Neurological Complications in Sickle Cell Disease: Clinical, Analytical and Radiological Correlations in a Multicentre Cohort

Sickle cell disease is a structural hemoglobinopathy with high global prevalence, especially in Africa. Neurological complications affect a high percentage of patients. An observational, retrospective, multicenter study was conducted in Aragon (Spain) among patients ≥12 years of age with sickle cell disease followed up between 2021 and 2022. Sociodemographic, clinical, analytical and therapeutic variables were analyzed, along with alterations in cerebral blood flow velocity assessed by transcranial and carotid Doppler ultrasound, and findings from imaging and magnetic resonance. Forty patients were evaluated (aged 12-58 years; 52.5% male). Forty percent of patients had pathological findings on magnetic resonance imaging (7.5% infarction, 17.5% silent infarcts, 5% cerebral aneurysms, and 5% stenosis). Only 7.5% of patients presented alterations in flow velocity assessed by transcranial Doppler ultrasound, with imaging of the carotid and middle cerebral arteries. The most severe phenotypes of sickle cell disease were associated with a higher incidence of neurological complications. Deficiencies in natural anticoagulant proteins were observed. This study shows low Doppler ultrasound sensitivity in screening for neurological complications in patients with sickle cell disease. Although the sample size is limited, this analysis provides relevant information and generates new study hypotheses about the value of incorporating structural neuroimaging into long-term follow-up strategies and thrombophilia studies in selected patients. However, larger prospective studies are needed to refine screening algorithms and determine the prognostic implications of neurological lesions in sickle cell disease.