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With no known cures and a median survival time of merely 13.5 months, prion diseases are becoming a rising topic of discussion. These pathogenic agents, affecting humans and animals alike, have been found to be transmissible, untreatable and always fatal.
Prion diseases are a group of progressive neurodegenerative disorders (disorders that affect the nervous system, which comprise of the brain and spinal cord) and are believed to be caused by prions. These prions are found to cause abnormal folding of specific normal proteins, called prion proteins, that are found in our brain. Although the functions of prion proteins in our brain remain unclear, this abnormal folding of the proteins leads to brain damage, which usually progresses rapidly and is always fatal.
This build up of folded protein in the brain presents itself through various harmful and serious symptoms such as memory impairment, personality changes, difficulties with movement and speech, rapidly developing dementia or in the form of mere confusion, fatigue and even muscle stiffness.
Although the presence of prion diseases can only be definitively confirmed by taking a sample of brain tissue during a biopsy or after death, healthcare professionals can perform a number of tests, such as MRI scans of the brain or blood tests, to help diagnose some specific types of prion diseases or to rule out other diseases which present themselves through similar symptoms.
These diseases are very rare but some risk factors include genetics and age (as some types of prion diseases are found more frequently in older adults). The consumption of contaminated animal products and usage of contaminated medical equipment during procedures can also lead to the transmittance of these diseases.
There is currently no cure for prion diseases and in order to develop potential treatments, researchers continue to work on understanding more about these life threatening diseases. In the meantime, care provided for individuals affected by this disease continues to focus solely on easing their symptoms.
By Elizza Miriam Mathew
CDC. (2021, November 17). Identified Prion Diseases. CDC. Retrieved July 27, 2023, from https://www.cdc.gov/prions/index.html
Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017. (2020, October 14). NCBI. Retrieved July 27, 2023, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7569055/
Prion Diseases. (2020, October 14). Johns Hopkins Medicine. Retrieved July 27, 2023, from https://www.hopkinsmedicine.org/health/conditions-and-diseases/prion-diseases
Prion Diseases | NIH: National Institute of Allergy and Infectious Diseases. (2019, October 21). National Institute of Allergy and Infectious Diseases (NIAID). Retrieved July 27, 2023, from https://www.niaid.nih.gov/diseases-conditions/prion-diseases
Seladi-Schulman, J. (2023, April 10). Prion Disease: Symptoms, Causes, Treatment, & Prevention. Healthline. Retrieved July 27, 2023, from https://www.healthline.com/health/prion-disease
(Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study From 1998 to 2017, 2020)
CDC. (2021, November 17). Identified Prion Diseases. CDC. Retrieved July 27, 2023, from https://www.cdc.gov/prions/index.html
Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017. (2020, October 14). NCBI. Retrieved July 27, 2023, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7569055/
Prion Diseases. (2020, October 14). Johns Hopkins Medicine. Retrieved July 27, 2023, from https://www.hopkinsmedicine.org/health/conditions-and-diseases/prion-diseases
Prion Diseases | NIH: National Institute of Allergy and Infectious Diseases. (2019, October 21). National Institute of Allergy and Infectious Diseases (NIAID). Retrieved July 27, 2023, from https://www.niaid.nih.gov/diseases-conditions/prion-diseases
Seladi-Schulman, J. (2023, April 10). Prion Disease: Symptoms, Causes, Treatment, & Prevention. Healthline. Retrieved July 27, 2023, from https://www.healthline.com/health/prion-disease
(Prion Diseases, 2020)
CDC. (2021, November 17). Identified Prion Diseases. CDC. Retrieved July 27, 2023, from https://www.cdc.gov/prions/index.html
Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017. (2020, October 14). NCBI. Retrieved July 27, 2023, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7569055/
Prion Diseases. (2020, October 14). Johns Hopkins Medicine. Retrieved July 27, 2023, from https://www.hopkinsmedicine.org/health/conditions-and-diseases/prion-diseases
Prion Diseases | NIH: National Institute of Allergy and Infectious Diseases. (2019, October 21). National Institute of Allergy and Infectious Diseases (NIAID). Retrieved July 27, 2023, from https://www.niaid.nih.gov/diseases-conditions/prion-diseases
Seladi-Schulman, J. (2023, April 10). Prion Disease: Symptoms, Causes, Treatment, & Prevention. Healthline. Retrieved July 27, 2023, from https://www.healthline.com/health/prion-disease
(Prion Diseases | NIH: National Institute of Allergy and Infectious Diseases, 2019)
CDC. (2021, November 17). Identified Prion Diseases. CDC. Retrieved July 27, 2023, from https://www.cdc.gov/prions/index.html
Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017. (2020, October 14). NCBI. Retrieved July 27, 2023, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7569055/
Prion Diseases. (2020, October 14). Johns Hopkins Medicine. Retrieved July 27, 2023, from https://www.hopkinsmedicine.org/health/conditions-and-diseases/prion-diseases
Prion Diseases | NIH: National Institute of Allergy and Infectious Diseases. (2019, October 21). National Institute of Allergy and Infectious Diseases (NIAID). Retrieved July 27, 2023, from https://www.niaid.nih.gov/diseases-conditions/prion-diseases
Seladi-Schulman, J. (2023, April 10). Prion Disease: Symptoms, Causes, Treatment, & Prevention. Healthline. Retrieved July 27, 2023, from https://www.healthline.com/health/prion-disease
(Seladi-Schulman, 2023)
CDC. (2021, November 17). Identified Prion Diseases. CDC. Retrieved July 27, 2023, from https://www.cdc.gov/prions/index.html
Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017. (2020, October 14). NCBI. Retrieved July 27, 2023, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7569055/
Prion Diseases. (2020, October 14). Johns Hopkins Medicine. Retrieved July 27, 2023, from https://www.hopkinsmedicine.org/health/conditions-and-diseases/prion-diseases
Prion Diseases | NIH: National Institute of Allergy and Infectious Diseases. (2019, October 21). National Institute of Allergy and Infectious Diseases (NIAID). Retrieved July 27, 2023, from https://www.niaid.nih.gov/diseases-conditions/prion-diseases
Seladi-Schulman, J. (2023, April 10). Prion Disease: Symptoms, Causes, Treatment, & Prevention. Healthline. Retrieved July 27, 2023, from https://www.healthline.com/health/prion-disease
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