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https://www.readbyqxmd.com/read/28549335/microstructural-alterations-of-white-matter-in-juvenile-myoclonic-epilepsy
#1
Jinnan Gong, Xuebin Chang, Sisi Jiang, Benjamin Klugah-Brown, Song Tan, Dezhong Yao, Cheng Luo
Juvenile myoclonic epilepsy (JME) is a common type of idiopathic generalized epilepsy that is characterized by myoclonic jerks of the upper limbs and generalized tonic-clonic seizures. Frontal cognitive dysfunctions and abnormal coupling of the thalamocortical system have been found in neuropsychological and neuroimaging studies. This study intended to explore white matter (WM) measurement changes in JME using MRI. Twenty-six patients with JME and 25 healthy controls (HC) were recruited for the acquisition of diffusion MRI and structural MRI data...
April 4, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28549276/different-seizure-onset-patterns-in-mesiotemporal-lobe-epilepsy-have-a-distinct-interictal-signature
#2
Birgit Frauscher, Nicolás von Ellenrieder, François Dubeau, Jean Gotman
OBJECTIVE: Experimental research demonstrated that distinct underlying mechanisms go along with different seizure-onset patterns on EEG. These different mechanisms may reflect different tissue abnormalities which, we hypothesize, could also be reflected in morphological differences in the interictal epileptic and background EEG activity. METHODS: We searched our database of intracranial EEG recordings for mesiotemporal lobe epilepsy patients with either predominant low-voltage fast activity (LVF) or periodic spiking (PS)...
May 9, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28549245/analysis-of-heart-rate-variability-and-risk-factors-for-sudep-in-patients-with-drug-resistant-epilepsy
#3
Leyla Baysal-Kirac, Nail Güven Serbest, Erdi Şahin, Hava Özlem Dede, Candan Gürses, Ayşen Gökyiğit, Nerses Bebek, Ahmet Kaya Bilge, Betül Baykan
BACKGROUND: Cardiac problems have been suggested as causes of sudden unexpected death in epilepsy (SUDEP). Our aim was to investigate possible associations of cardiac autonomic functions based on heart rate variability (HRV) parameters with risk factors of SUDEP in patients with drug-resistant epilepsy. METHODS: Forty-seven patients with drug-resistant seizures and 45 healthy control subjects were enrolled in the study. Interictal time domain parameters of HRV were evaluated with 24-hour Holter recordings...
May 23, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28548599/an-extent-of-resection-threshold-for-seizure-freedom-in-patients-with-low-grade-gliomas
#4
David S Xu, Al-Wala Awad, Chad Mehalechko, Jeffrey R Wilson, Lynn S Ashby, Stephen W Coons, Nader Sanai
OBJECTIVE Seizures are the most common presenting symptom of newly diagnosed WHO Grade II gliomas (low-grade glioma [LGG]) and significantly impair quality of life. Although gross-total resection of LGG is associated with better seizure control, it remains unclear whether an extent of resection (EOR) "threshold" exists for long-term seizure control. Specifically, what proportion of FLAIR-positive tissue in patients with newly diagnosed LGG must be removed to achieve Engel Class I seizure freedom? To clarify the EOR threshold for long-term seizure control, the authors analyzed data from a consecutive series of patients with newly diagnosed LGG who presented with seizures and subsequently underwent microsurgical resection...
May 26, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28548558/the-role-of-genetic-testing-in-epilepsy-diagnosis-and-management
#5
Yvonne G Weber, Saskia Biskup, Katherine L Helbig, Sarah von Spiczak, Holger Lerche
Epilepsy is a common neurological disorder characterized by recurrent unprovoked seizures. More than 500 epilepsy-associated genes have been described in the literature. Most of these genes play an important role in neuronal excitability, cortical development or synaptic transmission. A growing number of genetic variations have implications on diagnosis and prognostic or therapeutic advice in terms of a personalized medicine. Area covered: The review presents the different forms of genetic epilepsies with respect to their underlying genetic and functional pathophysiology and aims to give advice for recommended genetic testing...
