Latrogenic Creutzfeldt-Jakob disease with florid plaques

Brain Pathol. 2003 Jul;13(3):245-9. doi: 10.1111/j.1750-3639.2003.tb00025.x.

Abstract

Florid plaques indistinguishable from those found in vCJD were identified at a postmortem examination in the brain of a 58-year-old clinical suspect case of Creutzfeldt-Jakob disease (CJD). Western blotting of brain tissue revealed an unusual prion protein type. Since the patient had received a dura mater graft 20 years prior to death and florid plaques are not only found in new variant CJD, the findings argue in favor of an iatrogenic origin of the disease with the longest incubation time following a dura mater graft reported to date even though he may have been exposed to BSE. The peculiar pathological, clinical and biochemical features may define a new type of human prion disease.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Aged
  • Amyloid / genetics
  • Amyloid / metabolism
  • Blotting, Western / methods
  • Cerebral Cortex / drug effects
  • Cerebral Cortex / pathology*
  • Creutzfeldt-Jakob Syndrome / complications*
  • Creutzfeldt-Jakob Syndrome / genetics
  • Creutzfeldt-Jakob Syndrome / pathology
  • Creutzfeldt-Jakob Syndrome / surgery
  • Dura Mater / transplantation
  • Endopeptidase K / pharmacology
  • Female
  • Humans
  • Lectins, C-Type / metabolism
  • Male
  • Middle Aged
  • PrPSc Proteins / metabolism
  • Prion Proteins
  • Prions
  • Protein Precursors / genetics
  • Protein Precursors / metabolism
  • Receptors, Cell Surface / metabolism

Substances

  • Amyloid
  • Lectins, C-Type
  • PRNP protein, human
  • PrPSc Proteins
  • Prion Proteins
  • Prions
  • Protein Precursors
  • Receptors, Cell Surface
  • glycoform specific receptor, human
  • Endopeptidase K