Reversible posterior leukoencephalopathy syndrome in sickle-cell anemia

Abstract

A 10-year-old African American girl with sickle-cell anemia developed headaches and seizures associated with hypertension during hospitalization for a pulmonary abscess. Hypertension developed after multiple transfusions, associated with abnormally high hematocrit and headache. Magnetic resonance imaging was consistent with posterior leukoencephalopathy. Neurologic signs, hypertension, and high hematocrit resolved after erythrocytapheresis. Magnetic resonance imaging, 1 month after the episode, produced normal results. Because reversible posterior leukoencephalopathy syndrome was only described in sickle-cell anemia during severe acute chest syndrome, this report documents that milder illness can be associated with reversible posterior leukoencephalopathy syndrome in sickle-cell anemia, and also highlights subtle signs that may herald serious neurologic events in high-risk patients. Examination of the pathophysiology of reversible posterior leukoencephalopathy syndrome in the context of sickle-cell anemia suggests that patients with sickle-cell anemia and subtle neurologic signs should be treated with high vigilance.

Figure 1

Reversible Posterior Leukoencephalopathy Syndrome (RPLS). T2 and fluid attenuated inversion recovery (FLAIR) hyperintensities in the grey and white matter of the posterior cortex in this patient were transient and were absent on imaging three weeks after hospital discharge. Axial FLAIR images demonstrate occipital (A) and parietal (B) hyperintensities better than T2 imaging of the same area (E and F, respectively). The hyperintensities are also present on the coronal FLAIR image (C). A magnetic resonance angiogram (D) demonstrated no abnormalities.