-técnico en el estudio de la epilepsia. Desarrollo: El efecto sobrenatural atribuido a la epilepsia, con su consecuente repercusión social, es resultado de especulaciones provocadas por siglos de falsas creencias sobre esta enfermedad. El impacto social de la enfermedad es negativo, genera problemas financieros, de aislamiento, de exclusión social y discriminación. Se plantea que la epilepsia tiene gran influencia en todos los niveles de calidad de vida. Las anomalías detectadas por las novedosas técnicas de estudio por neuroimagen en la epilepsia se han relacionado con el deterioro cognitivo, refractariedad de la enfermedad y otros hallazgos que pueden estar relacionados indirectamente con las alteraciones psicosociales de los pacientes. Consideraciones finales: La epilepsia, además del daño orgánico, genera consecuencias psicosociales negativas que limitan el desempeño saludable de los enfermos. En los últimos años los adelantos científico-técnicos han limitado de forma parcial los efectos sociales negativos de la enfermedad con la incorporación de novedosas tecnologías para su estudio y tratamiento.

Introduction: Views has been expressed that there is no disease more linked with social problems than epilepsy. Objective: To describe the social impact of scientific and technological development on the study of epilepsy. Method: A narrative review was carried out supported on the documentary research of several bibliographic sources found in electronic databases. The main search criteria were as follow: articles published in the last 10 years, which had relation with aspects concerning the psychosocial impact of scientific and technological development on the study of epilepsy. Development: The supernatural effect attributes to epilepsy, including its social repercussions, is the result of centuries of speculative theories and false beliefs about this disease. Epilepsy has negative impact on social well-being, causing serious economic problems, isolation, social exclusion and discrimination. Epilepsy is described as a disease with a great influence on all levels of quality of life. The abnormalities detected using novel neuroimaging techniques referred to the presence of cognitive impairment, refractory period and other aspects which may be indirectly related to psychosocial alterations in patients. Final considerations: Epilepsy, in addition to its traumatic effects, has negative psychosocial consequences that affect the healthy performance of patients. In recent years, the scientific and technological advancements have partially limited the negative social effects causes by this disease with the use of new technologies for its study and treatment.

Introdução: Tem sido sugerido que não há outra doença associada a tantos problemas sociais quanto a epilepsia. Objetivo: Descrever o impacto social do desenvolvimento científico-técnico no estudo da epilepsia. Método: Realizou-se revisão narrativa a partir do estudo documental de diversas fontes bibliográficas encontradas em bases de dados eletrônicas. Os principais critérios de busca foram: artigos publicados nos últimos 10 anos sobre o impacto psicossocial do desenvolvimento científico-técnico no estudo da epilepsia. Desenvolvimento: O efeito sobrenatural atribuído à epilepsia, com sua consequente repercussão social, é fruto de especulações causadas por séculos de falsas crenças sobre essa doença. O impacto social da doença é negativo, gera problemas financeiros, isolamento, exclusão social e discriminação. Sugere-se que a epilepsia tenha grande influência em todos os níveis da qualidade de vida. As anormalidades detectadas pelas novas técnicas de estudo de neuroimagem na epilepsia têm sido relacionadas à deterioração cognitiva, refratariedade da doença e outros achados que podem estar indiretamente relacionados às alterações psicossociais dos pacientes. Considerações finais: A epilepsia, além dos danos orgânicos, gera consequências psicossociais negativas que limitam o desempenho saudável dos pacientes. Nos últimos anos, os avanços técnico-científicos limitaram parcialmente os efeitos sociais negativos da doença com a incorporação de novas tecnologias para seu estudo e tratamento.

Intracranial arachnoid cysts and epilepsy in children: Should this be treated surgically? Our 29-year experience and review of the literature / Quistes aracnoideos intracraneales y epilepsia en niños: ¿Debemos tratarlos quirúrgicamente? Nuestra experiencia de 29 años y revisión de la literatura

Introduction and objective: Arachnoid cysts (ACs) are relatively frequent lesions related to different neurological symptoms, being mostly incidentally diagnosed. This study aims to clarify whether AC surgery in epileptic patients is useful in their treatment. Material and methods: The patients registered in the database of the Neuropediatrics Section from May 1990 to August 2019 are analyzed retrospectively. Patients in whom the diagnosis of ACs and epilepsy coincide are studied. The location, size and number of ACs, neurological development, age at diagnosis, follow-up time, the performance of surgery on the cyst, evolution, anatomical relationship between brain electrical activity and location of AC, and type of epilepsy are analyzed. Results: After analyzing the database, we found 1881 patients diagnosed with epilepsy, of which 25 had at least one intracranial AC. In 9 of the patients, cerebral or genetic pathologies were the cause of epilepsy. Of the other 16, only 2 patients showed that the type of epilepsy and the epileptogenic focus coincided with the location of the AC; one of them was surgically treated without success, and the other one remained asymptomatic without receiving medical or surgical treatment(AU)

