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Febrile Seizure

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https://www.readbyqxmd.com/read/28448686/plasma-cytokines-associated-with-febrile-status-epilepticus-in-children-a-potential-biomarker-for-acute-hippocampal-injury
#1
William B Gallentine, Shlomo Shinnar, Dale C Hesdorffer, Leon Epstein, Douglas R Nordli, Darrell V Lewis, L Matthew Frank, Syndi Seinfeld, Ruth C Shinnar, Karen Cornett, Binyi Liu, Solomon L Moshé, Shumei Sun
OBJECTIVE: Our aim was to explore the association between plasma cytokines and febrile status epilepticus (FSE) in children, as well as their potential as biomarkers of acute hippocampal injury. METHODS: Analysis was performed on residual samples of children with FSE (n = 33) as part of the Consequences of Prolonged Febrile Seizures in Childhood study (FEBSTAT) and compared to children with fever (n = 17). Magnetic resonance imaging (MRI) was obtained as part of FEBSTAT within 72 h of FSE...
April 27, 2017: Epilepsia
https://www.readbyqxmd.com/read/28442529/coinheritance-of-novel-mutations-in-scn1a-causing-gefs-and-in-kdm6a-causing-kabuki-syndrome-in-a-family
#2
Jisun Kim, Cha Gon Lee
Because the differentiation between phenotypic expansion and blended phenotypes is not clear, the mixed phenotypes of blended rare genetic diseases make diagnosis difficult. We describe a family with the co-existence and co-segregation of generalized epilepsy with febrile seizures plus (GEFS+) and Kabuki syndrome (KS). The proband, a 7-year-old male, presented with GEFS+, dysmorphic facial features, short stature, developmental delay, and intellectual disability. Two novel missense mutations: p.G325A in the KDM6A gene responsible for KS and p...
March 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/28436815/moyamoya-in-a-patient-with-fires-a-first-case-report
#3
Taylor Kaufman, Andrew White
Febrile infection-related epilepsy syndrome (FIRES) is a form of epileptic encephalopathy with severe refractory epilepsy that presents in previously healthy, school-aged children after significant febrile illness with concomitant rise in body temperature. Suspected causes include genetic or acquired channelopathies, as well as mitochondrial disturbances. In FIRES, the EEG shows diffuse slowing, generalized, and/or multifocal discharges. Seizures are present and resistant to treatment. Moyamoya angiopathy (MMA) is characterized by progressive stenosis of cerebral arteries and subsequent development of a network of collateral circulation that is prone to rupture...
2017: Neurodiagnostic Journal
https://www.readbyqxmd.com/read/28435493/prehospital-care-for-the-adult-and-pediatric-seizure-patient-current-evidence-based-recommendations
#4
Eric C Silverman, Karl A Sporer, Justin M Lemieux, John F Brown, Kristi L Koenig, Marianne Gausche-Hill, Eric M Rudnick, Angelo A Salvucci, Greg H Gilbert
INTRODUCTION: We sought to develop evidence-based recommendations for the prehospital evaluation and treatment of adult and pediatric patients with a seizure and to compare these recommendations against the current protocol used by the 33 emergency medical services (EMS) agencies in California. METHODS: We performed a review of the evidence in the prehospital treatment of patients with a seizure, and then compared the seizure protocols of each of the 33 EMS agencies for consistency with these recommendations...
April 2017: Western Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28433581/therapeutic-burst-suppression-coma-in-pediatric-febrile-refractory-status-epilepticus
#5
Jainn-Jim Lin, Cheng-Che Chou, Shih-Yun Lan, Hsiang-Ju Hsiao, Yu Wang, Oi-Wa Chan, Shao-Hsuan Hsia, Huei-Shyong Wang, Kuang-Lin Lin
BACKGROUND: Evidence for the beneficial effect of therapeutic burst-suppression coma in pediatric patients with febrile refractory status epilepticus is limited, and the clinical outcomes of this treatment strategy are largely unknown. Therefore, the aim of this study was to explore the outcomes of therapeutic burst-suppression coma in a series of children with febrile refractory status epilepticus. METHODS: We retrospectively reviewed consecutive pediatric patients with febrile refractory status epilepticus admitted to our pediatric intensive care unit between January 2000 and December 2013...
