Can Neuroimaging Markers of Vascular Pathology Explain Cognitive Performance in Adults With Sickle Cell Anemia? A review of the Literature

Abstract

Adults with homozygous sickle cell anemia have, on average, lower cognitive function than unaffected controls. The mechanisms underlying cognitive deterioration in this population are poorly understood, but cerebral small vessel disease (CSVD) is likely to be implicated. We conducted a systematic review using the Prisma Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines of articles that included both measures of cognitive function and magnetic resonance imaging (MRI) neuroimaging markers of small vessel disease. While all five studies we identified reported small vessel disease by MRI, only two of them found a significant relationship between structural changes and cognitive performance. Differences in methodologies and small sample sizes likely accounted for the discrepancies between the studies. We conclude that while MRI is a valuable tool to identify markers of CSVD in this population, larger studies are needed to definitely establish a link between MRI-detectable abnormalities and cognitive function in sickle cell anemia.

Keywords:

• Cerebral small vessel disease (CSVD)
• cerebrovascular
• cognition
• neuroimaging
• sickle cell anemia

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.

Funding

Neurovascular Determinants of Cognitive Function in Adults with Sickle Cell Disease (PI: Enrico Novelli) National Heart, Lung, and Blood Institute, [2016–2021R01HL127107]; Cardiovascular Epidemiology Training Program Grant NHLBI [#T32: HL083825], Program Director: Dr. Trevor Orchard, MBBCh, MMedSci, FAHA.