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benign childhood epilepsy

Laura Escobar Fernández, Alejandra Coccolo Góngora, María Vázquez López, Ana Paloma Polo Arrondo, María Concepción Miranda Herrero, Estíbaliz Barredo Valderrama, Pedro Castro de Castro
INTRODUCTION: Continuous spikes and waves during slow sleep (CSWS) is an EEG pattern that appears during childhood, and is often associated with cognitive impairment. It can appear in the course of epileptic syndromes, as well as in benign epilepsy. The aim of this study is to analyse epidemiological and clinical characteristic of patients with CSWS, in order to describe possible predictive factors in their outcome. METHODS: A retrospective study was conducted on paediatric patients with CSWS treated in a third-level hospital from November 1997 to November 2017...
February 13, 2019: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
José Ferreira Penêda, Sandra Alves, Francisco Monteiro, Inês Sousa Ribeiro, Nuno Barros Lima, Artur Condé
The authors present the case of a 5-year-old girl referred to our institution due to several episodes of nocturnal stridor with ocular retroversion and parental notion of apnea. She has been previously submitted to adenotonsillectomy. Due to symptoms worsening she was referred to our hospital. Here, a nasal fiberoptic endoscopy evaluation was conducted and a diagnosis of laryngomalacia was done. The was submitted to CO2 laser ariepiglotoplasty with symptom improvement after surgery. During a follow-up appointment, parents reported self-limited clonic facial movements at sleep onset...
January 30, 2019: International Journal of Pediatric Otorhinolaryngology
Ye-Hwa Jun, Tae-Hoon Eom, Young-Hoon Kim, Seung-Yun Chung, In-Goo Lee, Jung-Min Kim
BACKGROUND: Several neuroimaging studies have reported neurophysiological alterations in patients with benign childhood epilepsy with centrotemporal spikes (BCECTS). However, reported outcomes have been inconsistent, and the progression of these changes in the brain remains unresolved. Moreover, background electroencephalography (EEG) in cases of BCECTS has not been performed often. METHODS: We investigated background EEG activity changes after six months of oxcarbazepine treatment to better understand the neurophysiological alterations and progression that occur in BCECTS...
January 3, 2019: BMC Neurology
Roberto H Caraballo, Santiago Chacón, Lorena Fasulo, Claudio Bedoya
Absence status epilepticus (ASE) is a prolonged generalized absence seizure that usually lasts for hours and can even last for days. The main symptom is the altered content of consciousness while the patient may be alert and partly responsive. We describe the electroclinical features, treatment, and evolution of three paediatric patients with de novo ASE with an excellent response to valproic acid (VPA). Three paediatric patients presented with non-convulsive status epilepticus and an acute confusional state with impaired consciousness and EEG abnormalities compatible with typical ASE, associated with generalized spike-and-wave paroxysms at 2...
December 1, 2018: Epileptic Disorders: International Epilepsy Journal with Videotape
Anita N Datta, Laura Wallbank, Peter K H Wong
OBJECTIVE: Benign epilepsy of childhood with central temporal spikes (BECTS) and absence epilepsy are common epilepsy syndromes in children with similar age of onset and favorable prognosis. However, the co-existence of the electrocardiogram (EEG) findings of rolandic spike and 3 Hz generalized spike-wave (GSW) discharges is extremely rare, with few cases reported in the literature. Our objective was to characterize the EEG findings of these syndromes in children in our center and review the electro-clinical features...
December 3, 2018: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
Marília Matos, Tiago Bara, Samira Clark, Bianca Simone Zeigelboim, Jair Mendes Marques, Paulo Breno Noronha Liberalesso
PURPOSE: The purpose of this study was to demonstrate the association between benign rolandic epilepsy of childhood (BREC) and central auditory processing disorders (CAPDs) and to test the hypothesis that an early onset of BREC could be associated with more cases of CAPD. METHOD: This study has a retrospective cross-sectional design conducted from January 2006 to January 2016 including 93 patients with BREC and without intellectual disability, dyslexia, and attention-deficit hyperactivity disorders...
