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benign childhood epilepsy

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https://www.readbyqxmd.com/read/29774303/myotonia-permanens-with-nav1-4-g1306e-displays-varied-phenotypes-during-course-of-life
#1
Frank Lehmann-Horn, Adele D'Amico, Enrico Bertini, Mauro Lomonaco, Luciano Merlini, Kevin R Nelson, Heike Philippi, Gabriele Siciliano, Frank Spaans, Karin Jurkat-Rott
Introduction: Myotonia permanens due to Nav1.4-G1306E is a rare sodium channelopathy with potentially life-threatening respiratory complications. Our goal was to study phenotypic variability throughout life. Methods: Clinical neurophysiology and genetic analysis were performed. Using existing functional expression data we determined the sodium window by integration. Results: In 10 unrelated patients who were believed to have epilepsy, respiratory disease or Schwartz-Jampel syndrome, we made the same prima facie diagnosis and detected the same heterologous Nav1...
September 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/29730501/language-skills-in-children-with-benign-childhood-epilepsy-with-centrotemporal-spikes-a-systematic-review
#2
REVIEW
Joana Teixeira, Maria Emília Santos
Benign childhood epilepsy with centrotemporal spikes (BECTS) is the epileptic syndrome that most affects preschool and school-age children. Despite being usually considered a benign condition, several studies have shown that this epileptic syndrome is responsible for cognitive morbidities in children, namely at the level of attention and memory, as well as language. However, language disorders are still superficially addressed by the literature. This review aimed to compile and synthesize recent literature in this area...
May 3, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29720203/detection-rate-of-causal-variants-in-severe-childhood-epilepsy-is-highest-in-patients-with-seizure-onset-within-the-first-four-weeks-of-life
#3
David Staněk, Petra Laššuthová, Katalin Štěrbová, Markéta Vlčková, Jana Neupauerová, Marcela Krůtová, Pavel Seeman
BACKGROUND: Epilepsy is a heterogeneous disease with a broad phenotypic spectrum and diverse genotypes. A significant proportion of epilepsies has a genetic aetiology. In our study, a custom designed gene panel with 112 genes known to be associated with epilepsies was used. In total, one hundred and fifty-one patients were tested (86 males / 65 females). RESULTS: In our cohort, the highest probability for the identification of the cause of the disease was for patients with a seizure onset within the first four weeks of life (61...
May 2, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29649723/the-relevance-of-attention-deficit-hyperactivity-disorder-in-self-limited-childhood-epilepsy-with-centrotemporal-spikes
#4
Ellen Marise Lima, Patricia Rzezak, Bernardo Dos Santos, Letícia Gentil, Maria A Montenegro, Marilisa M Guerreiro, Kette D Valente
In this study, we aimed to evaluate the attentional and executive functions in patients with benign childhood epilepsy with centrotemporal spikes (BCECTS) with and without attention-deficit hyperactivity disorder (ADHD) compared with controls and compared with patients with ADHD without epilepsy. We evaluated 12 patients with BCECTS and ADHD (66.7% boys; mean age of 9.67years); 11 children with non-ADHD BCECTS (63.6% boys; mean age of 11.91years); 20 healthy children (75% boys; mean age of 10.15years); and 20 subjects with ADHD without epilepsy (60% boys; mean age of 10...
April 9, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29629540/role-of-language-related-functional-connectivity-in-patients-with-benign-childhood-epilepsy-with-centrotemporal-spikes
#5
Hyeon Jin Kim, Jung Hwa Lee, Chang Hyun Park, Hye Sun Hong, Yun Seo Choi, Jeong Hyun Yoo, Hyang Woon Lee
BACKGROUND AND PURPOSE: Benign childhood epilepsy with centrotemporal spikes (BECTS) does not always have a benign cognitive outcome. We investigated the relationship between cognitive performance and altered functional connectivity (FC) in the resting-state brain networks of BECTS patients. METHODS: We studied 42 subjects, comprising 19 BECTS patients and 23 healthy controls. Cognitive performance was assessed using the Korean version of the Wechsler Intelligence Scale for Children-III, in addition to verbal and visuospatial memory tests and executive function tests...
