TY - JOUR
T1 - Immediate outcomes in early life epilepsy
T2 - A contemporary account
AU - Berg, Anne T.
AU - Wusthoff, Courtney
AU - Shellhaas, Renée A.
AU - Loddenkemper, Tobias
AU - Grinspan, Zachary M.
AU - Saneto, Russell P.
AU - Knupp, Kelly G.
AU - Patel, Anup
AU - Sullivan, Joseph E.
AU - Kossoff, Eric H.
AU - Chu, Catherine J.
AU - Massey, Shavonne
AU - Valencia, Ignacio
AU - Keator, Cynthia
AU - Wirrell, Elaine C.
AU - Coryell, Jason
AU - Millichap, John J.
AU - Gaillard, William D.
N1 - Funding Information:
This study was funded by the Pediatric Epilepsy Research Foundation , Dallas, TX.
Funding Information:
This study was funded by the Pediatric Epilepsy Research Foundation, Dallas, TX.This project was also supported by NIH/NCRR Colorado CTSI Grant Number UL1 RR025780. Its contents are the authors' sole responsibility and do not necessarily represent official NIH views.
Publisher Copyright:
© 2019
PY - 2019/8
Y1 - 2019/8
N2 - 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.
AB - 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.
KW - Developmental delay
KW - Drug resistance
KW - Infantile spasms
KW - Mortality
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U2 - 10.1016/j.yebeh.2019.05.011
DO - 10.1016/j.yebeh.2019.05.011
M3 - Article
C2 - 31181428
AN - SCOPUS:85066836188
SN - 1525-5050
VL - 97
SP - 44
EP - 50
JO - Epilepsy and Behavior
JF - Epilepsy and Behavior
ER -