SUMMARY Purpose: 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. Methods: 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. Key Findings: 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). Significance: 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.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1528-1167.2011.03250.x

Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children.

Summary
Encephalitis is generally presumed, even when seizures follow banal febrile infection, and pathogen detection in cerebrospinal fluid fails. This retrospective multicenter case series reports on 22 previously healthy children aged 3–15 years (median 6.5 years) with prolonged or recurrent seizures occurring 2–14 days (median 5 days) after fever onset (19 children with respiratory or nonspecific infections). Cerebrospinal fluid studies revealed 2–42 cells/μl (median 5 cells/μl) and no pathogens. Electroencephalography showed diffuse slowing or multifocal discharges. Neuroimaging demonstrated normal findings in 10 children. Brain biopsies were performed in seven children showing gliosis but no inflammation. Anesthetic barbiturates were used in 14 children with refractory status epilepticus, and immunotherapy in 9. Two children died, eight remained in a state of impaired consciousness, eight developed therapy-refractory epilepsies, two had behavioral disturbances, and two recovered. The lack of evidence for encephalitis suggests another infection-related pathogenesis of this disastrous epileptic encephalopathy. Therefore, we propose the term “febrile infection–related epilepsy syndrome” (FIRES).

https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1528-1167.2010.02535.x

Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood.

The causes of encephalitis are numerous, and extensive investigations for infectious agents and other etiologies are often negative The discovery that many of these encephalitis are immune mediated has changed the approach to the diagnosis and treatment of these disorders Moreover, the broad spectrum of symptoms including, psychosis, catatonia, alterations of behavior and memory, seizures, abnormal movements, and autonomic dysregulation usually requires a multidisciplinary treatment approach This review focuses in several forms of encephalitis that occur in children, and for which an autoimmune etiology has been demonstrated (eg, anti-N-methyl-d-aspartate receptor encephalitis) or is strongly suspected (eg, Rasmussen encephalitis, limbic encephalitis, opsoclonus-myoclonus) The authors also review several disorders that may be immune mediated, such as the rapid onset obesity with hypothalamic dysfunction, hypoventilation and autonomic dysregulation (ROHHAD) syndrome and some encephalopathies with fever

/pdf/autoimmune-encephalitis-in-children-9syyn1vdkk.pdf

Autoimmune Encephalitis in Children

Status epilepticus is one of the most common neurological emergencies in children and adults. Febrile status epilepticus cases are often associated with inflammatory neurological diseases caused by specific pathogens or antineuronal autoimmunity. In addition, there is a subgroup of super-refractory status epilepticus triggered by fever and having no known cause.1 This condition is designated as either acute encephalitis with refractory, repetitive partial seizures (AERRPS)2 or febrile infection-related epilepsy syndrome (FIRES).3 The pathogenesis of AERRPS/FIRES is currently unknown. A close relationship between febrile illness and status epilepticus suggests deleterious effects of inflammation and autoimmunity on the onset and progression of seizure. However, immune mechanisms in human status epilepticus associated with isolated fever have not been fully elucidated.

We report a comprehensive study of the inflammatory mediators in paediatric cases of AERRPS. We show a marked upregulation of proinflammatory cytokines and chemokines in the cerebrospinal fluid (CSF) of patients with this condition.

We defined AERRPS using the criteria shown in online supplementary table S1. Between April 2010 and July 2013, 14 patients with AERRPS and 14 patients with other inflammatory neurological diseases (OIND) were enrolled in the study. Serum and CSF specimens from patients with AERRPS and OIND were collected between 0 and 39 days from the onset of neurological symptoms. Eighteen patients with non-inflammatory neurological diseases (NIND) served as a control group. Additionally, the Shizuoka Institute of Epilepsy and Neurological Disorders provided us with 13 conserved CSF specimens from patients with AERRPS who fulfilled the same criteria, which we also included in our analysis. …

https://jnnp.bmj.com/content/jnnp/86/7/820.full.pdf

Intrathecal overproduction of proinflammatory cytokines and chemokines in febrile infection-related refractory status epilepticus

Purpose To summarize the clinical features, suggested work-up, treatment and prognosis of new-onset refractory status epilepticus (NORSE), a condition recently defined as the occurrence of refractory status epilepticus (RSE) in a patient without active epilepsy, and without a clear acute or active structural, toxic or metabolic cause; and of the related syndrome of febrile infection-related epilepsy syndrome (FIRES), also recently defined as a subgroup of NORSE preceded by a febrile illness between 2 weeks and 24 h prior to the onset of RSE. Method Narrative review of the medical literature about NORSE and FIRES. Results NORSE and FIRES mainly affect school-age children and young adults. A prodromal phase with flu-like symptoms precedes the SE onset in two third of NORSE cases, and by definition in all FIRES. Status epilepticus usually starts with repeated focal seizures with secondary bilateralization. Most cases evolve to super RSE (SRSE) and have unfavorable outcome, with short-term mortality of 12–27%, long-term disability and epilepsy. No specific imaging or laboratory abnormalities have been identified so far that allows an early diagnosis and half of adult cases remain of unknown etiology. A standardized diagnostic algorithm is provided and. Autoimmune encephalitis is the most frequent identified cause. In the absence of specific diagnosis, immunotherapy could be tried in addition to antiepileptic treatment. Conclusions This review presents the rare but devastating syndrome of NORSE, including the subcategory of FIRES. Early recognition with complete work-up is primordial to identify the underlying cause and promptly start appropriate treatment.

New onset refractory status epilepticus (NORSE).

Sakuma H, Awaya Y, Shiomi M, Yamanouchi H, Takahashi Y, Saito Y, Sugai K, Sasaki M. Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. 
Acta Neurol Scand: 2010: 121: 251–256.
© 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard.

Objective –  We conducted a nationwide multicenter study in Japan to elucidate the clinical and laboratory characteristics of acute encephalitis with refractory, repetitive partial seizures (AERRPS).

Materials and methods –  Clinical and laboratory features, treatment, and outcome were assessed using a structured questionnaire.

Results –  Twenty-nine children were enrolled in the study. Refractory and repetitive partial seizures accompanied by fever were the cardinal clinical features. Partial seizures consisted principally of eye deviation or facial twitching, being periodically repeated during the acute phase. These seizures were refractory to conventional anticonvulsants and were only suppressed by high-dose intravenous barbiturate administration. Rhythmic activities on electroencephalography and non-specific cerebral atrophy on neuroimaging were common. Serum or cerebrospinal antibodies against GluRe2 were positive in six patients. General prognosis was unfavorable due to intractable epilepsy and cognitive deficits.

Conclusion –  Based on the peculiar and homogenous features, AERRPS can be regarded as a distinct clinical entity.

Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis.

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Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis.