The aim of this study was to explore whether intellectual performance in children with Sickle Cell Disease and with low risk of stroke as determined with conventional transcranial Doppler ultrasonography (TCD) criteria was associated with hemodynamic parameters in imaging TCD, when controlling for hematological and socio-economical variables and presence of silent infarcts. We performed neuropsychological testing with Kaufman Brief Intelligence Test (K-BIT-IQ) and imaging TCD examinations to measure blood flow velocities and pulsatility indexes (PI) in the middle cerebral arteries (MCA) In 46 children with homozygous HbSS (mean age 108±34 months, range limits: 47–166 months; 24 females), without a history of stroke or transient ischemic attack, with no stenosis on magnetic resonance angiography and with velocities below 170 cm/s in screening conventional TCD. Mean K-BIT IQ Composite and Vocabulary scores (91±13 and 86±14 respectively) were significantly below the average scores of 100 for the age-matched population (one sample t-test=5.21, p<0.001). Using univariate and multivariate regression models, we found that lower PI in the right MCA was associated with lower K-BIT-IQ Composite and Vocabulary scores. Furthermore, we found that interhemispheric differences in PIs were even more strongly associated with neuropsychological performance, whereas flow velocities were not associated with the K-BIT-IQ score. Using a model of chronic anemia, we found that cognitive functioning was associated with cerebral hemodynamics.
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