Journals / İstanbul Tıp Dergisi / 2012 / Cilt: 13 - Sayı: 2

A case report of a newborn with early ınfantile epileptic encephalopathy and west syndrome in follow-up

Erken infantil epileptik ensefalopati ve izleminde west sendromu gelişen bir yenidoğan olgusu

Pages
89–92
Publication Date
2022-10-18
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Intractable seizures in the neonatal period with serious clinical manifestations and characteristic EEG pattern of early infantile epileptic encephalopathy and West syndrome in follow-up association should be recognized in attention towards the possibility of this rare diagnose.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Early infantile epileptic encephalopathy (Ohtahara snydome) is a rare epileptic encephalopathy with onset of frequent seizures in the neonatal and early infancy period.
📊
Key Findings & Data:It is characterized by suppresson-burst pattern during sleeping and waking states.
💡
Conclusion & Impact:Intractable seizures in the neonatal period with serious clinical manifestations and characteristic EEG pattern of early infantile epileptic encephalopathy and West syndrome in follow-up association should be recognized in attention towards the possibility of this rare diagnose.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Early infantile epileptic encephalopathy (Ohtahara snydome) is a rare epileptic encephalopathy with onset of frequent seizures in the neonatal and early infancy period. It is characterized by suppresson-burst pattern during sleeping and waking states. This report describes a case of a newborn with early infantile epileptic ecephalopathy and West syndrome in follow-up. Intractable seizures in the neonatal period with serious clinical manifestations and characteristic EEG pattern of early infantile epileptic encephalopathy and West syndrome in follow-up association should be recognized in attention towards the possibility of this rare diagnose.

Özet

Erken infantil epileptik ensefalopati (Ohtahara sendromu), hayatın ilk aylarında ortaya çıkan ve sık tekrarlayan tonik nöbetler ile karekterize nadir bir epileptik ensefalopatidir. En önemli interiktal EEG bulgusu uyku ve uyanıklıkta ortaya çıkan börst-süpresyon paternidir. Burada yenidoğan döneminde erken infantil epileptik ensefalopati ve izleminde West sendromu tanısı alan bir olgu sunulmuştur. Yaşamın erken döneminde başlayan dirençli nöbetler, ağır klinik ve EEG bulguları ile seyreden erken infantil epileptik ensefalopati ve izleminde West sendromu birlikteliği nadir görülmesi nedeni ile klinik yaklaşımda hatırlanmalıdır.

🔖

Cite This Article

ZENCİROĞLU, A., OKUMUŞ, N., ÖZKAN, M., KARAGÖL, B. S. (2022). A case report of a newborn with early ınfantile epileptic encephalopathy and west syndrome in follow-up. İstanbul Tıp Dergisi, Cilt: 13 - Sayı: 2, 89–92. https://ajindex.com/articles/552523

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (13)

  1. Yamatogi Y, Ohtahara S. Early-infantile epileptic en- cephalopathy with suppression-bursts, Ohtahara syn- drome; its overview referring to our 16 cases. Brain Dev 2002;24:13-23
  2. Roessler E, Muenke M. Holoprosencephaly: a para- digm for the complex genetics of brain development. J Inherit Metab Dis 1998;21:481-97
  3. Raybaud C, Girard N. The developmental disorders of the commissural plate of the telencephalon: MR im- aging study and morphologic classification. Child’s Nerv Syst 1999;24:348-57
  4. Kim SK, Na DG, Byun HS, et al. Focal cortical dys- plasia: comparison of MRI and FDG-PET. J Comput Assist Tomogr 2000;24:296-302
  5. Clarke M, Gill J, Noronha M, et al. Early infantile epi- leptic encephalopathy with suppression burst: Ohtaha- ra syndrome. Dev Med Child Neurol 1987;29:520-8
  6. Ohtahara S, Ohtsuka Y, Yamatogi Y, et al. Early infan- tile epileptic encephalopathy with suppression-burst. In: Roger J, Bureau M, Dravet CH, et al., editors. Epileptic syndromes in infancy childhood and adoles- cence. London: John Libbey; 1992. p. 25-34
  7. Arzimanoglou A, Guerrini R, Aicardi J. Aicardi’s epilepsy in children. 3rd ed. Philadelphia: Lippincott Williams and Wilkins; 2004
  8. Tatsuno M, Hayashi M, Iwamoto H, et al. Autopsy case of Leigh’s encephalopathy with wide lesions in central nervous system and early infantile epileptic encephalopathy with burst suppression. [Article in Japanese] No To Hattatsu 1984;16:68-75
  9. Williams AN, Gray RG, Poulton K, et al. A case of Ohtahara syndrome with cytochrome oxidase defi- ciency. Dev Med Child Neurol 1998;40:568-70
  10. Higgins C, Gray G, Ramani P, et al. Transient cyto- chrome oxidase deficiency with Ohtahara syndrome. Dev Med Child Neurol 2000;42:785-6
  11. Ohtahara S, Yamatogi Y. Epileptic encephalopathies in early infancy with suppression-burst. J Clin Neuro- physiol 2003;20:398-407
  12. Komaki H, Sugai K, Maehara T, et al. Surgical treat- ment of early-infantile epileptic encephalopathy with suppression-bursts associated with focal cortical dys- plasia. Brain Dev 2001;23:727-31
  13. Dulac O. Epileptic encephalopathy. Epilepsia 2001;42 Suppl 3:23-6.