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Identification of etiologies according to baseline clinical features of pediatric new-onset refractory status epilepticus in single center retrospective study

Authors
 Sangbo Lee  ;  Se Hee Kim  ;  Heung Dong Kim  ;  Joon Soo Lee  ;  Ara Ko  ;  Hoon-Chul Kang 
Citation
 SEIZURE-EUROPEAN JOURNAL OF EPILEPSY, Vol.120 : 49-55, 2024-08 
Journal Title
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY
ISSN
 1059-1311 
Issue Date
2024-08
MeSH
Adolescent ; Child ; Child, Preschool ; Drug Resistant Epilepsy* / diagnosis ; Encephalitis / complications ; Encephalitis / diagnosis ; Epilepsies, Myoclonic / diagnosis ; Epilepsies, Myoclonic / physiopathology ; Female ; Humans ; Infant ; Male ; Retrospective Studies ; Status Epilepticus* / diagnosis ; Status Epilepticus* / etiology
Keywords
Febrile infection-related epilepsy syndrome ; New-onset refractory status epilepticus ; Refractory status epilepticus ; Super refractory status epilepticus
Abstract
Purpose: New-onset refractory status epilepticus (NORSE) is defined as a state of prolonged seizure activity that does not improve despite the appropriate administration of medications, with underlying causes unknown after the initial diagnosis of status epilepticus. Because episodes of NORSE are accompanied by severe complications and a high risk of mortality, the prompt identification of the underlying cause is crucial for effective treatment and outcome prediction. This study assessed the relationship of NORSE etiologies with baseline clinical features in pediatric population.

Methods: Seventy-one pediatric patients, under 18 years of age at the initial diagnosis (4.50 ± 4.04, mean ± standard deviation), who experienced at least one episode of NORSE and underwent a comprehensive diagnostic evaluation between January 2005 and June 2020 at our center, were retrospectively selected. We reviewed clinical features at disease onset and long-term follow-up data. Uniform manifold approximation and projection (UMAP) was used to distinguish etiological clusters according to baseline clinical characteristics, and further analysis was performed based on underlying etiologies.

Results: Two distinct etiological groups-genetic and non-genetic-were identified based on the UMAP of clinical characteristics. Dravet syndrome (12/15, 80%) was more predominant in patients with a genetic diagnosis, whereas cryptogenic NORSE and encephalitis were prevalent in patients without a genetic diagnosis. The analysis of etiological categories revealed that age at the onset of status epilepticus (P=0.021) and progression to super refractory status epilepticus (SRSE) (P=0.038) were independently associated with differences in etiologies.

Conclusion: Several clinical features in patients with NORSE, including the age of onset and the development of SRSE, can help identify underlying causes, which necessitate prompt and adequate treatment.
Full Text
https://linkinghub.elsevier.com/retrieve/pii/S1059-1311(24)00167-5
DOI
10.1016/j.seizure.2024.06.009
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아과학교실) > 1. Journal Papers
Yonsei Authors
Kang, Hoon Chul(강훈철) ORCID logo https://orcid.org/0000-0002-3659-8847
Ko, A Ra(고아라)
Kim, Se Hee(김세희) ORCID logo https://orcid.org/0000-0001-7773-1942
Kim, Heung Dong(김흥동) ORCID logo https://orcid.org/0000-0002-8031-7336
Lee, Joon Soo(이준수) ORCID logo https://orcid.org/0000-0001-9036-9343
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/201331
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