1,089 results match your criteria Benign Childhood Epilepsy


Impairment of eye emotion discrimination in benign childhood epilepsy with centrotemporal spikes: A neuropsychological study.

Brain Behav 2021 May 4:e02154. Epub 2021 May 4.

Department of Neurology, Anhui Provincial Children's Hospital, Hefei, China.

Purpose: To explore the characteristics of the impairment of eye emotional recognition and related clinical factors in children with benign childhood epilepsy with centrotemporal spikes (BECT).

Methods: The Eye Basic Emotion Discrimination Task and Eye Complex Emotion Discrimination Task were used to study emotion discrimination in 33 recently diagnosed BECT patients and 33 BECT patients after complete remission compared to respective age- and gender-matched comparison participants.

Results: The scores for discrimination of sadness, fear, and disgust were significantly lower in the newly diagnosed BECT group than in the comparison group (p = . Read More

View Article and Full-Text PDF

Mutations Are Associated With Benign Epilepsy of Childhood With Centrotemporal Spikes With or Without Arrhythmia.

Front Neurosci 2021 7;15:629610. Epub 2021 Apr 7.

Institute of Neuroscience and Department of Neurology of the Second Affiliated Hospital of Guangzhou Medical University, Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou, China.

encodes ryanodine receptor 2 protein (RYR-2) that is mainly located on endoplasmic reticulum membrane and regulates intracellular calcium concentration. The RYR-2 protein is ubiquitously distributed and highly expressed in the heart and brain. Previous studies have identified the mutations in the etiology of arrhythmogenic right ventricular dysplasia 2 and catecholaminergic polymorphic ventricular tachycardia. Read More

View Article and Full-Text PDF

A follow-up study in children with status epilepticus during sleep: From clinical spectrum to outcome.

Epilepsy Behav 2021 04 25;117:107843. Epub 2021 Feb 25.

Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, China. Electronic address:

Purpose: To evaluate the correlation between clinical spectrum and therapeutic outcomes and neuropsychological deficits in children with status epilepticus during sleep (SES).

Methods: The clinical spectrum of patients with SES was defined as follows: status epilepticus of benign childhood epilepsy with centro-temporal spikes (SEBECTs), atypical benign focal epilepsy during childhood (ABFEC), non-idiopathic focal epilepsy (NIFE), and Landau-Kleffner syndrome (LKS). SES cases were divided into 4 groups according to neuropsychological findings before treatment: developmental delay/intellectual disability (DD/ID), cognitive impairment (CI), attention deficit and/or hyperactivity behaviors (AHD), and normal group (NG). Read More

View Article and Full-Text PDF

A Systematic Review of Seizure-Freedom Rates in Patients With Benign Epilepsy of Childhood With Centrotemporal Spikes Receiving Antiepileptic Drugs.

Clin Neuropharmacol 2021 Mar-Apr 01;44(2):39-46

Department of Pallative Care.

Objectives: The objective of this study was to evaluate seizure remission rates in patients with benign epilepsy of childhood with centrotemporal spikes (BECTS) receiving antiepileptic drugs.

Methods: PubMed and Web of Science were searched for studies on pharmacotherapy in patients with BECTS using free search terms or Medical Subject Headings. Only studies that used seizure-freedom rates as an indicator for pharmaceutical efficacy were considered. Read More

View Article and Full-Text PDF
February 2021

Early risk factors for encephalopathic transformation in children with benign childhood epilepsy with centrotemporal spikes.

Brain Dev 2021 Apr 15;43(4):603-604. Epub 2021 Feb 15.

Faculty of Medicine, Tel-Aviv University, Israel; Neurology Institute, Dana-Dwek Children's Hospital, Tel Aviv, Sourasky Medical Center, Israel.

View Article and Full-Text PDF

Effects of sulthiame on seizure frequency and EEG in children with electrical status epilepticus during slow sleep.

Epilepsy Behav 2021 03 4;116:107793. Epub 2021 Feb 4.

