Neuropathology, Immunohistochemistry, and Biochemistry in Human Prion Diseases

  • Diane L. RitchieEmail author
  • James W. Ironside
Part of the Neuromethods book series (NM, volume 129)


Human prion diseases are rare fatal neurodegenerative conditions that occur as sporadic, inherited, and acquired disorders. All are defined by characteristic neuropathological changes and by the accumulation of a disease-associated form of the prion protein in the brain. In variant CJD, prion protein also accumulates in lymphoid tissues and in the peripheral nervous system. Examination of the brain in human prion diseases is essential for a definite diagnosis and for disease surveillance.

In this chapter we bring over 20 years’ experience of research and diagnosis in human prion diseases to provide detailed information on how to handle brain tissue from a case of human prion disease safely in a laboratory. We also describe how to make the diagnosis of a human prion disease using histological and biochemical techniques for the detection of disease-associated prion protein in the brain, and provide helpful notes for practical guidance and troubleshooting in the laboratory.

Key words

Neuropathology Brain CJD prion protein Immunohistochemistry Western blot PET blot 


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Copyright information

© Springer Science+Business Media LLC 2017

Authors and Affiliations

  1. 1.National CJD Research & Surveillance UnitCentre for Clinical Brain Sciences, School of Clinical Sciences, University of Edinburgh, Western General HospitalEdinburghUK

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