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

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.

TITLE: Pronóstico de la epilepsia de la infancia con puntas centrotemporales: utilidad clínica de unos criterios diagnósticos objetivos.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.

[Frequency, semiology and prognosis of Panayiotopoulos syndrome]. / Frecuencia, semiologia y pronostico del sindrome de Panayiotopoulos.

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 morphology¼, focal spikes and slow wave complexes in any location, absence of a 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.

TITLE: Frecuencia, semiologia y pronostico del sindrome de Panayiotopoulos.Introduccion. El sindrome de Panayiotopulos (SP) es un sindrome epileptico sobre el que hasta la fecha se ha publicado unicamente un pequeño numero de estudios. Objetivo. Estudiar la frecuencia, la semiologia y el pronostico del SP. Pacientes y metodos. Todos los pacientes con una o mas crisis epilepticas 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 diagnostico de SP a los pacientes que cumplieron los siguientes criterios a los seis meses de evolucion: una o mas crisis no provocadas con sintomas predominantemente autonomicos, presencia de complejos de puntas y ondas lentas focales de gran amplitud y «morfologia funcional¼, ausencia de deficit neurologico previo y neuroimagen normal. Resultados. Cumplieron los criterios de SP 27 casos (3,3%). La semiologia de las crisis fue similar a la descrita por otros autores. Un 88% de casos alcanzo una remision inicial de tres años sin tratamiento antiepileptico (sin crisis y sin tratamiento durante tres años). Sesenta y dos pacientes (7,5%) cumplieron todos los criterios de SP, a excepcion de la presencia de las tipicas alteraciones en el electroencefalograma. La semiologia de estos casos fue similar, y un 85% alcanzo una remision inicial de tres años sin tratamiento antiepileptico. Conclusiones. En la practica diaria son frecuentes los pacientes con crisis sugestivas de SP, pero sin las tipicas alteraciones en el electroencefalograma. Este grupo de pacientes tambien presenta un buen pronostico.

[Frequency, semiology and prognosis of benign infantile epilepsy]. / Frecuencia, semiologia y pronostico de la epilepsia infantil benigna.

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.

TITLE: Frecuencia, semiologia y pronostico de la epilepsia infantil benigna.Introduccion. La epilepsia infantil benigna es un sindrome epileptico sobre el que hasta ahora se ha publicado tan solo un pequeño numero de series de casos. Objetivo. Estudiar la frecuencia, semiologia y pronostico de la epilepsia infantil benigna. Pacientes y metodos. Los 827 pacientes con una o mas crisis epilepticas 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 evolucion: una o mas crisis focales o generalizadas, inicio antes de los 24 meses, ausencia de deficits neurologicos y electroencefalograma y neuroimagen normales. Resultados. Cumplieron los criterios diagnosticos 77 casos (9%). La semiologia de las crisis fue similar a la de otras crisis focales en niños menores de 24 meses. Un 25% de los pacientes permanecio como con crisis aisladas. Entre los de dos o mas crisis epilepticas, la probabilidad de alcanzar una remision inicial de tres años sin tratamiento antiepileptico fue del 86%. En el subgrupo de pacientes con crisis focales sin antecedentes familiares, la probabilidad fue del 74%, y en cinco casos se detecto posteriormente un retraso psicomotor o discapacidad intelectual. Conclusiones. La epilepsia infantil benigna es un sindrome epileptico frecuente. La semiologia de las crisis no es util para caracterizar el sindrome. El diagnostico de epilepsia infantil benigna a los seis meses de evolucion implica un pronostico razonablemente bueno, pero posiblemente no tanto como el de otras epilepsias autolimitadas de la infancia.

[Epileptic encephalopathies]. / Encefalopatias epilepticas.

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.

TITLE: Encefalopatias epilepticas.Segun la Comision de Clasificacion y Terminologia de la Liga Internacional contra la Epilepsia (ILAE), el termino 'encefalopatia epileptica' refleja la nocion de que la actividad epileptica en si misma puede contribuir a la genesis de graves discapacidades cognitivas o comportamentales, mas alla de lo que seria de esperar de la patologia subyacente a la epilepsia. No obstante, en muchos casos resulta dificil deslindar la contribucion relativa de las crisis epilepticas y la causa subyacente en la genesis de los deficits cognitivos. Algunos sindromes epilepticos, como los de West, Lennox-Gastaut o Dravet, se asocian con una alta probabilidad de rasgos encefalopaticos. Las causas mas frecuentes de encefalopatia epileptica son la encefalopatia hipoxico-isquemica, las malformaciones cerebrales, incluyendo las displasias corticales, las alteraciones cromosomicas o geneticas, la esclerosis tuberosa y las enfermedades metabolicas.

