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New onset epilepsy

Zachary Niday, Anastasios V Tzingounis
Exome and targeted sequencing have revolutionized clinical diagnosis. This has been particularly striking in epilepsy and neurodevelopmental disorders, for which new genes or new variants of preexisting candidate genes are being continuously identified at increasing rates every year. A surprising finding of these efforts is the recognition that gain of function potassium channel variants are actually associated with certain types of epilepsy, such as malignant migrating partial seizures of infancy or early-onset epileptic encephalopathy...
March 1, 2018: Neuroscientist: a Review Journal Bringing Neurobiology, Neurology and Psychiatry
Timothée Proix, Viktor K Jirsa, Fabrice Bartolomei, Maxime Guye, Wilson Truccolo
Recent studies have shown that seizures can spread and terminate across brain areas via a rich diversity of spatiotemporal patterns. In particular, while the location of the seizure onset area is usually invariant across seizures in an individual patient, the source of traveling (2-3 Hz) spike-and-wave discharges during seizures can either move with the slower propagating ictal wavefront or remain stationary at the seizure onset area. Furthermore, although many focal seizures terminate synchronously across brain areas, some evolve into distinct ictal clusters and terminate asynchronously...
March 14, 2018: Nature Communications
S Liyanagedera, R P Williams
The International League Against Epilepsy Classification of the Epilepsies, first presented in 1981, has been widely adopted across the globe. In 2017 it was revised to allow for more robust, specific, flexible and logical classification of seizures. A number of new seizure types are recognised. Classification should be timely as it plays a vital role in the diagnosis and management of patients with epilepsy. Accurate classification also underpins epilepsy research from pathophysiology to public health. Here we review the basic and extended forms of the classification...
December 2017: Journal of the Royal College of Physicians of Edinburgh
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
Marcel Heers, Moritz Helias, Tanguy Hedrich, Matthias Dümpelmann, Andreas Schulze-Bonhage, Tonio Ball
The foremost aim of presurgical epilepsy evaluation is the delineation of the seizure onset zone (SOZ). There is increasing evidence that fast epileptic activity (FEA, 14-250 Hz) occurring interictally, i.e. between seizures, is predominantly localized within the SOZ. Currently it is unknown, which frequency band of FEA performs best in identifying the SOZ, although prior studies suggest highest concordance of spectral changes with the SOZ for high frequency changes. We suspected that FEA reflects dampened oscillations in local cortical excitatory-inhibitory neural networks, and that interictal FEA in the SOZ is a consequence of reduced oscillatory damping...
2018: NeuroImage: Clinical
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
Nicolas Gaspard, Lawrence J Hirsch, Claudine Sculier, Tobias Loddenkemper, Andreas van Baalen, Judette Lancrenon, Michel Emmery, Nicola Specchio, Raquel Farias-Moeller, Nora Wong, Rima Nabbout
We report the proceedings of the First International new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) Symposium. To promote awareness of this condition and foster research efforts, we conveyed the First International new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) Symposium. The conference was supported by The NORSE Institute ( This article summarizes the discussions that were held during the Symposium and presents our strategy to unravel the cause of these disorders and to improve patient care...
February 24, 2018: Epilepsia
Rui-Juan Lv, Hai-Tao Ren, Hong-Zhi Guan, Tao Cui, Xiao-Qiu Shao
Objective: The purpose of this study is to analyze the seizure semiologic characteristics of patients with autoimmune epilepsy (AE) and describe the investigation characteristics of AE using a larger sample size. Methods: This observational retrospective case series study was conducted from a tertiary epilepsy center between May 2014 and March 2017. Cases of new-onset seizures were selected based on laboratory evidence of autoimmunity. At the same time, typical mesial temporal lobe epilepsy (MTLE) patients with hippocampal sclerosis (HS) were recruited as the control group from the subjects who underwent presurgical evaluation during the same period...
February 2018: Annals of Clinical and Translational Neurology
Ildikó Nagy, Dániel Fabó
Neurophysiological methods are gaining ground in the diagnosis and therapy of cerebrovascular disease. While the role of the EEG (electroencephalography) in the diagnosis of post-stroke epilepsy is constant, quantitative EEG para-meters, as new indicators of early efficiency after thrombolysis or in prognosis of patient's condition have proved their effectiveness in several clinical studies. In intensive care units, continuous EEG monitoring of critically ill patients became part of neurointenzive care protocols...
