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Epilepsia

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https://www.readbyqxmd.com/read/28213908/a-randomized-controlled-trial-of-lacosamide-versus-sodium-valproate-in-status-epilepticus
#1
Usha K Misra, Deepanshu Dubey, Jayantee Kalita
OBJECTIVE: To compare the efficacy and safety of lacosamide (LCM) and sodium valproate (SVA) in lorazepam (LOR)-resistant status epilepticus (SE). METHODS: Patients with LOR-resistant SE were randomized to intravenous LCM 400 mg at a rate of 60 mg/kg/min or SVA 30 mg/kg at a rate of 100 mg/min. The SE severity score (STESS), duration of SE and its etiology, and magnetic resonance imaging (MRI) findings were noted. Primary outcome was seizure cessation for 1 h, and secondary outcomes were 24 h seizure remission, in hospital death and severe adverse events (SAEs)...
February 18, 2017: Epilepsia
https://www.readbyqxmd.com/read/28206669/characterization-of-focal-cortical-dysplasia-with-balloon-cells-by-layer-specific-markers-evidence-for-differential-vulnerability-of-interneurons
#2
Julia M Nakagawa, Catharina Donkels, Susanne Fauser, Andreas Schulze- Bonhage, Marco Prinz, Josef Zentner, Carola A Haas
OBJECTIVE: Focal cortical dysplasia (FCD) is a major cause of pharmacoresistant focal epilepsy. Little is known about the pathomechanisms underlying the characteristic cytoarchitectural abnormalities associated with FCD. In the present study, a broad panel of markers identifying layer-specific neuron subpopulations was applied to characterize dyslamination and structural alterations in FCD with balloon cells (FCD 2b). METHODS: Pan-neuronal neuronal nuclei (NeuN) and layer-specific protein expression (Reelin, Calbindin, Calretinin, SMI32 (nonphosphorylated neurofilament H), Parvalbumin, transducin-like enhancer protein 4 (TLE4), and Vimentin) was studied by immunohistochemistry on paraffin sections of FCD2b cases (n = 22) and was compared to two control groups with (n = 7) or without epilepsy (n = 4 postmortem cases)...
February 16, 2017: Epilepsia
https://www.readbyqxmd.com/read/28199007/accuracy-of-claims-based-algorithms-for-epilepsy-research-revealing-the-unseen-performance-of-claims-based-studies
#3
Lidia M V R Moura, Maggie Price, Andrew J Cole, Daniel B Hoch, John Hsu
OBJECTIVE: To evaluate published algorithms for the identification of epilepsy cases in medical claims data using a unique linked dataset with both clinical and claims data. METHODS: Using data from a large, regional health delivery system, we identified all patients contributing biologic samples to the health system's Biobank (n = 36K). We identified all subjects with at least one diagnosis potentially consistent with epilepsy, for example, epilepsy, convulsions, syncope, or collapse, between 2014 and 2015, or who were seen at the epilepsy clinic (n = 1,217), plus a random sample of subjects with neither claims nor clinic visits (n = 435); we then performed a medical chart review in a random subsample of 1,377 to assess the epilepsy diagnosis status...
February 15, 2017: Epilepsia
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
#4
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/28199000/overexpression-of-pregnane-x-and-glucocorticoid-receptors-and-the-regulation-of-cytochrome-p450-in-human-epileptic-brain-endothelial-cells
#5
Chaitali Ghosh, Mohammed Hossain, Jesal Solanki, Imad M Najm, Nicola Marchi, Damir Janigro
OBJECTIVE: Recent evidence suggests a metabolic contribution of cytochrome P450 enzymes (CYPs) to the drug-resistant phenotype in human epilepsy. However, the upstream molecular regulators of CYP in the epileptic brain remain understudied. We therefore investigated the expression and function of pregnane xenobiotic (PXR) and glucocorticoid (GR) nuclear receptors in endothelial cells established from post-epilepsy surgery brain samples. METHODS: PXR/GR localization was evaluated by immunohistochemistry in specimens from subjects who underwent temporal lobe resections to relieve drug-resistant seizures...
