Anne T Berg1, Courtney Wusthoff2, Renée A Shellhaas3, Tobias Loddenkemper4, Zachary M Grinspan5, Russell P Saneto6, Kelly G Knupp7, Anup Patel8, Joseph E Sullivan9, Eric H Kossoff10, Catherine J Chu11, Shavonne Massey12, Ignacio Valencia13, Cynthia Keator14, Elaine C Wirrell15, Jason Coryell16, John J Millichap17, William D Gaillard18. 1. Epilepsy Center, Ann & Robert H. Lurie Children's Hospital of Chicago; Department of Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, IL, United States of America. Electronic address: atberg@luriechildrens.org. 2. Division of Child Neurology, Stanford University, Palo Alto, CA, United States of America. 3. Department of Pediatrics, University of Michigan, Ann Arbor, MI, United States of America. 4. Division of Epilepsy and Clinical Neurophysiology, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States of America. 5. Weill Cornell Medicine, New York Presbyterian Hospital, Health Information Technology Evaluation Collaborative, New York, NY, United States of America. 6. Division of Pediatric Neurology, Seattle Children's Hospital, Department of Neurology, University of Washington, Seattle, WA, United States of America. 7. Department of Pediatrics and Neurology, School of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO, United States of America. 8. Department of Pediatrics, The Ohio State University; Nationwide Children's Hospital, Columbus, OH, United States of America. 9. Department of Neurology, University of California San Francisco, San Francisco, CA, United States of America. 10. Departments of Neurology and Pediatrics, Johns Hopkins Hospital, Baltimore, MD, United States of America. 11. Department of Neurology, Massachusetts General Hospital, Boston, MA, United States of America. 12. Departments of Neurology and Pediatrics, The Children's Hospital of Philadelphia and The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States of America. 13. Section of Neurology, St. Christopher's Hospital for Children, Drexel University College of Medicine, Philadelphia, PA, United States of America. 14. Cook Children's Health Care System, Jane and John Justin Neurosciences Center, Fort Worth, TX, United States of America. 15. Department of Neurology, Mayo Clinic, Rochester, MN, United States of America. 16. Departments of Pediatrics & Neurology, Oregon Health & Sciences University, Portland, OR, United States of America. 17. Epilepsy Center, Ann & Robert H. Lurie Children's Hospital of Chicago; Department of Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, IL, United States of America. 18. Department of Neurology, Children's National Health System, George Washington University School of Medicine, Washington, DC, United States of America.
Abstract
RATIONALE: Early-life epilepsies (ELEs) include some of the most challenging forms of epilepsy to manage. Given recent diagnostic and therapeutic advances, a contemporary assessment of the immediate short-term outcomes can provide a valuable framework for identifying priorities and benchmarks for evaluating quality improvement efforts. METHODS: Children with newly diagnosed epilepsy and onset <3 years were prospectively recruited through 17 US hospitals, from 2012 to 2015 and followed for 1 year after diagnosis. Short-term outcome included mortality, drug resistance, evolution of nonsyndromic epilepsy to infantile spasms (IS) and from IS to other epilepsies, and developmental decline. Multivariable analyses assessed the risk of each outcome. RESULTS: Seven hundred seventy-five children were recruited, including 408 (53%) boys. Median age at onset was 7.5 months (interquartile range (IQR): 4.2-16.5), and 509 (66%) had onset in the first year of life. Of 22 deaths that occurred within one year of epilepsy diagnosis, 21 were children with epilepsy onset in infancy (<12 months). Of 680 children followed ≥6 months, 239 (35%) developed drug-resistant seizures; 34/227 (15%) infants with nonsyndromic epilepsy developed IS, and 48/210 (23%) initially presenting with IS developed additional seizure types. One hundred of 435 (23%) with initially typical development or only mild/equivocal delays at seizure onset, had clear developmental impairment within one year after initial diagnosis. Each outcome had a different set of predictors; however, younger age and impaired development at seizure onset were broadly indicative of poorer outcomes. Type of epilepsy and early identification of underlying cause were not reliable predictors of these outcomes. CONCLUSION: Early-life epilepsies carry a high risk of poor outcome which is evident shortly after epilepsy diagnosis. Onset in infancy and developmental delay is associated with an especially high risk, regardless of epilepsy type. The likelihood of poor outcomes is worrisome regardless of specific clinical profiles.
