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refractory seizure

J M Mercadé Cerdá, F J López Gonzalez, P Serrano Castro, M D Castro Vilanova, D M Campos Blanco, M R Querol Pascual
INTRODUCTION: The study aims to quantify the types of antiepileptic drugs (AED) prescribed in neurology consultations. MATERIAL AND METHOD: This descriptive, observational study included a sample of 559 patients older than 14 years, diagnosed with epilepsy, and receiving pharmacological treatment. Data were collected at outpatient consultations by 47 Spanish neurologists in May 2016. Epilepsy was defined based on the International League Against Epilepsy classification...
March 9, 2018: Neurología: Publicación Oficial de la Sociedad Española de Neurología
Christopher S Parker, Jonathan D Clayden, M Jorge Cardoso, Roman Rodionov, John S Duncan, Catherine Scott, Beate Diehl, Sebastien Ourselin
Patients with medically-refractory focal epilepsy may be candidates for neurosurgery and some may require placement of intracranial EEG electrodes to localise seizure onset. Assessing cerebral responses to single pulse electrical stimulation (SPES) may give diagnostically useful data. SPES produces cortico-cortical evoked potentials (CCEPs), which infer effective brain connectivity. Diffusion-weighted images and tractography may be used to estimate structural brain connectivity. This combination provides the opportunity to observe seizure onset and its propagation throughout the brain, spreading contiguously along the cortex explored with electrodes, or non-contiguously...
2018: NeuroImage: Clinical
David Neubauer, Mirjana Perković Benedik, Damjan Osredkar
PURPOSE: Refractory epilepsies in children present a major burden for patients and their families. Cannabidiol (CBD) has been suggested as a potential treatment for refractory epilepsies. The aim of this study was to evaluate the effectiveness of add-on therapy with CBD for the treatment of refractory childhood epilepsies. METHOD: Patients with childhood-onset refractory epilepsy, treated at the tertiary epilepsy center of the University Children's Hospital Ljubljana, Slovenia, were included in the study...
March 8, 2018: Epilepsy & Behavior: E&B
O Fernández-Concepción, M López Jiménez, C Valencia-Calderón, A Calderón-Valdivieso, A Recasén-Linares, L Reyes-Haro, C Vásquez-Ham
INTRODUCTION: There is sufficient evidence on the usefulness of surgery as a therapeutic alternative for patients with drug-resistant epilepsy; however this treatment is underutilized, especially in developing countries. METHODS: We describe the outcomes of epilepsy surgery in 27 paediatric patients at Hospital Baca Ortiz in Quito, Ecuador. Our analysis considered the following variables: reduction in seizure frequency, surgery outcome according to the Engel classification, improvement in quality of life, and serious complications due to surgery...
March 7, 2018: Neurología: Publicación Oficial de la Sociedad Española de Neurología
Naila Ismayilova, Mary-Anne Leung, Ravi Kumar, Martin Smith, Ruth E Williams
PURPOSE: The Ketogenic Diet (KD) is a well-established treatment for epilepsy in children and adults. We describe our 10-year KD experience in children less than two years of age diagnosed with medically refractory epilepsy. METHODS: We conducted a retrospective case-note review of infants managed with KD at our centre between 2006 and 2016. RESULTS: Twenty-nine children between 2½ weeks and 23 months of age were identified, with mixed epilepsy aetiologies...
March 2, 2018: Seizure: the Journal of the British Epilepsy Association
Karl Roessler, Burkhard S Kasper, Roland Coras, Soheil Arinrad, Michael Scholz, Hajo H Hamer, Ingmar Blümcke, Michael Buchfelder
BACKGROUND: Temporal lobe resection (TLR) including amygdalo-hippocampectomy (AHE) is the most frequent performed procedure in epilepsy surgery. Due to close anatomical relationship of the mesial temporal structures and the midbrain and choroidal fissure, the risk of severe complications like postoperative stroke is up to 2.5%. METHODS: We developed a modification of the classical technique for AHE by early entering the choroidal fissure for identification of crus cerebri, posterior cerebri artery and oculomotor nerve via the anterior part of the fronto- mesial temporal horn cleft...
March 7, 2018: World Neurosurgery
Tommi Nora, Hanna Heinonen, Mirja Tenhunen, Sirpa Rainesalo, Soila Järvenpää, Kai Lehtimäki, Jukka Peltola
Deep brain stimulation (DBS) of the anterior nucleus of the thalamus (ANT) is a method of neuromodulation used for refractory focal epilepsy. We report a patient suffering from drug-resistant epilepsy who developed novel visual symptoms and atypical seizures with the onset of ANT-DBS therapy. Rechallenge under video electroencephalography recording confirmed that lowering the stimulation voltage alleviated these symptoms. Subsequent stimulation with the initial voltage value did not cause the recurrence of either the visual symptoms or the new seizure type, and appeared to alleviate the patient's seizures in long-term follow-up...
