Febrile Convulsions a Practical Guide
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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. -
Status Epilepticus: Pathophysiology, Epidemiology, and Outcomes
Arch Dis Child 1998;79:73–77 73 CURRENT TOPIC Arch Dis Child: first published as 10.1136/adc.79.1.73 on 1 July 1998. Downloaded from Status epilepticus: pathophysiology, epidemiology, and outcomes Rod C Scott, Robert A H Surtees, Brian G R Neville Convulsive status epilepticus (CSE) is the most consciousness being regained between the sei- common neurological medical emergency and zures. This gives the impression that status epi- continues to be associated with significant lepticus is always convulsive and is a single morbidity and mortality. Our approach to the entity. There are, however, as many types of epilepsies in childhood has been clarified by the status epilepticus as there are types of seizures, broad separation into benign and malignant and this definition is now probably outdated. syndromes. The factors that suggest a poorer To show that status epilepticus is a complex outcome in terms of seizures, cognition, and disorder, Shorvon has proposed the following behaviour include the presence of multiple sei- definition. Status epilepticus is a disorder in zure types, an additional, particularly cognitive which epileptic activity persists for 30 minutes disability, the presence of identifiable cerebral or more, causing a wide spectrum of clinical pathology, a high rate of seizures, an early age symptoms, and with a highly variable patho- of onset, poor response to antiepileptic drugs, physiological, anatomical, and aetiological and the occurrence of CSE.1 basis.2 CSE needs diVerent definitions for Convulsive status epilepticus is not a syn- diVerent purposes. Many seizures that last for drome in the same sense as febrile convulsions, five minutes will continue for at least 20 benign rolandic epilepsy, and infantile poly- minutes, and so treatment is required for most morphic epilepsy. -
Status Epilepticus: Initial Management
DATE: July 2018 TEXAS CHILDREN’S HOSPITAL EVIDENCE-BASED OUTCOMES CENTER Initial Management of Status Epilepticus Evidence-Based Guideline Definition: Status Epilepticus (SE) is a disease process Diagnostic Evaluation resulting in prolonged seizures of longer than 5 minutes. (1) The NOTE: Central nervous system (CNS) infection should be cause of SE can stem from the malfunction of the response to excluded. terminate a seizure or from the commencement of the mechanisms that result in prolonged seizures. (2) If SE History: Assess for continues for longer than 30 minutes, there can be permanent Seizure onset neurological damage, including neuronal death, neuronal Known seizure disorder injury, and alteration of neuronal networks. (2,3) Ingestion Fever (e.g., signs of serious infection) Epidemiology: Convulsive status epilepticus (CSE) is the Medications (4) most common neurological emergency seen in childhood. It o Received prior to presentation (e.g., type, dose, is also among the top five reasons for admission to the PICU at dosage, route) Texas Children’s Hospital and is the third most common o Current anticonvulsant medications reason for transport calls. SE is a medical emergency and is o Use of psychopharmacologic medications associated with an overall mortality rate of 8% in children and Toxic/Subtherapeutic anticonvulsant levels (4,5) o 30% in adults. Among children, the overall incidence of SE Nonadherence and/or recent change (4-6) o is approximately 1 to 6 per 10,000/year. The incidence Vagus Nerve Stimulation (VNS) appears to be higher in children under 1 year of age with over Metabolic abnormalities 50% of cases occurring in children under 3 years. -
Is It a Convulsion Or Dopamine Deficiency? a Case of Epilepsy in Dihydropteridine Reductase Deficiency
Letter to the editor pISSN 2635-909X • eISSN 2635-9103 Ann Child Neurol 2020;28(2):72-74 https://doi.org/10.26815/acn.2019.00304 Is It a Convulsion or Dopamine Deficiency? A Case of Epilepsy in Dihydropteridine Reductase Deficiency Jisu Ryoo, MD, Eun Sook Suh, MD, Jeongho Lee, MD Department of Pediatrics, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, Korea Received: December 20, 2019 Dihydropteridine reductase (DHPR) deficiency A 15-year-old man was admitted to Soon- Revised: February 13, 2020 Accepted: March 2, 2020 is a severe form of hyperphenylalaninemia due chunhyang University Seoul Hospital. At the age to impaired renewal of a substance known as tet- of 3 months, he experienced the first seizure, Corresponding author: rahydrobiopterin (BH4), caused by mutations in characterized by brief psychomotor arrest and Jeongho Lee, MD the quinoid dihydropteridine reductase (QDPR) right hand tremor. He had no family history of Department