Prolonged Refractory Status Epilepticus Research Articles

Cannabidiol in the acute phase of febrile infection-related epilepsy syndrome (FIRES).

Febrile infection-related epilepsy syndrome (FIRES) is a prolonged refractory status epilepticus (SE) that develops among healthy individuals after a febrile infection. FIRES treatment is challenging due to its poor response to antiseizure medications (ASMs) and anesthetic drugs. The use of cannabidiol (CBD) as an adjunctive treatment has been suggested, albeit data about its role in the acute phase is lacking. This report describes the use of purified CBD in the acute phase of two pediatric cases of FIRES and their long-term outcome. Both children were treated with several ASMs, immunomodulators, anesthetics, and nonpharmacological treatment (ketogenic diet). CBD was administered, as an adjunctive treatment, through nasogastric tube about 30 days after onset. SE resolved within 3 days of reaching the target dose and both were seizure-free for 1 year after. Although it is difficult to define the extent to which each previous therapy contributed to recovery, in both cases CBD therapy was a turning point, reinforcing its potential role as add-on treatment in the acute phase of FIRES.

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

AbstractMultisystem 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.

A case of very prolonged new onset refractory status epilepticus (NORSE) with no evidence of autoimmune activation and a good neurological recovery

A case of very prolonged new onset refractory status epilepticus (NORSE) with no evidence of autoimmune activation and a good neurological recovery

Consensus definition and development of new-onset refractory status epilepticus and the related syndromes

New-onset refractory status epilepticus(NORSE) is a clinical syndrome, not a specific diagnosis of diseases in patients with new onset of refractive status epilepticus without active epilepsy or other preexisting relevant neurological disorders and is not associated with a clearly acute or active structrue, toxic or metabolic cause.Febrile infection-related epilepsy syndrome(FIRES) is a subcategory of NORSE, applicable for all ages, occurring in a prior febrile infection starting between 24 hours and 2 weeks before onset of refractory status epilepticus(RSE) with fever or not.Proposed consensus definitions are also applicable for infantile hemiconvulsion-hemiplegia and epilepsy syndrome, RSE, super-RSE, prolonged RSE and prolonged super-RSE.These consensus definitions will promote much more effective communication and multicenter research, and ultimately improve recognition and treatment of these conditions. Key words: New-onset refractory status epilepticus; Febrile infection-related epilepsy syndrome; Infantile hemiconvulsion-hemiplegia and epilepsy syndrome; Super-refractory status epilepticus; Consensus definition; Clinical characteristics; Autoimmune encephalitis

FEBRILE INFECTION-RELATED EPILEPSY SYNDROME (FIRES): COMPLICATED DIAGNOSIS AND CASE REPORT

Febrile infection-related epilepsy syndrome (FIRES) is an acute disease provoked by the nonspecific febrile illness with the development of refractory status epilepticus. It frequently occurs in previously healthy children. The pathogenesis of this syndrome is still unknown. The leading initial symptoms include acute fever, seizures, consciousness disorders and imitate neuroinfection or autoimmune pathology of CNS. These features make diagnosis extremely difficult, and there are in addition some difficulties with the verification of the diagnosis. Interpretation of MRI results allows identifying involved parts of the brain. However, the EEG record pattern is not precise. We report a clinical case of FIRES occurred in a 14-year-old previously healthy boy. The disease began similarly to an acute neuroinfection disease with prolonged refractory status epilepticus

FEBRILE INFECTION-RELATED EPILEPSY SYNDROME (FIRES): COMPLICATED DIAGNOSIS AND CASE REPORT

Febrile infection-related epilepsy syndrome (FIRES) is an acute disease provoked by the nonspecific febrile illness with the development of refractory status epilepticus. It frequently occurs in previously healthy children. The pathogenesis of this syndrome is still unknown. The leading initial symptoms include acute fever, seizures, consciousness disorders and imitate neuroinfection or autoimmune pathology of CNS. These features make diagnosis extremely difficult, and there are in addition some difficulties with the verification of the diagnosis. Interpretation of MRI results allows identifying involved parts of the brain. However, the EEG record pattern is not precise. We report a clinical case of FIRES occurred in a 14-year-old previously healthy boy. The disease began similarly to an acute neuroinfection disease with prolonged refractory status epilepticus

