Vol.5, No 1, 1998 pp. 18 - 22
UDC: 612.82;616-005
MOYAMOYA DISEASE: CLINICAL AND ANGIOGRAPHIC FEATURES
Dragan Stojanov1, Petar Bošnjaković1, Zoran Milenković2,
Nebojša Stojanović2, Ivan Stefanović2, Miroslava Živković3
1Institute of Radiology, 2Neurosurgical Clinic, 3Clinic for Neurology, Clinical Center, Niš,  Yugoslavia
Summary. This is a retrospective study of 12 adults (8 female, 4 male, mean age 45.3; range 26-63) and two children with moyamoya disease (MD) identified among 3400 diagnostic cerebral angiographies performed in a period of 17 years. Diagnosis of MD was made on the basis of features and progression of angiographic findings. Clinical manifestations and angiographic findings were analysed with a review of the literature. Two children and six adult patients had a clinical feature of ischemic cerebral events. The other adult patients had clinical signs of subarachnoid and/or intracerebral and intraventricular hemorrhage. Cerebral angiography showed a typically fine network of vessels at the base of the brain with a hazy, puff-of-smoke appearance, and development of transdural and leptomeningeal anastomoses in children and 8 adults patients. Four adult patients were in the terminal stage, with complete cerebral supply via vertebrobasilar and external carotid branches. Two of the adult patients had accompanying saccular aneurysms with localisations on basilar and internal carotid bifurcations, of which the latter was bleeding. All angiographic changes were bilateral. Low incidence and adult predominance are characteristic of MD in our population. There was no familial occurence among our patients. The clinical features of ischemic stroke were present in both children and adults, and intracranial hemorrhage in adults only. A characteristic angiographic feature of moyamoya was found in both children and adults.
Key words: Moyamoya disease, incidence, familial occurence, clinical feature, cerebral angiography, aneurysm
MOYAMOYA OBOLJENJE: KLINIČKE I ANGIOGRAFSKE KARAKTERISTIKE

Retrospektivnom studijom obuhvaćeno je 12 odraslih i dvoje dece obolelih od oboljenja Moyamoya (MD) dijagnostikovanih u periodu od 17 godina. U radu su analizirane kliničke manifestacije i angiografski nalazi u obolelih. Dijagnoza MD postavljena je angiografskim pregledom. Dvoje dece i 6 odraslih bolesnika imalo je kliničku sliku ishemijskih cerebralnih promena. Klinički znaci subarahnoidalne i/ili intracerebralne i intraventrikularne hemoragije bili su prisutni u ostalih bolesnika. Cerebralnom angiografijom nađene su stenookluzivne promene unutrašnjih karotidnih arterija i tipična mreža krvnih sudova magličastog izgleda na bazi mozga sa razvojem transduralnih i leptomeningealnih anastomoza u dece i 8 odraslih bolesnika. Četiri odraslih bolesnika bilo je u terminalnom stadijumu bolesti sa okluzijom unutrašnjih karotidnih arterija i kompletnom vaskularizacijom mozga preko vertebrobazilarnog sistema i grana spoljašnjih karotidnih arterija. Dvoje odraslih bolesnika imalo je udružene sakularne aneurizme lokalizovane na bifurkaciji bazilarne i unutrašnje karotidne arterije, Angiografske promene u svih bolesnika bile su obostrane.
Ključne reči: Moyamoya oboljenje, incidenca, kliničke karakteristike, cerebralna arteriografija, aneurizma