Full Text Journal Articles about
Benign Childhood Epilepsy

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Electrical status epilepticus in sleep affects intrinsically connected networks in patients with benign childhood epilepsy with centrotemporal spikes.

Wen He, Hongsheng Liu, Zhenqing Liu, Qianqian Wu,

BACKGROUND:Although outcomes of benign childhood epilepsy with centrotemporal spikes (BECTS) are frequently excellent, some atypical forms of BECTS, especially electrical status epilepticus in sleep (ESES), are characterized by worse outcomes and negative impacts on cognitive development. METHODS:To explore specific ESES-related brain networks in patients with BECTS, we used resting-state functional ... Read more >>

Epilepsy Behav (Epilepsy & behavior : E&B)
[2020, 106:107032]

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Impact of CYP2C19 Phenotypes on Clinical Efficacy of Stiripentol in Japanese Patients With Dravet Syndrome.

Yoshiaki Yamamoto, Yukitoshi Takahashi, Hiroko Ikeda, Katsumi Imai, Yoshiyuki Kagawa, Yushi Inoue,

BACKGROUND:Stiripentol is a strong inhibitor of CYP2C19 and CYP3A4. This study compared the effect of stiripentol on the pharmacokinetics of clobazam and N-desmethyl-clobazam (NCLB; an active metabolite of clobazam) between different CYP2C19 phenotypes. We also evaluated the clinical impact of CYP2C19 phenotypes in Japanese patients with Dravet syndrome receiving a ... Read more >>

Ther Drug Monit (Therapeutic drug monitoring)
[2020, 42(2):302-308]

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Fenfluramine: New Treatment for Seizures in Dravet Syndrome.

Joanna Garcia Pierce, Divakar S Mithal,

Investigators for the FAiRE DS Study Group assessed the efficacy and safety of Fenfluramine for treating seizures in patients less than 18 y.o. with Dravet Syndrome in an international double-blind, placebo-controlled clinical trial. A total of 119 patients (mean age 9.0 y, 54% male) were enrolled in the study. ... Read more >>

Pediatr Neurol Briefs (Pediatric Neurology Briefs)
[2020, 34:8]

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Electroencephalographic abnormalities are correlated with cognitive deficits in children with benign childhood epilepsy with centrotemporal spikes: A clinical study of 61 cases.

Jun Zhang, Haoran Yang, Dan Wu, Chenxi Yang, Yanfeng Yang, Wei Zhou, Xiaoling Zhang, Wei Sun,

OBJECTIVE:The objective of this study was to explore the effect of spikes on cognition in patients with benign childhood epilepsy with centrotemporal spikes (BECTS) and to identify electroencephalography (EEG) markers enabling early detection of cognitive impairment. METHODS:Sixty-one children with BECTS diagnoses and 60 age- and education-matched healthy controls were enrolled. ... Read more >>

Epilepsy Behav (Epilepsy & behavior : E&B)
[2020, 106:107012]

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CACNA1H variants are not a cause of monogenic epilepsy.

Jeffrey D Calhoun, Alexandra M Huffman, Irena Bellinski, Lisa Kinsley, Elizabeth Bachman, Elizabeth Gerard, Jennifer A Kearney, Gemma L Carvill,

CACNA1H genetic variants were originally reported in a childhood absence epilepsy cohort. Subsequently, genetic testing for CACNA1H became available and is currently offered by commercial laboratories. However, the current status of CACNA1H as a monogenic cause of epilepsy is controversial, highlighted by ClinGen's recent re-classification of CACNA1H as disputed. We ... Read more >>

Hum. Mutat. (Human mutation)
[2020, :]

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Cortical Excitability, Synaptic Plasticity, and Cognition in Benign Epilepsy With Centrotemporal Spikes: A Pilot TMS-EMG-EEG Study.

Fiona M Baumer, Kristina Pfeifer, Adam Fogarty, Dalia Pena-Solorzano, Camarin E Rolle, Joanna L Wallace, Alexander Rotenberg, Robert S Fisher,

PURPOSE:Children with benign epilepsy with centrotemporal spikes have rare seizures emerging from the motor cortex, which they outgrow in adolescence, and additionally may have language deficits of unclear etiology. We piloted the use of transcranial magnetic stimulation paired with EMG and EEG (TMS-EMG, TMS-EEG) to test the hypotheses that net ... Read more >>

J Clin Neurophysiol (Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society)
[2020, 37(2):170-180]

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Abdominal Epilepsy, a Rare Cause of Abdominal Pain: The Need to Investigate Thoroughly as Opposed to Making Rapid Attributions of Psychogenic Causality.