of Pediatrics, gene [1]. If little or no BH4 is available to facili- seizures or genetic disorders. Brain magnetic res- Soonchunhyang University Seoul tate processing phenylalanine (Phe), this amino onance imaging (MRI) and EEG showed nor- Hospital, Soonchunhyang University College of Medicine, 59 acid can accumulate in the blood and other tis- mal results. The seizures were not controlled by Daesagwan- ro, Yongsan-gu, Seoul sues, and the levels of neurotransmitters (dopa- antiepileptic drugs, including topiramate, val- 04401, Korea mine, serotonin) and the folate in cerebrospinal proate, and lamotrigine; therefore, he discontin- Tel: +82-2-709-9341 fluid also decrease [1]. Symptoms such as mental ued the medication. -
Epilepsy and Seizure Disorders: a Resource Guide for Parents
$5HVRXUFH *XLGH IRU3DUHQWV www.chadkids.org/epilepsyonline Epilepsy and Seizure Disorder: A Parent Resource Guide ¡ ¡ ¨ © £ ¤ ¢ ¥ ¦ § ¦ ! " $ # ¡ ¨ ' ( £ ¤ ¢ ¥ ¦ %& ¥ - . , ! # ! ) * + * ' - / ! !! ! # ! + ) ' - 00 ! , ! # ! + ) ¡ ¡¡ ¨ ( ( £ ¤ ## ¢ ¥ ¦ % 12 - - 03 ! # # ) * 0 !! ) + 0 , # ! ! ) * + " ' 4 0$ ! ! , # ! + ) 5 8 9 £7 7 162 6 ¦ ¥ 2: ( - 0; ! < - 0> = ? @ 3A ! = ! # 'C B D 3 0 ! ! C D 33 ? ! ! D 3 B ! < 3; = ! # - 3> @ ! ! EF ¤ ¤ 7 ¢ G % H ? 3/ ! ! = C - A I 0 J www.chadkids.org/epilepsyonline Epilepsy and Seizure Disorder: A Parent Resource Guide 2 WWhathat is epilepsy/seizure disorder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
Dictionary of Epilepsy
DICTIONARY OF EPILEPSY PART I: DEFINITIONS .· DICTIONARY OF EPILEPSY PART I: DEFINITIONS PROFESSOR H. GASTAUT President, University of Aix-Marseilles, France in collaboration with an international group of experts ~ WORLD HEALTH- ORGANIZATION GENEVA 1973 ©World Health Organization 1973 Publications of the World Health Organization enjoy copyright protection in accord ance with the provisions of Protocol 2 of the Universal Copyright Convention. For rights of reproduction or translation of WHO publications, in part or in toto, application should be made to the Office of Publications and Translation, World Health Organization, Geneva, Switzerland. The World Health Organization welcomes such applications. PRINTED IN SWITZERLAND WHO WORKING GROUP ON THE DICTIONARY OF EPILEPSY1 Professor R. J. Broughton, Montreal Neurological Institute, Canada Professor H. Collomb, Neuropsychiatric Clinic, University of Dakar, Senegal Professor H. Gastaut, Dean, Joint Faculty of Medicine and Pharmacy, University of Aix-Marseilles, France Professor G. Glaser, Yale University School of Medicine, New Haven, Conn., USA Professor M. Gozzano, Director, Neuropsychiatric Clinic, Rome, Italy Dr A. M. Lorentz de Haas, Epilepsy Centre "Meer en Bosch", Heemstede, Netherlands Professor P. Juhasz, Rector, University of Medical Science, Debrecen, Hungary Professor A. Jus, Chairman, Psychiatric Department, Academy of Medicine, Warsaw, Poland Professor A. Kreindler, Institute of Neurology, Academy of the People's Republic of Romania, Bucharest, Romania Dr J. Kugler, Department of Psychiatry, University of Munich, Federal Republic of Germany Dr H. Landolt, Medical Director, Swiss Institute for Epileptics, Zurich, Switzerland Dr B. A. Lebedev, Chief, Mental Health, WHO, Geneva, Switzerland Dr R. L. Masland, Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, USA Professor F. -
Supplementary Table Term* Revised Term** Focal Seizure Without Impairment of a Subjective Sensory Or Psychic Experience As Part of Migraine Or Epilepsy
Supplementary table Term* Revised term** Focal seizure without impairment of A subjective sensory or psychic experience as part of migraine or epilepsy. In epilepsy it is a focal consciousness or awareness Aura seizure without loss of consciousness (a simple partial seizure). It may or may not be followed by involving subjective sensory or other seizure manifestations. psychic phenomena only Automatic behaviour associated with loss of awareness, such as lip smacking or hand wringing, Automatism Focal dyscognitive seizure occurring as part of a complex partial seizure. Convulsion (previously "grand Tonic-clonic seziure, convulsive Seizure with involuntary, irregular myoclonic, clonic or tonic–clonic movements of one or more mal") seizure limbs. Onset may be focal or primary generalised. Literally ‘already seen’, this refers to a false impression that a present experience is familiar. It is used to refer to something heard, experienced or seen. It can be the aura of a temporal lobe Déjà vu seizure, but also happens in other settings (normal experience, intoxication, migraine, psychiatric). Focal seizure (previously "partial, Focal seizure A seizure originating in a specific cortical location. May be due to a structural lesion. localization related") Literally a ‘blow’. Usually used for an epileptic seizure (but can refer to other paroxysmal events, Ictus such as migraine, transient neurological events and stroke). Similarly pre- and post-ictal are used may for the period before and after a seizure. Idiopathic Genetic In relation to epilepsy, this implies that there is an underlying genetic cause. Literally ‘never seen’, this refers to a false impression that a present experience is unfamiliar. It is Jamais vu used for something seen, heard or experienced. -
