Sestre Milosrdnice University hospital and Institute of Clinical Medical Research
Abstract
A 40-year-old male presented to emergency room with epileptic grand mal seizure. He had untreated hypertension, and prior diagnostic investigation showed duplex renal arteries of the right kidney with hyperreninemia in the left renal vein. He was nonsmoker, with moderate alcohol intake. Neurologic examination was normal except for high blood pressure and tongue bite. Electroencephalogram was nonspecific. Nuclear magnetic resonance (NMR) showed vascular lesions in the white matter and infratentorially an expansive lesion with no postcontrast imbibition in the right cerebellar hemisphere. Neurosonography revealed hypoplasia of both internal carotid arteries (ICA), mean diameter <2 mm, subtotal stenosis at the origin of both ICA, and development of collateral path, typical for moyamoya disease. Magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) confirmed the neurosonography diagnosis. Immunologic tests for vasculitis were negative, while hematologic examination showed 4G allele for PAI-1. Serum lipids were elevated. We recommended neurosurgical operation of brain tumor. Histopathologic finding showed meningioma. This case is interesting because of the rare complex cerebrovascular disease, i.e. coexistence of hypoplasia of both ICA, bilateral subtotal stenosis of ICA, intracranial moyamoya disease, and brain tumor.Muškarac u dobi od 40 godina pregledan je u hitnoj ambulanti zbog epileptičnog napada tipa grand mal. Bolovao je od neliječene hipertenzije, ranijom dijagnostičkom obradom verificirana je dvostruka bubrežna arterija desno i hipereninemija u lijevoj bubrežnoj veni. Bio je nepušač s umjerenom konzumacijom alkohola. Neurološki pregled je bio uredan osim povišenih vrijednosti krvnog tlaka i traga ugriza jezika. Elektroencefalogram je bio nespecifičan. Magnetskom rezonancijom mozga nađene su vaskularne lezije u bijeloj tvari te infratentorijalno u desnoj cerebelarnoj hemisferi ekspanzivni proces bez postkontrastne imbibicije. Neurosonološkim ispitivanjem potvrđena je hipoplazija obiju unutarnjih karotidnih arterija (ACI) s prosječnim promjerom <2 mm, subtotalna stenoza polazišta obiju ACI i razvoj kolateralnog puta, što je tipično za bolest moyamoya. Magnetska angiografija (MRA) i digitalna subtrakcijska angiografija (DSA) su potvrdile neurosonološku dijagnozu. Imunološki testovi za vaskulitis su bili negativni, dok je hematološkom obradom nađen 4G alel za plazminogen aktivator inhibitor-1 (PAI-1). Serumski lipidi su bili povišeni. Preporučili smo mu operaciju tumora mozga. Histopatološki nalaz je ukazivao na meningeom. Ovaj slučaj je zanimljiv zbog rijetke složene cerebrovaskularne bolesti, odnosno supostojeće hipoplazije obiju ACI, bilateralne subtotalne stenoze obiju ACI, intrakranijske bolesti moyamoya te tumora mozga
