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

Maryline Couette^{1

2

3}, Stéphanie Forté^{4

5

6}, Damien Oudin Doglioni^{1

7}, Armand Mekontso-Dessap^{2

3}, David Calvet⁸, Kevin H M Kuo^{9

10}, Pablo Bartolucci^{1

3

11}

Affiliations

¹ Sickle Cell Referral Centre-UMGGR, University of Paris Est Créteil, Henri Mondor APHP, 94010 Créteil, France.
² CARMAS (Cardiovascular and Respiratory Manifestations of Acute Lung Injury and Sepsis), University of Paris Est Créteil, 94010 Créteil, France.
³ IMRB, INSERM, University of Paris Est Créteil, 94010 Créteil, France.
⁴ Department of Medecine, Centre Hospitalier de l'Université de Montréal, Montréal, QC H2X 0C1, Canada.
⁵ Department of Medicine, Université de Montréal, Montréal, QC H3C 3J7, Canada.
⁶ Centre de Recherche du CHUM, Montréal, QC H2X 0A9, Canada.
⁷ Laboratoire Inter-Universitaire de Psychologie-Personnalité, Cognition, Changement Social (LIP/PC2S), Université Grenoble Alpes, 38058 Saint-Martin-d'Hères, France.
⁸ INSERM, UMR 1266, Psychiatry and Neurosciences Institute of Paris, Paris-Descartes University, Department of Neurology and Stroke Unit, Sainte-Anne Hospital, 75014 Paris, France.
⁹ Department of Medicine, University Health Network, Toronto, ON M5G 2C4, Canada.
¹⁰ Department of Medicine, University of Toronto, Toronto, ON M5S 1A8, Canada.
¹¹ INSERM-U955, Equipe 2, Laboratoire d'Excellence, GRex, Institut Mondor, 94000 Créteil, France.

PMID: 36836150
PMCID: PMC9967394
DOI: 10.3390/jcm12041615

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

Maryline Couette et al. J Clin Med. 2023.

. 2023 Feb 17;12(4):1615.

doi: 10.3390/jcm12041615.

Authors

Maryline Couette^{1

2

3}, Stéphanie Forté^{4

5

6}, Damien Oudin Doglioni^{1

7}, Armand Mekontso-Dessap^{2

3}, David Calvet⁸, Kevin H M Kuo^{9

10}, Pablo Bartolucci^{1

3

11}

Affiliations

¹ Sickle Cell Referral Centre-UMGGR, University of Paris Est Créteil, Henri Mondor APHP, 94010 Créteil, France.
² CARMAS (Cardiovascular and Respiratory Manifestations of Acute Lung Injury and Sepsis), University of Paris Est Créteil, 94010 Créteil, France.
³ IMRB, INSERM, University of Paris Est Créteil, 94010 Créteil, France.
⁴ Department of Medecine, Centre Hospitalier de l'Université de Montréal, Montréal, QC H2X 0C1, Canada.
⁵ Department of Medicine, Université de Montréal, Montréal, QC H3C 3J7, Canada.
⁶ Centre de Recherche du CHUM, Montréal, QC H2X 0A9, Canada.
⁷ Laboratoire Inter-Universitaire de Psychologie-Personnalité, Cognition, Changement Social (LIP/PC2S), Université Grenoble Alpes, 38058 Saint-Martin-d'Hères, France.
⁸ INSERM, UMR 1266, Psychiatry and Neurosciences Institute of Paris, Paris-Descartes University, Department of Neurology and Stroke Unit, Sainte-Anne Hospital, 75014 Paris, France.
⁹ Department of Medicine, University Health Network, Toronto, ON M5G 2C4, Canada.
¹⁰ Department of Medicine, University of Toronto, Toronto, ON M5S 1A8, Canada.
¹¹ INSERM-U955, Equipe 2, Laboratoire d'Excellence, GRex, Institut Mondor, 94000 Créteil, France.

PMID: 36836150
PMCID: PMC9967394
DOI: 10.3390/jcm12041615

Abstract

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.

Keywords: cognitive deficits; cognitive impairment; neuropsychological assessment; sickle cell anemia; sickle cell disease; stroke.

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Conflict of interest statement

Forté reported receiving grants from the Canadian Hematology Society and Pfizer during the conduct of the study and served as a consultant for Novartis. Kuo reported receiving grants from the National Institutes of Health National Heart, Lung, and Blood Institute (grant 1R33HL147845-01A1), Thalassemia Foundation Canada, Peter Munk Cardiac Centre, University of Toronto, Cincinnati Children’s Hospital Medical Center, Canadian Hematology Society and Pfizer during the conduct of the study, and served as a consultant for Agios, Alexion, Apellis, Aruvant, Bluebirdbio, Celgene, Novartis, and Pfizer, as chair of the data safety monitoring board for Bioverativ/Sanofi and had scientific collaboration with Abfero and Phoenicia Biosciences. No other disclosures were reported.

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

Affiliations

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

Authors

Affiliations

Abstract

Conflict of interest statement

References

LinkOut - more resources

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