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Epilepsy in children

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https://www.readbyqxmd.com/read/28821006/adherence-to-antiepileptic-drugs-in-children-and-adolescents-a-retrospective-study-in-primary-care-settings-in-germany
#1
Louis Jacob, Hajo M Hamer, Karel Kostev
OBJECTIVE: The goal of this study was to analyze adherence to antiepileptic drugs (AED) in children and adolescents treated in pediatric practices in Germany. METHODS: The present study included patients aged between 2 and 17years who were diagnosed with epilepsy (ICD-10: G40) and had received at least two prescriptions of AED between January 2006 and December 2015 in 243 pediatric practices in Germany. The medication possession ratio (MPR) was used to estimate adherence, and patients with a MPR greater than 80% were considered adherent...
August 15, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28820708/clinical-spectrum-etiology-and-outcome-of-neurological-disorders-in-the-rural-hospital-of-mosango-the-democratic-republic-of-congo
#2
Deby Mukendi, Jean-Roger Lilo Kalo, Alain Mpanya, Luigi Minikulu, Tharcisse Kayembe, Pascal Lutumba, Barbara Barbé, Philippe Gillet, Jan Jacobs, Harry Van Loen, Cedric P Yansouni, François Chappuis, Raffaella Ravinetto, Kristien Verdonck, Marleen Boelaert, Andrea S Winkler, Emmanuel Bottieau
There is little published information on the epidemiology of neurological disorders in rural Central Africa, although the burden is considered to be substantial. This study aimed to investigate the pattern, etiology, and outcome of neurological disorders in children > 5 years and adults admitted to the rural hospital of Mosango, province of Kwilu, Democratic Republic of Congo, with a focus on severe and treatable infections of the central nervous system (CNS). From September 2012 to January 2015, 351 consecutive patients hospitalized for recent and/or ongoing neurological disorder were prospectively evaluated by a neurologist, subjected to a set of reference diagnostic tests in blood or cerebrospinal fluid, and followed-up for 3-6 months after discharge...
August 14, 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/28819726/population-pharmacokinetics-of-stiripentol-in-paediatric-patients-with-dravet-syndrome-treated-with-stiripentol-valproate-and-clobazam-combination-therapy
#3
Sophie Peigné, Stéphanie Chhun, Michel Tod, Elisabeth Rey, Christelle Rodrigues, Catherine Chiron, Gérard Pons, Vincent Jullien
AIM: The aim of this study was to describe the pharmacokinetics of stiripentol in children with Dravet syndrome and to determine the concentrations of stiripentol achieved in this population for the usual 25 mg/kg twice-daily dose. METHODS: Thirty-five children with epilepsy were included in a prospective population pharmacokinetic study (using MONOLIX software). Four blood samples were drawn per patient. Stiripentol area under the plasma concentration-time curve (AUC) values and trough concentrations were simulated for 7000 theoretical children weighing between 10 and 70 kg for the 25 mg/kg twice-daily dose...
August 17, 2017: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/28816003/prevalence-of-gastrointestinal-symptoms-in-angelman-syndrome
#4
Laura W Glassman, Olivia R Grocott, Portia A Kunz, Anna M Larson, Garret Zella, Kriston Ganguli, Ronald L Thibert
Angelman syndrome (AS) is a neurogenetic disorder characterized by intellectual disability, expressive speech impairment, movement disorder, epilepsy, and a happy demeanor. Children with AS are frequently reported to be poor feeders during infancy and as having gastrointestinal issues such as constipation, reflux, and abnormal food related behaviors throughout their lifetime. To assess the prevalence of gastrointestinal disorders in individuals with AS, we retrospectively analyzed medical records of 120 individuals seen at the Angelman Syndrome Clinic at Massachusetts General Hospital and 43 individuals seen at the University of North Carolina Comprehensive Angelman Clinic...
