Central-variant posterior reversible encephalopathy syndrome: brainstem or basal ganglia involvement lacking cortical or subcortical cerebral edema

Abstract

Objective: Although posterior reversible encephalopathy syndrome (PRES) typically involves cortical or subcortical edema of the cerebrum, only individual cases have been described of a variant involving the central brainstem and basal ganglia and lacking cortical and subcortical edema. We evaluated FLAIR and T2-weighted images of 124 patients with confirmed PRES to determine the incidence of this uncommon variant, which we refer to as the "central variant"; to determine which structures are involved in this variant; and to determine the associated causes.

Conclusion: We found that five of the 124 patients (4%) with PRES had MR findings consistent with the central variant-that is, either brainstem or basal ganglia involvement and a lack of cortical or subcortical edema of the cerebrum. The thalami were involved in all five PRES patients with MR findings consistent with the central variant, but there was variable involvement of the posterior limb of the internal capsule (4/5), cerebellum (3/5), and periventricular white matter (3/5); in each patient, there was improvement both clinically and on MRI. The causes of PRES in these five patients were hypertension (n=2), cyclosporine (n=2), and eclampsia (n=1). The incidence of the central variant may be increasing because of an improving awareness of the diverse imaging patterns of PRES.