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https://www.readbyqxmd.com/read/28629487/learning-and-memory-in-adolescents-with-critical-biventricular-congenital-heart-disease
#1
Adam R Cassidy, Jane W Newburger, David C Bellinger
OBJECTIVES: Although evidence exists of broadly defined memory impairment among adolescents with critical congenital heart disease (CHD), nuanced investigations of declarative memory in this at-risk population have not been conducted. This study had two primary aims: (1) to conduct a fine-grained analysis of a range of relevant learning and memory processes in adolescents with critical biventricular CHD, and (2) to identify risk, odds, and predictors of memory impairment. METHODS: Data were combined from two single-center studies of neurodevelopmental outcomes in critical CHD...
June 20, 2017: Journal of the International Neuropsychological Society: JINS
https://www.readbyqxmd.com/read/28629473/emergency-treatment-with-levetiracetam-or-phenytoin-in-status-epilepticus-in-children-the-eclipse-study-study-protocol-for-a-randomised-controlled-trial
#2
Mark D Lyttle, Carrol Gamble, Shrouk Messahel, Helen Hickey, Anand Iyer, Kerry Woolfall, Amy Humphreys, Naomi E A Bacon, Louise Roper, Franz E Babl, Stuart R Dalziel, Mary Ryan, Richard E Appleton
BACKGROUND: Convulsive status epilepticus (CSE) is the most common life-threatening neurological emergency in childhood. These children are also at risk of significant morbidity, with acute and chronic impact on the family and the health and social care systems. The current recommended first-choice, second-line treatment in children aged 6 months and above is intravenous phenytoin (fosphenytoin in the USA), although there is a lack of evidence for its use and it is associated with significant side effects...
June 19, 2017: Trials
https://www.readbyqxmd.com/read/28624716/seizure-related-hospital-admissions-readmissions-and-costs-comparisons-with-asthma-and-diabetes-in-south-australia
#3
Michelle L Bellon, Christopher Barton, Nikki McCaffrey, Denise Parker, Claire Hutchinson
PURPOSE: Seizures are listed as an Ambulatory Care Sensitive Condition (ACSC), where, in some cases, hospitalisation may be avoided with appropriate preventative and early management in primary care. We examined the frequencies, trends and financial costs of first and subsequent seizure-related hospital admissions in the adult and paediatric populations, with comparisons to bronchitis/asthma and diabetes admissions in South Australia between 2012 and 2014. METHODS: De-identified hospital separation data from five major public hospitals in metropolitan South Australia were analysed to determine the number of children and adults admitted for the following Australian Refined Diagnosis Related Groups: seizure related conditions; bronchitis/asthma; and diabetes...
June 10, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28623545/analysis-of-genotypes-and-phenotypes-in-chinese-children-with-tuberous-sclerosis-complex
#4
Dayong Bai, Junyang Zhao, Li Li, Jun Gao, Xu Wang
Tuberous sclerosis complex (TSC) is a neurocutaneous syndrome with serious clinical presentations, an autosomal dominant genetic disorder involving multiple organs and systems. We retrospectively investigated the clinical manifestations and genotypes of 20 Chinese children with TSC to enable informed diagnostic and surveillance recommendations in China. A retrospective analysis of clinical manifestations in 20 children (7.00±5.30 years old) with TSC was conducted. A genetic testing of the genes TSC1 and TSC2 was performed in 14 children...
June 14, 2017: Science China. Life Sciences
https://www.readbyqxmd.com/read/28623069/neonatal-hypoglycemia-a-wide-range-of-electroclinical-manifestations-and-seizure-outcomes
#5
Ebru Arhan, Zeynep Öztürk, Ayşe Serdaroğlu, Kürşad Aydın, Tuğba Hirfanoğlu, Yılmaz Akbaş
PURPOSE: We examined the various types of epilepsy in children with neonatal hypoglycemia in order to define electroclinical and prognostic features of these patients. METHOD: We retrospectively reviewed the medical records of patients with a history of symptomatic neonatal hypoglycaemia who have been followed at Gazi University Hospital Pediatric Neurology Department between 2006 and 2015. Patients with perinatal asphyxia were excluded. Details of each patient's perinatal history, neurological outcome, epilepsy details, seizure outcome and EEG and brain MRI findings were reviewed...
