Probability of Remission of the Main Epileptic Syndromes in Childhood.

AIM: To determine the long-term probability of remission without antiepileptic treatment of common epileptic syndromes and of children without a specific syndromic diagnosis. PATIENTS AND METHODS: All children less than 14 years old with 2 or more unprovoked seizures seen at our hospital between June 1, 1994, and March 1, 2011 (n = 680), were included and prospectively followed up until August 15, 2020. Syndromic diagnosis was made retrospectively but blinded to subsequent evolution, employing the data available at 6 months after diagnosis and under predefined operational criteria. RESULTS: The Kaplan-Meier estimate of the probability of achieving a remission period of at least 5 years, with neither seizures nor antiepileptic treatment at 14 years was 97% for well-defined childhood epilepsy with centrotemporal spikes, 82% for uncertain childhood epilepsy with centrotemporal spikes, 85% for well-defined Panayiotopoulos syndrome, 88% for uncertain Panayiotopoulos syndrome, 93% for nonfamilial self-limited infantile epilepsy, 100% for familial self-limited infantile epilepsy, 86% for absence epilepsy, 6% for juvenile myoclonic epilepsy, 71% for cryptogenic West syndrome, 72% for patients with no associated neurologic deficits and no specific syndromic diagnosis, 65% for symptomatic West syndrome, and 40% for patients with associated neurologic deficits and no specific syndromic diagnosis. CONCLUSIONS: The study results highlight the long-term outcomes of the main epileptic syndromes and also of the patients with no syndromic diagnosis.

Pronóstico de la epilepsia de la infancia con puntas centrotemporales: utilidad clínica de unos criterios diagnósticos objetivos / Prognosis of benign childhood epilepsy with centrotemporal spikes: clinical utility of objective diagnostic criteria

INTRODUCCIÓN: Los estudios sobre el pronóstico de la epilepsia de la infancia con puntas centrotemporales (EIPCT), la mayoría retrospectivos, incluyen a pacientes con características clínicas muy heterogéneas. OBJETIVO: Investigar el valor pronóstico de unos criterios diagnósticos objetivos de EIPCT aplicados a los seis meses de evolución de la epilepsia. PACIENTES Y MÉTODOS: Se incluyó prospectivamente a todos los pacientes con una o más crisis epilépticas no provocadas (n = 827). Se investigó el pronóstico y las características clínicas de cinco grupos de pacientes, definidos según la presencia de punta-onda centrotemporal (POCT) y de los siguientes signos: bloqueo del habla o disartria, contracciones tónicas o clónicas hemifaciales y sialorrea prominente. Grupo A (dos o tres signos y POCT), grupo B (un signo y POCT), grupo C (ningún signo y POCT), grupo D (dos o tres signos sin POCT), grupo E (crisis tonicoclónicas generalizadas y POCT). RESULTADOS: Se clasificaron 52, 12, 12, 22 y 8 casos, respectivamente, en los grupos A, B, C, D y E. Los pacientes de los cinco grupos presentaron una semiología similar, pero se observaron diferencias en la probabilidad de alcanzar una remisión inicial de tres años sin crisis ni tratamiento antiepiléptico: grupo A (98%), grupo B (92%), grupo C (90%), grupo D (77%) y grupo E (100%). La diferencia entre los grupos A y D fue estadísticamente significativa. CONCLUSIÓN: Los casos del grupo A podrían considerarse como casos bien definidos de EIPCT; los casos de los grupos B, C y E, como casos probables, y los del grupo D deberían excluirse del diagnóstico

INTRODUCTION: Studies about childhood epilepsy with centrotemporal spikes (CECTS), most of them retrospective, include patients with highly heterogeneous features. AIM: To investigate the prognostic value of objective diagnostic criteria for CECTS applied at six month of evolution of epilepsy. PATIENTS AND METHODS: All patients with one or more unprovoked epileptic seizures (n = 827) were prospectively included. We investigated prognosis and clinical features of five groups of patients defined in accordance with the presence of centrotemporal spikes (CTS) and the following signs: speech arrest or dysarthria; hemifacial tonic or clonic contractions; and prominent sialorrhea. Group A (two or three signs and CTS), group B (one sign and CTS), group C (no sign and CTS), group D (two or three signs without CTS), group E (tonic-clonic generalized seizures and CTS). RESULTS: 52, 12, 12, 22 and 8 cases were classified respectively into groups A, B, C, D and E. Patients of the five groups presented a similar semiology but differences in the probability of attaining a 3-years remission without antiepileptic treatment were observed: group A (98%), group B (92%), group C (90%), group D (77%), group E (100%). The difference between groups A and D was statistically significant. CONCLUSION. Cases of group A could be considered as well-defined cases of CECTS; cases of groups B, C and E, as probable cases of CECTS, and cases of group D must be excluded from the diagnosis