Giuliano Lo Bianco, Simon Thomson, Simone Vigneri, Hannah Shapiro, Michael E Schatman,

J Pain Res (Journal of Pain Research)
[2020, 13:457-460]

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Cardiac arrhythmias in Dravet syndrome: an observational multicenter study.

Sharon Shmuely, Rainer Surges, Robert M Helling, W Boudewijn Gunning, Eva H Brilstra, Judith S Verhoeven, J Helen Cross, Sanjay M Sisodiya, Hanno L Tan, Josemir W Sander, Roland D Thijs,

OBJECTIVES:We ascertained the prevalence of ictal arrhythmias to explain the high rate of sudden unexpected death in epilepsy (SUDEP) in Dravet syndrome (DS). METHODS:We selected cases with clinical DS, ≥6 years, SCN1A mutation, and ≥1 seizure/week. Home-based ECG recordings were performed for 20 days continuously. Cases were matched for age and sex to ... Read more >>

Ann Clin Transl Neurol (Annals of clinical and translational neurology)
[2020, :]

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A propositional AI system for supporting epilepsy diagnosis based on the 2017 epilepsy classification: Illustrated by Dravet syndrome.

Kuo-Liang Chiang, Chin-Yin Huang, Liang-Po Hsieh, Kai-Ping Chang,

PURPOSE:The 2017 epilepsy and seizure diagnosis framework emphasizes epilepsy syndromes and the etiology-based approach. We developed a propositional artificial intelligence (AI) system based on the above concepts to support physicians in the diagnosis of epilepsy. METHODS:We analyzed and built ontology knowledge for the classification of seizure patterns, epilepsy, epilepsy syndrome, ... Read more >>

Epilepsy Behav (Epilepsy & behavior : E&B)
[2020, 106:107021]

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Focal and generalized seizure activity after local hippocampal or cortical ablation of NaV 1.1 channels in mice.

Nico A Jansen, Anisa Dehghani, Cor Breukel, Else A Tolner, Arn M J M van den Maagdenberg,

Early onset seizures are a hallmark of Dravet syndrome. Previous studies in rodent models have shown that the epileptic phenotype is caused by loss-of-function of voltage-gated NaV 1.1 sodium channels, which are chiefly expressed in γ-aminobutyric acid (GABA)ergic neurons. Recently, a possibly critical role has been attributed to the hippocampus ... Read more >>

Epilepsia (Epilepsia)
[2020, :]

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Interneuron Desynchronization Precedes Seizures in a Mouse Model of Dravet Syndrome.

Conny H Tran, Michael Vaiana, Johan Nakuci, Ala Somarowthu, Kevin M Goff, Nitsan Goldstein, Priya Murthy, Sarah F Muldoon, Ethan M Goldberg,

Recurrent seizures, which define epilepsy, are transient abnormalities in the electrical activity of the brain. The mechanistic basis of seizure initiation, and the contribution of defined neuronal subtypes to seizure pathophysiology, remains poorly understood. We performed in vivo two-photon calcium imaging in neocortex during temperature-induced seizures in male and female ... Read more >>

J. Neurosci. (The Journal of neuroscience : the official journal of the Society for Neuroscience)
[2020, 40(13):2764-2775]

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Epilepsy: key experimental therapeutics in early clinical development.

Claude Steriade, Jacqueline French, Orrin Devinsky,

Introduction: Antiseizure medications are the mainstay of epilepsy treatment. Currently therapies are not specific to epilepsy etiology, and control seizures in two-thirds of cases. Drugs in clinical development aim to bridge that gap by targeting novel receptors and epileptogenesis. While currently approved antiseizure medications target focal or generalized epilepsies regardless ... Read more >>

Expert Opin Investig Drugs (Expert opinion on investigational drugs)
[2020, :1-11]

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Dose-Ranging Effect of Adjunctive Oral Cannabidiol vs Placebo on Convulsive Seizure Frequency in Dravet Syndrome: A Randomized Clinical Trial.

