The relationship between brain injury and vasculopathy in 146 sickle cell (SCD) patients with hemoglobin SS, the most serious form of SCD, was evaluated by MRI and MRA at St Jude Children’s Research Hospital, Memphis, TN. At an average age of 10 years, 46% of patients had brain injury revealed by MRI in the form of cystic infarction, ischemic damage, encephalomalacia, or atrophy, and 64% had vasculopathy identified by MRA as arterial totuosity (limited vasculopathy), and stenosis or occlusion (extensive vasculopathy). Patients with abnormal MRA usually had abnormal MRI, and only 28% had normal neuroimaging and angiography. Vasculopathy is prodromal to brain injury with SCD; it was limited in patients with cystic infarction and extensive with encephalomalacia. Large arteries were affected in 31% of patients with brain injury and small arteries in 69%. The degree of brain injury with SCD is related to the degree of vasculopathy. 
COMMENT. Brain injury is a common complication of sickle cell disease with hemoglobin SS, and the severity of injury is correlated with the degree of vasculopathy. Patients with normal vasculature have normal MRI and MRA, those with mild vasculopathy have limited brain injury (cystic infarction and lacunae), and patients with extensive vasculopathy (stenosis or occlusion) have severe brain injury (encephalomalacia). Brain injury in SCD may be preventable if the anemia and vasculopathy are corrected and reversed.