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https://www.readbyqxmd.com/read/28926844/atypical-presentation-of-a-pediatric-cerebellar-ganglioglioma
#1
Richard Bram, Roberta J Seidman, David Chesler
BACKGROUND/AIMS: Gangliogliomas (GGs) are rare central nervous system tumors occurring primarily in the supratentorial compartment with infratentorial instances most often involving the brain stem. Infratentorial GGs typically present with signs and symptoms of increased intracranial pressure (ICP), cranial nerve deficits, or focal cerebellar findings; rarely, these tumors have been associated with focal seizures. METHODS: In this report, we describe an atypical presentation of a cerebellar GG in a 20-month-old male who initially presented with syncope and emesis in the absence of electrographic evidence of seizures, radiographic evidence of hydrocephalus, or elevated ICP...
September 20, 2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28922267/the-1-year-follow-up-clinic-for-neonates-and-children-after-respiratory-extracorporeal-membrane-oxygenation-support-a-10-year-single-institution-experience
#2
Suzan Kakat, Maura O'Callaghan, Liz Smith, Raymond Hreiche, Deborah A Ridout, Jo Wray, Timothy Thiruchelvam, Katherine L Brown, Aparna U Hoskote
OBJECTIVES: To establish the effectiveness of a "1-year extracorporeal membrane oxygenation follow-up clinic" and to characterize any neurodevelopmental concerns identified. DESIGN: Single-center retrospective cohort of respiratory extracorporeal membrane oxygenation survivors over 10 years. SETTING: Nationally commissioned center for neonatal and pediatric (> 28 d of life) respiratory extracorporeal membrane oxygenation. PATIENTS: Children attending the follow-up clinic 1 year after receiving respiratory extracorporeal membrane oxygenation between 2003 and 2013...
September 15, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28914984/hypercalcemic-disorders-in-children
#3
REVIEW
Victoria J Stokes, Morten F Nielsen, Fadil M Hannan, Rajesh V Thakker
Hypercalcemia is defined as a serum calcium concentration that is greater than 2 standard deviations above the normal mean, which in children may vary with age and sex, reflecting changes in the normal physiology at each developmental stage. Hypercalcemic disorders in children may present with hypotonia, poor feeding, vomiting, constipation, abdominal pain, lethargy, polyuria, dehydration, failure to thrive and seizures. In severe cases renal failure, pancreatitis and reduced consciousness may also occur and older children and adolescents may present with psychiatric symptoms...
September 15, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28914199/clinical-applications-of-intravenous-immunoglobulins-in-child-neurology
#4
Maria Gogou, Efimia Papadopoulou-Alataki, Martha Spilioti, Sofia Alataki, Athanasios Evangeliou
BACKGROUND: While there are guidelines for the use of intravenous immunoglobulins in children with Guillain-Barre syndrome and myasthenia gravis based on high-level evidence studies, data is fewer for the majority of neurologic disorders in this age group. Neuronal antibodies are detected in children with seizures of autoimmune etiology. Intravenous immunoglobulins with their broad immunomodulatory mechanism of action could be ideally effective in different forms of immune-dysregulated intractable epilepsies such as autoimmune epilepsy and autoimmune Rasmussen encephalitis...
September 15, 2017: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/28904568/pediatric-autoimmune-encephalitis
#5
REVIEW
Massimo Barbagallo, Giovanna Vitaliti, Piero Pavone, Catia Romano, Riccardo Lubrano, Raffaele Falsaperla
Autoimmune (antibody mediated) encephalitis (AE) is emerging as a more common cause of pediatric encephalopathy than previously thought. The autoimmune process may be triggered by an infection, vaccine, or occult neoplasm. In the latter case, onconeural autoantibodies are directed against intracellular neuronal antigens, but a recent heterogeneous group of encephalitic syndromes has been found not to have underlying tumor but is associated with autoantibodies to the neuronal surface or synaptic antigens. Neuropsychiatric symptoms are very common in autoimmune encephalopathy; as a result, affected children may be initially present to psychiatrists...
