BackgroundSickle 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 SESâmatched control group, in order to gain more insight into the specific deficits of these patients.MethodsFortyâone children with homozygous SCD (HbSS or HbSâÎČ0âthalassemia) and 38 controls were assessed on a comprehensive set of wellâdefined and validated measures of neurocognitive functioning. Besides general intelligence, we evaluated executive functioning extensively (including response inhibition, sustained attention, planning, visuoâspatial working memory, and verbal working memory) as well as visuoâmotor functioning.ResultsSCD was clearly associated with lower IQ scores. More than one in three children with SCD had a Fullâscale IQ below 75. Furthermore, children with SCD showed deficits in visuoâmotor functioning. Some evidence was found for executive dysfunction: Children with SCD displayed poor visuoâspatial 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.ConclusionsChildren with SCD are at increased risk of lower intelligence, visuoâmotor 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;56:783â788. © 2010 WileyâLiss, Inc.