January 2018: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/29610055/language-function-in-childhood-idiopathic-epilepsy-syndromes
#6
D C Jackson, J E Jones, D A Hsu, C E Stafstrom, J J Lin, D Almane, M A Koehn, M Seidenberg, B P Hermann
PURPOSE: To examine the impact of diverse syndromes of focal and generalized epilepsy on language function in children with new and recent onset epilepsy. Of special interest was the degree of shared language abnormality across epilepsy syndromes and the unique effects associated with specific epilepsy syndromes. METHODS: Participants were 136 youth with new or recent-onset (diagnosis within past 12 months) epilepsy and 107 healthy first-degree cousin controls...
March 30, 2018: Brain and Language
https://www.readbyqxmd.com/read/29602081/juvenile-myoclonic-epilepsy-refractory-to-treatment-in-a-tertiary-referral-center
#7
Gonçalo Cação, Joana Parra, Shahidul Mannan, Sanjay M Sisodiya, Josemir W Sander
INTRODUCTION: Juvenile myoclonic epilepsy (JME) is an epileptic syndrome often regarded as one in which seizures are relatively easy to control. Individuals with JME, however, often require lifelong therapy to remain seizure-free, and a few have refractory epilepsy. We ascertained a population with JME and characterized a subgroup with refractory epilepsy. MATERIAL AND METHODS: We audited and reviewed clinical records of individuals diagnosed with JME identified via a sample of 6600 individuals in a clinical database from a specialized epilepsy clinic at a tertiary referral center...
March 26, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29593509/tantrums-emotion-reactions-and-their-eeg-correlates-in-childhood-benign-rolandic-epilepsy-vs-complex-partial-seizures-exploratory-observations
#8
Michael Potegal, Elena H Drewel, John T MacDonald
We explored associations between EEG pathophysiology and emotional/behavioral (E/B) problems of children with two types of epilepsy using standard parent questionnaires and two new indicators: tantrums recorded by parents at home and brief, emotion-eliciting situations in the laboratory. Children with Benign Rolandic epilepsy (BRE, N = 6) reportedly had shorter, more angry tantrums from which they recovered quickly. Children with Complex Partial Seizures (CPS, N = 13) had longer, sadder tantrums often followed by bad moods...
2018: Frontiers in Behavioral Neuroscience
https://www.readbyqxmd.com/read/29571057/panayiotopoulos-syndrome-and-benign-partial-epilepsy-with-centro-temporal-spikes-a-comparative-incidence-study
#9
Elaine Weir, John Gibbs, Richard Appleton
PURPOSE: To compare the de novo incidence of Panayiotopoulos syndrome (PS, early-onset childhood occipital epilepsy) and a common epilepsy syndrome, benign epilepsy with centro-temporal spikes (BECTS), in children and young people. METHODS: The incidence of PS and BECTS was recorded over 16 months in a population of children and young people (aged <16 years) living within a specific geographic area and epilepsy network within the North West of England and North Wales and the catchment area of the tertiary paediatric epilepsy centre...
April 2018: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/29475094/effects-of-levetiracetam-and-sulthiame-on-eeg-in-benign-epilepsy-with-centrotemporal-spikes-a-randomized-controlled-trial
#10
Moritz Tacke, Ingo Borggraefe, Lucia Gerstl, Florian Heinen, Katharina Vill, Michaela Bonfert, Thomas Bast, Bernd Axel Neubauer, Friedrich Baumeister, Martina Baethmann, Karl Bentele, Christian Blank, Harald M Blank, Harald Bode, Friedrich Bosch, Ulrich Brandl, Knut Brockmann, Peter Dahlem, Jan-Peter Ernst, Evemarie Feldmann, Andreas Fiedler, Michael Gerigk, Soeren Heß, Christiane Hikel, Hans-Georg Hoffmann, Matthias Kieslich, Joerg Klepper, Gerhard Kluger, Hartmut Koch, Walter Koch, Rudolf Korinthenberg, Ilona Krois, Hermann Kühne, Gerhard Kurlemann, Michaela Mandl, Ulrike Mause, Peter Navratil, Joachim Opp, Johann Penzien, Viola Prietsch, Axel Quattländer, Dietz Rating, Ulrike Schara, Mohammed G Shamdeen, Andreas Sprinz, Hildegard Wendker-Magrabi, Ulrich Stephani, Hiltrud Muhle, Hans-Michael Straßburg, Bärbel Töpke, Regina Trollmann, Elisabeth Tuschen-Hofstätter, Stephan Waltz, Gabriele Weber, Frank U Wien, Markus Wolff, Tilman Polster, Hedwig Freitag, Ötzcam Sönmez, Klaus Reinhardt, Marion Traus, Zeecam Hoovey
PURPOSE: BECTS (benign childhood epilepsy with centrotemporal spikes) is associated with characteristic EEG findings. This study examines the influence of anti-convulsive treatment on the EEG. METHODS: In a randomized controlled trial including 43 children with BECTS, EEGs were performed prior to treatment with either Sulthiame or Levetiracetam as well as three times under treatment. Using the spike-wave-index, the degree of EEG pathology was quantified. The EEG before and after initiation of treatment was analyzed...