August 16, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28811646/pafah1b1-haploinsufficiency-disrupts-gaba-neurons-and-synaptic-e-i-balance-in-the-dentate-gyrus
#5
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/28811058/early-detection-of-tuberous-sclerosis-complex-an-opportunity-for-improved-neurodevelopmental-outcome
#6
Clara W T Chung, John A Lawson, Vanessa Sarkozy, Kate Riney, Orli Wargon, Antonia W Shand, Stephen Cooper, Harrison King, Sean E Kennedy, David Mowat
BACKGROUND: Tuberous sclerosis complex (TSC) is an autosomal dominant condition associated with epilepsy, benign tumors, and variable neurodevelopmental outcomes. The diagnosis is most commonly made after epilepsy onset, although a proportion are diagnosed prenatally. Presymptomatic or early treatment with agents such as vigabatrin offers the hope of improved neurodevelopmental outcome. Therefore early diagnosis, before the onset of seizures, is important. In a cohort of children with TSC, we evaluated the age and mode of initial presentation, assessed the neurocognitive and epilepsy outcome, and analyzed whether those diagnosed before the onset of seizures have a different outcome compared with those diagnosed postseizures...
May 26, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28808808/ketogenic-diet-versus-gluten-free-casein-free-diet-in-autistic-children-a-case-control-study
#7
Omnia El-Rashidy, Farida El-Baz, Yasmin El-Gendy, Randa Khalaf, Dina Reda, Khaled Saad
Many diet regimens were studied for patients with autism spectrum disorder (ASD) over the past few years. Ketogenic diet is gaining attention due to its proven effect on neurological conditions like epilepsy in children. Forty-five children aged 3-8 years diagnosed with ASD based on DSM-5 criteria were enrolled in this study. Patients were equally divided into 3 groups, first group received ketogenic diet as modified Atkins diet (MAD), second group received gluten free casein free (GFCF) diet and the third group received balanced nutrition and served as a control group...
August 14, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28807611/initial-treatment-for-nonsyndromic-early-life-epilepsy-an-unexpected-consensus
#8
Renée A Shellhaas, Anne T Berg, Zachary M Grinspan, Courtney J Wusthoff, John J Millichap, Tobias Loddenkemper, Jason Coryell, Russell P Saneto, Catherine J Chu, Sucheta M Joshi, Joseph E Sullivan, Kelly G Knupp, Eric H Kossoff, Cynthia Keator, Elaine C Wirrell, John R Mytinger, Ignacio Valencia, Shavonne Massey, William D Gaillard
OBJECTIVE: There are no evidence-based guidelines on the preferred approach to treating early-life epilepsy. We examined initial therapy selection in a contemporary US cohort of children with newly diagnosed, nonsyndromic, early-life epilepsy (onset before age three years). METHODS: Seventeen pediatric epilepsy centers participated in a prospective cohort study of children with newly diagnosed epilepsy with onset under 36 months of age. Details regarding demographics, seizure types, and initial medication selections were obtained from medical records...
June 27, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28806633/provision-of-sudden-unexpected-death-in-epilepsy-sudep-information-among-malaysian-parents-of-children-with-epilepsy
#9
Choong Yi Fong, Wei Kang Lim, Ann Nie Kong, Pei Lin Lua, Lai Choo Ong
Sudden unexpected death in epilepsy (SUDEP) is an important cause of mortality in epilepsy. To date, there is only one published UK study evaluating information provision of SUDEP among parents of children with epilepsy (CWE), and there are no studies published from Asia. Although SUDEP information provision is recommended among parents of CWE, it is uncertain if these recommendations are applicable to Asian countries due to the different cultural attitude towards epilepsy. Our prospective cohort study consisted of multiethnic parents of children with epilepsy (CWE) seen in a tertiary hospital in Malaysia...
