Ateşli Enfeksiyonla İlişkili Epilepsi Sendromu (Febrile Infection-Related Epilepsy Syndrome – FIRES)
Özet
Ateşli Enfeksiyonla İlişkili Epilepsi Sendromu (FİRES), özgül bir neden olmaksızın ateşli bir hastalık sonrası gelişen, nadir ve yüksek mortaliteye sahip bir epileptik ensefalopatidir. Patofizyolojisinde nöroenflamatuar süreçlerin anahtar rol oynadığı düşünülmekte olup, tanı süreci enfektif ve metabolik diğer nedenlerin dışlanmasına dayanır. Özgül bir tedavisi bulunmamakla birlikte, ketojenik diyet, anestetikler ve çeşitli immünomodülatör ajanlar destekleyici olarak kullanılmaktadır. Hastalık genellikle kronik, dirençli epilepsi ve nörolojik sekellerle sonuçlanan kötü bir prognoza sahiptir.
Febrile Infection-Related Epilepsy Syndrome (FIRES) is a rare, highly mortal epileptic encephalopathy occurring after a non-specific febrile illness without a clear underlying cause. Neuroinflammation driven by excessive pro-inflammatory cytokines is considered the primary pathogenic mechanism, and diagnosis relies primarily on the exclusion of other metabolic or infectious factors. While there is no specific cure, clinical management involves the use of the ketogenic diet, anesthetics, and various immunomodulatory agents. The prognosis is generally poor, often resulting in chronic drug-resistant epilepsy and lasting neurological sequelae.
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