Neobičan slučaj Parkes-Weber-ovog sindroma kod bolesnika sa spontanom subarahnoidnom hemoragijom
Sažetak
Uvod. Parkes Weber-ov sindrom (PWS) je kompleksna i retka genetska bolest, koja se manifestuje kombinovanim vaskularnim malformacijama, prvenstveno na krvnim sudovima ekstremiteta, mada mogu biti zahvaćeni i krvni sudovi karlice. Izuzetno retko bolest može imati endokranijalnu i spinalnu lokalizaciju. Lečenje takvih bolesnika predstavlja izazov neurohirurškim centrima i zahteva multidisciplinarni pristup. Prikaz bolesnika. Prikazujemo 46-godišnjeg bolesnika, koji je primljen u Urgentni centar zbog spontane subarahnoidne hemoragije (SAH), flakcidne parapareze i urinarne inkontinencije. Bolesniku je prethodno postavljena dijagnoza PWS, a genetskom analizom dokazana je mutacija RASA1 gena. Takođe, bolesnik je imao SAH i bio hospitalizovan pre 26 godina, dok je pre šest godina, zbog višestrukih hilarnih aneurizmi desne renalne arterije i njenih grana, načinjena desnostrana nefrektomija. Urađena je digitalna subtrakciona angiografija endokranijuma, pri čemu nisu otkrivene aneurizmatske dilatacije i arteriovenske malformacije (AVM). Metodom magnetne rezonance nađena je intraduralna AVM na nivou između pršljenova T12 i L3, koja je u potpunosti ispunila duralnu vreću. Nakon konzervativnog lečenja došlo je do značajnog poboljšanja subjektivnog i kliničkog stanja bolesnika. Zaključak. Prema nama dostupnim podacima u referentnoj naučnoj literaturi, ovo je jedini prikaz bolesnika sa PWS sa spinalnom intraduralnom AVM i spontanim SAH bez srčane insuficijencije i sa istorijom prethodne nefrektomije.
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