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 identified 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.

The Predictive Value of Salt Sensitivity and Osmotic Fragility in the Development of Cerebral Small Vessel Disease

“…This test has not previously been used in patients with CSVD. It should be noted that sickle cell anaemia is currently associated with CSVD development, as well as endothelial dysfunction and the formation of WMHs [28,29].…”

Disease severity and slower psychomotor speed in adults with sickle cell disease

“…13 Thus, our results suggest genotype is a stronger risk factor for poorer cognitive function than age in our cohort. This is important because although cognitive function has been well characterized in children with SCD, 8,24 few studies have examined factors that influence cognitive function during adulthood 13,25 or in milder forms of the disease.…”

An Assessment of the Relationship between Structural and Functional Imaging of Cerebrovascular Disease and Cognition-Related Fibers

“…Exclusion criteria: (1) patients with severe visual and hearing impairment, severe physical weakness, or aphasia affecting the examination or failing to cooperate; (2) patients with mental illness or disturbance of consciousness; (3) patients who had already presented cognitive dysfunction and their assessment scores exceeding 2 points or a short caregiver questionnaire totalling more than 56 points; (4) patients who have pseudocognitive impairment or dementia caused by depression, anxiety, or AD; (5) patients whose cognitive dysfunction caused by other diseases, including infection, cancer, poisoning, metabolic diseases, and congenital mental retardation; (6) patients with severe heart, liver, lung, kidney and other organ failure, and therefore the examination cannot be completed; (7) patients who have MRI scan contraindications or are unable to scan; (8) patients whose imaging examination indicates the presence of other intracranial diseases, including cerebral hemorrhage and subarachnoid hemorrhage; (9) patients or their family are not willing to participate in the experiment.…”

“…Although the voxel-based morphology (VBM) analysis method can be used not only to analyze the difference in the gray matter structure between Alzheimer's disease (AD) patients and normal individuals but also to suggest changes in white matter structure, but whether white matter atrophy exists and its degree of damage cannot pass [6]. is method is completely deterministic, suggesting that macroscopic white matter damage detected by voxel-based morphology (VBM) does not fully reflect microscopic white matter integrity changes [7]. Diffusion tensor imaging (DTI) is a more sensitive imaging method for detecting white matter microscopic tissue damage, and it has been confirmed that DTI parameters are more closely related to MRI image volume and cognition [8].…”