Abstract
Objective
Sickle cell disease (SCD) is associated with cerebrovascular disease, cerebral infarction, and cognitive dysfunction. This study aimed to detect the presence and extent of white matter abnormalities in individuals with SCD using voxelโbased morphometry (VBM).
Methods
Thirtyโsix children and adolescents with SCD (age range, 9โ24 years) and 31 controls (8โ25 years) underwent magnetic resonance investigations using T1โ and T2โweighted protocols. White and gray matter density maps were obtained from threeโdimensional magnetic resonance imaging (MRI) data sets. Using VBM, we compared the maps between controls and SCD individuals with silent white matter infarct lesions (SCD+L; n = 16), and those without visible abnormality (SCDโL; n = 20).
Results
In comparison with controls, intelligence quotients (IQs) were lower in both SCD groups irrespective of presence of visible lesions. VBM showed widespread bilateral white matter abnormalities in the SCD+L group, extending beyond the regions of focal infarction in the deep anterior and posterior white matter borderzones. Bilateral white matter abnormalities were also observed in the SCDโL group, in locations similar to those in the SCD+L group.
Interpretation
VBM is sensitive to detection of widespread white matter injury in SCD patients in borderzones between arterial territories even in the absence of evidence of infarction. Those changes may contribute to cognitive deficits in this population. Ann Neurol 2006