June 1, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28622546/the-semiology-of-febrile-seizures-focal-features-are-frequent
#6
Michihiko Takasu, Tetsuo Kubota, Takeshi Tsuji, Hirokazu Kurahashi, Shingo Numoto, Kazuyoshi Watanabe, Akihisa Okumura
OBJECTIVE: To clarify the semiology of febrile seizures (FS) and to determine the frequency of FS with symptoms suggestive of focal onset. METHODS: FS symptoms in children were reported within 24h of seizure onset by the parents using a structured questionnaire consisting principally of closed-ended questions. We focused on events at seizure commencement, including changes in behavior and facial expression, and ocular and oral symptoms. We also investigated the autonomic and motor symptoms developing during seizures...
June 13, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28620490/ketogenic-diet-predictors-of-seizure-control
#7
Nitin Agarwal, Dimitrios Arkilo, Osman Farooq, Cynthia Gillogly, Katelyn S Kavak, Arie Weinstock
BACKGROUND: The ketogenic diet is an effective non-pharmacologic treatment for medically resistant epilepsy. The aim of this study was to identify any predictors that may influence the response of ketogenic diet. METHODS: A retrospective chart review for all patients with medically resistant epilepsy was performed at a tertiary care epilepsy center from 1996 to 2012. Patient- and diet-related variables were evaluated with respect to seizure reduction at 1, 3, 6, 9 and 12-month intervals and divided into four possible outcome classes...
2017: SAGE Open Medicine
https://www.readbyqxmd.com/read/28619377/cumulative-incidence-of-seizures-and-epilepsy-in-ten-year-old-children-born-before-28%C3%A2-weeks-gestation
#8
Laurie M Douglass, Timothy C Heeren, Carl E Stafstrom, William DeBassio, Elizabeth N Allred, Alan Leviton, T Michael O'Shea, Deborah Hirtz, Julie Rollins, Karl Kuban
OBJECTIVE: We evaluated the incidence of seizures and epilepsy in the first decade of life among children born extremely premature (less than 28 weeks' gestation). METHOD: In a prospective, multicenter, observational study, 889 of 966 eligible children born in 2002 to 2004 were evaluated at two and ten years for neurological morbidity. Complementing questionnaire data to determine a history of seizures, all caregivers were interviewed retrospectively for postneonatal seizures using a validated seizure screen followed by a structured clinical interview by a pediatric epileptologist...
May 18, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28619360/further-delineation-of-cog8-cdg-a-case-with-novel-compound-heterozygous-mutations-diagnosed-by-targeted-exome-sequencing
#9
Aram Yang, Sung Yoon Cho, Ja-Hyun Jang, Jinsup Kim, Sook Za Kim, Beom Hee Lee, Han-Wook Yoo, Dong-Kyu Jin
Congenital disorders of glycosylation (CDG) are a rapidly expanding group of inherited metabolic disorders with highly variable clinical presentations caused by deficient glycosylation of proteins and/or lipids. CDG-IIh is a very rare subgroup of CDG caused by mutations in the conserved oligomeric Golgi (COG) complex gene, COG8, and so far, only two cases have been reported in the medical literature. Here, we describe an 8-year-old Korean boy with psychomotor retardation, hypotonia, failure to thrive, elevated serum liver enzymes, microcephaly, and talipes equinovarus...
June 13, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28615945/ictal-blinking-an-under-recognized-phenomenon-our-experience-and-literature-review
#10
Marco Andrea Nicola Saporito, Giovanna Vitaliti, Piero Pavone, Giuseppa Di Stefano, Pasquale Striano, Roberto Horacio Caraballo, Raffaele Falsaperla
Ictal blinking (IB) is a very rare disease manifesting as an epileptic motor event in children and adults. Until now it has not been included in any classification of focal seizures of the International League Against Epilepsy Commission. It could be unilateral or bilateral, isolated or in association with other motor manifestations such as limbs' clonus and spasms. Its pathogenesis has not been clearly established: paroxysmal discharges from different areas of the brain could cause IB by activation of trigeminal fibers...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28612538/-risk-factors-and-prognosis-of-secondary-epilepsy-in-children-with-viral-encephalitis
#11
Liang Huang, Dan Yu, Rong Luo, Min Li, Hui Zhou, Xiao-Tang Cai, Zhi-Ling Wang
OBJECTIVES: To investigate the clinical features of viral encephalitis in children at acute stage, and the relationship of these clinical features with post viral encephalitic epilepsy. METHODS: The children with viral encephalitis treated in West China Second University Hospital between January 2010 and January 2014 were enrolled. The clinical features included general conditions, clinical manifestations, video electroencephalogram (VEEG), neuroimaging, virology, prognosis and antiepileptic drugs...
