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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
#1
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/28209153/a-prospective-observational-longitudinal-study-of-new-onset-seizures-and-newly-diagnosed-epilepsy-in-dogs
#2
N Fredsø, N Toft, A Sabers, M Berendt
BACKGROUND: Seizures are common in dogs and can be caused by non-epileptic conditions or epilepsy. The clinical course of newly diagnosed epilepsy is sparsely documented. The objective of this study was to prospectively investigate causes for seizures (epileptic and non-epileptic) in a cohort of dogs with new-onset untreated seizures, and for those dogs with newly diagnosed epilepsy to investigate epilepsy type, seizure type and the course of disease over time, including the risk of seizure recurrence...
February 16, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28199002/validating-the-shortened-quality-of-life-in-childhood-epilepsy-questionnaire-qolce-55-in-a-sample-of-children-with-drug-resistant-epilepsy
#3
Lauryn Conway, Elysa Widjaja, Mary Lou Smith, Kathy N Speechley, Mark A Ferro
OBJECTIVE: The aim of this study was to validate the newly developed shortened Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55) in a sample of children with drug-resistant epilepsy. METHODS: Data came from 136 children enrolled in the Impact of Pediatric Epilepsy Surgery on Health-Related Quality of Life Study (PEPSQOL), a multicenter prospective cohort study. Confirmatory factor analysis was used to assess the higher-order factor structure of the QOLCE-55...
February 15, 2017: Epilepsia
https://www.readbyqxmd.com/read/28197949/the-impact-of-next-generation-sequencing-on-the-diagnosis-and-treatment-of-epilepsy-in-paediatric-patients
#4
REVIEW
Davide Mei, Elena Parrini, Carla Marini, Renzo Guerrini
Next-generation sequencing (NGS) has contributed to the identification of many monogenic epilepsy syndromes and is favouring earlier and more accurate diagnosis in a subset of paediatric patients with epilepsy. The cumulative information emerging from NGS studies is rapidly changing our comprehension of the relations between early-onset severe epilepsy and the associated neurological impairment, progressively delineating specific entities previously gathered under the umbrella definition of epileptic encephalopathies, thereby influencing treatment choices and limiting the most aggressive drug regimens only to those conditions that are likely to actually benefit from them...
February 14, 2017: Molecular Diagnosis & Therapy
https://www.readbyqxmd.com/read/28188045/treatment-issues-for-children-with-epilepsy-transitioning-to-adult-care
#5
REVIEW
Rima Nabbout, Carol S Camfield, Danielle M Andrade, Alexis Arzimanoglou, Catherine Chiron, Joyce A Cramer, Jacqueline A French, Eric Kossoff, Marco Mula, Peter R Camfield
This is the third of three papers that summarize the second symposium on Transition in Epilepsies held in Paris in June 2016. This paper focuses on treatment issues that arise during the course of childhood epilepsy and make the process of transition to adult care more complicated. Some AEDs used during childhood, such as stiripentol, vigabatrin, and cannabidiol, are unfamiliar to adult epilepsy specialists. In addition, new drugs are being developed for treatment of specific childhood onset epilepsy syndromes and have no indication yet for adults...
February 7, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28185967/intraoperative-ct-and-nexframe-guided-placement-of-bilateral-hippocampal-based-responsive-neurostimulation-a-technical-note
#6
Kunal Gupta, Jeffrey S Raskin, Ahmed M Raslan
INTRODUCTION: Surgical resection of a lesion that correlates with seizure onset in patients with epilepsy can dramatically improve seizure burden and quality of life. For bilateral hippocampal lesions, bilateral resection comes with a risk of severe cognitive deficits. Responsive neurostimulation (RNS) devices offers a new modality to treat multifocal lesions in a reversible manner, including bilateral hippocampal stimulation. We describe technical aspects of Nexframe-assisted placement of bilateral NeuroPace mesial temporal electrodes, and case examples...