Nuevos fármacos antiepilépticos en Pediatría / New anti-epileptic drugs in Paediatrics

Se estima que unos 70 millones de personas padecen epilepsia a nivel mundial de los cuales más de la mitad son niños, en los que la prevalencia estimada se sitúa en torno al 0,5-0,8%. Aunque existen diversas terapias, el tratamiento de la epilepsia se basa mayoritariamente en fármacos, que en función de su año de comercialización se clasifican como de primera, segunda o tercera generación. En el presente artículo se revisan las principales características de los fármacos antiepilépticos de última generación (lacosamida, acetato de eslicarbazepina, brivaracetam, perampanel, retigabina, everolimus y cannabidiol) que, con excepción de la retigabina (ya no está comercializada), se consideran seguros y efectivos en población pediátrica. El everolimus y el cannabidiol tienen indicaciones muy concretas (esclerosis tuberosa, síndrome de Dravet y síndrome de Lennox Gastaut) mientras que el resto están indicados en el manejo de crisis de origen focal en niños a partir de 4 años. Estas nuevas moléculas han sido desarrolladas para aportar un perfil farmacocinético y de tolerancia superior a los fármacos previamente disponibles y es previsible que a medida que aumente su uso, se vaya perfilando y ampliando su verdadero potencial. Además, por primera vez en epileptología pediátrica, se ha utilizado la extrapolación de datos de efectividad en adultos (junto con estudios de seguridad y farmacocinética específicos en población pediátrica), para acelerar la aprobación de uso en población infantil

It is estimated that about 70 million people all over the world suffer from epilepsy, half of which are children, in whom the prevalence is around 0.5 to 0.8%. Although there are several therapies, the treatment of epilepsy is based mainly on drugs, which, depending on the year of coming onto the market are classified as first, second, or third generation. In this article, a description is presented on the main characteristics of the latest generation of anti-epileptic drugs (lacosamide, eslicarbazepine acetate, brivaracetam, perampanel, retigabine, everolimus and cannabidiol). These, with the exception of retigabine (is not yet on the market), are considered safe and effective in the paediatric population. Everolimus and cannabidiol have very specific indications (tuberous sclerosis, Dravet syndrome, and Lennox Gastaut syndrome), while the rest are indicated in the management of seizures of focal origin in children from 4 years-old. These new molecules have been developed in order to provide a pharmaceutical profile and tolerance superior to the previously available drugs, and it is forecast that as their use increases, their true potential and profile will widen. Furthermore, for the first time in Paediatric Epileptology, the extrapolation of the efficacy data in adults have been used (together with specific safety and pharmacokinetic studies in the paediatric population), in order to speed up their approval for use in the child population

[New anti-epileptic drugs in Paediatrics]. / Nuevos fármacos antiepilépticos en Pediatría.

It is estimated that about 70 million people all over the world suffer from epilepsy, half of which are children, in whom the prevalence is around 0.5 to 0.8%. Although there are several therapies, the treatment of epilepsy is based mainly on drugs, which, depending on the year of coming onto the market are classified as first, second, or third generation. In this article, a description is presented on the main characteristics of the latest generation of anti-epileptic drugs (lacosamide, eslicarbazepine acetate, brivaracetam, perampanel, retigabine, everolimus and cannabidiol). These, with the exception of retigabine (is not yet on the market), are considered safe and effective in the paediatric population. Everolimus and cannabidiol have very specific indications (tuberous sclerosis, Dravet syndrome, and Lennox Gastaut syndrome), while the rest are indicated in the management of seizures of focal origin in children from 4 years-old. These new molecules have been developed in order to provide a pharmaceutical profile and tolerance superior to the previously available drugs, and it is forecast that as their use increases, their true potential and profile will widen. Furthermore, for the first time in Paediatric Epileptology, the extrapolation of the efficacy data in adults have been used (together with specific safety and pharmacokinetic studies in the paediatric population), in order to speed up their approval for use in the child population.

