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https://www.readbyqxmd.com/read/28870817/-prrt2-mutation-and-infantile-convulsions
#1
M Mathot, D Lederer, S Gerard, E Gueulette, M Deprez
New genetic techniques have made it possible to better understand the implications of the PRRT2 gene (proline rich transmembrane protein 2) in various neurological disorders. Mutations within this gene are responsible for kinesigenic paroxysmal dyskinesias (PKD) as well as for benign familial infantile epilepsy (BFIE), a disease associating infantile convulsions and choreoathetosis (ICCA), a form of familial hemiplegic migraine (FHM type 4), paroxysmal benign torticollis of childhood, and episodic ataxia. We describe the case of an infant, carrying a mutation of the PRRT2 gene, with a classical presentation...
September 1, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28866336/-atypical-forms-of-benign-epilepsy-with-centrotemporal-spikes-bects-how-to-diagnose-and-guide-these-children-a-practical-scientific-approach
#2
REVIEW
Pasquale Parisi, Maria Chiara Paolino, Umberto Raucci, Alessandro Ferretti, Maria Pia Villa, Dorothee Kasteleijn-Nolst Trenite
Benign epilepsy with centrotemporal spikes (BECTS) epilepsy, also known as rolandic epilepsy, is the most common childhood type of epilepsy. There is debate on its "benign" definition given the numerous literature data on its correlation to cognitive morbidity. Although its prognosis is often favorable, BECTS can present or evolve however to an atypical form, characterized by a worse prognosis and negative impact on cognitive development. It is possible that abnormal electrical activity, marker of neurological dysfunction, has the potential to disrupt neural network function and development...
August 31, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28822871/altered-language-network-in-benign-childhood-epilepsy-patients-with-spikes-from-non-dominant-side-a-resting-state-fmri-study
#3
Jiajia Fang, Sihan Chen, Cheng Luo, Qiyong Gong, Dongmei An, Dong Zhou
Benign childhood epilepsy with centrotemporal spikes (BECTS) is one of the most common childhood epilepsy syndromes, and language deficits associated with BECTS have become a hot topic. This study investigated alterations of the language network in BECTS children with spikes from the non-dominant side in comparison with healthy controls. Twenty-three children with BECTS and 20 age-matched healthy controls were enrolled. Region of interest -based whole brain functional connectivity analysis was used to identify the potential differences in the functional connectivity of the Broca's area between the two groups...
August 1, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28800038/subclinical-rhythmic-eeg-discharge-of-adult-sreda-in-a-child-with-generalized-epilepsy-and-literature-review-of-sreda-in-children
#4
Marcie Goeden, Lalit R Bansal
Subclinical rhythmic discharges of adult (SREDA) is a rare benign EEG variant in adults and is of unknown clinical significance. Its occurrence in children is extremely rare. In review of the literature, it has been described in only four children. We present a case of a 10-year-old female with generalized idiopathic childhood absence epilepsy who is noted to have SREDA in three subsequent EEGs performed across a 25-month span. She had no clinical change with these discharges and it was believed to be a benign variant...
August 9, 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28762475/behavioral-and-fmri-responses-to-fearful-faces-are-altered-in-benign-childhood-epilepsy-with-centrotemporal-spikes-bcects
#5
Carolina Ciumas, Agathe Laurent, Mani Saignavongs, Faustine Ilski, Julitta de Bellescize, Eleni Panagiotakaki, Karine Ostrowsky-Coste, Alexis Arzimanoglou, Vania Herbillon, Danielle Ibarrola, Philippe Ryvlin
OBJECTIVE: We hypothesized that children with benign childhood epilepsy with centrotemporal spikes (BCECTS) might have altered social cognitive skills and underlying neural networks. METHODS: We studied 13 patients with BCECTS and 11 age-matched controls using event-related functional magnetic resonance imaging (fMRI) with an emotional discrimination task consisting of viewing happy, fearful, scrambled, and neutral faces. Behavioral performance measured during the task was correlated with clinical variables and behavioral ratings...