May 26, 2017: Expert Review of Molecular Diagnostics
https://www.readbyqxmd.com/read/28547518/life-threatening-posterior-reversible-encephalopathy-syndrome-in-the-cerebellum-treated-by-posterior-fossa-decompression
#6
Yousef Abusabha, Athanasios K Petridis, Bastian Kraus, Marcel A Kamp, Hans-Jakob Steiger, Kerim Beseoglu
Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiological syndrome associated with various clinical conditions, such as headache, encephalopathy, and seizures. It is reversible if a prompt diagnosis is made and treatment undertaken. We report a 52-year-old male with hypertensive crisis. Progressing somnolence and an unresponsive left pupil occurred. MRI revealed an intra-axial hyperintensity of the cerebellum and brainstem and occlusive hydrocephalus suggestive of encephalitis or a tumor...
May 25, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28546310/heterozygous-gnal-mice-are-a-novel-animal-model-to-study-dystonia-pathophysiology
#7
Assunta Pelosi, Fabien Menardy, Daniela Popa, Jean-Antoine Girault, Denis Hervé
Dystonia is a movement disorder characterized by sustained or intermittent muscle contractions. Its pathophysiological mechanisms are still poorly understood. Dominant mutations of the GNAL gene are a cause of isolated dystonia (DYT25) in patients. Some mutations result in a complete loss of function of the encoded protein, Gαolf, an adenylyl-cyclase-stimulatory G protein highly enriched in striatal projection neurons, in which it mediates the actions of dopamine and adenosine. We used male and female heterozygous Gnal knockout mice (Gnal+/-) to study how GNAL haplodeficiency is implicated in dystonia...
May 25, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28545782/autoimmune-encephalopathy-for-psychiatrists-when-to-suspect-autoimmunity-and-what-to-do-next
#8
REVIEW
Mark Oldham
OBJECTIVE: To provide a critical review of autoimmune encephalopathy-broadly defined as neuropsychiatric features directly related to an autoimmune process-relevant for psychiatric practice. METHODS: We consulted rheumatology textbooks to define the scope of autoimmune conditions and identified recent reviews of rheumatic conditions, autoimmune vasculitis, and autoimmune encephalitis. We integrated these with primary reports to provide a clinically relevant overview of autoimmune encephalopathy...
May 2017: Psychosomatics
https://www.readbyqxmd.com/read/28545673/burst-suppression-pattern-on-electroencephalogram-secondary-to-valproic-acid-induced-hyperammonemic-encephalopathy
#9
Koshi A Cherian, Alan D Legatt
BACKGROUND: Valproic acid may induce hyperammonemic encephalopathy. Various electroencephalogram (EEG) abnormalities have been documented in association with this condition, but not burst suppression, an abnormal EEG pattern that is associated with severe encephalopathy. METHODS: Serial EEGs, clinical observations, and laboratory findings were analyzed. PATIENT DESCRIPTION: This 13-year-old girl with autism and intractable epilepsy experienced increased seizures; her valproic acid dose was increased and other antiepileptic drugs were administered...
January 4, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28544992/pharmacokinetics-pharmacodynamics-and-tolerability-of-usl261-midazolam-nasal-spray-randomized-study-in-healthy-geriatric-and-non-geriatric-adults
#10
Alexander K Berg, Michael J Myrvik, Peter J Van Ess
AIM: Characterize pharmacokinetics, pharmacodynamics, and safety/tolerability of USL261 in geriatric adults to inform its potential for treating bouts of increased seizure activity. METHODS: Phase 1, randomized, double-blind, 2-way crossover study in healthy geriatric (≥65years; n=18) and non-geriatric (18-40years; n=12) adults evaluated single USL261 doses (2.5 and 5.0mg) administered intranasally. Pharmacokinetic parameters were estimated for midazolam and 1-hydroxymidazolam (active metabolite), including area under the plasma concentration-time curve (AUC), maximum plasma concentration (Cmax), time to Cmax (Tmax), and half-life (t1/2)...