April 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28901536/validation-of-the-pedsql-epilepsy-module-a-pediatric-epilepsy-specific-health-related-quality-of-life-measure
#6
Avani C Modi, Katherine F Junger, Constance A Mara, Tanja Kellermann, Lauren Barrett, Janelle Wagner, Grace A Mucci, Laurie Bailey, Dace Almane, Shanna M Guilfoyle, Lauryn Urso, Brooke Hater, Heather Hustzi, Gigi Smith, Bruce Herrmann, M Scott Perry, Mary Zupanc, James W Varni
OBJECTIVE: To validate a brief and reliable epilepsy-specific, health-related quality of life (HRQOL) measure in children with various seizure types, treatments, and demographic characteristics. METHODS: This national validation study was conducted across five epilepsy centers in the United States. Youth 5-18 years and caregivers of youth 2-18 years diagnosed with epilepsy completed the PedsQL Epilepsy Module and additional questionnaires to establish reliability and validity of the epilepsy-specific HRQOL instrument...
September 13, 2017: Epilepsia
https://www.readbyqxmd.com/read/28892547/non-rapid-eye-movement-arousal-parasomnias-in-children
#7
Vijayabharathi Ekambaram, Kiran Maski
Parasomnia is a common pediatric sleep disorder that can cause parents or caregivers distress when experienced by their children. Based on the International Classification of Sleep Disorders, parasomnias can be divided into two subgroups: non-rapid eye movement (NREM) parasomnias and rapid eye movement (REM) parasomnias. REM sleep parasomnias include nightmares, REM behavior disorder, and sleep paralysis, whereas NREM sleep parasomnias include disorders of arousal such as confusional arousals, sleepwalking, sleep talking, night terrors, and sleep-related eating disorder...
September 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28888970/glial-overexpression-of-dube3a-causes-seizures-and-synaptic-impairments-in-drosophila-concomitant-with-down-regulation-of-the-na-k-pump-atp%C3%AE
#8
Kevin A Hope, Mark S LeDoux, Lawrence T Reiter
Duplication 15q syndrome (Dup15q) is an autism-associated disorder co-incident with high rates of pediatric epilepsy. Additional copies of the E3 ubiquitin ligase UBE3A are thought to cause Dup15q phenotypes, yet models overexpressing UBE3A in neurons have not recapitulated the epilepsy phenotype. We show that Drosophila endogenously expresses Dube3a (fly UBE3A homolog) in glial cells and neurons, prompting an investigation into the consequences of glial Dube3a overexpression. Here we expand on previous work showing that the Na(+)/K(+) pump ATPα is a direct ubiquitin ligase substrate of Dube3a...
September 6, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28884437/extrapolation-of-a-brivaracetam-exposure-response-model-from-adults-to-children-with-focal-seizures
#9
Rik Schoemaker, Janet R Wade, Armel Stockis
INTRODUCTION: Prediction of brivaracetam effects in children was obtained by scaling an existing adult pharmacokinetic/pharmacodynamic (PK/PD) model for brivaracetam to children, using an existing population PK model for brivaracetam in children. The scaling was supported by estimating the change from adults to children in the concentration-effect relationship parameters for levetiracetam, a compound interacting with the same target protein (synaptic vesicle protein SV2A). METHODS: The existing adult PK/PD model for brivaracetam was applied to a combined adult-pediatric dataset of levetiracetam...
September 7, 2017: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/28883872/therapeutic-effects-of-adrenocorticotropic-hormone-acth-in-children-with-severely-intractable-seizure
#10
Jafar Nasiri, Azam Sarajan, Mehri Salari, Maryam Sedghi
OBJECTIVE: Treatment of intractable seizures other than spasms is difficult and controversial. There are few studies on efficacy of adrenocorticotropic hormone (ACTH) in treatment of patients with intractable seizure. MATERIALS & METHODS: Twenty-five patients with intractable seizure other than spasm including 14 boys and 11 girls with median age of 58 months referred to university clinics of Pediatric Neurology in Isfahan, Iran, during 2014-2015 were prospectively investigated...
