Sickle cell disease (SCD) is the most common cause of stroke in children, such as in this patient with acute onset of right hemiplegia. Ischemic strokes are more common than hemorrhagic strokes in patients with SCD. Although the exact mechanism of ischemic stroke in patients with SCD is unclear, likely contributing factors include vaso-occlusion due to progressive stenosis from intimal proliferation as well as abnormal red blood cell-endothelial interactions.
Given the high risk of stroke in patients with SCD, transcranial Doppler (TCD) ultrasound should be performed on all children age 2-16 with this condition. TCD ultrasound measures the mean velocity of blood flow in the large cerebral arteries, with higher velocities observed in the stenotic arteries. A high TCD velocity is associated with stroke, and these patients should undergo chronic transfusion therapy to decrease their risk.
