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Lesion burden and cognitive morbidity in children with sickle cell disease
Jeffrey Schatz1, Desiree A White, Asif Moinuddin
1Department of Psychology, University of South Carolina, Columbia, SC 29208, USA. schatz@sc.edu
Insights
Children with sickle cell disease and silent cerebral infarcts experience varying IQ effects based on lesion size. Large infarcts significantly lower IQ, while small infarcts show no apparent change.
Area of Science:
- Pediatric Neurology
- Hematology
- Neuroimaging
Background:
- Sickle cell disease (SCD) is associated with an increased risk of cerebrovascular complications.
- Silent cerebral infarcts (SCIs) are common in children with SCD, but their impact on cognitive function is not fully understood.
Purpose of the Study:
- To investigate the relationship between the extent of brain tissue injury (lesion volume) and IQ scores in children with SCD and SCIs.
Main Methods:
- Magnetic resonance imaging (MRI) was used to assess lesion volume in 27 children with SCD.
- Participants were categorized into three groups based on lesion volume: small (n=9), large (n=9), and no infarcts (n=9).
- Wechsler Full-Scale IQ scores were compared across the groups.
Main Results:
- Children with large SCIs (group 2) had significantly lower mean IQ scores (76.1) compared to those with small SCIs (group 1, 87.7) and no infarcts (group 3, 89.9).
- Small lesion volume was associated with no significant change in IQ compared to the control group.
- Large tissue loss due to SCIs is linked to reduced intellectual functioning.
Conclusions:
- In children with SCD, larger silent cerebral infarcts are associated with lower IQ scores.
- The cumulative effect of silent infarcts may negatively impact intellectual development over time.
- Early detection and management of SCIs are crucial for preserving cognitive function in children with SCD.
Abstract:
The effect of increased tissue injury in children with sickle cell disease and silent cerebral infarcts is not known. We determined the relationship between the extent of injury and IQ scores in children with silent cerebral infarcts. Participants were 27 children with sickle cell disease who had received magnetic resonance imaging (MRI). Children were divided into three groups: group 1, small lesion volume (n = 9, < 6.8 cm3); group 2, large lesion volume (n = 9; > 6.8 cm3); and group 3, no cerebral infarcts (n = 9). The Wechsler Full-Scale IQ was significantly lower for group 2 (mean = 76.1) when compared with group 1 (mean = 87.7) or group 3 (mean = 89.9). In children with silent cerebral infarcts, large tissue loss is associated with lower Wechsler Full-Scale IQ and small tissue loss is associated with no apparent change in IQ compared with children with no cerebral infarcts. The progressive accumulation of silent infarcts may lead to poorer intellectual functioning.