February 15, 2017: Epilepsia
https://www.readbyqxmd.com/read/28195311/reduced-local-input-to-fast-spiking-interneurons-in-the-somatosensory-cortex-in-the-gabaa-%C3%AE-2-r43q-mouse-model-of-absence-epilepsy
#6
Stephen P Currie, Liliana L Luz, Sam A Booker, David J A Wyllie, Peter C Kind, Michael I Daw
OBJECTIVE: Absence seizures in childhood absence epilepsy are initiated in the thalamocortical (TC) system. We investigated if these seizures result from altered development of the TC system before the appearance of seizures in mice containing a point mutation in γ-aminobutyric acid A (GABAA ) receptor γ2 subunits linked to childhood absence epilepsy (R43Q). Findings from conditional mutant mice indicate that expression of normal γ2 subunits during preseizure ages protect from later seizures...
February 13, 2017: Epilepsia
https://www.readbyqxmd.com/read/28195308/ca-2-permeable-ampa-receptors-associated-with-epileptogenesis-of-hypothalamic-hamartoma
#7
Hiroki Kitaura, Masaki Sonoda, Sayaka Teramoto, Hiroshi Shirozu, Hiroshi Shimizu, Tadashi Kimura, Hiroshi Masuda, Yosuke Ito, Hitoshi Takahashi, Shin Kwak, Shigeki Kameyama, Akiyoshi Kakita
Hypothalamic hamartoma (HH), composed of neurons and glia without apparent cytologic abnormalities, is a rare developmental malformation in humans. Patients with HH often have characteristic medically refractory gelastic seizures, and intrinsic epileptogenesis within the lesions has been speculated. Herein we provide evidence to suggest that in HH neurons, Ca(2+) permeability through α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptors is aberrantly elevated. In needle biopsy specimens of HH tissue, field potential recordings demonstrated spontaneous epileptiform activities similar to those observed in other etiologically distinct epileptogenic tissues...
February 13, 2017: Epilepsia
https://www.readbyqxmd.com/read/28186331/adult-motor-phenotype-differentiates-dravet-syndrome-from-lennox-gastaut-syndrome-and-links-scn1a-to-early-onset-parkinsonian-features
#8
Danah Aljaafari, Alfonso Fasano, Fábio A Nascimento, Anthony E Lang, Danielle M Andrade
Distinguishing adult patients with Lennox-Gastaut syndrome from those with Dravet syndrome is challenging. We have previously reported that patients with Dravet syndrome present a very peculiar motor phenotype. Here we sought to confirm that this association was not linked to the chronic use of antiepileptic drugs or the many lifetime seizures. To this aim, we studied 14 adult patients with Lennox-Gastaut syndrome and 14 adults with Dravet syndrome because both conditions share similar seizure severity. We found that antecollis and parkinsonian gait were significantly more common in the Dravet group, thus suggesting that these features are part of the Dravet syndrome adult phenotype...
February 10, 2017: Epilepsia
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
#9
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/28166392/interictal-network-synchrony-and-local-heterogeneity-predict-epilepsy-surgery-outcome-among-pediatric-patients
#10
Samuel B Tomlinson, Brenda E Porter, Eric D Marsh
OBJECTIVE: Epilepsy is a disorder of aberrant cortical networks. Researchers have proposed that characterizing presurgical network connectivity may improve the surgical management of intractable seizures, but few studies have rigorously examined the relationship between network activity and surgical outcome. In this study, we assessed whether local and global measures of network activity differentiated patients with favorable (seizure-free) versus unfavorable (seizure-persistent) surgical outcomes...
February 6, 2017: Epilepsia
https://www.readbyqxmd.com/read/28166389/an-evaluation-of-national-birth-certificate-data-for-neonatal-seizure-epidemiology
#11
Kristen Berry, Michael F Pesko, Dale C Hesdorffer, Renée A Shellhaas, Joanna K Seirup, Zachary M Grinspan
OBJECTIVE: Seizures are a common manifestation of neurologic dysfunction in neonates and carry a high risk for mortality and adverse long-term outcomes. U.S. birth certificates are a potentially valuable source for studying the epidemiology of neonatal seizures. However, the quality of the data is understudied. METHODS: We reviewed all U.S. birth records from 2003 to 2013 to describe the following: (1) rates of missing data, (2) evidence of underreporting, and (3) effect of the 2003 revision of the birth certificate form...