July 31, 2017: Epilepsia
https://www.readbyqxmd.com/read/28734769/influence-of-epileptic-activity-during-sleep-on-cognitive-performance-in-benign-childhood-epilepsy-with-centrotemporal-spikes
#6
Andreea Nissenkorn, Adi Pappo, Yael Feldmann, Gali Heimer, Omer Bar-Yosef, Michal Tzadok, Orli Polack, Ayelet Bord, Miriam Levav, Bruria Ben-Zeev
BACKGROUND: Benign childhood epilepsy with centrotemporal spikes is benign childhood epilepsy, presenting between 4 and 10 years of age, characterized by typical clinical and EEG findings. Despite excellent prognosis, there are reports of mild cognitive, language, fine motor and behavioral difficulties. In its atypical form - electrical status epilepticus during slow wave sleep, continuous epileptiform activity during sleep lead to severe neurocognitive deterioration. Our objective was to investigate the influence of abundant sleep epileptiform activity, not fulfilling the criteria for electrical status epilepticus during Slow Wave Sleep, discovered randomly in children without overt intellectual impairment...
July 11, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28686619/rare-variants-of-small-effect-size-in-neuronal-excitability-genes-influence-clinical-outcome-in-japanese-cases-of-scn1a-truncation-positive-dravet-syndrome
#7
Michael F Hammer, Atsushi Ishii, Laurel Johnstone, Alexander Tchourbanov, Branden Lau, Ryan Sprissler, Brian Hallmark, Miao Zhang, Jin Zhou, Joseph Watkins, Shinichi Hirose
Dravet syndrome (DS) is a rare, devastating form of childhood epilepsy that is often associated with mutations in the voltage-gated sodium channel gene, SCN1A. There is considerable variability in expressivity within families, as well as among individuals carrying the same primary mutation, suggesting that clinical outcome is modulated by variants at other genes. To identify modifier gene variants that contribute to clinical outcome, we sequenced the exomes of 22 individuals at both ends of a phenotype distribution (i...
2017: PloS One
https://www.readbyqxmd.com/read/28623837/abnormal-asymmetry-in-benign-epilepsy-with-unilateral-and-bilateral-centrotemporal-spikes-a-combined-fmri-and-dti-study
#8
Weifang Cao, Yaodan Zhang, Changyue Hou, Fei Yang, Jinnan Gong, Sisi Jiang, Yue Huang, Ruhui Xiao, Cheng Luo, Xiaoming Wang, Dezhong Yao
Benign epilepsy with centrotemporal spikes (BECTS) is the most common idiopathic focal childhood epilepsy associated with either unilateral or bilateral epileptic discharge. Asymmetry as an important characteristic of the human brain is beneficial for brain functions. However, little is known about on asymmetry of BECTS patients with different epileptic spikes pattern. In the present study, we investigated functional and structural asymmetries in unilateral spikes BECTS (U_BECTS) patients and bilateral spikes BECTS (B_BECTS) patients using resting state functional magnetic resonance images and diffusion tensor imaging...
June 8, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28622557/the-executive-profile-of-children-with-benign-epilepsy-of-childhood-with-centrotemporal-spikes-and-temporal-lobe-epilepsy
#9
Ellen M Lima, Patricia Rzezak, Catarina A Guimarães, Maria A Montenegro, Marilisa M Guerreiro, Kette D Valente
RATIONALE: Benign Epilepsy of Childhood with Centrotemporal Spikes (BECTS) and temporal lobe epilepsy (TLE) represent two distinct models of focal epilepsy of childhood. In both, there is evidence of executive dysfunction. The purpose of the present study was to identify particular deficits in the executive function that would distinguish children with BECTS from children with TLE. METHODS: We prospectively evaluated 19 consecutive children and adolescents with TLE with hippocampal sclerosis (HS) (57...
June 13, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28602030/variable-expressivity-of-a-likely-pathogenic-variant-in-kcnq2-in-a-three-generation-pedigree-presenting-with-intellectual-disability-with-childhood-onset-seizures
#10
Stacy Hewson, Klajdi Puka, Saadet Mercimek-Mahmutoglu
KCNQ2 has been reported as a frequent cause of autosomal dominant benign familial neonatal seizures. De novo likely pathogenic variants in KCNQ2 have been described in neonatal or early infantile onset epileptic encephalopathy patients. Here, we report a three-generation family with six affected patients with a novel likely pathogenic variant (c.628C>T; p.Arg210Cys) in KCNQ2. Four family members, three adults and a child, presented with a childhood seizure onset with variability in the severity of seizures and response to treatment, intellectual disability (ID) as well as behavioral problems...
