ABSTRACT
En el presente trabajo se exponen los recientes avances sobre cefaleas infantiles que se han producido en los últimos 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 edición, versión beta, de la clasificación de la Sociedad Internacional de Cefaleas; entre ellas, cefaleas que han sufrido importantes matizaciones, la aparición de nuevas entidades y un apartado en el que se recoge la existencia de nuevas cefaleas, pero que aún no se han considerado como nuevas entidades. Además, se resalta la fuerte relación existente entre las cefaleas infantiles y el trastorno por déficit de atención/hiperactividad, la poca relación de la toma de glutamato con la aparición de cefaleas y la escasa utilidad de la resonancia cerebral en el diagnóstico de estos procesos. Por otra parte, se resalta la nueva modificación producida en los síndromes episódicos de la infancia
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
Subject(s)
Humans , Child , Headache/classification , Headache/complications , Headache/etiology , Attention Deficit Disorder with Hyperactivity/complicationsABSTRACT
BACKGROUND: The safety and effectiveness of lacosamide, an antiepileptic drug (AED) that selectively enhances the slow inactivation of voltage-gated sodium channels without affecting rapid inactivation, has been demonstrated in randomized, double-blind, placebo-controlled trials in adults with focal epileptic seizures. Although lacosamide is approved for use in patients over 16 years of age, limited clinical experience exists for younger patients. OBJECTIVE: To assess the efficacy and tolerability of lacosamide in children with refractory epilepsy. DesignMethods: The trial was a prospective, open-label, observational, multicenter study. A total of 130 patients aged less than 16 years (range 6 months to 16 years) with refractory epilepsy who had initiated treatment with lacosamide were enrolled at 18 neuropediatric units in hospitals across Spain. Patients with a variety of etiologies were enrolled, including those with partial epilepsies and symptomatic, generalized epilepsy syndromes. Lacosamide (VIMPAT®; UCB Pharma SA, Brussels, Belgium) was primarily administered once every 12 hours as an oral solution or as an oral tablet, with an initial dose of 1-2 mg/kg/day in the majority of cases. The majority of patients were also receiving stable concomitant therapy with ≥1 other AED. Treatment response to lacosamide was determined by assessing the change in seizure frequency after 3 months of lacosamide therapy. Responders were defined as patients who achieved a seizure frequency reduction of >50%. Tolerability was assessed by the reporting of adverse effects, laboratory testing, and electroencephalography recordings. RESULTS: Lacosamide was dosed at a mean of 6.80 ± 2.39 mg/kg/day. After 3 months of lacosamide therapy, 62.3% of patients achieved a >50% reduction in seizure frequency, with complete seizure suppression being reported in 13.8% of patients. Adverse effects occurred in 39 patients (30%), but no dose-response relationship was observed in terms of these events. In ten patients, instability, difficulty walking, an inability to relate to subjective elements, and blurred vision or dizziness were reported. A total of 13 patients discontinued treatment - in five of these patients, symptom intensity remained unchanged despite dose reduction, which led to treatment discontinuation. The symptoms were markedly different in each patient, preventing determination of a causal factor(s). CONCLUSIONS: The results of this study provide preliminary evidence for the efficacy of lacosamide in children with refractory epilepsy. Further evaluation in a randomized, controlled trial is needed to validate the efficacy in this population and to fully investigate the adverse effects described here. We recommend an initial dose of 1-2 mg/kg/day, uptitrated to 6-9 mg/kg/day over 4-6 weeks.
