[Childhood headaches: new concepts and recent advances]. / Cefaleas infantiles: nuevos conceptos y recientes avances.

This study reports on the latest advances in childhood headaches that have been made in the last few years, with special emphasis on the important new modifications that have been produced with respect to the previous ones, published in the beta version of the third edition of the International Classification of Headache Disorders. These include headaches that have undergone important qualifications, the appearance of new entities and a section which reports the existence of new headaches, but which have still not been considered as new entities. Additionally, other points that are highlighted include the strong relation between childhood headaches and attention deficit hyperactivity disorder, the weak relation between glutamate intake and the appearance of headaches, and the scant usefulness of magnetic resonance imaging of the brain in the diagnosis of these processes. Moreover, the new modification produced in childhood episodic syndromes is highlighted.

TITLE: Cefaleas infantiles: nuevos conceptos y recientes avances.En el presente trabajo se exponen los recientes avances sobre cefaleas infantiles que se han producido en los ultimos años, y se da una especial importancia a las nuevas e importantes modificaciones que se han producido con respecto a las anteriores, publicadas en la tercera edicion, version beta, de la clasificacion de la Sociedad Internacional de Cefaleas; entre ellas, cefaleas que han sufrido importantes matizaciones, la aparicion de nuevas entidades y un apartado en el que se recoge la existencia de nuevas cefaleas, pero que aun no se han considerado como nuevas entidades. Ademas, se resalta la fuerte relacion existente entre las cefaleas infantiles y el trastorno por deficit de atencion/hiperactividad, la poca relacion de la toma de glutamato con la aparicion de cefaleas y la escasa utilidad de la resonancia cerebral en el diagnostico de estos procesos. Por otra parte, se resalta la nueva modificacion producida en los sindromes episodicos de la infancia.

[West syndrome: aetiology, therapeutic options, clinical course and prognostic factors]. / Sindrome de West: etiologia, opciones terapeuticas, evolucion clinica y factores pronosticos.

INTRODUCTION: West syndrome is an age-specific form of epilepsy that associates infantile spasms, hypsarrhythmia and a delay in or the complete stoppage of psychomotor development, although this last case is not essential. AIMS: To define the profile of West syndrome in our environment by taking into account its aetiology, semiology, response to different therapeutic options and the appearance of side effects, as well as to establish prognostic factors that determine its course. PATIENTS AND METHODS: A data collection document stating the eligibility criteria was drafted. Data were collected by reviewing the medical records of patients diagnosed with West syndrome during the period between January 2003 and January 2009. Later, a statistical study was conducted with descriptive analysis and the level of statistical significance of the possible prognostic factors was established. RESULTS: The study included 70 patients. There was a predominance of symptomatic aetiology, with hypoxia-ischaemia as the main cause. Regardless of the aetiology, 58% of patients responded to treatment with vigabatrine. Over 80% of patients being treated with adrenocorticotropic hormone were finally seizure-free and without hypsarrhythmia. Almost half the patients progressed to other epilepsies. CONCLUSIONS: The statistically significant poor prognostic factors were: existence of a prenatal history, neonatal history, symptomatic aetiology, age of onset below 4 months, epileptic seizures before the onset of the spasms and outside the neonatal period, and delayed psychomotor development prior to the onset of the spasms.

Trombosis neonatal de senos venosos cerebrales / Neonatal thrombosis of the cerebral venous sinuses

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Alteraciones del gen ARX / ARX gene mutations

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Utilización de carbamazepinay oxcarbazepina en pacientespediátricos con epilepsia parcialen España. Estudio observacional / Utilization of carbamazepine and oxcarbazepine in pediatric patients with partial epilepsy in Spain. An observational study