March 2017: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/28606234/-efficacy-and-safety-of-cyclophosphamide-as-a-sequential-immunotherapy-drug-for-anti-n-methyl-d-aspartate-receptor-encephalitis-in-children
#12
Wei-Wen Zhu, Wei-Ping Liao, Yong-Hong Yi, Xing-Wang Song
OBJECTIVE: To evaluate the efficacy and safety of cyclophosphamide as a second-line drug in the treatment of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. METHODS: Six children with anti-NMDAR encephalitis, who showed poor response to steroids and intravenous immunoglobulin, were given cyclophosphamide as a second-line immunotherapy. Follow-up was performed to evaluate the efficacy and safety of cyclophosphamide. RESULTS: After first-line immunotherapy for 1-4 weeks, the six patients had reduced psychiatric symptoms, seizures, and involuntary movements; three patients had an improved level of consciousness and were able to make simple conversations...
June 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28605661/seizure-management-by-preschool-teachers-a-training-concept-focussing-on-practical-skills
#13
Henriette K Dumeier, Martina P Neininger, Almuth Kaune, Pia M Schumacher, Andreas Merkenschlager, Wieland Kiess, Matthias K Bernhard, Thilo Bertsche, Astrid Bertsche
PURPOSE: Prolonged seizures can cause severe harm and even death. For seizures lasting longer than 5min, an administration of rescue medication is therefore recommended. Caregivers such as preschool teachers should be able to administer correctly anticonvulsive rescue medication to children. METHODS: A training concept for preschool teachers on seizure management focussing on practical skills was developed. To assess the success of the training, a structured interview on attitudes relating to rescue medication administration was conducted...
June 9, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28605632/seizure-related-variables-are-predictive-of-attention-and-memory-in-children-with-epilepsy
#14
Danielle N Lordo, Ryan Van Patten, Eliana L Sudikoff, Lisa Harker
Children with epilepsy (CWE) are at greater risk for cognitive deficits and behavioral difficulties than are typically developing healthy children, and particular epileptic symptoms and treatments may contribute to this risk. The current study examined the relationships between four seizure-related variables and attention and memory functioning in a sample of 207 CWE (ages 6-16) using both neurocognitive and parent/teacher-report measures. Sociodemographic, medical, and neuropsychological data were collected from patients' medical charts in a retrospective fashion...
June 9, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28605630/cognitive-outcome-after-epilepsy-surgery-in-children-a-controlled-longitudinal-study
#15
Valentina Sibilia, Carmen Barba, Tiziana Metitieri, Giovanni Michelini, Flavio Giordano, Lorenzo Genitori, Renzo Guerrini
OBJECTIVE: To analyze the determinants of cognitive outcome two years after surgery for drug-resistant epilepsy in a cohort of 31 children when compared to a control group of 14 surgical candidates who had yet to undergo surgery two years after the first neuropsychological assessment. METHODS: Controlled longitudinal study including three evaluations of IQ (Intelligence Quotient) scores or GDQ (General Developmental Quotient) for each group depending on the patient's age: prior to surgery (T0), one year (T1) and two years (T2) after surgery for the surgical group; baseline (T0) and one year (T1) and 2years (T2) after the first evaluation for the control-group...
