[Ten years' experience with vagus nerve stimulation in a paediatric population]. / Diez años de experiencia con el estimulador del nervio vago en una poblacion pediatrica.

INTRODUCTION: Fifty million people are affected by epilepsy. Up to 30% are not controlled with the aid of antiepileptic drugs. The vagus nerve stimulator (VNS) is a therapeutic alternative that must be taken into account. AIMS: To determine the effect of the VNS in a cohort of paediatric patients with refractory epilepsy. PATIENTS AND METHODS: A retrospective study of children with a VNS implanted between 2008 and 2017 in a tertiary hospital. Epidemiological, aetiological, clinical and electrophysiological data, along with VNS parameters were analysed. RESULTS: The study included 35 patients, with a mean age when the VNS was implanted of 12.84 years (range: 3.1-18.7 years) and a mean time between onset of epilepsy and implantation of 7.2 years (range: 1.3-17.7 years). The causation was structural in 62.9% of cases. The most frequent epileptic conditions were: Lennox-Gastaut syndrome and focal epilepsy, with a predominance of tonic seizures (57.1%). The video electroencephalogram showed multifocal anomalies (54%) and a pattern of epileptic encephalopathies (34.3%). Intellectual disability was associated in 94% of the cases. The mean of previous antiepileptic drugs was 9.6 ± 3 (range: 4-16). 43% responded to treatment (>= 50% reduction in number of seizures), with a mean reduction of 67.3%, which improved with higher ages of onset of epilepsy. Three patients were seizure-free (8.5%). The number of seizures decreased by 33% at six months and by 47.4% at 24 months. There was also a notable degree of cognitive (57%) and behavioural improvement (53%). In 28% of cases there were some side effects, but in general they were mild. CONCLUSIONS: The VNS is a valid option in refractory epilepsy, with improvements not only in terms of seizures but also regarding cognitive-behavioural aspects, this being very important for the paediatric population.

TITLE: Diez años de experiencia con el estimulador del nervio vago en una poblacion pediatrica.Introduccion. La epilepsia afecta a 50 millones de personas. Hasta un 30% no se controla con farmacos antiepilepticos. El estimulador del nervio vago (ENV) constituye una alternativa terapeutica que hay que valorar. Objetivo. Determinar el efecto del ENV en una cohorte pediatrica con epilepsia refractaria. Pacientes y metodos. Estudio retrospectivo de niños con ENV implantado entre 2008 y 2017 en un hospital terciario. Se han analizado datos epidemiologicos, etiologicos, clinicos, electrofisiologicos y parametros del ENV. Resultados. Se incluyo a 35 pacientes, con una mediana de edad de implantacion de 12,84 años (rango: 3,1-18,7 años) y una mediana de evolucion entre el inicio de la epilepsia y la implantacion de 7,2 años (rango: 1,3-17,7 años). La etiologia fue estructural en el 62,9% de los casos. Cuadros epilepticos mas frecuentes: sindrome de Lennox-Gastaut y epilepsia focal, con predominio de las crisis tonicas (57,1%). El videoelectroencefalograma mostro anomalias multifocales (54%) y un patron de encefalopatia epileptica (34,3%). El 94% asociaba discapacidad intelectual. La media de farmacos antiepilepticos previos fue de 9,6 ± 3 (rango: 4-16). El 43% fueron respondedores (>= 50% reduccion de crisis), con una media de reduccion del 67,3%, mejor cuanto mayor era la edad de inicio de la epilepsia. Tres pacientes quedaron libres de crisis (8,5%). La reduccion de crisis fue del 33% a los 6 meses y del 47,4% a los 24 meses. Mejoria cognitiva (57%) y conductual (53%). El 28% tuvo efectos secundarios, generalmente leves. Conclusiones. El ENV es una opcion valida en la epilepsia refractaria con mejoria no solo de las crisis, sino tambien cognitiva y conductual, con la importancia que ello tiene para la poblacion pediatrica.

Specificity of electroclinical features in the diagnosis of ring chromosome 20.