February 6, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28176298/what-does-the-u-s-medicare-administrative-claims-database-tell-us-about-initial-antiepileptic-drug-treatment-for-older-adults-with-new-onset-epilepsy
#7
Roy C Martin, Edward Faught, Jerzy P Szaflarski, Joshua Richman, Ellen Funkhouser, Kendra Piper, Lucia Juarez, Chen Dai, Maria Pisu
OBJECTIVE: Disparities in epilepsy treatment are not uncommon; therefore, we examined population-based estimates of initial antiepileptic drugs (AEDs) in new-onset epilepsy among racial/ethnic minority groups of older US Medicare beneficiaries. METHODS: We conducted retrospective analyses of 2008-2010 Medicare administrative claims for a 5% random sample of beneficiaries augmented for minority representation. New-onset epilepsy cases in 2009 had ≥1 International Classification of Diseases, Ninth Revision (ICD-9) 345...
February 7, 2017: Epilepsia
https://www.readbyqxmd.com/read/28168208/cortical-excitability-correlates-with-seizure-control-and-epilepsy-duration-in-chronic-epilepsy
#8
Adam D Pawley, Fahmida A Chowdhury, Chayanin Tangwiriyasakul, Bryan Ceronie, Robert D C Elwes, Lina Nashef, Mark P Richardson
OBJECTIVE: Cortical excitability differs between treatment responders and nonresponders in new-onset epilepsy. Moreover, during the first 3 years of epilepsy, cortical excitability becomes more abnormal in nonresponders but normalizes in responders. Here, we study chronic active epilepsy, to examine whether cortical excitability continues to evolve over time, in association with epilepsy duration and treatment response. METHODS: We studied 28 normal subjects, 28 patients with moderately controlled epilepsy (≤4 seizures per year) and 40 patients with poorly controlled epilepsy (≥20 or more seizures per year)...
February 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28166369/heterozygous-truncation-mutations-of-the-smc1a-gene-cause-a-severe-early-onset-epilepsy-with-cluster-seizures-in-females-detailed-phenotyping-of-10-new-cases
#9
Joseph D Symonds, Shelagh Joss, Kay A Metcalfe, Suresh Somarathi, Jamie Cruden, Anita M Devlin, Alan Donaldson, Nataliya DiDonato, David Fitzpatrick, Frank J Kaiser, Anne K Lampe, Melissa M Lees, Ailsa McLellan, Tara Montgomery, Vivek Mundada, Lesley Nairn, Ajoy Sarkar, Jens Schallner, Jelena Pozojevic, Ilaria Parenti, Jeen Tan, Peter Turnpenny, William P Whitehouse, Sameer M Zuberi
OBJECTIVE: The phenotype of seizure clustering with febrile illnesses in infancy/early childhood is well recognized. To date the only genetic epilepsy consistently associated with this phenotype is PCDH19, an X-linked disorder restricted to females, and males with mosaicism. The SMC1A gene, which encodes a structural component of the cohesin complex is also located on the X chromosome. Missense variants and small in-frame deletions of SMC1A cause approximately 5% of Cornelia de Lange Syndrome (CdLS)...
February 6, 2017: Epilepsia
https://www.readbyqxmd.com/read/28166327/neurological-autoantibody-prevalence-in-epilepsy-of-unknown-etiology
#10
Divyanshu Dubey, Abdulradha Alqallaf, Ryan Hays, Matthew Freeman, Kevin Chen, Kan Ding, Mark Agostini, Steven Vernino
Importance: Autoimmune epilepsy is an underrecognized condition, and its true incidence is unknown. Identifying patients with an underlying autoimmune origin is critical because these patients' condition may remain refractory to conventional antiseizure medications but may respond to immunotherapy. Objective: To determine the prevalence of neurological autoantibodies (Abs) among adult patients with epilepsy of unknown etiology. Design, Setting, and Participants: Consecutive patients presenting to neurology services with new-onset epilepsy or established epilepsy of unknown etiology were identified...