2018: Frontiers in Neurology
Stéphane Auvin, Mohamed Jeljeli, Béatrice Desnous, Nadia Soussi-Yanicostas, Pascal Dournaud, Ghislaine Sterkers
Dravet syndrome (DS) is a refractory epileptic syndrome. Vaccination is the trigger of the first seizure in about 50% of cases. Fever remains a trigger of seizures during the course of the disease. We compared ex vivo cytokine responses to a combined aluminium-adjuvanted vaccine of children with DS to sex- and age-matched heathy children. Using ex vivo cytokine responses of peripheral-blood mononuclear cells and monocytes, we found that vaccine responsiveness is biased toward a proinflammatory profile in DS with a M1 phenotype of monocytes...
March 7, 2018: Epilepsia
Manish N Shah, Anish Mitra, Manu S Goyal, Abraham Z Snyder, Jing Zhang, Joshua S Shimony, David D Limbrick, Marcus E Raichle, Matthew D Smyth
PURPOSE: Temporal lobe epilepsy (TLE) affects resting state brain networks in adults. This study aims to correlate resting state functional MRI (rsMRI) signal latency in pediatric TLE patients with their laterality. METHODS: From 2006 to 2016, 26 surgical TLE patients (12 left, 14 right) with a mean age of 10.7 years (range 0.9-18) were prospectively studied. Preoperative rsMRI was obtained in patients with concordant lateralizing structural MRI, EEG, and PET studies...
March 6, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Kevin J Li, Ronald J Gurrera, Lynn E Delisi
Clozapine has been shown to be the most efficacious therapy for treatment resistant schizophrenia, estimated at one third of all schizophrenia cases. There is significant morbidity and mortality associated with clozapine including risk of agranulocytosis, aspiration pneumonia, bowel ischemia, myocarditis, seizures, and weight gain. Here we present a case of a 62-year-old man with chronic paranoid schizophrenia refractory to numerous antipsychotics who was started on clozapine therapy during an acute inpatient psychiatric admission...
March 1, 2018: Schizophrenia Research
Ling-Ling Xie, Xiao-Jie Song, Tian-Yi Li, Li Jiang
We report a case of 14-month-old male monozygotic twins showing early-onset intractable epilepsy, delayed psychomotor development, hypotonia, opisthotonus, and dysmorphism. They presented with refractory partial and secondary generalized tonic-clonic or myoclonic seizures since age of 6 months. Electroencephalograms mainly revealed fast activity in left occipital region and generalized high amplitude polyspikes and wave. Brain MRI was normal. A de novo germline hemizygous mutation, C.110 T > C (p...
March 1, 2018: Brain & Development
Sudhir Datar
Status epilepticus (SE) is a medical emergency and presents with either a continuous prolonged seizure or multiple seizures without full recovery of consciousness in between them. The goals of treatment are prompt recognition, early seizure termination, and simultaneous evaluation for any potentially treatable cause. Improved understanding of the pathophysiology has led to a more practical definition. New data have emerged regarding the safety and efficacy of alternative agents, which are increasingly used in the management of these patients...
April 2018: Neurosurgery Clinics of North America
Scott Mintzer, Robert T Wechsler, Joanne B Rogin, Barry E Gidal, Matthias Schwab, Elinor Ben-Menachem, Mar Carreño, Patrício Soares da Silva, Joana Moreira, Yan Li, David Blum, Todd Grinnell
PURPOSE: To evaluate the effects of eslicarbazepine acetate (ESL) on lipid metabolism and to determine whether reduced statin exposure during ESL therapy has clinical consequences. SUBJECTS AND METHODS: We conducted a post-hoc analysis of pooled data for serum lipids (laboratory values) from three phase III, multicenter, randomized, double-blind, placebo-controlled trials of adjunctive ESL therapy (400, 800, or 1200 mg once daily) in patients with treatment-refractory partial-onset seizures...
February 9, 2018: Epilepsy Research
J Bryan Iorgulescu, Sean Ferris, Ashima Agarwal, Sandro Casavilca Zambrano, D Ashley Hill, Robert Schmidt, Arie Perry
Meningioangiomatosis (MA) is a rare entity characterized by the perivascular spread of meningothelial and fibroblastic cells along the Virchow-Robin spaces of small leptomeningeal and intracortical blood vessels [1,2]. Sporadic cases of MA are thought to represent benign hamartomatous proliferations that are associated with refractory seizures and plaque-like cerebral hemispheric masses, primarily involving the temporal and/or frontal lobes. A distinct subset of MA presents in association with neurofibromatosis type 2 (NF2), where the MA is often multifocal, non-epileptogenic, and accompanied by perilesional glial microhamartomas [3,4]...
