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Neonatalseizureclassification-Proofforweb.Pdf Page 1 of 39 Epilepsia 1 2 3 4 The ILAE Classification of Seizures & the Epilepsies: 5 Modification for Seizures in the Neonate. Proposal from 6 7 the ILAE Task Force on Neonatal Seizures 8 9 10 Ronit M Pressler1,2, Maria Roberta Cilio3, Eli M Mizrahi4, Solomon L Moshé5, Magda L 11 6 7 8 9 10 12 Nunes , Perrine Plouin , Sampsa Vanhatalo , Elissa Yozawitz , Sameer M Zuberi 13 1 14 Clinical Neuroscience, UCL-Institute of Child Health, London, UK, r. [email protected] 15 2 Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children NHS 16 Foundation Trust, London, UK 17 3 18 Departments of Neurology and Pediatrics, Benioff Children’s Hospital, University of 19 California San Francisco, San Francisco, CA, USA, [email protected] 20 4 Departments of Neurology, Baylor College of Medicine, Houston, TX, [email protected] 21 5 Saul R. Korey DepartmentFor of Neurology, Review Dominick Only P. Purpura Department of 22 23 Neuroscience and Department of Pediatrics, Albert Einstein College of Medicine and 24 Montefiore Medical Center, Bronx, New York, USA, [email protected] 6 25 PUCRS School of Medicine, Porto Alegre, RS, Brazil, [email protected] 26 7 Department of Clinical Neurophysiology, Hospital Necker Enfant Malades, Paris, France 27 [email protected] 28 8 29 Department of Clinical Neurophysiology, Children's Hospital, HUS Imaging, Helsinki 30 University Central Hospital and University of Helsinki, Finland, 31 [email protected] 32 9 Saul R. Korey Department of Neurologyand Department of Pediatrics, Albert Einstein 33 College of Medicine and Montefiore Medical Center, Bronx, New York, USA 34 35 [email protected] 10 36 Paediatric Neurosciences Research Group, Fraser of Allander Neurosciences Unit Royal 37 Hospital for Sick Children, Glasgow, UK, [email protected] 38 39 40 41 Corresponding author: 42 Dr Ronit M Pressler 43 Department of Clinical Neurophysiology 44 Great Ormond Street Hospital for Children NHS Foundation Trust 45 46 London WC1N 3JH, UK 47 Tel +442078138471 48 [email protected] 49 50 51 Keywords: Neonatal seizures, EEG, semiology, classification, epilepsy 52 53 Text pages: 16 (incl. abstract, definitions, and disclosures, excl. references) 54 55 References: 99 56 57 Figures: 3 58 59 Tables: 5 60 Epilepsia Page 2 of 39 1 2 3 Abstract 4 5 6 Seizures are the most common neurological emergency in the neonatal period occurring in 7 8 1–5 per 1000 live births. In contrast to seizures in infancy and childhood, most seizures in 9 10 11 the neonate are symptomatic of an acute illness and can be electrographic only or 12 13 associated with clinical manifestations. Hence, neonatal seizures may not fit easily into 14 15 16 classification schemes for seizures and epilepsies primarily developed for older children and 17 18 adults. The Task Force on Neonatal Seizures was established with the aim of developing a 19 20 21 modification of the 2017For ILAE Classification Review of Seizures Only and Epilepsies, relevant to neonates. 22 23 The proposed neonatal classification framework emphasizes the role of EEG in the diagnosis 24 25 26 of seizures in the neonate and includes a classification of seizure types relevant to this age 27 28 group. The seizure type is typically determined by the predominant clinical feature. As 29 30 seizures in neonates are often electrographic only with no clinical feature, these are 31 32 33 included in the proposed classification. Clinical events without an EEG correlate are not 34 35 included. As seizures in the neonatal period have been shown to have a focal onset, a 36 37 38 division into focal and generalized is unnecessary. Seizures can have a motor (automatisms, 39 40 clonic, epileptic spasms, myoclonic, sequential, tonic) or non-motor (autonomic, behavior 41 42 43 arrest) presentation. The proposed classification allows the user to choose the degree of 44 45 detail when classifying seizures in this age group. 46 47 48 The proposed classification was successfully applied to 147 video-EEG recordings of 49 50 neonatal seizures. These included representative samples of the common etiologies; 51 52 hypoxic ischemic encephalopathy, stroke, infection, metabolic and genetic disorders. We 53 54 55 noted that certain seizure types were associated with particular etiologies emphasizing the 56 57 value in describing seizure types in this age group. 58 59 60 Keywords: Neonatal seizures, EEG, semiology, classification, epilepsy Page 3 of 39 Epilepsia 1 2 3 4 5 6 7 8 9 Key points: 10 11 • A new framework is proposed for seizures in neonates, in keeping with 12 13 2017 ILAE seizure classification while tailored to the neonatal period 14 15 • The framework emphasizes the necessity of EEG diagnosis of seizures in the 16 17 neonatal period. 