Early Strokes Are Associated with More Global Cognitive Deficits in Adults with Sickle Cell Disease

This study sought to link neurocognitive profiles in sickle cell disease (SCD) patients with clinical characteristics. We conducted a prospective cohort study of adults with SCD who underwent comprehensive neuropsychological assessment at the UMGGR clinic at Henri Mondor Hospital, Creteil (France). A cluster analysis was performed based on neuropsychological testing scores. The association between clusters and clinical profiles was assessed. Between 2017 and 2021, 79 patients with a mean age of 36 [range 19-65] years were included. On principal component analysis, a 5-factor model presented the best fit (Bartlett's sphericity test [chi(2) (171) = 1345; p < 0.001]), explaining 72% of the variance. The factors represent distinct cognitive domains and anatomical regions. On hierarchical classification, three clusters emerged. Cluster 1 (n = 24) presented deficits in all five factors compared to Cluster 3 (n = 33). Cluster 2 (n = 22) had deficits in all factors, but to a lesser extent than Cluster 1. MoCA scores mirrored the severity of these cognitive deficits. Age, genotype and stroke prevalence did not differ significantly between clusters. However, the time of first stroke occurrence differed significantly between Cluster 1 and 2-3: 78% of strokes occurred during childhood, whereas 80% and 83% occurred during adulthood in Clusters 2 and 3, respectively. Educational attainment was also reduced in Cluster 1. SCD patients with childhood stroke seem to be at increased risk of a global cognitive deficit profile. In addition to existing methods of primary and secondary stroke prevention, early neurorehabilitation should be prioritized in order to reduce the long-term cognitive morbidity of SCD.

Automated summary

This study aimed to investigate the relationship between neurocognitive profiles and clinical characteristics in sickle cell disease (SCD) patients. A cohort study was conducted in adults with SCD who received comprehensive neuropsychological assessments. Cluster analysis based on neuropsychological testing scores was performed and the association with clinical profiles was examined. The results suggest that SCD patients with childhood stroke may be at higher risk of experiencing a global cognitive deficit profile. Therefore, early neurorehabilitation should be prioritized to reduce the long-term cognitive morbidity of SCD, in addition to existing stroke prevention methods.