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epileptic status refractory

Poul Jennum, Anne Sabers, Jakob Christensen, Rikke Ibsen, Jakob Kjellberg
PURPOSE: Epilepsy surgery has been a standard treatment for refractory epilepsies that cannot be controlled by standard medical treatment. We aimed to evaluate the health and social consequences of resective surgery relative to controls from a study of national data. METHODS: Using the Danish National Patient Registry we identified all subjects with an epilepsy diagnosis between 1996 and 2009 and compared them with a group of patients with an epilepsy diagnosis who had had neither epilepsy surgery nor a vagus stimulation diagnosis by the index date, and who were matched by gender, index year for epilepsy diagnosis, and index year for epilepsy surgery...
October 6, 2016: Seizure: the Journal of the British Epilepsy Association
Daniel L Kenney-Jung, Annamaria Vezzani, Robert J Kahoud, Reghann G LaFrance-Corey, Mai-Lan Ho, Theresa Wampler Muskardin, Elaine C Wirrell, Charles L Howe, Eric T Payne
Febrile infection-related epilepsy syndrome (FIRES) is a devastating epileptic encephalopathy with limited treatment options and an unclear etiology. Anakinra is a recombinant version of the human interleukin-1 receptor antagonist used to treat autoinflammatory disorders. This is the first report of anakinra for treatment of a child with super-refractory status epilepticus secondary to FIRES. Anakinra was well-tolerated and effective. Cerebral spinal fluid analysis revealed elevated levels of proinflammatory cytokines before treatment that normalized on anakinra, suggesting a potential pathogenic role for neuroinflammation in FIRES...
October 22, 2016: Annals of Neurology
Dong Li, Hongjie Yuan, Xilma R Ortiz-Gonzalez, Eric D Marsh, Lifeng Tian, Elizabeth M McCormick, Gabrielle J Kosobucki, Wenjuan Chen, Anthony J Schulien, Rosetta Chiavacci, Anel Tankovic, Claudia Naase, Frieder Brueckner, Celina von Stülpnagel-Steinbeis, Chun Hu, Hirofumi Kusumoto, Ulrike B S Hedrich, Gina Elsen, Konstanze Hörtnagel, Elias Aizenman, Johannes R Lemke, Hakon Hakonarson, Stephen F Traynelis, Marni J Falk
N-methyl-D-aspartate receptors (NMDARs) are ligand-gated cation channels that mediate excitatory synaptic transmission. Genetic mutations in multiple NMDAR subunits cause various childhood epilepsy syndromes. Here, we report a de novo recurrent heterozygous missense mutation-c.1999G>A (p.Val667Ile)-in a NMDAR gene previously unrecognized to harbor disease-causing mutations, GRIN2D, identified by exome and candidate panel sequencing in two unrelated children with epileptic encephalopathy. The resulting GluN2D p...
October 6, 2016: American Journal of Human Genetics
Ludovica Pasca, Valentina De Giorgis, Joyce Ann Macasaet, Claudia Trentani, Anna Tagliabue, Pierangelo Veggiotti
UNLABELLED: Ketogenic diet is an established and effective non-pharmacologic treatment for drug-resistant epilepsy. Ketogenic diet represents the treatment of choice for GLUT-1 deficiency syndrome and pyruvate dehydrogenase complex deficiency. Infantile spasms, Dravet syndrome and myoclonic-astatic epilepsy are epilepsy syndromes for which ketogenic diet should be considered early in the therapeutic pathway. Recently, clinical indications for ketogenic diet have been increasing, as there is emerging evidence regarding safety and effectiveness...
October 2016: European Journal of Pediatrics
Jamil M Beg, Thomas D Anderson, Kevin Francis, Lisa M Meckley, David Fitzhenry, Todd Foster, Susheel Sukhtankar, Stephen J Kanes, Lidia M V R Moura
OBJECTIVE: To provide an estimate of the annual number of super-refractory status epilepticus (SRSE) cases in the US and to evaluate utilization of hospital resources by these patients. METHODS: The Premier Hospital Database was utilized to estimate the number of SRSE cases based on hospital discharges during 2012. Discharges were classified as SRSE cases based on an algorithm using seizure-related International Classification of Diseases-9 (ICD-9) codes, Intensive Care Unit (ICU) length of stay (LOS), and treatment protocols (e...