2017: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/28873364/refractory-spasms-of-focal-onset-a-potentially-curable-disease-that-should-lead-to-rapid-surgical-evaluation
#11
Mathilde Chipaux, Georg Dorfmüller, Martine Fohlen, Nathalie Dorison, Marie-Astrid Metten, Olivier Delalande, Sarah Ferrand-Sorbets, Delphine Taussig
PURPOSE: Infantile spasms (IS) can occur as the only seizure type in children with surgically amenable epilepsies. Although early surgery has shown positive effects, little is known regarding outcomes. METHODS: We retrospectively reviewed all children with IS referred to our tertiary center between 2002 and 2014 and try to define factors of outcome. RESULTS: Sixty-eight children with focal onset seizures were referred: twenty children with a hemispheric implication and 48 with one or more lobes involved...
August 31, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28866334/increasing-volume-and-complexity-of-pediatric-epilepsy-surgery-with-stable-seizure-outcome-between-2008-and-2014-a-nationwide-multicenter-study
#12
Carmen Barba, Nicola Specchio, Renzo Guerrini, Laura Tassi, Salvatore De Masi, Francesco Cardinale, Simona Pellacani, Luca De Palma, Domenica Battaglia, Gianpiero Tamburrini, Giuseppe Didato, Elena Freri, Alessandro Consales, Paolo Nozza, Nelia Zamponi, Elisabetta Cesaroni, Giancarlo Di Gennaro, Vincenzo Esposito, Marco Giulioni, Paolo Tinuper, Gabriella Colicchio, Raffaele Rocchi, Guido Rubboli, Flavio Giordano, Giorgio Lo Russo, Carlo Efisio Marras, Massimo Cossu
OBJECTIVE: The objective of the study was to assess common practice in pediatric epilepsy surgery in Italy between 2008 and 2014. METHODS: A survey was conducted among nine Italian epilepsy surgery centers to collect information on presurgical and postsurgical evaluation protocols, volumes and types of surgical interventions, and etiologies and seizure outcomes in pediatric epilepsy surgery between 2008 and 2014. RESULTS: Retrospective data on 527 surgical procedures were collected...
August 31, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28865255/parent-use-of-cannabis-for-intractable-pediatric-epilepsy-everyday-empiricism-and-the-boundaries-of-scientific-medicine
#13
Elisa J Sobo
Cannabis is an increasingly sought-after remedy for US children with intractable (biomedically uncontrollable) epilepsy. However, like other complementary-alternative medicine (CAM) modalities, and particularly as a federally illegal, stigmatized substance, it is unsanctioned by mainstream medicine. Parents are largely on their own when it comes to learning about, procuring, dispensing, and monitoring treatments. Exploring how they manage is crucial to better assist them. Moreover, it can illuminate how 'research' done on the ground by laypeople variously disrupts and reinforces lay-expert and science-non-science divides...
August 5, 2017: Social Science & Medicine
https://www.readbyqxmd.com/read/28862044/acute-lamotrigine-overdose-a-systematic-review-of-published-adult-and-pediatric-cases
#14
Bader Alyahya, Marjorie Friesen, Bénédicte Nauche, Martin Laliberté
CONTEXT: Lamotrigine is a broad-spectrum anticonvulsant commonly used to treat seizure and bipolar mood disorders. Evidence from case series and retrospective studies indicate that lamotrigine overdose is usually benign. However, there are reported cases of cardiac arrest and mortality following lamotrigine overdose. We undertook a systematic review of the literature on lamotrigine overdoses to better understand the clinical severity, the relevance of serum concentrations, and therapeutic interventions for overdose...
September 1, 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28846756/prevalence-of-pathogenic-copy-number-variation-in-adults-with-pediatric-onset-epilepsy-and-intellectual-disability
#15
Felippe Borlot, Brigid M Regan, Anne S Bassett, D James Stavropoulos, Danielle M Andrade
Importance: Copy number variation (CNV) is an important cause of neuropsychiatric disorders. Little is known about the role of CNV in adults with epilepsy and intellectual disability. Objectives: To evaluate the prevalence of pathogenic CNVs and identify possible candidate CNVs and genes in patients with epilepsy and intellectual disability. Design, Setting, and Participants: In this cross-sectional study, genome-wide microarray was used to evaluate a cohort of 143 adults with unexplained childhood-onset epilepsy and intellectual disability who were recruited from the Toronto Western Hospital epilepsy outpatient clinic from January 1, 2012, through December 31, 2014...