June 9, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28605011/use-of-the-ketogenic-diet-to-manage-refractory-epilepsy-in-cdkl5-disorder-experience-of-100-patients
#16
Zhan Lim, Kingsley Wong, Heather E Olson, Ann M Bergin, Jenny Downs, Helen Leonard
OBJECTIVE: Pathogenic variants involving the CDKL5 gene result in a severe epileptic encephalopathy, often later presenting with features similar to Rett syndrome. Cardinal features of epilepsy in the CDKL5 disorder include early onset at a median age of 6 weeks and poor response to antiepileptic drugs. The ketogenic diet (KD) was first introduced in the 1920s as a treatment option for refractory epilepsy in children. This study investigated use of the KD in the CDKL5 disorder and its influences on seizures...
June 12, 2017: Epilepsia
https://www.readbyqxmd.com/read/28604149/risk-of-second-seizure-in-pediatric-patients-with-idiopathic-autism
#17
Asad A Qadir, Emily Obringer, Joseph Hageman, Charles Marcuccilli
PURPOSE: Epilepsy is a comorbidity of idiopathic autism spectrum disorder. The aim was to characterize the risk and time of second seizure in children with idiopathic autism spectrum disorder. METHODS: A retrospective review was performed at the University of Chicago and NorthShore University HealthSystem. Patients with idiopathic autism spectrum disorder, ≥1 seizure, and age 2 to 23 years were included. RESULTS: 153 patients were included; 141 (92%) had a second seizure...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28603689/spiders-ladybugs-and-bees-a-case-of-unusual-sensations-in-a-child-with-cingulate-epilepsy
#18
Robyn Whitney, Sameer AlMehmadi, Cristina Go, Ayako Ochi, Hiroshi Otsubo, Laura Bradbury, Kevin Jones, Eisha Christian, James Rutka, Bláthnaid McCoy
Cingulate epilepsy is a rare form of epilepsy. Seizures from the anterior cingulate may present with mood change, fear, hypermotor activity, and autonomic signs, while posterior cingulate seizures resemble temporal lobe seizures. We describe a child with cingulate epilepsy who experienced unpleasant/painful sensory phenomenon. The sensations were described as spiders crawling on his forehead/right leg, ladybugs causing right ear pain and bees stinging his head/right extremities. Unpleasant sensory phenomenon/pain are rarely reported in cingulate epilepsy...
2017: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/28602890/posterior-reversible-encephalopathy-syndrome-after-hematopoietic-cell-transplantation-in-children-with-hemoglobinopathies
#19
Javid Gaziev, Simone Marziali, Katia Paciaroni, Antonella Isgrò, Francesca Di Giuliano, Giorgia Rossi, Marco Marziali, Gioia De Angelis, Cecilia Alfieri, Michela Ribersani, Marco Andreani, Maria Giuseppina Palmieri, Fabio Placidi, Andrea Romigi, Francesca Izzi, Roberto Floris, Nicola Biagio Mercuri
Posterior reversible encephalopathy syndrome (PRES) is a serious adverse event associated with calcineurin inhibitors (CI) used for graft-versus-host disease (GVHD) prophylaxis. We compared the incidence of PRES in children with thalassemia (n=222, 1.4-17.8 years old) versus sickle cell disease (SCD; n=59, 2-17 years old) who underwent hematopoietic cell transplantation (HCT) from HLA-matched siblings or alternative donors and analyzed the risk factors for PRES. Overall, 31 children developed CI-related PRES (11%), including 30 patients with seizures and 1 patient without seizures...
June 5, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28602636/extensive-phenotyping-of-two-arx-polyalanine-expansion-mutation-mouse-models-that-span-clinical-spectrum-of-intellectual-disability-and-epilepsy
#20
Matilda R Jackson, Kristie Lee, Tessa Mattiske, Emily J Jaehne, Ezgi Ozturk, Bernhard T Baune, Terence J O'Brien, Nigel Jones, Cheryl Shoubridge
The Aristaless-related homeobox gene (ARX) is a known intellectual disability (ID) gene that frequently presents with X-linked infantile spasm syndrome as a comorbidity. ID with epilepsy in children is a chronic and devastating disorder that has poor treatment options and disease outcomes. To gain a better understanding of the role that mutations in ARX play in ID and epilepsy, we investigate ARX patient mutations modelled in mice. Over half of all ARX mutations result from expansions of the first two polyalanine (PA1 and PA2 respectively) tracts...
June 7, 2017: Neurobiology of Disease
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