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https://www.readbyqxmd.com/read/28229394/epileptic-encephalopathies-clinical-syndromes-and-pathophysiological-concepts
#1
REVIEW
Markus von Deimling, Ingo Helbig, Eric D Marsh
Epileptic encephalopathies account for a large proportion of the intractable early-onset epilepsies and are characterized by frequent seizures and poor developmental outcome. The epileptic encephalopathies can be loosely divided into two related groups of named syndromes. The first comprises epilepsies where continuous EEG changes directly result in cognitive and developmental dysfunction. The second includes patients where cognitive impairment is present at seizure onset and is due to the underlying etiology but the epileptic activity may then worsen the cognitive abilities over time...
February 2017: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/28222432/extracellular-hmgb1-modulates-glutamate-metabolism-associated-with-kainic-acid-induced-epilepsy-like-hyperactivity-in-primary-rat-neural-cells
#2
Yuji Kaneko, Colleen Pappas, Teresita Malapira, Fernando Ĺ Vale, Naoki Tajiri, Cesar V Borlongan
BACKGROUND/AIMS: Neuroinflammatory processes have been implicated in the pathophysiology of seizure/epilepsy. High mobility group box 1 (HMGB1), a non-histone DNA binding protein, behaves like an inflammatory cytokine in response to epileptogenic insults. Kainic acid (KA) is an excitotoxic reagent commonly used to induce epilepsy in rodents. However, the molecular mechanism by which KA-induced HMGB1 affords the initiation of epilepsy, especially the role of extracellular HMGB1 in neurotransmitter expression, remains to be elucidated...
February 20, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28222338/effect-of-synaptic-adhesion-like-molecule-3-on-epileptic-seizures-evidence-from-animal-models
#3
Jie Li, Ling Chen, Na Wang, Guohui Jiang, Yuqing Wu, Yi Zhang
Axonal sprouting and synaptic reorganization are the primary pathophysiological characteristics of epilepsy. Recent studies demonstrated that synaptic adhesion-like molecule 3 (SALM3) is highly expressed in the central nervous system and plays important roles in neurite outgrowth, branching, and axon guidance, mechanisms that are also observed in epilepsy. However, the expression of SALM3 in the epileptic brain and the effect of SALM3 in the pathogenesis of epilepsy remain unclear. The aims of this study were to investigate SALM3 expression in rat models of epilepsy and to explore the functional significance of SALM3 in epilepsy...
February 17, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28222113/differentially-expressed-proteins-underlying-childhood-cortical-dysplasia-with-epilepsy-identified-by-itraq-proteomic-profiling
#4
Lu Qin, Xi Liu, Shiyong Liu, Yi Liu, Yixuan Yang, Hui Yang, Yangmei Chen, Lifen Chen
Cortical dysplasia accounts for at least 14% of epilepsy cases, and is mostly seen in children. However, the understanding of molecular mechanisms and pathogenesis underlying cortical dysplasia is limited. The aim of this cross-sectional study is to identify potential key molecules in the mechanisms of cortical dysplasia by screening the proteins expressed in brain tissues of childhood cortical dysplasia patients with epilepsy using isobaric tags for relative and absolute quantitation-based tandem mass spectrometry compared to controls, and several differentially expressed proteins that are not reported to be associated with cortical dysplasia previously were selected for validation using real-time polymerase chain reaction, immunoblotting and immunohistochemistry...
2017: PloS One
https://www.readbyqxmd.com/read/28218059/sudden-unexpected-death-in-epilepsy-sudep-what-every-neurologist-should-know
#5
Rohit Shankar, Elizabeth J Donner, Brendan McLean, Lina Nashef, Torbjörn Tomson
This review aims to empower general neurologists to provide better informed person-centred advice on sudden unexpected death in epilepsy (SUDEP) to people with epilepsy in order to help keep them safe. Past and present evidence is consolidated in order to inform readers about SUDEP, and up-to-date insights into the epidemiology, diagnostic classification, pathophysiology, risk factors, influence of co-morbidity, and importance of sensitive person-centred communication are outlined. This review provides "fingertip" information to the practicing neurologist with regards to identifying and communicating risks for SUDEP and suggests practical measures for managing these risks in partnership with the patient...
February 16, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/28216336/pathophysiology-of-absence-epilepsy-insights-from-genetic-models
#6
REVIEW
Antoine Depaulis, Stéphane Charpier
Absence Epilepsy (AE) is a prototypic epileptic syndrome that develops during brain maturation but cannot be fully explored in human patients. Genetic animal models, especially rats with spike-and-wave discharges recorded on the electroencephalogram, the hallmark of absence seizures, offer strong face validity with the human pathology that allows precise exploration of the pathophysiology of this form of epilepsy. Using an array of different methods, recent studies have demonstrated that spike-and-wave discharges are initiated in the primary somatosensory cortex and then rapidly propagate to motor cortices and thalamic nuclei...