Introducción. Es importante realizar estudios sobre la utilizaciónde los nuevos fármacos antiepilépticos para optimizar su uso. Nuestroobjetivo es describir los patrones de utilización de la carbamazepinay la oxcarbazepina.Métodos. Estudio observacional, transversal nacional con 58investigadores que incluyeron a 185 pacientes pediátricos con epilepsiaparcial. Registramos patrones de prescripción, calidad de vidamediante el Cuestionario de del niño con Epilepsia (CAVE) y utilizaciónde recursos.Resultados. Ciento treinta y cuatro pacientes estaban en tratamientocon oxcarbazepina (el 72,4%); dosis media de 22,3 mg/kg/día;desviación estándar (DE) del 8,04; intervalo de confianza (IC) 95%:20,9 a 23,7, y 51 (el 27,6%) con carbamazepina; dosis media de 14mg/kg/día; DE: 6,2; IC 95%: 12,3 a 15,8. El 19,4 y 21,6 %, respectivamente,seguía tratamiento con politerapia. Las puntuaciones mediasen dimensiones funcionales de CAVE fueron (sobre 5): asistenciaescolar: 4,5; DE: 0,7; relación social: 4,1; DE: 0,9, y autonomía: 3,9;DE: 1,9. Los pacientes en politerapia presentaron peores resultados encalidad de vida (p<0,0001) y mayor utilización de recursos sanitarios,visitas al especialista (p = 0,04) y del servicio de urgencias (32,4 frentea 7,4%).Conclusiones. La oxcarbazepina se utiliza en dosis inferiores alas recomendadas y la dosificación no se ajusta por el peso. La infradosificaciónpuede llevar a pautas de politerapia que deberían revisarseindividualmente (AU)

Introduction. It is important to conduct studies on the utilizationof new antiepileptic drugs in order to improve their use.Our objective is to describe the use patterns of carbamazepine andoxcarbazepine.Methods. Observational, cross-sectional, national study with58 investigators that included 185 pediatric patients with partialepilepsy. We recorded prescription patterns, quality of life (QoL)using the QoL scale in childhood epilepsy (CAVE) and use ofresources.Results. 134 patients were under treatment with oxcarbazepine(72.4 %), with a mean dose of 22.3 mg/kg/day; standard desviation(SD): 8.04; 95 % confidence interval (CI): 20.9 to 23.7, and51 (27.6%) with carbamazepine, mean dose of 14 mg/kg/day; SD:6.2; 95 % CI: 12.3 to 15.8. A total of 19.4% and 21.6 %, respectively,followed multiple drug treatment. The mean scores onfunctional dimensions of CAVE were (out of 5): school attendance:4.5; SD: 0.7; social relationships: 4.1; SD: 0.9, and autonomy:3.9; SD: 1.9. Patients receiving multiple drug therapy had worseresults in quality of life (p < 0.0001) and greater utilization ofhealth care resources, more visits to the specialists (p = 0.04) andto the emergency departments (32.4% vs 7.4%).Conclusions. Oxcarbazepine is used in lower doses thanrecommended and the dosing is not adjusted for weight. Underdosingmay lead to regimes of multiple drug therapy that shouldbe reviewed individually (AU)

[Utilization of carbamazepine and oxcarbazepine in pediatric patients with partial epilepsy in Spain. An observational study]. / Utilización de carbamazepina y oxcarbazepina en pacientes pediátricos con epilepsia parcial en España. Estudio observacional.

INTRODUCTION: It is important to conduct studies on the utilization of new antiepileptic drugs in order to improve their use. Our objective is to describe the use patterns of carbamazepine and oxcarbazepine. METHODS: Observational, cross-sectional, national study with 58 investigators that included 185 pediatric patients with partial epilepsy. We recorded prescription patterns, quality of life (QoL) using the QoL scale in childhood epilepsy (CAVE) and use of resources. RESULTS: 134 patients were under treatment with oxcarbazepine (72.4 %), with a mean dose of 22.3 mg/kg/day; standard deviation (SD): 8.04; 95 % confidence interval (CI): 20.9 to 23.7, and 51 (27.6%) with carbamazepine, mean dose of 14 mg/kg/day; SD: 6.2; 95 % CI: 12.3 to 15.8. A total of 19.4% and 21.6 %, respectively, followed multiple drug treatment. The mean scores on functional dimensions of CAVE were (out of 5): school attendance: 4.5; SD: 0.7; social relationships: 4.1; SD: 0.9, and autonomy: 3.9; SD: 1.9. Patients receiving multiple drug therapy had worse results in quality of life (p < 0.0001) and greater utilization of health care resources, more visits to the specialists (p = 0.04) and to the emergency departments (32.4% vs 7.4%). CONCLUSIONS: Oxcarbazepine is used in lower doses than recommended and the dosing is not adjusted for weight. Underdosing may lead to regimes of multiple drug therapy that should be reviewed individually.

