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Objective: To obtain perspective on epilepsy in patients referred to tertiary centers in France, and describe etiology, epilepsy syndromes, and identify factors of drug resistance and comorbidities., Methods: We performed a cross-sectional analysis of the characteristics of 5,794 pediatric and adult patients with epilepsy included in a collaborative database in France between 2007 and 2013. Comparisons between groups used Student's t-test or Fisher's exact test for binary or categorical variables. Factors associated with drug resistance and intellectual disability were evaluated in multi-adjusted logistic regression models., Results: Mean age at inclusion was 17.9 years; children accounted for 67%. Epilepsy was unclassified in 20% of patients, and etiology was unknown in 65%, including those with idiopathic epilepsies. Etiologies differed significantly in adult- when compared to pediatric-onset epilepsy; however, among focal structural epilepsies, mesial temporal lobe epilepsy with hippocampal sclerosis began as often in the pediatric as in adult age range. Drug resistance concerned 53% of 4,210 patients evaluable for seizure control and was highest in progressive myoclonic epilepsy (89%), metabolic diseases (84%), focal cortical dysplasia (70%), other cortical malformations (69%), and mesial temporal lobe epilepsy with hippocampal sclerosis (67%). Fifty-nine percent of patients with focal structural epilepsy and 69% with epileptic encephalopathies were drug resistant; however, 40-50% of patients with West syndrome and epileptic encephalopathy with continuous spike-and-waves during sleep were seizure-free. Ages at onset in infancy and in young adults shared the highest risk of drug resistance. Epilepsy onset in infancy comprised the highest risk of intellectual disability, whereas specific cognitive impairment affected 36% of children with idiopathic focal epilepsy., Significance: Our study provides a snapshot on epilepsy in patients referred to tertiary centers and discloses needs for diagnosis and treatment. Large databases help identify patients with rare conditions that could benefit from specific prospective studies., (Wiley Periodicals, Inc. © 2016 International League Against Epilepsy.)

Purpose: To analyze the results of callosotomy in 17 children with symptomatic generalized epilepsy after West syndrome, according to the different seizure types and surgical procedures, to define selection criteria for candidates to callosotomy., Methods: Callosotomy was performed in two successive stages. Partial callosotomy, anterior in 13 and posterior in three, was followed by completion in 14 cases in all but four patients (complete callosotomy in one stage in one, no completion in three). All patients had clinical, video-EEG, and neuropsychological evaluation before and after each stage of callosotomy, with a mean final follow-up of 4 years., Results: Seizure frequency improved in only two of 13 patients after anterior callosotomy, in none of three after posterior callosotomy, but in nine of 14 after complete callosotomy. After complete callosotomy, spasms disappeared in 80% of the cases, and drop attacks, the most severe ictal event, completely stopped or were dramatically reduced in 90% of the children. One patient no longer had episodes of status epilepticus, and another one acquired the ability to walk after complete callosotomy. From the cognitive viewpoint, nine patients with improved seizure frequency after complete callosotomy also had improved behavior and cognitive functions, but two others experienced speech deterioration after posterior callosotomy at age 11 years and completion of callosotomy at age 16 years., Conclusions: As in other severe generalized epilepsies in childhood, drop attacks provide the best indication for complete callosotomy in patients with previous West syndrome. Because drop attacks can be identifiable by falls only, the previous acquisition of walking should be considered as a key feature for any benefit to be obtained.

Hypsarrhythmia and spasms of West syndrome have been postulated by some investigators to have a brainstem origin. Cortical involvement is also postulated because of the frequent association with cortical lesions. Cortical discharges have been proposed to stimulate subcortical structures (i.e., brainstem) and hypsarrhythmia is a secondarily generalized phenomenon produced by the brainstem. Two children with cryptogenic West syndrome refractory for several years to steroids and conventional antiepileptic drugs underwent total callosotomy. Hypsarrhythmia that was bilateral before operation became unilateral after the procedure and spasms became asymmetrical, suggesting that a corticocortical pathway through the corpus callosum is important in the generalization of hypsarrhythmia.

Clinical, neuroradiological data and outcome of epilepsy of 32 patients with symptomatic infantile spasms caused by perinatal insults are reported. Neuroradiological investigations showed focal as well as diffuse cerebral lesions. Fifteen children had a porencephalic lesion, 12 had periventricular leukomalacia and five had diffuse bilateral cerebral atrophy, associated with status marmoratus in one child and bilateral ulegyria in another. Eight patients (25%) had epilepsy following West syndrome. In the group of 15 children with porencephalic cysts, the lesion was always unilateral, but topography and extension varied. Eight children had circumscribed cysts involving the rolandic or parieto-occipital regions, and the outcome of epilepsy was favorable. Seven patients had large lesions involving the frontal lobe, associated with unilateral hemispheric atrophy in four cases. These four children had an unfavorable epilepsy outcome. In the 12 patients with periventricular leukomalacia, all premature, eleven had no seizure relapse after the spasms; one of these children had epilepsy. In the five full-term patients with bilateral and diffuse cerebral lesions, three children had epilepsy at the last observation. In children with leukomalacia and in patients with localized porencephalic lesions the outcome of epilepsy appears to be better than in patients with diffuse cerebral lesions or in children with extensive porencephalic cysts, particularly those involving the frontal lobe.