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https://www.readbyqxmd.com/read/28808577/transition-from-intravenous-to-enteral-ketamine-for-treatment-of-nonconvulsive-status-epilepticus
#1
Michael A Pizzi, Prasuna Kamireddi, William O Tatum, Jerry J Shih, Daniel A Jackson, William D Freeman
BACKGROUND: Nonconvulsive status epilepticus (NCSE) is a diagnosis that is often challenging and one that may progress to refractory NCSE. Ketamine is a noncompetitive N-methyl-d-aspartate antagonist that increasingly has been used to treat refractory status epilepticus. Current Neurocritical Care Society guidelines recommend intravenous (IV) ketamine infusion as an alternative treatment for refractory status epilepticus in adults. On the other hand, enteral ketamine use in NCSE has been reported in only 6 cases (1 adult and 5 pediatric) in the literature to date...
2017: Journal of Intensive Care
https://www.readbyqxmd.com/read/28806589/novel-clinical-manifestations-in-patients-with-kcna2-mutations
#2
Monisha Sachdev, Marina Gaínza-Lein, Dmitry Tchapyjnikov, Yong-Hui Jiang, Tobias Loddenkemper, Mohamad A Mikati
PURPOSE: To report novel clinical manifestations of KCNA2 mutation related epileptic encephalopathy. METHODS: Blood samples were sent for whole exome and Sanger sequencing. Seizure types were characterized by clinical criteria and EEG recording. RESULTS: KCNA2 mutations have been reported in 10 cases who presented with focal, absence, generalized tonic-clonic or myoclonic astatic seizures. Here we describe 3 patients with previously unreported, more severe manifestations...
August 5, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28804898/epilepsy-surgery-of-low-grade-epilepsy-associated-neuroepithelial-tumors-a-retrospective-nationwide-italian-study
#3
Marco Giulioni, Gianluca Marucci, Veronica Pelliccia, Francesca Gozzo, Carmen Barba, Giuseppe Didato, Flavio Villani, Giancarlo Di Gennaro, Pier Paolo Quarato, Vincenzo Esposito, Alessandro Consales, Matteo Martinoni, Gianfranco Vornetti, Corrado Zenesini, Carlo Efisio Marras, Nicola Specchio, Luca De Palma, Raffaele Rocchi, Flavio Giordano, Giovanni Tringali, Paolo Nozza, Gabriella Colicchio, Guido Rubboli, Giorgio Lo Russo, Renzo Guerrini, Paolo Tinuper, Francesco Cardinale, Massimo Cossu
OBJECTIVE: To analyze the attitude and results of Italian epilepsy surgery centers in the surgical management of "low grade epilepsy associated neuroepithelial tumors" (LEATs). METHODS: We conducted a retrospective study enrolling 339 consecutive patients with LEATs who underwent surgery between January 2009 and June 2015 at eight Italian epilepsy surgery centers. We compared demographic, clinical, pathologic, and surgical features of patients with favorable (Engel class I) and unfavorable (Engel class II, III, and IV) seizure outcome...
August 14, 2017: Epilepsia
https://www.readbyqxmd.com/read/28802513/nonconvulsive-status-epilepticus-after-electroconvulsive-therapy-a-review-of-literature
#4
Awais Aftab, Ashley VanDercar, Ayham Alkhachroum, Christine LaGrotta, Keming Gao
BACKGROUND: The clinical presentation and risk factors of nonconvulsive status epilepticus (NCSE) in the context of electroconvulsive therapy (ECT) are poorly understood, and guidance regarding diagnosis and management remains scarce. In this article, we identify case reports of ECT-induced NCSE from literature, and discuss the presentation, diagnosis, and management of these cases in the context of what is known about NCSE from the neurology literature. METHODS: A literature search on PubMed for case reports of NCSE after ECT...
July 15, 2017: Psychosomatics
https://www.readbyqxmd.com/read/28799517/imaging-biomarkers-of-anti-epileptic-drug-action-insights-from-magnetic-resonance-imaging
#5
Lorenzo Caciagli, Fenglai Xiao, Britta Wandschneider, Matthias J Koepp
BACKGROUND: Approximately one third of patients with epilepsy are refractory to medical treatment. Adverse effects associated with anti-epileptic drugs (AEDs) are considered to affect quality of life often more than seizures themselves. Neuroimaging techniques, particularly magnetic resonance imaging (MRI), have proven instrumental in clinical decision making in relation to epilepsy surgery, but may also provide further insights into the mechanisms underlying treatment response and side effects associated with AEDs...