Department of Pediatric Neurology, Istanbul Medipol University Faculty of Medicine, Istanbul, Turkey.

Objective: It is argued that early and adequate treatment of electrical status epilepticus in sleep (ESES) is essential to preserve cognitive functions and possibly recovering lost skills. Although antiepileptic drugs (AEDs) are effective in ESES, there is not much experience in the use of sulthiame. In this study, we aimed to examine the efficiency and tolerability of sulthiame in ESES. Read More

View Article and Full-Text PDF

Sulthiame impairs mitochondrial function in vitro and may trigger onset of visual loss in Leber hereditary optic neuropathy.

Orphanet J Rare Dis 2021 02 4;16(1):64. Epub 2021 Feb 4.

Interdisciplinary Pediatric Center for Children With Developmental Disabilities and Severe Chronic Disorders, University Medical Center Göttingen, Göttingen, Germany.

Background: Leber hereditary optic neuropathy (LHON) is the most common mitochondrial disorder and characterized by acute or subacute painless visual loss. Environmental factors reported to trigger visual loss in LHON mutation carriers include smoking, heavy intake of alcohol, raised intraocular pressure, and some drugs, including several carbonic anhydrase inhibitors. The antiepileptic drug sulthiame (STM) is effective especially in focal seizures, particularly in benign epilepsy of childhood with centrotemporal spikes, and widely used in pediatric epileptology. Read More

View Article and Full-Text PDF
February 2021

Self-limited focal epilepsy and childhood apraxia of speech with WAC pathogenic variants.

Eur J Paediatr Neurol 2021 Jan 24;30:25-28. Epub 2020 Dec 24.

Division of Child Neurology, Department of Pediatrics, Montreal Children's Hospital, McGill University, Montreal, Quebec, Canada; Department of Neurology & Neurosurgery, Montreal Children's Hospital, McGill University, Montreal, Quebec, Canada; Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada. Electronic address:

Heterozygous pathogenic WAC variants cause Desanto-Shinawi syndrome; affected patients have dysmorphic features, developmental impairment and behavioral abnormalities. Seizures are reported in one quarter, including tonic-clonic, absence, and febrile seizures. This study aimed to better understand the phenotypic spectrum of epilepsy and development in Desanto-Shinawi syndrome. Read More

View Article and Full-Text PDF
January 2021

"Benign" temporal lobe epilepsy with hippocampal sclerosis: A forgotten entity?

Epilepsy Behav Rep 2020 17;14:100407. Epub 2020 Nov 17.

Department of Neuroscience, Monash Medical Centre, Clayton, Victoria, Australia.

Mesial temporal lobe epilepsy, a well-characterized epilepsy syndrome, is most commonly accompanied by the pathological feature of hippocampal sclerosis. Patients with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) typically present in childhood to early adolescence. We describe a cohort of patients who presented with their first epileptic seizure in late adulthood with atypical features. Read More

View Article and Full-Text PDF
November 2020

Surface-based morphometry study of the brain in benign childhood epilepsy with centrotemporal spikes.

Ann Transl Med 2020 Sep;8(18):1150

Department of Radiology, Affiliated Hospital of Zunyi Medical University, Medical Imaging Center of Guizhou Province, Zunyi, China.

Background: The study aimed to explore cortical morphology in benign childhood epilepsy with centrotemporal spikes (BECTS) and the relationship between cortical characteristics and age of onset and intelligence quotient (IQ).

Methods: Cortical morphometry with surface-based morphometry (SBM) was used to compare changes in cortical thickness, gyrification, sulcal depth, and fractal dimension of the cerebral cortex between 25 BECTS patients and 20 healthy controls (HCs) with two-sample -tests [P<0.05, family-wise error (FWE) corrected]. Read More

View Article and Full-Text PDF
September 2020

Early risk factors for encephalopathic transformation in children with benign childhood epilepsy with centrotemporal spikes.

Brain Dev 2021 Apr 16;43(4):601-602. Epub 2020 Nov 16.

