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Status epilepticus in children

Paresh Zanzmera, Ramshekhar N Menon, Kalyani Karkare, Himanshu Soni, Sujit Jagtap, Ashalatha Radhakrishnan
PURPOSE: The purpose of this article was to study the electroclinical characteristics and seizure outcome of children with epilepsy with myoclonic absences (EMA). METHOD: In this descriptive cohort study, we reviewed clinical records of patients who met the criteria for EMA. Each patient's demographic data, birth/developmental history, seizure semiology/pattern, antiepileptic drugs (AED), clinical examination, video-electroencephalography (VEEG), and neuroimaging data were reviewed...
October 19, 2016: Epilepsy & Behavior: E&B
Sarah Aminoff Kelley, Eric Heath Kossoff
Electrical status epilepticus of sleep (ESES), with the activation of profuse amounts of epileptiform discharges in sleep, may lead to intractable epilepsy and neurocognitive decline in children. Numerous varied treatments including antiseizure medications, steroids, and surgery have been investigated as possible treatment options. The ketogenic diet (KD) is an additional treatment option which may add to our treatment armamentarium for ESES. The KD may theoretically improve ESES by affecting GABA systems and reducing inflammation...
September 23, 2016: Epilepsy Research
Kimberly Statler Bennett
No abstract text is available yet for this article.
October 2016: Pediatric Critical Care Medicine
S Boronat, M Vicente, E Lainez, A Sánchez-Montañez, E Vázquez, L Mangado, L Martínez-Ribot, M Del Campo
Fetal alcohol spectrum disorders (FASD) cause neurodevelopmental abnormalities. However, publications about epilepsy and electroencephalographic features are scarce. In this study, we prospectively performed electroencephalography (EEG) and brain magnetic resonance (MR) imaging in 61 patients with diagnosis of FASD. One patient had multiple febrile seizures with normal EEGs. Fourteen children showed EEG anomalies, including slow background activity and interictal epileptiform discharges, focal and/or generalized, and 3 of them had epilepsy...
September 13, 2016: European Journal of Medical Genetics
A C McClelland, W A Gomes, S Shinnar, D C Hesdorffer, E Bagiella, D V Lewis, J A Bello, S Chan, J MacFall, M Chen, J M Pellock, D R Nordli, L M Frank, S L Moshé, R C Shinnar, S Sun
BACKGROUND AND PURPOSE: The pathogenesis of febrile status epilepticus is poorly understood, but prior studies have suggested an association with temporal lobe abnormalities, including hippocampal malrotation. We used a quantitative morphometric method to assess the association between temporal lobe morphology and febrile status epilepticus. MATERIALS AND METHODS: Brain MR imaging was performed in children presenting with febrile status epilepticus and control subjects as part of the Consequences of Prolonged Febrile Seizures in Childhood study...
September 15, 2016: AJNR. American Journal of Neuroradiology
Qi Huang, Yuan Wu, Rongfa Qin, Xing Wei, Meigang Ma
Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an acute neurological disorder affecting children and adults. We aimed to compare the clinical characteristics, treatments, and outcomes between children and adults with anti-NMDAR encephalitis and to assess the probable risk factors. In this observational study, patients who tested positive for anti-NMDAR antibody in the cerebrospinal fluid were enrolled. The patients were divided into children and adults group on the basis of age (whether <16 or not)...
September 15, 2016: Journal 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
Hideaki Kanemura, Fumikazu Sano, Tetsuo Ohyama, Kanji Sugita, Masao Aihara
AIMS: To develop and implement interventions to improve the quality of life (QOL) in children with epilepsy, it is important for clinicians and researchers to understand the effects of the children's parents' perception of stigma. The purpose of this study was to identify a relationship between patient clinical characteristics and perception of stigma in the parents of children with epilepsy. METHODS: Parents of children with epilepsy were recruited from our university hospital between April 1, 2005 and March 31, 2012...
