PURPOSE - A review of the literature regarding cognitive and behavioral function in children with sickle cell disease (SCD) is presented, including a discussion of methodological issues to be considered when evaluating these patients and research in this area.
DESIGN - Sixteen studies were examined that addressed cognition and behavior in children with SCD.
RESULTS - Most studies of children with SCD who were classified as neurologically normal probably included a substantial number of children who had experienced silent strokes; this misclassification error likely resulted in erroneous findings of deficits in children thought to be free of brain injury. In most studies of children with SCD-related infarct, investigators failed to consider that patterns of impairment will vary depending upon lesion location. Many studies relied upon data from inappropriate control groups when examining the function of children with SCD.
CONCLUSIONS - Future studies should address a number of methodological factors. Brain imaging must be conducted to verify the presence or absence of cerebral infarcts. In children with imaging-verified infarcts, greater attention must be given to the relationship between lesion location and distinct patterns of cognitive or behavioral deficits. Sibling controls are the best choice in studies of children with SCD.