Childhood strokes are associated with global and severe cognitive deficits in adult patients with sickle cell disease (SCD), according to a study published in the Journal of Clinical Medicine.

Due to advances in available therapeutics, patients with SCD have a longer life expectancy, meaning that new challenges surrounding chronic care have arisen. For example, scientists increasingly observe that adults with SCD lack cognitive abilities when compared to their healthy peers. 

In recognition of this fact, the American Society of Hematology now recommends cognitive surveillance in patients with SCD of all ages. However, comprehensive neurocognitive testing is still limited. Researchers are studying the suitability of neurocognitive questionnaires, such as the Montreal Cognitive Assessment (MoCA), in assessing neurocognition in these patients. 

The authors of this study sought to understand the cognitive profile of patients with SCD and the usefulness of the MoCA as a screening tool. They recruited 79 patients with SCD from Henri Mondor Hospital in Créteil, France. Participants were assessed neurocognitively using a number of scales, including the MoCA. Researchers also examined the clinical records of participants, paying special attention to past histories of cerebrovascular incidents. 

Read more about SCD etiology 

The research team reported that participants could be classified into 3 clusters based on their cognitive abilities: cluster 3 was normal or close to normal, cluster 2 depicted lower processing speeds and frontal lobe deficits, and cluster 1 represented critically altered cognitive domains. In addition, the researchers discovered that the MoCA was a reliable screening tool in routine clinical care. 

The authors of this study discovered that adults with the most severe neurocognitive deficits (belonging in cluster 1) were more likely to have experienced childhood stroke (78%). Patients who experienced childhood strokes were more likely to experience disruptions in their schooling and education, worsening their overall neurocognitive profile. 

“This study highlights the importance of stroke prevention in childhood and the provision of early cognitive support to help these patients succeed in school,” the authors of the study concluded. 


Couette M, Forté S, Oudin Doglioni DO, et al. Early strokes are associated with more global cognitive deficits in adults with sickle cell diseaseJ Clin Med. 2023;12(4):1615. doi:10.3390/jcm12041615