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https://www.readbyqxmd.com/read/28276062/ilae-classification-of-the-epilepsies-position-paper-of-the-ilae-commission-for-classification-and-terminology
#1
Ingrid E Scheffer, Samuel Berkovic, Giuseppe Capovilla, Mary B Connolly, Jacqueline French, Laura Guilhoto, Edouard Hirsch, Satish Jain, Gary W Mathern, Solomon L Moshé, Douglas R Nordli, Emilio Perucca, Torbjörn Tomson, Samuel Wiebe, Yue-Hua Zhang, Sameer M Zuberi
The International League Against Epilepsy (ILAE) Classification of the Epilepsies has been updated to reflect our gain in understanding of the epilepsies and their underlying mechanisms following the major scientific advances that have taken place since the last ratified classification in 1989. As a critical tool for the practicing clinician, epilepsy classification must be relevant and dynamic to changes in thinking, yet robust and translatable to all areas of the globe. Its primary purpose is for diagnosis of patients, but it is also critical for epilepsy research, development of antiepileptic therapies, and communication around the world...
March 8, 2017: Epilepsia
https://www.readbyqxmd.com/read/28262406/a-novel-inherited-scn1a-mutation-associated-with-gefs-in-benign-and-encephalopathic-epilepsy
#2
Angela C Gauthier, Louis N Manganas, Richard H Mattson
Generalized epilepsy with febrile seizures plus (GEFS+) is an autosomal dominant condition often caused by mutations in SCN1A that usually first manifests as childhood simple febrile seizures but may progress to a variety of afebrile generalized seizure types. Here, we describe the case of an 8-year-old boy with a novel SCN1A mutation who developed febrile seizures at 10months of age which eventually advanced to frequent afebrile tonic-clonic seizures. His condition was unresponsive to several antiepileptic drugs and the ketogenic diet, and he experienced gradual cognitive decline...
March 2, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28258599/less-typical-features-of-benign-rolandic-epilepsy-in-chinese-children-a-retrospective-study
#3
Xiuhe Zhao, Aiqin Wang, Shengjun Wang, Tongxia Zhang, Zhaofu Chi, Yiming Liu
BACKGROUD: Benign rolandic epilepsy is one of the most common focal epilepsy in childhood. However more and more data demonstrated that less typical clinical presentations may lead to misdiagnosis and mistreatment. The focus of this study was to retrospectively investigate the less typical features of benign rolandic epilepsy in Chinese children. METHODS: Data of 316 Chinese children with BRE were collected and analyzed. RESULTS: Of all children, 7...
March 4, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28239337/local-and-distant-dysregulation-of-synchronization-around-interictal-spikes-in-bects
#4
Emilie Bourel-Ponchel, Mahdi Mahmoudzadeh, Patrick Berquin, Fabrice Wallois
Objective: High Density electroencephalography (HD EEG) is the reference non-invasive technique to investigate the dynamics of neuronal networks in Benign Epilepsy with Centro-Temporal Spikes (BECTS). Analysis of local dynamic changes surrounding Interictal Epileptic Spikes (IES) might improve our knowledge of the mechanisms that propel neurons to the hypersynchronization of IES in BECTS. Transient distant changes in the dynamics of neurons populations may also interact with neuronal networks involved in various functions that are impaired in BECTS patients...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28238620/coexistence-of-childhood-absence-epilepsy-and-benign-epilepsy-with-centrotemporal-spikes-a-case-series
#5
Alberto Verrotti, Sara Casciato, Alberto Spalice, Marco Carotenuto, Pasquale Striano, Pasquale Parisi, Nelia Zamponi, Salvatore Savasta, Victoria Elisa Rinaldi, Renato D'Alonzo, Federico Mecarini, Anthony J Ritaccio, Giancarlo Di Gennaro
AIM: Childhood absence epilepsy (CAE) and benign childhood epilepsy with centrotemporal spikes (BECTS) are the most common forms of childhood epilepsy. Recent studies in animal models suggest that the two phenotypes may represent a neurobiological continuum. Although the coexistence of CAE and BECTS has been reported, this issue remains controversial. The purpose of this study was to analyse the electro-clinical characteristics of a group of children with contemporary or subsequent features of absence seizures and focal seizures consistent with BECTS...