June 11, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28593865/the-semiology-of-benign-focal-epilepsy-with-affective-symptoms
#11
Fábio A Nascimento, Márcio A Sotero de Menezes, Cristiane A Simão, Bruno T Takeshita, Samanta F Blattes da Rocha, Pedro A Kowacs
Benign focal epilepsy with affective symptoms (BFEAS) is a rare childhood epilepsy syndrome essentially characterized by "epileptic attacks with affective symptoms of a terrifying type". Since the original description, approximately 50 cases have been reported. To our knowledge, however, none of the studies included video-EEG data. Herein, we detail the electroclinical features of a neurodevelopmentally normal 9-year-old boy with epilepsy since the age of 2 years. His seizure semiology essentially consisted of nocturnal focal seizures featuring abrupt fear and autonomic phenomena (such as excessive sweating, repeated swallowing, and coughing), associated with impaired consciousness...
June 1, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/28575760/benign-childhood-epilepsy-with-centrotemporal-spikes-bects-and-developmental-co-ordination-disorder
#12
Amanda Kirby, Natalie Williams, Loes Koelewijn, Lisa M Brindley, Suresh D Muthukumaraswamy, Johann Te Water Naudé, Marie Thomas, Frances Gibbon, Krish D Singh, Khalid Hamandi
BACKGROUND: Benign epilepsy with centro-temporal spikes (BECTS) is a common childhood epilepsy syndrome also known as Rolandic Epilepsy (RE). Neurocognitive phenotypes have been described with greater focus on attention, reading and language domains but there have been far fewer studies focusing on motor functioning. This study included measures of motor, language and cognition in order to investigate the range, degree and pattern of difficulties associated with BECTS in a case series of children, but with a particular emphasis on motor skills...
May 30, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28535638/-application-of-long-term-video-electroencephalogram-and-resting-state-functional-magnetic-resonance-imaging-in-detection-of-cognition-in-patients-with-benign-epilepsy-of-childhood-with-centrotemporal-spikes
#13
X X Yan, Q Yu, Y T Gao, L T Li, D H Yu, Y Chen, X J Yao, W D Yang, Z J Chen, J Z Yin, Y An, K Tan
Objective: To study the relationship between the changes of brain network and cognition in patients with benign epilepsy of childhood with centrotemporal spikes (BECTS) by using long term video electroencephalogram (VEEG) and resting-state functional magnetic resonance imaging (RS-fMRI) technology. Methods: Eleven patients with right-handed were recruited (from April 2015 to September 2016) from epilepsy specialist outpatients and functional department of neurosurgery of Tianjin Medical University General Hospital...
May 23, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28533141/common-and-distinctive-patterns-of-cognitive-dysfunction-in-children-with-benign-epilepsy-syndromes
#14
Dazhi Cheng, Xiuxian Yan, Zhijie Gao, Keming Xu, Xinlin Zhou, Qian Chen
BACKGROUND: Childhood absence epilepsy and benign childhood epilepsy with centrotemporal spikes are the most common forms of benign epilepsy syndromes. Although cognitive dysfunctions occur in children with both childhood absence epilepsy and benign childhood epilepsy with centrotemporal spikes, the similarity between their patterns of underlying cognitive impairments is not well understood. To describe these patterns, we examined multiple cognitive functions in children with childhood absence epilepsy and benign childhood epilepsy with centrotemporal spikes...
July 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28518218/comparison-and-optimization-of-in-silico-algorithms-for-predicting-the-pathogenicity-of-sodium-channel-variants-in-epilepsy
#15
COMPARATIVE STUDY
Katherine D Holland, Thomas M Bouley, Paul S Horn
OBJECTIVE: Variants in neuronal voltage-gated sodium channel α-subunits genes SCN1A, SCN2A, and SCN8A are common in early onset epileptic encephalopathies and other autosomal dominant childhood epilepsy syndromes. However, in clinical practice, missense variants are often classified as variants of uncertain significance when missense variants are identified but heritability cannot be determined. Genetic testing reports often include results of computational tests to estimate pathogenicity and the frequency of that variant in population-based databases...
