The angiographic presentation of European Moyamoya angiopathy

BACKGROUND AND PURPOSE: Little is known about the angiographic presentation of Moyamoya angiopathy (MMA) in non-Asian patients. METHODS: Conventional cerebral angiograms from 155 Caucasian patients diagnosed as MMA were analyzed with respect to extracranial champagne bottle neck sign, Suzuki stages, collateral status, as well as presence of aneurysms and posterior cerebral artery stenosis. RESULTS: In 84 of 155 angiograms, the extracranial carotid artery was visualized, in 65 of them (77.4%), a champagne bottle neck sign was noted. Of the 278 analyzable hemispheres, 13.7%,11.2%, 37.8%, 27.3%, 8.6%, and 1.4% were classified as Suzuki stage I, stage II, stage III, stage IV, stage V, and stage VI, respectively. Among 280 hemispheres, in 53 hemispheres (18.9%) isolated basal collaterals (pathway I) and in 104 hemispheres (37.1%) choroidal and pericallosal collaterals (including basal collaterals, pathway II) were found. In 74 hemispheres (26.4%) ethmoidal collaterals (pathways III), and in 17 hemispheres (6.1%) vault collaterals were visualized. Patients with higher Suzuki stages IV–VI (p = 0.008) and ethmoidal collaterals (p < 0.001) suffered more often from cerebral hemorrhage. Transient ischemic attacks occurred more frequently in patients with Suzuki stage I to III (p < 0.001). In 10 of 155 patients (6.5%), the angiogram revealed a cerebral aneurysm. In 13 patients (8.4%), a stenotic P1 segment of the posterior cerebral artery was found. CONCLUSIONS: This is so far the largest observational study about angiography in Caucasian European MMA patients. A comparison with Asian data indicates similarity of disease in Caucasian and Asian patients.