Neurocognitive Deficits in Children With Sickle Cell Disease: A Comprehensive Profile

Background. Sickle cell disease (SCD) can lead to profound cerebral damage, associated with neurocognitive deficits. The aim of the current study was to evaluate a broad range of neurocognitive functions in children with SCD compared to a SES-matched control group, in order to gain more insight into the specific deficits of these patients. Methods. Forty-one children with homozygous SCD(HbSS or HbS-beta 0-thalassemia) and 38 controls were assessed on a comprehensive set of well-defined and validated measures of neurocognitive functioning. Besides general intelligence, we evaluated executive functioning extensively (including response inhibition, sustained attention, planning, visuo-spatial working memory, and verbal working memory) as well as visuo-motor functioning. Results. SCD was clearly associated with lower IQ scores. More than one in three children with SCD had a Full-scale IQ below 75. Furthermore, children with SCD showed deficits in visuo-motor functioning. Some evidence was found for executive dysfunction: Children with SCD displayed poor visuo-spatial working memory, as well as subtle deficits in sustained attention and planning. No significant differences were found between children with SCD and controls in terms of response inhibition and verbal working memory. Conclusions. Children with SCD are at increased risk of lower intelligence, visuo-motor impairments, and executive dysfunction. These neurocognitive deficits may underlie high rates of scholastic impairments in these children. The present findings further illuminate the importance of regular neurocognitive evaluations and future neurocognitive rehabilitation programs for children with SCD. Pediatr Blood Cancer 2011;56:783-788. (c) 2010 Wiley-Liss, Inc.