August 9, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28799142/what-to-do-in-failed-hemispherotomy-our-clinical-series-and-review-of-the-literature
#6
REVIEW
Andrea Bartoli, Y El Hassani, B Jenny, S Momjian, C M Korff, M Seeck, S Vulliemoz, K Schaller
Hemispherotomy is an established surgical technique to cure or palliate selected, mostly young patients suffering from refractory epilepsy. However, a few patients continue to have seizures despite the surgical hemispherical disconnection. We present a case series of patients who underwent redo hemispherotomy after a first unsuccessful hemispherical disconnection and provide a roadmap for subsequent workup and treatment. The institutional database of epilepsy surgery was reviewed. Twenty-four patients who underwent hemispherotomies for refractory epilepsy were identified between 2007 and 2016...
August 10, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28796326/gustatory-and-olfactory-responses-to-stimulation-of-the-human-insula
#7
Laure Mazzola, Jean-Pierre Royet, Hélène Catenoix, Alexandra Montavont, Jean Isnard, François Mauguière
OBJECTIVE: Despite numerous studies suggesting the role of insular cortex in the processing of gustatory and olfactory inputs, the exact location of olfacto-gustatory representation in the insula remains controversial. Here we provide a functional mapping of olfactory-gustatory responses to stimulation of the human insular cortex. METHODS: We reviewed 651 electrical stimulations of the insula that were performed in 221 patients, using stereotactically implanted depth electrodes, during the pre-surgical evaluation of drug refractory epilepsy...
August 10, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28794189/time-of-day-influences-on-respiratory-sequelae-following-maximal-electroshock-induced-seizures-in-mice
#8
Benton S Purnell, Michael A Hajek, Gordon F Buchanan
Sudden unexpected death in epilepsy (SUDEP) is the leading cause of death in refractory epilepsy patients. While specific mechanisms underlying SUDEP are not well understood, evidence suggests most SUDEP occurs due to seizure-induced respiratory arrest. SUDEP also tends to happen at night. While this may be due to circumstances humans find themselves in at night, such as being alone without supervision or sleeping prone, or due to independent influences of sleep state, there are a number of reasons why the night (i...
August 9, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28790971/recurrent-epileptic-auras-as-a-presenting-symptom-of-alzheimer-s-disease
#9
Rani A Sarkis, Kim C Willment, Seth A Gale, Barbara A Dworetzky
Seizures are a common co-morbidity during the course of Alzheimer's disease (AD) and in a subset of patients may be one of the presenting symptoms. In this case series, we highlight three patients with recurrent medically refractory epileptic auras whose work up ultimately lead to the diagnosis of AD. All three patients underwent prolonged EEG, serial neuropsychological testing, FDG-PET, cerebrospinal fluid (CSF) AD biomarkers, and MRI. CSF biomarkers were particularly helpful in two cases. These cases highlight the importance of having a high index of suspicion for AD in new onset "idiopathic" epilepsy in the elderly...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28790849/immunotargeting-relapsed-or-refractory-precursor-b-cell-acute-lymphoblastic-leukemia-role-of-blinatumomab
#10
REVIEW
Manon Queudeville, Rupert Handgretinger, Martin Ebinger
Patients with refractory or relapsed (R/R) acute lymphoblastic leukemia (ALL) have a dismal prognosis of around 5% long-term survival when treated with cytotoxic chemotherapy and allogenic stem cell transplantation. T-cell immunobased strategies open up new therapeutic perspectives. Blinatumomab is the first of a new class of antibody constructs that was labeled bispecific T-cell engager (BiTE): it consists of two single chain variable fragment connected with a flexible linker, one side binding CD3, the other CD19...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/28789990/descriptive-analysis-of-extracorporeal-cardiopulmonary-resuscitation-following-out-of-hospital-cardiac-arrest-an-elso-registry-study%C3%A2
#11
Nathan L Haas, Ryan A Coute, Cindy H Hsu, James A Cranford, Robert W Neumar
AIM: Extracorporeal cardiopulmonary resuscitation (ECPR) is an emerging therapy for refractory cardiac arrest. The purpose of this study was to analyze and report characteristics and outcomes of adult patients treated with ECPR after out-of-hospital cardiac arrest (OHCA) in a large international registry. METHODS: The Extracorporeal Life Support Organization's Extracorporeal Life Support Registry was queried for adult cardiac arrests with arrest location of "EMT Transport" or "Outside Hospital...