February 6, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28157720/depression-in-epilepsy
#11
Marco Mula
PURPOSE OF REVIEW: To review some aspects of the relationship between epilepsy and depression that have recently received increasing attention and may become major research topics in the near future. RECENT FINDINGS: Epidemiological studies show that depression and suicide are, in some cases, premorbid symptoms preceding the onset of the epilepsy. Suicide is also three times more frequent in epilepsy than in the general population. Reliable screening instruments for depression and suicidality in patients with epilepsy are now available but data from real life clinical settings are needed to develop shared clinical pathways between neurology and psychiatry...
February 2, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28143391/adam23-is-a-common-risk-gene-for-canine-idiopathic-epilepsy
#12
Lotta L E Koskinen, Eija H Seppälä, Jutta Weissl, Tarja S Jokinen, Ranno Viitmaa, Reetta L Hänninen, Pascale Quignon, Andrea Fischer, Catherine André, Hannes Lohi
BACKGROUND: Idiopathic or genetic adult-onset epilepsy is a common neurological disorder in domestic dogs. Genetic association has been reported only with ADAM23 on CFA 37 in few breeds. To identify novel epilepsy genes, we performed genome-wide association (GWA) analyses in four new breeds, and investigated the association of the previously reported ADAM23 haplotype with the epilepsy phenotype in eight breeds. RESULTS: GWA analysis did not reveal new epilepsy loci...
January 31, 2017: BMC Genetics
https://www.readbyqxmd.com/read/28141740/preventing-cognitive-impairment-in-children-with-epilepsy
#13
Kees P J Braun
PURPOSE OF REVIEW: Cognitive impairments are common in children with epilepsy. They may already be present before the onset of epilepsy or occur - and even progress - during its course. Many variables contribute to cognitive dysfunction. Those that can be targeted to prevent (further) cognitive impairment will be highlighted in this review. RECENT FINDINGS: Ideally, but not yet realistically, epileptogenesis is prevented to avert seizures and cognitive impairments in high-risk patients...
January 30, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28130605/novel-polg-mutations-and-variable-clinical-phenotypes-in-13-italian-patients
#14
Paola Da Pozzo, Elena Cardaioli, Anna Rubegni, Gian Nicola Gallus, Alessandro Malandrini, Alessandra Rufa, Carla Battisti, Maria Alessandra Carluccio, Raffaele Rocchi, Fabio Giannini, Amedeo Bianchi, Michelangelo Mancuso, Gabriele Siciliano, Maria Teresa Dotti, Antonio Federico
POLG gene encodes the catalytic subunit of DNA polymerase gamma, essential for mitochondrial DNA (mtDNA) replication and repair. Mutations in POLG have been linked to a spectrum of clinical phenotypes, resulting in autosomal recessive or dominant mitochondrial diseases. These mutations have been associated with heterogeneous phenotypes, presenting with varying severity and at different ages of onset, ranging from the neonatal period to late adult life. We screened 13 patients for POLG mutations. All patients underwent a complete neurological examination, and in most of cases, muscle biopsy was performed...
January 27, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28118301/cognition-in-epilepsy-current-clinical-issues-of-interest
#15
Juri-Alexander Witt, Christoph Helmstaedter
PURPOSE OF REVIEW: This review provides an update and summary of recent neuropsychological findings in epilepsy focusing on three major clinical topics among the many developments in the field. We will critically outline the current state with regard to cognition in new-onset epilepsies, social cognition in epilepsy, and the long-term outcome of epilepsy surgery and the cognitive outcomes of superselective surgical procedures. RECENT FINDINGS: Current studies indicate that neuropsychological impairments are prevalent already at the onset of epilepsy and even before, social cognition (i...
January 21, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28109992/acute-and-spontaneous-seizure-onset-zones-in-the-intraperitoneal-kainic-acid-model
#16
Phillip Connell, Arezou Bayat, Sweta Joshi, Mohamad Z Koubeissi
OBJECTIVE: Hippocampal monitoring is often used in the intraperitoneal kainic acid (KA) seizure model for detection and quantification of early ictal activity. Here, we investigated extra-hippocampal seizure onset zones (SOZs) in this model. METHODS: Eight male Sprague Dawley rats implanted with depth electrodes were continuously recorded during intraperitoneal KA injections until status epilepticus (SE) was induced. Another group of four rats was monitored chronically up to two weeks after emergence of spontaneous recurrent seizures...