February 14, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28214048/psychosocial-and-behavioral-functioning-and-their-relationship-to-seizure-timing-in-children-with-benign-epilepsy-with-centrotemporal-spikes
#6
Gonca Bektaş, Uğur Tekin, Edibe Pembegül Yıldız, Tuğçe Aksu Uzunhan, Burak Tatlı, Nur Aydınlı, Mine Çalışkan, Meral Özmen
BACKGROUND: Psychosocial and behavioral problems have been reported in children with benign epilepsy with centrotemporal spikes (BECTS). Distinctive features of typical BECTS associated with cognitive and behavioral problems have not clearly been defined. PURPOSE: We aimed to identify psychosocial and behavioral functioning and their relationship to seizure timing in BECTS. METHODS: Consecutive patients with BECTS were recruited from the pediatric neurology outpatient clinic between May 2015 and May 2016...
February 14, 2017: Brain & Development
https://www.readbyqxmd.com/read/28209266/changes-in-functional-organization-and-functional-connectivity-during-story-listening-in-children-with-benign-childhood-epilepsy-with-centro-temporal-spikes
#7
Jennifer Vannest, Thomas C Maloney, Jeffrey R Tenney, Jerzy P Szaflarski, Diego Morita, Anna W Byars, Mekibib Altaye, Scott K Holland, Tracy A Glauser
Children with Benign Epilepsy with Centrotemporal Spikes (BECTS), despite high likelihood for seizure remission, are reported to have subtle difficulties in language and other cognitive skills. We used functional MRI and a story listening task to examine the effect of BECTS on patterns of activation and connectivity. Language and cognitive skills were assessed using standardized measures. Twenty-four children with recently diagnosed BECTS and 40 typically-developing children participated. In a functionally-defined region of interest in right inferior frontal gyrus, BECTS patients showed a lower level of activation...
February 13, 2017: Brain and Language
https://www.readbyqxmd.com/read/28203222/unexpected-death-of-a-child-with-complex-febrile-seizures-pathophysiology-similar-to-sudden-unexpected-death-in-epilepsy
#8
Brian J Dlouhy, Michael A Ciliberto, Christina L Cifra, Patricia A Kirby, Devin L Shrock, Marcus Nashelsky, George B Richerson
Febrile seizures are usually considered relatively benign. Although some cases of sudden unexplained death in childhood have a history of febrile seizures, no documented case of febrile seizure-induced death has been reported. Here, we describe a child with complex febrile seizures who died suddenly and unexpectedly after a suspected seizure while in bed at night during the beginning phases of sleep. She was resuscitated and pronounced brain dead 2 days later at our regional medical center. Autopsy revealed multiorgan effects of hypoperfusion and did not reveal an underlying (precipitating) disease, injury, or toxicological cause of death...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28203158/a-28-year-old-male-patient-with-nail-tumors-skin-lesions-and-epilepsy
#9
Deepak M W Balak, Bernard A Zonnenberg, Juliette M J Spitzer-Naaijkens, Mieke M Hulshof
BACKGROUND: Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by the development of benign hamartomas in multiple organs. Most patients with TSC exhibit cutaneous manifestations. METHODS: We report a 28-year-old patient with multiple pink papules at the proximal nail fold of several toes. RESULTS: Histopathological analysis of a biopsy of a papule was consistent with an ungual fibroma. Histopathological analysis of a biopsy of an elevated skin-colored plaque at the lower back was diagnostic for a Shagreen patch...
January 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28202379/comprehensive-preoperative-work-up-and-surgical-treatment-of-low-grade-tumor-benign-lesion-related-temporal-lobe-epilepsy
#10
Rui Feng, Jie Hu, Jinsong Wu, Liqin Lang, Chengxin Ma, Shize Jiang, Bing Sun, Xin Gu, Li Pan
OBJECTIVE: Generally low-grade tumor/benign lesion related temporal lobe epilepsy (LGT/BL-TLE) is considered easier to treat and has better prognosis when compared to non-lesional TLE. However, multiple disputes exist in surgical management of this epilepsy entity. This study aims to discuss comprehensive preoperative work-up, surgical strategies and outcome of it. METHODS: A retrospective review of sixty LGT/BL-TLE cases which underwent comprehensive preoperative work-up and then resective surgeries was conducted...
