Neuropathology of a fatal case of posterior reversible encephalopathy syndrome

John N. Kheir, Michael W. Lawlor, Edward S. Ahn, Leslie Lehmann, James J. Riviello, V. Michelle Silvera, Michael McManus, Rebecca D. Folkerth

Research output: Contribution to journalArticle

Abstract

The pathology of posterior reversible encephalopathy syndrome (PRES) is undefined, since it is rarely fatal and is biopsied in only exceptional circumstances. We describe rapidly progressive PRES following stem cell transplant for acute lymphoblastic leukemia. After development of altered mental status, this 8-year-old girl had T2 prolongation of the white matter in a posterior-dominant distribution, eventually developing cerebellar edema, hemorrhage, hydrocephalus, and herniation. Despite surgical and medical management, she died 36 hours later. At autopsy, the occipital and cerebellar white matter and focal occipital cortical gray matter showed a spectrum of microvascular changes, including dilated perivascular spaces containing proteinaceous exudates and macrophages, as well as fibrinoid necrosis and acute hemorrhage, in a distribution corresponding to the neuroimaging abnormalities and reminiscent of those seen in patients with acute hypertensive encephalopathy. Of note, similar microvascular changes were not seen in the kidney or other systemic sites. Thus, the findings indicate a brain-specific microvascular compromise as the substrate of PRES, at least in the rare instance of cases progressing to fatal outcome.

Original languageEnglish (US)
Pages (from-to)397-403
Number of pages7
JournalPediatric and Developmental Pathology
Volume13
Issue number5
DOIs
StatePublished - Sep 1 2010

Keywords

  • Autopsy
  • Complication
  • Cyclosporine
  • Hemorrhage
  • Hypertension
  • Leukoencephalopathy
  • PRES
  • RPLS
  • Tacrolimus

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine

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  • Cite this

    Kheir, J. N., Lawlor, M. W., Ahn, E. S., Lehmann, L., Riviello, J. J., Silvera, V. M., McManus, M., & Folkerth, R. D. (2010). Neuropathology of a fatal case of posterior reversible encephalopathy syndrome. Pediatric and Developmental Pathology, 13(5), 397-403. https://doi.org/10.2350/09-04-0634-CR.1