Overview of Prion Diseases

Authors: Garland E. Pendergraph, PhD, JD, MLS(ASCP)SM, HCLD/CC(ABB); Kwi Simmons, MLS(ASCP)
Reviewer: Judi Bennett, MT, BSM
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Continuing Education Credits

P.A.C.E.® Contact Hours: 1 hour(s)

Florida Board of Clinical Laboratory Science CE Credit Hours

Florida Board of Clinical Laboratory Science CE - General (Microbiology/Mycology/Parasitology): 1 hour(s)

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This course includes an overview of the function of prion proteins and the differing theories as to how prions cause several different neurodegenerative diseases. The causes and symptoms of the four types of infectious and classical prion diseases will be explored, as well as the recommendations for safe handling of blood and body fluid specimens from individuals infected with prion diseases.

Objectives

  • Differentiate between prion proteins and prions.
  • Discuss the function of prion proteins and explain how prions effectuate several neurodegenerative diseases.
  • List several theories advocating causes for prion infections.
  • Discuss the causes and symptoms of the four types of infectious prion diseases and four types of classical prion diseases.
  • State the World Health Organization’s clinical laboratory recommendations for the handling of blood and body fluid specimens from individuals infected with prion diseases.
  • Summarize knowledge regarding assays directed toward prion detection.

Course Outline

  • What are Prions?
    • Acronyms Used in this Course
    • Prion Proteins
    • Prions
    • Transmissible Spongiform Encephalopathies
  • Theories Regarding Prion Infection
    • Research
    • Theories
    • Hypothesis
    • Multi-component Hypothesis
  • Symptoms and Histopathology of Prion Disease
    • How do prions kill?
    • Hypotheses and Theories on the Killing Mechanisms of Prions
    • Amyloid Fibers and Glial Cells
    • Signs and Symptoms of Prion Disease
    • Summary of Both Human and Non-human Prion Diseases
    • Summary of Both Human and Non-human Prion Disease Forms: Major Neuropathological Features and Etiology
  • Infectious Prion Diseases
    • Scrapie
    • Clinical Presentation: Scrapie
    • Bovine Spongiform Encephalopathy/Mad Cow Disease
    • Bovine Spongiform Encephalopathy/Mad Cow Disease: Statistics
    • Chronic Wasting Disease
    • Variant Creutzfeldt-Jakob Disease: Clinical Presentation and Relationship to Bovine Spongiform Encephalopathy
    • Variant Creutzfeldt-Jakob Disease: Statistics and Prevention
    • Variant Creutzfeldt-Jakob Disease: Safeguards
  • Other Human Prion Diseases
    • Creutzfeldt-Jakob Disease
    • Creutzfeldt-Jakob Disease: Sporadic versus Iatrogenic
    • Fatal Familial Insomnia
    • Fatal Familial Insomnia: Clinical Presentation
    • Kuru
    • Clinical Presentation: Kuru
    • Gerstmann-Sträussler-Scheinker Disease
    • Prion Diseases and Prion Concentration
  • Laboratory Safety
    • Safety Precautions
    • Infection Control Strategies
    • Autopsy in Suspected or Known Cases of Prion Disease
  • Diagnosis
    • Diagnosis
    • Final Thoughts
  • References
    • References

Additional Information

Level of Instruction: Intermediate
Intended Audience: Medical laboratory scientists, medical laboratory technicians, histotechnologists, and histotechnicians working in the clinical or pathology laboratory. This course is also appropriate for medical laboratory science students, histology students, and pathology residents.
Author Information: 
Garland E. Pendergraph, PhD, JD, MLS(ASCP)SM, HCLD/CC(ABB) received his MSPH from the University of Kentucky in Lexington, his PhD in medical parasitology/entomology and mycology from the University of North Carolina in Chapel Hill, and his law degree with a concentration in health care law from Concord Law School, Purdue University. He also did a Fellowship in Tropical Medicine at Louisiana State University School of Medicine. He is the author of a textbook on phlebotomy, a number of scientific articles, plus internet training programs. He is the director of five laboratories.
Kwi Simmons is employed as a Clinical Coordinator at Piedmont Macon Hospital. She is certified as a MLS (ASCP) receiving her BS degree in medical technology from Thomas University and her Master of Science degree in clinical molecular genetics from Northern Michigan University.
Reviewer Information: Judi Bennett is a program director for MediaLab, Inc. During her 30 year career as a medical technologist, she has served as a senior system analyst, laboratory manager, Point-of-Care coordinator, microbiology supervisor, and generalist. Judi has been a speaker at various LIS, AMT, and CLMA conferences and has been published in CLMA magazine.
Course Description: This course includes an overview of the function of prion proteins and the differing theories as to how prions cause several different neurodegenerative diseases. The causes and symptoms of the four types of infectious and classical prion diseases will be explored, as well as the recommendations for safe handling of blood and body fluid specimens from individuals infected with prion diseases.