January 18, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28109983/the-clinical-and-neurobehavioral-course-of-down-syndrome-and-dementia-with-or-without-new-onset-epilepsy
#17
Taha Gholipour, Sara Mitchell, Rani A Sarkis, Zeina Chemali
BACKGROUND: Adult patients with Down syndrome (DS) are at higher risk of developing Alzheimer-type dementia and epilepsy. The relationship between developing dementia and the risk of developing seizures in DS is poorly characterized to date. In addition, treatment response and medication tolerability have not been rigorously studied. METHODS: We identified 220 patients with a diagnosis of DS and dementia. Those without a history of developing seizures (DD) were compared to patients with new-onset seizures (DD+S) after the age of 35...
January 18, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28098938/towards-prognostic-biomarkers-from-bold-fluctuations-to-differentiate-a-first-epileptic-seizure-from-new-onset-epilepsy
#18
Lalit Gupta, Rick Janssens, Mariëlle C G Vlooswijk, Rob P W Rouhl, Anton de Louw, Albert P Aldenkamp, Shrutin Ulman, René M H Besseling, Paul A M Hofman, Vivianne H van Kranen-Mastenbroek, Danny M Hilkman, Jacobus F A Jansen, Walter H Backes
OBJECTIVE: The diagnosis of epilepsy cannot be reliably made prior to a patient's second seizure in most cases. Therefore, adequate diagnostic tools are needed to differentiate subjects with a first seizure from those with a seizure preceding the onset of epilepsy. The objective was to explore spontaneous blood oxygen level-dependent (BOLD) fluctuations in subjects with a first-ever seizure and patients with new-onset epilepsy (NOE), and to find characteristic biomarkers for seizure recurrence after the first seizure...
January 18, 2017: Epilepsia
https://www.readbyqxmd.com/read/28073790/clemizole-and-modulators-of-serotonin-signalling-suppress-seizures-in-dravet-syndrome
#19
Aliesha Griffin, Kyla R Hamling, Kelly Knupp, SoonGweon Hong, Luke P Lee, Scott C Baraban
Dravet syndrome is a catastrophic childhood epilepsy with early-onset seizures, delayed language and motor development, sleep disturbances, anxiety-like behaviour, severe cognitive deficit and an increased risk of fatality. It is primarily caused by de novo mutations of the SCN1A gene encoding a neuronal voltage-activated sodium channel. Zebrafish with a mutation in the SCN1A homologue recapitulate spontaneous seizure activity and mimic the convulsive behavioural movements observed in Dravet syndrome. Here, we show that phenotypic screening of drug libraries in zebrafish scn1 mutants rapidly and successfully identifies new therapeutics...
January 10, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28057450/antiepileptic-drugs-prevent-seizures-in-hyperbaric-oxygen-a-novel-model-of-epileptiform-activity
#20
Ivan T Demchenko, Sergei Yu Zhilyaev, Alexander N Moskvin, Alexander I Krivchenko, Claude A Piantadosi, Barry W Allen
Breathing oxygen at sufficiently elevated pressures can trigger epileptiform seizures. Therefore, we tested the hypothesis that pre-treatment with FDA-approved antiepileptic drugs could prevent seizure onset in hyperoxia at 5 atmospheres absolute. We selected drugs from two putative functional categories, Na(+)-channel antagonists and GABA enhancers, each administered intraperitoneally at four doses in separate groups of C57BL/6 mice. The drugs varied in efficacy at the doses used. Of the five tested Na(+)-channel antagonists, carbamazepine and lamotrigine more than tripled seizure latency compared to values seen in vehicle controls...
January 2, 2017: Brain Research
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