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1 ILAE Classification & Definition of Epilepsy Syndromes in the Neonate
ILAE Classification & Definition of Epilepsy Syndromes in the Neonate and Infant: Position Statement by the ILAE Task Force on Nosology and Definitions Authors: Sameer M Zuberi1, Elaine Wirrell2, Elissa Yozawitz3, Jo M Wilmshurst4, Nicola Specchio5, Kate Riney6, Ronit Pressler7, Stephane Auvin8, Pauline Samia9, Edouard Hirsch10, O Carter Snead11, Samuel Wiebe12, J Helen Cross13, Paolo Tinuper14,15, Ingrid E Scheffer16, Rima Nabbout17 1. Paediatric Neurosciences Research Group, Royal Hospital for Children & Institute of Health & Wellbeing, University of Glasgow, Member of European Reference Network EpiCARE, Glasgow, UK. 2. Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester MN, USA. 3. Isabelle Rapin Division of Child Neurology of the Saul R Korey Department of Neurology, Montefiore Medical Center, Bronx, NY USA. 4. Department of Paediatric Neurology, Red Cross War Memorial Children’s Hospital, Neuroscience Institute, University of Cape Town, South Africa. 5. Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesu’ Children’s Hospital, IRCCS, Member of European Reference Network EpiCARE, Rome, Italy 6. Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia. Faculty of Medicine, University of Queensland, Queensland, Australia. 7. Clinical Neuroscience, UCL- Great Ormond Street Institute of Child Health, London, UK. Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children NHS Foundation Trust, Member of European Reference Network EpiCARE London, UK 8. Université de Paris, AP-HP, Hôpital Robert-Debré, INSERM NeuroDiderot, DMU Innov-RDB, Neurologie Pédiatrique, Member of European Reference Network EpiCARE, Paris, France. 9. Department of Paediatrics and Child Health, Aga Khan University, East Africa. 1 10. Neurology Epilepsy Unit “Francis Rohmer”, INSERM 1258, FMTS, Strasbourg University, France. -
EEG in the Diagnosis, Classification, and Management of Patients With
EEG IN THE DIAGNOSIS, J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2005.069245 on 16 June 2005. Downloaded from CLASSIFICATION, AND MANAGEMENT ii2 OF PATIENTS WITH EPILEPSY SJMSmith J Neurol Neurosurg Psychiatry 2005;76(Suppl II):ii2–ii7. doi: 10.1136/jnnp.2005.069245 he human electroencephalogram (EEG) was discovered by the German psychiatrist, Hans Berger, in 1929. Its potential applications in epilepsy rapidly became clear, when Gibbs and Tcolleagues in Boston demonstrated 3 per second spike wave discharge in what was then termed petit mal epilepsy. EEG continues to play a central role in diagnosis and management of patients with seizure disorders—in conjunction with the now remarkable variety of other diagnostic techniques developed over the last 30 or so years—because it is a convenient and relatively inexpensive way to demonstrate the physiological manifestations of abnormal cortical excitability that underlie epilepsy. However, the EEG has a number of limitations. Electrical activity recorded by electrodes placed on the scalp or surface of the brain mostly reflects summation of excitatory and inhibitory postsynaptic potentials in apical dendrites of pyramidal neurons in the more superficial layers of the cortex. Quite large areas of cortex—in the order of a few square centimetres—have to be activated synchronously to generate enough potential for changes to be registered at electrodes placed on the scalp. Propagation of electrical activity along physiological pathways or through volume conduction in extracellular spaces may give a misleading impression as to location of the source of the electrical activity. Cortical generators of the many normal and abnormal cortical activities recorded in the EEG are still largely unknown. -
Epilepsy Syndromes E9 (1)
