FSIQFull-scale IQ SCD Sickle cell disease SCI Silent cerebral infarction AIM Sickle cell disease (SCD) is the commonest cause of childhood stroke worldwide.Magnetic resonance imaging (MRI) is routinely used to detect additional silent cerebral infarction (SCI), as IQ is lower in SCI as well as stroke. This review assesses the effect of infarction on IQ, and specifically whether, compared to healthy controls, IQ differences are seen in children with SCI with no apparent MRI abnormality. RESULTS Mean differences in IQ between all three groups were significant: stroke patients had lower IQ than patients with SCI by 10 points (six studies); patients with SCI had lower IQ than no patients with SCI by 6 points (17 studies); and no patients with SCI had lower IQ than healthy controls by 7 points (seven studies).INTERPRETATION Children with SCD and no apparent MRI abnormality have significantly lower IQ than healthy controls. In this chronic condition, other biological, socioeconomic, and environmental factors must play a significant role in cognition.Sickle cell disease (SCD) is a lifelong inherited genetic disease associated with a high prevalence of stroke and cognitive dysfunction in childhood. Approximately 10% of patients will experience an overt stroke. 1 However, in the first decade of life, around one-third of children with SCD will accumulate at least one silent cerebral infarct (SCI; i.e. an abnormality seen on T2-weighted magnetic resonance imaging [MRI] in the absence of overt stroke, or neurological symptoms lasting more than 24h).2 By definition, SCI are clinically silent and therefore age at which SCI occurred and time lapse between SCI and cognitive testing are unknown. Stroke and SCI have been associated with general cognitive dysfunction, including problems with sustained attention, cognitive flexibility, and working memory.
3-7Full-scale IQ is the most commonly reported and widely studied standardized measure of general cognitive ability in SCD. Chordokoff and Whitten 8 published the first study investigating IQ between patients with SCD and controls, and found no differences. However, from the 1980s/early 1990s there were many studies suggesting that patients have lowered global intelligence scores than matched controls, even when excluding those with history of stroke or abnormal neurological examination.9-14 The first study that used MRI to classify patients into groups based on whether SCI are present or absent was published in
1996.15 Collaborators in the large Cooperative Study in Sickle Cell Disease (CSSCD) in the United States linked the presence of MRI abnormality and measurable global cognitive dysfunction. Since then, several studies have confirmed that children with SCI (SCI+) generally have lower IQ scores than those without evidence of SCI (SCIÀ). [16][17][18][19][20][21] These findings established a potential link between the presence of lesions and lesion size as a mediating factor in a child's IQ score.The presence, nature, and aetiology of any differences in IQ between children wit...