Creutzfeldt-Jakob disease (CJD) in a case of suspected chronic heavy metal poisoning.

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