Chapter 91. Prion Diseases

  1. Robert P. Lisak MD, FAAN, FRCP Parker Webber Chair Professor Chair Neurologist-in-Chief Chief3,4,
  2. Daniel D. Truong MD, FAAN Head5,
  3. William M. Carroll MBBS, MD, FRACP, FRCP(E) Head6 and
  4. Roongroj Bhidayasiri MD, FRCP Director7,8
  1. Ellen Gelpi MD Researcher1 and
  2. Herbert Budka MD, MSc, Dhc Professor Director2

Published Online: 18 MAY 2010

DOI: 10.1002/9781444317008.ch91

International Neurology: A Clinical Approach

International Neurology: A Clinical Approach

How to Cite

Gelpi, E. and Budka, H. (2009) Prion Diseases, in International Neurology: A Clinical Approach (eds R. P. Lisak, D. D. Truong, W. M. Carroll and R. Bhidayasiri), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444317008.ch91

Editor Information

  1. 3

    Wayne State University School of Medicine, Detroit, MI, USA

  2. 4

    Detroit Medical Center, Harper University Hospital, Detroit, MI, USA

  3. 5

    The Parkinson and Movement Disorder Institute, Memorial Neuroscience Institute, Orange Coast Memorial Medical Center, Fountain Valley, CA, USA

  4. 6

    Sir Charles Gairdner Hospital, Nedlands, Perth, Australia

  5. 7

    Chulalongkorn Comprehensive Movement Disorders Center, Chulalongkorn University Hospital, Bangkok, Thailand

  6. 8

    University of California at Los Angeles, School of Medicine, Los Angeles, CA, USA

Author Information

  1. 1

    Institute of Neurology, Medical University of Vienna, Austrian Reference Centre for Human Prion Diseases, Vienna, Austria

  2. 2

    Institute of Neurology (Obersteiner Institute), Medical University of Vienna, Vienna, Austria

Publication History

  1. Published Online: 18 MAY 2010
  2. Published Print: 11 SEP 2009

ISBN Information

Print ISBN: 9781405157384

Online ISBN: 9781444317008



  • prion diseases;
  • transmissible spongiform encephalopathies (TSE) or prion diseases - irreversible and fatal degeneration of CNS;
  • sporadic Creutzfeldt–Jakob disease (sCJD);
  • Gerstmann–Sträussler–Scheinker disease (GSS);
  • iatrogenic CJD (iCJD) - inadvertent transmission by invasive medical procedures;
  • Kuru, recognized in late 1950s - Fore tribe in Papua New Guinea;
  • Creutzfeldt–Jakob disease, rare disease with worldwide incidence about 1–2 cases;
  • name “prion,” created to designate a “proteinaceous infectious particle”;
  • vCJD differs from classical sCJD - psychiatric and sensory symptoms are most frequent initial clinical features


This chapter contains sections titled:

  • Introduction

  • Epidemiology

  • Pathophysiology

  • Clinical features

  • Investigations

  • Therapeutic approaches to prion diseases

  • Preventive measures

  • Further reading