Overview of Prion Diseases
Continuing Education Credits
This course includes an overview of the function of prion proteins and the differing theories on how prions cause several 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 infectious and classical prion diseases.
- State the recommendations for the handling of blood and body fluid specimens from individuals infected with prion diseases.
Course Outline
- What are Prions?
- Acronyms Used in this Course
- Prion Proteins
- Prions
- Transmissible Spongiform Encephalopathies
- List of Transmissible Spongiform Encephalopathies
- What is the normal biological function of prion proteins (PrPC) in healthy cells?
- Theories Regarding Prion Infection
- Research
- Theories of Prion Disease
- Hypotheses About Prion Disease
- Multi-Component Hypothesis
- Symptoms and Histopathology of Prion Disease
- How Do Prions Kill?
- Killing Mechanisms of Prions
- Amyloid Fibers and Glial Cells
- Histopathology of Prion Disease Forms
- True or False: The symptoms of prion diseases are believed to result from dense aggregates of amyloid fibers.
- True or False: Once symptoms appear, human prion diseases are always fatal.
- Infectious Prion Diseases
- Scrapie
- Clinical Presentation: Scrapie
- Bovine Spongiform Encephalopathy
- Bovine Spongiform Encephalopathy: Statistics
- Chronic Wasting Disease
- Variant Creutzfeldt-Jakob Disease
- Variant Creutzfeldt-Jakob Disease: Statistics and Prevention
- Variant Creutzfeldt-Jakob Disease: Safeguards
- True or False: Cooking meat until well-done is a very important step in preventing the transmission of mad cow disease, or bovine spongiform encephalopathy (BSE), to humans.
- Other Human Prion Diseases
- Creutzfeldt-Jakob Disease
- Creutzfeldt-Jakob Disease: Sporadic versus Iatrogenic
- Fatal Familial Insomnia
- Fatal Familial Insomnia: Clinical Presentation
- Kuru
- Kuru: Clinical Presentation
- Gerstmann-Sträussler-Scheinker Disease
- Prion Diseases and Concentration
- What is the cause of Gerstmann-Sträussler-Scheinker disease (GSS)?
- Laboratory Safety
- Safety Precautions
- Infection Control Strategies
- Autopsy in Suspected or Known Cases of Prion Disease
- True or False: The World Health Organization (WHO) recommends that all blood and body fluid specimens from patients with prion disease be considered infectious and handled with extreme caution.
- Diagnosis
- Diagnosis
- Final Thoughts
- References
- References
Additional Information
Level of Instruction: Basic
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, MS, MLS(ASCP), is employed as a Clinical Coordinator at Piedmont Macon Hospital. She is certified as an MLS (ASCP), holds a BS degree in medical technology from Thomas University, and a Master of Science degree in clinical molecular genetics from Northern Michigan University.
The authors have no
conflict of interest to disclose.
Reviewer Information:
Laurie Bjerklie, MA, MLS(ASCP)CM, is
a Lead Education Developer. She earned a B.S. in Medical Laboratory Science
from the University of North Dakota and an M.A. in Curriculum and Instruction
from Saint Xavier University. She has over 15 years of experience in higher
education and has held program director and faculty positions in both MLT and
MLS programs.
Judi Bennett, BSM, MLS(AMT), MLT(ASCP), is an Education Developer with MediaLab by Vastian, bringing over 30 years of experience as a medical laboratory scientist. Before joining MediaLab in 2010, she held key leadership roles, including Clinical Laboratory Manager, Microbiology Supervisor, and Senior Systems Analyst at Gwinnett Health System in Lawrenceville, Georgia. Her extensive knowledge of laboratory operations, combined with a strong commitment to healthcare quality and education, fuels her passion for developing engaging and effective learning tools for the next generation of laboratorians.
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.