December 2018: Epilepsy & Behavior: E&B
N N Zavadenko, A A Kholin, A N Zavadenko, E S Michurina
Speech and language development may be impaired in all forms of epilepsy involving specialized functional areas in the dominant cerebral hemisphere and their connections. The concept of epilepsy-aphasia clinical spectrum was recently proposed, but the notion of aphasia is quite conditional here as many of these patients demonstrate disorders of speech and language development from their infancy. Those forms of epilepsy are considered as continuum from the most severe Landau-Kleffner syndrome (LKS) and epilepsy with continuous spike-and-wave during sleep (CSWS) (also indicating as electrical status epilepticus during sleep - ESES) to intermediate epilepsy-aphasia disorders (with incomplete correspondence to diagnostic criteria of LKS and epilepsy with CSWS)...
2018: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
P Dryżałowski, S Jóźwiak, M Franckiewicz, J Strzelecka
Benign epilepsy with centrotemporal spikes (BECTS) is the most common focal epilepsy of the childhood and also one of the best known. It has a proclivity to start at a particular age and remit spontaneously before adolescence. Majority of patients may avoid long-term treatment, because of the mild course and very good outcome. Only few patients may present cognitive deficits if the proper treatment is not implied. BECTS is a part of heterogeneous group of syndromes that consists of Landau-Kleffner Syndrome (LKS), Continuous Spike-and-Wave during Sleep (CSWS) and Atypical benign partial epilepsy (ABPE)...
September 7, 2018: Neurologia i Neurochirurgia Polska
Min Jeong Han, Sun Jun Kim
BACKGROUND AND PURPOSE: This study is to assess the responsiveness of electroencephalography (EEG) abnormalities and their effects on language ability after initiating different types of antiepileptic therapy in children with newly diagnosed benign epilepsy of childhood with centrotemporal spikes (BECTS). METHODS: The records of patients newly diagnosed with BECTS ( n =120; 69 males) were reviewed retrospectively. The patients were randomly treated with lamotrigine, oxcarbazepine, or topiramate monotherapy, and underwent at least two EEG and standardized language tests...
October 2018: Journal of Clinical Neurology
Manal Salmi, Radu Bolbos, Sylvian Bauer, Marat Minlebaev, Nail Burnashev, Pierre Szepetowski
OBJECTIVE: The epilepsy-aphasia spectrum (EAS) is a heterogeneous group of age-dependent childhood disorders characterized by sleep-activated discharges associated with infrequent seizures and language, cognitive, and behavioral deficits. Defects in the GRIN2A gene, encoding a subunit of glutamate-gated N-methyl-d-aspartate (NMDA) receptors, represent the most important cause of EAS identified so far. Neocortical or thalamic lesions were detected in a subset of severe EAS disorders, and more subtle anomalies were reported in patients with so-called "benign" phenotypes...
October 2018: Epilepsia
Navah Ester Kadish, Thomas Bast, Gitta Reuner, Kathrin Wagner, Hans Mayer, Susanne Schubert-Bast, Gert Wiegand, Karl Strobl, Armin Brandt, Rudolf Korinthenberg, Vera van Velthoven, Andreas Schulze-Bonhage, Josef Zentner, Georgia Ramantani
BACKGROUND: Although the majority of children undergoing epilepsy surgery are younger than 3 yr at epilepsy manifestation, only few actually receive surgical treatment in early childhood. Past studies have, however, suggested that earlier intervention may correlate with superior developmental outcomes. OBJECTIVE: To identify predictors for long-term seizure freedom and cognitive development following epilepsy surgery in the first 3 yr of life and determine the appropriate timing for surgical treatment in this age group...
August 21, 2018: Neurosurgery
Kuriko Kagitani-Shimono, Yoko Kato, Ryuzo Hanaie, Junko Matsuzaki, Junpei Tanigawa, Yoshiko Iwatani, Junji Azuma, Masako Taniike
OBJECTIVE: Benign childhood epilepsy with centrotemporal spikes (BECTS), also known as rolandic epilepsy, has recently been reported to be associated with variable degrees of cognitive dysfunction. Many studies reported poor language ability in children with BECTS compared with healthy control children. To elucidate the harmful effects of BECTS on language cognition, we studied the magnetoencephalographic activity elicited by an auditory language comprehension task. METHODS: The participants (N = 20) included 10 children diagnosed with BECTS (aged 10...
