Idiopathic supraclinoid and internal carotid bifurcation steno-occlusive disease in young American adults

We report a series of American adults with idiopathic steno-occlusive disease of the supraclinoid internal carotid artery and its bifurcation. We reviewed the clinical records and imaging of 18 patients, 16 women and 2 men, aged 20-53 years (mean 35 years). There were no predominating risk factors for vascular occlusive disease, such as oral contraceptive use, hypertension, diabetes mellitus, or smoking. Four patients had irregularity of their cervical internal carotid arteries in a pattern not classic but suspicious of fibromuscular dysplasia. Eleven patients met the criteria for moyamoya disease, having both bilateral disease and moyamoya collateral lenticulostriate arteries. Necropsy in one case showed intimal thickening with duplication of the internal elastic lamina involving the internal carotid artery bifurcation bilaterally. We found a marked predominance of young and middle-aged females in our American adults, but our findings do not support the association with birth-control pills previously reported.