Kuru: The World’s Deadliest Disease with a Near 100% Fatality Rate
Kuru is categorized as a prion disease. It has a notorious reputation due to its near 100% fatality rate. As awareness surrounding Kuru has increased, its occurrence has become essentially non-existent. Primarily, it was confined to specific regions, such as New Guinea. But what is a prion disease, and how does one even contract a disease like this? Let’s delve into the intricacies of prion diseases, explore the different types of prion diseases, and get a better understanding of Kuru: its origins, transmission, and its decline in recent years.
What Are Prion Diseases?
Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of rare and fatal disorders affecting humans and animals. Unlike typical infectious diseases caused by bacteria or viruses, prion diseases are caused by misfolded proteins called prions. These prions induce abnormal folding in normal proteins, leading to a cascade of neurological degradation. These diseases are characterized by long incubation periods ranging from months to several years, followed by a rapid decline in cognitive and physical functions.
Types of Prion Diseases
Apart from Kuru, there are five other well-known prion diseases. These include:
- Creutzfeldt-Jakob Disease (CJD)
- variant Creutzfeldt-Jakob Disease (vCJD)
- fatal familial insomnia (FFI)
- Gerstmann-Sträussler-Scheinker syndrome (GSS)
- sporadic fatal insomnia (SFI)
Each disease varies in terms of symptoms, onset, and transmission. Creutzfeldt-Jakob Disease (CJD) is the most common type of prion disease, accounting for at least 85% of all cases. CJD can be sporadic, meaning it occurs randomly in individuals with no family history of the condition or hereditary, which means it’s passed down through families.
