[Paget's disease, a slow virus disease?].
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Scrapie is an unusual slow-virus disease of sheep which is very much like kuru and Creutzfeldt-Jakob disease, both fatal, slow neuological diseases of man. In mice, scrapie usually has an incubation period of about 6 months. Intraperitoneal inoculation of virus particles into newborn mice caused no disease, and there was no detectable virus replication for 1 year, but high titers of scrapie were present in the spleen and brain at 18 months. Virus replication occurred in mice injected from 4 days after birth by all inoculation routes, wheter or not they were injected with scrapie virus on day 0. The results suggest that scrapie virus replicates peripherally only in thymocytes, which are not present in mice until a few days after birth. The latent state suggests that the comparable human diseases could appear in later life as a result of perinatal infection. In some respects these diseases resemble premature senility.
Slow virus diseases are characterized by a long asymptomatic period, often months or years in duration, between the introduction of the infectious agent and the appearance of clinical illness. Two distinct groups cause serious degenerative diseases of the brain and spinal cord. The first to be identified are those caused by "unconventional agents," kuru and Creutzfeldt-Jakob disease. The second category, "conventional virus diseases," include SSPE (subacute sclerosing panencephalitis), PML (progressive multifocal leukoencephalopathy), progressive rubella encephalitis, and HIV encephalopathy. The universal focus on acquired immune deficiency syndrome (AIDS) has stimulated new research on slow viruses. The extreme neurological deficits, the chronic nature of these diseases, and the possible concern with infection control make patients with these diseases a challenge to nursing.
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Scrapie-associated fibrils, first observed in brains of scrapie-infected mice, were also observed in scrapie-infected hamsters and monkeys, in humans with Creutzfeldt-Jakob disease, and in kuru-infected monkeys. These fibrils were not found in a comprehensive series of control brains from humans and animals affected with central nervous system disorders resulting in histopathologies, ultrastructural features, or disease symptoms similar to those of scrapie, kuru, and Creutzfeldt-Jakob disease. These fibrils are also found in preclinical scrapie and in the spleens of scrapie-infected mice; they are a specific marker for the "unconventional" slow virus diseases, and may be the etiological agent.
Serum samples from patients with endemic Balkan nephropathy (EBN) were inoculated intraperitoneally to four species of laboratory animals. It was only in the guinea pig that a serially transmissible experimental disease occurred, after a long incubation period. The morphopathological features in the guinea pig were similar to those encountered in the terminal phase of human EBN. The hypothesis that EBN might be a slow virus disease is discussed.
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