May 22, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28544889/juvenile-myoclonic-epilepsy-as-a-spectrum-disorder-a-focused-review
#11
REVIEW
Betül Baykan, Peter Wolf
In consequence of newer research juvenile myoclonic epilepsy (JME) is no longer seen as a homogeneous disease. The causes of the existing variance are only partially known yet. We discuss to what extent the phenotypical spectrum of this polygenetically determined disorder expresses genetically defined endophenotypes, or is due to mere quantitative differences in the expression of the core phenotype. Of the three common seizure types of JME, myoclonic, generalized tonic-clonic and absences, absences also occur independently and are strong candidates for an endophenotype...
May 18, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28544888/pre-stroke-seizures-a-nationwide-register-based-investigation
#12
Johan Zelano, David Larsson, Eva Kumlien, Signild Åsberg
PURPOSE: The relationship between cerebrovascular disease and seizures is clearly illustrated by poststroke epilepsy. Seizures can also be the first manifestation of cerebrovascular disease and case-control studies have demonstrated that seizures carry an increased risk of subsequent stroke. Thus, seizures could serve as a marker for vascular risk that merits intervention, but more data is needed before proper trials can be conducted. The occurrence of pre-stroke seizures has not been assessed on a national scale...
May 17, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28544746/clinical-outcomes-following-proton-therapy-for-children-with-central-nervous-system-tumors-referred-overseas
#13
Daniel J Indelicato, Julie A Bradley, Eric S Sandler, Philipp R Aldana, Amy Sapp, Jennifer E Gains, Adrian Crellin, Ronny L Rotondo
BACKGROUND: International, multidisciplinary care of children with central nervous system (CNS) tumors presents unique challenges. The aim of this study is to report patient outcomes of U.K. children referred for proton therapy to a North American facility. METHODS: From 2008 to 2016, 166 U.K. children with approved CNS tumors were treated with proton therapy at a single academic medical center in the United States. Median age was 7 years (range, 1-19). Median follow-up was 2...
May 24, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28544133/autoimmune-encephalitis-associated-with-voltage-gated-potassium-channels-complex-and-leucine-rich-glioma-inactivated-1-antibodies-a-national-cohort-study
#14
M Celicanin, M Blaabjerg, C Maersk-Moller, S Beniczky, L Marner, C Thomsen, F W Back, D Kondziella, H Andersen, F Somnier, Z Illes, L H Pinborg
BACKGROUND AND PURPOSE: The aim of this study was to describe clinical and paraclinical characteristics of all Danish patients who tested positive for anti-voltage-gated potassium channels (VGKC)-complex, anti-leucine-rich glioma-inactivated 1 (LGI1) and anti-contactin-associated protein-2 antibodies in the serum/cerebrospinal fluid between 2009 and 2013 with follow-up interviews in 2015 and 2016. METHODS: We evaluated antibody status, symptoms leading to testing, course of disease, suspected diagnosis and time of admission as well as diagnosis and treatment...
May 25, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28543780/intranasal-midazolam-versus-rectal-diazepam-for-the-management-of-canine-status-epilepticus-a-multicenter-randomized-parallel-group-clinical-trial
#15
M Charalambous, S F M Bhatti, L Van Ham, S Platt, N D Jeffery, A Tipold, J Siedenburg, H A Volk, D Hasegawa, A Gallucci, G Gandini, M Musteata, E Ives, A E Vanhaesebrouck
BACKGROUND: Intranasal administration of benzodiazepines has shown superiority over rectal administration for terminating emergency epileptic seizures in human trials. No such clinical trials have been performed in dogs. OBJECTIVE: To evaluate the clinical efficacy of intranasal midazolam (IN-MDZ), via a mucosal atomization device, as a first-line management option for canine status epilepticus and compare it to rectal administration of diazepam (R-DZP) for controlling status epilepticus before intravenous access is available...