March 1, 2018: Neuropathology and Applied Neurobiology
Hank Bink, Madineh Sedigh-Sarvestani, Ivan Fernandez-Lamo, Lohith Kini, Hoameng Ung, Duygu Kuzum, Flavia Vitale, Brian Litt, Diego Contreras
New devices that use targeted electrical stimulation to treat refractory localization-related epilepsy have shown great promise, though it is not well known which targets most effectively prevent the initiation and spread of seizures. To better understand how the brain transitions from healthy to seizing on a local scale, we induced focal epileptiform activity in the visual cortex of five anesthetized cats with local application of the GABAA blocker picrotoxin while simultaneously recording local field potentials on a high-resolution electrocorticography array and laminar depth probes...
February 28, 2018: Journal of Neurophysiology
Suchitra Joshi, Jaideep Kapur
The female reproductive hormones progesterone and estrogen regulate network excitability. Fluctuations in the circulating levels of these hormones during the menstrual cycle cause frequent seizures during certain phases of the cycle in women with epilepsy. This seizure exacerbation, called catamenial epilepsy, is a dominant form of drug-refractory epilepsy in women of reproductive age. Progesterone, through its neurosteroid derivative allopregnanolone, increases γ-aminobutyric acid type-A receptor (GABAR)-mediated inhibition in the brain and keeps seizures under control...
February 23, 2018: Brain Research
Shanika R Samarasekera, Samuel F Berkovic, Ingrid E Scheffer
The aim of this study was to evaluate the efficacy and tolerability of open-label lacosamide in patients with refractory sleep-related hypermotor epilepsy. The study was a case review of eight patients with refractory sleep-related hypermotor epilepsy treated with lacosamide. Seizure diaries compared the mean baseline seizure frequency with the most recent 3 months of follow-up. Five (62.5%) patients were responders, defined as ≥50% reduction in seizure frequency, over a mean duration of exposure of 21.5 months...
February 25, 2018: Journal of Sleep Research
Daniel San-Juan, Carlos Ignacio Sarmiento, Katia Márquez González, José Manuel Orenday Barraza
Transcranial direct current stimulation (tDCS) is a reemerged noninvasive cerebral therapy used to treat patients with epilepsy, including focal cortical dysplasia, with controversial results. We present a case of a 28-year-old female with left frontal cortical dysplasia refractory to antiepileptic drugs, characterized by 10-15 daily right tonic hemi-body seizures. The patient received a total of seven sessions of cathodal tDCS (2 mA, 30 min). The first three sessions were applied over three consecutive days, and the remaining four sessions of tDCS were given each at 2-week intervals...
2018: Frontiers in Neurology
Kazushi Ichikawa, Megumi Tsuji, Yu Tsuyusaki, Moyoko Tomiyasu, Noriko Aida, Tomohide Goto
Gamma-aminobutyric acid transaminase (GABA-T) deficiency is a rare, autosomal recessive disorder characterized by severe psychomotor retardation, early-onset epileptic encephalopathy, intractable seizures, hypotonia, and hyperreflexia. The disease is caused by mutation in the 4-aminobutyrate aminotransferase (ABAT) gene, which encodes an enzyme involved in GABA catabolism. In this chapter, a 10-year follow-up of GABA-T deficiency in a rare case of a long-term survivor patient is discussed. The patient showed a progression of clinical phases with increasing age...
February 25, 2018: JIMD Reports
Sandra Pereira, Mariana Adrião, Mafalda Sampaio, Margarida Ayres Basto, Esmeralda Rodrigues, Laura Vilarinho, Elisa Leão Teles, Isabel Alonso, Miguel Leão
INTRODUCTION: Combined oxidative phosphorylation deficiency 20 (COXPD20) is a mitochondrial respiratory chain complex (RC) disorder, caused by disease-causing variants in the VARS2 gene, which encodes a mitochondrial aminoacyl-tRNA synthetase. Here we describe a patient with fatal mitochondrial encephalopathy caused by a homozygous VARS2 gene missense variant. CASE REPORT: We report the case of a girl, the first child of non-consanguineous and healthy parents, born from an uneventful term pregnancy, who presented, in the neonatal period, major hypotonia and microcephaly...
February 25, 2018: JIMD Reports
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