March 2018: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/29467710/structural-covariance-network-of-cortical-gyrification-in-benign-childhood-epilepsy-with-centrotemporal-spikes
#11
Lin Jiang, Tijiang Zhang, Fajin Lv, Shiguang Li, Heng Liu, Zhiwei Zhang, Tianyou Luo
Benign childhood epilepsy with centrotemporal spikes (BECTS) is associated with cognitive and language problems. According to recent studies, disruptions in brain structure and function in children with BECTS are beyond a Rolandic focus, suggesting atypical cortical development. However, previous studies utilizing surface-based metrics (e.g., cortical gyrification) and their structural covariance networks at high resolution in children with BECTS are limited. Twenty-six children with BECTS (15 males/11 females; 10...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29453776/syndromic-sebaceous-nevus-current-findings
#12
Oumama El Ezzi, Anthony S de Buys Roessingh, Michèle Bigorre, Guillaume Captier
BACKGROUND: Sebaceous nevus is a congenital malformation of the skin that usually occurs on the scalp or face. Syndromic forms do rarely exist with associated cerebral and ocular malformations. The skin lesions are pale at birth and become irregular by puberty. In the adult patient, tumors (usually benign) develop from sebaceous nevus. Their surgical excision during childhood can give a better result in terms of the definitive scar. OBJECTIVES: The aim of this study is to analyze our cases of syndromic sebaceous hamartoma, perform a review of the existing literature, and propose guidelines for the therapeutic plan...
May 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29432243/epilepsy-syndromes-in-childhood
#13
Phillip L Pearl
PURPOSE OF REVIEW: Epilepsy syndromes are an important clinical construct in pediatric epilepsy, as they encompass recognizable patterns seen in patients with epilepsies, whether of the more benign variety or associated with encephalopathy. RECENT FINDINGS: Syndromes may be organized by age of onset: neonatal, infantile, childhood, or adolescent. The assignment of a syndrome has specific implications for diagnosis, management, and prognostication. The 2010 revised classification of the epilepsies by the International League Against Epilepsy preserved the syndrome approach, while progress in genetics continues to advance our understanding of the pathophysiology and overlap of the epilepsy syndromes...
February 2018: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29398625/brain-responses-to-auditory-oddball-task-in-children-with-benign-childhood-epilepsy-with-centrotemporal-spikes-quantitative-analysis-and-correlation-with-neuropsychological-assessment-scores
#14
Mostafa M Elkholy, Asmaa M Ebraheim, Neveen M ElFayoumy
OBJECTIVE: Variable degrees of cognitive dysfunction have been reported in children with benign childhood epilepsy with centrotemporal spikes (BCECTS). Our aim was to perform quantitative analyses of the brain responses to cognitive tasks using event-related desynchronization (ERD) and event-related synchronization (ERS) and correlating the results with the scores of neuropsychological tests in patients with BCECTS. METHODS: This case control study included 30 patients with BCECTS and 20 controls...
March 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29295801/prevalence-of-idiopathic-epilepsy-among-school-children-in-gharbia-governorate-egypt
#15
Azza Kamal Alshahawy, Amira Hamed Darwish, Safynaz Elsaid Shalaby, Wegdan Mawlana
BACKGROUND: Epilepsy is one of the most common neurological disorders among children. Data about its prevalence in Egypt is limited. Our aim was to study the prevalence of idiopathic epilepsy among school children in Gharbia governorate, Egypt. SUBJECTS AND METHODS: A Cross-sectional school-based survey study was conducted; a validated screening questionnaire was distributed among urban and rural primary and preparatory school children. Students with suspected epilepsy were subjected to clinical evaluation, Electroencephalogram (EEG), and neuroimaging...