August 11, 2016: Journal of Medical Economics
Marc Zanello, Mélanie Pagès, Alexandre Roux, Sophie Peeters, Edouard Dezamis, Stéphanie Puget, Bertrand Devaux, Christian Sainte-Rose, Michel Zerah, Guillaume Louvel, Sarah N Dumont, Jean-François Meder, Jacques Grill, Gilles Huberfeld, Fabrice Chrétien, Eduardo Parraga, Xavier Sauvageon, Pascale Varlet, Johan Pallud
AIM: Prevalence and predictors of epileptic seizures are unknown in the malignant variant of ganglioglioma. METHODS: In a retrospective exploratory dataset of 18 supratentorial anaplastic World Health Organization grade III gangliogliomas, we studied: (i) the prevalence and predictors of epileptic seizures at diagnosis; (ii) the evolution of seizures during tumor evolution; (iii) seizure control rates and predictors of epilepsy control after oncological treatments...
August 23, 2016: British Journal of Neurosurgery
Layla E Borham, Amal M Mahfoz, Ibrahim A A Ibrahim, Naiyer Shahzad, Abeer Ahmed ALrefai, Amira A Labib, Bassam Bin Sef, Abdulrahmman Alshareef, Meshal Khan, Ali Milibary, Saeed Al Ghamdi
Evidence shows that inflammatory and immune processes within the brain might account for the pathophysiology of epilepsy. Therefore, developing new antiepileptic drugs that can modulate seizures through mechanisms other than traditional drugs is required for the treatment of refractory epilepsy. This study aims to determine the relationship between brain inflammation and epilepsy, to examine the contribution of some biochemical parameters involved in brain inflammation, and to address the effect of pharmacological interventions using some anti-inflammatory and immunomodulatory drugs in an experimental epilepsy model...
October 1, 2016: Brain Research
Rushna Ali
Cardiac rhythm abnormalities in the context of epilepsy are a well-known phenomenon. However, they are under-recognized and often missed. The pathophysiology of these events is unclear. Bradycardia and asystole are preceded by seizure onset suggesting ictal propagation into the cortex impacting cardiac autonomic function, and the insula and amygdala being possible culprits. Sudden unexpected death in epilepsy (SUDEP) refers to the unanticipated death of a patient with epilepsy not related to status epilepticus, trauma, drowning, or suicide...
2016: Open Cardiovascular Medicine Journal
Pavel Mareš, Hana Kubová
Possible changes of cortical excitability after status epilepticus (SE) elicited in 12-day-old rats were studied by means of paired cortical afterdischarges (ADs). Consequences of lithium-pilocarpine status were studied in animals with implanted electrodes 3, 6, 9, 13, and 26 days after SE. Paired low-frequency stimulation with a 1-min interval was repeated after 10 min, and duration of ADs was measured. Control rats received saline instead of pilocarpine; other treatments were the same as in SE group. Postictal refractoriness (i...
August 2016: Epilepsia
Sandeep B Patil, Arun Grace Roy, Kollencheri Puthenveettil Vinayan
PURPOSE: To describe the clinical features and outcome of febrile infection-related epilepsy syndrome (FIRES), a catastrophic epileptic encephalopathy, in a cohort of South Indian children. MATERIALS AND METHODS: We performed a retrospective chart review of a cohort of children with previously normal development who presented with status epilepticus or encephalopathy with recurrent seizures following a nonspecific febrile illness during the period between January 2007 and January 2012...
April 2016: Annals of Indian Academy of Neurology
Ida A Nissen, Nicole E C van Klink, Maeike Zijlmans, Cornelis J Stam, Arjan Hillebrand
OBJECTIVE: Previous studies have associated network hubs and epileptiform activity, such as spikes and high frequency oscillations (HFOs), with the epileptogenic zone. The epileptogenic zone is approximated by the area that generates interictal epileptiform activity: the irritative zone. Our aim was to determine the relation between network hubs and the irritative zone. METHODS: Interictal resting-state MEG recordings of 12 patients with refractory epilepsy were analysed...
July 2016: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Pius S Fasinu, Sarah Phillips, Mahmoud A ElSohly, Larry A Walker
States and the federal government are under growing pressure to legalize the use of cannabis products for medical purposes in the United States. Sixteen states have legalized (or decriminalized possession of) products high in cannabidiol (CBD) and with restricted ∆(9) -tetrahydrocannabinol (∆(9) -THC) content. In most of these states, the intent is for use in refractory epileptic seizures in children, but in a few states, the indications are broader. This review provides an overview of the pharmacology and toxicology of CBD; summarizes some of the regulatory, safety, and cultural issues relevant to the further exploitation of its antiepileptic or other pharmacologic activities; and assesses the current status and prospects for clinical development of CBD and CBD-rich preparations for medical use in the United States...
July 2016: Pharmacotherapy
Carmen Barba, Francesca Darra, Raffaella Cusmai, Elena Procopio, Carlo Dionisi Vici, Liesbeth Keldermans, Sandrine Vuillaumier-Barrot, Dirk J Lefeber, Renzo Guerrini
AIM: Epilepsy is commonly observed in congenital disorders of glycosylation (CDG), but no distinctive electroclinical pattern has been recognized. We aimed at identifying a characteristic clinical presentation that might help targeted diagnostic work-up. METHOD: Based on the initial observation of an index case with CDG and migrating partial seizures, we evaluated 16 additional children with CDG and analysed their clinical course, biochemical, genetic, electrographic, and imaging findings...