May 24, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28543284/in-utero-oxcarbazepine-exposure-and-neonatal-abstinence-syndrome-case-report-and-brief-review-of-the-literature
#16
Chao-Yang Chen, Xing Li, Ling-Yue Ma, Peng-Hui Wu, Ying Zhou, Qi Feng, Yi-Min Cui
Oxcarbazepine is a second-generation antiepileptic drug that is used to treat partial seizures. Although it has been increasingly used in pregnant women, its fetal safety has not been fully validated. We describe a 12-hour-old neonate who developed neonatal abstinence syndrome (NAS) following intrauterine exposure to oxcarbazepine. The neonate was born by cesarean section to a mother who took oxcarbazepine 300 mg/day for treatment of seizures throughout her pregnancy. Approximately 12 hours after birth, the infant developed paroxysmal jitter, which mainly presented as increased excitability, irritability, limb shaking, and increased muscle tone...
May 20, 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28543030/defining-epileptogenic-networks-contribution-of-seeg-and-signal-analysis
#17
REVIEW
Fabrice Bartolomei, Stanislas Lagarde, Fabrice Wendling, Aileen McGonigal, Viktor Jirsa, Maxime Guye, Christian Bénar
Epileptogenic networks are defined by the brain regions involved in the production and propagation of epileptic activities. In this review we describe the historical, methodologic, and conceptual bases of this model in the analysis of electrophysiologic intracerebral recordings. In the context of epilepsy surgery, the determination of cerebral regions producing seizures (i.e., the "epileptogenic zone") is a crucial objective. In contrast with a traditional focal vision of focal drug-resistant epilepsies, the concept of epileptogenic networks has been progressively introduced as a model better able to describe the complexity of seizure dynamics and realistically describe the distribution of epileptogenic anomalies in the brain...
May 20, 2017: Epilepsia
https://www.readbyqxmd.com/read/28542985/cholinesterase-reactivators-and-bioscavengers-for-pre-and-post-exposure-treatments-of-organophosphorus-poisoning
#18
REVIEW
Patrick Masson, Florian Nachon
Organophosphorus agents (OPs) irreversibly inhibit acetylcholinesterase (AChE) causing a major cholinergic syndrome. The medical counter-measures of OP poisoning have not evolved for the last 30 years with carbamates for pretreatment, pyridinium oximes-based AChE reactivators, antimuscarinic drugs and neuroprotective benzodiazepines for post-exposure treatment. These drugs ensure protection of peripheral nervous system and mitigate acute effects of OP lethal doses. However, they have significant limitations...
May 21, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28542865/disruption-of-ywhae-gene-at-17p13-3-causes-learning-disabilities-and-brain-abnormalities
#19
Abdul Noor, Simina Bogatan, Nicholas Watkins, Wendy S Meschino, Dimitri J Stavropoulos
There is a broad phenotypic spectrum of patients with 17p13.3 deletions. One of the most prominent features is lissencephaly caused by haploinsufficiency of the gene PAFAH1B1. The deletion of this gene and those distal to it, results in Miller-Dieker syndrome, however there have been many reports of patients with haploinsufficiency of the distal genes alone. The deletions of these genes including YWHAE, CRK and TUSC5 have been studied extensively and YWHAE has been postulated to be the cause of neurological abnormalities...
May 23, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28542864/circadian-clustering-of-spontaneous-epileptic-seizures-emerges-after-pilocarpine-induced-status-epilepticus
#20
Julika Pitsch, Albert J Becker, Susanne Schoch, Johannes Alexander Müller, Marco de Curtis, Vadym Gnatkovsky
OBJECTIVE: Seizures in mesial temporal lobe epilepsy (MTLE) associated with hippocampal sclerosis are thought to develop with various latency intervals after an initial transient brain insult. To study seizure dynamics after an initial transient precipitating insult in a systematic fashion, we utilized continuous video-electroencephalography (EEG) monitoring after the induction of status epilepticus (SE) in a mouse MTLE model. METHODS: Continuous 24/7 video/telemetric hippocampal EEG recordings in the systemic pilocarpine MTLE mouse model...
May 24, 2017: Epilepsia
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