Neuroimaging and Cognitive Function in Sickle Cell Disease: A Systematic Review

• Published in: Children (Basel) Vol. 10; no. 3; p. 532
• Main Authors: Abdi, Suad S, De Haan, Michelle, Kirkham, Fenella J
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 01-03-2023; MDPI
• Subjects: Anemia; Brain research; Case studies; Cerebrovascular disease; Cognition & reasoning; Cognition disorders; Cognitive ability; Hemodynamics; Hemoglobin; intelligence quotient; Ischemia; Magnetic resonance imaging; Medical imaging; Neuroimaging; Neuropsychology; Pediatrics; Review; sickle cell; Sickle cell anemia; Sickle cell disease; Stroke; Systematic review; transcranial Doppler; Veins & arteries; Velocity; United Kingdom; magnetic resonance imaging; transcranial Doppler; sickle cell; neuropsychology; connectome; anemia; intelligence quotient
• ISSN: 2227-9067; 2227-9067
• DOI: 10.3390/children10030532

Sickle cell disease (SCD) is the most common inherited single-gene disease. Complications include chronic anaemia, reduced oxygen-carrying capability, and cerebral vasculopathy, resulting in silent cerebral infarction, stroke, and cognitive dysfunction with impairments in measures of executive function, attention, reasoning, language, memory, and IQ. This systematic review aims to investigate the association between neuroimaging findings and cognition in children with SCD. Searches of PubMed and Embase were conducted in March 2022. Studies were included if participants were <18 years, if original data were published in English between 1960 and 2022, if any genotype of SCD was included, and if the relationship between cognition and neuroimaging was examined. Exclusion criteria included case studies, editorials, and reviews. Quality was assessed using the Critical Appraisal Skills Programme Case Control Checklist. A total of 303 articles were retrieved; 33 met the eligibility criteria. The presence of overt or silent strokes, elevated blood flow velocities, abnormal functional connectivity, and decreased fMRI activation were associated with neuropsychological deficits in children with SCD when compared to controls. There is a critical need to address the disease manifestations of SCD early, as damage appears to begin at a young age. Most studies were cross-sectional, restricting the interpretation of the directionality of relationships. Future research employing longitudinal neuroimaging and neuropsychological assessments could improve our understanding of the cumulative consequences of SCD on the developing brain.