Subject(s)
Acetamides/adverse effects , Acetamides/therapeutic use , Anticonvulsants/adverse effects , Anticonvulsants/therapeutic use , Epilepsy/drug therapy , Adolescent , Child , Child, Preschool , Dose-Response Relationship, Drug , Female , Humans , Infant , Lacosamide , Male , Observation , Prospective Studies , Spain , Treatment OutcomeABSTRACT
No disponible
Subject(s)
Humans , Child , Psychoses, Substance-Induced/diagnosis , Methylphenidate/adverse effects , Attention Deficit Disorder with Hyperactivity/drug therapySubject(s)
Central Nervous System Stimulants/adverse effects , Methylphenidate/adverse effects , Psychoses, Substance-Induced/etiology , Attention Deficit Disorder with Hyperactivity/drug therapy , Central Nervous System Stimulants/therapeutic use , Child , Humans , Male , Methylphenidate/therapeutic use , Psychoses, Substance-Induced/physiopathologyABSTRACT
Introducción. El síndrome de West es una epilepsia dependiente de la edad que asocia espasmos infantiles, hipsarritmia y un retraso o detención en el desarrollo psicomotor, aunque este último no es imprescindible. Objetivos. Definir el perfil del síndrome de West en nuestro medio atendiendo a la etiología, semiología, respuesta a distintas opciones terapéuticas y aparición de efectos adversos, y establecer factores pronósticos que determinen la evolución. Pacientes y métodos. Se ha elaborado un documento de recogida de datos en el que se constatan los criterios de inclusión. La recogida de datos se ha realizado mediante la revisión de historias clínicas de los pacientes diagnosticados de síndrome de West en el período comprendido entre enero de 2003 y enero de 2009. Posteriormente, se ha realizado un estudio estadístico con análisis descriptivo y se ha establecido el nivel de significación estadística de los posibles factores pronósticos. Resultados. El estudio abarcó 70 pacientes. La etiología sintomática fue predominante, destacando la hipoxia-isquemia como causa principal. Respondió a vigabatrina el 58% de los pacientes, independientemente de la etiología. Más del 80% de los pacientes en tratamiento con hormona adrenocorticotropa quedaron libres de crisis y sin hipsarritmia. Casi la mitad de los pacientes evolucionó a otras epilepsias. Conclusiones. Los factores de mal pronóstico estadísticamente significativos fueron: existencia de antecedentes prenatales, antecedentes neonatales, etiología sintomática, edad de inicio inferior a 4 meses, crisis epilépticas antes del inicio de los espasmos y fuera del período neonatal, y retraso en el desarrollo psicomotor previo al inicio de los espasmos (AU)
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 (AU)
Subject(s)
Humans , Male , Female , Infant , Spasms, Infantile/etiology , Epilepsy/drug therapy , Spasms, Infantile/drug therapy , Vigabatrin/therapeutic use , Retrospective Studies , Adrenocorticotropic Hormone/therapeutic use , Drug Therapy, CombinationABSTRACT
INTRODUCTION: Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic syndromes in childhood. It is characterised by the presence of several types of seizures, a characteristic electroencephalographic trace and its frequent association with mental retardation. There are no accurate data on the epidemiology of LGS in Spain. AIM: To describe the epidemiological profile of LGS in Spain. PATIENTS AND METHODS: We conducted a retrospective epidemiological study of a series of 331 patients with LGS from 50 Spanish hospitals. Patients were considered to be cases of LGS if they had at least two of the three types of seizures that characterise LGS (axial tonic seizures, atypical absences and atonic seizures) and displayed slow diffuse intercritical spike-wave activity (lower than 3 Hz) in the electroencephalogram in wakefulness. RESULTS: The mean age of the patients was 18.2 +/- 13.5 years. Of the total sample, 62% were males and 97% had cognitive retardation. And 54% of cases had a symptomatic aetiology. The most frequent types of seizures were axial tonic (89%), followed by the atypical absent type (84%) and atonic seizures (69%). In all, 99% of patients were treated with polytherapy, the most commonly used drugs being valproic acid, lamotrigine and topiramate. CONCLUSIONS: The results of the study are in line with those from previous analyses carried out in populations with similar characteristics. The high percentage of LGS patients with cognitive retardation in this epileptic encephalopathy results in important family and social repercussions.
