Journal Published Online: 01 May 2001
Volume 46, Issue 3

Creutzfeldt-Jakob Disease (CJD) in a Case of Suspected Chronic Heavy Metal Poisoning



We describe a patient who died of suspected heavy metal poisoning after a nine-month history of rapidly worsening dementia. Autopsy at a forensic-pathological institute established the postmortem diagnosis of sporadic Creutzfeldt-Jakob disease (CJD) based on demonstration of the proteinase-resistant prion protein (PrPSc) in Western-Blot on native brain tissue. Microscopic examination of the macroscopically largely inconspicuous brain revealed marked spongiform changes in the gray matter—mainly affecting the cerebral cortex, nucleus caudatus, and putamen—with confluent vacuoles. Patchy or perivacuolar deposits of PrPSc were found as well as granular PrPSc deposits. The cerebellum contained focal PrPSc deposits. There was an astrogliosis in the white matter and a proliferation of microglia in the gray matter with a simultaneous clear reduction in neuronal elements. The differential diagnosis is discussed, as is the potential risk to those performing autopsy on forensic cases with a clinical picture of rapidly progressing dementia, especially in cases where a prion disease is not initially suspected.

Author Information

Oehmichen, M
Medical University of Lübeck, Lübeck, Germany
Schulz-Schaeffer, W
University of Göttingen, Germany
Kretzschmar, H
University of Göttingen, Germany
Theuerkauf, I
University of Bonn, Germany
Gerling, I
Medical University of Lübeck, Lübeck, Germany
Windl, O
University of Göttingen, Germany
Meissner, C
Medical University of Lübeck, Lübeck, Germany
Pages: 6
Price: $25.00
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Stock #: JFS15026J
ISSN: 0022-1198
DOI: 10.1520/JFS15026J