BACKGROUND: Ring chromosome 20 (R20) syndrome is a chromosomal disorder characterized mainly by drug-resistant frontal lobe seizures, recurrent nonconvulsive status epilepticus (NCSE), and typical EEG features. The aim of this study was to investigate if this triad is common and specific to all patients with R20. METHODS: In this cross-sectional study (from 2000 to 2011), we selected patients who fulfilled at least two out of three criteria: drug-resistant frontal lobe seizures, recurrent NCSE, and characteristic electroencephalography (EEG) features. In all patients, diagnosis was based on karyotype analysis of at least 100 metaphases. RESULTS: We identified 36 patients who met at least two of the selected criteria: six patients (16.7%) with R20 and 30 (83.3%) without R20 (non-R20). All patients with R20 met all three criteria. Eleven (36.7%) patients without R20, however, also displayed the full triad. In 19 patients without R20 (63.3%), one of the three clinical features was missing: frontal lobe seizures were not resistant to antiepileptic drugs (AED) in four (13.3%), recurrent NCSE was missing in six (20%), and nine (30%) patients did not have typical EEG features. Based on this data, specificity was 63.3%, positive predictive value was 35.3%, and sensitivity and negative predictive values were 100%. Additionally, a review of all publications describing the R20 phenotype revealed that 81.98% of patients with R20 display the full electroclinical triad. CONCLUSIONS: In our study, all patients with R20 displayed the three electroclinical characteristics. This is in line with previous reports (presenting high sensitivity and negative predictive value). However, these features can also be observed in other epilepsies and are not specific to R20. Our findings suggest that in the presence of the full triad of symptoms, karyotype analysis focused on chromosome 20 should be conducted.

¿Cómo conectar con un adolescente? / How to connect with and adolescent?

No disponible

Functional surgery in pediatric drug-resistant posterior cortex epilepsy: Electro-clinical findings, cognitive and seizure outcome.

PURPOSE: Epilepsies originated from the occipital, parietal and/or the posterior edge of the temporal lobe are grouped together into posterior cortex epilepsy (PCE). Our objective was firstly to describe electro-clinical and imaging findings in the presurgical evaluation of children with PCE, and secondly to identify potential factors associated with surgical and cognitive outcomes. METHOD: From the total of patients referred to the Epilepsy Monitoring Unit of 'Hospital Universitario Niño Jesús' from 2003 to 2016, 55 had drug-resistant PCE. Different variables obtained from the multimodal presurgical work-up were analyzed among patients achieving seizure freedom after surgery (ILAE class 1) and patients with persistent seizures. Categorical variables were compared with Fishers exact test and numeric variables with t-Student for independent samples, and multiple logistic regression were used to analyze predictive values. RESULTS: Median duration of epilepsy until surgery was 5 years [3-10 years]. Fifty patients showed lesions in the MRI, and 62.5% had concordant MRI-PET corregistration. 37 (67%) patients were operated (lesionectomy in 21 subjects, tailored resection based on intracranial studies in 16), and 23 (62,2%) reached ILAE class 1, with a mean follow-up period of 3.51 [1-12] years. A lower number of basal seizures and antiepileptic drugs, a well-defined lesion on the MRI, an epileptogenic zone (EZ) restricted to the posterior quadrant and the normalization of postsurgical EEGs were associated with seizure freedom (p<0.05). Additionally, 65% of patients had a long-term improvement of cognitive performances. CONCLUSIONS: Epilepsy surgery should be considered in children with drug-resistant PCE, especially in those with a restricted EZ.

[Reversible neuropsychological deterioration associated to zonisamide in a paediatric patient with tuberous sclerosis]. / Deterioro neuropsicologico reversible asociado a zonisamida en un paciente pediatrico con esclerosis tuberosa.

