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Epilepsy in childhood

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https://www.readbyqxmd.com/read/28822871/altered-language-network-in-benign-childhood-epilepsy-patients-with-spikes-from-non-dominant-side-a-resting-state-fmri-study
#1
Jiajia Fang, Sihan Chen, Cheng Luo, Qiyong Gong, Dongmei An, Dong Zhou
Benign childhood epilepsy with centrotemporal spikes (BECTS) is one of the most common childhood epilepsy syndromes, and language deficits associated with BECTS have become a hot topic. This study investigated alterations of the language network in BECTS children with spikes from the non-dominant side in comparison with healthy controls. Twenty-three children with BECTS and 20 age-matched healthy controls were enrolled. Region of interest -based whole brain functional connectivity analysis was used to identify the potential differences in the functional connectivity of the Broca's area between the two groups...
August 1, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28812061/behavioral-comorbidities-and-drug-treatments-in-a-zebrafish-scn1lab-model-of-dravet-syndrome
#2
Brian P Grone, Tiange Qu, Scott C Baraban
Loss-of-function mutations in SCN1A cause Dravet syndrome (DS), a catastrophic childhood epilepsy in which patients experience comorbid behavioral conditions, including movement disorders, sleep abnormalities, anxiety, and intellectual disability. To study the functional consequences of voltage-gated sodium channel mutations, we use zebrafish with a loss-of-function mutation in scn1lab, a zebrafish homolog of human SCN1A. Homozygous scn1lab(s552/s552) mutants exhibit early-life seizures, metabolic deficits, and early death...
July 2017: ENeuro
https://www.readbyqxmd.com/read/28811059/an-infant-with-epilepsy-and-recurrent-hemiplegia-due-to-compound-heterozygous-variants-in-atp1a2
#3
Colin Wilbur, Sarah E Buerki, Ilaria Guella, Eric B Toyota, Daniel M Evans, Marna B McKenzie, Anita Datta, Aspasia Michoulas, Shelin Adam, Margot I Van Allen, Tanya N Nelson, Matthew J Farrer, Mary B Connolly, Michelle Demos
BACKGROUND: Pathogenic heterozygous variants in the ATP1A2 gene have most commonly been associated with familial hemiplegic migraine. However, a wide spectrum of phenotypes that include alternating hemiplegia of childhood and epilepsy have been described. PATIENT DESCRIPTION: We describe a boy who presented at age three months with a complex phenotype that included epilepsy, nonepileptic paroxysmal events, and recurrent hemiplegia. Magnetic resonance imaging demonstrated unilateral cortical edema during a severe episode of hemiplegia that was followed by a persistent mild hemiparesis...
June 8, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28808808/ketogenic-diet-versus-gluten-free-casein-free-diet-in-autistic-children-a-case-control-study
#4
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/28802771/dmd-and-west-syndrome
#5
Ruxandra Cardas, Catrinel Iliescu, Nina Butoianu, Andreea Seferian, Svetlana Gataullina, Elena Gargaun, Juliette Nectoux, Thierry Bienvenu, Dana Craiu, Teresa Gidaro, Laurent Servais
Duchenne Muscular Dystrophy (DMD) is the most frequent muscular dystrophy in childhood, with a worldwide incidence of one in 5000 live male births. It is due to mutations in the dystrophin gene leading to absence of full-length dystrophin protein. Central nervous system involvement is well-known in Duchenne Muscular Dystrophy. The multiple dystrophin isoforms expressed in brain have important roles in cerebral development and functioning. The association of Duchenne Muscular Dystrophy with seizures has been reported, and there is a higher prevalence of epilepsy in Duchenne Muscular Dystrophy patients (between 6...
July 19, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28801085/measuring-in-vivo-cerebral-maturation-using-age-related-t2-relaxation-times-at-3t
#6
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
#7
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/28799511/treatment-of-epileptic-encephalopathies
#8
Simona Balestrini, Sanjay M Sisodiya
BACKGROUND: Epileptic encephalopathies represent the most severe epilepsies, with onset in infancy and childhood and seizures continuing in adulthood in most cases. New genetic causes are being identified at a rapid rate. Treatment is challenging and the overall outcome remains poor. Available targeted treatments, based on the precision medicine approach, are currently few. OBJECTIVE: To provide an overview of the treatment of epileptic encephalopathies with known genetic determinants, including established treatment, anecdotal reports of specific treatment, and potential tailored precision medicine strategies...