EPILEPSY SYNDROMES E9 (1) Epilepsy Syndromes Last updated: September 9, 2021 CLASSIFICATION .......................................................................................................................................... 2 LOCALIZATION-RELATED (FOCAL) EPILEPSY SYNDROMES ........................................................................ 3 TEMPORAL LOBE EPILEPSY (TLE) ............................................................................................................... 3 Epidemiology ......................................................................................................................................... 3 Etiology, Pathology ................................................................................................................................ 3 Clinical Features ..................................................................................................................................... 7 Diagnosis ................................................................................................................................................ 8 Treatment ............................................................................................................................................. 15 EXTRATEMPORAL NEOCORTICAL EPILEPSY ............................................................................................... 16 Etiology ................................................................................................................................................ 16 -
ILAE Classification and Definition of Epilepsy Syndromes with Onset in Childhood: Position Paper by the ILAE Task Force on Nosology and Definitions
ILAE Classification and Definition of Epilepsy Syndromes with Onset in Childhood: Position Paper by the ILAE Task Force on Nosology and Definitions N Specchio1, EC Wirrell2*, IE Scheffer3, R Nabbout4, K Riney5, P Samia6, SM Zuberi7, JM Wilmshurst8, E Yozawitz9, R Pressler10, E Hirsch11, S Wiebe12, JH Cross13, P Tinuper14, S Auvin15 1. Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesu’ Children’s Hospital, IRCCS, Member of European Reference Network EpiCARE, Rome, Italy 2. Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester MN, USA. 3. University of Melbourne, Austin Health and Royal Children’s Hospital, Florey Institute, Murdoch Children’s Research Institute, Melbourne, Australia. 4. Reference Centre for Rare Epilepsies, Department of Pediatric Neurology, Necker–Enfants Malades Hospital, APHP, Member of European Reference Network EpiCARE, Institut Imagine, INSERM, UMR 1163, Université de Paris, Paris, France. 5. Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia. Faculty of Medicine, University of Queensland, Queensland, Australia. 6. Department of Paediatrics and Child Health, Aga Khan University, East Africa. 7. Paediatric Neurosciences Research Group, Royal Hospital for Children & Institute of Health & Wellbeing, University of Glasgow, Member of European Refence Network EpiCARE, Glasgow, UK. 8. Department of Paediatric Neurology, Red Cross War Memorial Children’s Hospital, Neuroscience Institute, University of Cape Town, South Africa. 9. Isabelle Rapin Division of Child Neurology of the Saul R Korey Department of Neurology, Montefiore Medical Center, Bronx, NY USA. 10. Programme of Developmental Neurosciences, UCL NIHR BRC Great Ormond Street Institute of Child Health, Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children, London, UK 11. -
Investigation of Seizures in Infants
Chapter 23 Investigation of seizures in infants RICHARD E. APPLETON1 and AILSA McLELLAN2 1The Roald Dahl EEG Unit, Paediatric Neurosciences Foundation, Alder Hey Children’s NHS Foundation Trust, Liverpool, and 2Department of Paediatric Neurosciences, Royal Hospital for Sick Children, Edinburgh The investigation of seizures in infancy (i.e. within the first year of life) begins with establishing whether the seizures are epileptic or non-epileptic in origin. The ‘broad’ differential diagnosis of possible seizures and ‘epilepsy’ is multiple and is particularly difficult under the age of 12 months and includes: Gastro-oesophageal reflux (Sandifer’s syndrome) Pallid syncopal attacks (reflex anoxic seizures) Cyanotic breath-holding attacks Cardiac arrhythmias Münchausen syndrome by proxy (passive or active both representing a form of child abuse) Shuddering spells and jitteriness Hyperekplexia Benign neonatal sleep myoclonus Benign myoclonus of infancy Tonic reflex activity and involuntary movements (seen in children with neurological impairment including cerebral palsy or hydrocephalus). Once a non-epileptic disorder has been excluded or the episodes are considered to be obviously epileptic, then the following conditions/investigations should be considered on a chronological basis. Perinatal (first week of life) and neonatal (first month of life) seizures The newborn period is the time of life with the highest risk of seizures1-3. This is because of the relative lack, and immature development of inhibitory neurotransmitters and their pathways. The immature and developing brain is susceptible to a large number of both cerebral and systemic insults including: Asphyxia (hypoxic-ischaemic encephalopathy) the most common and also most serious cause of neonatal seizures – particularly in term infants Intra- and periventricular haemorrhage – particularly in pre-term infants Metabolic dysfunction (e.g. -