Introducción y objetivo: Los quistes aracnoideos (QAs) son lesiones relativamente comunes relacionados con diferentes síntomas neurológicos, siendo diagnosticados de forma incidental en su mayoría. Este estudio tiene como objetivo aclarar si la cirugía sobre el QA en pacientes epilépticos es útil en su tratamiento. Material y métodos: Se analizan retrospectivamente los pacientes registrados en la base de datos de la Sección de Neuropediatría desde mayo de 1990 a agosto de 2019. Se estudian los pacientes en los que coincide el diagnóstico de QA y epilepsia. Se analiza la localización, tamaño y número de los QA, el desarrollo neurológico, edad al diagnóstico, tiempo de seguimiento, realización de cirugía sobre el QA, evolución, relación anatómica entre la actividad eléctrica cerebral y la localización del QA, así como el tipo de epilepsia. Resultados: Tras el análisis de la base de datos encontramos 1.881 pacientes diagnosticados de epilepsia, entre ellos 25 con al menos un QA intracraneal. En nueve de los pacientes la patología cerebral o genética por sí misma era causa de epilepsia. De los otros 16, únicamente en dos casos se evidenció que el tipo de epilepsia y el foco epileptogénico coincidían con la localización del QA; uno de ellos fue tratado quirúrgicamente sin éxito y el otro permaneció asintomático sin recibir tratamiento médico ni quirúrgico. Conclusiones: Aunque es necesario diseñar un estudio prospectivo para establecer causalidad, los resultados de nuestro trabajo y la literatura disponible sugieren que no hay relación causal entre la presencia de QAs y epilepsia. El estudio y tratamiento de estos pacientes debería ser completado en una unidad multidisciplinar de cirugía de la epilepsia, sin asumir de inicio que el QA es la causa de la epilepsia(AU)

Stereoelectroencephalography in the preoperative assessment of patients with refractory focal epilepsy: experience at an epilepsy centre.

OBJECTIVE: Stereoelectroencephalography (SEEG) is a technique for preoperative evaluation of patients with difficult-to-localise refractory focal epilepsy (DLRFE), enabling the study of deep cortical structures. The procedure, which is increasingly used in international epilepsy centres, has not been fully developed in Spain. We describe our experience with SEEG in the preoperative evaluation of DLRFE. MATERIAL AND METHODS: In the last 8 years, 71 patients with DLRFE were evaluated with SEEG in our epilepsy centre. We prospectively analysed our results in terms of localisation of the epileptogenic zone (EZ), surgical outcomes, and complications associated with the procedure. RESULTS: The median age of the sample was 30 years (range, 4-59 years); 27 patients (38%) were women. Forty-five patients (63.4%) showed no abnormalities on brain MR images. A total of 627 electrodes were implanted (median, 9 electrodes per patient; range, 1-17), and 50% of implantations were multilobar. The EZ was identified in 64 patients (90.1%), and was extratemporal or temporal plus in 66% of the cases. Follow-up was over one year in 55 of the 61 patients undergoing surgery: in the last year of follow-up, 58.2% were seizure-free (Engel Epilepsy Surgery Outcome Scale class I) and 76.4% had good outcomes (Engel I-II). Three patients (4.2%) presented brain haemorrhages. CONCLUSION: SEEG enables localisation of the EZ in patients in whom this was previously impossible, offering better surgical outcomes than other invasive techniques while having a relatively low rate of complications.

Intracranial arachnoid cysts and epilepsy in children: Should this be treated surgically? Our 29-year experience and review of the literature.

INTRODUCTION AND OBJECTIVE: Arachnoid cysts (ACs) are relatively frequent lesions related to different neurological symptoms, being mostly incidentally diagnosed. This study aims to clarify whether AC surgery in epileptic patients is useful in their treatment. MATERIAL AND METHODS: The patients registered in the database of the Neuropediatrics Section from May 1990 to August 2019 are analyzed retrospectively. Patients in whom the diagnosis of ACs and epilepsy coincide are studied. The location, size and number of ACs, neurological development, age at diagnosis, follow-up time, the performance of surgery on the cyst, evolution, anatomical relationship between brain electrical activity and location of AC, and type of epilepsy are analyzed. RESULTS: After analyzing the database, we found 1881 patients diagnosed with epilepsy, of which 25 had at least one intracranial AC. In 9 of the patients, cerebral or genetic pathologies were the cause of epilepsy. Of the other 16, only 2 patients showed that the type of epilepsy and the epileptogenic focus coincided with the location of the AC; one of them was surgically treated without success, and the other one remained asymptomatic without receiving medical or surgical treatment. CONCLUSIONS: Although it is necessary to design a prospective study to establish causality, the results of our research and the available literature suggest that there is no causal relationship between the presence of ACs and epilepsy. The study and treatment of these patients should be carried out in a multidisciplinary epilepsy surgery unit, without initially assuming that the AC is the cause of epilepsy.

