Abstract
To delineate the natural progression of cerebral vascular accidents in SCD and to determine the resultant structural and functional defects we investigated 29 patients (27 SS, 2 SC) who sustained strokes. Subarachnoid or intra-cerebral hemorrhage and bone marrow embolism occurred in 9 patients ages 14-35 years. Cerebral infarction occurred in 20 (19 SS, 1 SC) with a modal age of onset of 5 years. We were able to evaluate 14 long term survivors who were observed for a mean period of 9.4 years following the initial stroke. 10 (71%) had 1 or more recurrences with a mean interstroke interval of 28 months. Residual functional impairment was noted in all 14. Computerized axial tomography on 9 showed areas of low density and ventricular dilatation indicating structural brain damage. These patients were not treated with a transfusion program. Their clinical course indicates that evaluation of such therapy requires long periods of observation.
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