Neuropsychological consequences of sickle cell disease

In this study we assessed neuropsychological abilities in patients with sickle cell disease (SCD) in the absence of clinically apparent stroke. Eighteen adult patients with SCD with no history of stroke or chronic transfusion were compared with a group of 18 unaffected 'nearest-relatives' who were comparable in age, education, and gender. Participants were assessed with a battery of tests measuring memory, attention, language, motor speed, abstract reasoning, executive function, and visuospatial abilities. Medical and neurological evaluations, magnetic resonance (MR) imaging, and regional cerebral blood flow (rCBF) studies were conducted. The SCD group demonstrated poorer performance on timed tests of attention and construction. Five SCD cases and three of the comparison group had noncortical abnormalities on MR imaging, and rCBF studies revealed hypofrontal flow patterns in the SCD cases. However, cognitive deficits in the SCD group were evident even among individuals without MR imaging or rCBF abnormalities. These results suggest that subtle neuropsychological deficits can be associated with SCD in the absence of stroke. Prospective studies are needed to assess etiologic factors.