Determinants of cognitive dysfunction in adults with sickle cell–related stroke or suspected neurological morbidity

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 mani-festation 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 SCD patients referred for a neurological assessment. An adapted bat-tery of neuropsychological tests was used to assess cognitive deficits. Brain (white matter lesions or infarcts) or arterial (stenosis or occlusion) abnormalities were assessed using brain MRI/MRA and a cervical and transcranial Doppler ultrasound. The battery was completed in 96 patients. The cognitive profile was characterized by deficits in processing speed (58% (95% CI [48-68])), short-term memory (34% (95% CI [24-43])) and working memory (24% (95% CI [15-33])). Brain in-farcts were found in 56% of patients and intracranial vasculopathy in 49%. Twenty percent of pa-tients had no brain abnormalities. Processing speed dysfunction was associated with territorial in-farcts (OR 3.1, p=0.03) and education outside of France (OR 4.7, p=0.02). Short-term memory dysfunction was associated with territorial infarcts (OR 3.4, p=0.01) and a low educational level (OR 8.2, p=0.01). Working memory dysfunction was associated with a low educational level (OR 4.3, p=0.05) and vasculopathy (OR 3.7, p=0.03). Cognitive dysfunction appears to be a hallmark sign of SCD, particularly for adults with sickle cell related stroke or suspected neurological mor-bidity. Assessment of such dysfunction could be used in longitudinal follow up and clinical trials.