Frecuencia, semiología y pronóstico del síndrome de Panayiotopoulos / Frequency, semiology and prognosis of Panayiotopoulos síndrome

Introducción. El síndrome de Panayiotopulos (SP) es un síndrome epiléptico sobre el que hasta la fecha se ha publicado únicamente un pequeño número de estudios. Objetivo. Estudiar la frecuencia, la semiología y el pronóstico del SP. Pacientes y métodos. Todos los pacientes con una o más crisis epilépticas no provocadas que consultaron en nuestro hospital entre el 1 de junio de 1994 y el 1 de marzo de 2011 (n = 827) fueron incluidos y seguidos prospectivamente hasta el 30 de abril de 2018. Se diagnosticó de SP a los pacientes que cumplieron los siguientes criterios a los seis meses de evolución: una o más crisis no provocadas con síntomas predominantemente autonómicos, presencia de complejos de puntas y ondas lentas focales de gran amplitud y «morfología funcional», ausencia de déficit neurológico previo y neuroimagen normal. Resultados. Cumplieron los criterios de SP 27 casos (3,3%). La semiología de las crisis fue similar a la descrita por otros autores. Un 88% de casos alcanzó una remisión inicial de tres años sin tratamiento antiepiléptico (sin crisis y sin tratamiento durante tres años). Sesenta y dos pacientes (7,5%) cumplieron todos los criterios de SP, a excepción de la presencia de las típicas alteraciones en el electroencefalograma. La semiología de estos casos fue similar, y un 85% alcanzó una remisión inicial de tres años sin tratamiento antiepiléptico. Conclusiones. En la práctica diaria son frecuentes los pacientes con crisis sugestivas de SP, pero sin las típicas alteraciones en el electroencefalograma. Este grupo de pacientes también presenta un buen pronóstico

Introduction. Panayiotopoulos syndrome (PS) is an epileptic syndrome of childhood. Until now only a small number of studies have been published about this syndrome. Aim. To study the frequency, semiology and prognosis of PS. Patients and methods. all patients with one or more unprovoked seizures seen at our hospital between 1 June 1994 and 1 March 2011 (n = 827) were included and prospectively followed until 30 April 2018. A diagnosis of PS was made in patients that fulfilled all the following criteria at six month of evolution: seizures with predominantly autonomic symptoms, presence of high amplitude, ‘functional morphology’, focal spikes and slow wave complexes in any location, absence of previous neurological deficit and normal neuroimaging. Results. 27 cases (3,3%) met the diagnostic criteria. Semiology of the seizures was similar to that described by other authors. 88% of these cases attained a 3-year initial remission without antiepileptic treatment (three years both without seizures and without treatment). 62 cases (7,5%) met all the diagnostic criteria with the exception of the presence of the EEG features. Semiology of these cases was similar and 85% attained a 3-year initial remission without antiepileptic treatment. Conclusions. In daily practice, patients with a clinical picture suggestive of PS but without the typical EEG features are common. This group of patients also have a good outcome

Frecuencia, semiología y pronóstico de la epilepsia infantil benigna / Frequency, semiology and prognosis of benign infantile epilepsy

Introducción. La epilepsia infantil benigna es un síndrome epiléptico sobre el que hasta ahora se ha publicado tan sólo un pequeño número de series de casos. Objetivo. Estudiar la frecuencia, semiología y pronóstico de la epilepsia infantil benigna. Pacientes y métodos. Los 827 pacientes con una o más crisis epilépticas no provocadas que consultaron en nuestro hospital entre el 1 de junio de 1994 y el 1 de marzo de 2011 fueron incluidos y seguidos prospectivamente. Se diagnosticaron de epilepsia infantil benigna los pacientes que cumplieron los siguientes criterios a los seis meses de evolución: una o más crisis focales o generalizadas, inicio antes de los 24 meses, ausencia de déficits neurológicos y electroencefalograma y neuroimagen normales. Resultados. Cumplieron los criterios diagnósticos 77 casos (9%). La semiología de las crisis fue similar a la de otras crisis focales en niños menores de 24 meses. Un 25% de los pacientes permaneció como con crisis aisladas. Entre los de dos o más crisis epilépticas, la probabilidad de alcanzar una remisión inicial de tres años sin tratamiento antiepiléptico fue del 86%. En el subgrupo de pacientes con crisis focales sin antecedentes familiares, la probabilidad fue del 74%, y en cinco casos se detectó posteriormente un retraso psicomotor o discapacidad intelectual. Conclusiones. La epilepsia infantil benigna es un síndrome epiléptico frecuente. La semiología de las crisis no es útil para caracterizar el síndrome. El diagnóstico de epilepsia infantil benigna a los seis meses de evolución implica un pronóstico razonablemente bueno, pero posiblemente no tanto como el de otras epilepsias autolimitadas de la infancia