Febrile Infection-Related Epilepsy Syndrome (FIRES): A Literature Review and Case Study

ABSTRACTFebrile infection-related epilepsy syndrome (FIRES) is a catastrophic epileptic syndrome that strikes previously healthy children aged 3–15 years and has an unknown pathogenesis and few treatments. These children experience a nonspecific febrile illness that is followed by prolonged refractory status epilepticus. Although the etiology is unknown, FIRES has a biphasic presentation, with the acute phase beginning as seizure activity lasting 1–12 weeks, then followed by the chronic phase, which is characterized by refractory seizures that cluster every 2–4 weeks, and may continue to be multifocal and independent. Treatment of FIRES is difficult, typically unresponsive to antiepileptic drugs. Some children resolve temporarily with drug-induced burst suppression comas. Other therapies such as a ketogenic diet have limited benefit. The outcome varies with the length of the acute phase and is usually poor, with up to 30% of cases ending in death and 66–100% of survivors having intellectual disability. The authors present a case of a 6-year-old child presenting with FIRES and refractory status epilepticus, which continued despite multidrug therapy. The patient underwent immunomodulatory therapy with the eventual resolution of status, but she developed a chronic, moderately severe encephalopathy, including intractable epilepsy. This case highlights the challenges of FIRES and the potential of immunomodulatory therapies for children with this disorder.

Acute Resective Surgery for the Treatment of Refractory Status Epilepticus.

To identify the role of acute surgical intervention in the treatment of refractory status epilepticus (RSE). Retrospective review of consecutive patients who underwent epilepsy surgery from 2006 to 2015 was done to identify cases where acute surgical intervention was employed for the treatment of RSE. In addition, the adult and pediatric RSE literature was reviewed for reports of surgical treatment of RSE. Nine patients, aged 20-68years, with various etiologies were identified to have undergone acute surgical resection for the treatment of RSE, aided by electrocorticography. Patients required aggressive medical therapy with antiepileptic drugs and intravenous anesthetic drugs for 10-54days and underwent extensive neurodiagnostic testing prior to resective surgery. Eight out of nine patients survived and five patients were seizure-free at the last follow-up. The literature revealed 13 adult and 48 pediatric cases where adequate historical detail was available for review and comparison. We present the largest cohort of consecutive adult patients who underwent resective surgery in the setting of RSE. We also reveal that surgery can be efficacious in aborting status and in some can lead to long-term seizure freedom. Acute surgical intervention is a viable option in prolonged RSE and proper evaluation for such intervention should be conducted, although the timing and type of surgical intervention remain poorly defined.

Is Favorable Outcome Possible After Prolonged Refractory Status Epilepticus?

When status epilepticus (SE) remains refractory to appropriate therapy, it is associated with high mortality and with substantial morbidity in survivors. Many outcome predictors such as age, seizure type, level of consciousness before treatment, and mostly, etiology, are well-established. A longer duration of SE is often associated with worse outcome, but duration may lose its prognostic value after several hours. Several terms and definitions have been used to describe prolonged, refractory SE, including "malignant SE," "prolonged" SE, and more recently, "super refractory" SE, defined as "SE that has continued or recurred despite 24 hours of general anesthesia (or coma-inducing anticonvulsants)." There are few data available regarding the outcome of prolonged refractory SE, and even fewer for SE remaining refractory to anesthetic drugs. This article reviews reports of outcome after prolonged, refractory, and "super refractory" SE. Most information detailing the clinical outcome of patients surviving these severe illnesses, in which seizures can persist for days or weeks (and especially those concerning "super-refractory" SE) come from case reports and retrospective cohort studies. In many series, prolonged, refractory SE has a mortality of 30% to 50%, and several studies indicate that most survivors have a substantial decline in functional status. Nevertheless, several reports demonstrate that good functional outcome is possible even after several days of SE and coma induction. Treatment of refractory SE should not be withdrawn from younger patients without structural brain damage at presentation solely because of the duration of SE.

