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, Créteil (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 [χ² (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.

Intensive Care Unit (ICU) patients often experience significant discomfort and distress due to both the medical environment and the nature of their stay. While long-term sequelae such as depression, anxiety, and post-traumatic stress are well-documented, few studies have examined psychological disorders present at the time of ICU discharge. Based on the model of Post-Intensive Care Syndrome, specifically the mental component (PICS-M), we defined DICS-M (Discharge Intensive Care Syndrome - Mental component). This study aimed to estimate the prevalence of psychological disorders at ICU discharge and to identify potential mediators and risk factors. We conducted a prospective observational study involving 243 patients admitted between January 2023 and April 2024. The prevalence of DICS-M was 53% [95% CI: 46–59], with acute stress, anxiety, and depression observed in 37%, 36%, and 23% of patients, respectively. The analyses revealed an overlap among these psychological components. Peritraumatic distress acted as the main mediator of DICS-M. Univariate and multivariable analyses identified female gender and a history of psychiatric and cardiac conditions as risk factors of DICS-M. Psychological disorders are common at ICU discharge, mediated by peritraumatic distress, and associated with identifiable risk factors. These findings may help guide interventions to prevent long-term sequelae of ICU stays.

Intensive care unit (ICU) patients often endure discomfort and distress brought about by their medical environment and the subjective experience of their stay. Distress, pain, and loss of control are important predictors of future neuropsychiatric disorders. Depression, anxiety, and post-traumatic stress are common after discharge. We aimed at mitigating acute stress and discomfort via a novel intervention based on body image rehabilitation and rehabilitation of senses performed following a holistic approach guided by positive communication (corporeal rehabilitation care, CRC). We conducted a prospective observational study on 297 consecutively enrolled patients participating in at least one CRC session. Benefits of CRC were assessed on both subjective analogical scales of stress, pain, and well-being criteria, and objective clinical measures of dyspnea, respiratory rate, and systolic arterial pressure, just after CRC and long after (a median of 72 min later) to estimate its remote effect. Results showed that CRC had a positive effect on all overt measures of distress (acute stress, pain, discomfort) just after CRC and remotely. This beneficial effect was also observed on dyspnea and respiratory rate. Results also showed that best CRC responders had higher baseline values of stress and heart rate and lower baseline values of well-being score, indicating that the care targeted the population most at risk of developing psychological sequelae. Interestingly, a positive CRC response was associated with a better survival even after adjustment for physiologic severity, indicating a potential to identify patients prompt to better respond to other therapeutics and/or rehabilitation. This study demonstrated the feasibility of an innovative holistic patient-centered care approach and its short-term positive effects on critical parameters that are considered risk factors for post-intensive care syndrome. Further studies are warranted to study long-term benefits for patients, and overall benefits for relatives as well as ICU staff.

Background: Cognitive impairment is a dreaded complication of sickle cell disease (SCD) that impacts quality of life, school performance and employment. In 2020, the American Society of Hematology issued a strong recommendation that clinicians supervising the care of adults with SCD conduct surveillance for cognitive impairment using simplified signaling questions (DeBaun, 2020). However, guidance on the optimal screening strategy is lacking and several available tools are biased by language and education. The Rowland Universal Dementia Assessment Scale (RUDAS) was specifically designed for cognitive screening in multicultural populations (Storey, 2004). In the general elderly population, RUDAS is less biased by education than the Montreal Cognitive Assessment (MoCA) (Naqvi, 2015). Hypothesis: In adults with SCD, performance on the RUDAS is less influenced by educational attainment when compared to the MoCA. Our primary aim was to estimate the prevalence of suspected cognitive impairment using RUDAS and MoCA in adult SCD patients. The secondary aims were to examine for the presence of educational bias and to develop mitigation strategies in case of such a bias. Methods:Study design: cross-sectional study at UMGRR clinic at Henri Mondor Hospital, Créteil (France). Inclusion criteria: out-patients ≥18 years-old; all SCD phenotypes. Exclusion criteria: inability to obtain informed consent and/or follow study instructions. Intervention: Cognitive screening was performed using the RUDAS (translated to French by Philippe Desmarais), MoCA (third alternative version) and an additional visuospatial task of copying overlapping triangles (from the French BEC96 assessment). RUDAS and MoCA scores <28 and <26, respectively, were considered suggestive of cognitive impairment per previous studies (Basic, 2009 and Nasredinne, 2005) and patients were referred for definite neuropsychological evaluation. Survey on demographics and screening for depression and anxiety using Hospital Anxiety Depression Scale (HADS) were completed by the participants. Educational attainment was scored based on the number of years of schooling for the highest completed diploma. Statistical plan: linear regression was performed to identify possible associations between RUDAS, MoCA and social determinants of health. Results: Among the first 45 consecutive adult SCD patients undergoing routine cognitive screening, the median age was 39 (range 19-67). RUDAS and MoCA scores suggestive of mild cognitive impairment were found in 33/45 (73.3%) and 29/45 (64.4%) participants, respectively. There was a strong correlation between both tests (r=0.48, p=0.001). Both RUDAS and MoCA scores increased significantly with increasing level of education (r=0.36, p=0.015 and r=0.39, p=0.007, respectively), but were not significantly influenced by the HADS score. RUDAS and MoCA test items most biased by education were visuoconstructional tasks. Tasks assessing executive functioning and language were also biased in MoCA. Substituting the 3D visuospatial task of the RUDAS by a 2D task reduced the educational bias (r=0.20, p=0.045). Adding 1 point for highest level of education £ 12 years after kindergarten did significantly mitigate the effect of education on the RUDAS but only partially for the MoCA (r=0.23, p=0.131 and r=0.30, p=0.047). Conclusions: Overall, these results suggest there is an educational bias in the neurocognitive screening of adult SCD patients using available tools such as the RUDAS and MoCA. Although RUDAS was less biased overall, visuospatial assessment remained biased. The task often considered more "culture-fair" is still subject to the impact of educational potential (Statucka, 2019). We provide different strategies to mitigate education bias when assessing with RUDAS. Thus, the RUDAS adjusted by the educational level allows to systematically identify SCD patients in need of comprehensive neurocognitive testing. Prospective validation is ongoing.