April 19, 2017: Brain & Development
https://www.readbyqxmd.com/read/28431291/temporal-pole-abnormalities-detected-by-3-t-mri-in-temporal-lobe-epilepsy-due-to-hippocampal-sclerosis-no-influence-on-seizure-outcome-after-surgery
#6
Sara Casciato, Angelo Picardi, Alfredo D'Aniello, Marco De Risi, Giovanni Grillea, Pier Paolo Quarato, Addolorata Mascia, Liliana G Grammaldo, Giulio Nicolo' Meldolesi, Roberta Morace, Vincenzo Esposito, Giancarlo Di Gennaro
PURPOSE: To assess the clinical significance of temporal pole abnormalities (temporopolar blurring, TB, and temporopolar atrophy, TA) detected by using 3 Tesla MRI in the preoperative workup in patients with temporal lobe epilepsy due to hippocampal sclerosis (TLE-HS) who underwent surgery. METHODS: We studied 78 consecutive patients with TLE-HS who underwent surgery and were followed up for at least 2 years. Based on findings of pre-surgical 3 Tesla MRI, patients were subdivided in subgroups according to the presence of TB or TA...
April 12, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28423465/febrile-status-epilepticus-due-to-respiratory-syncytial-virus-infection
#7
Kazuhiro Uda, Katsuhiko Kitazawa
BACKGROUND: Febrile status epilepticus can have neurological sequelae. The type of sequelae, however, may depend on the etiology, including infections by viral agents such as the influenza virus. Respiratory syncytial virus (RSV) infection in childhood suggests a similar role for this pathogen. The aim of this study was to characterize febrile status epilepticus associated with RSV infection, and to determine whether this type of infection is a risk factor for neurological sequelae with febrile status epilepticus...
April 19, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28396650/new-onset-refractory-status-epilepticus-with-claustrum-damage-definition-of-the-clinical-and-neuroimaging-features
#8
Stefano Meletti, Giada Giovannini, Giuseppe d'Orsi, Lisa Toran, Giulia Monti, Rahul Guha, Andreas Kiryttopoulos, Maria Grazia Pascarella, Tommaso Martino, Haris Alexopoulos, Martha Spilioti, Jana Slonkova
New-onset refractory status epilepticus (NORSE) is a rare but challenging condition occurring in a previously healthy patient, often with no identifiable cause. We describe the electro-clinical features and outcomes in a group of patients with NORSE who all demonstrated a typical magnetic resonance imaging (MRI) sign characterized by bilateral lesions of the claustrum. The group includes 31 patients (12 personal and 19 previously published cases; 17 females; mean age of 25 years). Fever preceded status epilepticus (SE) in 28 patients, by a mean of 6 days...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28391250/confirmation-of-mutations-in-prosc-as-a-novel-cause-of-vitamin-b6-dependent-epilepsy
#9
Barbara Plecko, Markus Zweier, Anaïs Begemann, Deborah Mathis, Bernhard Schmitt, Pasquale Striano, Martina Baethmann, Stella Maria Vari, Francesca Beccaria, Federico Zara, Lisa M Crowther, Pascal Joset, Heinrich Sticht, Sorina Mihaela Papuc, Anita Rauch
Vitamin-B6-dependent epilepsies are a heterogenous group of treatable disorders due to mutations in several genes (ALDH7A1, PNPO, ALPL or ALDH4A1). In neonatal seizures, defects in ALDH7A1 and PNPO explain a major fraction of cases. Very recently biallelic mutations in PROSC were shown to be a novel cause in five families. We identified four further unrelated patients harbouring a total of six different mutations, including four novel disease mutations. Vitamin B6 plasma profiles on pyridoxine did not enable the differentiation of patients with PROSC mutations...
April 8, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28380244/suppositories-for-fever-as-a-major-risk-for-phenobarbital-induced-fixed-drug-eruption-in-children
#10
E Özkaya, A Kutlay
Phenobarbital, an anticonvulsant agent, is a well-known cause of fixed drug eruption (FDE) in patients with epilepsy. In paediatric population without epilepsy however, phenobarbital-induced FDE is only rarely reported, and exclusively from its use in suppositories for fever. It is included in suppositories, along with acetaminophen or acetyl salicylic acid, because of its sedative properties, and to prevent febrile seizures in children. We herein report two children with FDE from phenobarbital-containing suppositories in order to point out to this uncommon but important side effect of suppositories for fever...