October 2016: Epilepsy & Behavior: E&B
Gudrun Gröppel, Christian Dorfer, Anastasia Dressler, Angelika Mühlebner, Barbara Porsche, Johann A Hainfellner, Thomas Czech, Martha Feucht
AIM: To study the effect of hemispherotomy on electrical status epilepticus in sleep (ESES) and language development. METHOD: Children with a confirmed diagnosis of ESES prior to surgery and a minimum of 24 months of developmental follow-up data were compared with age-matched controls without ESES. Language quotients (LQs) were calculated before and after surgery. RESULTS: Eleven patients (five females, six males) and 21 controls (11 females, 10 males) were included...
August 25, 2016: Developmental Medicine and Child Neurology
Jan Henje Döring, Anette Lampert, Georg F Hoffmann, Markus Ries
BACKGROUND: Epilepsy is a serious chronic health condition with a high morbidity impairing the life of patients and afflicted families. Many epileptic conditions, especially those affecting children, are rare disorders generating an urgent medical need for more efficacious therapy options. Therefore, we assessed the output of the US and European orphan drug legislations. METHODS: Quantitative analysis of the FDA and EMA databases for orphan drug designations according to STrengthening the Reporting of OBservational studies in Epidemiology (STROBE) criteria...
2016: PloS One
Raquel Farias-Moeller, Luca Bartolini, Archana Pasupuleti, R D Brittany Cines, Amy Kao, Jessica L Carpenter
BACKGROUND: Super-refractory status epilepticus (SRSE) ensues when there is no improvement of seizure control in response to anesthetic therapy or seizure recurrence after reduction of anesthetic agents. There is no consensus on standard of care for SRSE. Ketogenic diet (KD) has reported success, but technical challenges exist including inability to feed patients, concomitant steroid use, acidotic states, and lack of dieticians with experience. The optimal protocol for KD is yet to be determined...
August 23, 2016: Neurocritical Care
Sebastián Sánchez, Fred Rincon
Status epilepticus (SE) is defined as a continuous clinical and/or electrographic seizure activity lasting five minutes or more or recurrent seizure activity without return to baseline. There is a paucity of epidemiological studies of SE, as most research is derived from small population studies. The overall incidence of SE is 9.9 to 41 per 100,000/year, with peaks in children and the elderly and with febrile seizures and strokes as its main etiologies. The etiology is the major determinant of mortality. Governments and the academic community should predominantly focus on the primary prevention of etiologies linked to SE, as these are the most important risk factors for its development...
August 16, 2016: Journal of Clinical Medicine
Kathleen M Gorman, Amre Shahwan
Sultiame is recommended for the treatment of benign epilepsy of childhood with centrotemporal spikes, electrical status epilepticus during slow-wave sleep, as well as other genetic (idiopathic) focal epilepsies. Sultiame is not traditionally considered a treatment choice for idiopathic generalised epilepsy, and it does not appear on the list of drugs recommended for treatment of absence seizures. We report the efficacy of sultiame in treating three children with drug-resistant absence seizures and discuss the potential use of sultiame beyond the idiopathic focal epilepsies...
September 1, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
Robert C Tasker, Howard P Goodkin, Iván Sánchez Fernández, Kevin E Chapman, Nicholas S Abend, Ravindra Arya, James N Brenton, Jessica L Carpenter, William D Gaillard, Tracy A Glauser, Joshua Goldstein, Ashley R Helseth, Michele C Jackson, Kush Kapur, Mohamad A Mikati, Katrina Peariso, Mark S Wainwright, Angus A Wilfong, Korwyn Williams, Tobias Loddenkemper
OBJECTIVE: To describe pediatric patients with convulsive refractory status epilepticus in whom there is intention to use an IV anesthetic for seizure control. DESIGN: Two-year prospective observational study evaluating patients (age range, 1 mo to 21 yr) with refractory status epilepticus not responding to two antiepileptic drug classes and treated with continuous infusion of anesthetic agent. SETTING: Nine pediatric hospitals in the United States...
