Seizure Types and Epilepsy Syndromes for Primary Care
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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. -
Clinicians Using the Classification Will Identify a Seizure As Focal Or Generalized Onset If There Is About an 80% Confidence Level About the Type of Onset
GENERALIZED ONSET SEIZURES Generalized onset seizures are not characterized by level of awareness, because awareness is almost always impaired. Generalized tonic-clonic: Immediate loss of Generalized epileptic spasms: Brief seizures with awareness, with stiffening of all limbs (tonic phase), flexion at the trunk and flexion or extension of the followed by sustained rhythmic jerking of limbs and limbs. Video-EEG recording may be required to face (clonic phase). Duration is typically 1 to 3 minutes. determine focal versus generalized onset. The seizure may produce a cry at the start, falling, tongue biting, and incontinence. Generalized typical absence: Sudden onset when activity stops with a brief pause and staring, Generalized clonic: Rhythmical sustained jerking of sometimes with eye fluttering and head nodding or limbs and/or head with no tonic stiffening phase. other automatic behaviors. If it lasts for more than These seizures most often occur in young children. several seconds, awareness and memory are impaired. Recovery is immediate. The EEG during these seizures Generalized tonic: Stiffening of all limbs, without always shows generalized spike-waves. clonic jerking. Generalized atypical absence: Like typical absence Generalized myoclonic: Irregular, unsustained jerking seizures, but may have slower onset and recovery and of limbs, face, eyes, or eyelids. The jerking of more pronounced changes in tone. Atypical absence generalized myoclonus may not always be left-right seizures can be difficult to distinguish from focal synchronous, but it occurs on both sides. impaired awareness seizures, but absence seizures usually recover more quickly and the EEG patterns are Generalized myoclonic-tonic-clonic: This seizure is like different. -
Title in All Caps
Epilepsy Syndromes: Where does Dravet Syndrome fit in? Scott Demarest MD Assistant Professor, Departments of Pediatrics and Neurology University of Colorado School of Medicine Children's Hospital Colorado Disclosures Scott Demarest has consulted for Upsher-Smith on an unrelated subject matter. No conflicts of interest Objectives • What is an Epilepsy Syndrome? • How do we define epilepsy syndromes? • Genetic vs Phenotype (Features) • So what? Why do we care about Epilepsy Syndromes? • How do we organize and categorize Epilepsy Syndromes? • What epilepsy syndromes are similar to Dravet Syndrome and what is different about them? Good Resource International League Against Epilepsy Epilepsydiagnosis.org https://www.epilepsydiagnosis.org/syndrome/epilepsy- syndrome-groupoverview.html What is an Epilepsy Syndrome? A syndrome is a collection of common clinical traits. For Epilepsy this is usually about: • What type of seizures occur? • Age seizure start? Electroclinical • Development? Features or • What does the EEG look like? Phenotype • Other Co-morbidities… Course of an Epilepsy Syndrome Developmental Trajectories - Theoretical Model Normal Previously Normal with Epileptic Encephalopathy Development Never Normal Gray represents Epilepsy Onset the intensity of Age Epilepsy How distinct are Epilepsy Syndromes? A B C Many features might overlap, but the hope is that the cluster of symptoms are “specific” to that epilepsy syndrome…this is often better in theory than practice. How does the individual patient fit? A B C Is this patient at type A,B or C? What about Syndromes Defined by Genes? Is SCN1A the same as Dravet Syndrome? …I don’t have a perfect answer for this… many diseases are being defined by the gene (CDKL5, SCN8A, CHD2). -
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. -
Mesial Frontal Epilepsy
Epikpsia, 39(Suppl. 4):S49-S61. 1998 Lippincon-Raven Publishers, Philadelphia 0 International League Against Epilepsy Mesial Frontal Epilepsy Norman K. So Oregon Comprehensive Epilepsy Program, Legacy Portland Hospitals, Portland, Oregon, U.S.A. Summary: The mesiofrontal cortex comprises a number of occur. The task of localization of the epileptogenic zone can be distinct anatomic and functional areas. Structural lesions and challenging, whether EEG or imaging methods are used. Suc- cortical dysgenesis are recognized causes of mesial frontal epi- cessful localization can lead to a rewarding outcome after epi- lepsy, but a specific gene defect may also be important, as seen lepsy surgery, particularly in those with an imaged lesion. in some forms of familial frontal lobe epilepsy. The predomi- Key Words: Mesial frontal epilepsy-cingulate gyrus- nant seizure manifestations, which are not necessarily strictly Supplementary motor area-Absence seizure-Hypermotor correlated with a specific ictal onset zone, are absence, hyper- seizure-Postural tonic seizure-Epilepsy surgery. motor, and postural tonic seizures. Other seizure types also FUNCTIONAL ANATOMY convolution. Traditional cytoarchitectonics have further subdivided this anterior frontal region. The frontal pole The frontal lobe is the largest lobe in the brain, ac- refers to the anterior most portion of the frontal lobe, but counting for one-third to one-half of total brain volume there is little consensus on how far back this extends. and weight. On the medial surface, the most important One or more curved.sulci are seen anterior and inferior to landmark is the cingulate sulcus (Fig. 1). This runs as an the cingulate sulcus, called the superior and inferior ros- inverted “C” following the contour of the corpus callo- tral sulci. -