Functional outcome of prolonged refractory status epilepticus.

IntroductionTo characterize etiology, clinical course and outcomes of patients in prolonged refractory status epilepticus (PRSE) and looking for prognostic factors.MethodsRetrospective study conducted in patients hospitalized from January 1, 2001 to December 31, 2011 in 19 polyvalent intensive care units in French university and general hospitals. Patients were adults with a generalized convulsive refractory status epilepticus that lasted more than seven days, despite treatment including an anesthetic drug and mechanical ventilation. Patients with anoxic encephalopathy were excluded. Follow-up phone call was used to determine functional outcome using modified Rankin Scale (mRS) with mRS 0–3 defining good and mRS 4–6 poor outcome.Results78 patients (35 female) were included. Median age was 57 years. Causes of status epilepticus were various, mainly including prior epilepsy (14.1%), CNS infection (12.8%), and stroke (12.8%). No etiology was found in 27 (34.6%) patients. PRSE was considered controlled in only 53 (67.9%) patients after a median duration of 17 (IQR 12–26) days. The median length of ICU stay was 28 (19–48) days. Forty-one (52.5%) patients died in the ICU, 26 from multiple organ failure, 8 from care withdrawal, 2 from sudden cardiac arrest, 1 from brain death and 4 from unknown causes. PRSE was previously resolved in 20 patients who died in the ICU. At one-year follow-up, there were 12 patients with good outcome and 58 with poor outcome and 8 lost of follow-up. On multivariate analysis, only vasopressor use was a predictor of poor outcome (OR 6.54; 95%CI 1.09-39.29; p = 0.04).ConclusionPoor outcome was observed in about 80% of this population of PRSE. Most patients died from systemic complications linked to their ICU stay. Some patients can recover satisfactorily over time though we did not identify any robust factor of good outcome.

Aetiology and outcome of generalized convulsive status epilepticus in elderly

PurposeConvulsive status epilepticus (CSE) is a common neurologic emergency in elderly people. The current study elaborates the clinical characteristics and outcome of CSE in elderly patients. MethodsAnalysis of data of generalized CSE patients, aged 60 years and above admitted at the neurointensive care unit (NICU) was performed. The primary outcome for analysis was in-hospital mortality. The study population was divided into groups based on progression of CSE and mortality to analyze difference in study variables. Mortality of the group was analyzed using life tables. ResultsA total of 33 patients satisfied the inclusion criteria from medical records of 212 patients with CSE. Mean age of the study population was 67.0±6.8 years; 69.7% were men. Acute symptomatic aetiology was the commonest cause of CSE (60.6%); nine (27.3%) patients progressed to refractory status epilepticus (RSE) of which five patients had prolonged RSE. The overall mortality was 18.2%. Complications of mechanical ventilation and mean age were higher in patients who died. Though vascular aetiology was the leading cause of CSE (39.3%), it was not associated with progression to RSE or mortality. Acute symptomatic aetiology accounted for five out of the six deaths in the entire cohort. ConclusionLess than one-third of elderly patients with CSE progressed to RSE. Vascular aetiology, the leading cause of generalized CSE in elderly, was not associated with progression to RSE and mortality. Acute symptomatic aetiology was associated with high mortality.

Centromedian nuclei of thalamus contributes to working memory performance: evidence from electric stimulation of CMN in a human subject treated with DBS for refractory epilepsy

