Perfusion magnetic resonance abnormalities in patients with sickle cell disease

Neurological complications are common in sickle cell disease (SCD). However , it is often difficult to relate the clinical presentation to conventional neuroimaging, because subclinical infarction is common and stroke has been described in the absence of large-vessel disease. We studied 48 patients w ith SCD aged 4-34 (median 13) years with T2-weighted, diffusion and perfusi on magnetic resonance imaging (MRI) and with MR angiography. Forty-four und erwent transcranial Doppler (TCD). Abnormalities on perfusion imaging were seen in 25 cases, 24 of whom had been symptomatic. The remaining patient ha d evidence of executive dysfunction and reduced perfusion in the frontal lo bes. The perfusion abnormality was larger than the area of infarction in 9 patients and was seen in an arterial distribution with no infarction in a f urther 9. In 3 patients with transient ischemic attacks, perfusion abnormal ities were demonstrated in the absence of any other neuroimaging abnormalit ies, and perfusion changes were seen in 3 others despite normal MR angiogra phy and TCD. Perfusion abnormalities are associated with neurological sympt oms in patients with SCD, whether or not MRT, MR angiography, and TCD are a bnormal. It is likely that this technique will guide management in individu al patients.