Síndrome del túnel carpiano como primer síntoma en las formas leves de mucopolisacaridosis / Carpal tunnel syndrome as the first symptom in mild forms of mucopolysaccharidosis

Introducción: El síndrome del túnel carpiano (STC) es una neuropatía compresiva del nervio mediano en el túnel carpiano, entidad poco frecuente en la edad pediátrica y en adultos jóvenes. Está claramente documentada la relación existente entre la aparición del STC y las enfermedades de depósito, como las mucopolisacaridosis (MPS), una de las causas que cabe tener en cuenta en el diagnóstico de STC en una persona joven. Objetivos: Estudiar la existencia de enfermedad de depósito lisosomal en pacientes afectados de STC menores de 30 años, diagnosticados en el Servicio de Neurofisiología del Hospital Torrecárdenas de Almería en los últimos 5 años (fase retrospectiva). Resultados: Se diagnosticaron 91 pacientes con STC durante el periodo 2005-2010, de los que finalmente 30 cumplieron criterios de inclusión en el estudio, con un predominio de mujeres de 20-22 y 24-27 años de edad. Se encontraron 5 casos con sospecha de enfermedad de depósito (16%), 2 de los cuales (6%) eran falsos positivos y 3 (10%) fueron diagnosticados de MPS. Conclusión: La existencia de un STC en personas menores de 30 años debe considerarse como un posible signo de alerta de una enfermedad de depósito, como la MPS (AU)

Introduction: Carpal tunnel syndrome (CTS) is a compressive neuropathy of the median nerve in the carpal tunnel, and is a rare pathology in children and young adults. The relationship between the occurrence of CTS and storage diseases such as mucopolysaccharidosis (MPS) is clearly documented, and should be considered when faced with a young person presenting with an apparently idiopathic CTS. Objectives: To study the frequency of lysosomal storage disease in patients under the age of 30 diagnosed with carpal tunnel syndrome in the past five years (retrospective phase) by the Neurophysiology Service of Hospital de Torrecárdenas (Almería). Results: 91 patients with CTS were diagnosed in the period 2005-2010, of which 30, predominantly women aged between 20-22 and 24-27 years old, met the criteria for inclusion in the study. Five patients were found with suspected lysosomal storage disease (16%) of which two (6%) were false positives and three (10%) were diagnosed with MPS. Conclusion: The existence of CTS in patients aged under 30 years should alert the physician to suspect lysosomal storage diseases, such as MPS, in the differential diagnosis of the case (AU)

Hemiparesia como signo de comienzo de la enfermedad de la sustancia blanca evanescente. Identificación de una nueva mutación / Left hemiparesis as a sign of onset of vanishing white matter disease. Identification of a new mutation

La enfermedad de la sustancia blanca evanescente es una enfermedad genética de herencia autosómica recesiva que afecta a la sustancia blanca cerebral. Existen varios fenotipos que difieren en la gravedad y la edad de inicio. Clásicamente, se caracteriza por la aparición de ataxia, espasticidad y un deterioro motor progresivo con exacerbaciones desencadenadas por procesos febriles y traumatismos craneales leves. Niña de 2,5 años, que tras traumatismo craneal leve, presentó marcha atáxica, hemiparesia izquierda y reflejos osteotendinosos exaltados. En la resonancia magnética cerebral se observó afectación difusa y simétrica de la sustancia blanca cerebral, con hiposeñal en T1 e hiperseñal en T2, y FLAIR. Se sospechó enfermedad de la sustancia blanca evanescente que se confirmó en el estudio genético al encontrar 2 mutaciones consideradas patogénicas, en el gen EIF2B5, una de ellas no descrita previamente. La hemiparesia debe incluirse entre las manifestaciones clínicas de la enfermedad de la sustancia blanca evanescente. El diagnóstico precoz puede ayudar a evitar infecciones y traumatismos, así como a realizar un adecuado consejo genético a las familias. Nuestro caso aporta además la identificación de una nueva mutación en el gen EIF2B5 (p.Gly132Ala en la posición 395), no descrita previamente, cuyas características sugieren que es patogénica con elevada probabilidad, por lo que estimamos que debería ser considerada entre las mutaciones del complejo EIF2B responsables de la enfermedad (AU)

