Clinical presentation of new onset refractory status epilepticus in children (the pSERG cohort)

Sculier, Claudine; Aguilar, Cristina Barcia; Gaspard, Nicolas; Gaínza‐Lein, Marina; Fernández, Iván Sánchez; Amengual‐Gual, Marta; Anderson, Anne E.; Arya, Ravindra; Burrows, Brian T.; Brenton, J. Nicholas; Carpenter, Jessica; Chapman, Kevin; Clark, Justice; Gaillard, William D.; Glauser, Tracy A.; Goldstein, Joshua; Goodkin, Howard P.; Gorman, Mark; Lai, Yi‐Chen; McDonough, Tiffani L; Mikati, Mohamad A.; Nayak, Anuranjita; Peariso, Katrina; Riviello, James J.; Rusie, Allison; Sperberg, Katherine; Stredny, Coral M.; Tasker, Robert C.; Tchapyjnikov, Dmitry; Vasquez, Alejandra; Wainwright, Mark S.; Wilfong, Angus A.; Williams, Korwyn; Loddenkemper, Tobias; pSERG,

doi:10.1111/epi.16950

Cited by 24 publications

(38 citation statements)

References 40 publications

(129 reference statements)

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“…31 FIRES is a subset of NORSE, and all our cases having fever at the time of NORSE onset, fulfilled the diagnostic criteria of FIRES. 32 The complete recovery rate of 83% in our study was comparable with the “fever at onset” group of Sculler's study, 30 but was much higher than the “FIRES” group in Wu's study. 31…”

Section: Discussionsupporting

confidence: 79%

“…McMurray et al 29 also advocated a low threshold for using steroids in MIS-C, to downregulate the cytokine storm and the co-stimulators and adhesion molecules. In their study on pediatric NORSE, Sculier et al30 reported that return to baseline neurological function occurred in 25% of patients with preceding fever and 89% of patients with fever at the onset of NORSE. Wu et al 31 described a 22.8% mortality rate among pediatric NORSE patients.…”

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confidence: 99%

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The Clinical Characteristics and Prognosis of Children Presenting with New Onset Refractory Status Epilepticus in COVID-19 Related Multisystem Inflammatory Syndrome

Das

Chatterjee

Mondal

et al. 2022

J Pediatr Intensive Care

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Multisystem inflammatory syndrome in children (MIS-C) is a hyperinflammatory process leading to multiorgan failure and shock, occurring during the acute or post-infectious stage of severe acute respiratory syndrome coronavirus (SARS-CoV-2), and has two subtypes: para-infectious and post-infectious varieties. The new onset of refractory status epilepticus has rarely been described as the presenting feature of MIS-C. This retrospective study, conducted at Dr. B.C. Roy Post Graduate Institute of Pediatric Sciences, included children hospitalized between August 1, 2020 and July 31, 2021, with new-onset refractory status epilepticus (NORSE) and subsequently diagnosed to have MIS-C. Their clinico-demographic variables, treatment courses during hospital stays, laboratory reports, radiological and electrophysiological findings, and outcomes at discharge and follow-up over 1 year were recorded. At their 12 month visits, their motor disabilities (primary) and continuation of anti-epileptic drugs, and persistence of magnetic resonance imaging (MRI) brain abnormalities (secondary) were the outcome measures. The characteristics of the patients in the para-infectious and post-infectious groups were compared using the Mann-Whitney U test for continuous variables and the Chi-square test for categorical variables. There were eight and 10 patients in groups A and B, respectively. Patients in group B had significantly higher age, more prolonged refractory status epilepticus (RSE), use of anesthetics and ventilation, and longer pediatric intensive care unit (PICU) stay, while other clinical and laboratory parameters and short and long-term outcomes were not significantly different between the two groups. Eight patients developed hemiparesis, while two had quadriparesis in the acute stage, but 15 (83%) patients had complete recovery from their motor deficits by 1 year. At 1-year follow-up, 33 and 39% of patients, respectively, had abnormal MRI and electroencephalogram (EEG). Acute disseminated encephalitis and acute leukoencephalopathy were the most commonly observed MRI abnormalities in the acute phase, with prolonged persistence of cerebritis in patients in the post-infectious group, warranting long-term immunomodulation. Combined immunotherapy with intravenous immunoglobulin and steroids was effective in the acute phase. However, long-term anti-epileptic therapy was needed in both groups.