[Drug regimens in patients with medication-resistant epilepsy in neurology and epilepsy outpatient departments in Spain]. / Pautas terapéuticas en el paciente con epilepsia farmacorresistente en consultas ambulatorias de neurología y epilepsia en España.

INTRODUCTION: About 30% of epileptic patients suffer from drug-resistant epilepsy (DRE). Quality of life is worse and costs are higher than in controlled epilepsy. One of the aims of the LINCE study was to assess the prevalence of DRE in epilepsy-specialized and general neurology clinics in Spain and the clinical management of these patients in routine clinical practice. PATIENTS AND METHODS: Cross-sectional, retrospective study to evaluate clinical prevalence and cost of DRE in Spain. Every participant neurologist assessed the percentage of DRE among the first 40 patients with diagnosed epilepsy seen. Patients of both sexes, older than 18 years were recruited. Their treatment before and after DRE diagnosis was analyzed. RESULTS: DRE prevalence in Spain is 22.7% (36% in epilepsy-specialized and 18.5% in neurology clinics; p < 0.0001), with no differences between genders. More than 50% of these patients have hardly achieved a secondary education and only 44% are employed. The most frequent drugs used after DRE diagnosis are lamotrigine (33.5%), levetiracetam (32.4%), carbamazepine (31.9%) and topiramate (25.8%) in various combinations, but the highest efficacy (equal or more than 50% seizures reduction) is obtained with pregabaline (53.1%), oxcarbazepine (50.6%) and levetiracetam (49.5%) and topiramate (48%). CONCLUSIONS: 22.7% of epileptic outpatients in Spain are diagnosed with DRE in clinics of neurology. These will require certain social interventions and greater use of health resources, including treatment with more appropriate AEDs. Pregabaline, oxcarbazepine, levetiracetam and topiramate are among the most effective AEDs in this type of patients.

[Status epilepticus associated with mild rotavirus gastroenteritis]. / Estado epiléptico asociado a una gastroenteritis leve por rotavirus.

BACKGROUND: Convulsions with mild rotavirus gastroenteritis were first described by Morooka in 1982. This is a process of convulsions and gastroenteritis without any changes in complementary tests. They are few cases out of Asia and they are rarely reported in Spain. CLINICAL CASE: They were 2 infants, 4 and 7 months old with no background of interest who suffered from status epilepticus during a mild rotavirus gastroenteritis without resolution after treatment. CONCLUSIONS: Benign afebrile cluster convulsions with rotavirus gastroenteritis is not common and is not often diagnosed in our area. It should be included in the differential diagnosis of status epilepticus and it is crucial to look for a history of diarrhoea. Careful management of diagnosis and treatment is essential given its controversial character.

Estado epiléptico asociado a una gastroenteritis leve por rotavirus / Status epilepticus associated with mild rotavirus gastroenteritis

Antecedentes. Las convulsiones asociadas a gastroenteritis por rotavirus fueron descritas por Morooka en 1982. Se definen como convulsiones asociadas a gastroenteritis leve y pruebas normales. Se han descrito poco fuera de Asia y son excepcionales en España. Caso clínico. Dos lactantes varones, de 4 y 7 meses de edad, que, sin antecedentes de interés y en el contexto de una gastroenteritis leve a la postre por rotavirus, desarrollan un estado epiléptico con escasa respuesta al tratamiento. Tras la resolución, se aprecia una curación at integrum sin alteraciones del desarrollo psicomotor. Conclusiones. Las convulsiones asociadas a gastroenteritis leve por rotavirus son una entidad poco frecuente o, por lo menos, poco diagnosticada en nuestro medio. Es necesaria su inclusión en el diagnóstico diferencial del estado epiléptico y, por lo tanto, es esencial buscar el posible antecedente de diarrea. Se requiere un manejo prudente en el área diagnóstica y terapéutica dado su carácter controvertido