Event Abstract Back to Event Centromedian nuclei of thalamus contributes to working memory performance: evidence from electric stimulation of CMN in a human subject treated with DBS for refractory epilepsy Jari Peräkylä1, Lihua Sun1, Markus Polvivaara1, Juha Öhman2, Jukka Peltola2, Kai Lehtimäki2 and Kaisa Hartikainen1* 1 Tampere University Hospital, Behavioral Neurology Research Unit, Finland 2 Tampere University Hospital, Department of Neurosciences Neurology and Rehabilitation, Finland Background: Patients treated with deep brain stimulation (DBS) provide an opportunity to study cognitive and affective processes in humans with direct stimulation of key nodes in the associative and limbic systems such as thalamic nuclei. Centromedian nucleus of thalamus (CMN) is one of the targets used for refractory epilepsy. There is some imaging and animal literature implicating CMN in attention and working memory. However, there is no direct evidence from human subjects with electrical stimulation of the CMN supporting this. Methods: We studied a patient treated successfully with CMN-DBS due to prolonged refractory status epilepticus. The patients performed a computer based Executive-reaction time (RT) test, i.e. a Go-NoGo visual discrimination task with threat-related emotional distractors and rule switching, while the DBS was switched ON and OFF every few minutes. The subject had to respond to the orientation of a previously presented triangle after a Go-signal appeared with a permission to respond. Repeated Measures Analysis of Variance was carried out for reaction times (RTs) and for different error types with Stimulation (On, Off) and Emotion (neutral, threat) as factors. There were three different error types, i.e. commission errors, incorrect button presses and missed responses. Results: There was a main effect of Stimulation for incorrect errors (p < 0.05), with stimulation increasing the number of incorrect button presses reflecting lapses in working memory. There were no Main effects for RTs, commission errors or misses. Conclusion: We found behavioral evidence from direct electrical stimulation of CMN in a human subject for CMN's role in working memory performance. Stimulation increased the number of incorrect errors indicating lapses in working memory performance due to stimulation. The results are in line with previous imaging research on the role of CMN in attention and working memory. Keywords: Attention, Deep Brain Stimulation, executive functions, working memory, DBS Conference: XII International Conference on Cognitive Neuroscience (ICON-XII), Brisbane, Queensland, Australia, 27 Jul - 31 Jul, 2014. Presentation Type: Poster Topic: Cognition and Executive Processes Citation: Peräkylä J, Sun L, Polvivaara M, Öhman J, Peltola J, Lehtimäki K and Hartikainen K (2015). Centromedian nuclei of thalamus contributes to working memory performance: evidence from electric stimulation of CMN in a human subject treated with DBS for refractory epilepsy. Conference Abstract: XII International Conference on Cognitive Neuroscience (ICON-XII). doi: 10.3389/conf.fnhum.2015.217.00240 Copyright: The abstracts in this collection have not been subject to any Frontiers peer review or checks, and are not endorsed by Frontiers. They are made available through the Frontiers publishing platform as a service to conference organizers and presenters. The copyright in the individual abstracts is owned by the author of each abstract or his/her employer unless otherwise stated. Each abstract, as well as the collection of abstracts, are published under a Creative Commons CC-BY 4.0 (attribution) licence (https://creativecommons.org/licenses/by/4.0/) and may thus be reproduced, translated, adapted and be the subject of derivative works provided the authors and Frontiers are attributed. For Frontiers’ terms and conditions please see https://www.frontiersin.org/legal/terms-and-conditions. Received: 19 Feb 2015; Published Online: 24 Apr 2015. * Correspondence: Dr. Kaisa Hartikainen, Tampere University Hospital, Behavioral Neurology Research Unit, Tampere, Finland, kaisa.hartikainen@live.com Login Required This action requires you to be registered with Frontiers and logged in. To register or login click here. Abstract Info Abstract The Authors in Frontiers Jari Peräkylä Lihua Sun Markus Polvivaara Juha Öhman Jukka Peltola Kai Lehtimäki Kaisa Hartikainen Google Jari Peräkylä Lihua Sun Markus Polvivaara Juha Öhman Jukka Peltola Kai Lehtimäki Kaisa Hartikainen Google Scholar Jari Peräkylä Lihua Sun Markus Polvivaara Juha Öhman Jukka Peltola Kai Lehtimäki Kaisa Hartikainen PubMed Jari Peräkylä Lihua Sun Markus Polvivaara Juha Öhman Jukka Peltola Kai Lehtimäki Kaisa Hartikainen Related Article in Frontiers Google Scholar PubMed Abstract Close Back to top Javascript is disabled. Please enable Javascript in your browser settings in order to see all the content on this page.