Introduction. Benign infantile epilepsy is an epileptic syndrome of infancy. Until now, only a small number of case-series have been published. Aim. To study the frequency, semiology and prognosis of benign infantile epilepsy. Patients and methods. The 827 patients with one or more epileptic seizures seen at our hospital between 1 June 1994 and 1 March 2011 were included and prospectively followed. A diagnosis of benign infantile epilepsy was made in patients that fulfilled the following criteria at six month of evolution: one or more focal and/or generalised seizures, onset before 24 months, no neurological deficit and normal neuroimaging and interictal EEG. Results. 77 cases (9%) met the diagnostic criteria. Semiology of the seizures was similar to that of other focal seizures in children under 24 months. 25% of the patients remained as isolated seizures. Among those with two or more seizures, the probability of achieving a 3 year initial remission without antiepileptic treatment was 86%. In the subgroup of patients with focal seizures without family history the probability was 74% and in five cases a global developmental delay/intellectual disability was detected thereafter. Conclusions. Benign infantile epilepsy is a frequent epileptic syndrome. Semiology of seizures is not useful to characterize the syndrome. A diagnosis of benign infantile epilepsy at six month of evolution implies a reasonably good prognosis, but possibly not as good as for other self-limited epilepsies of infancy

Neuropsychological assessment and perinatal risk: A study amongst very premature born 4- and 5-year old children.

BACKGROUND: Prematurity and its consequences are serious problems that can result in numerous neurosensory disabilities and cerebral cognitive dysfunctions. The Perinatal Risk Index (PERI) might provide a predictive measure of these problems. AIM: This study compared the cognitive development of prematurely born children at 4 and 5 years of age with age-matched peers born at term. The secondary objective was to determine whether a correlation exists between perinatal risk and performance on neuropsychological tests among premature children. METHODS: A total of 54 children between four and five years of age were evaluated; 27 were born very premature (premature group; PG), and 27 were born at term (term group; TG). Executive function, attention, memory, language, visual perception, and spatial structuring were evaluated. Subtests from the Kaufman Assessment Battery for Children, the Rey Complex Figure Test, the McCarthy Scales of Children's Abilities, the Peabody Picture Vocabulary Test, Test A, Trails A and B, the spatial structuring questionnaire from the Child Neuropsychological Maturity Questionnaire, and the Wechsler Intelligence Scale for Children were used. A PERI score was also obtained for the PG. RESULTS: The PG showed significantly lower scores than the TG in all the studied cognitive domains. Visual-perceptive scores were significantly and negatively correlated with the PERI scores of the PG. CONCLUSIONS: The PG showed neurocognitive deficits compared with the TG. The PERI can be used to predict the development of visual-perceptive abilities in children between four and five years of age.

Clinical rating scale for pantothenate kinase-associated neurodegeneration: A pilot study.

BACKGROUND: Pantothenate kinase-associated neurodegeneration is a progressive neurological disorder occurring in both childhood and adulthood. The objective of this study was to design and pilot-test a disease-specific clinical rating scale for the assessment of patients with pantothenate kinase-associated neurodegeneration. METHODS: In this international cross-sectional study, patients were examined at the referral centers following a standardized protocol. The motor examination was filmed, allowing 3 independent specialists in movement disorders to analyze 28 patients for interrater reliability assessment. The scale included 34 items (maximal score, 135) encompassing 6 subscales for cognition, behavior, disability, parkinsonism, dystonia, and other neurological signs. RESULTS: Forty-seven genetically confirmed patients (30 ± 17 years; range, 6-77 years) were examined with the scale (mean score, 62 ± 21; range, 20-106). Dystonia with prominent cranial involvement and atypical parkinsonian features were present in all patients. Other common signs were cognitive impairment, psychiatric features, and slow and hypometric saccades. Dystonia, parkinsonism, and other neurological features had a moderate to strong correlation with disability. The scale showed good internal consistency for the total scale (Cronbach's α = 0.87). On interrater analysis, weighted kappa values (0.30-0.93) showed substantial or excellent agreement in 85% of the items. The scale also discriminated a subgroup of homozygous c.1583C>T patients with lower scores, supporting construct validity for the scale. CONCLUSIONS: The proposed scale seems to be a reliable and valid instrument for the assessment of pediatric and adult patients with pantothenate kinase-associated neurodegeneration. Additional validation studies with a larger sample size will be required to confirm the present results and to complete the scale validation testing. © 2017 International Parkinson and Movement Disorder Society.

Individualised prediction model of seizure recurrence and long-term outcomes after withdrawal of antiepileptic drugs in seizure-free patients: a systematic review and individual participant data meta-analysis.