August 28, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28844439/comorbidities-and-risk-factors-associated-with-newly-diagnosed-epilepsy-in-the-u-s-pediatric-population
#16
Ahyuda Oh, David J Thurman, Hyunmi Kim
Neurobehavioral comorbidities can be related to underlying etiology of epilepsy, epilepsy itself, and adverse effects of antiepileptic drugs. We examined the relationship between neurobehavioral comorbidities and putative risk factors for epilepsy in children with newly diagnosed epilepsy. We conducted a retrospective analysis of children aged ≤18years in 50 states and the District of Columbia, using the Truven Health MarketScan® commercial claims and encounters database from January 1, 2009 to December 31, 2013...
August 22, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28843460/safety-threats-during-the-care-of-infants-with-hypoglycemic-seizures-in-the-emergency-department-a-multicenter-simulation-based-prospective-cohort-study
#17
Barbara M Walsh, Sandeep Gangadharan, Travis Whitfill, Marcie Gawel, David Kessler, Robert A Dudas, Jessica Katznelson, Megan Lavoie, Khoon-Yen Tay, Melinda Hamilton, Linda L Brown, Vinay Nadkarni, Marc Auerbach
BACKGROUND: Errors in the timely diagnosis and treatment of infants with hypoglycemic seizures can lead to significant patient harm. It is challenging to precisely measure medical errors that occur during high-stakes/low-frequency events. Simulation can be used to assess risk and identify errors. OBJECTIVE: We hypothesized that general emergency departments (GEDs) would have higher rates of deviations from best practices (errors) compared to pediatric emergency departments (PEDs) when managing an infant with hypoglycemic seizures...
August 23, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28843212/the-influence-of-endophenotypic-disease-specific-and-environmental-variables-on-the-expression-of-anxiety-in-pediatric-epilepsy
#18
William A Schraegle, Jeffrey B Titus
Children and adolescents with epilepsy often show higher rates of anxiety, which carries an increased risk for reduced health-related quality of life (HRQOL). The current study assessed the role of parental psychiatric history (i.e., anxiety, depression, and/or bipolar disorder) on the rate of anxiety features in youth seen in a tertiary epilepsy clinic. Data included parental ratings on the Behavior Assessment System for Children (BASC-2) and the Quality of Life in Childhood Epilepsy (QOLCE) questionnaire for 180 children and adolescents (mean age=11...
August 23, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28841275/neuropsychiatric-manifestations-of-pediatric-nmda-receptor-autoimmune-encephalitis-a-case-series-from-a-tertiary-care-center-in-india
#19
Salah Basheer, Madhu Nagappa, Anita Mahadevan, Parayil Sankaran Bindu, Arun B Taly, Satish Chandra Girimaji
Objective: Although psychiatric manifestations are one of the most common presentations of pediatric N-methyl-D-aspartate receptor (NMDAR) encephalitis, there is a lack of studies that characterize psychiatric aspects of this disorder. This study was designed to address this gap. Methods: Initial clinical presentations including psychiatric symptoms, treatment details, and outcome with respect to psychiatric symptoms were collected from medical case records of children aged less than 18 years with seropositive NMDAR encephalitis from a single tertiary care center (May 2010-November 2016)...
August 17, 2017: Primary Care Companion to CNS Disorders
https://www.readbyqxmd.com/read/28839247/spatiotemporal-changes-in-regularity-of-gamma-oscillations-contribute-to-focal-ictogenesis
#20
Yosuke Sato, Simeon M Wong, Yasushi Iimura, Ayako Ochi, Sam M Doesburg, Hiroshi Otsubo
In focal ictogenesis, gamma oscillations (30-70 Hz) recorded by electroencephalography (EEG) are related to the epileptiform synchronization of interneurons that links the seizure onset zone (SOZ) to the surrounding epileptogenic zone. We hypothesized that the synchronization of interneurons could be detected as changes in the regularity of gamma oscillation rhythmicity. We used multiscale entropy (MSE) analysis, which can quantify the regularity of EEG rhythmicity, to investigate how the regularity of gamma oscillations changes over the course of a seizure event...
August 24, 2017: Scientific Reports
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