Background. Convulsions with mild rotavirus gastroenteritis were first described by Morooka in 1982. This is a process of convulsions and gastroenteritis without any changes in complementary tests. They are few cases out of Asia and they are rarely reported in Spain. Clinical case. They were 2 infants, 4 and 7 months old with no background of interest who suffered from status epilepticus during a mild rotavirus gastroenteritis without resolution after treatment. Conclusions. Benign afebrile cluster convulsions with rotavirus gastroenteritis is not common and is not often diagnosed in our area. It should be included in the differential diagnosis of status epilepticus and it is crucial to look for a history of diarrhoea. Careful management of diagnosis and treatment is essential given its controversial character

Estatus epiléptico asociado a una gastroenteritis leve por rotavirus / Epileptic status associated with mild gastroenteritis caused by rotavirus

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Crisis epilépticas en el síndrome de Angelman / Epileptic seizures in angelman syndrome

Introducción. El síndrome de Angelman (SA) es un trastorno de base genética heterogénea caracterizado por retraso mental grave, ausencia del lenguaje, ataxia, dismorfia craneofacial y un fenotipo conductual característico. Pacientes y métodos. Se analizan 12 pacientes diagnosticados de SA y epilepsia, con una edad media de 10,9 años. El estudio se centra en las características de la epilepsia y trata de correlacionar los hallazgos con el genotipo de la enfermedad. Resultados. Todos presentaron crisis generalizadas de inicio precoz y, salvo uno, crisis polimorfas. Ocho de ellos presentaron también crisis focales. Todos los pacientes mostraban alteraciones electroencefalográficas antes de los dos años. Aunque no hay alteracionespatognomónicas en el electroencefalograma (EEG), su conocimiento en el SA puede ser un importante elemento de valoración para el diagnóstico precoz de esta entidad. En nuestra serie, todos los pacientes con deleción 15q11-13 presentaron el EEG típico de la enfermedad. El fármaco antiepiléptico más usado y más eficaz fue el ácido valproico (utilizado en todos lospacientes), seguido de lamotrigina y clobazam. En algún paciente se ensayaron hasta 10 fármacos antiepilépticos. La epilepsia suele ser de inicio muy precoz, e incluso precede al diagnóstico de SA en la mayoría de los casos, por lo que las crisis epilépticas pueden ser un elemento importante para llegar a un diagnóstico precoz. Es fundamental la adecuada tipificación de dichas crisis. Conclusión. El SA debe considerarse como diagnóstico diferencial en aquellos niños que presenten una epilepsiaprecoz y grave, en unión de un retraso psicomotor, con importante afectación de la marcha y el lenguaje. Este diagnóstico está apoyado por los hallazgos típicos en el EEG

Introduction. Angelman syndrome (AS) is a heterogeneous genetically-based disorder that is characterised bysevere mental retardation, absence of language, ataxia, craniofacial dysmorphia and a characteristic behavioural phenotype.Patients and methods. We analyse 12 patients with a mean age of 10.9 years diagnosed with AS. The study focuses on the characteristics of epilepsy and attempts to correlate the findings with the genotype of the disease. Results. All the patients presented early-onset generalised seizures and all except one had polymorphic seizures. Eight of them also presented focalseizures. All the patients displayed electroencephalographic alterations before the age of two years. Although there are no pathognomonic abnormalities in the electroencephalogram (EEG), knowledge of them in AS can be an important element ofassessment for reaching an early diagnosis of this condition. In our series, all the patients with 15q11-13 deletion presented an EEG pattern that was typical of the disease. The most commonly used and most effective antiepileptic drug was valproic acid (used in all patients), followed by lamotrigine and clobazam. Up to 10 antiepileptic drugs had been tried in some patients. Epilepsy usually has a very early onset and even precedes the diagnosis of AS in most cases, which means that the epileptic seizures can be an important aid in reaching an early diagnosis. Suitable classification of such seizures is essential.Conclusions. AS must be considered as a differential diagnosis in children who present early severe epilepsy together with psychomotor retardation and important gait and language disorders. This diagnosis is backed by the typical findings in the EEG