Estereoelectroencefalografía en la evaluación prequirúrgica de epilepsias focales refractarias: experiencia de un centro de epilepsia / Stereoelectroencephalography in the preoperative assessment of patients with refractory focal epilepsy: Experience at an epilepsy centre

Objetivo: La estereoelectroencefalografía (E-EEG) es una técnica de evaluación prequirúrgica en pacientes con epilepsia focal refractaria de difícil localización (EFRDL) que permite explorar con electrodos profundos regiones cerebrales de difícil acceso y la profundidad de la corteza. Esta técnica, en auge en centros internacionales, apenas se ha desarrollado en España. Describimos nuestra experiencia con la E-EEG en la evaluación de pacientes con EFRDL. Material y métodos: En los últimos 8 años, 71 pacientes con EFRDL fueron evaluados con E-EEG en nuestro centro. Analizamos prospectivamente los resultados obtenidos en la localización, los resultados quirúrgicos y las complicaciones asociadas a la técnica. Resultados: La mediana de edad fue de 30 años (rango 4-59 años), 27 pacientes eran mujeres (38%). La RM cerebral fue negativa en 45 pacientes (63,4%). Se implantaron 627 electrodos (mediana de 9 electrodos por paciente, rango 1-17), con un 50% de implantaciones multilobares. En 64 (90,1%) pacientes se localizó la zona epileptógena (ZE), siendo extratemporal o temporal plus en el 66% de los casos. En 55 pacientes de los 61 intervenidos el seguimiento fue superior al año: en el último año de seguimiento 32/55 pacientes (58,2%) estaban libres de crisis (Engel I) siendo los resultados favorables (Engel I-II) en el 76,4% de las intervenciones. Tres pacientes (4,2%) presentaron una hemorragia cerebral. Conclusión: La E-EEG permite localizar la ZE en pacientes en quienes anteriormente no era posible, ofreciendo unos resultados quirúrgicos superiores a otras técnicas invasivas y una tasa de complicaciones relativamente baja. (AU)

Objective: Stereoelectroencephalography (SEEG) is a technique for preoperative evaluation of patients with difficult-to-localise refractory focal epilepsy (DLRFE), enabling the study of deep cortical structures. The procedure, which is increasingly used in international epilepsy centres, has not been fully developed in Spain. We describe our experience with SEEG in the preoperative evaluation of DLRFE. Material and methods: In the last 8 years, 71 patients with DLRFE were evaluated with SEEG in our epilepsy centre. We prospectively analysed our results in terms of localisation of the epileptogenic zone (EZ), surgical outcomes, and complications associated with the procedure. Results: The median age of the sample was 30 years (range, 4-59 years); 27 patients (38%) were women. Forty-five patients (63.4%) showed no abnormalities on brain MR images. A total of 627 electrodes were implanted (median, 9 electrodes per patient; range, 1-17), and 50% of implantations were multilobar. The EZ was identified in 64 patients (90.1%), and was extratemporal or temporal plus in 66% of the cases. Follow-up was over one year in 55 of the 61 patients undergoing surgery: in the last year of follow-up, 58.2% were seizure-free (Engel Epilepsy Surgery Outcome Scale class I) and 76.4% had good outcomes (Engel I-II). Three patients (4.2%) presented brain haemorrhages. Conclusion: SEEG enables localisation of the EZ in patients in whom this was previously impossible, offering better surgical outcomes than other invasive techniques while having a relatively low rate of complications. (AU)

Long term outcome of functional hemispherectomy for refractory epilepsy: Experience from a single center / Resultado a largo plazo de la hemisferectomía funcional para la epilepsia refractaria: experiencia en un único centro

Background: Hemispherectomy has an established role as a treatment of last resort in patients with unilateral hemispheric lesions suffering from refractory epilepsy. Methods: Seven patients were evaluated at our Epilepsy Unit. We compared the seizure outcome at 6 months, 1, 2, 5 years post-surgery, as well as at end follow-up (mean 7.1 years) using Engel classification. Reduction of antiepileptic drugs (AEDs) was also assessed utilizing equal time frames. Results: The mean age of seizure onset was 5.4 years. Engel I was achieved in 5 patients at 6 months (71.4%). Engel at 1 year was predicted by the Engel at 6 months (p=0.013) with a similar number of patients being classified as Engel I outcome. Engel at 2 years was also predicted by Engel at 6 months and at 1 year (p=0.030). At end follow-up only 3 patients (42.9%) remained categorized as Engel I outcome. There was a trend toward a stability in Engel classification. All patients with developmental causes for their epilepsy experienced some deterioration of the surgical outcomes. Conversely, all patients with acquired causes were stable throughout follow-up. Seizure outcome at 6 months was worse in the patients who had post-op complications (p=0.044). Adult and pediatric populations did not differ significantly in any tested variable. Conclusions: Hemispherectomy is a valuable resource for seizure control in properly selected patients. Engel patient's evolution could be predicted at 6 months interval. Hemispherectomy could be considered a useful attitude in difficult cases (AU)

