Prion Diseases

Pathophysiology
Prion diseases or transmissible spongiform encephalopathies  (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.

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 60,424,213², total 66 extrapolated incidences are recorded in Norway.

reatment
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.