[Epileptic seizures in Angelman syndrome]. / Crisis epilépticas en el síndrome de Angelman.

INTRODUCTION: Angelman syndrome (AS) is a heterogeneous genetically-based disorder that is characterised by severe mental retardation, absence of language, ataxia, craniofacial dysmorphia and a characteristic behavioural phenotype. PATIENTS AND METHODS: We analyse 12 patients with a mean age of 10.9 years diagnosed with AS. The study focuses on the characteristics of epilepsy and attempts to correlate the findings with the genotype of the disease. RESULTS: All the patients presented early-onset generalised seizures and all except one had polymorphic seizures. Eight of them also presented focal seizures. All the patients displayed electroencephalographic alterations before the age of two years. Although there are no pathognomonic abnormalities in the electroencephalogram (EEG), knowledge of them in AS can be an important element of assessment for reaching an early diagnosis of this condition. In our series, all the patients with 15q11-13 deletion presented an EEG pattern that was typical of the disease. The most commonly used and most effective antiepileptic drug was valproic acid (used in all patients), followed by lamotrigine and clobazam. Up to 10 antiepileptic drugs had been tried in some patients. Epilepsy usually has a very early onset and even precedes the diagnosis of AS in most cases, which means that the epileptic seizures can be an important aid in reaching an early diagnosis. Suitable classification of such seizures is essential. CONCLUSIONS: AS must be considered as a differential diagnosis in children who present early severe epilepsy together with psychomotor retardation and important gait and language disorders. This diagnosis is backed by the typical findings in the EEG.

[An observational study of the patterns of carbamazepine and oxcarbazepine utilisation in adult patients with partial epilepsy in Spain]. / Estudio observacional de los patrones de utilizacion de carbamacepina y oxcarbacepina en pacientes adultos con epilepsia parcial en Espana.

INTRODUCTION: The health outcomes research methodology is especially important for evaluating therapeutic efficiency and effectiveness in the case of patients with epilepsy. AIM: To describe the patterns of utilisation of carbamazepine and the new antiepileptic drug oxcarbazepine, as well as their relation with the quality of life (patient's satisfaction and social and occupational repercussion) and therapeutic efficiency (utilisation of health care resources). PATIENTS AND METHODS: We performed an observational study of 284 adult patients with partial epilepsy that was clinically controlled with carbamazepine or oxcarbazepine either in monotherapy or with the addition of some other pharmaceutical agent. The study was carried out using the Morisky-Green questionnaire (for therapeutic compliance), the Sheehan Disability Scale (SDS, social functioning) and direct questioning (patient satisfaction); therapeutic efficiency was assessed with the register of medical visits, complementary examinations and concomitant treatments. RESULTS: The study showed that 65.6% suffered from partial seizures of a single type (simple, complex or with secondary generalisation) and 34.4% had several types of seizures. Oxcarbazepine treatment was being administered to 52% (mean dose: 1134.4 mg/day) and 48% were receiving carbamazepine (mean dose: 876.3 mg/day). The SDS scores were: occupational: 2.1; social: 2.0; and familial: 1.4. Most of the patients admitted having skipped some doses under certain circumstances; for this reason, 98.9% of them presented a medium level of compliance. It was found that 8.5% of patients visited the emergency department and 3% used hospitalisation services. CONCLUSIONS: The doses of oxcarbazepine tended to be lower than those recommended on the patient information leaflet. Patients only consider themselves to be mildly affected in their social and occupational relations and are satisfied with the control of their seizures. Patients receiving combination therapy utilise more health care resources.

