Acute Silent Cerebral Ischemic Events in Children with Sickle Cell Anemia ASCIEs in Sickle Cell Anemia

Quinn CT, McKinstry RC, Dowling MM, et al. — Mon, 29 Oct 2012 00:00:00 GMT

Background

Irregular, sporadic episodes of ischemic brain injury are known to occur in sickle cell anemia (SCA), resulting in overt stroke and silent cerebral infarction. Ongoing ischemia in other organs is common in SCA but has never been documented in the brain.

Objective

To test the hypothesis that acute silent cerebral ischemic events (ASCIEs) are frequent and potentially transient.

Design

Cross-sectional and cohort study of children with SCA screened by magnetic resonance imaging (MRI) of the brain for a randomized clinical trial.

Setting

Clinical trial setting in tertiary care centers.

Patients

Asymptomatic children with SCA without known stroke, neurologic injury, or epilepsy not receiving treatment with transfusions or hydroxyurea.

Main Outcome Measure

Incidence of ASCIEs calculated using single diffusion-weighted MRI scans (acute ischemic events that occurred within 10 days of the MRI).

Results

Acute silent cerebral ischemic events were detected on 1.3% of MRIs (10 of 771) in 652 children (mean age, 10.0 years), with an incidence of 47.3 events per 100 patient-years (95% CI, 22.7-87.2). Two of 10 children with ASCIEs had follow-up MRIs of the brain; only 1 had silent cerebral infarction in the same location as the previously detected ASCIE.

Conclusions

Children with SCA experience ongoing (chronic, intermittent) cerebral ischemia, sometimes reversible, far more frequently than previously recognized. The brain in SCA is at constant threat of ischemia.

AMA Publishing Group: Sickle Cell Disease/Thalassemias Topic Collection

Acute Silent Cerebral Ischemic Events in Children with Sickle Cell Anemia ASCIEs in Sickle Cell Anemia