Vanishing white matter disease is a genetic disorder of autosomal recessive inheritance that affects the brain white matter There are various phenotypes that differ in severity and age at onset. Usually, it is characterized by ataxia, spasticity and a progressive motor decline with exacerbations triggered by fever and mild head traumas. The patient was a 2.5 year-old girl who developed unstable gait, left hemiparesis and increased tendon reflexes following a mild head trauma. Brain MRI showed diffuse and symmetric white matter abnormalities with decreased signal on T1 and increased signal on T2 and FLAIR sequences. Vanishing White Matter disease was suspected. The diagnosis was confirmed by genetic molecular testing that showed 2 mutations in EIF2B5 gene. Both mutations were considered pathogenic, although one had not been previously described. Hemiparesis must be included among clinical features of vanishing white matter disease. Early diagnosis can help to avoid infections and traumas and allows families to be genetically counselled. Our case contributes with the identification of a new mutation in EIF2B5 gene (p.Gly132Ala in position 395), not previously described. Its characteristics suggest a high probability of being pathogenic. We believe that it should be considered among the complex EIF2B mutations responsible for the disease (AU)

[Left hemiparesis as a sign of onset of vanishing white matter disease. Identification of a new mutation]. / Hemiparesia como signo de comienzo de la enfermedad de la sustancia blanca evanescente. Identificación de una nueva mutación.

Vanishing white matter disease is a genetic disorder of autosomal recessive inheritance that affects the brain white matter There are various phenotypes that differ in severity and age at onset. Usually, it is characterized by ataxia, spasticity and a progressive motor decline with exacerbations triggered by fever and mild head traumas. The patient was a 2.5 year-old girl who developed unstable gait, left hemiparesis and increased tendon reflexes following a mild head trauma. Brain MRI showed diffuse and symmetric white matter abnormalities with decreased signal on T1 and increased signal on T2 and FLAIR sequences. Vanishing White Matter disease was suspected. The diagnosis was confirmed by genetic molecular testing that showed 2 mutations in EIF2B5 gene. Both mutations were considered pathogenic, although one had not been previously described. Hemiparesis must be included among clinical features of vanishing white matter disease. Early diagnosis can help to avoid infections and traumas and allows families to be genetically counselled. Our case contributes with the identification of a new mutation in EIF2B5 gene (p.Gly132Ala in position 395), not previously described. Its characteristics suggest a high probability of being pathogenic. We believe that it should be considered among the complex EIF2B mutations responsible for the disease.

¿Es preciso realizar un electrocardiograma en todos los niños con trastorno por déficit de atención e hiperactividad previo al tratamiento con psicoestimulantes? / Is it necessary to perform an electrocardiogram in all children with attention deficit disorder before starting treatment with psychostimulants?

Los autores de los estudios revisados en este artículo no encontraron diferencias en el riesgo de muerte o eventos cardiacos agudos graves entre pacientes con medicación psicoestimulante y sin ella. En conjunto, no consideran adecuada la realización de un electrocardiograma (ECG) o remitir al cardiólogo de forma rutinaria a pacientes con trastorno por déficit de atención con hiperactividad (TDAH) antes de iniciar tratamiento con medicación psicoestimulante. Consideran que la derivación debe basarse en factores de riesgo cardiovasculares detectados a través de la historia clínica o la exploración física. Comentario de los revisores: con los resultados disponibles no hay evidencia suficiente que justifique la realización de un ECG a todos los niños con TDAH antes del inicio del tratamiento con psicoestimulantes. En estos pacientes, lo prudente sería realizar una historia clínica y una exploración física (incluidas la tensión arterial y la frecuencia cardiaca) y derivar al cardiólogo solo los casos que presenten factores de riesgo cardiovascular o síntomas de enfermedad cardiaca(AU)

The authors didn't find differences on the rate of cardiovascular events or death between children exposed and unexposed to stimulant medication. They don't find appropriate to perform an electrocardiogram (EKG) or referral to cardiology of all children with attention deficit and hyperactivity disorder (ADHD) before starting stimulant medication. Cardiology referral should be considered only if cardiac risk factors are detected. Reviewer's commentary: at present there isn't enough evidence to justify the performance of an EKG as screening previous to initiate medical stimulant medication in children with ADHD. It seems reasonable to perform a careful history and physical examination (blood pressure and cardiac frequency included) and referral to cardiology only when cardiac risk factors or cardiac diseases are detected(AU)

