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Patients with sickle cell anemia (SCA) are at risk of substantial neurologic morbidity, including overt stroke, silent cerebral infarction (SCI), and cognitive impairment.1 Monthly transfusions reduce overt stroke risk in children deemed at risk by the presence of SCI2 or transcranial Doppler abnormalities but can lead to burdensome complications, including alloimmunization and iron overload, and may not improve intelligence quotient.2 Abnormal transcranial Doppler and SCI on MRI are sensitive but not specific screening techniques for stroke risk, and the number needed to prophylactically transfuse to prevent 1 stroke is as high as 13.3 Many families feel that the risk-to-benefit ratio is high, and physicians would prefer more precise information to counsel their individual patients. In asymptomatic patients, the addition of magnetic resonance angiography may improve stroke prediction, but if MRI is to be undertaken in a young child, it should be optimized to obtain maximum prognostic information.
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- © 2018 American Academy of Neurology
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