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Acute stroke in sickle cell disease

Alex George, MD, PhD
Section Editors
Donald H Mahoney, Jr, MD
Douglas R Nordli, Jr, MD
Deputy Editors
Jennifer S Tirnauer, MD
John F Dashe, MD, PhD


Stroke is a common and potentially devastating manifestation of sickle cell disease (SCD) that can affect children and adults. Challenges in management include distinguishing acute stroke from other cerebrovascular manifestations of the disease such as meningitis, cerebral malaria, or seizure disorder, and distinguishing acute ischemic stroke from hemorrhagic stroke. Care of the patient with an acute stroke requires specialized expertise in exchange transfusion practices.

This topic discusses an approach to the acute assessment and treatment of stroke in children and adults with SCD.

Risk stratification and primary stroke prevention, as well as secondary prevention for individuals with SCD who have had an acute stroke or transient ischemic attack (TIA) are presented in detail separately. (See "Prevention of stroke (initial or recurrent) in sickle cell disease".)


Individuals with SCD are at risk of ischemic and hemorrhagic stroke, especially those with HbSS or HbS-beta0 thalassemia. It has been estimated that without intervention, 11 percent of patients with SCD will have a clinically apparent stroke by age 20 years, and one-fourth of patients with SCD will have a stroke by age 45. Stroke risk factors and epidemiology in patients with SCD are presented separately. (See "Prevention of stroke (initial or recurrent) in sickle cell disease".)

The diagnosis of suspected stroke involves a rapid initial clinical assessment accompanied by neuroimaging to differentiate ischemia from hemorrhage and to exclude stroke mimics.

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Literature review current through: Nov 2017. | This topic last updated: Mar 29, 2017.
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