August 14, 2018: Epilepsy & Behavior: E&B
Ge Tan, Fenglai Xiao, Sihan Chen, Haijiao Wang, Deng Chen, Lina Zhu, Da Xu, Dong Zhou, Ling Liu
OBJECTIVES: Spontaneous low-frequency oscillations in different frequency bands have diverse physiological meanings. The amplitude of low-frequency fluctuation (ALFF) and functional connectivity (FC) in different frequency bands in Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS) are unknown and worth exploring. METHOD: Resting-state functional magnetic resonance imaging data were collected in 51 drug-naïve BECTS patients and 76 healthy controls. The ALFF was calculated for the typical (0...
September 2018: Epilepsy Research
Alexander Kc Leung, Kam Lun Hon, Theresa Nh Leung
Background: Febrile seizures are the most common neurologic disorder in childhood. Physicians should be familiar with the proper evaluation and management of this common condition. Objective: To provide an update on the current understanding, evaluation, and management of febrile seizures. Methods: A PubMed search was completed in Clinical Queries using the key terms 'febrile convulsions' and 'febrile seizures'. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews...
2018: Drugs in Context
Isabella Tristano, Francesco Nicita, Giacomo Garone, Fabiana Ursitti, Consuelo Nardone, Valerio Rocchi, Cristiana Alessia Guido, Alberto Spalice
INTRODUCTION: Rolandic epilepsy, also known as benign childhood epilepsy with centrotemporal spikes (BECTS), is one of the most common epileptic syndromes in previously healthy children. Despite what was known about the benignity of this syndrome, there is always more evidence about the involvement of the cognitive functions with different deficits in several domains to be investigated. AIM OF THE STUDY: The aim of our study was to describe prognostic electroencephalogram (EEG) pattern of an adverse cognitive development to recognize patients at higher risk of lasting cognitive deficits that could need antiepileptic drugs (AEDs) or an improved neurocognitive therapy...
September 2018: Epilepsy & Behavior: E&B
Satoru Ikemoto, Shin-Ichiro Hamano, Susumu Yokota, Reiko Koichihara, Yuko Hirata, Ryuki Matsuura
OBJECTIVE: To determine whether the characteristics of scalp-recorded high frequency oscillations, especially ripples, can predict the "atypical forms" of benign epilepsy of childhood with centrotemporal spikes (ABECTS), in BECTS. METHODS: Seven patients with ABECTS and eighteen patients with BECTS underwent electroencephalography (EEG) in the secondary bilateral synchrony (SBS) and non-SBS periods for ABECTS patients. SBS period is that when more than 50% of the interictal epileptiform discharges (IEDs) are bilaterally synchronized...
September 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Q Zeng, Y H Zhang, X L Yang, J Zhang, A J Liu, X Y Liu, Y W Jiang, X R Wu
Objective: To summarize the phenotype of epileptic children with SCN2A mutations. Methods: Epileptic patients who were treated in the Pediatric Department of Peking University First Hospital from September 2006 to October 2017 and detected with SCN2A mutations by targeted next-generation sequencing were enrolled. Clinical manifestations of all patients were analyzed retrospectively. Results: A total of 21 patients (16 boys and 5 girls) with SCN2A mutations were collected. Twenty-one SCN2A mutations were identified...
July 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Ping Qian, Xiaoling Yang, Xiaojing Xu, Xiaoyan Liu, Yuehua Zhang, Zhixian Yang
OBJECTIVE: To detect potential mutations of the glutamate receptor subunit (GRIN2A) gene and delineate the clinical-genetic characteristics of patients with epilepsy-aphasia spectrum (EAS) disorders. METHODS: One hundred twenty two patients with Landau-Kleffner syndrome (LKS), epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS), benign childhood epilepsy with centrotemporal spikes (BECT) and BECT variants were recruited. Potential mutations of the GRIN2A gene were screened with Sanger sequencing...
June 10, 2018: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
Eun Hye Lee, Hoi Soo Yoon
No abstract text is available yet for this article.
August 2018: World Journal of Pediatrics: WJP
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
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