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Intractable epilepsy

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https://www.readbyqxmd.com/read/28821005/anticonvulsant-effect-of-cannabidiol-in-the-pentylenetetrazole-model-pharmacological-mechanisms-electroencephalographic-profile-and-brain-cytokine-levels
#1
Luciano R Vilela, Isabel V Lima, Érica B Kunsch, Hyorrana Priscila P Pinto, Aline S de Miranda, Érica Leandro M Vieira, Antônio Carlos P de Oliveira, Marcio Flávio D Moraes, Antônio L Teixeira, Fabricio A Moreira
Cannabidiol (CBD), the main nonpsychotomimetic compound from Cannabis sativa, inhibits experimental seizures in animal models and alleviates certain types of intractable epilepsies in patients. Its pharmacological profile, however, is still uncertain. Here we tested the hypothesis that CBD anticonvulsant mechanisms are prevented by cannabinoid (CB1 and CB2) and vanilloid (TRPV1) receptor blockers. We also investigated its effects on electroencephalographic (EEG) activity and hippocampal cytokines in the pentylenetetrazole (PTZ) model...
August 15, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28815955/the-role-of-iqsec2-in-syndromic-intellectual-disability-narrowing-the-diagnostic-odyssey
#2
Benjamin M Helm, Zoe Powis, Carlos E Prada, Olga L Casasbuenas-Alarcon, Tonya Balmakund, G B Schaefer, Stephen G Kahler, Julie Kaylor, Susan Winter, Yuri A Zarate, Samantha A Schrier Vergano
While X-linked intellectual disability (XLID) syndromes pose a diagnostic challenge for clinicians, an increasing number of recognized disorders and their genetic etiologies are providing answers for patients and their families. The availability of clinical exome sequencing is broadening the ability to identify mutations in genes previously unrecognized as causing XLID. In recent years, the IQSEC2 gene, located at Xp11.22, has emerged as the cause of multiple cases of both nonsyndromic and syndromic XLID. Herein we present a case series of six individuals (five males, one female) with intellectual disability and seizures found to have alterations in IQSEC2...
August 17, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28815309/elimination-of-medically-intractable-epileptic-drop-attacks-following-endoscopic-total-corpus-callosotomy-in-rett-syndrome
#3
Keisuke Ueda, Sandeep Sood, Eishi Asano, Ajay Kumar, Aimee F Luat
INTRODUCTION: Rett syndrome is a neurodevelopmental genetic disorder, characterized by developmental delay, hand stereotypies, abnormal gait, and acquired microcephaly. Epilepsy is very common in Rett syndrome and can be medically intractable. It remains uncertain if a patient with epileptic drop attacks associated with this genetic disease can benefit from corpus callosotomy. CASE REPORT: We report an 8-year-old girl with Rett syndrome and medically intractable epileptic drop attacks who underwent endoscopic total corpus callosotomy without any complications that led to the successful elimination of her seizures...
August 16, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28811646/pafah1b1-haploinsufficiency-disrupts-gaba-neurons-and-synaptic-e-i-balance-in-the-dentate-gyrus
#4
Matthew T Dinday, Kelly M Girskis, Sunyoung Lee, Scott C Baraban, Robert F Hunt
Hemizygous mutations in the human gene encoding platelet-activating factor acetylhydrolase IB subunit alpha (Pafah1b1), also called Lissencephaly-1, can cause classical lissencephaly, a severe malformation of cortical development. Children with this disorder suffer from deficits in neuronal migration, severe intellectual disability, intractable epilepsy and early death. While many of these features can be reproduced in Pafah1b1(+/-) mice, the impact of Pafah1b1(+/-) on the function of individual subpopulations of neurons and ultimately brain circuits is largely unknown...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28799062/long-term-epilepsy-associated-tumor-in-the-amygdala-of-a-16-year-old-boy-report-of-a-rare-case-having-intranuclear-filaments
#5
Yoon Jin Cha, Dong-Seok Kim, Seung-Koo Lee, Hoon-Chul Kang, Se Hoon Kim
The term "long-term epilepsy-associated tumor (LEAT)" encompasses brain lesions associated with drug-resistant epilepsy over a long duration (≥2 years). Notably, some LEATs do not fit into any of the classifications of the World Health Organization (WHO). Herein, we report a LEAT that occurred in the left amygdala of a 16-year-old patient with intractable epilepsy. Histological examination of the resected amygdala revealed diffusely infiltrating tumor cells in the cortex. Perineuronal satellitosis and perivascular aggregation of tumor cells were apparent, along with mild nuclear enlargement and cytologic atypia...