BACKGROUND: People with epilepsy who became seizure-free while taking antiepileptic drugs might consider discontinuing their medication, with the possibility of increased quality of life because of the elimination of adverse events. The risk with this action, however, is seizure recurrence. The objectives of our study were to identify predictors of seizure recurrence and long-term seizure outcomes and to produce nomograms for estimation of individualised outcomes. METHODS: We did a systematic review and meta-analysis, and identified eligible articles and candidate predictors, using PubMed and Embase databases with a last update on Nov 6, 2014. Eligible articles had to report on cohorts of patients with epilepsy who were seizure-free and had started withdrawal of antiepileptic drugs; articles also had to contain information regarding seizure recurrences during and after withdrawal. We excluded surgical cohorts, reports with fewer than 30 patients, and reports on acute symptomatic seizures because these topics were beyond the scope of our objective. Risk of bias was assessed using the Quality in Prognosis Studies system. Data analysis was based on individual participant data. Survival curves and proportional hazards were computed. The strongest predictors were selected with backward selection. Models were converted to nomograms and a web-based tool to determine individual risks. FINDINGS: We identified 45 studies with 7082 patients; ten studies (22%) with 1769 patients (25%) were included in the meta-analysis. Median follow-up was 5·3 years (IQR 3·0-10·0, maximum 23 years). Prospective and retrospective studies and randomised controlled trials were included, covering non-selected and selected populations of both children and adults. Relapse occurred in 812 (46%) of 1769 patients; 136 (9%) of 1455 for whom data were available had seizures in their last year of follow-up, suggesting enduring seizure control was not regained by this timepoint. Independent predictors of seizure recurrence were epilepsy duration before remission, seizure-free interval before antiepileptic drug withdrawal, age at onset of epilepsy, history of febrile seizures, number of seizures before remission, absence of a self-limiting epilepsy syndrome, developmental delay, and epileptiform abnormality on electroencephalogram (EEG) before withdrawal. Independent predictors of seizures in the last year of follow-up were epilepsy duration before remission, seizure-free interval before antiepileptic drug withdrawal, number of antiepileptic drugs before withdrawal, female sex, family history of epilepsy, number of seizures before remission, focal seizures, and epileptiform abnormality on EEG before withdrawal. Adjusted concordance statistics were 0·65 (95% CI 0·65-0·66) for predicting seizure recurrence and 0·71 (0·70-0·71) for predicting long-term seizure freedom. Validation was stable across the individual study populations. INTERPRETATION: We present evidence-based nomograms with robust performance across populations of children and adults. The nomograms facilitate prediction of outcomes following drug withdrawal for the individual patient, including both the risk of relapse and the chance of long-term freedom from seizures. The main limitations were the absence of a control group continuing antiepileptic drug treatment and a consistent definition of long-term seizure freedom. FUNDING: Epilepsiefonds.

Encefalopatías epilépticas / Epileptic encephalopathies

Según la Comisión de Clasificación y Terminología de la Liga Internacional contra la Epilepsia (ILAE), el término ‘encefalopatía epiléptica’ refleja la noción de que la actividad epiléptica en sí misma puede contribuir a la génesis de graves discapacidades cognitivas o comportamentales, más allá de lo que sería de esperar de la patología subyacente a la epilepsia. No obstante, en muchos casos resulta difícil deslindar la contribución relativa de las crisis epilépticas y la causa subyacente en la génesis de los déficits cognitivos. Algunos síndromes epilépticos, como los de West, Lennox-Gastaut o Dravet, se asocian con una alta probabilidad de rasgos encefalopáticos. Las causas más frecuentes de encefalopatía epiléptica son la encefalopatía hipóxico-isquémica, las malformaciones cerebrales, incluyendo las displasias corticales, las alteraciones cromosómicas o genéticas, la esclerosis tuberosa y las enfermedades metabólicas (AU)

According to the International League Against Epilepsy’s (ILAE) Commission on Classification and Terminology, the term ‘epileptic encephalopathy’ reflects the notion that epileptic activity in itself can contribute to the genesis of severe cognitive or behavioural disabilities, beyond what could be expected from the pathology underlying the epilepsy. However, in many cases it is difficult to define the boundary between the relative contribution of the epileptic seizures and the underlying cause in the genesis of cognitive deficits. Some epileptic syndromes, such as those of West, Lennox-Gastaut or Dravet, are associated to a high probability of encephalopathic traits. The most frequent causes of epileptic encephalopathy are hypoxic-ischaemic encephalopathy, brain malformations, including cortical dysplasias, chromosomal or genetic disorders, tuberous sclerosis and metabolic diseases (AU)

Evaluación neuropsicológica de procesos cognitivos en niños de siete años de edad nacidos pretérmino / Neuropsychological assessment of cognitive processes in seven-yearold children born prematurely

