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Neuroimaging and Cognitive Function in Sickle Cell Disease: A Systematic Review
Suad S Abdi1, Michelle De Haan1, Fenella J Kirkham1,2,3
1Developmental Neurosciences Section, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK.
Children (Basel, Switzerland)
|March 29, 2023
Summary
Neuroimaging abnormalities like strokes and altered brain activity are linked to cognitive deficits in children with sickle cell disease (SCD). Early intervention is crucial as brain damage begins in childhood.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Background:
- Sickle cell disease (SCD) is a prevalent inherited single-gene disorder.
- SCD complications include anemia, reduced oxygen transport, and cerebral vasculopathy, leading to stroke and cognitive impairment.
- Cognitive deficits in SCD affect executive function, attention, reasoning, language, memory, and IQ.
Purpose of the Study:
- To systematically review the association between neuroimaging findings and cognitive function in pediatric patients with SCD.
- To synthesize current evidence on how brain changes relate to cognitive outcomes in children with sickle cell disease.
Main Methods:
- Systematic literature search of PubMed and Embase databases (March 2022).
- Inclusion criteria: participants <18 years, English publications (1960-2022), any SCD genotype, examination of cognition-neuroimaging relationship.
- Exclusion criteria: case studies, editorials, reviews. Quality assessed using CASP Checklist.
Main Results:
- 33 studies met eligibility criteria from 303 retrieved articles.
- Overt/silent strokes, elevated blood flow velocities, abnormal functional connectivity, and reduced fMRI activation correlated with neuropsychological deficits in children with SCD.
- Neuroimaging abnormalities are associated with cognitive impairments in pediatric SCD patients.
Conclusions:
- Brain damage in SCD begins early, necessitating prompt management of disease manifestations.
- Most included studies were cross-sectional, limiting causal inference.
- Longitudinal studies are needed to understand the cumulative effects of SCD on brain development.