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

Objetivo. Conocer el estado de conocimiento y utilización de las principales fuentes de información bibliográfica y recursos de la Web 2.0 en una muestra de profesionales vinculados a la neurología infantil. Sujetos y métodos. Encuesta anónima de opinión a 44 pediatras (36 adjuntos de neuropediatría y ocho residentes) con dos apartados: fuentes de información bibliográfica (25 preguntas) y recursos de la Web 2.0 (14 preguntas). Resultados. Las revistas más consultadas son Revista de Neurología y Anales de Pediatría. Todos utilizan la base de datos PubMed y, con menor frecuencia, Índice Médico Español (40%) y Embase (27%); el resto de bases internacionales y nacionales tiene una utilización inferior al 20%. El 81% de los encuestados utiliza la Cochrane Library, y con menor frecuencia otras fuentes de medicina basada en la evidencia: Tripdatabase (39%), National Guideline Clearinghouse (37%), Excelencia Clínica (12%) y Sumsearch (3%). El 45% recibe regularmente alguna tabla electrónica de contenidos (e-TOC) de revistas biomédicas, pero sólo un 7% declara haber utilizado el sistema de sindicación de contenidos (RSS). Los lugares preferidos para iniciar la búsqueda de información son PubMed (55%) y Google (23%). Los cuatro recursos de la Web 2.0 más utilizados son: YouTube (73%), Facebook (43%), Picasa (27%) y blogs (25%). No encontramos diferencias de respuesta entre el grupo menor o igual de 34 años y mayor o igual de 35 años. Conclusiones. El conocimiento de los patrones de uso de fuentes de información y recursos de la Web 2.0 permite detectar las limitaciones y oportunidades de mejora en el campo de la formación e información en neuropediatría (AU)

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 nationaldatabases. 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 (AU)

Recurrence risk after withdrawal of antiepileptic drugs in children with epilepsy: a prospective study.

AIM: To study recurrence risk after withdrawal of antiepileptic drugs in children with epilepsy. METHODS: All children younger than 14 with two or more unprovoked seizures 24h apart who were seen at our Hospital between 1994 and 2004 were included consecutively and prospectively followed. Patients previously examined in other centres were excluded. All patients who entered a remission were proposed to stop medication and were followed. RESULTS: Three hundred and fifty three children with two or more unprovoked seizures were attended. A total of 238 entered a remission period and were proposed to stop medication, 216 accept. Mean seizure-free time before medication withdrawal was 2.2 years. Kaplan-Meier estimate of recurrence risk was 23% at 2 years (95% CI: 17-29) and 28% at 5 years (95% CI: 22-34). A remote symptomatic etiology, various seizure types and a history of prior febrile seizures or prior neonatal seizures were associated with a significant increase in recurrence risk in univariable and multivariable analyses using Cox proportional hazards model. Recurrence risk at 2 years was 17% (95% CI: 11-23) for idiopathic/cryptogenic epilepsies and 41% (85% CI: 28-54) for remote symptomatic epilepsies. Recurrence risks at 2 years by epileptic syndrome were West syndrome (0%), benign rolandic epilepsy (10%), epilepsy without unequivocal partial or generalized seizures (11%), benign infantile seizures (13%), absence epilepsy (16%), cryptogenic partial epilepsies (20%), symptomatic partial epilepsies (45%), symptomatic generalized epilepsies (54%). CONCLUSIONS: Recurrence risk after withdrawal of antiepileptic treatment in children is low. Etiology and syndromic diagnosis are the main predictive factors.

Guía de práctica clínica de la Sociedad Andaluza de Epilepsia sobre profilaxis y tratamiento de las crisis epilépticas sintomáticas agudas / Clinical practice guidelines of the Andalusian epilepsy society on prophylaxis and treatment of acute symptomatic epileptic seizures