Introducción. Diversas investigaciones han demostrado que el desarrollo cognitivo de los niños prematuros suele verse afectado, existiendo una estrecha relación entre prematuridad, déficits ejecutivos, trastornos del aprendizaje, alteraciones del lenguaje y problemas conductuales. Asimismo, destacan también las dificultades en tareas viso-perceptivas y de integración funcional. Objetivo. Evaluar los procesos cognitivos (funciones ejecutivas, integración viso-perceptiva, coordinación viso-motora, memoria, conocimiento del medio, lenguaje, procesamiento visomotor y aptitud motora) en niños de siete años de edad nacidos pretérmino y compararlos con un grupo control, igualado en las variables nivel sociocultural, sexo y edad. Sujetos y método. Participaron 20 niños con edades comprendidas entre siete años y dos meses y siete años y 11 meses, distribuidos homogéneamente en dos grupos (prematuro vs. control). Se elaboró un protocolo de pruebas neuropsicológicas estandarizadas para evaluar los diferentes dominios cognitivos mencionados. Resultados. Los datos obtenidos evidencian una peor ejecución en los niños prematuros en diversos dominios evaluados (v.g., funciones ejecutivas y procesamiento visomotor) en comparación con los controles. Conclusiones. Debido al escaso número de trabajos que analizan procesos cognitivos en niños nacidos pretérmino de habla hispana, en esta investigación se seleccionó la ventana de edad de 7 años, momento en el que se inician procesos esenciales -lectura, escritura y cálculo matemático-, para detectar problemas neuropsicológicos que pudiesen estar influyendo en los procesos académicos, aportando de este modo nuevos hallazgos para el ámbito de la neuropsicología infantil

Introduction. Several studies have shown that the cognitive development of premature children is affected. In fact, it appears to be a close relationship between prematurity, executive deficits, learning disorders, language disorders and behavioral problems. Premature children present also difficulties in viso-perceptive tasks and functional integration tasks. Objective. The main goal of this study was to assess cognitive processes (executive functions, viso-perceptual integration, viso-motor coordination, memory, environmental knowledge, language, viso-motor processing and motor skills) in seven-year-old children who were born preterm and to compare them with those of a control group, matching in sociocultural level, sex and age. Subjects and method. 20 children ranging in age between seven years and two months and seven years and 11 months, assigned to two groups (premature vs. control) participated in the present study. A protocol including standardized neuropsychological tests was designed to assess the different cognitive mentioned domains. Results. We observed that there was worse performance in several evaluated domains (v.g., executive functions and viso-motor processing) in premature children than in the control group. Conclusions. There are few studies that explore cognitive processes in hispanic-speaking children born prematurely. In the present study we selected the seven-year age window, a time in life in which essential processes begin –reading, writing and mathematical calculation-. To detect neuropsychological problems that might be influencing the academic achievement of this population, adding new findings to the field of child neuropsychology

[Combined treatment with antiepileptic drugs. Andalusian Epilepsy Guide 2015]. / Tratamiento combinado con fármacos antiepilépticos. Guía Andaluza de Epilepsia 2015.

AIMS: The aim of this study was to draw up a set of recommendations based on scientific evidence and in agreement with authors and reviewers, which address fundamental issues concerning the combination of antiepileptic drugs. DEVELOPMENT: A committee of 11 experts belonging to the Sociedad Andaluza de Epilepsia (SAdE--Andalusian Epilepsy Society), of whom seven were neurologists, three were neuropaediatricians and one was a neurologist-neurophysiologist, all of them with long experience in epilepsy, promoted a comprehensive literature review among 55 experts in epilepsy who were members of the SAdE, with the aim of searching for any evidence that might be available on diagnostic or therapeutic matters in epilepsy. The guidelines were set out in 35 chapters. One of the chapters addressed the combination of antiepileptic drugs in the treatment of epilepsy. Taking 77 bibliographical references and the consensus view of authors and reviewers as their starting point, a set of easily applicable recommendations were drawn up. CONCLUSIONS: Combining antiepileptic drugs in patients with epilepsy whose seizures are not controlled with a single drug can, on many occasions, result in their going back into remission. There are a series of factors related with the type of epilepsy and characteristics of the patient and with the antiepileptic drugs to be used in combination that may favour a successful therapeutic outcome. Over-treatment with the combination of antiepileptic drugs must be avoided as far as possible. The results of this review provide a set of recommendations regarding combined treatment with antiepileptic drugs, based on scientific evidence and the agreement of authors, that are simple, useful and easy to apply at the different levels of healthcare.