Antecedentes: La hemisferectomía tiene un rol establecido como último recurso de tratamiento en pacientes con lesiones hemisféricas unilaterales que padecen epilepsia refractaria. Métodos: En nuestra Unidad de Epilepsia fueron evaluados 7 pacientes. Comparamos el resultado de la crisis epiléptica a los 6 meses, 1, 2 y 5 años posteriores a la cirugía, así como durante el seguimiento final (media 7,1 años) utilizando la clasificación de Engel. También se evaluó la reducción de fármacos antiepilépticos (FAE) utilizando marcos temporales iguales. Resultados: La edad media de aparición de la crisis fue de 5,4 años. Se logró Engel I en 5 pacientes a los 6 meses (71,4%). Engel a 1año fue predicho por Engel a 6 meses (p=0,013) con un número similar de pacientes clasificados como resultado Engel I. Engel a 2 años fue también predicho por Engel a 6 meses y 1año (p=0,030). Durante el seguimiento final solo 3 pacientes (42,9%) siguieron categorizados como resultado Engel I. Se produjo una tendencia hacia la estabilidad en la clasificación Engel. Todos los pacientes con causas evolutivas para la epilepsia experimentaron cierto deterioro de los resultados quirúrgicos. Por contra, todos los pacientes con causas adquiridas permanecieron estables a lo largo del seguimiento. El resultado de las crisis a los 6 meses fue peor en los pacientes con complicaciones posquirúrgicas (p=0,044). Las poblaciones adulta y pediátrica no difirieron significativamente en ninguna de las variables probadas. Conclusiones: La hemisferectomía es un recurso válido para el control de la crisis en pacientes debidamente seleccionados. La evolución del paciente de Engel pudo predecirse a intervalos de 6 meses. La hemisferectomía podría considerarse una actitud útil en casos difíciles (AU)

Long term outcome of functional hemispherectomy for refractory epilepsy: Experience from a single center.

BACKGROUND: Hemispherectomy has an established role as a treatment of last resort in patients with unilateral hemispheric lesions suffering from refractory epilepsy. METHODS: Seven patients were evaluated at our Epilepsy Unit. We compared the seizure outcome at 6 months, 1, 2, 5 years post-surgery, as well as at end follow-up (mean 7.1 years) using Engel classification. Reduction of antiepileptic drugs (AEDs) was also assessed utilizing equal time frames. RESULTS: The mean age of seizure onset was 5.4 years. Engel I was achieved in 5 patients at 6 months (71.4%). Engel at 1 year was predicted by the Engel at 6 months (p=0.013) with a similar number of patients being classified as Engel I outcome. Engel at 2 years was also predicted by Engel at 6 months and at 1 year (p=0.030). At end follow-up only 3 patients (42.9%) remained categorized as Engel I outcome. There was a trend toward a stability in Engel classification. All patients with developmental causes for their epilepsy experienced some deterioration of the surgical outcomes. Conversely, all patients with acquired causes were stable throughout follow-up. Seizure outcome at 6 months was worse in the patients who had post-op complications (p=0.044). Adult and pediatric populations did not differ significantly in any tested variable. CONCLUSIONS: Hemispherectomy is a valuable resource for seizure control in properly selected patients. Engel patient's evolution could be predicted at 6 months interval. Hemispherectomy could be considered a useful attitude in difficult cases.

Rehabilitación neuropsicológica en pacientes pre y posquirúrgicos de lobectomía temporal por epilepsia fármaco resistente / Neuropsychological rehabilitation in pre- and post-surgical temporal lobectomy patients for drug-resistant epilepsy

Introducción. La epilepsia del lóbulo temporal suele producir déficits mnésicos, atencionales y del lenguaje. En la mayoría de los casos, se trata con fármacos an-tiepilépticos, pero falla en un tercio de ellos. Por tal razón, una opción terapéutica es la lobectomía temporal, que contribuye a menguar las crisis. Sin embargo, los procedimientos quirúrgicos pueden conllevar secuelas, entre ellas consecuencias a nivel cognitivo. Para contrarrestar dichos efectos, se acostumbra llevar a cabo una rehabilitación neuropsicológica que va en pro de recuperar, fortalecer y sostener en el tiempo habilidades que ya venían afectándose desde antes de la cirugía. Objetivo. Brindar una reflexión en torno a la intervención neuropsicológica de la epilepsia en el lóbulo temporal. Método. La reflexión sobre el tema parte de un interés clínico y posteriormente se fue ampliando a partir de la revisión de la literatura en diferentes bases de datos como PubMed, Medline y Scopus entre los años 2000 y 2021. Reflexión. Son amplias las opciones terapéuticas a nivel neuropsicológico y pueden contribuir de manera positiva en la recuperación del paciente, por lo cual los profe-sionales requieren conocer las posibilidades de ello para poder utilizar las estrategias más adecuadas según cada caso y brindar opciones que beneficien la calidad de vida, teniendo en cuenta que ninguna es más efectiva que otra. Conclusión. Como resultado, se presenta un panorama general de la rehabilitación neuropsicológica en pacientes pre y posquirúrgicos con lobectomía, haciendo énfasis en la rehabilitación neuropsicológica tradicional y la rehabilitación basada en inteli-gencia artificial, realidad virtual y computación

Introduction. Temporal lobe epilepsy usually produces mnestic, attentional, and language deficits. In most cases, it is treated with antiepileptic drugs, but one third of them fail, so one therapeutic option is temporal lobectomy, which helps to reduce seizures. However, surgical procedures can have sequelae, including cognitive con-sequences. To counteract these effects, neuropsychological rehabilitation is usually carried out in order to recover, strengthen, and sustain in time skills that were already affected before the surgery. Objective. To provide a reflection on the neuropsychological intervention of tem-poral lobe epilepsy. Method. The reflection on the subject starts from a clinical interest and was sub-sequently expanded from the review of the literature in different databases such as PubMed, Medline, and Scopus between 2000 and 2021. Reflection. There are many therapeutic options at the neuropsychological level and they can contribute positively to the patient's recovery, so professionals need to know the possibilities in order to use the most appropriate strategies according to each case and provide options that benefit the quality of life, taking into account that none is more effective than the other one.Conclusion. As a result, an overview of neuropsychological rehabilitation in pre- and post-surgical patients with lobectomy is presented, with emphasis on traditional neuropsychological rehabilitation and rehabilitation based on artificial intelligence, virtual reality, and computation

Intracranial arachnoid cysts and epilepsy in children: Should this be treated surgically? Our 29-year experience and review of the literature.