October 2016: Developmental Medicine and Child Neurology
Jill A Fahrner, Raymond Liu, Michael Scott Perry, Jessica Klein, David C Chan
DNM1L encodes dynamin-related protein 1 (DRP1/DLP1), a key component of the mitochondrial fission machinery that is essential for proper functioning of the mammalian brain. Previously reported probands with de novo missense mutations in DNM1L presented in the first year of life with severe encephalopathy and refractory epilepsy, with several dying within the first several weeks after birth. In contrast, we report identical novel missense mutations in DNM1L in two unrelated probands who experienced normal development for several years before presenting with refractory focal status epilepticus and subsequent rapid neurological decline...
August 2016: American Journal of Medical Genetics. Part A
Robertino Dilena, Gabriella Nebbia, Lorenzo Fiorica, Marcello Farallo, Irene Degrassi, Francesca Gozzo, Veronica Pelliccia, Sergio Barbieri, Massimo Cossu, Laura Tassi
INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) with status epilepticus may occur after liver transplant. This may rarely lead to refractory epilepsy and hippocampal sclerosis (HS). CASE DESCRIPTION: We report the first case of epilepsy surgery in a liver-transplanted patient with refractory temporal lobe epilepsy. A 3-year-old girl underwent liver transplant for congenital biliary atresia. Four days after transplant she manifested PRES with status epilepticus, but she recovered within a couple of weeks...
July 2016: European Journal of Paediatric Neurology: EJPN
Jafar Mehvari, Fataneh Gholami Motlagh, Mohamad Najafi, Mohammad Reza Aghaye Ghazvini, Amirmansour Alavi Naeini, Mohamad Zare
BACKGROUND: Oxidative stress has been a frequent finding in epileptic patients receiving antiepileptic drugs (AEDs). In this study, the influence of Vitamin E on the antiseizure activity and redox state of patients treated with carbamazepine, sodium valproate, and levetiracetam has been investigated. MATERIALS AND METHODS: This double-blind, placebo-controlled trial was carried out on 65 epileptic patients with chronic antiepileptic intake. The subjects received 400 IU/day of Vitamin E or placebo for 6 months...
2016: Advanced Biomedical Research
M Vinjam, R Newby, R Davey
A 22-year-old female migraineur presented with recurrent convulsive status epilepticus and ataxia. Her epilepsy proved refractory to treatment, necessitating the use of five anti-epileptic drugs and a course of steroids. Genetic testing revealed compound heterozygosity for two mutations of the polymerase-? gene. The case highlights the clinical features and therapeutic challenges associated with this relatively common, but probably underrecognised, mitochondrial disease.
2015: Journal of the Royal College of Physicians of Edinburgh
Yoshiko Hirano, Hirokazu Oguni, Satoru Nagata
Ring chromosome 20 [r(20)] syndrome is a rare chromosomal disorder that is characterized by the development of refractory epilepsy during childhood with gradual declines in cognitive performance and behavior. Although the prognoses of seizures and intellectual disability associated with this condition are poor, life-threatening complications have rarely been described. We herein presented a case of a 17-year-old female with [r(20)] syndrome who developed recurrent status epilepticus (SE) at 14years of age that evolved into unremitting SE in spite of vigorous antiepileptic treatments...
September 2016: Brain & Development
Adam Wallace, Elaine Wirrell, Daniel L Kenney-Jung
Dravet syndrome (DS) is an intractable pediatric epilepsy syndrome, starting in early childhood. This disorder typically manifests with febrile status epilepticus, and progresses to a multifocal epilepsy with febrile and non-febrile seizures with encephalopathy. Most cases are due to a mutation in the SCN1A gene. This article reviews treatments for DS, with an emphasis on pharmacotherapy. While many medications are used in treating the seizures associated with DS, these patients typically have medically refractory epilepsy, and polytherapy is often required...
June 2016: Paediatric Drugs
Eric H Grover, Yara Nazzal, Lawrence J Hirsch
Convulsive status epilepticus (CSE) is a medical emergency with an associated high mortality and morbidity. It is defined as a convulsive seizure lasting more than 5 min or consecutive seizures without recovery of consciousness. Successful management of CSE depends on rapid administration of adequate doses of anti-epileptic drugs (AEDs). The exact choice of AED is less important than rapid treatment and early consideration of reversible etiologies. Current guidelines recommend the use of benzodiazepines (BNZ) as first-line treatment in CSE...
March 2016: Current Treatment Options in Neurology
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