Major intracerebral arterial involvement in sickle cell disease.

It is commonly believed that strokes occurring in patients with sickle cell disease are due to abnormalities involving the small intracranial vessels, that is, at the level of small venules, capillaries, and precapillaries. The authors present a case of multiple major intracranial arterial disease in a 14-year-old boy with sickle cell disease. The angiographic findings and the pathophysiology of arterial changes are discussed along with a review of the literature. This report emphasizes that patients with sickle cell disease may present with major intracranial arterial involvement as a prominent feature.