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

    Volume 46, Issue 3 (May 2001)

    ISSN: 0022-1198

    CODEN: JFSOAD

    Page Count: 6


    Meissner, C
    Medical University of Lübeck, Lübeck,

    Kretzschmar, H
    University of Göttingen,

    Gerling, I
    Medical University of Lübeck, Lübeck,

    Theuerkauf, I
    University of Bonn,

    Oehmichen, M
    Medical University of Lübeck, Lübeck,

    Windl, O
    University of Göttingen,

    Schulz-Schaeffer, W
    University of Göttingen,

    (Received 10 April 2000; accepted 12 June 2000)

    Abstract

    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.


    Paper ID: JFS15026J

    DOI: 10.1520/JFS15026J

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    Title Creutzfeldt-Jakob Disease (CJD) in a Case of Suspected Chronic Heavy Metal Poisoning
    Symposium , 0000-00-00
    Committee E30