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https://www.readbyqxmd.com/read/28538134/trial-of-cannabidiol-for-drug-resistant-seizures-in-the-dravet-syndrome
#1
Orrin Devinsky, J Helen Cross, Linda Laux, Eric Marsh, Ian Miller, Rima Nabbout, Ingrid E Scheffer, Elizabeth A Thiele, Stephen Wright
BACKGROUND: The Dravet syndrome is a complex childhood epilepsy disorder that is associated with drug-resistant seizures and a high mortality rate. We studied cannabidiol for the treatment of drug-resistant seizures in the Dravet syndrome. METHODS: In this double-blind, placebo-controlled trial, we randomly assigned 120 children and young adults with the Dravet syndrome and drug-resistant seizures to receive either cannabidiol oral solution at a dose of 20 mg per kilogram of body weight per day or placebo, in addition to standard antiepileptic treatment...
May 25, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28533141/common-and-distinctive-patterns-of-cognitive-dysfunction-in-children-with-benign-epilepsy-syndromes
#2
Dazhi Cheng, Xiuxian Yan, Zhijie Gao, Keming Xu, Xinlin Zhou, Qian Chen
BACKGROUND: Childhood absence epilepsy and benign childhood epilepsy with centrotemporal spikes are the most common forms of benign epilepsy syndromes. Although cognitive dysfunctions occur in children with both childhood absence epilepsy and benign childhood epilepsy with centrotemporal spikes, the similarity between their patterns of underlying cognitive impairments is not well understood. To describe these patterns, we examined multiple cognitive functions in children with childhood absence epilepsy and benign childhood epilepsy with centrotemporal spikes...
January 4, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28529525/melatonin-in-tuberous-sclerosis-complex-analysis-using-modern-mathematical-modeling-methods
#3
Justyna Paprocka, Marek Kijonka, Łukasz Boguszewicz, Maria Sokół
Purpose. The aim of the study was to assess melatonin secretion pattern in children with TSC and to compare it with the secretion patterns in children with and without epilepsy. Material and Methods. Melatonin secretion was measured every three hours using the RIA method in four children with recognized TSC. The parameters of the melatonin secretion models were interpreted and compared with those obtained for the patients with epilepsy (n = 76) and the children from the control, nonepileptic group (n = 36)...
2017: International Journal of Endocrinology
https://www.readbyqxmd.com/read/28526280/an-overview-of-medical-risk-factors-for-childhood-psychosis-implications-for-research-and-treatment
#4
REVIEW
Marianna Giannitelli, Angèle Consoli, Marie Raffin, Renaud Jardri, Douglas F Levinson, David Cohen, Claudine Laurent-Levinson
OBJECTIVE: Psychotic disorders in childhood and early adolescence often progress to chronic schizophrenia, but in many cases there are diagnosable medical and genetic causes or risk factors. We reviewed our clinical experience and the relevant literature to identify these factors and to define their clinical features, appropriate work-up and treatment. METHOD: We reviewed the results of comprehensive medical evaluations of 160 psychotic children and adolescents in our center...
May 16, 2017: Schizophrenia Research
https://www.readbyqxmd.com/read/28523600/sodium-channel-blockers-in-the-treatment-of-epilepsy
#5
Martin J Brodie
Sodium channel blockers have been the mainstay of the pharmacological management of focal and generalised tonic-clonic seizures for more than 70 years. The focus of this paper will be on phenytoin, carbamazepine, lamotrigine, oxcarbazepine, rufinamide, lacosamide and eslicarbazepine acetate. All these antiepileptic drugs have similar efficacy and share similar dose-dependent, adverse effect profiles, although phenytoin, carbamazepine and oxcarbazepine are more likely to cause idiosyncratic reactions than the others...
