Ohtahara Sendromu
Özet
Ohtahara sendromu, yenidoğan ve erken sütçocukluğu döneminde görülen, kötü prognozlu, dirençli nöbetler ve gelişimsel gerilikle karakterize nadir bir epileptik ensefalopatidir. Hastalığın temelinde sıklıkla yapısal beyin malformasyonları, metabolik bozukluklar veya genetik mutasyonlar yer almaktadır. Karakteristik EEG bulgusu "burst-supresyon" paterni olup, tedavi başarısı büyük oranda altta yatan etiyolojinin hızlı belirlenmesine bağlıdır. Erken tanı ve agresif yaklaşım, nörogelişimsel prognozun iyileştirilmesi açısından kritik öneme sahiptir.
Ohtahara syndrome is a rare, poor-prognosis epileptic encephalopathy characterized by intractable seizures and developmental delay in neonates and early infancy. The condition is often caused by underlying structural brain malformations, metabolic disorders, or genetic mutations. Its characteristic EEG finding is a "burst-suppression" pattern, and treatment success heavily depends on the rapid identification of the underlying etiology. Early diagnosis and an aggressive, targeted approach are crucial for improving neurodevelopmental outcomes.
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