Pediatric Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand 249203, India. Electronic address:

View Article and Full-Text PDF

Heterozygous Variants Are Associated With Idiopathic Focal Epilepsy With Incomplete Penetrance.

Front Genet 2020 15;11:559080. Epub 2020 Oct 15.

Key Laboratory of Neurogenetics and Channelopathies of Guangdong Institute, Department of Neurology of the Second Affiliated Hospital of Guangzhou Medical University, of Neuroscience, Province and the Ministry of Education of China, Guangzhou, China.

Introduction: Idiopathic focal epilepsy (IFE) is a group of self-limited epilepsies. The etiology for the majority of the patients with IFE remains elusive. We thus screened disease-causing variants in the patients with IFE. Read More

View Article and Full-Text PDF
October 2020

The Phenotypic Spectrum of PRRT2-Associated Paroxysmal Neurologic Disorders in Childhood.

Biomedicines 2020 Oct 28;8(11). Epub 2020 Oct 28.

Department of Pediatric Neurology, Children's Hospital Datteln, Witten/Herdecke University, 45711 Datteln, Germany.

Pathogenic variants in , encoding the proline-rich transmembrane protein 2, have been associated with an evolving spectrum of paroxysmal neurologic disorders. Based on a cohort of children with PRRT2-related infantile epilepsy, this study aimed at delineating the broad clinical spectrum of PRRT2-associated phenotypes in these children and their relatives. Only a few recent larger cohort studies are on record and findings from single reports were not confirmed so far. Read More

View Article and Full-Text PDF
October 2020

A Comparative Study on the Efficacy of Levetiracetam and Carbamazepine in the Treatment of Rolandic Seizures in Children: An Open-Label Randomized Controlled Trial.

J Res Pharm Pract 2020 Apr-Jun;9(2):68-72. Epub 2020 Jun 26.

Child Growth and Developmental Research Center, Research Institute for Primordial Prevention of Noncommunicable Diseases, Isfahan University of Medical Sciences, Isfahan, Iran.

Objective: This study was performed to investigate whether levetiracetam should be preferred to carbamazepine as a treatment choice for benign childhood epilepsy with centro Temporal spikes (BCECTS), the most common partial epilepsy of childhood.

Methods: This randomized clinical trial study included 92 children with rolandic epilepsy aged 4-12 years referred to the Pediatric Neurology Clinic at Imam Hossein Hospital, Isfahan, Iran, from April 2019 to January 2020. Patients were selected consecutively and randomly assigned to two study groups (levetiracetam and carbamazepine groups). Read More

View Article and Full-Text PDF

Neurocutaneous melanocytosis (melanosis).

Childs Nerv Syst 2020 10 13;36(10):2571-2596. Epub 2020 Oct 13.

Pediatric Neurosurgery, International Neuroscience Institute (INI), Hannover, Germany.

Neurocutaneous melanosis (NCM; MIM # 249400; ORPHA: 2481], first reported by the Bohemian pathologist Rokitansky in 1861, and now more precisely defined as neurocutaneous melanocytosis, is a rare, congenital syndrome characterised by the association of (1) congenital melanocytic nevi (CMN) of the skin with overlying hypertrichosis, presenting as (a) large (LCMN) or giant and/or multiple (MCMN) melanocytic lesions (or both; sometimes associated with smaller "satellite" nevi) or (b) as proliferative melanocytic nodules; and (2) melanocytosis (with infiltration) of the brain parenchyma and/or leptomeninges. CMN of the skin and leptomeningeal/nervous system infiltration are usually benign, more rarely may progress to melanoma or non-malignant melanosis of the brain. Approximately 12% of individuals with LCMN will develop NCM: wide extension and/or dorsal axial distribution of LCMN increases the risk of NCM. Read More

View Article and Full-Text PDF
October 2020

Oral language in children with benign childhood epilepsy with centrotemporal spikes.

Epilepsy Behav 2020 10 9;111:107328. Epub 2020 Sep 9.

Departamento de Pediatria do Hospital Beatriz Ângelo, Portugal.

