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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 44-50 |
| Number of pages | 7 |
| Journal | Epilepsy and Behavior |
| Volume | 97 |
| DOIs | |
| State | Published - Aug 1 2019 |
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Keywords
- Developmental delay
- Drug resistance
- Infantile spasms
- Mortality
ASJC Scopus subject areas
- Neurology
- Clinical Neurology
- Behavioral Neuroscience
Cite this
Immediate outcomes in early life epilepsy : A contemporary account. / Berg, Anne T.; Wusthoff, Courtney; Shellhaas, Renée A.; Loddenkemper, Tobias; Grinspan, Zachary M.; Saneto, Russell P.; Knupp, Kelly G.; Patel, Anup; Sullivan, Joseph E.; Kossoff, Eric H; Chu, Catherine J.; Massey, Shavonne; Valencia, Ignacio; Keator, Cynthia; Wirrell, Elaine C.; Coryell, Jason; Millichap, John J.; Gaillard, William D.
In: Epilepsy and Behavior, Vol. 97, 01.08.2019, p. 44-50.Research output: Contribution to journal › Article
}
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.
PY - 2019/8/1
Y1 - 2019/8/1
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
UR - http://www.scopus.com/inward/record.url?scp=85066836188&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85066836188&partnerID=8YFLogxK
U2 - 10.1016/j.yebeh.2019.05.011
DO - 10.1016/j.yebeh.2019.05.011
M3 - Article
C2 - 31181428
AN - SCOPUS:85066836188
VL - 97
SP - 44
EP - 50
JO - Epilepsy and Behavior
JF - Epilepsy and Behavior
SN - 1525-5050
ER -