Occlusion of Large Cerebral Vessels in Sickle-Cell Anemia

Seven patients with sickle-cell anemia and neurologic deficits secondary to Central-nervous-system dysfunction were studied with cerebral angiography after careful preparation. Preparation of the patient reduced the level of S hemoglobin to less than 20 per cent before study, and all studies were performed without sequelae. Six of the seven patients were found to have partial or complete occlusion of large cerebral vessels. The internal carotid artery was involved in all six patients. Disease in the anterior and middle cerebral arteries as well as the vertebral artery was also observed. Two patients had repeat studies. The disease appeared to be progressive. These findings, rarely reported, indicate that the common clinical assumption that the Central-nervous-system manifestations of sickle-cell anemia are a consequence of exclusively small-cerebral-vessel obstruction may in fact be erroneous.