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ESES epilepsy

Iván Sánchez Fernández, Jurriaan M Peters, Alireza Akhondi-Asl, Jacquelyn Klehm, Simon K Warfield, Tobias Loddenkemper
PURPOSE: To test whether patients with Electrical Status Epilepticus in Sleep (ESES) and normal neuroimaging have a smaller thalamic volume than expected for age and for total brain volume. METHODS: Case-control study comparing three groups of subjects of 4-14 years of age and normal magnetic resonance imaging: 1) ESES patients, 2) patients with refractory epilepsy control group, and 3) healthy controls. Thalamic and total brain volumes were calculated using an algorithm for automatic segmentation and parcellation of magnetic resonance imaging...
February 2017: Epilepsy Research
Anna Jeong, Jennifer Strahle, Ananth K Vellimana, David D Limbrick, Matthew D Smyth, Mary Bertrand
OBJECTIVE Electrical status epilepticus of sleep (ESES) is a rare electrographic pattern associated with global regression, which is often poorly responsive to traditional epilepsy treatments and can have a devastating and permanent neurocognitive outcome. The authors analyzed clinical, electroencephalographic, and neuropsychological outcomes in 9 patients with refractory ESES treated with functional hemispherotomy to illustrate the wide clinical spectrum associated with the disease and explore the role of hemispherotomy in its treatment...
January 2017: Journal of Neurosurgery. Pediatrics
Sarah Aminoff Kelley, Eric Heath Kossoff
Electrical status epilepticus of sleep (ESES), with the activation of profuse amounts of epileptiform discharges in sleep, may lead to intractable epilepsy and neurocognitive decline in children. Numerous varied treatments including antiseizure medications, steroids, and surgery have been investigated as possible treatment options. The ketogenic diet (KD) is an additional treatment option which may add to our treatment armamentarium for ESES. The KD may theoretically improve ESES by affecting GABA systems and reducing inflammation...
November 2016: Epilepsy Research
S Boronat, M Vicente, E Lainez, A Sánchez-Montañez, E Vázquez, L Mangado, L Martínez-Ribot, M Del Campo
Fetal alcohol spectrum disorders (FASD) cause neurodevelopmental abnormalities. However, publications about epilepsy and electroencephalographic features are scarce. In this study, we prospectively performed electroencephalography (EEG) and brain magnetic resonance (MR) imaging in 61 patients with diagnosis of FASD. One patient had multiple febrile seizures with normal EEGs. Fourteen children showed EEG anomalies, including slow background activity and interictal epileptiform discharges, focal and/or generalized, and 3 of them had epilepsy...
January 2017: European Journal of Medical Genetics
Deb K Pal, Colin Ferrie, Laura Addis, Tomoyuki Akiyama, Giuseppe Capovilla, Roberto Caraballo, Anne de Saint-Martin, Natalio Fejerman, Renzo Guerrini, Khalid Hamandi, Ingo Helbig, Andreas A Ioannides, Katsuhiro Kobayashi, Dennis Lal, Gaetan Lesca, Hiltrud Muhle, Bernd A Neubauer, Tiziana Pisano, Gabrielle Rudolf, Caroline Seegmuller, Takashi Shibata, Anna Smith, Pasquale Striano, Lisa J Strug, Pierre Szepetowski, Thalia Valeta, Harumi Yoshinaga, Michalis Koutroumanidis
The term idiopathic focal epilepsies of childhood (IFE) is not formally recognised by the ILAE in its 2010 revision (Berg et al., 2010), nor are its members and boundaries precisely delineated. The IFEs are amongst the most commonly encountered epilepsy syndromes affecting children. They are fascinating disorders that hold many "treats" for both clinicians and researchers. For example, the IFEs pose many of the most interesting questions central to epileptology: how are functional brain networks involved in the manifestation of epilepsy? What are the shared mechanisms of comorbidity between epilepsy and neurodevelopmental disorders? How do focal EEG discharges impact cognitive functioning? What explains the age-related expression of these syndromes? Why are EEG discharges and seizures so tightly locked to slow-wave sleep? In the last few decades, the clinical symptomatology and the respective courses of many IFEs have been described, although they are still not widely appreciated beyond the specialist community...
September 1, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
O Bennett-Back, S Uliel-Siboni, U Kramer
BACKGROUND: Video EEG (VEEG) is performed for most pediatric patients in order to evaluate unclear paroxysmal events and improve our understanding of difficult to control epileptic patients. PURPOSE: To characterize the video EEG studies on children who are not candidates for surgery in order to identify the parameters that affect results in level of improving the rate of acquisition, as well as improving the ability to expect the likelihood of epilepsy and of gathering new information as a result of the VEEG...