August 5, 2017: Resuscitation
https://www.readbyqxmd.com/read/28777058/causal-evidence-from-humans-for-the-role-of-mediodorsal-nucleus-of-the-thalamus-in-working-memory
#12
Jari Peräkylä, Lihua Sun, Kai Lehtimäki, Jukka Peltola, Juha Öhman, Timo Möttönen, Keith H Ogawa, Kaisa M Hartikainen
The mediodorsal nucleus of the thalamus (MD) with its extensive connections to the lateral pFC has been implicated in human working memory and executive functions. However, this understanding is based solely on indirect evidence from human lesion and imaging studies and animal studies. Direct, causal evidence from humans is missing. To obtain direct evidence for MD's role in humans, we studied patients treated with deep brain stimulation (DBS) for refractory epilepsy. This treatment is thought to prevent the generalization of a seizure by disrupting the functioning of the patient's anterior nuclei of the thalamus (ANT) with high-frequency electric stimulation...
August 4, 2017: Journal of Cognitive Neuroscience
https://www.readbyqxmd.com/read/28776279/asparagine-synthetase-deficiency-report-of-a-novel-mutation-and-review-of-literature
#13
Neerja Gupta, Vishal Vishnu Tewari, Manoj Kumar, Nitika Langeh, Aditi Gupta, Pallavi Mishra, Punit Kaur, Vedam Ramprasad, Sakthivel Murugan, Reema Kumar, Manisha Jana, Madhulika Kabra
Asparagine synthetase deficiency is a rare inborn error of metabolism caused by a defect in ASNS, a gene encoding asparagine synthetase. It manifests with a severe neurological phenotype manifesting as severe developmental delay, congenital microcephaly, spasticity and refractory seizures. To date, nineteen patients from twelve unrelated families have been identified. Majority of the mutations are missense and nonsense mutations in homozygous or compound heterozygous state. We add another case from India which harbored a novel homozygous missense variation in exon 11 and compare the current case with previously reported cases...
August 3, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28775328/inhibition-of-glutamate-decarboxylase-gad-by-ethyl-ketopentenoate-ekp-induces-treatment-resistant-epileptic-seizures-in-zebrafish
#14
Yifan Zhang, Michiel Vanmeert, Aleksandra Siekierska, Annelii Ny, Jubi John, Geert Callewaert, Eveline Lescrinier, Wim Dehaen, Peter A M de Witte, Rafal M Kaminski
Epilepsy is a chronic brain disorder characterized by recurrent seizures due to abnormal, excessive and synchronous neuronal activities in the brain. It affects approximately 65 million people worldwide, one third of which are still estimated to suffer from refractory seizures. Glutamic acid decarboxylase (GAD) that converts glutamate into GABA is a key enzyme in the dynamic regulation of neural network excitability. Importantly, clinical evidence shows that lowered GAD activity is associated with several forms of epilepsy which are often treatment resistant...
August 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28771662/neurotoxic-side-effects-in-children-with-refractory-or-relapsed-t-cell-malignancies-treated-with-nelarabine-based-therapy
#15
Michaela Kuhlen, Kirsten Bleckmann, Anja Möricke, Martin Schrappe, Simon Vieth, Gabriele Escherich, Annika Bronsema, Annika Vonalt, Manon Queudeville, C Michel Zwaan, Martin Ebinger, Klaus-Michael Debatin, Thomas Klingebiel, Ewa Koscielniak, Claudia Rossig, Birgit Burkhardt, Reinhard Kolb, Cornelia Eckert, Arndt Borkhardt, Arend von Stackelberg, Christiane Chen-Santel
The prognosis in children with refractory or relapsed (r/r) T-cell acute lymphoblastic leukaemia (T-ALL) or lymphoblastic lymphoma (T-LBL) is poor. Nelarabine (Ara-G) has successfully been used as salvage therapy in these children, but has been associated with significant, even fatal, neurotoxicities. We retrospectively analysed 52 patients with r/r T-ALL/T-LBL aged ≤19 years who were treated with Ara-G alone (n = 25) or in combination with cyclophosphamide and etoposide (n = 27). The majority of patients (45/52) received 1-2 cycles of Ara-G...
