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

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.

Montreal cognitive assessment in Brazilian adults with sickle cell disease: The burdens of poor sociocultural background

“…However, Couette et al. [ 17 ] showed that, although the occurrence of stroke episodes did not affect global cognition, the time of the first stroke did: SCD patients with childhood stroke seem to be at increased risk of a global cognitive deficit profile. Furthermore, MoCA scores mirrored the severity of the cognitive deficits.…”

“…Furthermore, MoCA scores mirrored the severity of the cognitive deficits. The paediatric group also had lower educational attainment, which could be explained in part by the disruption of the patient's schooling due to childhood strokes [ 17 ].…”

Psychiatric comorbidities in adults with sickle cell disease: A narrative review

“…FSIQ predicts attendance to appointments 46 and executive dysfunction correlates with workforce engagement, independence with tasks and socialization 51 . Some literature has suggested that early CVA and SCI are associated with more severe or more global cognitive deficits, even in the absence of radiological evidence of a specific area of ischemia 52 …”

“…51 Some literature has suggested that early CVA and SCI are associated with more severe or more global cognitive deficits, even in the absence of radiological evidence of a specific area of ischemia. 52 Cerebrovascular accidents (CVAs), silent cerebral infarction (SCI) and ageing are common primary causes of neurocognitive symptoms in SCD. 47,53 Severe anaemia, lower foetal haemoglobin levels, pathological electroencephalograms and abnormal transcranial Doppler velocities join CVAs in predicting deficits in attention, memory, executive function and sensory-motor skillset.…”

Rehabilitation: a key service, yet highly underused, in the management of young patients with sickle cell disease after stroke in DR of Congo

“…Patients with sickle cell disease who have a history of childhood stroke are at an increased risk of global cognitive deficits. Despite the high prevalence of these cognitive disorders documented by several studies, there is no standardized approach to their screening and there is still, currently, a lack of data on rehabilitation for stroke and cognitive difficulties in people with sickle cell disease across the world, including even in high income countries ( 14 , 15 ).…”