Introducción y objetivo. Los fármacos antiepilépticos (FAE) se han utilizado tradicionalmente de forma empírica para prevenir la presentación de crisis epilépticas en pacientes con afecciones cerebrales agudas durante la fase precoz o tardía. Sin embargo, los FAE no están exentos de serios inconvenientes, por lo que su empleo debería sustentarse en bases racionales y científicas. Nos proponemos la realización de una guía de práctica basada en evidencias explícitas acerca de cuándo está indicado el tratamiento profiláctico con FAE y su duración en las crisis sintomáticas agudas (CSA). Desarrollo. Realizamos una búsqueda selectiva de la información científica de calidad relacionada con el tema propuesto en PubMed-Medline, Tripdatabase y Biblioteca Cochrane Plus. Los autores analizaron y discutieron las referencias seleccionadas y se extrajeron las recomendaciones de ellas derivadas. Se identificaron 14 documentos primarios y ocho guías de práctica, protocolos o recomendaciones de expertos. Nuestras recomendaciones se recogieron al final del documento de manera explícita. Conclusiones. La Sociedad Andaluza de Epilepsia recomienda: a) emplear FAE sólo para la prevención primaria de CSA en los traumatismos craneoencefálicos graves y como prevención secundaria de nuevas CSA por otras causas de afectación cerebral aguda; b) la duración del tratamiento de las CSA no deberá superar el tiempo de resolución de la causa que las ha provocado; y c) las benzodiacepinas son los fármacos de elección para en el tratamiento de las CSA por abstinencia de alcohol y el sulfatode magnesio para las CSA de la eclampsia (AU)

Introduction and aims. Antiepileptic drugs (AED) have traditionally been used empirically to prevent the presentation of epileptic seizures in patients with acute brain disorders during the early or late phase. However, AED are not free of serious drawbacks, which means that their use should be based on solid scientific foundations. Our aim is to produce a set of practice guidelines based on explicit evidence about when prophylactic treatment with AED is indicated and the length of time it should be continued in acute symptomatic seizures (ASS). Development. A selective search for quality scientific information on the subject was conducted on PubMed-Medline, Tripdatabase and the Biblioteca Cochrane Plus. The authors discussed and analysed the references that were selected and any recommendations that could be drawn from them were collected. A total of 14 primary documents and eight practice guidelines, protocols or experts’ recommendations were identified. Our recommendations were explicitly included at the end of the document. Conclusions. The Andalusian Epilepsy Society makes the following recommendations: a) AED must only be used for the primary prevention of ASS in severe traumatic brain injury and as secondary prevention of new ASS due to other causes of acute brain damage; b) duration of treatment of ASS must not exceed the time needed to resolve the cause that gave rise to them; and c) benzodiazepines are the preferred drugs for use in the treatment of ASS due to alcohol withdrawal and magnesium sulphate for the ASS of eclampsia (AU)

[Clinical practice guidelines of the Andalusian Epilepsy Society on prophylaxis and treatment of acute symptomatic epileptic seizures]. / Guía de práctica clínica de la Sociedad Andaluza de Epilepsia sobre profilaxis y tratamiento de las crisis epilépticas sintomáticas agudas.

INTRODUCTION AND AIMS: Antiepileptic drugs (AED) have traditionally been used empirically to prevent the presentation of epileptic seizures in patients with acute brain disorders during the early or late phase. However, AED are not free of serious drawbacks, which means that their use should be based on solid scientific foundations. Our aim is to produce a set of practice guidelines based on explicit evidence about when prophylactic treatment with AED is indicated and the length of time it should be continued in acute symptomatic seizures (ASS). DEVELOPMENT: A selective search for quality scientific information on the subject was conducted on PubMed-Medline, Tripdatabase and the Biblioteca Cochrane Plus. The authors discussed and analysed the references that were selected and any recommendations that could be drawn from them were collected. A total of 14 primary documents and eight practice guidelines, protocols or experts' recommendations were identified. Our recommendations were explicitly included at the end of the document. CONCLUSIONS: The Andalusian Epilepsy Society makes the following recommendations: a) AED must only be used for the primary prevention of ASS in severe traumatic brain injury and as secondary prevention of new ASS due to other causes of acute brain damage; b) duration of treatment of ASS must not exceed the time needed to resolve the cause that gave rise to them; and c) benzodiazepines are the preferred drugs for use in the treatment of ASS due to alcohol withdrawal and magnesium sulphate for the ASS of eclampsia.

Response to sequential treatment schedules in childhood epilepsy: risk for development of refractory epilepsy.