Fostering Epilepsy Care in Europe All Rights Reserved
EPILEPSY IN THE WHO EUROPEAN REGION: Fostering Epilepsy Care in Europe All rights reserved. No part of this publication may be reproduced, stored in a database or retrieval system, or published, in any form or any way, electronically, mechanically, by print, photoprint, microfilm or any other means without prior written permission from the publisher. Address requests about publications of the ILAE/IBE/WHO Global Campaign Against Epilepsy: Global Campaign Secretariat SEIN P.O. Box 540 2130 AM Hoofddorp The Netherlands e-mail: [email protected] ISBN NR. 978-90-810076-3-4 Layout/ Printing: Paswerk Bedrijven, Cruquius, Netherlands Table of contents Foreword ................................................................................................................................................. 4 Preface ................................................................................................................................................. 5 Acknowledgements ........................................................................................................................................ 6 Tribute ................................................................................................................................................. 7 Abbreviations ................................................................................................................................................. 8 Background information on the European Region ......................................................................................... -
Pediatric Epilepsy
PEDIATRIC EPILEPSY Ø Epilepsy is one of the most common chronic neurological disorders. It is characterized by recurrent unprovoked seizures or an enduring predisposition to generate epileptic seizures. If epilepsy begins in childhood, it is often outgrown. Seizures are common in childhood and adolescence. Approximately 3% of children will experience a seizure. Ø A seizure occurs when there is a sudden change in behavior or sensation caused by abnormal and excessive electrical hypersynchronization of neuronal networks in the cerebral cortex. Normal inhibition is overcome by excessive excitatory stimuli. Ø If the cause of the seizures is known (for example: genetic, inborn errors of metabolism, metabolic (eg: low glucose, electrolyte abnormalities), structural (eg: malformations, tumours, bleeds, stroke, traumatic brain injury), infectious, inflammatory, or toxins) it is classified as symptomatic. If the cause is unknown, it is classified as idiopathic. 1. WHERE DID THE SEIZURE START? / WHAT KIND OF SEIZURE IS IT? 2. IS AWARENESS YES FOCAL ONSET GENERALIZED UNKNOWN IMPAIRED? NO Seizure that originates ONSET ONSET in a focal cortical area Seizure that involves When it is unclear YES with associated clinical both sides of the where the seizure 3. PROGRESSION TO BILATERAL? features. brain from the onset. starts. NO SEIZURE SEMIOLOGY (The terminology for seizure types is designed to be useful for communicating the key characteristics of seizures) CLONIC: sustained rhythmical TONIC: muscles stiffen or ATONIC: sudden loss of muscle tone, MYOCLONUS: sudden lighting- jerking movements. tense. lasting seconds. like jerk, may cluster. EPILEPTIC SPASM: sudden AUTONOMIC: eg: AUTOMATISMS: ABSENCE: brief (≤ 10s), OTHERS: change flexion, extension, or flexion- rising epigastric stereotyped, purposeless frequent (up to 100’s) in cognition, extension of proximal and sensations, waves of movements. -
Childhood Epilepsy Syndromes April 2021.Indd