Estudio observacional de los patrones de utilización de carbamacepina y oxcarbacepina en pacientes adultos con epilepsia parcial en España / An observational study of the patterns of carb amazepine and oxcarbazepine utilisation in adult patients with partial epilepsy in Spain

La metodología de la investigación de resultados en salud es particularmente importante para evaluarla efectividad y la eficiencia terapéutica en el caso de pacientes con epilepsia. Objetivo. Describir los patrones de utilización de la carbamacepina y del nuevo fármaco antiepiléptico oxcarbacepina, y su relación con la calidad de vida (satisfacción del paciente y repercusión sociolaboral) y la eficiencia terapéutica (utilización de recursos sanitarios). Pacientes y métodos. Estudio observacional de 284 pacientes adultos con epilepsia parcial clínicamente controlada con carbamacepina u oxcarbacepina en monoterapia o con otro fármaco añadido. Se utilizaron el cuestionario de Morisky-Green (cumplimiento terapéutico), el cuestionario de incapacidad de Sheehan (SDS, funcionamiento social) y preguntas directas (satisfacción del paciente); la eficiencia terapéutica se evaluó con el registro de visitas médicas, exploraciones complementarias y tratamientosconcomitantes. Resultados. El 65,6% padecía crisis parciales de un solo tipo (simples, complejas o con generalización secundaria) y el 34,4%, varios tipos de crisis. El 52% estaba en tratamiento con oxcarbacepina (dosis media: 1.134,4 mg/día) y el 48% con carbamacepina (dosis media: 876,3 mg/día). Las puntuaciones del SDS son: laboral: 2,1; social: 2,0; y familiar:1,4. La mayoría de los pacientes reconoce dejar alguna toma en determinadas circunstancias; por ello, el 98,9% presenta un nivel de cumplimiento medio. Un 8,5% de los pacientes utilizó los servicios de urgencias y un 3% utilizó los servicios de hospitalización.Conclusiones. Las dosis de oxcarbacepina tienden a ser inferiores a las recomendadas en la ficha técnica. Lospacientes sólo se consideran levemente afectados en sus relaciones sociolaborales y están satisfechos con el control de sus crisis. Los pacientes en politerapia utilizan más recursos sanitarios

The health outcomes research methodology is especially important for evaluating therapeutic efficiencyand effectiveness in the case of patients with epilepsy. Aim. To describe the patterns of utilisation of carbamazepine and the new antiepileptic drug oxcarbazepine, as well as their relation with the quality of life (patient’s satisfaction and social andoccupational repercussion) and therapeutic efficiency (utilisation of health care resources). Patients and methods.We performed an observational study of 284 adult patients with partial epilepsy that was clinically controlled with carbamazepine oroxcarbazepine either in monotherapy or with the addition of some other pharmaceutical agent. The study was carried out using the Morisky-Green questionnaire (for therapeutic compliance), the Sheehan Disability Scale (SDS, social functioning) and direct questioning (patient satisfaction); therapeutic efficiency was assessed with the register of medical visits, complementary examinations and concomitant treatments. Results. The study showed that 65.6% suffered from partial seizures of a single type(simple, complex or with secondary generalisation) and 34.4% had several types of seizures. Oxcarbazepine treatment was being administered to 52% (mean dose: 1134.4 mg/day) and 48% were receiving carbamazepine (mean dose: 876.3 mg/day).The SDS scores were: occupational: 2.1; social: 2.0; and familial: 1.4. Most of the patients admitted having skipped some doses under certain circumstances; for this reason, 98.9% of them presented a medium level of compliance. It was found that 8.5% ofpatients visited the emergency department and 3% used hospitalisation services. Conclusions. The doses of oxcarbazepine tended to be lower than those recommended on the patient information leaflet. Patients only consider themselves to be mildlyaffected in their social and occupational relations and are satisfied with the control of their seizures. Patients receiving combination therapy utilise more health care resources