THE SPECTRUM OF BRAIN MR ABNORMALITIES IN SICKLE-CELL DISEASE - A REPORT FROM THE COOPERATIVE STUDY OF SICKLE-CELL DISEASE

PURPOSE: To define the spectrum of abnormalities in sickle-cell diseas e, including infarction, atrophy, and hemorrhage, that are identified by brain MR imaging. METHODS: All MR studies included T1, T2, and inte rmediate pulse sequences. Images were interpreted without knowledge of the clinical history or neurologic examination findings. Brain MR ima ging was performed in 312 children with sickle-cell disease. RESULTS: Seventy patients (22%) had infarction/ischemia and/or atrophy. Infarct ion/ischemia was noted in 39 children (13%) who had no history of a st roke (the ''silent'' group). The prevalence rates for silent lesions w ere 17% for sickle-cell anemia and 3% for hemoglobin sickle-cell disea se. For patients with sickle-cell anemia and a history of cerebrovascu lar accident, infarction/ischemia lesions typically involved both cort ex and deep white matter, while silent lesions usually were confined t o deep white matter. Within the age range studied, the prevalence of i nfarction/ischemia did not increase significantly with age, although o lder patients with lesions had more lesions than did younger patients with lesions. CONCLUSIONS: Brain MR imaging showed infarction/ischemia in the absence of a recognized cerebrovascular accident in 13% of pat ients. The prevalence of these lesions did not increase significantly between the ages of 6 and 14 years, suggesting that lesions are presen t by age 6. However, the increase in the average number of lesions per patient with age may indicate progressive brain injury.