Super-Refractory Status Epilepticus in Children: A Retrospective Cohort Study

Vasquez, Alejandra; Farias‐Moeller, Raquel; Sánchez-Fernández, Iván; Abend, Nicholas S.; Amengual‐Gual, Marta; Anderson, Anne E.; Arya, Ravindra; Brenton, J. Nicholas; Carpenter, Jessica; Chapman, Kevin; Clark, Justice; Gaillard, William D.; Glauser, Tracy A.; Goldstein, Joshua; Goodkin, Howard P.; Guerriero, Réjean M.; Lai, Yi‐Chen; McDonough, Tiffani L; Mikati, Mohamad A.; Morgan, Lindsey A.; Novotny, Edward J.; Ostendorf, Adam P.; Payne, Eric T.; Peariso, Katrina; Piantino, Juan; Riviello, James J.; Sands, Tristan T.; Sannagowdara, Kumar; Tasker, Robert C.; Tchapyjnikov, Dmitry; Topjian, Alexis; Wainwright, Mark S.; Wilfong, Angus A.; Williams, Korwyn; Loddenkemper, Tobias

doi:10.1097/pcc.0000000000002786

Cited by 13 publications

(15 citation statements)

References 69 publications

(105 reference statements)

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“…These findings are concordant with data supporting that super-refractory status epilepticus causes worse neurological outcomes and lower rates of return to baseline cognitive function than non-super-refractory status epilepticus ( 18 ). One study reported that 58% of patients with NORSE had neurocognitive deficits, of which 30% were severe ( 19 ).…”

Section: Discussionsupporting

confidence: 89%

Long-term neuropsychological outcomes in children with febrile infection-related epilepsy syndrome (FIRES) treated with anakinra

et al. 2023

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BackgroundFebrile-infection related epilepsy syndrome (FIRES) is a rare epilepsy syndrome in which a previously healthy individual develops refractory status epilepticus in the setting of a preceding febrile illness. There are limited data regarding detailed long-term outcomes. This study aims to describe the long-term neuropsychological outcomes in a series of pediatric patients with FIRES.MethodsThis is a retrospective multi-center case series of pediatric patients with a diagnosis of FIRES treated acutely with anakinra who had neuropsychological testing at least 12 months after status epilepticus onset. Each patient underwent comprehensive neuropsychological evaluation as part of routine clinical care. Additional data collection included the acute seizure presentation, medication exposures, and outcomes.ResultsThere were six patients identified with a median age of 11.08 years (IQR: 8.19–11.23) at status epilepticus onset. Anakinra initiation was a median of 11 days (IQR: 9.25–13.50) after hospital admission. All patients had ongoing seizures and none of the patients returned to baseline cognitive function with a median follow-up of 40 months (IQR 35–51). Of the five patients with serial full-scale IQ testing, three demonstrated a decline in scores over time. Testing results revealed a diffuse pattern of deficits across domains and all patients required special education and/or accommodations for academic learning.ConclusionsDespite treatment with anakinra, neuropsychological outcomes in this series of pediatric patients with FIRES demonstrated ongoing diffuse neurocognitive impairment. Future research will need to explore the predictors of long-term neurocognitive outcomes in patients with FIRES and to evaluate if acute treatment interventions improve these outcomes.

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Section: Discussionsupporting

confidence: 89%

Long-term neuropsychological outcomes in children with febrile infection-related epilepsy syndrome (FIRES) treated with anakinra

et al. 2023

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“…Two infusions with different drugs were utilized in 32.3% and three or more different drugs were used in 19.4% of cases. Most of the patients with SR SE (96.8%) received midazolam as the first choice [54]. The same study observed that SRSE patients had delayed initiation of first non-ben-zodiazepine antiseizure medication in comparison with the non-SRSE patients (149 min vs. 62 min; p = 0.030).…”

