ABSTRACT
The prion diseases constitute an unusual group of neurodegenerative disorders. Although
they are similar in many ways to other more common diseases, such as Alzheimer disease
and amyotrophic lateral sclerosis, they are set apart on the basis of their transmissible
nature. In addition to the unique feature of transmissibility, the prion diseases
demonstrate that the expression of diverse disease phenotypes is possible from a common
etiologic factor. This review provides the reader with a basic understanding of the
nature of prions and highlights the clinical and pathologic features of these fascinating
diseases.
KEYWORD
Prions - Creutzfeldt-Jakob disease - Gerstmann-Sträussler-Scheinker disease