AIM: To document reversible cognitive deterioration associated to high doses of zonisamide, using the Reliable Change Index to control practice effects derived from repetitive neuropsychological assessments. CASE REPORT: A 11 year-old boy with tuberous sclerosis complex and left frontal refractory epilepsy, evaluated within a paediatric epilepsy surgery program. The epileptogenic zone was found to be related with a tuber situated on the left inferior frontal gyrus. The effects of high doses of zonisamide simulate a disturbance of eloquent cortex within the epileptogenic zone and the impact of uncontrolled seizures on cognitive functioning over the language-dominant hemisphere. Drug withdrawal significantly improved total intelligence index, verbal comprehension intellectual index and specific language-sustained cognitive abilities, beyond practice effects. CONCLUSIONS: The differentiation between cognitive effects of drugs and functional deficits resulting from eloquent cortex involvement within the epileptogenic zone can be of crucial importance in the decision-making process for epilepsy surgery.

TITLE: Deterioro neuropsicologico reversible asociado a zonisamida en un paciente pediatrico con esclerosis tuberosa.Objetivo. Documentar el deterioro cognitivo reversible asociado a altas dosis de zonisamida, utilizando indices de cambio fiable para controlar los efectos de practica derivados de evaluaciones neuropsicologicas repetidas. Caso clinico. Niño de 11 años con complejo esclerosis tuberosa y epilepsia refractaria del lobulo frontal izquierdo, evaluado en el contexto de un programa de cirugia de la epilepsia pediatrica. La zona epileptogena se relaciono con un tuber epileptogeno localizado en el giro frontal inferior del hemisferio izquierdo. Los efectos de altas dosis de zonisamida mimetizaron una afectacion de la corteza elocuente en la zona epileptogena y un impacto de las crisis no controladas en el funcionamiento cognitivo asociado al hemisferio dominante para el lenguaje. La retirada del farmaco mejoro significativamente, mas alla de los efectos de practica, el cociente intelectual total, el indice intelectual de comprension verbal y habilidades cognitivas especificas sustentadas en el lenguaje. Conclusiones. La diferenciacion entre los efectos cognitivos de los farmacos y la existencia de un deficit funcional por afectacion de la corteza elocuente en el area epileptogena puede ser crucial para la toma de decisiones en cirugia de la epilepsia.

Deterioro neuropsicológico reversible asociado a zonisamida en un paciente pediátrico con esclerosis tuberosa / Reversible neuropsychological deterioration associated to zonisamide in a paediatric patient with tuberous sclerosis

Objetivo. Documentar el deterioro cognitivo reversible asociado a altas dosis de zonisamida, utilizando índices de cambio fiable para controlar los efectos de práctica derivados de evaluaciones neuropsicológicas repetidas. Caso clínico. Niño de 11 años con complejo esclerosis tuberosa y epilepsia refractaria del lóbulo frontal izquierdo, evaluado en el contexto de un programa de cirugía de la epilepsia pediátrica. La zona epileptógena se relacionó con un túber epileptógeno localizado en el giro frontal inferior del hemisferio izquierdo. Los efectos de altas dosis de zonisamida mimetizaron una afectación de la corteza elocuente en la zona epileptógena y un impacto de las crisis no controladas en el funcionamiento cognitivo asociado al hemisferio dominante para el lenguaje. La retirada del fármaco mejoró significativamente, más allá de los efectos de práctica, el cociente intelectual total, el índice intelectual de comprensión verbal y habilidades cognitivas específicas sustentadas en el lenguaje. Conclusiones. La diferenciación entre los efectos cognitivos de los fármacos y la existencia de un déficit funcional por afectación de la corteza elocuente en el área epileptógena puede ser crucial para la toma de decisiones en cirugía de la epilepsia (AU)

Aim. To document reversible cognitive deterioration associated to high doses of zonisamide, using the Reliable Change Index to control practice effects derived from repetitive neuropsychological assessments. Case report. A 11 year-old boy with tuberous sclerosis complex and left frontal refractory epilepsy, evaluated within a paediatric epilepsy surgery program. The epileptogenic zone was found to be related with a tuber situated on the left inferior frontal gyrus. The effects of high doses of zonisamide simulate a disturbance of eloquent cortex within the epileptogenic zone and the impact of uncontrolled seizures on cognitive functioning over the language-dominant hemisphere. Drug withdrawal significantly improved total intelligence index, verbal comprehension intellectual index and specific languagesustained cognitive abilities, beyond practice effects. Conclusions. The differentiation between cognitive effects of drugs and functional deficits resulting from eloquent cortex involvement within the epileptogenic zone can be of crucial importance in the ecision-making process for epilepsy surgery (AU)

Influence of Picual olive ripening on virgin olive oil alteration and stability during potato frying.

