Sickle cell anemia and central nervous system infarction: a neuropathological study.

CNS infarcts were demonstrated in 12 of 24 autopsied patients with sickle cell anemia. The infarcts occurred most extensively in the territory supplied by the distal branches of the internal carotid artery, especially the anterior-middle cerebral artery boundary zone. They were regularly associated with organizing and recanalizing thrombi involving the distal cervical and proximal intracranial divisions of the internal carotid system. On the basis of these findings, we infer that the pathogenesis of the infarction involves perfusion failure or intraarterial embolization in addition to intravascular sickling.