April 5, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28373025/early-life-febrile-seizures-worsen-adult-phenotypes-in-scn1a-mutants
#11
Stacey B B Dutton, Karoni Dutt, Ligia A Papale, Sandra Helmers, Alan L Goldin, Andrew Escayg
Mutations in the voltage-gated sodium channel (VGSC) gene SCN1A, encoding the Nav1.1 channel, are responsible for a number of epilepsy disorders including genetic epilepsy with febrile seizures plus (GEFS+) and Dravet syndrome (DS). Patients with SCN1A mutations often experience prolonged early-life febrile seizures (FSs), raising the possibility that these events may influence epileptogenesis and lead to more severe adult phenotypes. To test this hypothesis, we subjected 21-23-day-old mice expressing the human SCN1A GEFS+ mutation R1648H to prolonged hyperthermia, and then examined seizure and behavioral phenotypes during adulthood...
April 1, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28372289/dpp4-regulates-the-inflammatory-response-in-a-rat-model-of-febrile-seizures
#12
Qi Sun, Yusong Zhang, Jie Huang, Fang Yu, Jian Xu, Biwen Peng, Wanhong Liu, Song Han, Jun Yin, Xiaohua He
Febrile seizures (FS) are the most common seizure disorders in children aged 6 months to 5 years. Children suffering from complex FS have a high risk of developing subsequent temporal lobe epilepsy (TLE). Neuroinflammation is involved in the pathogenesis of FS although the mechanism remains unknown. Our previous study using the Whole Rat Genome Oligo Microarray determined that Dipeptidyl peptidase IV (DPP4) is potentially a related gene in FS rats. In this study, we demonstrated that DPP4 expression was significantly increased at both the protein and mRNA levels after hyperthermia induction...
2017: Bio-medical Materials and Engineering
https://www.readbyqxmd.com/read/28355170/comparing-practice-patterns-between-pediatric-and-general-emergency-medicine-physicians-a-scoping-review
#13
Nnenna O Chime, Jessica Katznelson, Sandeep Gangadharan, Barbara Walsh, Katie Lobner, Linda Brown, Marcie Gawel, Marc Auerbach
OBJECTIVE: Acutely ill infants and children presenting to the emergency department are treated by either physicians with pediatric emergency medicine (PEM) training or physicians without PEM training, a good proportion of which are general emergency medicine-trained physicians (GEDPs). This scoping review identified published literature comparing the care provided to infants and children (≤21 years of age) by PEM-trained physicians to that provided by GEDPs. METHODS: The search was conducted in 2 main steps as follows: (1) initial literature search to identify available literature with evolving feedback from the group while simultaneously deciding search concepts as well as inclusion and exclusion criteria and (2) modification of search concepts and conduction of search using finalized concepts as well as review and selection of articles for final analysis using set inclusion criteria...
April 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28342868/increased-interleukin-1%C3%AE-and-basic-fibroblast-growth-factor-levels-in-the-cerebrospinal-fluid-during-human-herpesvirus-6b-hhv-6b-encephalitis
#14
Masato Tamai, Nobuyuki Kobayashi, Kazuya Shimada, Naomi Oka, Mayumi Takahashi, Akiko Tanuma, Tomohiro Tanemoto, Hiroyuki Namba, Yoshihiro Saito, Yasuyuki Wada, Aikou Okamoto, Hiroyuki Ida, Kazuhiro Kondo
Human herpesvirus 6B (HHV-6B) causes exanthema subitum in infants and is known to be mildly pathogenic. However, HHV-6B infection can induce febrile seizures in a high percentage of patients, and in rare cases, result in encephalitis. We detected higher levels of interleukin (IL)-1β and basic fibroblast growth factor (bFGF) in the cerebrospinal fluid (CFS) of patients with HHV-6B encephalitis when compared to those in patients with non-HHV-6B-induced febrile seizures. In vitro, IL-1β and bFGF enhanced HHV-6B gene expression in infected U373 astrocytes during the initial and maintenance phases of infection, respectively...
March 22, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28336122/familial-acute-necrotizing-encephalopathy-with-ranbp2-mutation-the-first-report-in-northeast-asia
#15
Yun-Jeong Lee, Su-Kyeong Hwang, So Mi Lee, Soonhak Kwon
BACKGROUND: Acute necrotizing encephalopathy (ANE) is a rare but rapidly progressing encephalopathy following a febrile illness, commonly a viral infection. It is characterized by the features of acute encephalopathy such as seizure, alteration of consciousness, and symmetric involvement of the bilateral thalamus on neuroimaging tests. Although most ANE cases have occurred sporadically, familial or recurrent ANE has been reported in Caucasian patients, with genetic susceptibility to ANE noted in some patients due to a RANBP2 mutation...