RATIONALE: Early-life epilepsies (ELEs) include some of the most challenging forms of epilepsy to manage. Given recent diagnostic and therapeutic advances, a contemporary assessment of the immediate short-term outcomes can provide a valuable framework for identifying priorities and benchmarks for evaluating quality improvement efforts. METHODS:Children with newly diagnosed epilepsy and onset <3 years were prospectively recruited through 17 US hospitals, from 2012 to 2015 and followed for 1 year after diagnosis. Short-term outcome included mortality, drug resistance, evolution of nonsyndromic epilepsy to infantile spasms (IS) and from IS to other epilepsies, and developmental decline. Multivariable analyses assessed the risk of each outcome. RESULTS: Seven hundred seventy-five children were recruited, including 408 (53%) boys. Median age at onset was 7.5 months (interquartile range (IQR): 4.2-16.5), and 509 (66%) had onset in the first year of life. Of 22 deaths that occurred within one year of epilepsy diagnosis, 21 were children with epilepsy onset in infancy (<12 months). Of 680 children followed ≥6 months, 239 (35%) developed drug-resistant seizures; 34/227 (15%) infants with nonsyndromic epilepsy developed IS, and 48/210 (23%) initially presenting with IS developed additional seizure types. One hundred of 435 (23%) with initially typical development or only mild/equivocal delays at seizure onset, had clear developmental impairment within one year after initial diagnosis. Each outcome had a different set of predictors; however, younger age and impaired development at seizure onset were broadly indicative of poorer outcomes. Type of epilepsy and early identification of underlying cause were not reliable predictors of these outcomes. CONCLUSION: Early-life epilepsies carry a high risk of poor outcome which is evident shortly after epilepsy diagnosis. Onset in infancy and developmental delay is associated with an especially high risk, regardless of epilepsy type. The likelihood of poor outcomes is worrisome regardless of specific clinical profiles.
Authors: Paul A Harris; Robert Taylor; Robert Thielke; Jonathon Payne; Nathaniel Gonzalez; Jose G Conde Journal: J Biomed Inform Date: 2008-09-30 Impact factor: 6.317
Authors: Anne T Berg; Samuel F Berkovic; Martin J Brodie; Jeffrey Buchhalter; J Helen Cross; Walter van Emde Boas; Jerome Engel; Jacqueline French; Tracy A Glauser; Gary W Mathern; Solomon L Moshé; Douglas Nordli; Perrine Plouin; Ingrid E Scheffer Journal: Epilepsia Date: 2010-02-26 Impact factor: 5.864
Authors: Anne T Berg; Samya Chakravorty; Sookyong Koh; Zachary M Grinspan; Renée A Shellhaas; Russell P Saneto; Elaine C Wirrell; Jason Coryell; Catherine J Chu; John R Mytinger; William D Gaillard; Ignacio Valencia; Kelly G Knupp; Tobias Loddenkemper; Joseph E Sullivan; Annapurna Poduri; John J Millichap; Cynthia Keator; Courtney Wusthoff; Nicole Ryan; William B Dobyns; Madhuri Hegde Journal: PLoS One Date: 2018-03-08 Impact factor: 3.240
Authors: Fiona M Baumer; Nancy A McNamara; Anthony L Fine; Elia Pestana-Knight; Renée A Shellhaas; Zihuai He; Daniel H Arndt; William D Gaillard; Sarah A Kelley; Margot Nagan; Adam P Ostendorf; Nilika S Singhal; Laura Speltz; Kevin E Chapman Journal: J Pediatr Date: 2021-01-20 Impact factor: 4.406