Ripening modifies oil attributes and composition. However, the influence of olive ripening on virgin olive oil (VOO) thermal oxidative stability on food-frying has not been studied yet. Oils from Picual olives of low (VOO1), medium (VOO2), and high (VOO3) ripeness were obtained, and their thermal oxidative stability during 40 potato-fryings was tested. Unused VOO1 showed higher antioxidant content and oxidative stability than VOO2 and VOO3. Polar compounds (PC), oligomers, and altered fatty acid methyl esters (polar-FAME) increased, whereas linoleic acid, polyphenols, and tocopherols decreased in the three VOOs through frying. The alteration was lower in VOO1, followed by VOO2 (0.105, 0.117, and 0.042 g/100 g oil less of PC, oligomers and polar-FAME per frying, respectively, in VOO1 than in VOO3). In conclusion, VOO obtained from low-ripeness Picual olives should be preferred when frying fresh-potatoes due to its higher thermal and oxidative stability, permitting a higher number of potato-frying uses.

Epilepsia mioclónica benigna del lactante: evolución natural y pronóstico neurocognitivo y conductual / Benign myoclonic epilepsy in infancy: natural history and behavioral and cognitive outcome

Introducción. La epilepsia mioclónica benigna del lactante (EMBL) es un síndrome electroclínico de características homogéneas y bien definidas, considerado clásicamente de buen pronóstico. Sin embargo, en los últimos años se han publicado estudios con resultados variables en cuanto a evolución neuropsicológica. Objetivo. Analizar la evolución natural y el pronóstico neurocognitivo y conductual de los pacientes con EMBL. Pacientes y métodos. Estudio retrospectivo de 10 pacientes con EMBL, con un período de seguimiento de más de cinco años, durante los cuales se realizó una evaluación neurocognitiva y conductual. Resultados. En el 60% de los pacientes las crisis se controlaron con ácido valproico en monoterapia, y el 80% no presentó nuevas crisis durante su seguimiento. El cociente intelectual de la cohorte se situó entre 74 y 93; tres pacientes tuvieron un cociente intelectual en rango de inteligencia límite, y seis, en rango de inteligencia media-baja. Nueve pacientes cumplieron criterios de trastorno por déficit de atención/hiperactividad y dos asociaban otro trastorno del aprendizaje, uno de ellos trastorno de aprendizaje no verbal, y el otro, trastorno específico de la lectoescritura. Todos los pacientes presentaron datos de pobre coordinación motriz y visuoespacial, y tres fueron diagnosticados de trastorno de conducta. Conclusiones. El término ‘benigno’ en la EMBL debe utilizarse con precaución en cuanto a su pronóstico neurocognitivo y conductual. El inicio precoz y un peor control de las crisis podrían suponer factores de riesgo de evolución neuropsicológica desfavorable (AU)

Introduction. Benign myoclonic epilepsy in infancy (BMEI) is a well-defined electro-clinical syndrome, classically associated with a good prognosis. However, in the last years several studies have been published with variable results of neuropsychological outcome in BMEI. Aim. To analyze the natural history and the cognitive and behavioral outcome in BMEI patients. Patients and methods. We report a long-term follow-up of 10 patients with BMEI. During the follow-up, all the patients underwent neurocognitive and behavioral evaluations. Results. Sixty percent of patients became seizure free on valproic acid. The intelligence quotient of the whole cohort was between 74 and 93, with three patients in the range of borderline intelligence and six in the range of medium-to-low intelligence. Nine of the 10 patients met criteria for attention deficit hyperactivity disorder, and two patients associated another learning disorder. All patients showed poor motor and visuospatial coordination signs and three patients had a behavior disorder. Conclusions. The term ‘benign’ in BMEI has to be used with caution in refer to its behavioral and cognitive outcome. Early onset of seizures and a worse epilepsy control may be risk factors of a poor neuropsychological outcome (AU)

[Benign myoclonic epilepsy in infancy: natural history and behavioral and cognitive outcome]. / Epilepsia mioclónica benigna del lactante: evolución natural y pronóstico neurocognitivo y conductual.