August 10, 2017: Brain Tumor Pathology
https://www.readbyqxmd.com/read/28797973/frontal-fibers-connecting-the-superior-frontal-gyrus-to-broca-s-area-a-cortico-cortical-evoked-potential-study
#6
Satoshi Ookawa, Rei Enatsu, Aya Kanno, Satoko Ochi, Yukinori Akiyama, Tamaki Kobayashi, Yukihiro Yamao, Takayuki Kikuchi, Riki Matsumoto, Takeharu Kunieda, Nobuhiro Mikuni
BACKGROUND: The frontal aslant tract (FAT) is a deep frontal pathway connecting the superior frontal gyrus (SFG) to Broca's area. This fiber is assumed to be associated with language functions, especially speech initiation and spontaneity. OBJECTIVE: The aim of this study was to electrophysiologically investigate this network using cortico-cortical evoked potentials (CCEPs). METHOD: Eight patients with brain tumors or medically intractable focal epilepsies who underwent frontal craniotomy over the language dominant side were enrolled in this study...
August 7, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28782373/correlating-resting-state-functional-mri-connectivity-by-independent-component-analysis-based-epileptogenic-zones-with-intracranial-eeg-localized-seizure-onset-zones-and-surgical-outcomes-in-prospective-pediatric-intractable-epilepsy-study
#7
Varina Boerwinkle, Deepankar Mohanty, Stephen Foldes, Danielle Guffey, Charles G Minard, Lucia Mirea, Aditya Vedantam, Jeffery S Raskin, Sandi Lam, Margaret Bond, P David Adelson, Angus A Wilfong, Daniel J Curry
OBJECTIVE: Prospective investigation determining agreement between the epileptogenic zone(s) (EZ) localization by resting state functional MRI (rs-fMRI) and the seizure onset zone(s) (SOZ) identified by intracranial EEG (ic-EEG) using novel differentiating and ranking criteria of rs-fMRI abnormal independent components in a consecutive heterogeneous pediatric intractable epilepsy population prospectively was conducted. METHODS: The EZ criteria were developed from an initial cohort of 350 patients evaluated for epilepsy surgery over a 3-year period...
August 7, 2017: Brain Connectivity
https://www.readbyqxmd.com/read/28777720/delay-differential-analysis-of-seizures-in-multichannel-electrocorticography-data
#8
Claudia Lainscsek, Jonathan Weyhenmeyer, Sydney S Cash, Terrence J Sejnowski
High-density electrocorticogram (ECoG) electrodes are capable of recording neurophysiological data with high temporal resolution with wide spatial coverage. These recordings are a window to understanding how the human brain processes information and subsequently behaves in healthy and pathologic states. Here, we describe and implement delay differential analysis (DDA) for the characterization of ECoG data obtained from human patients with intractable epilepsy. DDA is a time-domain analysis framework based on embedding theory in nonlinear dynamics that reveals the nonlinear invariant properties of an unknown dynamical system...
August 4, 2017: Neural Computation
https://www.readbyqxmd.com/read/28776646/postoperative-seizure-freedom-does-not-normalize-altered-connectivity-in-temporal-lobe-epilepsy
#9
Luigi Maccotta, Mayra A Lopez, Babatunde Adeyemo, Beau M Ances, Brian K Day, Lawrence N Eisenman, Joshua L Dowling, Eric C Leuthardt, Bradley L Schlaggar, Robert Edward Hogan
OBJECTIVES: Specific changes in the functional connectivity of brain networks occur in patients with epilepsy. Yet whether such changes reflect a stable disease effect or one that is a function of active seizure burden remains unclear. Here, we longitudinally assessed the connectivity of canonical cognitive functional networks in patients with intractable temporal lobe epilepsy (TLE), both before and after patients underwent epilepsy surgery and achieved seizure freedom. METHODS: Seventeen patients with intractable TLE who underwent epilepsy surgery with Engel class I outcome and 17 matched healthy controls took part in the study...
