Journal Article Ppublish 1 citation
PubMed: 38815229 DOI: 10.1182/bloodadvances.2023010925
Determinants of cognitive dysfunction in adults with sickle cell-related stroke or suspected neurological morbidity.
Blood Adv 8(15):3993-4002
Authors: Despina Messimeris, Hugo Bismuth, Corentin Provost, Clémentine Emaer, Nicolas Mélé, Robert Kitenge, Jean-Benoit Arlet, Laure Joseph, Brigitte Ranque, Pablo Bartolucci, Pauline Narme, David Calvet
Affiliations
- Institute of Psychiatry and Neuroscience of Paris, INSERM U1266, Paris, France. Neurology Department, GHU Paris Psychiatrie et Neurosciences, Hôpital Sainte Anne, Université Paris Cité, Paris, France.
- Neurology Department, GHU Paris Psychiatrie et Neurosciences, Hôpital Sainte Anne, Université Paris Cité, Paris, France.
- Institute of Psychiatry and Neuroscience of Paris, INSERM U1266, Paris, France.
- Neuroradiology Department, GHU Paris Psychiatrie et Neurosciences, Hôpital Sainte Anne, Université Paris Cité, Paris, France.
- Centre de formation et d'appui sanitaire, Centre hospitalier Monkole, Kinshasa, Democratic Republic of the Congo.
- Internal Medicine Department, French National Sickle Cell Referral Center, Hôpital Européen Georges Pompidou, Assistance-Publique Hôpitaux de Paris, Université Paris Cité, Paris, France.
- France Centre de référence des syndromes drépanocytaires majeurs, service de biothérapie, Centre d'investigation clinique, Hôpital Universitaire Necker-Enfants Malades, Université Paris Cité, Paris, France.
- Referral Center for Sickle Cell Disease and Red Blood Cell Disorders, UMGGR, Paris Est Créteil University, IMRB, INSERM U955, Créteil, France.
- Laboratoire Mémoire Cerveau et Cognition, UR 7536, Institut de Psychologie, Université Paris Cité, Paris, France.
Abstract
The prognosis of sickle cell disease (SCD) in adults is determined primarily by damage to targeted organs such as the brain. Cognitive dysfunction in SCD is a common chronic neurological manifestation, but studies remain mostly descriptive in adults. The objective of this study was to better characterize the cognitive profile and the association between cognitive dysfunction and brain lesions. We included adult patients with SCD referred for a neurological assessment. An adapted battery of neuropsychological tests was used to assess cognitive deficits. Brain or arterial abnormalities were assessed using brain magnetic resonance imaging/magnetic resonance angiography and a cervical and transcranial Doppler ultrasound. The cognitive profile of 96 patients was characterized by deficits in processing speed (58%), short-term memory (34%), and working memory (24%). Brain infarcts were found in 56% of patients and intracranial vasculopathy in 49%. Twenty percent of patients had no brain abnormalities. Processing speed dysfunction was associated with territorial infarcts (odds ratio [OR], 3.1; P = .03) and education outside of France (OR, 4.7; P = .02). Short-term memory dysfunction was associated with territorial infarcts (OR, 3.4; P = .01) and a low educational level (OR, 8.2; P = .01). Working memory dysfunction was associated with a low educational level (OR, 4.3; P = .05) and vasculopathy (OR, 3.7; P = .03). Cognitive dysfunction appears to be a hallmark sign of SCD, particularly for adults with sickle cell-related stroke or suspected neurological morbidity. Assessment of such dysfunction could be used in longitudinal follow-up and clinical trials.
MeSH
Cells, Memory, Short-Term, Stroke, Brain, Magnetic Resonance Imaging, Cognitive Dysfunction, Morbidity, Education, Memory, Hematologic Diseases