January 30, 2018: Ideggyógyászati Szemle
Alvin Y Chan, Lilit Mnatsakanyan, Mona Sazgar, Indranil Sen-Gupta, Jack J Lin, Frank P K Hsu, Sumeet Vadera
BACKGROUND: Responsive neurostimulation (RNS) is a relatively new treatment option that has been shown to be effective for patients with medically refractory focal epilepsy when resection is not possible, especially in bilateral mesial temporal onset. Robotic devices are becoming increasingly popular for use in stereotactic procedures such as stereoelectroencephalography, but have yet to be used when implanting RNS devices. OBJECTIVE: To show that these 2 forms of advanced technology were compatible and could be used effectively in patient care...
March 1, 2018: Operative Neurosurgery (Hagerstown, Md.)
Hsiu-Fen Lee, Ching-Shiang Chi
PURPOSE: To understand the long-term neurological outcomes and chronological changes of brain MRIs in patients with febrile infection-related epilepsy syndrome (FIRES). METHODS: From December 2000 to May 2016, 29 patients diagnosed with FIRES were collected retrospectively. The demographic distribution, clinical manifestations, neuroimaging findings, and treatment methodology were described. Follow-up clinical outcomes and chronological evolution of neuroimaging findings were analyzed...
February 6, 2018: Seizure: the Journal of the British Epilepsy Association
Deepak Menon, Ramshekhar N Menon, Chandrasekharan Kesavadas, Anita Mahadevan, Ashalatha Radhakrishnan, Sudheeran Kannoth, Pradeep P Nair, Mathew Abraham, Bejoy Thomas, Sanjeev V Thomas
New-onset refractory focal epilepsy poses significant challenges to the clinician in the absence of specific diagnostic biomarkers. Differential diagnoses based on imaging may be expanded by a veritable spectrum of peri-ictal imaging findings that may mask the underlying substrate. We report a 13-year-old girl who presented with refractory focal seizures of left parieto-occipital origin with cytotoxic gyral oedema noted over the same region on imaging. Despite an initial negative autoantibody profile, the patient was treated with immunosuppression, followed by serial relapses requiring immune-modulation...
February 1, 2018: Epileptic Disorders: International Epilepsy Journal with Videotape
Zachary M Grinspan, Renée A Shellhaas, Jason Coryell, Joseph E Sullivan, Elaine C Wirrell, John R Mytinger, William D Gaillard, Eric H Kossoff, Ignacio Valencia, Kelly G Knupp, Courtney Wusthoff, Cynthia Keator, Nicole Ryan, Tobias Loddenkemper, Catherine J Chu, Edward J Novotny, John Millichap, Anne T Berg
Importance: More than half of infants with new-onset epilepsy have electroencephalographic and clinical features that do not conform to known electroclinical syndromes (ie, nonsyndromic epilepsy). Levetiracetam and phenobarbital are the most commonly prescribed medications for epilepsy in infants, but their comparative effectiveness is unknown. Objective: To compare the effectiveness of levetiracetam vs phenobarbital for nonsyndromic infantile epilepsy. Design, Setting, and Participants: The Early Life Epilepsy Study-a prospective, multicenter, observational cohort study conducted from March 1, 2012, to April 30, 2015, in 17 US medical centers-enrolled infants with nonsyndromic epilepsy and a first afebrile seizure between 1 month and 1 year of age...
February 12, 2018: JAMA Pediatrics
Alexander I Tröster
In the USA, the Food and Drug Administration (FDA) has approved deep brain stimulation (DBS) devices for the treatment of Parkinson's disease (PD), essential tremor (ET), dystonia, and obsessive compulsive disorder (OCD). DBS has been approved for these same conditions in Europe, but, additionally the European Commission (EC) has granted the Conformité Européenne (CE) Mark for DBS as adjunctive treatment for partial-onset seizures in adults with medically refractory epilepsy. In this issue of EJN Budman and colleagues [1] briefly review the range of neurologic conditions that might constitute promising new indications for DBS...
February 12, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
M P Kerr, L V Watkins, H Angus-Leppan, A Corp, M Goodwin, C Hanson, A Roy, R Shankar
PURPOSE: This article reflects the report by the British Branch of the International League Against Epilepsy (ILAE) Working Group on services for adults with epilepsy and intellectual disability (ID). Its terms of reference was to explore the current status of aspects of the care of people with an ID and epilepsy. METHODS: Survey content was developed from key themes identified by consensus of the working group. An electronic survey was distributed via email. The sample population was the membership of the ILAE UK, Royal College of Psychiatrists (RCPsych) Faculty of ID, Epilepsy Nurses Association (ESNA), and the Association of British Neurologists (ABN)...