The Double Generalization Phenomenon in Juvenile Absence Epilepsy
Epilepsy & Behavior 21 (2011) 318–320 Contents lists available at ScienceDirect Epilepsy & Behavior journal homepage: www.elsevier.com/locate/yebeh Case Report The double generalization phenomenon in juvenile absence epilepsy Daniel San-Juan a,b,⁎, Adriana Patricia M. Mayorga a, David J. Anschel c, Alvaro Moreno Avellán a, Maricarmen F. González-Aragón a, Andrew J. Cole d a Clinical Neurophysiology Department, National Institute of Neurology, Mexico City, Mexico b Centro Neurológico, Hospital ABC Santa Fe, Mexico City, Mexico c Clinical Neurophysiology Department, Saint Charles Hospital, Port Jefferson, NY, USA d Epilepsy Service, Massachusetts General Hospital, Boston, MA, USA article info abstract Article history: The characterization of a seizure as generalized or focal onset depends on a basic knowledge of the underlying Received 12 March 2011 pathophysiology. Recently, an uncommon phenomenon in generalized epilepsy—evolution of seizures Revised 7 April 2011 from generalized to focal followed by secondary generalization—was reported for the first time. We describe a Accepted 8 April 2011 15-year-old boy, initially classified as having partial epilepsy, who had a typical absence seizure that became Available online 14 May 2011 focal with second secondary generalization (double generalization). On the basis of these findings his epilepsy fi Keywords: was classi ed as juvenile absence epilepsy and his treatment was changed, resulting in seizure freedom. This fi Juvenile absence epilepsy is the rst report of this unusual electroclinical evolution in a patient with juvenile absence epilepsy. The Generalized epilepsy recognition of this particular pattern allows correct classification and impacts both treatment and prognosis. Focal findings © 2011 Elsevier Inc. -
Managing Children with Epilepsy School Nurse Guide
MANAGING CHILDREN WITH EPILEPSY SCHOOL NURSE GUIDE ACKNOWLEDGEMENTS TO THOSE WHO HAVE CONTRIBUTED TO THE NOTEBOOK Children’s Hospital of Orange County Melodie Balsbaugh, RN Sue Nagel, RN Giana Nguyen, CHOC Institutes Fullerton School District Jane Bockhacker, RN Orange Unified School District Andrea Bautista, RN Martha Boughen, RN Karen Hanson, RN TABLE OF CONTENTS I. EPILEPSY What is epilepsy? Facts about epilepsy Basic neuroanatomy overview Classification of epileptic seizures Diagnostic Tests II. TREATMENT Medications Vagus Nerve Stimulation Ketogenic Diet Surgery III. SAFETY First Aid IV. SPECIAL CONCERNS MedicAlert Helmets Driving Employment and the law V. EPILEPSY AT SCHOOL School epilepsy assessment tool Seizure record Teaching children about epilepsy lesson plan Creating your own individualized health care plan VI. RESOURCES/SUPPORT GROUPS VII. ACCESS TO HEALTHCARE CHOC Epilepsy Center After-Hours Care After Hours Health Care Advice Healthy Families California Kids MediCal CHOC Clinics Healthy Tomorrows VIII. REFERENCES EPILEPSY WHAT IS EPILEPSY? Epilepsy is a neurological disorder. The brain contains millions of nerve cells called neurons that send electrical charges to each other. A seizure occurs when there is a sudden and brief excess surge of electrical activity in the brain between nerve cells. This results in an alteration in sensation, behavior, and consciousness. Seizures may be caused by developmental problems before birth, trauma at birth, head injury, tumor, structural problems, vascular problems (i.e. stroke, abnormal blood vessels), metabolic conditions (i.e. low blood sugar, low calcium), infections (i.e. meningitis, encephalitis) and idiopathic causes. Children who have idiopathic seizures are most likely to respond to medications and outgrow seizures. -
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 ......................................................................................... -
Typical Absence Seizures and Their Treatment
Arch Dis Child 1999;81:351–355 351 Arch Dis Child: first published as 10.1136/adc.81.4.351 on 1 October 1999. Downloaded from CURRENT TOPIC Typical absence seizures and their treatment C P Panayiotopoulos Typical absences (previously known as petit there are inappropriate generalisations regard- mal) are generalised seizures that are distinc- ing their use in the treatment of other tively diVerent from any other type of epileptic epilepsies. fit.1 They are pharmacologically unique2–5 and demand special attention in their treatment.6 The prevalence of typical absences among Typical absence seizures children with epilepsies is about 10%, probably Typical absence seizures are defined according with a female preponderance.6 Typical ab- to clinical and electroencephalogram (EEG) 16 sences are easy to diagnose and treat. There- ictal and interictal expression. Clinically, the fore, it is alarming that 40% of children with hallmark of the absence is abrupt and brief typical absences were inappropriately treated impairment of consciousness, with interrup- with contraindicated drugs, such as car- tion of the ongoing activity, and usually unresponsiveness. The seizure lasts for a few to Department of Clinical bamazepine and vigabatrin, according to a 7 20 seconds and ends suddenly with resumption Neurophysiology and recent report from London, UK. Epilepsies, St Thomas’ The purpose of this paper is to oVer some of the pre-absence activity, as if had not been Hospital, London guidance to paediatricians regarding diagnosis interrupted. Although some absence seizures SE1 7EH, UK and management of typical absence seizures. can manifest with impairment of consciousness C P Panayiotopoulos This is also important because of the introduc- only, this is often combined with the following: tion of new antiepileptic drugs.