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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
By
Hayrettin Tumani ,
Albert C. Ludolph  and
Adriano Aguzzi
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
Publisher: Cambridge University Press
Print publication year: 2005

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