PURPOSE: To investigate response to sequential treatment schedules and risk of development of refractory epilepsy in childhood. METHODS: All children younger than 14 years with two or more unprovoked seizures seen at our hospital between 1994 and 2004 were included and prospectively followed. "Seizure control" was defined as a 2-year seizure-free interval without further recurrences except those related to attempts of medication withdrawal and "refractory epilepsy" as failure of >2 drugs plus >1 seizure/month for > or =18 months. RESULTS: 343 Patients were included, 191 males and 152 females. Mean age at diagnosis was 4y 10 mo (SD 3 year 10 month). Mean follow-up period was 76.2 mo (SD 35.2). The probability of achieving "seizure control" was 70% and 86% at 5 and 10 years. 59% of patients were "controlled" with the first drug used. Among patients failing the first, second and third therapeutic regimen due to lack of efficacy, 39%, 23% and 12% respectively were finally "controlled" with subsequent treatment schedules Risk of development of refractory epilepsy was 8% and 12% at 6 and 10 years. CONCLUSION: After failing a first drug, a significant proportion of children can still be controlled with subsequent therapeutic schedules. Only a small proportion develops refractory epilepsy.

Early prediction of refractory epilepsy in childhood.

PURPOSE: To investigate early predictors (6 months after diagnosis) of refractory epilepsy. STUDY DESIGN: prospective cohort study. INCLUSION CRITERIA: all consecutive children <14 years with two or more unprovoked seizures 24h apart, who were seen at our hospital between 1994 and 2004. EXCLUSION CRITERIA: patients previously examined in other centres. DEFINITIONS: refractory epilepsy: failure of >2 drugs plus >1 seizure/month for >or=18 months. ANALYSIS: risk of developing refractory epilepsy was calculated using Kaplan-Meier survival curves. Univariable and multivariable analyses of potential predictors of developing refractory epilepsy were carried out using Cox proportional hazards model. RESULTS: 343 patients were included. Mean age at diagnosis was 4.8 years (+/-3.8 SD). Mean follow-up period was 76.2 (+/-35.2 SD) months (range 24-139). Risk of developing refractory epilepsy was 8% at 6 years. Risk for idiopathic syndromes was 2%. For non-idiopathic syndromes the risk was 38% for patients with age at onset <1 year plus >1 seizure during the first 6 months after diagnosis, 9% for age at onset <1 year plus 0-1 seizures during the first 6 months, 22% for age at onset >or=1 year plus >1 seizures during the first 6 months and 3% for age at onset >or=1 year plus 0-1 seizures during the first 6 months. CONCLUSION: Risk of developing refractory epilepsy is very low in idiopathic syndromes. For the rest of patients, a simple model comprising three variables allows more accurate prediction of risk of refractoriness.

Recurrence risk after a first remote symptomatic unprovoked seizure in childhood: a prospective study.

The aim of this study was to assess recurrence risk after a first remote symptomatic unprovoked seizure in childhood. All consecutive patients younger than 14 years with a first remote symptomatic unprovoked seizure who were seen at our hospital between 1994 and 2006 were included in the study and prospectively followed. Only two patients received antiepileptic treatment. Sixty-three children were included, with 35 males and 28 females. Mean age at first seizure was 4 years (SD 3y 5mo). Kaplan-Meier estimate of recurrence risk was 59% (95% confidence interval [CI] 47-71), 76% (95% CI 65-87), 85% (95% CI 76-94), and 87% (95% CI 78-96) at 6, 12, 18, and 24 months respectively. A total of 55 children out of 63 were affected by a static encephalopathy of pre- or perinatal origin. In this subgroup, recurrence risk at 12 and 24 months was 79% (95% CI 68-90) and 89% (95% CI 80-98). Univariable analysis using the Cox proportional hazards model showed that presence of global developmental delay/intellectual disability and Todd's paresis were associated with a significant increase in recurrence risk. In multivariable analysis, only Todd's paresis was significantly associated. Recurrence risk after a first remote symptomatic unprovoked seizure in childhood is much higher than what some previous studies suggests.

[The Andalusia Epilepsy Society's Guide to Epilepsy Therapy 2005: IV. General principles of antiepileptic polytherapy and therapeutic strategies in refractory epilepsy]. / Guía terapéutica en epilepsia de la Sociedad Andaluza de Epilepsia 2005: IV. Principios generales de politerapia antiepiléptica y estrategias terapéuticas en epilepsia refractaria.

