Kuru and the tranmissible virus dementias have been classified in a group of virus-induced slow infections that we have described as subacute spongiform virus encephalopathies because of the strikingly similar histopathologic lesions they induce. Scrapie, mink encephalopathy, and the chronic wasting disease with spongiform encephalopathy of captive mule deer and captive elk all appear, from their histopathology, pathogenesis, and the similarities of their infectious agents, to belong to the same group [1–8]. The basic neurocytologic lesion in all these diseases is a progressive vacuolation in the dendritic and axonal processes and cell bodies of neurons and, to a lesser extent, in astrocytes and oligodendrocytes; an extensive astroglial hypertrophy and proliferation; and, finally, spongiform change or status spongiosus of gray matter [9–11]. These atypical infections differ from other diseases of the human brain, which have been subsequently demonstrated to be slow virus infectious in that they do not evoke a virus-associated inflammatory response in the brain (i.e., no perivascular cuffing or invasion of the brain parenchyma with leucocytes); they usually show no pleocytosis nor do they show marked rise in protein in the cerebrospinal fluid throughout the course of infection [12–14]. Furthermore, they show no evidence of an immune response to the causative virus and, unlike the situation in the other virus diseases, there are no recognizable virions in sections of the brain visualized by electron microscopy. Instead, they show ultrastructural alteration in the plasma membrane that lines the vacuoles .
KeywordsPaired Helical Filament Scrapie Agent Slow Virus Status Spongiosus Scrapie Associate Fibril
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