TITLE: Tratamiento combinado con farmacos antiepilepticos. Guia Andaluza de Epilepsia 2015.Objetivo. Elaborar unas recomendaciones basadas en evidencias cientificas y en consenso de los autores y revisores, que aborden las cuestiones basicas acerca de la combinacion de farmacos antiepilepticos. Desarrollo. Un comite de 11 expertos pertenecientes a la Sociedad Andaluza de Epilepsia (SAdE), constituido por siete neurologos, tres neuropediatras y un neurologo-neurofisiologo, todos con especial competencia en epilepsia, promovieron la realizacion de una revision bibliografica exhaustiva entre 55 expertos en epilepsia pertenecientes a la SAdE, en busca de evidencias disponibles relacionadas con temas diagnosticos o terapeuticos en epilepsia. La guia se estructuro en 35 capitulos. Uno de los capitulos abordo la combinacion de farmacos antiepilepticos en el tratamiento de la epilepsia. Basandose en 77 citas bibliograficas y en la opinion consensuada de autores y revisores, se confecciono una serie de recomendaciones de facil aplicacion. Conclusiones. La combinacion de farmacos antiepilepticos en los pacientes con epilepsia cuyas crisis no estan controladas con un solo farmaco puede conseguir en numerosas ocasiones que entren en remision. Existe una serie de factores relacionados con el tipo de epilepsia y caracteristicas del paciente y con los farmacos antiepilepticos que se van a utilizar en combinacion que pueden favorecer el exito terapeutico. Se debe evitar en lo posible el sobretratamiento con la combinacion de farmacos antiepilepticos. Los resultados de esta revision proveen unas recomendaciones sobre el tratamiento combinado con farmacos antiepilepticos, basadas en evidencias cientificas y en el consenso de los autores, utiles, sencillas y aplicables en los diferentes niveles asistenciales.

Tratamiento combinado con fármacos antiepilépticos. Guía Andaluza de Epilepsia 2015 / Combined treatment with antiepileptic drugs. Andalusian Epilepsy Guide 2015

Objetivo. Elaborar unas recomendaciones basadas en evidencias científicas y en consenso de los autores y revisores, que aborden las cuestiones básicas acerca de la combinación de fármacos antiepilépticos. Desarrollo. Un comité de 11 expertos pertenecientes a la Sociedad Andaluza de Epilepsia (SAdE), constituido por siete neurólogos, tres neuropediatras y un neurólogo-neurofisiólogo, todos con especial competencia en epilepsia, promovieron la realización de una revisión bibliográfica exhaustiva entre 55 expertos en epilepsia pertenecientes a la SAdE, en busca de evidencias disponibles relacionadas con temas diagnósticos o terapéuticos en epilepsia. La guía se estructuró en 35 capítulos. Uno de los capítulos abordó la combinación de fármacos antiepilépticos en el tratamiento de la epilepsia. Basándose en 77 citas bibliográficas y en la opinión consensuada de autores y revisores, se confeccionó una serie de recomendaciones de fácil aplicación. Conclusiones. La combinación de fármacos antiepilépticos en los pacientes con epilepsia cuyas crisis no están controladas con un solo fármaco puede conseguir en numerosas ocasiones que entren en remisión. Existe una serie de factores relacionados con el tipo de epilepsia y características del paciente y con los fármacos antiepilépticos que se van a utilizar en combinación que pueden favorecer el éxito terapéutico. Se debe evitar en lo posible el sobretratamiento con la combinación de fármacos antiepilépticos. Los resultados de esta revisión proveen unas recomendaciones sobre el tratamiento combinado con fármacos antiepilépticos, basadas en evidencias científicas y en el consenso de los autores, útiles, sencillas y aplicables en los diferentes niveles asistenciales (AU)

Aim. To draw up a set of recommendations based on scientific evidence and in agreement with authors and reviewers, which address fundamental issues concerning the combination of antiepileptic drugs. Development. A committee of 11 experts belonging to the Sociedad Andaluza de Epilepsia (SAdE - Andalusian Epilepsy Society), of whom seven were neurologists, three were neuropaediatricians and one was a neurologist-neurophysiologist, all of them with long experience in epilepsy, promoted a comprehensive literature review among 55 experts in epilepsy who were members of the SAdE, with the aim of searching for any evidence that might be available on diagnostic or therapeutic matters in epilepsy. The guidelines were set out in 35 chapters. One of the chapters addressed the combination f antiepileptic drugs in the treatment of epilepsy. Taking 77 bibliographical references and the consensus view of authors and reviewers as their starting point, a set of easily applicable recommendations were drawn up. Conclusions. Combining antiepileptic drugs in patients with epilepsy whose seizures are not controlled with a single drug can, on many occasions, result in their going back into remission. There are a series of factors related with the type of epilepsy and characteristics of the patient and with the antiepileptic drugs to be used in combination that may favour a successful therapeutic outcome. Over-treatment with the combination of antiepileptic drugs must be avoided as far as possible. The results of this review provide a set of recommendations regarding combined treatment with antiepileptic drugs, based on scientific evidence and the agreement of authors, that are simple, useful and easy to apply at the different levels of healthcare (AU)

A study of drug-resistant childhood epilepsy testing the new ILAE criteria.