February 12, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28199897/real-world-utility-of-whole-exome-sequencing-with-targeted-gene-analysis-for-focal-epilepsy
#11
Piero Perucca, Ingrid E Scheffer, A Simon Harvey, Paul A James, Sebastian Lunke, Natalie Thorne, Clara Gaff, Brigid M Regan, John A Damiano, Michael S Hildebrand, Samuel F Berkovic, Terence J O'Brien, Patrick Kwan
OBJECTIVE: Driven by advances in genomic technology and reduction in costs, next-generation sequencing (NGS) is venturing into routine clinical care. The 'real-world' clinical utility of NGS remains to be determined in focal epilepsies, which account for 60% of all epilepsies and for which the importance of genetic factors is just beginning to emerge. We investigated the diagnostic yield and management implications of whole exome sequencing (WES)-based screening of selected genes in the routine care of common focal epilepsies suspected to have a genetic basis...
February 7, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28121917/clinical-features-of-benign-epilepsy-of-childhood-with-centrotemporal-spikes-in-chinese-children
#12
MULTICENTER STUDY
Meng-Jia Liu, Xiao-Jun Su, Xiu-Yu Shi Md, Ge-Fei Wu, Yu-Qin Zhang, Li Gao, Wei Wang, Jian-Xiang Liao, Hua Wang, Jian-Ning Mai, Jing-Yun Gao, Xiao-Mei Shu, Shao-Ping Huang, Li Zhang, Li-Ping Zou
This multicenter clinical trial was conducted to examine current practice of benign epilepsy with centrotemporal spikes and especially address the question that in what circumstances 1 antiepileptic drug (AED) should be preferred.Twenty-five medical centers participate in this clinical trial. The general information, clinical information, and treatment status were collected under the guidance of clinicians and then analyzed. Difference between different treatment groups was compared, and usefulness of the most commonly used AEDs was evaluated...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28110926/distributed-source-localization-of-interictal-spikes-in-benign-childhood-epilepsy-with-centrotemporal-spikes-a-standardized-low-resolution-brain-electromagnetic-tomography-sloreta-study
#13
Tae-Hoon Eom, Jung-Hyun Shin, Young-Hoon Kim, Seung-Yun Chung, In-Goo Lee, Jung-Min Kim
Benign childhood epilepsy with centrotemporal spikes (BCECTS), also known as Rolandic epilepsy, is the most common benign childhood epilepsy. Centrotemporal spikes are characteristic findings on electroencephalography (EEG). Though the condition is considered benign, many studies have reported some degree of neuropsychological impairment in individuals with BCECTS. There is also growing evidence from neuroimaging studies that BCECTS may affect a larger portion of the brain than originally thought. We performed distributed source localization analysis of interictal spikes in BCECTS...
April 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28105459/mog-antibody-positive-benign-unilateral-cerebral-cortical-encephalitis-with-epilepsy
#14
Ryo Ogawa, Ichiro Nakashima, Toshiyuki Takahashi, Kimihiko Kaneko, Tetsuya Akaishi, Yoshiki Takai, Douglas Kazutoshi Sato, Shuhei Nishiyama, Tatsuro Misu, Hiroshi Kuroda, Masashi Aoki, Kazuo Fujihara
OBJECTIVE: To describe the features of adult patients with benign, unilateral cerebral cortical encephalitis positive for the myelin oligodendrocyte glycoprotein (MOG) antibody. METHODS: In this retrospective, cross-sectional study, after we encountered an index case of MOG antibody-positive unilateral cortical encephalitis with epileptic seizure, we tested for MOG antibody using our in-house, cell-based assay in a cohort of 24 consecutive adult patients with steroid-responsive encephalitis of unknown etiology seen at Tohoku University Hospital (2008-2014)...