Diagnosis and Management of Epilepsies in Children and Young People 81
81 ���� ������������������������������������������� Diagnosis and management of epilepsies 81 in children and young people A national clinical guideline 1 Introduction 1 2 Diagnosis 3 3 Investigative procedures 6 4 Management 11 5 Antiepileptic drug treatment 15 6 Management of prolonged or serial seizures and convulsive status epilepticus 21 7 Behaviour and learning 23 8 Models of care 25 9 Development of the guideline 27 10 Implementation and audit 31 Annexes 34 Abbreviations 47 References 48 March 2005 COPIES OF ALL SIGN GUIDELINES ARE AVAILABLE ONLINE AT WWW.SIGN.AC.UK KEY TO EVIDENCE STATEMENTS AND GRADES OF RECOMMENDATIONS LEVELS OF EVIDENCE 1++ High quality meta-analyses, systematic reviews of randomised controlled trials (RCTs), or RCTs with a very low risk of bias 1+ Well conducted meta-analyses, systematic reviews of RCTs, or RCTs with a low risk of bias 1 - Meta-analyses, systematic reviews of RCTs, or RCTs with a high risk of bias 2++ High quality systematic reviews of case control or cohort studies High quality case control or cohort studies with a very low risk of confounding or bias and a high probability that the relationship is causal 2+ Well conducted case control or cohort studies with a low risk of confounding or bias and a moderate probability that the relationship is causal 2 - Case control or cohort studies with a high risk of confounding or bias and a significant risk that the relationship is not causal 3 Non-analytic studies, eg case reports, case series 4 Expert opinion GRADES OF RECOMMENDATION Note: The grade of recommendation relates to the strength of the evidence on which the recommendation is based. -
What Makes a Simple Partial Seizure Complex.Pdf
Epilepsy & Behavior 22 (2011) 651–658 Contents lists available at SciVerse ScienceDirect Epilepsy & Behavior journal homepage: www.elsevier.com/locate/yebeh Review What makes a simple partial seizure complex? Andrea E. Cavanna a,b,⁎, Hugh Rickards a, Fizzah Ali a a The Michael Trimble Neuropsychiatry Research Group, Department of Neuropsychiatry, University of Birmingham and BSMHFT, Birmingham, UK b National Hospital for Neurology and Neurosurgery, Institute of Neurology, and UCL, London, UK article info abstract Article history: The assessment of ictal consciousness has been the landmark criterion for the differentiation between simple Received 26 September 2011 and complex partial seizures over the last three decades. After review of the historical development of the Accepted 2 October 2011 concept of “complex partial seizure,” the difficulties surrounding the simple versus complex dichotomy are Available online 13 November 2011 addressed from theoretical, phenomenological, and neurophysiological standpoints. With respect to con- sciousness, careful analysis of ictal semiology shows that both the general level of vigilance and the specific Keywords: contents of the conscious state can be selectively involved during partial seizures. Moreover, recent neuroim- Epilepsy fi Complex partial seizures aging ndings, coupled with classic electrophysiological studies, suggest that the neural substrate of ictal Consciousness alterations of consciousness is twofold: focal hyperactivity in the limbic structures generates the complex Experiential phenomena psychic phenomena responsible for the altered contents of consciousness, and secondary disruption of the Limbic system network involving the thalamus and the frontoparietal association cortices affects the level of awareness. These data, along with the localization information they provide, should be taken into account in the formu- lation of new criteria for the classification of seizures with focal onset. -
Epilepsy & My Child Toolkit
