Neurocognitive Sequelae of Sickle Cell Disease: Current Issues and Future Directions

Abstract

Sickle cell disease is a genetic disorder with an autosomal recessive pattern of inheritance. This disease is most prevalent among those of African American and Mediterranean descent. Cerebral vascular accident (CVA) or stroke is one of the major complications of hemoglobin SS (Hgb SS) disease. CVA has implications for physical as well as neurocognitive function for children. Recent literature suggests that some children with sickle cell disease without overt signs of CVA may still have evidence of neurological deficit, both on magnetic resonance imaging and neurological examination. There is a growing body of knowledge that further aids in delineation of risk factors for CVA, silent infarct, and neurocognitive deficits in children with Hgb SS disease. More research is needed to continue to explore avenues for identification and intervention. The purpose of this article is to delineate areas of ongoing research in this important area.