Final answer:
Creutzfeldt-Jakob disease (CJD) is the most common prion disease in humans, which manifests as a rapidly progressive neurodegenerative disorder with no known cure.
Step-by-step explanation:
Among the neurodegenerative diseases caused by prions, the most common prion disease is Creutzfeldt-Jakob disease (CJD). Transmissible Spongiform Encephalopathies, caused by misfolded prion proteins (PrPsc), include fatal neurodegenerative diseases like kuru and Bovine Spongiform Encephalopathy (BSE), also known as Mad Cow Disease. However, CJD is the one that's most frequently encountered in humans. It leads to rapidly progressive dementia and neurological decline, characterized by spongy degeneration of the brain as depicted in Figure 6.26.
Prions are transmissible and encourage the misfolding of normal cellular proteins. This abnormal folding is associated with the brain damage and sponge-like lesions shown in the brain scans of CJD patients. There is currently no cure for this condition, and it ultimately leads to death.