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Purpose: The role of sharps and spikes, interictal epileptiform discharges (IEDs), in guiding epilepsy surgery in children remains controversial, particularly with intracranial electroencephalography (IEEG). Although ictal recording is the mainstay of localizing epileptic networks for surgical resection, current practice dictates removing regions generating frequent IEDs if they are near the ictal...
Purpose: Given evidence of limitations in neuropsychological performance in epilepsy, we probed the integrity of components of cognition—including speed of processing, response inhibition, and spatial working memory—supporting executive function in pediatric epilepsy patients and matched controls.
Methods: A total of 44 pairs of controls and medically treated pediatric epilepsy patients with no...
Purpose: To examine baseline and prospective (2‐year) changes in third, fourth, and lateral ventricle volumes in children with new‐onset idiopathic epilepsies and controls (age 8–18 years).
Methods: Structural magnetic resonance imaging (MRI) were collected from children with idiopathic generalized epilepsy (IGE, n = 29), idiopathic localization‐related epilepsy (ILRE, n = 30), and healthy controls...
Cerebral hyaline astrocytic inclusions have been observed in a subset of patients with early onset epilepsy, brain structural anomalies, and developmental delay, which indicates that it may represent a unique clinicopathologic entity. To further characterize this condition we use proteomics to investigate differentially expressed proteins in epileptic brain tissue from three pediatric epileptic patients...
Purpose: Glut 1 deficiency syndrome (DS) is defined by hypoglycorrhachia with normoglycemia, acquired microcephaly, episodic movements, and epilepsy refractory to standard antiepileptic drugs (AEDs). Gold standard treatment is the ketogenic diet (KD), which provides ketones to treat neuroglycopenia. Our purpose is (1) to describe epilepsy phenotypes in a large Glut 1 DS cohort, to facilitate diagnosis;...
Purpose: Determine the optimal initial monotherapy for children with newly diagnosed childhood absence epilepsy (CAE) based on 12 months of double‐blind therapy.
Methods: A double‐blind, randomized controlled clinical trial compared the efficacy, tolerability, and neuropsychological effects of ethosuximide, valproic acid, and lamotrigine in children with newly diagnosed CAE. Study medications were...
Purpose: Determine if blood messenger RNA (mRNA) expression patterns in children with newly diagnosed untreated idiopathic epilepsy are different from those in healthy controls. Determine the differential expression patterns between epilepsy patients with generalized onset or partial onset seizures compared to healthy controls.
Methods: Whole blood was obtained from otherwise healthy pediatric patients...
PurposeThe white matter (WM) is considered critical for linking cortical processing networks necessary for cognition. The aim of this study was to assess diffusion tensor imaging (DTI) measures of regional WM in children with nonlesional localization‐related epilepsy in comparison to controls, and to determine the relation between lobar WM and neuropsychological performance.
MethodsForty children...
PurposeTo assess the efficacy and safety/tolerability of adjunctive zonisamide treatment in pediatric patients with partial epilepsy.
MethodsIn this phase III, double‐blind, randomized, placebo‐controlled, multicenter trial, 207 patients (age 6–17 years) with partial epilepsy, receiving one or two antiepileptic drugs, were randomized to receive adjunctive zonisamide or placebo. Zonisamide was initiated...
PurposeTo review the efficacy and tolerability of stiripentol in the treatment of U.S. children with Dravet syndrome.
MethodsU.S. clinicians who had prescribed stiripentol for two or more children with Dravet syndrome between March 2005 and 2012 were contacted to request participation in this retrospective study. Data collected included overall seizure frequency, frequency of prolonged seizures,...
PurposeTo examine the long‐term functional outcomes and their predictors using a patient/family centered approach in a cohort of children who had hemispherectomy. Functional outcome measures studied were the following: ambulation ability, visual symptoms, spoken language, reading skills, and behavioral problems.
MethodsWe reviewed 186 consecutive children who underwent hemispherectomy between 1997...
PurposeThe current concept for hemispherotomy includes various lateral techniques and the vertical perithalamic hemispherotomy introduced by Delalande in 1992. We have chosen the vertical approach because of advantages that possibly influence outcome: the possibility to completely disconnect the hemisphere at the level of the thalamus obviating both the need to resect the insula and the need to open...
ObjectiveTo investigate the safety/tolerability and efficacy of long‐term adjunctive zonisamide and its impact on growth and development in children (6–18 years) with partial epilepsy.
MethodsOpen‐label extension of a phase III, placebo‐controlled trial. Started with double‐blind transition period (2–11 weeks), during which patients on zonisamide continued at the same dose and those on placebo switched...
ObjectiveAstrocytic inclusions (AIs) have been identified on histologic specimens of patients with early onset seizures, and the proteomic contents have been described. The aim of this study was to compare the clinical, electroencephalography (EEG), magnetoencephalography (MEG), magnetic resonance imaging (MRI), and surgical outcomes of AIs relative to focal cortical dysplasia (FCD).
MethodsWe assessed...
ObjectiveUsing a hypothesis‐driven approach, subcortical and cortical regions implicated in anxiety disorders in the general population were examined in children with recent‐onset epilepsy with versus without anxiety compared to controls. This study reports frequency of anxiety disorders while examining familial, clinical, and demographic variables associated with anxiety in children with epilepsy...
Objective
Infantile spasms (IS) are a severe form of childhood epilepsy associated with autism spectrum disorders (ASD) in up to 35% of cases. The objective of this post hoc analysis of our randomized control trial was to determine whether rapid diagnosis and treatment of IS could limit the incidence of ASD while identifying risk factors related to ASD outcome.
Methods
Patients with IS were randomized...
Cognitive and developmental outcomes in patients with epileptic encephalopathy are hypothesized to result from an interplay between the underlying epileptic pathologic substrate and the acquired consequences of frequent and repetitive seizures and epileptiform discharges that often straddle the interictal and ictal boundaries. This article briefly reviews the evidence related to this assumption, presents...
The primary aim was to examine the preliminary efficacy of a family tailored problem‐solving intervention to improve antiepileptic drug (AED) adherence in families of children with new‐onset epilepsy. Secondary aims were to assess changes in targeted mechanisms and treatment feasibility and acceptability. Fifty families (Mage = 7.6 ± 3.0; 80% Caucasian; 42% idiopathic localization related) completed...
ObjectiveIn autoimmune encephalitis the etiologic role of neuronal cell‐surface antibodies is clear; patients diagnosed and treated early have better outcomes. Neuronal antibodies have also been described in patients with pediatric epilepsy without encephalitis. The aim was to assess whether antibody presence had any effect on long‐term outcomes in these patients.
MethodsPatients (n = 178) were recruited...
ObjectivesTo evaluate the effectiveness of lacosamide (LCM) in pediatric patients, using time to treatment failure as the outcome measure, and to assess the impact of concomitant sodium channel blocker (SCB) use on LCM retention.
MethodsThis is a retrospective cohort study of patients <21 years old receiving LCM from 2010 to 2015. Kaplan‐Meier survival curves were generated for time to LCM failure,...
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