NEUROPSYCHOLOGICAL CONSEQUENCES OF SICKLE-CELL DISEASE

In this study we assessed neuropsychological abilities in patients wit h sickle cell disease (SCD) in the absence of clinically apparent stro ke. Eighteen adult patients with SCD with no history of stroke or chro nic transfusion were compared with a group of 18 unaffected ''nearest- relatives'' who were comparable in age, education, and gender. Partici pants were assessed with a battery of tests measuring memory, attentio n, language, motor speed, abstract reasoning, executive function, and visuospatial abilities. Medical and neurological evaluations, magnetic resonance (MR) imaging, and regional cerebral blood flow (rCBF) studi es were conducted. The SCD group demonstrated poorer performance on ti med tests of attention and construction. Five SCD cases and three of t he comparison group had noncortical abnormalities on MR imaging, and r CBF studies revealed hypofrontal flow patterns in the SCD cases. Howev er, cognitive deficits in the SCD group were evident even among indivi duals 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 etiolo gic factors.