April 2018: Brain & Development
https://www.readbyqxmd.com/read/29229893/prognostic-factors-for-epilepsy-following-first-febrile-seizure-in-saudi-children
#16
Abdullah I Almojali, Anwar E Ahmed, Muhammed Y Bagha
BACKGROUND: Febrile seizure is the most common convulsive event during childhood, but it is generally considered benign. OBJECTIVES: To estimate the rate of epilepsy after first presentation of febrile seizure and to describe factors that can predispose children to have subsequent epilepsy after their first febrile seizure. DESIGN: Retrospective chart review. SETTING: A tertiary care center in Riyadh. PATIENTS AND METHODS: All children whose first febrile seizure developed between 2009-2012, and who were admitted to the pediatric wards...
November 2017: Annals of Saudi Medicine
https://www.readbyqxmd.com/read/29213044/-the-course-and-development-of-epilepsy-in-patients-with-typical-variant-of-rett-syndrome-and-mutations
#17
M Yu Bobylova, I V Ivanova, I V Nekrasova, O A Pylaeva, K Yu Mukhin, A A Kholin, Ek S Iljina, A V Kulikov, El S Iljina, Yu E Nesterovsky
AIM: To study the anamnesis, clinical state, electro-encephalographic and brain MRI characteristics in patients with Rett syndrome (МЕСР2) and epilepsy. MATERIAL AND METHODS: Eleven female patients, aged from 3 to 23 years, with Rett syndrome and MeCP2 mutations were studied. The study continued for 10 years (2006-2015). Assessment of neurological and mental status, night sleep video-EEG monitoring, MRI were performed. RESULTS AND CONCLUSION: Epilepsy was diagnosed in six cases (54...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/29208197/-treatment-of-rolandic-epilepsy
#18
Maria Jose Miranda, Banoo Bakir Ahmad
Recent literature indicates, that rolandic epilepsy/epilepsy of childhood with centrotemporal spikes may not be as benign as previously assumed. This study investigates the existing evidence, which describes the treatment effects on seizure frequency as well as improvement of cognition in children with rolandic epilepsy. We conclude, that treatment with anti-epileptic drugs could be justified, if treatment reduces seizures, prevents the evolution to atypical forms, or diminishes the negative cognitive consequences associated with the disease...
November 27, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/29106865/benign-rolandic-epilepsy-presenting-like-paradoxical-vocal-fold-motion
#19
Jennifer H Gross, Mary Bertrand, Keiko Hirose
Paradoxical vocal fold motion (PVFM) is characterized by vocal fold adduction during respiration. Benign Rolandic epilepsy (BRE) is the most common childhood epilepsy and can cause oropharyngolaryngeal or facial manifestations. A 9-year-old male presented with intermittent apnea lasting 30-60 seconds and presumed PVFM. The patient's physical and fiberoptic exam were normal. He was admitted and found to have episodes of oxygen desaturation, neck twitching, and tongue burning. An EEG revealed focal epilepsy. After starting anti-epileptic medications, he had resolution of symptoms...
November 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29106684/frontal-lobe-epilepsy-surgery-in-childhood-and-adolescence-predictors-of-long-term-seizure-freedom-overall-cognitive-and-adaptive-functioning
#20
Georgia Ramantani, Navah Ester Kadish, Hans Mayer, Constantin Anastasopoulos, Kathrin Wagner, Gitta Reuner, Karl Strobl, Susanne Schubert-Bast, Gert Wiegand, Armin Brandt, Rudolf Korinthenberg, Irina Mader, Vera van Velthoven, Josef Zentner, Andreas Schulze-Bonhage, Thomas Bast
BACKGROUND: Although frontal lobe resections account for one-third of intralobar resections in pediatric epilepsy surgery, there is a dearth of information regarding long-term seizure freedom, overall cognitive and adaptive functioning. OBJECTIVE: To identify outcome predictors and define the appropriate timing for surgery. METHODS: We retrospectively analyzed the data of 75 consecutive patients aged 10.0 ± 4.9 yr at surgery that had an 8...
July 5, 2017: Neurosurgery
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