February 16, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28215998/photosensitivity-in-generalized-epilepsies
#7
REVIEW
Shervonne Poleon, Jerzy P Szaflarski
Photosensitivity, which is the hallmark of photosensitive epilepsy (PSE), is described as an abnormal EEG response to visual stimuli known as a photoparoxysmal response (PPR). The PPR is a well-recognized phenomenon, occurring in 2-14% of patients with epilepsy but its pathophysiology is not clearly understood. PPR is electrographically described as 2-5Hz spike, spike-wave, or slow wave complexes with frontal and paracentral prevalence. Diagnosis of PPR is confirmed using intermittent photic stimulation (IPS) as well as video monitoring...
February 16, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28210509/recurrence-of-postoperative-stress-induced-cardiomyopathy-resulting-from-status-epilepticus
#8
Grant A Miller, Yousef M Ahmed, Nicki S Tarant
Introduction. Classically, stress-induced cardiomyopathy (SIC), also known as takotsubo cardiomyopathy, displays the pathognomonic feature of reversible left ventricular apical ballooning without coronary artery stenosis following stressful event(s). Temporary reduction in ejection fraction (EF) resolves spontaneously. Variants of SIC exhibiting mid-ventricular regional wall motion abnormalities have been identified. Recent case series present SIC as a finding in association with sudden unexplained death in epilepsy (SUDEP)...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/28209738/optogenetic-low-frequency-stimulation-of-specific-neuronal-populations-abates-ictogenesis
#9
Zahra Shiri, Maxime Lévesque, Guillaume Etter, Frédéric Manseau, Sylvain Williams, Massimo Avoli
Despite many advances made in understanding the pathophysiology of epileptic disorders, seizures remain poorly controlled in approximately one third of mesial temporal lobe epilepsy patients. Here, we established the efficacy of cell type-specific low-frequency stimulation (LFS) in controlling ictogenesis in the mouse entorhinal cortex (EC) in an in vitro brain slice preparation. Specifically, we used 1 Hz optogenetic stimulation of Ca(2+)/calmodulin-dependent protein kinase II-positive principal cells as well as of parvalbumin- or somatostatin-positive interneurons to study the effects of such repetitive activation on epileptiform discharges induced by 4-aminopyridine...
February 16, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28203222/unexpected-death-of-a-child-with-complex-febrile-seizures-pathophysiology-similar-to-sudden-unexpected-death-in-epilepsy
#10
Brian J Dlouhy, Michael A Ciliberto, Christina L Cifra, Patricia A Kirby, Devin L Shrock, Marcus Nashelsky, George B Richerson
Febrile seizures are usually considered relatively benign. Although some cases of sudden unexplained death in childhood have a history of febrile seizures, no documented case of febrile seizure-induced death has been reported. Here, we describe a child with complex febrile seizures who died suddenly and unexpectedly after a suspected seizure while in bed at night during the beginning phases of sleep. She was resuscitated and pronounced brain dead 2 days later at our regional medical center. Autopsy revealed multiorgan effects of hypoperfusion and did not reveal an underlying (precipitating) disease, injury, or toxicological cause of death...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28202819/-melas-mitochondrial-encephalomyopathy-lactic-acidosis-and-stroke-like-episodes
#11
Hidetomo Murakami, Kenjiro Ono
Mitochondrial disease is caused by a deficiency in the energy supply to cells due to mitochondrial dysfunction. Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) is a mitochondrial disease that presents with stroke-like episodes such as acute onset of neurological deficits and characteristic imaging findings. Stroke-like episodes in MELAS have the following features: 1) neurological deficits due to localization of lesions in the brain, 2) episodes often accompany epilepsy, 3) lesions do not follow the vascular supply area, 4) lesions are more often seen in the posterior brain than in the anterior brain, 5) lesions spread to an adjacent area in the brain, and 6) neurological symptoms often disappear together with imaging findings, but later relapse...
February 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/28202778/nodding-syndrome-preventable-and-treatable
#12
REVIEW
Robert Colebunders, Maarten J Titulaer
New insights into the pathophysiology of nodding syndrome, a form of childhood epilepsy associated with onchocerciasis, provide new opportunities for disease treatment and parasite control (Johnson et al).
February 15, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28199998/delayed-intraparenchymal-and-intraventricular-hemorrhage-requiring-surgical-evacuation-after-mri-guided-laser-interstitial-thermal-therapy-for-lesional-epilepsy
#13
Sean M Barber, Luke Tomycz, Timothy George, Dave F Clarke, Mark Lee
BACKGROUND: Symptomatic intracranial hemorrhage is a rare complication of laser interstitial thermal therapy (LITT). OBJECTIVES: To present a unique case of delayed symptomatic intracranial hemorrhage occurring after a LITT procedure for epilepsy in a high-volume center (68 LITT procedures for pediatric epilepsy have been performed). METHODS: An 18-year-old male with epilepsy underwent LITT to an area of heterotopia near the right lateral ventricle...
