Neuropathological findings from an autopsied case showing posterior reversible encephalopathy syndrome-like neuroradiological findings associated with premedication including tacrolimus for autologous peripheral blood stem cell transplantation

Abstract

Posterior reversible encephalopathy syndrome (PRES) is diagnosed based on neuroradiological findings. Typically, PRES is reversible and presents with a good outcome; however, fatal outcomes have been reported. We report an autopsied case showing PRES-like neuroradiological findings associated with premedication including tacrolimus for autologous peripheral blood stem cell transplantation in a 28-year-old woman with a 2-year history of acute myeloid sarcoma/acute myeloid leukemia. Neurological examination revealed disturbed consciousness, muscle weakness in all extremities, and bilaterally diminished tendon reflexes. Brain fluid attenuated inversion recovery MRI showed multiple bilateral hyper-intensity areas in the posterior white matter and left corona radiate. She died of respiratory arrest within 24h after PRES diagnosis. Neuropathological examination revealed diffuse cerebral edema, multiple cerebral hematomas that extended into the lateral ventricles and subarachnoid cavities, and multiple microbleeds predominantly in the inferior surface of the occipital white matter. Microscopic findings revealed paler myelin sheaths, enlargement of the vascular endothelium, leakage of plasma components and red blood cells, and clasmatodendrosis within the occipital white matter. Cerebral herniation and diffuse cerebral edema due to vascular endothelial dysfunction were concluded to be the cause of death. These pathological findings may aid in the pathophysiological recognition of acute-stage PRES.