Sevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection–related epilepsy syndrome

L’Erario, Manuela; Roperto, Rosa Maria; Rosati, Anna

doi:10.1002/epi4.12545

Cited by 10 publications

(13 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…All patients had chronic epilepsy at a median follow-up of about a year. Other case reports showed similar results [9] , [10] , [11] , [12] . Anakinra was suggested to be effective in 5/6 patients with chronic drug resistant epilepsies of unknown etiology [13] .…”

Section: Discussionsupporting

confidence: 71%

Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulation

Perulli

Cicala

Turrini

et al. 2022

Epilepsy & Behavior Reports

View full text Add to dashboard Cite

Section: Discussionsupporting

confidence: 71%

Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulation

Perulli

Cicala

Turrini

et al. 2022

Epilepsy & Behavior Reports

View full text Add to dashboard Cite

“…This hypothesis was corroborated by 8 (out of 11) subsequent case reports 37–42,45,46 . Anakinra was initiated at a median of 30 days (range = 12 days–12 weeks) after the onset of seizures.…”

Section: Acute Phasementioning

confidence: 62%

“…35 Since then, several case reports or case series and one larger international retrospective study cohort have been published, consisting of a total of 46 patients treated during the acute phase of NORSE (range = .6-38 years, median age = 8; Table 1). 5,15,[35][36][37][38][39][40][41][42][43][44][45][46] Almost all patients (42/46, 91%) were children, and 98% (45/46) of them qualified as FIRES. Most cases were cryptogenic (44/46, 96%), with one case reported as infectious (Bartonella henselae), and another genetic.…”

Section: Anakinramentioning

confidence: 99%

See 1 more Smart Citation

Second‐line immunotherapy in new onset refractory status epilepticus

Hanin,

Muscal,

Hirsch

2024

Epilepsia

View full text Add to dashboard Cite

Several pieces of evidence suggest immune dysregulation could trigger the onset and modulate sequelae of new onset refractory status epilepticus (NORSE), including its subtype with prior fever known as febrile infection‐related epilepsy syndrome (FIRES). Consensus‐driven recommendations have been established to guide the initiation of first‐ and second‐line immunotherapies in these patients. Here, we review the literature to date on second‐line immunotherapy for NORSE/FIRES, presenting results from 28 case reports and series describing the use of anakinra, tocilizumab, or intrathecal dexamethasone in 75 patients with NORSE. Among them, 52 patients were managed with anakinra, 21 with tocilizumab, and eight with intrathecal dexamethasone. Most had elevated serum or cerebrospinal fluid cytokine levels at treatment initiation. Treatments were predominantly initiated during the acute phase of the disease (92%) and resulted, within the first 2 weeks, in seizure control for up to 73% of patients with anakinra, 70% with tocilizumab, and 50% with intrathecal dexamethasone. Cytokine levels decreased after treatment for most patients. Anakinra and intrathecal dexamethasone were mainly initiated in children with FIRES, whereas tocilizumab was more frequently prescribed for adults, with or without a prior febrile infection. There was no clear correlation between the response to treatment and the time to initiate the treatment. Most patients experienced long‐term disability and drug‐resistant post‐NORSE epilepsy. Initiation of second‐line immunotherapies during status epilepticus (SE) had no clear effect on the emergence of post‐NORSE epilepsy or long‐term functional outcomes. In a small number of cases, the initiation of anakinra or tocilizumab several years after SE onset resulted in a reduction of seizure frequency for 67% of patients. These data highlight the potential utility of anakinra, tocilizumab, and intrathecal dexamethasone in patients with NORSE. There continues to be interest in the utilization of early cytokine measurements to guide treatment selection and response. Prospective studies are necessary to understand the role of early immunomodulation and its associations with epilepsy and functional outcomes.

show abstract

“…Phenobarbital can increase the Ih current in hippocampal pyramidal neurons (Jung et al, 2007 ). Anakinra (human interleukin-1 receptor antagonist), a drug with anti-inflammatory activity, has been reported to be effective in the acute phase of FIRES (Kenney-Jung et al, 2016 ; Dilena et al, 2019 ; Lai et al, 2020 ; L'Erario et al, 2021 ). The cerebrospinal fluid of FIRES cases demonstrated high levels of proinflammatory cytokines before treatment, but the levels were normalized after anakinra therapy (Kenney-Jung et al, 2016 ).…”

Section: Resultsmentioning

confidence: 99%

The Contribution of HCN Channelopathies in Different Epileptic Syndromes, Mechanisms, Modulators, and Potential Treatment Targets: A Systematic Review

Kessi

Peng

Duan

et al. 2022

Front. Mol. Neurosci.

View full text Add to dashboard Cite

BackgroundHyperpolarization-activated cyclic nucleotide-gated (HCN) current reduces dendritic summation, suppresses dendritic calcium spikes, and enables inhibitory GABA-mediated postsynaptic potentials, thereby suppressing epilepsy. However, it is unclear whether increased HCN current can produce epilepsy. We hypothesized that gain-of-function (GOF) and loss-of-function (LOF) variants of HCN channel genes may cause epilepsy.ObjectivesThis systematic review aims to summarize the role of HCN channelopathies in epilepsy, update genetic findings in patients, create genotype–phenotype correlations, and discuss animal models, GOF and LOF mechanisms, and potential treatment targets.MethodsThe review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement, for all years until August 2021.ResultsWe identified pathogenic variants of HCN1 (n = 24), HCN2 (n = 8), HCN3 (n = 2), and HCN4 (n = 6) that were associated with epilepsy in 74 cases (43 HCN1, 20 HCN2, 2 HCN3, and 9 HCN4). Epilepsy was associated with GOF and LOF variants, and the mechanisms were indeterminate. Less than half of the cases became seizure-free and some developed drug-resistant epilepsy. Of the 74 cases, 12 (16.2%) died, comprising HCN1 (n = 4), HCN2 (n = 2), HCN3 (n = 2), and HCN4 (n = 4). Of the deceased cases, 10 (83%) had a sudden unexpected death in epilepsy (SUDEP) and 2 (16.7%) due to cardiopulmonary failure. SUDEP affected more adults (n = 10) than children (n = 2). HCN1 variants p.M234R, p.C329S, p.V414M, p.M153I, and p.M305L, as well as HCN2 variants p.S632W and delPPP (p.719–721), were associated with different phenotypes. HCN1 p.L157V and HCN4 p.R550C were associated with genetic generalized epilepsy. There are several HCN animal models, pharmacological targets, and modulators, but precise drugs have not been developed. Currently, there are no HCN channel openers.ConclusionWe recommend clinicians to include HCN genes in epilepsy gene panels. Researchers should explore the possible underlying mechanisms for GOF and LOF variants by identifying the specific neuronal subtypes and neuroanatomical locations of each identified pathogenic variant. Researchers should identify specific HCN channel openers and blockers with high binding affinity. Such information will give clarity to the involvement of HCN channelopathies in epilepsy and provide the opportunity to develop targeted treatments.

show abstract

Sevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection–related epilepsy syndrome

Abstract: This is an open access article under the terms of the Creat ive Commo ns Attri bution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

Cited by 10 publications

References 20 publications

Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulation

Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulation

Second‐line immunotherapy in new onset refractory status epilepticus

The Contribution of HCN Channelopathies in Different Epileptic Syndromes, Mechanisms, Modulators, and Potential Treatment Targets: A Systematic Review

Contact Info

Product

Resources

About