Pediatric refractory and super-refractory status epilepticus

Vasquez, Alejandra; Farias‐Moeller, Raquel; Tatum, William O.

doi:10.1016/j.seizure.2018.05.012

Cited by 70 publications

(63 citation statements)

References 151 publications

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“…106 At this point, induced coma with anesthetic agents is the most common management, optionally preceded by repeated boluses of non-BZD ASDs. 107,108 While case series suggest the use of anesthetics, there is lack of randomized controlled trials evaluating their comparative effectiveness. 36 Therefore, clinical decisions among the available therapeutic options mostly rely on scarce evidence, and clinical goals during treatment are uncertain.…”

Section: Refractory Se and Continuous Infusions With Anestheticsmentioning

confidence: 99%

“…36 Therefore, clinical decisions among the available therapeutic options mostly rely on scarce evidence, and clinical goals during treatment are uncertain. 107,108 Moreover, a balance between the aim of controlling SE and the potential damage secondary to anesthetic therapies may need to be considered. Recent studies have focused on the increased risk of mortality related to continuous infusion treatments.…”

Section: Refractory Se and Continuous Infusions With Anestheticsmentioning

confidence: 99%

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Status Epilepticus—Work-Up and Management in Children

2020

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Status epilepticus (SE) is one of the most common neurological emergencies in children and has a mortality of 2 to 4%. Admissions for SE are very resource-consuming, especially in refractory and super-refractory SE. An increasing understanding of the pathophysiology of SE leaves room for improving SE treatment protocols, including medication choice and timing. Selecting the most efficacious medications and giving them in a timely manner may improve outcomes. Benzodiazepines are commonly used as first line and they can be used in the prehospital setting, where most SE episodes begin. The diagnostic work-up should start simultaneously to initial treatment, or as soon as possible, to detect potentially treatable causes of SE. Although most etiologies are recognized after the first evaluation, the detection of more unusual causes may become challenging in selected cases. SE is a life-threatening medical emergency in which prompt and efficacious treatment may improve outcomes. We provide a summary of existing evidence to guide clinical decisions regarding the work-up and treatment of SE in pediatric patients.

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Section: Refractory Se and Continuous Infusions With Anestheticsmentioning

confidence: 99%

Section: Refractory Se and Continuous Infusions With Anestheticsmentioning

confidence: 99%

Status Epilepticus—Work-Up and Management in Children

2020

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“…At this point, further trials of continuous infusion(s) and the addition of loading oral ASMs not available in IV formulations until seizure cessation or burst suppression is re-attained for an additional 24-48 h may be helpful. There is a paucity of data describing speed of titration and 'number of trials' or cycles of serial anesthetic therapy after which pharmacotherapy is considered futile for electrographic seizure control [11,51]. Midazolam, which enhances the action of GABA on the GABA A receptors, is preferred because it is fast-acting and has a short duration of action.…”

Section: Third-line Therapy Phase (40-60 Min Refractory and Super-rementioning

confidence: 99%

“…Once a steroid trial is initiated and it is ineffective within 2-3 days, IVIg or plasma exchange may be considered. If there is cessation of SE, ongoing immunotherapy may be considered depending on the clinical scenario and underlying etiology [49,51,[72][73][74][75]. While steroids and immunotherapy may be considered a last resort treatment option, we usually reserve this approach for patients with suggestions of an underlying inflammatory or autoimmune etiology.…”

Section: Immunomodulatory Therapiesmentioning

confidence: 99%

Pharmacotherapy for Pediatric Convulsive Status Epilepticus

2019

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Convulsive status epilepticus (CSE) is one of the most common pediatric neurological emergencies. Ongoing seizure activity is a dynamic process and may be associated with progressive impairment of gamma-aminobutyric acid (GABA)-mediated inhibition due to rapid internalization of GABA A receptors. Further hyperexcitability may be caused by AMPA (alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) and NMDA (N-methyl-d-aspartic acid) receptors moving from subsynaptic sites to the synaptic membrane. Receptor trafficking during prolonged seizures may contribute to difficulties treating seizures of longer duration and may provide some of the pathophysiological underpinnings of established and refractory SE (RSE). Simultaneously, a practice change toward more rapid initiation of first-line benzodiazepine (BZD) treatment and faster escalation to second-line non-BZD treatment for established SE is in progress. Early administration of the recommended BZD dose is suggested. For second-line treatment, non-BZD anti-seizure medications (ASMs) include valproate, fosphenytoin, or levetiracetam, among others, and at this point there is no clear evidence that any one of these options is better than the others. If seizures continue after second-line ASMs, RSE is manifested. RSE treatment consists of bolus doses and titration of continuous infusions under continuous electro-encephalography (EEG) guidance until electrographic seizure cessation or burst-suppression. Ultimately, etiological workup and related treatment of CSE, including broad spectrum immunotherapies as clinically indicated, is crucial. A potential therapeutic approach for future studies may entail consideration of interventions that may accelerate diagnosis and treatment of SE, as well as rational and early polytherapy based on synergism between ASMs by utilizing medications targeting different mechanisms of epileptogenesis and epileptogenicity.

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“…3,4 About 10 to 40% of children with SE will develop RSE. 5 RSE may occur in individuals with a history of known epilepsy or as a consequence of an acute brain injury due to infection, stroke, or trauma. In the majority of these cases, the cause is identified within the first 72 hours.…”

Section: Introductionmentioning

confidence: 99%

Febrile Infection-Related Epilepsy Syndrome

et al. 2019

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Febrile infection-related epilepsy syndrome (FIRES) is a subset or variant of new-onset refractory status epilepticus in children. FIRES is characterized by the occurrence of a febrile episode between 24 hours and 2 weeks before the onset of refractory status epilepticus. A infectious cause is rarely identified in FIRES and an inflammatory or autoimmune etiology is implied. Seizures in FIRES are very difficult to control, and treatments include antiepileptic drugs, ketogenic diet, intravenous immunoglobulin, plasmapheresis, and corticosteroid therapy. The prognosis for patients with FIRES is poor, and most children are left with refractory epilepsy and cognitive impairment. The new consensus guidelines on the terminology of FIRES and recent interest in new treatment approaches have been welcome developments for clinicians who face the challenge of diagnosing and managing status epilepticus in a previously healthy child that occurs following a minor febrile episode. This review aims to provide clinicians with an update on the current hypotheses for the etiology, pathogenesis, clinical evaluation, management, and future directions in the diagnosis and treatment of FIRES.

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Pediatric refractory and super-refractory status epilepticus

Abstract: RSE and SRSE are neurological emergencies with limited therapeutic options. Multi-national collaborative efforts are desirable to evaluate the safety and efficacy of current RSE/SRSE therapies, and potentially impact patients' outcomes.

Cited by 70 publications

References 151 publications

Status Epilepticus—Work-Up and Management in Children

Status Epilepticus—Work-Up and Management in Children

Pharmacotherapy for Pediatric Convulsive Status Epilepticus

Febrile Infection-Related Epilepsy Syndrome

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