May 18, 2017: CNS Drugs
https://www.readbyqxmd.com/read/28521267/epileptogenic-high-frequency-oscillations-skip-the-motor-area-in-children-with-multilobar-drug-resistant-epilepsy
#6
Yasushi Iimura, Kevin Jones, Kyoko Hattori, Yushi Okazawa, Atsuko Noda, Kana Hoashi, Yutaka Nonoda, Eishi Asano, Tomoyuki Akiyama, Cristina Go, Ayako Ochi, O Carter Snead, Elizabeth J Donner, James T Rutka, James M Drake, Hiroshi Otsubo
OBJECTIVE: Subtotal hemispherectomy involves the resection of multiple lobes in children with drug-resistant epilepsy, skipping the motor area (MA). We determined epileptogenicity using the occurrence rate (OR) of high-frequency oscillations (HFOs) and the modulation index (MI), demonstrating strength of coupling between HFO and slow wave. We hypothesized that epileptogenicity increased over the multiple lobes but skipped the MA. METHODS: We analyzed 23 children (14 subtotal hemispherectomy; 9 multilobar resections)...
March 24, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28521067/antiepileptic-drugs-for-the-treatment-of-infants-with-severe-myoclonic-epilepsy
#7
REVIEW
Francesco Brigo, Stanley C Igwe, Nicola Luigi Bragazzi
BACKGROUND: This is an updated version of the original Cochrane review published in 2015, Issue 10.Severe myoclonic epilepsy in infants (SMEI), also known as Dravet syndrome, is a rare, refractory form of epilepsy, for which stiripentol (STP) has been recently licensed as add-on therapy. OBJECTIVES: To evaluate the efficacy and tolerability of STP and other antiepileptic drug treatments (including ketogenic diet) for patients with SMEI. SEARCH METHODS: For the latest update we searched the Cochrane Epilepsy Group Specialized Register (20 December 2016), the Cochrane Central Register of Controlled Trials (CENTRAL) via the Cochrane Register of Studies Online (CRSO, 20 December 2016), MEDLINE (Ovid, 1946 to 20 December 2016) and ClinicalTrials...
May 18, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28520630/diagnostic-yield-of-routine-electroencephalography-with-concurrent-video-recording-in-detecting-interictal-epileptiform-discharges-in-relation-to-reasons-for-request-a-prospective-study-of-1-080-video-electroencephalograms
#8
Laia Grau-López, Marta Jiménez, Jordi Ciurans, Sonia Barambio, Alejandra Fumanal, Juan L Becerra
PURPOSE: The aim of this study was to prospectively analyze the sensitivity and specificity of routine electroencephalography with concurrent video recording (vEEG) in relation to the reasons for requesting the test and to investigate when routine vEEG should be requested. METHODS: We prospectively analyzed 1,080 consecutive vEEGs performed between April 2015 and April 2016. The requests for vEEG were classified as requests with a low suspicion of epilepsy (syncope, confusion or delirium, suspicion of psychogenic nonepileptic seizures, and paroxysmal focal neurological deficit) or requests with a high suspicion of epilepsy (first clinical seizure, suspected status epilepticus, follow-up study of a patient with epilepsy, and acute symptomatic seizures)...
May 16, 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28512714/comparison-of-the-diagnostic-accuracy-of-pet-mri-to-pet-ct-acquired-fdg-brain-exams-for-seizure-focus-detection-a-prospective-study
#9
Michael J Paldino, Erica Yang, Jeremy Y Jones, Nadia Mahmood, Andrew Sher, Wei Zhang, Shireen Hayatghaibi, Ramkumar Krishnamurthy, Victor Seghers
BACKGROUND: There is great interest in positron emission tomography (PET)/magnetic resonance (MR) as a clinical tool due to its capacity to provide diverse diagnostic information in a single exam. OBJECTIVE: The goal of this exam is to compare the diagnostic accuracy of PET/MR-acquired [F-18]2-fluoro-2-deoxyglucose (FDG) brain exams to that of PET/CT with respect to identifying seizure foci in children with localization-related epilepsy. MATERIALS AND METHODS: Institutional Review Board approval and informed consent were obtained for this Health Insurance Portability and Accountability Act-compliant, prospective study...