August 11, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28804872/lacosamide-in-the-treatment-of-patients-with-epilepsy-and-intellectual-disabilities-a-long-term-study-of-136-patients
#10
Stefan Böttcher, Martin T Lutz, Thomas Mayer
OBJECTIVE: This study aimed to analyze the retention rate of lacosamide (LCM) in patients with epilepsy and intellectual disabilities (IDs), to identify factors influencing retention rate, and to investigate the LCM retention rate with and without concomitant sodium channel blocker (SCB). We hypothesized that the retention rate of LCM with concomitant SCB would be lower than without SCB. METHODS: Using the Kaplan-Meier estimator, we conducted a monocentric, retrospective, observational, open-label study to evaluate LCM retention rates in patients with IDs and drug-resistant epilepsy...
August 14, 2017: Epilepsia
https://www.readbyqxmd.com/read/28801973/cognitive-functioning-in-children-with-self-limited-epilepsy-with-centrotemporal-spikes-a-systematic-review-and-meta-analysis
#11
REVIEW
Steven Wickens, Stephen C Bowden, Wendyl D'Souza
OBJECTIVE: It is now well appreciated that benign epilepsy with centrotemporal spikes (BECTS, or more recently, ECTS) is associated with a range of cognitive and behavioral disturbances. Despite our improved understanding of cognitive functioning in ECTS, there have been to date no efforts to quantitatively synthesize the available literature within a comprehensive cognitive framework. METHODS: The present systematic review and meta-analysis was conducted according to PRISMA guidelines...
August 12, 2017: Epilepsia
https://www.readbyqxmd.com/read/28801085/measuring-in-vivo-cerebral-maturation-using-age-related-t2-relaxation-times-at-3t
#12
Eva Bültmann, Loukia M Spineli, Hans Hartmann, Heinrich Lanfermann
OBJECTIVE: To examine age-related changes in T2 relaxation times during infancy and childhood in order to assess T2 values obtained from routine MRI as a biomarker. METHODS: From our pool of clinical pediatric MRI examinations at 3T all patients with normal conventional MRI scans were retrospectively selected. Depending on their clinical findings the identified 99 patients (0-199months) were divided into 43 healthy controls and 56 diseased children with various clinical abnormalities (developmental delay, epilepsy, prematurity, and deafness)...
August 8, 2017: Brain & Development
https://www.readbyqxmd.com/read/28800038/subclinical-rhythmic-eeg-discharge-of-adult-sreda-in-a-child-with-generalized-epilepsy-and-literature-review-of-sreda-in-children
#13
Marcie Goeden, Lalit R Bansal
Subclinical rhythmic discharges of adult (SREDA) is a rare benign EEG variant in adults and is of unknown clinical significance. Its occurrence in children is extremely rare. In review of the literature, it has been described in only four children. We present a case of a 10-year-old female with generalized idiopathic childhood absence epilepsy who is noted to have SREDA in three subsequent EEGs performed across a 25-month span. She had no clinical change with these discharges and it was believed to be a benign variant...
August 9, 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28799513/ketogenic-diets-in-the-treatment-of-epilepsy
#14
Maurizio Elia, Joerg Klepper, Baerbel Leiendecker, Hans Hartmann
BACKGROUND: Although a larger number of antiepileptic drugs became available in the last decades, epilepsy remains drug-resistant in approximately a third of patients. Ketogenic diet (KD), first proposed at the beginning of the last century, is complex and has anticonvulsant effects, yet not completely understood. Over the last decades, different types of ketogenic diets (KDs) have been developed, namely classical KD and modified Atkins diet (MAD). They offer an effective alternative for children and adults with drug-resistant epilepsies...
August 9, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28796925/neurological-comorbidity-in-children-with-neurofibromatosis-type-1
#15
Keiko Hirabaru, Muneaki Matsuo
OBJECTIVE: To determine the frequency of central nervous system comorbidities in children with neurofibromatosis type 1 (NF1). METHODS: We performed a nationwide survey to investigate neurological comorbidities in 3-15-year-old children with NF1 in Japan by sending questionnaires to pediatricians and pediatric neurologists. A secondary questionnaire was sent to the parents of identified NF1 patients to assess neurological comorbidities including headache, attention deficit hyperactivity disorder (ADHD)-Rating Scale (RS), and the Social Responsiveness Scale -2...