AIMS: The objective of this work was to produce a scientific evidence-based guide to clinical practice dealing with the basic questions concerning the treatment of epilepsy. DEVELOPMENT: A committee of 11 experts belonging to the Andalusia Epilepsy Society, made up of six neurologists, three neuropaediatricians, one neurosurgeon and a pharmacologist, all of whom were deeply involved and experienced in epilepsy, conducted a thorough review of the literature in search of all the evidence available on the proposed subject matter. The following databases were used: MEDLINE, Cochrane Library and the databases of several clinical practice guidelines (National Guideline Clearinghouse, National Institute of Clinical Excellence and the American Academy of Neurology's Clinical Guidelines). The Guide was set out in seven sections and was published in four parts. From a total number of 187 relevant documents, the committee found 63 examples of scientific evidence and 91 therapeutic recommendations. These were tabulated and classified according to the European Federation of Neurological Societies' criteria for producing Clinical Practice Guidelines. CONCLUSIONS: The results of this survey provide scientific evidence-based clinical guidelines that are useful, simple and applicable at different levels of health care.

[The Andalusia Epilepsy Society's Guide to Epilepsy Therapy 2005: II. Chronic therapy with antiepileptic drugs in adults and in children]. / Guia terapéutica en epilepsia de la Sociedad Andaluza de Epilepsia 2005: II. Terapéutica antiepiléptica crónica en el adulto y en el nino.

AIMS: The objective of this work was to produce a scientific evidence-based guide to clinical practice dealing with the basic questions concerning the treatment of epilepsy. DEVELOPMENT: A committee of 11 experts belonging to the Andalusia Epilepsy Society, made up of six neurologists, three neuropaediatricians, one neurosurgeon and a pharmacologist, all of whom were deeply involved and experienced in epilepsy, conducted a thorough review of the literature in search of all the evidence available on the proposed subject matter. The following databases were used: MEDLINE, Cochrane Library and the databases of several clinical practice guidelines (National Guideline Clearinghouse, National Institute of Clinical Excellence and the American Academy of Neurology's Clinical Guidelines). The Guide was set out in seven sections and was published in four parts. From a total number of 187 relevant documents, the committee found 63 examples of scientific evidence and 91 therapeutic recommendations. These were tabulated and classified according to the European Federation of Neurological Societies' criteria for producing Clinical Practice Guidelines. CONCLUSIONS: The results of this survey provide scientific evidence-based clinical guidelines that are useful, simple and applicable at different levels of health care.

[The Andalusia Epilepsy Society's Guide to Epilepsy Therapy 2005: III. Antiepileptic therapy in special situations]. / Guia terapeutica en epilepsia de la Sociedad Andaluza de Epilepsia 2005: III. Tratamiento antiepileptico en situaciones especiales.

AIMS: The objective of this work was to produce a scientific evidence-based guide to clinical practice dealing with the basic questions concerning the treatment of epilepsy. DEVELOPMENT: A committee of 11 experts belonging to the Andalusia Epilepsy Society, made up of six neurologists, three neuropaediatricians, one neurosurgeon and a pharmacologist, all of whom were deeply involved and experienced in epilepsy, conducted a thorough review of the literature in search of all the evidence available on the proposed subject matter. The following databases were used: MEDLINE, Cochrane Library and the databases of several clinical practice guidelines (National Guideline Clearinghouse, National Institute of Clinical Excellence and the American Academy of Neurology's Clinical Guidelines). The Guide was set out in seven sections and was published in four parts. From a total number of 187 relevant documents, the committee found 63 examples of scientific evidence and 91 therapeutic recommendations. These were tabulated and classified according to the European Federation of Neurological Societies' criteria for producing Clinical Practice Guidelines. CONCLUSIONS: The results of this survey provide scientific evidence-based clinical guidelines that are useful, simple and applicable at different levels of health care.

Guía terapéutica en epilepsia de la Sociedad Andaluza de Epilepsia 2005: III. Tratamiento antiepiléptico en situaciones especiales / The Andalusia epilepsy society´s guide toe pilepsy therapy 2005: III. Antiepileptic therapy in special situations

Aims. The objective of this work was to produce a scientific evidence-based guide to clinical practice dealing with the basic questions concerning the treatment of epilepsy. Development. A committee of 11 experts belonging to the Andalusia Epilepsy Society, made up of six neurologists, three neuropaediatricians, one neurosurgeon and a pharmacologist, all of whom were deeply involved and experienced in epilepsy, conducted a thorough review of the literature in search of all the evidence available on the proposed subject matter. The following databases were used: MEDLINE, Cochrane Library and the databases of several clinical practice guidelines (National Guideline Clearinghouse, National Institute of Clinical Excellence and the American Academy of Neurology’s Clinical Guidelines). The Guide was set out in seven sections and was published in four parts. From a total number of 187 relevant documents, the committee found 63 examples of scientific evidence and 91 therapeutic recommendations. These were tabulated and classified according to the European Federation of Neurological Societies’ criteria for producing Clinical Practice Guidelines. Conclusions. The results of this survey provide scientific evidence-based clinical guidelines that are useful, simple and applicable at different levels of health care (AU)

