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https://www.readbyqxmd.com/read/28719803/elevated-sterol-regulatory-elementary-binding-protein-1-and-glua2-levels-in-the-hippocampal-nuclear-fraction-of-genetic-absence-epilepsy-rats-from-strasbourg
#1
Sathiya Sekar, Entesar Omran, Venkat Gopalakrishnan, John G Howland, Terrance P Snutch, Changiz Taghibiglou
Studies in animal models and human tissues show that nuclear translocation of sterol regulatory element binding protein 1 (SREBP1) and glutamate A2 subunit (GluA2) of cell-surface AMPA receptor (AMPAR) trigger neuronal excitotoxicity-induced apoptosis in stroke. However, it is not known whether a similar type of underlying pathophysiology occurs in absence epilepsy. To explore this issue, we examined the levels of mature SREBP1, GluA2, glyceraldehyde 3-phosphate dehydrogenase (GAPDH), p53, and activated to total caspase 3 ratio in nuclear fractions (NF) of hippocampal homogenate from 8 to 10 week old male Genetic Absence Epilepsy Rats from Strasbourg (GAERS) and non-epileptic control (NEC) strains...
July 12, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28709121/spatiotemporal-propagation-patterns-of-generalized-ictal-spikes-in-childhood-absence-epilepsy
#2
Vasileios Kokkinos, Andreas M Koupparis, Michalis Koutroumanidis, George K Kostopoulos
OBJECTIVE: This work investigates the spatial distribution in time of generalized ictal spikes in the typical absences of childhood absence epilepsy (CAE). METHODS: We studied twelve children with CAE, who had more than two typical absences during their routine video-EEG. Seizures were identified, and ictal spikes were marked over the maximum electronegative peak, clustered, waveform-averaged and spatiotemporaly analyzed in 2D electrode space. RESULTS: Consistency of spatiotemporal patterns of ictal spikes was high between the absences of the same child, but low between children...
June 17, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28708842/evaluating-whole-genome-sequence-data-from-the-genetic-absence-epilepsy-rat-from-strasbourg-and-its-related-non-epileptic-strain
#3
Pablo M Casillas-Espinosa, Kim L Powell, Mingfu Zhu, C Ryan Campbell, Jessica M Maia, Zhong Ren, Nigel C Jones, Terence J O'Brien, Slavé Petrovski
OBJECTIVE: The Genetic Absence Epilepsy Rats from Strasbourg (GAERS) are an inbreed Wistar rat strain widely used as a model of genetic generalised epilepsy with absence seizures. As in humans, the genetic architecture that results in genetic generalized epilepsy in GAERS is poorly understood. Here we present the strain-specific variants found among the epileptic GAERS and their related Non-Epileptic Control (NEC) strain. The GAERS and NEC represent a powerful opportunity to identify neurobiological factors that are associated with the genetic generalised epilepsy phenotype...
2017: PloS One
https://www.readbyqxmd.com/read/28697377/absence-of-regulator-of-g-protein-signaling-4-does-not-protect-against-dopamine-neuron-dysfunction-and-injury-in-the-mouse-6-hydroxydopamine-lesion-model-of-parkinson-s-disease
#4
Amer Ashrafi, Pierre Garcia, Heike Kollmus, Klaus Schughart, Antonio Del Sol, Manuel Buttini, Enrico Glaab
Regulator of G-protein signaling 4 (RGS4), a member of the RGS family of proteins that inactivate G-proteins, has gained interest as a potential drug target for neurological disorders, such as epilepsy and Parkinson's disease (PD). In the case of PD, the main current options for alleviating motor symptoms are dopamine replacement therapies, which have limitations because of side effects and reduced effectiveness over the long term. Research on new nondopaminergic PD drug targets has indicated that inhibition of RGS4 could be an effective adjuvant treatment option...
