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2008, vol. 61, br. 11-12, str. 581-585
Ohtahara sindrom - epileptička enceflopatija najranijeg detinjstva
Univerzitet u Novom Sadu, Medicinski fakultet, Institut za zdravstvenu zaštitu dece i omladine

e-adresagodipo@eunet.yu
Ključne reči: Mioklone epilepsije; Novorođenče; Infantilni spazmi; Ohtahara sindrom
Sažetak
Epileptičke encefalopatije ranog detinjstva su teški poremećaji moždane funkcije ispoljeni epileptičkim napadima rezi-stentnim na terapiju, paroksizmalnim elektroencefalografskim promenama i neurološkim, kognitivnim i poremećajima ponašanja. U osnovi im je specifična reakcija nezrelog mozga na nespecifičnu traumu i uzročno-posledična interakcija sa abnormalnom električnom aktivnošću koja nastaje i biva modifikovana u mozgu u razvoju. Ohtahara sindrom je retka i teška forma epileptičke encefalopatije najranijeg uzrasta, sa supresija-paroksizam elektroencefalografskim zapisom. Pretežno je simptomske etiologije sa teškim malformacijama nervnog sistema i po pravilu prisutnim mikroskopskim abnormalnostima migracije. Klinički se ispoljava neposredno po rođenju ili u prvim danima života, učestalim epileptičkim spazmima u budnosti i spavanju, ređe i eratičnim žarišnim motornim napadima i/ili hemikonvulzijama. Nema preporučene ni pouzdano efikasne terapije mimo terapije za Westov sindrom. Značajno kompromituje razvoj dece napadima rezistentnim na terapiju, teškom psihomotornom retardacijom i čestom evolucijom u Westov sindrom. Polovina dece umire u neonatalnom ili ranom odojačkom uzrastu.
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O članku

jezik rada: srpski
vrsta rada: pregledni članak
DOI: 10.2298/MPNS0812581K
objavljen u SCIndeksu: 06.04.2009.

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