February 6, 2017: Epilepsia
https://www.readbyqxmd.com/read/28166388/toll-like-receptor-3-deficiency-decreases-epileptogenesis-in-a-pilocarpine-model-of-se-induced-epilepsy-in-mice
#12
Adi Gross, Felix Benninger, Ravit Madar, Tomer Illouz, Kathleen Griffioen, Israel Steiner, Daniel Offen, Eitan Okun
OBJECTIVE: Epilepsy affects 60 million people worldwide. Despite the development of antiepileptic drugs, up to 35% of patients are drug refractory with uncontrollable seizures. Toll-like receptors (TLRs) are central components of the nonspecific innate inflammatory response. Because TLR3 was recently implicated in neuronal plasticity, we hypothesized that it may contribute to the development of epilepsy after status epilepticus (SE). METHODS: To test the involvement of TLR3 in epileptogenesis, we used the pilocarpine model for SE in TLR3-deficient mice and their respective wild-type controls...
February 6, 2017: Epilepsia
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
#13
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/28166368/efficacy-of-mglu2-positive-allosteric-modulators-alone-and-in-combination-with-levetiracetam-in-the-mouse-6-hz-model-of-psychomotor-seizures
#14
Cameron S Metcalf, Brian D Klein, Misty D Smith, Tim Pruess, Marc Ceusters, Hilde Lavreysen, Stefan Pype, Nancy Van Osselaer, Roy Twyman, H Steve White
OBJECTIVE: The metabotropic glutamate receptor subtype 2 (mGlu2 ) possesses both orthosteric and allosteric modulatory sites, are expressed in the frontal cortex and limbic structures, and can affect excitatory synaptic transmission. Therefore, mGlu2 is a potential therapeutic target in the treatment of epilepsy. The present study seeks to evaluate the anticonvulsant potential of mGlu2 -acting compounds. METHODS: The anticonvulsant efficacy of two selective mGlu2 -positive allosteric modulators (PAMs) (JNJ-42153605 and JNJ-40411813/ADX71149) and one mGlu2/3 receptor agonist (LY404039) were evaluated alone and in combination with the antiseizure drug levetiracetam (LEV) in the mouse 6 Hz model...
February 6, 2017: Epilepsia
https://www.readbyqxmd.com/read/28166365/perampanel-in-12-patients-with-unverricht-lundborg-disease
#15
Arielle Crespel, Philippe Gelisse, Ngoc Phuong Loc Tang, Pierre Genton
OBJECTIVE: Perampanel (PER) was used in 12 patients with Unverricht-Lundborg disease (ULD) to evaluate its efficacy against myoclonus and seizures. METHODS: We treated 11 patients with EPM1 mutations (6 F, 5 M, aged 13-62 years) and a 43-year-old man with de novo KCNC1 mutation. PER was introduced by 2 mg steps at 2-4 week intervals until 6 mg/day, with a possible dose reduction or dose increase. RESULTS: Ten patients had a clear clinical response of myoclonus, and five were able to reduce concomitant therapy...
February 6, 2017: Epilepsia
https://www.readbyqxmd.com/read/28166364/interrater-agreement-in-the-interpretation-of-neonatal-electroencephalography-in-hypoxic-ischemic-encephalopathy
#16
Courtney J Wusthoff, Joseph Sullivan, Hannah C Glass, Renée A Shellhaas, Nicholas S Abend, Taeun Chang, Tammy N Tsuchida
OBJECTIVE: Research using neonatal electroencephalography (EEG) has been limited by a lack of a standardized classification system and interpretation terminology. In 2013, the American Clinical Neurophysiology Society (ACNS) published a guideline for standardized terminology and categorization in the description of continuous EEG in neonates. We sought to assess interrater agreement for this neonatal EEG categorization system as applied by a group of pediatric neurophysiologists. METHODS: A total of 60 neonatal EEG studies were collected from three institutions...