July 2017: Epilepsia
https://www.readbyqxmd.com/read/28506485/surgery-for-dysembryoplastic-neuroepithelial-tumors-and-gangliogliomas-in-eloquent-areas-functional-results-and-seizure-control
#16
B Devaux, F Chassoux, E Landré, B Turak, A Laurent, M Zanello, C Mellerio, P Varlet
INTRODUCTION: Dysembryoplastic neuroepithelial tumors and gangliogliomas are developmental glioneuronal tumors usually revealed by partial epilepsy. High epileptogenicity, childhood epilepsy onset, drug-resistance, temporal location, and seizure freedom after complete resection are common characteristics of both tumors. We report the specificity of surgical management, functional results and seizure outcome in cases of a tumor location in eloquent areas. METHODS: Among 150 patients (88 males, 3-55 years) operated on for refractory epilepsy due to a glioneuronal tumor (1990-2015), 30 (20%, dysembryoplastic neuroepithelial tumors=21; gangliogliomas=9) had a tumor located in an eloquent cortex (sensory-motor, insular or language areas)...
June 2017: Neuro-Chirurgie
https://www.readbyqxmd.com/read/28391031/evaluation-of-the-language-profile-in-children-with-rolandic-epilepsy-and-developmental-dysphasia-evidence-for-distinct-strengths-and-weaknesses
#17
M Verly, R Gerrits, L Lagae, S Sunaert, N Rommel, I Zink
Although benign, rolandic epilepsy (RE) or benign childhood epilepsy with centro-temporal spikes is often associated with language impairment. Recently, fronto-rolandic EEG abnormalities have been described in children with developmental dysphasia (DD), suggesting an interaction between language impairment and interictal epileptiform discharges. To investigate if a behavioral-linguistic continuum between RE and DD exists, a clinical prospective study was carried out to evaluate the language profile of 15 children with RE and 22 children with DD...
July 2017: Brain and Language
https://www.readbyqxmd.com/read/28384699/sudden-unexpected-death-in-epilepsy-among-patients-with-benign-childhood-epilepsy-with-centrotemporal-spikes
#18
Kyra Doumlele, Daniel Friedman, Jeffrey Buchhalter, Elizabeth J Donner, Jay Louik, Orrin Devinsky
Importance: Children with benign epilepsy with centrotemporal spikes (BECTS) have traditionally been considered to have a uniformly good prognosis. However, benign may be a misnomer because BECTS is linked to cognitive deficits, a more severe phenotype with intractable seizures, and the potential for sudden unexpected death in epilepsy (SUDEP). Objective: To determine if cases of BECTS are present in the North American SUDEP Registry (NASR). Design, Setting, and Participants: The NASR is a clinical and biospecimen repository established in 2011 to promote SUDEP research...
June 1, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28359223/epileptic-discharge-related-functional-connectivity-within-and-between-networks-in-benign-epilepsy-with-centrotemporal-spikes
#19
Rong Li, Gong-Jun Ji, Yangyang Yu, Yang Yu, Mei-Ping Ding, Ye-Lei Tang, Huafu Chen, Wei Liao
Benign epilepsy with centrotemporal spikes (BECTS) is a common childhood epilepsy syndrome associated with abnormalities in neurocognitive domains, particularly during interictal epileptiform discharges (IEDs). Here, we investigated the effects of IEDs on brain's intrinsic connectivity networks in 43 BECTS patients and 28 matched healthy controls (HCs). Patients were further divided into IED and non-IED subgroups based on simultaneous EEG-fMRI recordings. Functional connectivity within and between five networks, corresponding to seizure origination and cognitive processes, were analyzed to measure IED effects...
January 10, 2017: International Journal of Neural Systems
https://www.readbyqxmd.com/read/28351718/progress-in-unraveling-the-genetic-etiology-of-rolandic-epilepsy
#20
REVIEW
Weixi Xiong, Dong Zhou
Rolandic epilepsy (RE), or benign epilepsy of childhood with centrotemporal spikes (BECT), is the most frequent idiopathic partial epilepsy syndrome of childhood, where the "idiopathic" implies a genetic predisposition. Although RE has long been presumed to have a genetic component, clinical and genetic studies have shown a complex inheritance pattern. Furthermore, the underlying major genetic influence in RE has been challenged by recent reports of twin studies. Meanwhile, many genes or loci have been shown to be associated the RE/atypical RE (ARE) spectrum, with a higher frequency of causative variants in ARE...
April 2017: Seizure: the Journal of the British Epilepsy Association
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