18 19 • Seizures can occur with clinical manifestations (motor or non-motor) or 20 21 For Review Only without clinical manifestations (electrographic only). 22 23 • Descriptors are determined by the predominant clinical feature and divided 24 25 into motor and non-motor. 26 27 28 29 30 31 32 33 34 35 36 37 38 39 Definitions 40 41 1,2 42 For the purpose of this report, the following definitions are used: 43 44 • Gestational age (GA): time elapsed between the first day of the last menstrual period 45 46 47 and the day of deliver (completed weeks). 48 49 • Postmenstrual age (PMA): gestational age plus chronological age (in weeks). 50 51 52 • Conceptional age (CA): age from conception: PMA minus 2 weeks. 53 54 • Preterm infant: born before GA of 37 weeks. 55 56 57 • Neonatal period: Period from birth up to and under 28 days in term neonates, or from 58 59 birth up to a PMA of 40 weeks and under 28 days in preterm neonates. 60 Epilepsia Page 4 of 39 1 2 3 Introduction 4 5 6 The 2017 ILAE Position Papers on Classification of Seizure Types and the Epilepsies3,4 7 8 presented a framework for classification including seizure types, epilepsy types, and 9 10 11 syndromes. There is an emphasis on defining etiology at all levels of clinical classification in 12 13 addition to consideration of comorbidities. Terminology is updated with some new seizure 14 15 16 types included. The ILAE Commission on Classification & Terminology recognized that 17 18 seizures in the neonate require special considerations and therefore a Neonatal Task Force 19 20 21 was established with theFor aim of integratingReview seizures Onlyand epilepsies in this age group into the 22 23 2017 ILAE Classification. 24 25 26 Seizures are the most common neurological emergency in the neonatal period occurring in 27 28 1–5 per 1000 live births. The majority of neonatal seizures are symptomatic of an acute 29 30 illness with an underlying etiology either documented or suspected.5,6 Epilepsy syndromes 31 32 33 may present in the neonatal period and with the increasing availability of genetic testing 34 35 expanding numbers of neonatal epilepsies with genetic and metabolic etiologies are 36 37 7 38 recognized. Although many causes can give rise to neonatal seizures, a relatively small 39 40 number account for most seizures (Figure 1). 41 42 43 The clinical diagnosis of neonatal seizures is difficult, particularly in critically ill infants due to 44 45 the multitude of epileptic and non-epileptic clinical manifestations within the intensive care 46 47 12,13 14 48 setting. In the study by Malone, 20 video clips of paroxysmal events in neonates were 49 50 presented to 137 health professionals (mostly neonatologists and intensivists) with the aim 51 52 of classifying movements as seizure or non-seizure. Only 50% of events were correctly 53 54 55 classified. There was poor inter-observer agreement independent of observers’ specialty. 56 57 The immature state of the motor pathways15,16 in term and preterm neonates may account 58 59 17 60 for some of the difficulty in differentiating seizures from non-epileptic movements. In Page 5 of 39 Epilepsia 1 2 3 selected populations, particularly in infants with hypoxic-ischemic encephalopathy (HIE), 50- 4 5 6 80% of seizures are electrographic only and, as a result, the extent of the seizure burden 7 8 (time spent with seizures; defined as electrographic seizures in minutes per hour) may be 9 10 6,12-14,18 11 greatly underestimated. It has been suggested that hypothermia therapy for HIE 12 13 may also increase electro-clinical uncoupling of seizures, with a high incidence of 14 15 12 16 electrographic seizures in these infants. There is evidence that electrographic seizure 17 18 burden has a comparable effect on outcome as electro-clinical seizures.19-23 19 20 The ILAE defines a seizure as a transient occurrence of signs and/or symptoms due to 21 For Review Only 22 3,24 23 abnormal excessive or synchronous neuronal activity in the brain; however, a seizure 24 25 does not necessarily mean that a person has epilepsy. Electrographic only seizures are not 26 27 28 included in this definition. 29 30 Neonatal seizures are often categorized as clinical only, electro-clinical, or electrographic- 31 32 13,25 33 only. A clinical only seizure consists of a sudden paroxysm of abnormal clinical changes 34 35 without a definite EEG association. Whether or not all of these events are indeed of 36 37 38 epileptic origin remains uncertain in newborns. An electro-clinical seizure features definite 39 40 clinical signs simultaneously coupled with an electrographic seizure. An electrographic only 41 42 seizure refers to the presence of a definite EEG seizure that is not associated with any 43 44 45 evident clinical signs (synonyms: clinically silent or subclinical seizures).
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