February 8, 2018: Seizure: the Journal of the British Epilepsy Association
Francesco Mari, Beatrice Berti, Alessandro Romano, Jacopo Baldacci, Riccardo Rizzi, M Grazia Alessandrì, Alessandra Tessa, Elena Procopio, Anna Rubegni, Charles Marques Lourenḉo, Alessandro Simonati, Renzo Guerrini, Filippo Maria Santorelli
Spastic paraplegia 35 (SPG35) is a recessive condition characterized by childhood onset, progressive course, complicated by dystonia, dysarthria, cognitive impairment, and epilepsy. Mutations in the FA2H gene have been described in several families, leading to the proposal of a single entity, named fatty acid hydrolase-associated neurodegeneration (FAHN). Several reports have described a polymorphic radiological picture with white matter lesions of various degrees and a distinct form of neurodegeneration with brain iron accumulation...
February 8, 2018: Neurogenetics
Lawrence J Hirsch, Nicolas Gaspard, Andreas van Baalen, Rima Nabbout, Sophie Demeret, Tobias Loddenkemper, Vincent Navarro, Nicola Specchio, Lieven Lagae, Andrea O Rossetti, Sara Hocker, Teneille E Gofton, Nicholas S Abend, Emily J Gilmore, Cecil Hahn, Houman Khosravani, Felix Rosenow, Eugen Trinka
We convened an international group of experts to standardize definitions of New-Onset Refractory Status Epilepticus (NORSE), Febrile Infection-Related Epilepsy Syndrome (FIRES), and related conditions. This was done to enable improved communication for investigators, physicians, families, patients, and other caregivers. Consensus definitions were achieved via email messages, phone calls, an in-person consensus conference, and collaborative manuscript preparation. Panel members were from 8 countries and included adult and pediatric experts in epilepsy, electroencephalography (EEG), and neurocritical care...
February 5, 2018: Epilepsia
Tommaso Schirinzi, Federica Graziola, Raffaella Cusmai, Lucia Fusco, Francesco Nicita, Mirella Elia, Lorena Travaglini, Enrico Bertini, Paolo Curatolo, Federico Vigevano, Alessandro Capuano
BACKGROUND: Alternating Hemiplegia of Childhood (AHC) is a rare neurological disease caused by mutations in ATP1A3 gene codifying for alpha3 subunit of Na+-K+ ATPase pump. Repeated and transient attacks of hemiplegia, usually affecting one side of the body or the other, or both sides of the body at once, are the core features of AHC. Monocular nystagmus, other abnormalities in ocular movements, dystonic posturing and epilepsy are commonly associated to AHC. However, the spectrum of ATP1A3 related diseases is still expanding and new phenotypes have been reported...
January 26, 2018: Brain & Development
Su Liu, Candan Gurses, Zhiyi Sha, Michael M Quach, Altay Sencer, Nerses Bebek, Daniel J Curry, Sujit Prabhu, Sudhakar Tummala, Thomas R Henry, Nuri F Ince
High-frequency oscillations in local field potentials recorded with intracranial EEG are putative biomarkers of seizure onset zones in epileptic brain. However, localized 80-500 Hz oscillations can also be recorded from normal and non-epileptic cerebral structures. When defined only by rate or frequency, physiological high-frequency oscillations are indistinguishable from pathological ones, which limit their application in epilepsy presurgical planning. We hypothesized that pathological high-frequency oscillations occur in a repetitive fashion with a similar waveform morphology that specifically indicates seizure onset zones...
January 30, 2018: Brain: a Journal of Neurology
Yonathan Murin, Jeremy Kim, Josef Parvizi, Andrea Goldsmith
Epilepsy is one of the most common neurological disorders affecting about 1% of the world population. For patients with focal seizures that cannot be treated with antiepileptic drugs, the common treatment is a surgical procedure for removal of the seizure onset zone (SOZ). In this work we introduce an algorithm for automatic localization of the seizure onset zone (SOZ) in epileptic patients based on electrocorticography (ECoG) recordings. The proposed algorithm builds upon the hypothesis that the abnormal excessive (or synchronous) neuronal activity in the brain leading to seizures starts in the SOZ and then spreads to other areas in the brain...
January 2018: PLoS Computational Biology
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