&lt;p&gt;Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies&lt;/p&gt;

Serino, Domenico; Santarone, Marta Elena; Caputo, Davide; Fusco, Lucia

doi:10.2147/ndt.s177803

Cited by 24 publications

(31 citation statements)

References 52 publications

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“…Alternative treatment options such as anesthetic drugs, immunotherapy, ketogenic diet, therapeutic hypothermia, and cannabidiol have shown only partial efficacy. 119 – 121 …”

Section: Febrile-infection-related Epilepsy Syndromementioning

confidence: 99%

The off-label use of anakinra in pediatric systemic autoinflammatory diseases

Maniscalco

Abu‐Rumeileh

Mastrolia

et al. 2020

Therapeutic Advances in Musculoskeletal

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Interleukin 1 (IL-1), a central mediator of innate immunity, is considered a master cytokine of local and systemic inflammation. IL-1 has emerged as pivotal in the pathogenesis of autoinflammatory diseases (AIDs), and blockade of its pathway has become a crucial target for therapy. Anakinra (ANA), a recombinant IL-1β receptor antagonist, was the first anti-IL-1 agent employed in clinical practice. ANA is currently approved for the treatment of rheumatoid arthritis, systemic juvenile idiopathic arthritis, adult-onset Still’s disease, and cryopyrin-associated autoinflammatory syndrome. It has also been successfully used for off-label treatment of various monogenic, polygenic, or undefined etiology systemic AIDs. This review describes currently available evidence for the off-label use of ANA in pediatric rheumatologic diseases. Specifically, the use of ANA in Kawasaki disease, idiopathic recurrent pericarditis, Behçet disease, monogenic AIDs, undifferentiated AIDs, chronic non-bacterial osteomyelitis, macrophage activation syndrome, and febrile infection-related epilepsy, in terms of its safety and efficacy. In selected pediatric rheumatic disorders, the off-label administration of ANA appears to be effective and safe. In order to control severe and/or relapsing disease, ANA should be considered as a valuable treatment option in children suffering from rare inflammatory diseases. However, currently available data consist of retrospective studies and short case series; thus, randomized controlled trials and larger series with long-term follow up are mandatory to better assess the efficacy and cost effectiveness of ANA in these challenging patients.

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“…Alternative treatment options such as anesthetic drugs, immunotherapy, ketogenic diet, therapeutic hypothermia, and cannabidiol have shown only partial efficacy. 119 – 121 …”

Section: Febrile-infection-related Epilepsy Syndromementioning

confidence: 99%

The off-label use of anakinra in pediatric systemic autoinflammatory diseases

Maniscalco

Abu‐Rumeileh

Mastrolia

et al. 2020

Therapeutic Advances in Musculoskeletal

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“…Ann Child Neurol 2020;28(1): [8][9][10][11][12][13][14][15] Clinical manifestations FIRES in previously healthy children generally has three phases. The first phase is the unremarkable febrile illness.…”

Section: Etiology and Pathophysiologymentioning

confidence: 99%

Febrile Infection-Related Epilepsy Syndrome: Refractory Status Epilepticus and Management Strategies

Lee

2020

Ann Child Neurol

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Febrile infection-related epilepsy syndrome (FIRES) is a rare, catastrophic epileptic syndrome that strikes previously healthy children. Its pathogenesis is unknown, it has few treatments, and it is typically refractory. In FIRES, refractory status epilepticus or a cluster of seizures starts a few days after the onset of an acute febrile illness, and it may continue as drug-resistant epilepsy, with neuropsychological impairments occurring without latency. Clinical knowledge and guidelines on FIRES are limited because it is sporadic and extremely rare. To date, the absence of specific biomarkers poses a significant diagnostic challenge; nevertheless, early diagnosis is very important for optimal management. Despite treatment with multiple immunotherapies and anti-seizure medications, the majority of patients with FIRES are left with significant cognitive disabilities and refractory epilepsy. This review aims to highlight the most recent insights into the clinical features, terminology, epidemiology, pathogenesis, diagnostic challenges, and therapeutic options associated with FIRES.

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“…In this otherwise drug refractory condition ketogenic diet (KD), therapeutic hypothermia, cannabidiol (CBD), anakinra (recombinant, modified human interleukin-1 receptor antagonist) and continuous intravenous administration of magnesium sulfate have provided therapeutic benefit. 25 New-onset refractory status epilepticus is not a specific diagnosis. It is a clinical presentation in a patient with new onset refractory status epilepticus, with no other relevant neurological disorder/active epilepsy, without any acute/active structural o toxic or metabolic cause.…”

Section: Bickersta's Brainstem Encephalitismentioning

confidence: 99%

Acute Encephalitis Syndrome: Approach to a Changing Paradigm

Banerjee¹,

Muhammed²,

Acharya³

2019

Pediatric Infectious Disease

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Purpose: Acute encephalitis syndrome (AES) poses challenges to physicians owing to acute presentation, often rapid neurologic deterioration, myriad causes including noninfective inflammatory disorders of central nervous system (CNS) and low microbiologic yield. We broadly discuss common and less common causes of AES and their clinical, laboratory including radiologic features as specific diagnosis guides management and improves outcome. Materials and methods: Literature search was performed using keywords "Paediatric acute encephalitis" in MEDLINE database from 2009 to 2019 and all relevant articles (barring case reports) in English language were reviewed. Landmark articles prior to 2009 were also reviewed. Conclusion:Acute encephalitis remains a diagnostic and therapeutic challenge in neurocritical care. The recognition of etiological agent and encephalitis mimics by investigations is important for specific therapeutic measures. Judicious use of neuroimaging, cerebrospinal fluid (CSF) analysis and appropriate lab tests helps in diagnosing specific entities especially noninfective mimics of AES which has important treatment and prognostic implication. Initial stabilization and institution of supportive measures remains key to successful management.

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<p>Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies</p>

Cited by 24 publications

References 52 publications

The off-label use of anakinra in pediatric systemic autoinflammatory diseases

The off-label use of anakinra in pediatric systemic autoinflammatory diseases

Febrile Infection-Related Epilepsy Syndrome: Refractory Status Epilepticus and Management Strategies

Acute Encephalitis Syndrome: Approach to a Changing Paradigm

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