Ian Miller, Ingrid E Scheffer, Boudewijn Gunning, Rocio Sanchez-Carpintero, Antonio Gil-Nagel, M Scott Perry, Russell P Saneto, Daniel Checketts, Eduardo Dunayevich, Volker Knappertz, ,

Importance:Clinical evidence supports effectiveness of cannabidiol for treatment-resistant seizures in Dravet syndrome, but this trial is the first to evaluate the 10-mg/kg/d dose. Objective:To evaluate the efficacy and safety of a pharmaceutical formulation of cannabidiol, 10 and 20 mg/kg/d, vs placebo for adjunctive treatment of convulsive seizures in patients with ... Read more >>

JAMA Neurol (JAMA neurology)
[2020, :]

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What has been the impact of new drug treatments on epilepsy?

Zhibin Chen, Martin J Brodie, Patrick Kwan,

PURPOSE OF REVIEW:Nearly two dozen antiseizure medications (ASMs) with different mechanisms of action have been introduced over the past three decades with the aim of providing better efficacy or safety profile than the previous drugs. Several new ASMs with improvement on a classic drug family or have novel mechanisms of ... Read more >>

Curr. Opin. Neurol. (Current opinion in neurology)
[2020, 33(2):185-190]

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Moving more: physical activity and its positive effects on long term conditions in children and young people.

Paul Dimitri, Kush Joshi, Natasha Jones, ,

While the benefits of regular participation in physical activity in children and young people are clear, misconceptions have developed about the possible negative effects and potential complications of exercise on long-term conditions such as epilepsy, asthma and diabetes. Over the last decade evidence has emerged supporting the positive impact that ... Read more >>

Arch. Dis. Child. (Archives of disease in childhood)
[2020, :]

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Fenfluramine acts as a positive modulator of sigma-1 receptors.

Parthena Martin, Peter A M de Witte, Tangui Maurice, Arnold Gammaitoni, Gail Farfel, Bradley Galer,

OBJECTIVE:Adjunctive fenfluramine hydrochloride, classically described as acting pharmacologically through a serotonergic mechanism, has demonstrated a unique and robust clinical response profile with regard to its magnitude, consistency, and durability of effect on seizure activity in patients with pharmacoresistant Dravet syndrome. Recent findings also support long-term improvements in executive functions (behavior, ... Read more >>

Epilepsy Behav (Epilepsy & behavior : E&B)
[2020, 105:106989]

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Adjunctive Cannabidiol in Patients with Dravet Syndrome: A Systematic Review and Meta-Analysis of Efficacy and Safety.

Simona Lattanzi, Francesco Brigo, Eugen Trinka, Gaetano Zaccara, Pasquale Striano, Cinzia Del Giovane, Mauro Silvestrini,

BACKGROUND:Dravet syndrome (DS) is one of the most severe forms of drug-resistant epilepsy and available interventions fail to control seizures in most patients. Cannabidiol (CBD) is the first in a new class of antiepileptic drugs with a distinctive chemical structure and mechanism of action. OBJECTIVE:The aim of this systematic review ... Read more >>

CNS Drugs (CNS drugs)
[2020, 34(3):229-241]

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Focal cortical hypermetabolism in atypical benign rolandic epilepsy.

Sarah R Curnow, Simon J Vogrin, Sarah Barton, Catherine A Bailey, A Simon Harvey,

OBJECTIVE:Atypical benign rolandic epilepsy (BRE) is an underrecognized and poorly understood manifestation of a common epileptic syndrome. Most consider it a focal epileptic encephalopathy in which frequent, interictal, centrotemporal spikes lead to negative motor seizures and interfere with motor and sometimes speech and cognitive abilities. We observed focal cortical hypermetabolism ... Read more >>

Epilepsy Res. (Epilepsy research)
[2020, 161:106288]

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Vaccination management in an asymptomatic child with a novel SCN1A variant and family history of status epilepticus following vaccination: A case report on a potential new direction in personalised medicine.

Lucy Deng, Alan Ma, Nicholas Wood, Simone Ardern-Holmes,

PURPOSE:SCN1A variants cause a spectrum of epilepsy syndromes from Dravet Syndrome, a severe epileptic encephalopathy of early infancy to the milder disorder of genetic epilepsy with febrile seizures plus (GEFS+). These genetic epilepsies are associated with increased risk of poor outcome including complications of status epilepticus and early mortality. Individualised ... Read more >>

Seizure (Seizure)
[2020, 78:49-52]

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Distal esophageal spasm: Update on diagnosis and management in the era of high-resolution manometry.

Harika Gorti, Salih Samo, Nikrad Shahnavaz, Emad Qayed,

Distal esophageal spasm (DES) is a rare major motility disorder in the Chicago classification of esophageal motility disorders (CC). DES is diagnosed by finding of ≥ 20% premature contractions, with normal lower esophageal sphincter (LES) relaxation on high-resolution manometry (HRM) in the latest version of CCv3.0. This feature differentiates it ... Read more >>

World J Clin Cases (World Journal of Clinical Cases)
[2020, 8(6):1026-1032]

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Successful use of fenfluramine in nonconvulsive status epilepticus of Dravet syndrome.

Nicola Specchio, Nicola Pietrafusa, Alessandro Ferretti, Marina Trivisano, Federico Vigevano,

Epilepsia (Epilepsia)
[2020, :]

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Changing Landscape of Dravet Syndrome Management: An Overview.

Debopam Samanta,

Dravet syndrome (DS), previously known as severe myoclonic epilepsy of infancy, is a severe developmental and epileptic encephalopathy caused by loss-of-function mutations in one copy of SCN1A (haploinsufficiency), located on chromosome 2q24, with decreased function of Nav1.1 sodium channels in GABAergic inhibitory interneurons. Pharmacoresistant seizures in DS start in the ... Read more >>

Neuropediatrics (Neuropediatrics)
[2020, 51(2):135-145]

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Psychosis in Parkinson's disease and parkinsonism in antipsychotic-naive schizophrenia spectrum psychosis: clinical, nosological and pathobiological challenges.

John L Waddington,

Following the formulation of operational criteria for the diagnosis of psychosis in Parkinson's disease, a neurodegenerative disorder, the past decade has seen increasing interest in such nonmotor psychopathology that appears to be independent of dopaminergic therapy. Similarly, there has been a resurgence of interest in motor aspects of the neurodevelopmental ... Read more >>

Acta Pharmacol. Sin. (Acta pharmacologica Sinica)
[2020, 41(4):464-470]

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Cingulate gyrus epilepsy: semiology, invasive EEG, and surgical approaches.

Chien-Chen Chou, Cheng-Chia Lee, Chun-Fu Lin, Yi-Hsiu Chen, Syu-Jyun Peng, Fu-Jung Hsiao, Hsiang-Yu Yu, Chien Chen, Hsin-Hung Chen, Yang-Hsin Shih,

OBJECTIVE:The semiology of cingulate gyrus epilepsy is varied and may involve the paracentral area, the adjacent limbic system, and/or the orbitofrontal gyrus. Invasive electroencephalography (iEEG) recording is usually required for patients with deeply located epileptogenic foci. This paper reports on the authors' experiences in the diagnosis and surgical treatment of ... Read more >>

Neurosurg Focus (Neurosurgical focus)
[2020, 48(4):E8]

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New insights into the early mechanisms of epileptogenesis in a zebrafish model of Dravet syndrome.

Ettore Tiraboschi, Silvia Martina, Wietske van der Ent, Kamil Grzyb, Kinga Gawel, Maria Lorena Cordero-Maldonado, Suresh Kumar Poovathingal, Sarah Heintz, Somisetty Venkata Satheesh, Jarle Brattespe, Ju Xu, Maximiliano Suster, Alexander Skupin, Camila V Esguerra,

OBJECTIVE:To pinpoint the earliest cellular defects underlying seizure onset (epileptogenic period) during perinatal brain development in a new zebrafish model of Dravet syndrome (DS) and to investigate potential disease-modifying activity of the 5HT2 receptor agonist fenfluramine. METHODS:We used CRISPR/Cas9 mutagenesis to introduce a missense mutation, designed to perturb ion transport ... Read more >>

Epilepsia (Epilepsia)
[2020, 61(3):549-560]

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