2-Minute Neuroscience: Prion Diseases

Prion diseases, also known as transmissible spongiform encephalopathies, are a group of neurodegenerative diseases that involve the accumulation of an abnormal form of a protein called prion protein into aggregates that are resistant to being broken down by brain enzymes. In this video, I discuss the general characteristics of prion diseases, including the pathophysiology, epidemiology, and prognosis.

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TRANSCRIPT

Prion diseases, also known as transmissible spongiform encephalopathies, are a group of neurodegenerative diseases that involve the accumulation of an abnormal form of a protein called prion protein into aggregates that are resistant to being broken down by brain enzymes.

The formation of these protein aggregates is associated with pathological changes in the nervous system, such as the formation of tiny holes in grey matter, which gives the tissue a sponge-like appearance. There is also a loss of neurons and other signs of brain pathology, which are associated with symptoms that rapidly worsen over time. The symptoms vary depending on the specific prion disease, but include severe cognitive decline and a variety of neurological problems. Prion diseases are incurable and always result in death; there is currently no treatment recognized to be effective in improving someone’s prognosis.

Unlike other infectious agents, the agent that causes prion diseases seems to consist only of an abnormal form of a protein. Hence the term prion, which is an amalgamation of “proteinaceous infectious particle.” The pathogenic form of prion protein seems to be capable of transmitting its pathogenic state to other, previously healthy, proteins. Additionally, pieces of protein aggregates sometimes break off, and the particles can form additional aggregates. In these ways, prion diseases cause the spread of pathology throughout the central nervous system.

Prion diseases are not transmitted like typical infectious diseases. There is no evidence, for example, of them being transmitted through casual contact. Only a very small percentage of cases are due to an acquired infection. Documented cases of acquired infections have occurred through consumption of contaminated meat or accidental transmission through a medical procedure. Approximately 10-15% of prion diseases are inherited and clearly traceable back to a mutation in the prion protein gene, but most cases are sporadic, without a known cause.

REFERENCES

Piñar-Morales R, Barrero-Hernández F, Aliaga-Martínez L. Human prion diseases: An overview. Med Clin (Barc). 2023 Jun 23;160(12):554-560. English, Spanish. doi: 10.1016/j.medcli.2023.03.001. Epub 2023 Apr 21. PMID: 37088611.

Zerr I, Ladogana A, Mead S, Hermann P, Forloni G, Appleby BS. Creutzfeldt-Jakob disease and other prion diseases. Nat Rev Dis Primers. 2024 Feb 29;10(1):14. doi: 10.1038/s41572-024-00497-y. PMID: 38424082.

Picture of spongiform degeneration of gray matter from Tulemo on Wikipedia: https://en.wikipedia.org/wiki/Transmi...

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