March 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28101769/childhood-absence-epilepsy-and-benign-epilepsy-with-centro-temporal-spikes-a-narrative-review-analysis
#15
REVIEW
Alberto Verrotti, Renato D'Alonzo, Victoria Elisa Rinaldi, Sara Casciato, Alfredo D'Aniello, Giancarlo Di Gennaro
BACKGROUND: Recent studies have shown a possible coexistence of absence seizures with other forms of epilepsy. The purpose of this study was to ascertain the possible contemporary or subsequent presence of childhood absence epilepsy (CAE) and benign epilepsy with centro-temporal spikes (BECTS) in pediatric epileptic patients. METHODS: A PubMed systematic search indexed for MEDLINE, PubMed and EMBASE was undertaken to identify studies in children including articles written between 1996 and 2015...
January 19, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28098941/posterior-cortex-epilepsy-surgery-in-childhood-and-adolescence-predictors-of-long-term-seizure-outcome
#16
Georgia Ramantani, Angeliki Stathi, Armin Brandt, Karl Strobl, Susanne Schubert-Bast, Gert Wiegand, Rudolf Korinthenberg, Vera van Velthoven, Josef Zentner, Andreas Schulze-Bonhage, Thomas Bast
OBJECTIVE: We aimed to investigate the long-term seizure outcome of children and adolescents who were undergoing epilepsy surgery in the parietooccipital cortex and determine their predictive factors. METHODS: We retrospectively analyzed the data of 50 consecutive patients aged 11.1 (mean) ± 5.1 (standard deviation) years at surgery. All patients but one had a magnetic resonance imaging (MRI)-visible lesion. Resections were parietal in 40%, occipital in 32%, and parietooccipital in 28% cases; 24% patients additionally underwent a resection of the posterior border of the temporal lobe...
January 18, 2017: Epilepsia
https://www.readbyqxmd.com/read/28078877/congenital-muscular-dystrophy-and-epilepsy-a-prospective-case-series-of-pediatric-patients
#17
LETTER
G Vitaliti, P Pavone, C Romano, M Barbagallo, M Vecchio, C Ledda, R Lubrano, R Falsaperla
Congenital Muscular Dystrophies (CMDs) can be considered as a heterogeneous group of diseases characterized by marked weakness, generalized hypotonia and joint contractures. They are divided into pure and classical forms, without ocular and cerebral involvement, and complex forms, which are associated with cerebral abnormalities. Seizures have rarely been described in the pure forms while they seem to occur more frequently in complex forms. The aim of our study was to evaluate the incidence of seizure in CMD...
October 2016: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28064325/childhood-epilepsies-what-should-a-pediatrician-know
#18
Fahad A Bashiri
Seizures in children are among the most common neurological disorders. A pediatrician should know how to approach a child who presents with a seizure. This review will focus on points that are important in the evaluation of children who have experienced seizures. A comprehensive and neurologically focused framework for history taking and a thorough clinical examination are the cornerstones in diagnosing and managing seizures. This article reviews the clinical approach to the diagnosis, investigation, and management of epilepsy in children, excluding neonatal seizures...
January 2017: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
https://www.readbyqxmd.com/read/28038823/a-kcnq2-e515d-mutation-associated-with-benign-familial-neonatal-seizures-and-continuous-spike-and-waves-during-slow-wave-sleep-syndrome-in-taiwan
#19
Inn-Chi Lee, Jiann-Jou Yang, Shuan-Yow Li
BACKGROUND/PURPOSE: Pediatric epilepsy caused by a KCNQ2 gene mutation usually manifests as benign familial neonatal seizures (BFNS) during the 1(st) week of life. However, the exact mechanism, phenotype, and genotype of the KCNQ2 mutation are unclear. METHODS: We studied the KCNQ2 genotype from 75 nonconsanguineous patients with childhood epilepsy without an identified cause (age range: from 2 days to 18 years) and from 55 healthy adult controls without epilepsy...
December 27, 2016: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28026919/a-comprehensive-assessment-of-cognitive-function-in-the-common-genetic-generalized-epilepsy-syndromes
#20
A Loughman, S C Bowden, W J D'Souza
BACKGROUND AND PURPOSE: Considered to be benign conditions, the common genetic generalized epilepsy (GGE) syndromes are now known to be frequently accompanied by cognitive dysfunction. However, unresolved issues impede clinical management of this common comorbidity, including which cognitive abilities are most affected, whether there are differences between syndromes and how seizure type and mood symptoms affect cognitive dysfunction. We provide a detailed description of cognitive ability and evaluate factors contributing to cognitive dysfunction...
December 27, 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
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