INTRODUCTION: Benign myoclonic epilepsy in infancy (BMEI) is a well-defined electro-clinical syndrome, classically associated with a good prognosis. However, in the last years several studies have been published with variable results of neuropsychological outcome in BMEI. AIM. To analyze the natural history and the cognitive and behavioral outcome in BMEI patients. PATIENTS AND METHODS: We report a long-term follow-up of 10 patients with BMEI. During the follow-up, all the patients underwent neurocognitive and behavioral evaluations. RESULTS: Sixty percent of patients became seizure free on valproic acid. The intelligence quotient of the whole cohort was between 74 and 93, with three patients in the range of borderline intelligence and six in the range of medium-to-low intelligence. Nine of the 10 patients met criteria for attention deficit hyperactivity disorder, and two patients associated another learning disorder. All patients showed poor motor and visuospatial coordination signs and three patients had a behavior disorder. CONCLUSIONS: The term 'benign' in BMEI has to be used with caution in refer to its behavioral and cognitive outcome. Early onset of seizures and a worse epilepsy control may be risk factors of a poor neuropsychological outcome.

TITLE: Epilepsia mioclonica benigna del lactante: evolucion natural y pronostico neurocognitivo y conductual.Introduccion. La epilepsia mioclonica benigna del lactante (EMBL) es un sindrome electroclinico de caracteristicas homogeneas y bien definidas, considerado clasicamente de buen pronostico. Sin embargo, en los ultimos años se han publicado estudios con resultados variables en cuanto a evolucion neuropsicologica. Objetivo. Analizar la evolucion natural y el pronostico neurocognitivo y conductual de los pacientes con EMBL. Pacientes y metodos. Estudio retrospectivo de 10 pacientes con EMBL, con un periodo de seguimiento de mas de cinco años, durante los cuales se realizo una evaluacion neurocognitiva y conductual. Resultados. En el 60% de los pacientes las crisis se controlaron con acido valproico en monoterapia, y el 80% no presento nuevas crisis durante su seguimiento. El cociente intelectual de la cohorte se situo entre 74 y 93; tres pacientes tuvieron un cociente intelectual en rango de inteligencia limite, y seis, en rango de inteligencia media-baja. Nueve pacientes cumplieron criterios de trastorno por deficit de atencion/hiperactividad y dos asociaban otro trastorno del aprendizaje, uno de ellos trastorno de aprendizaje no verbal, y el otro, trastorno especifico de la lectoescritura. Todos los pacientes presentaron datos de pobre coordinacion motriz y visuoespacial, y tres fueron diagnosticados de trastorno de conducta. Conclusiones. El termino 'benigno' en la EMBL debe utilizarse con precaucion en cuanto a su pronostico neurocognitivo y conductual. El inicio precoz y un peor control de las crisis podrian suponer factores de riesgo de evolucion neuropsicologica desfavorable.

A role for the CXCR3/CXCL10 axis in Rasmussen encephalitis.

BACKGROUND: Rasmussen encephalitis is a devastating pediatric syndrome of unknown etiology that is characterized by progressive loss of neurological function and intractable focal epilepsy. Cytotoxic T lymphocytes have an active role in the pathogenic process of Rasmussen encephalitis. We studied the implication of CXCL10-CXCR3, a chemotactic axis involved in the pathogenesis of several cases of immune encephalitis. METHODS: We analyzed surgical specimens of children with Rasmussen encephalitis, and performed functional in vitro assays to test the implications of the pathological findings. RESULTS: We found that cytotoxic T lymphocytes infiltrating the damaged areas of primary biopsies expressed CXCR3, whereas neurons and astrocytes in the same areas expressed CXCL10. The in vitro assays demonstrated we found that astrocytes upregulated the expression of CXCL10 messenger RNA and the release of CXCL10 to the supernatants on stimulation with polyinosinic-polycyticylic acid, a synthetic double-stranded RNA that mimics infections with either RNA or DNA viruses. Activated T lymphocytes responded to the production of CXCL10 by astrocytes by increasing their migration in a transwell assay. Finally, the chemotaxis induced by the stimulated astrocytes was completely abrogated in the presence of a small molecule antagonist of CXCR3. CONCLUSIONS: Our results suggest that the CXCR3-CXCL10 axis has a role in recruiting pathogenic T lymphocytes into the brains of patients with Rasmussen encephalitis. This chemotactic mechanism may be targeted pharmacologically.