Purpose: Benign childhood epilepsy with centrotemporal spikes is one of the most common childhood disorders. Despite the benignity usually attributed to this epileptic syndrome, several studies have demonstrated that these children have cognitive disabilities. Among these disturbances, language disorders have been the less studied in depth. Read More

View Article and Full-Text PDF
October 2020

Altered Static and Dynamic Spontaneous Neural Activity in Drug-Naïve and Drug-Receiving Benign Childhood Epilepsy With Centrotemporal Spikes.

Front Hum Neurosci 2020 28;14:361. Epub 2020 Aug 28.

The Clinical Hospital of Chengdu Brain Science Institute, Key Laboratory for NeuroInformation of Ministry of Education, Center for Information in Medicine, High-Field Magnetic Resonance Brain Imaging Key Laboratory of Sichuan Province, School of Life Sciences and Technology, University of Electronic Science and Technology of China, Chengdu, China.

The present study aims to investigate intrinsic abnormalities of brain and the effect of antiepileptic treatment on brain activity in Benign childhood epilepsy with centrotemporal spikes (BECTS). Twenty-six drug-naïve patients (DNP) and 22 drug-receiving patients (DRP) with BECTS were collected in this study. Static amplitude of low frequency fluctuation (sALFF) and dynamic ALFF (dALFF) were applied to resting-state fMRI data. Read More

View Article and Full-Text PDF

The relationship between epilepsy and cognitive function in benign childhood epilepsy with centrotemporal spikes.

Brain Behav 2020 12 22;10(12):e01854. Epub 2020 Sep 22.

Department of Neurology, Nanjing Brain Hospital, Nanjing Medical University, Nanjing, China.

Introduction: This study was aimed to explore the relationship between neural network changes in newly diagnosed children with Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS) and cognitive impairment.

Methods: Children's cognition was evaluated using the Wechsler Intelligence Scale for Children-Fourth Edition (WISC-IV). Magnetoencephalographic (MEG) data of 18 healthy children and 22 BECTS patients were recorded in order to construct a functional connectivity (FC) network, which was quantified by graph theory (GT). Read More

View Article and Full-Text PDF
December 2020

Alterations of functional connectivity density in a Chinese family with a mild phenotype associated with a novel inherited variant of SCN8A.

Epilepsy Behav 2020 11 10;112:107379. Epub 2020 Sep 10.

Department of Neurology, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China; Chinese Academy of Sciences Sichuan Translation Medicine Research Hospital, Chengdu, China. Electronic address:

Objective: Only a few heritable SCN8A variants have been described in patients with a mild phenotype of epilepsy. Here, we describe a Chinese family with a novel inherited SCN8A variant and investigate changes in spontaneous cerebral activity during the resting-state in magnetic resonance imaging (MRI)-negative patients with epilepsy and their unaffected siblings.

Methods: A gene panel targeting 535 epilepsy genes was performed on the proband and his parents. Read More

View Article and Full-Text PDF
November 2020

Early prediction of encephalopathic transformation in children with benign epilepsy with centro-temporal spikes.

Brain Dev 2021 Feb 8;43(2):268-279. Epub 2020 Sep 8.

Sackler Faculty of Medicine, Tel-Aviv University, Israel; Pediatric Neurology Institute, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Israel.

Background: Most children with Benign epilepsy with centro-temporal spikes (BECTS) undergo remission during late adolescence and do not require treatment. In a small group of patients, the condition may evolve to encephalopathic syndromes including epileptic encephalopathy with continuous spike-and-wave during sleep (ECSWS), or Landau-Kleffner Syndrome (LKS). Development of prediction models for early identification of at-risk children is of utmost importance. Read More

View Article and Full-Text PDF
February 2021

The relationship between neuromagnetic activity and cognitive function in benign childhood epilepsy with centrotemporal spikes.

Epilepsy Behav 2020 11 25;112:107363. Epub 2020 Aug 25.

