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Human Prion Diseases - 1st Edition - ISBN: 9780444639455, 9780444639530

Human Prion Diseases, Volume 153

1st Edition

Series Volume Editors: Maurizio Pocchiari Jean Manson
eBook ISBN: 9780444639530
Hardcover ISBN: 9780444639455
Imprint: Elsevier
Published Date: 7th June 2018
Page Count: 512
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Table of Contents

1. Historical view

Section I: Pathophysiology of prions
2. The cellular and pathological prion protein
3. Cell biology of prion infection
4. Experimental models of human prion diseases and prion strains
5. The role of the immune system in prion infection

Section II: Animal prion diseases (clinical, epidemiology, neuropathological, biochemical, biomarker, and genotypes)
6. Typical and atypical scrapie
7. Typical and atypical BSE
8. Chronic Wasting Disease

Section III: Human prion diseases (clinical, epidemiology, neuropathological, biochemical, biomarker, and genotypes)
9. Sporadic Creutzfeldt-Jakob Disease
10. Variably protease-sensitive prionopathy
11. Variant Creutzfeldt-Jakob Disease
12. Iatrogenic Creutzfeldt-Jakob Disease
13. Genetic Creutzfeldt-Jakob Disease
14. Gerstmann-Sträussler-Scheinker disease
15. Sporadic and fatal Familial Insomnia

Section IV: Prion-like mechanisms in other neurodegenerative diseases
16. Prion-like mechanisms in Alzheimer
17. Prion-like mechanisms in Parkinson
18. Prion-like mechanisms in ALS

Section V: Diagnosis and treatment
19. Prion protein amplification techniques
20. Differential diagnosis with other rapid progressive dementias
21. Symptomatic treatment, care and support of CJD patients
22. Identifying therapeutic targets and treatment in model systems
23. Vaccination strategies
24. Clinical trials

Section VI: Public health issues
25. Animal diseases and the zoonotic potential
26. Safety of blood, blood derivatives and plasma-derived products
27. Safety in clinical practice
28. Concluding thoughts


Human Prion Diseases, Volume 153 is designed to update the reader on the latest advances and clinical aspects of prion diseases. The book is organized into five sections, including the pathophysiology of prions and a description of animal and human diseases. This is followed by detailed reports on recent advances in diagnosis strategies for the development of novel anti-prion molecules and possible designs of clinical trials in such a rare disease. An introductory chapter gives an extensive historical background of prion research, with a final chapter highlighting recent progress, and more importantly, unsolved problems.

Key Features

  • Offers an authoritative overview of prion diseases in humans, detailing the pathogenesis of the disease, clinical investigations, and the diagnosis of both the genetic and acquired forms
  • Provides clarity and context by presenting prion diseases in relation to other neurodegenerative diseases in humans
  • Emphasizes the unique properties of prion diseases and consequent problems they can cause, both clinically and in public health terms


Basic and clinical researchers in neuroscience; fellows, residents, and practicing clinicians in neurology, human and comparative neuropathology


No. of pages:
© Elsevier 2018
7th June 2018
eBook ISBN:
Hardcover ISBN:

Ratings and Reviews

About the Series Volume Editors

Maurizio Pocchiari

Maurizio Pocchiari

Professor Maurizio Pocchiari is a neurologist working at the Istituto Superiore di Sanità, Rome, Italy, where chairs the Department of Neuroscience. He heads a group of neurologists, geneticists, biochemists, and statisticians working on various aspects of human and experimental prion diseases. These include studies on clinical and molecular epidemiology, diagnosis, pathogenesis, experimental therapy, biochemistry and molecular and cellular biology of the prion protein and the prion protein gene, development of new techniques for the inactivation of prions, and the analysis of risk in transmitting prions from animal-to-human or from human-to-human. Professor Pocchiari runs the Italian Creutzfeldt-Jakob disease surveillance unit since 1993 and is a member of the Coordination Committee of the European Creutzfeldt-Jakob disease Surveillance Network (EuroCJD) of the European Centre for Disease Prevention and Control (ECDC).

Affiliations and Expertise

Professor and Director of Research, Department of Cellular Biology and Neuroscience, Istituto Superiore di Sanita (ISS), Rome, Italy

Jean Manson

Jean Manson

Prof. Manson is an internationally recognised leading scientist in TSE research and has developed, delivered and managed a high international scientific programme involving studies of animal and human Transmissible Spongiform Encephalopathies (TSEs). She develops and uses in vivo and in vitro model systems to define the basic mechanisms of disease and to address practical issues involving the TSEs. She heads a division of six group leaders and forty scientific staff all researching TSEs. She also runs a research group of seventeen people, including postdocs, technicians and PhD students.

The transmissible spongiform encephalopathies (TSEs) are a group of fatal neurodegenerative diseases affecting humans and animals. TSEs present with characteristic pathology which can include neuronal loss, reactive astrogliosis, deposition of disease-associated prion protein (PrP) and vacuolation in the brain. The aim of her group is to examine the role of host PrP in TSEs such as scrapie, bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob disease (CJD), the nature of the infectious and neurotoxic agent and to define routes of transmission between and within animal Basic research into neurodegenerative processes has also led into study of other diseases such as Alzheimer’s disease. They use a variety of models from the molecular to whole animal level of study. They have produced a number of unique transgenic mouse models via gene-targeting and with these models we are investigating the effects of PrP sequence, the species barrier, the influence of PrP glycosylation on disease susceptibility and also the expression of PrP in various cell-types or at specific time-points during disease. The group also investigates human to human transmission of vCJD via blood transfusion and other potential routes. Their research programme currently receives funding from a number of different sources including BBSRC, MRC, DEFRA, DoH and EU.

Affiliations and Expertise

Professor and Head, Neurobiology Division, The Roslin Institute, University of Edinburgh, UK