Cirugía de la epilepsia en niños con displasias corticales focales / Epilepsy surgery in children with focal cortical dysplasias

Introducción. Las displasias corticales focales (DCF) son la primera etiología de cirugía de la epilepsia pediátrica. La evaluación prequirúrgica en DCF a menudo es compleja, ya que son lesiones que pueden ser altamente epileptógenas y, a la vez, conservar función neurológica, y no visualizarse en la resonancia magnética. El éxito de la cirugía depende, en gran medida, de la adecuada identificación de la lesión y de la posibilidad de realizar una resección completa del tejido displá- sico. En este trabajo se revisa la bibliografía relacionada con el tema, en relación con la experiencia de los autores. Desarrollo. Se revisan algunos avances relacionados con la evaluación prequirúrgica y el abordaje neuroquirúrgico de la epilepsia en niños con DCF; se comentan los resultados de las series quirúrgicas en relación con las clasificaciones de DCF y los factores de pronóstico posquirúrgico; se describen algunos fenotipos anatomoelectroclínicos distintivos en niños con DCF y su abordaje quirúrgico; y se comentan brevemente los actuales retos y el futuro del tratamiento quirúrgico de la epilepsia en DCF. Conclusiones. El avance los métodos de diagnóstico prequirúrgico y de procedimientos quirúrgicos está permitiendo ofrecer un tratamiento exitoso en edades tempranas a pacientes con DCF previamente considerados ‘no lesionales’ y a pacientes con lesiones localizadas en la ‘corteza elocuente’. La identificación de fenotipos anatomoelectroclínicos de DCF permite establecer abordajes quirúrgicos y expectativas de pronóstico posquirúrgico adecuadas a cada situación, mejor en las DCF IIb transmantle y en las displasias de fondo de surco que en las DCF multilobares, en su mayoría DCF I (AU)

Introduction. Focal cortical dysplasias (FCD) are the first cause of surgery in paediatric epilepsy surgery. The pre-surgical assessment in FCD is often complex, since they are lesions that can be highly epileptogenic and at the same time can preserve neurological functioning and may not be displayed in magnetic resonance imaging. The success of the operation largely depends on the proper identification of the lesion and the possibility of performing a complete resection of the dysplastic tissue. In this work we review the literature related with this topic, in relation to the authors’ experience. Development. The study reviews some of the advances made as regards the pre-surgical assessment and the neurosurgical management of epilepsy in children with FCD; results from the surgical series regarding the classifications of FCD and the post-surgical prognostic factors are commented on; some anatomo-clinical phenotypes that are distinctive in children with FCD and their surgical management are described; and current challenges and the future of the surgical treatment of epilepsy in FCD are also briefly discussed. Conclusions. The advances being made in the methods of pre-surgical diagnosis and surgical procedures are making it possible to offer successful treatment at earlier ages in patients with FCD who were previously considered ‘non-lesional’ and in patients with localised lesions in the ‘eloquent cortex’. The identification of anatomo-electro-clinical phenotypes of FCD makes it possible to establish surgical approaches and post-surgical prognostic expectations that are well suited to each situation, which are better in the transmantle-type FCD IIb and in bottom-of-sulcus dysplasias than in multilobe FCD, which are mostly FCD I (AU)

[Epilepsy surgery in children with focal cortical dysplasias]. / Cirugía de la epilepsia en niños con displasias corticalesfocales.

