Reversible posterior leukoencephalopathy syndrome: Report of 3 autopsy cases

Abstract

Background: Reversible posterior leukoencephalopathy syndrome (RPLS) is an increasingly recognized clinicoradiological entity associated with multiple pathologies, the most common being hypertensive crisis. Because of the “reversible” nature of this syndrome, few autopsy/histological findings have been reported to date.(Case 1) A 48 y/o woman with a history of allogeneic renal transplant and hypertension presented with headache, blurred vision, confusion and died of pneumonia. (Case 2) A 48 y/o man who had a history of malignant lymphoma treated developed seizures and died of fungal pneumonia. (Case 3) A 74 y/o woman with a past medical history significant for hypertension, idiopathic thrombocytopenic purpura treated with splenectomy followed by Rituximab and steroid died of multiorgan failure. Radiologically, these 3 patients showed MR image findings characteristic of RPLS. Postmortem examination of these brains revealed leukoencephalopathic changes including diffuse white matter rarefaction with mild gliosis (Case 1) and relatively well‐circumscribed perivascular rarefied foci without gliosis (Cases 2, 3).Discussion: Our cases support the two proposed pathophysiologic mechanisms for PRLS; a breakdown in cerebrovascular autoregulation with ensuing interstitial accumulation of fluid (Cases 1), and cerebral vasospasm with resulting ischemia within the involved territories (Cases 2, 3).