August 2, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28767715/lafora-disease-in-miniature-wirehaired-dachshunds
#16
Lindsay Swain, Gill Key, Anna Tauro, Saija Ahonen, Peixiang Wang, Cameron Ackerley, Berge A Minassian, Clare Rusbridge
Lafora disease (LD) is an autosomal recessive late onset, progressive myoclonic epilepsy with a high prevalence in the miniature Wirehaired Dachshund. The disease is due to a mutation in the Epm2b gene which results in intracellular accumulation of abnormal glycogen (Lafora bodies). Recent breed-wide testing suggests that the carrier plus affected rate may be as high as 20%. A characteristic feature of the disease is spontaneous and reflex myoclonus; however clinical signs and disease progression are not well described...
2017: PloS One
https://www.readbyqxmd.com/read/28766701/rationale-for-an-adjunctive-therapy-with-fenofibrate-in-pharmacoresistant-nocturnal-frontal-lobe-epilepsy
#17
Monica Puligheddu, Miriam Melis, Giuliano Pillolla, Giulia Milioli, Liborio Parrino, Giovanni Mario Terzano, Sonia Aroni, Claudia Sagheddu, Francesco Marrosu, Marco Pistis, Anna Lisa Muntoni
OBJECTIVE: Nocturnal frontal lobe epilepsy (NFLE) is an idiopathic partial epilepsy with a family history in about 25% of cases, with autosomal dominant inheritance (autosomal dominant NFLE [ADNFLE]). Traditional antiepileptic drugs are effective in about 55% of patients, whereas the rest remains refractory. One of the key pathogenetic mechanisms is a gain of function of neuronal nicotinic acetylcholine receptors (nAChRs) containing the mutated α4 or β2 subunits. Fenofibrate, a common lipid-regulating drug, is an agonist at peroxisome proliferator-activated receptor alpha (PPARα) that is a ligand-activated transcription factor, which negatively modulates the function of β2-containing nAChR...
August 2, 2017: Epilepsia
https://www.readbyqxmd.com/read/28761628/modified-atkins-diet-in-adult-with-refractory-epilepsy-a-controlled-randomized-clinical-trial
#18
Mohammad Zare, Ali Asghar Okhovat, Ahmad Esmaillzadeh, Jafar Mehvari, Mohammad Reza Najafi, Mohammad Saadatnia
Background: The usefulness of the modified Atkins diet (mAD) in refractory epilepsy in adults has been rarely investigated. We aimed to evaluate the efficacy of mAD in adult with refractory epilepsy. Methods: In a controlled randomized clinical trial, we enrolled 66 refractory adult epileptic cases from February 2010 to December 2012. The patients were randomly divided into two groups, case groups (22 patients) used antiepileptic drugs and mAD and control group (32 patients) only use antiepileptic drugs. The primary outcome was at least 50% decrement in seizure frequency after 2 months of therapy...
April 4, 2017: Iranian Journal of Neurology
https://www.readbyqxmd.com/read/28758966/variability-of-creatine-metabolism-genes-in-children-with-autism-spectrum-disorder
#19
Jessie M Cameron, Valeriy Levandovskiy, Wendy Roberts, Evdokia Anagnostou, Stephen Scherer, Alvin Loh, Andreas Schulze
Creatine deficiency syndrome (CDS) comprises three separate enzyme deficiencies with overlapping clinical presentations: arginine:glycine amidinotransferase (GATM gene, glycine amidinotransferase), guanidinoacetate methyltransferase (GAMT gene), and creatine transporter deficiency (SLC6A8 gene, solute carrier family 6 member 8). CDS presents with developmental delays/regression, intellectual disability, speech and language impairment, autistic behaviour, epileptic seizures, treatment-refractory epilepsy, and extrapyramidal movement disorders; symptoms that are also evident in children with autism...
July 31, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28752473/safety-profile-of-eslicarbazepine-acetate-as-add-on-therapy-in-adults-with-refractory-focal-onset-seizures-from-clinical-studies-to-6-years-of-post-marketing-experience
#20
Helena Gama, Mariana Vieira, Raquel Costa, Joana Graça, Luís M Magalhães, Patrício Soares-da-Silva
INTRODUCTION: Eslicarbazepine acetate was first approved in the European Union in 2009 as adjunctive therapy in adults with partial-onset seizures with or without secondary generalization. OBJECTIVE: The objective of this study was to review the safety profile of eslicarbazepine acetate analyzing the data from several clinical studies to 6 years of post-marketing surveillance. METHODS: We used a post-hoc pooled safety analysis of four phase III, double-blind, randomized, placebo-controlled studies (BIA-2093-301, -302, -303, -304) of eslicarbazepine acetate as add-on therapy in adults...
July 27, 2017: Drug Safety: An International Journal of Medical Toxicology and Drug Experience
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