Episode 64. Metamorphosis – CJD and Other Brain Prion Shapeshifters

The focus of this podcast is the enigmatic theme of prion disorders. I trace the history of our understanding of this neurodegenerative disorder with how Nobel laureate D. Carleton Gajdusek, described the transmission of kuru - the first pathologically defined human prion disease.

I also narrate how the similarities in the clinical and pathological features of kuru and scrapie, the disease of sheep, opened the way to this breakthrough. I also chronicled how the efforts of another Nobel laureate, Stanley Prusiner, characterised the pathology, genetics and transmission of scrapie, and how he succeeded in a bitter scientific race that transformed the field of transmissible spongiform encephalopathies which he named prion diseases.

I also explored the pathology of prions, their normal functions, and how they cause disease when they become misfolded. In this theme, I discussed Creutzfeldt Jakob disease extensively, with reference to the roles played by Alfons Jakob and Hans Gerherdt Creutzfeldt, and depicting its classical manifestations and investigations. I illustrated the lived experience of CJD with such memoirs as Howie V CJD, by Sandy Bosman. I also exhaustively covered the clinical and management aspects of new variant CJD, and I illustrated this with the memoir Who Killed My Son, by Christine Lord.

The podcast also reviewed the other established human prion diseases such as fatal familial insomnia, and it explored the similarities between prion diseases and other neurodegenerative diseases that are also related to misfolded proteins, such as Alzheimer’s, Parkinson’s, and motor neurone diseases. I also covered mad cow and other prion disorders of animals. I cited other helpful books such as Collectors of Lost Souls by Warwick Anderson, Fatal Flaws by Jay Ingram, Madness and Memory by Stanley Prusiner, and Consciousness: A User’s Guide by Adam Zeman.