Objetivo. Elaborar una guía de práctica clínica basada en la evidencia científica que aborde las cuestiones básicas acerca del tratamiento de la epilepsia. Desarrollo. Un comité de 11 expertos pertenecientes a la Sociedad Andaluza de Epilepsia, en el que se incluían seis neurólogos, tres neuropediatras, un neurocirujano y una farmacóloga, todos con especial dedicación y competencia en epilepsia, realizó una revisión bibliográfica exhaustiva en busca de las evidencias disponibles relacionadas con el tema propuesto. Se utilizaron las siguientes bases de datos: MEDLINE, Cochrane Library y bases de datos de guías de práctica clínica (National Guideline Clearinghouse, National Institute of Clinical Excellence y Guías Clínicas de la Academia Americana de Neurología). La guía se estructuró en siete secciones y se dividió para su publicación en cuatro partes. Se identificaron 187 documentos relevantes, de los que se extrajeron un total de 63 evidencias científicas y 91 recomendaciones terapéuticas, que se tabularon y clasificaron según los criterios de elaboración de Guías de Práctica Clínica de la Federación Europea de Sociedades Neurológicas. Conclusión. Los resultados de esta revisión proveen unas guías de práctica clínica basadas en la evidencia científica útiles, sencillas y aplicables en los diferentes niveles asistenciales (AU)

Guía terapéutica en epilepsia de la Sociedad Andaluza de Epilepsia 2005: IV. Principios generales de politerapia antiepiléptica y estrategias terapéuticas en epilepsia refractaria / The andalusia epilepsy society´s guide to epilesy therapy 2005: IV general principles of antiepileptic polytherapy and therapeutic strategies in refractory epilepsy

Aims. The objective of this work was to produce a scientific evidence-based guide to clinical practice dealing with the basic questions concerning the treatment of epilepsy. Development. A committee of 11 experts belonging to the Andalusia Epilepsy Society, made up of six neurologists, three neuropaediatricians, one neurosurgeon and a pharmacologist, all of whom were deeply involved and experienced in epilepsy, conducted a thorough review of the literature in search of all the evidence available on the proposed subject matter. The following databases were used: MEDLINE, Cochrane Library and the databases of several clinical practice guidelines (National Guideline Clearinghouse, National Institute of Clinical Excellence and the American Academy of Neurology’s Clinical Guidelines). The Guide was set out in seven sections and was published in four parts. From a total number of 187 relevant documents, the committee found 63 examples of scientific evidence and 91 therapeutic recommendations. These were tabulated and classified according to the European Federation of Neurological Societies’ criteria for producing Clinical Practice Guidelines. Conclusions. The results of this survey provide scientific evidence-based clinical guidelines that are useful, simple and applicable at different levels of health care (AU)

Objetivo. Elaborar una guía de práctica clínica basada en la evidencia científica que aborde las cuestiones básicas acerca del tratamiento de la epilepsia. Desarrollo. Un comité de 11 expertos pertenecientes a la Sociedad Andaluza de Epilepsia, en el que se incluían seis neurólogos, tres neuropediatras, un neurocirujano y una farmacóloga, todos con especial dedicación y competencia en epilepsia, realizó una revisión bibliográfica exhaustiva en busca de las evidencias disponibles relacionadas con el tema propuesto. Se utilizaron las siguientes bases de datos: MEDLINE, Cochrane Library y bases de datos de guías de práctica clínica (National Guideline Clearinghouse, National Institute of Clinical Excellence y Guías Clínicas de la Academia Americana de Neurología). La guía se estructuró en siete secciones y se dividió para su publicación en cuatro partes. Se identificaron 187 documentos relevantes, de los que se extrajeron un total de 63 evidencias científicas y 91 recomendaciones terapéuticas, que se tabularon y clasificaron según los criterios de elaboración de Guías de Práctica Clínica de la Federación Europea de Sociedades Neurológicas. Conclusión. Los resultados de esta revisión proveen unas guías de práctica clínica basadas en la evidencia científica útiles, sencillas y aplicables en los diferentes niveles asistenciales (AU)