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Neuronal Degeneration and Cell Death in Prion Disease

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Prions and Brain Diseases in Animals and Humans

Part of the book series: NATO ASI Series ((NSSA,volume 295))

Abstract

The classical pathological features of spongiform encephalopathies include vacuolation of the neuropil, astrocytic gliosis and neuronal degeneration (Masters and Richardson, 1978). Neuronal degeneration is widespread in many cases of CJD and may lead to almost complete loss of nerve cells (Fig. 1A, B). Alfons Jakob described a variety of pathological changes including chromatolysis of Betz cells in the motor cortex as well as satellitosis and neuronophagia (“glial rosettes”). However, there is only limited data available on the extent and pattern of neuronal loss (Jeffrey et al., 1992; Jeffrey et al., 1995; Masters and Richardson, 1978; Hogan et al., 1981; Scott and Fraser, 1984), and even less is known about the mechanisms which underlie neuronal cell death in spongiform encephalopathies. It is not clear whether apoptotic nerve cell loss is caused by progressive loss of the function of PrPC or increasing toxicity of PrPSc in the course of the disease (“loss of function and gain of function hypotheses”).

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Authors and Affiliations

  1. Department of Neuropathology, University of Göttingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany

    Hans A. Kretzschmar, Armin Giese, Jochen W. Herms & David R. Brown

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  1. Hans A. Kretzschmar
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  2. Armin Giese
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  3. Jochen W. Herms
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  1. CH-1296, Coppet, Switzerland

    Douglas R. O. Morrison

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Kretzschmar, H.A., Giese, A., Herms, J.W., Brown, D.R. (1998). Neuronal Degeneration and Cell Death in Prion Disease. In: Morrison, D.R.O. (eds) Prions and Brain Diseases in Animals and Humans. NATO ASI Series, vol 295. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-1896-3_25

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