Subject(s)
Epilepsy/drug therapy , Epilepsy/epidemiology , Epilepsy/physiopathology , Adolescent , Adult , Anticonvulsants/therapeutic use , Child , Child, Preschool , Electroencephalography , Female , Humans , Infant , Intellectual Disability/epidemiology , Intellectual Disability/physiopathology , Male , Middle Aged , Retrospective Studies , Spain/epidemiology , Syndrome , Young AdultABSTRACT
Introducción. El síndrome de Lennox-Gastaut (SLG) es uno de los síndromes epilépticos más graves de la infancia. Se caracteriza por la presencia de varios tipos de crisis, un trazado electroencefalográfico característico y la frecuente asociación de retraso mental. No existen datos precisos sobre la epidemiología del SLG en España. Objetivo. Describir el perfil epidemiológico del SLG en España. Pacientes y métodos. Estudio epidemiológico retrospectivo de una serie de 331 pacientes con SLG procedentes de 50 hospitales españoles. Se consideraron casos de SLG aquellos pacientes que tenían al menos dos de los tres tipos de crisis características del SLG (crisis tónicas axiales, ausencias atípicas y crisis atónicas) junto con actividad intercrítica difusa de punta-onda lenta (< 3 Hz) en el electroencefalograma en vigilia. Resultados. La edad media de los pacientes fue de de 18,2 ± 13,5 años. El 62% eran varones y el 97% tenía retraso cognitivo. El 54% de los casos fue de etiología sintomática. Los tipos de crisis más frecuentes fueron las tónicas axiales (89%), seguidas de las ausencias atípicas (84%) y las crisis atónicas (69%). El 99% de los pacientes fue tratado con politerapia, siendo los fármacos más frecuentemente empleados el ácido valproico, la lamotrigina y el topiramato. Conclusiones. Los resultados del estudio son consistentes con los de estudios previos realizados en poblaciones de características similares. El elevado porcentaje de pacientes con SLG con retraso cognitivo condiciona en esta encefalopatía epiléptica una gran repercusión, tanto familiar como social (AU)
Introduction. Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic syndromes in childhood. It is characterised by the presence of several types of seizures, a characteristic electroencephalographic trace and its frequent association with mental retardation. There are no accurate data on the epidemiology of LGS in Spain. Aim. To describe the epidemiological profile of LGS in Spain. Patients and methods. We conducted a retrospective epidemiological study of a series of 331 patients with LGS from 50 Spanish hospitals. Patients were considered to be cases of LGS if they had at least two of the three types of seizures that characterise LGS (axial tonic seizures, atypical absences and atonic seizures) and displayed slow diffuse intercritical spikewave activity (< 3 Hz) in the electroencephalogram in wakefulness. Results. The mean age of the patients was 18.2 ± 13.5 years. Of the total sample, 62% were males and 97% had cognitive retardation. And 54% of cases had a symptomatic aetiology. The most frequent types of seizures were axial tonic (89%), followed by the atypical absent type (84%) and atonic seizures (69%). In all, 99% of patients were treated with polytherapy, the most commonly used drugs being valproic acid, lamotrigine and topiramate. Conclusions. The results of the study are in line with those from previous analyses carried out in populations with similar characteristics. The high percentage of LGS patients with cognitive retardation in this epileptic encephalopathy results in important family and social repercussions (AU)
Subject(s)
Humans , Epilepsy/epidemiology , Intellectual Disability/epidemiology , Retrospective Studies , Anticonvulsants/therapeutic use , Epilepsy/drug therapy , Electroencephalography , Age of Onset , Epilepsy, Generalized/epidemiology , Epilepsy, Absence/epidemiologyABSTRACT
Introducción. El síndrome de Lennox-Gastaut (SLG) es uno de los síndromes epilépticos más graves de la infancia. Se aracteriza por la presencia de varios tipos de crisis, un trazado electroencefalográfico característico y la frecuente asociación de retraso mental. No existen datos precisos sobre la epidemiología del SLG en España. Objetivo. Describir el perfil epidemiológico del SLG en España. Pacientes y métodos. Estudio epidemiológico retrospectivo de una serie de 331 pacientes con SLG procedentes de 50 hospitales españoles. Se consideraron casos de SLG aquellos pacientes que tenían al menos dos de los tres tipos de crisis características del SLG (crisis tónicas axiales, ausencias atípicas y crisis atónicas) junto con actividad intercrítica difusa de punta-onda lenta (< 3 Hz) en el electroencefalograma en vigilia. Resultados. La edad media de los pacientes fue de de 18,2 ± 13,5 años. El 62% eran varones y el 97% tenía retraso cognitivo. El 54% de los casos fue de etiología sintomática. Los tipos de crisis más frecuentes fueron las tónicas axiales (89%), seguidas de las ausencias atípicas (84%) y las crisis atónicas (69%). El 99% de los pacientes fue tratado con politerapia, siendo los fármacos más frecuentemente empleados el ácido valproico, la lamotrigina y el topiramato. Conclusiones. Los resultados del estudio son consistentes con los de estudios previos realizados en poblaciones de características similares. El elevado porcentaje de pacientes con SLG con retraso cognitivo condiciona en esta encefalopatía epiléptica una gran repercusión, tanto familiar como social (AU)