November 2016: European Journal of Paediatric Neurology: EJPN
Frank Besag, Giuseppe Gobbi, Albert Aldenkamp, Rochelle Caplan, David W Dunn, Matti Sillanpää
The categorisation of the childhood epilepsies into a number of different syndromes has allowed greater insight into the prognosis, not only with regard to seizure control but also in relation to cognitive and behavioural outcome. The role of genetics in determining both the syndrome and the behavioural outcome remains promising, although the promise is still largely unfulfilled. The behavioural/psychiatric outcome of a selection of the large number of childhood epilepsy syndromes is presented. The rate of autism in West syndrome, particularly in children who have tuberous sclerosis with temporal tubers, is high...
May 16, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
Jessica J Falco-Walter, Thomas Bleck
Status epilepticus is the most severe form of epilepsy, with a high mortality rate and high health care costs. Status epilepticus is divided into four stages: early, established, refractory, and super-refractory. While initial treatment with benzodiazepines has become standard of care for early status epilepticus, treatment after benzodiazepine failure (established status epilepticus (ESE)) is incompletely studied. Effective treatment of ESE is critical as morbidity and mortality increases dramatically the longer convulsive status epilepticus persists...
April 25, 2016: Journal of Clinical Medicine
Mar Carreño, Santiago Fernández
Sleep has a strong influence on interictal epileptiform discharges and on epileptic seizures. Interictal epileptiform discharges are activated by sleep deprivation and sleep, and some epilepsies occur almost exclusively during sleep. Treatment of sleep-related epilepsy should take in account the type of epileptic syndrome, the type of seizures, the patient characteristics, and also the pharmacokinetics of the drug. Proper characterization of the epilepsy is essential to choose appropriate antiepileptic drugs...
May 2016: Current Treatment Options in Neurology
Márta Hegyi, Zsuzsa Siegler, András Fogarasi, Péter Barsi, Péter Halász
OBJECTIVES: A retrospective study has been done at the Bethesda Children's Hospital Epilepsy Center with those patients whose EEG records fulfilled in one or more records the criteria of electrical status epilepticus in slow wave sleep (ESES) pattern, occupying at least 75% of NREM sleep with bilateral discharges, and had detailed disease history and long term follow-up data, between 2000 and 2012. PATIENTS AND METHODS--Thirty-three patients (mean 11.1 +/- 4.2 years of age) were studied by 171 sleep EEG records...
January 30, 2016: Ideggyógyászati Szemle
Elena Pavlidis, Guido Rubboli, Marina Nikanorova, Margarethe Sophie Kölmel, Elena Gardella
Encephalopathy with status epilepticus during sleep (ESES) is an age-related disorder characterized by neuropsychological regression, epilepsy and a typical EEG pattern of continuous epileptiform activity (> 85%) during NREM sleep. Cases of worsening or induction of ESES with phenytoin, carbamazepine and phenobarbital have been reported. We describe a child with benign epilepsy with centrotemporal spikes (BECTS) in whom treatment with oxcarbazepine (OXC) induced ESES. The patient was studied through repeated clinical-neuropsychological evaluations and 24-hour EEG recordings...
April 2015: Functional Neurology
Sang-Ahm Lee, Byung-In Lee
PURPOSE: We determined the level of knowledge about epilepsy in Korean people with epilepsy (PWE) and evaluated whether this is associated with self-efficacy, perceived stigma, anxiety, and depressive mood in these patients. METHODS: This was a cross-sectional multicenter study. A total of 530 PWE participated from 31 secondary or tertiary hospitals in Korea. Knowledge about epilepsy was assessed using 34 medical items (EKP-M) of the Epilepsy Knowledge Profile-General...
November 2015: Epilepsy & Behavior: E&B
Michelle Downes, Rebecca Greenaway, Maria Clark, J Helen Cross, Nicola Jolleff, William Harkness, Marios Kaliakatsos, Stewart Boyd, Steve White, Brian G R Neville
OBJECTIVE: To determine whether multiple subpial transection in the posterior temporal lobe has an impact on long-term outcome in children who have drug-resistant Landau-Kleffner syndrome (LKS) or other "electrical status epilepticus during sleep" (ESES)-related regression. Given the wide variability in outcomes reported in the literature, a secondary aim was to explore predictors of outcome. METHODS: The current study includes a surgery group (n = 14) comprising patients who underwent multiple subpial transection of the posterior temporal lobe and a nonsurgery comparison group (n = 21) comprising patients who underwent presurgical investigations for the procedure, but who did not undergo surgery...