INTRODUCTION AND OBJECTIVE: Arachnoid cysts (ACs) are relatively frequent lesions related to different neurological symptoms, being mostly incidentally diagnosed. This study aims to clarify whether AC surgery in epileptic patients is useful in their treatment. MATERIAL AND METHODS: The patients registered in the database of the Neuropediatrics Section from May 1990 to August 2019 are analyzed retrospectively. Patients in whom the diagnosis of ACs and epilepsy coincide are studied. The location, size and number of ACs, neurological development, age at diagnosis, follow-up time, the performance of surgery on the cyst, evolution, anatomical relationship between brain electrical activity and location of AC, and type of epilepsy are analyzed. RESULTS: After analyzing the database, we found 1881 patients diagnosed with epilepsy, of which 25 had at least one intracranial AC. In 9 of the patients, cerebral or genetic pathologies were the cause of epilepsy. Of the other 16, only 2 patients showed that the type of epilepsy and the epileptogenic focus coincided with the location of the AC; one of them was surgically treated without success, and the other one remained asymptomatic without receiving medical or surgical treatment. CONCLUSIONS: Although it is necessary to design a prospective study to establish causality, the results of our research and the available literature suggest that there is no causal relationship between the presence of ACs and epilepsy. The study and treatment of these patients should be carried out in a multidisciplinary epilepsy surgery unit, without initially assuming that the AC is the cause of epilepsy.

Long term outcome of functional hemispherectomy for refractory epilepsy: Experience from a single center.

BACKGROUND: Hemispherectomy has an established role as a treatment of last resort in patients with unilateral hemispheric lesions suffering from refractory epilepsy. METHODS: Seven patients were evaluated at our Epilepsy Unit. We compared the seizure outcome at 6 months, 1, 2, 5 years post-surgery, as well as at end follow-up (mean 7.1 years) using Engel classification. Reduction of antiepileptic drugs (AEDs) was also assessed utilizing equal time frames. RESULTS: The mean age of seizure onset was 5.4 years. Engel I was achieved in 5 patients at 6 months (71.4%). Engel at 1 year was predicted by the Engel at 6 months (p=0.013) with a similar number of patients being classified as Engel I outcome. Engel at 2 years was also predicted by Engel at 6 months and at 1 year (p=0.030). At end follow-up only 3 patients (42.9%) remained categorized as Engel I outcome. There was a trend toward a stability in Engel classification. All patients with developmental causes for their epilepsy experienced some deterioration of the surgical outcomes. Conversely, all patients with acquired causes were stable throughout follow-up. Seizure outcome at 6 months was worse in the patients who had post-op complications (p=0.044). Adult and pediatric populations did not differ significantly in any tested variable. CONCLUSIONS: Hemispherectomy is a valuable resource for seizure control in properly selected patients. Engel patient's evolution could be predicted at 6 months interval. Hemispherectomy could be considered a useful attitude in difficult cases.

Cirugía de zona epileptogénica localizada en un área elocuente dellóbulo frontal en un adolescente con epilepsia / Epileptogenic zone surgery located in an eloquent area of the frontal lobe in an adolescent with epilepsy

Objetivo: Describir los resultados de la evaluación prequirúrgica, transquirúrgica y postquirúrgica, en un adolescente con epilepsia farmacorresistente con zona epileptogénica estimada en un área elocuente del lóbulo frontal izquierdo.Caso clínico: Paciente masculino, de 18 años, con epilepsia frontal izquierdafarmacorresistente desde los tres años, con una frecuencia de crisis de desconexión, atónicas y, en ocasiones, tónico clónico bilateral entre 30 y 40 diarias, previo a la cirugía. Laevaluación prequirúrgica identificó la zona de inicio ictal frontal izquierda, no lesional por imagen de resonancia magnética de 3 tesla. Se indicó tomografía computarizada por emisiónde fotón único interictal / ictal corregistrada con resonancia magnética, donde se identificó hiperperfusión frontal izquierda. Se realizó la cirugía con el uso de potenciales evocadossomatosensoriales para identificar el surco central, la estimulación cortical directa para mapear el área motora primaria, y la electrocorticografía transoperatoria para delimitar la zona de resección. Se empleó la técnica combinada desconectiva (callosotomía anterior) y resectiva del giro frontal superior y medio izquierdos, con modificación del patrón eléctrico en la electrocorticografía posterior a la cirugía. El resultado anatomopatológico fue displasia cortical focal tipo IA. En el seguimiento postquirúrgico, el paciente presenta solo entre 2-3 crisis semanales.Conclusiones: La cirugía de epilepsia extratemporal no lesional y con zona epileptogénica que incluye áreas elocuentes del lóbulo frontal es factible de realizar en nuestro país con mínima invasividad y buenos resultados(AU)