Introduction. Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic syndromes in childhood. It is characterised by the presence of several types of seizures, a characteristic electroencephalographic trace and its frequent association with mental retardation. There are no accurate data on the epidemiology of LGS in Spain. Aim. To describe the epidemiological profile of LGS in Spain. Patients and methods. We conducted a retrospective epidemiological study of a series of 331 patients with LGS from 50 Spanish hospitals. Patients were considered to be cases of LGS if they had at least two of the three types of seizures that characterise LGS (axial tonic seizures, atypical absences and atonic seizures) and displayed slow diffuse intercritical spikewave activity (< 3 Hz) in the electroencephalogram in wakefulness. Results. The mean age of the patients was 18.2 ± 13.5 years. Of the total sample, 62% were males and 97% had cognitive retardation. And 54% of cases had a symptomatic aetiology. The most frequent types of seizures were axial tonic (89%), followed by the atypical absent type (84%) and atonic seizures (69%). In all, 99% of patients were treated with polytherapy, the most commonly used drugs being valproic acid, lamotrigine and topiramate. Conclusions. The results of the study are in line with those from previous analyses carried out in populations with similar characteristics. The high percentage of LGS patients with cognitive retardation in this epileptic encephalopathy results in important family and social repercussions (AU)
Subject(s)
Humans , Male , Female , Epilepsy/epidemiology , Intellectual Disability/epidemiology , Retrospective Studies , Anticonvulsants/therapeutic use , Epilepsy/drug therapy , Electroencephalography , Age of Onset , Epilepsy, Generalized/epidemiology , Epilepsy, Absence/epidemiologyABSTRACT
No disponible
Subject(s)
Humans , Male , Child , Methylphenidate/adverse effects , Cardiovascular Diseases/chemically induced , Attention Deficit Disorder with Hyperactivity/drug therapy , Hypertension/chemically induced , Tachycardia/chemically inducedABSTRACT
Contiene: ¿Conocemos la epilepsia?; crisis epiléptica. Síndrome epiléptico. Enfermedad epiléptica; qué debemos hacer cuando nos encontremos ante una crisis epiléptica; qué puede provocar o favorecer una crisis epiléptica; tratamiento farmacológico de la epilepsia; tratamiento quirúrgico de las epilepsias y otras terapias alternativas; calidad de vida y epilepsia. Reflexiones prácticas para las diferentes edades de la vida; legislación: Lo que se nos permite hacer a las personas epilépticas; embarazo y epilepsia; el papel de las asociaciones.
Subject(s)
EpilepsyABSTRACT
Studies designed specifically for the pediatric population are needed to assess the tolerability and safety of the new antiepileptic drugs. The purpose of this study was to document the safety, ease of dosing, and acceptance of oxcarbazepine oral suspension in pediatric patients in monotherapy and polytherapy. A prospective, multicenter, open-label study was conducted at the neurology services of three pediatric university hospitals over 12 months. After obtaining signed informed consent, we enrolled a series of 62 patients with epilepsy aged between 2 months and 14 years who began oxcarbazepine treatment in monotherapy or in combination with other antiepileptic drugs to assess the seizure frequency, safety (adverse events), and acceptance of the pharmaceutical form by the patient's family. Fifty patients (80.6%) reduced seizures by at least 50%, 44 (71%) saw a reduction in seizure frequency of over 75%, and 29 (46.8%) were seizure free at the end of the study. The difference in the number of seizures before and after the study was statistically significant, both overall and by type of pathology. Adverse events occurred in four patients (6.4%) and required withdrawal of the drug in two cases (skin rash); three patients (4.8%) withdrew for inefficacy. Five patients (8.1%) withdrew from the treatment. We concluded that, in this series of patients, oxcarbazepine in oral suspension form was seen to help reduce seizure frequency, to have few side effects, and to be accepted by parents and patients.
Subject(s)
Anticonvulsants/administration & dosage , Carbamazepine/analogs & derivatives , Epilepsy/drug therapy , Administration, Oral , Adolescent , Carbamazepine/administration & dosage , Child , Child, Preschool , Drug Administration Schedule , Female , Follow-Up Studies , Humans , Infant , Male , Patient Satisfaction , Prospective Studies , Suspensions , Treatment OutcomeABSTRACT
We report another case of cerebro-facio-thoracic dysplasia (Pascual-Castroviejo syndrome) characterized by mental retardation and characteristic facies: narrow forehead, synophris, hypertelorism, broad nasal bridge, long philtrum, micrognathia, triangular-shaped mouth and low posterior hairline, and also brachycephaly, calcified clinoid ligaments, and upper rib deformities. Although the severity of mental retardation within the syndrome varies, the reported case shows not only a severe degree, but also cerebral malformations not reported in any of the previous cases of cerebro-facio-thoracic syndrome. These include cortical-subcortical atrophy with hypoplasia of the corpus callosum, and of the cerebellar vermis. We also discuss the inheritance pattern and differential diagnosis, comparing this phenotype to other similar dysmorphic syndromes.
