Yer Değiştiren Foküslü İnfantil Parsiyel Nöbetler (İnfantil Malign Migratuar Parsiyel Epilepsi)

Özet

İnfantil malign migratuar parsiyel nöbet sendromu (MMPSI), yaşamın ilk 6 ayında başlayan, her iki hemisferde bağımsız gelişen refrakter fokal nöbetler ve ağır psikomotor gerilik ile karakterize, nadir ve kötü prognozlu bir infantil epileptik ensefalopatidir. Etiyolojisi tam olarak bilinmemekle birlikte genetik faktörlerin rol oynadığı düşünülmektedir ve tanı uzun süreli video-EEG monitörizasyonu ile konulur. Mevcut antiepileptik tedavilere dirençli seyreden bu sendrom, ciddi mikrosefali, gelişimsel duraklama ve yüksek mortalite riski ile ilişkilidir.

 

Malignant migrating partial seizures in infancy (MMPSI) is a rare and severe infantile epileptic encephalopathy characterized by intractable focal seizures emerging independently in both hemispheres within the first six months of life, accompanied by profound psychomotor retardation. While the exact etiology remains unknown, genetic factors are suspected, and diagnosis relies heavily on long-term video-EEG monitoring. The condition is notoriously resistant to conventional antiepileptic therapies and typically leads to significant developmental arrest, acquired microcephaly, and a high risk of early mortality.

Referanslar

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Gelecek

25 Ocak 2023

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