Transmissible spongiform encephalopathies are a unique group of brain
diseases of different animal species and man that can be transmitted b
etween individuals by inoculation or ingestion of diseased nervous sys
tem tissues. The causative agents induce non inflammatory spongiform d
egeneration of the central nervous system which leads to a progressive
loss of brain function including ataxia and paralysis resulting alway
s in death. The agent might consist only of a conformationally altered
cellular protein of the host species devoid of nucleic acid. The natu
ral occurance of transmissible spongiform encephalopathies is restrict
ed to a few species. Thus, under certain conditions, i.e. the feeding
of infectious bone meal or scientific experiments, the agents can be t
ransmitted to other species causing lethal disease.