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.

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 10,348,2762 total 11 extrapolated incidences are recorded in Belgium.

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.