August 3, 2017: Epilepsia
https://www.readbyqxmd.com/read/28774647/discrimination-of-a-medial-functional-module-within-the-temporal-lobe-using-an-effective-connectivity-model-a-ccep-study
#10
Julien Krieg, Laurent Koessler, Jacques Jonas, Sophie Colnat-Coulbois, Jean-Pierre Vignal, Christian G Bénar, Louis G Maillard
The temporal lobe is classically divided in two functional systems: the ventral visual pathway and the medial temporal memory system. However, their functional separation has been challenged by studies suggesting that the medial temporal lobe could be best understood as an extension of the hierarchically organized ventral visual pathway. Our purpose was to investigate (i) whether cerebral regions within the temporal lobe could be grouped into distinct functional assemblies, and (ii) which regions were central within these functional assemblies...
July 31, 2017: NeuroImage
https://www.readbyqxmd.com/read/28765064/treatment-of-epilepsy-in-patients-with-myasthenia-gravis-is-really-harder-than-it-looks
#11
Paulo José Lorenzoni, Renata Dal-Prá Ducci, Tallulah Spina Tensini, Giuliano Dalledone, Claudia Suemi Kamoi Kay, Luciano de Paola, Lineu Cesar Werneck, Rosana Herminia Scola, Carlos Silvado
The relationship between myasthenia gravis (MG) and epilepsy has been rarely reported. As consequence, there are no specific guidelines for the management of these conditions when they mutually occur. We reported on three patients in whom epilepsy and MG are coexisting, but in different clinical settings. Two patients were treated with antiepileptic drugs which improved their symptoms. One patient has controlled the seizures after a successful anterior temporal lobectomy with no appreciable consequences to her MG...
July 29, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28763722/glutamate-metabolism-in-temporal-lobe-epilepsy-as-revealed-by-dynamic-proton-mrs-following-the-infusion-of-u-13-c-glucose
#12
Brenda L Bartnik-Olson, Daniel Ding, John Howe, Amul Shah, Travis Losey
Focal metabolic dysfunction commonly observed in temporal lobe epilepsy (TLE), and is associated with the development of medical intractability and neurocognitive deficits. It has not been established if this dysfunction is due to cell loss or biochemical dysfunction in metabolic pathways. To explore this question, dynamic (1)H MRS following an infusion of [U(13)- C] glucose was performed to measure glutamate (Glu) metabolism. Subjects (n=6) showed reduced Glu levels (p<0.01) in the ipsilateral mesial temporal lobe (MTL) compared with controls (n=4)...
July 23, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28754198/practice-paper-of-the-academy-of-nutrition-and-dietetics-classic-and-modified-ketogenic-diets-for-treatment-of-epilepsy
#13
Kelly Roehl, Sarika L Sewak
Ketogenic diet (KD) therapy is an established form of treatment for both pediatric and adult patients with intractable epilepsy. Ketogenic diet is a term that refers to any diet therapy in which dietary composition would be expected to result in a ketogenic state of human metabolism. While historically considered a last-resort therapy, classic KDs and their modified counterparts, including the modified Atkins diet and low glycemic index treatment, are gaining ground for use across the spectrum of seizure disorders...
August 2017: Journal of the Academy of Nutrition and Dietetics
https://www.readbyqxmd.com/read/28735133/neurologic-outcome-after-resection-of-parietal-lobe-including-primary-somatosensory-cortex-implications-of-additional-resection-of-posterior-parietal%C3%A2-cortex
#14
Young-Hoon Kim, June Sic Kim, Sang Kun Lee, Chun Kee Chung
OBJECTIVE: Postoperative neurologic outcomes after primary somatosensory cortex (S1) resection have not been well documented. This study was designed to evaluate the neurologic deterioration that follows resection of the S1 areas and to assess the risk factors associated with these morbidities. METHODS: We reviewed 48 consecutive patients with medically intractable epilepsy who underwent resection of the S1 and/or the adjacent cortex. The 48 patients were categorized into 4 groups according to the resected area as seen on postoperative magnetic resonance images: group 1 (resection of S1 only; n = 4), 2 (the posterior parietal cortex [PPC] only; n = 24), 3 (S1 and PPC; n = 10), and 4 (S1 and precentral gyrus; n = 10)...
July 19, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28715338/closed-loop-neurostimulators-a-survey-and-a-seizure-predicting-design-example-for-intractable-epilepsy-treatment
#15
Hossein Kassiri, Sana Tonekaboni, M Tariqus Salam, Nima Soltani, Karim Abdelhalim, Jose Luis Perez Velazquez, Roman Genov
First, existing commercially available open-loop and closed-loop implantable neurostimulators are reviewed and compared in terms of their targeted application, physical size, system-level features, and performance as a medical device. Next, signal processing algorithms as the primary strength point of the closed-loop neurostimulators are reviewed, and various design and implementation requirements and trade-offs are discussed in details along with quantitative examples. The review results in a set of guidelines for algorithm selection and evaluation...
