Neonatal Seizures: Is there a relationship between ictal electro-clinical features and etiology? – A critical appraisal based on a systematic literature review

Abstract The aim of this study was to evaluate whether specific etiologies of neonatal seizures have distinct ictal electro- clinical features. A systematic review of English articles using the PubMed database since 2004 (last update 9/26/16). Search terms included text words and MeSH terms related to neonatal seizures. Eligible articles included reports of neonates with seizures with a full description of seizure semiology and electroclinical findings. Independent extraction of data was performed by two authors using predefined data fields, including study quality indicators. Data was collected for every individual patient described in the articles. The dataset was analyzed with the Fisher?s exact test. The initial search led to 8507 titles; using filters, 2910 titles and abstracts were identified, with 177 full texts selected to be read. Fifty seven studies were included in the analysis with 151 neonates (37.7 male and 62.9% term). Genetic etiologies (51%) and sequential seizures (41.1%) predominated in this sample and hypoxic ischemic encephalopathy (HIE) accounted for only 4%. The low prevalence of HIE observed was probably due to a publication bias. A significant association was found between etiology and seizure type: hemorrhage with autonomic seizures (p=0.003), CNS infection and stroke with clonic seizures (p=0.042, pPeer reviewe

The ILAE classification of seizures and the epilepsies : Modification for seizures in the neonate. Position paper by the ILAE Task Force on Neonatal Seizures

Seizures are the most common neurological emergency in the neonatal period and in contrast to those in infancy and childhood, are often provoked seizures with an acute cause and may be electrographic-only. Hence, neonatal seizures may not fit easily into classification schemes for seizures and epilepsies primarily developed for older children and adults. A Neonatal Seizures Task Force was established by the International League Against Epilepsy (ILAE) to develop a modification of the 2017 ILAE Classification of Seizures and Epilepsies, relevant to neonates. The neonatal classification framework emphasizes the role of electroencephalography (EEG) in the diagnosis of seizures in the neonate and includes a classification of seizure types relevant to this age group. The seizure type is determined by the predominant clinical feature. Many neonatal seizures are electrographic-only with no evident clinical features; therefore, these are included in the proposed classification. Clinical events without an EEG correlate are not included. Because seizures in the neonatal period have been shown to have a focal onset, a division into focal and generalized is unnecessary. Seizures can have a motor (automatisms, clonic, epileptic spasms, myoclonic, tonic), non-motor (autonomic, behavior arrest), or sequential presentation. The classification allows the user to choose the level of detail when classifying seizures in this age group.Peer reviewe

EPILEPSIE A L'EAU CHAUDE DU NOURRISSON

PARIS7-Villemin (751102101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Mutations in TCF4, Encoding a Class I Basic Helix-Loop-Helix Transcription Factor, Are Responsible for Pitt-Hopkins Syndrome, a Severe Epileptic Encephalopathy Associated with Autonomic Dysfunction

Pitt-Hopkins syndrome (PHS) is a rare syndromic encephalopathy characterized by daily bouts of hyperventilation and a facial gestalt. We report a 1.8-Mb de novo microdeletion on chromosome 18q21.1, identified by array–comparative genomic hybridization in one patient with PHS. We subsequently identified two de novo heterozygous missense mutations of a conserved amino acid in the basic region of the TCF4 gene in three additional subjects with PHS. These findings demonstrate that TCF4 anomalies are responsible for PHS and provide the first evidence of a human disorder related to class I basic helix-loop-helix transcription-factor defects (also known as “E proteins”). Moreover, our data may shed new light on the normal processes underlying autonomic nervous system development and maintenance of an appropriate ventilatory neuronal circuitry

Application of the International League Against Epilepsy Neonatal Seizure Framework to an international panel of medical personnel

