Rates of Status Epilepticus and Sudden Unexplained Death in Epilepsy in People with Genetic Developmental and Epileptic Encephalopathies

AM Donnan; AL Schneider; S Russ-Hall; L Churilov; IE Scheffer

Journal article

Rates of Status Epilepticus and Sudden Unexplained Death in Epilepsy in People with Genetic Developmental and Epileptic Encephalopathies

AM Donnan, AL Schneider, S Russ-Hall, L Churilov, IE Scheffer

Neurology | LIPPINCOTT WILLIAMS & WILKINS | Published : 2023

DOI: 10.1212/WNL.0000000000207080

Abstract

Background and ObjectivesThe genetic developmental and epileptic encephalopathies (DEEs) comprise a large group of severe epilepsy syndromes, with a wide phenotypic spectrum. Currently, the rates of convulsive status epilepticus (CSE), nonconvulsive status epilepticus (NCSE), and sudden unexplained death in epilepsy (SUDEP) in these diseases are not well understood. We aimed to describe the proportions of patients with frequently observed genetic DEEs who developed CSE, NCSE, mortality, and SUDEP. Understanding the risks of these serious presentations in each genetic DEE will enable earlier diagnosis and appropriate management.MethodsIn this retrospective analysis of patients with a genetic ..

View full abstract

University of Melbourne Researchers

Amy Schneider Author

Leonid Churilov Author

Ingrid Scheffer Author

Grants

Awarded by National Health and Medical Research Council

Funding Acknowledgements

The authors gratefully acknowledge support provided by the Australian Epilepsy Research Foundation grant, Australian National Health and Medical Research Council (NHMRC) Center for Research Excellence Grant (GNT2006841), and NHMRC Synergy Grant (GNT2010562). Professor Scheffer is also supported by a NHMRC Senior Investigator Fellowship (GNT1172897).