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Semin Neurol 2000; Volume 20(Number 03): 337-352
DOI: 10.1055/s-2000-9396
Copyright © 2000 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

The Prion Diseases

James A. Mastrianni, Raymond P. Roos
  • Department of Neurology, The University of Chicago Medical Center, Chicago, Illinois
Further Information

Publication History

Publication Date:
31 December 2000 (online)

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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

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