Prion diseases

doi:10.1017/CBO9780511544873.037

36 - Prion diseases

from Part VI - Other Dementias

Published online by Cambridge University Press: 04 August 2010

M. Flint Beal ,

Anthony E. Lang and

Albert C. Ludolph

Hayrettin Tumani ,

Albert C. Ludolph and

Adriano Aguzzi

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M. Flint Beal: Affiliation:
Cornell University, New York
Anthony E. Lang: Affiliation:
University of Toronto
Albert C. Ludolph: Affiliation:
Universität Ulm, Germany
Hayrettin Tumani: Affiliation:
Department of Neurology, University of Ulm, Germany
Albert C. Ludolph: Affiliation:
Department of Neurology, University of Ulm, Germany
Adriano Aguzzi: Affiliation:
Institute of Neuropathology, University of Zürich, Switzerland

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Summary

Introduction

Prion diseases represent a disease complex that is clinically rare. However, they are epidemiologically important severe diseases, and very likely to be important for our basic understanding of neurodegeneration in principle. This is reflected in this introductory clinical overview, which presents the clinical diagnostic and differential diagnostic approach to the patient; then a review follows which focuses on the current status of the field of basic sciences of prion diseases.

The history of prion diseases started in the year 1920 when Hans Gerhard Creutzfeldt described a mysterious focal disease of the central nervous system of a 22-year-old female patient, which clinically was characterized by progressive psychomotor disturbances and cortical symptoms (Creutzfeldt, 1920). In his autopsy he observed a pronounced gliosis which accompanied non-inflammatory focal lesions of the cortex. One year later, Alfons Jakob saw three similar cases which he also considered a new entity – he described a spastic pseudosclerosis and encephalomyelitis with disseminated focal degeneration (Jakob, 1921). Based on the similarity of the reported cases, Walter Spielmeyer suggested the name Creutzfeldt–Jakob disease (CJD). In 1936, the Gerstmann–Sträussler–Schenker (GSS) syndrome was first described and in 1957 fatal familial insomnia (FFI) (Gerstmann & Sträussler 1936; Lugaresi et al., 1986). In the 1960s, interest in the epidemiology of prion diseases increased when Gajdusek and Gibbs showed that Kuru – a disease first observed by the German Walter Zigas and Carleton Gajdusek among the Fore in Papua New Guinea and thought to be associated with cannibalism – could be transmitted to non-human primates and had similarities with the spongiform encephalopathy of sheep-scrapie (Zigas & Gajdusek, 1957; Hadlow, 1959; Gajdusek & Gibbs, 1966).

Type: Chapter
Information: Neurodegenerative Diseases
Neurobiology, Pathogenesis and Therapeutics
, pp. 512 - 548

DOI: https://doi.org/10.1017/CBO9780511544873.037 [Opens in a new window]

Publisher: Cambridge University Press

Print publication year: 2005

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