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19 - Prion diseases
from Part II - Neurological diseases
- By Esther A. Croes, Cornelia M. van Duijn
Albert Hofman, Erasmus Universiteit Rotterdam, Richard Mayeux, Columbia University, New York
Book:

Investigating Neurological Disease

Published online:

29 September 2009

Print publication:

30 August 2001, pp 259-269
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Summary

This chapter discusses the etiology, diagnosis, occurrence, and prognosis of human prion disorders. It emphasizes on the Creutzfeldt-Jakob disease (CJD), the most common and complex prion disorder in humans. Prion diseases may be transmitted from man to man. The classical example is kuru, in which the disease has been transmitted through cannibalistic rituals. New developments may be anticipated with regard to the use of magnetic resonance imaging (MRI) in the diagnosis of CJD. For all prion disorders, DNA diagnostics may be used to support the diagnosis. Several dominant mutations in the prion protein (PRNP) gene are known to lead to CJD. The most common prion disease, CJD, is reported worldwide with a mortality of around one death per million persons. Within months after diagnosis of CJD, there is a progressive deterioration of the clinical condition to akinetic mutism.