Department of Neurology, Nanjing Brain Hospital, Nanjing Medical University, Nanjing, Jiangsu 210029, China. Electronic address:

Purpose: Our aim was to explore the pathophysiological mechanism of cognitive function changes in early untreated children with benign childhood epilepsy with centrotemporal spikes (BECTS).

Methods: Magnetoencephalography (MEG) was performed in 33 children with BECTS and 18 healthy children. Wechsler Intelligence Scale for Children, fourth edition (WISC-IV) was used to divide children with BECTS into two groups. Read More

View Article and Full-Text PDF
November 2020

Multilobar Epilepsy Surgery in Childhood and Adolescence: Predictors of Long-Term Seizure Freedom.

Neurosurgery 2020 12;88(1):174-182

Medical Faculty, University of Freiburg, Freiburg im Breisgau, Germany.

Background: Although multilobar resections correspond to one-fifth of pediatric epilepsy surgery, there are little data on long-term seizure control.

Objective: To investigate the long-term seizure outcomes of children and adolescents undergoing multilobar epilepsy surgery and identify their predictors.

Methods: In this retrospective study, we considered 69 consecutive patients that underwent multilobar epilepsy surgery at the age of 10. Read More

View Article and Full-Text PDF
December 2020

Reliability and availability of granger causality density in localization of Rolandic focus in BECTS.

Brain Imaging Behav 2020 Jul 31. Epub 2020 Jul 31.

Shenzhen Mental Health Centre, Shenzhen Kangning Hospital, Shenzhen, 518003, China.

A new method, called granger causality density (GCD), could reflect the directed information flow of the epileptiform activity, which is much closely match with excitatory and inhibitory imbalance theory of epilepsy. Here, we investigated if GCD could effectively localize the Rolandic focus in 50 patients with benign childhood epilepsy with central-temporal spikes (BECTS) from 27 normal children. The BECTS were classified into ictal epileptiform discharges (IEDs; 12 females, 15 males;age, 8. Read More

View Article and Full-Text PDF

Seizures in self-limited epilepsy with centrotemporal spikes: video-EEG documentation.

Childs Nerv Syst 2020 09 13;36(9):1853-1857. Epub 2020 Jul 13.

Hospital Israelita Albert Einstein, Av. Albert Einstein, 627 - Morumbi, São Paulo, SP, CEP: 05652-900, Brazil.

Purpose: Self-limited epilepsy with centrotemporal spikes, formerly called benign epilepsy with centrotemporal spikes, or rolandic epilepsy, is an age-related and well-defined epileptic syndrome. Since seizures associated with rolandic spikes are infrequent and usually occur during sleep, and repetitive or prolonged EEG recording for diagnostic purposes is not necessary for diagnosis, reports of ictal video-electroencephalographic seizures in this syndrome are rare. We aimed to show ictal video-EEG of typical rolandic seizures. Read More

View Article and Full-Text PDF
September 2020

Oxcarbazepine monotherapy in children with benign epilepsy with centrotemporal spikes improves quality of life.

Chin Med J (Engl) 2020 Jul;133(14):1649-1654

Department of Neurology, Capital Institute of Pediatrics, Beijing 100020, China.

Background: Benign epilepsy with centrotemporal spikes (BECTS) is the most common type of childhood idiopathic focal epilepsy. BECTS is associated with pervasive cognitive deficits and behavior problems. While seizures can be easily controlled, it is crucial to select anti-epileptic drugs that do not impair cognition, do not cause psychosocial effects, and improve the quality of life. Read More

View Article and Full-Text PDF

Cortical and subcortical volume differences between Benign Epilepsy with Centrotemporal Spikes and Childhood Absence Epilepsy.

Epilepsy Res 2020 10 23;166:106407. Epub 2020 Jun 23.

