alexa Prion Diseases | Spain | PDF | PPT| Case Reports | Symptoms | Treatment

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Prion Diseases

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  • Prion Diseases

    Pathophysiology
    Prion diseases, or transmissible spongiform encephalopathies, are fatal, neurodegenerative diseases. Central to prion pathogenesis is the conversion of a host-encoded prion protein, PrPC, into a partially- protease resistant isoform, PrPSc, which accumulates in the brain and is associated with infectivity. The ability of transmission to be effected both naturally and experimentally by disease-associated prion protein , in the absence of associated nucleic acid , led to the ‘protein only hypothesis’ of prion propagation and infectivity.

  • Prion Diseases

    Statistics
    This study describes the occurrence and epidemiology of CJD and vCJD . The following attempts to extrapolate the above incidence rate for Prion disease to the populations of 40,280,7802total 44  extrapolated incidences are recorded in Argentina.

  • Prion Diseases

    Treatment
    A number of potentially useful drugs have been used in experimental animal prion diseases or in patients in an attempt to alter the course of the disease. Quinacrine: The antimalarial compound, Pentosan polysulphate (PPS): PPS is a semi-synthetic chemical derived from shavings of beech wood, Tetracyclic Compounds, Flupirtine are commonly used drugs.

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