Factsheet 26 Childhood epilepsy syndromes What is a syndrome? A syndrome is a group of signs or symptoms that Many children with severe epilepsy syndromes have happen together and help to identify a unique additional difficulties with learning and behaviour and medical condition. may need extra support. See our leafletmedication for epilepsy and our What is a ‘childhood epilepsy syndrome’? factsheet ketogenic diet for more information. If your child is diagnosed with an epilepsy syndrome, Examples of childhood syndromes it means that their epilepsy has some specific signs and symptoms. These include: Benign rolandic epilepsy (BRE) This syndrome affects 15% of children with epilepsy • the type of seizure or seizures they have; and can start any time between the ages of 3 and 10. • the age when the seizures start; • a specific pattern on an electroencephalogram Children may have very few seizures and most (EEG); and become seizure-free by the age of 16. They may have • sometimes a pattern on a brain imaging scan. focal motor aware seizures (previously simple partial seizures), which means they involve movement. They An EEG test is painless and records patterns of are often at night, and usually involve one side of electrical activity in the brain. Some epilepsy syndromes the face and/or the muscles that involve speech and have a particular pattern, so the EEG can be helpful in swallowing, causing gurgling or grunting noises, mouth finding the correct diagnosis. A magnetic resonance movements, and dribbling. Speech can be temporarily imaging (MRI) brain scan is also painless and looks at affected and symptoms may develop into a tonic clonic the structure of the brain for any underlying abnormality. -
A Proposed Diagnostic Scheme for People with Epileptic Seizures and with Epilepsy: Report of the ILAE Task Force on Classification and Terminology
Epilepsia, 42(6):796–803, 2001 Blackwell Science, Inc. © International League Against Epilepsy ILAE Commission Report A Proposed Diagnostic Scheme for People with Epileptic Seizures and with Epilepsy: Report of the ILAE Task Force on Classification and Terminology Jerome Engel, Jr. UCLA School of Medicine, Los Angeles, California, U.S.A. The International League Against Epilepsy (ILAE) and dynamic classifications will be revised periodically made a major contribution when it established standard- based not only on rapidly emerging new information, but ized classifications and terminology for epileptic sei- also on the resolution of problems that will inevitably be zures and syndromes. This provided a universal vocabu- identified through use. At this point, the proposal does lary that not only facilitated communication among cli- include several definitive changes in concepts and ter- nicians, but also established a taxonomic foundation for minology (Table 2), and classifications are presented as performing quantitative clinical and basic research on examples of what could be devised in the future. epilepsy. Much, however, has changed since the adop- tion of the currently used Classification of Epileptic Sei- zures in 1981 (1) and the Classification of Epilepsies and RATIONALE FOR THE PROPOSAL Epileptic Syndromes in 1989 (2). Consequently, the Ex- ecutive Committee of the ILAE, which took office in Although each new ILAE classification has repre- July 1997, agreed that review and revision of the current sented considerable effort on the part of acknowledged classification system would be a priority for this Execu- experts from many different countries, they have always tive term. met with a certain degree of resistance from the interna- A Task Force on Classification and Terminology was tional epileptology community. -
Catastrophic Epilepsies of Childhood
NE40CH07-Baraban ARI 8 June 2017 9:56 Catastrophic Epilepsies of Childhood MacKenzie A. Howard1 and Scott C. Baraban2 1Center for Learning and Memory and Department of Neuroscience, University of Texas at Austin, Texas, 78712; email: [email protected] 2Epilepsy Research Laboratory in the Department of Neurological Surgery, Weill Institute for Neurosciences, University of California, San Francisco, California 94143; email: [email protected] Annu. Rev. Neurosci. 2017. 40:149–66 Keywords The Annual Review of Neuroscience is online at development, genetic, seizure disorder, plasticity, syndrome, neuro.annualreviews.org electrophysiology https://doi.org/10.1146/annurev-neuro-072116- 031250 Abstract Copyright c 2017 by Annual Reviews. The tragedy of epilepsy emerges from the combination of its high preva- All rights reserved lence, impact upon sufferers and their families, and unpredictability. Child- Annu. Rev. Neurosci. 