Ketamine Continuous Infusion for Refractory Status Epilepticus in a Patient With Anticonvulsant Hypersensitivity Syndrome

Refractory status epilepticus (RSE) requires aggressive management with multiple antiepileptic drugs (AEDs) often requiring the initiation of continuous infusions of propofol, midazolam, or pentobarbital to achieve adequate control in addition to intermittent agents. Ketamine has been implicated in several case reports as a successful agent for treating RSE given that it blocks the N-methyl-D-aspartate receptor, which is overexpressed in prolonged status epilepticus. We describe a previously healthy 27-year-old woman who presented with prolonged RSE requiring the initiation of multiple AEDs, including high-dose propofol and midazolam continuous infusions. As a result of hypotension from propofol and inadequate seizure control with midazolam, the patient was successfully transitioned to a pentobarbital infusion in combination with multiple AEDs. Although the patient achieved control of her RSE, her course was complicated by the development of an anticonvulsant hypersensitivity syndrome (AHS) with transaminitis. Limited with the options of AED that could have been used, it was decided to initiate the patient on a continuous ketamine infusion plus midazolam and slowly wean the patient off pentobarbital as well as to avoid further use of phenytoin and phenobarbital. The patient was successfully transitioned off pentobarbital to a ketamine infusion plus midazolam with complete seizure control after several dose escalations. Her AHS and transaminitis resolved on a ketamine infusion for a total of 12 days, and she was successfully discharged from the hospital after 60 days in the ICU. This is the first case report to describe a successful transition to a ketamine infusion in a patient with AHS and transaminitis.

Pathology of bilateral pulvinar degeneration following long duration status epilepticus

PurposeTo define the neuropathological findings of pulvinar degeneration seen in long duration status epilepticus. MethodsWe review the clinical, radiologic, neurophysiologic, investigational and neuropathological findings on a 27 year old woman who died after 162 days of prolonged refractory status epilepticus. ResultsContinuous EEG monitoring confirmed recurrent uncontrolled seizure activity bilaterally and independently, most frequent in the right fronto-temporal region. Initial MRI of the brain was normal. Repeat study until on day 127 of admission showed advanced changes, with bilateral pulvinar T2/FLAIR hyperintensities. The autopsy revealed sharply defined, grey, soft, granular nodules in each medial pulvinar nucleus. Microscopically these consisted of sharply defined paucicellular areas with loss of neurons and myelin and with numerous macrophages in their centers, surrounded by reactive astrocytes with relatively spared of axons. The spinal cord at cervical and thoracic levels showed symmetric spongy vacuolation in the central part of the dorsal columns and lateral corticospinal tracts, with mild myelin loss, relatively preserved axons. The pathological lesions found in this case in thepulvinar are somewhat similar to the pathologic lesions described in Wernicke's encephalopathy. Those found in the spinal cord of our patient resemble characteristic features of B12 related subacute combined degeneration. ConclusionCharacteristic pulvinar degeneration may be found as an acquired phenomenon in prolonged refractory status epilepticus. We hypothesize that the neuropathological findings result from an excessive focal metabolic demand, secondary to neuronal network over activation in the setting of prolonged, frequent bi-temporal seizures.

Histopathological evidence that hippocampal atrophy following status epilepticus is a result of neuronal necrosis

Medial temporal lobe epilepsy is commonly associated with hippocampal atrophy on MRI and hippocampal sclerosis on histopathological examination of surgically-resected specimens. Likewise, it is well-established that prolonged seizures and status epilepticus can lead to hippocampal edema as noted on MRI.In this paper, the authors present an unusual patient with prolonged refractory status epilepticus, due to limbic encephalitis associated with anti-GAD antibody, who underwent palliative epilepsy surgery. Bilateral hippocampal edema was noted on preoperative MRI. Histologic evaluation confirmed presence of acute necrosis and neuronal loss in the left hippocampal formation. Follow-up MRI several months after surgery demonstrated severe atrophy of the contralateral right hippocampus.This is the first clear histopathological evidence that hippocampal atrophy following status epilepticus is the result of acute neuronal necrosis and cell loss.

