Latrogenic Creutzfeldt-Jakob disease with florid plaques.
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.
Kretzschmar
Hans A
HA
Institut für Neuropathologie, Ludwig-Maximilians-Universität München, Germany. Hans.Kretzschmar@inp.med.uni-muenchen.de
Sethi
Shneh
S
Földvári
Zsuzsanna
Z
Windl
Otto
O
Querner
Veronika
V
Zerr
Inga
I
Poser
Sigrid
S
D000368:Aged; D000682:Amyloid / Q000235:genetics / Q000378:metabolism; D015153:Blotting, Western / Q000379:methods; D002540:Cerebral Cortex / Q000187:drug effects / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D004388:Dura Mater / Q000637:transplantation; D019286:Endopeptidase K / Q000494:pharmacology; D005260:Female; D006801:Humans; D037181:Lectins, C-Type / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D018620:PrPSc Proteins / Q000378:metabolism; D000072002:Prion Proteins; D011328:Prions; D011498:Protein Precursors / Q000235:genetics / Q000378:metabolism; D011956:Receptors, Cell Surface / Q000378:metabolism
D000682:Amyloid; D037181:Lectins, C-Type; C512656:PRNP protein, human; D018620:PrPSc Proteins; D000072002:Prion Proteins; D011328:Prions; D011498:Protein Precursors; D011956:Receptors, Cell Surface; C120053:glycoform specific receptor, human; D019286:Endopeptidase K