Objective: To describe the results of pre-surgical, trans-surgical and post-surgical assessment of an adolescent with drug-resistant epilepsy and an estimated epileptogenic zone in an eloquent area of the left frontal lobe.Clinical case: Male patient, 18 years old, who had drug-resistant left frontal epilepsy since the age of three. Before surgery, the patient suffered 30 and 40 daily disconnection episodes, atonic and, occasionally, daily bilateral clonic tonic. The pre-surgical evaluation identifiedthe left frontal, non-lesional ictal onset zone using 3-tesla magnetic resonance imaging. An interictal / ictal single photon emission computed tomography corrected with magnetic resonance imaging was indicated, which identified left frontal hyperperfusion. Surgery wasperformed using somatosensory-evoked potentials to identify the central sulcus, direct cortical stimulation to map the primary motor area, and intraoperative electrocorticography to delimit the resection area. Combined disconnective technique (anterior callosotomy) andresective technique of the left superior and middle left gyrus was used, modifying the electrical pattern in the electrocorticography after surgery. The pathological result was type IA focal cortical dysplasia. In the post-surgical follow-up, the patient only has 2-3 weekly crises.Conclusions: Surgery is feasible in a non-lesional extra-temporal epilepsy with an epileptogenic zone that includes eloquent frontal lobe areas in our country with minimal invasiveness and good results(AU)

Stereoelectroencephalography in the preoperative assessment of patients with refractory focal epilepsy: Experience at an epilepsy centre. / Estereoelectroencefalografía en la evaluación prequirúrgica de epilepsias focales refractarias: experiencia de un centro de epilepsia.

OBJECTIVE: Stereoelectroencephalography (SEEG) is a technique for preoperative evaluation of patients with difficult-to-localise refractory focal epilepsy (DLRFE), enabling the study of deep cortical structures. The procedure, which is increasingly used in international epilepsy centres, has not been fully developed in Spain. We describe our experience with SEEG in the preoperative evaluation of DLRFE. MATERIAL AND METHODS: In the last 8 years, 71 patients with DLRFE were evaluated with SEEG in our epilepsy centre. We prospectively analysed our results in terms of localisation of the epileptogenic zone (EZ), surgical outcomes, and complications associated with the procedure. RESULTS: The median age of the sample was 30 years (range, 4-59 years); 27 patients (38%) were women. Forty-five patients (63.4%) showed no abnormalities on brain MR images. A total of 627 electrodes were implanted (median, 9 electrodes per patient; range, 1-17), and 50% of implantations were multilobar. The EZ was identified in 64 patients (90.1%), and was extratemporal or temporal plus in 66% of the cases. Follow-up was over one year in 55 of the 61 patients undergoing surgery: in the last year of follow-up, 58.2% were seizure-free (Engel Epilepsy Surgery Outcome Scale class I) and 76.4% had good outcomes (Engel I-II). Three patients (4.2%) presented brain haemorrhages. CONCLUSION: SEEG enables localisation of the EZ in patients in whom this was previously impossible, offering better surgical outcomes than other invasive techniques while having a relatively low rate of complications.

[Neuronavigation in the surgical planning of callosotomy]. / Utilidad de la neuronavegación en la planificación quirúrgica de la callosotomía.

OBJECTIVE: To describe the usefulness of 3D computer-assisted preoperative neuronavigation for stereoscopic location of the venous sinuses, arterial branches, and corpus callosum, to extrapolate anatomical landmarks on the surgical field and make decisions before the intervention. METHODS: A prospective analysis was performed on patients with refractory epilepsy who underwent neuronavigation-assisted callosotomy (BRAIN LAB Dual). RESULTS: A total of 10 neuronavigation-assisted callosotomies were performed in the year 2014. The ages of the patients (4 males and 6 females) were between 4 and 13 years (mean 7; SD 3.02). The most common indication for callosotomy in our sample was Lennox Gastoux (5 patients). A right parasagittal craniotomy was performed in 8 patients. An anterior two-thirds callosotomy was performed in 8 patients and anterior three-quarters in 2 patients. The mean accuracy of the neuronavigation procedure was less than 2mm. In no cases were there significant intraoperative surgical complications. CONCLUSION: Callosotomy using frameless guided neuronavigation is an accurate and safe technique in patients with epilepsy refractory to surgical resection.

Anatomic study of the optical radiation and its relationship in the temporal lobe: definition of a secure approach for amigdalohipocampectomy in epilepsy surgery / Estudio anatómico de la radiación óptica y su relación en el lóbulo temporal: definición de un abordaje seguro para amigdalohipocampectomía en cirugía de la epilepsia

