From kuru to BSE: a comprehensive review of prion diseases

Prions are protein molecules that naturally occur in neural membranes (PrP). These components challenge our understanding due to their multiple functions, which are not yet fully unraveled. Structural changes can convert these molecules into a pathogenic form, known as PrPSc, causing insoluble aggregates that lead to neurodegenerative diseases, such as Creutzfeldt-Jakob disease, Fatal Familial Insomnia, and Gerstmann-Sträussler-Scheinker disease. These diseases have a long incubation period, approximately 30 years, without early detection by common diagnostic tests, highlighting the complexity of prion diseases. Once symptoms appear, prion diseases progress rapidly and are fatal, with an average life expectancy of just one year. Diagnosis is challenging; after symptom onset, a series of differential diagnoses is necessary, including electroencephalography, magnetic resonance imaging, computed tomography, and cerebrospinal fluid analysis, to exclude other degenerative diseases such as Alzheimer’s. Due to the rapid and devastating progression of symptoms, treatment is palliative, focused on providing comfort to the individual.