Hemikonvülziyon Hemipleji Epilepsi Sendromu
Özet
Hemikonvülziyon hemipleji epilepsi (HHE) sendromu, genellikle 4 yaş altı çocuklarda ateşli hastalık döneminde görülen, fokal motor nöbetler ve bunları izleyen hemipleji ile karakterize nadir bir tablodur. Akut dönemde tek hemisferde sitotoksik ödem ve nöbet aktivitesi izlenirken, uzun dönemde bu hemisferde atrofi ve sıklıkla dirençli epilepsi gelişimi görülür. Patofizyolojisi tam olarak aydınlatılamamış olsa da inflamasyonun nöbetlerin tetiklenmesinde ve nöronal hasarın oluşumunda kritik bir rol oynadığı düşünülmektedir. Günümüzde tedavi daha çok destekleyici olup, epilepsinin dirençli olduğu vakalarda cerrahi seçenekler değerlendirilebilir.
Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a rare condition primarily affecting children under 4 years of age, characterized by focal motor seizures during febrile illnesses followed by hemiplegia. While the acute phase manifests as cytotoxic edema and seizure activity in one hemisphere, the long-term progression often involves cerebral atrophy and the development of refractory epilepsy. Although its exact pathophysiology remains unclear, inflammation is believed to play a critical role in triggering seizures and causing neuronal damage. Current management is primarily supportive, with surgical interventions considered for cases involving refractory epilepsy.
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