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Section: Discussionsupporting

confidence: 79%

mentioning

confidence: 99%

The Clinical Characteristics and Prognosis of Children Presenting with New Onset Refractory Status Epilepticus in COVID-19 Related Multisystem Inflammatory Syndrome

Das

Chatterjee

Mondal

et al. 2022

J Pediatr Intensive Care

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“…Although the mechanisms underlying RSE may be distinct in adult and pediatric patients, FIRES can thus develop at any age. It was recently demonstrated that FIRES is distinguishable from febrile SE in children not only by the timing of fever (part of the definition), but also by younger age and shorter SE duration in febrile SE 10 NORSE/FIRES has no evident geographical trend (M = 8, MA = 7, MP = 9, LA = 81.2%, LD = 2.1%).No studies have systematically assessed the geographical distribution of NORSE/FIRES.A recent systematic review of literature on pediatric FIRES reported that the incidence of NORSE was higher in certain parts of Asia.…”

Section: Resultsmentioning

confidence: 99%

International consensus recommendations for management of new onset refractory status epilepticus including febrile infection‐related epilepsy syndrome: Statements and supporting evidence

et al. 2022

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Objective This study was undertaken to develop consensus‐based recommendations for the management of adult and pediatric patients with new onset refractory status epilepticus (NORSE)/febrile infection‐related epilepsy syndrome (FIRES) based on best evidence and experience. Methods The Delphi methodology was followed. A facilitator group of nine experts was established, who defined the scope, users, and suggestions for recommendations. Following a review of the current literature, recommendation statements concerning diagnosis, treatment, and research directions were generated, which were then rated on a scale of 1 (strongly disagree) to 9 (strongly agree) by a panel of 48 experts in the field. Consensus that a statement was appropriate was reached if the median score was ≥7 and inappropriate if the median score was ≤3. The analysis of evidence was mapped to the results of each statement included in the Delphi survey. Results Overall, 85 recommendation statements achieved consensus. The recommendations are divided into five sections: (1) disease characteristics; (2) diagnostic testing and sampling; (3) acute treatment; (4) treatment in the postacute phase; and (5) research, registries, and future directions in NORSE/FIRES. The detailed results and discussion of all 85 statements are outlined herein. A corresponding summary of findings and practical flowsheets are presented in a companion article. Significance This detailed analysis offers insight into the supporting evidence and the current gaps in the literature that are associated with expert consensus statements related to NORSE/FIRES. The recommendations generated by this consensus can be used as a guide for the diagnosis, evaluation, and management of patients with NORSE/FIRES, and for planning of future research.

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“…Febrile infection-related epilepsy syndrome (FIRES), a subtype of new-onset refractory status epilepticus (NORSE), is a catastrophic epileptic syndrome that strikes previously healthy children between the age of 2 and early adulthood and has unknown pathogenesis and few treatments ( Fox et al, 2017 ; Sculier et al, 2021 ; Lattanzi et al, 2022 ; Nausch et al, 2022 ). Affected children experience a non-specific illness with fever starting between 2 weeks and 24 h before the onset of prolonged refractory status epilepticus.…”

Section: Seizures and Epilepsy Associated With Viral Infections Of Th...mentioning

confidence: 99%

Molecular Mechanisms in the Genesis of Seizures and Epilepsy Associated With Viral Infection

Löscher

Howe

2022

Front. Mol. Neurosci.

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Seizures are a common presenting symptom during viral infections of the central nervous system (CNS) and can occur during the initial phase of infection (“early” or acute symptomatic seizures), after recovery (“late” or spontaneous seizures, indicating the development of acquired epilepsy), or both. The development of acute and delayed seizures may have shared as well as unique pathogenic mechanisms and prognostic implications. Based on an extensive review of the literature, we present an overview of viruses that are associated with early and late seizures in humans. We then describe potential pathophysiologic mechanisms underlying ictogenesis and epileptogenesis, including routes of neuroinvasion, viral control and clearance, systemic inflammation, alterations of the blood-brain barrier, neuroinflammation, and inflammation-induced molecular reorganization of synapses and neural circuits. We provide clinical and animal model findings to highlight commonalities and differences in these processes across various neurotropic or neuropathogenic viruses, including herpesviruses, SARS-CoV-2, flaviviruses, and picornaviruses. In addition, we extensively review the literature regarding Theiler’s murine encephalomyelitis virus (TMEV). This picornavirus, although not pathogenic for humans, is possibly the best-characterized model for understanding the molecular mechanisms that drive seizures, epilepsy, and hippocampal damage during viral infection. An enhanced understanding of these mechanisms derived from the TMEV model may lead to novel therapeutic interventions that interfere with ictogenesis and epileptogenesis, even within non-infectious contexts.

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Clinical presentation of new onset refractory status epilepticus in children (the pSERG cohort)

Abstract: This is an open access article under the terms of the Creative Commons Attribution-NonCommercial License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.

Cited by 24 publications

References 40 publications

The Clinical Characteristics and Prognosis of Children Presenting with New Onset Refractory Status Epilepticus in COVID-19 Related Multisystem Inflammatory Syndrome

The Clinical Characteristics and Prognosis of Children Presenting with New Onset Refractory Status Epilepticus in COVID-19 Related Multisystem Inflammatory Syndrome

International consensus recommendations for management of new onset refractory status epilepticus including febrile infection‐related epilepsy syndrome: Statements and supporting evidence

Molecular Mechanisms in the Genesis of Seizures and Epilepsy Associated With Viral Infection

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