August 9, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28794080/the-immune-system-in-pediatric-seizures-and-epilepsies
#9
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/28778058/response-to-adrenocorticotropic-in-attention-deficit-hyperactivity-disorder-like-symptoms-in-electrical-status-epilepticus-in-sleep-syndrome-is-related-to-electroencephalographic-improvement-a-retrospective-study
#10
Attila Altunel, Emine Özlem Altunel, Ali Sever
INTRODUCTION: Encephalopathy with electrical status epilepticus in sleep (ESES) syndrome is a rare epilepsy syndrome of childhood that is characterized by sleep-induced epileptiform discharges and problems with cognition or behavior. The neuropsychiatric symptoms in ESES syndrome, among which the ADHD-like symptoms are prominent, bear a close resemblance to symptoms in various developmental disorders. Positive response to adrenocorticotropic hormone (ACTH) is associated with the normalization of the EEG and improvement of neuropsychiatric function...
August 1, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28772100/catastrophic-epilepsies-of-childhood
#11
MacKenzie A Howard, Scott C Baraban
The tragedy of epilepsy emerges from the combination of its high prevalence, impact upon sufferers and their families, and unpredictability. Childhood epilepsies are frequently severe, presenting in infancy with pharmaco-resistant seizures; are often accompanied by debilitating neuropsychiatric and systemic comorbidities; and carry a grave risk of mortality. Here, we review the most current basic science and translational research findings on several of the most catastrophic forms of pediatric epilepsy. We focus largely on genetic epilepsies and the research that is discovering the mechanisms linking disease genes to epilepsy syndromes...
July 25, 2017: Annual Review of Neuroscience
https://www.readbyqxmd.com/read/28762475/behavioral-and-fmri-responses-to-fearful-faces-are-altered-in-benign-childhood-epilepsy-with-centrotemporal-spikes-bcects
#12
Carolina Ciumas, Agathe Laurent, Mani Saignavongs, Faustine Ilski, Julitta de Bellescize, Eleni Panagiotakaki, Karine Ostrowsky-Coste, Alexis Arzimanoglou, Vania Herbillon, Danielle Ibarrola, Philippe Ryvlin
OBJECTIVE: We hypothesized that children with benign childhood epilepsy with centrotemporal spikes (BCECTS) might have altered social cognitive skills and underlying neural networks. METHODS: We studied 13 patients with BCECTS and 11 age-matched controls using event-related functional magnetic resonance imaging (fMRI) with an emotional discrimination task consisting of viewing happy, fearful, scrambled, and neutral faces. Behavioral performance measured during the task was correlated with clinical variables and behavioral ratings...
July 31, 2017: Epilepsia
https://www.readbyqxmd.com/read/28762286/childhood-onset-epileptic-encephalopathy-associated-with-isolated-focal-cortical-dysplasia-and-a-novel-tsc1-germline-mutation
#13
Hannes Hoelz, Eva Coppenrath, Konstanze Hoertnagel, Timo Roser, Moritz Tacke, Lucia Gerstl, Ingo Borggraefe
Tuberous sclerosis complex (TSC) is an autosomal-dominant inheritable neurocutaneous disease due to mutations within the TSC1 and TSC2 genes. Many patients present with West syndrome, a severe epilepsy syndrome characterized by the triad of infantile spasms, an interictal electroencephalogram (EEG) pattern termed hypsarrhythmia (continuous slow activity with an amplitude higher than 300 µV and multiregional spikes/polyspikes/sharp waves) and developmental regression. In this study, we report on a previously healthy patient with positive family history of epilepsy with new-onset epileptic encephalopathy at the age of 9 years...
March 1, 2017: Clinical EEG and Neuroscience: Official Journal of the EEG and Clinical Neuroscience Society (ENCS)
https://www.readbyqxmd.com/read/28754226/febrile-seizures-and-epilepsy-association-with-autism-and-other-neurodevelopmental-disorders-in-the-child-and-adolescent-twin-study-in-sweden
#14
Christopher Gillberg, Sebastian Lundström, Elisabeth Fernell, Gill Nilsson, Brian Neville
BACKGROUND: There is a recently well-documented association between childhood epilepsy and earlysymptomaticsyndromeselicitingneurodevelopmentalclinicalexaminations (ESSENCE) including autism spectrum disorder, but the relationship between febrile seizures and ESSENCE is less clear. METHODS: The Child and Adolescent Twin Study in Sweden (CATSS) is an ongoing population-based study targeting twins born in Sweden since July 1, 1992. Parents of 27,092 twins were interviewed using a validated DSM-IV-based interview for ESSENCE, in connection with the twins' ninth or twelfth birthday...
September 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28747835/non-epileptic-paroxysmal-events-in-childhood
#15
Burak Tatlı, Serhat Güler
Non epileptic paroxysmal events are recurrent movement disorders with acute onset and ending, which may mimic epilepsy. The duration, place, timing of the attacks, and state of conciousness may confuse pediatricians about the diagnosis of epilepsy and non epileptic paroxysmal events. The key point in the diagnosis is taking an accurate and detailed history. Wrong diagnosis can give rise to anxiety of both the family and the child, interruptions in the child's education, limitations in career planning, and irreversible damages in the long term...