February 16, 2017: Stereotactic and Functional Neurosurgery
https://www.readbyqxmd.com/read/28193459/epileptiform-activity-and-behavioral-arrests-in-mice-overexpressing-the-calcium-channel-subunit-%C3%AE-2%C3%AE-1
#14
Leonardo C Faria, Feng Gu, Isabel Parada, Ben Barres, Z David Luo, David A Prince
: The alpha2delta-1 subunit (α2δ-1) of voltage-gated calcium channels is a receptor for astrocyte-secreted thrombospondins that promote developmental synaptogenesis.Alpha2delta-1 receptors are upregulated in models of injury-induced peripheral pain and epileptogenic neocortical trauma associated with an enhancement of excitatory synaptic connectivity. These results lead to the hypothesis that overexpression of α2δ-1 alone in neocortex of uninjured transgenic (TG) mice might result in increased excitatory connectivity and consequent cortical hyperexcitability and epileptiform activity...
February 10, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28166388/toll-like-receptor-3-deficiency-decreases-epileptogenesis-in-a-pilocarpine-model-of-se-induced-epilepsy-in-mice
#15
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/28153692/chronic-demyelination-induced-seizures
#16
Andrew S Lapato, Jenny I Szu, Jonathan P C Hasselmann, Anna J Khalaj, Devin K Binder, Seema K Tiwari-Woodruff
Multiple sclerosis (MS) patients are three to six times more likely to develop epilepsy compared to the rest of the population. Seizures are more common in patients with early onset or progressive forms of the disease and prognosticate rapid progression to disability and death. Gray matter atrophy, hippocampal lesions, interneuron loss, and elevated juxtacortical lesion burden have been identified in MS patients with seizures; however, translational studies aimed at elucidating the pathophysiological processes underlying MS epileptogenesis are limited...
January 30, 2017: Neuroscience
https://www.readbyqxmd.com/read/28131205/altered-degree-centrality-in-childhood-absence-epilepsy-a-resting-state-fmri-study
#17
Xueyu Wang, Dongmei Jiao, Xinying Zhang, Xiangtao Lin
Modern network studies have suggested that the pathology of many neurological diseases is in fact not equally distributed over the brain but preferentially affects the hub regions. This study aims to investigate how hub regions were affected in Children with Childhood absence epilepsy (CAE) using resting-state fMRI (rs-fMRI). As one important measures obtained from rs-fMRI, degree centrality (DC) calculates the number of direct connections between a given node and the rest of the brain within the entire connectivity matrix of the brain...
February 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28131095/a-potential-mechanism-for-the-ameliorative-effect-of-thymoquinone-on-pentylenetetrazole-induced-kindling-and-cognitive-impairments-in-mice
#18
Ahmed O Abdel-Zaher, Hanan S M Farghaly, Magda M Y Farrag, Mahran S Abdel-Rahman, Basel A Abdel-Wahab
Cognitive dysfunction is commonly observed in epileptic patients. Pentylenetetrazole (PTZ) kindling is a well established animal model which simulates clinical epilepsy. This study evaluated the potential role of glutamate, oxidative stress and nitric oxide (NO) overproduction in pentylenetetrazole (PTZ)-induced kindling and associated cognitive impairments in mice and effect of thymoquinone on these parameters. Repeated treatment of mice with a subconvulsive dose of PTZ (35mg/kg i.p.) once every alternate-day for 12 injections induced kindling...
January 25, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28124054/metal-maps-of-sclerotic-hippocampi-of-patients-with-mesial-temporal-lobe-epilepsy
#19
Miloš Opačić, Aleksandar J Ristić, Danijela Savić, Vid Simon Šelih, Marko Živin, Dragoslav Sokić, Savo Raičević, Vladimir Baščarević, Ivan Spasojević
The loss of metal homeostasis has been implicated in the pathophysiology of mesial temporal lobe epilepsy associated with hippocampal sclerosis (mTLE-HS). Here we applied laser ablation inductively coupled plasma mass spectrometry imaging to establish the spatial distribution of Zn, Fe, Cu and Mn in coronal sections of hippocampi of four patients with drug-resistant mTLE-HS who underwent amygdalohippocampectomy. Detailed maps of the metal concentrations in the different morphological areas/layers were built and analyzed...
January 26, 2017: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/28104639/hippocampal-atrophy-is-associated-with-altered-hippocampus-posterior-cingulate-cortex-connectivity-in-mesial-temporal-lobe-epilepsy-with-hippocampal-sclerosis
#20
Y C Shih, C E Tseng, F-H Lin, H H Liou, W Y I Tseng
BACKGROUND AND PURPOSE: Unilateral mesial temporal lobe epilepsy and hippocampal sclerosis have structural and functional abnormalities in the mesial temporal regions. To gain insight into the pathophysiology of the epileptic network in mesial temporal lobe epilepsy with hippocampal sclerosis, we aimed to clarify the relationships between hippocampal atrophy and the altered connection between the hippocampus and the posterior cingulate cortex in patients with mesial temporal lobe epilepsy with hippocampal sclerosis...
January 19, 2017: AJNR. American Journal of Neuroradiology
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