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

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

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

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

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

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

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

Complex motor behaviors in temporal lobe epilepsy.

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

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

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