Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE)
in cattle are classified as transmissible spongiform encephalopathies (TSEs) or prion
diseases. TSEs are rapidly progressive, invariably fatal, neurodegenerative diseases
that occur in humans and many animal species. Most TSEs are characterized by a long
incubation period, and a neuropathologic feature of spongiosis, gliosis, neuronal
loss, and absence of inflammatory reaction. CJD is the most common form of TSE in
humans and occurs in many countries of the world, with an annual incidence of about
1 case per million population. Other human TSEs endemic in many countries include
Gerstmann–Sträussler–Scheinker syndrome and fatal familial insomnia, diseases that
are primarily associated with specific prion protein gene mutations and an autosomal
dominant inheritance pattern [
[1]
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© 2002 Elsevier Science (USA). Published by Elsevier Inc. All rights reserved.