Division of Neurology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave. Cincinnati, OH, 45229, USA; Pediatric Neuroimaging Research Consortium, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave. Cincinnati, OH, 45229, USA. Electronic address:

Objective: Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS) and Childhood Absence Epilepsy (CAE) are the most common childhood epilepsy syndromes and they share a similar age-dependence. However, the two syndromes clearly differ in seizures and EEG patterns. The aim of this study is to investigate whether children of the same age with BECTS, CAE and typically-developing children have significant differences in grey matter volume that may underlie the different profiles of these syndromes. Read More

View Article and Full-Text PDF
October 2020

Clinical and electroencephalographic features of benign childhood epilepsy with centrotemporal spikes comorbidity with attention-deficit hyperactivity disorder in Southwest China.

Epilepsy Behav 2020 10 27;111:107240. Epub 2020 Jun 27.

Department of Neurology, West China Hospital, Sichuan University, Wai Nan Guo Xue Lane 37#, Chengdu, Sichuan 610041, China. Electronic address:

Purpose: This study was conducted to analyze the clinical and electroencephalographic (EEG) features of attention-deficit hyperactivity disorder (ADHD) in children with benign partial epilepsy with centrotemporal spikes (BECTS) in Southwest China, to address the question of what the risk factors are for patients with BECTS who suffer from ADHD.

Methods: Overall 118 right-handed children with BECTS were included from two medical centers. Of them, 29 patients were with diagnosed ADHD according to the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-V) at baseline, and the remaining were considered as typical BECTS. Read More

View Article and Full-Text PDF
October 2020

Exome sequencing in 57 patients with self-limited focal epilepsies of childhood with typical or atypical presentations suggests novel candidate genes.

Eur J Paediatr Neurol 2020 Jul 29;27:104-110. Epub 2020 May 29.

Department of Medical Genetics, Member of the ERN EpiCARE, University Hospital of Lyon, Lyon, France; Institut NeuroMyoGène, CNRS UMR 5310 - INSERM U1217, Lyon, France; Université de Lyon - Université Claude Bernard Lyon 1, Lyon, France. Electronic address:

Objective: Self-limited focal epilepsies of childhood (SFEC) are amongst the best defined and most frequent epilepsy syndromes affecting children with usually normal developmental milestones. They include core syndromes such as Rolandic epilepsy or "Benign" epilepsy with Centro-Temporal Spikes and the benign occipital epilepsies, the early onset Panayiotopoulos syndrome and the late-onset Gastaut type. Atypical forms exist for all of them. Read More

View Article and Full-Text PDF

Identification of susceptibility variants to benign childhood epilepsy with centro-temporal spikes (BECTS) in Chinese Han population.

EBioMedicine 2020 Jul 21;57:102840. Epub 2020 Jun 21.

Guy's & St Thomas' NHS Foundation Trust and King's College London, NIHR Biomedical Research Centre, London, England United Kingdom. Electronic address:

Background: Benign Childhood Epilepsy with Centro-temporal Spikes (BECTS) is the most common form of idiopathic epilepsy in children, accounting for up to 23% of pediatric epilepsy. The pathogenesis of BECTS is unknown, but it is thought that genetic factors play a role in susceptibility to the disease.

Methods: To investigate the role of common genetic variants in BECTS pathogenesis, a 2-stage genome-wide association study (GWAS) was performed in 1,800 Chinese Han BECTS patients, and 7,090 healthy controls. Read More

View Article and Full-Text PDF

Epilepsy-causing STX1B mutations translate altered protein functions into distinct phenotypes in mouse neurons.

Brain 2020 07;143(7):2119-2138

Laboratory of Protein Biochemistry, Institute for Chemistry and Biochemistry, Freie Universität Berlin, Germany.

Syntaxin 1B (STX1B) is a core component of the N-ethylmaleimide-sensitive factor attachment protein receptor (SNARE) complex that is critical for the exocytosis of synaptic vesicles in the presynapse. SNARE-mediated vesicle fusion is assisted by Munc18-1, which recruits STX1B in the auto-inhibited conformation, while Munc13 catalyses the fast and efficient pairing of helices during SNARE complex formation. Mutations within the STX1B gene are associated with epilepsy. Read More

View Article and Full-Text PDF