May 16, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28511630/corpus-callosotomy-for-intractable-epilepsy-revisited-the-children-s-hospital-of-michigan-series
#10
Aimee F Luat, Eishi Asano, Ajay Kumar, Harry T Chugani, Sandeep Sood
Corpus callosotomy is a palliative procedure performed to reduce the severity of drug-resistant epilepsy. The authors assessed its efficacy on different seizure types in 20 subjects (age range 5-19 years); 8 with active vagus nerve stimulator. Fifteen had complete callosotomy, 3 had anterior 2/3, and 2 had anterior 2/3 followed later by complete callosotomy. Ten had endoscopic approach. In all, 65% had ≥ 50% reduction of generalized seizures leading to falls (atonic, tonic, myoclonic); 35% became seizure-free (follow-up period: 6 months to 9 years; mean 3 years)...
June 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28511603/clinical-experience-with-perampanel-for-refractory-pediatric-epilepsy-in-one-canadian-center
#11
Anita N Datta, Qi Xu, Shafina Sachedina, Cyrus Boelman, Linda Huh, Mary B Connolly
Perampanel (PER) is a new antiseizure medication that inhibits the α-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid (AMPA) class of glutamate receptors. It is important for physicians to be aware of the efficacy and tolerability of new drugs in the postmarketing phase. We performed a retrospective review of our experience with perampanel at BC Children's Hospital. Twenty-four pediatric patients prescribed perampanel from 2014 to 2016 were identified. Fifteen (63%) discontinued perampanel, and 10 (42%) had greater than 50% reduction in seizures...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28506347/-association-between-autism-spectrum-disorder-and-epilepsy-in-children
#12
Song-Li Mei, Zhao Zhang, Xin Liu, Ting-Ting Gao, Xin-Xian Peng
OBJECTIVE: To examine the association between autism spectrum disorder (ASD) and epilepsy in children. METHODS: A total of 190 children with ASD were enrolled. A self-designed questionnaire, Childhood Autism Rating Scale, and Autism Behavior Checklist were used to determine the association between ASD and epilepsy. RESULTS: Among the 190 children with ASD, 20 (10.5%) had epileptic seizures and 12 (6.3%) were diagnosed with epilepsy. The rates of abnormal physical development and hearing disorders before the age of one year were significantly higher in ASD children with epileptic seizures than in those without epileptic seizures (P<0...
May 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28506334/-mecp2-duplication-syndrome-a-clinical-analysis-of-three-cases-and-literature-review
#13
Dan-Xia Tang, Dong-Fang Li, Ruo-Hao Wu, Li-Na Zhang, Xiang-Yang Luo
MECP2 duplication syndrome (MDS) is a rare pediatric disease and mainly manifests as delayed motor development, language loss or delay, recurrent infection, severe intellectual disability, epilepsy, autistic symptoms, and early infantile hypotonia. In this article, the three children with this disease were all boys. Cases 1 and 2 had delayed motor development, and language loss or delay as initial manifestations, and case 3 had recurrent infection as initial manifestation. Physical examination showed hypotonia and negative pathological signs in each case...
May 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28503985/diagnostic-and-therapeutic-management-of-a-first-unprovoked-seizure-in-children-and-adolescents-with-a-focus-on-the-revised-diagnostic-criteria-for-epilepsy
#14
Arnold J Sansevere, Jennifer Avalone, Lauren Doyle Strauss, Archana A Patel, Anna Pinto, Maya Ramachandran, Ivan Sanchez Fernandez, Ann M Bergin, Amir Kimia, Phillip L Pearl, Tobias Loddenkemper
By definition, unprovoked seizures are not precipitated by an identifiable factor, such as fever or trauma. A thorough history and physical examination are essential to caring for pediatric patients with a potential first unprovoked seizure. Differential diagnosis, EEG, neuroimaging, laboratory tests, and initiation of treatment will be reviewed. Treatment is typically initiated after 2 unprovoked seizures, or after 1 seizure in select patients with distinct epilepsy syndromes. Recent expansion of the definition of epilepsy by the ILAE allows for the diagnosis of epilepsy to be made after the first seizure if the clinical presentation and supporting diagnostic studies suggest a greater than 60% chance of a second seizure...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28501751/the-state-of-everyday-quantitative-eeg-use-in-canada-a-national-technologist-survey
#15
Marcus C Ng, Kara Gillis
PURPOSE: This study sought to determine the state of quantitative EEG (QEEG) use in Canada, as QEEG may provide a partial solution to the issue of escalating EEG demand against insufficient health care resources. METHODS: A 10-item survey questionnaire was administered to participants at the annual meeting of the Canadian Association of Electroneurophysiology Technologists, which was held in parallel with the annual meeting of the Canadian Neurological Sciences Federation...
