2017
DOI: 10.24953/turkjped.2017.04.014
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Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature

Abstract: Alparslan C, Kamit-Can F, Anıl AB, Olgaç-Dündar N, Çavuşoğlu D, Göç Z. Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature. Turk J Pediatr 2017; 59: 463-466. Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epilepsy syndrome which is characterized by acute onset of refractory status epilepticus following a febrile infection occurring in previously normal children. Despite the various treatment options that have… Show more

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Cited by 5 publications
(3 citation statements)
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“…Taking this diagnostic uncertainty into account, between 29% (38/130) and 48% (63/130) of patients with NORSE will have an identifiable etiology and between 18% (23/130) and 37% (48/130) will have an autoimmune/paraneoplastic cause 4 . Given that the majority of cases where a cause is identified consist of autoimmune/paraneoplastic etiologies, patients with cryptogenic NORSE are often treated empirically with immunotherapy, albeit with variable results 126–129 . Although the range of etiologic mechanisms at play in cryptogenic disease may explain some of the variability, there is currently no biomarker to accurately predict response to therapy.…”
Section: Inflammatory Status Epilepticusmentioning
confidence: 99%
See 1 more Smart Citation
“…Taking this diagnostic uncertainty into account, between 29% (38/130) and 48% (63/130) of patients with NORSE will have an identifiable etiology and between 18% (23/130) and 37% (48/130) will have an autoimmune/paraneoplastic cause 4 . Given that the majority of cases where a cause is identified consist of autoimmune/paraneoplastic etiologies, patients with cryptogenic NORSE are often treated empirically with immunotherapy, albeit with variable results 126–129 . Although the range of etiologic mechanisms at play in cryptogenic disease may explain some of the variability, there is currently no biomarker to accurately predict response to therapy.…”
Section: Inflammatory Status Epilepticusmentioning
confidence: 99%
“…4 Given that the majority of cases where a cause is identified consist of autoimmune/paraneoplastic etiologies, patients with cryptogenic NORSE are often treated empirically with immunotherapy, albeit with variable results. [126][127][128][129] Although | 315 the range of etiologic mechanisms at play in cryptogenic disease may explain some of the variability, there is currently no biomarker to accurately predict response to therapy. RE is associated with an aberrant immune reaction to an unknown trigger which, on histopathology, involves cytotoxic T cells, reactive microglia, and significant neuronal loss without evidence of viral infection.…”
Section: Inflammatory Status Epilepticusmentioning
confidence: 99%
“…FIRES-синдром встречается крайне редко (примерно 1 случай на 1 млн детского населения), чаще всего дебютирует в возрасте от 3 до 15 лет, второй пик заболеваемости приходится на 65 лет. Семейный анамнез пациентов по эпилепсии и фебрильным судорогам не отягощен [5,6].…”
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