June 19, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28689466/early-diagnosis-and-treatment-of-lennox-gastaut-syndrome
#5
Trevor Resnick, Raj D Sheth
Lennox-Gastaut syndrome (LGS) is a severe form of childhood-onset epilepsy associated with high morbidity and mortality. The peak period for manifestations of Lennox-Gastaut syndrome is between ages 3 and 5 years, a time of critical brain development and corresponding vulnerability to the electroclinical dysfunction arising from Lennox-Gastaut syndrome. Diagnosis is based on a triad of symptoms: multiple seizure types, cognitive impairment, and slow spike-and-wave pattern on electroencephalography. In practice, Lennox-Gastaut syndrome presentation is diverse, and there may be a delay between initial symptoms and emergence of the full triad of clinical features...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28688851/characterization-of-the-dominant-inheritance-mechanism-of-episodic-ataxia-type-2
#6
Kevin Dorgans, Julie Salvi, Federica Bertaso, Ludivine Bernard, Philippe Lory, Frederic Doussau, Alexandre Mezghrani
Episodic Ataxia type 2 (EA2) is an autosomal dominant neuronal disorder linked to mutations in the Cav2.1 subunit of P/Q-type calcium channels. In vitro studies have established that EA2 mutations induce loss of channel activity and that EA2 mutants can exert a dominant negative effect, suppressing normal Cav2.1 activity through protein misfolding and trafficking defects. To date, the role of this mechanism in the disease pathogenesis is unknown because no animal model exists. To address this issue, we have generated a mouse bearing the R1497X nonsense mutation in Cav2...
July 5, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28687722/a-meta-analysis-on-progressive-atrophy-in-intractable-temporal-lobe-epilepsy-time-is-brain
#7
REVIEW
Lorenzo Caciagli, Andrea Bernasconi, Samuel Wiebe, Matthias J Koepp, Neda Bernasconi, Boris C Bernhardt
OBJECTIVE: It remains unclear whether drug-resistant temporal lobe epilepsy (TLE) is associated with cumulative brain damage, with no expert consensus and no quantitative syntheses of the available evidence. METHODS: We conducted a systematic review and meta-analysis of MRI studies on progressive atrophy, searching PubMed and Ovid MEDLINE databases for cross-sectional and longitudinal quantitative MRI studies on drug-resistant TLE. RESULTS: We screened 2,976 records and assessed eligibility of 248 full-text articles...
July 7, 2017: Neurology
https://www.readbyqxmd.com/read/28686997/management-of-epilepsy-in-merrf-syndrome
#8
REVIEW
Josef Finsterer, Sinda Zarrouk-Mahjoub
Myoclonic epilepsy with ragged-red fibers (MERRF) syndrome is a rare syndromic mitochondrial disorder (MID) with a broad phenotypic but narrow genotypic heterogeneity. One of the predominant phenotypic features in addition to myopathy is epilepsy. The most frequent seizure type in MERRF is generalised myoclonic seizure but also focal myoclonic, focal atonic, generalised tonic-clonic, generalised atonic, generalised myoclonic-atonic, typical absences, or tonic-clonic seizures of unknown onset have been reported...
June 24, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28673977/lgi1-tunes-intrinsic-excitability-by-regulating-the-density-of-axonal-kv1-channels
#9
Michael Seagar, Michael Russier, Olivier Caillard, Yves Maulet, Laure Fronzaroli-Molinieres, Marina De San Feliciano, Norah Boumedine-Guignon, Léa Rodriguez, Mickael Zbili, Fabrice Usseglio, Christine Formisano-Tréziny, Fahamoe Youssouf, Marion Sangiardi, Morgane Boillot, Stéphanie Baulac, María José Benitez, Juan-José Garrido, Dominique Debanne, Oussama El Far
Autosomal dominant epilepsy with auditory features results from mutations in leucine-rich glioma-inactivated 1 (LGI1), a soluble glycoprotein secreted by neurons. Animal models of LGI1 depletion display spontaneous seizures, however, the function of LGI1 and the mechanisms by which deficiency leads to epilepsy are unknown. We investigated the effects of pure recombinant LGI1 and genetic depletion on intrinsic excitability, in the absence of synaptic input, in hippocampal CA3 neurons, a classical focus for epileptogenesis...
July 3, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28673029/predictive-factors-of-surgical-outcome-in-frontal-lobe-epilepsy-explored-with-stereoelectroencephalography
#10
Francesca Bonini, Aileen McGonigal, Didier Scavarda, Romain Carron, Jean Régis, Henry Dufour, Jean-Claude Péragut, Virginie Laguitton, Nathalie Villeneuve, Patrick Chauvel, Bernard Giusiano, Agnès Trébuchon, Fabrice Bartolomei
BACKGROUND: Resective surgery established treatment for pharmacoresistant frontal lobe epilepsy (FLE), but seizure outcome and prognostic indicators are poorly characterized and vary between studies. OBJECTIVE: To study long-term seizure outcome and identify prognostic factors. METHODS: We retrospectively analyzed 42 FLE patients having undergone surgical resection, mostly preceded by invasive recordings with stereoelectroencephalography (SEEG)...