June 2017: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/28745663/-differential-diagnosis-of-paroxysms-of-tonic-muscle-tension-in-children-of-early-age-with-delay-of-psychomotor-development-and-abnormal-neurologic-status
#16
M B Mironov, M Yu Bobylova, I V Nekrasova, T M Krasilschikova, M M Gunchenko, M N Sarzhina, A S Petrukhin, S G Burd, T T Batisheva
AIM: To study neurologic status, results of video-EEG monitoring and magnetic resonance imaging in children under 3 years old with paroxysms of tonic muscle tension. MATERIAL AND METHODS: One hundred and forty-six infants and young children with motor disturbances and different variants of clinically similar epileptic seizures, hyperkinesis and stereotypes were examined. RESULTS AND CONCLUSION: Cerebral palsy (91%), genetic and chromosomal abnormalities (6%), brain malformations (2%) were identified...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28740620/use-and-cost-comparison-of-clobazam-to-other-antiepileptic-drugs-for-treatment-of-lennox-gastaut-syndrome
#17
Clément François, John M Stern, Augustina Ogbonnaya, Tasneem Lokhandwala, Pamela Landsman-Blumberg, Amy Duhig, Vivienne Shen, Robin Tan
Background: Lennox-Gastaut syndrome (LGS) is a severe form of childhood-onset epilepsy associated with serious injuries due to frequent and severe seizures. Of the antiepileptic drugs (AEDs) approved for LGS, clobazam is a more recent market entrant, having been approved in October 2011. Recent AED budget impact and cost-effectiveness analyses for LGS suggest that adding clobazam to a health plan formulary may result in decreased medical costs; however, research on clinical and economic outcomes and treatment patterns with these AED treatments in LGS is limited...
2017: Journal of Market Access & Health Policy
https://www.readbyqxmd.com/read/28739361/gender-affects-long-term-neurological%C3%A2-outcome-of-neonates
#18
Amir Freud, Eyal Sheiner, Tamar Wainstock, Daniella Landau, Asnat Walfisch
OBJECTIVE: We evaluated the possible association between fetal gender and long-term pediatric neurological morbidity. METHODS: We performed a population-based retrospective cohort analysis comparing the risk of long-term neurological morbidity (up to age 18 years) of children born during the years 1991 to 2013 according to their gender. Neurological morbidity evaluated included hospitalizations in childhood involving pervasive developmental disorder, obstructive sleep apnea, cerebral palsy, epilepsy, and infantile spasms and disorders of eating as recorded in the hospital files...
September 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28738275/early-childhood-myoclonic-epilepsy-an-independent-genetic-generalized-epilepsy-with-myoclonic-seizures-as-the-main-seizure-type
#19
Zhixian Yang, Jiao Xue, Hui Li, Ping Qian, Xiaoyan Liu, Yuwu Jiang, Yuehua Zhang
OBJECTIVE: To elucidate the characteristics of the myoclonic seizures alone, or predominant myoclonus combined with generalized tonic-clonic seizures (GTCS) and/or absences, in early childhood, and discuss its classification. METHODS: Forty-two children were retrospectively recruited between January 2006 and June 2015. RESULTS: The mean age of seizure onset was 40.5months. They were divided into 4 groups: myoclonic seizures alone; predominant myoclonus combined with GTCS; predominant myoclonus combined with absences; predominant myoclonus combined with both GTCS and absences...
September 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28734769/influence-of-epileptic-activity-during-sleep-on-cognitive-performance-in-benign-childhood-epilepsy-with-centrotemporal-spikes
#20
Andreea Nissenkorn, Adi Pappo, Yael Feldmann, Gali Heimer, Omer Bar-Yosef, Michal Tzadok, Orli Polack, Ayelet Bord, Miriam Levav, Bruria Ben-Zeev
BACKGROUND: Benign childhood epilepsy with centrotemporal spikes is benign childhood epilepsy, presenting between 4 and 10 years of age, characterized by typical clinical and EEG findings. Despite excellent prognosis, there are reports of mild cognitive, language, fine motor and behavioral difficulties. In its atypical form - electrical status epilepticus during slow wave sleep, continuous epileptiform activity during sleep lead to severe neurocognitive deterioration. Our objective was to investigate the influence of abundant sleep epileptiform activity, not fulfilling the criteria for electrical status epilepticus during Slow Wave Sleep, discovered randomly in children without overt intellectual impairment...
July 11, 2017: European Journal of Paediatric Neurology: EJPN
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