Section: Super-refractory Status Epilepticusmentioning

confidence: 76%

Management and prognosis of pediatric status epilepticus

et al. 2022

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Background Pediatric status epilepticus is a neurological emergency with the potential for severe developmental and neurological consequences. Prompt diagnosis and management are necessary. Objectives To outline the existing best available evidence for managing pediatric and neonatal status epilepticus, in the light of emerging randomized controlled studies. We also focus on short and long-term prognoses. Materials and methods This is a systematic overview of the existing literature. Results Status epilepticus, its treatment, and prognosis are usually based on the continuation of seizure activity at 5 and 30 min. Refractory and super-refractory status epilepticus further complicates management and requires continuous EEG monitoring with regular reassessment and adjustment of therapy. Benzodiazepines have been accepted as the first line of treatment on the basis of reasonable evidence. Emerging randomized controlled trials demonstrate equal efficacy for parenterally administered phenytoin, levetiracetam, and valproic acid as second-line agents. Beyond this, the evidence for third-line options is sparse. However, encouraging evidence for midazolam and ketamine exists with further data required for immunological, dietary, and surgical interventions. Conclusion Our overview of the management of pediatric and neonatal status epilepticus based on available evidence emphasizes the need for evidence-based guidelines to manage status epilepticus that fails to respond to second-line treatment.

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“…Vasquez et al showed that pediatric SRSE patients with delayed non‐BZD ASM initiation had more medical complications, higher mortality, and lower return to neurological baseline than non‐SRSE patients. However, these associations were not adjusted for potential confounders 17 . Delayed BZD use and underdosing were associated with SE refractoriness and unfavorable outcomes 18 .…”

Section: Discussionmentioning

confidence: 97%

“…Furthermore, data from adults might not directly apply to children since the physical and neurochemical characteristics of the developed brain differ from those of the developing brain. However, several recent studies on children and adolescents with SE, refractory SE (RSE), and super‐refractory SE (SRSE) have been published 9‐18 …”

Section: Introductionmentioning

confidence: 99%

“…However, several recent studies on children and adolescents with SE, refractory SE (RSE), and super-refractory SE (SRSE) have been published. [9][10][11][12][13][14][15][16][17][18] This prospective nationwide surveillance study was performed to provide up-to-date real-world data on the underlying etiologies, diagnostic and therapeutic approaches-both out-of-and in-hospital-and outcomes of children and adolescents with community-or hospitalonset SE requiring PICU admission for ≥4 hours. In contrast to previous reports, this prospective nationwide study over a 2-year period included all German children´s hospitals, thus providing a comprehensive and representative data with regard to this important medical issue.…”

Section: Introductionmentioning

confidence: 99%

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Epileptic Status in a PEDiatric cohort (ESPED) requiring intensive care treatment: A multicenter, national, two‐year prospective surveillance study

et al. 2023

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Objective:The aim of this study was to provide seizure etiology, semiology, underlying conditions, and out-of-and in-hospital diagnostics, treatment, and outcome data on children with out-of-or in-hospital-onset status epilepticus (SE) according to the International League Against Epilepsy definition that required admission to the pediatric intensive care unit (PICU) for ≥4 hours. Methods: This prospective national surveillance study on SE in childhood and adolescence was conducted over 2 years (07/2019-06/2021). Results: This study examined 481 SE episodes in 481 children with a median age of 43 months (1 month to 17 years 11 months), of which 46.2% were female and 50.7% had a previous seizure history. The most frequent acute SE cause was a prolonged, complicated febrile seizure (20.6%). The most common initial seizure types were generalized seizures (49.9%), focal seizures (18.0%), and unknown types (12.1%); 40.5% of patients suffered from refractory SE and 5.0% from superrefractory SE. The three most common medications administered by nonmedically trained individuals were diazepam, midazolam, and antipyretics. The three most frequent anti-seizure medications (ASMs) administered by the emergency physician were midazolam, diazepam, and propofol. The three most common ASMs used in the clinical setting were midazolam, levetiracetam, and phenobarbital. New ASMs administered included lacosamide, brivaracetam, perampanel, stiripentol, and eslicarbazepine. Status epilepticus terminated in 16.0% in the preclinical setting, 19.1% in the emergency department, and 58.0% in the PICU;the outcome was unknown for 6.9%. The median PICU stay length was 2 (1-121) days. The median modified Rankin scale was 1 (0-5) on admission and 2 (0-6) at discharge. New neurological deficits after SE were observed in 6.2%. The mortality rate was 3.5%.

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Super-Refractory Status Epilepticus in Children: A Retrospective Cohort Study

Cited by 13 publications

References 69 publications

Long-term neuropsychological outcomes in children with febrile infection-related epilepsy syndrome (FIRES) treated with anakinra

Long-term neuropsychological outcomes in children with febrile infection-related epilepsy syndrome (FIRES) treated with anakinra

Management and prognosis of pediatric status epilepticus

Epileptic Status in a PEDiatric cohort (ESPED) requiring intensive care treatment: A multicenter, national, two‐year prospective surveillance study

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