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1.
Epilepsia ; 65(6): e87-e96, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38625055

RESUMO

Febrile infection-related epilepsy syndrome (FIRES) is a subset of new onset refractory status epilepticus (NORSE) that involves a febrile infection prior to the onset of the refractory status epilepticus. It is unclear whether FIRES and non-FIRES NORSE are distinct conditions. Here, we compare 34 patients with FIRES to 30 patients with non-FIRES NORSE for demographics, clinical features, neuroimaging, and outcomes. Because patients with FIRES were younger than patients with non-FIRES NORSE (median = 28 vs. 48 years old, p = .048) and more likely cryptogenic (odds ratio = 6.89), we next ran a regression analysis using age or etiology as a covariate. Respiratory and gastrointestinal prodromes occurred more frequently in FIRES patients, but no difference was found for non-infection-related prodromes. Status epilepticus subtype, cerebrospinal fluid (CSF) and magnetic resonance imaging findings, and outcomes were similar. However, FIRES cases were more frequently cryptogenic; had higher CSF interleukin 6, CSF macrophage inflammatory protein-1 alpha (MIP-1a), and serum chemokine ligand 2 (CCL2) levels; and received more antiseizure medications and immunotherapy. After controlling for age or etiology, no differences were observed in presenting symptoms and signs or inflammatory biomarkers, suggesting that FIRES and non-FIRES NORSE are very similar conditions.


Assuntos
Febre , Estado Epiléptico , Humanos , Estado Epiléptico/etiologia , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Febre/etiologia , Febre/complicações , Adulto Jovem , Adolescente , Epilepsia Resistente a Medicamentos/etiologia , Criança , Convulsões Febris/etiologia , Eletroencefalografia , Idoso , Imageamento por Ressonância Magnética , Síndromes Epilépticas , Pré-Escolar
2.
Epilepsia ; 65(8): e148-e155, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38837761

RESUMO

In response to the evolving treatment landscape for new-onset refractory status epilepticus (NORSE) and the publication of consensus recommendations in 2022, we conducted a comparative analysis of NORSE management over time. Seventy-seven patients were enrolled by 32 centers, from July 2016 to August 2023, in the NORSE/FIRES biorepository at Yale. Immunotherapy was administered to 88% of patients after a median of 3 days, with 52% receiving second-line immunotherapy after a median of 12 days (anakinra 29%, rituximab 25%, and tocilizumab 19%). There was an increase in the use of second-line immunotherapies (odds ratio [OR] = 1.4, 95% CI = 1.1-1.8) and ketogenic diet (OR = 1.8, 95% CI = 1.3-2.6) over time. Specifically, patients from 2022 to 2023 more frequently received second-line immunotherapy (69% vs 40%; OR = 3.3; 95% CI = 1.3-8.9)-particularly anakinra (50% vs 13%; OR = 6.5; 95% CI = 2.3-21.0), and the ketogenic diet (OR = 6.8; 95% CI = 2.5-20.1)-than those before 2022. Among the 27 patients who received anakinra and/or tocilizumab, earlier administration after status epilepticus onset correlated with a shorter duration of status epilepticus (ρ = .519, p = .005). Our findings indicate an evolution in NORSE management, emphasizing the increasing use of second-line immunotherapies and the ketogenic diet. Future research will clarify the impact of these treatments and their timing on patient outcomes.


Assuntos
Dieta Cetogênica , Imunoterapia , Estado Epiléptico , Humanos , Estado Epiléptico/terapia , Estado Epiléptico/tratamento farmacológico , Masculino , Feminino , Dieta Cetogênica/métodos , Imunoterapia/métodos , Imunoterapia/tendências , Adolescente , Adulto , Epilepsia Resistente a Medicamentos/terapia , Epilepsia Resistente a Medicamentos/dietoterapia , Criança , Anticorpos Monoclonais Humanizados/uso terapêutico , Pessoa de Meia-Idade , Pré-Escolar , Anticonvulsivantes/uso terapêutico , Adulto Jovem , Rituximab/uso terapêutico , Gerenciamento Clínico
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