Epilepsy & My Child Toolkit A Resource for Parents with a Newly Diagnosed Child 136EMC Epilepsy & My Child Toolkit A Resource for Parents with a Newly Diagnosed Child “Obstacles are the things we see when we take our eyes off our goals.” -Zig Ziglar EPILEPSY & MY CHILD TOOLKIT • TABLE OF CONTENTS Introduction Purpose .............................................................................................................................................5 How to Use This Toolkit ....................................................................................................................5 About Epilepsy What is Epilepsy? ..............................................................................................................................7 What is a Seizure? .............................................................................................................................8 Myths & Facts ...................................................................................................................................8 How is Epilepsy Diagnosed? ............................................................................................................9 How is Epilepsy Treated? ..................................................................................................................10 Managing Epilepsy How Can You Help Manage Your Child’s Care? ...............................................................................13 What Should You Do if Your Child has a Seizure? ............................................................................14 -
Clinical Decision Making in Seizures and Status Epilepticus
January 2015 Clinical Decision Making Volume 17, Number 1 Authors In Seizures And Felipe Teran, MD Department of Emergency Medicine, Icahn School of Medicine at Mount Sinai, New York, NY; Faculty, Emergency Department, Clínica Status Epilepticus Alemana, Santiago, Chile Katrina Harper-Kirksey, MD Abstract Anesthesia Critical Care Fellow, Department of Anesthesia, Stanford Hospital and Clinics, Stanford, CA Andy Jagoda, MD, FACEP Seizures and status epilepticus are frequent neurologic emergen- Professor and Chair, Department of Emergency Medicine, Icahn cies in the emergency department, accounting for 1% of all emer- School of Medicine at Mount Sinai, New York, NY; Medical Director, Mount Sinai Hospital, New York, NY gency department visits. The management of this time-sensitive and potentially life-threatening condition is challenging for both Peer Reviewers prehospital providers and emergency clinicians. The approach to J. Stephen Huff, MD Professor of Emergency Medicine and Neurology, University of seizing patients begins with differentiating seizure activity from Virginia, Charlottesville, VA mimics and follows with identifying potential secondary etiolo- Jason McMullan, MD gies, such as alcohol-related seizures. The approach to the patient Assistant Professor, Department of Emergency Medicine, University of in status epilepticus and the patient with nonconvulsive status Cincinnati, Cincinnati, OH epilepticus constitutes a special clinical challenge. This review CME Objectives summarizes the best available evidence and recommendations Upon completion of this article, you should be able to: regarding diagnosis and resuscitation of the seizing patient in the 1. Describe the diagnostic approach to patients who have recovered from a seizure and patients in status epilepticus. emergency setting. 2. Recognize and treat patients with alcohol withdrawal seizures. -
Epilepsy in Children – Brain Growth and Behavior
logy & N ro eu u r e o N p h f y o s l i a o l n o r Kanemura and Aihara, J Neurol Neurophysiol 2013, S2 g u y o J Journal of Neurology & Neurophysiology ISSN: 2155-9562 DOI: 10.4172/2155-9562.S2-006 ReviewResearch Article Article OpenOpen Access Access Epilepsy in Children – Brain Growth and Behavior Hideaki Kanemura1* and Masao Aihara2 1Department of Pediatrics, Faculty of Medicine, University of Yamanashi, Japan 2Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Japan Abstract The degree of behavioral problems associated with epilepsy in children is greater than would be expected on the basis of the existence of a chronic illness alone. Involvement of the frontal lobe such as atypical evolution of benign childhood epilepsy with centrotemporal spikes (BCECTS), epilepsy with continuous spike-waves during slow sleep (CSWS), and frontal lobe epilepsy (FLE) are characterized by impairment of neuropsychological abilities, and are frequently associated with behavioral disorders. In volumetric studies using three-dimensional (3D) magnetic resonance imaging (MRI), frontal and prefrontal lobe volumes revealed growth disturbance in patients with atypical evolution of BCECTS, CSWS, and FLE compared with those of normal subjects. These studies also revealed that in patients with shorter seizure durations and abnormal EEG periods, developmental abnormalities of the frontal lobe were soon restored to a more normal growth ratio. Conversely, growth disturbances of the prefrontal lobes were persistent in the patients with longer seizure durations and abnormal EEG periods. These findings suggest that seizure and the duration of paroxysmal anomalies may be associated with prefrontal lobe growth abnormalities, which are associated with neuropsychological problems.