November 2015: Epilepsia
Bart van den Munckhof, Violet van Dee, Liora Sagi, Roberto H Caraballo, Pierangelo Veggiotti, Elina Liukkonen, Tobias Loddenkemper, Iván Sánchez Fernández, Marga Buzatu, Christine Bulteau, Kees P J Braun, Floor E Jansen
OBJECTIVE: Epileptic encephalopathy with electrical status epilepticus in sleep (ESES) is a pediatric epilepsy syndrome with sleep-induced epileptic discharges and acquired impairment of cognition or behavior. Treatment of ESES is assumed to improve cognitive outcome. The aim of this study is to create an overview of the current evidence for different treatment regimens in children with ESES syndrome. METHODS: A literature search using PubMed and Embase was performed...
November 2015: Epilepsia
Gabriela Pesántez-Ríos, Antonio Martínez-Bermejo, Joaquín Arcas, Milagros Merino-Andreu, Arturo Ugalde-Canitrot
INTRODUCTION: The development of atypical features in rolandic epilepsy is part of a clinical spectrum of phenotypes that are variable, idiopathic and age-dependent, as well as having a genetically determined predisposition. AIM: To study the electroclinical characteristics suggesting an atypical development in rolandic epilepsy. PATIENTS AND METHODS: A retrospective search was performed in 133 children diagnosed with atypical benign focal epilepsy (ABFE), Landau-Kleffner syndrome and continuous spike-wave during sleep (CSWS)...
August 1, 2015: Revista de Neurologia
Robert T Fraser, Erica K Johnson, Steven Lashley, Jason Barber, Naomi Chaytor, John W Miller, Paul Ciechanowski, Nancy Temkin, Lisa Caylor
OBJECTIVE: Self-management challenges facing adults with epilepsy include limited understanding of the condition and treatment, associated psychosocial issues, and lack of community integration. Self-management interventions improve patients' medical, life role, and emotional management. Previous interventions, developed from expert opinion, indicated issues with participant engagement/retention, and limited follow-up periods. PACES in Epilepsy addressed methodologic concerns by utilizing patient needs assessment data (n = 165) to derive self-management content and program features for evaluation via randomized controlled trial (RCT)...
August 2015: Epilepsia
John D Hixson, Deborah Barnes, Karen Parko, Tracy Durgin, Stephanie Van Bebber, Arianne Graham, Paul Wicks
OBJECTIVE: The study objective was to test whether engaging in an online patient community improves self-management and self-efficacy in veterans with epilepsy. METHODS: The study primary outcomes were validated questionnaires for self-management (Epilepsy Self-Management Scale [ESMS]) and self-efficacy (Epilepsy Self-Efficacy Scale [ESES]). Results were based on within-subject comparisons of pre- and postintervention survey responses of veterans with epilepsy engaging with the PatientsLikeMe platform for a period of at least 6 weeks...
July 14, 2015: Neurology
E Pavlidis, G Rubboli, Marina Nikanorova, Margarethe Sophie Kölmel, Elena Gardella
Encephalopathy with status epilepticus during sleep (ESES) is an age-related disorder characterized by neuropsychological regression, epilepsy and a typical EEG pattern of continuous epileptiform activity (> 85%) during NREM sleep. Cases of worsening or induction of ESES with phenytoin, carbamazepine and phenobarbital have been reported. We describe a child with benign epilepsy with centrotemporal spikes (BECTS) in whom treatment with oxcarbazepine (OXC) induced ESES. The patient was studied through repeated clinical-neuropsychological evaluations and 24-hour EEG recordings...
June 8, 2015: Functional Neurology
Bernhard Schmitt
Sleep and epilepsy have a close relationship. About 20% of patients suffer seizures only during the night, approximately 40% only during the day and approximately 35% during the day and night. In certain epilepsy syndromes, the occurrence of seizures is strongly related to sleep or awakening. Infantile spasms appear predominately on awakening, and hypsarrhythmia is sometimes visible only in sleep. Children with Panayiotopoulos syndrome or benign epilepsy with centrotemporal spikes (BECTS) have seizures mostly when asleep, and in both syndromes interictal spike waves are markedly accentuated in slow wave sleep...
June 2015: Neuropediatrics
Deepa Dash, Teenu Mary Sebastian, Meena Aggarwal, Manjari Tripathi
PURPOSE: This study was conducted to observe the effect of a structured educational program on drug adherence and self-care management in people with epilepsy in a developing country. METHODS: A total of 180 consecutive people with epilepsy were enrolled from the epilepsy clinic of a tertiary care hospital in North India. Out of these, 90 were randomized to the epilepsy health education group and received the educational program and 90 were in the control group and received the standard of care but did not receive any structured educational program...
March 2015: Epilepsy & Behavior: E&B
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