Investigate prevalence of radiological proven acute stroke in patients with sickle cell anemia (SCA) who develop focal neurological deficits.  

SCA is a known risk factor for developing intracranial vasculopathy and stroke. However neurological symptoms can also be attributable to stroke mimics.  

A total of 147 patients were included (82% with sickle cell disease (SCD) and 18% with sickle cell trait). Mean age±SD was 43±17 years. Prevalent medical histories were strokes (71%), pain crisis (37%) and acute chest syndrome (31%). Outpatient treatment consisted of hydroxyurea (33%) and exchange transfusion (28%). Most common reason for neurological consultation was presence of a focal deficit-35% of cases (one or more symptoms of ataxia, blurry vision, unilateral weakness, sensory loss, or acute onset of dizziness). Mean±SD of HgB S was 43±28 at time of event. MRI was performed in 123 (84%) patients and showed acute ischemia in 36 (29%) patients. Vascular imaging showed Moya-Moya vasculopathy (12%) and intracranial stenosis causing ≥50% narrowing in 15%. Most common neurological symptoms in patients with acute brain ischemia were focal weakness (42%), confusion (22%) and symptoms recurrence due to history of stroke (19%). The presence of acute brain ischemia changed management for 33% of patients who received exchange transfusion during hospital stay. Most common discharge diagnoses were vaso-occlusive crisis, acute stroke and others (acute chest syndrome, VTE, seizures). Most patients with acute stroke had good outcomes, being discharged either to home (56%) or acute rehab (33%).

SCA patients presenting with acute neurological deficits have a high prevalence of stroke mimics, with neurological symptoms related to vaso-occlusive crisis being most common. Outcome is overall good for patients with SCD and acute ischemia.