Introducción/Objetivos: El lóbulo temporal anterior tiene importantes estructuras subcorticales, especialmente fibras blancas que llevan la información visual. La comprensión de esta región anatómica, importantes para la práctica microquirúrgica, se basa en técnicas de disección de fibras. Ellos proporcionan perspectiva tridimensional de esta región y añaden un enfoque quirúrgico exitoso para el tratamiento de las lesiones temporales mesiales. El propósito de este trabajo es el estudio de la anatomía de la pared lateral del ventrículo lateral con el fin de determinar una zona libre de la radiación óptica. Métodos: Se diseccionaron diez hemisferios cerebrales, preparados de acuerdo con técnicas de Klingler. Se utilizan espátulas de madera con puntas de diferentes tamaños. La radiación óptica fue delimitada y las medidas se tomaron a partir de esta estructura para el polo temporal, que se utiliza como punto de referencia. Resultados: Abordajes para el cuerno temporal superior a 27 mm más allá del polo temporal pueden cruzar asa de Meyer y determinar un perjuicio a la radiación óptica con los consiguientes déficits en los campos visuales. Conclusión: La determinación de la zona de libre de fibras de radiación óptica es factible. En este trabajo se podría inferir que el área libre de la radiación óptica se encuentra en la región anterioinferior del lóbulo temporal a una distancia de hasta 2,7 centímetros desde el polo temporal y permite el acceso a el hipocampo y la amígdala durante la cirugía de la epilepsia. Resecciones más grandes que estas medidas permiten aclarar de una lesión a la radiación óptica con los consiguientes déficits en los campos visuales.

Introduction/Objective: The anterior temporal lobe has important subcortical structures, especially white fibers that lead visual information. Understanding this anatomical region, important for microsurgical practice, is based on fibers dissection techniques. They provide three-dimensional perspective for this region and add a successful surgical approach for the treatment of mesial temporal lesions. The purpose of this paper is to study the anatomy of the lateral wall of the lateral ventricle in order to determine a free area of the optical radiation. Methods: Ten cerebral hemispheres were dissected, prepared according to Klingler´s techniques. Wooden spatulas with tips of various sizes were used. The optical radiation was delimited and measures were taken from this structure to the temporal pole, used as a reference point. Results: Approaches to the temporal horn larger than 27 mm beyond the temporal pole can cross Meyer´s loop and determine injury to the optical radiation with consequent postoperatively deficits in visual fields. Conclusion: The determination of free area of optical radiation fibers is feasible. In this work we could infer that free area of optical radiation is located in the anterioinferior region of the temporal lobe at a distance of up to 2.7 centimeters from the temporal pole and allows access to the hippocampus and amygdala during epilepsy surgery. Larger resections than these measures can possibly determine injury to the optical radiation with consequent deficits in visual fields.

Epilepsia refractaria / Refractory epilepsy

La epilepsia refractaria o farmacorresistente es una condición frecuente en nuestra práctica neurológica cotidiana. Se considera que aproximadamente el 30% de pacientes con epilepsia de cualquier tipo van a persistircon crisis epilépticas a pesar de la terapia farmacológica. La definición de epilepsia farmacorresistente ha sido motivo de controversia entre los grupos de investigación a lo largo de las últimas décadas. La nueva propuesta de la Liga Internacional contra la Epilepsia (ILAE) para definir la falla del tratamiento médico y facilitar la selección de pacientes candidatos a procedimientos quirúrgicos es un gran avance hacia un mejor tratamiento para esta población. El objetivo del presente artículo es dar pautas que le permitan al neurólogo clínico identificar a los pacientes con epilepsia refractaria y dirigir los estudios diagnósticos que permitan seleccionar aquellos candidatos a un procedimiento quirúrgico curativo o paliativo. El éxito de dichos procedimientos está condicionado por la precisión diagnóstica en el protocolo prequirúrgico, donde el neurólogo desempeña un papel fundamental.

Drug resistant epilepsy is a very frequent condition seen at our clinical practice. Despite of antiepileptic drugsabout 30% of patients suffering from epilepsy will continue having seizures even if they are in combined schemes. The definition of pharmacoresistant epilepsy is subject of current controversy between researchgroups. However, the International League against Epilepsy (ILAE) has published recently a new consensusproposal defining drug resistant epilepsy. This new definition permits to select the group of patients that couldbe candidates to surgery. The aim of this review is to guide neurologists on the selection of patients sufferingfrom drug resistant epilepsy and to describe the main diagnosis procedures needs as preoperative assessment.

Displasias corticales como causa de epilepsia y sus representaciones en las imágenes: [revisión] / Cortical dysplasia as a cause of epilepsy and its images respresentation: [review]

Epilepsy is a chronic neurological disorder characterized by spontaneous recurrent seizures, which are clinically classified as generalized or partial. Approximately, 30 percent of patients with partial epilepsy is refractory to medical treatment. Within the refractory group we must discard the presence of cortical dysplasia as an underlying cause of the crisis. Cortical dysplasias are a type of malformations of cortical development (MCD) that are increasingly recognized as a cause of refractory epilepsy. From the radiological point of view this kind of pathology is of particular interest since imaging manifestations can be subtle or may show completely normal examinations. The aim of this paper is to review the literature, describing the imaging appearance of the normal cortical development, the classifications of cortical malformations, mainly cortical dysplasias, by highlighting the most frequent radiological signs. We also examine the current role of positron emission tomography (PET) in epilepsy, which in conjunction with magnetic resonance imaging findings and electrophysiological studies are used to define a possible surgical treatment. Through this treatment we expect to be provided with details of histopathological alterations found in the surgical specimen to be compared to the radiographic changes revealed in the pre-surgical study.