February 6, 2017: Epilepsia
https://www.readbyqxmd.com/read/28150296/radiofrequency-thermocoagulation-of-the-seizure-onset-zone-during-stereoelectroencephalography
#17
Petia Dimova, Luca de Palma, Anne-Sophie Job-Chapron, Lorella Minotti, Dominique Hoffmann, Philippe Kahane
OBJECTIVE: To assess long-term outcome and identify prognostic factors of radiofrequency thermocoagulation (RFTC) following stereoelectroencephalography (SEEG) explorations in particularly complex cases of focal epilepsy. METHODS: We retrospectively reviewed the medical charts, video-SEEG recordings, and outcomes for 23 patients (aged 6-53 years) treated with SEEG-guided RFTC, of whom 15 had negative magnetic resonance imaging (MRI) findings, and 10 were considered noneligible for resective surgery after SEEG...
February 2, 2017: Epilepsia
https://www.readbyqxmd.com/read/28139826/neonatal-nonepileptic-myoclonus-is-a-prominent-clinical-feature-of-kcnq2-gain-of-function-variants-r201c-and-r201h
#18
Sarah B Mulkey, Bruria Ben-Zeev, Joost Nicolai, John L Carroll, Sabine Grønborg, Yong-Hui Jiang, Nishtha Joshi, Megan Kelly, David A Koolen, Mohamad A Mikati, Kristen Park, Phillip L Pearl, Ingrid E Scheffer, Rebecca C Spillmann, Maurizio Taglialatela, Silvia Vieker, Sarah Weckhuysen, Edward C Cooper, Maria Roberta Cilio
OBJECTIVE: To analyze whether KCNQ2 R201C and R201H variants, which show atypical gain-of-function electrophysiologic properties in vitro, have a distinct clinical presentation and outcome. METHODS: Ten children with heterozygous, de novo KCNQ2 R201C or R201H variants were identified worldwide, using an institutional review board (IRB)-approved KCNQ2 patient registry and database. We reviewed medical records and, where possible, interviewed parents and treating physicians using a structured, detailed phenotype inventory focusing on the neonatal presentation and subsequent course...
January 31, 2017: Epilepsia
https://www.readbyqxmd.com/read/28130784/time-to-electroencephalography-is-independently-associated-with-outcome-in-critically-ill-neonates-and-children
#19
Iván Sánchez Fernández, Arnold J Sansevere, Rejean M Guerriero, Ersida Buraniqi, Phillip L Pearl, Robert C Tasker, Tobias Loddenkemper
OBJECTIVE: To identify factors associated with in-hospital mortality in neonates and children undergoing continuous electroencephalography (cEEG) monitoring in the intensive care unit (ICU). METHODS: We performed a retrospective observational study in patients from birth to 21 years of age who underwent clinically indicated cEEG in the ICU from 2011 to 2013. The main outcome measure was in-hospital mortality. RESULTS: Six-hundred and twenty-five patients (54...
January 28, 2017: Epilepsia
https://www.readbyqxmd.com/read/28117493/shedding-light-on-interictal-epileptic-spikes-an-in-vivo-study-using-fast-optical-signal-and-electrocorticography
#20
Mana Manoochehri, Mahdi Mahmoudzadeh, Victoria Osharina, Fabrice Wallois
OBJECTIVE: Interictal epileptic spikes (IESs), apart from being a key marker of epileptic neuronal networks, constitute a nice model of the widespread endogenous phenomenon of neuronal hypersynchronization. Many questions concerning the mechanisms that drive neurons to hypersynchronize remain unresolved, but synaptic as well as nonsynaptic events are likely to be involved. In this study, changes in optical properties of neural tissues were observed in rats with penicillin-induced IES using fast optical signal (FOS) concomitantly with electrocorticography (ECoG)...
January 24, 2017: Epilepsia
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