March 20, 2017: Brain & Development
https://www.readbyqxmd.com/read/28334860/genetic-regulation-of-gene-expression-in-the-epileptic-human-hippocampus
#16
Nasir Mirza, Richard Appleton, Sasha Burn, Daniel du Plessis, Roderick Duncan, Jibril Osman Farah, Bjarke Feenstra, Anders Hviid, Vivek Josan, Rajiv Mohanraj, Arif Shukralla, Graeme J Sills, Anthony G Marson, Munir Pirmohamed
Epilepsy is a serious and common neurological disorder. Expression quantitative loci (eQTL) analysis is a vital aid for the identification and interpretation of disease-risk loci. Many eQTLs operate in a tissue- and condition-specific manner. We have performed the first genome-wide cis-eQTL analysis of human hippocampal tissue to include not only normal (n = 22) but also epileptic (n = 22) samples. We demonstrate that disease-associated variants from an epilepsy GWAS meta-analysis and a febrile seizures (FS) GWAS are significantly more enriched with epilepsy-eQTLs than with normal hippocampal eQTLs from two larger independent published studies...
March 3, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28332711/long-term-white-matter-tract-reorganization-following-prolonged-febrile-seizures
#17
Suresh S Pujar, Kiran K Seunarine, Marina M Martinos, Brian G R Neville, Rod C Scott, Richard F M Chin, Chris A Clark
OBJECTIVE: Diffusion magnetic resonance imaging (MRI) studies have demonstrated acute white matter changes following prolonged febrile seizures (PFS), but their longer-term evolution is unknown. We investigated a population-based cohort to determine white matter diffusion properties 8 years after PFS. METHODS: We used diffusion tensor imaging (DTI) and applied Tract-Based Spatial Statistics for voxel-wise comparison of white matter microstructure between 26 children with PFS and 27 age-matched healthy controls...
March 23, 2017: Epilepsia
https://www.readbyqxmd.com/read/28327685/recurrence-risk-of-febrile-seizures-in-children
#18
J Agrawal, P Poudel, G S Shah, S Yadav, S Chaudhary, S Kafle
BACKGROUND: Identifying children with febrile seizure who are at risk for recurrence is important so that special attention can be given to them. The objective of this study was to identify the risk factors for recurrence of febrile seizures in children. METHODS: This prospective hospital based study was conducted from July 2013 to August 2014 'among children of 6 months to 6 years of age at Bishweshwar Prasad Koirala Institute of Health Sciences (BPKIHS), Nepal...
September 2016: Journal of Nepal Health Research Council
https://www.readbyqxmd.com/read/28325560/long-term-prognosis-of-childhood-absence-epilepsy
#19
C Martínez-Ferrández, E Martínez-Salcedo, C Casas-Fernández, H Alarcón-Martínez, S Ibáñez-Micó, R Domingo-Jiménez
INTRODUCTION: Childhood absence epilepsy (CAE) is considered easily manageable with medication provided that a strict patient classification system is employed. It accounts for 10% of all childhood epilepsy cases starting before the age of 15 and it is most frequent in school-aged girls. The aim of this study is to analyse long-term outcomes of patients diagnosed with CAE according to the Loiseau and Panayiotopoulos criteria and treated during childhood. METHODS: We conducted a retrospective study including 69 patients with CAE who are currently older than 11; data were gathered from medical histories, EEG records, and telephone questionnaires...
March 18, 2017: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/28302661/severe-co-trimoxazole-induced-hypoglycaemia-in-a-patient-with-microscopic-polyangiitis
#20
Thomas Edward Conley, Atif Mohiuddin, Noshaba Naz
A 69-year-old man presented to the emergency department with lower respiratory tract infection and febrile neutropaenia. He was recently discharged following a 50-day hospital stay with newly diagnosed microscopic polyangiitis, complicated by pulmonary haemorrhage and severe renal dysfunction requiring renal replacement therapy, plasma exchange and immunosuppression (cyclophosphamide and methylprednisolone). High risk of pneumocystis pneumonia (PCP) led to an escalation in treatment from prophylactic to therapeutic oral co-trimoxazole, alongside broad-spectrum antibiotics...
March 16, 2017: BMJ Case Reports
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