May 5, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28491900/screening-of-conventional-anticonvulsants-in-a-genetic-mouse-model-of-epilepsy
#16
Nicole A Hawkins, Lyndsey L Anderson, Tracy S Gertler, Linda Laux, Alfred L George, Jennifer A Kearney
OBJECTIVE: Epilepsy is a common neurological disorder that affects 1% of the population. Approximately, 30% of individuals with epilepsy are refractory to treatment, highlighting the need for novel therapies. Conventional anticonvulsant screening relies predominantly on induced seizure models. However, these models may not be etiologically relevant for genetic epilepsies. Mutations in SCN1A are a common cause of Dravet Syndrome, a severe epileptic encephalopathy. Dravet syndrome typically begins in infancy with seizures provoked by fever and then progresses to include afebrile pleomorphic seizure types...
May 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/28490751/unexpected-efficacy-of-a-novel-sodium-channel-modulator-in-dravet-syndrome
#17
Lyndsey L Anderson, Nicole A Hawkins, Christopher H Thompson, Jennifer A Kearney, Alfred L George
Dravet syndrome, an epileptic encephalopathy affecting children, largely results from heterozygous loss-of-function mutations in the brain voltage-gated sodium channel gene SCN1A. Heterozygous Scn1a knockout (Scn1a (+/-)) mice recapitulate the severe epilepsy phenotype of Dravet syndrome and are an accepted animal model. Because clinical observations suggest conventional sodium channel blocking antiepileptic drugs may worsen the disease, we predicted the phenotype of Scn1a (+/-) mice would be exacerbated by GS967, a potent, unconventional sodium channel blocker...
May 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28489764/mutual-associations-between-intellectual-disability-and-epilepsy-related-psychiatry-disability-population-based-study
#18
Zhenjie Wang, Chao Guo, Gong Chen, Lei Zhang, Xun Wen, Xiaoying Zheng
Epilepsy is the third-leading cause of psychiatry disability in China, and intellectual disability (ID) is also 1 major type of disabilities in China. This study estimates the prevalence of comorbidities with ID and epilepsy-related psychiatry disability (EPD) and examines mutual associations within ID and EPD.Data were taken from the Second China National Sample Survey on Disability, which was a nationally representative, population-based survey. To derive a nationally representative sample, the survey used multistage, stratified, cluster random sampling with probability proportional to size...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28489143/the-use-of-a-formula-based-ketogenic-diet-in-children-with-refractory-epilepsy
#19
Letícia Pereira de Brito Sampaio, Cristina Takakura, Maria Luiza Giraldes de Manreza
The ketogenic diet (KD) is a nonpharmacologic treatment that has been used for refractory epilepsy since 1921. The KD is a high-fat, low-carbohydrate, and restricted protein diet, which is calculated and weighed for each individual patient. Introducing and maintaining the diet for a long time remains a challenge. In this study, we evaluated the acceptability, tolerance, and efficacy of a formula-based KD in 10 children with refractory epilepsy. The ketogenic formula tested herein caused only mild KD-related adverse events and adequate adherence...
April 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/28488681/practices-and-views-of-neurologists-regarding-the-use-of-whole-genome-sequencing-in-clinical-settings-a-web-based-survey
#20
Iris Jaitovich Groisman, Thierry Hurlimann, Amir Shoham, Béatrice Godard
The use of Whole-Genome Sequencing (WGS) in clinical settings has brought up a number of controversial scientific and ethical issues. The application of WGS is of particular relevance in neurology, as many conditions are difficult to diagnose. We conducted a worldwide, web-based survey to explore neurologists' views on the benefits of, and concerns regarding, the clinical use of WGS, as well as the resources necessary to implement it. Almost half of the 204 neurologists in the study treated mostly adult patients (48%), while the rest mainly children (37...
May 10, 2017: European Journal of Human Genetics: EJHG
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