Objective: The International League Against Epilepsy (ILAE) Neonatal Seizure Framework was tested by medical personnel.// Methods: Attendees at the 2016 ILAE European Congress on Epileptology in Prague, the International Video-EEG Course in Pediatric Epilepsies in Madrid 2017, and a local meeting in Utrecht 2018, were introduced to the proposed ILAE neonatal classification system with teaching videos covering the seven types of clinical seizures in the proposed neonatal classification system. Five test digital video recordings of electroencephalography (EEG)-confirmed motor neonatal seizures were then shown and classified by the rater based on their knowledge of the proposed ILAE Neonatal Seizure Framework. A multi-rater Kappa statistic was used to assess the agreement between observers and the true diagnosis. // Results: The responses of 194 raters were obtained. There was no single predominant classification system that was currently used by the raters. Using the ILAE framework, 78%–93% of raters correctly identified the clinical seizure type for each neonate; the overall inter-rater agreement (Kappa statistic) was 0.67. The clonic motor seizure type was most frequently accurately identified (93% of the time; κ = 0.870). EEG technicians correctly identified all presented motor seizure types more frequently than any other group (accuracy = 0.9).// Significance: The ILAE Neonatal Seizure Framework was judged by most raters to be better than other systems for the classification of clinical seizures. Among all seizure types presented, clonic seizures appeared to be the easiest to accurately identify. Average accuracy across the five seizure types was 84.5%. These data suggest that the ILAE neonatal seizure classification may be used by all healthcare professionals to correctly identify the predominant clinical seizure type

Application of the International League Against Epilepsy Neonatal Seizure Framework to an international panel of medical personnel

Objective The International League Against Epilepsy (ILAE) Neonatal Seizure Framework was tested by medical personnel.Methods Attendees at the 2016 ILAE European Congress on Epileptology in Prague, the International Video-EEG Course in Pediatric Epilepsies in Madrid 2017, and a local meeting in Utrecht 2018, were introduced to the proposed ILAE neonatal classification system with teaching videos covering the seven types of clinical seizures in the proposed neonatal classification system. Five test digital video recordings of electroencephalography (EEG)-confirmed motor neonatal seizures were then shown and classified by the rater based on their knowledge of the proposed ILAE Neonatal Seizure Framework. A multi-rater Kappa statistic was used to assess the agreement between observers and the true diagnosis.Results The responses of 194 raters were obtained. There was no single predominant classification system that was currently used by the raters. Using the ILAE framework, 78%-93% of raters correctly identified the clinical seizure type for each neonate; the overall inter-rater agreement (Kappa statistic) was 0.67. The clonic motor seizure type was most frequently accurately identified (93% of the time; ? = 0.870). EEG technicians correctly identified all presented motor seizure types more frequently than any other group (accuracy = 0.9).Significance The ILAE Neonatal Seizure Framework was judged by most raters to be better than other systems for the classification of clinical seizures. Among all seizure types presented, clonic seizures appeared to be the easiest to accurately identify. Average accuracy across the five seizure types was 84.5%. These data suggest that the ILAE neonatal seizure classification may be used by all healthcare professionals to correctly identify the predominant clinical seizure type.Peer reviewe

Electroencephalography in premature and full-term infants. Developmental features and glossary.: Electroencephalography in premature and full-term infants

International audienceFollowing the pioneering work of C. Dreyfus-Brisac and N. Monod, research into neonatal electroencephalography (EEG) has developed tremendously in France. French neurophysiologists who had been trained in Paris (France) collaborated on a joint project on the introduction, development, and currently available neonatal EEG recording techniques. They assessed the analytical criteria for the different maturational stages and standardized neonatal EEG terminology on the basis of the large amount of data available in the French and the English literature. The results of their work were presented in 1999. Since the first edition, technology has moved towards the widespread use of digitized recordings. Although the data obtained with analog recordings can be applied to digitized EEG tracings, the present edition, including new published data, is illustrated with digitized recordings. Herein, the reader can find a comprehensive description of EEG features and neonatal behavioural states at different gestational ages, and also a definition of the main aspects and patterns of both pathological and normal EEGs, presented in glossary form. In both sections, numerous illustrations have been provided. This precise neonatal EEG terminology should improve homogeneity in the analysis of neonatal EEG recordings, and facilitate the setting up of multicentric studies on certain aspects of normal EEG recordings and various pathological patterns