Overt and incomplete (silent) cerebral infarction in sickle cell anemia: diagnosis and management

Regional complete infarctions in children with sickle cell anemia (HbSS) are often associated with stenosis of the large intracranial arteries and result in lifetime disability. Incomplete infarction occurs more frequently than previously recognized and has far-reaching effects on neurocognitive development and the risk for overt secondary strokes into adulthood. Clinical and neuroimaging modalities have been highlighted in an algorithmic approach, with the studies giving the highest yield in results and most likely to be available listed in sequential order. The recognition of an emerging "second peak" incidence in the third decade of life is worrisome and warrants more intense scrutiny and diagnosis of subtle findings of stroke in this young adult population.