July 17, 2017: IEEE Transactions on Biomedical Circuits and Systems
https://www.readbyqxmd.com/read/28714074/altered-metabolomic-genomic-signature-a-potential-noninvasive-biomarker-of-epilepsy
#16
Helen C Wu, Fabien Dachet, Farhad Ghoddoussi, Shruti Bagla, Darren Fuerst, Jeffrey A Stanley, Matthew P Galloway, Jeffrey A Loeb
OBJECTIVE: This study aimed to identify noninvasive biomarkers of human epilepsy that can reliably detect and localize epileptic brain regions. Having noninvasive biomarkers would greatly enhance patient diagnosis, patient monitoring, and novel therapy development. At the present time, only surgically invasive, direct brain recordings are capable of detecting these regions with precision, which severely limits the pace and scope of both clinical management and research progress in epilepsy...
July 17, 2017: Epilepsia
https://www.readbyqxmd.com/read/28710408/non-ketogenic-combination-of-nutritional-strategies-provides-robust-protection-against-seizures
#17
Glenn Dallérac, Julien Moulard, Jean-François Benoist, Stefan Rouach, Stéphane Auvin, Angèle Guilbot, Loïc Lenoir, Nathalie Rouach
Epilepsy is a neurological condition that affects 1% of the world population. Conventional treatments of epilepsy use drugs targeting neuronal excitability, inhibitory or excitatory transmission. Yet, one third of patients presents an intractable form of epilepsy and fails to respond to pharmacological anti-epileptic strategies. The ketogenic diet is a well-established non-pharmacological treatment that has been proven to be effective in reducing seizure frequency in the pharmaco-resistant patients. This dietary solution is however extremely restrictive and can be associated with complications caused by the high [fat]:[carbohydrate + protein] ratio...
July 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28705496/the-effect-of-cxcr2-inhibition-on-seizure-activity-in-the-pilocarpine-epilepsy-mouse-model
#18
Tao Xu, Xinyuan Yu, Teng Wang, Ying Liu, Xi Liu, Shu Ou, Yangmei Chen
C-X-C motif chemokine receptor 2 (CXCR2) is one of the most well characterized chemokine receptors and is a potential target for treating brain pathologies involving inflammatory processes, including epilepsy. However, the role of CXCR2 in epilepsy has not been investigated, and whether CXCR2 modulates seizure activity in temporal lobe epilepsy (TLE) remains unknown. In this study, we aimed to determine the potential role of CXCR2 in intractable TLE patients and in pilocarpine-induced epileptic mice. Here, through Western blotting and semi-quantitative immunohistochemistry, we detected that CXCR2 protein expression was up-regulated (by nearly 50%) in the temporal neocortex of TLE patients and in the hippocampus and adjacent temporal cortex of pilocarpine mice model...
July 10, 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/28704161/cardiovascular-safety-of-low-dose-fenfluramine-in-dravet-syndrome-a-review-of-its-benefit-risk-profile-in-a-new-patient-population
#19
An-Sofie Schoonjans, Fabienne Marchau, Bernard P Paelinck, Lieven Lagae, Arnold Gammaitoni, Milka Pringsheim, Martin G Keane, Berten Ceulemans
OBJECTIVE: Dravet syndrome (DS) is a rare, treatment-resistant epilepsy syndrome for which current treatment regimens are often ineffective. Fenfluramine is currently in development for treatment of DS based on reports in the 1980s and 1990s of its antiepileptic activity in pediatric patients with intractable epilepsy. However, fenfluramine was withdrawn from global markets in 1997 following reports of its association with pulmonary hypertension and heart valve disease in adult patients treated for obesity...
July 13, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28702333/cough-syncope-induced-by-post-nasal-drip-successfully-managed-by-gabapentin
#20
Hussein Algahtani, Bader Shirah
Syncope is a common complaint in both neurology clinic and emergency department. It is defined as transient loss of consciousness with loss of postural tone, which is usually self-limited and followed by a spontaneous recovery. Our report describes a case of cough syncope resulting from chronic intractable cough caused by post nasal drip. Although his experience was debilitating, we were able to control his symptoms significantly using a small dose of Gabapentin. This dose is much lower when compared with the already established licensed indicated higher doses used for the treatment of neuropathic pain and epilepsy...
2017: Respiratory Medicine Case Reports
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