Overwhelming leukoencephalopathy as the only sign of neuropsychiatric lupus

J Rheumatol. 2005 Sep;32(9):1843-5.

Abstract

We describe a patient with diffuse leukoencephalopathy, a rare central nervous system complication of systemic lupus erythematosus, who died of brain herniation despite aggressive management. Brain magnetic resonance imaging revealed diffuse white matter hyperintensities consistent with vasogenic edema. Autopsy revealed only widespread cerebral edema. Early recognition and persistent, aggressive treatment will be required to avoid this fatal and rare manifestation of neuropsychiatric lupus.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Adult
  • Biopsy, Needle
  • Combined Modality Therapy
  • Disease Progression
  • Fatal Outcome
  • Female
  • Humans
  • Immunohistochemistry
  • Leukoencephalopathy, Progressive Multifocal / diagnosis*
  • Leukoencephalopathy, Progressive Multifocal / etiology
  • Leukoencephalopathy, Progressive Multifocal / therapy
  • Lupus Erythematosus, Systemic / complications*
  • Lupus Erythematosus, Systemic / diagnosis
  • Lupus Vasculitis, Central Nervous System / diagnosis*
  • Lupus Vasculitis, Central Nervous System / etiology
  • Lupus Vasculitis, Central Nervous System / therapy
  • Magnetic Resonance Imaging / methods
  • Risk Assessment
  • Severity of Illness Index
  • Spinal Puncture