August 10, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28796763/national-and-state-estimates-of-the-numbers-of-adults-and-children-with-active-epilepsy-united-states-2015
#16
Matthew M Zack, Rosemarie Kobau
Epilepsy, a brain disorder leading to recurring seizures, has garnered increased public health focus because persons with epilepsy experience pronounced and persistent health and socioeconomic disparities despite treatment advances, public awareness programs, and expanded rights for persons with disabilities (1,2). For almost all states, epilepsy prevalence estimates do not exist. CDC used national data sources including the 2015 National Health Interview Survey (NHIS) for adults (aged ≥18 years), the 2011-2012 National Survey of Children's Health (NSCH), and the 2015 Current Population Survey data, describing 2014 income levels, to estimate prevalent cases of active epilepsy, overall and by state, to provide information for state public health planning...
August 11, 2017: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/28794249/not-all-scn1a-epileptic-encephalopathies-are-dravet-syndrome-early-profound-thr226met-phenotype
#17
Lynette G Sadleir, Emily I Mountier, Deepak Gill, Suzanne Davis, Charuta Joshi, Catherine DeVile, Manju A Kurian, Simone Mandelstam, Elaine Wirrell, Katherine C Nickels, Hema R Murali, Gemma Carvill, Candace T Myers, Heather C Mefford, Ingrid E Scheffer
OBJECTIVE: To define a distinct SCN1A developmental and epileptic encephalopathy with early onset, profound impairment, and movement disorder. METHODS: A case series of 9 children were identified with a profound developmental and epileptic encephalopathy and SCN1A mutation. RESULTS: We identified 9 children 3 to 12 years of age; 7 were male. Seizure onset was at 6 to 12 weeks with hemiclonic seizures, bilateral tonic-clonic seizures, or spasms...
August 9, 2017: Neurology
https://www.readbyqxmd.com/read/28794080/the-immune-system-in-pediatric-seizures-and-epilepsies
#18
REVIEW
Christian M Korff, Russell C Dale
The relation between the immune system and epilepsy has been studied for a long time. Immune activation may precede or follow the appearance of seizures. Depending on the situation, the innate and acquired immunity may be involved to various degrees. The intense, ongoing research has opened encouraging management and therapeutic perspectives for a significant number of patients suffering from seizures. These include the use of various drugs and less conventional approaches with anti-inflammatory or immunomodulatory properties...
August 9, 2017: Pediatrics
https://www.readbyqxmd.com/read/28792705/homocysteine-metabolism-and-hematological-parameters-in-early-stage-of-phenytoin-treated-epileptic-children
#19
Tarun K Sharma, S K Vardey, S Sitaraman
BACKGROUND: Long-term antiepileptic drug (AED) therapy has been associated with metabolic consequences that lead to an increase in risk of atherosclerosis in patients with epilepsy. Earlier published studies showed conflicting results about the levels of hematological parameters, serum homocysteine, folate, and vitamin B12, in epileptics treated with phenytoin monotherapy. Therefore, we evaluated homocysteine metabolism and hematological parameters in early stage of phenytoin treated epileptic children...
July 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28783116/teratogenic-effects-of-topiramate-in-a-zebrafish-model
#20
Yu-Heng Lai, Yu-Ju Ding, David Moses, Yau-Hung Chen
Topiramate is commonly used for treating epilepsy in both children and adults. Recent clinical data suggests that administration of topiramate to women during pregnancy increases the risk of oral clefts in their offspring. To better understand the potential effects of topiramate, we dosed adult female zebrafish with topiramate, and investigated the altered morphologies in adult females and their offspring. It showed that topiramate-treated female fish had reduced oocyte maturation, and the survival rates of their offspring were seriously decreased during embryogenesis...
August 7, 2017: International Journal of Molecular Sciences
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