June 29, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28671982/clinical-services-for-adults-with-an-intellectual-disability-and-epilepsy-a-comparison-of-management-alternatives
#11
Adam P Wagner, Tim J Croudace, Naomi Bateman, Mark W Pennington, Elizabeth Prince, Marcus Redley, Simon R White, Howard Ring
BACKGROUND: Intellectual disability (ID) is relatively common in people with epilepsy, with prevalence estimated to be around 25%. Surprisingly, given this relatively high frequency, along with higher rates of refractory epilepsy than in those without ID, little is known about outcomes of different management approaches/clinical services treating epilepsy in adults with ID-we investigate this area. MATERIALS & METHODS: We undertook a naturalistic observational cohort study measuring outcomes in n = 91 adults with ID over a 7-month period (recruited within the period March 2008 to April 2010)...
2017: PloS One
https://www.readbyqxmd.com/read/28669649/-dress-syndrome-and-agranulocytosis-a-rare-combination
#12
P Lavenant, J-M Roue, F Huet, C Abasq, L Misery, S Rioualen
INTRODUCTION: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe toxidermia that can lead to death from multivisceral failure. We report a case of DRESS associated with febrile agranulocytosis in a child. OBSERVATION: An 8-year-old child was hospitalized for diffuse maculopapular exanthema with edema of the extremities and face associated with cheilitis and febrile agranulocytosis. This symptomatology began 1month after the introduction of carbamazepine for partial epilepsy...
June 29, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28664455/neuroprotective-effects-of-the-absence-of-jnk1-or-jnk3-isoforms-on-kainic-acid-induced-temporal-lobe-epilepsy-like-symptoms
#13
Luisa de Lemos, Felix Junyent, Antoni Camins, Rubén Darío Castro-Torres, Jaume Folch, Jordi Olloquequi, Carlos Beas-Zarate, Ester Verdaguer, Carme Auladell
The activation of c-Jun-N-terminal kinases (JNK) pathway has been largely associated with the pathogenesis and the neuronal death that occur in neurodegenerative diseases. Altogether, this justifies why JNKs have become a focus of screens for new therapeutic strategies. The aim of the present study was to identify the role of the different JNK isoforms (JNK1, JNK2, and JNK3) in apoptosis and inflammation after induction of brain damage. To address this aim, we induced excitotoxicity in wild-type and JNK knockout mice (jnk1 (-/-) , jnk2 (-/-) , and jnk3 (-/-) ) via an intraperitoneal injection of kainic acid, an agonist of glutamic-kainate-receptors, that induce status epilepticus...
June 29, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28661008/antiepileptic-drug-monotherapy-for-epilepsy-a-network-meta-analysis-of-individual-participant-data
#14
REVIEW
Sarah J Nevitt, Maria Sudell, Jennifer Weston, Catrin Tudur Smith, Anthony G Marson
BACKGROUND: Epilepsy is a common neurological condition with a worldwide prevalence of around 1%. Approximately 60% to 70% of people with epilepsy will achieve a longer-term remission from seizures, and most achieve that remission shortly after starting antiepileptic drug treatment. Most people with epilepsy are treated with a single antiepileptic drug (monotherapy) and current guidelines from the National Institute for Health and Care Excellence (NICE) in the United Kingdom for adults and children recommend carbamazepine or lamotrigine as first-line treatment for partial onset seizures and sodium valproate for generalised onset seizures; however a range of other antiepileptic drug (AED) treatments are available, and evidence is needed regarding their comparative effectiveness in order to inform treatment choices...
June 29, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28658605/sociability-impairments-in-genetic-absence-epilepsy-rats-from-strasbourg-reversal-by-the-t-type-calcium-channel-antagonist-z944
#15
Mark T Henbid, Wendie N Marks, Madeline J Collins, Stuart M Cain, Terrance P Snutch, John G Howland
Childhood absence epilepsy (CAE) is associated with interictal co-morbid symptoms including abnormalities in social behaviour. Genetic Absence Epilepsy Rats from Strasbourg (GAERS) is a model of CAE that exhibits physiological and behavioural alterations characteristic of the human disorder. However, it is unknown if GAERS display the social deficits often observed in CAE. Sociability in rodents is thought to be mediated by neural circuits densely populated with T-type calcium channels and GAERS contain a missense mutation in the Cav3...
