Relationship of structural magnetic resonance imaging, magnetic resonance perfusion, and other disease factors to neuropsychological outcome in sickle cell disease.

OBJECTIVE To investigate the relationship between neuropsychological functioning and radiographic findings in children with sickle cell disease (SCD) with no history of clinical neurological events. METHODS Thirty-one patients with SCD randomly selected from a regional treatment center underwent neuropsychological and disease severity assessments. Of these, 22 also had structural magnetic resonance imaging and magnetic resonance perfusion studies performed. RESULTS Forty-five percent of the imaged subgroup showed imaging abnormalities that were found to be correlated with disease severity but not neuropsychological level of performance indices. A significant relationship, however, was found between imaging abnormalities and increased variability in neuropsychological performance. CONCLUSIONS These results corroborate the high rate of rheologic and vascular pathology in SCD and underscore the importance of representing neuropsychological functioning in multiple ways.