Clinical Outcomes Following Prolonged Refractory Status Epilepticus (PRSE)

To define the clinical profile and outcome of patients in prolonged refractory status epilepticus (PRSE), and investigate possible predictors of outcome. We reviewed 63 consecutive patients with PRSE cared for in the medical and neurointensive care units of three academic medical centers over a 9-year period. For this multi-center retrospective cohort study, PRSE was defined as SE that persisted despite at least 1 week of induced coma. Variables examined for their relationship to outcome included etiology, EEG, neuroimaging, and age. Forty-two (66%) of 63 patients in PRSE survived to discharge from hospitalization. Fourteen (22%) patients had a good outcome (mRS ≤ 3) at last available follow up (at least 6 months post-PRSE). Of these, 6 (10%) individuals had no significant disability and were able to carry out all usual activities (mRS = 1). Normal neuroimaging and a reactive EEG at onset of PRSE were associated with good outcome. Good or excellent clinical outcomes were possible in patients in PRSE for up to 79 days, and in patients up to 69 years old. Good outcome is not unusual in PRSE, including in some older patients, in a variety of diagnoses, and despite months of coma.

Efficacy and Safety of Intravenous Sodium Valproate in Convulsive Status Epilepticus in Children in Shahid Sadoughi Hospital

Efficacy and Safety of Intravenous Sodium Valproate in Convulsive Status Epilepticus in Children in Shahid Sadoughi Hospital

Corpus callosotomy for treatment of pediatric refractory status epilepticus

Medically refractory status epilepticus (RSE) causes high morbidity and mortality in children. There are no evidence-based guidelines for treatment. Epilepsy surgery is a treatment option for RSE. We describe a 9-year-old boy treated successfully for RSE with complete corpus callosotomy (CC). Epilepsy surgery should be considered for prolonged RSE. In the absence of evidence of focal epileptogenesis, complete corpus callosotomy may be effective in select cases.

Febrile infection–related epilepsy syndrome (FIRES): Does duration of anesthesia affect outcome?

We conducted a retrospective multicenter study on children who had been included in eight studies published between November 2001 and July 2010 to explore the correlations between burst-suppression coma (BSC) with outcome in febrile infection-related epilepsy syndrome (FIRES). The 77 enrolled patients presented with prolonged refractory status epilepticus. BSC was induced in 46 patients. Cognitive levels at follow-up were significantly associated with duration of a BSC (p=0.005). The outcome of FIRES is poor. Treatment by inducing a prolonged BSC was associated with a worse cognitive outcome.

Febrile infection-related epilepsy syndrome (FIRES): Pathogenesis, treatment, and outcome

To explore the correlations between treatment modalities and selected disease parameters with outcome in febrile infection-related epilepsy syndrome (FIRES), a catastrophic epileptic encephalopathy with a yet undefined etiology. We conducted a retrospective multicenter study on children who had been included in eight studies published between November 2001 and July 2010. Additional data were retrieved from six of the eight participating centers. The 77 enrolled patients presented with prolonged refractory status epilepticus. A preceding febrile infection had been reported in 96% of them. Treatment modalities included antiepileptic drugs (a median of six), intravenous immunoglobulin (IVIG, 30 patients), steroids (29 patients), burst-suppression coma (BSC, 46 patients), and other less conventional agents. There was no evidence of efficacy for those treatment modalities except for IVIG (two patients), a ketogenic diet (one patient), and a prolonged cycle of barbiturate anesthesia coma (one patient). Nine patients (11.7%) died during the acute phase of FIRES. Only 12 of the 68 surviving patients (18%) retained normal cognitive level, but most of them had learning disabilities. Sixty-three patients (93%) had refractory epilepsy at follow-up. Cognitive levels at follow-up were significantly associated with duration of BSC (p = 0.005) and younger age at FIRES onset (p = 0.02). The outcome of FIRES is poor. No therapeutic agent was efficacious in shortening the acute phase, with the possible exception of a ketogenic diet. Treatment by inducing a prolonged BSC was associated with a worse cognitive outcome.