PURPOSE: To test the new ILAE definition of drug-resistant epilepsy in a cohort study. METHODS: All children younger than 14 with two or more unprovoked seizures observed at our hospital between 1994 and 2008 were included. RESULTS: Five hundred and eight patients were followed for an average of 90 months (range 24-168). The probabilities of achieving seizure freedom, according to the ILAE criteria, with the first, second, third and fourth and subsequent therapeutic regimens were 65%, 29%, 27% and 21%, respectively. In the cohort, 87 patients met the criteria for drug-resistant epilepsy, which represents 19% of the treated patients (n=459) and 17% of the overall sample. The probability of meeting the criteria for drug-resistant epilepsy was 11%, 11% and 13% at 2, 6 and 10 years respectively. Sixty two percent of drug resistant cases were younger than 4 years old, 73% had an associated developmental delay and/or motor deficit, 42% had an identifiable structural cause of epilepsy and 32% had a specific epileptic syndrome. For drug-resistant patients who tried additional therapeutic regimens, the probability of achieving a seizure-free state without further recurrences was 23% and 27% at three and five years, respectively. CONCLUSIONS: Compared with more stringent criteria, the new ILAE criteria classify a greater number of patients with drug-resistant epilepsy. A significantly higher proportion of cases meeting this definition subsequently enter remission. A definition of drug-resistance that includes the additional criteria of failure of a third antiepileptic drug or high seizure frequency may better identify patients with truly drug-resistant epilepsy.

Training with differential outcomes enhances discriminative learning and visuospatial recognition memory in children born prematurely.

Previous studies have demonstrated that discriminative learning is facilitated when a particular outcome is associated with each relation to be learned. When this training procedure is applied (the differential outcome procedure; DOP), learning is faster and more accurate than when the more common non-differential outcome procedure is used. This enhancement of accuracy and acquisition has been called the differential outcome effect (DOE). Our primary purpose in the present study was to explore the DOE in children born with great prematurity performing a discriminative learning task (Experiment 1) or a delayed visuospatial recognition task (Experiment 2). In Experiment 1, participants showed a faster learning and a better performance when differential outcomes were used. In Experiment 2, a significant DOE was also observed. That is, premature children performed the visuospatial recognition task better when they received differential outcomes following their correct responses. By contrast, the overall performance of full-term children was similar in both differential and non-differential conditions. These results are first to show that the DOP can enhance learning of conditional discriminations and recognition memory in children born prematurely with very low birth-weight.

[The use of bibliographic information resources and Web 2.0 by neuropaediatricians]. / Uso de recursos de información bibliográfica y Web 2.0 por neuropediatras.

AIM: To determine the state of knowledge and use of the main sources of bibliographic information and Web 2.0 resources in a sample of pediatricians linked professionally to child neurology. SUBJECTS AND METHODS: Anonymous opinion survey to 44 pediatricians (36 neuropediatric staffs and 8 residents) with two sections: sources of bibliographic information: (25 questions) and Web 2.0 resources (14 questions). RESULTS: The most consulted journals are Revista de Neurología and Anales de Pediatría. All use PubMed database and less frequently Índice Médico Español (40%) and Embase (27%); less than 20% use of other international and national databases. 81% of respondents used the Cochrane Library, and less frequently other sources of evidence-based medicine: Tripdatabase (39%), National Guideline Clearinghouse (37%), Excelencia Clínica (12%) and Sumsearch (3%). 45% regularly receive some e-TOC (electronic table of contents) of biomedical journals, but only 7% reported having used the RSS (really system syndication). The places to start searching for information are PubMed (55%) and Google (23%). The four resources most used of Web 2.0 are YouTube (73%), Facebook (43%), Picasa (27%) and blogs (25%). We don't found differences in response between the group of minus or equal to 34 and major or equal to 35 years. CONCLUSIONS: Knowledge of the patterns of use of information databases and Web 2.0 resources can identify the limitations and opportunities for improvement in the field of pediatric neurology training and information.