Neuropsychological Changes in Children with Sickle Cell Disease and Their Correlation to the Imaging Studies

Abstract

Background: Early detection of neuropsychological changes in children with sickle cell disease (SCD) is essential to improve their quality of life. Aim of the Work: To assess neurological and psychological disorders in children with sickle cell disease (SCD) using multimodal approach through clinical, laboratory, neuroimaging and neurophysiological studies in a trial to detect etiological risk factors. Study Design: Cross-sectional study. Place and Duration of Study: Sample: Department of Pediatric (Hematology Unit) and Department of Neurology, Tanta University Hospital Egypt, between April 2016 and April 2018. Methodology: This study was conducted on 50 children (27 male and 23 female; age range 2-18 years) with SCD and 25 healthy children matched age and sex. All subjects were subjected to full history taking, neurologic examination using pediatric neurological sheet, laboratory investigations, neuroimaging including: CT and /or MRI, MRA and/or CT angiography, also MR, EEG and  Stanford-Binet Intelligence scales-Fifth Edition. Results: Most of patients presented with headache 66%, cognitive decline 48%, seizures 28%, and visual affection 24%. Less common presentations were, ischemic and hemorrhagic stroke 6% and 4% respectively. SCD children showed many abnormalities on neurological examination and on different modalities of MR imaging on the brain with positive correlation (X2=7.641, p-value <0.001*, r=0.248) with many risk factors. Prophylactic blood transfusion in SCD patients with abnormal TCD had a role in reducing the incidence of stroke. Conclusion: Children with SCD were presented with variable neuropsychological disturbance that correlated with the brain imaging.