La epilepsia es una alteración neurológica crónica caracterizada por crisis convulsivas recurrentes y espontáneas, que clínicamente se clasifican como generalizadas o parciales, dentro de las cuales aproximadamente el 30 por ciento de los pacientes con epilepsia parcial son refractarios al tratamiento médico. Dentro del grupo refractario debemos descartar la presencia de una displasia cortical como causa subyacente de las crisis. Las displasias corticales son un tipo de malformaciones del desarrollo cortical que en forma cada vez más frecuente se reconocen como causante de epilepsia refractaria. Desde el punto de vista radiológico, este tipo de patología tiene especial interés debido a que las manifestaciones imaginológicas pueden ser sutiles o presentar exámenes completamente normales. El objetivo de este artículo es realizar una revisión de la literatura, describiendo el desarrollo cortical normal con su aspecto en imágenes, las clasificaciones de las malformaciones corticales y en especial de las displasias corticales, destacando los signos radiológicos más frecuentes. Además revisaremos el rol en la actualidad de la Tomografía por Emisión de Positrones (PET) en epilepsia, que en conjunto con las imágenes por resonancia magnética y los estudios electrofisiológicos se utilizan para definir un eventual tratamiento quirúrgico, el que una vez realizado nos da detalles del análisis de las alteraciones histopatológicas en la pieza quirúrgica versus las alteraciones radiológicas visualizadas en el estudio pre-quirúrgico.

Volumetría y electroencefalografía digital en pacientes con epilepsiadel lóbulo temporal medial farmacorresistente sometidos a cirugía / Volumetric measurement and digital electroencephalography in patients with medication-resistant medial temporal lobe epilepsy submitted to surgery

Objetivo. Evaluar la contribución de la volumetría mediante resonancia magnética (RM) y el electroencefalograma(EEG) interictal en la evaluación pre y posquirúrgica de pacientes con epilepsia del lóbulo temporal medial (ELTM) farmacorresistente sometidos a cirugía. Pacientes y métodos. Se evaluaron 12 estudios volumétricos por RM y 24 registros de EEG digital correspondientes a seis pacientes con crisis parciales complejas refractarias a tratamiento médico de origen temporal.Se realizó el análisis volumétrico por RM, para el estudio de la zona epileptogénica, y se calculó la frecuencia de aparición de descargas epileptiformes interictales (DEI/minuto) antes, a los seis meses y al año de la cirugía, y se correlacionó el análisis volumétrico con la zona irritativa y epileptogénica. Resultados. Los volúmenes de los hipocampos tanto ipsi como contralaterales están disminuidos de tamaño en comparación con el aumento de la frecuencia DEI en las zonas mesiales. Loslóbulos temporales inferiores y la corteza parahipocámpica se encontraron disminuidos de volumen ipsilaterales a la zona epileptogénica. La frecuencia de DEI disminuye a los seis meses de realizada la lobectomía temporal respecto a las DEI prequirúrgica.Se encontró correlación negativa entre el volumen resecado de la corteza parahipocámpica y el lóbulo temporalinferior con la frecuencia DEI posquirúrgica al año. Conclusiones. En pacientes con ELTM farmacorresistente se encuentran disminuidos los volúmenes de otras estructuras del lóbulo temporal medial, además del hipocampo, y se constata un menorvolumen de éstas, en el lado ipsilateral a la zona epileptogénica. El volumen de hipocampo resecado se relaciona con la frecuencia de DEI posquirúrgica de los pacientes con ELTM sometidos a lobectomía temporal exitosa. El análisis volumétrico mediante RM de la lesión epileptogénica aporta información localizadora de utilidad en la evaluación prequirúrgica de pacientes........(AU)

Aim. To assess the value of volumetric measurement by means of magnetic resonance imaging (MRI) and interictalelectroencephalogram (EEG) in pre- and post-operative assessment of patients with medication-resistant medial temporal lobe epilepsy (MTLE) who were submitted to surgery. Patients and methods. We evaluated 12 volumetric studies carried out using MRI and 24 digital EEG records for six patients suffering from complex partial seizures that were resistant to medicaltreatment and had their origin in the temporal lobe. A volumetric analysis was performed using MRI to study the epileptogenic region and the frequencies at which interictal epileptiform discharges (IED/minute) appeared before, at six months and at oneyear after surgery were calculated; a correlation was observed between the volumetric analysis and the irritative andepileptogenic region. Results. The volumes of both the ipso and contralateral hippocampuses were smaller in comparison to the increased frequency of the IED in the mesial regions. The inferior temporal lobes and the parahippocampal cortex havereduced volumes ipsolateral to the epileptogenic region. At six months after performing the temporal lobectomy, the IED frequency decreased with respect to the pre-operative IED. A negative correlation was found between the resected volume of the parahippocampal cortex and the inferior temporal lobe, and the post-operative IED frequency at one year. Conclusions. In patients with medication resistant MTLE the volumes of other structures in the medial temporal lobe are diminished, inaddition to the hippocampus, and they are seen to have a smaller volume on the side that is ipsolateral to the epileptogenic region. There is a relation between the volume of the resected hippocampus and the post-operative IED frequency in patients with MTLE who successfully underwent a temporal lobectomy. Volumetric analysis of the epileptogenic lesion using MRIprovides localising information.......(AU)