June 26, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28653281/fingolimod-exerts-only-temporary-antiepileptogenic-effects-but-longer-lasting-positive-effects-on-behavior-in-the-wag-rij-rat-absence-epilepsy-model
#16
Antonio Leo, Rita Citraro, Nicola Amodio, Caterina De Sarro, Maria Eugenia Gallo Cantafio, Andrew Constanti, Giovambattista De Sarro, Emilio Russo
One of the major challenges in the epilepsy field is identifying disease-modifying drugs in order to prevent or delay spontaneous recurrent seizure onset or to cure already established epilepsy. It has been recently reported that fingolimod, currently approved for the treatment of relapsing-remitting multiple sclerosis, has demonstrated antiepileptogenic effects in 2 different preclinical models of acquired epilepsy. However, to date, no data exist regarding the role of fingolimod against genetic epilepsy. Therefore, we have addressed this issue by studying the effects of fingolimod in Wistar Albino Glaxo/Rijswijk (WAG/Rij) rats, a well-established genetic model of absence epilepsy, epileptogenesis, and neuropsychiatric comorbidity...
June 26, 2017: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/28644200/electrical-stimulations-of-the-human-insula-their-contribution-to-the-ictal-semiology-of-insular-seizures
#17
Laure Mazzola, François Mauguière, Jean Isnard
INTRODUCTION: Stereotactic stimulations of the insular cortex through intracranial electrodes aim at characterizing the semiology of insular seizures. These stimulations, carried out in the context of Stereo-Electro-Encephalography (SEEG) during presurgical monitoring of epilepsy, reproduce the ictal symptoms observed during the development of insular seizures. METHODS: The authors reviewed the results of insular stimulations performed in 222 patients admitted between 1997 and 2015 for presurgical SEEG exploration of atypical temporal or perisylvian epilepsy...
July 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28633970/unified-neural-field-theory-of-brain-dynamics-underlying-oscillations-in-parkinson-s-disease-and-generalized-epilepsies
#18
E J Müller, S J van Albada, J W Kim, P A Robinson
The mechanisms underlying pathologically synchronized neural oscillations in Parkinson's disease (PD) and generalized epilepsies are explored in parallel via a physiologically-based neural field model of the corticothalamic-basal ganglia (CTBG) system. The basal ganglia (BG) are approximated as a single effective population and their roles in the modulation of oscillatory dynamics of the corticothalamic (CT) system and vice versa are analyzed. In addition to normal EEG rhythms, enhanced activity around 4 Hz and 20 Hz exists in the model, consistent with the characteristic frequencies observed in PD...
June 17, 2017: Journal of Theoretical Biology
https://www.readbyqxmd.com/read/28624414/corneal-kindled-c57bl-6-mice-exhibit-saturated-dentate-gyrus-long-term-potentiation-and-associated-memory-deficits-in-the-absence-of-overt-neuron-loss
#19
Gregory J Remigio, Jaycie L Loewen, Sage Heuston, Colin Helgeson, H Steve White, Karen S Wilcox, Peter J West
Memory deficits have a significant impact on the quality of life of patients with epilepsy and currently no effective treatments exist to mitigate this comorbidity. While these cognitive comorbidities can be associated with varying degrees of hippocampal cell death and hippocampal sclerosis, more subtle changes in hippocampal physiology independent of cell loss may underlie memory dysfunction in many epilepsy patients. Accordingly, animal models of epilepsy or epileptic processes exhibiting memory deficits in the absence of cell loss could facilitate novel therapy discovery...
June 15, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28623753/precuneal-epilepsy-clinical-features-and-surgical-outcome
#20
Adil Harroud, Olivier Boucher, Thi Phuoc Yen Tran, Louis Harris, Jeffery Hall, François Dubeau, Ismail Mohamed, Alain Bouthillier, Dang Khoa Nguyen
OBJECTIVE: The precuneus is a complex and highly connected structure located in the medial portion of the superior parietal lobule. The clinical presentation of precuneal epilepsy is poorly characterized, mostly because these patients have seldom been distinguished from those with other types of parietal lobe epilepsy. The present study aims to improve the understanding of precuneal epilepsy by detailing its clinical features and surgical outcomes. METHODS: Six previously unreported cases of drug-resistant precuneal epilepsy investigated between 2002 and 2014 were retrospectively studied...
June 14, 2017: Epilepsy & Behavior: E&B
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