INTRODUCTION: Focal cortical dysplasias (FCD) are the first cause of surgery in paediatric epilepsy surgery. The pre-surgical assessment in FCD is often complex, since they are lesions that can be highly epileptogenic and at the same time can preserve neurological functioning and may not be displayed in magnetic resonance imaging. The success of the operation largely depends on the proper identification of the lesion and the possibility of performing a complete resection of the dysplastic tissue. In this work we review the literature related with this topic, in relation to the authors' experience. DEVELOPMENT: The study reviews some of the advances made as regards the pre-surgical assessment and the neurosurgical management of epilepsy in children with FCD; results from the surgical series regarding the classifications of FCD and the post-surgical prognostic factors are commented on; some anatomo-clinical phenotypes that are distinctive in children with FCD and their surgical management are described; and current challenges and the future of the surgical treatment of epilepsy in FCD are also briefly discussed. CONCLUSIONS: The advances being made in the methods of pre-surgical diagnosis and surgical procedures are making it possible to offer successful treatment at earlier ages in patients with FCD who were previously considered 'non-lesional' and in patients with localised lesions in the 'eloquent cortex'. The identification of anatomo-electro-clinical phenotypes of FCD makes it possible to establish surgical approaches and post-surgical prognostic expectations that are well suited to each situation, which are better in the transmantle-type FCD IIb and in bottom-of-sulcus dysplasias than in multilobe FCD, which are mostly FCD I.

TITLE: Cirugia de la epilepsia en niños con displasias corticales focales.Introduccion. Las displasias corticales focales (DCF) son la primera etiologia de cirugia de la epilepsia pediatrica. La evaluacion prequirurgica en DCF a menudo es compleja, ya que son lesiones que pueden ser altamente epileptogenas y, a la vez, conservar funcion neurologica, y no visualizarse en la resonancia magnetica. El exito de la cirugia depende, en gran medida, de la adecuada identificacion de la lesion y de la posibilidad de realizar una reseccion completa del tejido displasico. En este trabajo se revisa la bibliografia relacionada con el tema, en relacion con la experiencia de los autores. Desarrollo. Se revisan algunos avances relacionados con la evaluacion prequirurgica y el abordaje neuroquirurgico de la epilepsia en niños con DCF; se comentan los resultados de las series quirurgicas en relacion con las clasificaciones de DCF y los factores de pronostico posquirurgico; se describen algunos fenotipos anatomoelectroclinicos distintivos en niños con DCF y su abordaje quirurgico; y se comentan brevemente los actuales retos y el futuro del tratamiento quirurgico de la epilepsia en DCF. Conclusiones. El avance los metodos de diagnostico prequirurgico y de procedimientos quirurgicos esta permitiendo ofrecer un tratamiento exitoso en edades tempranas a pacientes con DCF previamente considerados 'no lesionales' y a pacientes con lesiones localizadas en la 'corteza elocuente'. La identificacion de fenotipos anatomoelectroclinicos de DCF permite establecer abordajes quirurgicos y expectativas de pronostico posquirurgico adecuadas a cada situacion, mejor en las DCF IIb transmantle y en las displasias de fondo de surco que en las DCF multilobares, en su mayoria DCF I.

Analysis of genetic diversity of southern Spain fig tree (Ficus carica L.) and reference materials as a tool for breeding and conservation.

The common fig tree (Ficus carica L.) is a Mediterranean crop with problematic cultivar identification. The recovery and conservation of possible local varieties for ecological production requires the previous genetic characterization of the available germplasm. In this context, 42 lines corresponding to 12 local varieties and two caprifigs, in addition to 15 reference samples have been fingerprinted using 21 SSR markers. A total of 77 alleles were revealed, detecting a useful level of genetic variability within the local germplasm pools. UPGMA clustering analysis has revealed the genetic structure and relationships among the local and reference germplasm. Eleven of the local varieties could be identified and defined as obtained clusters, showing that SSR analysis is an efficient method to evaluate the Andalusian fig tree diversity for on-farm conservation.

