The Human Spongiform Encephalopathies: Kuru, Creutzfeldt-Jakob Disease, and the Gerstmann-Sträussler-Scheinker Syndrome

  • P. Brown
  • D. C. Gajdusek
Part of the Current Topics in Microbiology and Immunology book series (CT MICROBIOLOGY, volume 172)


Kuru and the transmissible virus dementias Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker syndrome (GSS) belong to the group of virus-induced slow infections that we have described as subacute spongiform virus encephalopathies because of the strikingly similar histopathological lesions they induce (Table 1). Scrapie, mink encephalopathy, chronic wasting disease with spongiform encephalopathy of captive mule deer and of captive elk, and bovine spongiform encephalopathy all appear from their histopathology, pathogenesis, and the similarities of their infectious agents to belong to the same group. The basic neurocytological lesions in all these diseases are a progressive vacuolation in the dendritic and axonal processes and cell bodies of neurons and, to a lesser extent, astrocytes and oligodendrocytes; an extensive astroglial hypertrophy and proliferation; and spongiform change or status spongiosis of gray matter with extensive neuronal loss.


Amyotrophic Lateral Sclerosis Bovine Spongiform Encephalopathy Transmissible Spongiform Encephalopathy Abnormal Slow Wave Activity Subacute Spongiform Encephalopathy 
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Copyright information

© Springer-Verlag Berlin · Heidelberg 1991

Authors and Affiliations

  • P. Brown
    • 1
  • D. C. Gajdusek
    • 1
  1. 1.Laboratory of CNS Studies, National Institute of Neurological Disorders and StrokeNational Institutes of HealthBethesdaUSA

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