Abstract
Neurocognitive disorders (NCD) are frequent yet underrecognized in adults with sickle cell disease (SCD), and practical strategies for routine cognitive assessment remain limited. We evaluated the psychometric properties of the Rowland Universal Dementia Assessment Scale (RUDAS) in the cognitive evaluation of adults with SCD.Between 2018 and 2023, adults with SCD were recruited from three comprehensive centers in Canada and France. Participants completed either the French or English version of the RUDAS. A prespecified subset underwent comprehensive multidisciplinary neurocognitive evaluation to establish a clinical diagnosis of minor or major NCD. The diagnostic performance of clinically relevant RUDAS cutoffs was assessed against a multidisciplinary reference diagnosis of minor or major NCD.A total of 440 adults with SCD were included (median age, 34 years; 56.4% female). The median RUDAS score was 26 (range, 16-30). A RUDAS cutoff score <27 demonstrated a specificity of 83% for detecting NCD. Adjustment for educational attainment increased specificity to 89%, with corresponding reduction in sensitivity from 50% to 45%. Latent class analysis identified two distinct cognitive profiles; individuals in the lower-performing group were older and more likely to have a history of prior stroke, vascular risk factors, impaired kidney function, lower educational attainment, and unemployment.In adults with SCD, the RUDAS provides a pragmatic and accessible approach to support brief cognitive assessment in routine clinical care. While its role in isolation is limited by moderate sensitivity, its high specificity may facilitate targeted referral for comprehensive neurocognitive evaluation and interventions.