Evaluación neuropsicológica prequirúrgica en epilepsias focales pediátricas / Presurgery neuropsychological evaluation in pediatric focal epilepsies

Introducción. La evaluación neuropsicológica está incorporada a los protocolos de valoración de un paciente candidato a cirugía de la epilepsia, proporciona información de las disfunciones cognitivas presentes en cada paciente, permite predecir los posibles riesgos cognitivos de la cirugía y proporciona medidas objetivas de cambio postquirúrgico. Las alteraciones neuropsicológicas son una importante comorbilidad de la epilepsia resistente a los fármacos. Su aparición precoz en la infancia puede determinar que las disfunciones cognitivas con las que cursa puedan ser atípicas con respecto a los principios de localización cerebral, debido a procesos de plasticidad y reorganización del cerebro inmaduro; el análisis de los perfiles neuropsicológicos con los que cursan las epilepsias focales pediátricas es mucho más complejo que en adultos. Desarrollo y conclusiones. En el presente trabajo se revisan las alteraciones neuropsicológicas que acompañan a las epilepsias focales de córtex posterior, temporales y frontales, señalando la escasez de trabajos publicados a pesar de que la evaluación neuropsicológica forma parte indispensable de la evaluación prequirúrgica (AU)

Introduction. Neuropsychological assessment is included in the protocols for evaluation of epilepsy surgery candidates, providing information about the patient’s cognitive dysfunctions, allowing for prediction of possible cognitive deficits derived from surgery and yielding objective measures of any post-surgical changes. Neuropsychological disturbances constitute an important co-morbidity of medically intractable epilepsy. An early epilepsy onset in infancy may lead to cognitive dysfunctions that are atypical in terms of brain localization, due to the inherent plasticity and reorganization processes of the immature brain. The analysis of the neuropsychological profiles of paediatric focal epilepsies is much more complex than in the adult population. Development and conclusions. In this paper, we review the neuropsychological disturbances associated to focal epilepsies (posterior cortex, temporal and frontal epilepsies), stressing the point that there is a considerable lack of rigorous studies on the topic in the literature, in spite of this being an essential part of the presurgical work-up in epilepsy patients (AU)

Complex motor behaviors in temporal lobe epilepsy.

Complex motor behaviors differing from typical automatisms were found in 12 of 502 patients with temporal lobe epilepsy. Movements involved proximal limb segments (6) or body axis (6) and were often preceded by auras and followed by automatisms. Seven of 12 patients are seizure free after surgery. The other 5 patients declined surgery.

[Psychosis in a case of temporal lobe epilepsy associated with a dysembryoplastic neuroepithelial tumour]. / Psicosis en un caso de epilepsia temporal asociado a un tumor neuroepitelial disembrioplasico.

INTRODUCTION: Psychosis is an acknowledged, although infrequent, complication that occurs in 0.5 9% of all cases of epilepsy. In this work, we present a case of complex partial epilepsy due to a dysembryoplastic neuroectodermal tumour in the right hippocampus, which began as acute psychosis and was cured following its surgical removal. Both its aetiology and its pathogenesis are discussed. CASE REPORT: We describe the case of a 17 year old male with a family history of schizophrenia. While studying his last years of secondary education, and apparently well adapted, he was taken into custody and hospitalised urgently because of personality disorders. The patient suffered from somatic and auditory hallucinations, simple visual pseudohallucinations, formal alterations to his thinking, cosmovisions, delusions of influence, persecution, grandeur and of a mystic nature, with no other neurological focus. At the same time, the patient also had complex partial seizures. Magnetic resonance imaging revealed a cystic tumour in the right temporal lobe. Pathology laboratory findings showed a dysembryoplastic neuroepithelial tumour. For a period of two years following surgical removal, the schizophrenic symptoms and the complex partial seizures disappeared and the patient no longer required antipsychotic medication. CONCLUSIONS: Epileptic psychosis may be related to pathological conditions of the hippocampus. In this study we present what we believe to be the first case of a dysembryoplastic neuroepithelial tumour giving rise to such psychosis. Surgical removal of the tumour cured both entities.