October 2016: Pediatric Critical Care Medicine
Brian Appavu, Lisa Vanatta, John Condie, John F Kerrigan, Randa Jarrar
PURPOSE: We aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm. METHOD: A retrospective review was performed at Phoenix Children's Hospital on patients with super-refractory status epilepticus undergoing ketogenic diet therapy from 2011 to 2015. RESULTS: Ten children with super-refractory status epilepticus, ages 2-16 years, were identified...
October 2016: Seizure: the Journal of the British Epilepsy Association
Alicia Mirás Veiga, David Conejo Moreno, Ana Isabel Gómez Menéndez, Ignacio Muñoz Siscart, Marta Del Olmo Fernández, Esteban Gómez Sánchez, María García González, Fernando Gómez Sáez
Febrile infection-related epilepsy syndrome (FIRES) is a rare condition which evolves into refractory status epilepticus (SE), with poor outcome in most cases. Conventional antiepileptic drugs fail to control SE in FIRES patients. We report the case of a previously healthy 4-year-old boy who was diagnosed with FIRES. One week after pharyngitis and high fever he started seizures, followed by refractory SE. Benzodiazepines, phenytoin, high-dose barbiturates that induce burst suppression, high doses of corticosteroids, plasmapheresis, immunoglobulins, propofol, lidocaine, ketamine, inhaled desflurane, ketogenic diet, lacosamide, and therapeutic hypothermia were tried at different times in a period of 8 weeks, but all of them were ineffective...
July 19, 2016: Neuropediatrics
Melissa J Maguire, Cerian F Jackson, Anthony G Marson, Sarah J Nolan
BACKGROUND: Sudden Unexpected Death in Epilepsy (SUDEP) is defined as sudden, unexpected, witnessed or unwitnessed, non-traumatic or non-drowning death of people with epilepsy, with or without evidence of a seizure, excluding documented status epilepticus and in whom postmortem examination does not reveal a structural or toxicological cause for death. SUDEP has a reported incidence of 1 to 2 per 1000 patient years and represents the most common epilepsy-related cause of death. The presence and frequency of generalised tonic-clonic seizures (GTCS), male sex, early age of seizure onset, duration of epilepsy, and polytherapy are all predictors of risk of SUDEP...
July 19, 2016: Cochrane Database of Systematic Reviews
Fenella Jane Kirkham
Pediatric neurology relies on ultrasound, computed tomography (CT), and magnetic resonance (MR) imaging. CT prevails in acute neurologic presentations, including traumatic brain injury (TBI), nontraumatic coma, stroke, and status epilepticus, because of easy availability, with images of diagnostic quality, e.g., to exclude hemorrhage, usually completed quickly enough to avoid sedation. Concerns over the risks of ionizing radiation mean re-imaging and higher-dose procedures, e.g., arteriography and venography, require justification...
2016: Handbook of Clinical Neurology
Yen-Ping Chen, Hsiu-Fen Lee, Tai-Tong Wong
PURPOSE: The aim of this study is to describe epileptic seizures in patients with primary intracranial sarcomas. METHODS: We report a 17-year-old girl diagnosed with primary high-grade intracranial sarcoma with initial clinical manifestation of nonconvulsive status epilepticus. Literature reports between 2000 and 2014, relevant to primary intracranial sarcomas in children, were reviewed. The clinical presentations and neurological outcomes were analyzed. RESULTS: Eleven of 29cases (38 %), 8 males and 3 females, who exhibited epileptic seizures as one of the initial symptoms and diagnosed with primary intracranial sarcomas were collected...
September 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Simone M Dustin, Carl E Stafstrom
BACKGROUND AND PURPOSE: The high-fat, low-carbohydrate ketogenic diet (KD) is effective in many cases of drug-resistant epilepsy, particularly in children. In the classic KD, fats consist primarily of long-chain saturated triglycerides. Polyunsaturated fatty acids (PUFAs), especially the n-3 type, decrease neuronal excitability and provide neuroprotection; pilot human studies have raised the possibility of using PUFAs to control seizures in patients. METHODS: To determine the relative roles of the KD and PUFAs in an animal model, we induced epilepsy in juvenile rats (P29-35) using intraperitoneal kainic acid (KA)...
June 2016: Journal of Epilepsy Research
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