2017.40:149-166. Downloaded from www.annualreviews.org Access provided by University of Texas - Austin on 08/31/17. For personal use only. hood epilepsies are frequently severe, presenting in infancy with pharmaco- resistant seizures; are often accompanied by debilitating neuropsychiatric ANNUAL and systemic comorbidities; and carry a grave risk of mortality. Here, we REVIEWS Further review the most current basic science and translational research findings Click here to view this article's online features: on several of the most catastrophic forms of pediatric epilepsy. We focus • Download figures as PPT slides • Navigate linked references largely on genetic epilepsies and the research that is discovering the mech- • Download citations anisms linking disease genes to epilepsy syndromes. We also describe the • Explore related articles • Search keywords strides made toward developing novel pharmacological and interventional treatment strategies to treat these disorders. -
Treatment for Patients with Lennox-Gastaut Syndrome Roundtable
Treatment for Patients with Lennox-Gastaut Syndrome Roundtable Video 2/5 – Issues of Epidemiology Dr. Raj Sheth Chief of Neurology at Nemours in Jacksonville. Professor of Neurology at the Mayo Clinic college of Medicine, Professor of Pediatrics from the University of Florida. Well, I’m very pleased to be talking about a topic that most physicians are familiar with, but oftentimes, causes a lot of confusion when you get into the nitty-gritty of trying to figure out what the syndrome is and what the best way of treating a patient is. How do you evaluate them and manage them? When you talk about Lennox-Gastaut Syndrome, about one to ten percent of all epilepsy clinics will have a patient with Lennox-Gastaut Syndrome in them. The reason that the range is so wide is because it depends on the level of the clinic. If you have a new onset seizure clinic, you’re going to see Lennox-Gastaut Syndrome very rarely. But if you have a chronic epilepsy patient clinic, you’re going to see a lot of Lennox-Gastaut patients. In a tertiary care epilepsy center, the incidents would be about ten percent. When you look at population-based surveys, the incidents are a quarter of one in a thousand. So it would be .26 per 1,000 patients is what would be in the general population. Of course, most of you who have worked in neurology clinics will realize that they occupy a significant portion of our time and resources. To try and understand that, we could go to the next slide. -
ILAE Definition of the Idiopathic Generalized Epilepsy Syndromes: Position Statement by the ILAE Task Force on Nosology and Definitions
1 ILAE Definition of the Idiopathic Generalized Epilepsy Syndromes: Position Statement by the ILAE Task Force on Nosology and Definitions E Hirsch1, J French2, IE Scheffer3, SM Zuberi4, E Trinka5,6, N Specchio7, E Somerville8, P Samia9, K Riney10, R Nabbout11, S Jain12, A Bogacz13, T Alsaadi14, JM Wilmshurst15, S Auvin16, S Wiebe17, P Tinuper18,19*, E Wirrell20* *Co senior authors 1. Neurology Epilepsy Units “Francis Rohmer”, INSERM 1258, FMTS, Strasbourg University, France. 2. NYU Grossman School of Medicine and NYU Langone Health, New York, NY, USA. 3. University of Melbourne, Austin Health and Royal Children’s Hospital, Florey Institute, Murdoch Children’s Research Institute, Melbourne, Australia. 4. Paediatric Neurosciences Research Group, Royal Hospital for Children & Institute of Health & Wellbeing, University of Glasgow, Member of European Reference Network EpiCARE, Glasgow, UK. 5. Department of Neurology and Neuroscience Institute, Christian Doppler University Hospital, Paracelsus Medical University, and Centre for Cognitive Neuroscience, Affiliated Member of EpiCARE, Salzburg, Austria. 6. Department of Public Health, Health Services Research and Health Technology Assessment, UMIT – University for Health Sciences, Medical Informatics and Technology, Hall in Tirol, Austria. 7. Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesu’ Children’s Hospital, IRCCS, Member of European Reference Network EpiCARE, Rome, Italy 8. Prince of Wales Hospital and University of New South Wales, Sydney, Australia. 9. Department of Paediatrics and Child Health, Aga Khan University, East Africa. 10. Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia. Faculty of Medicine, University of Queensland, Queensland, Australia. 11. Reference Centre for Rare Epilepsies, Department of Pediatric Neurology, Necker–Enfants Malades Hospital, APHP, Member of European Reference Network EpiCARE, Institut Imagine, INSERM, UMR 1163, Université de Paris, Paris, France.